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41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 4.0-75.0, Epilepsy Focal-Onset Seizure Before the start of the trial, to obtain the informed consent approved by the ethics committee voluntarily signed by each subject. For underage subjects, the informed consent jointly signed by the subjects themselves (≥10 years ) and their parents or legal guardian (in which the underage needs the signature with their parents or legal guardian, and the underage is defined as the subject under 18 years of age) Male and female, between the ages of 4 and 75 years Diagnostic of focal onset seizures (with or without focal to bilateral tonic clonic seizures) was based on the 2017 Classification of Epileptic Seizures from the International League Against Epilepsy (ILAE) In the 4 weeks before enrollment and during the baseline period, patients have been on only one stable dosage of antiepileptic drug and suitable for lacosamide add-on therapy according to their investigators criteria During the 8-week retrospective baseline period, patients must have had at least 4 focal onset seizures per 28 days on average Patients had received previous lacosamide treatment Female patients are pregnant, breast-feeding, and will not use contraception during the trial Patients had known allergies to lacosamide or any ingredients of the drug, or with allergic constitution Patients have a history of status epilepticus in the last 12 months History of chronic alcohol or drug abuse; 6.history of suicide attempt or suicidal ideation in the past 6 months Current use of Antidepressants, anxiolytics or antipsychotics Patients suffer from progressive diseases that affect the patient's brain and its function Sychogenic nonepileptic seizures Patients suffer from serious lung and blood system diseases, malignant tumor, lower immune function and psychosis Patients wil receive ketogenic diet therapy, or Four weeks before entering the screening period, patients used other drugs that may affect the absorption, distribution, metabolism and excretion of lacosamide, such as antipsychotics, monoamine oxidase inhibitors, barbiturates (except for combined use as anticonvulsant therapy), narcotic analgesics | 1 |
41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 18.0-65.0, Glioma Glioma Intracranial Gliomas Benign Brain Tumor Brain Cancer Motor Cortex; Lesion Age 18-65 years old 2. Ability to understand a written informed consent document, and the willingness to sign it 3. Radiographic evidence of likely low-grade glioma on MRI (i.e. non-enhancing) invading primary motor cortex in the non-dominant hemisphere. 4. Karnofsky performance status (KPS) ≥ 75 5. Normal or near normal motor strength (i.e., at least 3/5 in relevant areas) 6. Normal or near normal speech (Can consistently name at least 4/5 cards) 7. No medical contraindication to surgery 8. Free of other illness that may shorten life expectancy Presence of other malignancy not in remission 2. Evidence of bi-hemispheric or widespread tumor involvement 3. Likely candidate to receive GTR on initial resection 4. Medically high-risk surgical candidate 5. History of recent scalp or systemic infection 6. Presence of other implants or foreign bodies in the head 7. Inability to receive an MRI for any reason 8. Inability to receive cortical stimulation for any reason 9. Coagulation disorders and/or use of anti-thrombotic therapies 10. Platelet count < 50 11. Diathermy procedures 12. Electroconvulsive Therapy (ECT) 13. Transcranial Magnetic Stimulation (TMS) 14. Presence of implanted cardiac device (such as a pacemaker or defibrillator) 15. Pregnant women | 0 |
41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 9.0-999.0, Refractory Epilepsy years old or older Diagnosis of epilepsy with focal seizures with or without focal to bilateral tonic clonic seizures (International League Against Epilepsy classification). Diagnosis established by both clinical history and an electroencephalogram (EEG) consistent with focal seizures The subject has demonstrated compliance, according to medical records, on at least two (2) FDA-approved ASDs at a daily dose considered therapeutic for the patient's demographic according to package labeling, within approximately the last 3 years Seizure frequency ≥3 and ≤20 per month, over the past year Currently on 1-4 ASDs with no changes in antiepileptic drug doses in the 3 weeks prior to enrollment in the study and no planned dose changes during the trial. Changes after enrollment are permitted only if clinically necessary An MRI scan of the brain using 1.5 Tesla magnet, or greater, with T1, T2, and FLAIR sequences, performed within past 3 years and more recently than any craniotomy or skull burr hole procedure Seizure focus that allows design of an appropriate stimulation montage (determined by sponsor review of seizure history) Available seizure history and supporting data for review by a sponsor committee All female study subjects of child-bearing age are required to have a pregnancy test. Additionally, all females of childbearing potential will be required to use an effective method of birth control (defined as having a documented failure rate of <=1%; for women using enzyme-inducing ASDs hormonal contraceptives will not be considered as effective) Written informed consent obtained from study subject or subject's legal representative and ability for study subject to comply with the requirements of the study Presence of a condition or abnormality that in the opinion of the Investigator would compromise the safety of the subject or the integrity of the data Evidence for more than one seizure focus. (NOTE: For this study, a seizure focus is defined as a neocortical region confined to one hemisphere and either one lobe or on a junction of two adjacent lobes from which seizures arise, as documented by scalp or intracranial EEG, that is either supported or not refuted by MRI, and either supported or not refuted by clinical semiology) Seizure focus is one of: interhemispheric, cingulate, mesiotemporal, or orbitofrontal Seizure focus is hemispheric or poorly defined History of psychogenic nonepileptic seizures, or physiologic nonepileptic seizures and Non-epileptogenic events, including suspicion for or a significant history of syncope Seizures of generalized onset Status epilepticus in the last 12 months Presence of any disease, medical condition or physical condition that, in the opinion of the Investigator, may compromise interfere, limit, effect or reduce the subject's ability to complete a study of 24 weeks duration Presence of any disease, medical condition or physical condition that, in the opinion of the Investigator, may adversely impact the safety of the subject or the integrity of the data Damaged skin on scalp that may interfere with tDCS stimulation | 1 |
41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 18.0-999.0, Refractory Epilepsy adult patients treated between 2010 and 2019 for refractory epilepsy by DBS of the anterior nucleus os the thalamus using microendoscopy minimum follow-up of 1-year refractory epilepsy defined as failure of at least 3 antiepileptic drugs age < 18 years | 1 |
41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 18.0-999.0, Epilepsy Age ≥ 18 years Informed Consent as documented by signature Neurosurgical patient, i.e. patient under surgical care who will undergo one of the following specific procedures Step 1: Patient in the operating room undergoing clinically indicated brain surgery either for resection (e.g. tumor), cortical mapping (including awake) or intracranial electrode implantation Step 2: patients with pharmaco-resistant epilepsy hospitalized in the epilepsy monitoring unit (EMU) for the purpose of intracranial EEG monitoring Patients with increased risk of infection Pregnant or breast-feeding women Severe neuropsychiatric disorders Severe cognitive problems: the patients need to be able to understand instructions and provide consent Chronic headache disorders, such as migraine and related disorders, as well as trigeminal neuralgia Medical conditions contraindicating cranial surgery (e.g. skin disorders causing poor wound healing, blood or cardiac disorder requiring chronic anti-coagulation, osteomyelitis, active systemic infection, haemorrhagic disease, diabetes, hepatitis, any documented allergy to implantation material) Other chronic, unstable medical conditions that could interfere with subject participation Existing scalp lesions or skin breakdown Scalp infections Implanted neurosurgical devices that are incompatible with Epios leads, which may DBS leads | 1 |
41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 18.0-999.0, Tooth Wear Randomized Controlled Trial Vertical Dimension of Occlusion Composite Resins Generalized moderate to severe tooth wear with patient demand for treatment Full dental arches, one diastema due to one missing posterior tooth was allowed Estimated need for an increase in VDO of at least 3 mm in the first molar region Limited mouth opening (History of) Temporomandibular dysfunction Advanced periodontitis, deep caries lesions, or multiple large restorations including teeth with endodontic problems Patients with specific individual risk factors, such as parafunctional habits of grinding/clenching or patients with Gastro-Oesophageal Reflux Disease (GORD), were not excluded | 0 |
41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 18.0-999.0, Intractable Epilepsy Intractable focal epilepsy with undergoing pre-surgical evaluation intracerebral electrodes implantation in auditory cortices, temporal lobe, superior temporal gyrus, limbic areas, frontal lobe Given Consent to Deafness Pregnancy | 1 |
41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 18.0-100.0, Metastatic Squamous Cell Carcinoma of the Head and Neck (HNSCC) Subjects must be ≥18 years of age. 2. Subjects or their legal representative must be able to provide written informed consent to participate in the trial prior to the performance of any study-specific procedures. 3. Subjects must have histologically or cytologically confirmed HNSCC; eligible histologies SCC of the oropharynx, oral cavity, hypopharynx, and larynx. 4. Subjects must have PD-L1 IHC (including CPS score using an FDA approved test) testing completed within 6 months of screening or at screening. 5. Have measurable disease per 1.1 as assessed by the central imaging vendor or the local site investigator/radiology. Lesions situated in a previously irradiated area are considered measurable if progression has been demonstrated in such lesions. 6. Subjects must have locally advanced, recurrent or metastatic neoplastic disease that is not curable by currently available local therapies. 7. Subjects must have an Eastern Cooperative Oncology Group (ECOG) PS of 0 or1. 8. Subjects must have a life expectancy of at least 12 weeks. 9. Subjects must have adequate hematologic reserve based on the following: 1. ANC ≥1,500/μL 2. Platelet count >100,000/μL 3. Hemoglobin >9 g/dL 10. Subjects must have adequate hepatic function based on the following: 1. Total bilirubin <1.5 × upper limit of normal (ULN) 2. Alanine aminotransferase (ALT)/aspartate aminotransferase (AST) ≤2.5 x ULN (≤5 x ULN for subjects with known hepatic metastases). 11. Subjects must have adequate renal function based on the following: 1. Serum creatinine ≤1.5 × ULN; or 2. Calculated creatinine clearance of >30 mL/min. 12. Human immunodeficiency virus (HIV) infected subjects must be on antiretroviral therapy (ART) and have a well-controlled HIV infection/disease defined as: 1. Subjects on ART must have a CD4+ T cell count 350 cells/mm3 at time of screening 2. Subjects on ART must have achieved and maintained virologic suppression defined as confirmed HIV RNA level below 50 copies/mL or the lower limit of qualification (below the limit of detection) using the locally available assay at the time of screening and for at least 12 weeks prior to screening 3. Subjects on ART must have been on a stable regimen, without changes in drugs or dose modification, for at least 4 weeks prior to study entry (Day 1). 13. Subjects with oropharyngeal cancer must have archival tissue available for p16 testing or be willing to undergo pre-study biopsy to obtain tissue for p16 testing. 14. All subjects must have archival or recently obtained tissue available for biomarker analysis. 15. Female subjects of childbearing potential must have a negative pregnancy test within 72 hours of first dose of study treatment. Female subjects of childbearing potential must use a highly effective mode of contraception or abstain from heterosexual activity for the duration of the trial and for 120 days following the last dose of study medication. A female is NOT of childbearing potential if she has undergone bilateral salpingoophorectomy or is menopausal, defined as an absence of menses for 12 consecutive months. Male subjects must agree to use highly effective contraception Subjects with SCC of the nasopharynx. 2. Subjects who have received systemic treatment for recurrent or metastatic HNSCC; however, subjects who have received adjuvant systemic therapy or systemic therapy for locally advanced disease which was completed more than 6 months prior to study enrollment are eligible. 3. Subjects must have recovered from the effects of any prior radiation therapy or surgery. 4. Subjects who have received investigational therapy within 5 half-lives of the investigational agent or 4 weeks, whichever is shorter. 5. Subjects with primary immunodeficiency. 6. Subjects who require immunosuppressive therapy including, but not limited to, treatment with corticosteroids in pharmacologic doses (equivalent to ≥10 mg prednisone daily), cyclosporine, mycophenolate, azathioprine, methotrexate, adalimumab, infliximab, vedolizumab, tofacitinib, dupilumab, rituximab, etc. 7. Subjects with autoimmune conditions requiring treatment in the previous 2 years; however, subjects on replacement hormonal therapy alone for autoimmune endocrinopathies are eligible for enrollment. 8. Subjects with active central nervous system (CNS) metastases; however, subjects who have undergone radiation and/or surgery for the treatment of CNS metastases, who are neurologically stable and who are no longer taking pharmacologic doses of corticosteroids are eligible; subjects with leptomeningeal metastases are not eligible. 9. Has received prior radiotherapy within 2 weeks of start of study treatment. Subjects must have recovered from all radiation-related toxicities, not require corticosteroids, and not have had radiation pneumonitis. A 1-week washout is permitted for palliative radiation (≤2 weeks of radiotherapy) to non-CNS disease. 10. Subjects with a prior malignancy (other than the malignancy under study) in the 2 years prior to enrollment; however, subjects with curatively treated nonmelanoma skin cancers, intra-epithelial cervical neoplasia or in situ carcinoma of the breast are eligible for enrollment. 11. Subjects with prior allogenic transplants. 12. Has a history of (noninfectious) pneumonitis that required steroids or has current pneumonitis. 13. Subjects with an active infection requiring treatment with systemic antibiotics. 14. Subjects who are pregnant or lactating. 15. Subjects who have received treatment with a prior anti-PD-1 or anti-PD-L1, anti-CTLA-4, or anti-LAG3 agent or who have received prior treatment with pepinemab. 16. HIV-infected subjects with a history of Kaposi sarcoma and/or Multicentric Castleman Disease. 17. Subjects who are hepatitis B surface antigen positive are eligible if they have received hepatitis B virus (HBV) antiviral therapy for at least 4 weeks and have undetectable HBV viral load prior to enrollment. Note: Subjects should remain on antiviral therapy throughout study intervention and follow local guidelines for HBV antiviral therapy post completion of study intervention. Hepatitis B screening tests are not required unless: 1. Known history of HBV infection 2. As mandated by local health authority. 18. Subjects with a history of hepatitis C virus (HCV) infection are eligible if HCV viral load is undetectable at screening. Note: Subjects must have completed curative antiviral therapy at least 4 weeks prior to enrollment. Hepatitis C screening tests are not required unless: 1. Known history of HCV infection 2. As mandated by local health authority. 19. Subjects who have received a live vaccine within 30 days of study enrollment. 20. Current alcohol or drug abuse. 21. Subjects with any intercurrent medical condition where the known risks of participation in the trial outweigh any potential benefits; subjects with psychiatric or social circumstances that preclude responsible participation in the trial; subjects with severe nutritional deficiencies or marked hypoalbuminemia. 22. History of significant hypersensitivity, intolerance, or allergy to any drug compound, food, or other substance, unless approved by the investigator (or designee). 23. Inability to comply with visit schedule or other protocol requirements | 0 |
41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 18.0-65.0, Covid19 This study will volunteers household contact of COVID 19 confirmed cases who attend the triage clinic in the participating centers. Included persons should be > 18 years old and below 65 years, without any contraindications for Mefloquine usage • People with history of previous confirmed COVID-19 infection Pregnant and lactating females People with neuropsychiatric disorders: myasthenia gravis, epilepsy, psychotic disorder, schizophrenia, repeated episodes of anxiety, depression People with liver cirrhosis or raised liver enzymes People with arrhythmias or prolonged QT interval on EKG People with a history of Quinidine-Quinine analogs allergy | 0 |
41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 50.0-85.0, Alzheimer Disease Preclinical Alzheimer's Disease Prodromal Alzheimer's Disease Alzheimer's Disease (Incl Subtypes) Mild Cognitive Impairment Subjects are fully eligible for and have completed the (Amyloid Prediction in early stage Alzheimer's disease from acoustic and linguistic patterns of speech) study. (See https://clinicaltrials.gov/ct2/show/NCT04828122) Subject has access to audio or written recordings created by them that are available for collection Subject consents to take part in PAST extension study Subject hasn't completed the full visit day in the study | 0 |
41 year old man with history of severe intellectual disability, CHF, epilepsy presenting with facial twitching on the right and generalized shaking in at his NH which required 20 mg valium to cease seizure activity. Per outside medical patient was felt to have focal epilepsy with secondary generalization, likely due to anoxic brain injury at birth, and probably related to the atrophic changes seen on MRI, particularly in the left temporal lobe. The patient first developed seizures at age 13 found by family to have a generalized convulsion. He had a second seizure two years after his first episode. He was maintained on Dilantin and phenobarbital. The patient went 20 years without another seizure. He was recently tapered off Dilantin, and it was felt that perhaps this medication was necessary to maintain him seizure free. The patient had no further events during the hospital course and was back at his baseline at the time of discharge. Full EEG reports are pending at the time of dictation. Past Medical History: Epilepsy as above, CHF, depression | eligible ages (years): 20.0-999.0, Middle Cerebral Artery Stroke Patients with acute MCA infarct (NIHSS > 8) and confirmed by brain MRI studies (excluding lacunar infarct) during hospital admission Patients with prior clinical history of major stroke, significant head injury, brain tumor, major psychiatric illness, progressive neurodegenerative disorder, CNS infection, epilepsy, which might be precipitating to seizures or history of use of AEDs, including LEV or PER, during pregnancy or lactation, history of inadequate medical compliance, or any other significant major systemic disease with safety concern, determined by physician | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 12.0-999.0, Pneumonia, Pneumocystis Carinii HIV Infections Concurrent Medication: Allowed physiologic replacement doses of steroids Pneumocystis carinii pneumonia (PCP) in patient who is HIV positive by ELISA, HIV culture, or p24 antigenemia, or is a member of a risk group for HIV infection Failed at least 4 but not > 14 full days' therapy with either sulfamethoxazole/trimethoprim (SMX/TMP) or parenteral pentamidine. Patients must have received therapy with only one of the two conventional agents prior to enrollment Patients in whom an unequivocal diagnosis of this episode of PCP has been or can be established by morphologic confirmation of three or more typical Pneumocystis carinii organisms in sputum, bronchoalveolar lavage fluid, or lung tissue obtained by transbronchial or open lung biopsy within 15 days prior to study entry Patients in whom no significant improvement in arterial-alveolar oxygen pressure (defined as a decrease of at least 15mm Hg) is observed in the 24 hours prior to entry Patient is willing to have maximal medical support, including pressors, invasive monitoring, and/or mechanical ventilation, during at least the first 7 days of protocol therapy if such support is necessary. Continuation of maximal medical support beyond 7 days is at discretion of investigator and patient Patients with history of hypersensitivity less severe than type I may be enrolled if, in opinion of investigator, these adverse effects do not prohibit rechallenge with the drug. Prior Medication: Required At least 4 full days but no greater than 14 full days of parenteral and/or oral therapy with sulfamethoxazole/trimethoprim (SMX/TMP) or pentamidine. Allowed Zidovudine (AZT) Co-existing Condition: Excluded Patients with history of type I hypersensitivity (urticaria, angioedema, anaphylaxis), exfoliative dermatitis, or other life-threatening reaction secondary to trimetrexate, sulfamethoxazole/trimethoprim, or pentamidine Presence of any process that, in the opinion of investigator, would be adversely and seriously affected by steroid therapy Failure to meet criteria. Concurrent Medication: Excluded Zidovudine (AZT) Myelosuppressive agents Nephrotoxic agents AZT may be resumed at completion of study. Excluded Patients with history of type I hypersensitivity (urticaria, angioedema, anaphylaxis), exfoliative dermatitis, or other life-threatening reaction secondary to trimetrexate, sulfamethoxazole/trimethoprim, or pentamidine Presence of any process that, in the opinion of investigator, would be adversely and seriously affected by steroid therapy | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Pneumonia, Pneumocystis Carinii HIV Infections Patients must have HIV antibody positive CD4 cell counts less than 500/mm3 Adequate general health No significant deterioration in performance status within the past month Prior treatment with a stable regimen of antiretroviral medication for at least 4 weeks prior to study. Prior Medication: Required Stable regimen of antiretroviral medication for at least 4 weeks prior to study entry. Allowed Aerosolized pentamidine for PCP prophylaxis Co-existing Condition: Patients with the following symptoms or conditions are excluded Intercurrent infection Clinically significant abnormality on EKG Known hypersensitivity to quinolines Known hemoglobin M abnormality Known NADH methemoglobin reductase deficiency Positive test for G6PD deficiency Fever. Prior Medication: Excluded Other systemic medication (other than AZT, ddC, ddI, methadone, acyclovir, and NSAIDs) within 3 days prior to study entry | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 13.0-999.0, Sarcoma, Kaposi HIV Infections Concurrent Medication: Required Antiretroviral therapy during study treatment only in patients with CD4 count < 500 cells/mm3 (per 12/30/94 amendment). Allowed G-CSF for a second occurrence of grade 3 or 4 neutropenia (per 12/30/94 amendment) Nonsteroidal anti-inflammatory agents including acetaminophen for drug-related fevers Systemic steroids for no more than 1 week in any 30-day period PCP prophylaxis with TMP/SMX, dapsone, or inhaled pentamidine, if patient has a history of PCP or a CD4 count < 250 cells/mm3. Allowed only in patients with CD4 count < 100 cells/mm3 Maintenance doses of ganciclovir, pyrimethamine/sulfa and TMP/SMX for stable, well-controlled opportunistic infections Non-myelosuppressive treatment IND medications. Prior Medication: Required: PER 11/20/95 Stable dose of antiretroviral therapy required for at least 21 days prior to study entry for all patients. (Changed from Stable dose of antiretroviral therapy for at least 21 days prior to study entry in patients with CD4 count < 500 cells/mm3 (per 12/30/94 amendment). Patients must have Co-existing Condition: Patients with the following symptoms or conditions are excluded Other active malignancies (except basal cell carcinoma of the skin and in situ cervical cancer) Alteration in mental status that may prevent compliance Cardiac functional capacity of Class II or worse OR regional wall abnormalities or abnormal ejection fraction on two-dimensional echocardiogram, if performed. Concurrent Medication: Excluded Chemotherapy, interferons, or immune modulators for Kaposi's sarcoma Myelosuppressive agents such as induction doses of ganciclovir, Fansidar (pyrimethamine/sulfadoxine), or any other investigational drugs (with the exception of non-myelosuppressive treatment IND medications in specific patients) GM-CSF or erythropoietin (except for a second grade 3/4 neutropenia or anemia) G-CSF. Patients with the following prior conditions are excluded History of myocardial infarction or significant arrhythmias History of symptomatic hypoglycemia. Prior Medication: Excluded | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 13.0-999.0, Toxoplasmosis, Cerebral HIV Infections Concurrent Medication: Allowed Aerosolized pentamidine for PCP prophylaxis. PER 4/3/96 History of treatment limiting toxicity to pyrimethamine. Patients with a history of treatment limiting toxicity to both pyrimethamine and sulfonamides will be assigned to receive atovaquone plus clarithromycin. Patients must have Documented HIV infection or diagnosis of AIDS (except for CD4 count < 200 cells/mm3) Toxoplasmic encephalitis Ability to give informed consent or legal designee who could give consent. PER 4/3/96 NOTE A history of treatment limiting toxicity to both pyrimethamine and sulfonamides will result in the patient being enrolled in the atovaquone plus clarithromycin arm Co-existing Condition: Patients with the following symptoms or conditions are excluded Coma Opportunistic infection that requires either acute or maintenance treatment with disallowed medications Any infections or neoplasms of the central nervous system other than Toxoplasma, HIV encephalopathy, or syphilis Unable to take oral study drugs Malabsorption (i.e., three or more episodes of diarrhea per day that has caused >= 10 percent loss of body weight over the past 4 weeks) Positive CSF or serum for Cryptococcus antigen or culture (a positive serum antigen only is acceptable, provided patient received prior antifungal therapy and is on maintenance, and the likelihood of recurrence is low) Malignancy requiring use of cytotoxic chemotherapy Medical or social condition that would adversely affect study participation or compliance. Concurrent Medication: Excluded Trimethoprim-sulfamethoxazole | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, HIV Infections Pregnancy A woman may be eligible for this study if She is HIV-positive She is at least 34 weeks pregnant She has a history of at least 4 weeks of continuous oral ZDV during her current pregnancy and tolerated it well She has given consent for her newborn to participate in this study. (The father must also give consent if he is available after reasonable attempts to contact him. A woman under 18 needs the consent of a parent or legal guardian for her and her infant to participate.) A woman will not be eligible for this study if She is taking part in another study of HIV treatment during pregnancy Her infant has a life-threatening illness indicated in an ultrasound Her infant does not appear to be growing normally in the womb She has a cesarean section She has abnormal blood test results She has severe nausea, vomiting, or other problems of the stomach and intestines at the time of study entry She has an active opportunistic (AIDS-related) infection or other serious infection at the time of study entry The study staff cannot find a usable vein The study doctor feels that she cannot take drugs by mouth | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-0.25, HIV Infections Concurrent Medication: Allowed Recommended Standard immunizations. Should repeat MMR 3 months after discontinuing study Benadryl and/or aspirin Pneumocystis carinii pneumonia prophylaxis Systemic ketoconazole and acyclovir, or oral nystatin for acute therapy Aerosol ribavirin for short-term treatment of RSV. Concurrent Treatment: Allowed Blood transfusion. Patients must have the following Parent or guardian available to give written informed consent Protocol requires prior Institutional Review Board (IRB) approval before any subject is entered into study. Prior Medication: Allowed Co-existing Condition: Patients with the following conditions or symptoms are excluded Symptomatic of any class P-2 symptoms (except lymphadenopathy at time of study entry Presence of serious acute infection requiring parenteral treatment at time of study entry. Concurrent Medication: Excluded Prophylaxis for oral candidiasis or otitis media or other infections Immunoglobulin therapy (except single dose or for hypogammaglobulinemia) Ketoconazole, acyclovir, or nystatin for prophylaxis. Patients with the following are excluded Symptomatic of any class P-2 symptoms (except lymphadenopathy at time of study entry Presence of serious acute infection requiring parenteral treatment at time of study entry. Prior Medication: Excluded Antiretroviral treatment or experimental treatment within 2 weeks of entry | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 13.0-999.0, HIV Infections Histoplasmosis Concurrent Medication: Allowed Vincristine, vinblastine, bleomycin, or interferon for Kaposi's sarcoma Erythropoietin Didanosine by exemption for 10 patients Barbiturates Coumarin-type anticoagulants Oral contraceptives Digoxin Methadone Narcotics Co-existing Condition: Patients with the following conditions or symptoms are excluded Severely ill, or at risk of dying from histoplasmosis within the first week of treatment, as indicated by systolic blood pressure less than 90 mm Hg , or PO2 less than 60 Active CNS lesions, malignancies, or infections other than MAI Severe malabsorption syndrome (persistent diarrhea more than 4 weeks duration with at least 4 loose stools per day accompanied by a 10 percent or greater weight loss) Requiring cytotoxic therapy for malignancies Any systemic fungal infection other than histoplasmosis Systemic Mycobacterium avium intracellulare Receiving treatment for other acute opportunistic infections whose signs and symptoms have not yet resolved or stabilized History of allergy to or intolerance of imidazoles or azoles. Concurrent Medication: Excluded All other systemic antifungal agents | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-60.0, Pneumonia, Pneumocystis Carinii HIV Infections Patient must Have Pneumocystis carinii pneumonia (PCP) that has not been treated for current episode. PCP must be documented by observation of > 1 cluster of organisms in sputum, bronchial secretion, or lung tissue Have clinical symptoms of respiratory disease or radiologic abnormalities Patient cannot have significant emotional disorder. Concurrent Medication: Excluded Drugs likely to be bone marrow toxic Investigational drugs. Prior Medication: Excluded Three patients in each group cannot have had zidovudine (AZT) for at least 2 months prior to administration of trimetrexate | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 12.0-999.0, Pneumonia, Pneumocystis Carinii HIV Infections Concurrent Medication: Allowed Acetaminophen mg prescribed as necessary for temperature > 38.7 degrees C. Acetaminophen should not be prescribed as a standing order for more than 48 hours. Prior Medication: Allowed Zidovudine (AZT) as long as such therapy is suspended prior to randomization and not reinstituted until therapy for the acute episode is completed and the patient's white blood cell count is acceptable Other myelosuppressive therapies which may be handled in the same manner as AZT Prophylaxis for Pneumocystis carinii pneumonia (PCP) Unequivocal diagnosis of Pneumocystis carinii pneumonia (PCP) by morphologic confirmation of three or more typical P. carinii organisms in sputum, bronchoalveolar lavage fluid, or lung tissue obtained by transbronchial or open-lung biopsy within 3 days before or after randomization. If morphologic confirmation is not possible prior to therapy, patients may be randomized if the investigator believes there is a high suspicion of PCP based on clinical presentation. If morphologic diagnosis cannot be established within 6 days of randomization, the patient will be withdrawn from study therapy. Resting (A-a) DO2 < 30 torr on room air. Patient, parent, guardian, or person with power of attorney gives informed consent Co-existing Condition: Patients will be excluded for the following reasons History of Type I hypersensitivity (i.e., urticaria, angioedema, or anaphylaxis), exfoliative dermatitis, or other life-threatening reaction secondary to antibiotics containing sulfa, trimethoprim, or trimetrexate History of life-threatening pentamidine toxicity. Concurrent Medication: Excluded Other drugs for the treatment or prevention of AIDS or Pneumocystis carinii pneumonia (PCP) Disalcid Aspirin Acetaminophen q4h as a standing order for more than 48 hours. Prior Medication: Excluded within 14 days of study entry Systemic steroids exceeding physiological replacement Other investigational drugs including ganciclovir Excluded within 6 weeks of study entry | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 12.0-999.0, Pneumonia, Pneumocystis Carinii HIV Infections Concurrent Medication: Allowed Acetaminophen 650 mg prescribed as necessary for temperature > 38.7 degrees C. Acetaminophen q4h should not be prescribed as a standing order for more than 48 hours. Prior Medication: Allowed Zidovudine as long as such therapy is suspended prior to randomization and not reinstituted until therapy for the acute episode is completed Prophylaxis for Pneumocystis carinii pneumonia (PCP) Unequivocal diagnosis of Pneumocystis carinii pneumonia (PCP) by morphologic confirmation of three or more typical P. carinii organisms in sputum, bronchoalveolar lavage fluid, or lung tissue obtained by transbronchial or open-lung biopsy within 3 days before or after randomization. If morphologic confirmation is not possible prior to therapy, patients may be randomized if the investigator believes there is a high suspicion of PCP based on clinical presentation. If morphologic diagnosis cannot be established within 6 days of randomization, the patient will be withdrawn from study therapy Resting alveolar-arterial oxygen differences = or > 30 mm Hg on room air Patients with the following are excluded Inability to have alveolar blood gas analysis on room air Medically unable to receive a liter of intravenous fluid (5 percent dextrose in water) per 24 hours. This procedure is required in order to maintain blinding. Prior Medication: Excluded within 14 days of study entry Systemic steroids exceeding physiological replacement Other investigational drugs Excluded within 6 weeks of study entry Antiprotozoal regimen for this episode consisting of pentamidine, eflornithine, DFMO, or dapsone, for therapy of active Pneumocystis carinii pneumonia (PCP) History of Type I hypersensitivity (i.e., urticaria, angioedema, or anaphylaxis), exfoliative dermatitis, or other life-threatening reaction secondary to antibiotics containing sulfa, trimethoprim, or trimetrexate History of life-threatening pentamidine toxicity Requirement for treatment with agents that are known to be myelosuppressive or nephrotoxic during the period of acute Pneumocystis carinii pneumonia (PCP) therapy | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Inflammation Vasculitis Wegener's Granulomatosis Diagnosis: Wegener's granulomatosis. Age: 10-80 years. Qualifications to Prior documentation of vasculitis based on clinical characteristics and histopathological and/or angiographic evidence of vasculitis. Patients will be eligible for this study regardless of whether they are currently receiving immunosuppressive therapies. Failure to respond to prior therapy with other cytotoxic agents or toxicity from such agents, in the setting of persistent disease, will constitute one reason for for this study. In the absence of histopathological and/or angiographic evidence of vasculitis, patients with the following will also be eligible: A. Positive C-ANCA (done at the NIH), and B. Glomerulonephritis as evidenced by the presence of red blood cell casts and proteinuria or renal biopsy showing necrotizing glomerulonephritis in the absence of positive immunofluorescence for immunoglobulin and complement, and C. One or more of the following: Inflammatory sinusitis with histopathological evidence of granulomatous inflammation and negative special stains for mycobacteria and fungi. Sinusitis must be present for at least 3 months and have failed to respond to at least 2 weeks of antibiotic therapy directed against likely pathogens (H. influenza, S. pneumonia, and upper respiratory tract anaerobic bacteria); Pulmonary nodule or infiltrates in a patient in the absence of infection. Evidence of active disease as defined by a Vasculitis Disease Activity Index of greater than or equal to 3 (Appendix I) or if begun on immunosuppressive therapy at an outside institution, a history of a Vasculitis Disease Activity Index greater than or equal to 3 during the past 6 months Evidence of infection by gram stain and/or culture specimens. In those instances in which infection cannot be ruled out by gram stain and culture of secretions or collections of fluid in involved organs, it may be necessary to obtain a biopsy of the affected tissue for microbiological and histopathological studies. Recent (within four weeks) increase in GC or cytotoxic drug therapy. Patients who are pregnant or nursing infants will not be eligible. Fertile women should have a negative pregnancy test within one week prior to study entry and should be using effective means of birth control. Processes that would predispose to enhanced risk of MTX toxicity: acute or chronic liver disease, alcohol abuse (greater than 14 oz of 100 proof liquor or equivalent per week), active peptic ulcer disease, and inability to comply with study guidelines. Serological evidence of infection with human immunodeficiency virus (a serological determination will be performed within two weeks of study entry) | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Dermatomyositis Inclusion Body Myositis Polymyositis Selected patients should have PM, IBM or DM. Specifically they should have a) proximal muscle weakness; b) no evidence of clinical, histological or family history of another neuromuscular illness; c) elevation of muscle enzymes during the course of the disease; d) typical skin rash in case of DM; and e) diagnostic muscle biopsy. Suitable candidates for IVIg should be patients with active, bonefide disease who: 1. have been treated with steroids but had: a) no response or incomplete response (as defined by continued muscle weakness) to high-dose therapy or b) a good response to steroids but inability to taper the dose without a flare of disease activity or c) unacceptable steroid side effects such as gastrointestinal hemorrhages, osteonecrosis, hyperglycemia, extreme weight gain etc., and 2. have been treated with one immunosuppressive drug (such as azathioprine, Methotrexate, Cyclophosphamide, Cyclosporine) but without benefit or with unacceptable side effects Pregnant or nursing women (confirmed by a screening pregnancy test). Critically ill patients such as those requiring intravenous pressors for maintenance of cardiac output due to severe cardiomyopathy, patients with respiratory insufficiency and patients with severe muscle weakness requiring help for basic self care. Children below age 18. Patients with severe renal or hepatic disease, severe COPD or coronary artery disease or other systemic medical problems often seen when PM or DM is associated with severe cases of lupus, rheumatoid arthritis or scleroderma. Patients with known allergic reaction to IVIg. Serum IgA less than 11mg/dl | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Mastocytosis Subjects children and adults from birth to 80 years of age. Participants must have histologic evidence of an increased mast cell number in at least one organ system. Must be willing to be seen at the NIH according to protocol guidelines. Routine medical care must be available through their referral physician. Patients with mastocytosis now followed at the NIH on protocol 88-I-0190 will be continued on this study | 2 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Sjogren's Syndrome Xerostomia Primary Sjogren's Syndrome; symptoms of dry eyes and dry mouth; 6 week period off disease modifying agents, such as antimalarials or steroids. No males. No females with childbearing potential. No patients with hypersensitivity to thalidomide. No confounding medical illness or abnormal laboratory test that in the judgment of the investigators would pose added risk for study participants | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 8.0-99.0, Pulmonary Disease Patients 8 years old and older with signs or symptoms of pulmonary disease of medical interest to the professional staff of CPB will be eligible for participation in this protocol. Only standard diagnostic procedures and conventional therapy will be performed. Patients will not be subjected to any research procedures. This protocol does not commit the NIH to medical or surgical treatment of protocol participants after discharge. Patients will be discharged to the referring physician. Consenting to pregnancy testing in minors of childbearing age: We will inform the minor during the assent process that for safety, we need to do a pregnancy test. She will also be told that if it is positive, we will counsel her and help her tell her parents. If the minor does not want to proceed she will be advised not to sign assent and her enrollment on this training protocol will end Patients without symptoms of pulmonary disease will be excluded from this protocol | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-70.0, Mastocytosis Healthy Volunteer Healthy Volunteers must: 1. Be 18-70 years of age 2. Be healthy 3. Have adequate peripheral venous access 4. Have normal renal function (creatinine less than or equal to 1.5mg/dL; less than or equal to 1 plus proteinuria) 5. Have normal hepatic function (bilirubin less than or equal to 1.5 mg/dL) 6. Have normal hematologic function (WBC greater than or equal to 3000/mm(3); granulocytes greater than or equal to 1500/mm(3) ; platelets greater than or equal to 175,000; hemoglobin greater than or equal to 12.5 g/dL) Patients must: 1. Be 18-70 years of age 2. Have mast cell hyperplasia compatible with a diagnosis of systemic mastocytosis (applicable to systemic mastocytosis patients only) or other allergic, hematologic, or immunologic condition 3. Have adequate peripheral venous access or be willing to have a central line placed. 4. First be admitted as inpatients under an existing NIH protocol 5. Have preserved renal function (creatinine less than or equal to 2 mg/dL; less than or equal to 2 plus proteinuria) 6. Have preserved hepatic function (bilirubin less than or equal to 1.5 mg/dL) 7. Have preserved hematologic function (WBC greater than or equal to 3000/mm(3); granulocytes greater than or equal to 1500/mm(3) ; platelets greater than or equal to 175,000; hemoglobin greater than or equal to 12.5 g/dL) All female subjects of childbearing potential: 1. May be enrolled if using effective contraception 2. Have a negative serum or urine pregnancy test determined within 72 hours before beginning Plerixafor or G-CSF administration All subjects must not meet any of the following Healthy Volunteers and patients must not: 1. Have active bacterial, fungal or viral infections 2. Have viral screens positive for HIV or hepatitis B or C 3. Be pregnant or lactating 4. Have a history of autoimmune disease such as rheumatoid arthritis, vasculitis, pyoderma gangrenosum or similar disorder 5. Have any condition, which in the judgment of the investigator, might place the subject at undue risk Healthy Volunteers with any of the following will be excluded: 1. Splenomegaly, pulmonary fibrosis and other related conditions 2. Use of any investigative drugs within the past 12 months 3. Have a significant coagulation disorder Systemic Mastocytosis and Mast Cell Related Condition Patients with any of the following will be excluded: 1. Patients taking any other growth factors, cytokines or investigative drugs 2. Patients who are hemodynamically unstable (blood pressure systolic of lower than 105 or diastolic lower than 65) | 1 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Atopic Dermatitis Healthy Mastocytosis General: Age equal to or greater than 18. Access to a primary medical care provider outside of the NIH. Able to give informed consent. No history of malignancy or autoimmune disease such as rheumatoid arthritis, vasculitis, pyoderma gangrenosum, psoriasis. No use of systemic corticosteroids within the past month. No use of local corticosteroids at the proposed blistering site within the past month. No evidence of current acute infection. INR less than or equal to 1.5, PTT less than or equal to 40, platelet count greater than or equal to 100,000/mm(3). No personal or family history of keloid formation. Blood glucose less than or equal to 160. No use of any investigative drugs within the past month. No allergy to lidocaine. Healthy volunteers must not have a history of atopic dermatitis, mastocytosis or chronic urticaria. Mastocytosis: Histologic evidence of mast cell hyperplasia in at least one organ system. Atopic Dermatitis: Must have at least 3 major and 3 minor criteria. No history of mastocytosis | 1 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Vasculitis Wegener's Granulomatosis Documentation of Wegener's Granulomatosis (WG) based on clinical characteristics and histopathological evidence of vasculitis. Patient with a positive C or P-ANCA and glomerulonephritis as evidenced by the presence of red blood cell casts and proteinuria or renal biopsy showing necrotizing glomerulonephritis in the absence of positive immunofluorescence for immunoglobulin and complement will also be eligible. Patients must be of the ages of 18-80 years. Patients on the CYC to MTX protocol (#95-I-0091) who experience a relapse of disease while on MTX maintenance therapy. Relapse is defined by a Vasculitis Disease Activity Index of greater than or equal to 3. Patients from outside the NIH will also be eligible if they have been treated with a CYC to MTX regimen identical to that used in #95-I-0091 and experience a relapse of disease while on MTX maintenance therapy. If treatment for this relapse has already been commenced at the outside institution with daily CYC and glucocorticoid, patients will still be eligible if there is a history of a Vasculitis Disease Activity Index greater than or equal to 3 at the time of CYC and glucocorticoid initiation. Patients who experience a relapse of disease after MTX has been stopped or while tapering the MTX dose (following 2 years of maintenance therapy) will not be eligible. Patients with active disease who have a contraindication to MTX therapy will be eligible. Evidence of active disease as defined by a Vasculitis Disease Activity Index of greater than or equal to 3. Patients with inactive disease who have a contraindication to CYC and. Evidence of active disease as defined by a Vasculitis Disease Activity Index of greater than or equal to 3. Patients with inactive disease on MTX while on the CYC to MTX protocol (95-I-0091) or the MTX protocol (90-I-0086) who develop an contraindication necessitating discontinuation of MTX. Patients from outside the NIH will also be eligible if they similarly develop a contraindication to MTX while on treatment. Patients with inactive disease on the CYC protocol (#76-I-0041 or 76-I-0042) who develop a contraindication necessitating CYC discontinuation and also have a contraindication to receiving MTX. Patients from outside the NIH will also be eligible if they similarly develop a contraindication to CYC while on treatment and cannot receive MTX. Patients with inactive disease who are receiving treatment with CYC and prednisone in a manner similar to #76-I-0042 will be eligible if they have a contraindication to receiving MTX and have been in remission for less 3 months Evidence of active infection which, in the judgment of the investigator, is of greater danger to the patient than the underlying vasculitis. In those instances in which infection cannot be ruled out by gram stain and culture of secretions or collections of fluid in involved organs, it may be necessary to obtain a biopsy of the affected tissue for microbiological and histopathological studies. Patients who are pregnant or who are nursing infants will not be eligible. Fertile women must have a negative pregnancy test within one week prior to study entry and must be using an effective means of birth control. Patients with active disease who are eligible for the CYC to MTX protocol (#95-I-0091) or the MTX protocol (#90-I-0086). Active peptic ulcer disease. Serological evidence of infection with human immunodeficiency virus. A serological determination will be performed within two weeks of beginning study participation. Inability to comply with study guidelines. Creatinine clearance less than 25ml/min | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Sjogren's Syndrome Female gender only, ages 18 to 75. Patients should have documented primary Sjogren's syndrome as determined by protocol 84-D-0056. The diagnosis of primary Sjogren's syndrome will be based on the presence of all three of the following: Salivary gland abnormalities; Lacrimal gland abnormalities; Serologic abnormalities. Controls should have no signs or symptoms of Sjogren's syndrome, and negative serologic testing reconfirmed within 2 weeks of the OGTT. Willingness to: a) participate in phase I and II of the protocol; b) follow the guidelines set to prepare for the OGTT; and c) modify current medical therapy if necessary. Must be able to comply with protocol procedures. No patients with "incomplete" Sjogren's syndrome, i.e., with less than three positive findings as described above. No presence of physical or mental conditions that may interfere with the protocol. These the following diseases and conditions: (Present at time of phase I/II): significant disruption of sleep-wake pattern (i.e., less than 4 hours of continuous sleep, on the night before the OGTT only), BMI less than 18 or equal to/greater than 30, anemia with Hgb less than 10 gm/dl, use of tobacco or alcohol; (Within the past 6 weeks): acute weight change (greater than 5%), use of contraceptives -BCP-, Depoprovera or Norplant), irregular menstrual cycle, lactation, blood drawing in excess of 50 ml, use of recreational drugs, modification of estrogen replacement therapy; (Within the past 6 months): chronic pattern of weight change (greater than 10%), eating disorders, uncontrolled hypo/hyperthyroidism, breast cancer, lymphoma or other malignancy, pregnancy, treatment for depression, insulinoma, VIPoma, pheochromocytoma, atrophic gastritis, active tuberculosis or treatment for it; (A history of): HIV+, sarcoidosis, secondary Sjogren's syndrome, bleeding diathesis, organ transplant, severe neuroendocrine, renal, cardiovascular, pulmonary or gastrointestinal disease (e.g., renal insufficiency, unstable angina, heart failure, severe emphysema, Crohn's disease), diabetes mellitus, acromegaly and mental retardation. No contraindications to OGTT. These Hospital inpatient status, acute illness, immobilization, starvation, severe emotional distress within one week of the OGTT; Low carbohydrate diet (less than 150 g/day) for 3 days preceding the OGTT; Pregnancy; Therapy which impairs glucose tolerance: e.g., niacin, thiazide diuretics, phenytoin, excess thyroxine or psychotropic drugs within 1 month of phase I, oral contraceptives within 6 weeks of phase I, glucocorticoid treatment within the past 6 months (or one year if treatment lasted over 2 weeks), Beta blockers/agonists (e.g., terbutaline) within 2 days of phase I; Diabetes mellitus; Glucose intolerance. Subjects may not take medications that can affect somatostatin levels, such as antidepressants, benzodiazepines, neuromodulators (e.g., cholinergic, alpha/beta adrenergic), oral contraceptives (BCP), steroid hormones (with the exception of estrogen replacement therapy -ERT-, for which subjects will be matched), immunomodulators, anticonvulsants (e.g., carbamazepine), cimetidine -Tagamet® (Registered Trademark)-, herbal remedies (because of their variable substance content). Subjects may not take drugs affecting gastrin secretion: antacids (e.g., Maalox® (Registered Trademark), Mylanta® (Registered Trademark), H(2) receptor antagonists (e.g., famotidine -Pepcid® (Registered Trademark)-, ranitidine -Zantac® (Registered Trademark)-), cathecolamines, atropine, haloperidol. To become eligible, subjects should be able to safely discontinue these medications at least one month prior to phase I. Antacids, H(2) antagonists and neuromodulators (e.g., Salagen) may be discontinued 2 days prior to phase I. Patients cannot have take experimental drugs within 1 month of the beginning of the protocol | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Pneumonia, Pneumocystis Carinii HIV Infections Patients must have the following Clinical diagnosis of acute mild to moderate Pneumocystis carinii pneumonia Dose-limiting intolerance to TMP / SMX or inadequate response to TMP / SMX Willingness and ability to give informed consent. The clinical condition of the patient and appropriate physiologic should be used in evaluating patients for therapy with 566C80. Patients who are rapidly progressing in disease severity or have severe disease as evidenced by sustained tachypnea (e.g., sustained respiratory rate > 30 breaths/minute), rapid deterioration in (A-a)DO2 and chest radiograph may not be appropriate candidates for oral therapy, including 566C80. Therapy with parenteral pentamidine should be undertaken in these cases. Treatment with IV trimetrexate or primaquine / clindamycin should be considered for these patients unless the patient is known to have dose-limiting intolerance to these agents. Patients with severe PCP who are intolerant and/or unresponsive to therapy with TMP / SMX and parenteral pentamidine may enroll in the 566C80 open-label compassionate plea protocol Patients with the following prior conditions are excluded: History of serious or dose-limiting adverse experience during previous 566C80 therapy, thought to be attributable to the drug | 1 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Graft Versus Host Disease Histologically or clinically proven chronic graft versus host disease (GVHD) that has failed to respond to at least 1 month of treatment with the following: Steroids (greater than 0.5 mg/kg/day) AND Cyclosporine or a cytotoxic agent (azathioprine or mercaptopurine) OR Other experimental treatment (such as chloroquine) All allogeneic bone marrow or peripheral blood stem cell transplantation patients eligible regardless of underlying disease for which transplantation was performed if: At least 45 days since prior transplantation No relapse of underlying disease No loss of donor hematopoiesis Patients with a rapid deterioration of GVHD that is considered life threatening if not controlled are eligible after receiving high dose steroids (greater than 1 mg/kg/day) for at least 10 days Age: Not specified Performance status: Not specified Life expectancy: Not specified Hematopoietic: Absolute neutrophil count at least 1,300/mm3 Platelet count at least 75,000/mm3 Hepatic: Not specified Renal: Creatinine no greater than 2 mg/dL Other: Not pregnant or nursing Negative pregnancy test Fertile patients must use effective contraception PRIOR Biologic therapy: See Disease Characteristics Chemotherapy: No other concurrent cytotoxic drugs Endocrine therapy: Concurrent steroids allowed but must be tapered to less than 0.5 mg/kg/day of prednisone or equivalent prior to starting study drug (if symptomatic flare develops during taper, patients may continue on the lowest dose thought to produce stabilization) Radiotherapy: Not specified Surgery: Not specified Other: Concurrent cyclosporine and other nonmyelosuppressive drugs allowed No concurrent myelosuppressive agents (azathioprine, mercaptopurine) | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 8.0-999.0, Angioedema ENTRY --Disease Characteristics-- Confirmed diagnosis of hereditary angioedema (HAE) based upon C4 antigenic level, positive family history, and C1 inhibitor (C1INH) antigenic level (Type I HAE) or functional level (Type II HAE) Functional C1 within normal levels if diagnosis of acquired C1INH is possible Negative family history will not as long as patient meets all other has normal C1 level, and has no evidence of acquired C1INH deficiency History of symptoms of HAE including episodic swelling, abdominal pain, and/or respiratory difficulty Experiencing a minimum of 1-2 HAE episodes within 6 weeks --Prior/Concurrent Therapy-- Endocrine therapy: Anabolic steroids allowed as long as dose has not been changed in at least 30 days Other: No concurrent and at least 1 week since prior aspirin, nonsteroidal antiinflammatory agents, dipyridamole, Coumadin-like drugs, or any other medication having an effect on coagulation or platelets --Patient Characteristics-- Hematopoietic: No clinically significant history of hematologic disease Hepatic: No clinically significant history of hepatic disease | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 2.0-80.0, Mastocytosis Ages greater than or equal to 2 to 80. Wt greater than 40 kg. Patients with systemic mastocytosis proven by a bone marrow biopsy and one of the following: Category I disease with poor prognosis secondary to extensive bone marrow involvement of mast cells (i.e. greater than 50 percent of the bone marrow cavity replaced by mast cells) and evidence of a bone marrow failure state defined as presence of one of the following for greater than or equal to 2 months Neutrophil count less than 500/ mm3 Platelet count less than 20,000/mm3 Dependency on transfusion of RBC or platelets. Category II disease with an associated hematologic disorder Myelodysplastic syndromes Myeloproliferative states except essential thrombocytosis or polycythemia vera Increased blasts in peripheral blood or bone marrow greater than 10 percent Bone marrow failure states as described above. Category III disease with enlarged peripheral lymph nodes with histopathological evidence of the replacement of the entire lymph node architecture with mast cells and/or accompanying peripheral eosinophilia. We also consider aggressive systemic mastocytosis with local tissue invasion as category III disease. Category IV disease with mast cell leukemia, characterized by the presence of malignant mast cells in the peripheral blood, which constitute greater than 10 percent of the nucleated cells. No major organ dysfunction precluding transplantation. DLCO greater than or equal to 65 percent predicted. Left ventricular ejection fraction of greater than or equal to 40%. ECOG performance status of 0 or 1. Life expectancy of more than 3 months. HLA-identical sibling available as donor. Patients or their parent(s)/responsible guardian(s) must be able to comprehend the investigational nature of the study and be willing to sign an informed consent. DONOR HLA identical family donor, (only HLA identical). Weight greater than or equal to 18 kg. Age less than or equal to 80 years old. Informed consent given (Any of the following) Pregnant or lactating Age less than 2 or age greater than 80 years. Weight less than or equal to 40 kg. ECOG performance status of 2 or more. Psychiatric disorder or mental deficiency severe as to make compliance with the BMT treatment unlikely, and making informed consent impossible. Major anticipated illness or organ failure incompatible with survival from PBSC transplant. DLCO less than 65 percent predicted. Left ventricular ejection fraction less than 40%. Baseline GFR less than 50ml/min: measured or calculated by the following formula: GFR = {(140-age) x body weight in kg}/72 x serum creatinine (for females, multiply by 0.85). Serum bilirubin greater than 4 mg/dl, transaminases greater than 5 times the upper limit of normal. Other malignant diseases liable to relapse or progress within 5 years. Life expectancy of less than 3 months DONOR (Any of the following) Pregnant or lactating. Donor unfit to receive G-CSF and undergo apheresis (Uncontrolled hypertension, history of congestive heart failure or unstable angina, thrombocytopenia). HIV positive. Donors who are positive for HBV, HCV or HTLV-1 may be used if the risk-benefit ratio is considered acceptable by the patient and investigator. Weight greater than 18 kg. Age greater than 2 or less than 80 years | 1 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 13.0-999.0, Lupus Erythematosus, Systemic A diagnosis of systemic lupus erythematosus | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Chronic Granulomatous Disease The following conditions must be met before a patient may be enrolled in the study: Patients ages 2 to 17 years; minimum weight of 12 kg. DHR proven chronic granulomatous disease with gp91 phox or p22 phox deficiency. History of at least one life-threatening infection (defined as any infection requiring treatment with intravenous antibiotic therapy) or a family member with CGD and a history of life-threatening infection. Free of active infection. Patients with consenting HLA-matched related donors that meet donor selection (test patients), or patients without an eligible HLA-matched related donor (control patients). Patients with eligible donors who choose not to undergo stem cell transplantation or patients with eligible but non-consenting donors may be enrolled in the control arm of the study. Patients enrolled in the control arm who complete a 24 month follow-up period may enroll in the transplant arm should an eligible, consenting donor become available. Patients with adequate organ function as measured by: Cardiac: Asymptomatic or, if symptomatic, then left ventricular ejection fraction at rest must be greater than 35%. Hepatic: SGOT within 4 times normal range and total bilirubin less than 2mg/dL. Renal: Creatinine clearance greater than or equal to 50 mL/min/ 1.73m(2). A maximum age adjusted serum creatinine will be used for patients who are unable to provide an accurate 24 hour urine collection. For children less than or equal to 5 years of age, the maximum serum creatinine (mg/dl) is 0.8; for children whose age is greater than 5 to less than or equal to 10, the maximum serum creatinine (mg/dl) is 1.0; for children whose age is greater than 10 to less than or equal to age 15, the maximum serum creatinine (mg/dl) is 1.2; and for children whose age is greater than 15, the maximum serum creatinine (mg/dl) is 1.5. Pulmonary: DLCO (diffusion capacity) and FEV1 greater than 45 percent of predicted (corrected for hemoglobin). Minors in whom pulmonary function tests are not possible will be evaluated for significant pulmonary dysfunction by a pulmonary consultant. Written informed consent/assent conforming to institutional guidelines obtained from patient and parent. Absence of co-existing medical problems that would significantly increase the risk of a transplant procedure in the judgment of the principal investigator Any one of the following conditions eliminates a patient from participating in this protocol: Female patients who are pregnant or lactating. ECOG performance status of 2 or more or less than 50 percent on the Lansky scale for ages 0-10. Seropositivity for HIV due to excessive risk of infection and neurotoxicity of antiretroviral medications. Evidence of rapid deterioration due to progressive infection and/or organ damage. Malignant diseases liable to relapse or progress within 5 years. DONOR Donors must be fit to receive G-CSF and give peripheral blood stem cells (normal blood count, normotensive, no history of stroke, no history of severe heart disease, greater than 12kg). Related to the patient and HLA-phenotypically identical with the patient for HLA-A, B and DRB1 alleles. Matching assessed minimally by serology for Class I and DNA typing for Class II antigens. Female x-linked CGD carriers must have greater than 30 percent normal neutrophils. If donor is a sibling who is a minor, he/ she is the oldest eligible sibling and no adult siblings are eligible donors. Written informed consent from donor. Donors who are minors will be evaluated by a social worker, psychologist or psychiatrist prior to the assent process to determine willingness to participate. If willingness to participate has been confirmed, informed consent will be obtained from adult parent or legal guardian. Informed assent will be obtained from minor donor in the presence of a third party who will assess comprehension and voluntary participation | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Recurrent Melanoma Stage IV Melanoma Histologically or cytologically confirmed stage IV melanoma At least 1 measurable lesion Greater than 20 mm by conventional techniques Greater than 10 mm by spiral CT scan Known brain metastases allowed if all of the following are met Radiologically stable for at least 6 weeks after completion of whole brain radiotherapy Stable at time of study No mass effect present radiologically No concurrent steroids to control symptoms of brain metastases Performance status | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 2.0-80.0, Mastocytosis Monoclonal Bone Marrow Tryptase Two to 80 years of age. Histologic evidence of increased mast cell number by bone marrow and/or skin biopsy or documentation of mastocytosis in the skin supported with a photograph of diagnostic skin lesion Must be under the care of a primary care physician to be enrolled. Ability to provide informed consent Anemia with hemoglobin less than 8 g/dL, hematocrit less than 24. Any condition that in the opinion of the investigator contraindicates participation in this study. Two to 80 years of age. A biological relative without the diagnosis of mastocytosis by skin examination or histologic evidence in a skin or bone marrow biopsy Subject has a primary medical care provider outside the NIH Ability to provide informed consent Any condition that in the opinion of the investigator contraindicates participation in this study | 2 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-45.0, Depression Mothers and infants must meet certain to be included in the study. Selection are: maternal age between 20 and 45 years, ability to read and write in English, normal pregnancy/delivery status without birth complications, term birth (+/ days from due date), and singleton status of the child. Approximately equal numbers of male and female infants will be recruited, and to the extent possible, the two groups of depressed and nondepressed mothers will be matched on ethnic composition. Participants will be seen at 5, 12, and 24 months None | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 2.0-100.0, Eosinophilic Myeloid Neoplasm Hypereosinophilic Syndrome All subjects must meet the established diagnostic for hypereosinophilic syndrome: eosinophilia greater than 1,500/mm(3) on two occasions, no secondary etiology for the eosinophilia despite careful clinical evaluation, and evidence of end organ damage (histologic evidence of tissue infiltration by eosinophils and/or objective evidence of clinical pathology in any organ system that is temporally associated with eosinophilia and not clearly attributable to another cause). All subjects must fit one of the following three categories: 1. refractory to or intolerant of steroids 2. presence of FIP1L1/PDGFRA by RT-PCR 3. presence of greater than or equal to 4 of the following laboratory suggestive of a myeloid disorder: i. dysplastic eosinophils on peripheral smear ii. serum B12 level greater than or equal to 1000 pg/ml iii. serum tryptase level greater than or equal to 12 iv. anemia and/or thrombocytopenia v. bone marrow cellularity greater than 80% with left shift in maturation vi. dysplastic (spindle-shaped) mast cells on bone marrow biopsy vii. evidence of fibrosis on bone marrow biopsy viii. dysplastic megakaryocytes on bone marrow biopsy 3. All subjects must be at least 2 years of age. 4. Negative serum beta-hCG within 24 hours prior to drug administration for women of childbearing potential to early pregnancy. 5. All subjects (men and women) must agree to practice abstinence or effective contraception during administration of imatinib mesylate or ruxolitinib and for 6 months after discontinuation of drug. Of note, failure of the standard chemotherapeutic agents (steroids, hydroxyurea, and interferon alpha) will not be a prerequisite for participation in this protocol for the following reasons. 1) There is no approved therapy for HES. 2) steroid therapy in the myeloid subset of HES patients is generally ineffective. 3) Although hydroxyurea and interferon alpha are initially effective in most cases, a majority of patients become refractory to or intolerant of these agents within a relatively short period of time (less than 1 year). 4) Data from other myeloid neoplasms and disorders, including CML, suggest that interferon, imatinib mesylate, and ruxolitinib, but not hydroxyurea, are associated with cytogenetic remission. 5) The reported incidence and severity of side effects from imatinib mesylate in patients with CML and ruxolitinib in myelofibrosis and polycythemia vera appear comparable to (or less than) those associated with interferon alpha. Subjects who meet but are already receiving imatinib, may be enrolled in the dose de-escalation portion of the study at the investigator s discretion. Although a private physician is not required for in the study, it is strongly recommended that all subjects have a physician outside the NIH for routine medical care and emergencies Pregnancy or nursing women 2. HIV positivity or other known immunodeficiency 3. D816V KIT-positive systemic mastocytosis 4. Absolute neutrophil count less than 1000/mm(3) or platelet count less than 10,000/mm(3) or less than 50,000/m(3) with clinical evidence of bleeding. 5. Elevated transaminases (greater than 5 times the upper limit of normal) or elevated bilirubin (greater than 3 times the upper limit of normal) 6. Any condition that, in the investigator s opinion, places the patient at undue risk by participating in the study 7. Evidence of B cell clonality by PCR or flow cytometry ( for ruxolitinib only) | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Mastocytosis Anaphylaxis FOR WITH Age equal to or greater than 18 years. Diagnosis of mastocytosis established by presence of typical urticaria pigmentosa skin lesions or a bone marrow biopsy. Letter of referral from prospective study participant's local doctor. Ability to give informed consent FOR WITH Age less than 18 years. Lack of a referral physician. Presence of co-morbid conditions which, in the judgment of the investigator or the referring physician, may put the patient at undue risk for travel (such as an acute infection, severe thrombocytopenia). Inability to provide informed consent. Inability or refusal to undergo a bone marrow biopsy and aspirate. Known allergy to latex or Lidocaine. FOR WITH OR Age equal to or greater than 18 years. Presence of flushing or anaphylaxis with negative workup for known causes such as carcinoid syndrome, pheochromocytoma, food allergy. Ability to give informed consent FOR WITH OR Same as for patients with mastocytosis. Known cause for anaphylaxis or flushing. Frequent life-threatening anaphylactic episodes: History of 6 or more separate attacks resulting in ER visits in the 6 months preceding the referral. FOR To have a first-degree relative accepted to the protocol as a patient with unexplained anaphylaxis, flushing or mastocytosis. Ability to give informed consent. For minors, ability of the parent to give informed consent. There are no age restrictions | 1 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-21.0, Mastocytosis Children with tissue-diagnosed pediatric-onset mastocytosis formerly enrolled in protocols 90-I-0120, and 93-I-0136 or per physician referral with more severe disease as indicated by one of the following parameters Hepatomegaly or splenomegaly Diffuse cutaneous mastocytosis History of gastrointestinal bleeding or peptic ulcer disease Bone marrow biopsy with abnormal numbers or shaped mast cells or abnormal flow cytometry Serum tryptase greater than 20ng/ml Hematologic abnormalities such as an increase WBC, thrombocytosis, and/or an increase in PT and/or PTT Age birth to 21.0 years of age at the time of entry into the protocol Diagnosis of mastocytosis by skin examination or histologic evidence in a skin or bone marrow biopsy Subject has a primary medical care provider outside the NIH Age greater than or equal to 21.0 years No primary care physician Has AIDS or is HIV Positive | 1 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Alcohol Consumption Subjects who are included: 1. will be between the ages of 21 and 25. 2. will have a score on the telephone administered SRE at least one standard deviation above the mean, in the case of subjects to be evaluated further as potential Low-Response group participants. 3. will have a score on the telephone administered SRE at least one standard deviation below the mean, in the case of persons to be evaluated further as potential High Responders. 4. will be willing to undergo a complete medical and psychiatric evaluation, as described in NIAAA protocol 98-AA-0009. 5. will be willing to undergo indicated laboratory and EKG examination, as described in NIAAA protocol 98-AA-0009. 6. will be willing to have the results of their intake evaluation become part of their record at NIH, as described in NIAAA protocol 98-AA-0009. 7. will be willing to submit a urine sample for the purpose of drug screening and, in the case of females, for pregnancy testing. 8. will be willing to submit blood samples for the genetic testing described herein. 9. must be essentially physically and emotionally healthy, as determined by the screening procedures in 4& 5. 10. must be willing to be contacted at five year intervals for purpose of interview about their drinking behavior in the interim, and provide identification and contact information which would facilitate such follow-up A subject will be excluded from the study if they: 1. have an intermediate score on the telephone administered SRE (within one standard deviation of the mean). 2. have an abnormal physical exam or laboratory values outside the normal ranges. 3. are pregnant. 4. have a positive drug screen. 5. have fulfilled DSM-IV for ethanol or other substance dependency at any time within the past 5 years. 6. have a history of stimulant use or abuse. 7. have a history of smoking within the last year, or a lifetime history of smoking more than 100 cigarettes. 8. have fulfilled DSM-IV for any major (non substance-related) Axis I psychiatric disorder. 9. have taken any prescribed, non-prescribed, or over-the-counter medications or drugs within 14 days prior to the study days (excluding oral contraceptive agents, aspirin, or acetaminophen). 10. have a history of claustrophobia. 11. have a history of facial flushing syndrome in response to alcohol exposure | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Crohn Disease Patients must meet each of the following to be eligible for enrollment in this study. 1. Patients with Crohn's disease of at least 6 months duration (with colitis, ileitis, or ileocolitis) confirmed by radiography or endoscopy. 2. Patient must have a CDAI score less than 220. 3. Patients with corticosteroid-dependent Crohn's disease who have failed at least one attempt at corticosteroid tapering within the previous 6 months. Corticosteroid-dependent patients are defined as those patients who have relapse of Crohn's disease within 60 days following completion of corticosteroid treatment; OR during corticosteroid tapering at doses greater than or equal to 10 mg/day (prednisone equivalent); OR within 3 months following corticosteroid tapering, while receiving a corticosteroid dose greater than or equal to 10 mg/day. 4. Patients with a CDAI score of less than 150 MUST be on oral corticosteroids (other than oral budesonide) greater than or equal to 10 mg/day to be on oral corticosteroids (prednisone equivalent) for Crohn's disease be on a stable dose of oral corticosteroids (other than oral budesonide) for at least 2 weeks prior to screening have had clinically inactive Crohn's disease for at least 2 weeks prior to screening. OR Patients with a CDAI score of greater than or equal to 150 to less than 220 MUST be on oral corticosteroids (othern than oral budesonide) greater than or equal to 10 mg/day to less than or equal to 40 mg/day (prednisone equivalent) for Crohn's disease have had no worse than mild disease for at least 2 weeks prior to screening. 5. Patients on aminosalicylates must have been on a stable dose for at least 4 weeks prior to screening; and patients on the immunosuppressants, azathioprine, 6-mercaptopurine, or methotrexate must have been on a stable dose for at least 8 weeks prior to screening. 6. Patients not using aminosalicylates must have discontinued treatment at least 4 weeks prior to screening. Patients not using immunosuppressants such as azathioprine, 6-mercaptopurine, or methotrexate must have discontinued treatment at least 4 weeks prior to screening. Patients who had been receiving infliximab must have stopped therapy at least 8 weeks prior to screening. Patients who had been receiving adalimumab, cyclosporine, tacrolimus, or mycophenolate mofetil must have stopped therapy for at least 4 weeks prior to screening. 7. Patients who have incidental (e.g. perianal) fistulae are permitted, provided patients have predominantly luminal Crohn's disease, and fistulae are not associated with retention. 8. Patient's platelet count must be greater than or equal to 20,000/cmm. 9. Female patients must be one of the following: postmenopausal, surgically incapable of bearing children, practicing an acceptable method of birth control (acceptable methods may hormonal contraceptives, intrauterine device, and spermicide and barrier). Abstinence or partner/spouse sterility may also qualify at the Investigator's discretion. If a female patient is of childbearing potential she must have a negative urine pregnancy test at screening. 10. Patients must be able and willing to comply with all study procedures. 11. Signed informed consent must be obtained prior to conducting any study procedures. 12. Patients must be men and women greater than or equal to 18 years of age. 13. Patients must have a body weight greater than or equal to 40 kg (88 lb) The presence of any of the following will the patient from from participating in the study: 1. Patients with symptomatic intestinal strictures. 2. Patients with local manifestations of Crohn's disease such as abscesses, or disease manifestations for which surgery might be indicated, or which might preclude utilization of a CDAI to assess response to therapy (such as "short gut" syndrome). 3. Patients with stomas. 4. Patients with rectovaginal fistulae. 5. Patients who require antibiotics for the treatment of Crohn's disease. 6. Patients using oral budesonide. 7. Patients with diarrhea, due to conditions other than inflammatory Crohn's disease (e.g. bacterial or parasitic gastroenteritis, bile salt diarrhea, or bacterial overgrowth). 8. Patients who are concomitantly using an anti-TNF agent, antibiotics, nonsteroidal anti-inflammatory drugs (NSAIDs), cyclosporine, tacrolimus, mycophenolate mofetil, or investigational therapies. 9. Patients unable to tolerate the extracorporeal volume shifts associated with ECP treatment due to the presence of any of the following conditions: uncompensated congestive heart failure, pulmonary edema, severe chronic obstructive pulmonary disease, severe asthma, renal failure, or hepatic failure. 10. Patients with a poor tolerability of venipuncture or a lack of adequate venous access for required blood sampling. 11. Patients receiving total parenteral nutrition (TPN), as the sole source of nutrition, within 3 weeks of screening. 12. Patients with hypersensitivity or allergy to psoralen (methoxsalen). 13. Patients with hypersensitivity or allergy to both heparin and citrate products. 14. Patients with active bleeding. 15. Females who are pregnant and/or lactating. 16. Patients must not have been enrolled in any investigational study for 30 days prior to enrollment | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Osteoporosis Female, 5 years postmenopause Ambulatory Community dwelling Able to ingest calcium and vitamin D supplements Willing to restrict vitamin K intake Stable thyroid dose if appropriate No history of hyperthyroidism, hyperparathyroidism or other metabolic bone diseases Absence of hardware in hip and spine History of malignancy within the last five years Not currently using coumadin or warfarin | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-75.0, Hypereosinophilia Hypereosinophilic Syndrome Documented history of Hypereosinophilic Syndrome (HES) Eosinophil count greater than 1500 cells for 6 months Signs and symptoms of organ system involvement No evidence of parasitic, allergic or other causes of eosinophilia after comprehensive evaluation Achieve and maintain a stable prednisone (corticosteroid) dose prior to starting study medication Not pregnant or nursing Churg-Strauss Syndrome Wegener's Granulomatosis Lymphoma, hematological malignancy, advanced and metastatic solid tumors Chemotherapy, radiotherapy or interleukin 2 treatment | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, GITR GITR Ligand Uveitis Immunosuppression Suppressor of Cytokine Secretion Patients with bilateral sight threatening uveitis requiring systemic immunotherapy who are 18 years and older are eligible. Disease can be active or quiescent, but subjects must be on a minimum prescribed therapy upon enrollment of a dose averaging at least 20 mg/day (or greater than or equal to 0.25 mg/kg/day) of systemic prednisone or a more intensive immunosuppression regimen. More intensive regimens may from one to three anti-inflammatory treatments for uveitis that any one of the following (or related) compounds: corticosteroids (including systemic or periorbital administration), topical corticosteroids (when used in combination with other agents), cyclophosphamide, cyclosporine, azathioprine, chlorambucil, tacrolimus, leflunomide, mycophenolate mofetil, or methotrexate Patients who have non-infectious intermediate, posterior, or panuveitis of at least 3 months duration. Included conditions may but are not limited to intermediate uveitis of the pars planitis subtype, sarcoidosis, the Vogt-Koyanagi-Harada (VKH) syndrome, birdshot retinochoroidopathy, retinal vasculitis and sympathetic ophthalmia Patients who are 18 years of age or older Subjects will not be able to enroll if they Are unwilling or unable to give blood at the designated times in the protocol Have another disease or condition affecting vision that will interfere with obtaining study data Are pregnant | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Kidney Neoplasms CELL Patients must have metastatic renal cell cancer age greater than or equal to 18 years Clinical performance status of Eastern Cooperative Oncology Group (ECOG) 0, 1 at entry to the trial Life expectancy of greater than three months Seronegative for human immunodeficiency virus (HIV) antibody. (The experimental treatment being evaluated in this protocol depends on an intact immune system. Patients who are human immunodeficiency virus (HIV) seropositive can have decreased immune competence and can thus be less responsive to the experimental treatment and more susceptible to it's toxicities.) Seronegative for hepatitis B antigen Seropositive for Epstein-Barr Virus (EBV) Patients with electrocardiogram (EKG) abnormalities, symptoms of cardiac ischemia or arrythmias or age greater than 50 years must have a normal stress cardiac test (stress thallium, stress multi-gated acquisition scan (MUGA), dobutamine echocardiogram or other stress test) Patients who have a recent prolonged history of cigarette smoking or symptoms of respiratory dysfunction must have pulmonary function testing with an forced expiratory volume in 1 second (FEV(1)) greater than 60% predicted CELL -Active systemic infections, coagulation disorders, contra-indications to receiving interleukin-2 (IL-2) or major medical illnesses of the cardiovascular, respiratory or immune system. CELL Patients must have measurable metastatic renal cell cancer and have tumor progression after therapy with interleukin-2 (IL-2) Clinical performance status of Eastern Cooperative Oncology Group (ECOG) 0, 1 at entry to the treatment phase of this trial Platelet count greater than 100,000/mm^3 Serum alanine aminotransferase (ALT)/aspartate aminotransferase (AST) less than three times the upper limit of normal Serum creatinine less than or equal to 1.6 mg/dl Total bilirubin less than or equal to 1.6 mg/dl or direct bilirubin less than or equal to 0.5 mg/dl Life expectancy of greater than three months At the time of T-cell transfer, the patient must have a T-cell population which has met the attached Certificate of Analysis for tumor recognition and safety testing Any patient receiving interleukin-2 (IL-2) must sign a durable power of attorney | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Malignant Carcinoid Syndrome Histologically confirmed diagnosis of a neuroendocrine tumor of the carcinoid type Documented evidence of carcinoid syndrome (flushing and/or diarrhea) attributable to a primary tumor of the lung, stomach or mid-gut Previous positive Octreoscan World Health Organization (WHO) performance score lower than 2. At the baseline visit patients MUST satisfy the following before they are randomized to receive study treatment Stool and/or flushing frequency of greater than or equal to 3 episodes/day (average over a minimum five consecutive days) Patients who have previously been treated with somatostatin analogues must have discontinued treatment for a sufficient period of time (a washout period of at least 7 days for immediate release formulations and up to 2 months for prolonged release formulations is usually required). Compared with their "controlled" state on treatment, these patients must show a clinically significant deterioration (at least two episodes) of either symptom. For example, a patient considered to be controlled on their previous treatment with an estimated stool frequency of two episodes per day, must achieve a stool frequency of at least four episodes per day (average over a minimum five consecutive days) WHO performance score lower than 2 VIPoma or other non-carcinoid tumor Treatment with interferon, chemotherapy or radiotherapy given within 30 days prior to or planned during the study Radionuclide treatment within three months prior to or planned during the study Presence of other active malignant pathology (except basal cellular carcinoma of the skin and/or in situ carcinoma of the cervix/uterus) Surgical procedure or embolization procedure (with or without cytotoxic agents) of the tumor within three months prior to or planned during the study Life expectancy of less than 6 months Any investigational drug given within 30 days prior to or expected to be given during the study No access to a telephone for completion of the daily telephone diary | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Clear Cell Renal Cell Carcinoma Recurrent Renal Cell Cancer Stage III Renal Cell Cancer Stage IV Renal Cell Cancer Patients must have histologically or cytologically confirmed renal cell carcinoma which is either metastatic (M1) or unresectable (M0); patients must have a component of conventional clear cell renal carcinoma; patients with true papillary renal cell carcinoma, sarcomatoid features without any clear cell component, chromophobe, oncocytoma, collecting duct tumors and transitional cell carcinoma are NOT eligible Patients must have measurable disease; soft tissue disease, which has been radiated in the 2 months prior to registration, is not assessable as measurable disease; X-rays, scans, or physical examinations used for tumor measurement must have been completed within 28 days prior to registration; X-rays, scans or physical examinations for non-measureable disease must have been completed within 42 days prior to registration; all disease must be assessed Patients with metastatic disease who have a resectable primary tumor and are deemed a surgical candidate may have undergone resection and have recovered from surgery; at least 28 days must have elapsed since surgery and patient must have recovered from any adverse effects of surgery Patients must not have received any prior systemic therapy for renal cell carcinoma, including chemotherapy, interferon, interleukin-2, other biologic response modifiers, anti-angiogenic therapy, hormonal therapy, or any other experimental systemic therapy; prior treatment with thyroid medications is allowed Patients may have received prior radiation therapy to less than 25% of the bone marrow; at least 28 days must have elapsed since completion of prior radiation therapy; patients must have recovered from all associated toxicities at the time of registration Total bilirubin =< institutional upper limit of normal SGOT or SGPT =< 2.5 x institutional upper limit of normal Serum creatinine =< institutional upper limit of normal or calculated creatinine clearance >= 60 mL/min for patients with creatinine levels above institutional normal Patients who have had a prior nephrectomy must have a serum creatinine =< 2 x institutional upper limit of normal Patients must be offered the opportunity to consent for specimen submission | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Stomatitis Male or female patients ages > 18 yrs Patients undergoing high dose chemotherapy with or without radiation therapy treatment as conditioning for autologous hematopoietic stem cell transplantation. The conditioning regimens at least one of the following: high dose melphalan (Mel 200), busulfan, or etoposide, with or without total body irradiation Patients with Karnofsky performance scores > or = 70% Informed consent for participation in study Patients who weigh < 33 kg Premenopausal female patients who are pregnant, lactating or are likely to become pregnant Patients with active medical conditions that preclude autologous hematopoietic stem cell transplantation Patients diagnosed with active acquired immunodeficiency syndrome (AIDS) or Hepatitis B/C Patients with known hypersensitivity to recombinant protein therapeutics Patients who have taken CG53135-05, palifermin or other investigational drugs in the past 30 days Patients who have untreated symptomatic dental infection Patients with a history of sensitivity or allergy to E. coli-derived products Patients with WHO Grade 3 or 4 oral mucositis (OM) | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Systemic Lupus Erythematosus Has SLE by ACR revised (meets <4 criteria) Has SLE with at least one elevated lupus antibody Has new onset of severe lupus disease flare in at least one body or organ system, excluding renal or neurologic Active severe CNS or Renal disease defined by BILAG as Level A Allergy to murine or human antibodies Antiphospholid antibodies AND a history of thrombocytopenic events | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 10.0-70.0, Crohn's Disease 70 years-old., Weigh 20-100 kg (44-220 lbs) CD of the ileum, colon or ileocolon, verified by colonoscopy, barium enema, or small bowel series performed within 36 months Perianal fistulae will be eligible provided that the perianal disease does not account for the preponderance of symptoms Have steroid-dependent, steroid-refractory or steroid naive CD Steroid-dependent CD: CDAI or mCDAI of < 150 while receiving prednisone 10-40 mg/day or budesonide 3-9 mg/day for at least 12 weeks prior to screening, but unable to taper prednisone below 10 mg/day or budesonide below 3 mg/day without experiencing a flare within the previous 6 months. Steroids must be at a stable dose for 2 weeks prior to screening (week #-2), prednisone at a dose of 10-40 mg/day and budesonide at a dose of 3-9 mg/day Steroid-refractory CD: currently moderately active CD (CDAI or mCDAI 200 despite treatment with 40 kg) or 0.5 mg/kg/day (if weighing 20 mg/day (if weighing prednisone <40 9 mg/day for the previous 4 weeks prior to the screening kg), or budesonide evaluation. Prednisone or budesonide must be at a stable dose for 2 weeks prior to screening (week #-2) Steroid-naïve CD: currently moderately active CD, (CDAI or mCDAI 200 and one of the following: 1. Despite treatment with aminosalicylates and/or antibiotics for the previous 4 weeks prior to the screening evaluation, who are candidates for prednisone or budesonide. 2. Not currently on therapy, who are candidates for prednisone or budesonide 3. Patients with prior exposure to steroids, who have not been treated with steroids for 4 weeks prior to screening, and are candidates for prednisone or budesonide Prednisone or budesonide will be started at the screening visit, at a dose of 40 mg/day or 9 mg/day and tapered per the steroid taper. Patients who have started steroids up to 14 days prior to screening will also qualify as steroid naïve, however patient needs to be on 40 mg prednisone or 9 mg budesonide Discontinue oral or rectal 5-Aminosalicylic acid (5-ASA) therapies, rectal steroids, ciprofloxacin or metronidazole at the screening visit CDAI > 450 CD requiring hospitalization and intravenous (iv) corticosteroids, iv antibiotics or total parenteral nutrition (TPN) TPN or enteral nutrition of >1000 Calories/day (both TPN and elemental diets impact the CDAI) History of resection of more than 100 cm of small bowel, total proctocolectomy, or subtotal colectomy with ileorectal anastomosis Ileostomy or colostomy Severe fixed symptomatic stenosis of the small or large intestine Blood transfusion within 3 months before screening Treatment with 6-MP or AZA within the 6 months prior to screening Immunosuppressants or biologics 3 months before screening Treatment 2 weeks before screening | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Lupus Erythematosus, Systemic Has SLE by American College of Rheumatology revised (meets >/= 4 criteria) Has had SLE for at least 6 months prior to study entry Has at least one elevated autoantibody level at study entry Has moderately active SLE disease at study entry in any body/organ system (Full not present here due to length of considerations. The protocols should be consulted regarding the complete list of entry criteria.) Active Severe Lupus as defined by BILAG Index Level A in any body system or organ Allergy to human antibodies or Murine Prior therapy with other anti-B cell antibodies | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-50.0, Asthma Healthy Asthmatic Subjects Clinical diagnosis of asthma Steroid naive or subjects not using their inhaled steroids for the last 4 weeks, short-acting beta-agonist for rescue medication FEV1 > 70% of predicted Positive skin prick test for house dust mite, cat or grass pollen PC20 methacholine or histamine < 8 mg/ml Asthmatic Subjects Use of systemic steroids in the previous 6 weeks Asthma exacerbations in the previous 6 weeks Current or ex-smokers with ≥ 10 pack-years (≥ 2 pipe pack-years), ex smokers who stopped less than 1 year ago Healthy Subjects No airway complaints FEV1 > 90% of predicted Negative skin prick test PC20 methacholine or histamine > 8 mg/ml Healthy Subjects Use of steroids in the previous 6 weeks Current or ex-smokers with ≥ 10 pack-years (≥ 2 pipe pack-years), ex smokers who stopped less than 1 year ago | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Systemic Lupus Erythematosus participants must be diagnosed with SLE and be experiencing an active lupus flare in at least one of three organ systems: skin (discoid lesions), inflammation of the lining of the heart (pericarditis), or inflammation of the lining of the lung (pleuritis/pleurisy); or inflammation of more than 4 joints within 14 days of a screening visit (arthritis) Stable dose of prednisone (<30mg) for at least one month participants experiencing an active lupus flare in the kidney or central nervous systems Treatment with a stable dose of azathioprine, mycophenolate mofetil, hydroxychloroquine, chloroquine, or methotrexate for less than three months prior to the study participants with active viral or bacterial infections participants with any other autoimmune disease as a main diagnosis Prior treatment with rituximab | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Chronic Myeloproliferative Disorders Leukemia Lymphoma Nonneoplastic Condition Precancerous Condition Histologically confirmed systemic mastocytosis Objective evidence of disease, as defined by the following Hemoglobin < 10 g/dL Recurrent mast cell mediator-release symptoms that impair the patient's quality of life Symptomatic hepatosplenomegaly Ascites Symptomatic bone disease Profound constitutional symptoms (e.g., fatigue, asthenia, flushing, hyperpyrexia, weight loss, myalgia, and arthralgia) Elevated serum tryptase level Mast cell leukemia allowed | 1 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 40.0-85.0, Cardiovascular Disease Outpatients who are required a combination therapy with sitting systolic blood pressure ≥ 140 mmHg or diastolic blood pressure ≥ 90 mmHg Outpatients aged over 40 years and less than 85 years (inclusive), regardless of sex Previously untreated patients or patients who are on other therapy, which can be converted to 4mg of benidipine Patients who can be treated with benidipine, angiotensin receptor blockers, β-blockers, and thiazide diuretics Seated systolic blood pressure ≥ 200 mmHg or seated diastolic blood pressure ≥ 120 mmHg Secondary hypertension Type I diabetes mellitus or type 2 diabetes on insulin treatment History of cerebrovascular disorder, myocardial infarction, angina pectoris, coronary angioplasty or coronary artery bypass graft surgery within 6 months prior to enrolment in the study Heart failure (New York Heart Association [NYHA] functional classification II, III or IV) Chronic atrial fibrillation or atrial flutter Congenital heart disease or a history of rheumatic heart disease Severe peripheral arterial disease (Fontaine Class II, III or IV) Serious liver dysfunction (AST or ALT ≥100 IU / l) Serious renal dysfunction (serum creatinine ≥ 2mg/dl) | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-30.0, Fasting Healthy lean women (with body mass indices [BMI] < 25 kg/m2) Overweight otherwise healthy men (with BMI > 27 kg/m2) Overweight otherwise healthy women (with BMI > 27 kg/m2) A history of any illness that may affect the concentrations of the hormones to be studied, e.g. infectious diseases, renal or hepatic failure, type 1 or type 2 diabetes mellitus, cancer or lymphoma, hypogonadism, malabsorption or malnourishment, hypo or hyperthyroidism, hypercortisolism, alcoholism or drug abuse, anemia, or eating disorder On medications known to affect the hormones to be measured (glucocorticoids, anti-seizure medications, and thyroid hormones) A known history of anaphylaxis or anaphylactoid-like reactions, or a known hypersensitivity to E. coli derived proteins | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Head and Neck Cancer Patients must have documented advanced, locally recurrent, or metastatic head and neck carcinoma, which is untreatable by surgical resection or radiation therapy Prior chemotherapy for advanced/metastatic disease is allowed (1 regimen only) Patients must be taxane-naïve (no prior docetaxel or paclitaxel) Patients who have received chemoradiation as a primary therapy for advanced head and neck cancer are eligible Patients must have measurable or evaluable disease. Pre-study imaging for disease assessment must be done within 28 days of registration Patients with brain metastases are eligible if they have been stable for at least six weeks post-radiation therapy Aged 18 years or older Performance status of 0-2 by Zubrod criteria Life expectancy of at least 12 weeks Hematologic: absolute neutrophil count (ANC) equal to or > 1,500/mm3; hemoglobin equal to or > 8.0 g/dl; platelets equal to or > 100,000/mm3 Patients with congestive heart failure, second or third degree heart block or recent myocardial infarction within 12 months from registration are not eligible Peripheral neuropathy equal to or greater than grade 2 Patients with a history of severe hypersensitivity reaction to drugs formulated with polysorbate 80 Use of standard chemotherapy or investigational agents for treatment of head and neck cancer within 28 days of 1st dose of study drug Any medical or psychiatric illness which, in the opinion of the principal investigator, would compromise the patient's ability to tolerate this treatment regimen Prior unanticipated severe reaction to fluoropyrimidine therapy or known hypersensitivity to 5-fluorouracil Pregnant or lactating women, women of childbearing potential with either a positive pregnancy test (PPT) at baseline, or sexually active females not using a reliable contraceptive method while on study and for at least six months after chemotherapy. (Postmenopausal women must have been amenorrheic for at least 12 months to be considered of non-childbearing potential.) Sexually active patients not using a reliable contraceptive method while on study and for at least six months after chemotherapy Patients with malabsorption syndromes will be excluded. Administration of capecitabine through feeding tubes is permitted Serious concurrent infections | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 45.0-999.0, Osteoporosis Women who are postmenopausal with at least one year since the last menstruation Women who are 45-54 years of age with the following bone mineral density (BMD) and/or vertebral fracture BMD 3.0 standard deviations (SDs) or more below peak bone mass of young females at the lumbar spine, femoral neck, or total hip; or BMD 2.5 SDs or more below peak bone mass of young females at the lumbar spine, femoral neck or total hip with the presence of a vertebral fracture verified by the central imaging organization before the patient is enrolled into the study Women 55 or more years of age with the following BMD and/or vertebral fracture BMD 2.5 SDs or more below peak bone mass of young females at the lumbar spine, femoral neck or total hip; or BMD 2.0 SDs or more below peak bone mass of young females at the lumbar spine, femoral neck or total hip with the presence of a vertebral fracture verified by the central imaging organization before the patient is enrolled into the study The following types of vertebral fractures should not be considered for patient enrollment into this trial Pathological fractures due to malignant disease or infection Fractures due to excessive trauma sufficient to cause a fracture in young individuals with normal bone mass A. Vertebral Deformity Patient has 5 or more vertebral (thoracic and lumbar) deformities Patient has 2 or more lumbar vertebral deformities (L1 to L4) Severe lumbar scoliosis (>15 degrees) which precludes a reliable evaluation of the dual x-ray absorptiometry (DXA) B. DXA Imaging Inability to have a DXA scan performed. C. History or Concurrent Illness Disorders of immunity Endocrine system Gastrointestinal system Kidney and collecting system Liver, biliary tract and pancreatic systems | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Osteoarthritis Peptic Ulcer Must require the chronic use of a non-steroidal anti-inflammatory drug for the treatment of osteoarthritis Must be taking daily aspirin for cardiovascular prophylaxis Clinical Laboratory values within normal limits for this population History of gastric, duodenal or esophageal surgery except simple oversew of an ulcer Evidence of uncontrolled, clinically significant disease History of cancer within the past 5 years Presence of gastroduodenal ulcers, esophageal ulcer or >= 10 gastroduodenal erosions during the screening endoscopy. Known history of gastroduodenal ulcer or bleeding within the past year. Esophageal stricture requiring dilatation Presence of Barrett's esophagus with dysplastic changes Systemic disease affecting the esophagus or a history of caustic or physiochemical trauma or irradiation to the esophagus Sero-tests positive for H. pylori Evidence of Zollinger-Ellison syndrome, esophageal varices, cholecystitis, or pancreaticobiliary tract disease Requires treatment with an excluded medication such as proton pump inhibitors, histamine H2 receptor antagonists, antacids, corticosteroids, lithium, fluconazole, misoprostol, probenecid, methotrexate, anticoagulants, St. John's wart, dong quai, feverfew, garlic, ginger, horse chestnut, red clover or white willow supplements or bisphosphonates | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Arthritis Arthroscopic Hip Surgery primary THA (cemented or cementless) preoperative diagnosis of osteoarthritis (primary or secondary) meets objective discharge-to-home test following hip replacement ability to speak English and reply to questionnaires written in English ability to provide informed consent may patients presenting for their second primary hip arthroplasty Discharge to Home independent supine-to-sit transfer independent sit-to-stand transfer walk with walking aid 30 meters ascend and descend 3 stairs surgery as part of a workers' compensation claim arthroplasty performed for trauma or following open reduction and internal fixation, malignancy or inflammatory arthropathy intra-operative complication of fracture or sciatic nerve injury procedure requiring complex surgical approach other than posterior or anterolateral (ie trochanteric osteotomy/micro 2 incision approach) indications for additional primary or revision arthroplasty within the time frame of the study (24 months) in other hip or knee joints which would adversely affect quality of life and functional scores pertaining to the joint of interest | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-76.0, Leukemia, Mast-Cell Mantle-cell Lymphoma Recipient Between 18 and 76 years of age Chronic lymphocytic leukemia (CLL) Unmutated IgG VH gene status Mutated IgG VH genes (> 2% nucleotide change compared to somatic sequence) Complete remission benefit most from allogeneic hematopoietic stem cell transplant (HSCT). (Physicians will be encouraged to provide aggressive chemotherapy prior to nonmyeloablative transplantation.) Mantle cell lymphoma (MCL): Transplant physicians believe subject would benefit from allogeneic HSCT Adequate renal (Cr < 2.4 mg/dL) and hepatic (Bilirubin < 3.0 mg/dL, Aspartate aminotransferase (AST) < 100 IU) function Men and women of reproductive potential must agree to use an acceptable method of birth control during treatment and for six months after completion of treatment All subjects must provide written informed consent Donor Recipient has a 9 of 10 or 10 of 10 HLA identical donor (high resolution molecular genotyping at HLA A, B, C and DrB1, and DQ) Pregnancy Lactating Serious uncontrolled infection HIV seropositivity Hepatitis B or C seropositivity Cardiac function: ejection fraction < 40% or uncontrolled cardiac failure Pulmonary: Diffusing capacity carbon monoxide (DLCO) < 50% predicted Liver function abnormalities: elevation of bilirubin to ≥ 3 mg/dL and/or AST > 100 | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Nasal Polyps Gastroesophageal Reflux Subjects will Be adults with nasal polyposis recruited from the PI's private practice Not be currently taking a PPI Be able and willing to undergo a noninvasive 24 hour pH probe study; and Take a PPI Patients who Are pregnant Have a history of surgical treatment for reflux disease History of allergic or adverse reaction to Prevacid or adverse reaction to Prevacid during the study period; and Do not meet criteria | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Recurrent Melanoma Recurrent Renal Cell Cancer Stage III Melanoma Stage IV Melanoma Stage IV Renal Cell Cancer Histologically confirmed diagnosis of 1 of the following Melanoma Unresectable stage III disease Stage IV disease Renal cell carcinoma Unresectable and/or stage IV disease Measurable disease No untreated brain metastases or leptomeningeal disease Patients with previously treated brain metastases are eligible provided they have no evidence of progression for ≥ 4 weeks following treatment and do not require steroids Performance status | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Systemic Mastocytosis, Aggressive (ASM) Leukemia, Mast Cell Hematological Non-mast Cell Lineage Disease (AHNMD) At least 18 years of age Karnofsky performance status (KPS) of > 30% (equivalent to ECOG 0 to 3) Mast cell disease, histologically confirmed and documented to be Aggressive systemic mastocytosis (ASM) OR Mast cell leukemia (MCL) meeting the following Meets for systemic mastocytosis Biopsy indicates diffuse infiltration by atypical, immature mast cells Bone marrow aspirate smears show at least 20% mast cells Confirmed availability of tissue sample within 6 months prior to entry into study, for evaluation of KIT mutation status of the tumor cells. Subjects who have systemic mastocytosis PLUS eosinophilia AND known positivity for FIP1L1-PDGFR-alpha fusion are eligible only if they have demonstrated relapse or disease progression on prior imatinib therapy Blood levels of liver enzymes within normal limits (EXCEPTION: If the sole cause of elevated blood levels of liver enzymes is ASM/MCL, then AST and ALT ≤ 4X upper limit of normal (ULN), and/or bilirubin ≤ 4X ULN) Active pulmonary disease based on physical assessment or lateral chest X-ray, considered by the investigator to be unrelated to mastocytosis Any pulmonary infiltrate or abnormality on the baseline chest X-ray known to be new in the previous 4 weeks (EXCEPTION: pleural effusion related to systemic mastocytosis, eg, secondary to ascites, AND not causing symptomatic respiratory complaints, may be eligible) Cardiovascular disease, including congestive heart failure Myocardial infarction within 6 months Poorly-controlled hypertension with any Grade 3/4 cardiac problems (per New York Heart Association Criteria) Uncontrolled diabetes Chronic renal disease Active uncontrolled infection Known malignant disease involving the central nervous system (CNS) Known confirmed diagnosis of HIV infection or active viral hepatitis | 1 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Breast Cancer Signed informed consent, premenopausal women 18 years or older, histologically or cytologically confirmed locally advanced or metastatic breast cancer, suitable for endocrine treatment, no curative treatment available,, one or more measurable lesions, life expectancy more than 6 months, normal biological parameters Presence of life-threatening metastases, previous endocrine therapy or chemotherapy for advanced or metastatic disease, any previous treatment with hormone (LH-RH) severe or uncontrolled systemic disease, pituitary adenoma, High risk of medullar compression | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-80.0, Arthritis, Rheumatoid Previous diagnosis of RA according to the revised 1987 of the American Rheumatism Association (Arnett et al., 1988) patients must have a minimum of 8 tender and 4 swollen joints with disease diagnosed at least 3 months prior to screening patients must have been using oral or parenteral MTX for at least the previous 3 months, and at a stable dose of 7.5 to 25 mg per week for at least the previous 1 month women of childbearing potential must test negative for pregnancy and be using adequate birth control measures patients must have a documented purified protein derivative (PPD) skin test performed at prescreening Patients who have received any prior treatment with infliximab or with any other therapeutic agent targeted at reducing tumor necrosis factor (TNF) (e.g., etanercept, pentoxifylline or thalidomide) within the previous 3 months patients who are incapacitated history of infected joint prosthesis within the previous 5 years patients with a concomitant diagnosis of congestive heart failure (CHF), history of or known malignancy within the previous 5 years, cases of active or latent tuberculosis (TB), acute or chronic serious infections within the past 3 months known substance abuse (drug or alcohol) within the previous 3 years | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 20.0-70.0, Non-Hodgkin's Lymphoma Histologically proven NHL, and for which intensive chemotherapy is considered treatment-of-choice. 2. HBsAg-positive. 3. No previous chemotherapy and radiotherapy. 4. No concurrent radiotherapy. AGC ≧ 2,000/mm3, Platelet ≧ 100,000/mm3 of peripheral blood. 5. Total bilirubin ≦ 2.5 mg/dl. Alanine aminotransferase (SGPT) < 200 I.U/L 6. Serum creatinine ≦1.5 mg/dl Blood urea nitrogen (BUN) ≦ 25 mg/dl 7. Objectively measurable or evaluable disease 8. Signed informed consent Age > 75 years, or Age < 15 years 2. Pregnant or breast-feeding women. 3. Patients with history of brain metastasis or CNS involvement. 4. Child's class B or C in patients with liver cirrhosis. 5. Impaired cardiac function with NYHA (New York Heart Association) classification ≧ GrII. 6. Concurrent glucocorticoids use (for other reasons). The conventional use of glucocorticoids for antiemetic purpose is also not allowed | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 8.0-999.0, Sickle Cell Disease Vaso-occlusive Crisis Sickle cell and acute pain Age 8 and up English or Spanish-speaking Fever greater than 101 Acute chest syndrome or pneumonia Other SS complications (sequestration, aplastic crisis) Other explanation for pain (chronic, AVN, surgical) History of GI bleeding, HTN, or hyperglycemia/DM | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-75.0, Anemia Acquired Immunodeficiency Syndrome Patients with a confirmed diagnosis of AIDS, exhibiting signs and symptoms of AIDS characterized by lymphocytopenia (decrease in the number of lymphocytes [cells that are typical elements of lymph tissue] in the blood) as determined by a finding of <1,000 cells/cubic millimeter having a documented HIV antibody (either a history of infections due to the patient's impaired resistance or the presence of severe symptoms, such as persistent fever or night sweats associated with significant weight loss) receiving AZT therapy of at least 400 mg/day having a hematocrit <=30%, and a history of a >=15% decrease in hematocrit since starting AZT therapy, or that the patient has become dependent on transfusions clinically stable for at least 1 month before study entry, with a performance score of 0, 1, or 2 Patients with a history of any important blood disease or clinically significant disease or malfunction of the lungs, heart, hormones, neurological, gastrointestinal, reproductive or urinary systems, which are not caused by the AIDS infection having a sudden onset of infections, dementia due to AIDS, uncontrolled high blood pressure, or an iron deficiency received androgen therapy within 2 months of study entry having anemia caused by other conditions than AIDS or AZT therapy (for example, certain vitamin deficiencies or bleeding from the gastrointestinal tract) having a history of seizures, history of cell damage due to chemotherapy within 1 month before study entry, or a history of substance abuse | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 15.0-70.0, Autoimmune Thyroiditis Hashimotos Thyroiditis Clinically approved AIT patients who do not use any medication other than LT4 to keep TSH in the lower half of normal range Any kind of drug use other than LT4 or any kind of known pathology which may effect GIS absorption | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Kidney Diseases Patients undergoing a single renal transplant from deceased or living donor Adults 18 years and older First or second renal transplant Capable of understanding the purposes of the study, has given written informed consent, and agrees to comply with the study requirements Women of child bearing age should have a negative serum pregnancy test Greater than 2 renal transplants Age < 18 years Patients receiving immunosuppressive therapy within the preceding 28 days for first transplant and 3 months for the second transplant Cold ischemia time > 30 hours History of malignancy in the last 5 years except successfully treated localized non-melanoma skin cancer HIV, hepatitis B virus (HBV), or hepatitis C virus (HCV) positive serology Loss of previous transplant in < 1 year History of non-compliance Any form of substance abuse, psychiatric disorder or condition which, in the opinion of the investigator, may impair communication with the investigator or compliance with study procedures Multiple organ transplant | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-90.0, Abscess Cysts Female or male, aged between 18 and 90 years Written informed consent Abscess formation or abdominal cyst scheduled to drainage Plasma creatinine <1.5 mg/dL Pregnancy or lactation Hemodialysis or hemofiltration Allergy or hypersensitivity against study drugs Massive edemata or hypernatremia Reduced liver function (Child-Pugh A, B, C) Relevant prolongation of QT-interval CNS-diseases which predispose for cramps | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-60.0, Rheumatoid Arthritis Participant Age > 18 and < 60 years at time of pre-transplant evaluation An established clinical diagnosis of rheumatoid arthritis by American College of Rheumatology criteria Patients must have failed an autologous hematopoietic transplant or have failed to respond to either methotrexate or leflunomide in combination with a TNF inhibitor. Failure is defined as an inability to tolerate treatment with at least 6 swollen joints and 20 involved joints or inability to answer at least 70% of HAQ questions with "no difficulty" despite 2 or more months of treatment Ability to give informed consent Patient must have a HLA matched sibling donor at the A, B, C, and DR loci to proceed or HLA matched cord blood donor If donor is HLA matched cord blood, cord blood stem cells will be obtained from the NMDP (1-800-548-1375) and one or two units of HLA matched cord blood will be infused on day zero. Participant History of coronary artery disease, or documented congestive heart failure HIV positive Uncontrolled diabetes mellitus, or any other illness that in the opinion of the investigators would jeopardize the ability of the patient to tolerate aggressive chemoradiotherapy Prior history of malignancy except localized basal cell or squamous skin cancer. Other malignancies for which the patient is judged to be cured by local surgical therapy, such as head and neck cancer, or stage I breast cancer will be considered on an individual basis Positive pregnancy test, inability or unwillingness to pursue effective means of birth control, failure to willingly accept or comprehend irreversible sterility as a side effect of therapy Psychiatric illness or mental deficiency making compliance with treatment or informed consent impossible FEV1/FVC < 70% of predicted, DLCO < 40% of predicted Resting LVEF < 45 % Bilirubin > 2.0 mg/dl (unless due to Gilberts), transferase (AST) > 2.5 x upper limit of normal Serum creatinine > 2.0 mg/dl. Donor | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-72.0, Liver Cirrhosis Liver Transplant Disorder Male and female patients between 18 and 72 years of age Male or female patients who are primary cadaveric liver transplant recipients Cold ischemia time must be <20 hours Females capable of becoming pregnant must have a negative pregnancy test at baseline and are required to practice an approved method of birth control for the duration of the study and for a period of three months following discontinuation of study medication Patient has given written informed consent to participate in the study Patients meeting any of the following at baseline will be excluded from study participation Patients who have previously received an organ transplant Patients who are recipients of a multiple organ transplants Women of childbearing potential not using the contraception method(s) specified in this study, as well as women who are breastfeeding Known sensitivity to Simulect or class of Simulect Patients with severe medical condition(s) that in the view of the investigator prohibits participation in the study Use of any other investigational agent in the last 30 days | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 13.0-65.0, Pneumonia, Interstitial Plasma Cell Pneumocystis Carinii Pneumonia Pneumonia, Pneumocystis Carinii HIV Infections Documented or presumptive HIV infection Signs and symptoms of PCP present for at least 5 days Pneumocystis jiroveci confirmed in BAL fluid or induced sputum sample Suitable candidate for oral therapy Alveolar-arterial oxygen (A-a) gradient < or = 45 mm Hg on room air and partial pressure of oxygen (pO2) > or = 60 mm Hg No more than 48 hours of prior treatment in the preceding 7 days for PCP treatment at full doses; failure of Pneumocystis prophylaxis or use of medications recommended for treatment of PCP at doses less than recommended by the CDC Guidelines is acceptable Unwilling or unable to discontinue use of other medications with anti-PCP activity AIDS related cachexia (weight loss that is more than 10% of ideal body weight) Severe diarrhea and/or vomiting History of hypersensitivity or severe or life threatening toxicity to TMP-SMX, other sulfonamides or pentamidine Active illicit drug use Impending respiratory failure or need for intubation AST and ALT levels > 3 times the upper limit of normal History of pancreatitis Severe PCP Karnofsky score < or = 20 | 1 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-65.0, Breast Neoplasms Fibrocystic Disease of Breast Mammaplasty first visit to a Breast Disease Ambulance for consultation no pregnancy no severe heart disease (EF > 40%) no therapy with digitalis no acute/chronic inflammatory disease (e.g. RA, M. Crohn) no dysfunction of the Hypothalamo-Pituitary-Adrenal system no severe mental disorder | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Hypercholesterolemia Patients are eligible for study entry based on the following 1. Males or females greater than or equal to 18 years of age 2. Females must not be pregnant or lactating. Females of childbearing potential and males must use a reliable means of contraception. 3. LDL-C level greater than the NCEP goals, as determined by patients' risk category according to NCEP ATP III 4. Risk category for coronary heart disease and coronary heart disease equivalent with LDL goal of < 100 mg/dL 5. Baseline lipid LDL-C = 100 to160 mg/dL and triglyceride level = 100 to 500 mg/dL 6. Normal thyroid function tests (total T3, total T4, and thyroid-stimulating hormone [TSH]) 7. Hemoglobin A1C < 8.5% on a stable oral hypoglycemic or insulin regimen 8. On stable lipid modification pharmacotherapy (including a statin) for at least 2 weeks prior to study entry. Patients must be on at least half of the maximal doses of statins (as assessed by the Investigator), or be intolerant to statins such that the doses are not achievable. 9. Able to give informed consent Pre-Randomization Patients will not be eligible for the study based on the following 1. History of thyroid disorders of any form within 24 weeks prior to study entry 2. Active liver disease and/or liver transaminases greater than 1.5 X upper limit of normal 3. Active myocarditis, hypertrophic cardiomyopathy, uncorrected primary valvular disease, restrictive cardiomyopathy, uncorrected congenital heart disease, or constrictive pericarditis 4. Myocardial infarction, unstable ischemic heart disease, stroke, or coronary revascularization procedure within 24 weeks prior to study entry 5. Moderate or severe symptomatic congestive heart failure (New York Heart Association class III and IV) 6. Drug or alcohol dependence, or other conditions which may affect study compliance 7. Renal insufficiency (serum creatinine > 2 mg/dL) 8. Subjects taking other hormonal therapies (other than oral contraceptive agents and postmenopausal hormone replacement therapy) e.g., glucocorticoids, androgens, or growth hormones 9. Use of thyroid supplements (levothyroxine, liothyronine, etc.) or any preparation containing thyromimetic agents within 24 weeks prior to study entry 10. History of coagulopathy or use of anticoagulants such as warfarin 11. Unstable endocrine/metabolic syndrome that may affect lipid metabolism 12. History of atrial or ventricular arrhythmia 13. Diagnosis of other non-cardiac underlying medical conditions expected to impact mortality within 24 weeks after randomization | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-39.0, McCune-Albright Syndrome Children with MAS ranging from toddlers to young adults Diagnosis of MAS will be made on a clinical basis. Blood testing is not helpful in this condition, as bone marrow progenitor cells with the Gαs mutation display a survival disadvantage. All patients are mixed chimeras, as this mutation is lethal if it occurs in germline cells Patients who exhibit two or more of the following clinical findings fit the diagnosis of MAS GnRH independent precocious puberty Polyostotic fibrous dysplasia Café-au-lait spots with coast of Maine borders and respect for the midline Non-autoimmune hyperthyroidism Ten controls will also be recruited from family members of patients with MAS with no known allergies. An additional control group of ten unrelated subjects, also with no known allergies, will be recruited from the Endocrine Clinic for comparison Any MAS patient or control who has not, or cannot, discontinue(d) any home regimens of antihistamines or glucocorticoids (including inhaled steroids) at least seven days prior to skin testing Any MAS patient or control on tricyclic antidepressants within two weeks prior to skin testing | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-999.0, Mastocytosis Confirmed or suspected diagnosis of mastocytosis Ability to give informed consent (by the patient or legal guardian if minor) Inability or not willing to provide informed consent | 2 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Cancer of Head and Neck Histologically proved locally advanced squamous cell carcinoma of the head and neck of all primary sites. The following TNM stages by sites will be eligible.Oral cavity, Pharynx, Larynx, Nasopharynx, paranasal sinuses, Oral cavity, Pharynx, Larynx, Nasopharynx, paranasal sinuses T4 N0 N1 N2-A,B,C N3, T3 N0 N1 N2-A,B,C N3 Any T N2-A,B,C N3 Unknown primary Tx N2-A,B,C N3 Note: Only clearly unresectable T4 N0 lesions are eligible for study provided the reasons for unresectability are due to extensive anatomic involvement and are outlined by the surgeon 2. Patients must have signed an approved informed consent. 3. Patients with Performance Status 0-2. 4. No evidence of distant metastatic disease. 5. No previous radiation therapy. 6. No previous chemotherapy. 7. Patients must be greater than 18 years of age. 8. Women of child bearing potential (WOCBP) must have a negative pregnancy test within 7 days of treatment. Patients are considered not of child bearing potential if they are surgically sterile (they have undergone a hysterectomy, bilateral tubal ligation, or bilateral oophorectomy) or they are postmenopausal. 9. Pretreatment evaluations History and physical examination within four weeks prior to study entry Dental evaluation Medical oncology examination to evaluate medical contraindications prior to start of chemotherapy 10. Adequate renal & bone marrow function determined by the following laboratory parameters: ANC greater than or equal to 1500/mm3; platelets greater than or equal to 100,000/mm3; hemoglobin greater than or equal to 8.0 g/dl; Serum Creatinine less than or equal to 2.0 mg/dl, Total bilirubin less than 1.5 X the ULIN; AST/ALT less than 3 times the ULN, Creatinine Clearance greater than or equal to 50 cc/min 11. Evidence of measurable disease. 12. No evidence of concomitant malignancy except for non-melanomatous skin cancer (controlled or controllable) or carcinoma in situ of the cervix Any of the following will make the patient ineligible to participate in this study: 1. Acute hepatitis or known HIV. 2. Active or uncontrolled infection. 3. Significant history of concomitant life threatening / uncontrolled cardiac disease; i.e., uncontrolled hypertension, unstable angina, recent myocardial infarction (within prior 6 months), uncontrolled congestive heart failure, and known cardiomyopathy with decreased ejection fraction, cardiac arrhythmia 4. Prior therapy which specifically and directly targets the EGFR pathway. 5. Prior severe infusion reaction to a monoclonal antibody. 6. Any concurrent chemotherapy not indicated in the study protocol or any other investigational agent(s). 7. Women of childbearing potential (WOCBP) and male participants who are unwilling or unable to use an effective method to avoid pregnancy for the entire study period 8. Preexisting clinically significant neuropathy. 9. Patients with loco-regional recurrences from any site with no prior radiation therapy and not amenable for salvage surgery are not eligible for study | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-90.0, Heart Failure, Congestive Patients hospitalized due to decompensated heart failure who require IV inotropic and/or vasodilator and diuretic therapy 2. Patients receiving appropriate medical therapy for heart failure, defined as ACE inhibitors and beta blockers (all unless not tolerated or contraindicated) and diuretics, for 1 month prior to study entry 3. For at least 24 hours prior to into the study, the patient should be treated with a minimum dose of the following: dobutamine 2.5μg/kg/min or milrinone 0.3μg/kg/min or dopamine 5μg/kg/min. or nesiritide 0.01μg/kg/min or nitroglycerin 0.3 µg/kg/min or nitroprusside 0.3 µg/kg/min or a combination of any of these agents, with diuretic therapy. Doses of the above stated medications should be stable for 6 hours prior to into study. An increase in this dosage within the first 8 hours after enrollment is determined by the attending physician to be unlikely and the following definition of "not responding adequately to IV inotropic and/or vasodilator and diuretic therapy" is exhibited PCWP is ≥ 20 mmHg at time of randomization and PCWP was ≥ 18 mmHg continuously for 24 hours or PCWP ≥ 20 mmHg continuously for 12 hours prior to randomization Cardiac Index < 2.4 L/min/m2 There is evidence for abnormal renal function and/or diuretic resistance defined as: Serum creatinine > 1.2 mg/dL or Diuretic dosage of intravenous Furosemide ≥ 120 mg daily, or equivalent 4. LVEF < 35% 5. Male or female 18-90 years of age 6. If female, no child-bearing potential or negative pregnancy test 7. Written informed consent 8. Willingness to participate in required follow-up exams Acute Q-wave myocardial infarction within past 7 days 2. Post cardiotomy shock within past 30 days 3. Cardiac surgery within past 14 days 4. Bridge to transplant 5. History of severe COPD as defined as FEV1 < 1.0 liter 6. History of malignant arrhythmias defined as either sustained ventricular tachycardia > 15 beats or more in length, not associated with a correctable cause, within the preceding 3 months, unless the patient presently has an implantable cardiac defibrillator history of ventricular fibrillation or sudden death, unless the patient presently has an implantable cardiac defibrillator 7. Patients implanted with a resynchronization device within the previous 14 days or if there is a possibility of implant within 60 days following randomization 8. Systolic pressure <80 mmHg 9. Requiring cardiopulmonary support type devices 10. Platelets < 50,000/mm3 or other evidence of coagulopathy, INR greater than 1.5 in the absence of anticoagulation therapy. 11. Infection (WBC ≥ 12.5 x 103/ml, and or temperature ≥ 100.5°F/38°C) 12. History of cerebral vascular accident (CVA) or transient ischemic attacks (TIA) within the last 3 months 13. Unwilling or unable to receive blood transfusion 14. Inability to undergo treatment with heparin 15. Patients on dialysis or serum creatinine > 4.0 mg/dl 16. Primary liver disease with bilirubin, SGOT, or SGPT > 4X upper limit of normal 17. Life expectancy from other disease < 12 months 18. Patients who are active on the cardiac transplantation waiting list, unless there is a documented reason (large body habitus, highly sensitized, O blood group) to anticipate that transplant is unlikely within the subsequent 65 days. 19. Symptomatic patent foramen ovale or intracardiac shunt 20. Patients diagnosed with clinically significant peripheral vascular disease, defined as absent pedal pulse or signs or symptoms of limb ischemia, including a history of intermittent claudication 21. Patients with amyloidosis, thyroid induced heart failure, high output failure secondary to an arterio-venous fistula, and significant uncorrected primary valvular disease (with the exception of mitral regurgitation believed secondary to LV dilatation) | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 3.0-10.0, Myocardial Repolarization Health children (ASA I-II) undergoing procedures that require general anesthesia without pre-medication Children with long QT syndrome, a family history of long QT syndrome or taking medication that is known to prolong the QT interval IV access unobtainable pre-operatively | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 12.0-65.0, Asthma Will meet American Thoracic Society for asthma 2. Pulmonary function tests consistent with asthma. This includes a baseline FEV1 < 80% predicted as well as a 12% improvement in FEV1 following up to 4 puffs of albuterol. 3. Subjects must be 12 to 65 years old Viral infection within four weeks of the starting date. 2. Abnormal hepatic function. 3. History of COPD 4. Pregnancy. 5. History of smoking. 6. Anemia (hemoglobin less than 12 gm %) 7. Concurrent therapy with anticonvulsants, erythromycin, rifampin and any systemic asthma medication including Singular®, Xolair® or oral prednisone. 8. Greater than 500 mcg per day of inhaled corticosteroids 9. Suspected non-compliance with medical care. 10. Abnormal prednisone pharmacokinetics (applies to phase 2 of trial) 11. Patients with severe medical conditions that in the view of the investigator prohibits participation in the study (specify as required) 12. Use of any investigational agent in the last 30 days | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Low Back Pain At least 18 years of age History of chronic, function-limiting low back pain of at least 6 months duration Able to give voluntary, written informed consent to participate Able to understand the investigation, cooperate with the procedures, and willing to return for follow-up No recent surgical procedures within last three months Cauda Equina symptoms and/or compressive radiculopathy Narcotic use of no greater than 100mg/day hydrocodone, 60mg Methadone. or 100mg morphine Uncontrolled major Depression or uncontrolled psychiatric disorder Uncontrolled or acute medical illnesses Chronic severe conditions that could interfere with outcome assessments Women who are pregnant or lactating Subjects who have participated in a clinical study with an investigational product within 30 days of enrollment Patients with multiple complaints involving concomitant hip osteoarthritis Inability to achieve proper positioning and inability to understand informed consent and protocol History of adverse reaction to local anesthetic or anti-inflammatory drugs and history of gastrointestinal bleeding or ulcers | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 0.0-0.096, Vitamin D Deficiency Healthy singleton term born infants of appropriate size for gestational age (AGA) according to the Centers for Disease Control growth charts based on weight at birth between the 5th and 95th percentiles for sex; and born to healthy breastfeeding women. This allows us to assess growth and bone mineral accretion without bias of intrauterine growth restriction Infants of mothers with history of gestational diabetes or hypertension in pregnancy; chronic alcohol use; malabsorption syndromes due to malabsorption of vitamin D (celiac disease, Crohn's etc.); and multiple births Should any be identified after recruitment, the infant will be excluded from the main analyzes, but followed in a separate arm as intent-to-treat | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Pain, Postoperative Over 18 years old Scheduled (within two weeks of the screening visit) to undergo elective orthopedic surgery (e.g., bunionectomy, arthroscopic knee surgery, rotator cuff repair) Moderate to severe pain within 8 hours following completion of the required surgery Previous anaphylactic or serious allergic reaction to shellfish or opioids History of sleep apnea Other Inclusion/ May Apply | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Breast Neoplasms Patients with histologically confirmed HER-2/neu-overexpressing adenocarcinoma of the breast; this is defined as HER-2+ by immunohistochemistry (IHC) 3+ staining or Fluorescence In-Situ Hybridization (FISH). Prior adjuvant Trastuzumab therapy is permitted. Patients must not be eligible for therapy of known curative potential for metastatic breast cancer if it is identified during the course of the study. 2. Patients may have measurable or evaluable disease. 3. Stable central nervous system (CNS) disease that has been adequately treated and is not under active treatment allowed. 4. Age 18 years or older. 5. Able to give informed consent. 6. Patients with an Eastern Cooperative Oncology Group (ECOG) performance score of 0 or 1. 7. No systemic oral steroids administered within 28 days prior to initiating treatment on protocol. Topical, ocular, and nasal steroids are allowed, as are those applied to mucus membranes. 8. No prior or currently active autoimmune disease requiring management with systemic immunosuppression. This includes inflammatory bowel disease, systemic vasculitis, scleroderma, psoriasis, multiple sclerosis, hemolytic anemia or immune-mediated thrombocytopenia, rheumatoid arthritis, systemic lupus erythematosus, Sjogren's syndrome, sarcoidosis, or other rheumatologic disease. Asthma or chronic obstructive pulmonary disease that does not require daily systemic corticosteroids is acceptable. 9. Not pregnant, and on appropriate birth control if of child-bearing potential. 10. No history of other malignancies within the prior five years (excluding a history of carcinoma in situ of the cervix, superficial non-melanoma skin cancer, and superficial bladder cancer). 11. Adequate bone marrow reserve with absolute neutrophil count (ANC) > 1000 and platelets > 100,000. 12. Adequate renal function with serum creatinine < 2.0. 13. Adequate hepatic reserve with serum bilirubin < 2.0, aspartate transaminase (AST) and alanine aminotransferase (ALT) < 2X the upper limit of normal, and alkaline phosphatase < 5X the upper limit of normal. Serum bilirubin > 2.0 is acceptable in the setting of known Gilbert's syndrome. 14. Adequate cardiac reserve with a cardiac ejection fraction within the lower limit of facility normal by MUGA, or 45% by echocardiogram. 15. No active major medical or psychosocial problems that could be complicated by study participation. 16. HIV negative No histologic documentation of breast adenocarcinoma. 2. Breast adenocarcinoma that is not amplified for HER-2/neu gene expression by at least 2-fold by FISH analysis, or that is less than IHC 3+ when FISH negative. 3. Cardiac dysfunction documented by an ejection fraction less than the lower limit of the facility normal by multi-gated acquisition (MUGA) scan, or 45% by echocardiogram. 4. Symptomatic intrinsic lung disease or extensive tumor involvement of the lungs resulting in dyspnea at rest. 5. History of autoimmune disease as detailed above. 6. Systemic oral corticosteroid treatment within 28 days prior to initiating treatment on study. 7. Uncontrolled medical problems. 8. Evidence of active acute or chronic infection. 9. Chemotherapy, radiation therapy, or biologic therapy (except Trastuzumab) within 28 days prior to initiating treatment on study. Hormonal therapy and supportive therapy with bisphosphonates will be allowed. 10. Participation in an investigational new drug trial within 28 days prior to initiating treatment on study. 11. Pregnant or breast feeding. 12. Hepatic, renal, or bone marrow dysfunction as detailed above. 13. Concurrent malignancy or history of other malignancy within the last five years except as noted above. 14. Corn allergy. 15. Known severe hypersensitivity to Trastuzumab (excluding mild to moderate infusion reactions that are easily managed and do not recur) | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Post-surgical Inflammation Patients scheduled for cataract extraction Under 18 years | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Acute Coronary Syndrome Patients who have undergone PCI within the preceding 72 hrs 2. Patients on aspirin and clopidogrel 3. Age greater than 18 years and less than 80 years 4. Written informed consent Active bleeding 2. Pregnancy 3. Patients already on H2 antagonists 4. Patients already on PPI 5. Patients with implanted cardiac defibrillator (ICD) 6. Patients with GI strictures, swallowing disorders, or bowel obstruction and fistulas. 7. Patients with significant gastrointestinal diverticular disease 8. Patients likely to require MRI imaging during the time the PillCam is still in the digestive tract (8 to 72 hours after the PillCam procedure) | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-70.0, Renal Transplantation Primary donor kidney transplant Panel reactive antibody (PRA) ≤ 20% Multi-organ transplantation including dual kidneys or previous transplant with any other organ different from kidney Non-heart beating donor or kidney from a non-compatible donor Other protocol-defined inclusion/ | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Cystic Fibrosis Phlebitis Diagnosis of cystic fibrosis, made accordingly to the Cystic Fibrosis Foundation Guideline (Rosenstein BJ. J Pediatr 1998;132: 589-595) age of 18 years or more and ability to consciously express owns informed consent have a prescription done by one of the CF Centre specialist Physicians of an IV antibiotic course of the expected duration of 2 weeks, due to a pulmonary exacerbation, with the association of ceftazidime 3 times daily and tobramycin once daily diluted in Normal Saline absence of clinical conditions that contraindicate the administration of 350ml of Normal Saline in 30 minutes 3 times daily and of 400ml of Normal Saline in 40 minutes no simultaneous anti-inflammatory therapy administered orally, IM or IV days have passed from the end of the previous course The IV course will be given to the subject as an inpatient, and he or she will be admitted to our hospital | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-80.0, Pemphigus Vulgaris Histologic evidence compatible with pemphigus vulgaris and direct immunofluorescence evidence of pemphigus vulgaris Chronic disease that has been controlled with steroids and/or cytotoxics, e.g. maintenance phase On prednisone 15 or more mg/day to around 40 mg/day or on prednisone 15 or more mg every other day (qod) to around 40 mg qod Failure to taper steroids below a range of 15 mg/day to around 40 mg/day or 15 mg/qod to around 40 qod without flaring the disease The steroid dosage at which the most recent flare occurred should not be less than 85% of the last (within 30 days) dosage which controlled the disease, i.e. 85% of the baseline steroid dosage. This is to ensure that patients will not have had a recent acute flare at the time of entry into the study, and be in the rapid steroid taper portion of their disease after such a flare Two baseline steroid dosages as determined by prior flares. It is common that patients will be repetitively unable to taper below a certain baseline steroid dose without experiencing a mild flare of their disease. This baseline dose will be determined on two occasions during attempted tapers, and the baseline number then averaged to determine the dose of steroid the patient is on at the time of entry into the study No pulse steroids, pulse cyclosphosphamide, or plasmapheresis within two months of beginning the protocol. This will patients who had recent acute flares of their disease and may be on the rapid steroid taper portion of their disease. The patient must be in maintenance phase, as defined in the listed in e Patient understands the procedures and agrees to participate in the study program by giving written informed consent Patients able to taper steroids without recurrence of disease Patients with early, severe disease that have not responded to high doses of prednisone, cytotoxics, plasmapheresis, or other modalities Contraindications to the use of Dapsone, including severe anemia or G6PD deficiency Patient has behavioral problems that might interfere with compliance Pregnancy or breast-feeding Younger than 18 or older than 80 years of age. Since PV is rare in patients younger than 18, it was decided to this potentially different population. It is unlikely that this will many patients. Dapsone induces a hemolytic anemia, which would be a particular problem for patients over age 80, who are more likely to have ischemic heart disease or other atherosclerotic vascular disease History of allergy to dapsone Ischemic heart disease | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Hypertension Aged over 18 years Hypertension (essential or secondary) managed in Sheffield Hypertension Clinic or general practice or both Blood pressures not adequately controlled (systolic blood pressure (SBP) >140 mmHg and/or diastolic blood pressure (DBP) >85 mmHg in clinic and on ambulatory blood pressure monitoring) despite treatment with the maximum tolerated dose of three antihypertensive agents Additional antihypertensive treatment deemed appropriate by the patients' doctor Patients' current antihypertensive treatment includes a thiazide diuretic and at least one of a beta-blocker, angiotensin converting enzyme inhibitor or angiotensin II receptor antagonist Definite indication or contraindication for spironolactone Known Conn's syndrome (definite indication for spironolactone) Heart failure NYHA class III or IV (definite indication for spironolactone) Known hepatic failure or significant cirrhosis Known pregnancy or women planning pregnancy Women of child bearing potential not using adequate contraceptive methods Serum creatinine > 221µmol/l Serum Potassium > 5.0mmol/l Clinic blood pressure or daytime ambulatory blood pressure >240/120 | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-50.0, Healthy Healthy subjects (Men or women of nonchildbearing potential ), aged 18 to 50 years Body mass index in the range of 18 to 30 Kg/m2 and body weight greater than 50 Kg Healthy as determined by the investigator on the basis of medical history, physical examination, clinical laboratory test results, vital signs, and 12-lead electrocardiogram | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Systemic Mastocytosis Patients with SM; including mast cell leukemia. 2. Age >/= 18 years 3. Minimum of two weeks since any major surgery or completion of radiation. 4. Eastern Cooperative Oncology Group (ECOG) performance status </= 2 5. Adequate liver function as shown by serum bilirubin </= 1.5 x upper limit of normal (ULN), and serum Alanine transaminase (ALT) </= 3 x ULN 6. Prothrombin Time (PT)/Partial thromboplastin time (PTT)/International normalized ratio (INR) within normal institutional limits 7. Signed informed consent Treatment with any conventional (specifically, interferon or cladribine) or investigational medicine for SM within the preceding 4 weeks 2. Chronic treatment with systemic steroids or another immunosuppressive agent 3. Other malignancies within the past 3 years except for adequately treated carcinoma of the cervix or basal or squamous cell carcinomas of the skin, unless patient has SM-associated clonal hematologic disease that does not require therapy, as judged by treating physician and approved by principal investigator. 4. Other concurrent severe and/or uncontrolled medical disease which could compromise participation in the study as judged by the Principal Investigator (i.e., severely impaired lung function, uncontrolled diabetes, uncontrolled hypertension, severe infection, severe malnutrition, unstable angina, or congestive heart failure New York Heart Association Class III or IV, ventricular arrhythmias, active ischemic heart disease, myocardial infarction within six months, chronic liver or renal disease, active upper GI tract ulceration) 5. A known history of Human immunodeficiency virus (HIV) seropositivity 6. Impairment of gastrointestinal function or gastrointestinal disease that may significantly alter the absorption of RAD001 as judged by the Principal Investigator (e.g., ulcerative disease; uncontrolled nausea, vomiting or diarrhea; malabsorption syndrome or small bowel resection) 7. Patients with a bleeding diathesis or on oral anti-vitamin K medication 8. Women who are pregnant or breast feeding, or women/men able to conceive and unwilling to practice an effective method of birth control (women of childbearing potential must have a negative urine or serum pregnancy test within 48 hours prior to administration of RAD001; protocol definition of post-menopausal women is: 12 months of natural (spontaneous) amenorrhea or 6 months of spontaneous amenorrhea with serum FSH levels > 40 mIU/m or 6 weeks post-surgical bilateral oophorectomy with or without hysterectomy) 9. Patients who have received prior treatment with an mTOR inhibitor (e.g., sirolimus, temsirolimus) 10. Patients with a known hypersensitivity to RAD001 (everolimus) or other rapamycins (sirolimus, temsirolimus) or to its excipients 11. Patients unwilling to or unable to comply with the protocol | 1 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Graft Rejection Low risk keratoplasty High risk keratoplasty | 0 |
Pt is a 22yo F otherwise healthy with a 5 yr history of the systemic mastocytosis, with flares normally 3/year, presenting with flushing and tachycardia concerning for another flare. This is patient's 3rd flare in 2 months, while still on steroid taper which is new for her. She responded well to 125 mg IV steroids q 8 hrs and IV diphenydramine in addition to her continuing home regimen. CBC was at her baseline, w/normal differential. Serum tryptase revealed a high value at 84. The patient failed aspirin challenge due to adverse reaction. She was stabilized on IV steroids and IV benadryl and transferred back to the medical floor. She continued on her home histamine receptor blockers and was transitioned from IV to PO steroids and benadryl and observed overnight and was discharged on her home meds, prednisone taper, GI prophylaxis with PPI, Calcium and vitamin D, and SS bactrim for PCP. | eligible ages (years): 18.0-999.0, Coronary Artery Disease Drug Resistance Patients undergoing elective coronary stenting Pretreatment with a bolus dose of 600mg of clopidogrel prior to coronary stent implantation Pretreatment with aspirin ≥ 100 mg per day for at least 7 days Age > 18 years Written consent Troponin T on admission > 0.03 ng/mL Myocardial infarction or fibrinolytic therapy within the previous 14 days Cardiogenic shock Contraindication for aspirin or clopidogrel Oral anticoagulation Pretreatment with heparin or a thienopyridine within the previous 14 days Use of a GP IIb/IIIa-receptor antagonist during PCI Platelet count < 100.000/µl Severe disorders of the coagulation system Severe impairment of liver or kidney function | 0 |
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