Clinical trials: Hyper IgE Syndromes
DOCK8 Deficiency
Pilot Study of Reduced-Intensity Hematopoietic Stem Cell Transplant of DOCK8 Deficiency
Background: -DOCK8 deficiency is a genetic disorder that affects the immune system and can lead to severe recurrent infections and possible death from infections or certain types of cancers, including blood cancers.
- Trial name
- NCT01176006
- Testing
- Reduced-intensity hematopoietic stem cell, Fludarabine(Fludara, Berlex Laboratories), Cyclophosphamide(CTX, Cytoxan)
- Where
- United States
- Volunteers
- 76 people, healthy volunteers welcome
- Sponsor
- National Cancer Institute (NCI)
Chediak Higashi Syndrome, Chronic Granulomatous Disease
Learning and Behavior Problems in Children With Chronic Granulomatous Disease and Related Disorders
This study will try to determine what causes learning, behavioral and emotional problems in children with chronic granulomatous disease (GCD) and other phagocyte disorders.
- Conditions
- Chediak Higashi Syndrome, Chronic Granulomatous Disease, Job's Syndrome, Leukocyte Disorder
- Trial name
- NCT00005933
- Where
- United States
- Volunteers
- 150 people
- Sponsor
- National Institute of Allergy and Infectious Diseases (NIAID)
Primary Immunodeficiencies, APECED
Participation in a Research Registry for Immune Disorders
Background: People with primary immune deficiency diseases (PIDD) have weak immune systems. This makes it hard for their bodies to fight infection.
- Conditions
- Primary Immunodeficiencies, APECED, CGD, Wiskott-Aldrich Syndrome
- Trial name
- NCT01953016
- Where
- United States
- Volunteers
- 716 people
- Sponsor
- National Human Genome Research Institute (NHGRI)
Job Syndrome
NFIL3-induced Pathological Enhancement of IgE Class Switch Recombination in Hyper-IgE Syndrome
Hyper IgE syndrome (HIES) is a rare and complex primary immunodeficiency that affects multiple systems. It is characterized by elevated Immunoglobulin E(IgE), recurrent skin and pulmonary infections and eczematoid…
- Trial name
- NCT02228941
- Volunteers
- 40 people, healthy volunteers welcome
- Sponsor
- Shanghai Children's Medical Center
Granulomatous Disease, Chronic, Job's Syndrome
Posaconazole to Treat Invasive Fungal Infections
This study will evaluate the safety and effectiveness of posaconazole for treating invasive fungal infections. New therapies for these infections are needed for patients who do not respond, to or cannot tolerate, standard…
- Conditions
- Granulomatous Disease, Chronic, Job's Syndrome, Infection
- Trial name
- NCT00033982
- Testing
- Posaconazole
- Where
- United States
- Volunteers
- 50 people
- Sponsor
- National Institute of Allergy and Infectious Diseases (NIAID)
JOB's Syndrome, Hyper-IgE Recurrent Infection Syndrome
Effect of Ranitidine on Hyper-IgE Recurrent Infection (Job's) Syndrome
This study will examine the safety and effectiveness of ranitidine (Zantac) in patients with Hyper-IgE recurrent infection syndrome, a disease characterized by recurrent infections of the ears, sinuses, lungs and skin, and…
- Conditions
- JOB's Syndrome, Hyper-IgE Recurrent Infection Syndrome, Immune Deficiency
- Trial name
- NCT00527878
- Testing
- Ranitidine
- Where
- United States
- Volunteers
- 16 people
- Sponsor
- National Institute of Allergy and Infectious Diseases (NIAID)
Severe Combined Immunodeficiency (SCID), Immunodeficiency With Predominant T-cell Defect, Unspecified
Sequential Cadaveric Lung and Bone Marrow Transplant for Immune Deficiency Diseases
The purpose of this study is to determine whether bilateral orthotopic lung transplantation (BOLT) followed by cadaveric partially-matched hematopoietic stem cell transplantation (HSCT) is safe and effective for patients aged…
- Conditions
- Severe Combined Immunodeficiency (SCID), Immunodeficiency With Predominant T-cell Defect, Unspecified, Severe Chronic Neutropenia, Chronic Granulomatous Disease (CGD)
- Trial name
- BOLT+BMT (NCT01852370)
- Testing
- CD3/CD19 negative allogeneic hematopoietic stem cells
- Where
- United States
- Volunteers
- 16 people
- Sponsor
- Paul Szabolcs
Infections, Pneumonia
Natural History, Management, and Genetics of the Hyperimmunoglobulin E Recurrent Infection Syndrome (HIES)
The Hyper IgE Syndromes (HIES) are primary immunodeficiencies resulting in eczema and recurrent skin and lung infections. Autosomal dominant Hyper IgE syndrome (AD-HIIES; Job's syndrome) is caused by STAT3 mutations, and is a…
- Conditions
- Infections, Pneumonia, Immune System Diseases, STAT3 Transcription Factor
- Trial name
- NCT00006150
- Where
- United States
- Volunteers
- 600 people, healthy volunteers welcome
- Sponsor
- National Institute of Allergy and Infectious Diseases (NIAID)
Candidiasis, Chronic Mucocutaneous
CAMB/MAT2203 in Patients With Mucocutaneous Candidiasis
This is an open-label, dose-titration trial to study the efficacy, safety, and pharmacokinetics of oral cochleate amphotericin B (CAMB) in the treatment of mucocutaneous candidiasis infections in patients who are refractory or…
- Trial name
- CAMB (NCT02629419)
- Testing
- Amphotericin B
- Where
- United States
- Volunteers
- 4 people
- Sponsor
- Matinas BioPharma Nanotechnologies, Inc.
Hyper-IgE Syndrome, Job's Syndrome
Omalizumab to Treat Hyper-IgE (Job's) Syndrome
This study will evaluate the safety and effectiveness of a laboratory-made antibody called omalizumab in patients with Job's syndrome, or hyper-IgE syndrome (HIES).
- Trial name
- NCT00260702
- Testing
- Omalizumab (Xolair)
- Where
- United States
- Volunteers
- 1 people
- Sponsor
- National Institute of Allergy and Infectious Diseases (NIAID)
Autosomal-dominant Hyper-IgE Syndrome
Safety, Tolerability, and Immunogenicity of One Dose of NDV 3A Vaccine in People With STAT3-Mutated Hyper-IgE Syndrome
Background: AD-HIES is a disease that weakens the immune system. It puts people at risk for infections, particularly Staph and Candida infections.
- Trial name
- NCT02996448
- Testing
- NDV-3A
- Where
- United States
- Volunteers
- 3 people, healthy volunteers welcome
- Sponsor
- National Institute of Allergy and Infectious Diseases (NIAID)
Bronchogenic Carcinoma, Chronic Granulomatous Disease
Diagnostic Effectiveness of Virtual Bronchoscopy
This study will evaluate a new technique for examining the air passages of the lungs called "virtual bronchoscopy." It involves using computed tomography (CT) images of the chest to generate a 3-dimensional model of the walls of…
- Conditions
- Bronchogenic Carcinoma, Chronic Granulomatous Disease, Job's Syndrome, Mycobacterium Infection
- Trial name
- NCT00001515
- Testing
- virtual bronchoscopy
- Where
- United States
- Volunteers
- 120 people
- Sponsor
- National Institutes of Health Clinical Center (CC)
Hyper IgE Syndrome From STAT3 Mutation, Job s Syndrome
Evaluating the Safety and Tolerability of Baricitinib in Patients With Job Syndrome With Lupus-Like Disease and/or Atopic Dermatitis
Background: Autosomal dominant hyper-IgE syndrome (HIES), also called Job syndrome, is a genetic disorder that affects the immune system.
- Conditions
- Hyper IgE Syndrome From STAT3 Mutation, Job s Syndrome, HIES, Lupus
- Trial name
- NCT07262983
- Testing
- baricitinib
- Where
- United States
- Volunteers
- 20 people
- Sponsor
- National Institute of Allergy and Infectious Diseases (NIAID)
Hypercholesterolemia, Hyperbilirubinemia
Study of Efficacy and Safety of the Plasmapheresis Method With Albumin Compensation Compared With the Plasmapheresis Method Without Albumin Compensation for Aging Biomarkers Correction in Men and Women Aged 40 to 55 Years Old
Reaching active aging makes it important to implement new methods affecting the biological age of a person. Biochemical parameters of a blood test are aging biomarkers that are ones of the most accessible for testing.
- Conditions
- Hypercholesterolemia, Hyperbilirubinemia, Hyper IgE Syndrome, Hyper Beta Lipoproteinemia
- Trial name
- NCT04897113
- Testing
- plasma exchange with albumin, plasma exchange without albumin
- Where
- 2 sites in Russia
- Volunteers
- 80 people, healthy volunteers welcome
- Sponsor
- National Medical Research Center for Rehabilitation and Balneology