Effect of Ranitidine on Hyper-IgE Recurrent Infection (Job's) Syndrome

• Register: ClinicalTrials.gov
• Last refreshed on: 19 October 2017
• Main ID: NCT00527878
• Date of registration: 08/09/2007
• Prospective Registration: No
• Primary sponsor: National Institute of Allergy and Infectious Diseases (NIAID)
• Public title: Effect of Ranitidine on Hyper-IgE Recurrent Infection (Job's) Syndrome
• Scientific title: A Double-Blind, Randomized, Placebo-Controlled Cross-Over Study Assessing the Role of Pathogen-Specific IgE and Histamine Release in the Hyper-IgE Syndrome and the Effect of Ranitidine on Laboratory and Clinical Manifestations
• Date of first enrolment: September 2007
• Target sample size: 16
• Recruitment status: Terminated
• URL: https://clinicaltrials.gov/show/NCT00527878
• Study type: Interventional
• Phase: Phase 2

Countries of recruitment

United States

Key inclusion & exclusion criteria

- INCLUSION CRITERIA:

1. Male and female patients with the diagnosis of Hyper IgE Recurrent Infection
(Job) syndrome. Mutations in the STAT3 gene account for the majority, if not all
cases of HIES. However as the full genetics of HIES remains unknown, we will use
clinical criteria, including the expert opinion of the investigators, as well as
a score greater than 40 by the diagnostic scoring system used in protocol
00-I-0159.

2. A chronic (greater than 4 weeks duration) infection or greater than 2 acute
infections within the last 12 months. Acute infections can include but are not
limited to: pneumonia, abscesses, sinusitis, skin infections, mucocutaneous
candidiasis and ear infections. Chronic infections include continuous or
intermittent symptoms despite appropriate therapeutic interventions for at least
4 weeks, including but not limited to chronic lung infiltrates with productive
cough, chronic ear drainage despite topical therapy, chronic or intermittent
drainage from a single abscess site, and/or chronic signs of sinusitis on sinus
CT scan.

3. Patients aged 2 years and above. There is no upper age limit. We are excluding
children less than 2 years of age, as we do not expect them to meet the first
inclusion criterion, having a score high enough to be diagnosed with HIES.

4. Patients have to be at their own personal clinical baseline for at least 2 weeks
duration. Patients will not start the study medication during an acute
exacerbation of and infection.

5. The patient or the patient's guardian will be willing and capable of providing
informed consent after initial counseling by clinical staff. Separate consent
forms for all interventional procedures will be obtained after explanation of the
specific procedure.

6. Patients must agree to have blood stored for future studies of the immune system
and/or other medical conditions.

7. Women of childbearing potential must agree to use adequate contraception
(hormonal or barrier method of birth control; abstinence) prior to study entry
and for the duration of study participation.

8. Patients may be concurrently enrolled on other protocols as long as the Principal
Investigator (PI) is informed.

EXCLUSION CRITERIA:

1. Pregnancy. Ranitidine is pregnancy class B, and likely safe in pregnancy, but as this
has not been studied, pregnant patients will be excluded. In addition, hormonal
changes that occur during pregnancy may affect the skin manifestations and frequency
of infection.

2. Hypersensitivity to ranitidine or any of the ingredients in ranitidine.

3. Pre-existing medications or conditions for which the investigators judge that
ranitidine should not be given.

4. Patient or investigators unwilling to stop baseline H2 receptor antagonist therapy
(over the counter or prescription) such as Tagamet (Cimetidine), Pepcid (Famotidine),
and Axid (Nizatidine). H2 receptor antagonist therapy must be stopped for 3 months
prior to study initiation. Patients who are receiving H2 receptor antagonist therapy
for gastritis, acid reflux, or peptic ulcer disease will be offered changing their
regimen to a proton pump inhibitor or other non-H2 receptor antagonist therapy to
allow for study enrollment (3 months after stopping the H2 receptor antagonist).

5. Patients under the age of 2 years

6. Patients with HIV, receiving chemotherapy or who have a malignancy.

7. Any condition that in the judgment of the investigator would place the subject at
undue risk or compromise the results or interpretation of the study.



Age minimum: 2 Years
Age maximum: N/A
Gender: All

Health Condition(s) or Problem(s) studied

Hyper-IgE Recurrent Infection Syndrome

JOB's Syndrome

Immune Deficiency

Intervention(s)

Drug: Placebo

Drug: Ranitidine

Primary Outcome(s)

Number of Infections in Subjects With HIES. [Time Frame: 1 year on intervention]

Secondary Outcome(s)

New Lung Infections [Time Frame: 12 months placebo and 12 months ranitidine]

Clinical Severity Score [Time Frame: one year on ranitidine and one year on placebo]

New Skin Infections [Time Frame: 12 months placebo/12 months ranitidine]

Secondary ID(s)

07-I-0218

070218

Source(s) of Monetary Support

Please refer to primary and secondary sponsors

Results

• Results available: Yes
• Date Posted: 04/02/2013
• URL: https://clinicaltrials.gov/ct2/show/results/NCT00527878