- ICH GCP
- US Clinical Trials Registry
- Clinical Trial NCT00005933
Learning and Behavior Problems in Children With Chronic Granulomatous Disease and Related Disorders
Cognitive Function in Leukocyte 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. (Phagocytes are a type of white blood cell.) Children with these disorders have frequent severe infections that require hospitalization, sometimes for long periods of time. Many of them also have problems with school, learning, behavior, anxiety and depression. This study will explore whether these latter problems are a direct result of the illness itself or are a consequence of frequent, long hospitalizations, or are due to other factors. Test findings in these children will be compared with those of children with cystic fibrosis-another disease that causes frequent infections requiring prolonged hospitalization.
Patients age 2 or older with GCD or other phagocytic disorders or cystic fibrosis may be eligible for this study. Participants (or a parent or guardian) will complete questionnaires including personal information such as age, gender and marital status, a family medical history, and information on their illness. Patients will be given various psychological and intelligence tests, and they and their parents or guardians will be interviewed by a child psychiatrist. The tests and interviews take a total of about 5 hours and are given in two or three separate sessions.
The tests may reveal problems such as learning disorders, attention-deficit hyperactivity disorder, anxiety, or depression. If any of these problems are identified, appropriate referrals will be made for specialized services, such as special school placement, tutoring, or counseling.
Study Overview
Status
Detailed Description
Leukocyte disorders are predominantly genetic diseases in which phagocytes fail to function normally resulting in recurrent infections. Children with these disorders are subject to recurrent, severe, often life-threatening infections and are hospitalized more frequently than their peers. Frequent hospitalization and chronic illness can affect growth, development, socialization, and educational opportunities.
Specifically, chronic granulomatous disease (CGD) is an inherited disorder in which phagocytes fail to generate an oxidative burst. In 26 patients followed at the NIH tested for behavioral problems at the request of a parent or staff member, we have observed a 23% rate of mild mental retardation. However, it is not clear whether this is due to CGD per se or the recurrent infections and hospitalizations. We seek to determine the prevalence of cognitive disabilities in children with CGD and other leukocyte disorders. We seek to determine whether abnormal leukocyte functioning may be related to specific behavioral phenotypes or impaired cognitive functioning. We also seek to clarify whether impaired cognitive functioning is related to the effects of frequent and prolonged hospitalization or other variables such as severity of illness in this population.
Study Type
Enrollment
Contacts and Locations
Study Locations
-
-
Maryland
-
Bethesda, Maryland, United States, 20892
- National Institute of Allergy and Infectious Diseases (NIAID)
-
-
Participation Criteria
Eligibility Criteria
Ages Eligible for Study
- Child
- Adult
- Older Adult
Accepts Healthy Volunteers
Genders Eligible for Study
Description
INCLUSION CRITERIA
Presence of a confirmed diagnosis of CGD or other phagocytic disorder.
Age 2 or older.
English spoken in the home.
EXCLUSION CRITERIA
Unconfirmed phagocytic disorder.
Under age 2.
First language is other than English.
Study Plan
How is the study designed?
Collaborators and Investigators
Publications and helpful links
General Publications
- Batshaw ML. Mental retardation. Pediatr Clin North Am. 1993 Jun;40(3):507-21. doi: 10.1016/s0031-3955(16)38547-9.
- Boyle IR, di Sant'Agnese PA, Sack S, Millican F, Kulczycki LL. Emotional adjustment of adolescents and young adults with cystic fibrosis. J Pediatr. 1976 Feb;88(2):318-26. doi: 10.1016/s0022-3476(76)81011-6.
- Eliez S, Reiss AL. Genetics of childhood disorders: XI. Fragile X syndrome. J Am Acad Child Adolesc Psychiatry. 2000 Feb;39(2):264-6. doi: 10.1097/00004583-200002000-00029. No abstract available.
Study record dates
Study Major Dates
Study Start
Study Completion
Study Registration Dates
First Submitted
First Submitted That Met QC Criteria
First Posted (Estimate)
Study Record Updates
Last Update Posted (Estimate)
Last Update Submitted That Met QC Criteria
Last Verified
More Information
Terms related to this study
Additional Relevant MeSH Terms
- Pathologic Processes
- Immunologic Deficiency Syndromes
- Immune System Diseases
- Lymphoproliferative Disorders
- Lymphatic Diseases
- Eye Diseases
- Disease
- Hematologic Diseases
- Genetic Diseases, Inborn
- Genetic Diseases, X-Linked
- Eye Diseases, Hereditary
- Phagocyte Bactericidal Dysfunction
- Primary Immunodeficiency Diseases
- Albinism
- Syndrome
- Granuloma
- Granulomatous Disease, Chronic
- Leukocyte Disorders
- Chediak-Higashi Syndrome
- Job Syndrome
Other Study ID Numbers
- 000164
- 00-I-0164
This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.
Clinical Trials on Chronic Granulomatous Disease
-
National Institute of Allergy and Infectious Diseases...Enrolling by invitationChronic Granulomatous Disease (CGD) | X-Linked Chronic Granulomatous DiseaseUnited States
-
University of California, Los AngelesNational Heart, Lung, and Blood Institute (NHLBI); Boston Children's Hospital; California Institute for Regenerative Medicine (CIRM) and other collaboratorsRecruitingGranulomatous Disease, Chronic, X-linkedUnited States
-
National Institute of Allergy and Infectious Diseases...Active, not recruitingChronic Granulomatous Disease TransplantUnited States
-
GenethonActive, not recruitingX-Linked Chronic Granulomatous DiseaseFrance
-
Children's Hospital of Fudan UniversityRecruitingBCG | Vitamin D3 | Chronic-granulomatous DiseaseChina
-
Great Ormond Street Hospital for Children NHS Foundation...WithdrawnGranulomatous Disease, Chronic, X-linked, VariantUnited Kingdom
-
National Institute of Allergy and Infectious Diseases...RecruitingChronic Granulomatous Disease-associated ColitisUnited States
-
GenethonActive, not recruitingX-Linked Chronic Granulomatous DiseaseUnited Kingdom
-
National Institute of Allergy and Infectious Diseases...Rare Diseases Clinical Research Network; Primary Immune Deficiency Treatment...Enrolling by invitationGranulomatous Disease, ChronicUnited States, Canada
-
Hubert Serve, Prof., MDUnknown