Five cases of the Chediak-Higashi Syndrome (CHS) among Saudi Arab children were diagnosed between June 1978 and December 1990. All patients were males, ages 18 months to ten years, born to first degree consanguineous parents. All showed the typical somatic and laboratory characteristics of this syndrome with characteristic hyperpigmented irides in four patients. Three patients were in the accelerated phase, two of whom responded to a combination chemotherapy (vincristine and prednisone) and one improved after splenectomy. All patients died before they passed the first decade of their lives. This report is the first detailed description of CHS among Arab children in the Arabian peninsula.
Chediak-Higashi syndrome: Report on five Saudi Arab children and review of the literature. Publishing Authors By Initials
Chediak-Higashi syndrome: Report on five Saudi Arab children and review of the literature. Journal Published:
PUBLICATION TYPE: Journal Article
Journal: Annals of Saudi medicine
VOLUME: 13
Page Numbers: 321-7
Journal Abbreviation:
ISSN: 0256-4947
DAY: 25
MONTH: Jul
YEAR: 1993
Chediak-Higashi syndrome: Report on five Saudi Arab children and review of the literature. Information
Number of References:
LANGUAGE: eng
NlmUniqueID: 8507355
Chediak-Higashi syndrome: Report on five Saudi Arab children and review of the literature. Keywords Mesh Terms:
KEYWORDS:
MESH TERMS:
Chemical & Substance for Abstract: Chediak-Higashi syndrome: Report on five Saudi Arab children and review of the literature. Information
Substance Name:
Registry Number:
Grant and Affiliation Information for Chediak-Higashi syndrome: Report on five Saudi Arab children and review of the literature.
AFFILIATION: Department of Oncology, Division of Pediatric Hematology/Oncology, Department of Pediatrics, and Division of Allergy and Immunology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Country: Saudi Arabia
AGENCY:
GRANT:
ACRONYM:
MEDLINETA: Ann Saudi Med
REFSOURCE:
DATABASENAME:
ACCESSION NUMBER:
Number Hits: 0
Chediak-Higashi syndrome: Report on five Saudi Arab children and review of the literature Related Publications