Chediak-Higashi syndrome: Report on five Saudi Arab children and review of the literature.
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.
Al-Nasser
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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.
Harfi
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Sabbah
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Malik
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