CHEDIAK-HIGASHI-SYNDROME - REPORT ON 5 SAUDI-ARAB CHILDREN AND REVIEWOF THE LITERATURE

Five cases of the Chediak-Higashi Syndrome (CHS) among Saudi Arab chil dren were diagnosed between June 1978 and December 1990. All patients were males, ages 18 months to ten years, born to first degree consangu ineous parents. All showed the typical somatic and laboratory characte ristics of this syndrome with characteristic hyperpigmented irides in four patients. Three patients were in the accelerated phase, two of wh om responded to a combination chemotherapy (vincristine and prednisone ) and one improved after splenectomy. All patients died before they pa ssed the first decade of their lives. This report is the first detaile d description of CHS among Arab children in the Arabian peninsula.