AUTOSOMAL RECESSIVE OSTEOPETROSIS IN ARAB CHILDREN

Nineteen Arab children including six boys and 13 girls in ten sibships were diagnosed as having osteopetrosis over a 5-year period in variou s hospitals in Kuwait. Eighteen patients had an isolated autosomal rec essive form and one had autosomal recessive osteopetrosis associated w ith renal tubular acidosis. The mean age of diagnosis was 24 months. P arental consanguinity was high amongst them (68%). Anaemia, hepatosple nomegaly, failure to thrive, recurrent infections and neurological man ifestations were common. Associated congenital abnormalities were foun d in 26%. Deafness, hydrocephalus and dental caries were relatively le ss common. A high mortality (37%) owing to infection was noted. The me dical management and recommendations for patient care are discussed br iefly.