OSTEOPETROSIS IN CHILDREN

Over a 10-year period, 28 Arab children with autosomal recessive osteo petrosis were seen in two hospitals in Riyadh, Saudi Arabia. Eighteen (64%) had osteopetrosis associated with metabolic acidosis probably du e to a renal tubular defect; nine (32%) had a malignant infantile form of osteopetrosis and one had a mild form with delayed onset, Parental consanguinity was 56% and 40% among patients with and without acidosi s respectively. Somatic and psychomotor retardation and recurrent bone fractures were common in both groups, Dental caries, cerebral calcifi cation and optic atrophy were more frequent in patients with acidosis, while anaemia, hepatosplenomegaly and deafness were more common in pa tients without acidosis, To guarantee optimal rehabilitation, children with this progressive disease require an early multiteam approach.