Carpenter syndrome in eight Arab patients,dominant inheritance suspected. S.S. Al Arrayed. Genetic department Salmaniya Medical Ctr, Manama, Bahrain.

   Carpenter syndrome is rare autosomal recessive syndrome. The diagnostic criteria are acrocephaly, poly dactyly and syndactyly of fingers and toes. Some patients have congenital heart disease hypogonadism, mild obesity and frequent mental retardation. Thirty-five cases had been reported worldwide, since 1961. We are reporting eight cases with carpenter syndrome, four alive and four diseased from two families. In both families parents are consanguineous. All patients have typical features of carpenter syndrome. All the patients have mild mental retardation. None of the patients have congenital heart disease. The karyotypes in all of them are normal. The father in one family has some of the syndrome features such as, hepertelerism, acrocephaly, epicanthus folds, but without polysyndactyly, and with normal intelligence. Conclusion: In this family the consanguinity supports autosomal recessive inheritance. The fact that the father shows some of the syndrome features may indicate either codominant inheritance or dominant inheritance.