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June 1988

Cape Malay Hypospadias/Mental Retardation Syndrome-Reply

Author Affiliations

Department of Human Genetics University of Cape Town Medical School Observatory 7925 Cape Town Republic of South Africa

Am J Dis Child. 1988;142(6):590. doi:10.1001/archpedi.1988.02150060023010

In Reply.—We are grateful for Dr Hecht's gracious comments concerning our description of a new syndrome of hypospadias with mental retardation.

The Cape Malay community is a socioreligious isolate estimated to number approximately 4000 individuals. The term Malay is a misnomer, as their origin is not the Malayan Archipelago. Their ancestors arrived at the Cape approximately three centuries ago from the Dutch East Indian islands of Sri Lanka and India. They are currently a small and endogamous community, distinguished by their adherence to the Muslim faith. Owing to limited population size, only two exceptional genetic disorders have been reported from this community.1,2 They are also known to have a high incidence of diabetes mellitus, and the common genetic disorders are represented in their expected frequencies.

With regard to the genetic nature of this new disorder, no further light was shed by extensive family analysis. There were no other

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