Browsing by Author "Ogah, S. O."

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    Hunter syndrome: case report and review of literature
    (2006-06) Ogunbiyi, A.; Adeyinka, A. O.; Ogah, S. O.; Baiyeroju, A. M.
    Hunter syndrome is one of the mucopolysaccharidosis, which are a rare group of genetic diseases, It is due to a deficiency in the enzyme Iduronate 2-sulphatase. This in turn leads to the accumulation of glycosaminoglycans, dermatan and heparan sulphate. The intra and extracellular accumulation of this substances lead to multisystemic organ abnormality. We present a patient with Hunter syndrome who presented with involvement of the skin, cardiovascular system, the eyes and musculoskeletal system. We have also included a literature review. As far as we know, it is the first case reported in a Nigerian.