Niemann-Pick Disease is an autosomal recessive disorder of infancy characterized by failure to thrive, hepatosplenomegaly and neurodegenerative changes that leads to sphingomyelin and cholesterol storage within the lysosome.Although the disease is common among Jewish individual but is rare in South Asian particularly in Bangladesh. So, we present the case as a rare incidence in our country.
A total of 7667 patients were admitted In Paediatrics and Paediatric surgery Department of Rajshahi Medical College Hospital from January 1999 to December 2000. Among these patients congenital malformation was found in 222 cases. The result showed that the occurrence of major congenital malformation was 2.26% and imperforated anus topped the list followed by congenital heart diseases. Minor malformation was 0.62% of which hypospadiasis and syndactyly were highest and lowest in number respectively. TAJ 2002; 15(1): 22-24
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