12/04/2012

Niemann-Pick

Niemann-Pick disease is caused by a disturbance in the metabolism of phosphatides (phospholipids), characterized by the deposition of sphingomyelin in the reticuloendothelial system cells and lymph nodes, but also the cells and the endothelial tissue. Niemann-Pick disease is inherited in a recessive and seen mainly in children. Typical signs of the disease are gastrointestinal disorders, anemia, hypertrophy and abnormalities of the liver and spleen, as well as neurological deficits. Typically, children who suffer from this disease do not survive three years.

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