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Enteropathy in Niemann-Pick disease

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Abstract

Three consecutive cases of Niemann-Pick disease with predominant enteropathy were seen. The diagnosis was confirmed by the demonstration of typical foamy cells in the bone marrow, small intestinal mucosa, and liver. The enteropathy was apparent in steatorrhoea, xylose malabsorption, protein loss etc. The hypothesis of foamy cell infiltration of the lamina propria of the small intestinal mucosa as a cause of malabsorption is proposed.

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Hager-Małecka, B., Szczepański, Z., Karczewska, K. et al. Enteropathy in Niemann-Pick disease. Z. Kinder-Heilk. 115, 71–75 (1973). https://doi.org/10.1007/BF00438992

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Key words

  • Lipoid histiocytosis
  • Niemann-Pick disease
  • Enteropathy
  • Malabsorption