Enteropathy in Niemann-Pick disease

descriptionPublicationkeyboard_double_arrow_right Article 01 Mar 1973 English Publisher:Springer Science and Business Media LLCJournal:Zeitschrift für Kinderheilkunde, volume 115, pages 71-75 (issn: 0340-6199, eissn: 1432-1076,

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Authors: B, Hager-Malecka; Z, Szczepański; K, Karczewska; D, Sońta-Jakimczyk;

doi: 10.1007/bf00438992

pmid: 4757188

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.

Keywords

Male, Niemann-Pick Diseases, Xylose, Infant, Liver, Malabsorption Syndromes, Bone Marrow, Intestine, Small, Humans, Female, Intestinal Mucosa, Immunoelectrophoresis

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