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Case Reports
December 1946


Author Affiliations

From the Department of Pathology, University of Pittsburgh School of Medicine and the Children's Hospital of Pittsburgh.

Am J Dis Child. 1946;72(6):720-727. doi:10.1001/archpedi.1946.02020350087006

NIEMANN-PICK disease is a congenital lipid dyscrasia, frequently familial, in which certain phosphatides because of defective metabolisms tend to accumulate within the body. The excess phosphatide is phagocytosed by cells of the reticuloendothelial system, and these cells become amassed in various organs, mainly spleen, liver and lymph nodes. This condition, known by the names of the first contributors,1 resembles Gaucher's disease, from which it is differentiated by the earlier age incidence, mortality occurring before the end of the second year, racial proclivities and the type of stored lipid. A definite differential diagnosis depends on the identification of the abnormally occurring lipids. Chemical analysis of the phospholipids in the liver and spleen of a patient dying of Niemann Pick disease is reported in this paper.

Recent reviews by Baumann, Klenk and Scheidegger,2 Atkinson3 and Canmann adequately cover the clinical bibliography which is not repeated here. A total of 60

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