An unusual case of phospholipidosis

We present the results of a structural, histochemical and lipid-chromatographic study of tissues obtained at postmortem from an unusual case of phospholipidosis. A previous biopsy of the appendix and liver (Elleder et al., 1975a) had revealed a predominance of phosphoglyceride storage, principally o...

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Bibliographic Details
Published in:Virchows Archiv A Pathological Anatomy and Histology 1978-12, Vol.377 (4), p.329-338
Main Authors: Elleder, M, Jirásek, A, Smíd, F, Harzer, K, Schlegerová, D
Format: Article
Language:English
Subjects:
Appendix - enzymology
Child, Preschool
Humans
Lymph Nodes - analysis
Neurons - analysis
Phosphatidic Acids - analysis
Sphingolipidoses - pathology
Sphingomyelin Phosphodiesterase - analysis
Sphingomyelins - analysis
Sphingomyelins - metabolism
Spleen - analysis
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Summary:We present the results of a structural, histochemical and lipid-chromatographic study of tissues obtained at postmortem from an unusual case of phospholipidosis. A previous biopsy of the appendix and liver (Elleder et al., 1975a) had revealed a predominance of phosphoglyceride storage, principally of lysobisphosphatidic acid (LBPA) postmortem material showed that this lipid was stored exclusively in central neurons. In the spleen and the lymph node, however, sphingomyelin (SP) was shown, histochemically and chromatographically, to be the main lipid stored. Total sphingomyelinase (SPase) activity in the appendix was reduced to about 50% of normal. Neuroaxonal dystrophy (NAD) and a conspicuous discrepancy between the degree of distension of some neurons and their lipid content deserve special mention. The case is contrasted with classical sphingomyelinosis; the complexity of the Niemann-Pick group of diseases is discussed as an indication of the difficulties of classification of any atypical case.
ISSN:0340-1227
1432-2307
DOI:10.1007/BF00507133