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Infantile neurological Gaucher's disease in three siblings

An ultrastructural study

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Summary

The clinical, histological and histochemical aspects of infantile neurological Gaucher's disease in three siblings are described.

In one of them, electron-microscopic examination of Gaucher cells from a cervical lymphnode biopsy was carried out. We believe that the term “tubes” is inadequate to describe Gaucher bodies, since we have shown that most Gaucher bodies consist of flattened sac-like structures. A fragment of the cerebral cortex obtained from the same subject four hours after death was also studied with the electron microscope; surprisingly, Gaucher bodies were observed in some neurons.

The various factors which can favour the deposition of kerasin are discussed.

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Hernández, F., Bueno, M. Infantile neurological Gaucher's disease in three siblings. Virchows Arch. Abt. A Path. Anat. 360, 27–32 (1973). https://doi.org/10.1007/BF00548655

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  • DOI: https://doi.org/10.1007/BF00548655

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