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Elektronenmikroskopische Befunde an inneren Organen bei Morbus Fabry

Electron microscopic observations in internal organs in morbus fabry

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Summary

Electron microscopic findings are reported on the localization and fine structure of glycolipid inclusions in different organs (heart, kidney, lymph nodes, arterial blood vessels, pancreas) in Fabry's disease in a female. The intracellular and extracellular inclusions were made up of multilamellar membraneous systems in concentric, excentric, and parallel arrangement. This fine structure is characteristic of liquid-crystalline phases of phospholipid-water systems.

The same type of inclusions are found in the internal organs of heterozygotic women as in homozygotic men. The relationship between the glycolipid inclusions and the lysosomes is discussed.

Zusammenfassung

Es werden elektronenmikroskopische Beobachtungen zur Lokalisation und zur Feinstruktur der Glykolipideinschlüsse in verschiedenen inneren Organen bei Morbus Fabry berichtet. Die intrazellulÄren und extrazellulÄren Einschlüsse bestehen aus vielschichtig lamellÄren Membransystemen in konzentrischer und exzentrischer sowie in stapeiförmiger paralleler Anordnung. Diese lamellÄre Anordnung ist charakteristisch für flüssigkristalline Phasen von Phospholipid-Wasser-Systemen. Erstmalig werden ultrastrukturelle Befunde an den inneren Organen einer erkrankten Frau mitgeteilt. Demzufolge lassen sich bei heterozygoten Frauen gleiche Glykolipidablagerungen wie bei homozygoten MÄnnern nachweisen. Die Beziehung der Glykolipideinschlüsse zu den Lysosomen wird erörtert.

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  1. Pathologisches Institut und Klinik für Hautkrankheiten, der Friedrich-Schiller-UniversitÄt Jena, DDR

    Jürgen Roth & Hannelore Roth

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  1. Jürgen Roth
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  2. Hannelore Roth
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Herrn Prof. Dr. F. Bolck, Direktor des Pathologischen Instituts der Friedrich-Schiller-UniversitÄt Jena, zum 60. Geburtstag gewidmet.

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Roth, J., Roth, H. Elektronenmikroskopische Befunde an inneren Organen bei Morbus Fabry. Virchows Arch. A Path. Anat. and Histol. 378, 75–90 (1978). https://doi.org/10.1007/BF00427188

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