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Die hämolytische Krise bei Morbus Wilson

Acute hemolysis in morbus wilson

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Abstract

Three brothers with M. Wilson are presented. A severe hemolytic crisis of the youngest revealed the same disorder in the family. An acute hemolysis has to be considered as an early symptom of the disease. Its significance is discussed and compared with analogue cases in the leterature. Characteristics of copper metabolism and liver function in the acute hepatic phase of the lenticular degeneration are described. The high cupriuria observed in such cases points to the mechanism of hemolysis. Copper is deliberated from the liver, enters the red cells and damages the normal reduction processes. Hemoglobin, enzymes and membrane proteins are oxidized.

Every cirrhosis in the youth, mainly in connection with an acute hemolysis should direct the attention to M. Wilson. Increased direct bilirubin in a hemolytic crisis is suspicious and needs a control of cupriuria, which is of greater diagnostic significance than serum copper and coeruloplasmin.

Zusammenfassung

Drei Brüder mit Morbus Wilson werden vorgestellt; die schwere hämolytische Krise des jüngsten, ließ das Leiden in der Familie erkennen. Das Frühsymptom der hämolytischen Krise wird mit den Fällen der Literatur verglichen. Besonderheiten von Kupferstoffwechsel und Leberfunktion in der “akuten hepatischen Phase” der hepatolentikulären Degeneration werden besprochen. — Die hohe Cupriurie im beobachteten Fall weist auf den Mechanismus der Hämolyse: Kupfer wird aus der Leber freigesetzt, überschwemmt den Kreislauf, dringt in die Erythrocyten ein, schaltet normale Reduktionsprozesse aus; Hämoglobin, Enzyme und Membranproteine werden oxidiert.

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Authors and Affiliations

  1. Kinderspital Luzern, Luzern, Schweit

    H. -U. Furrer & O. Tönz

  2. Kinderspital, CH-8032, Zürich, Schweiz

    H. -U. Furrer

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  1. H. -U. Furrer
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  2. O. Tönz
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Herrn Professor K. Betke zum 60. Geburtstag gewidmet.

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Furrer, H.U., Tönz, O. Die hämolytische Krise bei Morbus Wilson. Z. Kinder-Heilk. 118, 147–162 (1974). https://doi.org/10.1007/BF00440029

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