Die hämolytische Krise bei Morbus Wilson

✍ Scribed by H. -U. Furrer; O. Tönz

• Publisher: Springer
• Year: 1974
• Tongue: English
• Weight: 919 KB
• Volume: 118
• Category: Article
• ISSN: 0340-6997
• DOI: 10.1007/bf00440029

✦ Synopsis

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 literature. 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.