Skórne manifestacje chorób wątroby. Część I

Aldona Pietrzak¹, Dorota Czelej¹, Bogusław Pietrzak²

Streszczenie
Summary
We describe the association between hepar diseases and skin manifestations of its improper function. The pathomechanism of many diseases is associated with the icterus. Fascinating group of many disorders share the same clinical abnormalities of the bilirubin biosynthetic pathway. Some of metabolic diseases are characterized by a dinstinctive biochemical pattern like porphirias, or other clinical distinctive features like Alagille syndrom, haemochromatosis, von Gierke disese, Andersen disease, Hurler diseaes, Hunter disease, Sanfilippo disease, Maroteaux-Lamy disease, hyperlipoproteinemia and xanthomatosis, GM1 gangliosydosis, Gaucher disese, Fabry disease, Niemann-Pick disease, Wolman disease, Refsum disease, Wilson and Menkes disease.

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