Clinical Hepatology by Henryk Dancygier

Author:Henryk Dancygier
Language: eng
Format: epub
Publisher: Springer Berlin Heidelberg, Berlin, Heidelberg

Erythropoietic Protoporphyria

The hepatocellular uptake and excretion of the lipo-phile protoporphyrin into the bile is the most important pathophysiological aspect for liver pathology. One quarter of all patients with EPP have an associated liver damage that can progress to cirrhosis. 10% of all patients develop gallstones; these gallstones consist mainly of protoporphyrin. The capacity of the liver for the clearance of the protoporphyrin is limited. In case of an uncomplicated course with only cutaneous symptoms, the deposition of protoporphyrin in the liver is insignificant. In contrast, overtaxing the liver excretory capacity leads to a crystalline storage of protoporphyrin in “Maltese Cross” structures that show a clear birefringence in polarized light (Fig. 84.3). Protoporphyrin crystallizes in a high concentration and is probably hepa-totoxic in concentrations greater than 500 μg/g (normal approximately 1 μg/g). The protoporphyrin induced liver damage can progress into fibrosis and cholestatic cirrhosis. Irreversible liver damage is characterized by decreasing fecal protoporphyrin excretion as a “signum malum ominis”. Enterohepatic reuptake and circulation of protoporphyrin further contributes to retention.

Fig. 84.3Hepatic “Maltese Cross” structures (arrow) in EPP that show a clear birefringence in polarized light (Kindly provided by PD Dr. med. J.O. Habeck, Department of Pathology, Klinikum Chemnitz, Germany)

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