Lock, G and Holstege, A and Mueller, AR and Christe, W and Doss, MO and Scholmerich, J and Neuhaus, P (1996) Liver failure in erythropoietic protoporphyria associated with choledocholithiasis and severe post-transplantation polyneuropathy. LIVER, 16 (3). pp. 211-217. ISSN 0106-9543,
Full text not available from this repository.Abstract
In a 58-year-old woman with erythropoietic protoporphyria, asymptomatic liver involvement had been diagnosed 12 years earlier. For more than 20 years the patient had been known to have symptomatic gallstones. A mild polyneuropathy of the lower limbs had been diagnosed several years ago. In December 1992, she presented with colicky upper abdominal pain, dyspepsia and mild jaundice. Diagnosis of beginning cholestasis in erythrohepatic protoporphyria and coincidental choledocholithiasis was made. A causal relation between choledocholithiasis acid deterioration of liver function was assumed. Endoscopic extraction of the bile duct stones, however, could not prevent the development of terminal hepatic failure. Biochemically, an excessive protoporphyrinemia and coproporphyrinuria were found. Five weeks after presentation, the patient underwent orthotopic liver transplantation. Immediately after the operation she developed a severe axonal neuropathy with cranial nerve involvement. One year after transplantation, her general condition has markedly improved, but there is still a disabling polyneuropathy. Recently, there were single reports on patients with very similar neurological symptoms following liver transplantation in erythropoietic protoporphyria. This case supports the assumption of a distinct protoporphyrin-induced neural damage in severe hepatic failure.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | ERYTHROHEPATIC PROTOPORPHYRIA; TRANSFUSION THERAPY; COMPLICATIONS; DISEASE; DYSFUNCTION; CIRRHOSIS; PATIENT; SEPSIS; SKIN; cholestasis; erythrohepatic protoporphyria; liver transplantation; neurotoxicity |
| Depositing User: | Dr. Gernot Deinzer |
| Last Modified: | 19 Oct 2022 08:35 |
| URI: | https://pred.uni-regensburg.de/id/eprint/51682 |
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