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Liver failure in erythropoietic protoporphyria associated with choledocholithiasis and severe post-transplantation polyneuropathy.
- Source :
-
Liver [Liver] 1996 Jun; Vol. 16 (3), pp. 211-7. - Publication Year :
- 1996
-
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 and 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.
- Subjects :
- Alanine Transaminase blood
Alkaline Phosphatase blood
Aspartate Aminotransferases blood
Bilirubin blood
Cholangiography
Cholelithiasis therapy
Cholinesterases blood
Cyclosporine pharmacology
Cyclosporine therapeutic use
Female
Gallstones physiopathology
Humans
Liver Transplantation
Middle Aged
Nervous System Diseases physiopathology
Neurology
Porphyria, Hepatoerythropoietic complications
Protoporphyrins blood
Protoporphyrins urine
Gallstones metabolism
Liver Failure physiopathology
Porphyria, Hepatoerythropoietic metabolism
Subjects
Details
- Language :
- English
- ISSN :
- 0106-9543
- Volume :
- 16
- Issue :
- 3
- Database :
- MEDLINE
- Journal :
- Liver
- Publication Type :
- Academic Journal
- Accession number :
- 8873010
- Full Text :
- https://doi.org/10.1111/j.1600-0676.1996.tb00730.x