Lebertransplantation bei erythrohepatischer Protoporphyrie
| Autor: | I. Sieg, M. Frank, R. Kretschmar, Peter Neuhaus, R. Steffen, Thomas Steinmüller, Wolf O. Bechstein, M. O. Doss, Gerhard Blumhardt |
|---|---|
| Rok vydání: | 2008 |
| Předmět: |
medicine.medical_specialty
integumentary system business.industry Bilirubin General Medicine Jaundice medicine.disease Gastroenterology Ursodeoxycholic acid Transaminase Transplantation chemistry.chemical_compound chemistry Internal medicine medicine Portal hypertension Protoporphyrin Erythropoietic protoporphyria medicine.symptom business medicine.drug |
| Zdroj: | DMW - Deutsche Medizinische Wochenschrift. 117:1097-1102 |
| ISSN: | 1439-4413 0012-0472 |
| DOI: | 10.1055/s-2008-1062416 |
| Popis: | A 51-year-old man had for 5 years been known to have erythropoietic protoporphyria. GPT levels were raised up to 40 U/l, gamma-GT up to 120 U/l. After lengthy exposure to sun radiation an erythema with blisters, abdominal discomfort and jaundice developed (total bilirubin 7.3 mg/dl) and biliary liver cirrhosis with portal hypertension and splenomegaly were diagnosed. Because the acute hepatobiliary complications were not improved by conservative treatment (daily 750 mg ursodeoxycholic acid and 12 g colestyramine), an orthotopic liver transplantation was performed without complication. The excised liver showed small nodular parenchymal transformation and contained reddish brown protoporphyrin pigment in the hepatocellular cytoplasm, the Kupffer cells, the canaliculi and in some biliary ducts. Bilirubin and transaminase levels in blood became normal after the transplantation, as did the urinary excretion of coproporphyrin. However, isomer I was still dominant. The protoporphyrin level in erythrocytes and plasma remained elevated. After a symptom-free interval of one year biochemical and histological tests demonstrated protoporphyrin-induced damage in the transplanted liver. |
| Databáze: | OpenAIRE |
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