We report a case of acquired porphyrinemia in a liver transplant recipient. Thrombosis of the portal vein and hepatic artery led to massive necrosis of the patient's first donor liver, necessitating its removal. After an 18-hour anhepatic period, a second donor liver was transplanted. During this interval, the patient was warmed with an infrared heat lamp that emitted bright visible light. Following the anhepatic period, a photodistributed cutaneous erythema was noted. The level of total serum porphyrins during the anhepatic period was significantly elevated at 1.548 mumol/L (normal, less than 0.018 mumol/L). Fractionation showed that the levels of the hydrophobic porphyrins, coproporphyrin and protoporphyrin, were elevated, while the levels of the hydrophilic porphyrins remained normal. Following the successful second liver transplantation, the patient's porphyrin levels declined. This is the first reported case of acquired porphyrinemia in a liver transplant recipient.
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Arch Dermatol Res
April 1997
Division of Clinical Biochemistry, Faculty of Medicine, Philipps University, Marburg, Germany.
Various clinical and biochemical observations point to a relationship between degree of disease expression and metabolic disturbance in autosomal recessive congenital erythropoietic porphyria (Günther's disease). Although the clinical manifestations have been well described since Günther's fundamental observations, an interdependence between disease severity and porphyrin excess has yet to be elucidated. We investigated porphyrin metabolism in nine Indian patients suffering from the characteristic clinical symptoms: skin photosensitivity, red-colored urine as a sign of extremely elevated porphyrinuria and mild to severe hemolytic anemia.
View Article and Find Full Text PDFArch Dermatol
May 1991
Department of Dermatology, Indiana University School of Medicine, Indianapolis 46202.
We report a case of acquired porphyrinemia in a liver transplant recipient. Thrombosis of the portal vein and hepatic artery led to massive necrosis of the patient's first donor liver, necessitating its removal. After an 18-hour anhepatic period, a second donor liver was transplanted.
View Article and Find Full Text PDFAltogether 98 patients have been examined, suffering from latent porphyria (n = 18) and manifest porphyria cutanea tarda (n = 80). 90 (91.8%) examinees abused alcohol.
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