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황달을 주소로 발현한 만발피부포르피린증

Porphyria Cutanea Tarda Presenting as Jaundice

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Abstract

Porphyria cutanea tarda (PCT) is a disease characterized by an early appearance of severe photosensitivity with an increase in urinary uroporphyrin excretion and other porphyrins, high isocoproporphyrin fecal levels and an accumulation of protoporphyrin in erythrocyte. This disease has been known to be caused by a dramatic decrease in the activity of the uroporphyrinogen decarboxylase. Multiple infectious and environmental factors may contribute to PCT by influencing oxidative stress in the hepatocytes. We report here on a case of porphyria cutanea tarda that presented with jaundice and photosensitivity.

참고문헌 (14)

  1. "Porphyria Cutanea Tarda의 1예"
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  5. "Association between reactive oxygen species and disease activity in chronic hepatitis C", 21, pp.291~295 (1996)
  6. "Porphyria cutanea tarda: multiplicity of risk factors including HFE mutations, hepatitis C, and inherited uroporphyrinogen decarboxylase deficiency", 47, pp.419~426 (2002)
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  8. "Hepatocellular carcinoma in porphyria cutanea tarda:frequency and factors related to its occurrence", 12, pp.56~61 (1992)
  9. "Iron removal therapy in porphyria cutanea tarda:phlebotomy versus slow subcutaneous desferrioxamine infusion", 114, pp.621~629 (1986)
  10. "Treatment of porphyria cutanea tarda of end-stage renal disease with erythropoietin", 29, pp.499~500 (1993)
  11. "Agents affecting porphyrin formation and secretion:implications for porphyria cutanea tarda treatment", 26, pp.16~23 (1989)
  12. "Low-dose oral chloroquine in the treatment of porphyria cutanea tarda", 111, pp.609~613 (1984)
  13. "Low-dose oral chloroquine in patients with porphyria cutanea tarda and low-moderate iron overload", 7, pp.169~175 (1994)
  14. "Liver changes in porphyria cutanea tarda patients treated with chloroquine", 102, pp.261~266 (1980)

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