Liver Cirrhosis Induced by Porphyria Cutanea Tarda: A Case Report and Review
0
조회
0
다운로드
0
인용
0
참고문헌
초록
Porphyria cutanea tarda (PCT) is a metabolic disorder that results in a decrease in uroporphyrinogen decarboxylase activity. It is characterized by photosensitivity,bullae formation, and skin pigmentation. There are four types of PCT: acquired, familial, toxic,and hepatoerythropoietic. Uroporphyrin levels are elevated in the urine of PCT patients. PCT can be differentiated from other porphyrias by its clinical characteristics and the porphyrin levels in the serum, erythrocytes,urine, and feces. This metabolic disorder can lead to liver dysfunction as well as histological changes such as fatty infiltration or hepatic fibrosis. PCT rarely manifests as liver cirrhosis. We report herein a case of PCT-induced liver cirrhosis that progressed to hepatic failure.
Abstract
Porphyria cutanea tarda (PCT) is a metabolic disorder that results in a decrease in uroporphyrinogen decarboxylase activity. It is characterized by photosensitivity,bullae formation, and skin pigmentation. There are four types of PCT: acquired, familial, toxic,and hepatoerythropoietic. Uroporphyrin levels are elevated in the urine of PCT patients. PCT can be differentiated from other porphyrias by its clinical characteristics and the porphyrin levels in the serum, erythrocytes,urine, and feces. This metabolic disorder can lead to liver dysfunction as well as histological changes such as fatty infiltration or hepatic fibrosis. PCT rarely manifests as liver cirrhosis. We report herein a case of PCT-induced liver cirrhosis that progressed to hepatic failure.
참고문헌 (31)
- Yachimski P, "Porphyria cutanea tarda", Clin Gastroenterol Hepatol, 5, pp.e6~ (2007)
- Fritsch C, "Porphyria cutanea tarda", Skin Pharmacol Appl Skin Physiol, 11, pp.321~335 (1998)
- Moran MJ, "Hepatic uroporphyrinogen decarboxylase activity in porphyria cutanea tarda patients: the influence of virus C infection", Hepatology, 27, pp.584~589 (1998)
- Martinez di Montemuros F, "Gene symbol: UROD. Disease: Porphyria, cutanea tarda", Hum Genet, 113, pp.368~ (2003)
- Cassiman D, "Porphyria cutanea tarda and liver disease. A retrospective analysis of 17 cases from a single centre and review of the literature", Acta Gastroenterol Belg, 71, pp.237~242 (2008)
- Sarkany RP, "The management of porphyria cutanea tarda", Clin Exp Dermatol, 26, pp.225~232 (2001)
- Elder GH, "Porphyria cutanea tarda", Semin Liver Dis, 18, pp.67~75 (1998)
- Young LC, "Porphyria cutanea tarda associated with Cys282Tyr mutation in HFE gene in hereditary hemochromatosis: a case report and review of the literature", Cutis, 80, pp.415~418 (2007)
- Lamoril J, "Hemochromatosis (HFE) and transferrin receptor-1 (TFRC1) genes in sporadic porphyria cutanea tarda (sPCT)", Cell Mol Biol (Noisy-le-grand), 48, pp.33~41 (2002)
- Cohen DJ, "Porphyria cutanea tarda: a clinical review", Compr Ther, 22, pp.175~178 (1996)
- Bleasel NR, "Porphyria cutanea tarda", Australas J Dermatol, 41, pp.197~206 (2000)
- Nordmann Y, "Biological diagnosis of porphyria cutanea tarda", Ann Dermatol Venereol, 127, pp.860~861 (2000)
- Counter SA, "Zinc protoporphyrin levels, blood lead levels and neurocognitive deficits in Andean children with chronic lead exposure", Clin Biochem, 41, pp.41~47 (2008)
- Hershko C, "Combined use of zinc protoporphyrin (ZPP), mean corpuscular volume and haemoglobin measurements for classifying microcytic RBC disorders in children and young adults", Clin Lab Haematol, 7, pp.259~269 (1985)
- Murphy GM, "The cutaneous porphyrias: a review. The British Photodermatology Group", Br J Dermatol, 140, pp.573~581 (1999)
- Sampietro M, "Iron overload in porphyria cutanea tarda", Haematologica, 84, pp.248~253 (1999)
- Hammerschmidt DE, "Porphyria cutanea tarda", J Lab Clin Med, 136, pp.490~ (2000)
- Siersema PD, "The difference in liver pathology between sporadic and familialforms of porphyria cutanea tarda: the role of iron", J Hepatol, 23, pp.259~267 (1995)
- Cortes JM, "Hepatic pathology in porphyria cutaneatarda", Liver, 3, pp.413~414 (1983)
- Chevallier P, "Hepatic multi-nodular focal fatty metamorphosis in acquired porphyria cutanea tarda. Sonographic, CT, and MRI features", Clin Imaging, 22, pp.418~421 (1998)
- Gisbert JP, "Hepatocellular carcinoma risk in patients with porphyria cutanea tarda", Eur J Gastroenterol Hepatol, 16, pp.689~692 (2004)
- Elder GH, "Alcohol intake and porphyria cutanea tarda", Clin Dermatol, 17, pp.431~436 (1999)
- Wolff Fernandez C, "Effect of chloroquine treatment of porphyria cutanea tarda on the associated liver disease", Rev Invest Clin, 52, pp.211~213 (2000)
- Pizzino D, "Pathology of the skin appendages in porphyria cutanea tarda", G Ital Dermatol Venereol, 123, pp.607~608 (1988)
- Krajnc I, "The significance of histologic analysis of skin lesions in porphyria cutanea tarda. Light microscopy, electron microscopy, immunohistochemical and immunofluorescence analysis", Wien Klin Wochenschr, 110, pp.651~654 (1998)
- Mascaro JM, "New reasons for an archaic treatment: phlebotomy in sporadic porphyria cutanea tarda", Arch Dermatol, 139, pp.379~380 (2003)
- Bygum A, "Iron overload in porphyria cutanea tarda", Br J Dermatol, 143, pp.1116~ (2000)
- Wolff C, "Treatment of porphyria cutanea tarda with chloroquine and its effect on associated liver disease: retrospective analysis", Rev Med Chil, 124, pp.456~460 (1996)
- Herrero C, "Management of patients with porphyria cutanea tarda", Photodermatol Photoimmunol Photomed, 14, pp.64~65 (1998)
- Drago F, "Very lowdose chloroquine treatment for porphyria cutanea tarda", Acta Derm Venereol, 75, pp.329~330 (1995)
- Fracanzani AL, "Liver cancer risk is increased in patients with porphyria cutanea tarda in comparison to matched control patients with chronic liver disease", J Hepatol, 35, pp.498~503 (2001)
