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Porto-Sinusoidal Vascular Disease in Congenital Erythropoietic Porphyria Needing Liver Transplantation.
Gopalakrishna, Harish; Mironova, Maria; Malik, Shahid; Faust, Alison; Khurram, Nigar; Koh, Christopher; Kleiner, David E; Heller, Theo.
Afiliación
  • Gopalakrishna H; Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD.
  • Mironova M; Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD.
  • Malik S; Division of Gastroenterology, Hepatology, and Nutrition, University of Pittsburgh, Pittsburgh, PA.
  • Faust A; Penn State Health, Pennsylvania State University, Hershey, PA.
  • Khurram N; Department of Pathology, University of Pittsburgh, Pittsburgh, PA.
  • Koh C; Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD.
  • Kleiner DE; Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD.
  • Heller T; Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD.
ACG Case Rep J ; 11(5): e01336, 2024 May.
Article en En | MEDLINE | ID: mdl-38682079
ABSTRACT
Porphyria caused by inherited disorders in heme biosynthesis can lead to accumulation of porphyrins in various organs. Liver involvement due to porphyria mostly results in cholestasis leading to liver cirrhosis or hepatocellular carcinoma. Congenital erythropoietic porphyria (CEP), a rare porphyria due to deficiency of uroporphyrinogen III synthase, mostly results in cutaneous manifestations. There are reports of liver involvement including varying degree of fibrosis in patients with CEP. We report a unique case of a patient with CEP who developed porto-sinusoidal vascular disease with complications of portal hypertension that necessitated liver transplantation.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: ACG Case Rep J Año: 2024 Tipo del documento: Article Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: ACG Case Rep J Año: 2024 Tipo del documento: Article Pais de publicación: Estados Unidos