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Reappraisal of liver transplantation for erythropoietic protoporphyria: A deadly combination of disease recurrence and biliary complication.

Yutaka EndoTaizo HibiMasahiro ShinodaHideaki ObaraMinoru KitagoHiroshi YagiYuta AbeYasushi HasegawaKentaro MatsubaraShutaro HoriMasayuki TanakaSatomi MakiuchiYutaka NakanoOsamu ItanoTatsuo KurodaYuko Kitagawa
Published in: Pediatric transplantation (2022)
Physicians should recognize the potentially rapid and life-threatening progression of protoporphyric hepatopathy that leads to liver failure. For young patients with EPP, LT and sequential BMT should thoroughly be considered by a multidisciplinary team as soon as hepatic reserve deterioration becomes evident. Split liver transplantation should preferably be avoided and appropriate post-transplant management is critical before protoporphyrin depositions to the bile duct and hepatocyte causes irreversible damage to the liver graft.
Keyphrases
  • liver failure
  • hepatitis b virus
  • primary care
  • quality improvement
  • oxidative stress
  • palliative care
  • free survival
  • liver injury