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

Yutaka Endo, Taizo Hibi, Masahiro Shinoda, Hideaki Obara, Minoru Kitago, Hiroshi Yagi, Yuta Abe, Yasushi Hasegawa, Kentaro Matsubara, Shutaro Hori, Masayuki Tanaka, Satomi Makiuchi, Yutaka Nakano, Osamu Itano, Tatsuo Kuroda, Yuko Kitagawa

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)


Background: Erythropoietic protoporphyria (EPP) is a rare inherited disorder that causes the accumulation of protoporphyrin in the erythrocytes, skin, and liver. Severe protoporphyric hepatopathy results in liver failure, requiring both liver and bone marrow transplantation as a life-saving procedure and to correct the underlying enzymatic defect, respectively. Case presentation: We report a 20-year-old man who underwent split liver transplantation using a right trisegment and caudate lobe graft for EPP-induced liver failure, but succumbed to a deadly combination of early relapse of EPP and subsequent, intractable, late-onset bile leakage from the cut surface of segment 4. EPP recurrence most likely created a high-risk situation for bile leakage from the non-communicating bile ducts of segment 4; therefore, this case shed light on the potential relationship between EPP recurrence and biliary complications. Conclusion: 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.

Original languageEnglish
Article numbere14261
JournalPediatric Transplantation
Issue number4
Publication statusPublished - 2022 Jun


  • bile leakage
  • disease recurrence
  • erythropoietic protoporphyria
  • postoperative management
  • split liver transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation


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