Reappraisal of liver transplantation for erythropoietic protoporphyria: A deadly combination of disease recurrence and biliary complication.
Pediatr Transplant
; 26(4): e14261, 2022 06.
Article
in En
| MEDLINE
| ID: mdl-35225415
ABSTRACT
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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Liver Transplantation
/
Liver Failure
/
Protoporphyria, Erythropoietic
/
Liver Diseases
Limits:
Adult
/
Humans
/
Male
Language:
En
Journal:
Pediatr Transplant
Journal subject:
PEDIATRIA
/
TRANSPLANTE
Year:
2022
Type:
Article
Affiliation country:
Japan