Optimal liver transplant procedure in progressive familial intrahepatic cholestasis type 1 treated with biliary diversion or intestinal transplantation: Lessons learned from three cases treated with different approaches.
Pediatr Transplant
; 27(6): e14566, 2023 09.
Article
em En
| MEDLINE
| ID: mdl-37417206
ABSTRACT
BACKGROUND:
Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an autosomal recessive cholestatic liver disorder caused by ATP8B1 gene mutations. Although liver transplantation (LT) is indicated for progressive liver disease, postoperative complications, including severe diarrhea and graft steatohepatitis leading to graft loss, have been reported. CASES The first patient had jaundice, pruritus, diarrhea, and growth retardation (weight z-score -2.5; height z-score -3.7). She underwent LT with total internal biliary diversion (TIBD) to the colon at 2 years of age. Graft biopsy at the 7-year follow-up examination revealed microvesicular steatosis (60%). Her diarrhea improved, and her growth failure was recovering (weight z-score -1.0; height z-score -1.7). The second patient underwent sequential intestine-liver transplantation at 8 years of age due to end-stage liver disease (ESLD) and short bowel syndrome caused by massive bowel resection for internal hernia after partial external biliary diversion (PEBD) at 21 months of age. She developed severe pancreatitis induced by steroid-bolus therapy for rejection after transplantation. She died 1.7 years after intestinal transplantation due to an uncontrollable pancreatic abscess and acute respiratory distress syndrome. The third patient underwent PEBD at 15 months of age and received LT with TEBD at 15 years of age due to ESLD with hepatic encephalopathy. Throughout the perioperative period, she showed no abdominal symptoms, including diarrhea and pancreatitis. Graft biopsy at the 2-year follow-up examination revealed macrovesicular steatosis (60%) with inflammation.CONCLUSIONS:
The patients showed different outcomes. Effective therapeutic options to mitigate post-LT complications in patients with PFIC1 must be considered individually.Palavras-chave
Texto completo:
1
Bases de dados:
MEDLINE
Assunto principal:
Colestase Intra-Hepática
/
Transplante de Fígado
/
Fígado Gorduroso
Tipo de estudo:
Etiology_studies
Limite:
Female
/
Humans
/
Infant
Idioma:
En
Revista:
Pediatr Transplant
Assunto da revista:
PEDIATRIA
/
TRANSPLANTE
Ano de publicação:
2023
Tipo de documento:
Article
País de afiliação:
Japão