Outcomes of pediatric liver transplantation for progressive familial intrahepatic cholestasis

Background Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. We present our data of LT for each type of PFIC and compare their...

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Published in:Pediatric transplantation 2023-12, Vol.27 (8), p.e14600-n/a
Main Authors: Vasudevan, Anu K., Shanmugam, Naresh, Rammohan, Ashwin, Valamparampil, Joseph J., Rinaldhy, Kshetra, Menon, Jagadeesh, Thambithurai, Ravikumar, Namasivayam, Saravanapandian, Kaliamoorthy, Ilankumaran, Rela, Mohamed
Format: Article
Language:English
Subjects:
Child
Children
Cholestasis
Cholestasis, Intrahepatic - surgery
Clinical aspects
Diarrhea
Disease Progression
Fatty Liver
Gallbladder diseases
Humans
Ischemia
Liver Transplantation
Morbidity
outcomes
Pediatrics
progressive familial intrahepatic cholestasis
quality of life
Steatosis
Total internal biliary diversion
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Summary:Background Progressive familial intrahepatic cholestasis (PFIC) is a heterogenous group of inherited hepatocellular disorders and the clinical aspects, role of liver transplantation (LT), and its outcomes remain largely unelucidated. We present our data of LT for each type of PFIC and compare their early, and long‐term outcomes, highlighting their individual differences and management strategies. Methods Prospectively collected data over a decade (2011–2022) of children with PFIC who underwent LT was analyzed. The groups (PFIC 1–4) were compared with regard to early and long‐term outcomes including attainment of catch‐up growth. Results Of 60 children with PFIC who underwent LT, 13, 11, 31 & 5 were of PFIC 1, 2, 3 & 4, respectively. There were no significant differences in gender, PELD scores, BMI, type of grafts, cold and warm ischemia times, intraoperative blood loss, and morbidity among the groups. Post‐LT chronic diarrhea was observed in 6 (46.1%) children with PFIC‐I, and of them, 3 (23%) developed graft steatohepatitis. Three of these children underwent total internal biliary diversion (TIBD) and on 1‐year follow‐up, their graft steatosis resolved and they attained catch‐up growth. Catch‐up growth was significantly poorer in the PFIC1 group (44.4% vs. 88%, 90%, 100% p 
ISSN:1397-3142
1399-3046
1399-3046
DOI:10.1111/petr.14600