TY - JOUR
T1 - Optimal transplant strategy of pediatric liver transplantation for fibropolycystic liver disease
T2 - Multicenter retrospective study in Japan
AU - Uchida, Hajime
AU - Inui, Ayano
AU - Okamoto, Tatsuya
AU - Yasui, Toshihiro
AU - Honda, Masaki
AU - Mizuta, Koichi
AU - Bessho, Kazuhiko
AU - Okajima, Hideaki
AU - Ueno, Takehisa
AU - Matsuura, Toshiharu
AU - Okada, Noriki
AU - Sakamoto, Seisuke
AU - Kasahara, Mureo
N1 - Publisher Copyright:
© 2024 Japan Society of Hepatology.
PY - 2024
Y1 - 2024
N2 - Aim: To assess the preoperative disease characteristics and indications for living donor liver transplantation (LDLT), complications, patient survival, and prognosis after LDLT for fibropolycystic liver disease (FLD) in children. Methods: We undertook a cross-sectional survey of patients who underwent LDLT for FLD between January 2002 and December 2020. Results: A total of 35 patients (22 male and 13 female individuals) with FLD were included in this study, of whom 19 (54.3%) had isolated congenital hepatic fibrosis and 16 (45.6%) had Caroli syndrome. Refractory gastrointestinal bleeding was the most frequent symptom related to the indication for LDLT, being found in 48.6% of our patients, followed by uncontrollable cholangitis and ascites. The median age at the time of LDLT was 8.1 years old. Of the 27 patients presenting with renal involvement, 13 patients required kidney transplantation (KT). Overall, the renal function after LDLT decreased regardless of renal involvement; however, patients with renal involvement had a significantly lower estimated glomerular filtration rate than those without renal involvement throughout the course of this study (p < 0.01). The 5-year overall patient survival rate was 97.1%. Two patients died with a median follow-up of 8.9 years after LDLT; one died due to sepsis 2 weeks after simultaneous liver–kidney transplantation and the other committed suicide 10 years after LDLT. Conclusion: The prognosis of the pediatric patients who underwent LDLT for FLD was excellent. However, an individualized treatment approach based on the status of the renal function and liver disease is important, as a certain proportion of patients require KT.
AB - Aim: To assess the preoperative disease characteristics and indications for living donor liver transplantation (LDLT), complications, patient survival, and prognosis after LDLT for fibropolycystic liver disease (FLD) in children. Methods: We undertook a cross-sectional survey of patients who underwent LDLT for FLD between January 2002 and December 2020. Results: A total of 35 patients (22 male and 13 female individuals) with FLD were included in this study, of whom 19 (54.3%) had isolated congenital hepatic fibrosis and 16 (45.6%) had Caroli syndrome. Refractory gastrointestinal bleeding was the most frequent symptom related to the indication for LDLT, being found in 48.6% of our patients, followed by uncontrollable cholangitis and ascites. The median age at the time of LDLT was 8.1 years old. Of the 27 patients presenting with renal involvement, 13 patients required kidney transplantation (KT). Overall, the renal function after LDLT decreased regardless of renal involvement; however, patients with renal involvement had a significantly lower estimated glomerular filtration rate than those without renal involvement throughout the course of this study (p < 0.01). The 5-year overall patient survival rate was 97.1%. Two patients died with a median follow-up of 8.9 years after LDLT; one died due to sepsis 2 weeks after simultaneous liver–kidney transplantation and the other committed suicide 10 years after LDLT. Conclusion: The prognosis of the pediatric patients who underwent LDLT for FLD was excellent. However, an individualized treatment approach based on the status of the renal function and liver disease is important, as a certain proportion of patients require KT.
KW - Caroli disease
KW - congenital hepatic fibrosis
KW - fibropolycystic kidney disease
KW - kidney transplantation
KW - liver transplantation
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U2 - 10.1111/hepr.14122
DO - 10.1111/hepr.14122
M3 - Article
AN - SCOPUS:85206298691
SN - 1386-6346
JO - Hepatology Research
JF - Hepatology Research
ER -