Long-term results of pediatric liver transplantation for autoimmune liver disease - 14/05/21
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Highlights |
• | Autoimmune liver diseases (autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are rare indications for liver transplantation (LT) in children. |
• | Retrospective data from 30 children diagnosed with AIH or PSC who underwent a first LT from January 1988 to February 2018 were collected. |
• | Recurrence of initial disease was observed in 6 patients (20.0%), all of them with type 1 AIH. Overall patient survival rates were 96.4%, 84.6%, 74.8%, 68.0%, 68.0%, 68.0% and 68.0% at 1, 5, 10, 15, 20, 25 and 30 years, respectively. Age at LT<1year, LT for fulminant failure and LT for type 2 AIH were significant predictive factors of death. |
Abstract |
Background |
Autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are rare indications for liver transplantation (LT) in children. The aim of the present retrospective multicenter study was to evaluate long-term outcome after LT for autoimmune liver disease in childhood.
Methods |
Retrospective data from 30 children who underwent a first LT from 1988 to 2018 were collected.
Results |
The study population consisted of 18 girls and 12 boys, transplanted for AIH type 1 (n=14), AIH type 2 (n=7) or PSC (n=9). Mean age at LT was 11.8±5.2 years. The main indications for LT were acute (36.7%) or chronic end-stage liver failure (63.3%). Graft rejection occurred in 19 patients (63.3%); 6 pts required retransplantation for chronic rejection. Recurrence of initial disease was observed in 6 patients (20.0%), all of them with type 1 AIH, after a median time of 42 months, requiring retransplantation in 2 cases. Overall patient survival rates were 96.4%, 84.6%, 74.8%, 68.0%, 68.0%, 68.0% and 68.0% at 1, 5, 10, 15, 20, 25 and 30 years, respectively. Age at LT<1year (p<0.0001), LT for fulminant failure (p=0.023) and LT for type 2 AIH (p=0.049) were significant predictive factors of death.
Conclusion |
Long-term outcome after LT for pediatric autoimmune liver disease is impaired in patients with AIH because of consistent complications such as rejection and disease recurrence.
Il testo completo di questo articolo è disponibile in PDF.Abbreviations : AIH, ALF, AZA, CNI, CST, LT, mTORi, PSC, MMF
Keywords : Liver transplantation, Pediatric, Auto-immune disease, Outcome, Survival
Mappa
Vol 45 - N° 3
Articolo 101537- Maggio 2021 Ritorno al numero
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