Twenty-Year Transplant-Free Survival Rate Among Patients With Biliary Atresia

Vries, Willemien de; Veen, J. Homan-van der; Hulscher, Jan B F; Hoekstra-Weebers, Josette E. H. M.; Houwen, Roderick H. J.; Verkade, Henkjan J.

doi:10.1016/j.cgh.2011.07.024

Cited by 70 publications

(67 citation statements)

References 26 publications

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“…In a Dutch cohort of 106 patients with BA between 1977 and 1988, 27% survived for 20 years without transplantation . Overall, 20‐year survival with and without transplantation was 43%.…”

Section: Biliary Atresiamentioning

confidence: 99%

Transitional care for patients with surgical pediatric hepatobiliary disease: Choledochal cysts and biliary atresia

Aspelund

Mahdi

Rothstein

et al. 2019

J of Gastro and Hepatol

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Choledochal cysts (CDCs) and biliary atresia (BA) are rare pediatric hepatobiliary anomalies that require surgical intervention due to increased risk of malignancy and liver failure, respectively. The underlying disease and operative procedures place patients at risk for long‐term complications, which may continue to affect them into adulthood. Lack of a transitional care model in the health‐care system potentiates the challenges they will face following aging out of their pediatric providers' care. We sought to elucidate the long‐term complications and challenges patients with CDCs and BA face, review the current literature regarding transitioning care, and propose guidelines aiding adult providers in continued care and surveillance of these patients. A literature review was performed to assess short‐term and long‐term complications after surgery and the current standards for transitioning care in patients with a history of CDCs and BA. While transitional programs exist for patients with other gastrointestinal diseases, there are few that focus on CDCs or BA. Generally, authors encourage medical record transmission from pediatric to adult providers, ensuring accuracy of information and compliance with treatment plans. Patients with CDCs are at risk for developing biliary malignancies, cholangitis, and anastomotic strictures after resection. Patients with BA develop progressive liver failure, necessitating transplantation. There are no consensus guidelines regarding timing of follow up for these patients. Based on the best available evidence, we propose a schema for long‐term surveillance.

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“…In a Dutch cohort of 106 patients with BA between 1977 and 1988, 27% survived for 20 years without transplantation . Overall, 20‐year survival with and without transplantation was 43%.…”

Section: Biliary Atresiamentioning

confidence: 99%

Transitional care for patients with surgical pediatric hepatobiliary disease: Choledochal cysts and biliary atresia

Aspelund

Mahdi

Rothstein

et al. 2019

J of Gastro and Hepatol

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“…Studies with almost exclusively term BA patients showed that early KPE is associated with better transplant-free survival; a KPE after 60 days of age has a negative effect on transplant-free survival (2,(8)(9)(10). In total, 70% to 80% of infants with BA will need a liver transplantation (LTx) due to an ongoing process of liver fibrosis (11). In fact, BA is the main indication for pediatric LTx (12,13).…”

mentioning

confidence: 99%

Preterm Infants With Biliary Atresia

Wessel

Boere²,

Hulzebos

et al. 2017

J. pediatr. gastroenterol. nutr.

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Objectives: Biliary atresia (BA) occurs in 0.54 of 10.000 of overall live births in the Netherlands. BA has an unfavorable prognosis: <40% of patients are cleared of jaundice after Kasai portoenterostomy (KPE), 4-year transplant-free survival rate is 46% and the 4-year survival rate is ∼75%. Little is known on difficulties in diagnosis and the outcome of BA in preterm infants. We aimed to analyze the incidence and outcome of BA in preterm infants in the Netherlands. Methods: Retrospective study including Dutch preterm infants treated for BA. Parameters included gestational age, congenital anomalies, age at KPE, days between first symptoms, and KPE and referral interval (first hospital to KPE). Outcome parameters were clearance of jaundice (COJ) and (transplant-free) survival. Data are presented as medians (ranges). Results: Included 28 preterm infants (13 boys/15 girls) between March 1988 and December 2015. The incidence of BA was 1.06 of 10.000 preterm live births. Gestational age was 34.8 (27.3–36.9) weeks. Congenital anomalies were present in 11 of 28 (39%) infants. Time between first symptoms and KPE was 57 (9–138) days. Referral interval was 28 (8–86) days. Age at KPE was 70 (35–145) days. COJ was achieved in 23% of cases. Four-year transplant-free survival rate was 21%. Four-year overall survival was 61%. Conclusions: BA has a higher incidence in the preterm population compared to the overall BA population. The outcome of BA in preterm infants is poor, regarding COJ and (transplant-free) survival. We speculate that timely recognition of BA-related signs and symptoms in preterm infants will improve prognosis.

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“…It can be speculated that patients who did not undergo transplantation reaching adulthood have a relatively benign course of their disease and are therefore less likely delayed in COL. One-fifth of patients surviving BA without OLT have normal liver biochemistry up to 20 years and have no clinical or ultrasonographic signs of liver cirrhosis [2,9]. The patients who underwent transplantation had a worse course of disease culminating in life-threatening liver failure.…”

Section: Discussionmentioning

confidence: 98%