Biliary Atresia: Lessons Learned from the Voluntary German Registry

Leonhardt, Johannes; Kuebler, Jochen F.; Leute, Philip Johannes Felix; Turowski, Carmen; Becker, Thomas; Pfister, Eva‐Doreen; Ure, Benno M.; Petersen, Claus

doi:10.1055/s-0030-1268476

Cited by 38 publications

(35 citation statements)

References 15 publications

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“…A recent large German multi-center study confirmed a relation between outcome and experience of the surgical center and reported a 2-year survival of 20% with their native liver (40). The 2-year survival with the native liver was even less (12%) in our cohort.…”

Section: Discussionmentioning

confidence: 89%

Diversity of Disorders Causing Neonatal Cholestasis â€“ The Experience of a Tertiary Pediatric Center in Germany

et al. 2014

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Background and Objective: Rapidly establishing the cause of neonatal cholestasis is an urgent matter. The aim of this study was to report on the prevalence and mortality of the diverse disorders causing neonatal cholestasis in an academic center in Germany.Methods: Clinical chemistry and cause of disease were retrospectively analyzed in 82 infants (male n = 42, 51%) that had presented with neonatal cholestasis to a tertiary medical center from January 2009 to April 2013.Results: Altogether, 19 disorders causing neonatal cholestasis were identified. Biliary atresia was the most common diagnosis (41%), followed by idiopathic cases (13%), progressive familial intrahepatic cholestasis (PFIC, 10%), cholestasis in preterm infants (10%), α1AT deficiency, Alagille syndrome, portocaval shunts, mitochondriopathy, biliary sludge (all 2%), and others. Infants with biliary atresia were diagnosed with a mean age of 62 days, they underwent Kasai portoenterostomy ~66 days after birth. The majority of these children (~70%) received surgery within 10 weeks of age and 27% before 60 days. The 2-year survival with their native liver after Kasai procedure was 12%. The time span between Kasai surgery and liver transplantation was 176 ± 73 days. Six children (7%), of whom three patients had a syndromic and one a non-syndromic biliary atresia, died prior to liver transplantation. The pre- and post-transplant mortality rate for children with biliary atresia was ~12 and ~17%, respectively.Conclusion: Neonatal cholestasis is a severe threat associated with a high risk of complications in infancy and it therefore requires urgent investigation in order to initiate life saving therapy. Although in the last 20 years new causes such as the PFICs have been identified and newer diagnostic tools have been introduced into the clinical routine biliary atresia still represents the major cause.

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Section: Discussionmentioning

confidence: 89%

Diversity of Disorders Causing Neonatal Cholestasis â€“ The Experience of a Tertiary Pediatric Center in Germany

et al. 2014

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“…Several investigators have reported that KPE plays a role in gaining time for liver transplantation but is not a curative treatment [3,4]. However, the results of KPE seem to steadily improve, especially in Japanese studies.…”

Section: Discussionmentioning

confidence: 99%

Late complications and current status of long-term survivals over 10 years after Kasai portoenterostomy

2011

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PurposeWhereas the Kasai portoenterostomy (KPE) is an accepted first line of surgery for bile drainage in infants with biliary atresia, its long-term effectiveness is not clear because its etiology and pathogenesis remains unknown. This study was aimed to investigate the late complications occurring in long-term survivors and the current status of living patients who survived over 10 years after KPE.MethodsA retrospective analysis of the medical records of 32 patients who underwent KPE from 1990 to 2000 was done. We analyzed 10-year survival rates with the Kaplan-Meier method and the current status of the long-term survivors.ResultsThe overall 10-year survival rate by Kaplan-Meier method after KPE was 76.2%. Eight (25%) patients had died, including 4 who were transplanted. Nineteen (59.4%) patients survived over 10 years. Among them, 6 (31.6%) patients had portal hypertension, and 5 (26.3%) had episodes of cholangitis. Two had intrahepatic cyst and 2 had intestinal obstruction. Six (31.6%) patients have been well without any complications.ConclusionThe long-term survival rate of biliary atresia is slightly improving. However, two thirds of patients suffer from various complications. One-third of survivors go on without any complication. As biliary atresia is known as a progressive inflammatory disease, careful life-long follow- up is needed in long-term survivals after KPE.

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“…Centralization of the surgical and medical treatment of rare conditions in children such as orofacial clefts [10], brain tumors [11], cardiac malformations [12] and biliary atresia [13][14][15] has been recommended by numerous authors. However, this survey showed that 88 % of the centers treat less than 20 new ARM cases per year while having 3 or more surgeons who are independently performing anorectal repairs.…”

Section: Discussionmentioning

confidence: 99%

Survey on the management of anorectal malformations (ARM) in European pediatric surgical centers of excellence

et al. 2015

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Biliary Atresia: Lessons Learned from the Voluntary German Registry

Cited by 38 publications

References 15 publications

Diversity of Disorders Causing Neonatal Cholestasis â€“ The Experience of a Tertiary Pediatric Center in Germany

Diversity of Disorders Causing Neonatal Cholestasis â€“ The Experience of a Tertiary Pediatric Center in Germany

Late complications and current status of long-term survivals over 10 years after Kasai portoenterostomy

Survey on the management of anorectal malformations (ARM) in European pediatric surgical centers of excellence

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