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Diagnosis and therapy

Aspartate Aminotransferase-to-Platelet ratio index (APRi) in infants with biliary atresia: prognostic value at presentation.

Grieve A, Makin E, Davenport M.

J Pediatr Surg. 2013 Apr;48(4):789-95. doi: 10.1016/j.jpedsurg.2012.10.010.

Department of Paediatric Surgery, University of the Witwatersrand.

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Abstract


Diagnosis and therapy

Aspartate Aminotransferase-to-Platelet ratio index (APRi) in infants with biliary atresia: prognostic value at presentation.

Grieve A, Makin E, Davenport M.

J Pediatr Surg. 2013 Apr;48(4):789-95. doi: 10.1016/j.jpedsurg.2012.10.010.

Department of Paediatric Surgery, University of the Witwatersrand.

BACKGROUND:

Biliary atresia (BA) is a progressive obliterative cholangiopathy leading to liver fibrosis and cirrhosis. The aspartate aminotransferase-to-platelet ratio index (APRi) has been used in other liver diseases and in older children with BA as a surrogate marker of liver fibrosis. The aim of this study was to calculate APRi at time of presentation and relate this to operative findings and early outcome.

METHODS:

Prospective single surgeon cohort study of infants with BA (January 1999-December 2010). Initial APRi values were related to other biochemical indices and liver appearance at the time of Kasai portoenterostomy. Data are expressed as median (interquartile range). Non-parametric comparison was performed and a P-value of≤0.05 was regarded as significant.

RESULTS:

Overall 260 infants were included in the study. Median APRi was 0.67 (0.43-1.12) at a median age of surgery of 58 (range 14-209) days. APRi correlated with age (rs=0.44; P<0.0001), spleen size (rs=0.48; P<0.0001) and bilirubin (rs=0.45; P<0.0001). Liver assessment at operation was divided into cirrhosis [n=28 (10.8%)] or non-cirrhosis. Using a cut-off value of 1.22 [AUC 0.83 (95% CI 0.73-0.90)] showed a sensitivity of 75% and a specificity of 84% for macroscopic cirrhosis. Native liver survival was significantly different but improved only for those in the lowest APRi quartile (<0.43; P<0.009). APRi values at presentation had no significant association with later development of significant oesophageal varices.

CONCLUSION:

APRi at the time of KP is a useful adjunct in evaluating severity of liver disease in BA at presentation.

 

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Liver transplantation > Outcome

Outcomes in children with biliary atresia following liver transplantation.

Sun LY, Yang YS, Zhu ZJ, Gao W, Wei L, Sun XY, Qu W, Rao W, Zeng ZG, Dong C, Tu JP, Wang J, Liu YH, Liu Y, Yu LX, Wang Y, Li J, Shen ZY.

Hepatobiliary Pancreat Dis Int. 2013 Apr;12(2):143-8.

Nankai University School of Medicine, Tianjin 300071, China.

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Abstract


Outcome

Outcomes in children with biliary atresia following liver transplantation.

Sun LY, Yang YS, Zhu ZJ, Gao W, Wei L, Sun XY, Qu W, Rao W, Zeng ZG, Dong C, Tu JP, Wang J, Liu YH, Liu Y, Yu LX, Wang Y, Li J, Shen ZY.

Hepatobiliary Pancreat Dis Int. 2013 Apr;12(2):143-8.

Nankai University School of Medicine, Tianjin 300071, China.

BACKGROUND:

Congenital biliary atresia is a rare condition characterized by idiopathic dysgenesis of the bile ducts. If untreated, congenital biliary atresia leads to liver cirrhosis, liver failure and premature death. The present study aimed to evaluate the outcomes of orthotopic liver transplantation in children with biliary atresia.

METHOD:

We retrospectively analyzed 45 patients with biliary atresia who had undergone orthotopic liver transplantation from September 2006 to August 2012.

RESULTS:

The median age of the patients was 11.0 months (5-102). Of the 45 patients, 41 were younger than 3 years old. Their median weight was 9.0 kg (4.5-29.0), 34 of the 45 patients were less than 10 kg. Thirty-one patients had undergone Kasai portoenterostomy prior to orthotopic liver transplantation. We performed 30 living donor liver transplants and 15 split liver transplants. Six patients died during a follow-up. The median follow-up time of surviving patients was 11.4 months (1.4-73.7). The overall 1-, 2- and 3-year survival rates were 88.9%, 84.4% and 84.4%, respectively.

CONCLUSION:

With advances in surgical techniques and management, children with biliary atresia after liver transplantation can achieve satisfactory survival in China, although there remains a high risk of complications in the early postoperative period.

 

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Diagnosis and therapy

Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature.

Kianifar HR, Tehranian S, Shojaei P, Adinehpoor Z, Sadeghi R, Kakhki VR, Keshtgar AS.

Pediatr Radiol. 2013 Mar 22.

Paediatric Gastroenterology Ward, Mashhad University of Medical Sciences, Ghaem Hospital, Mashhad, Iran.

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Abstract


Diagnosis and therapy

Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature.

Kianifar HR, Tehranian S, Shojaei P, Adinehpoor Z, Sadeghi R, Kakhki VR, Keshtgar AS.

Pediatr Radiol. 2013 Mar 22.

Paediatric Gastroenterology Ward, Mashhad University of Medical Sciences, Ghaem Hospital, Mashhad, Iran.

Hepatobiliary scintigraphy is an important diagnostic modality for work-up of neonatal cholestasis. Therefore, our objective was to evaluate the literature regarding the accuracy of hepatobiliary scintigraphy in differentiating biliary atresia from non-biliary atresia causes of cholestasis (collectively called neonatal hepatitis). Our search included Medline, SCOPUS and Google Scholar. Only studies using Tc-99 m-labeled immunodiacetic acid (IDA) derivatives were included. Overall, 81 studies were included in the meta-analysis. Pooled sensitivity and specificity were 98.7% (range 98.1-99.2%) and 70.4% (range 68.5-72.2%), respectively. Factors that increased specificity included the use of radiotracers with high hepatic extraction, administration of hepatic-inducing drugs (such as phenobarbital), use of a calculated dose/kg and administration of a booster dose in cases of non-excretion of the tracer in the bowel. SPECT imaging and duodenal fluid sampling also had high specificity; however, they need further validation because of the low number of studies. Semiquantitative imaging methods do not seem to have any incremental value. We conclude that hepatobiliary scintigraphy using IDA derivatives can be very useful for diagnostic work-up of neonatal cholestasis. To improve the specificity, several measures can be followed regarding type and dose of the radiotracer and imaging protocols. Non-imaging methods seem to be promising and warrant further validation

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Etiology & basic research > Immunology

Lack of HLA predominance and HLA shared epitopes in biliary Atresia.

Mack CL, Anderson KM, Aubrey MT, Rosenthal P, Sokol RJ, Freed BM.

Springerplus. 2013 Dec;2(1):42. Epub 2013 Feb 8.

Departments of Medicine and Immunology, Division of Allergy and Clinical Immunology, University of Colorado School of Medicine, 80045 Aurora, CO USA ; Department of Pediatrics, Division of Pediatric Gastroenterology, Digestive Health Institute, Children's Hospital Colorado, Hepatology and Nutrition, 13123 East 16th Ave. B290, 80045 Aurora, CO USA.

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Abstract


Immunology

Lack of HLA predominance and HLA shared epitopes in biliary Atresia.

Mack CL, Anderson KM, Aubrey MT, Rosenthal P, Sokol RJ, Freed BM.

Springerplus. 2013 Dec;2(1):42. Epub 2013 Feb 8.

Departments of Medicine and Immunology, Division of Allergy and Clinical Immunology, University of Colorado School of Medicine, 80045 Aurora, CO USA ; Department of Pediatrics, Division of Pediatric Gastroenterology, Digestive Health Institute, Children's Hospital Colorado, Hepatology and Nutrition, 13123 East 16th Ave. B290, 80045 Aurora, CO USA.

Biliary atresia (BA) is characterized by progressive inflammation and fibrosis of bile ducts. A theory of pathogenesis entails autoimmune-mediated injury targeting bile duct epithelia. One of the strongest genetic associations with autoimmunity is with HLA genes. In addition, apparently dissimilar HLA alleles may have similar antigen-binding sites, called shared epitopes, that overlap in their capacity to present antigens. In autoimmune disease, the incidence of the disease may be related to the presence of shared epitopes, not simply the HLA allelic association. Aim: To determine HLA allele frequency (high-resolution genotyping) and shared epitope associations in BA. Results: Analysis of every allele for HLA-A, -B, -C, -DRB1, -DPB1 and -DQB1 in 180 BA and 360 racially-matched controls did not identify any significant HLA association with BA. Furthermore, shared epitope analysis of greater than 10 million possible combinations of peptide sequences was not different between BA and controls. Conclusions: This study encompasses the largest HLA allele frequency analysis for BA in the United States and is the first study to perform shared epitope analysis. When controlling for multiple comparisons, no HLA allele or shared epitope association was identified in BA. Future studies of genetic links to BA that involve alterations of the immune response should include investigations into defects in regulatory T cells and non-HLA linked autoinflammatory diseases.

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Review and overview papers

Scottish outcomes for extra hepatic biliary atresia post-rationalisation of services.

Tayler R, Barclay AR, Rogers P, Mcintyre K, Russell RK, Devadason D, Bisset WM, Ling SC, McGrogan P.

Arch Dis Child. 2013 May;98(5):381-3

Department of Paediatric Gastroenterology abd Nutrition, Royal Hospital for Sick Children, Glasgow G3 8SJ, UK. andrew.barclay@ggc.scot.nhs.uk

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Abstract


Review and overview papers

Scottish outcomes for extra hepatic biliary atresia post-rationalisation of services.

Tayler R, Barclay AR, Rogers P, Mcintyre K, Russell RK, Devadason D, Bisset WM, Ling SC, McGrogan P.

Arch Dis Child. 2013 May;98(5):381-3

Department of Paediatric Gastroenterology abd Nutrition, Royal Hospital for Sick Children, Glasgow G3 8SJ, UK. andrew.barclay@ggc.scot.nhs.uk

AIMS:

To evaluate the outcome of Scottish children with extra hepatic biliary atresia (EHBA) since rationalisation of Kasai services to three English centres in 2002 (The 'Group A' centres).

METHODS:

All Scottish children with EHBA diagnosed between 2002 and 2009 were identified via the Scottish Society of Paediatric Gastroenterology, Hepatology and Nutrition (SSPGHAN) clinicians. A case-note review was conducted with demographics, presentation and outcome data recorded. These data were compared with historical Scottish data and data published previously by the supraregional liver units.

RESULTS:

25 patients were identified, of whom 22 were referred for Kasai in the group A centres, and of whom 19 had a Kasai. 2 year transplant-free survival (TFS) was significantly lower in the SSPGHAN 2002-2009 group than the group A centres in (1) (6/18 (33%) vs 36/57 (63%), p=0.023).

CONCLUSIONS:

These postrationalisation data are disappointing. The emphasis for care will now focus on improved communication between, primary care, general paediatricians and surgical centres through regional and national managed clinical networks, aiming to improve future outcomes for Scottish children with BA.

 

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Portoenterostomy > Outcome

Improving outcomes of biliary atresia: French national series 1986-2009.

Chardot C, Buet C, Serinet MO, Golmard JL, Lachaux A, Roquelaure B, Gottrand F, Broué P, Dabadie A, Gauthier F, Jacquemin E.

J Hepatol. 2013 Jun;58(6):1209-17

Observatoire français de l'atrésie des voies biliaires, Hôpital Necker - Enfants malades, Université Paris Descartes, Paris, France. Electronic address: christophe.chardot@nck.aphp.fr.

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Abstract


Outcome

Improving outcomes of biliary atresia: French national series 1986-2009.

Chardot C, Buet C, Serinet MO, Golmard JL, Lachaux A, Roquelaure B, Gottrand F, Broué P, Dabadie A, Gauthier F, Jacquemin E.

J Hepatol. 2013 Jun;58(6):1209-17

Observatoire français de l'atrésie des voies biliaires, Hôpital Necker - Enfants malades, Université Paris Descartes, Paris, France. Electronic address: christophe.chardot@nck.aphp.fr.

BACKGROUND & AIMS:

This study analyses the prognosis of biliary atresia (BA) in France since liver transplantation (LT) became widely available.

METHODS:

The charts of all BA patients living in France and born between 1986 and 2009 were reviewed. Patients were divided into 3 cohorts according to their years of birth: 1986-1996, 1997-2002, and 2003-2009.

RESULTS:

1107 BA children were identified, 990 born in metropolitan France (incidence 1/18,400 live births). Kasai operation was performed in 1044 (94%), leading to complete clearance of jaundice (total serum bilirubin⩽20μmol/L) in 38% of patients. Survival with native liver (SNL) after Kasai operation was 40%, 36%, and 30% at 5, 10, and 20years, stable in the 3 cohorts. Median age at Kasai operation was 59days, unchanged over time. Twenty-year SNL was 39%, 32%, 28%, and 19% after Kasai operation performed in the first, second, third months of life or thereafter (p=0.0002). 588 children underwent 692 LTs. Mortality without transplantation decreased over time: 16%, 7%, and 4% in the 3 cohorts (p<0.0001). Survival after transplantation was 83%, 82%, and 77% at 5, 10, and 20years in the whole series. Five-year post-transplant survival was 75%, 90%, and 89% in the 3 cohorts (p<0.0001). In the whole series, overall BA patient survival was 81%, 80%, and 77% at 5, 10, and 20years. Five-year BA patient overall survival increased over time: 72%, 88%, and 89% in the 3 cohorts (p<0.0001).

CONCLUSIONS:

BA patients currently have an 89% live expectancy, and a 30% chance to reach adulthood without transplantation. Early Kasai operation, without age threshold, reduces the need for liver transplantation until adulthood.

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Etiology & basic research > Animal model

Clues to the etiology of bile duct injury in biliary atresia.

Mack CL, Feldman AG, Sokol RJ.

Semin Liver Dis. 2012 Nov;32(4):307-16

Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, and Digestive Health Institute, Children's Hospital Colorado, Aurora, Colorado 80045, USA.

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Abstract


Animal model

Clues to the etiology of bile duct injury in biliary atresia.

Mack CL, Feldman AG, Sokol RJ.

Semin Liver Dis. 2012 Nov;32(4):307-16

Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Colorado School of Medicine, and Digestive Health Institute, Children's Hospital Colorado, Aurora, Colorado 80045, USA.

Biliary atresia (BA) is an infantile obstructive cholangiopathy of unknown etiology with suboptimal therapy, which is responsible for 40 to 50% of all pediatric liver transplants. Although the etiology of bile duct injury in BA in unknown, it is postulated that a pre- or perinatal viral infection initiates cholangiocyte apoptosis and release of antigens that trigger a Th1 immune response that leads to further bile duct injury, inflammation, and obstructive fibrosis. Humoral immunity and activation of the innate immune system may also play key roles in this process. Moreover, recent investigations from the murine BA model and human data suggest that regulatory T cells and genetic susceptibility factors may orchestrate autoimmune mechanisms. What controls the coordination of these events, why the disease only occurs in the first few months of life, and why a minority of infants with perinatal viral infections develop BA are remaining questions to be answered.

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Liver transplantation

Current options for management of biliary atresia.

Gallo A, Esquivel CO.

Pediatr Transplant. 2013 Mar;17(2):95-8

Division of Abdominal Transplantation, Stanford University School of Medicine, Lucile Packard Children's Hospital at Stanford, Stanford, CA 94305, USA.

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Abstract


Liver transplantation

Current options for management of biliary atresia.

Gallo A, Esquivel CO.

Pediatr Transplant. 2013 Mar;17(2):95-8

Division of Abdominal Transplantation, Stanford University School of Medicine, Lucile Packard Children's Hospital at Stanford, Stanford, CA 94305, USA.

It is encouraging that we are improving the technical aspects of treatment modalities for biliary atresia. However, it is clear that more needs to be done to best develop new treatment plans while applying the modalities we have (porto-enterostomy or liver transplantation or both) in a way that will afford the best survival and quality-of-life. This review article will discuss a number of points that are vital to improving care and illustrates the need to further scrutinize treatment decisions.

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Review and overview papers

International incidence and outcomes of biliary atresia.

Jimenez-Rivera C, Jolin-Dahel KS, Fortinsky KJ, Gozdyra P, Benchimol EI.

J Pediatr Gastroenterol Nutr. 2013 Apr;56(4):344-54

Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada. cajimenez@cheo.on.ca

show abstract

Abstract


Review and overview papers

International incidence and outcomes of biliary atresia.

Jimenez-Rivera C, Jolin-Dahel KS, Fortinsky KJ, Gozdyra P, Benchimol EI.

J Pediatr Gastroenterol Nutr. 2013 Apr;56(4):344-54

Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada. cajimenez@cheo.on.ca

OBJECTIVES:

International trends in incidence and outcomes of biliary atresia (BA) are controversial and a wide range of estimates have been reported worldwide. We reviewed the population-based literature to assess international variation of BA incidence and outcomes, and to assess the evidence for seasonal variation in incidence, centralization of Kasai hepatoportoenterostomy, and newborn screening.

METHODS:

We conducted a systematic review (registration number CRD42011001441) of observational or interventional research within MEDLINE, EMBASE, and the Cochrane Database, which reported incidence, prevalence, or outcomes of infants with BA. Population-based studies, defined by inclusion of an entire population or representative sample, were included. Outcomes included overall survival, native liver survival (NLS), and time to Kasai hepatoportoenterostomy. Single- or multicenter studies were excluded unless those centers captured all potential patients within a jurisdiction. Two independent data extractors reviewed the abstracts and articles.

RESULTS:

A total of 40 studies were included following review of 3128 references. A wide range of incidence was reported internationally. Ten-year overall survival ranged from 66.7% to 89%. NLS ranged from 20.3% to 75.8% at 1 to 3 years and 24% to 52.8% at 10 years. Earlier age at Kasai was a predictor of improved NLS. Seasonality was reported in 11 studies, and 3 reported an increased incidence during the months of August to March. The evidence for centralization of Kasai to high-volume centers is promising but does not account for all case-mix, provider, or health system factors involved in volume-outcome relations. Stool color card screening resulted in earlier Kasai and improved NLS in Taiwan.

CONCLUSIONS:

Large, international studies could help fill the gaps in knowledge identified by this review.

 

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Portoenterostomy > Outcome

Early surgical outcomes and pathological scoring values of older infants (? 90 d old) with biliary atresia.

Chen G, Zheng S, Sun S, Xiao X, Ma Y, Shen W, Chen L, Song Z.

J Pediatr Surg. 2012 Dec;47(12):2184-8

Surgical Department, Children's Hospital of Fudan University, Shanghai 201102, China. chengongzlp@hotmail.com

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Abstract


Outcome

Early surgical outcomes and pathological scoring values of older infants (? 90 d old) with biliary atresia.

Chen G, Zheng S, Sun S, Xiao X, Ma Y, Shen W, Chen L, Song Z.

J Pediatr Surg. 2012 Dec;47(12):2184-8

Surgical Department, Children's Hospital of Fudan University, Shanghai 201102, China. chengongzlp@hotmail.com

PURPOSE:

This study aimed to analyze the impact of age at Kasai operation on the short-term outcome of type III biliary atresia (BA) and to discuss if pathological scoring can be the prediction of effect in the Kasai procedure for the older (≥ 90 days) infant.

METHODS:

During the period 2004 through 2010, 452 infants with type III BA were reviewed. The relationship between ages at Kasai operation and jaundice clearance rates and two year native liver survival rates were assessed, retrospectively. Pathological slides were analyzed with a histological scoring system.

RESULTS:

All of the patients were divided into 3 groups according to their ages at operation (group A: aged 60 days or less (n=146), group B: age between 60 and 90 days (n=222) , and group C: age on or over 90 days (n=84)). The worst outcome of clearance of jaundice was found in group A but not in group C 2 weeks after the operation (P<0.05). Jaundice clearance rates showed no difference among the three groups either at 3-months or 6-months after operation. Moreover, in group C patients, the pathological scores showed no difference between the jaundice clearance group and jaundice persistence group 6 months after surgery. In group C, two year survival rate of patients with native livers was 36.1%.

CONCLUSION:

Some patients over 90 days of age at surgery can still benefit from a Kasai procedure. The pathological scoring system does not play a role in predicting jaundice clearance in patients over 90 days of age at surgery.

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Portoenterostomy > Outcome

Detection of hepatotropic viruses has no impact on the prognosis after Kasai procedure.

Schukfeh N, Al-Gamrah A, Petersen C, Kuebler JF.

J Pediatr Surg. 2012 Oct;47(10):1828-32.

Department of Pediatric Surgery of Hannover Medical School, Hannover, Germany. nagoud.schukfeh@uk-essen.de

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Abstract


Outcome

Detection of hepatotropic viruses has no impact on the prognosis after Kasai procedure.

Schukfeh N, Al-Gamrah A, Petersen C, Kuebler JF.

J Pediatr Surg. 2012 Oct;47(10):1828-32.

Department of Pediatric Surgery of Hannover Medical School, Hannover, Germany. nagoud.schukfeh@uk-essen.de

BACKGROUND/PURPOSE:

A viral origin of biliary atresia (BA) is discussed, and several studies have demonstrated different viral strains in liver biopsies of patients undergoing Kasai portoenterostomy. We hypothesized that the presence of hepatotropic viruses in patients undergoing portoenterostomy contributes to the progression of the disease and negatively affect the outcome.

METHODS:

Liver biopsies were prospectively taken from 70 patients undergoing portoenterostomy in our department from April 1996 to April 2004. Samples were screened by polymerase chain reaction for all common hepatic viruses. Primary outcome parameter was survival with the native liver. Secondary parameters were postoperative serum activity of liver enzymes and serum bilirubin levels at different time points. Patients underwent regular follow-up until October 2008.

RESULTS:

Twenty-eight patients (40%) were positive for 1 or more hepatotropic viruses. Four patients were lost to follow-up. In the remaining 66 patients, there was no significant difference in survival with their native liver between virus-positive and virus-negative patients. After a mean follow-up of 7.7 years (range, 4.6-16.1 years), 15 (23%) of 66 patients still lived with their native liver. There was no difference in liver enzymes, C-reactive protein, or bilirubin at any time point between both groups.

CONCLUSION:

A significant number of our patients tested positive for hepatotropic viruses in liver biopsies at the time of the Kasai procedure, but the presence of virus had no influence on the course of BA. This suggests that the ongoing inflammatory process of BA leading to liver cirrhosis in most Kasai-treated patients is not affected by hepatotropic viruses. Our data question the necessity to aggressively screen for and treat viral infections in patients with BA.

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Etiology & basic research > Epidemiology - Genetics

+276 G/T single nucleotide polymorphism of the adiponectin gene is associated with the susceptibility to biliary atresia.

Udomsinprasert W, Tencomnao T, Honsawek S, Anomasiri W, Vejchapipat P, Chongsrisawat V, Poovorawan Y.

World J Pediatr. 2012 Nov;8(4):328-34

Program in Medical Sciences, Department of Biochemistry, Faculty of Medicine, Chulalongkorn University, Bangkok, 10330, Thailand.

show abstract

Abstract


Epidemiology - Genetics

+276 G/T single nucleotide polymorphism of the adiponectin gene is associated with the susceptibility to biliary atresia.

Udomsinprasert W, Tencomnao T, Honsawek S, Anomasiri W, Vejchapipat P, Chongsrisawat V, Poovorawan Y.

World J Pediatr. 2012 Nov;8(4):328-34

Program in Medical Sciences, Department of Biochemistry, Faculty of Medicine, Chulalongkorn University, Bangkok, 10330, Thailand.

BACKGROUND:

Biliary atresia (BA) is an intractable neonatal inflammatory and obliterative cholangiopathy, leading to progressive fibrosis and cirrhosis. Adiponectin, an anti-inflammatory adipokine, is known to play a possible role in liver diseases. The objective of our study was to determine the relationship between adiponectin gene polymorphisms and BA susceptibility.

METHODS:

A total of 106 BA patients and 107 healthy controls were included in this study. Two single nucleotide polymorphisms (SNPs) of the adiponectin gene, +45T/G (rs2241766) and +276G/T (rs1501299), were genotyped by polymerase chain reaction-restriction fragment length polymorphism analysis.

RESULTS:

Genotype distributions of +45 T/G and +276 G/T SNPs were seen in the Hardy-Weinberg equilibrium for both BA patients and controls. The frequency of the G/G genotype at +276G/T was significantly higher in BA patients than in the controls (P=0.009). Regarding +45T/ G in BA patients, the frequency of the T/T genotype tended to be lower than in the controls, but the difference was not significant. Moreover, the G allele at +276G/T in BA patients was more common than in the controls (P=0.0043). In contrast, the frequency of the T allele at +45T/G was not significantly different between BA patients and the controls. None of the haplotypes studied was found to significantly influence the risk of BA.

CONCLUSIONS:

+276G/T SNP is strongly associated with BA, particularly with the G allele. We postulate that the +276G/T adiponectin gene polymorphism confers increased susceptibility to BA.

 

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Portoenterostomy > Technical aspects

Laparoscopic versus open Kasai portoenterostomy in infant with biliary atresia: a retrospective review on the 5-year native liver survival.

Chan KW, Lee KH, Tsui SY, Wong YS, Pang KY, Mou JW, Tam YH.

Pediatr Surg Int. 2012 Nov;28(11):1109-13

Division of Paediatric Surgery and Paediatric Urology, Department of Surgery, The Chinese University of Hong Kong, The Prince of Wales Hospital, Hong Kong SAR, China. edwinchan@surgery.cuhk.edu.hk

show abstract

Abstract


Technical aspects

Laparoscopic versus open Kasai portoenterostomy in infant with biliary atresia: a retrospective review on the 5-year native liver survival.

Chan KW, Lee KH, Tsui SY, Wong YS, Pang KY, Mou JW, Tam YH.

Pediatr Surg Int. 2012 Nov;28(11):1109-13

Division of Paediatric Surgery and Paediatric Urology, Department of Surgery, The Chinese University of Hong Kong, The Prince of Wales Hospital, Hong Kong SAR, China. edwinchan@surgery.cuhk.edu.hk

PURPOSE:

Laparoscopic Kasai portoenterostomy was reported to be a safe and feasible procedure in infant with biliary atresia. We aimed to investigate the long-term results after laparoscopic portoenterostomy as such data in the literature are lacking.

METHODS:

Sixteen infants underwent laparoscopic Kasai portoenterostomy from 2002 to 2006. The age and the sex of the patient, the bilirubin level before the operation, the early clearance of jaundice (total bilirubin <20 μmol/L within 6 months of portoenterostomy), the native liver survival at 2 and 5 years after the operation were reviewed. The results were retrospectively compared with 16 consecutive infants who underwent open Kasai portoenterostomy before 2002.

RESULTS:

All infants had type III biliary atresia. The early clearance of jaundice rate at 6 months was 50 % (8/16) after laparoscopic operation and was 75 % (12/16) after open operation (p = 0.144). Two years after the operation, the native liver survival was 50 % (8/16) in the laparoscopic group and was 81 % (13/16) in the open group (p = 0.076). Five years after the operation, the native liver survival rate was 50 % (8/16) in the laparoscopic group and was 81 % (13/16) in the open group (p = 0.076). The jaundice-free native liver survival rate at 5 years was 50 % (8/16) in laparoscopic group and was 75 % (12/16) in the open group. In the laparoscopic group, all patients with early clearance of jaundice survived and remained jaundice freed 5 years after the operation.

CONCLUSION:

The 5-year native liver survival rate after laparoscopic portoenterostomy was 50 %. Apparently superior result was observed in the open group (81 %) although the figures did not reach statistical difference because of the small sample size. A larger scale study is required to draw a more meaningful conclusion.

 

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Diagnosis and therapy

Early detection of biliary atresia: past, present & future.

Muraji T.

Expert Rev Gastroenterol Hepatol. 2012 Sep;6(5):583-9

Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito, Ibaraki 311-4145, Japan. t-muraji@ibaraki-kodomo.com

show abstract

Abstract


Diagnosis and therapy

Early detection of biliary atresia: past, present & future.

Muraji T.

Expert Rev Gastroenterol Hepatol. 2012 Sep;6(5):583-9

Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito, Ibaraki 311-4145, Japan. t-muraji@ibaraki-kodomo.com

The age threshold at the time of Kasai hepatic portoenterostomy associated with a prognosis of biliary atresia (BA) is becoming clearer as 10-year native liver survival data become more frequent in the recent literature, whereas the age at diagnosis has not dramatically decreased during the last 3 decades. A stool color card screening implemented in 1994 in Japan is now expanding worldwide. However, the contribution of this modality will probably be limited because of the nature of this disease, for example, 'progressive obliterative cholangiopathy'. A cholic stool was actually observed only in 50% before diagnosis according to the Japanese BA Registry data. Thus, color card screening does not appear to be instrumental in detecting patients with BA early enough before 1 month of age. A highly sensitive, adequately specific, noninvasive and quantitative method may be expensive, but the overall cost would be lower than that of liver transplant.

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Diagnosis and therapy

How reliably can paediatric professionals identify pale stool from cholestatic newborns?

Bakshi B, Sutcliffe A, Akindolie M, Vadamalayan B, John S, Arkley C, Griffin LD, Baker A.

Arch Dis Child Fetal Neonatal Ed. 2012 Sep;97(5):F385-7.

King’s College Hospital, London, UK.

show abstract

Abstract


Diagnosis and therapy

How reliably can paediatric professionals identify pale stool from cholestatic newborns?

Bakshi B, Sutcliffe A, Akindolie M, Vadamalayan B, John S, Arkley C, Griffin LD, Baker A.

Arch Dis Child Fetal Neonatal Ed. 2012 Sep;97(5):F385-7.

King’s College Hospital, London, UK.

BACKGROUND:

The success of surgery in infants with hepatobiliary disease is inversely proportional to the age when surgery was performed. Pale stool colour is a major indicator of biliary obstruction. However, simple recognition has been inadequate, resulting in late diagnosis and referral. Objective To assess the skills of healthcare professionals in recognising pale stools.

METHOD:

Photographs of normal, acholic and indeterminate infant stools were shown to paediatric professionals who have regular contact with jaundiced babies at three London teaching hospitals. Each stool was classified as 'healthy' or 'suspect'.

RESULTS:

One-third of the stools were not correctly identified by physicians and nurses.

CONCLUSION:

Experienced professionals often do not recognise stool colour associated with biliary obstruction. The authors propose that stool colour cards similar to those used in Japan and Taiwan may improve early detection of hepatobiliary disease at a minimal cost.

 

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Diagnosis and therapy > Drug therapy

Efficacy of fat-soluble vitamin supplementation in infants with biliary atresia.

Shneider BL, Magee JC, Bezerra JA, Haber B, Karpen SJ, Raghunathan T, Rosenthal P, Schwarz K, Suchy FJ, Kerkar N, Turmelle Y, Whitington PF, Robuck PR, Sokol RJ; Childhood Liver Disease Research Education Network (ChiLDREN).

Pediatrics. 2012 Sep;130(3):e607-14.

Department of Pediatrics, Children’s Hospital Pittsburgh of UPMC, Pittsburgh, PA 15224, USA. benjamin.shneider@chp.edu

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Abstract


Drug therapy

Efficacy of fat-soluble vitamin supplementation in infants with biliary atresia.

Shneider BL, Magee JC, Bezerra JA, Haber B, Karpen SJ, Raghunathan T, Rosenthal P, Schwarz K, Suchy FJ, Kerkar N, Turmelle Y, Whitington PF, Robuck PR, Sokol RJ; Childhood Liver Disease Research Education Network (ChiLDREN).

Pediatrics. 2012 Sep;130(3):e607-14.

Department of Pediatrics, Children’s Hospital Pittsburgh of UPMC, Pittsburgh, PA 15224, USA. benjamin.shneider@chp.edu

OBJECTIVE:

Cholestasis predisposes to fat-soluble vitamin (FSV) deficiencies. A liquid multiple FSV preparation made with tocopheryl polyethylene glycol-1000 succinate (TPGS) is frequently used in infants with biliary atresia (BA) because of ease of administration and presumed efficacy. In this prospective multicenter study, we assessed the prevalence of FSV deficiency in infants with BA who received this FSV/TPGS preparation.

METHODS:

Infants received FSV/TPGS coadministered with additional vitamin K as routine clinical care in a randomized double-blinded, placebo-controlled trial of corticosteroid therapy after hepatoportoenterostomy (HPE) for BA (identifier NCT 00294684). Levels of FSV, retinol binding protein, total serum lipids, and total bilirubin (TB) were measured 1, 3, and 6 months after HPE.

RESULTS:

Ninety-two infants with BA were enrolled in this study. Biochemical evidence of FSV insufficiency was common at all time points for vitamin A (29%-36% of patients), vitamin D (21%-37%), vitamin K (10%-22%), and vitamin E (16%-18%). Vitamin levels were inversely correlated with serum TB levels. Biochemical FSV insufficiency was much more common (15%-100% for the different vitamins) in infants whose TB was ≥2 mg/dL. At 3 and 6 months post HPE, only 3 of 24 and 0 of 23 infants, respectively, with TB >2 mg/dL were sufficient in all FSV.

CONCLUSIONS:

Biochemical FSV insufficiency is commonly observed in infants with BA and persistent cholestasis despite administration of a TPGS containing liquid multiple FSV preparation. Individual vitamin supplementation and careful monitoring are warranted in infants with BA, especially those with TB >2 mg/dL.

 

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Etiology & basic research

Biliary atresia: cellular dynamics and immune dysregulation.

Feldman AG, Mack CL

Semin Pediatr Surg. 2012 Aug;21(3):192-200

Section of Pediatric Gastroenterology, Hepatology and Nutrition, Digestive Health Institute, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO 80045, USA.

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Abstract


Etiology & basic research

Biliary atresia: cellular dynamics and immune dysregulation.

Feldman AG, Mack CL

Semin Pediatr Surg. 2012 Aug;21(3):192-200

Section of Pediatric Gastroenterology, Hepatology and Nutrition, Digestive Health Institute, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, CO 80045, USA.

The cause of biliary atresia is unknown; in the past few decades, the majority of investigations related to its pathogenesis have centered on viral infections and immunity. The acquired or perinatal form of biliary atresia entails a progressive inflammatory injury of bile ducts, leading to fibrosis and obliteration of both the extrahepatic and intrahepatic bile ducts. Theories of pathogenesis include viral infection, chronic inflammatory or autoimmune-mediated bile duct injury, and abnormalities in bile duct development. This review will focus solely on human studies pertaining to a potential viral trigger of bile duct injury at diagnosis and provide insight into the interplay of the innate and adaptive immune responses in the pathogenesis of disease.

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Etiology & basic research > Animal model

Biliary atresia: the animal models.

Petersen C.

Semin Pediatr Surg. 2012 Aug;21(3):185-91

Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany. petersen.claus@mh-hannover.de

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Abstract


Animal model

Biliary atresia: the animal models.

Petersen C.

Semin Pediatr Surg. 2012 Aug;21(3):185-91

Department of Pediatric Surgery, Hannover Medical School, Hannover, Germany. petersen.claus@mh-hannover.de

Biliary atresia (BA) is a progressive fibrosing process of the neonatal biliary tree and liver, of unknown origin, and an as-yet unexplained pathologic mechanism. The crucial point is to elucidate the origin of this rare disease to change palliative surgery to etiology-related procedures. Patient-based research can only begin at the time of the Kasai procedure and does not allow retracing of the pathology back to its origin. Basic research has focused on similar diseases in the veterinary literature and started to simulate BA in animal models. Unfortunately, even after 50 years of research, no knowledge has been gained from such models, which has led to a single clinical application. This article reviews BA in the context of the animal models available and discusses whether future studies are promising or futile.

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Review and overview papers

Biliary atresia: clinical aspects

Davenport M.

Semin Pediatr Surg. 2012 Aug;21(3):175-84

Department of Paediatric Surgery, King's College Hospital, Denmark Hill, London, United Kingdom. Markdav2@ntlworld.com

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Abstract


Review and overview papers

Biliary atresia: clinical aspects

Davenport M.

Semin Pediatr Surg. 2012 Aug;21(3):175-84

Department of Paediatric Surgery, King's College Hospital, Denmark Hill, London, United Kingdom. Markdav2@ntlworld.com

Biliary atresia (BA) remains an enigmatic disease with a degree of etiologic heterogeneity. A number of variants can be defined clinically, and these include the syndromic group (typically BA splenic malformation), cystic BA, and cytomegalovirus (CMV) IgM +ve associated BA. The remainder, and still the largest group, may be termed isolated BA(.) There is a wide variation in incidence across the globe from 1 in 5000 in Taiwan to 1 in 20,000 live births in Northern Europe, although the reasons for such a disparity remain obscure. Management remains primarily surgical with an attempt to restore bile flow by resection of extrahepatic biliary remnants and a reconstruction portoenterostomy (the Kasai procedure), reserving liver transplantation for those where this fails or complications of chronic liver disease supervene. Clearance of jaundice to normal values has been achieved in 40%-55% of cases in large series from around the world, with an expectation of 5-year native liver survival of similar proportions.

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Diagnosis and therapy

Endoscopic retrograde cholangiopancreatography in neonatal cholestasis.

Shteyer E, Wengrower D, Benuri-Silbiger I, Gozal D, Wilschanski M, Goldin E.

J Pediatr Gastroenterol Nutr. 2012 Aug;55(2):142-5

Pediatric Gastroenterology Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. eyals@hadassah.org.il

show abstract

Abstract


Diagnosis and therapy

Endoscopic retrograde cholangiopancreatography in neonatal cholestasis.

Shteyer E, Wengrower D, Benuri-Silbiger I, Gozal D, Wilschanski M, Goldin E.

J Pediatr Gastroenterol Nutr. 2012 Aug;55(2):142-5

Pediatric Gastroenterology Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. eyals@hadassah.org.il

BACKGROUND:

Endoscopic retrograde cholangiopancreatography (ERCP) is not as widely used in children as in adults and is performed in few specialized centers. The aim of the present study was to review the experience of ERCP in children younger than 3 months in a national referral center.

METHODS:

A retrospective chart review was performed of all of the babies younger than 3 months who underwent ERCP between 2000 and 2010. Data on demographics, diagnosis, type of anesthesia, treatments, and complications were collected.

RESULTS:

A total of 27 babies, 14 boys, were examined. Median age was 55 days (range 33-89). Ultrasound was normal in 16 infants, whereas others included small gallbladder (4), biliary stones (3), and dilated bile ducts (3). Thirteen infants underwent earlier liver biopsy, which was inconclusive. ERCP led to the diagnosis of biliary atresia in 13 infants who had subsequent surgery. In others, ERCP showed choledochal cyst (1), biliary stones (2), dilated bile ducts (1), and normal examination (6); there were 5 failures. The final diagnoses in our cohort were extrahepatic biliary atresia (15), biliary stones (5), neonatal hepatitis (4), choledochal cyst (1), paucity of intrahepatic bile duct (1), and congenital hepatic fibrosis (1). Diagnoses in the failed ERCP group included biliary atresia (2), bile duct paucity (1), and biliary stones (2). In 4 (19%) infants with clinical suspicion of extrahepatic biliary atresia, a normal ERCP ruled out the diagnosis and avoided an intraoperative cholangiogram. No complications, including pancreatitis, were reported.

CONCLUSIONS:

ERCP in infants is feasible and has no complications. It may serve as an additional diagnostic tool in neonatal cholestasis in inconclusive cases and may prevent more invasive procedures. ERCP may be part of the algorithm of neonatal cholestasis when it is available and other investigations fail to confirm a diagnosis.

 

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Claus Petersen | Department of Pediatric Surgery | Hannover Medical School | Carl-Neuberg-Strasse 1 | 30625 Hannover, Germany
Tel.: +49-(0)511-532-9040 | Fax: +49-(0)511-532-8052 | www.bard-online.com | biliary-atresia@mh-hannover.de