A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice

Timothy Jancelewicz, Rebecca Barmherzig, Catherine T.S. Chung, Simon C. Ling, Binita M. Kamath, Vicky L. Ng, Joao Amaral, Constance O'Connor, Annie Fecteau, Jacob C. Langer

Research output: Contribution to journalArticle

18 Citations (Scopus)

Abstract

Background Neonates with cholestasis may undergo many tests before biliary atresia (BA) or an alternative diagnosis is reached, and delayed intervention may worsen outcomes. An optimal diagnostic approach to reduce risk, cost, and delay has yet to be defined. The purpose of this study was to develop an algorithm that rapidly and accurately excludes BA for infants with cholestatic jaundice. Methods A single-center retrospective comparison of diagnostic workup was made between cholestatic infants with BA, and those without BA who underwent hepatobiliary iminodiacetic acid (HIDA) scan during admission. Patients were born between 2000 and 2010 and those older than 100 days at assessment were excluded. Sensitivity and specificity analysis of predictive variables was performed and an algorithm constructed. Results There were 45 BA and 167 non-BA patients. Some variables were 100% sensitive for the exclusion of BA: conjugated bilirubin < 2.5 mg/dL, gamma-glutamyl transpeptidase < 150 U/L, excretion on HIDA, or a normal percutaneous cholangiogram. Clinical variables and ultrasound were less useful as screening tests owing to low specificity and sensitivity, respectively. Liver biopsy was 98% sensitive and 84% specific in the diagnosis of BA. An algorithm was constructed that rules out BA with a negative laparotomy rate of 3-22%. Conclusion We propose a screening algorithm for infants with conjugated hyperbilirubinemia that permits efficient exclusion of BA with minimal invasive testing and with a low risk of negative laparotomy. This algorithm now requires prospective evaluation to determine its diagnostic accuracy and its ability to reduce hospital costs, patient morbidity, and time to Kasai portoenterostomy in patients with BA.

Original languageEnglish (US)
Pages (from-to)363-370
Number of pages8
JournalJournal of pediatric surgery
Volume50
Issue number3
DOIs
StatePublished - Mar 1 2015

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Biliary Atresia
Obstructive Jaundice
Laparotomy
Sensitivity and Specificity
Hyperbilirubinemia
gamma-Glutamyltransferase
Hospital Costs
Cholestasis
Bilirubin
Newborn Infant
Morbidity
Biopsy

All Science Journal Classification (ASJC) codes

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice. / Jancelewicz, Timothy; Barmherzig, Rebecca; Chung, Catherine T.S.; Ling, Simon C.; Kamath, Binita M.; Ng, Vicky L.; Amaral, Joao; O'Connor, Constance; Fecteau, Annie; Langer, Jacob C.

In: Journal of pediatric surgery, Vol. 50, No. 3, 01.03.2015, p. 363-370.

Research output: Contribution to journalArticle

Jancelewicz, T, Barmherzig, R, Chung, CTS, Ling, SC, Kamath, BM, Ng, VL, Amaral, J, O'Connor, C, Fecteau, A & Langer, JC 2015, 'A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice', Journal of pediatric surgery, vol. 50, no. 3, pp. 363-370. https://doi.org/10.1016/j.jpedsurg.2014.08.014
Jancelewicz, Timothy ; Barmherzig, Rebecca ; Chung, Catherine T.S. ; Ling, Simon C. ; Kamath, Binita M. ; Ng, Vicky L. ; Amaral, Joao ; O'Connor, Constance ; Fecteau, Annie ; Langer, Jacob C. / A screening algorithm for the efficient exclusion of biliary atresia in infants with cholestatic jaundice. In: Journal of pediatric surgery. 2015 ; Vol. 50, No. 3. pp. 363-370.
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AU - Barmherzig, Rebecca

AU - Chung, Catherine T.S.

AU - Ling, Simon C.

AU - Kamath, Binita M.

AU - Ng, Vicky L.

AU - Amaral, Joao

AU - O'Connor, Constance

AU - Fecteau, Annie

AU - Langer, Jacob C.

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N2 - Background Neonates with cholestasis may undergo many tests before biliary atresia (BA) or an alternative diagnosis is reached, and delayed intervention may worsen outcomes. An optimal diagnostic approach to reduce risk, cost, and delay has yet to be defined. The purpose of this study was to develop an algorithm that rapidly and accurately excludes BA for infants with cholestatic jaundice. Methods A single-center retrospective comparison of diagnostic workup was made between cholestatic infants with BA, and those without BA who underwent hepatobiliary iminodiacetic acid (HIDA) scan during admission. Patients were born between 2000 and 2010 and those older than 100 days at assessment were excluded. Sensitivity and specificity analysis of predictive variables was performed and an algorithm constructed. Results There were 45 BA and 167 non-BA patients. Some variables were 100% sensitive for the exclusion of BA: conjugated bilirubin < 2.5 mg/dL, gamma-glutamyl transpeptidase < 150 U/L, excretion on HIDA, or a normal percutaneous cholangiogram. Clinical variables and ultrasound were less useful as screening tests owing to low specificity and sensitivity, respectively. Liver biopsy was 98% sensitive and 84% specific in the diagnosis of BA. An algorithm was constructed that rules out BA with a negative laparotomy rate of 3-22%. Conclusion We propose a screening algorithm for infants with conjugated hyperbilirubinemia that permits efficient exclusion of BA with minimal invasive testing and with a low risk of negative laparotomy. This algorithm now requires prospective evaluation to determine its diagnostic accuracy and its ability to reduce hospital costs, patient morbidity, and time to Kasai portoenterostomy in patients with BA.

AB - Background Neonates with cholestasis may undergo many tests before biliary atresia (BA) or an alternative diagnosis is reached, and delayed intervention may worsen outcomes. An optimal diagnostic approach to reduce risk, cost, and delay has yet to be defined. The purpose of this study was to develop an algorithm that rapidly and accurately excludes BA for infants with cholestatic jaundice. Methods A single-center retrospective comparison of diagnostic workup was made between cholestatic infants with BA, and those without BA who underwent hepatobiliary iminodiacetic acid (HIDA) scan during admission. Patients were born between 2000 and 2010 and those older than 100 days at assessment were excluded. Sensitivity and specificity analysis of predictive variables was performed and an algorithm constructed. Results There were 45 BA and 167 non-BA patients. Some variables were 100% sensitive for the exclusion of BA: conjugated bilirubin < 2.5 mg/dL, gamma-glutamyl transpeptidase < 150 U/L, excretion on HIDA, or a normal percutaneous cholangiogram. Clinical variables and ultrasound were less useful as screening tests owing to low specificity and sensitivity, respectively. Liver biopsy was 98% sensitive and 84% specific in the diagnosis of BA. An algorithm was constructed that rules out BA with a negative laparotomy rate of 3-22%. Conclusion We propose a screening algorithm for infants with conjugated hyperbilirubinemia that permits efficient exclusion of BA with minimal invasive testing and with a low risk of negative laparotomy. This algorithm now requires prospective evaluation to determine its diagnostic accuracy and its ability to reduce hospital costs, patient morbidity, and time to Kasai portoenterostomy in patients with BA.

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