Skip to main content
Top
Published in: BMC Pediatrics 1/2019

Open Access 01-12-2019 | Nutrition | Case report

A case report of a challenging diagnosis of biliary atresia in a patient receiving total parenteral nutrition

Authors: Atu Agawu, Andrew Wehrman, Jennifer Pogoriler, Natalie A. Terry, Henry C. Lin

Published in: BMC Pediatrics | Issue 1/2019

Login to get access

Abstract

Background

Total parenteral nutrition (TPN) and biliary atresia (BA) are common causes of cholestasis in infancy. The diagnosis of BA is time sensitive due to an inverse correlation between age at intervention (hepatic portoenterostomy - HPE) and survival without liver transplantation. Clinical, laboratory, and histologic features of BA and parenteral nutrition associated cholestasis (PNAC) are similar, creating a diagnostic dilemma for cholestatic infants on parenteral nutrition. There is limited published information about the natural history of PNAC including time to resolution, or diagnostic tests that distinguish BA from other etiologies of cholestasis.

Case presentation

We present a case of a child diagnosed with BA whose cholestasis began while receiving TPN. His clinical course was notable for transient resolution of his cholestasis after stopping parenteral nutrition and ultimate intraoperative diagnosis.

Conclusions

Clinicians who care for patients who frequently receive TPN should be aware that clinical, laboratory, imaging, and biopsy findings can be similar between BA and PNAC.
Literature
1.
go back to reference Mieli-Vergani G, Hadžic N. Biliary atresia and neonatal disorders of the bile ducts. In: Wyllie R, Hyams JS, Kay M, editors. Pediatric gastrointestinal and liver disease. 5th ed. Philadelphia: Elsevier; 2016. p. 838–50. Mieli-Vergani G, Hadžic N. Biliary atresia and neonatal disorders of the bile ducts. In: Wyllie R, Hyams JS, Kay M, editors. Pediatric gastrointestinal and liver disease. 5th ed. Philadelphia: Elsevier; 2016. p. 838–50.
5.
go back to reference Shneider BL, Magee JC, Karpen SJ, Rand EB, Narkewicz MR, Bass LM, et al. Total serum bilirubin within 3 months of Hepatoportoenterostomy predicts short-term outcomes in biliary atresia. J Pediatr. 2016;170:211–7.CrossRef Shneider BL, Magee JC, Karpen SJ, Rand EB, Narkewicz MR, Bass LM, et al. Total serum bilirubin within 3 months of Hepatoportoenterostomy predicts short-term outcomes in biliary atresia. J Pediatr. 2016;170:211–7.CrossRef
10.
go back to reference Russo P, Magee JC, Anders RA, Bove KE, Chung C, Cummings OW, et al. Key histopathologic features of liver biopsies that distinguish biliary atresia from other causes of infantile cholestasis and their correlation with outcome: a multicenter study. Am J Surg Pathol. 2016;40:1601–15.CrossRef Russo P, Magee JC, Anders RA, Bove KE, Chung C, Cummings OW, et al. Key histopathologic features of liver biopsies that distinguish biliary atresia from other causes of infantile cholestasis and their correlation with outcome: a multicenter study. Am J Surg Pathol. 2016;40:1601–15.CrossRef
11.
go back to reference Kianifar HR, Tehranian S, Shojaei P, Adinehpoor Z, Sadeghi R, Kakhki VRD, et al. Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature. Pediatr Radiol. 2013;43:905–19.CrossRef Kianifar HR, Tehranian S, Shojaei P, Adinehpoor Z, Sadeghi R, Kakhki VRD, et al. Accuracy of hepatobiliary scintigraphy for differentiation of neonatal hepatitis from biliary atresia: systematic review and meta-analysis of the literature. Pediatr Radiol. 2013;43:905–19.CrossRef
12.
go back to reference Russo P, Magee JC, Boitnott J, Bove KE, Raghunathan T, Finegold M, et al. Design and validation of the biliary atresia research consortium histologic assessment system for cholestasis in infancy. Clin Gastroenterol Hepatol. 2011;9:357–362.e2.CrossRef Russo P, Magee JC, Boitnott J, Bove KE, Raghunathan T, Finegold M, et al. Design and validation of the biliary atresia research consortium histologic assessment system for cholestasis in infancy. Clin Gastroenterol Hepatol. 2011;9:357–362.e2.CrossRef
Metadata
Title
A case report of a challenging diagnosis of biliary atresia in a patient receiving total parenteral nutrition
Authors
Atu Agawu
Andrew Wehrman
Jennifer Pogoriler
Natalie A. Terry
Henry C. Lin
Publication date
01-12-2019
Publisher
BioMed Central
Keyword
Nutrition
Published in
BMC Pediatrics / Issue 1/2019
Electronic ISSN: 1471-2431
DOI
https://doi.org/10.1186/s12887-019-1446-2

Other articles of this Issue 1/2019

BMC Pediatrics 1/2019 Go to the issue