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J Hepatol. 2014 Jul;61(1):116-23. doi: 10.1016/j.jhep.2014.03.016. Epub 2014 Mar 18.

Design and validation of a diagnostic score for biliary atresia.

Author information

1
Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
2
Department of Pediatric Hepatology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt. Electronic address: msira@liver-eg.org.
3
Department of Radiology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.
4
Department of Pathology, National Liver Institute, Menofiya University, 32511 Shebin El-koom, Menofiya, Egypt.

Erratum in

  • J Hepatol. 2015 Jul;63(1):289.

Abstract

BACKGROUND & AIMS:

The dilemma of early diagnosis of biliary Atresia (BA), particularly distinguishing it from other causes of neonatal cholestasis is challenging. The aim was to design and validate a scoring system for early discrimination of BA from other causes of neonatal cholestasis.

METHODS:

A twelve-point scoring system was proposed according to clinical, laboratory, ultrasonographic, and histopathological parameters. A total of 135 patients with neonatal cholestasis in two sets were recruited to design (n=60) and validate (n=75) a scoring system. Parameters with significant statistical difference between BA (n=30) and non-BA (n=30) patients in the design set were analyzed by logistic regression to predict the presence or absence of BA then a scoring system was designed and validated.

RESULTS:

The total score ranged from 0 to 37.18 and a cut-off value of >23.927 could discriminate BA from other causes of neonatal cholestasis with sensitivity and specificity of 100% each. By applying this score in the validation set, the accuracy was 98.83% in predicting BA. The diagnosis of BA was proposed correctly in 100% and the diagnosis of non-BA was proposed correctly in 97.67% of patients. By applying this model, unnecessary intraoperative cholangiography would be avoided in non-BA patients.

CONCLUSIONS:

This scoring system accurately separates infants with BA and those with non-BA, rendering intraoperative cholangiography for confirming or excluding BA unnecessary in a substantial proportion of patients.

KEYWORDS:

Clay stool; Doppler ultrasonography; Hepatic subcapsular flow; Liver biopsy; Neonatal cholestasis

PMID:
24657403
DOI:
10.1016/j.jhep.2014.03.016
[Indexed for MEDLINE]

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