Guideline for the Evaluation of Cholestatic Jaundice in Infants

Publication Date: January 1, 2017
Last Updated: March 14, 2022

Recommendations

EVALUATION OF THE JAUNDICED INFANT

1. Any formula-fed infant noted to be jaundiced after 2 weeks of age should be evaluated for cholestasis with measurement of total and conjugated (direct) serum bilirubin. (Category IA)
Depending upon local practice, breast-fed babies that appear otherwise well may be followed clinically until 3 weeks of age, at which time if they appear icteric should then undergo serum evaluation of total and conjugated (direct) serum bilirubin.
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2. Measurements of serum bilirubin should always be fractionated into unconjugated (indirect) or conjugated (direct) hyperbilirubinemia (Category IA)
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3. Conjugated (direct) hyperbilirubinemia (>1.0 mg/dL, 17 ╬╝mol/L) is considered pathological and warrants diagnostic evaluation. (Category IA)
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PHYSICAL EXAMINATION

4. A thorough physical examination is crucial to the proper evaluation of the jaundiced infant. Attention to hepatomegaly, splenomegaly, and ill appearance warrants special considerations. (Category IA)
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5. Direct visualization of stool pigment is a key aspect of a complete evaluation of the jaundiced infant. (Category IA)
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LABORATORY EVALUATION

6. The abdominal ultrasound is useful in excluding choledochal cyst or gallstone disease causing extrahepatic bile duct obstruction. It may demonstrate an absent or abnormal gallbladder, or other features suggestive, but not diagnostic, of BA. (Category IA)
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7. Limited specificity precludes the use of the HBS scan as a stand-alone test in making a definitive diagnosis of BA. (Category IB)
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Definitively demonstrated bile flow by selective use of HBS may be of value in excluding BA. (Category IB)
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8. Limited specificity of MRCP, ERCP, PTCC provides a limited role in the general guidance to caregivers toward diagnosing BA in the present era. ()
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9. In the hands of an experienced pediatric pathologist, histopathological findings of bile duct proliferation, bile plugs, and fibrosis in an appropriately timed liver biopsy is the most supportive test in the evaluation of the infant with protracted conjugated hyperbilirubinemia. (Category IB)
Diseases other than BA that cause cholestasis can be determined via histologic examination of the liver.
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INTRAOPERATIVE CHOLANGIOGRAM

10. Evaluation by intraoperative cholangiogram and histological examination of the duct remnant is considered the gold standard to diagnose biliary atresia. (Category IA)
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Recommendation Grading

Disclaimer

Overview

Title

Guideline for the Evaluation of Cholestatic Jaundice in Infants

Authoring Organization

Publication Month/Year

January 1, 2017

Last Updated Month/Year

August 2, 2023

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Inclusion Criteria

Female, Male, Infant

Health Care Settings

Ambulatory, Hospital, Outpatient

Intended Users

Nurse, nurse practitioner, physician, physician assistant

Scope

Assessment and screening, Diagnosis, Management, Treatment

Diseases/Conditions (MeSH)

D007567 - Jaundice, Neonatal

Keywords

biliary atresia, hepatoportoenterostomy, Kasai, neonatal cholestasis, neonatal jaundice, radionuclide scan

Supplemental Methodology Resources

Data Supplement, Data Supplement