Note from the National Guideline Clearinghouse (NGC): The following key points summarize the content of the guideline recommendations. Refer to the full text for additional information, including detailed information about the initial evaluation of the jaundiced infant, the initial evaluation of the infant with conjugated hyperbilirubinemia, and diagnostic tests used for further evaluation of the infant with cholestasis.
Levels of evidence (A-D) are defined at the end of "Major Recommendations" field.
It is recommended that any infant noted to be jaundiced at 2 weeks of age be clinically evaluated for cholestasis with measurement of total and direct serum bilirubin. However, breast-fed infants who can be reliably monitored and who have an otherwise normal history (no dark urine or light stools) and physical examination may be asked to return at 3 weeks of age and, if jaundice persists, have measurement of total and direct serum bilirubin at that time. (C)
Retest any infant with an acute condition or other explanation for jaundice whose jaundice does not resolve with appropriate management of the diagnosed condition. (D)
Ultrasound is recommended for infants with cholestasis of unknown etiology. (A)
Liver biopsy is recommended for most infants with cholestasis of unknown etiology. (A)
Gamma-glutamyl transpeptidase (GGTP) and lipoprotein X are not routinely recommended in the evaluation of cholestasis in young infants. (C)
Scintigraphy and duodenal aspirate are not routinely recommended but may be useful in situations in which other tests are not readily available. (A)
Magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) are not routinely recommended, although ERCP may be useful in experienced hands. (C)
Definitions:
Coding Scheme for Quality of Evidence
Level A
Recommendation based on 2 or more studies that compared the test to a criterion standard in an independent, blind manner in an unselected population of infants similar to those addressed in the guideline.
Level B
Recommendation based on a single study that compared the test to a criterion standard in an independent, blind manner in an unselected population of infants similar to those addressed in he guideline.
Level C
Recommendation based on lower quality studies or studies for which inadequate information is provided to assess quality, together with expert opinion and consensus of the committee.
Level D
No studies available; recommendations based on expert opinion and consensus of the committee.