Know how to sequentially evaluate neonatal cholestasis
Know how to sequentially evaluate neonatal cholestasis: Excerpt from Avoiding Common Pediatric Errors
Author: Anjali Subbaswamy, MD
What to Do - Gather Appropriate Data,
Interpret the Data
Neonatal cholestasis is a pathologic state of reduced bile formation or flow,
where contents normally excreted into bile are retained and measurable in
blood. The incidence is approximately one in 2,500 live births. It is a typical
presenting feature of neonatal liver disease rather than a late manifestation.
Mechanisms are hepatocellular (viral hepatitis, Dubin-Johnson syndrome)
or obstructive (choledochal cyst, biliary atresia). There is an extensive differential diagnosis, including neonatal sclerosing cholangitis, cystic fibrosis, hypothyroidism, and tyrosinemia. When pathologic jaundice is noted, a
workup should begin promptly and include:
• Fractionated bilirubin levels
• Blood type and Rh determination in mother and infant
• Direct Coombs testing in the infant
• Hemoglobin and hematocrit values
• Serum albumin levels: albumin binds bilirubin in a ratio of 1:1
• Ultrasound examination of the liver and bile ducts
• A radionuclide liver scan for uptake of hepatoiminodiacetic acid (HIDA)
is indicated for suspected extrahepatic biliary atresia
• The only definitive diagnosis for biliary atresiais an intraoperative cholangiogram.
If the cause of the cholestasis is biliarya tresia, expeditious surgical repair
is necessary to avoid worsening cholestasis, hepatic fibrosis, and cirrhosis,
which lead to portal hypertension, hepatic failure and death. Most infants
presentat 4 to 6weeks of age,withahistoryofpersistentjaundiceandacholic
stools. Operating before 60 days of age and having type 1 biliary atresia
(obliteration of the common bile duct) are associated with favorable surgical
outcomes. The aim of surgical repair, via the Kasai portoenterostomy, is to
restore bile flow, alleviate jaundice, and abbreviate the cholangiodestructive
process within the liver. Orthotopic liver transplantation is an option typically reserved for a failed Kasai procedure. Preoperative preparation for
a Kasai procedure includes optimizing nutrition, administering 1 mg/day
vitamin K by mouth/intramuscularly/intravenously to minimize perioperative coagulopathy. Some advocate preoperative antibiotics to minimize gut
flora and postoperative trimethoprim-sulfa to minimize cholangitis. There
are no studies proving the efficacy of any of these recommendations.
Suggested Readings
Bates MD, Bucuvalas JC, Alonso MH, et al. Biliary atresia: pathogenesis and treatment. Semin
Liv Dis. 1998;18(3):281–293.
Middlesworth W, Altman RP. Biliary atresia. Curr Opin Pediatr. 1997;9(3):265–269.
Suchy FJ. Neonatal cholestasis. Pediatr Rev. 2004;25:388–396.
Book Source Details
- Book Title: Avoiding Common Pediatric Errors
- Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
- Year of Publication: 2008
- Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.
More About Neonatal myasthenia
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Avoiding Common Pediatric Errors
Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7489-6
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