Approach to Neonatal Cholestasis

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The incidence of NC is ~1 in 2500 live births.

Investigations:

First Tier:

  • Serum Total Bilirubin, Direct Bilirubin, Indirect Bilirubin
  • Coagulation Profile – PT, APTT, INR
  • Hemolysis Params

Second Tier:

  • USG W/A (Fasting)
  • TORCH Profile
  • Live Function Test (LFT)
  • Thyroxine, TSH

Differntial Diagnosis:

  • biliary atresia (BA) (35%)
  • progressive familial intrahepatic cholestasis (PFIC) (10%)
  • preterm birth (10%)
  • metabolic and endocrinological disorders (9–17%)
    • Alpha1 AT deficieny
    • Cystic Fibrosis
  • Alagille syndrome (AS) (2–6%)
  • infectious diseases (1–9%) -> TORCH
  • mitochondriopathy (2%)
  • biliary sludge (2%)
  • idiopathic cases (13–30%)

Management:

In patients with advanced disease, insufficient hepatic synthetic function and hypovitaminosis, a vitamin K-dependent bleeding disorder may occur.

  1. vitamin K (1 mg/day),
  2. vitamin A (1500 U/kg/day),
  3. vitamin D (cholecalciferol; 500 U/kg/day), and
  4. vitamin E (50 U/kg/day)

should be initiated immediately.

Associated Conditions with Bile Atresia:

  1. polysplenia (100%)
  2. situs inversus (50%)
  3. cardiac anomalies (50%)
  4. vascular malformations, e.g., preduodenal portal vein (60%)

References:

  1. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4470262/

Last updated on November 6th, 2022.