title: Neonatal cholestasis syndrome: an appraisal at a tertiary center.
abstract: OBJECTIVE
  To know the magnitude, etiology and clinical profile, the efficacy of various investigations and the outcome in patients with neonatal cholestasis syndrome (NCS).
  
  
  METHODS
  Prospective evaluation of 60 consecutive infants with NCS (mean age 3.9 +/- 1.9 months; 49 males) over a period of 3.5 years.
  
  
  METHODS
  Tertiary level referral gastroenterology center in North India.
  
  
  METHODS
  Liver function tests, urine examination, serum antibodies against Cytomegalovirus (CMV), Rubella and Toxoplasma; abdominal ultrasonography, hepatobiliary scintigraphy and liver biopsy were done. In appropriate setting, laparotomy and surgical corrections were done for biliary tract disorders.
  
  
  RESULTS
  NCS constituted 19% of pediatric liver diseases. Considerable delay in presentation was observed [mean delay, extrahepatic biliary atresia (EHBA) = 4 +/- 2.0 months, neonatal hepatitis (NH) = 2.2 +/- 1.3 months]. Thirty three (55%) infants had EHBA, 14 (23%) NH (4 CMV, 2 galactosemia, 1 urinary tract infection and 7 idiopathic), 2 (3%) paucity of intralobular bile ducts and 11 (18%) were of indeterminate etiology. Liver biopsy was the most accurate (96.4%) investigation in discriminating between EHBA and NH. Of the 18 operated infants with EHBA (portoenterostomy-15 and hepatico-jejunostomy-3), 10 were alive (mean follow up = 22.8 +/- 8.6 months) of which 4 were completely asymptomatic.
  
  
  CONCLUSIONS
  (i) NCS is an important cause of liver disease in Indian children. (ii) It requires prompt referral, quick investigative approach and targeted management. (iii) Liver biopsy is highly accurate in differentiating EHBA and NH. (iv) infants with EHBA and compensated status of liver should undergo corrective surgery irrespective of age at presentation.
authors.affiliation: Department of Gastroenterology (Pediatric GE), Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow.
authors.forename: S K
authors.initials: SK
authors.lastname: Yachha
authors.forename: A
authors.initials: A
authors.lastname: Khanduri
authors.forename: M
authors.initials: M
authors.lastname: Kumar
authors.forename: S S
authors.initials: SS
authors.lastname: Sikora
authors.forename: R
authors.initials: R
authors.lastname: Saxena
authors.forename: R K
authors.initials: RK
authors.lastname: Gupta
authors.forename: J
authors.initials: J
authors.lastname: Kishore
mesh_terms: D001656:Biliary Atresia / Q000175:diagnosis; D002779:Cholestasis / Q000150:complications* / Q000175:diagnosis; D003937:Diagnosis, Differential; D005260:Female; D006505:Hepatitis / Q000175:diagnosis; D006801:Humans; D007223:Infant; D008107:Liver Diseases / Q000209:etiology*; D008297:Male; D011446:Prospective Studies; D013577:Syndrome