Skip to main content

Advertisement

Log in

Management of neonatal cholestasis: Consensus statement of the pediatric gastroenterology chapter of Indian academy of pediatrics

  • Recommendations
  • Published:
Indian Pediatrics Aims and scope Submit manuscript

Abstract

Justification

Neonatal cholestasis is an important cause of chronic liver disease in young children. Late referral and lack of precise etiological diagnosis are reasons for poor outcome in substantial number of cases in India. There is a need to create better awareness among the pediatricians, obstetricians and primary care physicians on early recognition, prompt evaluation and referral to regional centers.

Process

Eminent national faculty members were invited to participate in the process of forming a consensus statement. Selected members were requested to prepare guidelines on specific issues, which were reviewed by two other members. These guidelines were then incorporated into a draft statement, which was circulated to all members. A round table conference was organized; presentations, ensuing discussions, and opinions expressed by the participants were incorporated into the final draft.

Objectives

To review available published data on the subject from India and the West, to discuss current diagnostic and management practices in major centers in India, and to identify various problems in effective diagnosis and ways to improve the overall outcome. Current problems faced in different areas were discussed and possible remedial measures were identified. The ultimate aim would be to achieve results comparable to the West.

Recommendations

Early recognition, prompt evaluation and algorithm-based management will improve outcome in neonatal cholestasis. Inclusion of stool/urine color charts in well baby cards and sensitizing pediatricians about differentiating conjugated from the more common unconjugated hyperbilirubinemia are possible effective steps. Considering the need for specific expertise and the poor outcome in suboptimally managed cases, referral to regional centers is warranted.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Consensus report on neonatal cholestasis syndrome. Pediatric Gastroenterology Subspecialty Chapter of Indian Academy of Pediatrics. Indian Pediatr. 2000;37:845–851.

    Google Scholar 

  2. Yachha SK, Sharma A. Neonatal cholestasis in India. Indian Pediatr. 2005;42:491–492.

    PubMed  CAS  Google Scholar 

  3. Yachha SK. Cholestatic jaundice during infancy. Indian J Gastroenterol. 2005;24:47–48.

    PubMed  Google Scholar 

  4. Davis AR, Rosenthal P, Escobar GJ, Newman TB. Interpreting conjugated bilirubin levels in newborns. J Pediatr. 2011;158:562–565.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  5. Moyer V, Freese DK, Whitington PF, Olson AD, Brewer F, Colletti RB, et al. Guideline for the evaluation of cholestatic jaundice in infants: recommendations of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition. J Pediatr Gastroenterol Nutr. 2004;39:115–128.

    Article  PubMed  Google Scholar 

  6. Yachha SK, Khanduri A, Kumar M, Sikora SS, Saxena R, Gupta RK, et al. Neonatal cholestasis syndrome: an appraisal at a tertiary center. Indian Pediatr. 1996;33:729–734.

    PubMed  CAS  Google Scholar 

  7. Arora NK, Arora S, Ahuja A, Mathur P, Maheshwari M, Das MK, et al. Alpha 1 antitrypsin deficiency in children with chronic liver disease in North India. Indian Pediatr. 2010;47:1015–1023.

    Article  PubMed  Google Scholar 

  8. Yachha SK, Sharma BC, Khanduri A, Srivastava A. Current spectrum of hepatobiliary disorders in northern India. Indian Pediatr. 1997;34:885–890.

    PubMed  CAS  Google Scholar 

  9. Alagille D, Habib EC, Thomassin N. L’atresie des voies biliaires extrahepatiques permeables chez l’enfant. J Par Pediatr 1969:301–318.

    Google Scholar 

  10. Kamath BM, Spinner NB, Piccoli DA. Alagille Syndrome. In: Suchy F, Sokol RJ, Balistreri WF, editors. Liver Disease in Children. Third ed. New York: Cambridge University Press; 2007. p. 326–345.

    Chapter  Google Scholar 

  11. Mizuta K, Sanada Y, Wakiya T, Urahashi T, Umehara M, Egami S, et al. Living-donor liver transplantation in 126 patients with biliary atresia: single-center experience. Transplant Proc. 2010;42:4127–4131.

    Article  PubMed  CAS  Google Scholar 

  12. Sanghai SR, Shah I, Bhatnagar S, Murthy A. Incidence and prognostic factors associated with biliary atresia in Western India. Ann Hepatol. 2009;8:120–122.

    PubMed  Google Scholar 

  13. Ahmad M, Jan M, Ali W, Shabir ud d, Bashir C, Iqbal Q, et al. Neonatal cholestasis in Kashmiri children. JK Pract. 2000;7:125–126.

    PubMed  CAS  Google Scholar 

  14. Khalil S, Shah D, Faridi MM, Kumar A, Mishra K. Prevalence and outcome of hepatobiliary dysfunction in neonatal septicaemia. J Pediatr Gastroenterol Nutr. 2012;54:218–222.

    Article  PubMed  Google Scholar 

  15. Poddar U, Thapa BR, Das A, Bhattacharya A, Rao KL, Singh K. Neonatal cholestasis: differentiation of biliary atresia from neonatal hepatitis in a developing country. Acta Paediatr. 2009;98:1260–1264.

    Article  PubMed  Google Scholar 

  16. Rastogi A, Krishnani N, Yachha SK, Khanna V, Poddar U, Lal R. Histopathological features and accuracy for diagnosing biliary atresia by prelaparotomy liver biopsy in developing countries. J Gastroenterol Hepatol. 2009;24:97–102.

    Article  PubMed  Google Scholar 

  17. Shah I, Bhatnagar S. Clinical profile of chronic hepatobiliary disorders in children: experience from tertiary referral centre in Western India. Trop Gastroenterol. 2010;31:108–110.

    PubMed  Google Scholar 

  18. Yachha SK, Mohindra S. Neonatal cholestasis syndrome: Indian scene. Ind J Pediatr. 1999;66:S94–S96.

    CAS  Google Scholar 

  19. Chen SM, Chang MH, Du JC, Lin CC, Chen AC, Lee HC, et al. Screening for biliary atresia by infant stool color card in Taiwan. Pediatrics. 2006;117:1147–1154.

    Article  PubMed  Google Scholar 

  20. Burton BK. Inborn errors of metabolism in infancy: a guide to diagnosis. Pediatrics. 1998;102:E69.

    Article  PubMed  CAS  Google Scholar 

  21. Lien TH, Chang MH, Wu JF, Chen HL, Lee HC, Chen AC, et al. Effects of the infant stool color card screening program on 5-year outcome of biliary atresia in Taiwan. Hepatology. 2011;53:202–208.

    Article  PubMed  Google Scholar 

  22. Lally KP, Kanegaye J, Matsumura M, Rosenthal P, Sinatra F, Atkinson JB. Perioperative factors affecting the outcome following repair of biliary atresia. Pediatrics. 1989;83:723–726.

    PubMed  CAS  Google Scholar 

  23. Serinet MO, Wildhaber BE, Broue P, Lachaux A, Sarles J, Jacquemin E, et al. Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening. Pediatrics. 2009;123:1280–1286.

    Article  PubMed  Google Scholar 

  24. Lampela H, Ritvanen A, Kosola S, Koivusalo A, Rintala R, Jalanko H, et al. National centralization of biliary atresia care to an assigned multidisciplinary team provides highquality outcomes. Scand J Gastroenterol. 2012;47:99–107.

    Article  PubMed  Google Scholar 

  25. McKiernan PJ, Baker AJ, Kelly DA. The frequency and outcome of biliary atresia in the UK and Ireland. Lancet. 2000;355:25–29.

    Article  PubMed  CAS  Google Scholar 

  26. Whitington PF, Freese DK, Alonso EM, Schwarzenberg SJ, Sharp HL. Clinical and biochemical findings in progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr. 1994;18:134–141.

    Article  PubMed  CAS  Google Scholar 

  27. Hsiao CH, Chang MH, Chen HL, Lee HC, Wu TC, Lin CC, et al. Universal screening for biliary atresia using an infant stool color card in Taiwan. Hepatology. 2008;47:1233–1240.

    Article  PubMed  Google Scholar 

  28. Niedbala A, Lankford A, Boswell WC, Rittmeyer C. Spontaneous perforation of the bile duct. Am Surg. 2000;66:1061–1063.

    PubMed  CAS  Google Scholar 

  29. Lee WS, Looi LM. Usefulness of a scoring system in the interpretation of histology in neonatal cholestasis. World J Gastroenterol. 2009;15:5326–5333.

    Article  PubMed Central  PubMed  Google Scholar 

  30. Singh R, Thapa BR, Kaur G, Prasad R. Biochemical and molecular characterization of GALT gene from Indian galactosemia patients: identification of 10 novel mutations and their structural and functional implications. Clin Chim Acta. 2012;414:191–196.

    Article  PubMed  CAS  Google Scholar 

  31. Singh R, Kaur G, Thapa BR, Prasad R, Kulkarni K. A case of classical galactosemia: identification and characterization of 3 distinct mutations in galactose-1-phosphate uridyl transferase (GALT) gene in a single family. Indian J Pediatr. 2011;78:874–876.

    Article  PubMed  Google Scholar 

  32. Bijarnia S, Puri RD, Ruel J, Gray GF, Jenkinson L, Verma IC. Tyrosinemia type I—diagnostic issues and prenatal diagnosis. Indian J Pediatr. 2006;73:163–165.

    Article  PubMed  Google Scholar 

  33. Suchy FJ. Neonatal cholestasis. Pediatr Rev. 2004;25:388–396.

    PubMed  Google Scholar 

  34. Feranchak AP, Sokol RJ. Medical and nutritional mManagement of cholestasis in infants and children. In: Suchy FJ, Sokal RJ, Balistreri WF, editors. Liver Diseases in Children. 3rd ed. New York: Cambridge University Press; 2007. p. 190–231.

    Chapter  Google Scholar 

  35. Venigalla S, Gourley GR. Neonatal cholestasis. Semin Perinatol. 2004;28:348–355.

    Article  PubMed  Google Scholar 

  36. Sokol RJ, Shepherd RW, Superina R, Bezerra JA, Robuck P, Hoofnagle JH. Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop. Hepatology. 2007;46:566–581.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  37. Bassett MD, Murray KF. Biliary atresia: recent progress. J Clin Gastroenterol. 2008;42:720–729.

    Article  PubMed  Google Scholar 

  38. Sharma D, Shah UH, Sibal A, Chowdhary SK. Cholecystoappendicostomy for progressive familial intrahepatic cholestasis. Indian Pediatr. 2010;47:626–628.

    Article  PubMed  Google Scholar 

  39. Bhatia V, Bavdekar A, Yachha SK. Management of acute liver failure in infants and children: Consensus statement of the Pediatric Gastroenterology Chapter, Indian Academy of Pediatrics. Indian Pediatr. 2013;50:477–482.

    Article  PubMed  Google Scholar 

  40. Poonacha P, Sibal A, Soin AS, Rajashekar MR, Rajakumari DV. India’s first successful pediatric liver transplant. Indian Pediatr. 2001;38:287–291.

    PubMed  CAS  Google Scholar 

  41. Sibal A. Pediatric Liver transplantation in India: Past, Present and future. 21th Annual Conference of Indian National Association for Study of the Liver [INASL] 2013; Hyderabad, India.

  42. Utterson EC, Shepherd RW, Sokol RJ, Bucuvalas J, Magee JC, McDiarmid SV, et al. Biliary Atresia: Clinical Profiles, Risk Factors, and Outcomes of 755 Patients Listed for Liver Transplantation. J Pediatr. 2005;147:180–185.

    Article  PubMed  Google Scholar 

  43. Wang SH, Chen CL, Concejero A, Wang CC, Lin CC, Liu YW, et al. Living donor liver transplantation for biliary atresia. Chang Gung Med J. 2007;30:103–108.

    PubMed  Google Scholar 

  44. Kaur S, Wadhwa N, Sibal A, Jerath N, Sasturkar S. Outcome of live donor liver transplantation in Indian children with bodyweight 7.5 kg. Indian Pediatr. 2011;48:51–54.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Vidyut Bhatia.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Bhatia, V., Bavdekar, A., Matthai, J. et al. Management of neonatal cholestasis: Consensus statement of the pediatric gastroenterology chapter of Indian academy of pediatrics. Indian Pediatr 51, 203–210 (2014). https://doi.org/10.1007/s13312-014-0375-2

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13312-014-0375-2

Keywords

Navigation