Title: Neonatal Liver Biopsy- Experience from a Tertiary Care Centre

Author: Sujay Chaudhuri

 DOI:  https://dx.doi.org/10.18535/jmscr/v6i5.129

Abstract

Objectives: To clinch the diagnosis of complex neonatal liver diseases (where all other diagnostic modalities fail) this study was conducted in a Tertiary Care Centre of Northern India.

Materials and Methods: The neonates who attended pediatric Gastroenterology Clinic of PGIMER – Chandigarh from July 2000 to June 2003 having history of liver disease (i.e. Jaundice , clay colored stool, high colored urine, hepatosplenomegaloy, syndromic features, sibling history of liver disease etc) were subjected to liver biopsy when history, clinical examination, USG abdomen, LFT, HIDA Scan, per operative cholangiogram, enzyme study  of metabolic disease) fail to establish diagnosis. Liver biopsy was done as per standard protocol considering all safety measures.

Result: Out of total 50 neonates (who had liver  biopsy), 15 (30%) had biliary atresia (bile duct proliferation, bile plugs , portal fibrosis, normal lobular architecture), 30 (60%) had ideopathic neonatal hepatitis (multi nucleated giant cells, pseudoglandular transformation, dilated canaliculi, inflammatory  infiltrates in heaptocellular lobules with necrosis, mild portal tract fibrosis, bile duct proliferation), One (2%) had galactosemia (diffuse hepatocellural damage, marked steatosis, cholestasis, pseudoacinar transformation), one (2%) had alfa one antitrypsin deficiency (PAS positive Diastase resistant alfa one  antitrypsin inclusion in peripheral hepatocytes, bile stasis, minimal inflammation), one (2%) had non syndromic paucity of intrahepatic bile ducts  having low ratio of <.9 of intra lobular ducts to portal tracts).

Conclusion: Neonatal liver biopsy is an important investigation for making accurate diagnosis of complex liver disease

Keywords: neonatal liver biopsy, complex liver disease.

References

  1. The liver biopsy in modern clinical practice – a pediatric point of view By N.ovchiarsky et al Adv Anat Pathol July 2012 ,19 (4): 250-262
  2. Liver biopsy in neonatal cholestasis – a review on statistical ground Zerbina MC et al Mod pathol 1997 Aug: 10(8) : 793 to 799
  3. Diagnostic liver biopsy, Drugs and diseases – clinical procedure Medscape, Author – Kenneth Ingram Updated Feb 22, 2017
  4. Differential diagnosis of extra hepatic biliary atresia from neonatal hepatitis -a prospective study, Lai MW et al , J pediatr gastoenterol nutr , 1994 feb 18(2):121-7
  5. liver biopsy in children – Position paper of the ESPGHAN hepatology committee, Antal Dezsofi et al, Medical position paper Nov 5, 2014
  1. Whatever happened to neonatal hepatitis ? Balistreri WF et al Clin liver dis 2006; 10(1):27-53
  2. Alpha 1- anti trypsin globules in liver biopsy, Clausen PP et al, Acta pathol microbial scand A , 1980 Jul;88(4):225-30
  3. Detection of PiZ Phenotype individuals by alpha 1 anti trypsin ( AAT) immuno histo chemistry in paraffin embedded liver tissue specimen, Callea F et al, J Hepatol 1986;2(3):389-401
  4. The diagnostic value of alpha 1 anti trypsin globules in liver cells as a morphological marker of alpha – 1 – anti trypsin deficiency, Clausen PP et al, Liver 1984 Dec 4(6):353-9.
  5. Liver changes suggesting alpha -1 anti trypsin deficiency (a neglected disease), (Article in Hungarian)Bartok I et al, Orv Hetil 1989 Sep 3;130(36):1911-6,1919
  1. Paucity of intra hepatic bile ducts in neonates, The first case series for Iran Mozhgan Z et al, Iran   pediatrics 2013 Feb 23(1): 65 to 70
  2. Non syndromic paucity of intra hepatic bile ducts in infancy and ideopathic ductopenia in adult hood: the same syndrome? Bruguera M et al, Hepotology 1992 May 15(5):830-4
  3. Reversibility of extensive liver damage in galactosemia, Applebaum MN et al Gastroenterology 1975 Aug 69(2):496-502
  4. Galactosemia – a not to be missed inborn error of metabolism, Madhulika Kabra and Neerja gupta, Division of genetics, department of pediatrics ,AIIMSIndian  pediatrics – 2016 Jan-19-20
  1. Duarte variant galactosemia, Fridovich- Keil JL et al, Gene review, Biochemical tests of Historic interest, Dec 2014
  2. Editorial – clouds over galactosemia Lancet 1984, 1379 – 1380
  3. Emerging concepts and human trials in Alpha -1- Antitrypsin Deficiency Liver Disease, Jeffrey H Teckman et al, Thieme E- Journals- seminars in Liver Disease/ Abstract. Publication date 31 May 2017 (online) (www.thieme-connect.com/produ-cts/ejournals/abstract/10.1055/s-0037-1602586)
  4. Liver and intrahepatic bile ducts- nontumor metabolic diseases, Galact-osemia, Author Komal Arora, Pathology outlines- galactossemia, April 2012
  5. Sweet and sour: an update on classic galactossemia, Ana I. Coelho et al J Inherit Metab Dis 2017, 40:325-342
  6. The value of preoperative liver biopsy in the diagnosis of extrahepatic biliary atresia: A systematic  review and metaanalysis, Lee JY et al, J Pediatr Surg. 2018 May 51(5):753-61
  7. Ideopathic neonatal hepatitis or extra hepatic biliary atresia?, By AEM Kheir Sudan J pediatr 2016 vol 16, Issue No. 1
  8. Neonatal hepatitis: liver biopsy findings and clinical features, Shibuya T et al, Tohoku J Exp Med. 1964 Jun 25;83:29-46 Pubmed- NCBI
  9. Extra hepatic biliary atresia – correlation of histopathology and liver function trials with surgical outcome, Lucky Gupta, Siddharth D Gupta, Journal of Indian association of pediatric surgeons 2012 Oct – Dec 17(4):147-162
  10. Extra hepatic biliary artesia – current concepts and future direction de carvalho E et al, J pediatr (Rio J) 2007 Mar- Apr,83(2):106-20
  11. Pediatric biliary atresia, Drugs and diseases, September 24, 2017, Author Steven N Schwarz
  12. Ideopathic neonatal giants cells hepatitis presenting with acute hepatic failure in post natal day one, By correa KK et al, J perinatol 2002, April- may 22 (3)–249-251
  13. Histopathology of vanishing bile ducts disease, Desmet VJ et al Adv clin patho 1998, April 2(2) 87 – 99
  14. Pathology and pathogenesis of intra hepatic bile ducts loss, Nakanuma Y et al J hepato biliary pancreatic surgery 2001 – 8 (4) :303-315
  1. Clinical and molecular genetics of Alagille syndrome, Krantz Ian D et al Curr. Opin. Pediatr 1999, December 11(6),:558-564
  2. Congenital colestatic syndrome – what happens when children grow up ? Ling SC, Can J gastroenternal , Nov 2007 Vol 21 No (11) 743-751
  3. Clinical practice – neonatal cholestasis De Bruyne R et al Eur. J. pediatr 2011 March 170 (3):279-284
  4. Clinical problem with developmental anomalies of biliary tract Suchy FJ  Semin gastroenterol dis 2003 Oct 14(4):156-6

Corresponding Author

Sujay Chaudhuri

Division of Pediatric Gastroenterology, Department of Gastroenterology, PGIMER Chandigarh

Address: Akshaytara Apartment, Flat No. 1/7, 2nd Mile Sevoke Road, Siliguri- 734001, Near Don Bosco

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