Skip to main content

Neonatal and Structural Disease

  • Chapter
  • 255 Accesses

Part of the book series: Current Histopathology ((CUHI,volume 23))

Abstract

Unconjugated bilirubinaemia is extremely common in the neonatal period and is usually physiological. However, unconjugated bilirubinaemia of any degree occurring in the first 24 hours of life is pathological. Haemolysis is probably the most common cause, and the others too are mostly non-hepatological, including conditions such as infection, hypoxia and hypoglycaemia. Galactosaemia and fructosaemia (Chapter 9) are rare treatable conditions which should always be sought (by the detection, initially, of non-glucose reducing substances in the urine).

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

Buying options

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   39.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Ishak, K. G. and Sharp, H. L. (1987). Developmental abnormalities and liver disease in childhood. In MacSween, R. N. M., Anthony, P. P. and Scheuer, P. J. (eds). Pathology of the Liver. 3rd edn. pp. 66–98. Edinburgh: Churchill Livingstone

    Google Scholar 

  2. Lau, J. Y. N., Koukoulis, G., Mieli-Vergani, G., Portmann, B. C. and Williams, R. (1992). Syncytial giant-cell hepatitis-a specific disease entity? J. Hepatol., 15,216–219

    Article  PubMed  CAS  Google Scholar 

  3. Phillips, M. J., Blendis, L. M., Poucell, S., Patterson, J., Petric, M., Roberts, E., Levy, G. A., Superina, R. A., Greig, P. D., Cameron, R., Langer, B. and Purcell, R. H. (1991). Syncytial giant-cell hepatitis. Sporadic hepatitis with distinctive pathological features, a severe clinical course, and paramyxoviral features. N. Engl. J. Med., 324, 455–460

    Article  PubMed  CAS  Google Scholar 

  4. Gautier, M., Jehan, P. and Odievre, M. (1976). Forty eight cases of extrahepatic biliary atresia. Correlation with postoperative bile flow restoration. J. Pediatr., 89, 704–709

    Article  PubMed  CAS  Google Scholar 

  5. Chandra, R. S. and Altman, R. P. (1978). Ductal remnants in extrahepatic biliary atresia. A histopathological study with clinical correlation. J. Pediatr., 93, 196–200

    Article  PubMed  CAS  Google Scholar 

  6. Mujano, T., Suruga, A., Tsuchiya, A. and Suda, K. (1977). A histopathological study of the remnants of extrahepatic bile duct in so-called ‘non-correctable’ biliary atresia. J Pediatr. Surg., 12, 19–25

    Article  Google Scholar 

  7. Psacharopoulos, H. T., Howard, E. R., Portmann, B. and Mowat, A. P. (1980). Extrahepatic biliary atresia; pre operative assessment and surgical results in 47 consecutive cases. Arch. Dis. Child, 55. 851–856

    Article  CAS  Google Scholar 

  8. Howard, E. R. and Davenport, M. (1992). Paediatric liver disease: surgical aspects. In Millward-Sadler, G. H., Wright, R. and Arthur, M. J. P. (eds). Wright’s Liver and Biliary Disease. 3rd edn. pp. 1208–1232. London: W B Saunders

    Google Scholar 

  9. Howard, E. R., Driver, M., McClement, J. and Mowat, A. P. (1982). Results of surgery in 88 consecutive cases of extrahepatic biliary atresia. J. R. Soc. Med.. 75, 408–413

    PubMed  CAS  Google Scholar 

  10. Wight, D. G. D. (1993). The pathology of liver transplantation. In Wight, D. G. D. (ed.). Liver, Biliary Tract and Exocrine Pancreas. Vol. 11. Systemic Pathology. Symmers, W. S. C. (series ed.) Vol 11. Edinburgh: Churchill Livingstone

    Google Scholar 

  11. Mieli-Vergani, G., Howard, E. R., Portmann, B. and Mowat, A. P. (1989). Later referral for biliary atresia-missed opportunities for effective surgery. Lancet, 1,421–423

    Article  PubMed  CAS  Google Scholar 

  12. Hays, D. M. Woolley, M. M., Snyder, W. H., Reed, G. B., Gwinn, J. L. and Landing, B. H. (1967). Diagnosis of biliary atresia. Relative accuracy of percutaneous liver biopsy, open biopsy and operative cholangiography. J. Pediatr., 71,598–607

    Article  PubMed  CAS  Google Scholar 

  13. Brough, A. J. and Bernstein, J. (1974). Conjugated hyper bilirubinaemia in early infancy. A reassessment of liver biopsy. Hum. Pathol., 5,507–516

    CAS  Google Scholar 

  14. Mieli-Vergani, G. and Mowat, A. P. (1992). Paediatric liver disease: medical aspects. In Millward-Sadler, G. H., Wright, R. and Arthur, M. J. P. (eds). Wright’s Liver and Biliary Disease. 3rd Edn., pp. 1138–1154. London: W. B. Saunders

    Google Scholar 

  15. Alagille, D. (1979). Cholestasis in the first 3 months of life. In Popper, H. and Schaffner, F. (eds). Progress in Liver Disease VI. pp. 471–485. New York: Grune and Stratton

    Google Scholar 

  16. Desmet, V. J. (1991). Embryology of the liver and intrahepatic biliary tract, and an overview of malformations of the bile duct. In McIntyre, N., Benhamou, J.-P., Bircher, J., Rizetto, M. and Rodes, J. (eds). Oxford Textbook of Clinical Hepatology, pp. 497–519. Oxford: Oxford University Press

    Google Scholar 

  17. Desmet, V. J. (1987). Cirrhosis: aetiology and pathogenesis: cholestasis. In Boyer, J. L. and Bianchi, L. (eds). Liver Cirrhosis, pp. 101–118. Lancaster: MTP Press

    Google Scholar 

  18. Desmet, V. J. (1990). Cholestatic syndromes of infancy and childhood. In Zakim, D. and Boyer, T. D. (eds). Hepatology. A Textbook of Liver Disease. pp. 1355–1395. Philadelphia: W B Saunders

    Google Scholar 

  19. Landing, B. H. (1974). Consideration of the pathogenesis of neonatal hepatitis, biliary atresia and choledochal cyst. The concept of infantile obstructive cholangiopathy. Prog. Pediatr. Surg., 6, 113–119

    PubMed  CAS  Google Scholar 

  20. Landing, B. H., Mahnovski, V. and Dahms, B. (1979). Considerations of certain aspects of the pathogenesis of neonatal hepatitis and biliary atresia. In Javitt, N. B. (eds). Neonatal Hepatitis and Biliary Atresia. pp.79–1296. 315–321 US Dept Health Education Welfare, NIH

    Google Scholar 

  21. Witzleben, C. L., Buck, B. E., Schnanfer, L. and Brzosko, W. J. (1978). Studies in the pathogenesis of biliary atresia. Lab. Invest., 38, 525–532

    PubMed  CAS  Google Scholar 

  22. Haas, J. E. (1978). Bile duct and liver pathology in biliary atresia. World J. Surg., 2, 561–569

    CAS  Google Scholar 

  23. Witzleben, C. L. (1976). Etiologies of infantile obstructive cholangiopathy. J. Pediatr., 88, 909–910

    Article  PubMed  CAS  Google Scholar 

  24. Witzelben, C. L. (1990). Cystic diseases of the liver. In Zakim, D. and Boyer, T. D. (eds). Hepatology. A Textbook of Liver Disease. 3rd edn. pp. 1395–1411. Philadelphia: W B Saunders

    Google Scholar 

  25. Henson, S. W., Gray, H. K. and Dockerty, M. B. (1956). Benign tumours of the liver. III. Solitary cysts. Surg. Gynecol. Obstet., 103, 607–612

    Google Scholar 

  26. Milutinovic, J., Fialkow, P. J., Rudd, T. G., Agoda, L. Y., Phillips, L. A. and Bryant, J. I. (1980). Liver cysts in patients with autosomal dominant polycystic kidney disease. Am. J. Med., 68, 741–744

    Article  PubMed  CAS  Google Scholar 

  27. Grunefeld, J.-P., Albouze, G., Junger, P., Landais, P., Dana, A., Droz, D., Moynot, A., Lafforgue, B., Boursztyn, E., and Franco, D., (1985). Liver changes and complications in adult polycystic renal disease. Adv. Nephrol., 14, 1–20

    Google Scholar 

  28. Desmet, V. J. (1992). Congenital diseases of intrahepatic bile ducts: variations on the theme ‘ductal plate malformation’. Hepatology, 16, 1069–1083

    Article  PubMed  CAS  Google Scholar 

  29. MacSween, R. N. M. and Burt, A. D. (1989). Pathology of the intrahepatic bile ducts. In Anthony, P. P. and MacSween, R. N. M. (eds). Recent Advances in Histopathology. 14. pp. 161–184. Edinburgh: Churchill Livingstone

    Google Scholar 

  30. Landing, B. H., Wells, T. R. and Claireaux, A. E. (1980). Morpho-metric analysis of liver lesions in cystic diseases of childhood. Hum. Pathol., 11, 549–560

    CAS  Google Scholar 

  31. Adams, C. M., Danks, D. M. and Campbell, P. E. (1974). Comments upon the classification of infantile polycystic diseases of the liver and kidney, based upon three dimensional reconstruction of the liver. J. Med. Genet., 11, 234–243

    Article  PubMed  CAS  Google Scholar 

  32. Summerschild, H. C., Langmark, F. and Maurseth, K. (1973). Congenital hepatic fibrosis; report of two new cases and review of the literature. Surgery. 73, 53–58

    Google Scholar 

  33. Desmet, V. J. (1992). What is congenital hepatic fibrosis? Histopathology, 20, 465–477

    Article  PubMed  CAS  Google Scholar 

  34. Desmet, V. J. (1985). Intrahepatic bile ducts under the lens. J. Hepatol., 1, 545–559

    Article  PubMed  CAS  Google Scholar 

  35. Nonomura, A., Ohta, G., Yoshida, K., Kurachi, M., Matsubara, F. and Takazakura, E. (1978). Congenital hepatic fibrosis. A case report with study of three-dimensional reconstruction of serial sections of the liver. Acta Pathol. Jpn., 28, 949–956

    CAS  Google Scholar 

  36. Caroli, J. (1973). Disease of the intrahepatic biliary tree. Clin. Gastroenterol.. 2, 147–161

    CAS  Google Scholar 

  37. Mercadier, M., Chigot, J. P., Clot, J. P., Langlois, P. and Lansieux, P. (1984). Caroli’s disease. World J. Surg., 8. 22–29

    CAS  Google Scholar 

  38. Hadad, A. R., Westbrook, K. C., Campbell, F. T. and Morris, W. D. (1976). Congenital dilatation of the bile ducts. Am. J. Surg., 132, 799–804

    Article  PubMed  CAS  Google Scholar 

  39. Khobayashi, A. and Ohbe, Y. (1977). Choledochal cyst in infancy and childhood: analysis of 16 cases. Arch. Dis. Child., 52, 121–128

    Article  Google Scholar 

  40. Todani, T., Watanabe, Y., Narusue, M., Tabuchi, K. and Okajima, K. (1977). Congenital bile duct cysts. Classification, operative procedures, and review of thirty-seven cases including cancer arising from choledochal cyst. Am. J. Surg., 134. 263–269

    Article  PubMed  CAS  Google Scholar 

  41. Perrault, J. (1981). Paucity of interlobular bile ducts: Getting to know it better. Dig. Dis. Sci., 26, 481–484

    CAS  Google Scholar 

  42. Berman, M. D., Ishak, K. G., Schaefer, E. J., Barnes, S. and Jones, E. A. (1981). Syndromatic hepatic ductular hypoplasia (arteriohepatic dysplasia), A clinical and hepatic histologic study of three patients. Dig. Dis. Sci., 26, 485–497

    CAS  Google Scholar 

  43. Hadchouel, M., Hugon, R. N. and Gautier, M. (1978). Reduced ratio of portal tracts to paucity of intrahepatic bile ducts. Arch. Pathol. Lab. Med., 102, 402

    PubMed  CAS  Google Scholar 

  44. Alagille, D., Estrada, A., Hadchouel, M., Gautier, M., Odievre, M. and Dommergues, J. P. (1987). Syndromatic paucity of interlobular bile ducts (Alagille syndrome or arteriohepatic dysplasia). J. Pediatr.. 110,195–200

    Article  PubMed  CAS  Google Scholar 

  45. Emery, J. L. (1974). Pathology with reference to the bile retention syndrome. Postgrad. Med. J., 50, 344–347

    Article  PubMed  CAS  Google Scholar 

  46. Klein, S. and Nealon, W. H. (1988). Hepatobiliary abnormalities associated with total parenteral nutrition. Semin. Liver Dis., 8, 237–246

    Article  CAS  Google Scholar 

  47. Chang, M. H., Hsu, H. C., Lee, C. Y. et al. (1987). Neonatal hepatitis: a follow-up study. J. Pediatr. Gastroenterol. Nutr., 6, 203–207

    Article  PubMed  CAS  Google Scholar 

  48. Sveger, T. (1976). Liver disease in a, -antitrypsindeficiency detected by screening 200,000 infants. N. Engl. J. Med., 294, 1316–1321

    Article  PubMed  CAS  Google Scholar 

  49. Talbot, I. C. and Mowat, A. P. (1975). Liver disease in infancy: histological features and relationship to a1-antitrypsin deficiency. J. Clin. Pathol., 28, 559–563

    Article  PubMed  CAS  Google Scholar 

  50. Callea, F., Brisigotti, M., Faa, G., Lucini, L. and Eriksson, S. (1991). Identification of PiZ gene products in liver tissue by a monoclonal antibody specific for the Z mutant of a,-antitrypsin. J. Hepatol., 12, 372–376

    Article  PubMed  CAS  Google Scholar 

  51. Aägenaes, O., Henriksen, T. and Sorland, S. (1976). Hereditary neonatal cholestasis with vascular malformation. In Berenberg, S. R. (eds). Liver Diseases in Infancy and Childhood. pp. 198–206. Baltimore: Williams and Wilkins

    Google Scholar 

  52. Ghishan, F. K. and Greene, H. L. (1988). Liver disease in children with PiZZ alpha, antitrypsin deficency. Hepatology, 8, 307–310

    Article  PubMed  CAS  Google Scholar 

  53. Hadchouel, M. and Gautier, M. (1976). Histopathological study of the liver in the early cholestatic phase of a,-antitrypsin deficiency. J. Pediatr., 89, 211–215

    Article  PubMed  CAS  Google Scholar 

  54. Scheuer, P. J. (1993). Chronic hepatitis. In Wight, D. G. D. (eds). Liver, Biliary Tract and Exocrine Pancreas. Vol. 11. Systemic Pathology. Symmers, W. S. C. Series ed. 3rd edn. Edinburgh: Churchill Livingstone

    Google Scholar 

  55. Perlmutter, D. H. (1991). The cellular basis for liver injury in a,antitrypsin deficency. Hepatology, 13, 172–185

    Article  PubMed  CAS  Google Scholar 

  56. Eriksson, S., Carlson, J. and Velez, R. (1986). Risk of cirrhosis and primary liver cancer in alpha,-antitrypsin deficiency. N. Engl. J. Med., 314, 736–739

    Article  PubMed  CAS  Google Scholar 

  57. Thung, S. N., Gerber, M. A., Sarno, E. and Popper, H. (1979). Distribution of five antigens in hepatocellular carcinoma. Lab. Invest., 41, 101–105

    CAS  Google Scholar 

  58. Palmer, P. E. and Wolfe, H. J. (1976). a,-Anti-trypsin deficiency in primary hepatic carcinomas. Arch. Pathol. Lab. Med., 100. 232–236.

    PubMed  CAS  Google Scholar 

  59. Sveger, T. (1988). The natural history of liver disease in a,antitrypsin deficiency. Acta.Paediatr. Scand., 77, 847–851

    CAS  Google Scholar 

  60. Morin, T., Martin, J.-P., Geldmann, G. D., Benhamou, J.-P. and Ropartz, C. (1975). Heterozygous a, anti-trypsin deficiency and cirrhosis in adults. a fortuitous association. Lancet, 1, 250–251

    Article  PubMed  CAS  Google Scholar 

  61. Clausen, P. P. (1980). alAnti-trypsin globules in liver biopsies. Acta Pathol. Microbiol. Scand A, 88, 225–230

    CAS  Google Scholar 

  62. Bradfield, J. W. B. and Blenkinsopp, W. K. (1977). a,-Anti-trypsin globules in the liver and Pi M phenotype. J. Clin. Pathol.. 30. 464–466

    Article  PubMed  CAS  Google Scholar 

  63. Pariente, E.-A., Degott, C., Mastin, J., Feldmann, G., Potet, F. and Benhamou, J.-P. (1981). Hepatocytic PAS-positive diastase resistant inclusions in the absence of a,-anti-trypsin deficiency high prevalence in alcoholic cirrhosis. Am. J. Clin. Pathol, 76, 299–302

    PubMed  CAS  Google Scholar 

  64. Qizilbash, A. and Youn-Pong, O. (1983). Alpha, -antitrypsin disease: differential diagnosis of PAS positive, diastase resistant globules in liver cells. Am. J. Clin. Path., 79, 697–702

    PubMed  CAS  Google Scholar 

  65. Callea, F., Ray, M. B. and Desmet, V. J. (1981). a,-Anti-trypsin and copper in the liver. Histopathology, 5. 415–424

    Article  PubMed  CAS  Google Scholar 

  66. Carrell, R. W. (1987). a,-Antitrypsin deficiency. In Weatherall, D. J., Ledingham, J. G. G. and Warrell, D. A. (eds). Oxford Textbook of Medicine. 2nd edn. pp. 9.44–9.46. Oxford: Oxford University Press

    Google Scholar 

  67. Lomas, D. A., Evans, D. L., Finch, J. T., and Carrell, R. W. (1992). The mechanism of Z a1-antitrypsin accumulation in the liver. Nature, 357, 605–607

    Article  PubMed  CAS  Google Scholar 

  68. Curiel, D. T., Holmes, M. D., Okayama, H., Brantly, M. L., Vogelmeier, C., Travis, W. D., Stier, L. E., Perks, W. H. and Crystal, R. G. (1989). Molecular basis of the liver and lung disease associated with the alpha,-antitrypsin deficiency allele Mmalton. J. Biol. Chem., 264, 13938–13945

    PubMed  CAS  Google Scholar 

  69. Portmann, B. C. (1993). Paediatric liver disease. In Wight, D. G. D. (ed). Liver. Biliary Tract and Exocrine Pancreas. Vol. 11. Systemic Pathology. Symmers, W. S. C., series ed. 3rd edn. Edinburgh: Churchill Livingstone

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

Copyright information

© 1993 D. G. D. Wight

About this chapter

Cite this chapter

Wight, D.G.D. (1993). Neonatal and Structural Disease. In: Atlas of Liver Pathology. Current Histopathology, vol 23. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-2212-2_8

Download citation

  • DOI: https://doi.org/10.1007/978-94-011-2212-2_8

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-4980-1

  • Online ISBN: 978-94-011-2212-2

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics