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. 1981 May;56(5):373–376. doi: 10.1136/adc.56.5.373

Long-term prognosis for infants with intrahepatic cholestasis and patent extrahepatic biliary tract.

M Odièvre, M Hadchouel, P Landrieu, D Alagille, N Eliot
PMCID: PMC1627434  PMID: 6973322

Abstract

One hundred and three infants with prolonged cholestasis beginning before 3 months were classified as having alpha-1-antitrypsin deficiency (17 patients), scanty interlobular bile ducts (16 patients), or "neonatal hepatitis" (70 patients). Twenty-two gradually developed chronic liver disease and the remaining 81 recovered within a few months. Prognosis was found to be poor for infants with alpha-1-antitrypsin deficiency, scanty interlobular bile ducts, and familial "idiopathic" hepatitis. Patients who developed cirrhosis often presented with severe and persistent neonatal cholestasis, mimicking extrahepatic biliary atresia and leading to laparotomy. Thus, a high-risk group of infants-defined by aetiology, family history, and degree of cholestasis-can be recognised in the first months of life.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Aagenaes O., Sigstad H., Bjorn-Hansen R. Lymphoedema in hereditary recurrent cholestasis from birth. Arch Dis Child. 1970 Oct;45(243):690–695. doi: 10.1136/adc.45.243.690. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Alagille D., Odièvre M., Gautier M., Dommergues J. P. Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental, and sexual development, and cardiac murmur. J Pediatr. 1975 Jan;86(1):63–71. doi: 10.1016/s0022-3476(75)80706-2. [DOI] [PubMed] [Google Scholar]
  3. Danks D. M., Campbell P. E., Jack I., Rogers J., Smith A. L. Studies of the aetiology of neonatal hepatitis and biliary atresia. Arch Dis Child. 1977 May;52(5):360–367. doi: 10.1136/adc.52.5.360. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Danks D. M., Campbell P. E., Smith A. L., Rogers J. Prognosis of babies with neonatal hepatitis. Arch Dis Child. 1977 May;52(5):368–372. doi: 10.1136/adc.52.5.368. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Grand R. J., Watkins J. B., Katz A. J., Lawson E. E. Letter: Neonatal jaundice: recent developments. N Engl J Med. 1975 May 8;292(19):1028–1028. doi: 10.1056/NEJM197505082921912. [DOI] [PubMed] [Google Scholar]
  6. Hadchouel M., Gautier M. Histopathologic study of the liver in the early cholestatic phase of alpha-1-antitrypsin deficiency. J Pediatr. 1976 Aug;89(2):211–215. doi: 10.1016/s0022-3476(76)80450-7. [DOI] [PubMed] [Google Scholar]
  7. Heathcote J., Deodhar K. P., Scheuer P. J., Sherlock S. Intrahepatic cholestasis in childhood. N Engl J Med. 1976 Oct 7;295(15):801–805. doi: 10.1056/NEJM197610072951503. [DOI] [PubMed] [Google Scholar]
  8. Lawson E. E., Boggs J. D. Long-term follow-up of neonatal hepatitis: safety and value of surgical exploration. Pediatrics. 1974 May;53(5):650–655. [PubMed] [Google Scholar]
  9. Mowat A. P., Psacharopoulos H. T., Williams R. Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated infants. Arch Dis Child. 1976 Oct;51(10):763–770. doi: 10.1136/adc.51.10.763. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Thaler M. M. Cryptogenic liver disease in young infants. Prog Liver Dis. 1976;5:476–493. [PubMed] [Google Scholar]
  11. Thaler M. M., Gellis S. S. Studies in neonatal hepatitis and biliary atresia. I. Long-term prognosis of neonatal hepatitis. Am J Dis Child. 1968 Sep;116(3):257–261. doi: 10.1001/archpedi.1968.02100020259005. [DOI] [PubMed] [Google Scholar]
  12. Thaler M. M., Gellis S. S. Studies in neonatal hepatitis and biliary atresia. II. The effect of diagnostic laparotomy on long-term prognosis of neonatal hepatitis. Am J Dis Child. 1968 Sep;116(3):262–270. doi: 10.1001/archpedi.1968.02100020264006. [DOI] [PubMed] [Google Scholar]
  13. Thaler M. M. Letter: Questions regarding neonatal hepatitis. Pediatrics. 1975 Feb;55(2):296–297. [PubMed] [Google Scholar]

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