Significance of α1-proteinase inhibitor deficiency in etiopathogenesis of liver diseases in children

Significance of α1-proteinase inhibitor deficiency in etiopathogenesis of liver diseases in children

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ABSTRACT α1-proteinase inhibitor (α1PI) phenotypes were examined in 27 children with chronic persistent hepatitis (1st group) and in 70 newborns and infants with protracted icterus and neonatal hepatitis (2nd group). 1442 adults and children in population study served as controls (3rd group). Prevalence of phenotype PI MZ was equal to 7.41% in the 1st group, 4,29% in the 2nd group, and 3,47% in the 3rd group. Prevalence of phenotype PI MS in the groups studied was equal to 1,87%, 2,86%, and 3,33%, respectively, α1PI phenotype PI ZZ responsible for α1PI deficiency was not encountered in the 1st group. Its prevalence in the 2nd group reached 5,71% and in the 3rd group equaled to 0,07%. Thus, the cause of hepatitis in newborns and infants may be attributed to α1PI deficiency approximately in 6% of cases. Hepatitis related to α1PI deficiency was characterized by persistent jaundice, hepatomegaly, elevated serum level of conjugated and unconjugated bilirubin and hemorrhagic syndrome. ARTICLE PDF AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Department of Pediatrics, Vilnius State University, Vilnius, Lithuanian SSR, USSR Petras Kaltenis & Vytautas Basys Authors * Petras Kaltenis View author publications You can also search for this author inPubMed Google Scholar * Vytautas Basys View author publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Kaltenis, P., Basys, V. SIGNIFICANCE OF α1-PROTEINASE INHIBITOR DEFICIENCY IN ETIOPATHOGENESIS OF LIVER DISEASES IN CHILDREN. _Pediatr Res_ 26, 516 (1989). https://doi.org/10.1203/00006450-198911000-00103 Download citation * Issue Date: 01 November 1989 * DOI: https://doi.org/10.1203/00006450-198911000-00103 SHARE THIS ARTICLE Anyone you share the following link with will be able to read this content: Get shareable link Sorry, a shareable link is not currently available for this article. Copy to clipboard Provided by the Springer Nature SharedIt content-sharing initiative

ABSTRACT α1-proteinase inhibitor (α1PI) phenotypes were examined in 27 children with chronic persistent hepatitis (1st group) and in 70 newborns and infants with protracted icterus and


neonatal hepatitis (2nd group). 1442 adults and children in population study served as controls (3rd group). Prevalence of phenotype PI MZ was equal to 7.41% in the 1st group, 4,29% in the


2nd group, and 3,47% in the 3rd group. Prevalence of phenotype PI MS in the groups studied was equal to 1,87%, 2,86%, and 3,33%, respectively, α1PI phenotype PI ZZ responsible for α1PI


deficiency was not encountered in the 1st group. Its prevalence in the 2nd group reached 5,71% and in the 3rd group equaled to 0,07%. Thus, the cause of hepatitis in newborns and infants may


be attributed to α1PI deficiency approximately in 6% of cases. Hepatitis related to α1PI deficiency was characterized by persistent jaundice, hepatomegaly, elevated serum level of


conjugated and unconjugated bilirubin and hemorrhagic syndrome. ARTICLE PDF AUTHOR INFORMATION AUTHORS AND AFFILIATIONS * Department of Pediatrics, Vilnius State University, Vilnius,


Lithuanian SSR, USSR Petras Kaltenis & Vytautas Basys Authors * Petras Kaltenis View author publications You can also search for this author inPubMed Google Scholar * Vytautas Basys View


author publications You can also search for this author inPubMed Google Scholar RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Kaltenis, P., Basys, V.


SIGNIFICANCE OF α1-PROTEINASE INHIBITOR DEFICIENCY IN ETIOPATHOGENESIS OF LIVER DISEASES IN CHILDREN. _Pediatr Res_ 26, 516 (1989). https://doi.org/10.1203/00006450-198911000-00103 Download


citation * Issue Date: 01 November 1989 * DOI: https://doi.org/10.1203/00006450-198911000-00103 SHARE THIS ARTICLE Anyone you share the following link with will be able to read this content:


Get shareable link Sorry, a shareable link is not currently available for this article. Copy to clipboard Provided by the Springer Nature SharedIt content-sharing initiative