PMID: 6984276Nov 1, 1982Paper

Liver disease in children with alpha 1-antitrypsin deficiency without neonatal cholestasis

Acta paediatrica Scandinavica
A Nemeth, B Strandvik

Abstract

Thirteen children with alpha 1-antitrypsin deficiency (8 PiZ and 5 PiSZ) were investigated at ages ranging from 4 to 6. None had had neonatal cholestasis. Nine, mainly the PiZ individuals, had increased serum concentration of transaminases. Liver biopsy was performed in 7 patients with increased serum levels of transaminases. One of these patients had cirrhosis and 4 had moderate to severe fibrosis. The results indicate that alpha 1-antitrypsin deficient individuals, also without neonatal cholestasis syndrome run a high risk of developing serious liver disease, already in childhood. The cirrhotic patient was the only one who had increased excretion of bile acids in urine.

References

Dec 1, 1978·The American Journal of Medicine·C H ChanE A Jones
Jan 1, 1977·Scandinavian Journal of Gastroenterology·C Larsson, S Eriksson
Jun 10, 1976·The New England Journal of Medicine·T Sveger
Aug 1, 1974·The Journal of Pediatrics·E J WilkinsonT A Hosty
Sep 10, 1970·The New England Journal of Medicine·G Menghini
Nov 1, 1982·Acta paediatrica Scandinavica·A Nemeth, B Strandvik

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Citations

Oct 3, 2003·American Journal of Respiratory and Critical Care Medicine·UNKNOWN American Thoracic Society, UNKNOWN European Respiratory Society
Nov 1, 1982·Acta paediatrica Scandinavica·A Nemeth, B Strandvik
Mar 1, 1988·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·F K Ghishan, H L Greene
Feb 16, 2018·Alimentary Pharmacology & Therapeutics·S A TownsendA M Turner
Oct 17, 2017·Molecular Therapy : the Journal of the American Society of Gene Therapy·Florie BorelChristian Mueller

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