Liver disease in adults with α1-antitrypsin deficiency.

Mattias MandorferTheresa BucsicsVeronika HutyaKarin Schmid-ScherzerBenedikt SchaeferHeinz ZollerArnulf FerlitschMarkus Peck-RadosavljevicMichael TraunerPeter FerenciMeinhard KneusslThomas Reiberger

Published in: United European gastroenterology journal (2018)

Heterozygosity for the Z-allele predisposes for the development of CSPH, confirming its role as a genetic (co)factor in liver disease. Pi*ZZ/SZ patients rarely develop liver fibrosis ≥F3 during adulthood; however, liver fibrosis ≥F2 is common. Elevated CAP values hint at underlying hepatic steatosis, which might promote liver fibrosis progression.

Keyphrases

liver fibrosis
end stage renal disease
ejection fraction
newly diagnosed
chronic kidney disease
depressive symptoms
genome wide
gene expression
patient reported outcomes
copy number
replacement therapy