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• W2907576011 abstract "Abstract Background & Aims To identify prognostic factors for liver disease in children with alpha‐1 antitrypsin deficiency, irrespective of phenotype, using the DEFI‐ALPHA cohort. Methods Retrospective, then prospective from 2010, multicentre study including children known to have alpha‐1 antitrypsin blood concentration below 0.8 g/L, born in France since 1989. Clinical and biological data were collected. Liver disease was classified as “severe” (portal hypertension, liver failure, liver transplantation or death); “moderate” (persistent abnormal liver biology without portal hypertension); and “mild/none” (normal or almost normal liver biology and native liver). Prognostic factors for severe liver disease were evaluated using a Cox semiparametric model. Results In January 2017, 153 patients from 19 centres had been included; genotypes were PIZZ in 81.9%, PISZ in 8.1%, other in 10.0%. Mean ± SD follow‐up was 4.7 ± 2.1 years. Half of patients had moderate liver disease. Twenty‐eight children (18.3%) had severe liver disease (mean age 2.5 years, range: 0‐11.6): diagnosis of alpha‐1 antitrypsin deficiency was made before two months of age in 65.4%, genotypes were PIZZ in 25 (89.3%), PISZ in 2, PIM like Z in 1, 15 children underwent liver transplantation, 1 child died at 3 years of age. Neonatal cholestasis was significantly associated with severe liver disease ( P = 0.007). Conclusion Alpha‐1 antitrypsin‐deficient patients presenting with neonatal cholestasis were likely to develop severe liver disease. Some patients with non‐homozygous ZZ genotype can develop severe liver disease, such as PISZ and M variants, when associated with predisposing factors. Further genetic studies will help to identify other factors involved in the development of liver complications." @default.
• W2907576011 created "2019-01-11" @default.
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• W2907576011 date "2019-02-01" @default.
• W2907576011 modified "2023-10-09" @default.
• W2907576011 title "Liver disease related to alpha1‐antitrypsin deficiency in French children: The DEFI‐ALPHA cohort" @default.
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• W2907576011 doi "https://doi.org/10.1111/liv.14035" @default.
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