Other clinical studyThe liver in adolescents with α1-antitrypsin deficiency☆
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Cited by (185)
Rare liver diseases in Egypt: Clinical and epidemiological characterization
2024, Arab Journal of GastroenterologyAlpha-1 Antitrypsin Deficiency Liver Disease
2022, Clinics in Liver DiseaseCitation Excerpt :Majority remained asymptomatic through childhood. Life-threatening liver disease occurred in about 3% to 5% of ZZ children in the first few months or years of life.27 However, there is concern that the outcomes reported might not be fully representative of a less homogenous genetic population, such as North America, which may carry a different array of modifier genes.28
Alpha-1 antitrypsin deficiency: A re-surfacing adult liver disorder
2022, Journal of HepatologyCitation Excerpt :Although this review is focused on liver disease in adults, it needs to be highlighted that AATD also causes paediatric liver injury, typically in the form of neonatal cholestasis.49 The strongest evidence stems from the Swedish neonatal screening programme that identified 120 newborns with Pi∗ZZ out of 200,000 newborns, while a variety of other reports relied on data from tertiary centres.50–52 In the population-based cohort, 12% of Pi∗ZZ neonates displayed prolonged jaundice, and 8% of these neonates had severe liver disease.50
UK guideline on the transition and management of childhood liver diseases in adulthood
2024, Alimentary Pharmacology and TherapeuticsDanish children with ZZ-homozygous alpha-1 antitrypsin deficiency are more affected on liver parameters than children with heterozygosity
2024, Acta Paediatrica, International Journal of Paediatrics
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This study was supported by The Swedish Heart and Lung Foundation, Lundström's and Crafoord Foundations, Sweden.