학술논문
Meta‐analysis: Prevalence of significant or advanced fibrosis in adults with alpha‐1‐antitrypsin deficiency.
Document Type
Article
Author
Huang, Daniel Q.; Chan, Kai En; Tan, Caitlyn; Zeng, Rebecca Wenling; Koh, Benjamin; Ong, Elden Yen Hng; Ong, Charlotte Chung Hui; Ong, Christen En Ya; Tan, Darren J. H.; Lim, Wen Hui; Cho, Elina; Tan, Eunice X. X.; Teng, Margaret L. P.; Ng, Cheng Han; Nah, Benjamin; Lim, Mei Chin; Muthiah, Mark; Clark, Virginia C.; Loomba, Rohit
Source
Subject
*ALPHA 1-antitrypsin deficiency
*ACOUSTIC radiation force
*ACOUSTIC radiation
*FIBROSIS
*HEPATIC fibrosis
*ACOUSTIC resonance
*MAGNETIC resonance
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Language
ISSN
0269-2813
Abstract
Summary: Background: The prevalence of liver fibrosis detected by non‐invasive imaging in alpha‐1‐antitrypsin (AAT) deficiency has not been systematically assessed. Aims: We conducted a systematic review and meta‐analysis to determine the prevalence of significant fibrosis and advanced fibrosis in AAT deficiency based on non‐invasive imaging. Methods: Medline and Embase electronic databases were searched for studies from inception to 13 November 2022 that provided data for the prevalence of fibrosis in adults with AAT deficiency. A generalised linear mixed model with Clopper–Pearson intervals was used to pool single‐arm outcomes. Results: Of the 214 records identified, 8 studies were included. Five studies assessed fibrosis using vibration‐controlled transient elastography. The prevalence of significant fibrosis (defined as ≥7.1 kPA) in Z homozygosity, Z heterozygosity and non‐carrier status was 22.10% (five studies, 95% CI: 17.07–28.12), 9.24% (three studies, 95% CI: 4.68–17.45) and 5.38% (one study, 95% CI: 3.27–8.73), respectively, p < 0.0001, and the prevalence of advanced fibrosis (defined as ≥9.5 kPa) was 8.13% (five studies, 95% CI: 4.60–13.96), 2.96% (three studies, 95% CI: 1.49–5.81) and 1.08% (one study, 95% CI: 0.35–3.28), respectively, p = 0.003. There were limited data regarding the use of magnetic resonance elastography or acoustic radiation force impulse to assess for fibrosis. Conclusion: More than one in five adult individuals with AAT deficiency and Z homozygosity harbour significant fibrosis, and nearly 1 in 10 harbours advanced fibrosis. The risk of fibrosis increases incrementally with the frequency of Pi*Z mutations. [ABSTRACT FROM AUTHOR]