ec6745cb861f0d530cbd168fea60494f pmc_33923569.pdf bc6747ef0fc7c24f2f5d4869550d458761693c37 pmc_33923569.pdf f37ca07e48d1e350a8804e297b9e556730d8fc120ce1bde28fa8a1d209c6e66c pmc_33923569.pdf Title: Utility of Transient Elastography for the Screening of Liver Disease in Patients with Alpha1-Antitrypsin Deficiency Subject: Screening of liver disease in alpha-1 antitrypsin deficiency (AATD) is usually carried out with liver enzymes, with low sensitivity. We conducted a multicenter cross-sectional study aiming to describe the utility of transient elastography for the identification of liver disease in patients with AATD. A total of 148 AATD patients were included. Among these, 54.7% were Pi*ZZ and 45.3% were heterozygous for the Z allele. Between 4.9% and 16.5% of patients had abnormal liver enzymes, without differences among genotypes. Liver stiffness measurement (LSM) was significantly higher in Pi*ZZ individuals than in heterozygous Z (5.6 vs. 4.6 kPa; p = 0.001). In total, in 8 (5%) individuals LSM was >7.5 kPa, considered significant liver fibrosis, and 10 kPa in 3 (1.9%) all being Pi*ZZ. Elevated liver enzymes were more frequently observed in patients with LSM > 7.5 kPa, but in 5 out of 8 of these patients all liver enzymes were within normal range. In patients with AATD, the presence of abnormal liver enzymes is frequent; however, most of these patients do not present significant liver fibrosis. Transient elastography can help to identify patients with liver fibrosis even with normal liver enzymes and should be performed in all Z-allele carriers to screen for liver disease. Keywords: alpha1-antitrypsin deficiency; liver disease; transient elastography Author: Mònica Pons, Alexa Núñez, Cristina Esquinas, María Torres-Durán, Juan Luis Rodríguez-Hermosa, Myriam Calle, Ramón Tubio-Pérez, Irene Belmonte, Francisco Rodríguez-Frías, Esther Rodríguez, Joan Genescà, Marc Miravitlles and Miriam Barrecheguren Creator: LaTeX with hyperref Producer: pdfTeX-1.40.21 CreationDate: Fri Apr 16 12:25:11 2021 CEST ModDate: Fri Apr 16 12:32:24 2021 CEST Custom Metadata: no Metadata Stream: no Tagged: no UserProperties: no Suspects: no Form: none JavaScript: no Pages: 13 Encrypted: no Page size: 595.276 x 841.89 pts (A4) Page rot: 0 File size: 865080 bytes Optimized: no PDF version: 1.7 name type encoding emb sub uni object ID ------------------------------------ ----------------- ---------------- --- --- --- --------- AWHDWW+VnURWPalladioL Type 1 Custom yes yes no 10 0 YOWCKL+URWPalladioL-Ital Type 1 Custom yes yes no 15 0 ZHETQZ+URWPalladioL-Bold Type 1 Custom yes yes no 20 0 EDDHQY+URWPalladioL-Roma Type 1 Custom yes yes no 24 0 KQDAYE+CMSY10 Type 1 Builtin yes yes no 28 0 AHXVRP+VnURWPalladioL-Bold Type 1 Custom yes yes no 32 0 XPUSBJ+TimesNewRomanPS-BoldItalicMT Type 1C WinAnsi yes yes no 40 0 BSDWLC+TimesNewRomanPSMT Type 1C WinAnsi yes yes no 43 0 MCBCCI+SourceSansRoman-Regular Type 1C Custom yes yes no 65 0 MCBCCM+SourceSansRoman.613wght Type 1C Custom yes yes no 69 0 MCBCCO+SourceSansRoman-Bold Type 1C Custom yes yes no 73 0 QLBIEB+URWPalladioL-BoldItal Type 1 Custom yes yes no 91 0 FNTSBS+PalatinoLinotype-Bold CID TrueType Identity-H yes yes yes 103 0 FNTSBS+PalatinoLinotype-Roman CID TrueType Identity-H yes yes yes 109 0 FNTSBS+PalatinoLinotype-Italic CID TrueType Identity-H yes yes yes 115 0 FNTSBS+PalatinoLinotype-Bold CID TrueType Identity-H yes yes yes 130 0 FNTSBS+PalatinoLinotype-Roman CID TrueType Identity-H yes yes yes 136 0 FNTSBS+PalatinoLinotype-Italic CID TrueType Identity-H yes yes yes 142 0 FNTSBS+PalatinoLinotype-Bold CID TrueType Identity-H yes yes yes 152 0 FNTSBS+PalatinoLinotype-Roman CID TrueType Identity-H yes yes yes 158 0 FNTSBS+PalatinoLinotype-Italic CID TrueType Identity-H yes yes yes 164 0 CKBPVA+EURM10 Type 1 Builtin yes yes no 185 0 Jhove (Rel. 1.28.0, 2023-05-18) Date: 2024-10-02 03:20:06 CEST RepresentationInformation: pmc_33923569.pdf ReportingModule: PDF-hul, Rel. 1.12.4 (2023-03-16) LastModified: 2023-03-31 12:09:51 CEST Size: 865080 Format: PDF Version: 1.7 Status: Well-Formed and valid SignatureMatches: PDF-hul MIMEtype: application/pdf PDFMetadata: Objects: 362 FreeObjects: 1 IncrementalUpdates: 0 DocumentCatalog: PageLayout: SinglePage PageMode: UseNone Outlines: Item: Title: Introduction Destination: section.1 Item: Title: Materials and Methods Destination: section.2 Children: Item: Title: Variables Destination: subsection.2.1 Item: Title: Liver Stiffness Measurement by Transient Elastography Destination: subsection.2.2 Item: Title: Statistical Analysis Destination: subsection.2.3 Item: Title: Results Destination: section.3 Children: Item: Title: Demographic and Clinical Findings Destination: subsection.3.1 Item: Title: Clinical and Laboratory Signs of Liver Disease Destination: subsection.3.2 Item: Title: Transient Elastography Destination: subsection.3.3 Item: Title: Characteristics of Pi*ZZ Patients According to LSM Values Destination: subsection.3.4 Item: Title: Comparison between Pi*ZZ Patients with or without COPD Destination: subsection.3.5 Item: Title: Discussion Destination: section.4 Item: Title: References Destination: section.4 Info: Title: Utility of Transient Elastography for the Screening of Liver Disease in Patients with Alpha1-Antitrypsin Deficiency Author: Mònica Pons, Alexa Núñez, Cristina Esquinas, María Torres-Durán, Juan Luis Rodríguez-Hermosa, Myriam Calle, Ramón Tubio-Pérez, Irene Belmonte, Francisco Rodríguez-Frías, Esther Rodríguez, Joan Genescà, Marc Miravitlles and Miriam Barrecheguren Subject: Screening of liver disease in alpha-1 antitrypsin deficiency (AATD) is usually carried out with liver enzymes, with low sensitivity. We conducted a multicenter cross-sectional study aiming to describe the utility of transient elastography for the identification of liver disease in patients with AATD. A total of 148 AATD patients were included. Among these, 54.7% were Pi*ZZ and 45.3% were heterozygous for the Z allele. Between 4.9% and 16.5% of patients had abnormal liver enzymes, without differences among genotypes. Liver stiffness measurement (LSM) was significantly higher in Pi*ZZ individuals than in heterozygous Z (5.6 vs. 4.6 kPa; p = 0.001). In total, in 8 (5%) individuals LSM was >7.5 kPa, considered significant liver fibrosis, and 10 kPa in 3 (1.9%) all being Pi*ZZ. Elevated liver enzymes were more frequently observed in patients with LSM > 7.5 kPa, but in 5 out of 8 of these patients all liver enzymes were within normal range. In patients with AATD, the presence of abnormal liver enzymes is frequent; however, most of these patients do not present significant liver fibrosis. Transient elastography can help to identify patients with liver fibrosis even with normal liver enzymes and should be performed in all Z-allele carriers to screen for liver disease. 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