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      Liver function in alpha-1-antitrypsin deficient individuals at 37 to 40 years of age

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          Abstract

          Severe alpha-1-antitrypsin (AAT) deficiency (PiZZ) is a risk factor for liver disease, but the prevalence of liver cirrhosis and hepatocellular cancer in PiZZ adults is unknown. The risk of liver disease in adults with moderate AAT deficiency (PiSZ) is also unknown. A cohort of 127 PiZZ, 2 PiZnull, 54 PiSZ, and 1 PiSnull individuals were identified by the Swedish national neonatal AAT screening program between 1972 and 1974, when all 200,000 newborn infants in Sweden were screened for AAT deficiency. The cohort has been followed up since birth. Our aim was to study liver function and signs of liver disease in this cohort at 37 to 40 years of age in comparison with a matched, random sample of control subjects identified from the population registry.

          Eighty seven PiZZ, 32 PiSZ, and 92 control subjects (PiMM) answered a questionnaire on medication and alcohol consumption and provided blood samples. Liver stiffness was assessed by Acoustic Radiation Force Impulse (ARFI) elastography in 32 PiZZ, 15 PiSZ, and 51 PiMM subjects.

          The median of liver function tests and procollagen-III-peptide were within the normal range in all Pi subgroups. However, the PiZZ men had significantly higher plasma bilirubin than the PiMM men ( P = 0.018). Plasma ɣ-glutamyl transferase (GGT) was significantly higher in the PiZZ men ( P = 0.009) and the PiSZ men ( P = 0.021) compared with the PiMM men. The median of liver stiffness was significantly higher in the PiZZ men ( P = 0.037) and the PiSZ men ( P = 0.032) compared with the PiMM men. The PiZZ women taking medication influencing liver enzymes had significantly higher GGT than the PiMM women on the corresponding treatment ( P = 0.023).

          These AAT-deficient individuals identified by neonatal screening have normal plasma levels of liver function tests, and no clinical signs indicating liver disease at the age of 37 to 40 years. However, bilirubin, GGT, and liver stiffness are significantly higher in PiZZ men than PiMM men.

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          EFSUMB guidelines and recommendations on the clinical use of ultrasound elastography. Part 1: Basic principles and technology.

          J. Bamber, D. Cosgrove, C Dietrich … (2013)
          The technical part of these Guidelines and Recommendations, produced under the auspices of EFSUMB, provides an introduction to the physical principles and technology on which all forms of current commercially available ultrasound elastography are based. A difference in shear modulus is the common underlying physical mechanism that provides tissue contrast in all elastograms. The relationship between the alternative technologies is considered in terms of the method used to take advantage of this. The practical advantages and disadvantages associated with each of the techniques are described, and guidance is provided on optimisation of scanning technique, image display, image interpretation and some of the known image artefacts. © Georg Thieme Verlag KG Stuttgart · New York.
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            WFUMB guidelines and recommendations for clinical use of ultrasound elastography: Part 3: liver.

            Giovanna Ferraioli, Carlo Filice, Laurent Castéra … (2015)
            The World Federation for Ultrasound in Medicine and Biology (WFUMB) has produced these guidelines for the use of elastography techniques in liver disease. For each available technique, the reproducibility, results, and limitations are analyzed, and recommendations are given. Finally, recommendations based on the international literature and the findings of the WFUMB expert group are established as answers to common questions. The document has a clinical perspective and is aimed at assessing the usefulness of elastography in the management of liver diseases.
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              Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants.

              T Sveger (1976)
              We prosepctively studied 200,000 newborns to determine the frequency and clinical characteristics of alpha1-antitrypsin deficiency. One hundred and twenty Pi Z, 48 Pi SZ, two PI Z-and one Pi S-infants were identified and followed to the age of six months. Fourteen of 120 Pi Z infants had prolonged obstructive jaundice, nine with severe clinical and laboratory evidence of liver disease. Five had only laboratory evidence of liver disease. Eight other Pi Z infants had minimal abnormalities in serum bilirubin and hepatic enzyme activity and variable hepatosplenomegaly. All 22 Pi Z infants with hepatic abnormalities, two thirds of whom were made, appeared healthy at six months of age. Ninety-eight Pi Z infants did not have clinical liver disease, but liver-function tests gave abnormal results in 44 of 84 at three months, and in 36 of 60 at six months of age. The number of small-for-gestational-age infants was greater (P less than 0.001) among those with clinical liver disease. None of the 48 Pi SZ infants had clinical liver disease, but 10 of 42 at three months and one of 22 at six months of age had abnormal liver function. The Pi Z and Pi SZ phenotypes are associated with covert or readily apparent hepatic dysfunction in the first three months of life.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Medicine (Baltimore)
                Journal ID (iso-abbrev): Medicine (Baltimore)
                Journal ID (publisher-id): MEDI
                Title: Medicine
                Publisher: Wolters Kluwer Health
                ISSN (Print): 0025-7974
                ISSN (Electronic): 1536-5964
                Publication date Collection: March 2017
                Publication date (Electronic): 24 March 2017
                Volume: 96
                Issue: 12
                Electronic Location Identifier: e6180
                Affiliations
                [a ]Department of Respiratory Medicine and Allergology Malmö
                [b ]Department of Clinical Radiology Malmö, Skåne University Hospital, Lund University, Sweden.
                Author notes
                [∗ ]Correspondence: Eeva Piitulainen, Department of Respiratory Medicine and Allergology, Skåne University Hospital, Lund University, Jan Waldenströms gata 24, plan 4, S-205 02 Malmö, Sweden (e-mail: eeva.piitulainen@ 123456med.lu.se ).
                Article
                Publisher ID: MD-D-16-03863 Accession ID: 06180
                DOI: 10.1097/MD.0000000000006180
                PMC ID: 5371441
                PubMed ID: 28328804
                SO-VID: b34e7439-0865-4026-92a9-fb1ce7dd9756
                Copyright © Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0

                History
                Date received : 31 May 2016
                Date revision received : 31 January 2017
                Date accepted : 1 February 2017
                Categories
                Subject: 4500
                Subject: Research Article
                Subject: Observational Study
                Custom metadata
                OPEN-ACCESS TRUE

                Keywords: alpha-1-antitrypsin deficiency,elastography,liver disease,liver function,screening
                Data availability:
                Keywords: alpha-1-antitrypsin deficiency, elastography, liver disease, liver function, screening

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