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

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Mostafavi, BehrouzLund University,Lunds universitet,Lungmedicin, allergologi och palliativ medicin,Sektion II,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Respiratory Medicine, Allergology, and Palliative Medicine,Section II,Department of Clinical Sciences, Lund,Faculty of Medicine,Skåne University Hospital (author)

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

Article/chapterEnglish2017

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2017

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LIBRIS-ID:oai:lup.lub.lu.se:28d5c2ad-b244-4d8a-b7d9-b8f6b4fb3b95
https://lup.lub.lu.se/record/28d5c2ad-b244-4d8a-b7d9-b8f6b4fb3b95URI
https://doi.org/10.1097/MD.0000000000006180DOI
http://kipublications.ki.se/Default.aspx?queryparsed=id:135521725URI

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Language:English
Summary in:English

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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 ?-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.

Subject headings and genre

MEDICIN OCH HÄLSOVETENSKAP Klinisk medicin Lungmedicin och allergi hsv//swe
MEDICAL AND HEALTH SCIENCES Clinical Medicine Respiratory Medicine and Allergy hsv//eng
Alpha-1-antitrypsin deficiency
Elastography
Liver disease
Liver function
Screening

Added entries (persons, corporate bodies, meetings, titles ...)

Diaz, SandraKarolinska Institutet,Lund University,Lunds universitet,Diagnostisk radiologi, Malmö,Forskargrupper vid Lunds universitet,Radiology Diagnostics, Malmö,Lund University Research Groups,Skåne University Hospital(Swepub:lu)ront-sdi (author)
Tanash, Hanan A.Lund University,Lunds universitet,Lungmedicin, allergologi och palliativ medicin,Sektion II,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Respiratory Medicine, Allergology, and Palliative Medicine,Section II,Department of Clinical Sciences, Lund,Faculty of Medicine,Skåne University Hospital(Swepub:lu)lung-hat (author)
Piitulainen, EevaLund University,Lunds universitet,Lungmedicin, allergologi och palliativ medicin,Sektion II,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Respiratory Medicine, Allergology, and Palliative Medicine,Section II,Department of Clinical Sciences, Lund,Faculty of Medicine,Skåne University Hospital(Swepub:lu)lung-epi (author)
Lungmedicin, allergologi och palliativ medicinSektion II (creator_code:org_t)

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In:Medicine96:120025-79741536-5964

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