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₁-Antitrypsin Deficiency in 26-Year-Old Subjects^*

Lung, Liver, and Protease/Protease Inhibitor Studies

^* From the Departments of Respiratory Medicine (Dr. Piitulainen), Clinical Chemistry (Dr. Carlson), Surgical Pathophysiology (Dr. Ohlsson), and Pediatrics (Dr. Sveger), Lund University, University Hospital, Malmö, Sweden.

Correspondence to: Tomas Sveger, MD, PhD, Department of Pediatrics, University Hospital, SE-20502 Malmö, Sweden; e-mail: tomas.sveger{at}pediatrik.mas.lu.se

Background: Clinical and biochemical signs of lung and liver disease have been followed prospectively in a birth cohort of individuals with ₁-antitrypsin (AAT) deficiency.

Objective: At age 26 years, the focus was on clinical health, lung and liver function tests, and plasma markers of the protease/antiprotease balance. The effect of early childhood environment and symptoms was also studied.

Methods: Eligible individuals were 26-year-old subjects with AAT deficiency (PiZ, n = 122; PiZ –, n = 2; PiSZ/S–, n = 53) and control subjects (PiMM, n = 44). Of the original AAT-deficient subjects, 119 completed the clinical examination and 134 answered the questionnaire.

Results: The prevalence of respiratory symptoms did not differ between the PiZ and SZ groups. Sixteen percent of PiZ and 14% of PiSZ subjects had asthma. Four current smokers (67%) and 22% of ex-smokers/never-smokers reported recurrent wheezing (p = 0.03). No difference in FEV₁ or FEV₁/FVC ratio was found between the PiZ, SZ (5% being smokers), and MM individuals (all nonsmokers). A decreased FEV₁/FVC ratio was found in PiZ subjects with neonatal cholestasis, compared to remaining PiZ subjects (p = 0.02). Recurrent wheezers at age 2 years with AAT deficiency had decreased FEV₁/FVC ratio (p = 0.025) at age 26 years. None had clinical symptoms of liver disease. Six percent of PiZ and 9% of PiSZ subjects had a marginal increase of serum alanine aminotransferase; 7% of PiZ and 4% of PiSZ had abnormal -glutamyl transferase test results. The PiZ and SZ individuals had decreased plasma albumin (p = 0.0002). Secretory leukocyte protease inhibitor (SLPI) was increased in PiZ and SZ subjects compared to PiMM subjects (p = 0.0001). Neutrophil lipocalin was decreased in PiZ subjects (p = 0.0004) and PiSZ subjects (p = 0.001) compared to PiMM individuals. The elastase/AAT complex concentration was lower in AAT-deficient subjects (p = 0.0001).

Conclusion: Twenty-six-year-old PiZ and SZ individuals (5% smokers) had normal lung function test results, and 4 to 9% had marginal deviations in liver test results. Analyses of SLPI and neutrophil lipocalin, a marker of neutrophil activity, indicate compensatory changes in the AAT-deficiency state.

Key Words: ₁-antitrypsin deficiency • epidemiology • liver disease • lung disease • neonatal screening • neutrophil lipocalin • protease • protease inhibitor

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