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- W3037508104 abstract "TYPE: Abstract Publication TOPIC: Diffuse Lung Disease PURPOSE: Analyze the natural history of AATD subjects identified during the twenty-five years’ experience of the Referral Center of Spedali Civili, Brescia (Italy). METHODS: Retrospective clinical analysis of patients referred to our Center since January 1996 up to January 2020. The inclusion criterion was a diagnosis of AATD via genotyping or protein phenotyping. RESULTS: 1021 patients were included (53% males). At the time of diagnosis, mean AAT serum level was 79±27 mg/dL. We diagnosed 825 simple heterozygotes (genotype PiMZ 65%), 117 compound heterozygotes (genotype PiSZ 54%) and 79 homozygotes (genotype PiZZ 82%); 151 (15%) patients were carriers of rare variants, especially Mmalton (35). Index cases (diagnosed due to diseases correlated with AATD) were 223 (22%), 70 of them suffered from emphysema. Among non-index cases, family history for AATD was the most common cause of diagnosis (70%). Augmentation therapy was prescribed in 38 patients; no adverse drug reactions were observed. FEV1 annual decline (mL/year) and the annual trend of blood transaminases have been evaluated. CONCLUSIONS: Z-AAT resulted the most common variant, although many rarer ones are present. Experienced Referral Centers are crucial for AATD correct management and to carry out a timely diagnosis. Our final data regarding natural history of AATD will let clinicians better understand the prognosis of AATD, supporting them to make the better medical decision. CLINICAL IMPLICATIONS: This research increases the knowledge on AATD natural history and its optimal management. DISCLOSURE: No significant relationships. KEYWORDS: Alpha1-Antitrypsin deficiency, Retrospective Analysis, AATD" @default.
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- W3037508104 date "2020-06-01" @default.
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- W3037508104 title "ALPHA1-ANTITRYPSIN DEFICIENCY: A 25-YEAR EXPERIENCE" @default.
- W3037508104 doi "https://doi.org/10.1016/j.chest.2020.05.199" @default.
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