Alpha-1 Antitrypsin Replacement Improves Walking Capacity in Subjects With Severe Deficiency.
This article focuses on evaluating the clinical and functional effects of alpha-1 antitrypsin replacement therapy in subjects with severe alpha-1 antitrypsin deficiency, a genetic disorder linked to pulmonary emphysema and chronic obstructive pulmonary disease (COPD). In a retrospective study of sev...
| Publicado en: | Respiratory Care Vol. 71; no. 5; pp. 496 - 501 |
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| Autores principales: | , , , , , , , , , , |
| Formato: | research tables/charts Journal Article |
| Publicado: |
Mary Ann Liebert, Inc.
May2026
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=193488579&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 193488579 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 00201324 4GG jtl: Respiratory Care issn: 00201324 maglogo: N pubinfo: dt: May2026 vid: 71 iid: 5 pid: 1365 pub: Mary Ann Liebert, Inc. place: New Rochelle, New York artinfo: ui: 193488579 193488579 193488579 10.1177/19433654251412331 193488579 ppf: 496 ppct: 5 formats: tig: atl: Alpha-1 Antitrypsin Replacement Improves Walking Capacity in Subjects With Severe Deficiency. aug: au: Marchese, Alessandra Frizzelli, Annalisa De Simoni, Alessandro Bondarenko, Olha Pisi, Roberta Aluia, Agnese Pelà, Giovanna Ledda, Roberta Eufrasia Caramori, Gaetano Chetta, Alfredo Aiello, Marina affil: Drs. Marchese, Frizzelli, De Simoni, Bondarenko, Pisi, Aluia, Prof. Caramori, Professors Chetta and Aiello are affiliated with Division of Pneumology, Department of Medicine and Surgery, University of Parma, Parma, Italy. sug: subj: alpha 1-Antitrypsin Deficiency Therapy Walking Genotype Lung Physiology Disease Exacerbation Dyspnea Outcomes (Health Care) Human Italy Italy Male Female Adult Middle Age Aged Retrospective Design Record Review Descriptive Statistics Paired T-Tests Data Analysis Software Adult: 19-44 years Middle Aged: 45-64 years Aged: 65+ years Male Female ab: This article focuses on evaluating the clinical and functional effects of alpha-1 antitrypsin replacement therapy in subjects with severe alpha-1 antitrypsin deficiency, a genetic disorder linked to pulmonary emphysema and chronic obstructive pulmonary disease (COPD). In a retrospective study of seven patients with both common and rare severe genotypes, at least 12 months of weekly intravenous augmentation therapy significantly improved quality of life, dyspnea (breathlessness), and six-minute walk test (6MWT) distance, while reducing the frequency of bronchial exacerbations. However, no significant changes were observed in standard lung function parameters or arterial blood gases. The study highlights potential benefits of replacement therapy on physical fitness and symptom relief but notes limitations including a small sample size and lack of a control group, suggesting the need for further research. pubtype: Academic Journal doctype: research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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