title: Key current clinical issues in alpha-1 antitrypsin deficiency.
abstract: Alpha-1 antitrypsin deficiency is a common but under-recognized condition on which respiratory therapists can have a large impact. A key recent development is the issuance of an international evidence-based standards document regarding diagnosis and management of individuals with alpha-1 antitrypsin deficiency. This report summarizes that standards document, which recommends more widespread testing for alpha-1 antitrypsin deficiency, in order to extend the benefits of diagnosis to individuals found to have alpha-1 antitrypsin deficiency. An important aspect of the standards document is that evidence regarding the clinical efficacy of intravenous alpha-1 antitrypsin augmentation therapy is reviewed. Though no definitive support from a randomized clinical trial is available, the weight of evidence favors the clinical efficacy of alpha-1 antitrypsin augmentation therapy, at least in individuals with moderate degrees of established airflow obstruction. On that basis the standards document recommends intravenous augmentation therapy in specified clinical circumstances.
authors.affiliation: Department of Pulmonary and Critical Care Medicine, A90, The Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland OH 44195, USA. stollej@ccf.org.
authors.forename: James K
authors.initials: JK
authors.lastname: Stoller
mesh_terms: D000293:Adolescent; D000328:Adult; D002985:Clinical Protocols; D006801:Humans; D007231:Infant, Newborn; D007262:Infusions, Intravenous; D008403:Mass Screening / Q000592:standards*; D016896:Treatment Outcome; D000515:alpha 1-Antitrypsin / Q000008:administration & dosage*; D019896:alpha 1-Antitrypsin Deficiency / Q000175:diagnosis* / Q000188:drug therapy*
chemical_list: D000515:alpha 1-Antitrypsin