PMID- 37813825 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20240307 IS - 2372-952X (Print) IS - 2372-952X (Electronic) IS - 2372-952X (Linking) VI - 11 IP - 1 DP - 2024 Jan 25 TI - Pulmonary Rehabilitation for Chronic Obstructive Pulmonary Disease Patients with Underlying Alpha-1 Antitrypsin Deficiency: A Systematic Review and Practical Recommendations. PG - 121-132 LID - 10.15326/jcopdf.2023.0434 [doi] AB - BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) is an often-overlooked genetic condition that makes individuals susceptible to early onset of chronic obstructive pulmonary disease (COPD). The established benefits of exercise-based pulmonary rehabilitation (PR) for usual COPD patients are unclear for those with underlying AATD, especially given potentially differing muscle adaptations to exercise. This review seeks to compare PR outcomes between AATD and usual COPD patients and to consolidate current knowledge on exercise intervention outcomes for the AATD population. METHODS: A thorough search of 4 databases (Ovid, Medline, CINAHL, CENTRAL) was conducted based on 3 search concepts: (1) alpha-1 antitrypsin deficiency, (2) pulmonary rehabilitation OR exercise, and (3) muscle morphology. A dual review process and quality assessment were independently implemented throughout all stages of the review. RESULTS: Four studies highlighted modest exercise capacity and quality of life in AATD patients undergoing PR. However, one study reported unique muscle and mitochondrial responses compared to usual COPD patients. Additionally, a moderate exercise session did not alter pro-inflammatory cytokine levels in AATD patients, despite higher levels of tumor necrosis factor-alpha levels in muscle biopsies compared to usual COPD patients. CONCLUSIONS: The current literature base insufficiently addresses the efficacy of PR on AATD, with indications that exercise adaptation may deviate from that of usual COPD patients. Further research is needed to optimize PR, particularly in identifying the most suitable exercise intensity, and delivery setting, and addressing specific educational needs for individuals with AATD. CI - JCOPDF (c) 2024. FAU - Alwadani, Fawaz A AU - Alwadani FA AD - Institute for Applied Health Research, University of Birmingham, Birmingham, United Kingdom. AD - Department of Physical Therapy, Jazan University, Jazan, Saudi Arabia. FAU - Wheeler, Kyrie AU - Wheeler K AD - Heartlands Hospital, Birmingham, United Kingdom. FAU - Pittaway, Harriet AU - Pittaway H AD - Institute of Inflammation and Ageing, University of Birmingham, Birmingham, United Kingdom. FAU - Turner, Alice M AU - Turner AM AD - Institute for Applied Health Research, University of Birmingham, Birmingham, United Kingdom. LA - eng PT - Journal Article PL - United States TA - Chronic Obstr Pulm Dis JT - Chronic obstructive pulmonary diseases (Miami, Fla.) JID - 101635411 PMC - PMC10913928 OTO - NOTNLM OT - AATD OT - alpha-1 antitrypsin deficiency OT - chronic obstructive pulmonary disease OT - exercise therapy OT - pulmonary rehabilitation COIS- All authors declare no conflict of interest. EDAT- 2023/10/10 00:41 MHDA- 2023/10/10 00:42 PMCR- 2023/10/09 CRDT- 2023/10/09 22:53 PHST- 2023/10/10 00:42 [medline] PHST- 2023/10/10 00:41 [pubmed] PHST- 2023/10/09 22:53 [entrez] PHST- 2023/10/09 00:00 [pmc-release] AID - 10.15326/jcopdf.2023.0434 [doi] PST - ppublish SO - Chronic Obstr Pulm Dis. 2024 Jan 25;11(1):121-132. doi: 10.15326/jcopdf.2023.0434.