PMID- 27564669 OWN - NLM STAT- MEDLINE DCOM- 20180105 LR - 20180924 IS - 2325-6621 (Electronic) IS - 2325-6621 (Linking) VI - 13 Suppl 4 DP - 2016 Aug TI - Alpha-1 Antitrypsin Deficiency: Phenotypes and Quality of Life. PG - S332-5 LID - 10.1513/AnnalsATS.201507-436KV [doi] AB - Alpha-1 antitrypsin deficiency (AATD) is a recognized genetic cause of rapidly progressive loss of lung function conventionally assessed by the decline in FEV1. However, there is less information concerning other physiological measures and the impact on quality of life. Data derived predominantly from the UK national registry show that AATD presents with various physiological phenotypes with differing clinical impact and progression. In general, the decline in quality of life is surprisingly slow despite rapid loss of lung function, which may reflect the benefits of centralized services for patients with AATD. Use of the new GOLD classification identifies patient characteristics that relate to mortality and disparate symptomatology despite similar spirometric impairment. FAU - Stockley, Robert A AU - Stockley RA AD - Lung Investigation Unit, Queen Elizabeth Hospital, Edgbaston, Birmingham, United Kingdom. LA - eng PT - Journal Article PT - Review PL - United States TA - Ann Am Thorac Soc JT - Annals of the American Thoracic Society JID - 101600811 SB - IM MH - Age Factors MH - Health Status MH - Humans MH - Phenotype MH - Pulmonary Emphysema/etiology/mortality/*physiopathology MH - *Quality of Life MH - Spirometry MH - alpha 1-Antitrypsin Deficiency/complications/mortality/*physiopathology OTO - NOTNLM OT - GOLD OT - alpha-1 antitrypsin deficiency OT - health status OT - lung function EDAT- 2016/08/27 06:00 MHDA- 2018/01/06 06:00 CRDT- 2016/08/27 06:00 PHST- 2016/08/27 06:00 [entrez] PHST- 2016/08/27 06:00 [pubmed] PHST- 2018/01/06 06:00 [medline] AID - 10.1513/AnnalsATS.201507-436KV [doi] PST - ppublish SO - Ann Am Thorac Soc. 2016 Aug;13 Suppl 4:S332-5. doi: 10.1513/AnnalsATS.201507-436KV.