PMID- 26653189 OWN - NLM STAT- MEDLINE DCOM- 20160920 LR - 20220318 IS - 2325-6621 (Electronic) IS - 2329-6933 (Print) IS - 2325-6621 (Linking) VI - 12 IP - 12 DP - 2015 Dec TI - Characteristics of Alpha-1 Antitrypsin-Deficient Individuals in the Long-term Oxygen Treatment Trial and Comparison with Other Subjects with Chronic Obstructive Pulmonary Disease. PG - 1796-804 LID - 10.1513/AnnalsATS.201506-389OC [doi] AB - RATIONALE: Alpha-1 antitrypsin deficiency (AATD) predisposes to chronic obstructive pulmonary disease, but is underrecognized. Oxygenation and exercise desaturation in individuals with AATD-associated chronic obstructive pulmonary disease has been sparsely studied. The Long-term Oxygen Treatment Trial (LOTT) permits comparing these features of individuals with AATD with alpha-1 antitrypsin-replete (called "usual chronic obstructive pulmonary disease") LOTT participants. OBJECTIVES: Compare demographic, clinical, baseline oxygenation, and exercise desaturation features in participating AATD subjects with those of other LOTT subjects. METHODS: LOTT is a multicenter randomized controlled trial comparing use of supplemental oxygen versus not in subjects with chronic obstructive pulmonary disease and moderate hypoxemia (resting oxygen saturation as measured by pulse oximetry, 89-93%) or normal oxygen saturation at rest and significant exercise desaturation. MEASUREMENT AND MAIN RESULTS: Among the 597 LOTT participants with nonmissing alpha-1 antitrypsin levels, 11 (1.8%) had severe AATD and 44 (7.4%) had mild/moderate AATD. Comparison of the 11 severely AAT-deficient individuals with the 542 LOTT participants with usual chronic obstructive pulmonary disease showed that the AATD subjects were younger and despite less smoking, had lower FEV1/FVC (mean post-bronchodilator FEV1/FVC, 0.38 +/- 0.06 vs. 0.46 +/- 0.13; P = 0.002). Comparison with 27 age-, sex-, and FEV1-matched alpha-1 antitrypsin-normal LOTT participants showed no baseline difference in resting room air pulse oximetry saturation (AATD, 93.6% +/- 2.3% vs. 92.7% +/- 2.2%; P = 0.64). Exercise-related desaturation was more severe in the individuals with AATD based on desaturation to 88% or less sooner during a 6-minute-walk test, having a higher percentage of desaturation points (e.g., <90%) during exercise, and having a higher distance-saturation product (defined as the distance walked in 6 min multiplied by the nadir saturation achieved during the 6-minute-walk test). CONCLUSIONS: These data suggest that individuals with AATD experience more profound desaturation with exercise than age-, sex-, race-, and FEV1-matched control subjects with usual chronic obstructive pulmonary disease. Clinical trial registered with www.clinicaltrials.gov (NCT 00692198). FAU - Stoller, James K AU - Stoller JK AD - 1 Respiratory Institute, and. AD - 2 Education Institute, Cleveland Clinic, Cleveland, Ohio. FAU - Aboussouan, Loutfi S AU - Aboussouan LS AD - 1 Respiratory Institute, and. FAU - Kanner, Richard E AU - Kanner RE AD - 3 Division of Pulmonary Medicine, University of Utah, Salt Lake City, Utah. FAU - Wilson, Laura A AU - Wilson LA AD - 4 Epedemiology Department, Johns Hopkins School of Public Health, Baltimore, Maryland. FAU - Diaz, Phil AU - Diaz P AD - 5 Pulmonary, Allergy, Critical Care, and Sleep Medicine Division, Ohio State University, Columbus, Ohio. FAU - Wise, Robert AU - Wise R AD - 6 Pulmonary and Critical Care Medicine, John Hopkins, Baltimore, Maryland; and. CN - LOTT Research Group LA - eng SI - ClinicalTrials.gov/NCT00692198 GR - HHSN268200736197C/PHS HHS/United States GR - HHSN268200736191C/PHS HHS/United States GR - HHSN268200736196C/PHS HHS/United States GR - HHSN268200736184C/PHS HHS/United States GR - HHSN268200736189C/PHS HHS/United States GR - Y1-HR-8076-01/HR/NHLBI NIH HHS/United States GR - HHSN268200736183C/PHS HHS/United States GR - HHSN268200736190C/PHS HHS/United States GR - HHSN268200736186C/PHS HHS/United States GR - HHSN268200736192C/PHS HHS/United States GR - Y1-HR-7019-01/HR/NHLBI NIH HHS/United States GR - HHSN268200736195C/PHS HHS/United States GR - HHSN268200736193C/PHS HHS/United States GR - HHSN268200736187C/PHS HHS/United States GR - HHSN268200736185C/PHS HHS/United States GR - HHSN268200736194C/PHS HHS/United States GR - HHSN268200736188C/PHS HHS/United States PT - Journal Article PT - Multicenter Study PT - Randomized Controlled Trial PT - Research Support, N.I.H., Extramural PT - Research Support, U.S. Gov't, P.H.S. PL - United States TA - Ann Am Thorac Soc JT - Annals of the American Thoracic Society JID - 101600811 RN - 0 (alpha 1-Antitrypsin) SB - IM MH - Aged MH - Exercise/*physiology MH - Exercise Test MH - Female MH - Follow-Up Studies MH - Forced Expiratory Volume MH - Humans MH - Male MH - Middle Aged MH - Oximetry MH - Oxygen Inhalation Therapy/*methods MH - Pulmonary Disease, Chronic Obstructive/complications/diagnosis/*therapy MH - Retrospective Studies MH - Time Factors MH - Treatment Outcome MH - alpha 1-Antitrypsin/*blood MH - alpha 1-Antitrypsin Deficiency/*complications/diagnosis PMC - PMC4722829 OTO - NOTNLM OT - Long-term Oxygen Treatment Trial OT - alpha-1 antitrypsin deficiency OT - desaturation OT - oxygenation FIR - Bailey, William C IR - Bailey WC FIR - Fuhlbrigge, Anne L IR - Fuhlbrigge AL FIR - Sampong, Ernestina IR - Sampong E FIR - Sloan, Karin IR - Sloan K FIR - Wagner, Ashley IR - Wagner A FIR - Anderson, Susan IR - Anderson S FIR - Moy, Marilyn IR - Moy M FIR - Okunbor, Osarenoma IR - Okunbor O FIR - Stoller, James K IR - Stoller JK FIR - Marlow, Scott IR - Marlow S FIR - Meli, Yvonne IR - Meli Y FIR - Rice, Richard IR - Rice R FIR - Aboussouan, Loutfi S IR - Aboussouan LS FIR - Castele, Robert IR - Castele R FIR - Parambil, Joseph IR - Parambil J FIR - Khatri, Sumita IR - Khatri S FIR - Pande, Aman IR - Pande A FIR - Zein, Joe IR - Zein J FIR - Olbrych, Thomas IR - Olbrych T FIR - Alkins, Stephan IR - Alkins S FIR - Jocko, Christine IR - Jocko C FIR - Rahaghi, Franck IR - Rahaghi F FIR - Barton, Jean IR - Barton J FIR - Albert, Richard K IR - Albert RK FIR - Underwood, Jennifer IR - Underwood J FIR - Make, Barry IR - Make B FIR - Underwood, Jennifer IR - Underwood J FIR - MacIntyre, Neil IR - MacIntyre N FIR - Davies, John IR - Davies J FIR - Stibolt, Thomas IR - Stibolt T FIR - Mularski, Richard IR - Mularski R FIR - Naleway, Allison IR - Naleway A FIR - Vertrees, Sarah IR - Vertrees S FIR - Casaburi, Richard IR - Casaburi R FIR - Porszasz, Janos IR - Porszasz J FIR - Walker, Peggy IR - Walker P FIR - Indelicato, Renee IR - Indelicato R FIR - Specht, Lennard IR - Specht L FIR - Ellstrom, Kathleen IR - Ellstrom K FIR - Portillo, Jamie IR - Portillo J FIR - Horak, David IR - Horak D FIR - Tiep, Brian IR - Tiep B FIR - Barnett, Mary IR - Barnett M FIR - Diaz, Philip IR - Diaz P FIR - Drake, Janice IR - Drake J FIR - Rittinger, Mahasti IR - Rittinger M FIR - Compton, Rachael IR - Compton R FIR - Panos, Ralph IR - Panos R FIR - Lach, Laura A IR - Lach LA FIR - Criner, Gerard IR - Criner G FIR - Grabianowski, Carla IR - Grabianowski C FIR - Cordova, Francis IR - Cordova F FIR - Desai, Parag IR - Desai P FIR - Krachman, Samuel IR - Krachman S FIR - Mamary, James IR - Mamary J FIR - Marchetti, Nathaniel IR - Marchetti N FIR - Satti, Aditi IR - Satti A FIR - Mumm, Eileen IR - Mumm E FIR - Vega-Olivo, Michelle IR - Vega-Olivo M FIR - Hua, Jenny IR - Hua J FIR - Tauch, Vanna IR - Tauch V FIR - Criner, Lii-Yoong IR - Criner LY FIR - Jacobs, Michael IR - Jacobs M FIR - Rising, Peter IR - Rising P FIR - Simonelli, Paul IR - Simonelli P FIR - Mitchell, Michele IR - Mitchell M FIR - Lammi, Matthew IR - Lammi M FIR - Romaine, Connie IR - Romaine C FIR - Lee, Howard IR - Lee H FIR - Ianacone, Mary IR - Ianacone M FIR - Scharf, Steven IR - Scharf S FIR - Bell-Farrell, Wanda IR - Bell-Farrell W FIR - Mador, M Jeffery IR - Mador M FIR - Rahman, Ayesha IR - Rahman A FIR - Zaman, Mumtaz IR - Zaman M FIR - Hill, Lisa IR - Hill L FIR - Platt, Alec IR - Platt A FIR - Cooper, J Allen Jr IR - Cooper J Jr FIR - Harrington, Kathleen IR - Harrington K FIR - Dransfield, Mark IR - Dransfield M FIR - Smith, Patti IR - Smith P FIR - Davis, Donald IR - Davis D FIR - Cooper, J Allen Jr IR - Cooper J Jr FIR - Smith, Patti IR - Smith P FIR - Sriram, Peruvemba IR - Sriram P FIR - Herring, Katherine IR - Herring K FIR - Gay, Steven IR - Gay S FIR - Martinez, Fernando IR - Martinez F FIR - Han, Meilan IR - Han M FIR - Rysso, Kelly IR - Rysso K FIR - Meldrum, Catherine IR - Meldrum C FIR - Ravikrishnan, K P IR - Ravikrishnan K FIR - Keena, Daniel IR - Keena D FIR - DeRidder, Jennifer IR - DeRidder J FIR - Kring, Beth IR - Kring B FIR - Anzueto, Antonio IR - Anzueto A FIR - Aguilera, Alex IR - Aguilera A FIR - Houlihan, Timothy IR - Houlihan T FIR - Girgis, Reda IR - Girgis R FIR - Cannestra, Jennifer IR - Cannestra J FIR - Sciurba, Frank IR - Sciurba F FIR - Kelly, Benjamin IR - Kelly B FIR - Kanner, Richard IR - Kanner R FIR - Scholand, Mary Beth IR - Scholand MB FIR - Villegas, G Martin IR - Villegas G FIR - Carle, Judy IR - Carle J FIR - Au, David IR - Au D FIR - Udris, Edmunds IR - Udris E FIR - Curtis, Randall IR - Curtis R FIR - Au, David IR - Au D FIR - Feemster, Laura C IR - Feemster LC FIR - Goodman, Richard IR - Goodman R FIR - Moss, Brianna IR - Moss B FIR - Reinke, Lynn IR - Reinke L FIR - Udris, Edmunds IR - Udris E FIR - Aitken, Moira IR - Aitken M FIR - Culver, Bruce IR - Culver B FIR - Yusen, Roger IR - Yusen R FIR - Castro, Mario IR - Castro M FIR - Mittler, Brigitte IR - Mittler B FIR - Heaghney, Jeanne IR - Heaghney J FIR - Jacobs, Myron IR - Jacobs M FIR - Joo, Min IR - Joo M FIR - Bracken, Nina IR - Bracken N FIR - Diamond, Edward IR - Diamond E FIR - Joseph, Mary K IR - Joseph MK FIR - Soler, Xavier IR - Soler X FIR - Villa, Arianna IR - Villa A FIR - Layish, Daniel IR - Layish D FIR - Silverman, Edwin IR - Silverman E FIR - Kelly, Roxanne IR - Kelly R FIR - Cossette, Daniel IR - Cossette D FIR - Tonascia, James IR - Tonascia J FIR - Belt, Patricia IR - Belt P FIR - Blackford, Amanda IR - Blackford A FIR - Collison, Betty IR - Collison B FIR - Dodge, John IR - Dodge J FIR - Donithan, Michele IR - Donithan M FIR - Ewing, Cathleen IR - Ewing C FIR - Jackson, Rosetta IR - Jackson R FIR - May, K Patrick IR - May KP FIR - Meinert, Jill IR - Meinert J FIR - Piantadosi, Steven IR - Piantadosi S FIR - Reyes, Girlie IR - Reyes G FIR - Shade, David IR - Shade D FIR - Smith, Michael IR - Smith M FIR - Sternberg, Alice L IR - Sternberg AL FIR - Wilson, Laura IR - Wilson L FIR - Wagoner, Annette IR - Wagoner A FIR - Wise, Robert IR - Wise R FIR - Yates, Katherine P IR - Yates KP FIR - Hakim, Rosemarie IR - Hakim R FIR - Punturieri, Antonello IR - Punturieri A FIR - Bamdad, Julie IR - Bamdad J FIR - Croxton, Thomas IR - Croxton T FIR - Deshler, Joanne IR - Deshler J FIR - McCord-Reynolds, Pamela IR - McCord-Reynolds P FIR - Stylianou, Mario IR - Stylianou M FIR - Weinmann, Gail IR - Weinmann G FIR - Bernard, Gordon IR - Bernard G FIR - Anderson, James IR - Anderson J FIR - Lo, Bernard IR - Lo B FIR - Ries, Andrew IR - Ries A FIR - Stoloff, Stuart IR - Stoloff S FIR - Thomashow, Byron IR - Thomashow B FIR - Tilley, Barbara IR - Tilley B FIR - Weiss, Kevin IR - Weiss K EDAT- 2015/12/15 06:00 MHDA- 2016/09/22 06:00 PMCR- 2016/12/01 CRDT- 2015/12/15 06:00 PHST- 2015/12/15 06:00 [entrez] PHST- 2015/12/15 06:00 [pubmed] PHST- 2016/09/22 06:00 [medline] PHST- 2016/12/01 00:00 [pmc-release] AID - 10.1513/AnnalsATS.201506-389OC [doi] PST - ppublish SO - Ann Am Thorac Soc. 2015 Dec;12(12):1796-804. doi: 10.1513/AnnalsATS.201506-389OC.