Comparing patients with ZZ versus SZ alpha-1 antitrypsin deficiency: Findings from AlphaNet's disease management program

Radmila Choate; David M. Mannino; Kristen E. Holm; Robert A. Sandhaus

doi:10.15326/JCOPDF.6.1.2018.0134

Comparing patients with ZZ versus SZ alpha-1 antitrypsin deficiency: Findings from AlphaNet's disease management program

Radmila Choate, David M. Mannino, Kristen E. Holm, Robert A. Sandhaus

Research output: Contribution to journal › Article › peer-review

13 Scopus citations

Abstract

Background: The aim of this study was to examine differences in demographic, health, and behavioral characteristics in individuals with ZZ and SZ genotypes of alpha-1 antitrypsin deficiency (AATD) within AlphaNet's Disease Management and Prevention Program (ADMAPP). Methods: Self-reported data from 3535 patients with AATD, including 3031 (85.7%) patients with ZZ, ZNull, and NullNull genotypes (referred to here as ZZ), and 504 (14.3%) with the SZ genotype were analyzed using t-tests, ANOVAs, and Chi-squared tests. Results: The average age of the cohort was 56.3±10.6 years. The majority of respondents were male (51.2%), white (98.2%) and married (65.2%). SZs reported having more frequent exacerbations (p < 0.001) and hospitalizations (p=0.012) than ZZs. A higher proportion of SZs than ZZs had been diagnosed with high blood pressure, diabetes, congestive heart failure, and other comorbid conditions. SZs were more likely than ZZs to report "poor" health (p=0.005). Over a third (38.4%) of SZs do not exercise compared to 27.1% of ZZs (p < 0.001). A greater proportion of SZs compared to ZZs view themselves as being overweight (p < 0.001) or "out of shape" (p=0.001). A higher proportion of SZs than ZZs reported any history of smoking and current smoking (p < 0.001). Conclusions: In patients with AATD and lung disease participating in a disease management program, a higher proportion of SZs than ZZs report exacerbations, comorbidities, and overall poor health, as well as unhealthy behaviors such as lack of exercise and current smoking. Future work should consider the extent to which genotype-specific health promotion interventions would be useful.

Original language	English
Pages (from-to)	29-39
Number of pages	11
Journal	Chronic Obstructive Pulmonary Diseases
Volume	6
Issue number	1
DOIs	https://doi.org/10.15326/JCOPDF.6.1.2018.0134
State	Published - 2019

Bibliographical note

Funding Information:
Abbreviations: alpha-1 antitrypsin deficiency, AATD; AlphaNet’s Disease Management and Prevention Program, ADMAPP; protease inhibitor, PI; negative binomial, NB; Charlson Comorbidity Index, CCI; zero-inflated negative binomial, ZINB; Big Fat Referene Guide, BFRG; Akike information criterion, AIC; incident rate ratio, IRR; confidence interval, CI; standard deviation, SD; gastroesophageal reflux disease, GERD Funding Support: This study was funded by an unrestricted research grant from AlphaNet. Date of Acceptance: July 16, 2018 Citation: Choate R, Mannino DM, Holm KE, Sandhaus RA. Comparing patients with ZZ versus SZ alpha-1 antitrypsin deficiency: findings from AlphaNet’s disease management program. Chronic Obstr Pulm Dis. 2019;6(1):29-39. doi: https://doi.org/10.15326/ jcopdf.6.1.2018.0134

Publisher Copyright:
© 2019.

Keywords

ADMAPP
Alpha-1 antitrypsin deficiency
AlphaNet Disease Management and Prevention Program
Genotype
SZ
ZZ

ASJC Scopus subject areas

Pulmonary and Respiratory Medicine

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.15326/JCOPDF.6.1.2018.0134

Cite this

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title = "Comparing patients with ZZ versus SZ alpha-1 antitrypsin deficiency: Findings from AlphaNet's disease management program",

abstract = "Background: The aim of this study was to examine differences in demographic, health, and behavioral characteristics in individuals with ZZ and SZ genotypes of alpha-1 antitrypsin deficiency (AATD) within AlphaNet's Disease Management and Prevention Program (ADMAPP). Methods: Self-reported data from 3535 patients with AATD, including 3031 (85.7%) patients with ZZ, ZNull, and NullNull genotypes (referred to here as ZZ), and 504 (14.3%) with the SZ genotype were analyzed using t-tests, ANOVAs, and Chi-squared tests. Results: The average age of the cohort was 56.3±10.6 years. The majority of respondents were male (51.2%), white (98.2%) and married (65.2%). SZs reported having more frequent exacerbations (p < 0.001) and hospitalizations (p=0.012) than ZZs. A higher proportion of SZs than ZZs had been diagnosed with high blood pressure, diabetes, congestive heart failure, and other comorbid conditions. SZs were more likely than ZZs to report {"}poor{"} health (p=0.005). Over a third (38.4%) of SZs do not exercise compared to 27.1% of ZZs (p < 0.001). A greater proportion of SZs compared to ZZs view themselves as being overweight (p < 0.001) or {"}out of shape{"} (p=0.001). A higher proportion of SZs than ZZs reported any history of smoking and current smoking (p < 0.001). Conclusions: In patients with AATD and lung disease participating in a disease management program, a higher proportion of SZs than ZZs report exacerbations, comorbidities, and overall poor health, as well as unhealthy behaviors such as lack of exercise and current smoking. Future work should consider the extent to which genotype-specific health promotion interventions would be useful.",

keywords = "ADMAPP, Alpha-1 antitrypsin deficiency, AlphaNet Disease Management and Prevention Program, Genotype, SZ, ZZ",

author = "Radmila Choate and Mannino, {David M.} and Holm, {Kristen E.} and Sandhaus, {Robert A.}",

note = "Funding Information: Abbreviations: alpha-1 antitrypsin deficiency, AATD; AlphaNet{\textquoteright}s Disease Management and Prevention Program, ADMAPP; protease inhibitor, PI; negative binomial, NB; Charlson Comorbidity Index, CCI; zero-inflated negative binomial, ZINB; Big Fat Referene Guide, BFRG; Akike information criterion, AIC; incident rate ratio, IRR; confidence interval, CI; standard deviation, SD; gastroesophageal reflux disease, GERD Funding Support: This study was funded by an unrestricted research grant from AlphaNet. Date of Acceptance: July 16, 2018 Citation: Choate R, Mannino DM, Holm KE, Sandhaus RA. Comparing patients with ZZ versus SZ alpha-1 antitrypsin deficiency: findings from AlphaNet{\textquoteright}s disease management program. Chronic Obstr Pulm Dis. 2019;6(1):29-39. doi: https://doi.org/10.15326/ jcopdf.6.1.2018.0134 Publisher Copyright: {\textcopyright} 2019.",

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doi = "10.15326/JCOPDF.6.1.2018.0134",

language = "English",

volume = "6",

pages = "29--39",

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T1 - Comparing patients with ZZ versus SZ alpha-1 antitrypsin deficiency

T2 - Findings from AlphaNet's disease management program

AU - Choate, Radmila

AU - Mannino, David M.

AU - Holm, Kristen E.

AU - Sandhaus, Robert A.

N1 - Funding Information: Abbreviations: alpha-1 antitrypsin deficiency, AATD; AlphaNet’s Disease Management and Prevention Program, ADMAPP; protease inhibitor, PI; negative binomial, NB; Charlson Comorbidity Index, CCI; zero-inflated negative binomial, ZINB; Big Fat Referene Guide, BFRG; Akike information criterion, AIC; incident rate ratio, IRR; confidence interval, CI; standard deviation, SD; gastroesophageal reflux disease, GERD Funding Support: This study was funded by an unrestricted research grant from AlphaNet. Date of Acceptance: July 16, 2018 Citation: Choate R, Mannino DM, Holm KE, Sandhaus RA. Comparing patients with ZZ versus SZ alpha-1 antitrypsin deficiency: findings from AlphaNet’s disease management program. Chronic Obstr Pulm Dis. 2019;6(1):29-39. doi: https://doi.org/10.15326/ jcopdf.6.1.2018.0134 Publisher Copyright: © 2019.

PY - 2019

Y1 - 2019

N2 - Background: The aim of this study was to examine differences in demographic, health, and behavioral characteristics in individuals with ZZ and SZ genotypes of alpha-1 antitrypsin deficiency (AATD) within AlphaNet's Disease Management and Prevention Program (ADMAPP). Methods: Self-reported data from 3535 patients with AATD, including 3031 (85.7%) patients with ZZ, ZNull, and NullNull genotypes (referred to here as ZZ), and 504 (14.3%) with the SZ genotype were analyzed using t-tests, ANOVAs, and Chi-squared tests. Results: The average age of the cohort was 56.3±10.6 years. The majority of respondents were male (51.2%), white (98.2%) and married (65.2%). SZs reported having more frequent exacerbations (p < 0.001) and hospitalizations (p=0.012) than ZZs. A higher proportion of SZs than ZZs had been diagnosed with high blood pressure, diabetes, congestive heart failure, and other comorbid conditions. SZs were more likely than ZZs to report "poor" health (p=0.005). Over a third (38.4%) of SZs do not exercise compared to 27.1% of ZZs (p < 0.001). A greater proportion of SZs compared to ZZs view themselves as being overweight (p < 0.001) or "out of shape" (p=0.001). A higher proportion of SZs than ZZs reported any history of smoking and current smoking (p < 0.001). Conclusions: In patients with AATD and lung disease participating in a disease management program, a higher proportion of SZs than ZZs report exacerbations, comorbidities, and overall poor health, as well as unhealthy behaviors such as lack of exercise and current smoking. Future work should consider the extent to which genotype-specific health promotion interventions would be useful.

AB - Background: The aim of this study was to examine differences in demographic, health, and behavioral characteristics in individuals with ZZ and SZ genotypes of alpha-1 antitrypsin deficiency (AATD) within AlphaNet's Disease Management and Prevention Program (ADMAPP). Methods: Self-reported data from 3535 patients with AATD, including 3031 (85.7%) patients with ZZ, ZNull, and NullNull genotypes (referred to here as ZZ), and 504 (14.3%) with the SZ genotype were analyzed using t-tests, ANOVAs, and Chi-squared tests. Results: The average age of the cohort was 56.3±10.6 years. The majority of respondents were male (51.2%), white (98.2%) and married (65.2%). SZs reported having more frequent exacerbations (p < 0.001) and hospitalizations (p=0.012) than ZZs. A higher proportion of SZs than ZZs had been diagnosed with high blood pressure, diabetes, congestive heart failure, and other comorbid conditions. SZs were more likely than ZZs to report "poor" health (p=0.005). Over a third (38.4%) of SZs do not exercise compared to 27.1% of ZZs (p < 0.001). A greater proportion of SZs compared to ZZs view themselves as being overweight (p < 0.001) or "out of shape" (p=0.001). A higher proportion of SZs than ZZs reported any history of smoking and current smoking (p < 0.001). Conclusions: In patients with AATD and lung disease participating in a disease management program, a higher proportion of SZs than ZZs report exacerbations, comorbidities, and overall poor health, as well as unhealthy behaviors such as lack of exercise and current smoking. Future work should consider the extent to which genotype-specific health promotion interventions would be useful.

KW - ADMAPP

KW - Alpha-1 antitrypsin deficiency

KW - AlphaNet Disease Management and Prevention Program

KW - Genotype

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KW - ZZ

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Comparing patients with ZZ versus SZ alpha-1 antitrypsin deficiency: Findings from AlphaNet's disease management program

Abstract

Bibliographical note

Keywords

ASJC Scopus subject areas

UN SDGs

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