Alpha-1

research

PROJECTS

Hypothesis: Antibiotic requiring airway infections worsens emphysem and FEV1 in patients homozygotic for Alpha-1-antitrypsin deficiency. Investigated by looking at yearly reductions in FEV1 and lungdensity in patients having either 0, 1, 2 or 3+ antibiotic requiring lunginfections per year.

Hypothesis: Living in a large city worsens emphysem and FEV in patients homozygotic for Alfa-1-antitrypsin deficiency. Investigated by looking into yearly reductions in FEV1 and lungdensity in patients living in urbanized vs. rural areas.

Hypothesis: Having a fireplace lightened up at least once a week worsens emphysem and FEV1 in patients homozygotic for Alfa-1-antitrypsin deficiency. Investigated by looking at yearly reductions in FEV1 and lung density in patients using their fireplace weekly versus patients who doesn’t.

Collaborators

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Publications

Articles published since 1994

Clinical considerations in individuals with α 1-antitrypsin PI*SZ genotype

Published June 2020 in European Respiratory Journal

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Protocol for the EARCO Registry: a pan-European observational study in patients with α 1-antitrypsin deficiency

Published March 2020 in ERJ Open Research

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Efficacy and safety of inhaled α1-antitrypsin in patients with severe α1-antitrypsin deficiency and frequent exacerbations of COPD

Published November 2019 in European Respiratory Journal

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Long-term efficacy and safety of α1 proteinase inhibitor treatment for emphysema caused by severe α1 antitrypsin deficiency: an open-label extension trial (RAPID-OLE)

Published January 2017 in The Lancet Respiratory Medicine

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Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial

Published July 2015 in The Lancet

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Short-term reproducibility of computed tomography-based lung density measurements in alpha-1 antitrypsin deficiency and smokers with emphysema

Published July 2004 in Acta Radiologica

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Alpha1-antitrypsin deficiency. 1: epidemiology of alpha1-antitrypsin deficiency

Published February 2004 in Thorax

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Attitudes toward an unsolicited approach in relation to status of genetic disease: exemplified by alpha(1)-antitrypsin deficiency

Published September 2000 in American Journal of Medical Genetics

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Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) alpha1-AT study group

Published October 1997 in The European Respiratory Journal

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Body mass index and mortality in patients with severe alpha 1-antitrypsin deficiency

Published February 1997 in Respiratory Medicine

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Decline in FEV1 among patients with severe hereditary alpha 1-antitrypsin deficiency type PiZ

Published December 1995 in American Journal of Respiratory and Critical Care Medicine

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Survival in relation to lung function and smoking cessation in patients with severe hereditary alpha 1-antitrypsin deficiency

Published February 1995 in American Journal of Respiratory and Critical Care Medicine

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Airways obstruction and two year survival in patients with severe alpha 1-antitrypsin deficiency

Published November 1994 in European Respiratory Journal

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Survival of patients with severe alpha 1-antitrypsin deficiency with special reference to non-index cases

Published July 1994 in Thorax

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research

PROJECTS

Collaborators

Publications

Clinical considerations in individuals with α 1-antitrypsin PI*SZ genotype

Protocol for the EARCO Registry: a pan-European observational study in patients with α 1-antitrypsin deficiency

Efficacy and safety of inhaled α1-antitrypsin in patients with severe α1-antitrypsin deficiency and frequent exacerbations of COPD

Long-term efficacy and safety of α1 proteinase inhibitor treatment for emphysema caused by severe α1 antitrypsin deficiency: an open-label extension trial (RAPID-OLE)

Intravenous augmentation treatment and lung density in severe α1 antitrypsin deficiency (RAPID): a randomised, double-blind, placebo-controlled trial

Prognosis of patients with alpha1-antitrypsine deficiency on long-term oxygen therapy

Randomised controlled trial for emphysema with a selective agonist of the γ-type retinoic acid receptor

Alpha1-antitrypsin deficiency: current perspective on research, diagnosis, and management

Short-term reproducibility of computed tomography-based lung density measurements in alpha-1 antitrypsin deficiency and smokers with emphysema

Alpha1-antitrypsin deficiency. 1: epidemiology of alpha1-antitrypsin deficiency

Attitudes toward an unsolicited approach in relation to status of genetic disease: exemplified by alpha(1)-antitrypsin deficiency

Risk of hospital admission for obstructive pulmonary disease in alpha(1)-antitrypsin heterozygotes of phenotype PiMZ

A randomized clinical trial of alpha(1)-antitrypsin augmentation therapy

Transmitting genetic risk information in families: attitudes about disclosing the identity of relatives

Clinical features and prognosis of life time non-smokers with severe alpha 1-antitrypsin deficiency

Intermediate alpha 1-antitrypsin deficiency PiSZ: a risk factor for pulmonary emphysema?

Does alpha1-antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary alpha1-antitrypsin deficiency? Wissenschaftliche Arbeitsgemeinschaft zur Therapie von Lungenerkrankungen (WATL) alpha1-AT study group

Body mass index and mortality in patients with severe alpha 1-antitrypsin deficiency

Decline in FEV1 among patients with severe hereditary alpha 1-antitrypsin deficiency type PiZ

Survival in relation to lung function and smoking cessation in patients with severe hereditary alpha 1-antitrypsin deficiency

Airways obstruction and two year survival in patients with severe alpha 1-antitrypsin deficiency

Survival of patients with severe alpha 1-antitrypsin deficiency with special reference to non-index cases