Alpha-1

Alpha-1 Antitrypsin Deficiency

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: 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.

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.

Collaborators

Publications

Articles published since 1994

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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Alpha-1 Antitrypsin Deficiency

research

PROJECTS

Collaborators

Publications

Alpha-1-antitrypsin deficiency

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