Investigació Aplicada en Malalties Respiratòries

Research Lines

Alpha-1-antitrypsin deficiency (AATD)

  • To enroll patients in the Spanish Registry of AATD
  • To collaborate with the initiatives of the Alpha One International registry (A.I.R.)
  • To identify the best regimens of administration of AAT based on PK/PD models
  • To identify the role of AAT as a protective antiinflammatory mediator in exacerbations of Chronic Obstructive Pulmonary Disease (COPD)
Projects in Progress
  • Development of the Spanish registry of patients with AATD
  • Detection and identification of rare variants of AAT
  • Role of AAT in exacerbations of COPD
Milestones achieved
  • Consolidation of the Spanish registry of AATD
  • Collaboration with A.I.R.
  • Identification of some rare mutations of AAT (Ybarcelona)
  • Development of a model to estimate the best therapeutic regimen of intravenous AAT for augmentation therapy
Future challenges
  • To ascertain the role of AAT as antiinflammatory mediator in smoking-related COPD
  • To participate in new clinical trials of inhaled AAT and new drugs for patients with the deficiency


  • Augmentation therapy for emphysema due to alpha-1-antitrypsin deficiency

    Gema Tirado-Conde, Beatriz Lara, Marc Miravitlles.

    Therapeutic Advances in Respiratory Diseases 2008; 2 (1): 13-21.

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  • Laboratory testing of individuals with severe AAT deficiency in three European centres.

    Miravitlles M, Herr C, Ferrarotti I, Jardi R, Rodriguez-Frias F, Luisetti M, Bals R

    Eur Respir J 2010; 35: 960-968.

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