[Profiles of cases included in the Spanish registry of patients with alpha-1 antitrypsin deficiency].

نویسندگان

  • B Lara Gallego
  • S Castillo Carullón
  • M T Martínez Martínez
  • M García Reymundo
  • M Miravitlles
چکیده

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Development and results of the Spanish registry of patients with alpha-1-antitrypsin deficiency

The Spanish registry of alpha-1 antitrypsin deficiency was founded in 1993 and became a member of the International Registry (AIR) in 1999. We describe the updating process following its incorporation into AIR and compare the data collected in the first period (1993-1999) and the second period (1999-2005), during which time patients were included exclusively by internet. The registry included 3...

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Prevalence of Alpha-1 Antitrypsin (A1AT) Deficiency among Patients with COPD in Kerman, Iran

Background: One of the genetic risk factors for chronic obstructive pulmonary disease (COPD) is deficiency of Alpha-1 Antitrypsin (A1AT). There is no exact statistics about the prevalence of this disease in different regions of Iran. The present study aimed to determine the prevalence of alpha-1 antitrypsin (A1AT) deficiency in COPD patients in Kerman, Iran. Metho...

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Assessment of Alpha-1 Antitrypsin Deficiency in Patients with Severe Chronic Obstructive Pulmonary Disease

Background and Aims: Chronic obstructive pulmonary disease (COPD) is a kind of pulmonary diseases characterized by chronic obstruction of lung that is in the form of a diffuse narrowing of airways resulting in air flow resistance. Alpha-1 antitrypsin (AAT) deficiency is genetically relatively common risk factor in patients with COPD throughout the world and the exact cause of its prevalence is ...

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Determination of Alpha 1-Antitrypsin Phenotypes and Genotypes in Iranian Patients

Alpha 1-antitrypsin (AAT) or alpha 1-protease inhibitor (PI) is the principal inhibitor of proteolytic enzyme in serum. Its phenotypic variability has been reported to be associated with liver, lung diseases and rheumatoid arthritis in humans. There is much documentation about high risk phenotypes of PI in some regions of the world, however, there are no reliable reports on these phenotypes and...

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Activity of the alpha-1 antitrypsin deficiency registry in Belgium.

A Belgian alpha-1-antitrypsin (AAT) deficiency registry has been established in 2003. Currently 55 patients are included. At the same time, a working group has been set up for publishing national guidelines. In 2014, several Belgian patients founded Alpha-1 Global. We hope that the integrated activities of all the stakeholders involved in AAT deficiency will permit a high quality care for all p...

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عنوان ژورنال:
  • Anales de pediatria

دوره 84 4  شماره 

صفحات  -

تاریخ انتشار 2016