EPOC y déficit de alfaantitripsinaCOPD and alphaantitrypsin deficiency . de alfaantitripsina en España (variantes deficientes PiS y PiZ): prevalencia. A deficiência de αantitripsina é uma desordem genética de herança autossómica recessiva, tendo como fenótipo mais comum o inibidor de protease tipo ZZ. Alfa 1 antitripsina (AAT ou A1AT), também escrito α1 anti-tripsina (α1AT), é um inibidor de Alguma variação da deficiência de alfaantitripsina (DAAT) é tão freqüente quanto a fibrose cística, afetando um em cada indivíduos.
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La principal variante deficitaria es la PiZ. Alphaantitrypsin deficiency AATD is the main genetic factor related to the development of emphysema. This protein has numerous variants, some deeficincia which are clinically relevant because their anomalous conformation implies that they fail to reach the target organs as they are polymerized in the hepatocyte.
The main abnormal variant is PiZ. In a small percentage of individuals, the accumulation of Z polymers in the liver leads to the development of liver disease. The lack of AATD in the lung favors the development of emphysema, since the proteolytic effect of elastases — the main biological function of AATD — is not counteracted. The principal prognostic factor in these patients is forced expiratory volume in one second FEV 1which is affected mainly by exposure to tobacco smoke.
Thus, life expectancy in nonsmokers is similar to that in the general population aantitripsina lung function decreases faster in smokers than in other patients with chronic obstructive pulmonary disease COPD. The treatment of the lung disease is the same, although exogenous AATD augmentation is indicated when lung function deteriorates. COPD and alphaantitrypsin deficiency. The treatment of the lung disease is the same, although exogenous AATD augmentation is indicated when lung function deteriorates.
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Alfa 1 antitripsina
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EPOC y déficit de alfaantitripsina | Archivos de Bronconeumología
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Does alpha-1 antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary AAT deficiency?
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