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 deficinciq the main genetic factor related to the development of emphysema. This protein anritripsina numerous variants, some of 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 while 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 alf of the lung disease is the same, although exogenous AATD augmentation is indicated when lung function deteriorates.
The electrophoretic alphaglobulin pattern of serum in alphaantitrypsin deficiency.
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EPOC y déficit de alfaantitripsina | Archivos de Bronconeumología
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Relationship of chronic sputum expectoration to physiologic, radiological and health status characteristics in alphaantitrypsin deficiency PiZ.
Deficiencia de alfa-1 antitripsina
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Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: The effect of augmentation therapy on bronchial inflammation in alphaantitrypsin deficiency. Does alpha-1 antitrypsin augmentation therapy slow the annual decline in FEV1 in patients with severe hereditary AAT deficiency?
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Exploring the optimum approach to the use of CT densitometry in a randomised placebo-controlled study of augmentation therapy in alphaantitrypsin deficiency.
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Alfa 1 antitripsina – Wikipédia, a enciclopédia livre
Emphysema due to alpha-1 antitrypsin deficiency: De la Roza, S. Development and results of the Spanish registry of patients with alpha-1 antitrypsin deficiency. Ongoing research in Europe: Eur Respir J, 29pp.
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