000			nab a22 7a 4500
999			_c17094 _d17094
003			PC17094
005			20230413062748.0
008			221125b xxu||||| |||| 00| 0 eng d
040			_cH12O
041			_aeng
100			_91308 _aMartínez Martínez, María Teresa _eNeumología
245	0	0	_aIndications for active case searches and intravenous alpha-1 antitrypsin treatment for patients with alpha-1 antitrypsin deficiency chronic pulmonary obstructive disease: an update. _h[artículo]
260			_bArchivos de bronconeumología, _c2015
300			_a51(4):185-92.
500			_aFormato Vancouver: Casas F, Blanco I, Martínez MT, Bustamante A, Miravitlles M, Cadenas S et al. Indications for active case searches and intravenous alpha-1 antitrypsin treatment for patients with alpha-1 antitrypsin deficiency chronic pulmonary obstructive disease: an update. Arch Bronconeumol. 2015 Apr;51(4):185-92.
501			_a PMID: 25027067
504			_aContiene 111 referencias
520			_aThe effect of hereditary alpha-1 antitrypsin (AAT) deficiency can manifest clinically in the form of chronic obstructive pulmonary disease (COPD). AAT deficiency (AATD) is defined as a serum concentration lower than 35% of the expected mean value or 50 mg/dl (determined by nephelometry). It is associated in over 95% of cases with Pi*ZZ genotypes, and much less frequently with other genotypes resulting from combinations of Z, S, rare and null alleles. A systematic qualitative review was made of 107 articles, focusing mainly on an active search for AATD in COPD patients and intravenous (iv) treatment with AAT. On the basis of this review, the consultant committee of the Spanish Registry of Patients with AATD recommends that all COPD patients be screened for AATD with the determination of AAT serum concentrations, and when these are low, the evaluation must be completed with phenotyping and, on occasions, genotyping. Patients with severe AATD COPD should receive the pharmacological and non-pharmacological treatment recommended in the COPD guidelines. There is enough evidence from large observational studies and randomized placebo-controlled clinical trials to show that the administration of iv AAT reduces mortality and slows the progression of emphysema, hence its indication in selected cases that meet the inclusion criteria stipulated in international guidelines. The administration of periodic infusions of AAT is the only specific treatment for delaying the progression of emphysema associated with AATD.
710			_988 _aServicio de Neumología
856			_uhttp://pc-h12o-es.m-hdoct.a17.csinet.es/pdf/pc/1/pc17094.pdf _ySolicitar documento
942			_2ddc _cART _n0