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Titolo:
Exhaled nitric oxide in patients with PiZZ Phenotype-related alpha 1-anti-trypsin deficiency
Autore:
Malerba, M; Clini, E; Cremona, G; Radaeli, A; Bianchi, L; Corda, L; Pini, L; Ricclardolo, F; Grassi, V; Ambrosino, N;
Indirizzi:
Fdn S Maugeri, IRCCS, Div Pneumol, Lung Funct Unit,Sci Inst Gussago, I-25064 Gussago, BS, Italy Fdn S Maugeri Gussago BS Italy I-25064 ussago, I-25064 Gussago, BS, Italy Univ Brescia, Inst Internal Med, Brescia, Italy Univ Brescia Brescia Italy v Brescia, Inst Internal Med, Brescia, Italy San Raffaele Sci Inst, Resp Med Unit, I-20132 Milan, Italy San Raffaele Sci Inst Milan Italy I-20132 Med Unit, I-20132 Milan, Italy Osped Riuniti Bergamo, Div Pulm, I-24100 Bergamo, Italy Osped Riuniti Bergamo Bergamo Italy I-24100 Pulm, I-24100 Bergamo, Italy
Titolo Testata:
RESPIRATORY MEDICINE
fascicolo: 6, volume: 95, anno: 2001,
pagine: 520 - 525
SICI:
0954-6111(200106)95:6<520:ENOIPW>2.0.ZU;2-E
Fonte:
ISI
Lingua:
ENG
Soggetto:
OBSTRUCTIVE PULMONARY-DISEASE; ALPHA-1-ANTITRYPSIN DEFICIENCY; LUNG-DISEASE; AIR; SYNTHASE; ASTHMA; COPD; PIG;
Keywords:
chronic airway obstruction; bronchial hyper-responsiveness; chemiluminescence;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
33
Recensione:
Indirizzi per estratti:
Indirizzo: Clini, E Fdn S Maugeri, IRCCS, Div Pneumol, Lung Funct Unit,Sci Inst Gussago, Via Pinidolo 23, I-25064 Gussago, BS, Italy Fdn S Maugeri Via Pinidolo 23 Gussago BS Italy I-25064 BS, Italy
Citazione:
M. Malerba et al., "Exhaled nitric oxide in patients with PiZZ Phenotype-related alpha 1-anti-trypsin deficiency", RESP MED, 95(6), 2001, pp. 520-525

Abstract

There is no report of exhaled NO (eNO) in subjects with different phenotypes of alpha (1)-anti-trypsin (AAT) deficiency. Exhaled nitric oxide was evaluated by means of single-breath chemiluminescence analysis (fractional exhaled concentration at the plateau level [p1FE(No)]) in 40 patients with AAT deficiency. Patients were divided according to the protease inhibitor (Pi) phenotype: PiMZ/MS, n = 25; PiSZ n = 6; PiZZ,n = 9. Nineteen healthy subjects served as controls. Levels of eNO in PiZZpatients were also compared with those of subjects, without AAT deficiency(PiMM), matched for diagnosis, sex, age, smoking habit and forced expiratory volume in 1 sec (FEV1). In AAT deficiency subjects airway hyper-responsiveness to methacholine (PD20 FEV1) was also assessed.p1FE(No) was significantly lower in the PiZZ group (4.5 +/- 1.4 ppb) than in matched PiMM subjects (8.2 +/- 3.8 ppb), in healthy controls (9.3 +/- 2.8 ppb) and in patients of other phenotypes. Dynamic lung volumes and DLCO were significantly lower in PiZZ than in other AAT-deficient patients. Bronchial hyper-responsiveness was not different among AAT phenotypes. These results suggest that eNO may be significantly reduced in PiZZ as compared to healthy control subjects and to AAT subjects with other phenotypes, independent of the level of airway obstruction. Whether, at least potentially, eNO may be considered as an early marker of lung involvement in AAT deficiency must be confirmed with studies on larger number of subjects.

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Documento generato il 22/02/20 alle ore 11:53:21