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  1. Article ; Online: Characterization of the severity of dyspnea in patients with bronchiectasis: correlation with clinical, functional, and tomographic aspects.

    Nucci, Maria Cecília Nieves Maiorano de / Fernandes, Frederico Leon Arrabal / Salge, João Marcos / Stelmach, Rafael / Cukier, Alberto / Athanazio, Rodrigo

    Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia

    2020  Volume 46, Issue 5, Page(s) e20190162

    Abstract: Objective: To characterize a population of patients with bronchiectasis, correlating clinical, radiological, and functional aspects with the severity of dyspnea.: Methods: This was a cross-sectional study involving adult patients with HRCT-confirmed ... ...

    Abstract Objective: To characterize a population of patients with bronchiectasis, correlating clinical, radiological, and functional aspects with the severity of dyspnea.
    Methods: This was a cross-sectional study involving adult patients with HRCT-confirmed bronchiectasis, categorized according to the severity of dyspnea (as being mildly or severely symptomatic, on the basis of the modified Medical Research Council scale). We correlated the severity of dyspnea with clinical parameters, functional parameters (spirometry values, lung volumes, and DLCO), and CT parameters.
    Results: We evaluated 114 patients, 47 (41%) of whom were men. The median age (interquartile range) was 42 years (30-55 years). The most common form was idiopathic bronchiectasis. Of the 114 patients, 20 (17.5%) were colonized with Pseudomonas aeruginosa and 59 (51.8%) were under continuous treatment with macrolides. When we applied the Exacerbation in the previous year, FEV1, Age, Colonization, Extension, and Dyspnea score, the severity of dyspnea was categorized as moderate in 54 patients (47.4%), whereas it was categorized as mild in 50 (43.9%) when we applied the Bronchiectasis Severity Index. The most common lung function pattern was one of obstruction, seen in 95 patients (83.3%), and air trapping was seen in 77 patients (68.7%). The prevalence of an obstructive pattern on spirometry was higher among the patients with dyspnea that was more severe, and most functional parameters showed reasonable accuracy in discriminating between levels of dyspnea severity.
    Conclusions: Patients with bronchiectasis and dyspnea that was more severe had greater functional impairment. The measurement of lung volumes complemented the spirometry data. Because bronchiectasis is a complex, heterogeneous condition, a single variable does not seem to be sufficient to provide an overall characterization of the clinical condition.
    MeSH term(s) Adult ; Bronchiectasis/complications ; Bronchiectasis/diagnostic imaging ; Bronchiectasis/epidemiology ; Cross-Sectional Studies ; Dyspnea/etiology ; Humans ; Male ; Multidetector Computed Tomography ; Respiratory Function Tests ; Severity of Illness Index ; Spirometry ; Tomography, X-Ray Computed/methods
    Language Portuguese
    Publishing date 2020-06-15
    Publishing country Brazil
    Document type Journal Article
    ZDB-ID 2223157-2
    ISSN 1806-3756 ; 1806-3713
    ISSN (online) 1806-3756
    ISSN 1806-3713
    DOI 10.36416/1806-3756/e20190162
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: The patient profile of individuals with Alpha-1 antitrypsine gene mutations at a referral center in Brazil.

    Felisbino, Manuela Brisot / Fernandes, Frederico Leon Arrabal / Nucci, Maria Cecília Nieves Maiorano de / Pinto, Regina Maria de Carvalho / Pizzichini, Emilio / Cukier, Alberto

    Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia

    2018  Volume 44, Issue 5, Page(s) 383–389

    Abstract: Objective: The clinical, functional, radiological and genotypic descriptions of patients with an alpha-1 antitrypsin (A1AT) gene mutation in a referral center for COPD in Brazil.: Methods: A cross-sectional study of patients with an A1AT gene ... ...

    Abstract Objective: The clinical, functional, radiological and genotypic descriptions of patients with an alpha-1 antitrypsin (A1AT) gene mutation in a referral center for COPD in Brazil.
    Methods: A cross-sectional study of patients with an A1AT gene mutation compatible with deficiency. We evaluated the A1AT dosage and genotypic, demographic, clinical, tomographic, and functional characteristics of these patients.
    Results: Among the 43 patients suspected of A1AT deficiency (A1ATD), the disease was confirmed by genotyping in 27 of them. The A1AT median dosage was 45 mg/dL, and 4 patients (15%) had a normal dosage. Median age was 54, 63% of the patients were male, and the respiratory symptoms started at the age of 40. The median FEV1 was 1.37L (43% predicted). Tomographic emphysema was found in 77.8% of the individuals. The emphysema was panlobular in 76% of them and 48% had lower lobe predominance. The frequency of bronchiectasis was 52% and the frequency of bronchial thickening was 81.5%. The most common genotype was Pi*ZZ in 40.7% of participants. The other genotypes found were: Pi*SZ (18.5%), PiM1Z (14.8%), Pi*M1S (7.4%), Pi*M2Z (3.7%), Pi*M1I (3.7%), Pi*ZMnichinan (3.7%), Pi*M3Plowell (3.7%), and Pi*SF (3.7%). We did not find any significant difference in age, smoking load, FEV1, or the presence of bronchiectasis between the groups with a normal and a reduced A1AT dosage, neither for 1 nor 2-allele mutation for A1ATD.
    Conclusions: Our patients presented a high frequency of emphysema, bronchiectasis and bronchial thickening, and early-beginning respiratory symptoms. The most frequent genotype was Pi*ZZ. Heterozygous genotypes and normal levels of A1AT also manifested significant lung disease.
    MeSH term(s) Cross-Sectional Studies ; Female ; Genotype ; Humans ; Male ; Middle Aged ; Mutation/genetics ; Phenotype ; Respiratory Function Tests ; Tomography, X-Ray Computed ; alpha 1-Antitrypsin/genetics ; alpha 1-Antitrypsin Deficiency/diagnosis ; alpha 1-Antitrypsin Deficiency/genetics
    Chemical Substances alpha 1-Antitrypsin
    Language Portuguese
    Publishing date 2018-12-05
    Publishing country Brazil
    Document type Journal Article
    ZDB-ID 2223157-2
    ISSN 1806-3756 ; 1806-3713
    ISSN (online) 1806-3756
    ISSN 1806-3713
    DOI 10.1590/S1806-37562017000000420
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Recommendations for the pharmacological treatment of COPD: questions and answers.

    Fernandes, Frederico Leon Arrabal / Cukier, Alberto / Camelier, Aquiles Assunção / Fritscher, Carlos Cezar / Costa, Cláudia Henrique da / Pereira, Eanes Delgado Barros / Godoy, Irma / Cançado, José Eduardo Delfini / Romaldini, José Gustavo / Chatkin, Jose Miguel / Jardim, José Roberto / Rabahi, Marcelo Fouad / Nucci, Maria Cecília Nieves Maiorano de / Sales, Maria da Penha Uchoa / Castellano, Maria Vera Cruz de Oliveira / Aidé, Miguel Abidon / Teixeira, Paulo José Zimermann / Maciel, Renato / Corrêa, Ricardo de Amorim /
    Stirbulov, Roberto / Athanazio, Rodrigo Abensur / Russo, Rodrigo / Minamoto, Suzana Tanni / Lundgren, Fernando Luiz Cavalcanti

    Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia

    2018  Volume 43, Issue 4, Page(s) 290–301

    Abstract: The treatment of COPD has become increasingly effective. Measures that range from behavioral changes, reduction in exposure to risk factors, education about the disease and its course, rehabilitation, oxygen therapy, management of comorbidities, and ... ...

    Abstract The treatment of COPD has become increasingly effective. Measures that range from behavioral changes, reduction in exposure to risk factors, education about the disease and its course, rehabilitation, oxygen therapy, management of comorbidities, and surgical and pharmacological treatments to end-of-life care allow health professionals to provide a personalized and effective therapy. The pharmacological treatment of COPD is one of the cornerstones of COPD management, and there have been many advances in this area in recent years. Given the greater availability of drugs and therapeutic combinations, it has become increasingly challenging to know the indications for, limitations of, and potential risks and benefits of each treatment modality. In order to critically evaluate recent evidence and systematize the major questions regarding the pharmacological treatment of COPD, 24 specialists from all over Brazil gathered to develop the present recommendations. A visual guide was developed for the classification and treatment of COPD, both of which were adapted to fit the situation in Brazil. Ten questions were selected on the basis of their relevance in clinical practice. They address the classification, definitions, treatment, and evidence available for each drug or drug combination. Each question was answered by two specialists, and then the answers were consolidated in two phases: review and consensus by all participants. The questions answered are practical questions and help select from among the many options the best treatment for each patient and his/her peculiarities.
    MeSH term(s) Disease Management ; Humans ; Pulmonary Disease, Chronic Obstructive/drug therapy ; Severity of Illness Index ; Surveys and Questionnaires
    Language Portuguese
    Publishing date 2018-01-24
    Publishing country Brazil
    Document type Journal Article ; Review
    ZDB-ID 2223157-2
    ISSN 1806-3756 ; 1806-3713
    ISSN (online) 1806-3756
    ISSN 1806-3713
    DOI 10.1590/S1806-37562017000000153
    Database MEDical Literature Analysis and Retrieval System OnLINE

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