Brazilian Journal of Pulmonology

ISSN (on-line): 1806-3756 | ISSN (printed): 1806-3713

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Highlights of the Brazilian Thoracic Association Guidelines for Interstitial Lung Diseases

Destaques das Diretrizes de Doenças Pulmonares Intersticiais da Sociedade Brasileira de Pneumologia e Tisiologia

Bruno Guedes Baldi, Carlos Alberto de Castro Pereira, Adalberto Sperb Rubin, Alfredo Nicodemos da Cruz Santana, André Nathan Costa, Carlos Roberto Ribeiro Carvalho, Eduardo Algranti, Eduardo Mello de Capitani, Eduardo Pamplona Bethlem, Ester Nei Aparecida Martins Coletta, Jaquelina Sonoe Ota Arakaki, José Antônio Baddini Martinez, Jozélio Freire de Carvalho, Leila John Marques Steidle, Marcelo Jorge Jacó Rocha, Mariana Silva Lima, Maria Raquel Soares, Marlova Luzzi Caramori, Miguel Abidon Aidé, Rimarcs Gomes Ferreira, Ronaldo Adib Kairalla, Rudolf Krawczenko Feitoza de Oliveira, Sérgio Jezler, Sílvia Carla Sousa Rodrigues, Suzana Pinheiro Pimenta

J Bras Pneumol.2012;38(3):282-291

Abstract PDF PT PDF EN Portuguese Text

Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.

 


Keywords: Lung diseases, interstitial; Guidelines as topic; Brazil.

 


Doxycycline use in patients with lymphangioleiomyomatosis: safety and efficacy in metalloproteinase blockade

Doxiciclina em pacientes com linfangioleiomiomatose: segurança e eficácia no bloqueio de metaloproteinases

Suzana Pinheiro Pimenta, Bruno Guedes Baldi, Milena Marques Pagliarelli Acencio, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho

J Bras Pneumol.2011;37(4):424-430

Abstract PDF PT PDF EN Portuguese Text

Objective: To determine the frequency of near-fatal asthma in a group of severe asthma patients, as well as the clinical characteristics and prognosis of these patients within a one-year follow-up period. Methods: A prospective study involving 731 low-income patients with severe asthma treated at a referral outpatient clinic located in the city of Salvador, Brazil. The patients were submitted to spirometry at admission, received medications for asthma, and were monitored regarding the frequency of asthma exacerbations during the follow-up period. A subsample of 511 patients also completed questionnaires regarding asthma symptoms and asthma-related quality of life. Results: Of the 731 patients studied, 563 (77%) were female. The median age was 47 years, and 12% were illiterate. Most of the patients had rhinitis, and 70 patients (10%) reported near-fatal asthma prior to admission. Of these 70 patients, 41 (59%) reported having been intubated previously. The patients reporting a history of near-fatal asthma at admission were more likely to have asthma exacerbations during the follow-up period and to respond poorly to therapy than were those not reporting such a history. At the end of the follow-up period, the scores on the two questionnaires were similar between the two groups of patients. Conclusions: The frequency of near-fatal asthma was high in this group of low-income patients with severe asthma. The patients with a history of near-fatal asthma had a worse prognosis than did those without such a history, although both groups had received the same kind of treatment. Curiously, the intensity of symptoms and the quality of life at the end of the study were similar between the two groups.

 


Keywords: Asthma/prevention and control; Asthma/complications; Quality of life; Prognosis.

 


Doxycycline use in patients with lymphangioleiomyomatosis: biomarkers and pulmonary function response

Doxiciclina em pacientes com linfangioleiomiomatose: biomarcadores e resposta funcional pulmonar

Suzana Pinheiro Pimenta, Bruno Guedes Baldi, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho

J Bras Pneumol.2013;39(1):5-15

Abstract PDF PT PDF EN Appendix

Objective: To assess blockade of matrix metalloproteinase (MMP)-2 and MMP-9, as well as the variation in FEV1, in patients with lymphangioleiomyomatosis (LAM) treated with doxycycline (a known MMP inhibitor) for 12 months. Methods: An open-label, single-arm, interventional clinical trial in which LAM patients received doxycycline (100 mg/day) for 12 months. Patients underwent full pulmonary function testing, a six-minute walk test, and quality of life assessment, as well as blood and urine sampling for quantification of MMP-2, MMP-9, and VEGF-D levels-at baseline, as well as at 6 and 12 months after the initiation of doxycycline. Results: Thirty-one LAM patients received doxycycline for 12 months. Although there was effective blockade of urinary MMP-9 and serum MMP-2 after treatment, there were no significant differences between pre‑and post-doxycycline serum levels of MMP-9 and VEGF-D. On the basis of their response to doxycycline (as determined by the variation in FEV1), the patients were divided into two groups: the doxycycline-responder (doxy-R) group (n = 13); and the doxycycline-nonresponder (doxy-NR) group (n = 18). The patients with mild spirometric abnormalities responded better to doxycycline. The most common side effects were mild epigastric pain, nausea, and diarrhea. Conclusions: In patients with LAM, doxycycline treatment results in effective MMP blockade, as well as in improved lung function and quality of life in those with less severe disease. However, these benefits do not seem to be related to the MMP blockade, raising the hypothesis that there is a different mechanism of action. (Brazilian Registry of Clinical Trials - ReBEC; identification number RBR-6g8yz9 [http://www.ensaiosclinicos.gov.br])

 


Keywords: Lymphangioleiomyomatosis; Doxycycline; Matrix metalloproteinases; Respiratory function tests.

 


Evolution of pulmonary function after treatment with goserelin in patients with lymphangioleiomyomatosis

Evolução da função pulmonar após tratamento com goserelina em pacientes com linfangioleiomiomatose

Bruno Guedes Baldi, Pedro Medeiros Junior, Suzana Pinheiro Pimenta, Roberto Iglesias Lopes, Ronaldo Adib Kairalla, Carlos Roberto Ribeiro Carvalho

J Bras Pneumol.2011;37(3):375-379

Abstract PDF PT PDF EN Portuguese Text

In the atypical smooth muscle cells that are characteristic of lymphangioleiomyomatosis (LAM), there are estrogen and progesterone receptors. Therefore, anti-hormonal therapy, despite having produced controversial results, can be considered a treatment option. The objective of this retrospective study was to evaluate hormonal and spirometric data for nine women with LAM after one year of treatment with goserelin. The mean increase in FEV1 and FVC was 80 mL and 130 mL, respectively. There was effective blockage of the hormonal axis. It is still not possible to exclude a potential beneficial effect of the use of gonadotropin-releasing hormone analogues in LAM patients, which underscores the need for randomized trials.

 


Keywords: Lymphangioleiomyomatosis; Spirometry; Goserelin.

 


 

 


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