Brazilian Journal of Pulmonology

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

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Determination of alpha 1-antitrypsin levels and of the presence of S and Z alleles in a population of patients with chronic respiratory symptoms

Avaliação da concentração de alfa 1-antitripsina e da presença dos alelos S e Z em uma população de indivíduos sintomáticos respiratórios crônicos

Heliane Guerra Serra, Carmen Sílvia Bertuzzo, Mônica Corso Pereira, Cláudio Lúcio Rossi, Walter Pinto Júnior, Ilma Aparecida Paschoal

J Bras Pneumol.2008;34(12):1019-1025

Abstract PDF PT PDF EN Portuguese Text

Abstract Objective: To determine the levels of alpha-1 antitrypsin (AAT) and the presence of S and Z alleles in patients with chronic respiratory symptoms. Methods: Patients with chronic cough and dyspnea were submitted to clinical evaluation, pulmonary function tests, high-resolution computed tomography, nephelometric determination of AAT and determination of S and Z alleles by polymerase chain reaction. Smoking and AAT levels were considered the dependent variables. Results: Of the 89 patients included in the study, 44 were female. The mean age was 51.3 ± 18.2 years. The S and Z alleles were detected in 33.3% and 5.7%, respectively, and the gene frequency was 0.16 and 0.028, respectively. Two patients were SZ heterozygotes (AAT levels ≤ 89 mg/dL). The patients were divided into groups based on AAT level: ≤ 89 mg/­dL (deficiency, no group); 90-140 mg/dL (intermediate, Group 1, n = 30); and ≥ 141 mg/dL (normal, Group 2, n = 57). The frequency of smokers was the same in both groups, although tobacco intake was greater in Group 2. The S allele was present in 13 and 14 patients in Groups 1 and 2, respectively, whereas the Z allele was present in 2 and 1 patient in the same groups. There was no difference in the results of pulmonary function tests or in the frequency of bronchiectasis or emphysema between the two groups. Spirometric values and AAT levels were similar in smokers and nonsmokers. Bronchiectasis was more common in nonsmokers, and emphysema was more common in smokers. Conclusions: Thirty patients presented AAT levels lower than the mean values found in patients with the MM or MS genotype, and this fact could not be explained by an increased frequency of S and Z alleles.

 


Keywords: Alpha 1-antitrypsin; Emphysema: Lung diseases; Alleles.

 


Volumetric capnography as a noninvasive diagnostic procedure in acute pulmonary thromboembolism

Capnografia volumétrica como auxílio diagnóstico não-invasivo no tromboembolismo pulmonar agudo

Marcos Mello Moreira, Renato Giuseppe Giovanni Terzi, Mônica Corso Pereira, Tiago de Araújo Guerra Grangeia4, Ilma Aparecida Paschoal

J Bras Pneumol.2008;34(5):328-332

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Pulmonary thromboembolism is a common condition. Its diagnosis usually requires pulmonary scintigraphy, computed angiotomography, pulmonary arteriography and, in order to rule out other diagnoses, the measurement of D-dimer levels. Due to the fact that these diagnostic methods are not available in most Brazilian hospitals, the validation of other diagnostic techniques is of fundamental importance. We describe a case of a woman with chronic pulmonary hypertension who experienced a pulmonary thromboembolism event. Pulmonary scintigraphy, computed angiotomography and pulmonary arteriography were used in the diagnosis. The D-dimer test result was positive. Volumetric capnography was performed at admission and after treatment. The values obtained were compared with the imaging test results.

 


Keywords: Thromboembolism; Hypertension, pulmonary; Capnography; Schistosomiasis.

 


Brazilian consensus on non-cystic fibrosis bronchiectasis

Consenso brasileiro sobre bronquiectasias não fibrocísticas

Mônica Corso Pereira1,a, Rodrigo Abensur Athanazio2,b, Paulo de Tarso Roth Dalcin3,4,c, Mara Rúbia Fernandes de Figueiredo5,d, Mauro Gomes6,7,e, Clarice Guimarães de Freitas8,f, Fernando Ludgren9,g, Ilma Aparecida Paschoal1,h, Samia Zahi Rached2,i, Rosemeri Maurici10,j

J Bras Pneumol.2019;45(4):e20190122-e20190122

Abstract PDF PT PDF EN Portuguese Text

Bronchiectasis is a condition that has been increasingly diagnosed by chest HRCT. In the literature, bronchiectasis is divided into bronchiectasis secondary to cystic fibrosis and bronchiectasis not associated with cystic fibrosis, which is termed non-cystic fibrosis bronchiectasis. Many causes can lead to the development of bronchiectasis, and patients usually have chronic airway symptoms, recurrent infections, and CT abnormalities consistent with the condition. The first international guideline on the diagnosis and treatment of non-cystic fibrosis bronchiectasis was published in 2010. In Brazil, this is the first review document aimed at systematizing the knowledge that has been accumulated on the subject to date. Because there is insufficient evidence on which to base recommendations for various treatment topics, here the decision was made to prepare an expert consensus document. The Brazilian Thoracic Association Committee on Respiratory Infections summoned 10 pulmonologists with expertise in bronchiectasis in Brazil to conduct a critical assessment of the available scientific evidence and international guidelines, as well as to identify aspects that are relevant to the understanding of the heterogeneity of bronchiectasis and to its diagnostic and therapeutic management. Five broad topics were established (pathophysiology, diagnosis, monitoring of stable patients, treatment of stable patients, and management of exacerbations). After this subdivision, the topics were distributed among the authors, who conducted a nonsystematic review of the literature, giving priority to major publications in the specific areas, including original articles, review articles, and systematic reviews. The authors reviewed and commented on all topics, producing a single final document that was approved by consensus.

 


Keywords: Bronchiectasis; Tomography, X-ray; Radiography, thoracic.

 


Oxygen desaturation in healthy subjects undergoing the incremental shuttle walk test

Dessaturação em indivíduos saudáveis submetidos ao incremental shuttle walk test

Daniel Machado Seixas, Daniela Miti Tsukumo Seixas, Monica Corso Pereira, Marcos Mello Moreira, Ilma Aparecida Paschoal

J Bras Pneumol.2013;39(4):-

Abstract PDF PT PDF EN Portuguese Text

Objective: To determine the probability of oxygen desaturation in healthy individuals undergoing the incremental shuttle walk test (ISWT). Methods: We enrolled 83 healthy subjects: 55 males (including 1 smoker) and 28 females. We determined pre-ISWT FEV1, FEV6, HR and SpO2, as well as post-ISWT HR and SpO2. Results: Mean values overall were as follows: age, 35.05 ± 12.53 years; body mass index, 24.30 ± 3.47 kg/m2; resting HR, 75.12 ± 12.48 bpm; resting SpO2, 97.96 ± 1.02%; FEV1, 3.75 ± 0.81 L; FEV6, 4.45 ± 0.87 L; FEV1/FEV6 ratio, 0.83 ± 0.08 (no restriction or obstruction); incremental shuttle walk distance, 958.30 ± 146.32 m; post-ISWT HR, 162.41 ± 18.24 bpm; and post-ISWT SpO2, 96.27 ± 2.21%. In 11 subjects, post-ISWT SpO2 was higher than was pre-ISWT SpO2. In 17 subjects, there was a 4% decrease in SpO2 after the ISWT. There were no statistically significant differences between the groups with and without post-ISWT oxygen desaturation in terms of age, gender, FEV1, FEV6, FEV1/FEV6, pre-ISWT SpO2, incremental shuttle walk distance, HR, or percentage of maximal HR. In the individuals with post-ISWT oxygen desaturation, the body mass index was higher (p = 0.01) and post-ISWT SpO2 was lower (p = 0.0001). Conclusions: Healthy individuals can present oxygen desaturation after the ISWT. Using the ISWT to predict subtle respiratory abnormalities can be misleading. In healthy subjects, oxygen desaturation is common after the ISWT, as it is during any intense physical activity.

 


Keywords: Heart function tests; Respiratory function tests; Body mass index; Oximetry

 


Near-fatal pulmonary embolism: capnographic perspective

Embolia pulmonar quase fatal: perspectiva capnográfica

Marcos Mello Moreira1,2,a, Luiz Claudio Martins3,b, Konradin Metze4,c, Marcus Vinicius Pereira2,d, Ilma Aparecida Paschoal1,e

J Bras Pneumol.2018;44(6):525-528

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Acute respiratory failure as a manifestation of eosinophilia-myalgia syndrome associated with L-tryptophan intake

Insuficiência respiratória aguda como manifestação da síndrome de eosinofilia-mialgia associada à ingestão de L-triptofano

Tiago de Araujo Guerra Grangeia, Marcelo Schweller, Ilma Aparecida Paschoal, Lair Zambon, Mônica Corso Pereira

J Bras Pneumol.2007;33(6):747-751

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Eosinophilia-myalgia syndrome was described in 1989 in patients who presented progressive and incapacitating myalgia and eosinophilia in blood, fluids and secretions. Most patients report previous L-tryptophan intake. Respiratory manifestations are found in up to 80% of the cases, occasionally as the only manifestation. Treatment includes drug discontinuation and administration of corticosteroids. Here, we describe the case of a 61-year-old female admitted with acute respiratory failure after using L-tryptophan, hydroxytryptophan and other drugs. The patient presented eosinophilia, together with elevated eosinophil counts in the bronchoalveolar lavage and pleural effusion. After discontinuation of the drugs previously used, corticosteroids were administered, resulting in clinical and radiological improvement within just a few days.

 


Keywords: Respiratory insufficiency; Tryptophan; Eosinophilia-myalgia syndrome.

 


Chronic respiratory failure in patients with neuromuscular diseases: diagnosis and treatment

Insuficiência respiratória crônica nas doenças neuromusculares: diagnóstico e tratamento

Ilma Aparecida Paschoal, Wander de Oliveira Villalba, Mônica Corso Pereira

J Bras Pneumol.2007;33(1):81-92

Abstract PDF PT PDF EN Portuguese Text

Neuromuscular diseases affect alveolar air exchange and therefore cause chronic respiratory failure. The onset of respiratory failure can be acute, as in traumas, or progressive (slow or rapid), as in amyotrophic lateral sclerosis, muscular dystrophies, diseases of the myoneural junction, etc. Respiratory muscle impairment also affects cough efficiency and, according to the current knowledge regarding the type of treatment available in Brazil to these patients, it can be said that the high rates of morbidity and mortality in these individuals are more often related to the fact that they cough inefficiently rather than to the fact that they ventilate poorly. In this review, with the objective of presenting the options of devices available to support and substitute for natural ventilation in patients with neuromuscular diseases, we have compiled a brief history of the evolution of orthopedic braces and prostheses used to aid respiration since the end of the 19th century. In addition, we highlight the elements that are fundamental to the diagnosis of alveolar hypoventilation and of failure of the protective cough mechanism: taking of a clinical history; determination of peak cough flow; measurement of maximal inspiratory and expiratory pressures; spirometry in two positions (sitting and supine); pulse oximetry; capnography; and polysomnography. Furthermore, the threshold values available in the literature for the use of nocturnal ventilatory support and for the extension of this support through the daytime period are presented. Moreover, the maneuvers used to increase cough efficiency, as well as the proper timing of their introduction, are discussed.

 


Keywords: Respiratory insufficiency/diagnosis; Chronic disease; Respiratory insufficiency/therapy;

 


More than good lungs!

Mais do que bons pulmões!

Ilma Aparecida Paschoal

J Bras Pneumol.2009;35(10):947-948

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Idiopathic bilateral diaphragmatic paresis

Paresia diafragmática bilateral idiopática

Mônica Corso Pereira, Rodrigo Frange Miziara Mussi, Reinaldo Alexandre de Carvalho Massucio, Ana Maria Camino, Aristóteles de Souza Barbeiro, Wander de Oliveira Villalba, Ilma Aparecida Paschoal

J Bras Pneumol.2006;32(5):481-485

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We report the case of a patient with severe dyspnea upon reclining. Lung disease, neuromuscular disorders and heart disease were ruled out. However, during the course of the investigation, bilateral diaphragmatic paresis was discovered. A key sign leading to the diagnosis was evidence of paradoxical respiration in the dorsal decubitus position. When the patient was moved from the orthostatic position to the dorsal decubitus position, oxygenation and forced vital capacity worsened. The orthostatic fluoroscopy was normal. Maximal inspiratory pressure was severely reduced. The responses to transcutaneous electric stimulation of the diaphragm were normal. However, electric stimulation of the phrenic nerve produced no response, leading to the diagnosis of bilateral diaphragmatic paresis.

 


Keywords: Paresis; Respiratory insufficiency; Diaphragm; Respiration

 


 

 


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