Brazilian Journal of Pulmonology

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


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Year 2008 - Volume 34  - Number 10  (/October)


1 - Tuberculosis/HIV co-infection

Co-infecção tuberculose/HIV

Antônio Carlos Moreira Lemos

J Bras Pneumol.2008;34(10):753-755

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Original Article

2 - Evaluation of the Asthma Control Questionnaire validated for use in Brazil

Avaliação do Questionário de Controle da Asma validado para uso no Brasil

Mylene Leite, Eduardo Vieira Ponte, Jaqueline Petroni, Argemiro D`Oliveira Júnior, Emílio Pizzichini, Álvaro Augusto Cruz

J Bras Pneumol.2008;34(10):756-763

Abstract PDF PT PDF EN Portuguese Text

Objective: To evaluate whether the Portuguese version of the Asthma Control Questionnaire (ACQ) is a valid instrument to measure asthma control in adult outpatients in Brazil. Methods: We selected 278 outpatients diagnosed with asthma. All of the patients completed the questionnaire, underwent spirometry and were clinically evaluated by a physician in order to characterize the control of the disease in the first visit. The questionnaire was evaluated in three versions, with 5, 6 and 7 questions, respectively, and scores of 0.75 and 1.50 were used as cut-off points. Results: Of the 278 patients, 77 (27.7%) had intermittent asthma, 39 (14.0%) had mild persistent asthma, 40 (14.4%) had moderate persistent asthma and 122 (43.9%) had severe persistent asthma. The sensitivity of ACQ to identify uncontrolled asthma ranged from 77% to 99%, and the specificity ranged from 36% to 84%. The positive predictive value ranged from 73% to 90%, and the negative predictive value ranged from 67% to 95%. The positive likelihood ratio ranged from 1.55 to 4.81, and the negative likelihood ratio ranged from 0.03 to 0.27. In the 5- and 6-question versions of the ACQ, the intraclass correlation coefficient was 0.92. These two versions were both responsive to clinical changes in the patients. Conclusions: All three versions of the ACQ satisfactorily discriminated between patients with uncontrolled asthma and those with controlled asthma. The 5- and 6-question versions also presented good reliability and responsiveness. Therefore, the ACQ is a valid tool for evaluating asthma control in adult outpatients in Brazil.


Keywords: Asthma; Therapeutics; Diagnosis; Questionnaires; Reproducibility of results.


3 - Asthma and lung function in a birth cohort at 6-7 years of age in southern Brazil

Asma e função pulmonar aos 6-7 anos de idade em uma coorte de nascimentos no Sul do Brasil

Moema Nudilemon Chatkin, Ana Maria Baptista Menezes, Silvia Elaine Cardozo Macedo, Edgar Fiss

J Bras Pneumol.2008;34(10):

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Objective: Asthma and respiratory symptoms are common in children, and many studies have shown associations between childhood symptoms and impaired lung function in adult life. The aim of the present study was to investigate the association of various respiratory symptoms with wheezing patterns (persistent, early, and late-onset) and lung function, as well as to determine whether lung function was associated with atopy or with demographic, socioeconomic, environmental, and gestational factors, in a birth cohort at 6-7 years of age. Methods: The target population consisted of children aged 6-7 years from a birth cohort of 5,304 children born in southern Brazil in 1993. For this follow-up evaluation, 532 of those children were randomly selected, and a sub-sample was submitted to spirometry and skin prick tests. A questionnaire was administered to the parent(s) or legal guardian(s) of each child. Results: Spirometric values were lower in the children with respiratory symptoms or asthma. Mean forced expiratory volume in one second/forced vital capacity ratio (FEV1/FVC ratio) was lower in children with any of the following: current wheezing and asthma; asthma ever; four or more episodes of wheezing within the preceding 12 months; sleep disturbance due to wheezing; and exercise-induced wheezing. Persistent wheezing was associated with lower FEV1/FVC ratio. After multiple linear regression, exercise-induced wheezing was also associated with reduced FEV1/FVC ratio. Nonwhite skin color and wheezing severe enough to limit speech were associated with lower FEV1. Conclusions: Children with persistent wheezing and symptoms of severe asthma have impaired lung function at 6-7 years of age.


Keywords: Asthma; Respiratory function tests; Respiratory sounds; Signs and symptoms, respiratory.


4 - Prevalence of gastroesophageal reflux in lung transplant candidates with advanced lung disease

Prevalência de refluxo gastroesofágico em pacientes com doença pulmonar avançada candidatos a transplante pulmonar

Gustavo Almeida Fortunato, Mirna Mota Machado, Cristiano Feijó Andrade, José Carlos Felicetti, José de Jesus Peixoto Camargo, Paulo Francisco Guerreiro Cardoso

J Bras Pneumol.2008;34(10):772-778

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Objective: To assess the esophageal function profile and the prevalence of gastroesophageal reflux (GER) in lung transplant candidates. Methods: From July of 2005 to November of 2006, a prospective study was conducted involving 55 candidates for lung transplantation at the Santa Casa de Misericórdia Hospital in Porto Alegre, Brazil. Prior to transplantation, patients underwent outpatient stationary esophageal manometry and 24-h esophageal pH-metry using one and two electrodes. Results: Abnormal esophageal manometry was documented in 80% of the patients, and 24% of the patients presented pathological acid reflux. Digestive symptoms presented sensitivity and specificity for GER of 50% and 61%, respectively. Of the patients with chronic obstructive pulmonary disease, 94% presented abnormal esophageal manometry, and 80% presented lower esophageal sphincter hypotonia, making it the most common finding. Patients with bronchiectasis presented the highest prevalence of GER (50%). Conclusions: In patients with advanced lung disease, GER is highly prevalent. In the population studied, digestive symptoms of GER were not predictive of pathological acid reflux. The role that GER plays in chronic rejection should be examined and clarified in future studies.


Keywords: Gastroesophageal reflux; Lung transplantation; Manometry; Esophageal pH monitoring.


5 - Idiopathic pulmonary fibrosis and emphysema in smokers

Fibrose pulmonar idiopática simultânea a enfisema em pacientes tabagistas

Denise Rossato Silva, Marcelo Basso Gazzana, Sérgio Saldanha Menna Barreto, Marli Maria Knorst

J Bras Pneumol.2008;34(10):779-786

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Objective: To describe the clinical and functional findings recently reported in the medical literature for patients diagnosed with emphysema involving the upper lobes and idiopathic pulmonary fibrosis (IPF) involving the lower lobes. Methods: Eleven patients with emphysema and IPF were identified retrospectively. All of the patients underwent high-resolution computed tomography of the lung and pulmonary function tests. Results: Of the 11 patients, 8 were male and 3 were female. The mean age was 70.7 ± 7.2 years (range, 61-86 years). All of the patients were smokers (mean smoking history, 61.5 ± 43.5 pack-years). The mean values of forced vital capacity (FVC), forced expiratory volume in one second (FEV1) and FEV1/FVC were 72.1 ± 12.7%, 68.2 ± 11.9% and 74.4 ± 10.8, respectively. Lung volumes were normal in 7 patients. A restrictive pattern was observed in 3 patients, and hyperinflation was present in one. The diffusing capacity was moderatelyto- severely reduced in all of the patients (mean, 27.7% ± 12.9% of predicted). Ten of the 11 patients performed the six-minute walk test. The mean distance covered was 358.4 ± 143.1 m, and 9 of the 10 patients presented desaturation ≥ 4%. Echocardiographic findings suggestive of pulmonary hypertension were present in 4 patients (mean systolic pulmonary artery pressure, 61.8 mmHg; range, 36-84 mmHg). Conclusions: The concomitant presence of emphysema and IPF causes characteristic changes on pulmonary function tests. The most significant finding is a discrepancy between diffusing capacity and spirometry results.


Keywords: Pulmonary emphysema; Pulmonary fibrosis; Lung Diseases, interstitial; Anoxia; Hypertension, pulmonary.


6 - Immunohistochemical study of lung remodeling in mice exposed to cigarette smoke

Estudo imunohistoquímico do remodelamento pulmonar em camundongos expostos à fumaça de cigarro

Samuel Santos Valença, Luís Cristóvão Porto

J Bras Pneumol.2008;34(10):787-795

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Objective: Various studies of emphysema involve long-term exposure of animals to cigarette smoke, focusing on the cell type involved in the protease/antiprotease imbalance and on extracellular matrix degradation. In emphysema, increased expression of metalloproteinases is associated with cytokines, and evidence suggests that the matrix metalloproteinase-12 (MMP-12) plays an important role. Our objective was to investigate tissue inhibitor of metalloproteinase-2 (TIMP-2), tumor necrosis factor-alpha (TNF-α) and interleukin-6 (IL-6) detection by immunohistochemical methods in mouse lung. Methods: Male C57BL/6 mice were exposed 3 times a day to the smoke of 3 cigarettes over a period of 10, 20, 30 or 60 days in an inhalation chamber (groups CS10, CS20, CS30 and CS60, respectively). Controls were exposed to the same conditions in room air. Results: A progressive increase in the number of alveolar macrophages was observed in the bronchoalveolar lavage fluid of the exposed mice. The mean linear intercept, an indicator of alveolar destruction, was greater in all exposed groups when compared to control group. In the CS10, CS20 and CS30 mice, the immunohistochemical index (II) for MMP-12 increased in parallel with a decrease in II for TIMP-2 in the CS10, CS20 and CS30 mice. The II for the cytokines TNF-α and IL-6 was greater in all exposed groups than in the control group. Emphysema, with changes in volume density of collagen and elastic fibers, was observed in the CS60 group. Conclusions: These findings suggest that cigarette smoke induces emphysema with major participation of TNF-α and IL-6 without participation of neutrophils.


Keywords: Macrophages, alveolar; Emphysema; Tissue inhibitor of metalloproteinase-2; Interleukin-6.


7 - Airway resistance in children measured using the interrupter technique: reference values

Resistência de vias aéreas em crianças medida pela técnica do interruptor: valores de referência

Viviane Viegas Rech, Paula Cristina Vasconcellos Vidal, Hilário Teixeira de Melo Júnior, Renato Tetelbom Stein, Paulo Márcio Condessa Pitrez, Marcus Herbert Jones

J Bras Pneumol.2008;34(10):796-803

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Objective: The interrupter technique is used for determining interrupter resistance (Rint) during quiet breathing. This noninvasive method requires minimal cooperation and can therefore be useful in evaluating airway obstruction in uncooperative children. To date, no reference values have been determined for Rint in a Brazilian population. The objective of this study was to define a prediction equation for airway resistance using the interrupter technique for healthy children aged 3-13 years. Methods: This was a prospective, cross-sectional study involving preschool and school children in Porto Alegre, Brazil, in whom Rint was measured during peak expiratory flow. Results: ­One-hundred and ninety-three children were evaluated. Univariate analysis using linear regression showed that height, weight and age correlated significantly and independently with Rint. Multiple regression with height, weight, age and gender as variables resulted in a model in which only height and weight were significant, independent predictors of Rint. Collinearity was identified among height, weight and age. ­Conclusions: Reference values and an equation for calculating Rint in healthy children were obtained and are adjusted for height.


Keywords: Respiratory function tests; Airway resistance; Diagnostic techniques, respiratory system; Airway obstruction/diagnosis; Reference values.


8 - Using electron microscopy and multivariate cluster analysis to determine diagnosis and prognosis in cases of neuroendocrine lung carcinoma

Diagnóstico e prognóstico dos tumores pulmonares neuroendócrinos mediante microscopia eletrônica e análise multivariavel de agrupamento

Cecília Aparecida Vaiano Farhat, Edwin Roger Parra, Andrew V. Rogers, Silvia Nagib Elian, Mary N. Sheppard, Vera Luiza Capelozzi

J Bras Pneumol.2008;34(10):804-811

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Objective: To establish reproducible electron microscopic criteria for identifying the four major types of neuroendocrine tumors of the lung: carcinoid; atypical carcinoid; large cell neuroendocrine carcinoma; and small cell carcinoma. Methods: Measurements were made on electron micrographs using a digital image analyzer. Sixteen morphometric variables related to tumor cell differentiation were assessed in 27 tumors. The examination under electron microscopy revealed that all of the tumors could be classified as belonging to one of the four categories listed above. Cluster analysis of the morphometry variables was used to group the tumors into three clusters, and Kaplan-Meier survival function curves were employed in order to draw correlations between each cluster and survival. Results: All three clusters of neuroendocrine carcinomas were found to be associated with survival curves, demonstrating the prognostic significance of electron microscopic features. The tumors fell into three well-defined clusters, which represent the spectrum of neuroendocrine differentiation: typical carcinoid (cluster 1); atypical carcinoid and large cell neuroendocrine carcinoma (cluster 2); and small cell carcinoma (cluster 3). Cluster 2 represents an intermediate step in neuroendocrine carcinogenesis, between typical carcinoid tumors and small cell carcinomas. Conclusions: Our findings confirm that electron microscopy is useful in making the diagnosis and prognosis in cases of lung tumor.


Keywords: Neuroendocrine tumors/lung; Microscopy, electron; Cluster analysis; Survival analysis.


9 - Extramedullary hematopoiesis: findings on computed tomography scans of the chest in 6 patients

Hematopoese extramedular: achados em tomografia computadorizada do tórax de 6 pacientes

Edson Marchiori, Dante Luiz Escuissato, Klaus Loureiro Irion, Gláucia Zanetti, Rosana Souza Rodrigues, Gustavo de Souza Portes Meirelles, Bruno Hochhegger

J Bras Pneumol.2008;34(10):812-816

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Objective: To present findings on computed tomography scans of the chest indicative of extramedullary hematopoiesis in six patients. Methods: We retrospectively analyzed computed tomography scans of six adult patients-five males and one female-with a mean age of 36.5 years. Two radiologists independently reviewed the scans, and a consensus was reached in discrepant cases. Results: The most common finding in the scans was lower paravertebral masses with heterogeneous content (four patients). The scans of two patients showed a solitary parietal and pleural mass. Conclusions: There are findings in computed tomography scans that are highly suggestive of extramedullary hematopoiesis, especially when those findings correlate with underlying blood diseases. Such findings, in most of the cases, allow physicians to dispense with histopathological confirmation.


Keywords: Tomography, X-ray computed; Hematopoiesis, extramedullary; Mediastinum/physiopathology; Anemia, sickle cell.


10 - Alterations in peak inspiratory pressure and tidal volume delivered by manually operated self-inflating resuscitation bags as a function of the oxygen supply rate

Alterações da pressão de pico inspiratório e do volume corrente fornecidos por reanimadores manuais com balão auto-inflável em função do fluxo de entrada de oxigênio utilizado

Armando Carlos Franco de Godoy, Ronan José Vieira, Eduardo Mello De Capitani

J Bras Pneumol.2008;34(10):817-821

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Objective: To assess possible alterations in the tidal volume and peak inspiratory pressure delivered by seven models of manually operated self-inflating resuscitation bags as a function of the oxygen supply rate used. Methods: The resuscitation bags tested were the following: Oxigel, models A and B; CE Reanimadores; ProtecSolutions; Missouri; Axmed; and Narcosul. For the measurements, a wall oxygen flow meter, a flow meter/respirometer, a resuscitation bag, a sensor (Tracer 5 unit), and a test lung were connected. In addition, the Tracer 5 unit was connected to a notebook computer. Oxygen supply rates of 1, 5, 10, and 15 L/min were used. Results: The tidal volume delivered by the Oxigel model A resuscitation bag when receiving oxygen at a rate of 15 L/min was approximately 99% greater than that delivered when receiving oxygen at a rate of 1 L/min. Similarly, peak inspiratory pressure was approximately 155% greater. Under the same conditions, the tidal volume delivered by the Narcosul resuscitation bag was 48% greater, and peak inspiratory pressure was 105% greater. The remaining resuscitation bags tested showed no significant alterations in the tidal volume or peak inspiratory pressure delivered. Conclusions: Under the resistance and compliance conditions used, the resuscitation bags in which the oxygen inflow is directed to the interior of the bag had the patient valve stuck at the inspiratory position when receiving oxygen at a rate ≥ 5 L/min, significantly increasing the tidal volume and peak inspiratory pressure delivered. This did not occur with the resuscitation bags in which the oxygen inflow is directed to the exterior of the bag.


Keywords: Oxygen/administration & dosage; Resuscitation; Equipment and supplies; Pulmonary ventilation; Intensive care.


11 - Mycobacterium tuberculosis and nontuberculous mycobacterial isolates among patients with recent HIV infection in Mozambique

Doença pulmonar por Mycobacterium tuberculosis e micobactérias não-tuberculosas entre pacientes recém-diagnosticados como HIV positivos em Moçambique, África

Elizabete Abrantes Nunes, Eduardo Mello De Capitani, Elizabete Coelho, Alessandra Costa Panunto, Orvalho Augusto Joaquim, Marcelo de Carvalho Ramos

J Bras Pneumol.2008;34(10):822-828

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Objective: Mycobacteriosis is frequently diagnosed among HIV-infected patients. In Mozambique, where few patients are under antiretroviral therapy and the prevalence of tuberculosis is high, there is need for better characterization of mycobacteria at the species level, as well as for the identification of patterns of resistance to antituberculous drugs. Methods: We studied a sample of 503 HIV-infected individuals suspected of having pulmonary tuberculosis. Of those 503, 320 tested positive for mycobacteria through sputum smear microscopy or culture of bronchoalveolar lavage fluid. Results: Acid-fast bacilli were observed in the sputum of 73% of the individuals presenting positive cultures. Of 277 isolates tested, only 3 were nontuberculous mycobacteria: 2 were identified as Mycobacterium avium and one was identified as M. simiae. Strains initially characterized as M. tuberculosis complex through polymerase chain reaction restriction analysis (PRA) of the hsp65 gene were later confirmed as such through PRA of the gyrB gene. Among the M. tuberculosis isolates, resistance patterns were as follows: to isoniazid, 14%; to rifampin, 6%; and multidrug resistance, 5%. Previously treated cases showed significantly higher rates of resistance to first-line antituberculous drugs. The most common radiological pattern was interstitial infiltrate (in 67%), followed by mediastinal lymph node enlargement (in 30%), bronchiectasis (in 28%), miliary nodules (in 18%) and cavitation (in 12%). Patients infected with nontuberculous mycobacteria presented clinical profiles indistinguishable from those of other patients. The median CD4 lymphocyte count in this group was 134 cells/mm3. Conclusions: There is a strong association between tuberculosis and AIDS in Mozambique, as expected in a country with a high prevalence of tuberculosis. Although drug resistance rates are high, the isoniazid-rifampin regimen continues to be the appropriate choice for initial therapy.


Keywords: Tuberculosis; Mycobacteria, atypical; HIV; Acquired immunodeficiency syndrome; Drug Resistance, Multiple; Mozambique.


Review Article

12 - Cystic fibrosis-related dyslipidemia

Dislipidemia relacionada à fibrose cística

Crésio de Aragão Dantas Alves, Daniela Seabra Lima

J Bras Pneumol.2008;34(10):829-837

Abstract PDF PT PDF EN Portuguese Text

This article aims to review the physiopathology, diagnosis and treatment of cystic fibrosis-related dyslipidemia (CFD). Bibliographic searches of the Medline and Latin American and Caribbean Health Sciences Literature databases were made (year range, 1987-2007), and the most representative papers on the theme were selected. The characteristic symptoms of CFD are hypertriglyceridemia-with or without hypocholesterolemia-and essential fatty acid deficiency. The principal CFD risk factors are pancreatic insufficiency, high-carbohydrate diet, liver diseases, inflammatory state and corticosteroid therapy. There are no specific recommendations regarding screening, which is typically performed based on the diagnosis, and at regular intervals, and more frequently in individuals belonging to high-risk groups. Treatment includes a balanced diet, micronutrient supplementation, and regular physical exercise according to individual tolerance. In the great majority of the cases, CFD-related hypertriglyceridemia does not reach values for which the use of hypolipidemic drugs is indicated. We conclude that there are few articles in the literature regarding the frequency, etiology and management of CFD. Preventive and therapeutic recommendations for hypertriglyceridemia are extrapolated from studies in individuals without cystic fibrosis. Further research is necessary to investigate the association of essential fatty acid deficiency and the physiopathology of cystic fibrosis. Since hypertriglyceridemia is an important risk factor for coronary artery disease, prospective studies will contribute for a better understanding of the natural history of this condition and define how to prevent and treat it.


Keywords: Cystic fibrosis; Dyslipidemias; Hypertriglyceridemia; Fatty Acids, nonesterified.


Brief Communication

13 - Testing pulmonary vasoreactivity

Teste de vasorreatividade pulmonar

Edmundo Clarindo Oliveira, Carlos Faria Santos Amaral, Marco Antonio Moura, Frederico Thadeu Assis Figueiredo Campos, Helder Machado Pauperio

J Bras Pneumol.2008;34(10):838-844

Abstract PDF PT PDF EN Portuguese Text

Pulmonary arterial hypertension is classified as idiopathic or secondary (associated with collagenoses, heart disease, portal hypertension, pulmonary thromboembolism, and pulmonary vascular diseases). Pulmonary vasoreactivity should be tested in order to define the best treatment option. Of the many drugs that have been used to test pulmonary vasoreactivity, inhaled nitric oxide is the best choice, due to its specific pulmonary effect and very short half-life (5-10 s). The results of this test identify candidates for heart surgery among patients with congenital heart disease and candidates for the use of calcium antagonists among patients with other forms of pulmonary arterial hypertension. Performing and interpreting the results of such tests are a great responsibility, since mistakes can lead to incorrect treatment decisions, resulting in the death of patients.


Keywords: Hypertension, pulmonary/diagnosis; Nitric oxide/diagnostic use; Administration, inhalation.


Guidelines SBPT

14 - Smoking cessation guidelines - 2008

Diretrizes para cessação do tabagismo - 2008

Jonatas Reichert, Alberto José de Araújo, Cristina Maria Cantarino Gonçalves, Irma de Godoy, José Miguel Chatkin, Maria da Penha Uchoa Sales, Sergio Ricardo Rodrigues de Almeida Santos e Colaboradores

J Bras Pneumol.2008;34(10):845-880

Abstract PDF PT PDF EN Portuguese Text

These guidelines are an up-to-date and comprehensive tool to aid health professionals in treating smokers, recommending measures and strategies for managing each case based on clinical evidence. Written in a simplified and objective manner, the text is divided into two principal sections: Evaluation and Treatment. The sections both present comments on and levels of evidence represented by the references cited, as well as some proposals for the reduction of damage and for intervening in specific and still poorly explored situations, such as relapse, passive smoking, physician smoking, and tobacco use in specific environments.


Keywords: Smoking/adverse effects; Smoking cessation/methods; Guideline.


Case Report

15 - Primary tracheobronchial amyloidosis

Amiloidose traqueobrônquica primária

José Wellington Alves dos Santos, Ayrton Schneider Filho, Alessandra Bertolazzi, Gustavo Trindade Michel, Lauro Vinícius Schvarcz da Silva, Carlos Renato Melo, Vinícius Dallagasperina Pedro, Daniel Spilmann, Juliana Kaczmareck Figaro

J Bras Pneumol.2008;34(10):881-884

Abstract PDF PT PDF EN Portuguese Text

Tracheobronchial amyloidosis is an uncommon localized form of amyloidosis, characterized by amyloid deposits restricted to the trachea, main bronchi and segmental bronchi. We present the case of a retired 67-year-old man with long-term progressive dyspnea, wheezing and chest pain. A diagnosis of tracheobronchial amyloidosis was made after the third fiberoptic bronchoscopy and histological confirmation through Congo red staining of tissue samples.


Keywords: Amiloidose/traquéia; Broncoscopia; Doenças da traquéia/diagnóstico.


16 - Pregnancy in a patient with severe pulmonary alveolar microlithiasis

Gravidez em paciente com microlitíase alveolar pulmonar grave

José Osmar Bezerra de Souza Filho, Cristiane Maria Cavalcante Silveira, Aline Barreto da Cunha, Valéria Goes Ferreira Pinheiro, Francisco Edson de Lucena Feitosa, Marcelo Alcântara Holanda

J Bras Pneumol.2008;34(10):885-888

Abstract PDF PT PDF EN Portuguese Text

Pulmonary alveolar microlithiasis (PAM) is a rare disease that affects both lungs. It is characterized by the presence of small calculi (calcium phosphate) within the alveolar spaces. We report the case of a 26-year-old female whose diagnosis was based on characteristic findings on chest X-rays and high-resolution computed tomography scans. The patient, 28 weeks pregnant, was rehospitalized 10 months after the diagnosis, presenting hypoxemic acute respiratory failure and severe restrictive ventilatory defect on spirometry. After 32 completed weeks of gestation (228 days), she was submitted to cesarean section, and the outcome was successful for mother and newborn. PAM has a variable clinical course. It is suggestive of an autosomal recessive inheritance pattern and has been associated with positive family history. The etiology of PAM is unclear, and many authors speculate that there is a local enzymatic defect responsible for the intra-alveolar accumulation of calcium. Reports of patients with PAM who become pregnant are exceptional, and this is the first case described in Brazil. The course of this disease is usually slow and progressive, and patients typically die of cardiorespiratory failure. The present case illustrates the need to offer female patients, especially those with advanced disease, genetic counseling and orientation regarding the risks of pregnancy. Currently, the only effective therapy is lung transplantation.


Keywords: Pulmonary alveoli; Pregnancy; Respiratory insufficiency.



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