OBJETIVO: Utilizar os dados clínicos e espirométricos para distinguir entre os padrões restritivo e inespecífico dos resultados dos testes de função pulmonar em pacientes com CVF reduzida e relação VEF1/CVF normal ou elevada. MÉTODOS: Foram avaliados resultados de testes de função pulmonar de 211 pacientes adultos submetidos à espirometria e a medidas de volumes pulmonares. O diagnóstico clínico na solicitação do exame e diversos dados funcionais foram utilizados para diferenciar pacientes com o padrão restritivo "verdadeiro" (CPT reduzida) daqueles com o padrão inespecífico (CPT normal). RESULTADOS: Na amostra estudada, a CPT estava reduzida em 144 casos e estava dentro da faixa normal em 67. As causas mais comuns do padrão inespecífico foram doenças pulmonares obstrutivas, insuficiência cardíaca congestiva, obesidade, bronquiolite, doenças intersticiais e doenças neuromusculares. Em pacientes com hipótese diagnóstica de fibrose pulmonar, doenças pleurais ou doenças da parede torácica, o valor preditivo positivo (VPP) para restrição foi > 90%. Em homens, a CVF < 60% do previsto teve um VPP para restrição de 98,8%. Em mulheres, o padrão restritivo foi encontrado em 84,4% daquelas com CVF < 50% do previsto. Uma diferença entre VEF1% e CVF% > 0% teve um VPP para restrição de 89,5%. Após regressão logística, uma escala de pontos foi desenvolvida para predizer o padrão restritivo. CONCLUSÕES: O padrão restritivo pode ser identificado com segurança em diversos casos com VEF1 e CVF reduzidos e relação VEF1/CVF normal usando-se um algoritmo que leva em conta o diagnóstico clínico e alguns achados espirométricos.

OBJECTIVE: To use clinical and spirometry findings in order to distinguish between the restrictive and nonspecific patterns of pulmonary function test results in patients with low FVC and a normal or elevated FEV1/FVC ratio. METHODS: We analyzed the pulmonary function test results of 211 adult patients submitted to spirometry and lung volume measurements. We used the clinical diagnosis at the time spirometry was ordered, together with various functional data, in order to distinguish between patients presenting with a "true" restrictive pattern (reduced TLC) and those presenting with a nonspecific pattern (normal TLC). RESULTS: In the study sample, TLC was reduced in 144 cases and was within the normal range in 67. The most common causes of a nonspecific pattern were obstructive disorders, congestive heart failure, obesity, bronchiolitis, interstitial diseases, and neuromuscular disorders. In patients given a working diagnosis of pulmonary fibrosis, pleural disease, or chest wall disease, the positive predictive value (PPV) for restriction was > 90%. In males, an FVC < 60% of predicted had a PPV for restriction of 98.8%. In females, the restrictive pattern was found in 84.4% of those with an FVC < 50% of predicted. A difference of > 0% between the FEV1% and the FVC% had a PPV for restriction of 89.5%. After performing logistic regression, we developed a point scale for predicting the restrictive pattern. CONCLUSIONS: In many patients with reduced FEV1, reduced FVC, and a normal FEV1/FVC ratio, the restrictive pattern can be identified with confidence through the use of an algorithm that takes the clinical diagnosis and certain spirometry measurements into account.

Paciente masculino, 27 anos, com sintomas respiratórios, linfonodomegalia cervical anterior bilateral e hepatomegalia. Os estudos de imagem evidenciaram linfonodomegalia hilar bilateral e infiltrado pulmonar. O paciente foi submetido a biópsias pulmonar e hepática, que evidenciaram presença de granulomas não caseosos. Também foi submetido à biópsia de linfonodo hilar, que revelou a presença de material amilóide. Os achados clínicos, radiológicos e histopatológicos foram compatíveis com sarcoidose e amiloidose ganglionar. A associação entre sarcoidose e amiloidose é raramente descrita.

A 27-year-old male patient presented with respiratory symptoms, bilateral enlargement of the cervical lymph nodes and enlarged liver. In the imaging studies, bilateral enlargement of the hilar nodes was observed, together with pulmonary infiltrate. The patient was submitted to lung and liver biopsies, which revealed noncaseating granulomas. The clinical, radiological and histopathological findings were consistent with sarcoidosis and lymph node amyloidosis. The combination of sarcoidosis and amyloidosis has rarely been reported.

A linfangiomatose, doença rara e de origem controversa, ocorre em indivíduos de todas as faixas etárias, predominando entre os mais jovens, sem predileção por sexo. Comumente cursa com envolvimento torácico, porém órgãos como ossos, baço e fígado podem ser acometidos. Histologicamente, o envolvimento pulmonar cursa com proliferação, anastomoses complexas e dilatação secundária do sistema linfático. Clinicamente, a apresentação é variável. Os achados radiográficos podem ser sugestivos e o diagnóstico é definido pela histologia. Relatamos dois casos da doença; uma das pacientes era oligossintomática e encontra-se em tratamento; na outra, a doença foi mais agressiva e o diagnóstico muito tardio, culminando em óbito.

Lymphangiomatosis, a rare diseases of controversial origin, occurs in individuals of any age, regardless of gender, but is predominantly seen in younger individuals. It often presents with thoracic involvement, although, the bones, spleen and liver can also be affected. Histologically, the pulmonar involvement includes proliferation, complex anastomoses and secondary dilatation of the lymphatic vessels. Clinically, the presentation is variable. Although radiographic findings can be suggestive of the disease, the final diagnosis is made histologically. We report two cases of lymphangiomatosis, both in females: one was oligosymptomatic and is being treated for the disease; the other had a more progressive form, was diagnosed quite late and ultimately died of the disease.

O quilotórax ocorre quando há ruptura, laceração ou obstrução do ducto torácico, com liberação de quilo no espaço pleural. Pode acontecer em malformações linfáticas congênitas, linfomas, tumores de mediastino, doenças infecciosas, procedimentos cirúrgicos, traumas automobilísticos, ou ser idiopático. Apresenta sinais clínicos de dispnéia, hipotensão, edema generalizado e cianose. O diagnóstico geralmente é feito por toracocentese e o tratamento é conservador. O quilotórax espontâneo é uma condição incomum de derrame pleural, e somente é hipótese diagnóstica após a exclusão das demais causas. Descrevemos um caso de quilotórax espontâneo associado a atividade física leve em academia de ginástica.

Chylothorax occurs when there is rupture, laceration or obstruction of the thoracic duct, resulting in the release of chyle into the pleural space. Chylothorax can occur in cases of congenital lymphatic malformation, lymphoma, mediastinal tumor and infectious disease, as well as during surgical procedures and after traffic accident-related trauma. It can also be idiopathic. The condition presents clinical signs of dyspnea, hypotension, generalized edema and cyanosis. The diagnosis is usually made through thoracocentesis, and the treatment is conservative. Spontaneous chylothorax is an uncommon form of pleural effusion, and its diagnosis should be hypothesized only after all other causes have been ruled out. Herein, we describe a case of spontaneous chylothorax associated with light physical activity at a fitness center.

BACKGROUND: Several factors influence the pulmonary function values considered normal. In children of short stature, there are difficulties in interpreting the pulmonary function. OBJECTIVE: To assess spirometric values in children and adolescents with short stature and to identify a correction factor to adequately predict the expected values for this population. METHOD: A prospective selection of 77 patients was made, all with short stature and no respiratory disease. These patients were submitted to spirometry, transcutaneous hemoglobin oxygen saturation, chest perimeter measurement, and immediate hypersensitivity testing. Bone age was assessed by wrist X-rays. The data obtained by spirometry (FVC, FEV1, and FEF25-75%) were compared with those of Polgar and Promadhat (1971), predicted in three ways: a) by actual height; b) by height estimated at the 50th percentile for chronological age (CA); c) by height estimated at the 50th percentile for bone age (BA). RESULTS: The mean height was 133.3 ± 13.2 cm, and the deficit in relation to the third percentile was 5.4 ± 6.0 cm. The values obtained for FVC, FEV1, FEF25-75%, were significantly higher than those predicted by actual height. The mean FEV1 obtained was 2.42 ± 0.71 L, and the predicted (actual height) was 2.10 ± 0.64 L; according to the height estimated by BA and CA, the values were 2.27 and 2.86 L, respectively. The mean FVC1 was 2.20 ± 0.6 L, and the predicted was 1.90 ± 0.55 L. With the height estimated for bone age and chronologic age, the predicted values were 2.10 and 2.60 L, respectively. CONCLUSION: Children and adolescents with short stature have higher spirometric values than predicted for their actual height. These findings suggest that the height estimated at the 50th percentile for bone age can be used to evaluate pulmonary function.

Racional - A síndrome hepatopulmonar caracteriza-se pela presença de hipoxemia arterial resultante de dilatações vasculares intra-pulmonares em portadores de doença hepática crônica e/ou hipertensão portal. A síndrome, que resulta em hipoxemia arterial por vezes grave, pode alcançar completa resolução após o transplante hepático. Objetivo - Determinar a prevalência da síndrome hepatopulmonar em portadores de cirrose hepática, candidatos a transplante de fígado no Serviço de Transplante Hepático do Hospital de Clinicas da Universidade Federal do Paraná, Curitiba, PR. Pacientes e Métodos - Cinqüenta e quatro pacientes adultos (idade > ou = 18 anos) com cirrose hepática avançada foram submetidos a análise dos gases arteriais em ar ambiente na posição sentada. Os pacientes com hipoxemia arterial (PaO2 < 70 mm Hg) foram avaliados com ecocardiografia com microbolhas e estudo da função pulmonar. Resultados - Sete dos 54 pacientes (12,9%) apresentaram hipoxemia arterial em ar ambiente, embora apenas um deles referisse dispnéia aos esforços. Os sete pacientes apresentavam disfunção hepática variável de acordo com a classificação de Child (A = 1, B = 4, C = 2). Em todos os sete pacientes a ecocardiografia com microbolhas foi positiva, caracterizando a presença de síndrome hepatopulmonar. O estudo da função pulmonar não revelou anormalidades nos sete pacientes hipoxêmicos. Conclusão - A prevalência da síndrome hepatopulmonar em candidatos a transplante hepático é elevada. Essa síndrome nem sempre se acompanha de sintomas respiratórios, devendo ser rastreada rotineiramente no processo de seleção dos candidatos a transplante hepático.

Background - Hepatopulmonary syndrome is an important clinical problem associated with chronic liver disease. Liver transplantation can result in complete resolution of the arterial hypoxemia associated with this syndrome, even in its most severe presentation. Aim - To determine the prevalence of hepatopulmonary syndrome in adult liver transplant candidates. Patients and Methods - Fifty-four consecutives adult patients ( > or = 18 years) with severe liver disease waiting for liver transplant were screenned for arterial hypoxemia (PaO2 <70 mm Hg) while they were seated, at rest, and breathing room air. Patients with arterial hypoxemia underwent contrast-enhanced two-dimensional echocardiography and pulmonary function testing. Hypoxemia (PaO2 <70 mm Hg) was present in 7 of 54 patients (12,9%), although only 1 of them complained of dyspnea. The Child's classification of the patients were: A = 1, B = 4, and C = 1. All seven hypoxemic patients had positive contrast-enhanced two-dimensional echocardiography, consistent with hepatopulmonary syndrome. Conclusion - The prevalence of hepatopulmonary syndrome in adult liver transplant candidates is elevated. The screenning for hepatopulmonay syndrome should be part of the routine evaluation of liver transplant candidates even in the absence of pulmonary symptoms.