Resp Care
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Review Case Reports
Congenital pulmonary airway malformation: a case report and review of the literature.
Congenital parenchymal lung malformations have an estimated incidence at 1:25,000-1:35,000 births. We present a case of this rare congenital abnormality in a 38 year-old male, review the current literature with discussion of proposed causes, malignant potential, and management strategies. A 38-year-old white male presented with a 4-day history of chronic stable hemoptysis. ⋯ Defects in thyroid transcription factor 1 have also been proposed. With the increasing use and image resolution of ultrasound in modern obstetric practice, congenital pulmonary airway malformations rarely go undetected into adulthood. Management remains controversial; however, most authors agree with early surgical excision.
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Over the years a great deal of effort has been made to standardize all pulmonary function tests on adults. Many of the "rules" concerning the interpretation of the spirogram have been based entirely on adult observations. In the age of increasing conformity, and attempts to relate "adult" literature to the pediatric population, the latter was given much less emphasis than the former. ⋯ Lung function testing, particularly spirometry, has much to offer in the diagnosis of lung disease in children and the monitoring of response to therapy. With better standardization of pulmonary function testing in children, and more trained technologists, the age limits for testing can be extended to below 6 years of age and sometimes below 5. Also with better standardization the results obtained are meaningful and when interpreted in context of age offer excellent diagnostic information to better treat the child with lung disease.
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Spirometry is considered the primary method to detect the air flow limitation associated with obstructive lung disease. However, air flow limitation is the end-result of many factors that contribute to obstructive lung disease. One of these factors is increased airway resistance. ⋯ Furthermore, the FOT provides unique information about lung mechanics that is not available from analysis using spirometry, body plethysmography, or the interrupter technique. However, it is unclear whether any of these measures of airway resistance contribute clinically important information to the traditional measures derived from spirometry (FEV(1), FVC, and FEV(1)/FVC). The purpose of this paper is to review the physiology and methodology of these measures of airway resistance, and then focus on their clinical utility in relation to each other and to spirometry.
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Review Case Reports
What is the best pulmonary diagnostic approach for wheezing patients with normal spirometry?
Asthma is characterized by airway inflammation, airway hyper-responsiveness (AHR) and variable air flow obstruction. The diagnosis of asthma, however, is often based upon nonspecific clinical symptoms of cough, wheeze, and shortness of breath. Furthermore, the physical examination and measurements of pulmonary function are often unremarkable in patients with asthma, thereby complicating the diagnosis of the disease. The following discussion will review approaches to the diagnosis of asthma when lung functions are normal, and will largely focus on the use of bronchial provocation tests to detect underlying AHR.
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Pulmonary function testing is often considered the basis for diagnosis in many categories of pulmonary disease. Although most of the testing methodologies are well established and widely employed, there are still many questions regarding how tests should be performed, how to ensure that reliable data are produced, what reference values and rules should be used, and how pulmonary function tests (PFTs) should be interpreted to best support clinical decision making. This conference was organized around a set of questions aimed at many of these issues. ⋯ These topics included testing of adults and children, with concentration on important disease entities such as COPD, asthma, and unexplained dyspnea. Special emphasis was given to discussing reference values, lower limits of normal, interpretive strategies to optimize disease classification, and those factors directly affecting data quality. Established techniques for spirometry, lung volumes, diffusing capacity, exercise testing, and bronchial challenges were compared and contrasted with new technologies, and with technologies that might be part of pulmonary function laboratories in the near future.