Diffuse pulmonary lymphangiomatosis treated with bevacizumab.

Diffuse pulmonary lymphangiomatosis (DPL) is a rare disease caused by uncontrolled lymphatic vessel proliferation resulting in respiratory dysfunction. Lymphatic vessel growth is influenced by vascular endothelial growth factor (VEGF). It has been shown that bevacizumab, a monoclonal antibody to VEGF type A, may be helpful in treating diseases characterized by excessive vessel proliferation. We report the case of a 51-year-old man with DPL treated with 1 mg/kg bevacizumab every three weeks for 6 months. A significant improvement in lung infiltrates was seen on post-treatment computed tomography (CT) chest with a 17.5% improvement in forced expiratory volume in one second (FEV1). The patient reported improved respiratory symptoms, and no significant adverse drug side effects were reported. The authors believe this is the first case of DPL to report lung function improvement [FEV1, forced vital capacity (FVC), and Diffusion Capacity for Carbon Monoxide (DLCO)] following bevacizumab therapy.

Evaluating the appropriateness of hospital doctors' requests for pulmonary function tests beyond basic spirometry: results from a prospective observational study.

OBJECTIVES: Hospitalists request 'complete' pulmonary function tests (PFTs), typically comprising of spirometry, diffusion capacity of the lung for carbon monoxide (DLCO) and absolute lung volumes (ALVs), the results of which assist in the management of patients with respiratory disorders. Recently, concerns have been raised about over-requesting of 'complete' PFTs, but there is a paucity of information on the proportion of requests that can be considered clinically inappropriate. This study prospectively evaluated the 'complete' PFTs requested in a hospital service and assessed the impact of medical review of the requests. METHODS: A six-month prospective study on requests to two teaching hospital PFT laboratories from non-respiratory doctors was undertaken. Requests at one laboratory underwent review by a respiratory doctor ('intervention laboratory') while requests at the second laboratory were not reviewed ('control laboratory'). The appropriateness of requests was measured against pre-specified criteria. RESULTS: PFT requests for 335 subjects were included in the study. In the intervention laboratory, 8 of 110 ALV and 122 of 134 DLCO requests fulfilled pre-specified criteria for appropriate test indications. Fewer ALV (7% vs. 100%, p < 0.001) and DLCO tests (91% vs. 100%, p = 0.031) could have been performed in the intervention laboratory compared to the control laboratory. CONCLUSION: A considerable proportion of 'complete' PFT requests from non-respiratory hospital doctors may be unwarranted. Using a simple screening method, the number of unnecessary PFTs could be reduced, resulting in substantial time and cost savings for hospital PFT laboratories.

Effect of changing from the National Health and Nutritional Examination Survey III spirometry reference range to that of the Global Lung Initiative 2012 at Gold Coast Hospital and Health Service.

The categorisation of lung disease into obstructive ventilatory defect (OVD) and tendency to a restrictive ventilatory defect (TRVD) patterns using spirometry is used to guide both prognostication and treatment. The effectiveness of categorisation depends upon having reference ranges that accurately represent the population they describe. The Global Lung Initiative 2012 (GLI 2012) has spirometry reference ranges drawn from the largest sample size to date. This study aimed to determine whether using spirometry reference ranges from the new GLI 2012 dataset, compared to the previously used National Health and Nutritional Examination Survey III (NHANES III) dataset, resulted in a change in diagnosis between OVD, TRVD and normal ventilatory pattern (NVP). Spirometry data were collected from 301 patients, aged 18-80 years, undergoing investigation at the Gold Coast Hospital and Health Service (GCHHS) throughout February and March 2014. OVD was defined as a forced expiratory volume in 1 second (FEV1) divided by forced vital capacity (FVC) less than lower limit of normal (LLN). TRVD was defined as FEV1/FVC ≥ LLN, FEV1 < LLN, and FVC < LLN. The LLN values were determined by equations from the GLI and NHANES datasets. Spirometry interpreted using the NHANES III equations showed: 102 individuals (33.9%) with normal spirometry, 136 (45.2%) with an OVD pattern, 52 (17.3%) with a TRVD pattern, and 11 (3.7%) with a mixed pattern. When the spirometry data were interpreted using the GLI 2012 equations 2 (0.7%) individuals changed from OVD to NVP, 2 (0.7%) changed from NVP to OVD and 14 (4.7%) changed from TRVD to NVP. Using the GLI 2012 reference range resulted in a change in diagnosis of lung disease in 5.9% of the individuals included in this study. This variance in diagnosis when changing reference ranges should be taken into account by clinicians as it may affect patient management.

Communication of Pulmonary Function Test Results: A Survey of Patient's Preferences.

INTRODUCTION: Physician-patient communication in patients suffering from common chronic respiratory disease should encompass discussion about pulmonary function test (PFT) results, diagnosis, disease education, smoking cessation and optimising inhaler technique. Previous studies have identified that patients with chronic respiratory disease/s often express dissatisfaction about physician communication. Currently there is a paucity of data regarding patient awareness of their PFT results (among those who have undergone PFTs previously) or patient preferences about PFT result communication. METHODS: We undertook a three-month prospective study on outpatients referred to two Pulmonary Function Laboratories. If subjects had undergone PFTs previously, the awareness of their previous test results was evaluated. All subjects were asked about their preferences for PFT result communication. Subjects were determined to have chronic respiratory disease based on their past medical history. RESULTS: 300 subjects (50% male) with a median age (± SD) of 65 (± 14) years participated in the study. 99% of the study participants stated that they were at least moderately interested in knowing their PFT results. 72% (217/300) of the subjects had undergone at least one PFT in the past, 48% of whom stated they had not been made aware of their results. Fewer subjects with chronic respiratory disease preferred that only a doctor discuss their PFT results with them (28% vs. 41%, p = 0.021). CONCLUSION: Our study demonstrates that while almost all subjects want to be informed of their PFT results, this does not occur in a large number of patients. Many subjects are agreeable for their PFT results to be communicated to them by clinicians other than doctors. Further research is required to develop an efficient method of conveying PFT results that will improve patient satisfaction and health outcomes.