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Query: UMLS:C0231807 (exertional dyspnea)
 3,402 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The syndrome resulting from combined pulmonary fibrosis and emphysema has not been comprehensively described. The current authors conducted a retrospective study of 61 patients with both emphysema of the upper zones and diffuse parenchymal lung disease with fibrosis of the lower zones of the lungs on chest computed tomography. Patients (all smokers) included 60 males and one female, with a mean age of 65 yrs. Dyspnoea on exertion was present in all patients. Basal crackles were found in 87% and finger clubbing in 43%. Pulmonary function tests were as follows (mean+/-sd): total lung capacity 88%+/-17, forced vital capacity (FVC) 88%+/-18, forced expiratory volume in one second (FEV1) 80%+/-21 (% predicted), FEV1/FVC 69%+/-13, carbon monoxide diffusion capacity of the lung 37%+/-16 (% predicted), carbon monoxide transfer coefficient 46%+/-19. Pulmonary hypertension was present in 47% of patients at diagnosis, and 55% during follow-up. Patients were followed for a mean of 2.1+/-2.8 yrs from diagnosis. Survival was 87.5% at 2 yrs and 54.6% at 5 yrs, with a median of 6.1 yrs. The presence of pulmonary hypertension at diagnosis was a critical determinant of prognosis. The authors hereby individualise the computer tomography-defined syndrome of combined pulmonary fibrosis and emphysema characterised by subnormal spirometry, severe impairment of gas exchange, high prevalence of pulmonary hypertension, and poor survival.
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PMID:Combined pulmonary fibrosis and emphysema: a distinct underrecognised entity. 1645 4

A nationwide epidemiological survey of idiopathic interstitial pneumonias (IIPs) in 2005 was performed using clinical personal records. According to the data from the Japan Intractable Diseases Information Center, 4,396 patients with IIPs were covered by public insurance. The prevalence of IIPs in Japan was estimated to be 3.44 per 100,000. The forms of 1543 patients (new: 658, recurrent: 885) were collected. Of 1543 cases, 1322 cases (85.7%) had idiopathic pulmonary fibrosis (IPF), therefore details were investigated in patients with IPF. The mean age at onset was 65.4 years and men were more frequent than women (878 in men, 444 in women). The severity, diagnostic method, symptoms, imaging findings, and pulmonary function tests were analyzed in the new forms. Severity levels of I, II, III and IV were seen in 32, 28, 177, and 287 cases, respectively. A pathological diagnosis was made in 67 cases (12%). The positive rates of fine crackles, dry cough, exertional dyspnea, and finger clubbing were 98%, 94%, 98%, and 53%, respectively. On HRCT, honeycomb was noted in the subpleural lung lesions in more than 90% of the cases. As for pulmonary function tests, restrictive and diffusing capacity impairment were noted in 86% and 92%, respectively, and SpO2 was below than 90% in 91% on a 6-minute walking test. The serum KL-6 level was elevated in 95%. Most of the IPF analyzed cases were at a severity level of III or IV because the analysis aimed at those coverage by public insurance. Since this was only the initial nationwide epidemiological survey in Japan, the current situation should be investigated.
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PMID:[Nationwide epidemiological survey of patients with idiopathic interstitial pneumonias using clinical personal records]. 1801 23

Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease, generally treated with total correction within the first two years of life. Occasionally, some unoperated cases can reach older ages. A 68-year-old woman with diabetes mellitus presented with swelling in legs and abdomen, weakness, exertional dyspnea, and orthopnea. On physical examination, she had mild cyanosis with clubbing. Her blood pressure was 110/60 mmHg and pulse rate was 79 beat/min. She had a systolic ejection murmur and bilateral rales on basal lung areas. Massive edema was noted in both lower limbs. Electrocardiography showed atrial fibrillation with normal ventricular response. Chest radiography showed an increased cardiothoracic ratio and bilateral minimal pleural effusion. Echocardiography showed biatrial dilatation (right atrium 62 mm, left atrium 49 mm) and thickening of left ventricular walls. There was right ventricular hypertrophy with decreased systolic function. A very large ventricular septal defect and severe pulmonary stenosis were noted. The patient did not accept any interventional procedure. To our knowledge, this is the oldest unoperated TOF case reported from our country.
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PMID:Unoperated tetralogy of Fallot in a 68-year-old patient. 1862 10

A congenital fistula between the right pulmonary artery (RPA) and left atrium (LA) is a rare condition that results in central cyanosis. An 11-year-old boy was admitted with exertional dyspnea and easy fatigability. He had severe cyanosis of the lips and limbs with clubbing of the fingers. Systemic oxygen saturation was 70%. There was no abnormal finding on electrocardiography, chest radiography, and echocardiography. Agitated saline injection showed early appearance of contrast bubbles in the LA. A pulmonary arteriovenous fistula was suspected and diagnostic cardiac catheterization was performed. Angiography demonstrated a large fistula between the proximal RPA and LA. The narrowest part of the fistula was 13.8 mm in balloon sizing. A 14-mm Amplatzer septal occluder was deployed at the narrowest site; however, the device migrated to the LA and then to the aortic arch. The device was removed and was successfully reimplanted to the fistula. After the procedure, arterial oxygen saturation increased from 70% to 96% and control angiography demonstrated complete occlusion of the fistula. The patient was symptom-free on follow-up evaluations at 6, 12, and 18 months, with a mean oxygen saturation of 96%. This case represents the first pediatric patient in whom a septal occluder was used.
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PMID:Transcatheter closure of a fistula between the right pulmonary artery and left atrium using the Amplatzer septal occluder. 2153 1

Pulmonary arteriovenous malformation (PAVM) is a rare condition in which there is abnormal connection between pulmonary arteries and veins. The disorder usually appears in late childhood or early adult life, with dyspnea on exertion, clubbing or cyanosis. We present two patients with severe cyanosis and their work-up to diagnosis of PAVM, as a rare cause of cyanosis in childhood.
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PMID:Congenital pulmonary arteriovenous malformation: a rare cause of cyanosis in childhood. 2153 21

About 673 small-scale asbestos mining and milling facilities and 33 large - scale asbestos manufacturing plants, (17 asbestos-cement product manufacturing plants and 16 other than asbestos-cement product plants) are situated in India. The present study reveals the exposure of commercial asbestos (chrysotile) in the occupational as well as ambient air environment of the asbestos-cement (AC) sheets industry using membrane filter method of Bureau of Indian Standards (BIS). The fibre concentrations in 15 samples collected in the occupational environment at ingredient feeding site, sheet-producing site, fibre godown were 0.079, 0.057 and 0.078 f/cc, respectively and in five samples from surrounding ambient air at factory gate resulted fibre concentration of 0.071 f/cc. All the samples have shown fibre concentration lower than the threshold limit values (TLVs) prescribed by BIS. Morphological analysis of samples, further under phase contrast and polarized microscopy indicates the presence of chrysotile asbestos, which acts as carcinogen as well as co-carcinogen. A clinical examination of exposed subjects reveals that there was no case of clubbing, crepitation, ronchi and dyspnea on exertion; however, obstruction and restriction were 10.9 per cent and 25 per cent in exposed subjects, respectively while in control there were 12 per cent and 28 per cent, respectively. The study revealed that chrysotile asbestos is emitted in the occupational as well as ambient environment that may cause adverse health impact.
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PMID:Environmental health survey in asbestos cement sheets manufacturing industry. 2195 67

Idiopathic pulmonary fibrosis (IPF) is a differentiated disease within the idiophatic interstitial pneumonias. IPF is progressive and fibrosing and is limited to the lungs. This entity generally affects persons older than 50 years old and is associated with the radiological and/or histological pattern of usual interstitial pneumonia (UIP). Clinically, IPF causes progressive exertional dyspnea and nonproductive cough. In most patients, physical examination reveals fine bibasilar inspiratory crackles and 50% of patients have digital clubbing. There are no specific laboratory alterations. Bronchoalveolar lavage and transbronchial biopsy will not establish the diagnosis of IPF but are useful to exclude other entities. Definitive diagnosis requires: a) exclusion of other, defined clinical entities or diffuse pulmonary diseases of known cause, and b) the presence of a histological pattern of UIP on analysis of pulmonary tissue from surgical biopsy, radiological evidence of the defined pattern of UIP on high-resolution computed tomography, or both.
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PMID:[From exclusion to uncertainty: the route to diagnosing idiopathic pulmonary fibrosis]. 2311 2

Pulmonary arteriovenous malformations are rare vascular anomalies which rarely present in childhood. We discuss a nine-year-old male child who presented with the classical triad of exertional dyspnea, cyanosis and clubbing since six years of age. Pulmonary arteriovenous malformation was suspected on chest radiography and was later confirmed on computed tomography scan. A bubble contrast echocardiography showed the presence of an extracardiac shunt. Pulmonary angiography was done to delineate the anatomy of the pulmonary vasculature and to plan embolization. The child has undergone two sittings of transcatheter coil embolization with improvement in oxygenation.
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PMID:An unusual cause of central cyanosis in a nine-year-old boy. 2329 35

Direct communication of the right pulmonary artery and the left atrium is an extremely rare congenital malformation of the pulmonary vasculature. A 41-year-old woman with a history of cyanosis since childhood presented with mild exertional dyspnea. On physical examination, she had central cyanosis, clubbing of the fingers, and an upright position caused by orthodeoxia. Imaging studies showed a very large aneurysm in the distal right pulmonary artery with a direct communication to the left atrium. The patient underwent successful repair, with resolution of hypoxia and exertional symptoms.
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PMID:Unexpected cause of cyanosis and dyspnea in an adult: direct communication of the right pulmonary artery and left atrium. 2479 66

Idiopathic Pulmonary Fibrosis (IPF) is relentless progressive interstitial lung disease (ILD) of unknown etiology. Main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Majority of IPF patients have been elderly men with smokers. However, there are important differential diagnosis such as fibrotic non-specific interstitial pneumonia (NSIP), Connective Tissue Disease (CTD) associated ILD, chronic hypersensitivity pneumonia (CHP). Clinical point of view, non-productive cough and progressive exertional dyspnea are main symptoms. In addition, scalene muscle hypertrophy, fine crackles and finger clubbing are key findings. Serum marker such as lactate deydrogenase (LDH), Krebs von den Lungeng-6 (KL-6) are sensitive for ILD detection and activity. Pulmonary function test and 6 minute walk test (6MWT) are quite meaningful physiological examination. Serial change of forced vital capacity 6MWT distance predict mortality of IPF. International IPF guideline published recently and highlighted on the importance of high resolution computed tomography (HRCT) findings. Key findings of IPF are honeycombing, traction bronchiectasis and subpleural reticular opacity. IPF is chronic progressive disease. Therefore, tracing disease behavior is crucial and unifying clinical, physiological, imaging information over time provide useful information for physicians.In management, many candidate agent failed to have positive result. Pirfenidone which is anti-fibrotic agent showed to slow the decline of vital capacity and prevent of acute exacerbation. Molecular agent such as nintedanib is promising agent for prevention of progression of IPF. In this review, we review the clinical information of IPF and IPF guideline. Lastly, we show the clinical algorithm of this devastated disease.
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PMID:Practical management of Idiopathic Pulmonary Fibrosis. 2627 87


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