Hypoxaemia during chest physiotherapy in patients with cystic fibrosis.
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Biomedical subjects
Publications and source records attributed to M X FitzGerald.
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A localized area of hypertransradiance often leads to surgical referral. Among 608 cases, 115 were due to local lesions of airways, blood vessels, or parenchyma. Among the remaining 493 with bullae from diffuse emphysema, 21% underwent surgery. Good restoration of function occurred in patients with rapidly progressive dyspnea who did not have a bronchitic component, recurrent infections, or CO2 retention. Physiologically, preoperative findings suggestive of tension pneumothorax, including severe restriction, marked air trapping, and little ventilation/perfusion mismatch suggested good results. Favorable radiographic findings included well-defined, large air spaces without stigmata of diffuse emphysema, serial films showing rapid enlargement of bullae, and expiration films with good thoracic motion and obscuration of lung around bullae. Compressed but otherwise intact lung was best demonstrated by angiography and CT scans. Palliative bullectomy in severe diffuse emphysema sometimes had gratifying clinical results. Resection of small bullae never caused improvement. Localized giant bullae most often were associated with paraseptal or periacinar emphysema, and the best surgical results were obtained in this group.
We report a patient with both sarcoidosis and Crohn's colitis, an association not previously documented. We review the literature and discuss the areas of overlap in these two conditions.
Progression of lung fibrosis induced in rabbits by intratracheal bleomycin (BLM, 10 mg/kg) was monitored by biochemical and morphological measurements. These indicated that the evolution of fibrosis in the rabbit was slower than in other experimental animals. Histology revealed cellular and fibrocellular lesions 4 weeks after BLM. At 8 weeks these lesions still predominated, indicating continuing active disease accompanied by a progression to fibrosis. The gradual progression of the response was reflected in alterations in lung composition. Changes in lung content of collagen, protein and DNA were evident at 8 weeks after BLM, at which time concentration (micrograms/mg dry weight) of collagen and protein were also elevated (P less than 0.05). Plasma angiotensin converting enzyme (ACE), a marker of endothelial injury, decreased 1 week after BLM (P less than 0.01) and then returned to normal, indicating that endothelial injury does not parallel the fibrotic response. In summary, the continuing active inflammation and slow progress of fibrosis induced by i.t. BLM in the rabbit suggests that this model has advantages over others for the serial study of the cellular and biochemical evolution of lung fibrosis.
Steroids are used in progressive lung sarcoid despite arguments that spontaneous remission occur and therapy may not alter outcome. We studied a unique group of 6 patients with dyspnea and advancing pulmonary sarcoid, who had documented untreated progressive disease for 6.8 +/- 2.4 years. Raised SACE, Ga 67 lung uptake, and lymphocyte counts in lung lavage fluid indicated continued active alveolitis. After 3-6 months on steroid, MRC dyspnea grade fell from 2.5 to 0.3 and FVC, FEV, and DLCO increased by 36%, 27% and 16% respectively. This was associated with a fall in small opacity profusion scores on x-ray lung uptake of Ga 67 and serum ACE. These improvements were sustained for the duration of follow up (mean 22 months). These data show that steroids can alter the natural history of progressive sarcoid and reversible alveolitis may coexist with established fibrosis.
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This study reports serum 25-hydroxy vitamin D (25-(OH)D) levels, bone mineral content and bone maturation in 20 adolescent and adult patients with cystic fibrosis, and their response to the internationally recommended dose of supplementary vitamin D (800 iu/day; 20 micrograms/day). Serum 25-(OH)D values were below normal in 75 per cent of patients and serum alkaline phosphatase values, corrected for age, were increased in 60 per cent. Bone mineral content, measured by photon beam absorptiometry, was below the normal range in 45 per cent of patients and bone age retarded in 45 per cent. Following supplementation with vitamin D 40 per cent of patients failed to achieve normal serum 25-(OH)D levels. We concluded that hypovitaminosis D occurs frequently in older patients with cystic fibrosis and is accompanied by osteopenia and retarded bone maturation.
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Acute upper airway obstruction is a potentially life-threatening event. The most common causes include foreign body inhalation, thermal injury, inflammation, angioedema and trauma. Airway obstruction caused by submucosal extension of subcutaneous emphysema has only been previously reported once. We report the case of a patient who suffered a respiratory arrest as a result of hypopharyngeal and laryngeal swelling associated with massive subcutaneous emphysema.
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A 14-year-old girl whose sole presenting features were symptoms of pulmonary embolism, was found to have bi-atrial myxomas. The diagnosis was made preoperatively, and the patient had a successful outcome following elective surgery. The absence of other features such as systemic embolism, atrioventricular valvular obstruction, fever and constitutional symptoms makes this a most unusual case. Right atrial myxoma should be considered in any patient presenting with pulmonary embolism for which there is no obvious source.
Treatment with thioridazine resulted in hyperpyrexia and ventricular tachycardia in a young woman who was later shown to have thyrotoxicosis. The reasons why hyperthyroidism may enhance thioridazine toxicity are reviewed.
A case of lymphocytic lymphoma in a patient with coincidental sarcoidosis is described, and the possible relationship of the two conditions is discussed. A revised set of diagnostic criteria is proposed to overcome previous difficulties encountered in validating such dual pathology.
The history of surgery for the treatment of emphysema and its complications is long and complex and, with improved understanding of the pathophysiology, most procedures have been discarded. Present clear indications for surgery are mainly large or increasing bullae that result in compression of apparently good lung tissue, and complications of bullous emphysema such as pneumothorax and infection. With localized giant bullae the results of local resection can be as dramatic as the effects of pleural drainage for tension pneumothorax. Lobectomy should not be undertaken until bullae have been removed locally and application of positive pressure has failed to reveal any expandable lung tissue--a rare situation. Resection of small bullae generally has no effect on lung function. The indications for resection of large bullae in the presence of diffuse emphysema require careful individual study. In such cases even a small increment of function may be of great clinical benefit, and there is evidence that longevity can be increased. Finally, two-stage local drainage of tension bullae may be indicated in those rare cases in which open thoracotomy cannot be considered for other reasons.
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