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Hospital case notes and chest radiographs of 100 patients with Marfan syndrome were investigated for evidence of pulmonary disease. The criteria for inclusion of details of a given patient in the study were the occurrence of Marfan abnormalities in at least two separate body systems (skeletal, cardiovascular, ocular) or in one body system where there was a family history of a classically affected first degree relative. Selection of cases was biased towards those with cardiorespiratory problems by virtue of the hospitals from which the patients were drawn. Forty eight patients underwent cardiac surgery. Eleven patients had a history of spontaneous pneumothorax, which had been recurrent in 10 cases and bilateral in six. Eight had had pneumonia or excessively frequent respiratory infections and two had bronchiectasis. Chest radiographs showed emphysematous bullae in five, upper lobe fibrosis in four, and aspergilloma in two. The cases reviewed together with other published evidence suggest that spontaneous pneumothorax and bullae are causally related to Marfan syndrome. The presence of idiopathic upper lobe fibrosis in four Marfan patients is interesting but provides insufficient evidence to assess possible causality. [ABSTRACT FROM PUBLISHER]

In patients with radiological evidence of pulmonary emphysema the bronchodilator drugs fenoterol and ipratropium bromide produced a considerable increase in vital capacity and reduction in residual volume. The response to fenoterol was virtually complete 15 minutes after administration, but after ipratropium bromide vital capacity was still increasing at 60 minutes. The change in vital capacity was slightly greater with a combination of the two drugs than with either used alone. Changes in FEV1 and peak flow rate were small. [ABSTRACT FROM PUBLISHER]

Fifty-six male patients with pulmonary emphysema and normal serum alpha 1-antitrypsin were followed for a minimum period of three years (range 3-13 yr) to observe the effects of the radiological distribution of emphysema on the annual rate of change of lung function indices. The subjects were placed in three categories--upper zone (UZ), lower zone (LZ), and generalised (G). The influence of smoking was taken into account with subjects in each radiographic category being divided into ex-smokers (group EX-S) and continuing smokers (group S). The rate of decline of FEV1, VC, TCO, and KCO was greater in group S than EX-S in all three x-ray categories. In group S, FEV1, VC, TCO, and KCO declined significantly faster in UZ emphysema than in G emphysema. In group EX-S, FEV1, TCO, and PaO2 declined significantly faster in UZ than G emphysema but VC remained unaltered. KCO and PaCO2 did not change significantly in any radiographic category in either group S or EX-S. Patients with G and LZ emphysema underwent very similar physiological changes over the course of time. Those with UZ emphysema, however, complained of exertional dyspnoea at an earlier age; a number of their lung function indices deteriorated at a faster rate than in patients from the other two categories, suggesting that UZ emphysema is a different disease entity. [ABSTRACT FROM AUTHOR]

Forty-four adult patients with acute severe asthma were studied on admission to a general hospital to determine the mode of presentation and clinical severity of the acute illness. Most patients described poorly controlled wheezing for a mean period of five weeks before a more rapid deterioration, usually over 24 hours, caused them to seek medical attention. The clinical severity of the acute attach was not related to the duration of acute wheezing before admission to hospital. Recovery, studied in 11 of the patients, was slow, and most had reached only 70% of their predicted spirometric values after seven days' treatment in hospital. The rate of recovery was not influenced by the antecedent history of acute wheezing. Very sudden deterioration from a background of good control appears to be uncommon in adults with asthma who present to hospital as emergencies, and it is likely that improvement in the standard of routine management of asthmatics at home would prevent many such admissions. [ABSTRACT FROM PUBLISHER]

Sixteen patients with intrinsic non-atopic asthma with persistent wheezing who had never been treated with corticosteroids showed normal adrenocortical responses to prolonged stimulation with Tetracosactin Depot. In a subgroup of six patients the hypothalamo-pituitary-adrenal (HPA) response to a standard insulin stress test was normal. It is concluded that impaired responsiveness of the HPA axis is unlikely to be a common factor in initiating or maintaining airways obstruction in patients with intrinsic asthma. [ABSTRACT FROM PUBLISHER]