Pulmonary dysfunction is not a well-recognized feature of facioscapulohumeral muscular dystrophy (FSHD). The aim of this study was to establish the prevalence and type of pulmonary and respiratory muscle dysfunction in FSHD. Sixteen patients with moderately advanced FSHD and 16 healthy controls were evaluated. Standard lung and respiratory muscle function tests were performed. Diaphragm muscle inspiratory action was evaluated with transdiaphragmatic pressure measurements. Lung function tests showed an increased residual volume in five patients. There was a significant difference in global respiratory muscle function in patients versus controls; weakness was mild, and it affected expiratory more than inspiratory muscles. There was no significant difference in the diaphragm inspiratory action of patients versus controls. The dystrophic process that underlies FSHD did not significantly involve the muscles of the diaphragm, but it caused mild global respiratory muscle weakness that affected expiratory more than inspiratory muscles. It is probably not necessary to routinely monitor respiratory muscle function in ambulant FSHD patients who lack symptoms or signs of respiratory impairment.

Thoracic abnormalities and respiratory muscle function were investigated in nine patients with rigid spine syndrome. A severe restrictive chest wall defect and limited mobility of the spine associated with clinically significant respiratory muscle weakness were present in all patients. Respiratory muscle strength and endurance were less than 60% of control values. Slight to moderate scoliosis was present in five patients and absent in four. Scoliosis appeared to have only a minor additional effect on respiratory muscle function. Six patients were emaciated, and one patient was underweight, but no relationship was seen between body mass index and respiratory muscle strength. Respiratory muscle function was more impaired in patients with hypoventilation than in normocapnic patients. Respiratory muscle involvement appears to be a significant feature of rigid spine syndrome, terminating in hypercapnic ventilatory failure in some patients.

Water-pipes (hookah, narghile, shisha, goza, hubble bubble and in South Africa hubbly-bubbly) have been used to smoke tobacco for more than 400 years. Traditionally in the Middle East water-pipe smoking has been the habit of older men, but it is increasing in popularity worldwide, especially among students, other young people and children. 1 The popularity of water-pipe smoking appears to be based on its social nature and the assumption that its effects are less harmful than those of cigarette smoking.2 The increase in water-pipe smoking can partly be attributed to the popularity of the sweetened and variously flavoured tobaccos that are used in the water-pipe.A water-pipe is essentially a glass bottle with hoses and a mouthpiece through which to smoke. 3 The head contains the tobacco, and since the tobacco is too moist to burn on its own, charcoal is placed on top of the tobacco-filled head. The body is fixed to the neck of the bottle, and its central conduit is submerged in the bottle. The bottle is half-filled with water and placed on the ground. The hose is attached to an aperture in the side of the pipe (Fig. 1). When the charcoal is lit, the smoker inhales through the hose. This creates a vacuum above the water, drawing air through the body and over the tobacco and charcoal. The smoke bubbles through the water, where it is diluted and cooled, and then inhaled by the smoker.Carbon monoxide (CO) is produced by the incomplete combustion of carbon-containing material, notably tobacco, biomass fuel and fossil fuel. The carboxyhaemoglobin (COHb) level in the blood (half-life 2 -6 hours) after inhalation of CO will depend on concentrations of inspired CO, duration of exposure, pulmonary ventilation and the COHb level before the inhalation. 4 COHb is a sensitive specific physiological marker of atmospheric CO exposure from both indoor and outdoor sources.In water-pipe smokers expired CO was reported to increase by 300% after an hour of smoking, while in cigarette smokers it only increased by 60%. 5During a single water-pipe smoking session a smoker may produce a 24-hour urinary cotinine level that is equivalent to smoking 10 cigarettes a day (95% confidence interval (CI) 7 -13 cigarettes a day).6 Contrary to popular belief, noxious substances such as nicotine, tar and heavy metals (chromium, arsenic, lead) are found in the smoke of water-pipes.7 Although water-pipe smoking is so widespread, its adverse health effects are not well documented. Data on CO levels in the blood after a controlled smoking session comparing water-pipe smoking with cigarette smoking are limited.We aimed to measure COHb levels in the blood before and after water-pipe smoking and cigarette smoking under controlled conditions. Materials and methodsOnly self-confessed smokers of cigarettes and water-pipes aged 18 years and older were recruited from students and hospital workers. There were no regular pipe smokers among Water-pipe smoking is growing in popularity, especially among young people, because of the social nature of the smoking sessio...

Background-Pulmonary involvement is frequently observed in patients with limb girdle muscular dystrophy and occurs early in the disease. The aim of this study was to establish the prevalence of pulmonary dysfunction; the type of dysfunction; and any correlation between patient age, disease duration, or limb weakness and lung or respiratory muscle dysfunction.Methods -Twenty patients with strictly delineated limb girdle muscular dystrophy and 20 healthy controls were evaluated. Full inspiration chest radiographs were obtained. Standard lung and respiratory muscle function tests were performed and the data were statistically analysed.