The Quarterly journal of medicine
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The aetiology, clinical features and outcome of 40 patients presenting with Goodpasture's syndrome (glomerulonephritis with haemoptysis and pulmonary infiltrates) are reviewed. The diseases of the patients studied could be divided into three groups: antiglomerular basement membrane (anti-GBM) antibody-induced disease (7/40); systemic vasculitis (22/40) and idiopathic Goodpasture's syndrome (i.e. no systemic disease or anti-GBM antibody detected) (11/40). Overall mortality was 57.5 per cent (anti-GBM disease 4/7; systemic vasculitis 15/22; and idiopathic Goodpasture's syndrome 4/11). ⋯ Review of renal histology showed that all had proliferative nephritis, with 80 per cent of patients having more than 30 per cent crescents. Thus Goodpasture's syndrome was associated with a wide variety of underlying disease. It had a poor prognosis, with the degree of renal impairment at presentation, the extent of crescent formation and the nature of the underlying disease being the major determinants of outcome.
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Hypercalcaemia in six patients with sarcoidosis was associated with elevated circulating 1,25-dihydroxy vitamin D3 (187-475 pmol/l): the concentration of this metabolite of vitamin D was a function of the concentration of its precursor, 25-hydroxy vitamin D which remained within the normal range. Corticosteroids, in reducing serum calcium, eliminated this abnormal substrate--product relationship by rapidly reducing circulating 1,25-dihydroxy vitamin D3 while having no effect on 25-hydroxy vitamin D. ⋯ Studies on the clearance of exogenous 1,25-dihydroxy vitamin D3 indicated that during hypercalcaemic episodes, the plasma disappearance time of the sterol was delayed and that this reverted to normal with steroid therapy. Administration of vitamin D3 to these patients with sarcoidosis increased the circulating concentration of 1,25-dihydroxy vitamin D3 and this resulted in hypercalcaemia, thus accounting for their hypersensitivity to vitamin D and sunlight.
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Case Reports
Clinical and biochemical studies in Engelmann's disease (progressive diaphyseal dysplasia).
The clinical and biochemical features of four adults with Engelmann's disease (Camurati-Engelmann disease; progressive diaphyseal dysplasia) are presented. One young patient, with a particularly severe form of the disease, is discussed in detail. Biochemical abnormalities were found in three of the patients. ⋯ The most severely affected patients, who had had progressive and generalized bone disease from age two and a half years, also had persistent hypocalcaemia and hyperphosphataemia, a positive calcium balance, and a very low urine calcium excretion. It is suggested that some patients with Engelmann's disease may have a previously unrecognized metabolic disorder associated with increased retention of calcium and excessive bone formation. The possible role of abnormal phosphate metabolism in this increased formation of bone, and the relationship of Engelmann's disease to other hyperostoses, are discussed.
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The Saw-scaled or Carpet Viper (Echis carinatus) whose range extends from Senegal to Bengal probably bites and kills more people than any other species of snake. One hundred and fifteen patients with poisoning caused by its bite were studied in the savanna region of Nigeria, where victims of this snake may occupy 10 per cent of hospital beds. Patients showing no signs of envenoming were excluded. ⋯ Blood coagulability was restored in two to 39 (mean 12) hours after the first dose of antivenom. Immediate-type serum reactions were observed in 21 per cent of cases. Additional treatment included blood transfusion for patients in haemorrhagic shock and ealry surgical débridement of necrotic tissue at the site of the bite.
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In sixteen patients with upper airway obstruction, breathlessness was a symptom in all with maximum mid vital capacity flow rates in inspiration or expiration of 1-7 litres per second or less. With one exception, all these patients had stridor. The stridor was inspiratory in nine, expiratory in one and both inspiratory and expiratory in two. ⋯ The importance of a standard posture during serial measurements is emphasized. The ratio of forced expired volume in one second measured in millilitres, to the peak expiratory flow measured in litres per minute, was of limited value if differentiating upper from lower airway obstruction in these patients. It is concluded that upper airway obstruction is likely to become more common and that respiratory function tests, in particular the flow volume loop, play an essential part in the recognition and management of this problem.