[Letter: Quadruple paraneoplastic endocrine syndrome in primary bronchial cancer].
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Biomedical subjects
Publications and source records attributed to J Marsac.
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Paraneoplastic hypercalcemia is frequently observed in squamous cell carcinoma of the bronchus. Clinically, apart from the general symptoms which are always very marked, neurological signs are the most common. This hypercalcemia, accompanied by hypophosphatemia, is of sudden onset and immediately very high. Metabolic alkalosis, with hypokalemia and hypochloremia, differentiates it from true hyperparathyroidism. Symptomatic treatment is only transiently effective. However, the obvious effect of mitramycin may be useful before surgical operation, which alone produces a lasting normalisation of serum calcium. Inappropriate secretion of a parathormone substance by the tumour may be demonstrated by radio-immunoassay, and this explains a large number of cases of hypercalcemia but this does not exclude other possible factors in the etiology. Finally, hypercalcemia originally attributed to bony lysis, may be explained in some cases by a paraneoplastic process.
Three cases of pulmonary lymphangiomyomatosis are described, with emphasis on the ultrastructural changes. The clinicopathologic features corresponded to those previously described. Each patient was a female in the reproductive years; breathlessness and recurrent pneumothoraces were the predominant clinical features. Histologically, the lungs showed a focal interstitial infiltrate of short, spindle-shaped mononuclear cells compatible with primitive smooth muscle, which was associated with irregular emphysema and hemosiderosis. Electron microscopy confirmed the smooth muscle nature of the pulmonary infiltrate and showed the presence of cells intermediate between smooth muscle and fibroblasts. Abnormalities were also noted in the pulmonary connective tissue that are possibly related to the fragility of the lung in this condition.
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A classical definition of Pickwickian syndrome associates alveolar hypoventilation, obesity and hypersomnia with periodic breathing. Obesity in itself is enough to explain the alveolar hypoventilation and some of the sleep disorders, but in fact all three elements of this syndrome are intricated. Obesity, whether associated with a Pickwickian syndrome or not, affects ventilatory mechanics similarly. With the cause of sleep disorders are associated central ventilatory pauses, appearing periodically followed by phases of apnoea secondary to buccopharyngial hypotony. The alveolar hypoventilation is therefore the consequence of obesity and periodic apnoea. It also results from a low respiratory frequency considering that the tidal volume is also decreased. These different elements suggest some disorder of the centers controling ventilation. One can describe different nosological forms, all having as a common factor hypersomnia with periodic respiration, the latter being a determining factor in the diagnosis. Therapy, including weight reduction and symptomatic treatment of alveolar hypoventilation, is now augmented by new drugs acting on the central nervous system.
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