Adult maltase acid deficiency myopathy: treatment with long-term home mechanical ventilation.

We report a study of an adult with a maltase acid deficiency myopathy. A restrictive respiratory syndrome due to respiratory muscle weakness is associated with paralysis of other muscular groups. In 1982 the patient presented with an alveolar hypoventilation, and mechanical ventilation was required after acute respiratory failure. The patient has received nocturnal mechanical ventilation by tracheostomy at home for 5 years. His clinical status gradually improved in parallel to amelioration of his respiratory condition. Functional respiratory tests improved: initial hypoxia-hypercapnia disappeared, vital capacity increased. The possible mechanisms underlying the improvement are discussed. Increase in pulmonary compliance is an argument to explain the functional improvement observed. Ventilatory response to carbon dioxide was abnormal whereas the ventilatory response to exercise and maxima minute ventilation test were normal. Results are consistent with a respiratory control impairment. The role of mechanical ventilation is difficult to assess in the improvement we observed.

[Chronic pulmonary artery thrombosis. Therapeutic modality. Apropos of 6 cases]. / Thromboses artérielles pulmonaires chroniques. Modalités thérapeutiques. A propos de 6 observations

Six cases of post-embolic chronic cor pulmonale are presented. All six were treated with thrombolytic agents (4 with streptokinase, 2 with urokinase), and in only one case was improvement maintained at the end of one year. Two patients underwent a disobliterative procedure of the pulmonary artery, together with ligation of the inferior vena cava. One of these operations was unsuccessful, and the other had a successful outcome, as confirmed by objective assessment with angiography, scintigraphy and haemodynamic studies. The literature is reviewed at this stage. It was found that the fibrinolytic agents had some chance of working only if the condition was less than a few months old. One major drawback to surgical disobliteration that cannot be foreseen before operation is the presence of thromboses at the arteriolar level in subjects whose main arteries are already blocked proximally. Nervertheless it is possible to obtain good results by surgery, and the operation is worth attempting in young subjects, given the poor prognosis of the untreated condition.

[Auto-immune hypoglycemic syndrome induced by pyritinol]. / Syndrome hypoglycémique auto-immun induit par le pyritinol.

The auto-immune hypoglycemic syndrome is characterized by the association of hypoglycemia (clinical and/or biological) and anti-insulin antibodies in patients who have never received exogenous insulin. Initially this syndrome was most often described in Japanese patients some of whom were treated with drugs containing a sulfydril group. We now recall the case of a female caucasian patient treated with Pyritinol for rhumatoid polyarthritis and who presented severe spontaneous hypoglycemia linked with the presence of anti-insulin antibodies in her serum. The level of her antibodies decreased abruptly on suspension of the drug. The recent and more developed characterization techniques of the different forms of circulating insulin and of their antibodies may help to differenciate an auto-immune hypoglycemia from hypoglycemia due to the secret auto-administration of bovine and porcine insuline, and permit us to suggest that an abnormality in the structure of the molecule of insulin might be a cause of this syndrome. However, the exact mechanism of hypoglycemia linked with the presence of anti-insulin auto-antibodies is not yet clear as is the predisposition of a drug with a sulfydril group to induce such an auto-immune phenomenon.

[Churg-Strauss syndrome. Its nosological place. Apropos of a new case diagnosed before death]. / Syndrome de Churg et Strauss. Place nosologique. A propos d'un nouveau cas diagnostiqué pré-mortem

Allergic granulomatous angeitis or Churg and Strauss disease, seems to form part of the disease of necrotising angeitis. Clinically, it may be considered when periarteritis nodosa is associated with asthma and marked eosinophilia. The diagnosis depends on the pathological findings including the venous and pulmonary lesions, giant cells and the presence of extra-vascular granulomas. The recognition of this disease does not, however, permit one to give a precise prognosis. In the absence of progress concerning the pathogenesis, treatment will be the same as that given in other forms of necrotising angeitis.