Biomedical subjects
Publications and source records attributed to J Saint-Martin.
The authors report on a case of mesenteric fibromatosis in a 5 month-old infant. Surgical excision was available and the clinical course was good, without recurrence after 30 months. Clinical and histological features of mesenteric desmoïd tumor in children are discussed and diagnostic difficulties are examined in depth.
Major macrocytosis seems to be suggestive of triploidy in dysmature infants with multiple malformations. This marker may prove ethically useful since its presence may justify withholding special therapies until results of the karyotype are obtained.
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The authors report the exceptional case of a cerebral neuroblastoma responsible for intracranial hypertension at birth. In spite of neurosurgical intervention on the seventh day the newborn died. Histological and immunohistochemical study with Protein S 100 and "anti LEU 7" are consistent with the diagnosis of neuroblastoma.
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Esophageal pH was systematically recorded for 3 hours in 30 neonates under 1,700 g, of which 18 were under assisted ventilation. Gastric acid secretion occurs early. Varying degrees of gastroesophageal reflux were observed in 21 neonates. Positioning in the prone position at 30 degrees allowed for the disappearance of reflux in 19 and an improvement in the remaining 2. We recommend the adoption of this position in all premature infants including those under assisted ventilation.
The authors report a case of giant hemangioma with thrombocytopenia, whose very important increase was responsible for acute congestive heart failure with pre-renal failure. In order to limit the tumoral volume, a compressive dressing was applied which had to be removed at first, as it had respiratory and hemodynamic consequences. A dressing was reapplied, and with concomitant diuresis obtained a dramatic improvement. The volemic changes observed in this case are discussed.
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On the occasion of a case of Gaucher's disease associated with portal hypertension and after reviewing similar cases in the literature, the following conclusions can be proposed: portal hypertension is a very rare complication of Gaucher's disease. An intrahepatic block or, more rarely, a constrictive pericarditis should be discussed. Splenectomy, which could be responsible for postoperative portal thrombosis, should be carried out only in patients with severe hypersplenism. In cases with proven portal hypertension, the indication for portacaval shunting should take into account the peculiar circumstances related to Gaucher's disease.
A 17-year-old patient with insulin-dependent diabetes since the age of 2 developed limitation in the range of joint movements (wrist, interphalangeal, metacarpophalangeal, elbows, knees) which progressively worsened. It is important to seek such articular complications which would seem to be common and probably underestimated in terms of their importance regarding the professional future of these patients. Associated dwarfism (as seen in our own patient) would seem to be rare. The aetiopathogenesis remains hypothetical. It is only by verification of these hypotheses, in particular that of the possibility of increased polymerisation of collagen, that therapeutic deductions (d-penicillamine) may be envisaged.
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A new lethal disorder affecting two brothers is described. The features were curvature of the long bones, multiple fractures, dysmorphic facies, syndactyly and absence of ossification in the cranial vault. The condition could be inherited as an autosomal recessive or sex linked condition.
Two new cases of ventricular tachycardia (VT) in the infant are reported, and reviewed in the light of the 23 case histories found in the literature. The diagnosis rests upon eliminating a pre-excitation syndrome, which is so common in this age group. The VTs found in infants are rapid, irregular, and take many different forms. They often necessitate urgent treatment with electric shocks. Preventive treatment consists of a combination of procainamide and beta-blockers in relatively large doses. The search for an aetiological agent should include a haemodynamic and angiocardiographic study of all the chambers of the heart to exclude cardiomyopathy, tumours, papyraceous right ventricle and congenital heart defects. Where no cause can be demonstrated, preventive treatment should be given, with regular electrical testing and other follow-up investigations. An attempt to reduce the drug dosage should be made every 6 months, in hospital. In cases which prove resistant despite adequate treatment, it seems justifiable to carry out a pericardial exploration with the aim of diathermising the ectopic focus; this approach is suggested because of the poor natural history of this type of case.
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Explore the source record for details and available documents.