Pediatrie最新文献

Whereas clinical and preventive aspects of father-daughter incest are now better-known, mother-child incest remains mysterious. Reporting four recent cases, the authors attempt to describe its main clinical features, particularly those which could allow earlier diagnosis. An original "constructive" therapeutic approach of the most severely mentally disturbed children among these victims is then developed: the emphasis is put on how medical, social and legal procedures might be coordinated.

Three cases of diffuse subcortical gray matter heterotopias in children are reported. Generalised seizures and mild mental retardation were the most frequent signs. No specific electroencephalographic pattern was recognized. Magnetic resonance imaging scans showed the thick diffuse layer of heterotopic gray matter which was surrounded by normal white matter. Gyration was normal, and no associated malformation was observed. This neuronal migrational disorder happens between the 10th and 16th gestational week. Nineteen observations (17 girls) are reported in the literature. The filiation with agyria-pachygyria and the possible genetic transmission are discussed.

Cervical tumors of thymic origin are considered to be uncommon lesions in the differential diagnosis of neck masses. They can be either cystic or solid. The authors present one case of solid cervical thymic tumor in a 2 month-old baby. The embryogenesis of the thymus explains the cervical location of these tumors. Theories of physiopathology are presented. The clinical presentation is variable and their nature is often recognized only upon surgery and preoperative pathologic examination. Some cases of thymoma and respiratory complications resulting from ectopic thymus have been described in the literature, so that total excision of the mass must be performed. This lesion may be more common than suggested in the literature.

We report two cases of localized tracheomalacia, one associated with esophageal atresia, and one isolated. The pathophysiology explains that the symptoms are more important during or shortly after eating, the alimentary bowl crushing the trachea against the aorta, or the innominate artery. The exact cause of tracheomalacia is unknown. Esophageal atresia is frequently associated. The tracheal compression is more often due to the innominate artery, because its origin is located on the left side of the trachea in infants. The aorta or a vascular anomaly are rarely implicated. The symptoms of tracheomalacia are largely due to airway obstruction during expiration: stridor, baking cough, and the life-threatening "dying spell". For diagnosis, the endoscopy is the most important investigation. Among the many methods of treatment which have been proposed, the aortopexy appears to be the technique giving the best results. A single acute apneic attack is an absolute indication for surgery. It is also important to rule out severe gastroesophageal reflux, which can produce the same symptoms.

In a multicentre trial we compared the clinical efficacy of amoxicillin/clavulanate used as a single-agent therapy with that of the three-agent combination usually prescribed in the post-operative period for appendicular peritonitis in children. Only bacteriologically documented peritoneal infections were included. Sixty-four patients were randomly distributed between two groups: Group A (29 cases) treated with amoxicillin/clavulanate, first administered iv (100 mg/kg/d), followed by conversion to the oral route (50 mg/kg/d) once the patient had been afebrile for 48 hours; Group B (35 cases) first treated by the iv route with benzylpenicillin (100,000 IU/kg/d) plus netilmicin (5 mg/kg/d) plus metronidazole (30 mg/kg/d), followed by conversion to the oral route for metronidazole (30 mg/kg/d). In both groups, the total duration of parenteral and oral treatment was not less than 5 days. One hundred and seventy nine bacterial strains were recovered from peritoneal fluid samples obtained during surgery; 86% of these were sensitive to amoxicillin/clavulanate. Clinical efficacy, assessed on the basis of time until return to normal temperature and gut transit and duration of hospitalization, was identical in both groups, with follow-up monitoring on day 30 showing recovery in all cases. Cure was obtained without any problems of infection in 25/29 patients in group A and in 34/35 patients in group B (non significant difference). Tolerance was excellent and identical in the two groups with the exception of three cases of thrombophlebitis which occurred in group B. The results of this study suggest that amoxicillin/clavulanate may be useful as single-agent therapy as a first-line curative treatment for appendicular peritonitis in children.

Imerslund-Najman-Grasbeck disease is a rare inherited megaloblastic anaemia secondary to a selective malabsorption of vitamin B12 by ileal enterocytes. The authors report on a 4 year-old tunisian girl who presented as visceral infantile leishmaniasis because of huge splenomegaly and major anaemia. The diagnosis of Imerslund disease was performed on the basis of the association of typical megaloblastic cells in the marrow, permanent proteinuria and favourable outcome under parenteral B12 administration. In addition, ther were no folate deficiency, no anti-intrinsic factor antibodies and no intrinsic factor deficiency. The outcome of the disease is always favourable if parenteral administration of vitamin B12 maintained.

The authors report a case of aortic arch thrombosis in a neonate probably related to perinatal placental emboli. However the finding of a fibroblastic endarteritis raised the possibility of a primitive lesion of the aortic wall. The evolution was complicated by cerebral ischemia. A surgical thrombectomy was performed. The outcome was favourable with persistence of a moderate right motor deficiency at 9 months. The authors advise the addition of an enlargement patch and the use of anticoagulation therapy in case of intimal lesion or very adherent thrombus.