Pediatrie最新文献

Three children presented with an association of pains, infectious syndrome, acute renal failure, hepatitis and meningitis, that lead to the diagnosis of leptospirosis. The clinical spectrum of this rare disease are recalled.

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.

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.

Acute neurogenic pulmonary edema (NPE) can dramatically complicate a serious brain injury. From bibliographic data and four personal cases documented by and haemodynamical study, the authors analyse the pathophysiological mechanisms and the haemodynamical changes resulting from massive sympathic outflow, the main mechanism of NPE being haemodynamical rather than lesional. This rare complication needs to be recognized in order to undergo an intensive care treatment generally leading to a favourable evolution. This treatment lies on circulatory and ventilatory supports with positive and expiratory pressure in order to maintain an optimal oxygenation and an optimal cerebral perfusion pressure. With this treatment the outcome of NPE is usually favourable with complete recovery within 2 to 5 days.

We report two cases of neonatal ascites. The first case is a 24 day old male referred for abdominal distention and edema. Peritoneal tap removed a transudative fluid. Ultrasonographic evaluation revealed obstructive posterior urethral valves. Bladder drainage led to resolution of the urinary ascites and renal function normalization. Long term follow-up after endoscopic resection of valves was good. The second case is a male infant who presented at birth with abdominal distention. Radiology revealed an urinoma and a left side hydronephrosis secondary to ureteropyelic junction syndrome which underwent a successful surgical treatment. Urinary ascites is a rare entity which calls for immediate diagnosis and management to preserve renal function.

The authors report two cases of citrullinemia in siblings which add to 68 observations from the literature. They overview the clinical presentation, diagnosis and therapeutic management of the disease. The prognosis of severe neonatal form remains poor but an early adequate management may contribute to an acceptable outcome.

The authors report two cases of scurvy in two encephalopathic 3 year-old girls. The first case was misleading and was initially operated with the diagnosis of osteomyelitis. The second one presented with bleeding syndrome and subperiosteal hemorrhage. The diagnosis of scurvy was based upon deficient diet, clinical features, and periosteal cleavage at ultrasonographic evaluation. Treatment with ascorbic acid, 400 mg/d, led to a complete resolution of the clinical features. The authors emphasize the misleading features of scurvy at bone ultrasonographic evaluation and recommend a supplementation with ascorbic acid of children with severe mental retardation.

The weight and height data of 9261 children from birth onwards were gathered on their admission to primary school in 1989 in the area of Lyon, and compared to French references. In comparison with the latter data, the weight and height for age were found to be higher, and there was an excess of children with a high weight for height. The weight for height was higher than the mean of the reference distribution plus two standard deviations in 6.2% of the children. Birth hypertrophy increased the risk of obesity at age six. A new criterion of neonatal hypertrophy, taking various constitutional characteristics into account, was found to be associated to a twofold risk of subsequent obesity, and was shown to be more appropriate in predicting subsequent obesity than classical definitions based on the weight or on the weight for gestational age or sex.