Pediatrie最新文献

The basic principles and indications of high frequency oscillatory ventilation are presented. The authors have used this technics in more than 400 neonates with respiratory failure. High frequency oscillatory ventilation appears as a simple, efficient and safe method of ventilation in the treatment of neonatal respiratory distress.

The authors present the principle and discusses the indications and contraindications of two technics of extracorporeal membrane oxygenation in the treatment of neonatal respiratory failure, respectively veno-arterial and veno-venous. The two technics appear to give comparable results. They are indicated in neonates with refractory hypoxemia after failure of other methods such as surfactant, high frequency oscillatory ventilation and nitrous monoxyde.

The results of liver transplantation in children have dramatically improved from the wide use of cyclosporine A and because of technical advances such as the use of reduced-size adult liver transplants. It is now accurate to consider that 70 to 80% of transplanted children will be alive with a normal life 5 years post-transplant. Within a few years, liver transplantation has thus become the regular treatment of many liver diseases in childhood.

Accumulating evidence tends to demonstrate that inflammatory processes are responsible for neurological damage and sequelae in bacterial meningitis in children and infants. Massive liberation of bacterial cell wall components (Lipopolysaccharide, acid teichoic polymers) induce a cascade of reactions including the secretion of many cytokines (such as TNF alpha and IL-1 beta) and prostaglandins (such as PAF and PGE2) which in turn leads to the development of cerebral oedema, intracranial hypertension and cerebral blood flow reduction. Dexamethasone (DXM) is effective at the beginning of the inflammatory cascade and its utilisation in the meningitis experimental model in animals has shown significant reduction in the inflammatory response to bacterial meningitis. The first clinical studies using DXM as an adjunctive therapy to antibiotics have demonstrated its beneficial effect in terms of complications and long-term neurological sequelae in Haemophilus influenzae meningitis in children and infants. It seems that a similar effect can be obtained in meningococcal and pneumococcal meningitis. Little information is actually available concerning the use of DXM in penicillin-resistant pneumococcal meningitis. The rare reported cases of ceftriaxone failure with DXM as treatment of penicillin-resistant pneumococcal meningitis had a favorable outcome with the use of vancomycin.

The authors report on the observation of a premature infant (gestational age 31 weeks, birth weight 1440 g) who experienced a necrotizing enterocolitis and a congenital tuberculosis. Congenital tuberculosis is a rare infection; transmission of Koch's bacilli is achieved through hematogenous route or more frequently through inhalation or ingestion of infected amniotic fluid. The diagnosis was done when miliary patterns were found on the mother's chest X-ray. At the same time, the newborn presented with a chronic lung disease, a poor clinical status with biological signs of inflammation. Usual antibiotics were unsuccessful. A rapid improvement was obtained with a specific antituberculous treatment.

The authors report on one case of 3-hydroxy-3-methylglutaryl-coenzyme A lyase (HMG-Co A lyase) deficiency in a 3 year-old girl, presenting as Reye's syndrome. Urinary organic profile suggested this inherited metabolic disease; decreased activity of HMG-Co A lyase was demonstrated in cultured fibroblasts. The metabolic etiologies of Reye's syndrome are summarized; clinical, biological, and therapeutic assessment of HMG Co A lyase deficiency are developed. The pattern of organic aciduria must be studied in all patients presenting with Reye's syndrome.

The reported case concerns a 12-year-old boy with a congenital hypoplasia of the left pulmonary artery without associated cardiac malformation. At rest, pulmonary function tests were within the normal range, while the patient demonstrated an abnormal dyspnea and hypoxemia during exercise. These symptoms disappeared after left pneumonectomy. Unilateral pulmonary artery hypoplasia can be responsible for exercise hypoxemia due to an intermittent right-to-left shunt or a ventilation-to-perfusion mismatching.

A 4-year-old boy was treated with oxybutinine and desmopressine because of bladder instability associated with secondary enuresis. He was admitted with obnubilation, vomiting and experienced two seizure episodes concomitantly with hyponatremia and hypoosmolality. The child healed promptly under water restriction and intravenous administration of sodium chloride. This case report suggests that desmopressine may be responsible for severe side-effects. This drug should not be widely used and its indications should be restricted to patients with proven antidiuretic hormone secretion abnormalities.

Magnetic resonance imaging (MRI) of the hypothalamic hypophyseal area was performed in seven children with multiple hypophyseal deficiency including growth hormone deficiency. In all cases stalk transection syndrome was found. In four cases MRI showed cranial or cerebral malformations. Perinatal asphyxia was found in only one child, and congenital hypopituitarism was present in two cases. The discussion focuses on the mechanisms that might be responsible for this syndrome. The malformation theory, while not taking into account all situations, seems to be the most appropriate, the reasons being the MRI finding and the patients' case histories.