Pediatrie最新文献

From 1988 until 1992, 94 children with end-stage liver disease were put forward for orthotopic liver transplantation (OLT) by the surgical and anesthesic teams of the Bicetre Hospital. Due to a majority of adult donors, and to the young age of the recipients (mean = 3 years 3 months), a reduced-size graft was prepared in 64 out of the 111 transplants performed (58%). A number of children (42) had to be reoperated on due to complications: hepatic artery thrombosis, one of the most severe complications following pediatric OLT, occurred in 14 cases, and was an indication for seven out of the 17 retransplantations in this series. The eight deaths which occurred in the early postoperative period were the toll of this particularly challenging surgery.

A 15-year-old boy with thalassaemia major treated with desferrioxamine, experienced fever associated with digestive disorders. These symptoms led to the discovery of multiple liver abscesses. Therapy included antibiotics (third generation cephalosporin and aminoglycoside) and desferrioxamine discontinuation. Recovery occurred within one month approximately. Systemic yersiniosis was further confirmed by the finding of antibodies to Yersinia enterocolitica O5. The systemic diffusion of Yersinia is favoured by iron-overload and iron-chelating agents.

We report the results of a retrospective epidemiologic study on neuroblastoma in Brittany, a region of France characterized by an homogeneous sedentary population living on a granitic ground. During a period of 10 years (1980-1989), 48 cases of neuroblastoma were observed, which gives an incidence of 7.92 cases per million of children under 15 years. There were 25 boys and 23 girls and 20 infants under 1 year (41%). The localisation was abdominal in 39 cases (81%). Among the 28 children older than 1 year, 25 (89%) had stage III and stage IV neuroblastomas, half of them with metastases. We conclude that the incidence and the clinical presentation of neuroblastoma in Brittany are similar to those reported in other countries.

It appears to be necessary to consider, during a bone marrow transplantation, brothers and sisters who are donors. These children face psychological difficulties, which are not always understood by medical staff and families who are mainly concerned with the health of the sick children. Systematic meetings with a hundred donors point out the distress experienced in these situations.

The circumstances and the prognosis of six gunshot craniocerebral wounds in civilian children are presented. The cause is a suicide in two cases and an accident in four cases. In three out of these four cases, another person is responsible for this accident, an adult two times, a child in one case. When the accident is due to a child, it is always by play and one time the accident is the autofact of a 2-year-old boy. In all cases, the fire arm was loaded and an adult's imprudence is present. The mortality is important (4/6). More than the initial clinical state, the seriousness of which is not a pejorative indice, the prognosis is subordinated to the CT scan aspect about the missile tract, and to the initial value of the intracranial pressure.

Langerhans cell histiocytosis is a non malignant disorder of immune regulation. Patients with multisystem disease and organ dysfunction are considered to have a poor prognosis, and particularly children younger than 2 years of age. Clinical trials using human alpha-interferon, cyclosporine A and bone marrow transplantation have been reported recently.

Four cases of psychosocial dwarfism are reported. The growth follow-up of these four children does not fit to the classical description of rapid improvement once separated from their defective or careless family. This illustrates the difficulties for some children to adapt themselves to a foster family and the need to refer them to an institutional center in order to solve their different somatic, nutritional and psychological problems, together with those of their family. In such cases the important growth retardation and hormonal changes may hide severe psychopathological troubles such as hyperkinetic syndrome, anxiety and, possibly, depression.

A five month-old infant presented with gastro-intestinal symptoms followed by a multiple organ failure with: shock, status epilepticus, disseminated intravascular coagulation, hepatic and renal failure. The infant survived with major neurological sequelae. The diagnosis and the actuality of the so-called "hemorrhagic shock and encephalopathy syndrome" are discussed.