Annales de pediatrie最新文献

Pediatric intensive care units use sophisticated medical technology and are staffed by deeply committed nurses who are subjected to significant psychological stress. This stress varies with the type of patient and influences the style and quality of care. With this respect, children and adolescents admitted after a suicidal attempt are considered catalysts. However, there have been no systematic studies of how pediatric intensive care nurses respond emotionally to their interactions with these patients. This epidemiological study conducted in five Parisian Teaching Hospital pediatric intensive care units used a specially designed questionnaire to evaluate nurses' responses on the basis of style of care. Children under 16 years of age admitted after attempted suicide were studied comparatively with same age children admitted for status asthmaticus or encephalopathy with seizures. Results highlighted the differences in nurses' psychological responses to these situations and their difficulties in interacting with patients. This study provides strict methodological guidelines for investigating an issue often discussed emotionally or on the basis of anecdotal data.

Posttraumatic stress disorder is a form of anxiety disorder which is poorly known in children and manifests as a series of symptoms occurring after an extraordinary event outside the range of usual human experiences and responsible for a feeling of terror. Virtually pathogmonic symptoms include re-experiencing the event through play and ceaselessly repeated behaviors (re-enactments), cognitive distortions when relating the facts (chronological errors, belief in omens), changes in attitudes towards others and life in general, and neurovegetative hyperactivity (hypervigilance, startle responses, difficulties controlling impulses). Although adequate follow-up data are lacking, the disorder can probably become chronic in children, as in adults. This nosographic entity raises the theoretical issue of the roles of life events and individual vulnerability. A number of factors may either "predispose" or "protect" the child: degree and duration of exposure to the trauma, nature of the event, preexistence of psychiatric disorders, level of cognitive development, sex, degree of social support, and containing or noncontaining attitude of the family. Treatments advocated in adults (pharmacotherapy, cognitive and behavioral therapies, group therapy) remain to be used and evaluated in children.

The need for quantitative clinical evaluation tools for use in child psychiatry is obvious. Behavior rating scales are useful for comparing clinical and laboratory data, monitoring the effects of treatments, and enhancing communication between clinicians and investigators. The methodological principles used to construct and validate such tools are described. This approach is of benefit in most psychiatric disorders of children and adolescents. The questionnaires and scales most widely used throughout the world and available in French are reviewed. The advantages and drawbacks of evaluation scales in everyday practice and in research are discussed, with the BSE-A scale (Behavior Summarized Evaluation of Austism) as an example.

In adults, suicidal attempts by jumping from elevated sites have been extensively studied and usually occur in patients with major psychiatric disorders such as schizophrenia or melancholia. Among the children and adolescents admitted to the Robert Debré Hospital in Paris for attempted suicide from 1989 through 1992, 8 (1 boy and 7 girls) had jumped out of a window. Ages ranged from 11 1/2 years to 15 years. Two patients had depression but in the six other cases there was no evidence of a psychiatric disorder. The suicidal attempt occurred after a prolonged period of conflicts within the family. Management of such patients proved difficult because of the presence of severe injuries and above all of massive denial on the part of the patient and family.

A total of 5,473 pharmacological provocative growth hormone release tests were carried out in 3,143 children. Mean age was 9 years 9 months (range 3-16 years) and mean bone age was 7 years 6 months (range 2-14 years). Tests were of 9 different types: 1) arginine (n = 625); 2) clonidine (n = 339); 3) insulin (n = 198); 4) ornithine (n = 162); 5) insulin + arginine (n = 203); 6) clonidine + betaxolol (n = 2,003); 7) L-dopa (n = 685); 8) glucagon = propranolol (n = 443); 9) glucagon + betaxolol (n = 815). All growth hormone determinations were performed using the same radioimmunoassay. Distribution of values obtained with each test was gausso-logarithmic. Mean peak levels with their 95% confidence limit were as follows: 1) 10.2 and 0.45; 2) 11.5 and 0.7; 3) 11.8 and 0.8; 4) 14.2 and 1.2; 5) 14.3 and 0.9; 6) 15.7 and 1.1; 7) 19.8 and 2.1; 8) 20.8 and 2.3; 9) 21.0 and 2.5. These data indicate low specificity, with up to two-fold differences in mean peak levels from one test to another; proportions of peaks under 10 ng/ml ranged from 29% to 69%. Thus, the rate of patients diagnosed with growth hormone deficiency may vary substantially according to the test used. To reduce these discrepancies, we suggest adjustment of test results using a weighting coefficient of 1) 1.9; 2) 1.48; 3) 1.4; 4) 1.16; 5) 1.06; 6) 1.01; 7) 0.73; 8) 0.69; 9) 0.66.

Sertoli-Leydig tumors stem from the mesenchyma and sexual cords of the embryonic gonad. Two cases are reported. One manifested as symptoms of virilization in a 12 year old girl. The other patient developed adnexal torsion at the age of five years. Pelvic ultrasonography visualized the tumor in both cases. Increased production of ovarian androgens suggested the diagnosis in the first case. Histological studies disclosed intermediate differentiation in the first case and tubular differentiation in the second. These tumors usually exhibit low-grade malignancy and unilateral salpingo-oophorectomy ensures recovery in most instances.

Prenatal treatment based on administration of dexamethasone to the mother during pregnancy was initiated early during nine pregnancies with a high risk of congenital adrenal hyperplasia due to 21-hydroxylase deficiency. The purpose of this treatment was to prevent fetal virilization by reducing production of androgens by the adrenal glands. Prenatal diagnosis was achieved by comparing amniotic fluid cell HLA genotypes and more recently by subjecting trophoblasts to molecular genetic studies. Together with prenatal determination of fetal sex, this allowed to determine that only two female fetuses were affected. Efficacy of continued prenatal treatment in these two cases was good in one case and mediocre in the other. The treatment was well tolerated by the mothers and fetuses.

In pediatric patients, endogenous Cushing syndrome is an infrequent condition almost always due to one of two conditions. 1) Adrenal gland tumors account for 70% of Cushing syndromes in young pediatric patients. They cause rapidly progressive hypercorticism not due to increased ACTH production (elevated plasma and urine cortisol levels, very low ACTH and LPH levels unchanged by dexamethasone, metyrapone or CRH). Imaging techniques determine the side and spread of the tumor and look for metastases. Following surgical removal, patients with indicators of malignant disease (tumor weight above 30 g, extracapsular spread or metastases, independently from pathological data) are given op'DDD. 2) Cushing disease occurs in peripubertal patients and causes overweight with delayed statural gain. ACTH production is increased (positive dexamethasone suppression test and provocative metopirone and CRH tests) as a result of a pituitary adenoma which should be looked for by magnetic resonance imaging and whose removal ensures recovery in 50% of cases. Other therapeutic tools include op'DDD, radiation to the pituitary, and bilateral adrenalectomy as the last resort given the high risk of post-adrenalectomy pituitary tumor (50% of pediatric patients). Other causes are exceedingly rare: primary nodular hyperplasia of the adrenal glands and production of ACTH by a nonpituitary tumor. Corticosteroid treatment is the most common cause of Cushing syndrome in children.

Four-year-old schoolchildren with a positive family history for atherogenic dyslipidemia and/or clinical atheroma before 55 years of age were screened for hypercholesterolemia. Investigations included determination of serum levels of total cholesterol, triglycerides, HDL cholesterol, apolipoprotein A1, apolipoprotein B, and Lp(a); an agarose lipidogram; acrylamide gradient electrophoresis; and determination of LDL composition by ultracentrifugation. Normal values were defined as values under the 90th centile, i.e., 1.97 g/l for total cholesterol, 0.89 g/l for triglycerides, 1.36 g/l for LDL-cholesterol, and 1.26 g/l for apolipoprotein B. Among 3,565 children routinely evaluated at 4 years of age, 525 (16.2%) had a positive family history; of these, 72 underwent lipid investigations. Eight children (11%) had hypercholesterolemia type IIA, eight had a variety of lipid disorders, and 14 (20.6%) had increased Lp(a) levels as an isolated anomaly or concomitantly with an atherogenic dyslipidemia. Because Lp(a) is a cardiovascular risk factor independent from total cholesterol levels, we believe this parameter should be determined in high risk children.

A case of hypothalamic dysfunction in a girl with a twelve-year follow-up is reported. Onset occurred at the age of three with severe obesity, hypothermia, hypersomnia, and lethargy. Somatotropic, gonadotropic, and thyrotropic hormones were low, whereas prolactin was increased. Imaging techniques failed to disclose any lesion of the hypothalamus or pituitary. Clomipramine improved the vegetative disorders. The literature on clinical and hormonal disorders of hypothalamic dysfunction is reviewed.