Annales de pediatrie最新文献

Obesity is a multifactorial syndrome involving both a genetic susceptibility and environmental factors such as early eating experiences in infancy. Interviews, drawings, and a personality test (Rorschach) were used to classify obese children according to their personality. Results suggest that the eating disorder is only one component of a more global alteration in psychological organization. These data support the need for psychological intervention.

To clarify risk factors for obesity in children and look for factors affecting prognosis, simple clinical parameters were analyzed in 113 obese children at the first visit and during treatment. Height and weight charts were constructed. The main risk factors were parental obesity, excess weight at the age of one and above all young age at the time of the rebound in body mass index (W/H2). Severity of obesity was unrelated to spontaneous caloric intake but was negatively correlated with age at the time of rebound in body mass index (r = -0.34; p = 0.017) and above all with age at onset of obesity (r = -0.53; p < 0.001). Closely spaced measurements of weight, stature, and body mass index in high risk children would allow appropriate preventive treatment before the phase of established obesity. Curative treatment is disappointing: only 28% of the study subjects achieved significant reductions in weight.

A case of cerebral sarcoidosis of fortuitous discovery is reported in an adolescent. Neurosarcoidosis is exceedingly rare in the pediatric age groups. Clinical manifestations are widely variable and nonspecific, making diagnosis difficult in patients without extraneurologic involvement. The mainstay of management is early, prolonged corticosteroid therapy. Outcome varies with location of the lesions.

A case of intralobar pulmonary sequestration in an infant is reported. The lesion presented as recurrent bronchopulmonary infections. The chest film showed a density in the right lower lobe, in which air-filled images and air-fluid levels developed rapidly. Aortography demonstrated systemic blood supply to the lesion from three arteries stemming from the thoracic aorta. The pathological study of the operative specimen confirmed the diagnosis. Postoperative outcome was satisfactory.

A 21-month-old infant developed coma with hypotonia during a viral infection. Acyl CoA dehydrogenase deficiency was diagnosed on the basis of results of the chromatographic study of organic acids performed on a urine specimen collected during the acute episode. However, other disorders of mitochondrial and fatty acid oxygenation can generate similar symptoms. Emphasis is put on the need for collecting urine specimens in patients who develop alterations in consciousness and hypoglycemia without ketonuria during prolonged fasting or repeated vomiting due to a viral infection. Urine chromatography can suggest which enzyme is defective, although the diagnosis should always be confirmed by a study of fatty acid oxygenation in lymphocytes or fibroblasts.

Type II primordial microcephalic dwarfism is a rare form of bird-headed dwarfism individualized in 1982 by Majewski. A case in a female patent with completed growth illustrates the main features, which include severe growth retardation (greater than 4 SD reduction in height) of prenatal onset, short limbs, coxa vara with epiphysiolysis of the hips, metaphyseal flaring and, in some instances, shortness of the ulnas and curvature of the radiuses. A genetic cause (with autosomal recessive inheritance) is very likely. A number of features in the case reported herein may be of pathogenetic relevance: growth hormone levels were elevated before closure of the epiphyses and normal thereafter, no growth spurt occurred at puberty, polycystic ovaries with hirsutism developed after puberty, and surgical wound healing was unusually slow.

Childhood obesity results from excessive dietary intake as compared with energy expenditure. This imbalance can result from either overeating or a constitutional decrease in one or several of the components of energy expenditure: resting metabolic rate, diet-induced thermogenesis, or exercise-related thermogenesis. Decreased energy expenditure is probably at least partly genetic in origin, contributing to the significant clustering of obesity in families. Management aims at reducing the imbalance by decreasing the caloric intake. Once the desired weight is achieved, intake must be adjusted to expenditure to avoid weight regain.

The effectiveness of early tenotomy of the psoas and adductor muscles for the prevention of dislocation of the hip dislocation in children with cerebral palsy precluding walking was studied in 40 hips in 24 children with severe disability. Age at the time of the procedure ranged from 1 1/2 years to 10 years. Results were evaluated by measuring Reimers' migration percentage (MP). Mean follow-up was three years. A satisfactory outcome (at least 10% improvement in MP at last follow-up) was seen in 67% of cases. The study of correlations with age and degree of displacement at surgery showed that results were satisfactory in 90% of children who had surgery before 4 years of age and whose MP was under 33%. To have preventive value, this procedure should be carried out at 2 years of age, before development of dislocation of the hip.

This retrospective review included 133 patients with one or several of the following defects: diastematomyelia, neurenteric cyst, dermal sinus or cyst, meningeal malformation, sacral agenesis, tethering of the spinal cord, and lumbosacral lipoma. Physical evaluation readily identified most lumbosacral lipomas, as well as most cases of diastematomyelia since hypertrichosis over the defect was common. Some patients developed life-threatening clinical manifestations, e.g., meningitis due to a dermal sinus. On the basis of this retrospective review and a review of the literature, the clinical and therapeutic aspects of each defect are discussed.

The knowledge acquired over the last few years on Duchenne's muscular dystrophy (DMD) serves as the basis for management of all types of myopathy. After reviewing the current classification of muscle dystrophies and the principles of orthopedic management of these conditions, a historical perspective of surgical procedures used in DMD is presented. The various modes of discovery of myopathy are described. Deformities of the limbs and spine related to DMD are reviewed and their outcome is detailed. In DMD patients, the surgical procedures described allowed to delay loss of the ability to walk by 1 year 7 months to 3 years 8 months according to the severity of the condition, and provided significant improvements in quality of life. Surgical stabilization of the scoliosis ensured preservation of comfortable sitting in every case, as well as preservation of autonomous upper limb motion, with a low rate of complications. Surgical indications in DMD are well-established and serve as the basis for making surgical decisions in the other myopathies, except during early infancy. The principles and orientations of treatment of the other myopathies are defined, in the light of the variability of lesions and of the rate of muscle function loss. In these non-DMD myopathies, medical and surgical treatments are of as yet unquantifiable benefit but provide a substantial improvement in quality of life.