[Encopresis revealing myotonic dystrophy in 2 children]. / Encoprésie révélatrice d'une dystrophie myotonique de Steinert : à propos de 2 observations.

Gastrointestinal symptoms are very frequent in myotonic dystrophy but largely unrecognized. They can be the revealing factors of the disease. We report 2 cases of 10 and 17-year-old children with persistent encopresis starting at the age of 3 and 5 years in spite of laxative treatment. Neurological examination and anorectal manometry provided the diagnosis of myotonic dystrophy. Procainamide treatment was introduced and the digestive symptoms improved. Any child with encopresis should have complete evaluation to rule out the diagnosis of myotonic dystrophy and physicians should look for upper and/or lower gastrointestinal symptoms in every patient with myotonic dystrophy.

The KBG syndrome: an additional sporadic case.

We report the sporadic case of a boy with clinical features of KBG syndrome, including slight mental retardation, characteristic facies, macrodontia, and skeletal anomalies.

[Validation of the French version of a pleasure scale for children (The Pleasure Scale for Children, PSC, Kazdin, 1989) in 214 hospitalized children in pediatrics]. / Etude de validation de la version française de l'échelle de plaisir pour infants (EPE) de Kazdin (the pleasure scale for children, PSC, Kazdin, 1989) chez 214 enfants hospitalisés en pédiatrie.

UNLABELLED: The aim of this study was to determine the metrological parameters of a french version of the Pleasure Scale for Children (PSC): 214 (121 males and 93 females) with a mean age of 8.69 years (sd: 1.95) ranging from 6 years to 12 years were included in the study. The children were inpatients presenting various somatic disorders. STATISTICAL ANALYSIS: First a principal component analysis was done on the 39 items of the correlation matrix. Several guidelines were used to limit the number of factors (Kaiser criteria, Cattell scree test, Horn parallel analysis). Secondly the construct validity was studied using the alpha Cronbach coefficient and by calculing the Pearson correlation coefficient between each item and the total score. Thirdly the concurrent validity was determinated using two items of the Children Depression Rating Scale--Revised (CDRS-R) measuring pleasure (social withdrawal and enjoyment capacity). Fourthly the discriminant validity of the PSC was studied by comparing non depressive children (score lower than 30 to the CDRS-R) and depressive children (score higher than 30 to the CDRS-R). RESULTS: The principal component analysis showed a one factor solution with 33 items among the 39 having a higher than 0.3 saturation. The Cronbach alpha coefficient was 0.84. All the items correlated with the total score. The mean value was 0.37. The correlations between the total score of the scale and the CDRS-R enjoyment capacity and social withdrawal items were respectively -0.37 (p < 0.01) and -0.38 (p < 0.01). PSC score were significantly lower in depressive children (m = 86.96; sd = 8.33) than in non depressive children (m = 94.67; sd = 10) (t = 5.32; df = 212; p < 0.001).