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Biomedical subjects

L Laberge

Publications and source records attributed to L Laberge.

10 recordsLinked to original sources

The correlation of CTG repeat length with material and social deprivation in myotonic dystrophy.

Socioeconomic deprivation has long been recognized as a prominent feature of myotonic dystrophy type 1 (DM1), but studies performed before the discovery of the mutation causing DM1 may have suffered an ascertainment bias towards the more severe forms of the disease. We have sought to clarify the relationship between CTG repeats, muscular impairment, and socioeconomic characteristics of 200 patients with DM1. Patients with DM1 reported lower educational attainment, lower employment rate, lower family income, and higher reliance on social assistance than the reference population. Logistic regression showed, on one hand, that CTG repeats and marital status were significant predictors of social assistance recipiency and, on the other hand, that CTG repeats and gender were significant predictors of low social support from family, after adjustment for age, gender, degree of muscular impairment, CTG repeats, educational level, and marital status. For example, each additional 100 CTG repeats was found to increase the odds of relying on social assistance by about 35% and having low social support by about 22%. The chances of experiencing socioeconomic deprivation are loaded heavily against patients with DM1. The relationship between increased CTG repeat length and higher risk of material and social deprivation must be acknowledged in the clinical management of DM1.

Adult↗

Fatigue and daytime sleepiness rating scales in myotonic dystrophy: a study of reliability.

OBJECTIVES: To assess the reliability of the Epworth Sleepiness Scale (ESS), Daytime Sleepiness Scale (DSS), Chalder Fatigue Scale (CFS), and Krupp's Fatigue Severity Scale (KFSS) in patients with myotonic dystrophy type 1 (DM1). METHODS: In total, 27 patients with DM1 were administered the questionnaires on two occasions, with a 2 week interval. Internal consistency and test retest reliability were measured using intraclass correlation coefficients (ICCs), and Cronbach's alpha, Cohen's kappa, and Goodman-Kruskal's gamma coefficients. RESULTS: Internal consistency of the CFS and KFSS were adequate (alpha > 0.70) but that of the ESS was weak (alpha = 0.24). Both daytime sleepiness and fatigue rating scales showed significant test retest reliability. Test retest reliability for individual items revealed inconsistencies for some ESS and CFS items. CONCLUSIONS: Reliability of the CFS, DSS, and KFSS was high, allowing their use for individual patients with DM1, but that of the ESS was lower, rendering its current usage in DM1 questionable. Fatigue rating scales such as the KFSS, which are based on the behavioural consequences of fatigue, may constitute a more accurate and comprehensive measure of fatigue severity in the DM1 population.

Adult↗

Development of sleep patterns in early adolescence.

This study examines the developmental changes of sleep patterns as a function of gender and puberty and assesses the prevalence of sleep habits and sleep disturbances in early adolescence. It also investigates the relationship between sleep patterns, sleep habits and difficulty falling asleep and nocturnal awakenings. The present analyses are based on results available for 588 boys and 558 girls for whom mothers completed questions concerning demographics and sleep at annual intervals when their child was aged 10--13 years. The results indicated that nocturnal sleep times decreased, bedtimes were delayed and differences between weekend and school day sleep schedules progressively increased with age. Gender and puberty were both associated with the timing of sleep on weekends. Girls presented longer weekend time in bed (TIB) and later weekend wake time than boys. Similarly, subjects with higher pubertal status showed longer weekend TIB and later weekend wake time than subjects with lower pubertal status. Difficulty falling asleep was associated with later weekend wake time and with sleeping with a night light. In conclusion, the gender differences commonly reported in adolescents' sleep patterns are most likely explained by girls' higher pubertal status. This study emphasizes the link between puberty and a putative physiological need for more sleep, in presence of a general reduction of sleep times during adolescence. From age 10--13 years, the delay and lengthening of the sleep period on weekends in comparison to schooldays is associated with difficulty falling asleep.

Adolescent↗

Development of disturbing dreams during adolescence and their relation to anxiety symptoms.

STUDY OBJECTIVES: This work assesses the prevalence and development of disturbing dreams among adolescents and the association of these dreams with anxiety. DESIGN: Sex differences in prevalence were analyzed with chi-square analyses. Changes over time were assessed with Wilcoxon tests and cross-tabulation tables. Associations with anxiety and DSM-III-R symptoms were assessed with ANOVA designs. SETTING: N/A. PARTICIPANTS: A total of 610 boys and girls rated their recall of disturbing and normal dreams at both 13 and 16 years of age. Subgroups of subjects were evaluated for anxiety symptoms at age 13 and for DSM-III-R symptoms of separation anxiety (SA), overanxious disorder (OD) and generalized anxiety disorder (GAD) at age 16. INTERVENTIONS: N/A. MEASUREMENTS AND RESULTS: The recall of disturbing dreams was more prevalent for girls than for boys at both ages, and increased over time for girls while it decreased for boys. The recall of normal dreams was also more prevalent for girls at both ages, but this difference could not fully account for the difference in recall of disturbing dreams. Normal dream recall increased from age 13 to 16 for both sexes. The frequent occurrence of disturbing dreams was associated with anxiety at age 13 and with GAD, SA and OD symptoms at age 16 for both sexes. Evidence of more numerous OD symptoms for girls with frequent disturbing dreams suggests that this form of anxiety may partially account for the observed sex difference in disturbing dream prevalence. CONCLUSIONS: The findings highlight a prevalence of disturbing dreams that is especially marked for adolescent girls. Unlike previous cross-sectional studies, which have found the same sex difference, this longitudinal design also calls attention to within-subjects changes in disturbing dream recall. The results also confirm that the frequent recall of disturbing dreams is associated with pathological symptoms of trait anxiety-apparently even as young as 13 years of age. Further study of disturbing dreams may contribute to understanding of associated pathophysiological factors which, too, vary by sex (e.g., PTSD, insomnia, depression).

Adolescent↗

Sleep and circadian phase characteristics of adolescent and young adult males in a naturalistic summertime condition.

Our aim was to compare the circadian phase characteristics of healthy adolescent and young adult males in a naturalistic summertime condition. A total of 19 adolescents (mean age 15.7 years) and 18 young adults (mean age 24.5 years) with no sleep problems took part in this study. Two-night polysomnographic (PSG) sleep recordings and 24h secretion patterns of urinary 6-sulfatoxymelatonin were monitored in all 37 subjects. Sleep-wake patterns were initially assessed at home using a standard sleep diary. Circadian assessment included the measure of dim light melatonin offset (DLMOff) and the morningness-eveningness (M/E) questionnaire. As expected, compared to young adults, adolescents habitually spent more nocturnal time in bed and spent more time (and percentage) in delta sleep. No difference was found between adolescents and young adults on multiple sleep latency test (MSLT) sleep onset latencies, M/E, melatonin secretion measures (24h total, nighttime, daytime, and night ratio), and DLMOff. For the subjects as a whole, correlational analyses revealed a significant association between the DLMOff and M/E and between both these phase markers and habitual bedtimes, habitual rising times, and melatonin secretion measures (daytime levels and the night ratio). No association was found between phase markers and daytime sleepiness or sleep consolidation parameters such as sleep efficiency or number of microarousals. These results together indicate that adolescents and young adults investigated during summertime showed similar circadian phase characteristics, and that, in these age groups, an evening phase preference is associated with a delayed melatonin secretion pattern and delayed habitual sleep patterns without a decrease in sleep consolidation or vigilance.

Adolescent↗

Development of parasomnias from childhood to early adolescence.

OBJECTIVES: This study examines the prevalence and developmental changes of parasomnias and assesses gender differences, relationships between parasomnias, and associations with anxiety and family adversity using data collected during the course of a longitudinal study of a representative sample of children from Québec. METHOD: The present analyses are based on results available for 664 boys and 689 girls for whom mothers have completed questions concerning demographics, parasomnias, and anxiety level. For the prevalence and developmental aspects of parasomnias, prospective data were collected at annual intervals from 11 to 13 years old and retrospective data for the period between ages 3 and 10 years were collected when the children were 10 years old. RESULTS: Somniloquy, leg restlessness, and sleep bruxism are the most frequent parasomnias. More girls were afflicted with leg restlessness, while enuresis and somniloquy were more common in boys. High anxiety scores were found in children suffering from night terrors, somniloquy, leg restlessness, sleep bruxism, and body rocking. Parasomnias were unrelated to the index of family adversity. CONCLUSIONS: Although sleepwalking, night terrors, enuresis, and body rocking dramatically decreased during childhood, somniloquy, leg restlessness, and sleep bruxism were still highly prevalent at age 13 years, paralleling results found in adults. Sleepwalking, night terrors, and somniloquy are conditions often found together. The only robust gender difference was for enuresis. High anxiety scores in parasomnias are reported for the first time in a large, controlled study. Sociodemographic variables do not seem to play a major role in the occurrence of parasomnias.

Adolescent↗

Lymphangiomas in children: percutaneous sclerotherapy with an alcoholic solution of zein.

PURPOSE: To determine the efficacy of percutaneous sclerotherapy with an alcoholic solution of zein in lymphangiomas in pediatric patients. MATERIALS AND METHODS: Fourteen patients with macrocystic and mixed lymphangiomas were treated with use of Ethibloc as a sclerosing agent. The procedure was performed with ultrasonographic and/or fluoroscopic guidance. Follow-up clinical and sequential computed tomographic examinations were performed to evaluate regression of the lesion. RESULTS: Regression was excellent in nine (64%) of the 14 lesions: Regression was complete in four lymphangiomas and was to less than 5% of the initial volume in five. Satisfactory results were obtained in the remaining five (36%) of 14 lesions. The most frequent complication was leakage of Ethibloc without important sequelae in 10 of the 14 lymphangiomas. CONCLUSION: Percutaneous sclerotherapy with Ethibloc was a safe and effective procedure in the treatment of macrocystic and mixed lymphangiomas.

Adolescent↗