Cognitive and behavioral phenotype of children with pseudohypoparathyroidism type 1A.

Pseudohypoparathyroidism 1A (PHP1A) is a rare, genetic disorder. Most patients with PHP1A have cognitive impairment but this has not been systematically studied. We hypothesized that children with PHP1A would have lower intelligent quotient (IQ) scores than controls. To evaluate cognition and behavior, we prospectively enrolled children with PHP1A, one unaffected sibling (when available) and controls matched on BMI/age/gender/race. Evaluations included cognitive and executive function testing. Parents completed questionnaires on behavior and executive function. We enrolled 16 patients with PHP1A, 8 unaffected siblings, and 15 controls. Results are presented as mean (SD). The PHP1A group had a composite IQ of 85.9 (17.2); 25% had a composite IQ < -2 SD. The PHP1A group had significantly lower IQs than matched controls (composite IQ -17.3, 95%CI -28.1 to -6.5, p < 0.01) and unaffected siblings (composite IQ -21.5, 95%CI -33.9 to -9.1, p < 0.01). Special education services were utilized for 93% of the patients with PHP1A. Deficits were observed in executive function and parents reported delayed adaptive behavior skills and increased rates of attention deficit hyperactivity disorder. In conclusion, children with PHP1A have lower intelligence quotient scores, poorer executive function, delayed adaptive behavior skills, and increased behavior problems.

Sleep in Type 1 Diabetes: Implications for Glycemic Control and Diabetes Management.

PURPOSE OF REVIEW: To highlight recent findings from studies of sleep in type 1 diabetes (T1D), with a focus on the role of sleep in self-management, the cognitive and psychosocial outcomes related to sleep disturbances, and factors associated with sleep disturbances specific to T1D. RECENT FINDINGS: People with T1D experience higher rates of sleep disturbances than people without diabetes, and these disturbances have negative implications for glycemic control and diabetes management, as well as psychosocial and cognitive outcomes. Inconsistent sleep timing (bedtime and wake time) has emerged as a potential target for interventions, as variability in sleep timing has been linked with poorer glycemic control and adherence to treatment. Sleep-promoting interventions and new diabetes technology have the potential to improve sleep in people with T1D. Sleep is increasingly considered a critical factor in diabetes management, but more multi-method and longitudinal research is needed. We emphasize the importance of sufficient and consistent sleep for people with T1D, and the need for providers to routinely assess sleep among patients with T1D.

Sleep habits in adolescents with type 1 diabetes: Variability in sleep duration linked with glycemic control.

OBJECTIVE: To describe adolescents' sleep on school and weekend nights using multiple methods and to examine the links between sleep variability, quality, and duration with diabetes indicators. METHODS: Adolescents with type 1 diabetes (N = 65, mean age = 15.0, 52.3% female, mean HbA1c = 8.9% or 74 mmol/mol) wore an actigraph and kept daily diaries recording sleep, activities, and blood glucose monitoring (BGM) habits for at least 7 days. Average daily BGM and blood glucose (BG) levels were obtained through glucometer downloads. HbA1c was obtained as part of clinic visits. Adolescents completed a sleep quality questionnaire (Pittsburgh sleep quality index [PSQI]), and adolescents and caregivers reported on adherence to diabetes treatment. RESULTS: Adolescents reported a mean PSQI global score of 5.37, which is above the clinical cutoff for poor sleep quality. Actigraphy data revealed that mean adolescent total sleep time was 6:54 (h:min), and participants slept more on weekend nights than on school nights (P < .001). Additionally, variability in sleep duration was significantly related to HbA1c, frequency of BGM, and average BG. Total sleep time and self-reported sleep quality were not significantly associated with adherence or glycemic control. CONCLUSIONS: Few adolescents with type 1 diabetes met recommendations for sleep duration, and many reported poor sleep quality. We identified significant associations between variability in sleep duration with poorer glycemic control and less frequent BGM, supporting the need to consider sleep patterns as a modifiable factor that may affect adherence and glycemic control.

Engagement with a Text-Messaging Intervention Improves Adherence in Adolescents with Type 1 Diabetes: Brief Report.

Adherence to diabetes management is a challenge for adolescents with type 1 diabetes (T1D). Positive psychology interventions have improved adherence to treatment recommendations in adults with chronic health conditions but have not been widely tested in pediatric populations. We hypothesized that higher engagement with a text-messaging intervention to promote positive affect would increase the effects on diabetes management among adolescents with T1D. Adolescents with T1D (n = 48) and their caregivers were randomized to either an attention control condition or a novel positive psychology intervention delivered through personalized automated text messaging. We examined rates of engagement (percent response to text messages) in relation to demographic factors, and we explored the effect of engagement in relation to adherence and glycemic control. Adolescent engagement was good (mean response rate of 76%) over the 8-week intervention. Engagement was related to adolescents' gender, race, baseline glycemic control, and blood glucose monitoring, but not to treatment type (pump vs. injection), diabetes duration, age, or household income. There was a significant effect of level of engagement on better caregiver-reported adherence, but adolescents' engagement was not related to self-reported adherence or glycemic control. These results indicate feasibility and initial efficacy of using automated text-messaging to deliver an intervention aimed at promoting adherence in adolescents with T1D.

Obstructive Sleep Apnea and Otolaryngologic Manifestations in Children with Pseudohypoparathyroidism.

BACKGROUND/AIMS: Pseudohypoparathyroidism (PHP) is a rare, genetic disorder. Patients with PHP may have increased prevalence of obstructive sleep apnea (OSA) but this has not been prospectively studied. METHODS: We enrolled children aged 6-18 years with PHP and matched controls. Evaluation included physical examination, medical history, and polysomnography. RESULTS: Fifteen children with PHP type 1A (PHP1A) and 15 controls completed the study. Both groups were obese (BMI 32.2 ± 8.7 vs. 31.7± 6.5). The majority of PHP1A patients required tympanostomy tubes (86.7%) and adenotonsillectomy (73.3%). The primary outcome, i.e., the obstructive disturbance index, was significantly higher in PHP1A children versus controls (1.8 ± 2.3 vs. 0.6 ± 0.5, p = 0.045). Children with PHP1A were more likely to have OSA compared with controls (60.0 vs. 13.3%, p = 0.008). Three siblings with PHP type 1B (PHP1B) were also studied (BMI 25.9 ± 9.0). None had a history of adenotonsillectomy, one had tympanostomy tubes. The obstructive disturbance index (2.0 ± 2.3) was similar to that of children with PHP1A. Two (66.7%) PHP1B participants had OSA. CONCLUSION: Children with PHP1A are at an increased risk for OSA compared with similarly obese peers. They also have higher rates of otitis media and adenotonsillar hypertrophy. Screening for OSA should be considered in all patients with PHP1A and possibly PHP1B though more research is needed.