The moderating role of diabetes distress on the effect of a randomized eHealth intervention on glycemic control in Black adolescents with type 1 diabetes.

OBJECTIVE: Due to systemic inequities, Black adolescents with type 1 diabetes are more likely to have suboptimal glycemic control and high rates of diabetes distress, but tailored interventions for this population are lacking. In primary outcomes of a randomized clinical trial, a family-based eHealth intervention improved glycemic control in Black adolescents with type 1 diabetes and elevated depressive symptoms. The present study is a secondary analysis of these clinical trial data examining the moderating effect of diabetes distress on the efficacy of the intervention. METHODS: Using secondary data from a multicenter randomized clinical trial (Clinicaltrials.gov [NCT03168867]), caregiver-adolescent dyads were randomly assigned to either up to three sessions of an eHealth parenting intervention (n = 75) or a standard medical care control group (n = 74). Black adolescents (10 years, 0 months to 14 years, 11 months old) with type 1 diabetes and a caregiver willing to participate were eligible. Adolescents reported their diabetes distress at baseline, and hemoglobin A1c (HbA1c) data were collected at baseline, 6-, 13-, and 18-month follow-up. RESULTS: No between-group contrasts emerged in a linear mixed-effects regression (p's > .09). Within-group contrasts emerged such that adolescents assigned to the intervention who reported high diabetes distress had lower HbA1c at the 18-month follow-up relative to baseline (p = .004); the 18-month decrease in HbA1c was -1.03%. CONCLUSIONS: Black adolescents with type 1 diabetes and high levels of diabetes distress showed significant decreases in HbA1c following a family-based eHealth intervention, suggesting diabetes distress may be a key moderator of intervention efficacy within this population.

Psychometric Properties of the Patient Activation Measure in Family Caregivers of Patients With Chronic Illnesses.

BACKGROUND: The Patient Activation Measure (PAM) is used clinically and in research to measure an individual's knowledge, skills, and confidence related to their health management engagement. Despite the use of "patient" in the title, the instrument can be used in nonpatient populations. A group at high risk for low activation concerning their own health is family caregivers of patients with chronic illnesses. The psychometric properties of the PAM have not been established in family caregivers. OBJECTIVES: This study aimed to examine the psychometric properties of the PAM 10-item version (PAM-10) in a sample of family caregivers of patients with chronic illnesses. Our focus was on family caregivers' health activation of their own healthcare needs. METHODS: We evaluated the internal consistency reliability of the PAM-10 in a sample of 277 family caregivers. Item-total correlations and interitem correlations were used to assess item homogeneity. Construct validity of the PAM-10 was examined using exploratory factor analysis and testing hypotheses on known relationships. RESULTS: The PAM-10 demonstrated adequate internal consistency. Item-total correlation coefficients and interitem correlation coefficients were acceptable. Construct validity of the instrument was supported. Factor analysis yielded two factors that explained 62.3% of the variance in the model. Lower levels of depressive symptoms were significantly associated with better activation, providing evidence of construct validity. Caregivers with high activation levels were significantly more likely to engage in and adhere to self-care behaviors such as regular exercise, eating a healthy diet, and engaging in stress reduction strategies. DISCUSSION: This study demonstrated that the PAM-10 is a reliable and valid measure for family caregivers of patients with chronic illnesses to measure caregivers' health activation of their own healthcare needs.

Subcutaneous fat necrosis of the newborn: A retrospective study of 32 infants and care algorithm.

OBJECTIVE: To describe the clinical and laboratory outcomes of infants with subcutaneous fat necrosis of the newborn (SCFN) and propose a care algorithm. METHODS: This single-center, retrospective study of infants diagnosed with SCFN at Ann & Robert H. Lurie Children's Hospital of Chicago from 2009 to 2019. RESULTS: Of 32 infants who met inclusion criteria, most were born full-term (84%), born via cesarean section (58%), had normal weight for gestational age (69%), and experienced delivery complications (53%). Twenty-nine infants (91%) had calcium drawn, and all had hypercalcemia. Three infants developed clinical symptoms of hypercalcemia, two required hospital admission, two developed nephrocalcinosis, and one developed acute kidney injury. The majority of infants (62%) had a peak ionized calcium between 1.5 and 1.6 mmol/L. No infants with peak ionized calcium less than 1.5 mmol/L developed complications of hypercalcemia. Most patients were diagnosed with hypercalcemia (86%) and demonstrated peak ionized calcium levels (59%) within the first 28 days of life. No patients developed hypercalcemia after 3 months of age. CONCLUSION: Hypercalcemia occurred in 100% of infants who had laboratory monitoring. We recommend obtaining an initial ionized calcium level when SCFN is suspected, and monitoring for the first 3 months of life if hypercalcemia has not been detected. In patients with asymptomatic hypercalcemia less than 1.5 mmol/L, there appears to be low likelihood of related complications. For symptomatic, markedly elevated (>1.6 mmol/L), or persistently elevated levels (>6 months) we suggest coordinated care with endocrinology or nephrology, consider hospitalization, and urinary system ultrasound.

Health Literacy and Perceived Control: Intermediary Factors in the Relationship Between Race and Cardiovascular Disease Risk in Incarcerated Men in the United States.

BACKGROUND: Black race, inadequate health literacy, and poor perceived control are predictors of increased cardiovascular disease (CVD) risk. The purpose of this study was to explore the relationships among race, health literacy, perceived control, and CVD risk while controlling for known risk factors in incarcerated men. METHODS: We included data from 349 incarcerated men to examine race and CVD risk (Framingham Risk Score) using a serial mediation model with health literacy and perceived control using 95% confidence intervals (CIs) from 5000 bootstrap samples. RESULTS: Of the participants (age, 36 ± 10; education, 12 ± 2; body mass index, 28.3 ± 5.0), 64.2% were White and 35.8% were Black. Black incarcerated men were younger (P = .047) with lower levels of health literacy (P < .001). All 3 indirect effects of race on CVD were significant, whereas the direct effect of race was not. Black incarcerated men had higher levels of CVD risk through health literacy (a1b1 = 0.3571; 95% CI, 0.0948-0.7162) and lower levels of CVD risk through perceived control (a2b2 = -0.1855; 95% CI, -0.4388 to -0.0077). Black incarcerated men had higher levels of CVD risk through health literacy influenced by perceived control (a1b2d21 = 0.0627; 95% CI, 0.0028-0.1409), indicating that despite the protective effect of higher levels of perceived control in Black incarcerated men, CVD risk remained higher compared with their White counterparts. CONCLUSION: Future CVD risk reduction interventions in incarcerated men, specifically Black incarcerated men, should include goals of improving health literacy and perceived control as modifiable risk factors.

The Arduous Path to Drug Approval for the Management of Prader-Willi Syndrome: A Historical Perspective and Call to Action.

Prader-Willi syndrome (PWS) is a neuroendocrine genetic disorder resulting from the loss of paternally expressed imprinted genes in chromosome 15q11-q13 [...].

Linkage of Optimism With Depressive Symptoms Among the Stroke Survivor and Caregiver Dyads at 2 Years Post Stroke: Dyadic Mediation Approach.

BACKGROUND: Depressive symptoms are substantial among stroke survivors and their caregivers in poststroke management. Optimism and social support are known to protect against depressive symptoms. However, little is known about how optimism and social support contribute to depressive symptoms among stroke survivor-caregiver dyads. The study's purpose was to examine actor and partner effects of optimism on depressive symptoms through perceived social support among stroke survivors and caregiver dyads in the chronic stage of rehabilitation. METHODS: Stroke survivors and caregivers (N = 105 dyads) completed the survey at 2 years of follow-up after the first stroke. Depressive symptoms, optimism, and perceived social support were assessed using the Center for Epidemiologic Studies-Depression, the Life Orientation Test, and the Interpersonal Support Evaluation List. The Actor-Partner Interdependence Model Extended to Mediation analysis was used to test the indirect effect of optimism on depressive symptoms through perceived social support. RESULTS: Higher optimism was significantly associated with lower depressive symptoms for caregivers (direct actor effect, -0.6844; 95% confidence interval [CI], -0.9844 to -0.3844) and stroke survivors (direct partner effect, -0.4189; 95% CI, -0.0789 to -0.0889). Perceived social support availability significantly mediated the association between optimism and depressive symptoms for stroke survivors (indirect effect, -0.1957; 95% CI, -0.3923 to -0.0670). Caregiver perceived social support availability was also a significant mediator between caregivers' optimism and stroke survivors' depressive symptoms (indirect effect, 0.1658; 95% CI, 0.0559-0.3128). CONCLUSIONS: Intervention improving dyad members' optimism and social support would be beneficial to improve depressive symptoms of the stroke survivors and caregivers in chronic stroke management.

Follow-up of SARS-CoV-2 positive subgroup from the Asymptomatic novel CORonavirus iNFection study.

A nested longitudinal study within theAsymptomatic novel CORonavirus iNFfection study followed participants with positive nasopharyngeal swab to query for development of symptoms and assess duration of positive reverse transcription-polymerase chain reaction (RT-PCR) test results. Of the 91 participants initially testing positive, 86 participated in follow-up approximately 14 days after study enrollment; of those 86 participants, 19 (22.1%) developed at least one symptom at any time after the initial positive severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) test result. The median number of days to symptom development after their initial positive test result was 6 (range 1-29 days). No participants reported a SARS-CoV-2-related hospitalization. The most frequently reported symptoms were fatigue or muscle aches (10.5%), headache (9.3%), fever (5.8%), and shortness of breath (5.8%). Of the 78 participants who submitted a nasopharyngeal swab for repeat RT-PCR testing, 17 (21.8%) remained positive at Day 14, 4 of which continued to test positive at Day 28. These findings reinforce the probable role of silent SARS-CoV-2 infections in community transmission, and that reliance on symptom development will miss a large proportion of infections. Broad testing programs not limited to individuals presenting with symptoms are critical for identifying persons with SARS-CoV-2 infection and ultimately slowing transmission.

Influence of molecular classes and growth hormone treatment on growth and dysmorphology in Prader-Willi syndrome: A multicenter study.

Prader-Willi syndrome (PWS) is a complex genetic disorder with three molecular classes but clinical ascertainment is based on distinctive features. The prevalence of dysmorphic features was studied in 355 PWS participants (61% deletion, 36% maternal disomy [UPD], and 3% imprinting defects) from the National Institute of Health PWS Rare Diseases Clinical Research Network. The effect of growth hormone (GH) treatment on growth and dysmorphic features was compared. Among participants, upslanting palpebral fissures were seen in 23%; strabismus in 42%; abnormal dentition in 32%; small hands in 63% and small feet in 70%; hypopigmentation in 30%; striae in 32% and skin picking in 26%. Compared to those with UPD, participants with deletions were found to be heavier (p = 0.002), had smaller head circumference (HC) (p = 0.009), higher incidence of a flat occiput (p = 0.005); low-anterior hairline (p = 0.04); abnormal dentition (p = 0.009); abdominal striae (p = 0.045), nail abnormalities (p = 0.050), and fair-haired (p < 0.001). Participants in both genetic groups receiving GH were taller (p = 0.005), had larger HCs (p = 0.005), and longer hands (p = 0.049). This study suggested that PWS genetic subtypes and GH treatment can influence growth and dysmorphic features that may impact clinical diagnosis of PWS, such as stature, head shape and appearance of the eyes, nose, and genitalia.

Effects of family and neighborhood risks on glycemic control among young black adolescents with type 1 diabetes: Findings from a multi-center study.

While individual and family risk factors that contribute to health disparities in children with type 1 diabetes have been identified, studies on the effects of neighborhood risk factors on glycemic control are limited, particularly in minority samples. This cross-sectional study tested associations between family conflict, neighborhood adversity and glycemic outcomes (HbA1c) in a sample of urban, young Black adolescents with type 1 diabetes(mean age = 13.4 ± 1.7), as well as whether neighborhood adversity moderated the relationship between family conflict and HbA1c. Participants (N = 128) were recruited from five pediatric diabetes clinics in two major metropolitan US cities. Diabetes-related family conflict was measured via self-report questionnaire (Diabetes Family Conflict Scale; DFCS). Neighborhood adversity was calculated at the census block group level based on US census data. Indictors of adversity were used to calculate a neighborhood adversity index (NAI) for each participant. Median family income was $25,000, suggesting a low SES sample. In multiple regression analyses, DFCS and NAI both had significant, independent effects on glycemic control (ß = 0.174, P = 0.034 and ß = 0.226 P = 0.013, respectively) after controlling for child age, family socioeconomic status and insulin management regimen. Tests of effects of the NAI and DFCS interaction on HbA1c found no significant moderating effects of neighborhood adversity. Even within contexts of significant socioeconomic disadvantage, variability in degree of neighborhood adversity predicts diabetes-related health outcomes in young Black adolescents with type 1 diabetes. Providers should assess social determinants of health such as neighborhood resources that may impact adolescents' ability to maintain optimal glycemic control.

Optimizing Recruitment of Black Adolescents into Behavioral Research: A Multi-Center Study.

OBJECTIVES: Adolescents of color are underrepresented in behavioral health research. Study aims were to quantify the amount and types of outreach effort needed to recruit young Black adolescents with type 1 diabetes and their primary caregiver into a clinical trial evaluating a parenting intervention and to determine if degree of recruitment difficulty was related to demographic, diabetes-related, or family characteristics. METHODS: Data were drawn from a multi-center clinical trial. Participants (N = 155) were recruited from seven pediatric diabetes clinics. Contact log data were used to quantify both number/type of contacts prior to study enrollment as well as length of time to enrollment. Families were coded as having expedited recruitment (ER) or prolonged recruitment (PR). Baseline study data were used to compare ER and PR families on sociodemographic factors, adolescent diabetes management and health status and family characteristics such as household organization and family conflict. RESULTS: Mean length of time to recruit was 6.6 months and mean number of recruitment contacts was 10.3. Thirty-nine percent of the sample were characterized as PR. These families required even higher levels of effort (mean of 9.9 months to recruit and 15.4 contacts). There were no significant between-group differences on any baseline variable for ER and PR families, with the exception of family income. CONCLUSIONS: Researchers need to make persistent efforts in order to successfully enroll adolescents of color and their caregivers into clinical trials. Social determinants of health such as family resources may differentiate families with prolonged recruitment within such samples.

Incidence of strabismus, strabismus surgeries, and other vision conditions in Prader-Willi syndrome: data from the Global Prader-Willi Syndrome Registry.

BACKGROUND: There is a relative lack of information on the incidence and treatment of vision problems in Prader-Willi syndrome (PWS). Using data from the Global PWS Registry, we performed a cross-sectional study of vision problems in PWS. METHODS: Data, reported by caregivers who completed the Vision Survey in the Global PWS Registry between May of 2015 and March of 2020, were analyzed using descriptive statistics. RESULTS: There were 908 participants in this survey, with a mean age of 14.5 years (range 0-62 years). The prevalence of strabismus in this population was 40 %, with no statistically significant difference in prevalence by genetic subtype. Ninety-one percent of participants with strabismus were diagnosed before 5 years of age. Of those with strabismus, 42 % went on to have strabismus surgery, with 86 % of those having their first strabismus surgery before 5 years of age and 10.1 % having more than one strabismus surgery. Additional vision issues reported included myopia (41 %), hyperopia (25 %), astigmatism (25 %), and amblyopia (16 %). CONCLUSIONS: The prevalence of strabismus, amblyopia, and hyperopia are considerably higher in the PWS population represented in the Global PWS Registry as compared to the general population. People with PWS should be screened early and regularly for vision problems.

Depressive Symptom Trajectories in Family Caregivers of Stroke Survivors During First Year of Caregiving.

BACKGROUND: The purpose of this study was to identify patterns of depressive symptom trajectory and examine the associations of the symptom trajectory with caregiving burden, family function, social support, and perceived health status of caregivers of stroke survivors during the first year of caregiving after discharge from rehabilitation center. METHODS: Caregivers of stroke survivors completed a survey of depressive symptoms, caregiving burden, family function, perceived availability of social support, and perceived health status at postdischarge and 1 year. Patterns of depressive symptom trajectory (ie, symptom-free, symptom relieved, symptom developed, and persistent symptom groups) were identified by grouping depressive symptoms based on 2 assessments using the Center for Epidemiologic Studies-Depression. Repeated-measures analysis of variance and multinomial logistic regression were used to examine the associations. RESULTS: Of the 102 caregivers, 57.8% were symptom-free, 20.6% experienced persistent depressive symptoms, 11.8% relieved depressive symptoms, and 9.8% developed depressive symptoms. There were significant changes in family function (Wilks λ = 0.914, P = .038) and perceived health status (Wilks λ = 0.914, P = .033) among the groups during the first year of caregiving. The persistent symptom group reported the highest level of burden and the lowest level of family function and perceived availability of social support at both assessment times. Compared with symptom-free caregivers, caregivers with persistent depressive symptoms were 7 times more likely to have fair/poor health rather than excellent/very good health at 1 year (odds ratio, 7.149; P = .012). CONCLUSION: Caregivers with persistent depressive symptoms are the most vulnerable to negative psychosocial outcomes and poor perceived health status during the first year of caregiving from discharge for stroke survivors.

Molecular genetic classification in Prader-Willi syndrome: a multisite cohort study.

BACKGROUND: Prader-Willi syndrome (PWS) is due to errors in genomic imprinting. PWS is recognised as the most common known genetic cause of life-threatening obesity. This report summarises the frequency and further characterises the PWS molecular classes and maternal age effects. METHODS: High-resolution microarrays, comprehensive chromosome 15 genotyping and methylation-specific multiplex ligation probe amplification were used to describe and further characterise molecular classes of maternal disomy 15 (UPD15) considering maternal age. RESULTS: We summarised genetic data from 510 individuals with PWS and 303 (60%) had the 15q11-q13 deletion; 185 (36%) with UPD15 and 22 (4%) with imprinting defects. We further characterised UPD15 findings into subclasses based on the presence (size, location) or absence of loss of heterozygosity (LOH). Additionally, significantly older mothers (mean age=32.5 years vs 27.7 years) were found in the UPD15 group (n=145) compared with the deletion subtype (n=200). CONCLUSIONS: We report on molecular classes in PWS using advanced genomic technology in the largest cohort to date. LOH patterns in UPD15 may impact the risk of having a second genetic condition if the mother carries a recessive mutant allele in the isodisomic region on chromosome 15. The risk of UPD15 may also increase with maternal age.

Perceived Control Predicts Symptom Status in Patients With Heart Failure.

BACKGROUND: Symptom status is an important indicator of disease progression in patients with heart failure (HF). Perceived control is a target of most self-care interventions and is associated with better outcomes in HF; however, little is known about the relationship between perceived control and symptom status in patients with HF. OBJECTIVE: The aims of this study were to (1) determine the relationship of perceived control to HF symptom status and (2) examine the associations of perceived control to self-care and of self-care to symptom status. METHODS: A total of 115 patients with HF were included. Data on symptom status (Memorial Symptom Assessment Scale-HF), perceived control (Control Attitudes Scale-Revised), and self-care (Self-Care of Heart Failure Index) were collected. Other covariates included were age, gender, New York Heart Association class, comorbidity burden, and depressive symptoms. Multiple regression analyses were performed to analyze the data. RESULTS: Lower perceived control predicted worse symptom status after controlling for covariates (P = .009). Other covariates predictive of worse symptom status were younger age, New York Heart Association class III/IV, and higher levels of depressive symptoms. Higher levels of perceived control were associated with better self-care (P = .044). Better self-care was associated with better symptom status (P = .038). CONCLUSIONS: Lower levels of perceived control were independently associated with worse symptom status in patients with HF. Intervention strategies targeting perceived control should be tested to determine whether they could improve symptom status.

Birth seasonality studies in a large Prader-Willi syndrome cohort.

Prader-Willi syndrome (PWS) is generally due to sporadic paternal deletions of the chromosome 15q11-q13 region followed by maternal disomy 15. Advanced maternal age is more commonly seen in those with maternal disomy 15. Environmental factors (e.g., drug use, occupational chemical exposure, infectious agents, and irradiation) could account for chromosome changes. Previous evidence of differences in male and female gametogenesis could suggest an environmental role in the causation of the paternal 15q11-q13 deletion seen in PWS. Certain occupations such as hydrocarbon-exposing occupations (e.g., landscaping, farming, and painting) and viral exposure (e.g., human coronavirus 229E causing upper respiratory infections in adults with an incorporation site in the human genome at chromosome 15q11) can be seasonal in nature and contribute to chromosome damage. To assess, we reviewed birth seasonality data in a large cohort of individuals with PWS recruited nationally (N = 355) but no significant differences were seen by month between those with the 15q11-q13 deletion compared with maternal disomy 15 when analyzing quarterly seasonal patterns. Although early evidence supported birth seasonality differences in PWS, a larger number of individuals in our recent study using advanced genetic testing methods did not find this observation.

Structural Data Showing the Existence of LDI Bonds between the Rings of Dimeric Cofacial Siloxysilicon Phthalocyanines.

In a pair of earlier papers, the existence of long directional interaction bonds, LDI bonds, was postulated on the basis of data for cofacial oligomeric siloxysilicon phthalocyanines from this laboratory and data for other cofacial oligomeric phthalocyanines from the literature. However, the combined data are not fully suited to the purpose for which they were used. Here an alternative approach is taken in which a carefully chosen group of dimeric cofacial siloxysilicon phthalocyanines is used. Structural data derived from these phthalocyanines is examined in some detail to determine where it conforms to normal expectations and where it does not. To a high degree of certainty, consideration of the results obtained shows that long directional (LDI) bonds exist in dimeric cofacial siloxysilicon phthalocyanines. The new data also provide an opportunity for other research on chemical bonds.

Hormonal, metabolic and skeletal phenotype of Schaaf-Yang syndrome: a comparison to Prader-Willi syndrome.

BACKGROUND: Nonsense and frameshift mutations in the maternally imprinted, paternally expressed gene MAGEL2, located in the Prader-Willi critical region 15q11-15q13, have been reported to cause Schaaf-Yang syndrome (SYS), a genetic disorder that manifests as developmental delay/intellectual disability, hypotonia, feeding difficulties and autism spectrum disorder. Prader-Willi syndrome (PWS) is a genetic disorder characterised by severe infantile hypotonia, hypogonadotrophic hypogonadism, early childhood onset obesity/hyperphagia, developmental delay/intellectual disability and short stature. Scoliosis and growth hormone insufficiency are also prevalent in PWS.There is extensive documentation of the endocrine and metabolic phenotypes for PWS, but not for SYS. This study served to investigate the hormonal, metabolic and body composition phenotype of SYS and its potential overlap with PWS. METHODS: In nine individuals with SYS (5 female/4 male; aged 5-17 years), we measured serum ghrelin, glucose, insulin-like growth factor 1 (IGF-1), insulin-like growth factor binding protein 3, follicle-stimulating hormone, luteinising hormone, thyroid-stimulating hormone, free T4, uric acid and testosterone, and performed a comprehensive lipid panel. Patients also underwent X-ray and dual-energy X-ray absorptiometry analyses to assess for scoliosis and bone mineral density. RESULTS: Low IGF-1 levels despite normal weight/adequate nutrition were observed in six patients, suggesting growth hormone deficiency similar to PWS. Fasting ghrelin levels were elevated, as seen in individuals with PWS. X-rays revealed scoliosis >10° in three patients, and abnormal bone mineral density in six patients, indicated by Z-scores of below -2 SDs. CONCLUSION: This is the first analysis of the hormonal, metabolic and body composition phenotype of SYS. Our findings suggest that there is marked, but not complete overlap between PWS and SYS.

Multicentre study of maternal and neonatal outcomes in individuals with Prader-Willi syndrome.

INTRODUCTION: Prader-Willi syndrome (PWS) is a complex genetic disorder associated with three different genetic subtypes: deletion of the paternal copy of 15q11-q13, maternal UPD for chromosome 15 and imprinting defect. Patients are typically diagnosed because of neonatal hypotonia, dysmorphism and feeding difficulties; however, data on the prenatal features of PWS are limited. OBJECTIVE: The aim of the study was to identify and compare frequencies of prenatal and neonatal clinical features of PWS among the three genetic subtypes. METHODS: Data from 355 patients with PWS from the Rare Diseases Clinical Research Network PWS registry were used to analyse multiple maternal and neonatal factors collected during an 8-year multisite study. RESULTS: Among our cohort of 355 patients with PWS (61% deletion, 36% UPD and 3% imprinting defect) 54% were born by caesarean section, 26% were born prematurely and 34% with a low birth weight (frequencies 32%, 9.6% and 8.1%, respectively, in the general population). Fetal movements were reported as decreased in 72%. All babies were hypotonic, and 99% had feeding difficulties. Low Apgar scores (<7) were noted in 17.7% and 5.6% of patients, respectively, compared with 1% and 1.4%, respectively, in the general population. Maternal age and pre-pregnancy weight were significantly higher in the UPD group (p=0.01 and <0.001, respectively). CONCLUSION: We found a higher rate of perinatal complications in PWS syndrome compared with the general population. No significant differences in the genetic subtypes were noted except for a higher maternal age and pre-pregnancy weight in the UPD subgroup.

Diabetes management mediating effects between diabetes symptoms and health-related quality of life in adolescents and young adults with type 1 diabetes.

OBJECTIVES: The primary objective was to investigate the mediating effects of diabetes management in the relationship between diabetes symptoms and generic health-related quality of life (HRQOL) in adolescents and young adults (AYAs) with type 1 diabetes. The secondary objective explored patient health communication and perceived treatment adherence barriers as mediators in a serial multiple mediator model. METHODS: The PedsQL 3.2 Diabetes Module 15-item diabetes symptoms summary score, 18-item diabetes management summary score, and PedsQL 4.0 generic core scales were completed in a 10-site national field test study by 418 AYA aged 13 to 25 years with type 1 diabetes. Diabetes symptoms and diabetes management were tested for bivariate and multivariate linear associations with overall generic HRQOL. Mediational analyses were conducted to test the hypothesized mediating effects of diabetes management as an intervening variable between diabetes symptoms and generic HRQOL. RESULTS: The predictive effects of diabetes symptoms on HRQOL were mediated in part by diabetes management. In predictive analytics models utilizing multiple regression analyses, demographic and clinical covariates, diabetes symptoms, and diabetes management significantly accounted for 53% of the variance in generic HRQOL (P < 0.001), demonstrating a large effect size. Patient health communication and perceived treatment adherence barriers were significant mediators in an exploratory serial multiple mediator model. CONCLUSIONS: Diabetes management explains in part the effects of diabetes symptoms on HRQOL in AYA with type 1 diabetes. Patient health communication to healthcare providers and perceived treatment adherence barriers further explain the mechanism in the relationship between diabetes symptoms and overall HRQOL.

Diabetes symptoms predictors of health-related quality of life in adolescents and young adults with type 1 or type 2 diabetes.

OBJECTIVES: The objective was to investigate the patient-reported diabetes symptoms predictors of generic health-related quality of life (HRQOL) in adolescents and young adults (AYA) with type 1 or type 2 diabetes. METHODS: The 15-item PedsQL™ 3.2 Diabetes Module Diabetes Symptoms Summary Score and PedsQL™ 4.0 Generic Core Scales were completed in a 10-site national field test study by 513 AYA ages 13-25 years with type 1 (n = 424) or type 2 (n = 89) diabetes. Diabetes symptoms were tested for bivariate and multivariate linear associations with generic HRQOL. RESULTS: Diabetes symptoms were associated with decreased HRQOL in bivariate analyses. In predictive analytics models utilizing hierarchical multiple regression analyses controlling for relevant demographic and clinical covariates, diabetes symptoms accounted for 38 and 39% of the variance in patient-reported generic HRQOL for type 1 and type 2 diabetes, respectively, reflecting large effect sizes. The diabetes symptoms facets hyperglycemia symptoms, hypoglycemia symptoms, and nonspecific diabetes symptoms individually accounted for a significant percentage of the variance in separate exploratory predictive analytics models after controlling for demographic and clinical covariates, with small-to-large effect sizes. CONCLUSIONS: Diabetes symptoms are potentially modifiable predictors of generic HRQOL in AYA with diabetes. Identifying specific diabetes symptoms or symptoms facets that are the most important predictors from the patient perspective facilitates a patient-centered approach in clinical research, clinical trials, and practice designed to enhance overall generic HRQOL in AYA with diabetes.