Differences in self-reported weekend catch up sleep between children and adolescents with and without primary hypertension

Background: The data on the association of sleep duration and blood pressure in the pediatric age group have been mixed and most studies have focused on weekday sleep duration. The purpose of this study was to compare the weekday and weekend sleep patterns between children and adolescents with newly diagnosed primary hypertension and a normotensive control group. Methods: Children and adolescents from a pediatric nephrology clinic, aged 6-18 years with newly diagnosed primary hypertension were compared to an age and sex matched normotensive control group from a general pediatric clinic. The questions about bed time and getting out of bed times from the Pediatric Sleep Questionnaire (PSQ) were used to obtain weekday and weekend bed time, getting out of bed time and sleep duration. The Pediatric Daytime Sleepiness Scale (PDSS) was used to assess subjective sleepiness. Results: In both groups of 60 subjects each, weekday total sleep time was similar. Subjects in both groups went to bed later and woke up later on the weekends. However, in the hypertensive group, weekend getting out of the bed time was earlier (8:52 AM +/-93 min vs. 9:36 AM +/-88 min, p = 0.013) and weekend catchup sleep was about 40 min less (62.8 +/- 85.5 vs. 102.7 +/- 84.9, p = 0.035). Hypertensive children perceived less subjective sleepiness (PDSS scores 8.28 +/- 4.88 vs. 10.63 +/- 5.41, p = 0.007). The p values were calculated after adjusting for body mass index (BMI), race, daytime nap, caffeine use, sleep related breathing disorder (SRBD) scale and periodic limb movement of sleep (PLMS) scale subcomponents of the PSQ. Conclusions: Hypertensive children obtained less weekend catch up sleep and reported less subjective sleepiness compared to the control group. More weekend sleep may potentially mitigate the effect of weekday sleep deprivation on blood pressure

Knowledge and Beliefs of EMS Providers toward Lights and Siren Transportation

INTRODUCTION: The use of warning lights and siren (WLS) increases the risk of ambulance collisions. Multiple studies have failed to demonstrate a clinical benefit to the patients. We sought to investigate the degree to which providers understand the data and incorporate it into their practice. METHODS: The authors distributed an anonymous survey to prehospital providers under their medical direction at staff and quality assurance meetings. The surveys asked the providers\u27 degree of agreement with four statements: transport with lights and siren shortens transport times; transport with lights and siren improves patient outcome; transport with lights and siren increases the risk of collision during transport; and transport with lights and siren reduces the utilization of mutual aid service. We compared responses between providers who had been in prior ambulance collisions and those who had not. RESULTS: Few responses reached statistical significance, but respondents tended towards agreement that WLS use shortens transport times, that it does not improve outcomes, and that it increases the risk of collision. Despite the overall agreement with the published literature, respondents report \u3e 80% of transports are conducted using WLS. CONCLUSION: The data demonstrate the surveyed providers are aware of the risk posed by WLS to themselves, their patients, and the public. Nevertheless, their practice in the absence of rigid protocols suggests they disregard this knowledge. Despite a large number of prior ambulance collisions among the surveyed group, a high number of transports are conducted using WLS

Interpretation of the Epigenetic Signature of Facioscapulohumeral Muscular Dystrophy in Light of Genotype-Phenotype Studies

Facioscapulohumeral muscular dystrophy (FSHD) is characterized by incomplete penetrance and intra-familial clinical variability. The disease has been associated with the genetic and epigenetic features of the D4Z4 repetitive elements at 4q35. Recently, D4Z4 hypomethylation has been proposed as a reliable marker in the FSHD diagnosis. We exploited the Italian Registry for FSHD, in which FSHD families are classified using the Clinical Comprehensive Evaluation Form (CCEF). A total of 122 index cases showing a classical FSHD phenotype (CCEF, category A) and 110 relatives were selected to test with the receiver operating characteristic (ROC) curve, the diagnostic and predictive value of D4Z4 methylation. Moreover, we performed DNA methylation analysis in selected large families with reduced penetrance characterized by the co-presence of subjects carriers of one D4Z4 reduced allele with no signs of disease or presenting the classic FSHD clinical phenotype. We observed a wide variability in the D4Z4 methylation levels among index cases revealing no association with clinical manifestation or disease severity. By extending the analysis to family members, we revealed the low predictive value of D4Z4 methylation in detecting the affected condition. In view of the variability in D4Z4 methylation profiles observed in our large cohort, we conclude that D4Z4 methylation does not mirror the clinical expression of FSHD. We recommend that measurement of this epigenetic mark must be interpreted with caution in clinical practice

Is vitamin D deficiency a feature of pediatric celiac disease?

Background: Celiac disease (CD) is an autoimmune enteropathy characterized by villus atrophy and malabsorption of essential nutrients. Vitamin D deficiency has been described in autoimmune diseases, but its status in prepubertal children with CD has not been adequately studied. Objective: To determine the vitamin D status of prepubertal children with CD. Study design: A retrospective study of prepubertal children aged 3–12 years with CD (n=24) who were compared to prepubertal, non-CD children of the same age (n=50). Children were included in the study if they had a diagnosis of CD by intestinal biopsy, and were not on a gluten-free diet (GFD). Patients were excluded if they had diseases of calcium or vitamin D metabolism, or were receiving calcium or vitamin D supplementation or had other autoimmune diseases. All subjects had their serum 25-hydroxyvitamin D [25(OH)D] level measured. Results: There was no difference in 25(OH)D level between the CD and non-CD children (27.58±9.91 vs. 26.20±10.45, p=0.59). However, when the patients were subdivided into obese and non-obese groups, the non-obese CD patients had a significantly higher 25(OH)D level than the obese normal children (28.39±10.26 vs. 21.58±5.67, p=0.009). In contrast, there was no difference in 25(OH)D level between non-obese CD patients and non-obese normal children (28.39±10.26 vs. 30.64±12.08, p=0.52). The season of 25(OH)D measurement was not a significant confounder (p=0.7). Conclusions: Our data showed no difference in 25(OH)D levels between normal children and those with CD when adjusted for body mass index

Serum 25-hydroxyvitamin D levels do not correlate with asthma severity in a case-controlled study of children and adolescents

Background: There is no consensus on the association between vitamin D and asthma. Objective: To determine the relationship between 25-hydroxyvitamin D [25(OH)D] levels and asthma symptom severity in children and adolescents. Methods: A retrospective, case-control study of 263 subjects of ages 2–19 years with asthma who were compared to 284 non-asthmatic controls of similar ages. Subjects were excluded if they had diseases of calcium or vitamin D metabolism or were receiving calcium or vitamin D supplementation. Serum 25(OH)D was measured in all subjects. Asthma symptom severity, usually stratified into 6 steps, was stratified into five steps [1–5] based on the number and dose of controller medications used as outlined by the National Heart, Lung, and Blood Institute’s guidelines. Mean 25(OH)D values were compared between the asthmatic patients and controls, as well as among the five steps of asthma symptom severity. Results were adjusted for age, sex, BMI, race and severity of asthma symptoms. Results:There was no difference in 25(OH)D between asthmatic patients and controls (28.64±10.09 vs. 28.42±11.47, p=1.0). However, there was a significant difference in 25(OH)D between obese and non-obese asthmatic patients (23.33±7.67 vs. 30.16±10.20, p Conclusions: There were no differences in mean 25(OH)D levels between asthmatic patients and controls. Mean 25(OH)D level was significantly lower in both the obese asthmatic patients and obese controls. Asthma severity had no relationship to mean 25(OH)D levels

The relationship between subnormal peak-stimulated growth hormone levels and auxological characteristics in obese children

Context: The hypothesis that obese children are overdiagnosed with growth hormone deficiency (GHD) has not been adequately investigated in the context of adiposity-related differences in auxology. Aim: To investigate the differences in auxological parameters between short, prepubertal, obese children, and normal-weight peers who underwent growth hormone stimulation testing (GHST). Hypothesis: Over-weight/obese children with GHD [peak growth hormone (GH) \u3c 10 μg/L] will have higher values for growth velocity (GV) standard deviation score (SDS), bone age minus chronological age (BA − CA), and child height SDS minus mid-parental height (MPTH) SDS when compared to normal-weight GHD peers. Subjects and Methods: A retrospective review of anthropometric and provocative GHST data of 67 prepubertal, GH-naïve children of age 10.21 ± 2.56 years (male n = 45, age 10.8 ± 2.60 years; female n = 22, age 8.94 ± 2.10). Inclusion criteria: GHST using arginine and clonidine. Exclusion criteria: hypopituitarism, abnormal pituitary magnetic resonance imaging scan, syndromic obesity, or syndromic short stature. Data were expressed as mean ± SD. Results: The over-weight/obese children with peak GH of \u3c10 μg/L had significantly lower value for natural log (ln) peak GH (1.45 ± 0.09 vs. 1.83 ± 0.35, p = 0.022), but similar values for GV SDS, insulin-like growth factor-I, insulin-like growth factor binding protein-3, bone age, BA − CA, MPTH, and child height SDS minus MPTH SDS compared to normal-weight peers with GHD. After adjusting for covariates, the over-weight/obese children (BMI ≥ 85th percentile) were \u3e7 times more likely than normal-weight subjects (BMI \u3c 85th percentile) to have a peak GH of \u3c10 μg/L, and 23 times more likely to have a peak GH of \u3c7 μg/L (OR = 23.3, p = 0.021). There was a significant inverse relationships between BMI SDS and the ln of peak GH (β = −0.40, r2 = 0.26, p = 0.001), but not for BMI SDS vs. GV SDS, ln peak GH vs. BA, or ln peak GH vs. GV SDS. Conclusion: Subnormal peak GH levels in obese prepubertal children are not associated with unique pre-GHST auxological characteristics

Adiposity is associated with early reduction in bone mass in pediatric inflammatory bowel disease

Background: The effect of adiposity on bone mass in the early phases of inflammatory bowel disease (IBD) in children and adolescents is unclear. Aims: To determine the role of adiposity on bone mass in the first 3 years of diagnosis of IBD. Hypothesis: Increased adiposity will be associated with increased bone mass in both the controls and IBD subjects. Setting: University tertiary institution. Methods: Height-adjusted bone mineral density (BMD) z-scores of 25 subjects, age 13.97 ± 2.70y, diagnosed with IBD for \u3c 4 years were compared to 24 controls, age 13.65 ± 2.60y. Overweight was defined as BMI of ≥85th but \u3c95th percentile, and obesity as BMI ≥95thpercentile. Severity of IBD was determined by the Pediatric Crohn’s Disease Activity Index and Lichtiger Colitis Activity Index. Results: Prior to stratification by BMI criterion, height-adjusted BMD z-scores were non-significantly lower in IBD subjects vs. controls for both the femoral neck (-0.8 ± 1.1 vs. -0.06 ± 1.1, p=0.070) and lumbar vertebrae (-0.4 ± 1.2 vs. 0.2 ± 1.2, p=0.086). Following stratification, height-adjusted BMD z-scores were significantly lower in the overweight/obese IBD subjects vs. overweight/obese controls for femoral neck (-0.9 ± 0.9 vs. 0.3 ± 1.3, p=0.032); and non-significantly lower for the lumbar spine z-score (-0.4 ± 1.6 vs. 0.5 ± 1.3, p=0.197). BMD z-score had no relationship with the duration of disease, steroid therapy, and the severity of disease. Conclusion: Adiposity was associated with reduced bone mass in the early phases of IBD, but with increased bone mass in the controls

Health-Care Access during the Ebola Virus Epidemic in Liberia

The Ebola virus disease (EVD) epidemic, which began in West Africa in December 2013, claimed more than 11,000 lives, with more than 4,800 of these deaths occurring in Liberia. The epidemic had an additional effect of paralyzing the health-care systems in affected countries, which led to even greater mortality and morbidity. Little is known about the impact that the epidemic had on the provision of basic health care. During the period from March to May 2015, we undertook a nationwide, community-based survey to learn more about health-care access during the EVD epidemic in Liberia. A cluster sampling strategy was used to administer a structured in-person survey to heads of households located within the catchment areas surrounding all 21 government hospitals in Liberia. A total of 543 heads of household from all 15 counties in Liberia participated in the study; more than half (67%) of urban respondents and 46% of rural respondents stated that it was very difficult or impossible to access health care during the epidemic. In urban areas, only 20-30% of patients seeking care during the epidemic received care, and in rural areas, only 70-80% of those seeking care were able to access it. Patients requiring prenatal and obstetric care and emergency services had the most difficulty accessing care. The results of this survey support the observation that basic health care was extremely difficult to access during the EVD epidemic in Liberia. Our results underscore the critical need to support essential health-care services during humanitarian crises to minimize preventable morbidity and mortality

A 5-year clinical follow-up study from the Italian National Registry for FSHD

BACKGROUND: The natural history of facioscapulohumeral muscular dystrophy (FSHD) is undefined. METHODS: An observational cohort study was conducted in 246 FSHD1 patients. We split the analysis between index cases and carrier relatives and we classified all patients using the Comprehensive Clinical Evaluation Form (CCEF). The disease progression was measured as a variation of the FSHD score performed at baseline and at the end of 5-year follow-up (DeltaFSHD score). FINDINGS: Disease worsened in 79.4% (112/141) of index cases versus 38.1% (40/105) of carrier relatives and advanced more rapidly in index cases (DeltaFSHD score 2.3 versus 1.2). The 79.1% (38/48) of asymptomatic carriers remained asymptomatic. The highest DeltaFSHD score (1.7) was found in subject with facial and scapular weakness at baseline (category A), whereas in subjects with incomplete phenotype (facial or scapular weakness, category B) had lower DeltaFSHD score (0.6) p \u3c 0.0001. CONCLUSIONS: The progression of disease is different between index cases and carrier relatives and the assessment of the CCEF categories has strong prognostic effect in FSHD1 patients

The relationship between adiposity and stature in prepubertal children with celiac disease

Background and Aim: The pathogenesis of short stature in celiac disease (CD) is unknown. Obese children are generally taller than their non-obese peers; however, the role of adiposity on stature in CD is unclear. Our aim was to determine the association between adiposity and stature in CD. Subjects and methods: We compared the anthropometric characteristics of prepubertal children of ages 3-12 years, with biopsy-proven CD (n=40) and who were not on gluten-free diet, to same aged, prepubertal non-CD children (n=50). Body mass index (BMI) was calculated using the formula weight/height2. Sex-adjusted midparental target height (MPTH) standard deviation score (SDS) was calculated using National Children Health Statistics data for 18-year-old adults. Data were expressed as mean±standard deviation. Results: CD subjects had significantly lower BMI SDS than controls (0.61±1.22 vs. 1.28±1.60, p=0.027) but were not significantly shorter than the controls (-0.05±1.21 vs. 0.21±1.71, p=0.41). When the patients were subdivided into the normal-weight and overweight/obese groups, the normal-weight CD patients were of similar height as the normal-weight controls (p=0.76) but were significantly shorter than both the overweight/obese controls (p=0.003). The MPTH SDS did not differ between the groups. Conclusions: Overweight/obese prepubertal children with CD were taller than both their normal-weight CD peers and the normal-weight controls, but were of similar height as the overweight/obese control subjects