Background Head circumference (HC) charts are important for early detection of hydrocephalus during childhood. In low-income countries where population-based HC charts are rarely available, ...hydrocephalus occurs more commonly than in developed countries, and is usually not diagnosed early enough to prevent severe brain damage. This applies to Ethiopia as well. The World Health Organization (WHO) has provided standard HC charts advocated for global use, but recent studies cast doubts whether these charts are equally applicable in various populations. The aim of the study was therefore to establish reference ranges for early childhood HC in Ethiopia. Methods In this prospective, observational cross-sectional study, measurements of HC were collected from healthy children of different ethnicities between birth and 24 months, in health centers situated in 5 Ethiopian cities. Reference ranges for HC were estimated using the LMS method and compared with those recommended by WHO. Results A total of 4019 children were included. Overall, 6.7% of boys and 7.1% of girls were above the +2 standard deviation (SD) of the WHO reference ranges, whereas the corresponding figures below −2 SD were 2.8% and 2.1%. Similarly, the +2 SD lines of the Ethiopian reference curves were considerably higher than those of the WHO growth standards, whereas the median and −2 SD lines were more comparable. Conclusions Ethiopian HC reference ranges for children from birth to 24 months of age were found to differ significantly from those established by WHO and should correspondingly be considered as the first choice for screening for hydrocephalus in that population.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
KCNJ11 mutations cause permanent neonatal diabetes through pancreatic ATP-sensitive potassium channel activation. 90% of patients successfully transfer from insulin to oral sulfonylureas with ...excellent initial glycaemic control; however, whether this control is maintained in the long term is unclear. Sulfonylurea failure is seen in about 44% of people with type 2 diabetes after 5 years of treatment. Therefore, we did a 10-year multicentre follow-up study of a large international cohort of patients with KCNJ11 permanent neonatal diabetes to address the key questions relating to long-term efficacy and safety of sulfonylureas in these patients.
In this multicentre, international cohort study, all patients diagnosed with KCNJ11 permanent neonatal diabetes at five laboratories in Exeter (UK), Rome (Italy), Bergen (Norway), Paris (France), and Krakow (Poland), who transferred from insulin to oral sulfonylureas before Nov 30, 2006, were eligible for inclusion. Clinicians collected clinical characteristics and annual data relating to glycaemic control, sulfonylurea dose, severe hypoglycaemia, side-effects, diabetes complications, and growth. The main outcomes of interest were sulfonylurea failure, defined as permanent reintroduction of daily insulin, and metabolic control, specifically HbA1c and sulfonylurea dose. Neurological features associated with KCNJ11 permanent neonatal diabetes were also assessed. This study is registered with ClinicalTrials.gov, number NCT02624817.
90 patients were identified as being eligible for inclusion and 81 were enrolled in the study and provided long-term (>5·5 years cut-off) outcome data. Median follow-up duration for the whole cohort was 10·2 years (IQR 9·3–10·8). At most recent follow-up (between Dec 1, 2012, and Oct 4, 2016), 75 (93%) of 81 participants remained on sulfonylurea therapy alone. Excellent glycaemic control was maintained for patients for whom we had paired data on HbA1c and sulfonylurea at all time points (ie, pre-transfer for HbA1c, year 1, and most recent follow-up; n=64)—median HbA1c was 8·1% (IQR 7·2–9·2; 65·0 mmol/mol 55·2–77·1) before transfer to sulfonylureas, 5·9% (5·4–6·5; 41·0 mmol/mol 35·5–47·5; p<0·0001 vs pre-transfer) at 1 year, and 6·4% (5·9–7·3; 46·4 mmol/mol 41·0–56·3; p<0·0001 vs year 1) at most recent follow-up (median 10·3 years IQR 9·2–10·9). In the same patients, median sulfonylurea dose at 1 year was 0·30 mg/kg per day (0·14–0·53) and at most recent follow-up visit was 0·23 mg/kg per day (0·12–0·41; p=0·03). No reports of severe hypoglycaemia were recorded in 809 patient-years of follow-up for the whole cohort (n=81). 11 (14%) patients reported mild, transient side-effects, but did not need to stop sulfonylurea therapy. Seven (9%) patients had microvascular complications; these patients had been taking insulin longer than those without complications (median age at transfer to sulfonylureas 20·5 years IQR 10·5–24·0 vs 4·1 years 1·3–10·2; p=0·0005). Initial improvement was noted following transfer to sulfonylureas in 18 (47%) of 38 patients with CNS features. After long-term therapy with sulfonylureas, CNS features were seen in 52 (64%) of 81 patients.
High-dose sulfonylurea therapy is an appropriate treatment for patients with KCNJ11 permanent neonatal diabetes from diagnosis. This therapy is safe and highly effective, maintaining excellent glycaemic control for at least 10 years.
Wellcome Trust, Diabetes UK, Royal Society, European Research Council, Norwegian Research Council, Kristian Gerhard Jebsen Foundation, Western Norway Regional Health Authority, Southern and Eastern Norway Regional Health Authority, Italian Ministry of Health, Aide aux Jeunes Diabetiques, Societe Francophone du Diabete, Ipsen, Slovak Research and Development Agency, and Research and Development Operational Programme funded by the European Regional Development Fund.
Aim To compare the prevalence of gastrostomy tube feeding (GTF) of children with cerebral palsy (CP) in six European countries.
Method Data on 1295 children (754 males, 541 females; mean age 5y ...11mo, range 11y 2mo, min 6mo, max 11y 8mo) with CP born from 1999 to 2001 were collected from geographically defined areas in six European countries; four of the areas covered the whole country. Distribution of CP was unilateral 37%, bilateral 51%, dyskinetic 8%, and ataxic 4%. Sixty children were classified in Gross Motor Function Classification System (GMFCS) levels I and II, 6 in level III and 34 in levels IV and Vas Outcome measures were GTF, age at placement, feeding difficulties and the children’s height and weight for age standard deviation scores (z‐scores).
Results The use of GTF among all children with CP was highest in western Sweden (22%, 95% confidence interval CI 16–29), and lowest in Portugal (6%, 95% CI 3–10), northern England (6%, 95% CI 3–9) and in Iceland (3%, 95% CI 0–13; p<0.001). The difference between areas was greater among children in GMFCS levels IV and V (non‐ambulant); in this group, lower height z‐scores were more prevalent in the areas with lower prevalence of GTF. The children’s age at placement of gastrostomy also varied between areas (p<0.002).
Interpretation The observed differences in the use of GTF may reflect differences in access to treatment or clinical practice, or both. Our results suggest that the use of GTF may improve growth in height and weight among children with more severely affected gross motor function – the group most likely to have associated feeding difficulties.
This article is commented on by Sullivan on page 876‐877 of this issue.
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BFBNIB, FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
► Children’s Big Five personality traits predict eating fattening foods and overweight. ► Associations are partly gender-specific, but independent of age. ► In boys, all the Big Five predict eating ...fruit and vegetables. In girls, this is true for imagination and benevolence. ► In girls, but not boys, neuroticism is associated with drinking sweet drinks. ► Introverted boys and girls, as well as unbenevolent, unconscientious, and neurotic girls tend to be overweight.
The role of children’s personality traits in the consumption of potentially obesogenic foods was investigated in a sample of Norwegian children aged 6–12years (N=327, 170 boys, 157 girls). Mothers rated their child’s personality on the traits of the Five Factor Model (i.e., extraversion, benevolence, conscientiousness, neuroticism, and imagination). Mothers also completed a food frequency questionnaire assessing their child’s consumption of sweet drinks, sweet foods, and fruit and vegetables, and reported their child’s height and weight. Controlling for age and mothers’ education, boys and girls who were less benevolent consumed more sweet drinks, and girls who were less conscientious and more neurotic consumed more sweet drinks. Boys and girls who were more benevolent and imaginative consumed more fruits and vegetables, and boys who were more extraverted, more conscientious, and less neurotic consumed more fruits and vegetables. Controlling for maternal education, boys and girls who were less extraverted, and girls who were less benevolent, less conscientious, and more neurotic were more likely to be overweight or obese. These findings suggest that children’s personality traits play an important yet understudied role in their diet. Further investigation of mechanisms that relate child traits to obesogenic eating and overweight would be valuable.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
A 4-generation family with multiple synostoses syndrome type 4 (SYNS4) is reported, the third family identified so far. The phenotype segregated with a previously undescribed Asn399Lys (c.1197C>A) ...substitution in GDF6. N399 is part of a hydrophobic pocket critical for binding the BMP/GDF antagonist noggin. The N399K substitution renders GDF6 more similar to noggin-resistant members of the BMP family, namely GDF2 and BMP10, both of which contain lysine in the corresponding position. To further define the SYNS4 phenotype, we examined 6 of 9 affected family members. The phenotype was carpal and tarsal synostoses with painful feet after walking, but the condition could also be asymptomatic. Interestingly, unlike the previous SYNS4 families, the family presented here has no history of hearing loss, and a 73-year-old mutation carrier had normal audiometry for his age. Based on structure modelling, BMPR2 binding should not be affected by the GDF6-N399K substitution, unlike the S429R and Y444N mutations found in the 2 other families. Hypothetically, this difference may be related to lack of hearing loss.
Normal Liver Stiffness Values in Children Mjelle, Anders B.; Mulabecirovic, Anesa; Havre, Roald F. ...
Journal of pediatric gastroenterology and nutrition,
20/May , Volume:
68, Issue:
5
Journal Article
Peer reviewed
Open access
ABSTRACT
Objectives:
Noninvasive tests for the evaluation of liver fibrosis are particularly helpful in children to avoid general anesthesia and potential complications of invasive tests. We aimed to ...establish reference values for 2 different elastography methods in a head‐to‐head comparison for children and adolescents 4 to 17 years, using transient elastography as common reference in a subset.
Methods:
A total of 243 healthy participants aged 4 to 17 years were examined by a single observer with a full liver B‐mode scan before elastography, following a minimum of 3 hours fasting. Liver stiffness measurements (LSMs) using 2‐dimensional shear wave elastography (2D‐SWE, GE Logiq E9) and point shear wave elastography (pSWE, Samsung RS80A with Prestige) were performed in all participants, and compared to transient elastography (TE, FibroScan) in a subset (n = 87). Interobserver agreement was evaluated in 50 children aged 4 to 17 years.
Results:
Valid measurements were obtained in 242 of 243 (99.6%) subjects for 2D‐SWE, 238 of 243 (97.9%) for pSWE, and in 83 of 87 (95.4%) for TE. Median liver stiffness overall was 3.3 (interquartile range IQR 2.7–4.3), 4.1 (IQR 3.6–4.7), and 4.1 kPa (IQR 3.5–4.6) for 2D‐SWE, pSWE, and TE, respectively. Intraclass correlation coefficients between observers were 0.84 and 0.83 for 2D‐SWE and pSWE, respectively. LSM values were significantly lower for 2D‐SWE compared to pSWE and TE, and increased with advancing age. Higher LSM values in males were observed in adolescents.
Conclusions:
All methods showed excellent feasibility. 2D‐SWE showed significantly lower LSM values than pSWE and TE, and lower failure rate compared to TE. Our results further indicate an age and sex effect on LSM values.
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FZAB, GIS, IJS, KILJ, NLZOH, NUK, OILJ, SBCE, SBMB, UL, UM, UPUK
Experiments in animal models have shown a positive association between in utero exposure to pharmacologic sex hormones and offspring obesity. The developmental effects of such hormones on human ...obesity are unknown.
Using data from a large, prospective pregnancy cohort study (n=19 652), with linkage to a national prescription registry, we evaluated the association between use of hormonal contraceptives before and after conception (defined from dispensed prescription data and characterized by last date of use relative to conception, 12 to >4 months before (n=3392), 4 to >1 months before (n=2541), 1 to >0 months before (n=2997) and 0-12 weeks after (n=567)) in relation to offspring overweight or obesity at age 3 years.
We observed a weak, inverse association between early pregnancy use of a combination oral contraceptive and offspring overweight or obesity at age 3 (adjusted odds ratio (OR): 0.75, 95% confidence interval (CI): 0.53, 1.08) and a positive, but imprecise, association with use of a progestin-only oral contraceptive in early pregnancy (adjusted OR: 1.26, 95% CI: 0.79, 2.02). In general, no association was observed between the use of a hormonal contraceptive before conception and offspring overweight or obesity. A sensitivity analysis comparing combination oral contraceptive users in early pregnancy to other unplanned pregnancies without hormonal contraceptive use further strengthened the inverse association (adjusted OR: 0.70, 95% CI: 0.48, 1.02). Other sensitivity analyses were conducted to evaluate the robustness of the associations observed given varying assumptions.
Pharmacologic sex hormones in early pregnancy may be inversely or positively associated with offspring overweight or obesity at age 3, depending on the specific formulation used. The present study provides support for the potential for environmental sources of hormonally active agents to exert developmental effects.
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DOBA, EMUNI, FIS, FZAB, GEOZS, GIS, IJS, IMTLJ, IZUM, KILJ, KISLJ, MFDPS, NLZOH, NUK, OILJ, PILJ, PNG, SAZU, SBCE, SBJE, SBMB, SBNM, UILJ, UKNU, UL, UM, UPUK, VKSCE, ZAGLJ
Summary
Background
Personality may be an important yet understudied influence on childhood obesity.
Objective
We investigated the association between children's personality traits and weight status ...in a sample of 1533 6–12 year olds.
Methods
Mothers rated their child's personality using the Norwegian Hierarchical Personality Inventory for Children, and reported on their child's height and weight.
Results
Relative to their normal weight peers, overweight children were rated lower on energy, optimism, compliance, concentration, perseverance, and self‐confidence, and higher on egocentricity, irritability, and anxiety.
Conclusions
These findings suggest possible mechanisms to investigate in future research relating personality to childhood obesity.
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BFBNIB, FZAB, GIS, IJS, IZUM, KILJ, NLZOH, NUK, OILJ, PILJ, PNG, SAZU, SBCE, SBMB, UL, UM, UPUK, VSZLJ