Associations between food portion sizes, insulin resistance, VO2 max and metabolic syndrome in European adolescents: The HELENA study.

BACKGROUND AND AIMS: This study aims to examine the associations of food portion size (PS) with markers of insulin resistance (IR) and clustered of metabolic risk score in European adolescents. METHODS: A total of 495 adolescents (53.5% females) from the Healthy Lifestyle in Europe by Nutrition in Adolescence (HELENA) study were included. The association between PS from food groups and homeostasis model assessment of insulin resistance (HOMA-IR) index, VO2 max, and metabolic risk score was assessed by multilinear regression analysis adjusting for several confounders. Analysis of covariance (ANCOVA) was used to determine the mean differences of food PS from food groups by HOMA-IR cutoff categories by using maternal education as a covariable. RESULTS: Larger PS from vegetables in both gender and milk, yoghurt, and milk beverages in males were associated with higher VO2 max, while larger PS from margarines and vegetable oils were associated with lower VO2 max (p < 0.05). Males who consumed larger PS from fish and fish products; meat substitutes, nuts, and pulses; cakes, pies, and biscuits; and sugar, honey, jams, and chocolate have a higher metabolic risk score (p < 0.05). Males with lower HOMA-IR cutoff values consumed larger PS from vegetables, milk, yoghurt, and milk beverages (p < 0.05). Females with lower HOMA-IR cutoff values consumed larger PS from breakfast cereals, while those with higher HOMA-IR cutoff values consumed larger PS from butter and animal fats (p = 0.018). CONCLUSION: The results show that larger PS from dairy products, cereals, and high energy dense foods are a significant determinant of IR and VO2 max, and larger PS from food with higher content of sugar were associated with higher metabolic risk score.

Is it important to achieve physical activity recommendations at early stages of life to improve bone health?

Research in bone health during childhood is limited and important to prevent future diseases, particularly, osteoporosis. Bone parameters using DXA and pQCT in 295 Spanish children were evaluated and we found a benefit of meeting the World Health Organization physical activity recommendations in bone composition in childhood. PURPOSE: To investigate the association between physical activity (PA) and bone health in a Spanish paediatric cohort, considering the influence of meeting/not meeting the current World Health Organization (WHO) PA recommendations and to elucidate if there are differences between boys and girls. METHODS: In a cohort of children born in the region of Aragon (Spain) in 2009, followed until the age of 7 years, bone parameters were assessed using dual-energy X-ray absorptiometry (DXA) (whole body scan) and peripheral quantitative computed tomography (pQCT) (tibia scanned at the 8% (distal) and 38% (diaphyseal) of the total tibia length) in 295 7-year-old children (154 boys) in the last evaluation performed between 2016 and 2017. PA was assessed using GT3X Actigraph accelerometers. RESULTS: Boys had significantly higher areal bone mineral density (aBMD), higher total bone mineral content (BMC) at the diaphyseal site and higher trabecular BMC and vBMD, and higher total bone area at the distal site than girls (p<0.01 for all of them). Both boys and girls complying with the WHO PA recommendations had significantly higher trabecular BMC than their inactive counterparts. CONCLUSIONS: Meeting WHO PA recommendations has a beneficial effect in bone composition in childhood both in boys and in girls.

Enlargement of the Optic Chiasm: A Novel Imaging Finding in Glutaric Aciduria Type 1.

Patients with glutaric aciduria type 1, without early diagnosis and initiation of preventive treatment, often develop movement disorders and various degrees of motor disability due to striatal area-specific damage induced by an acute episode of metabolic decompensation. The neuroimaging phenotype of patients with glutaric aciduria type 1 includes characteristic cyst-like bilateral enlargement of the Sylvian fissures and anterior subarachnoid spaces and signal abnormalities including supratentorial white matter and deep gray matter structure T2 hyperintensities, frequently associated with restricted diffusion. In this retrospective study, we add to the neuroimaging spectrum of glutaric aciduria type 1, a novel imaging finding present regardless of a previous metabolic crisis: the enlargement of the optic chiasm associated with signal abnormalities in the anterior intracranial visual structures observed in 6 of 10 patients. These optic pathway abnormalities are suggested as useful diagnostic clues for glutaric aciduria type 1, and possible pathophysiologic mechanisms are discussed.

Effectiveness and process evaluation in obesity and type 2 diabetes prevention programs in children: a systematic review and meta-analysis.

BACKGROUND: Obesity in children is one of the most severe public health challenges of the current century and Type 2 Diabetes Mellitus (T2DM) frequency is also escalating. More so, the importance of process evaluation (PE) in complex interventions is increasingly recognized. The present review, aims to identify the effectiveness in terms of body composition parameters in a generation of articles to prevent obesity and T2DM in children. We hypothesise that those studies reporting PE applying the latest implementation guidelines suggested by the researchers would potentially show positive changes in body composition compared to those not reporting it. Additionally, we will evaluate the implementation degree of PE in those articles considering it and describe the PE subcomponents. Lastly, we aim to assess the intervention target used and its results. METHODS: A literature review was performed in parallel by 2 independent reviewers. A final number of 41 studies were selected for inclusion criteria. RESULTS: Meta-analysis of BMI and zBMI found non-significant effects of the proposed interventions. Sub-group analysis revealed only a significant effect in studies which performed PE. Moreover, PE was reported in 42% effective studies and 57% non-effective studies. Fidelity and satisfaction were the most implemented PE subcomponents, although there was a generally low grade of PE use (7/41). The highest proportion of effectiveness (83%) was shown in interventions of physical activity alone while the intervention most used was 3-arm target (diet, PA and BS). CONCLUSIONS: Overall, obesity and T2DM prevention studies included in this review are not effective in terms of BMI and zBMI. Those studies performing PE reported to be effective in terms of BMI, while studies not reporting PE did not have positive results in terms of BMI and zBMI. In addition, none of the intervention studies included all PE indicators and most studies, which included PE in their interventions, did not provide full report of the PE components, according to the guidelines used for the present review. PROSPERO registration number: CRD42018093667.

Incidence and morphology of the incisive suture in CT scanning of young children and human fetuses.

PURPOSE: Incisive suture is a suture classically described on the oral face of the palate in fetuses and young children. The aim of our study was to describe the evolution of the incisive suture in human fetuses and to evaluate the incidence of this suture in a population of young children under 4 years, to determine if there is a possibility of improving the anterior growth of the maxilla, by stimulation of this suture. METHODS: One hundred and thirty CT scan images of patients aged from birth to 48 months have been studied and nine fetal palates aged from 18 to 26 weeks of development, have been scanned using high-resolution X-ray micro-computed tomography RESULTS: The CT scan images of patients showed that an incisive suture was present in 33/130 cases (25,4%). All the patients with a suture were under 2 years old. The fetal palate study showed that the suture was present in the inferior aspect of the palate (oral cavity) in all cases. The incisive suture increased from 18 to 24 weeks. At 26 weeks it stopped growing although the intercanine length increased. Considering the closure of the suture in a vertical plane, our study on fetuses has shown that the incisive suture is closing from its superior side (nasal side) to its inferior side. CONCLUSIONS: Considering all these results it appears to us that the incisive suture is partially ossified after birth, it cannot be stimulated by orthodontic appliances.

Fluid consumption, total water intake and first morning urine osmolality in Spanish adolescents from Zaragoza: data from the HELENA study.

BACKGROUND/OBJECTIVES: The objective of the study is to describe the hydration status and to assess the main food- and/or fluid intake-associated factors in healthy adolescents. SUBJECTS/METHODS: A total of 194 adolescents from the city of Zaragoza aged 12.5-17.5 years (99 males) participating in the 'Healthy Lifestyle in Europe by Nutrition in Adolescence' (HELENA) cross-sectional study were included. First morning urine was collected, and osmolality was determined by freezing point depression osmometer. A self-reported computer-based 24-h dietary recall was applied the same day of the urine collection. Analysis of variance, Kruskal-Wallis procedure or Pearson's χ(2) analyses was used to examine the group associations. RESULTS: Seventy-one percent of adolescents did not meet the European Food Safety Agency (EFSA) recommendations for average total water intake (TWI), and 68% had high first morning urine osmolality values. TWI and the proportion of those meeting EFSA reference values significantly (P<0.05) decreased with increased osmolality. Males who met the EFSA recommendations consumed significantly (P<0.05) more plain water (1035.13 vs 582.68 ml) and dairy drinks (368.13 vs 226.68 ml) than those who did not. In females, the consumption of water (1359.41 vs 620.44 ml) and sugar-sweetened beverages (214.61 vs 127.42 ml) was significantly higher in those meeting the EFSA recommendations than in those who did not. First morning urine osmolality was associated with vegetables (unstandardized ß: -0.6; 95% confidence interval (CI): -1.02 and -0.18) and fruits intake (ß: -0.41; 95% CI: -0.63 and -0.19) in males and with dairy drinks (ß: -0.39; 95% CI: -0.76 and -0.02) and fruits (ß: -0.41; 95% CI: -0.73 and -0.10) in females. CONCLUSIONS: There was a high prevalence of inadequate TWI and high urine osmolality among these Spanish adolescents. Lower urine osmolality was associated with higher consumption of vegetables in males, dairy drinks in females and fruits in both males and females.

SOX10 mutations mimic isolated hearing loss.

Ninety genes have been identified to date that are involved in non-syndromic hearing loss, and more than 300 different forms of syndromic hearing impairment have been described. Mutations in SOX10, one of the genes contributing to syndromic hearing loss, induce a large range of phenotypes, including several subtypes of Waardenburg syndrome and Kallmann syndrome with deafness. In addition, rare mutations have been identified in patients with isolated signs of these diseases. We used the recent characterization of temporal bone imaging aspects in patients with SOX10 mutations to identify possible patients with isolated hearing loss due to SOX10 mutation. We selected 21 patients with isolated deafness and temporal bone morphological defects for mutational screening. We identified two SOX10 mutations and found that both resulted in a non-functional protein in vitro. Re-evaluation of the two affected patients showed that both had previously undiagnosed olfactory defects. Diagnosis of anosmia or hyposmia in young children is challenging, and particularly in the absence of magnetic resonance imaging (MRI), SOX10 mutations can mimic non-syndromic hearing impairment. MRI should complete temporal bones computed tomographic scan in the management of congenital deafness as it can detect brain anomalies, cochlear nerve defects, and olfactory bulb malformation in addition to inner ear malformations.

Simplified gyral pattern in severe developmental microcephalies? New insights from allometric modeling for spatial and spectral analysis of gyrification.

The strong positive-allometric relationship between brain size, cortical extension and gyrification complexity, recently highlighted in the general population, could be modified by brain developmental disorders. Indeed, in case of brain growth insufficiency, the pathophysiological relevance of the "simplified gyral pattern" phenotype is strongly disputed since almost no genotype-phenotype correlations have been found in primary microcephalies. Using surface scaling analysis and newly-developed spectral analysis of gyrification (Spangy), we tested whether the gyral simplification in groups of severe microcephalies related to ASPM, PQBP1 or fetal-alcohol-syndrome could be fully explained by brain size reduction according to the allometric scaling law established in typically-developing control groups, or whether an additional disease effect was to be suspected. We found the surface area reductions to be fully explained by scaling effect, leading to predictable folding intensities measured by gyrification indices. As for folding pattern assessed by spectral analysis, scaling effect also accounted for the majority of the variations, but an additional negative or positive disease effect was found in the case of ASPM and PQBP1-linked microcephalies, respectively. Our results point out the necessity of taking allometric scaling into account when studying the gyrification variability in pathological conditions. They also show that the quantitative analysis of gyrification complexity through spectral analysis can enable distinguishing between even (predictable, non-specific) and uneven (unpredictable, maybe disease-specific) gyral simplifications.

[Audiophonological evaluation of 16 children fitted with cochlear implants for sensorineural hearing loss induced by bacterial meningitis]. / Implants cochléaires dans les surdités après méningite bactérienne: suivi audiologique de 16 enfants.

BACKGROUND: Bacterial meningitis (BM) is the primary etiology of acquired sensorineural hearing loss (SNHL) in children and may compromise language development. Since the 1990 s, cochlear implants (CIs) have become part of the management of children with profound SNHL with encouraging results. The aim of this study was to analyze the audiophonological performance of children before and after cochlear implantation for SNHL following bacterial meningitis. METHODS: Retrospective study of all children fitted with CIs for bilateral severe to profound SNHL after bacterial meningitis in the Robert-Debré pediatric ENT department between August 1990 and March 2009. Audiophonological performance was assessed using the APCEI profile. RESULTS: Of the 283 children receiving implants during that period, 16 children (6%; 6 boys, 10 girls) underwent CI implantation after bacterial meningitis (Streptococcus pneumoniae in 8 cases, Neisseria meningitidis in 2 cases, and Haemophilus influenzae in 4 cases). The mean time from meningitis to SNHL was 8.3 months (median, 1.5 months; range, 1 day to 13 years). The mean time from meningitis to cochlear implantation was 2 years and 3 months (median, 7 months; range, 1 month to 13 years 3 months). Twelve children (75%) presented partial cochlear and/or vestibular ossification on presurgical CT scan. Three children received bilateral implants. DISCUSSION: Thirteen children (81%) developed early SNHL in the first 3 months, whereas 3 children developed SNHL more than 10 months after meningitis. As for the benefits of cochlear implantation, 11 children presented near to normal intelligibility and optimal use of their cochlear implant; 5 children presented partial benefits due to neurological sequelae (1), a long delay before implantation (1), technical problems (2), or a social problem in relation to low socioeconomic status (1). CONCLUSION: After bacterial meningitis, audiological evaluation must be made carefully during the first 3 months to detect early SNHL, but SNHL may also develop several years later. In case of profound SNHL and a modified signal of the labyrinth on the MRI, cochlear implantation must be performed without delay before cochlear and/or vestibular ossification. Cochlear implantation is an effective technique with good long-term audiologic results. The coexistence of neurological lesions may compromise the results, but it should not contraindicate a cochlear implantation.

Spectrum of temporal bone abnormalities in patients with Waardenburg syndrome and SOX10 mutations.

BACKGROUND AND PURPOSE: Waardenburg syndrome, characterized by deafness and pigmentation abnormalities, is clinically and genetically heterogeneous, consisting of 4 distinct subtypes and involving several genes. SOX10 mutations have been found both in types 2 and 4 Waardenburg syndrome and neurologic variants. The purpose of this study was to evaluate both the full spectrum and relative frequencies of inner ear malformations in these patients. MATERIALS AND METHODS: Fifteen patients with Waardenburg syndrome and different SOX10 mutations were studied retrospectively. Imaging was performed between February 2000 and March 2010 for cochlear implant work-up, diagnosis of hearing loss, and/or evaluation of neurologic impairment. Eleven patients had both CT and MR imaging examinations, 3 had MR imaging only, and 1 had CT only. RESULTS: Temporal bone abnormalities were bilateral. The most frequent pattern associated agenesis or hypoplasia of ≥1 semicircular canal, an enlarged vestibule, and a cochlea with a reduced size and occasionally an abnormal shape, but with normal partition in the 13/15 cases that could be analyzed. Three patients lacked a cochlear nerve, bilaterally in 2 patients. In addition, associated abnormalities were found when adequate MR imaging sequences were available: agenesis of the olfactory bulbs (7/8), hypoplastic or absent lacrimal glands (11/14), hypoplastic parotid glands (12/14), and white matter signal anomalies (7/13). CONCLUSIONS: In the appropriate clinical context, bilateral agenesis or hypoplasia of the semicircular canals or both, associated with an enlarged vestibule and a cochlear deformity, strongly suggests a diagnosis of Waardenburg syndrome linked to a SOX10 mutation.

Sex- and tissue-specific expression of "similar to nothepsin" and cathepsin D in relation to egg yolk formation in Gallus gallus.

Egg yolk constitutes the main storage compartment of the avian egg and the first nutritional source that supports embryonic growth. Most egg yolk components are synthesized by the liver of laying hens at sexual maturity and are secreted into the blood to be further transferred into the ovarian oocyte (yolky follicle) by receptor-mediated endocytosis. Egg yolk proteins are secreted as precursors and must undergo proteolytic processing to be bioactive. It is assumed that chicken cathepsin D, an aspartic protease, is a key enzyme in this process. Very recently, a novel aspartic protease, namely "similar to nothepsin," has been identified in the egg yolk. Previous experiments conducted in Antarctic fish have shown that the expression of nothepsin is tissue- and sex-specific. To gain insight into the specificities of expression of both cathepsin D and "similar to nothepsin" in Gallus gallus, we compared their distribution in various tissues, in male and females. Cathepsin D is ubiquitously expressed in all tissues examined, including liver of both male and female adults, and its expression is stable during sexual maturation. In contrast, "similar to nothepsin" expression is unique to the liver of adult females and is sex steroid-dependent as it increases gradually in the liver of hens during sexual maturation. The sexual dimorphic expression of the "similar to nothepsin" gene suggests that the activity of this protein is regulated by the steroid environment of laying hens and is specifically adapted for inclusion in the yolk. Further studies are needed to assess whether "similar to nothepsin" assists cathepsin D in the proteolytic processing of egg yolk proteins during follicular growth.

[Computed tomography of the normal and pathologic temporal bone]. / Anatomie tomodensitométrique de l'oreille normale et malformée.

High-resolution computed tomography scanning (CT) allows depiction of microanatomic structures of the temporal bone. CT is useful for detecting several pathologic conditions of the temporal bone such as congenital malformations, particularly in young children with sensorineural hearing loss. Some external, middle and inner ear structures are difficult to evaluate. The objective of this study has been to provide the key planes in coronal and axial planes (five coronal planes and three axial planes) but also with oblique planes reconstruction (two planes) for normal temporal bones evaluation. These standardized planes help to improve visualization of the main congenital malformations. Identification of obvious morphogenetic malformations (Michel aplasia, Mondini deformity….) is not difficult. However, less severe dysplasia may be missed or normal micro anatomic structures in newborn misreaded.

Sialoblastoma of salivary glands in children: chemotherapy should be discussed as an alternative to mutilating surgery.

Sialoblastoma is a very rare congenital salivary gland tumor. No consensus has been reached concerning the treatment of this tumor due to its rarity. The treatment of reference is surgery, which can be mutilating, in the case of a locally invasive tumor. The treatment of metastatic disease is also controversial. The authors report a new case of a 6-year-old girl with a progressively growing left parotid mass since birth. The first cytological diagnosis was that of pleomorphic adenoma. Due to local progression, superficial parotidectomy was performed at the age of 3.5 years and revealed a diagnosis of sialoblastoma. Six months later, local recurrence and lung metastasis were treated by neoadjuvant chemotherapy with a very good partial response on the local recurrence and the lung metastasis, allowing complete parotidectomy with sacrifice of the facial nerve. Bilateral lung biopsies after adjuvant chemotherapy showed total necrosis. No recurrence was observed with a follow-up of 1 year. This case and a review of the literature confirm the very good chemosensitivity of this tumor and argue in favor of neoadjuvant chemotherapy for locally invasive tumors rather than extensive mutilating surgery.