Translation and validation of the French version of the child perceptions questionnaire for children aged 11 to 14 years old (CPQ11-14) short-form.

OBJECTIVES: The COHQoL is a set of questionnaires used to evaluate the impact of oral health on children's quality of life. Although the CPQ8-10 and the P-CPQ have been translated and validated in French, the CPQ11-14 14 has not yet been validated. The aim was to develop a French version of the CPQ11-14 16-items. MATERIALS AND METHODS: The French version of CPQ11-14 was obtained by a forward-backward translation process and pretested. The final version was tested on children aged 11-14 and divided into three groups: children with orofacial clefts, children with rare dental diseases other than clefts, and children without anomalies. We conducted a cross-sectional study and evaluated the reliability with test-retest and internal consistency, and the questionnaire validity with construct validity and discriminant validity. We performed an Exploratory Factory Analysis (EFA). RESULTS: 187 children tested the questionnaire. The ICC of the test-retest was 0.76 and the Cronbach's alpha was 0.77. The correlation between the CPQ11-14 and self-assessment of oral health and general well-being was > 0.2. Patients with orofacial clefts and rare diseases had significantly higher scores for overall short-form CPQ11-14. The EFA revealed six factors. CONCLUSION: The French CPQ11-14 is valid to assess the impact of oral health on children's quality of life. CLINICAL RELEVANCE: The translation of this questionnaire into French will enable us to assess the impact of oral health on the quality of life of adolescents. This questionnaire complements the 8-10 years version of the CPQ, as well as the parental version that can be used in conjunction with the questionnaire.

Transfontanellar shear wave elastography of the neonatal brain for quantitative evaluation of white matter damage.

Cerebral white matter damage (WMD) is the most frequent brain lesion observed in infants surviving premature birth. Qualitative B-mode cranial ultrasound (cUS) is widely used to assess brain integrity at bedside. Its limitations include lower discriminatory power to predict long-term outcomes compared to magnetic resonance imaging (MRI). Shear wave elastography (SWE), a promising ultrasound imaging modality, might improve this limitation by detecting quantitative differences in tissue stiffness. The study enrolled 90 neonates (52% female, mean gestational age = 30.1 ± 4.5 weeks), including 78 preterm and 12 term controls. Preterm neonates underwent B-mode and SWE assessments in frontal white matter (WM), parietal WM, and thalami on day of life (DOL) 3, DOL8, DOL21, 40 weeks, and MRI at term equivalent age (TEA). Term infants were assessed on DOL3 only. Our data revealed that brain stiffness increased with gestational age in preterm infants but remained lower at TEA compared to the control group. In the frontal WM, elasticity values were lower in preterm infants with WMD detected on B-mode or MRI at TEA and show a good predictive value at DOL3. Thus, brain stiffness measurement using SWE could be a useful screening method for early identification of preterm infants at high WMD risk.Registration numbers: EudraCT number ID-RCB: 2012-A01530-43, ClinicalTrial.gov number NCT02042716.

Association between Portal Vein Thrombosis after Umbilical Vein Catheterization and Neonatal Asphyxia.

INTRODUCTION: Neonatal portal vein thrombosis (PVT) is frequently related to umbilical venous catheterization (UVC), but risk factors remain unclear. This study aims to analyze the variables associated to PVT in near- to full-term newborns with UVC, with a focus on newborns exposed to controlled therapeutic hypothermia (CTH) for hypoxic ischemic encephalopathy (HIE). METHODS: This is retrospective cohort study of infants delivered at or after 36 weeks and with a birthweight over 1,500 g. All infants were assessed for UVC location and PVT using ultrasonography performed between day 5 and day 10 after catheterization. RESULTS: Among 213 eligible patients, PVT was diagnosed in 57 (27%); among them, 54 (95%) were localized in the left portal vein branch. With all significant factors in univariate analysis considered, higher gestational age at birth (adjusted OR 1.35; 95% CI: 1.12-1.64, p = 0.002) and duration of UVC placement (adjusted OR 1.36; 95% CI: 1.11-1.67, p = 0.004) were the main risk factors of PVT. Among 87 infants who were cooled for HIE, 31 (36%) had PVT compared to 26 (21%) in infants without CTH. Using a multivariate model including variables linked to treatment procedures only, an increased PVT incidence was statistically associated with UVC duration (adjusted OR 1.33; 95% CI: 1.08; 1.63, p = 0.01) and CTH (adjusted OR 1.94; 95% CI: 1.04-3.65, p = 0.04). CONCLUSION: Left PVT was frequently observed in near- to full-term neonates with UVC. Among factors linked to treatment procedures, both duration of UVC and CTH exposure for HIE were found to be independent risk factors of PVT.

Calculating the fraction of Kawasaki disease potentially attributable to seasonal pathogens: a time series analysis.

Background: Kawasaki disease is an acute, febrile, systemic vasculitis of children that primarily affects medium-sized blood vessels with a tropism for the coronary arteries. Although the etiological factors remain unknown, infections have been suggested as the trigger of Kawasaki disease. We sought to calculate the fraction of Kawasaki disease potentially attributable to seasonal infections. Methods: This cohort study used a population-based time series analysis from the French hospitalisation database (Programme de Médicalisation des Systèmes d'Information), which includes all inpatients admitted to any public or private hospital in France. We included all children aged 0-17 years hospitalised for Kawasaki disease in France over 13 years. The monthly incidence of Kawasaki disease per 10,000 children over time was analysed by a quasi-Poisson regression model. The model accounted for seasonality by using harmonic terms (a pair of sines and cosines with 12-month periods). The circulation of eight common seasonal pathogens (adenovirus, influenza, metapneumovirus, Mycoplasma pneumoniae, norovirus, rhinovirus, rotavirus, respiratory syncytial virus, and Streptococcus pneumonia) over the same period was included in the model to analyse the fraction of Kawasaki disease potentially attributable to each pathogen. Infections were identified on the basis of polymerase chain reaction or rapid antigen testing in hospital laboratories. Findings: Between Jan 1, 2007, and Dec 31, 2019, we included 10,337 children with Kawasaki disease and 442,762 children with the selected infectious diseases. In the Kawasaki disease cohort, the median age [IQR] was 2 [0-4] years, 6164 [59.6%] were boys. Adenovirus infection was potentially responsible for 24.4% [21.5-27.8] (p < 0.001) of Kawasaki diseases, Norovirus for 6.7% [1.3-11.2] (p = 0.002), and RSV 4.6% [1.2-7.8] (p = 0.022). Sensitivity analyses found similar results. Interpretation: This cohort study of data from a comprehensive national hospitalisation database indicated that approximately 35% of Kawasaki diseases was potentially attributable to seasonal infections. Funding: None.

Cervical vestibular evoked myogenic potentials in healthy children: Normative values for bone and air conduction.

Objectives: To characterize cervical vestibular evoked myogenic potentials (c-VEMPs) in bone conduction (BC) and air conduction (AC) in healthy children, to compare the responses to adults and to provide normative values according to age and sex. Design: Observational study in a large cohort of healthy children (n = 118) and adults (n = 41). The c-VEMPs were normalized with the individual EMG traces, the amplitude ratios were modeled with the Royston-Wright method. Results: In children, the amplitude ratios of AC and BC c-VEMP were correlated (r = 0.6, p < 0.001) and their medians were not significantly different (p = 0.05). The amplitude ratio was higher in men than in women for AC (p = 0.04) and BC (p = 0.03). Children had significantly higher amplitude ratios than adults for AC (p = 0.01) and BC (p < 0.001). Normative values for children are shown. Amplitude ratio is age-dependent for AC more than for BC. Confidence limits of interaural amplitude ratio asymmetries were less than 32%. Thresholds were not different between AC and BC (88 ± 5 and 86 ± 6 dB nHL, p = 0.99). Mean latencies for AC and BC were for P-wave 13.0 and 13.2 msec and for N-wave 19.3 and 19.4 msec. Conclusion: The present study provides age- and sex-specific normative data for c-VEMP for children (6 months to 15 years of age) for AC and BC stimulation. Up to the age of 15 years, c-VEMP responses can be obtained equally well with both stimulation modes. Thus, BC represents a valid alternative for vestibular otolith testing, especially in case of air conduction disorders.

Viral Identification Using Multiplex Polymerase Chain Reaction Testing Does Not Reduce Antibiotic Prescribing in Paediatric Intensive Care Units.

PCR tests for viral identification, performed on nasopharyngeal secretions, have experienced a major boom in the last few years. Their use is very frequent, but their indications are still not well defined, especially in Paediatric Intensive Care Units (PICU). These tests are used for the microbiological diagnosis of lower respiratory infections but can be used in other situations. The aim of the study was to investigate the effect of viral identification on antibiotic therapy management. We conducted a single-centre retrospective study from 1 October 2017 to 31 December 2019. This study included all consecutive FilmArray® Respiratory Panel tests performed in patients hospitalised in a PICU. Patients were identified using the microbiology laboratory prospective database and data were extracted from the medical record. 544 tests corresponding to 408 patients were included. The main reasons for testing were pneumonia (34%) and bronchiolitis (24%). In 70% of cases, at least one virus was identified, with Human Rhinovirus (56%) and Respiratory Syncytial Virus (28%) being the two predominant. Bacterial co-infection was present in 25% of cases. Viral identification was not associated with reduced antibiotic therapy. On multivariate analysis, antibiotic management was significantly associated with clinical gravity, CRP value or radiology findings regardless of virus identification. Viral identification has an epidemiological value, but antibiotic prescription relies on other factors.

Association between early nutrition support and 28-day mortality in critically ill patients: the FRANS prospective nutrition cohort study.

BACKGROUND: Current guidelines suggest the introduction of early nutrition support within the first 48 h of admission to the intensive care unit (ICU) for patients who cannot eat. In that context, we aimed to describe nutrition practices in the ICU and study the association between the introduction of early nutrition support (< 48 h) in the ICU and patient mortality at day 28 (D28) using data from a multicentre prospective cohort. METHODS: The 'French-Speaking ICU Nutritional Survey' (FRANS) study was conducted in 26 ICUs in France and Belgium over 3 months in 2015. Adult patients with a predicted ICU length of stay > 3 days were consecutively included and followed for 10 days. Their mortality was assessed at D28. We investigated the association between early nutrition (< 48 h) and mortality at D28 using univariate and multivariate propensity-score-weighted logistic regression analyses. RESULTS: During the study period, 1206 patients were included. Early nutrition support was administered to 718 patients (59.5%), with 504 patients receiving enteral nutrition and 214 parenteral nutrition. Early nutrition was more frequently prescribed in the presence of multiple organ failure and less frequently in overweight and obese patients. Early nutrition was significantly associated with D28 mortality in the univariate analysis (crude odds ratio (OR) 1.69, 95% confidence interval (CI) 1.23-2.34) and propensity-weighted multivariate analysis (adjusted OR (aOR) 1.05, 95% CI 1.00-1.10). In subgroup analyses, this association was stronger in patients ≤ 65 years and with SOFA scores ≤ 8. Compared with no early nutrition, a significant association was found of D28 mortality with early enteral (aOR 1.06, 95% CI 1.01-1.11) but not early parenteral nutrition (aOR 1.04, 95% CI 0.98-1.11). CONCLUSIONS: In this prospective cohort study, early nutrition support in the ICU was significantly associated with increased mortality at D28, particularly in younger patients with less severe disease. Compared to no early nutrition, only early enteral nutrition appeared to be associated with increased mortality. Such findings are in contrast with current guidelines on the provision of early nutrition support in the ICU and may challenge our current practices, particularly concerning patients at low nutrition risk. Trial registration ClinicalTrials.gov Identifier: NCT02599948. Retrospectively registered on November 5th 2015.

Spatiotemporal variation of childhood hyperthyroidism: a 10-year nationwide study.

Objective: Childhood hyperthyroidism is mostly caused by Graves' disease, a rare autoimmune disease in children. Epidemiological data are scarce and the variability of within-region incidence is unknown. We aimed to provide the first description of temporal trends in pediatric hyperthyroidism in France and to explore spatial trends, with a view to identifying possible environmental triggers. Design and methods: We performed an observational population-based study on data collected from the National Health Data System, covering the 2008-2017 period and the whole of France. We identified patients with an indicator reflecting incident cases of treated hyperthyroidism, in children aged 6 months-17.9 years, localized at the scale of the département (equivalent to a county) of residence. We performed descriptive analyses of incidence rate by sex, age, and year, and used a spatiotemporal model for estimation at département level. Results: We identified 4734 incident cases: 3787 girls (80%) and 947 boys (20%). The crude incidence rate was 3.35 (95% CI: 3.26; 3.45) per 100 000 person-years over the study period. We estimated the increase in incidence between 2008 and 2017 at 30.1% (19.0%; 42.3%). Annual incidence rate increased linearly over the 10-year period in both girls and boys, rising similarly in all age groups and in all départements. The spatial model highlighted marked heterogeneity in the risk of childhood hyperthyroidism across France. Conclusion: The trend toward increasing incidence observed may reflect changes in genetic and environmental interactions, and the marked spatial heterogeneity may reflect localized ethnic or environmental factors worthy of further investigation.

Incidence of Acute Chest Syndrome in Children With Sickle Cell Disease Following Implementation of the 13-Valent Pneumococcal Conjugate Vaccine in France.

Importance: Acute chest syndrome (ACS) is one of the leading acute severe complications of sickle-cell disease (SCD). Although Streptococcus pneumoniae (S pneumoniae) is highly prevalent in children with SCD, its precise role in ACS is unclear. The efficacy of 13-valent pneumococcal conjugate vaccine (PCV13) implementation on ACS is still unknown. Objective: To assess the association of PCV13 implementation in the general pediatric population with the incidence of ACS in children with SCD. Design, Setting, and Participants: This cohort study used an interrupted time-series analysis of patient records from a national hospital-based French surveillance system. All children younger than 18 years with SCD (based on the International Statistical Classification of Diseases and Related Health Problems, Tenth Revision definition) hospitalized in France between January 2007 and December 2019 were included. Exposures: PCV13 implementation. Main Outcomes and Measures: Monthly incidence of ACS per 1000 children with SCD over time as analyzed by segmented linear regression with autoregressive error; monthly incidence of hospitalization for vaso-occlusive crisis, asthma crisis, and acute pyelonephritis per 1000 children with SCD over the same period as the control outcomes. Results: Among the 107 694 hospitalizations of children with SCD, 4007 episodes of ACS were included (median [IQR] age, 8 [4-12] years; 2228 [55.6%] boys). PCV13 implementation in 2010 was followed by a significant decrease in the incidence of ACS (-0.9% per month; 95% CI, -1.4% to -0.4%; P < .001), with an estimated cumulative change of -41.8% (95% CI, -70.8% to -12.7%) by 2019. Sensitivity analyses yielded the same results, including the incidence of ACS adjusted for that of vaso-occlusive crisis over time. The results were similar among different age groups. By contrast, no change was found for the 3 control outcomes over the study period. Conclusions and Relevance: PCV13 implementation was associated with an important reduction in the incidence of ACS in children with SCD. This vaccine benefit provides new evidence of the key role of S pneumoniae in ACS and should be considered when estimating outcomes associated with current PCVs and the potential benefit of next-generation PCVs in children.

Cognitive status of patients judged fit for discharge from the post-anaesthesia care unit after general anaesthesia: a randomized comparison between desflurane and propofol.

BACKGROUND: The Aldrete's score is used to determine when a patient can safely leave the Post-Anaesthesia Care Unit (PACU) and be transferred to the surgical ward. The Aldrete score is based on the evaluation of vital signs and consciousness. Cognitive functions according to the anaesthetic strategy at the time the patient is judged fit for discharge from the PACU (Aldrete's score ≥ 9) have not been previously studied. The aim of this trial was to assess the cognitive status of inpatients emerging either from desflurane or propofol anaesthesia, at the time of PACU discharge (Aldrete score ≥ 9). METHODS: Sixty adult patients scheduled for hip or knee arthroplasty under general anaesthesia were randomly allocated to receive either desflurane or propofol anaesthesia. Patients were evaluated the day before surgery using Digit Symbol Substitution Test (DSST), Stroop Color Test and Verbal Learning Test. After surgery, the Aldrete score was checked every 5 min until reaching a score ≥ 9. At this time, the same battery of cognitive tests was applied. Each test was evaluated separately. Cognitive status was reported using a combined Z score pooling together the results of all 3 cognitive tests. RESULTS: Among the 3 tests, only DSST was significantly reduced at Aldrete Score ≥ 9 in the Desflurane group. Combined Z-scores at Aldrete Score ≥ 9 were (in medians [interquartils]): - 0.2 [- 1.2;+ 0.6] and - 0.4 [- 1.1;+ 0.4] for desflurane and propofol groups respectively (P = 0.62). Cognitive dysfunction at Aldrete score ≥ 9 was observed in 3 patients in the Propofol group and in 2 patients in the Desflurane group) (P = 0.93). CONCLUSION: No difference was observed in cognitive status at Aldrete score ≥ 9 between desflurane and propofol anaesthesia. Although approximately 10% of patients still had cognitive dysfunctions, an Aldrete score ≥ 9 was associated with satisfactory cognitive function recovery in the majority of the patients after lower limb arthroplasty surgery under general anaesthesia. TRIAL REGISTRATION: Clinical Trials identifier NTC02036736 .

Abnormal bone mineral density and content in girls with early-onset anorexia nervosa.

BACKGROUND: Early-onset anorexia nervosa (EO-AN) represents a significant clinical burden to paediatric and mental health services. The impact of EO-AN on bone mineral abnormalities has not been thoroughly investigated due to inadequate control for pubertal status. In this study, we investigated bone mineral abnormalities in girls with EO-AN regardless of pubertal development stage. METHOD: We conducted a cross-sectional study of 67 girls with EO-AN (median age = 12.4 [10.9-13.7 years]) after a median duration of disease of 1.3 [0.6-2.0] years, and 67 healthy age-, sex-, pubertal status- matched control subjects. We compared relevant bone mineral parameters between groups: the total body bone mineral density [TB-BMD], the lumbar spine BMD [LS-BMD], the total body bone mineral content [TB-BMC] and the ratio of the TB-BMC to lean body mass [TB-BMC/LBM]. RESULTS: TB-BMD, TB-BMC, LS-BMD and TB-BMC/LBM were all significantly lower in patients with AN compared to controls. In the EO-AN group, older age, later pubertal stages and higher lean body mass were associated with higher TB-BMC, TB-BMD, and LS-BMD values. DISCUSSION: Girls with EO-AN displayed deficits in bone mineral content and density after adjustment for pubertal maturation. Age, higher pubertal stage and lean body mass were identified as determinants of bone maturation in the clinical population of patients with EO-AN. Bone health should be promoted in patients, specifically in those with an onset of disorder before 14 years old and with a delayed puberty.

Peripheral obstruction without airflow limitation is rare and not specific to asthma in children.

Peripheral (or small) airway obstruction (PAO) is considered a marker of childhood asthma but the techniques able to directly measure it are rarely used in routine. Usual spirometry and plethysmography can detect a certain degree of PAO when reduced forced vital capacity (FVC) is associated to normal forced expiratory volume in 1 s (FEV1 ) to FVC ratio, and normal total lung capacity (TLC). The frequency of this functional pattern has never been studied in different pediatric respiratory conditions. To assess the prevalence and outcome of PAO in children with different diseases or symptoms, we retrospectively extracted from our database all files of Caucasian subjects encompassing spirometry and plethysmography measurements. Spirometry patterns (normal, airflow limitation [AFL; low FEV1 /FVC], low FVC [with normal FEV1 /FVC]) and final functional patterns (normal, AFL, PAO, restrictive [low TLC], or mixed) were described. We included 4394 files recorded in 1794 children (median [IQR] age: 10.7 [9.2-12.9] years). At inclusion, 125 (7%) children had low FVC of which 56 (44.8%, and 3.1% [95% CI 2.3-3.9] of the whole population) had PAO. PAO prevalence increased with age (OR (95% CI) per 2-year-increase: 2.26 (1.59-3.23); p < .001), and was more frequent in chronic bronchial diseases other than asthma (1.6% vs. 8.0%). On repeated tests, PAO frequently normalized (26.1%) or persisted (43.5%), but it less often progressed into AFL (13%) or restrictive (13%) patterns. PAO is an infrequent nonspecific and persistent functional pattern. Its prevalence increases with age and in diagnosis of chronic respiratory diseases other than asthma.

International consensus validation of the POPI tool (Pediatrics: Omission of Prescriptions and Inappropriate prescriptions) to identify inappropriate prescribing in pediatrics.

INTRODUCTION: While drug prescription should be based on established recommendations stemming from clinical trials but in pediatrics, many drugs are used without marketing authorization. Consequently recommendations are often based on clinical experience and the risk of inappropriate prescription (IP) is high. A tool for detecting IP in pediatrics-called POPI (Pediatrics: Omission of Prescriptions and Inappropriate prescriptions)-has been developed in France. However the relevance of its use at an international level is not known. Our aim has been to adapt POPI for a worldwide use. MATERIAL AND METHOD: A two-round Delphi online questionnaire was completed and validated by international experts to identify consensual items. They were asked to rate the validity of each items taking into account the recommendations and practices in their countries. Only propositions obtaining a median score in the upper tertile with an agreement of more than 75% of the panel-for the first round-and 85%-for the second round-were retained. RESULTS: Our panel included 11 pharmacists (55%) and 9 physicians (45%). The panelists came from 12 different countries: England, Belgium, Brazil, Canada, China, Ivory Coast, Ireland, Malaysia, Portugal, Switzerland, Turkey and Vietnam. At the end of the first round, of the 105 items of the original POPI tool, 80 items were retained including 16 items reworded and 25 items were deleted. In the second round, 14 experts participated in the study. This final international POPI tool is composed of 73 IP and omissions of prescriptions in the fields of neuropsychiatry, dermatology, infectiology, pneumology, gastroenterology, pain and fever. DISCUSSION AND CONCLUSION: This study highlights international consensus on prescription practice in pediatrics. The use of this tool in everyday practice could reduce the risk of inappropriate prescription. The impact of the diffusion of POPI tool will be assessed in a prospective multicentric study.

Children with uncontrolled asthma and significant reversibility might show hypoxaemia.

Asthmatic children free of exacerbation with airway obstruction may have low partial pressure of oxygen (PaO2) which can be a marker for future risk, but PaO2 is scarcely measured during pulmonary function testing. We prospectively included asthmatic children with airway obstruction referred for pulmonary function testing, including blood gas analysis (n = 51). Hypoxaemia, defined as a value lower than - 2 z-score, was present in 15 (29%) children, and 37 (72%) children had a significant reversibility after bronchodilator administration. The multivariable model showed a positive influence of baseline forced expiratory volume in 1 s (FEV1) on PaO2 (ß coefficient 0.69, [95% CI: 0.07; 1.30]; P = 0.03), whereas uncontrolled asthma and FEV1 reversibility negatively influenced it (ß coefficient - 1.59 [95% CI: - 2.74; - 0.44]; P = 0.01; and - 0.07 [95% CI: - 0.13; - 0.02]; P = 0.01, respectively). As a consequence, children with uncontrolled symptoms of asthma and FEV1 reversibility ≥ 12% were significantly more at risk of having hypoxaemia compared to children with well/partly controlled asthma or no significant reversibility of FEV1.Conclusion: Among obstructive asthmatic children without current exacerbation, hypoxaemia is more likely to be seen in children with uncontrolled asthma and a significant post-bronchodilator FEV1 reversibility, in favour of different pathophysiology and treatment requirements of their airway obstruction.What is Known:• Recommendations are to treat asthmatic children in order to control respiratory symptom and maintain normal pulmonary function.• Asthmatic children free of exacerbation may have different pathophysiology for airway obstruction (central, peripheral, inflammatory, spasticity, remodelling) and should be treated according the pathophysiology of their airway disease.What is New:• In obstructive asthmatic children free of current exacerbation, the presence of hypoxaemia (ventilation-perfusion mismatch) is influenced by asthma control and post-bronchodilator reversibility, independently of the level of baseline airway obstruction.• The presence of hypoxaemia in obstructive asthmatic children free of current exacerbation can be highly suspected by the composite index "uncontrolled asthma + FEV1reversibility ≥ 12%" which may guide treatment.

Dizziness and Convergence Insufficiency in Children: Screening and Management.

OBJECTIVE: In children screened for dizziness with vergence disorders, we tested short and long term efficacy of orthoptic vergence training (OVT) and instructions to reduce screen usage. METHODS: Prospective study: Of the 179 children referred for vertigo or dizziness (over 3 years) with ophthalmological disorder as the only problem after complete oto-neuro-vestibular testing, 69 presented vergence insufficiency, and 49 accepted to participate in this study. 109 healthy children served as controls. All subjects had classic orthoptic evaluation and video binocular movement recordings during various oculomotor tasks. Patients were evaluated before OVT (M0), 3 months after the end of OVT (M3) and 9 months after the end of OVT (M9). Statistics compared orthoptic and oculomotor parameters between patients and controls over time with one-way ANCOVA, and mixed models, controlling for age and gender. RESULTS: Patients reported vertigo that was usually rotatory, lasting <15 min, associated with or alternating with headache (50%). Their exposure to small video screens and TV was intensive (∼3.6 h per day). At M0, all orthoptic and oculomotor parameters were statistically different in patients relative to controls (p < 0.0001) except for divergence. At M3, vertigo symptoms had disappeared in all of the patients, and all eye movement parameters improved significantly (p < 0.0001). At M9, this improvement remained stable or continued. CONCLUSION: Vergence disorders (assessed by abnormal orthoptic and oculomotor parameters) can generate symptoms of dizziness in children. Orthoptic treatment and instruction to reduce screen usage has a significant and long term effect on vertigo symptoms as well as oculomotor performances. Dizzy children should be screened for vergence disorders. WHAT THIS STUDY ADDS: Dizziness in children can be associated exclusively with insufficient convergence. Orthoptic training and instructions to reduce screen exposure made dizziness symptoms disappear and improved all eye movement parameters for 6 months. Vergence disorders should be screened for in dizzy children.

Oral health related quality of life of children and adolescents affected by rare orofacial diseases: a questionnaire-based cohort study.

BACKGROUND: Rare diseases affecting the teeth, the oral cavity and the face are numerous, each of them present specific characteristics, and is a life-long condition. The aim of the study was to assess the association between Oral health-related quality of life (OHRQoL), and demographic characteristics, clinical and dental factors, and psycho-social characteristics to investigate that oral symptoms are not the main factors underlying a decrease in OHRQoL. MATERIAL AND METHODS: We conducted a national cohort study in French centres for rare diseases (RD) specialized in orofacial diseases. The inclusion criteria were: to have received care in RD centres over the last 5 years (2012-2017) and to have been between 6 and 17 years of age on September 1, 2017. Patients were invited to answer a questionnaire composed of socio-demographic, clinical and dental questions, psychosocial questions and then fill in the Child-OIDP Index. At the end of the questionnaire, a free space was left for the patient to add a verbatim comment to provide qualitative data. Thematic analysis was used to analyze the verbatim answers. RESULTS: Complete data were available for 110 patients. The sample included 44.5% boys and 55.5% girls. Ages ranged from 6 to 17 years old and 68.2% were between 6 to 12 years old and 31.8% were between 13 and 17 years old. Factor associated with a lower OHRQoL were: being a girl (p = 0.03), renouncement to dental care for financial reasons (p = 0.01), having syndromic disease (p = 0.01), having a problem with tooth shape and color (p = 0.03), feeling isolated, alone and different from other children (p = 0.003 and p = 0.02). Qualitative analysis highlighted very little recourse to psychological care and patients reported great anxiety and fear about the future. CONCLUSION: OHRQoL of children suffering from these diseases is impaired, especially from the psychosocial point of view but also from that of the course of treatment and access to care. There is a need to improve the legibility of care pathways and the financial coverage of treatments.

Melatonin Levels in Preterm and Term Infants and Their Mothers.

The prevention of perinatal brain damage following preterm birth remains a public health priority. Melatonin has been shown to be a promising neuroprotectant in neonatal preclinical models of brain damage, but few studies have investigated melatonin secretion in newborns. We hypothesized that melatonin circulating levels would be lower in preterm compared to term infants. We conducted a prospective, longitudinal, multicenter study to assess melatonin, and 6-sulfatoxy-melatonin (aMT6s) concentrations, measured by radioimmunoassay. Among 209 neonates recruited, 110 were born before 34 gestational weeks (GW) and 99 born after 34 GW. Plasma melatonin concentrations, measured at birth and on Day 3 were below detectable levels (≤7 pg/mL) in 78% and 81%, respectively, of infants born before 34 GW compared to 57% and 34%, respectively, of infants born after 34 GW. The distribution of plasma melatonin concentrations was found to be correlated with gestational age at both time-points (p < 0.001). Median urine aMT6s concentrations were significantly lower in infants born before 34 GW, both on Day 1 (230 ng/L vs. 533 ng/L, p < 0.0001) and on Day 3 (197 ng/L vs. 359 ng/L, p < 0.0001). In conclusion, melatonin secretion appears very low in preterm infants, providing the rationale for testing supplemental melatonin as a neuroprotectant in clinical trials.

X chromosome gene dosage as a determinant of congenital malformations and of age-related comorbidity risk in patients with Turner syndrome, from childhood to early adulthood.

Objective Turner Syndrome is associated with several phenotypic conditions associated with a higher risk of subsequent comorbidity. We aimed to evaluate the prevalence of congenital malformations and the occurrence of age-related comorbid conditions and to determine whether the frequencies of congenital and acquired conditions depend on X chromosome gene dosage, as a function of karyotype subgroup. Design and methods This national retrospective observational cohort study includes 1501 patients. We evaluated the prevalence of congenital malformations and the cumulative incidence of subsequent specific comorbidities at five-year intervals, from the ages of 10 to 30 years, with stratification by karyotype subgroup: 45,X (n = 549), 45,X/46,isoXq (n = 280), 46,X,r(X)/46,XX (n = 106), 45,X/46,XX (n = 221), presence of Y (n = 87). Results Median age was 9.4 (3.7-13.7) years at first evaluation and 16.8 (11.2-21.4) years at last evaluation. Congenital heart (18.9%) malformations were more frequent in 45,X patients, and congenital renal (17.2%) malformations were more frequent in 45,X, 45,X/46,isoXq and 46,X,r(X)/46,XX patients than in those with 45,X/46,XX mosaicism or a Y chromosome (P < 0.0001). The cumulative incidence of subsequent acquired conditions, such as thyroid disease, hearing loss, overweight/obesity, dyslipidemia and, to a lesser extent, celiac disease, glucose intolerance/type 2 diabetes, hypertension and liver dysfunction increased with age, but less markedly for patients with mosaicism than for those with other karyotypes. Patients with a ring chromosome were more prone to metabolic disorders. Conclusion These data suggest that X gene chromosome dosage, particularly for Xp genes, contributes to the risk of developing comorbidities.

Factors Affecting Loss to Follow-Up in Children and Adolescents with Chronic Endocrine Conditions.

OBJECTIVE: Most children with endocrine diseases require long-term continuity of care. We investigated the prevalence of loss to follow-up (LTFU) in pediatric patients with chronic endocrine diseases and the risk factors associated with LTFU. METHODS: This observational cohort study included all children with chronic endocrine diseases included in the database of a single academic pediatric care center over a period of 8 years. LTFU was defined as a lack of attendance at clinical visits for over 2 years, for unknown reasons. RESULTS: LTFU was recorded for 154 of the 1,067 patients included (14%). Median age at diagnosis was 5.8 (0.3-11.8) vs. 1.2 (0.0-6.9) years, and age at last visit was 14.1 (9.7-16.1) vs. 11.7 (6.1-15.8) years, for the LTFU and no-LTFU groups, respectively. In multivariate analysis, the risk of LTFU increased with age at diagnosis (OR 1.18; 95% CI 1.12-1.24) and was higher for patients diagnosed before 2006 (vs. after 2006; OR 4.80; 95% CI 3.00-7.66), with fewer visits in the last 3 years (OR 0.72; 95% CI 0.65-0.80; p < 0.0001) and a lower health insurance classification (OR 1.79; 95% CI 1.10-2.89; p = 0.02). The risk of LTFU was higher for patients with isolated growth hormone deficiency than for those with other endocrine conditions, such as multiple pituitary deficiencies, hypogonadotropic hypogonadism, Turner syndrome, or thyroid, adrenal, or gonadal disorders (OR 5.24; 95% CI 1.13-24.37; p = 0.03). CONCLUSION: This study provides the first epidemiological data for LTFU in children and adolescents with chronic endocrine diseases. It should facilitate the targeting of interventions to improve adherence to medical care and healthcare organization during the pediatric period.