Health-related quality of life among children with Turner syndrome: controlled cross-sectional study

International audienceBACKGROUND : The aim of the study was to assess health-related quality of life (HR-QoL) in children with Turner syndrome in comparison with controls.METHODS : We prospectively recruited 16 female girls with Turner syndrome (mean age 15.2±2.6 years) and 78 female controls (mean age 12.7±2.8 years) in randomly selected schools. We used the PedsQL, a generic HR-QoL questionnaire (self and parents' versions).RESULTS : Global HR-QoL scores in Turner syndrome were lower than controls for self-reports (respectively, 74.3±3.0 vs. 82.8±1.3, p=0.01) and parents' reports (62.7±3.8 vs. 80.1±1.7, p<0.0001). In Turner syndrome, self-reported HR-QoL was impaired in school functioning (70.6±4.0 vs. 80.71±1.7, p=0.02), social functioning (78.2±4.0 vs. 90.4±1.8, p<0.01) and physical functioning (78.5±3.2 vs. 87.1±1.4, p=0.02), but not in emotional functioning. Parents' reported HR-QoL was impaired in all four dimensions.CONCLUSIONS : HR-QoL was impaired in this cohort of young females with Turner syndrome, as in previously reported adult studies. In addition to medical treatment and routine clinical follow-up, female girls and teenagers with Turner syndrome should also be supported psychologically by social, educational and psychotherapeutic interventions that aim to address their self-esteem and emotional difficulties

Quality of life in children participating in a non-selective INR self-monitoring VKA-education programme

International audienceThe quality of life (QoL) of children receiving vitamin K antagonist (VKA) treatment has been scarcely studied

Cardiopulmonary fitness in children with congenital heart diseases versus healthy children.

International audienceOBJECTIVE : We aimed to compare the cardiopulmonary fitness of children with congenital heart diseases (CHD) with that of age-adjusted and gender-adjusted controls. We also intended to identify clinical characteristics associated with maximum oxygen uptake (VO2max) in this population.METHODS ANS RESULTS : We included in a cross-sectional multicentre study a total of 798 children (496 CHD and 302 controls) who underwent a complete cardiopulmonary exercise test (CPET). The association of clinical characteristics with VO2max was studied using a multivariate analysis. Mean VO2max in the CHD group and control represented 93%±20% and 107%±17% of predicted values, respectively. VO2max was significantly lower in the CHD group, overall (37.8±0.3vs 42.6±0.4 mL/kg/min, P<0.0001) and for each group (P<0.05). The mean VO2max decline per year was significantly higher in CHD than in the controls overall (−0.84±0.10 vs −0.19±0.14 mL/kg/min/year, P<0.01), for boys (−0.72±0.14vs 0.11±0.19 mL/kg/min/year, P<0.01) and for girls (−1.00±0.13 vs −0.55±0.21 mL/kg/min/year, P=0.05). VO2max was associated with body mass index, ventilatory anaerobic threshold, female gender, restrictive ventilatory disorder, right ventricle systolic hypertension, tricuspid regurgitation, the number of cardiac catheter or surgery procedures, and the presence of a genetic anomalyCONCLUSIONS : Although the magnitude of the difference was not large, VO2max among children with CHD was significantly lower than in normal children. We suggest performing CPET in routine follow-up of these patients

Active Bleeding after Cardiac Surgery: A Prospective Observational Multicenter Study.

MAIN OBJECTIVES:To estimate the incidence of active bleeding after cardiac surgery (AB) based on a definition directly related on blood flow from chest drainage; to describe the AB characteristics and its management; to identify factors of postoperative complications. METHODS:AB was defined as a blood loss > 1.5 ml/kg/h for 6 consecutive hours within the first 24 hours or in case of reoperation for hemostasis during the first 12 postoperative hours. The definition was applied in a prospective longitudinal observational study involving 29 French centers; all adult patients undergoing cardiac surgery with cardiopulmonary bypass were included over a 3-month period. Perioperative data (including blood product administration) were collected. To study possible variation in clinical practice among centers, patients were classified into two groups according to the AB incidence of the center compared to the overall incidence: "Low incidence" if incidence is lower and "High incidence" if incidence is equal or greater than overall incidence. Logistic regression analysis was used to identify risk factors of postoperative complications. RESULTS:Among 4,904 patients, 129 experienced AB (2.6%), among them 52 reoperation. Postoperative bleeding loss was 1,000 [820;1,375] ml and 1,680 [1,280;2,300] ml at 6 and 24 hours respectively. Incidence of AB varied between centers (0 to 16%) but was independent of in-centre cardiac surgical experience. Comparisons between groups according to AB incidence showed differences in postoperative management. Body surface area, preoperative creatinine, emergency surgery, postoperative acidosis and red blood cell transfusion were risk factors of postoperative complication. CONCLUSIONS:A blood loss > 1.5 ml/kg/h for 6 consecutive hours within the first 24 hours or early reoperation for hemostasis seems a relevant definition of AB. This definition, independent of transfusion, adjusted to body weight, may assess real time bleeding occurring early after surgery

NUTRI-REAPED study:nutritional assessment of French critically ill children and nutrition practice survey in French-speaking pediatric intensive care units

Abstract Background Impaired nutritional status is adversely associated with suboptimal outcomes in critically ill children. Undernutrition at pediatric intensive care unit (PICU) admission ranges from 15 to 65%. A lack of knowledge of the nutritional status of children in French PICUs prevents us from specifically targeting education. This study aims to describe the nutritional status of children in French PICUs and to assess nutritional practices and physicians’ knowledge of nutrition, in order to focus NutriSIP (the French-speaking PICU nutrition group) future education programs. A prospective observational multicenter point prevalence study was conducted in French PICUs, recruiting all children admitted over three different weeks. Anthropometric measurements were taken (weight, height/length, mid-upper arm, and head circumferences), in order to calculate nutritional indices. Nutritional status was defined according to WHO Body Mass Index z-score and dynamic assessment based on growth faltering detection. Concurrently, PICU physicians and PICU nurses from seven French-speaking countries completed a survey to ascertain knowledge about local nutritional care practices and overall nutrition knowledge. PICU physicians’ responses were compared to PICU nurses’ responses (previously published). Results Four hundred and thirty-two children were included in the observational study from 27 French PICUs. Undernutrition was diagnosed in 18.5% of them, young age and underlying chronic condition being the two independent risk factors. Faltering growth was diagnosed in 4.8% and overweight in 7.4%. Subjective nutritional assessment was not accurate. Thirty-eight French-speaking PICUs completed the survey. These showed nutritional practices frequently did not comply with international guidelines, especially regarding nutritional goals, and the reasons for withholding enteral nutrition. Comparison between physicians’ and nurses’ responses to the survey showed large discrepancies. Conclusion Undernutrition is frequent at admission in French PICUs. Nutritional status should be assessed using a holistic approach, because of the potential impact on outcome. French-speaking PICU healthcare professionals need further nutrition education, in order to improve nutritional practices to comply with international recommendations. This study will serve as a baseline to focus NutriSIP teaching programs in the future

The French Version of the Revised Ritvo Autism and Asperger Diagnostic Scale: A Psychometric Validation and Diagnostic Accuracy Study

International audienceThe early recognition of ASD in adults is challenging, in particular due to the lack of appropriate and robust diagnostic tools. We performed a psychometric validation and diagnostic accuracy study of the French version of the RAADS-R on a sample of 305 adults: 105 with ASD without ID, 99 with psychiatric disorders, and 103 non-psychiatric control groups. The French version of the RAADS-R demonstrates good reliability and diagnostic validity, suggesting that it can help clinicians during the diagnostic process in adults with ASD without ID. However, the finding that a two-factor structure better fits the results requires further validation. This study point out the need of further study of RAADS in psychiatric disorders group due to the relatively high false positive rate (55.6%) of ASD

Effects of the two types of anorexia nervosa (binge eating/purging and restrictive) on bone metabolism in female patients

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Postoperative management during the first 24 hours.

<p>Values are presented as N(%) or Median [Q25;Q75]. Significant difference between “Low incidence” and “High incidence” are denoted by *p<0.10, **p<0.05 and ***p<0.01, tested by Student /Mann-Whitney tests or Chi²/Fisher tests.</p

Preoperative characteristics (<i>N = 129)</i>.

<p>Values are presented as N(%) or Median [Q25;Q75]. Significant difference between “Low incidence” and “High incidence” are denoted by *p<0.10, **p<0.05 and ***p<0.01, tested by Student /Mann-Whitney tests or Chi²/Fisher tests.</p

Risks factors of occurrence of at least one postoperative complication.

<p>Univariate and multivariate analyses.</p