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Excepting for the increased eosinophils in a subset of the subjects, all had a normal appearance of the colonic mucosa. CONCLUSION Colonic mucosa of children and adolescents with LD has a normal histological appearance in majority of the patients. However, 35 % of the patients could demonstrate elevated eosinophils. In primary LD without any comorbidity there is no increase of mast cells and lymphocytic cells in the colonic mucosa. Adenylosuccinate synthase (ADSSL1) is a muscle specific enzyme involved in the purine nucleotide cycle and responsible for the conversion of inosine monophosphate to adenosine monophosphate. Since 2016, when mutations in the ADSSL1 gene were first described to be associated with an adult onset distal myopathy, nine patients with compound heterozygous variants in the ADSSL1 gene, all of Korean origin, have been identified. Here we report a novel ADSSL1 mutation and describe two sporadic cases of Turkish and Indian origin. Many of the clinical features of both patients and muscle histopathology and muscle MRI findings, were in accordance with previously reported findings in the adult onset distal myopathy individuals. However, one of our patients presented with progressive, proximally pronounced weakness, severe muscle atrophy and early contractures. Thus, mutations in ADSSL1 have to be considered in patients with both distal and proximal muscle weakness and across various ethnicities. V.BACKGROUND According to the INPES 2014 health barometer, the prevalence of smoking in pregnant women in France is the highest in Europe  17.8% of expectant mothers who smoke continue to do so during pregnancy. Several epidemiological studies have confirmed multiple risks for tobacco-exposed infants (low birth weight; digestive, respiratory, neurological, and psychological disorders; obesity; type 1 diabetes). PURPOSE This study compared a cohort of infants exposed to tobacco in utero (T+) with those unexposed (T-). Birth weight, diet, presence of colic (ROME III criteria) and regurgitations (Vandenplas scale) were specifically analyzed. METHODS This observational, cross-sectional, and multicenter survey was conducted in France by pediatricians and general practitioners from September 2016 to February 2017. Infants with a chronic pathology and those with parents under 18 years of age were excluded. The data were collected by the physician and by the mother through a self-administered questionnaire. RESULTS A tve/respiratory symptoms and lower birth weight than unexposed infants. Preventive and educational actions need to be further strengthened in the face of this public health problem. OBJECTIVE The suitability of the injectables may vary across different age groups especially for children; therefore, knowledge on their usage patterns is critical in terms of rational pharmacotherapy. This study aimed to investigate pediatric injectable drug utilization in primary care with a focus on different age groups. METHOD By simple sampling method, 100 prescriptions that contained at least one injectable drug were randomly selected for each month of the year in 32 provinces of Turkey (n=38.400). Among these prescriptions, injectable drugs that were for children ( less then 18 years) were analyzed. Patterns of injectable drug utilization were compared according to the pediatric age group of "infants", "children", and "adolescents". RESULTS We identified 5446 patients (14.2%) with a mean age of 7.4±5.2 years and a slight male tendency in distribution (53.8%). The most common indication for these patients was for the respiratory system (65.4%), of which 96.3% were respiratory tract infections. While les study indicates an inappropriate use of injectable drugs by primary care physicians for managing medical conditions in the pediatric population. AIM The literature includes few reports on the prehospital care of pediatric casualties of urban house fires. Here we aimed to describe the epidemiology of pediatric fire victims, focusing on their injuries, prehospital care, and survival. METHODS This retrospective study included children under 15 years of age who were victims of urban house fires and who received care from prehospital medical teams. The variables analyzed included epidemiology, specific care provided by prehospital emergency services, the number of cardiac arrests, and survival rates. RESULTS Over the 15-month study period, 365 house fires required the presence of at least one prehospital medical team. Casualties of these fires included 121 pediatric victims (median age, 4 years [interquartile range 2-9 years]). All children were initially treated by a prehospital medical team that was not specialized in pediatrics. Six children (4.9%) received secondary treatment from a pediatric support team. Of the 121 children, 114 (94.2%) suffered from smoke inhalation and seven (5.8%) from burns. Two patients who were in cardiac arrest at their initial medical care did not survive. CONCLUSION Pediatric fire casualties were initially managed by prehospital medical teams that were not specialized in pediatrics. As in adults, the main injuries were secondary to smoke inhalation, but this has increased toxicity in children. Prehospital teams not specialized in pediatrics can optimize their practice via the sharing of experiences, team training, and cognitive aid checklist for pediatric fire victims. selleck inhibitor INTRODUCTION Vancomycin is an old antibiotic whose use is still being debated today. The objective of this work was to establish an inventory of the use of vancomycin in the various pediatric and neonatal hospital services in the New Aquitaine region. MATERIALS AND METHODS A declaratory practice survey was conducted in 49 pediatric and neonatal hospital units. These practices were compared with the guidelines of several learned societies. RESULTS A total of 36 responses could be analyzed 12 units (33%) used vancomycin in discontinuous administration, 18 (50%) had opted for continuous infusion, and six used it in both modalities (17%). The reported dosages were highly variable. Blood tests were performed by 26 units (72%), but the target values of the trough serum concentration were also highly variable. After dosing, all units reported adjusting the dosage and re-dosing after modification (26/26). Finally, 21 units (58%) reported taking into account the MIC of the possibly isolated bacterium. CONCLUSION Our study shows that vancomycin is used in very different ways from one unit to another, within the same region, including in ways not recommended by the main learned societies.