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Background: The treatment of aggressive behavior and agitation in pediatric patients with autism spectrum disorder (ASD) in the emergency department is topical and challenging., Case Presentation: We described an adolescent with autism spectrum disorder treated ten times in the pediatric emergency department for severe episodes of aggressiveness and agitation. After resolving the acute phase of these behavioural crises, sedation was maintained with a continuous infusion of dexmedetomidine to prevent the resurgence of agitation and to organize discharge properly, considering the family's needs. The continuous infusion of dexmedetomidine allowed the patient to remain asleep most of the time during his stay at the emergency department. No adverse events were recorded., Conclusions: The continuous infusion of dexmedetomidine could represent a safe and valuable tool to facilitate the permanence of the patient in the PED.

Hyponatremia is the most common electrolyte disorder in the pediatric population. Symptoms are related to the time in which hyponatremia has developed. The acute presentation could be dramatic, with neurological symptoms like headache, seizure, impaired mental status and even coma. It is essential for the physician to be aware of the possible causes of hyponatremia in the child in order to start a prompt treatment.

Competing Interests: Competing interests: None declared.

Competing Interests: Competing interests: None declared.

Background: Patients with cognitive impairment due to cerebral palsy experience pain more often than healthy peers and frequently require diagnostic and therapeutic painful procedures. Analgesia and procedural sedation outside the operating room are often required, but they may not adequately be provided because of the inability to accurately recognize and classify the state of pain and for the perceived higher risk of complications., Data Sources: We reviewed the available literature to highlight the specific risk factors and area of criticism, that should be further improved. We searched the Cochrane Library, Medline, Pubmed from 1987 to September 2018 using key words such as 'cerebral palsy and children and pain' or 'sedation and cerebral palsy and children'., Results: While different pain scales are useful in recognizing pain expressions, anxiety scales are not available. Moreover, studies on non-pharmacological techniques do not always have comparable results. Several risk factors, from anatomic abnormalities to liver and kidney functioning, should be kept in mind before proceeding with sedation., Conclusions: Large trials are needed to assess the impact of non-pharmacological techniques and to evaluate which pain control strategy (pharmacological and non-pharmacological) should be used in different settings.

Competing Interests: Competing interests: None declared.

This study evaluates the association between the long noncoding RNA GAS5 levels and the anti-proliferative effect of the glucocorticoid (GC) methylprednisolone (MP) alone and in combination with rapamycin in peripheral blood mononuclear cells (PBMCs) obtained from healthy donors. The effect of MP, rapamycin, and MP plus rapamycin was determined in 17 healthy donors by labelling metabolically active cells with [methyl-3H] thymidine and the expression levels of GAS5 gene were evaluated by real-time RT-PCR TaqMan analysis. We confirmed a role for GAS5 in modulating GC response: poor responders presented higher levels of GAS5 in comparison with good responders. Interestingly, when PBMCs were treated with the combination of rapamycin plus MP, the high levels of GAS5 observed for each drug in the MP poor responders group decreased in comparison with rapamycin (P value = 0.0134) or MP alone (P value = 0.0193). GAS5 is involved in GC resistance and co-treatment of rapamycin with GCs restores GC effectiveness in poor responders through the downregulation of the long noncoding RNA. GAS5 could be considered a biomarker to personalize therapy and a novel therapeutic target useful for the development of new pharmacological approaches to restore GC sensitivity., (© 2016 John Wiley & Sons Australia, Ltd.)

Unlabelled: The aim of the study is to investigate if the presence of medical clowns during painful procedures in the emergency department (ED) affects children's anxiety and pain. Forty children (4-11 years) admitted to the ED with the need of painful procedures were prospectively enrolled. They were randomly assigned to the clown group, where children interacted with clowns or to the control group in which they were entertained by parents and ED nurses. The children's anxiety was assessed by the Children's Anxiety and Pain Scales; pain was evaluated with the Numerical Rating Scale and Wong-Backer Scale, according to the children's age. Staff and clown's opinions were evaluated by means of dedicated questionnaires. Children's anxiety levels in the clown group were significantly lower than those compared with the control group, while children's pain levels did not change between the two groups., Conclusion: The presence of clowns in the ED before and during painful procedures was effective in reducing children's anxiety., What Is Known: • Anxiety and fear caused by medical procedures exacerbate children's pain and may interfere with the procedure. • To reduce anxiety, fear, and pain and to facilitate patient's evaluation, different non-pharmacological approaches have been proposed and positive effects of laughter and humor have been reported. What is New: • The presence of clowns in the waiting room and in the ED during medical evaluation and painful procedures helps to reduce children's anxiety.

We present the case of two twin brothers with surfactant protein C deficiency who were treated with hydroxychloroquine for three years, with apparent success. The exact physiopathology of this disease is not known and there is no specific treatment for it. There is merely news from a few previous descriptions in the literature about the use of hydroxychloroquine for surfactant protein C deficiency with satisfactory results. Two years after the treatment was withdrawn, the twins were evaluated once again: they presented no new infections, growth and general state were normal and chest CT showed a notable additional reduction in the interstitial pneumopathy. These data seem to cast some doubt on the efficacy of hydroxychloroquine, and they suggest that the clinical improvement was simply the natural evolution of the disease., (Copyright © 2012 SEPAR. Published by Elsevier Espana. All rights reserved.)

Introduction: Behçet's disease is a chronic multisystem vasculitis characterized by mucocutaneous, articular, neurological, gastrointestinal and ophthalmological lesions. Ocular involvement is mainly represented by recurrent uveitis, especially posterior uveitis; however, iridocyclitis, retinal and choroidal vasculitis, optic neuritis and retinal vascular occlusion can also occur., Case Presentation: A 12-year-old Caucasian boy with a history of recurrent buccal aphthosis and nonspecific gastrointestinal symptoms was admitted to our hospital with blurred vision associated with acute episcleritis and papillitis. The patient's pathergy test was positive, suggesting a diagnosis of Behçet's disease. Corticosteroid and cyclosporine therapy was started, but further episodes were noted in both eyes. The patient was then switched to intravenous infliximab, with complete resolution of the inflammation after the second infusion., Conclusion: Episcleritis and papillitis should be added to the list of uncommon manifestations of pediatric Behçet's disease. Infliximab is an effective, new therapeutic approach for Behçet's disease that is refractory to the conventional corticosteroid and immunosuppressive therapy.