Severe hyponatremia in children: a review of the literature through instructive cases.

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.

Sedation and analgesia in children with cerebral palsy: a narrative review.

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.

Differential expression of GAS5 in rapamycin-induced reversion of glucocorticoid resistance.

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.

A quasi randomized-controlled trial to evaluate the effectiveness of clowntherapy on children's anxiety and pain levels in emergency department.

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.

Diagnostic accuracy of ultrasonography for hand bony fractures in paediatric patients.

OBJECTIVE: Hand fractures are common in childhood, and radiography is the standard diagnostic procedure. US has been used to evaluate bone injuries, mainly in adults for long-bone trauma; there are only a few studies about hand fractures in children. The purpose of this study was to evaluate and confirm the safety and applicability of the US diagnostic procedure in comparison to X-ray diagnosis. STUDY DESIGN: This cross-sectional study involved a convenience sample of young patients (between 2 and 17 years old) who were taken to the emergency department due to hand trauma. After clinical assessment, patients with a suspected hand fracture first underwent X-ray, and subsequently US examination by two different operators; a radiologist experienced in US and a trained emergency physician in "double-blind" fashion. US and radiographic findings were then compared, and sensitivity as well as specificity was calculated. RESULTS: A total of 204 patients were enrolled in the study. Seventy-nine fractures of phalanges or metacarpals were detected by standard radiography. When US imaging was performed by an expert radiologist, 72 fractures were detected with sensitivity and a specificity of 91.1% and 97.6%, respectively. Sensitivity and specificity were found to be (respectively) 91.5% and 96.8% when US was performed by the ED physicians. CONCLUSIONS: US imaging showed excellent sensitivity and specificity results in the diagnosis of hand fractures in children. The study also showed a great agreement between the results of the US carried out by the senior radiologist and those carried out by the paediatric emergency physician, suggesting that US can be performed by an ED physician, allowing a rapid and accurate evaluation in ED and could become the first diagnostic approach whenever a hand fracture is suspected.

¿Es eficaz el tratamiento con hidroxicloroquina en el déficit de proteína C surfactante? / Is Treatment With Hydroxychloroquine Effective in Surfactant Protein C Deficiency?

Presentamos el caso de 2 hermanos gemelos con déficit de proteína C surfactante que fueron tratados mediante el empleo de hidroxicloroquina durante 3 años, con aparente éxito. La fisiopatología exacta de esta enfermedad no se conoce, y no disponemos de ningún tratamiento específico para ella; tan solo tenemos noticia de unas pocas descripciones previas en la literatura sobre el uso de hidroxicloroquina para el déficit de proteína C surfactante con resultados satisfactorios. Dos años después de la retirada del tratamiento se volvió a evaluar a los gemelos: no presentaron nuevas infecciones, el crecimiento y el estado general fueron normales, y la TC de tórax mostró una notable reducción adicional de la neumopatía intersticial. Estos datos parecen poner en duda la eficacia de la hidroxicloroquina, y sugieren que la mejoría clínica fue simplemente la evolución natural de la enfermedad (AU)

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 (AU)