Ital J Pediatr
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Patients suffering from mucopolysaccharidosis are among the most complex from the anesthesiological point of view, especially regarding the management of the airway. The evidence base for anesthesia management is often limited to case reports and small case series. ⋯ Patients with mucopolysaccharidosis are at high risk for anesthesia-related complications and require a high level of attention. However, a multidisciplinary approach, combined with expertise in the use of new techniques and new devices for airway management, makes anesthesiological management safer. Further research with prospective studies would be useful.
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In childhood B-precursor acute lymphoblastic leukemia (B-ALL), the ETV6/RUNX1 fusion transcript is considered to have an excellent outcome. However, few studies of children with ETV6/RUNX1-positive ALL from China have been conducted. It is largely unknown whether clinical outcomes for patients with this genotype and important factors that influence such outcomes are similar to those reported in other countries. Therefore, it is important to analyze the outcomes of children with ETV6/RUNX1-positive ALL treated at our institution with the aim of identifying significant prognostic variables in a Chinese population. ⋯ In conclusion, patients with ETV6/RUNX1 fusion transcript can achieve a high rate of complete remission and the long-term curative effect was excellent under risk-stratified treatment. In case of relapse, the MRD level at the end of induction therapy should be taken into consideration while deciding the appropriate chemotherapy dosage.
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Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a serious life-treating condition characterized by skin eruption, fever, haematologic abnormalities, and multi-organ involvement that can be fatal if unrecognized, especially in patients with liver failure. Diagnosis may be difficult because it is rarely described in children and can mimic many different conditions. ⋯ Our cases highlight that paediatricians should be aware of the clinical presentations of and therapeutic approaches for DRESS syndrome, especially in children receiving long-term antibiotic treatment. The removal of the offending drug is crucial and may be the only life-saving measure. In more aggressive cases, corticosteroid or other immunosuppressive drugs should be considered to achieve the best outcome.
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Point-of-care ultrasonography (POCUS) allows to obtain real-time images to correlate with the patient's presenting signs and symptoms. It can be used by various specialties and may be broadly divided into diagnostic and procedural applications. ⋯ Bedside emergency ultrasound is routinely used by adult emergency physicians and in the last 10 years its application is recognized and applied in PED. Pediatric emergency physicians are encouraged to familiarize with POCUS as it is a safe technology and can be extremely helpful in performing diagnosis, managing critical situations and guiding procedures, which results in globally improving pediatric patients care.
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The Italian Society of Pediatric Surgery (SICP) together with The Italian Society of Pediatric Anesthesia (SARNePI) through a systematic analysis of the scientific literature, followed by a consensus conference held in Perugia on 2015, have produced some evidence based guidelines on the feasibility of day surgery in relation to different pediatric surgical procedures. The main aspects of the pre-operative assessment, appropriacy of operations and discharge are reported.