Meyer Children Research Articles

Behçet syndrome in children and adults: discovering similarities and differences by a comparative study.

Behçet's syndrome (BS) is a rare disorder with a relapsing-remitting course. Clinical variance across geographical regions and different age groups has been observed. This study matched the demographic, clinical and treatment features of adult- and juvenile-onset BS in the Italian population. Two clinical databases of BS patients were compared. The paediatric BS database was collected at the Meyer Children's Hospital, Florence, while the adult BS database was collected at the Careggi University Hospital, Florence. A familiar predisposition for BS was significantly more frequent in the paediatric cohort (3/33 vs 1/165, P = 0.015). No difference emerged in terms of prevalence of HLA-B51 positivity. The proportion of patients meeting the revised ICBD and/or the ISG criteria at BS diagnosis was comparable in the two cohorts. No significant difference emerged between the two cohorts in terms of muco-cutaneous, ocular and neurological involvement, and gastrointestinal symptoms. Articular manifestations resulted as more common in the paediatric cohort, whereas venous vascular events were more frequent in the adult cohort. Regarding treatment strategy, paediatric patients more frequently received no treatment or corticosteroid monotherapy. Conversely, the use of DMARDs, both traditional and biologic, was significantly higher in the adult cohort. Remarkable differences between juvenile-onset and adult-onset BS, both in terms of gender, familiar predisposition and clinical manifestations have been observed and a different therapeutic approach in the real clinical practice of the two settings emerged. Prospective, comparison studies with a longer follow-up are encouraged to provide further data about the disease course for juvenile- and adult-onset BS.

QOL-25. Neurocognitive assessment of pediatric medulloblastoma treated by MBMET_MEYER trial. Censullo M.L1, Bertoluzzo G2, Fonte C1, Pavone R1, Guidi M1, Enrico G1, Gori C.G1, Martin R2, Teodori C2, Sardi I1 1Neuro-Oncology Unit, Department of Pediatric Oncology, Meyer Children’s University Hospital, Florence, Italy2Psychology Unit, Meyer Children’s University Hospital, Florence, Italy

Abstract The MBMET_MEYER is an interventistic monocentric trial of Meyer Children’s Hospital. The goal of this protocol is to reduce toxicity by use of protontherapy and reducing chemo-induced neurotoxicity by limited use of high dose chemotherapy. To date, a neurocognitive assessment is the standard in medulloblastomas but it is very difficult an assessment at baseline for clinical problems such as age, intracranial hypertension and immediate intervention. 10 neurocognitive assessments were reviewed in patients with medulloblastoma treated by MBMET_MEYER protocol: 3 anaplastic medulloblastoma M0 and 7 classic (2 M0, 1 M1, 1 M2, 3 M3). The median age was 10 years (range 5-18). All the patients were treated with surgery (gross total removal), 2 received chemotherapy and conventional radiotherapy, 8 chemotherapy and protontherapy and only 1 received autologos-hemotopoietic stem cell transplant for progression disease after induction chemotherapy. As for protocol, the neurocognitive assessment was defined by Weschler Intelligence Scales, fonemic and semantic fluency, immediate and deferred memory, tests for working memory, attention and visuo-spatial tests at baseline, after treatment and at the end of follow-up. For 3 patients it was not possible a baseline assessment for bad clinic conditions, 8 are still in treatment. From interviews and assessments emerged that 80% of patients had neurocognitive deficits: at baseline 5 had speed elaboration difficulties, 1 of these also presented verbal deficit, 1 showed problems in recalling verbal material and 1 in working memory; after two years from chemotherapy and radiotherapy 2 presented speed processig and working memory deficit. In conclusion, an assessment at baseline is very difficult for post-surgical problems but necessary to perform as soon. In this way, it is possible to evaluate the impact of the treatment on neurocognitive impairment. Further investigations are necessary to well understand the appropriate schedule of neurocognitive assessment of pediatric medulloblastoma.

Amateur dubbing as a healthcare activity in the pediatric hospital setting: a pilot project.

Dubbing is a mode of audiovisual translation (AVT) usually performed by experienced dubbing actors for professional purposes. Nowadays, thanks to advances in technology, everybody can create personal dubbed versions of audiovisual content for humorous and parodic as well as therapeutic and pedagogical purposes. The aim of this pilot project was to investigate the potential and applicability of amateur dubbing within the hospital setting, targeting children and adolescents. The project was carried out at Meyer Children's Hospital in Florence (Italy). Like other non-pharmacological activities, amateur dubbing was designed to make hospitalization a less traumatic experience for young patients and promote their general well-being. A research team (including psychologists, dubbing actors and a linguist) developed a theoretically sound and replicable set of procedures that combine new technologies with traditional pain management methods. The amateur dubbing workshops, carried out from January 2017 until the end of December 2019, involved 297 children and adolescents (male=29.8%; female=70.2%), aged 6-17, from different wards (i.e. Oncology, Neuropsychiatry, Pediatric Unit, Diabetology, Neurology, Surgery, etc.). This pilot project has proven to be a positive experience for all the patients and their families, in terms of the children's well-being, quality of life and socialization (i.e. expression of emotions, distraction and reduction of distress). Amateur dubbing as a healthcare activity has resulted in interesting recreational and psychological implications and benefits. Clearly, its implementation as a type of non-pharmacological technique needs to be further refined.

Impact of screen exposure on pediatric vernal Keratoconjunctivitis: a survey during the COVID-19 pandemic in Italy

BackgroundThe SARS-CoV-2 outbreak pushed the Italian government to start a strict lockdown, replacing school attendance with long-distance learning. This caused reduced exposure to sunlight but increased exposure to screens. Vernal keratoconjunctivitis (VKC) is a chronic inflammatory ocular condition in which exposure to light plays a cardinal role. We conducted an online survey to evaluate the impact of screen exposure on children with VKC during the COVID-19 lockdown.MethodsWe performed a survey-based observational study, asking patients followed at the Allergology clinics of Meyer Children’s University Hospital in Florence and of Policlinico Umberto I in Rome to provide grading on 6 subjective ocular clinical manifestations presented during the lockdown and to give an estimate of their hours/day of screen exposure.ResultsMean scores of signs and symptoms increased homogeneously when studying patients exposed to longer screen time. When comparing scores collected in 2019 to those in 2020, there was not a significant reduction in clinical manifestations, although the situation differed between the two centers due to geographical differences in sunlight exposure.ConclusionDuring the lockdown, there was a reduction in sunlight exposure but conversely an increase in the time spent in front of screens that correlated with the worsening of VKC signs and symptoms in direct proportion to the hours/day of screen exposure. Our results also showed a statistically significant difference in the relative impact of long-distance learning on VKC clinical manifestations in the different Italian regions.

Pelvic Pyomyositis in Childhood: Clinical and Radiological Findings in a Tertiary Pediatric Center.

Pyomyositis (PM) is an infrequent but increasing bacterial infection of the skeletal muscle, with muscles of the pelvis and thigh frequently involved. The diagnosis is often challenging, especially when a deep muscle is affected. We present a single-center pediatric cohort affected by pelvic PM. A retrospective analysis was performed, including children admitted to Meyer Children’s Hospital between 2010 and 2020. Demographic, anamnestic, clinical, laboratory, radiological and management data were collected. Forty-seven patients (range 8 days–16.5 years, 66% males) were selected. Pain (64%), functional limitations (40%) and fever (38%) were the most common presenting symptoms; 11% developed sepsis. The median time to reach the diagnosis was 5 days (IQR 3–9). Staphylococcus aureus was the most common organism (30%), Methicillin-Resistant S aureus (MRSA) in 14%. PM was associated with osteomyelitis (17%), arthritis (19%) or both (45%). The infection was multifocal in 87% of children and determined abscesses in 44% (40% multiple). Pelvic MRI scan, including diffusion-weighted imaging (DWI), always showed abnormalities when performed. Clinical and laboratory findings in pelvic PM are unspecific, especially in infancy. Nevertheless, the infection may be severe, and the suspicion should be higher. MRI is the most useful radiological technique, and DWI sequence could reveal insidious infections.

Gastrointestinal involvement in IgA vasculitis: a single-center 11-year study on a cohort of 118 children.

Immunoglobulin A vasculitis (IgAV), the most common childhood vasculitis, is associated with gastrointestinal (GI) involvement in 50-75% of cases. The aim of this study was to describe the characteristics of GI involvement in a cohort of hospitalized children with IgAV. We retrospectively evaluated patients hospitalized for IgAV at Meyer Children's University Hospital, from January 2010 to December 2020. The children's families were interviewed by phone and asked about disease relapses. In the study period, 118 children had GI involvement, corresponding to 75% of children hospitalized for IgAV. Their median age was 7years (interquartile range 6-9). The most frequent GI manifestations were abdominal pain (96%), bleeding (71%, mostly occult), vomiting (58%), and diarrhea (17%). GI complications, observed in 18%, were intussusception (14%), appendicitis (3%), gallbladder hydrops (2%), and ileal perforation (1%). Abdomen ultrasound, performed in all cases, was abnormal in 68%. Abdomen X-ray, performed in 19 patients, showed pathologic findings in 84% of them. Selected children with severe manifestations also underwent abdomen computed tomography (2/118; 2%) and GI endoscopy (5/118; 4%). Steroids were used in 80 (67.8%) cases. The use of second- and third-line therapies was necessary in three cases. Relapses, investigated in 80 cases (68%), occurred in 21% of them. Key Points • The present retrospective study describes a cohort of 118 patients hospitalized for gastrointestinal (GI) involvement in immunoglobulin A vasculitis (IgAV) at a tertiary care children's hospital from January 2010 to December 2020. • The most frequent GI manifestations were abdominal pain (96%), vomiting (58%), and diarrhea (17%). GI bleeding was reported in 71% of children, and it was occult in most cases. • GI complications, occurring in 18% of cases, were intussusception (14%), appendicitis (3%), gallbladder hydrops (2%), and ileal perforation (1%). • Steroid treatment has been successfully used in severe GI manifestations; however, our data do not support its association with low relapse risk. The present study describes a large pediatric cohort of GI involvement in IgAV. Steroid treatment should be used when GI manifestations are severe. The association of steroid use with relapse risk is not currently established.

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Handheld Optical System for Pectus Excavatum Assessment

Disruptive 3D technologies, such as reverse engineering (RE) and additive manufacturing (AM), when applied in the medical field enable the development of new methods for personalized and non-invasive treatments. When referring to the monitoring of pectus excavatum, one of the most common thoracic malformations, 3D acquisition of the patient chest proved to be a straightforward method for assessing and measuring chest deformation. Unfortunately, such systems are usually available in a dedicated facility, can be operated only by specialized doctors with the support of engineers and can be used only with patients on site. It is therefore impossible to perform any routine check-up when the patient is unable to reach the outpatient clinic. The COVID19 pandemic situation has placed even greater restrictions on patient mobility, worsening this problem. To deal with this issue, a new low-cost portable optical scanner for monitoring pectus excavatum is proposed in this work. The scanner, named Thor 2.0, allows a remote diagnostic approach, offering the possibility to perform routine check-ups telematically. Usability tests confirmed the user-friendly nature of the devised system. The instrument was used at the Meyer Children’s Hospital (Florence, Italy) chest-malformations center to treat PE patients. The performed measurements proved to be in line with the current state of the art.

Discrepancies between Vaccine Documentation and Serologic Status for Diphtheria, Tetanus, and Hepatitis B in Internationally Adopted Children.

Internationally Adopted Children (IAC) often show suboptimal immunisation coverage, but available data are discordant. Data at the first evaluation of 2073 IAC (median age: 6 years) referred to the Meyer Children’s University Hospital (Florence, Italy) in 2009–2019 were analysed in order to evaluate their immunisation status against diphtheria, tetanus, and hepatitis B. Negative antibody titres were observed in 11.5% of the IAC for diphtheria, 18.6% for tetanus, and 39.0% for hepatitis B. At multivariate analysis, originating from Africa was an independent risk factor for seronegativity for the three diseases (p < 0.001), while age below four years was an independent factor associated with protective immunity, only considering hepatitis B (p < 0.001). Vaccine documentation was an additional factor independently associated with protective immunity. However, a discrepancy between documentation (indicating previous vaccinations) and serology (showing negative antibody titres) was evidenced in 3.8% of the children for diphtheria, 12.6% for tetanus, and 29.6% for hepatitis B. This finding suggests that although vaccine documentation may reflect the presence of protective antibody titres, it should not be accepted as absolute evidence of protective immunity, underlining the importance of a complete assessment of immunisation status in IAC, particularly in those originating from Africa and aged over four years.

P1813CLINICAL CHARACTERIZATION OF CONGENITAL SOLITARY FUNCTIONING KIDNEY IN CHILDREN

Abstract Background and Aims Congenital anomalies of the kidney and urinary tract (CAKUT) are the major cause of chronic kidney disease and end-stage kidney disease in childhood. Solitary functioning kidney (SFK) is part of the spectrum of CAKUT. Congenital SFK is mainly due to unilateral renal agenesis (URA) and multi-cystic dysplastic kidney (MCDK). With the implementation of routine fetal ultrasound screening, SFK is increasingly recognized before birth, significantly raising the number of patients referred to paediatric nephrology units for clinical monitoring during childhood. Understanding the pathophysiology of SFK is pivotal for guiding the clinical management and informing long-term outcome. However, observational studies performed in children with SFK are controversial, especially about the need, methodology and timing of functional assessment. This may be at least in part due to the fact that different models of SFK, including congenital and acquired after unilateral nephrectomy, are often grouped together. The aim of this study was to assess the clinical, laboratory and functional features of congenital SFK caused by URA and MCDK in children, with a particular focus on the role of renal scintigraphy in estimating kidney function during childhood and adolescence. Method We retrospectively collected clinical, laboratory and instrumental records of all consecutive pediatric patients (aged 0-18 years) affected by congenital SFK caused by URA or MCDK referred to the Nephrology and Dialysis Unit of Meyer Children’s Hospital of Florence (Italy) from 1992 to 2019. Patients with unilateral kidney hypodysplasia were excluded. In particular, we reviewed data from ultrasound scanning and sequential renal scintigraphy over time. URA and MCDK were compared for clinical features, long-term course and outcome. Results A total of 155 patients with congenital SFK were included in the study and divided in two groups according to the cause of SFK (URA, n=100; MCDK, n=55). The median length of follow-up was 47 and 45 months, respectively. Male sex and ethnicity were equally distributed in the two groups. Prenatal diagnosis was more frequent in MCDK group. We did not observe either preterm birth or low birth weight in patients enrolled. Overall, the clinical features were not statistically different between the two groups. In particular, SFK associated CAKUT, including vesicoureteral reflux, occurred at a comparable frequency. Also, measurement of kidney length by ultrasound scanning, which is often considered suggestive of compensatory hypertrophy, did not differ between groups. Although renal clearance from sequential renal scintigraphy appeared not statistically different between URA and MCDK, the latter seems to reach complete functional adaptation more rapidly and earlier in the first two years of life. Conclusion The clinical course and long-term outcome of SFK has been a topic of extensive debate. Due to poor-quality of data (unclear inclusion/exclusion criteria, lack of uniformity in data collection and outcome definition), generalization of findings from observational studies to all patients with SFK could be inappropriate. Congenital SFK could represent the most unbiased group to analyze and this study provides a thorough clinical characterization of a large and strictly selected cohort. Insights from sequential renal scintigraphy suggest a different trend in reaching single kidney complete functional adaptation in URA and MCDK. These results could potentially reveal significant differences in the pathophysiologic mechanisms of reaching compensatory hypertrophy and functional adaptation by the solitary kidney in the two models. Whether confirmed in larger cohorts, these findings could provide important implications for follow-up planning, informing the need, methodology and timing for function assessment, tailoring the clinical management and understanding long-term prognosis.

Invasive Haemophilus influenzae Type b Disease in the Post Hexavalent Era: Ten Years of Molecular Surveillance in Tuscany.

The epidemiologic characteristics of invasive Haemophilus influenzae type b disease (HIBD) have markedly changed since the introduction of the Haemophilus influenzae type b (Hib) conjugate vaccine worldwide. The immunization schedule against Haemophilus influenzae type b differs in Europe. This is a retrospective observational study which evaluates all the data included in the molecular surveillance register for invasive infectious diseases at the Laboratory of Molecular Diagnosis at Meyer Children's University Hospital from December 2008 to December 2018 with a diagnosis of invasive HIBD in children <5 years of age. We identified 4 cases of HIBD: all the cases presented signs or symptoms of invasive infection and the H. influenzae type b was identified in cerebrospinal fluid, or blood or bronchoalveolar lavage by molecular test. The crude incidence for Hib invasive disease in Tuscany is 0.26/100,000 p-y in children younger than 5 years, significantly different from the incidence rate before the introduction of the Hib vaccination. Vaccination effectiveness can be estimated at 97.9% and the impact of hexavalent (2p+1) vaccine at 99.6%. This work confirms the high impact of the hexavalent vaccine 2p+1 schedule for HIBD in children <5 years, emphasizing the role of molecular test for HIBD diagnosis and surveillance.

Heterotopic pancreas in the gastrointestinal tract in children: a single-center experience and a review of the literature

BackgroundHeterotopic pancreas, that is the abnormal localization of a well-differentiated pancreatic tissue, is a rare occurrence in pediatric patients. Most lesions are found incidentally; in some circumstances, the presence of heterotopic pancreas may cause gastrointestinal symptoms, such as obstructive symptoms or bleeding.Patients and methodsThe clinical notes of patients with histological diagnosis of heterotopic pancreas treated at Meyer Children’s Hospital between 2009 and 2017 have been retrospectively examined.Four variables have been examined: clinical presentation, age at diagnosis, timing of surgery and localization of the heterotopic pancreas. Patients have been classified accordingly.ResultsFourteen patients were diagnosed with heterotopic pancreas at a single institution. In half cases, heterotopic pancreas caused symptoms that warranted surgical exploration. Symptomatic patients were significantly older than patients in whom heterotopic pancreas was an incidental finding (mean age 9 years and 5 months vs 2 years and 9 months; p = 0.02). Heterotopic pancreas was more frequently found in patients who underwent urgent surgical procedure than in patients who underwent elective surgery (2.61% vs 0.22%; p < 0.0001). In all cases, foci of heterotopic pancreas were resected.ConclusionHeterotopic pancreas is usually discovered in the submucosa of the stomach, duodenum and small bowel. Heterotopic tissue may cause symptoms related to mechanical complications, bleeding from the surrounding intestinal mucosa or, occasionally, to the development of malignancy.Heterotopic tissue is a rare but clinically relevant cause of gastrointestinal symptoms. The presence of heterotopic tissue should be considered in children with gastrointestinal symptoms of unclear origin and surgical resection is advisable.

Long-term efficacy of add-on stiripentol treatment in children, adolescents, and young adults with refractory epilepsies: A single center prospective observational study.

To assess the long-term efficacy and tolerability of stiripentol (STP) as an adjunctive treatment in different forms of refractory epilepsies. The medical records of all individuals consecutively treated with STP as add-on therapy for refractory epilepsies, irrespective of their being focal, generalized, or both, and followed at Meyer Children's Hospital between January 2007 and May 2018, were reviewed. The drug scheme administration consisted of a starting dose of STP of 10-15mg/kg/d with increments every week, up to a maximum of 50mg/kg/d, based on both age and weight. Etiology of epilepsy was codified as structural, genetic, infectious, immune, metabolic, and unknown. Responders were defined as patients who achieved a seizure frequency reduction of ≥50%. Retention rate was defined as the probability of continuing STP without additional therapy. Tolerability was assessed by reporting adverse events. A total of 132 individuals aged from 5months to 43years received add-on STP, including 30 patients with Dravet syndrome (DS). The median follow-up was 14.8months (range = 4months-18years, interquartile range = 25.72). Twenty-nine individuals (22%) received more than two antiepileptic drugs. Benzodiazepines, mainly clobazam, were the most commonly used add-on drugs. Sixty-six patients (50%) were responders, and 13 of them (9.8%) were seizure-free. Responder rate was higher in the genetic etiology group (57%), especially in DS (18/30; 60%), and in patients with refractory focal onset epilepsy without bilateral tonic-clonic seizures (5/15; 33%). The median relapse-free survival was 27months in the 66 responders. The median time to STP failure was 24.6months in all 132 individuals. This study confirms the long-term efficacy of add-on STP treatment in patients with different types of refractory epilepsies, including focal onset epilepsy without bilateral tonic-clonic seizures. Further confirmations based on prospectively designed studies are required to confirm STP efficacy in focal epilepsy.

Automatic detection and sonification of nonmotor generalized onset epileptic seizures: Preliminary results

Long-term video-EEG monitoring has improved diagnosis and treatment of epilepsy, especially in children. However, the amount of data neurophysiologists must analyze has grown remarkably.The main purpose of this paper is to provide a diagnostic support to speed up and ease EEG interpretation for a specific application concerning absence seizures, a type of non-motor generalized epileptic seizures.The proposed method consists of a pre-processing step where signals are filtered through the Stationary Wavelet Transform for the reduction of possible artefacts. Subsequently, a supervised automatic classification method is implemented for seizure detection, based on the Support Vector Machine Fine Gaussian method. Finally, a post-processing step is implemented in which spatial and temporal thresholds are defined for both online and offline application.In addition, a method that applies sonification techniques is developed. Sonification techniques could speed up the process of interpreting information, allowing rapid clinical intervention and a continuous monitoring of the event.The dataset consists of 30 EEG recordings performed in 24 children with absence seizures, clinically evaluated at the Meyer Children's Hospital in Firenze, Italy.The method shows encouraging results both in terms of balanced accuracy (about 96%) and latency times (1.25 s on average), which might make it suitable for online clinical trials. In fact, it was implemented in the perspective of a possible real-time application in clinical practice.

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Pathological ASXL1 Mutations and Protein Variants Impair Neural Crest Development.

SummaryThe neural crest (NC) gives rise to a multitude of fetal tissues, and its misregulation is implicated in congenital malformations. Here, we investigated molecular mechanisms pertaining to NC-related symptoms in Bohring-Opitz syndrome (BOS), a developmental disorder linked to mutations in the Polycomb group factor Additional sex combs-like 1 (ASXL1). Genetically edited human pluripotent stem cell lines that were differentiated to NC progenitors and then xenotransplanted into chicken embryos demonstrated an impairment of NC delamination and emigration. Molecular analysis showed that ASXL1 mutations correlated with reduced activation of the transcription factor ZIC1 and the NC gene regulatory network. These findings were supported by differentiation experiments using BOS patient-derived induced pluripotent stem cell lines. Expression of truncated ASXL1 isoforms (amino acids 1–900) recapitulated the NC phenotypes in vitro and in ovo, raising the possibility that truncated ASXL1 variants contribute to BOS pathology. Collectively, we expand the understanding of truncated ASXL1 in BOS and in the human NC.

Autonomic modulation and epileptic seizures: Biofeedback therapy for epilepsy

Long-term video-EEG monitoring has improved diagnosis and treatment of epilepsy, especially in children. However, the amount of data neurophysiologists must analyze has grown remarkably.The main purpose of this paper is to provide a diagnostic support to speed up and ease EEG interpretation for a specific application concerning absence seizures, a type of non-motor generalized epileptic seizures.The proposed method consists of a pre-processing step where signals are filtered through the Stationary Wavelet Transform for the reduction of possible artefacts. Subsequently, a supervised automatic classification method is implemented for seizure detection, based on the Support Vector Machine Fine Gaussian method. Finally, a post-processing step is implemented in which spatial and temporal thresholds are defined for both online and offline application.In addition, a method that applies sonification techniques is developed. Sonification techniques could speed up the process of interpreting information, allowing rapid clinical intervention and a continuous monitoring of the event.The dataset consists of 30 EEG recordings performed in 24 children with absence seizures, clinically evaluated at the Meyer Children's Hospital in Firenze, Italy.The method shows encouraging results both in terms of balanced accuracy (about 96%) and latency times (1.25 s on average), which might make it suitable for online clinical trials. In fact, it was implemented in the perspective of a possible real-time application in clinical practice.

“SIMBurns”: A high-fidelity simulation program in emergency burn management developed through international collaboration

Acute management of a severely burned patient is an infrequent and stressful situation that requires medical knowledge as well as immediate coordinated action. Many adverse events in health care result from issues related to the application of ‘non-technical' skills such as communication, teamwork, leadership and decision making rather than lack of medical knowledge. Training in these skills is known as Crisis Resource Management (CRM) training.In order to create well-prepared burn teams, it is critical to teach CRM principles through high-fidelity simulation (HFS).While CRM teaches foundational non-technical skills, HFS incorporates lifelike, whole-body, fully-responsive mannequins in order to provide a realistic emergency situation.The aim of the study is to describe the development of a novel high-fidelity simulation course called “SIMBurns: High Fidelity Simulation Program for Emergency Burn Management” that uses CRM as its foundation and is focused on management of burn injuries. The course was designed by a panel of simulation and burns experts from Meyer Children’s Hospital in Italy and Birmingham Children’s Hospital in the U.K. Simulation Program experts were certified by Boston Children’s Hospital’s Simulation Program. In this paper, we describe the course’s design, development, structure, and participant’s assessment of the course. Since the creation of the SIMBurns course in 2013, 9 courses have been conducted and 101 participants have attended the course. The course was well-received and its “Overall Satisfaction” was rated at 4.8/5. The primary objective in the SIMBurns course – to teach teamwork and CRM skills to medical staff involved in emergency burn care – was also met at 4.8/5. Participants felt that the course developed their ability to interact with other team members, further improved their understanding of how to appropriately use resources, emphasized the importance of role clarity and developed their communication skills. Additional quantitative and qualitative analyses obtained from participants were also reviewed after each course. The SIMBurns course aims to contribute to the education of those in healthcare in order to improve patient safety and to continue advancing the education of our emergency burn care teams.

Endoscopic endonasal skull base surgery in pediatric patients. A single center experience

ObjectAt present, a minimally invasive endoscopic endonasal approach is considered an efficient option for lesions affecting the anterior and middle skull base with sellar and parasellar region involvement. In this study we will retrospectively analyze the pediatric patients that we have been treating in the same medical center for the past four years, using an endoscopic approach in the skull base. MethodsWe performed a retrospective chart and imaging review of pediatric patients who underwent endoscopic endonasal skull base surgery (ESBS) at the Meyer Children's Hospital, (Azienda Ospedaliero Universitaria Meyer), in Florence, from January 2012 to July 2016. ResultsMean age was 12,5 years; 28 (65%) of the 44 patients were females. Skull base lesions were broadly classified as either bony abnormalities (4 cases) or skull base tumors (40 cases). The postoperative clinical follow-up duration ranged from 2 to 36 months. The two more frequently occurring diseases in our study were: craniopharyngioma and pituitary adenoma. ConclusionsAfter reviewing the international literature about pediatric endoscopic endonasal approach to skull base, we can affirm that our study is the world's second broadest work for number of records. While compiling this report, we have examined the first 40 consecutive pediatric patients to undergo ESBS at our institute. The appearance of postoperative complications is consistent with other international studies, confirming ESBS being feasible and safe even in the pediatric population. Level of evidence4.

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