We report on a 10-month-old boy with hypotonic dehydration and metabolic alkalosis. Sweat test was borderline and genetic analysis was negative for common mutations. Analysis of the whole coding regions of the CFTR gene revealed the rare mutation D579G in homozygosity.

Background In Italy, the State Regions Conference on 1st August 2019 approved the Guidelines for Short-Stay Observation (SSO). At the beginning of 2022, the main Scientific Societies of the pediatric hospital emergency-urgency area launched a national survey to identify the extent to which these national guidelines had been adopted in the emergency rooms and pediatric wards of the Italian Regions. Methods A survey has been widespread, among Pediatric Wards and Pediatric Emergency Departments (EDs), using both a paper questionnaire and a link to a database on Google Drive, for those who preferred to fill it directly online. Those who did not spontaneously answer, where directly contacted, via email and/or through a phone call and invited to participate. The data collected have been: age of managed children, presence of triage, presence of Sub-intensive Care Unit and Intensive Care Unit and special questions about Pediatric SSO, availability of training courses for workers, number of ED access in the last 4 years. Results This survey is still ongoing, without a definite deadline, so we presented the preliminary data. Currently, 8/20 Regions have not yet adopted the Guidelines. Till 02 January 2023, data from 253 hospitals were collected. There are currently 180/253 active Pediatric SSO (71.03% of the Hospitals). There are not active SSO in 33.27% of first level ED, in 19.35% of second level ED and in 33.66% of General Hospitals with Pediatric Wards. Active SSO are located mainly (75.97%) within Pediatric Wards. At the moment, the survey has been completed in 16 Regions: in the 8 Regions which are using guidelines, pediatric SSOs are active in all the second level ED (compared to 60.87% of the other 8 regions), in the 91.66% of first level ED (compared to the 33.3%), and in the 97.1% of General Hospitals (compared to 33.3%), with a statistically significance (p < 0.0001). The territorial analysis of these 16 regions highlighted geographical differences in the percentage of SSOs active: 35.22% are active in hospitals in Southern Italy, 88.64% in Central Italy and 91.67% in those of the North. Conclusions The delay in adopting specific guidelines negatively influences activation of pediatric SSOs in hospital system and prevents the adjustment of welfare level to new needs. To facilitate the activation of SSOs in hospitals, it is also necessary to guarantee adequate economic recognition. It is essential to implement public interventions to overcome the current inequalities in the interest of children and their families: the current delay seriously penalizes emergency pediatric hospital care, especially in the southern Italian Regions.

Bezoar is an accumulation of organic substances in the gastrointestinal tract. This condition is extremely rare, their incidence in the pediatric population is unknown. Bezoars usually occur in females, adolescents, and children with psychiatric or neurological disorders. Trichobezoar refers to the accumulation of hair usually in the stomach and even beyond it (Rapunzel syndrome). We present the clinical case of a schoolage female patient who started with neurological symptoms (headache, syncope) and then developed gastrointestinal symptoms. Only later was clarified a history of bullying at school and consequent anxious behaviors (trichotillomania and trichophagia). It's very important a thorough medical history, especially in patients with Trichobezoar, to highlight psychological disorders that can cause trichotillomania and trichophagia.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe multiorgan hypersensitivity reaction mostly caused by several eliciting drugs in patients with a genetic predisposition. Incidence of DRESS in children is very variable, frome 1:1000 to 1:10.000, and the mortality rate seems to be lower than 10%. Anti-convulsants are the main drugs involved both in adults and in children. The treatment of choice is the prompt withdrawn of the offending drug and using intravenous immunoglobulins and corticosteroids used in synergy. In recent years, emerging studies have outlined the disease more clearly. We present a pediatric case in which the patient developed DRESS syndrome as a result of exposure to lamotrigine before and carbamazepine after and a relapse after exposure to omeprazole. Starting from this case report we provide an overview on DRESS Syndrome.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe multiorgan hypersensitivity reaction mostly caused by several eliciting drugs in patients with a genetic predisposition. Anti-convulsants are the main drugs involved both in adults and in children. Incidence of DRESS in children is very variable, from 1:1,000 to 1:10,000, and the mortality rate seems to be lower than 10%. The treatment of choice is the prompt withdrawn of the offending drug and using intravenous immunoglobulins and corticosteroids used in synergy. In recent years, emerging studies have outlined the disease more clearly. We present a paediatric case in which the patient developed DRESS syndrome as a result of exposure to lamotrigine before and carbamazepine after and a relapse after exposure to omeprazole. Starting from this case report we provide an overview on DRESS syndrome.