Lung transplant list withdrawal in a liver transplant patient thanks to elexacaftor-tezacaftor-ivacaftor: a case report.

Background: Elexacaftor-tezacaftor-ivacaftor (ETI) is a transmembrane conductance regulator modulator that significantly improves lung function in patients affected by cystic fibrosis (CF). This triple drug is currently not indicated in liver transplant patients, as clinical trials including subjects with previous solid organ transplantation are lacking.

Case Presentation: We report on a liver transplant girl with CF-related advanced pulmonary disease meeting clinical criteria for lung transplant, who started the triple modulator because she could not get on the lung transplant waiting list due to psycho-social motivations.

T2-Low Asthma: A Discussed but Still Orphan Disease.

Asthma affects 10% of the worldwide population; about 5% of cases are severe with the need for target therapies such as biologics. All the biologics approved for asthma hit the T2 pathway of inflammation. T2-high asthma is classified as allergic and non-allergic, whereas T2-low asthma can be further defined as paucigranulocytic asthma, Type 1 and Type-17 inflammation and the neutrophilic form that accounts for 20-30% of all patients with asthma.

Oral ibuprofen versus oral ketorolac for children with moderate and severe acute traumatic pain: a randomized comparative study.

This study is to compare ibuprofen and ketorolac for children with trauma-related acute pain. We conducted a multicentre randomized, double-blind, controlled trial in the Paediatric Emergency Department setting. We enrolled patients aged 8 to 17 who accessed the emergency department for pain related to a limb trauma that occurred in the previous 48 h.

Increased use of high-flow nasal cannulas after the pandemic in bronchiolitis: a more severe disease or a changed physician's attitude?

Unlabelled: After the SARS-CoV-2 pandemic, we noticed a marked increase in high-flow nasal cannula use for bronchiolitis. This study aims to report the percentage of children treated with high-flow nasal cannula (HFNC) in various seasons. The secondary outcomes were admissions for bronchiolitis, virological results, hospital burden, and NICU/PICU need.

Hypoventilation and sleep hypercapnia in a case of congenital variant-like Rett syndrome.

Background: Breathing disturbances are often a primary clinical concern especially during wakefulness of the classic form of Rett syndrome, but data for atypical forms are lacking.

Case Presentation: We report the case of a 20-month-old female affected by Rett syndrome with congenital variant-like onset, characterized by severe hypotonia and neurodevelopment impairment. She presented hypoventilation, persistent periodic breathing, and sustained desaturation during sleep, without obstructive or mixed events.

Biological Treatments and Target Therapies for Pediatric Respiratory Medicine: Not Only Asthma.

We present a description of pediatric pneumology biological medications and other target therapies. The article aims at introducing the importance of a molecular approach to improve treatments. The first item treated was T2-High asthma and its current biological treatment and prescribing indications to propose a flow-chart to guide the clinical choice.

Does a standard triage tool adequately detect the needs of children and adolescents admitted for mental health problem?

Background: the visits to the paediatric emergency department for mental problems are increasing exponentially, but the emergency department team in not ready enough to manage them, due to the lack of adequate training. This study aimed to evaluate how the Italian Society of Paediatric Emergency Medicine and Urgency triage system was able to estimate urgency in patients accessing the paediatric emergency department for a mental health problem.

Methods: We conducted a retrospective study at the emergency department of the Institute for Maternal and Child Health, IRCCS Burlo garofolo of Trieste (Italy), from December 2015 to April 2017.

Pulmonary vasculitis in Hughes-Stovin syndrome (HSS): a reference atlas and computed tomography pulmonary angiography guide-a report by the HSS International Study Group.

Introduction: Hughes-Stovin syndrome (HSS) is a systemic vasculitis characterized by widespread venous/arterial thrombosis and pulmonary artery aneurysms (PAAs), which is associated with serious morbidity and mortality. All fatalities reported in HSS resulted from unpredictable fatal suffocating hemoptysis. Therefore, it is necessary to recognize pulmonary complications at an early stage of the disease.

Central apnea and periodic breathing in children with underlying conditions.

Central sleep apneas and periodic breathing are poorly described in childhood. The aim of the study was to describe the prevalence and characteristics of central sleep apnea and periodic breathing in children with associated medical conditions, and the therapeutic management. We retrospectively reviewed all poly(somno)graphies with a central apnea index ≥ 5 events per hr in children aged > 1 month performed in a paediatric sleep laboratory over a 6-year period.

Topical Clobetasol: An Overlooked Cause of Cushing Syndrome.

Background: We describe the case of an 11-year-old boy affected by chronic granulomatous disease complicated by a Crohn's like colitis needing prolonged treatment with oral corticosteroids.

Case Presentation: His therapy for the control of severe oral mucositis was based on topical clobetasol, which did not decrease once the steroids were discontinued. Two years after the oral interruption of the steroids, cushingoid characteristics persisted, the cause of which, after a thorough investigation, was found to be the persistence of the topical clobetasol oral gel.

Mental health problems in children admitted with physical symptoms.

The aim of the study was to investigate the burden of mental health problems in children and adolescents admitted to a pediatric ward with physical complaints. We conducted a retrospective study, considering all patients admitted to the pediatric ward of the IRCCS Burlo Garofolo, Trieste, Italy, between January 2015 and September 2016. We selected all patients, from 5 to 17 years old, who were admitted with physical complaints and were discharged with a diagnosis suggestive of a mental health problem: somatic symptom disorder, anxiety disorder, depressive disorder, factious disorders.

How Age Matters in the Assessment of Vertigo in the Pediatric Emergency Department: A 10-Year Age-Stratified Etiology Survey.

Vertigo is a relatively frequent cause for referral to the pediatric emergency department, and it is usually caused by benign or self-limiting etiology. However, it could be difficult to evaluate especially in the younger child and could also conceal serious illness as encephalitis or cerebellitis. Our survey collected in a 10-year period 757 children assessed in pediatric emergency department for vertigo and stratified this population for etiology and for group of age: younger than 6 years (113, 14.

Is the use of alternative therapy in children just another form of medicalisation? A prospective study.

Aim: The use of complementary and alternative medicines (CAM) seems widespread in adults and children, despite the conflicting evidence regarding its effectiveness and safety. This study aims to investigate the prevalence of the use of CAM in a population of Italian children.

Methods: This is a prospective observational study conducted in 4 family paediatric clinics, in Friuli Venezia Giulia, Italy, from February to June 2019.

Deep sedation for laser treatment of cutaneous vascular anomalies in children.

Pulse laser treatment is useful for aesthetic improvement in children with capillary vascular malformation and residual superficial haemangiomas. However, pulse laser treatment is painful, and repeated procedures are usually required to achieve a satisfactory clinical result. Evidence shows that analgesia in children undergoing pulse laser treatment may vary from general anaesthesia to no treatment at all.

Adolescents with chronic disease and social media: a cross-sectional study.

Objective: This study aims to explore the attitude of adolescents with chronic diseases toward social media exposure, focusing in particular on Facebook.

Design: Cross-sectional study.

Setting: An anonymous semistructured survey was distributed to an Italian hospital-based cohort of adolescents with chronic disease to explore the role of Facebook in their daily life.