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Vasculitis is defined by a state of inflammation of the blood vessel walls. It can result in formation of either aneurysms or stenosis with ischemia and necrosis. The heterogeneity of clinical presentation depends on the type and size of the vessels involved, which leads to different patterns of organ damage. The skin is usually involved but not necessarily as a first manifestation of disease. Indeed, significant organ damage may occur at any time in the disease course and may precede other symptoms with significant morbidity and mortality, especially in case of diagnostic delay. The most common vasculitides in childhood are IgA-associated vasculitis (Schönlein-Henoch purpura) and Kawasaki disease. Other vasculitides are much rarer, often arising with insidious symp- toms that make them life-threatening. This paper aims to review the literature on paediatric vasculitides providing a practical guide to disentangle between different diagnoses. It also provides an overview that underlines the key differences in vasculitis presentation, management and follow-up.

Aim To explore the cardio-pulmonary function of children returning to play sports after mild or asymptomatic SARS-CoV-2 infection. Methods This is a consecutive case series conducted at the Institute for Maternal and Child Health, Trieste, Italy. Paediatric patients who accessed the Institute for cardiologic and pneumological evaluation before the return-to-play competitive sports were recruited, according to the Italian Sports Medical Federation recommendations. Echocardiogram, electrocardiogram, treadmill ECG test and pulmonary function tests were performed. Results One hundred and thirty-two patients (aged 8-17 years old, mean age 12.8 ± 2.5) were recruited. Among these, 127 children were considered for the final analysis (49.6% females). Out of 127, 84 (66.1%) had a mild symptomatic form of SARS-CoV-2 infection, while 43 (33.9%) were asymptomatic. The main referred symptoms were fever (n = 37, 44%), asthenia (n = 14, 16.7%), rhinitis (n = 16, 19%), ageusia (n = 19, 22.6%), anosmia (n = 24, 28.6%), sore throat (n = 3, 3.6%), cough (n = 9, 10.7%), arthralgia-myalgia (n = 11, 13.1%), headache (n = 23, 27.4%) and gastrointestinal symptoms (n = 7, 8.3%). No child presented evidence of cardio-pulmonary function impairment after an average time of 77.3 days (SD 35) from SARS-CoV-2 swab positivity and a median of 68 days (IQ1 52, IQ3 92.5). Conclusion This preliminary study suggests that, in the absence of specific symptoms, the diagnostic yield of cardio-pulmonary tests before returning to play sports may be very low.

Purpose: Compare ibuprofen and ketorolac for children with trauma-related acute pain. Methods: 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 hours. At the admission, patients were classified based on numeric rating scale-11 (NRS-11) in moderate (NRS 4-6) and severe (NRS 7-10) pain groups. Each patient was randomized to receive either ibuprofen (10 mg/kg) or ketorolac (0.5 mg/kg) and the placebo of the not given drug in a double dummies design. NRS-11 was asked every 30 minutes until 2 hours after drug and placebo administration. The primary outcome was NRS-11 reduction at 60 minutes. Results: Among 125 patients with severe pain, NRS-11 reduction after 60 minutes from drug administration was 2.0 (IQR 1.0-4.0) for ibuprofen and 1.0 (IQR 1.0-3.0) for ketorolac (p=0.36). Ibuprofen was significantly better, considering secondary outcomes, at 90 minutes with a lower median of NRS-11 (p 0.008), more patients with NRS-11 less than 4 (p 0.01) and a reduction of pain score of more than 3 NRS-11 points (p 0.01). Among 87 patients with moderate pain, the NRS-11 reduction after 60 minutes from drug administration was 1.63 (±1.8) for ibuprofen and 1.8 (±1.6) for ketorolac, with no statistically significant difference. Conclusions: Oral ibuprofen and ketorolac are similarly effective in children and adolescents with acute traumatic musculoskeletal pain. ClinicalTrial.gov registration number: NCT04133623

The case of a 7-year-old girl presenting with several cutaneous hypopigmented patches is described. The diagnosis of lichen sclerosus was finally made based on the presence of the typical perivulvar ivory white atrophic lesions.

Sex differences play a relevant role in cancer susceptibility, incidence and survival. Exploring such differences is difficult because of the close interplay of genetic, epigenetic and hormonal factors. However, a better understanding of the role of such disparities in cancer mechanisms could improve its prevention and therapy. Our study explores how sex differences in pediatric outcomes vary after undergoing first and advanced-line therapy for hematological malignancies. The primary goal was to evaluate if sex differences in pediatric outcomes after first-line therapy persist after allogeneic hematopoietic stem cell transplantation (HSCT). The secondary goal was to analyze sex differences in disease risk at onset and pediatric outcomes after first-line therapy to compare our results with the literature's reported results. Among a total of 485 patients (280 males, 205 females) admitted for hematological malignancies, disease risk at the onset was significantly higher in males (P < .05). One hundred and seventy-four patients (111 males and 63 females) had a high-risk disease requiring HSCT. Before HSCT, all patients underwent myeloablative conditioning, which substantially impaired gonadal function. Although the number of boys undergoing HSCT was almost double that of girls, there were no sex-related differences in overall survival, cancer relapse and complications after HSCT exposure (P > .05). These findings suggest that the existing sex differences in cancer risk ab initio can be somehow flattened by a conditioning regimen, shedding new light on the role of hormonal factors in cancer mechanism and management.

Aim We compared visits to a tertiary level paediatric emergency department (PED) in Italy, during winter 2020-2021, when COVID-19 social distancing measures were in place, with winter 2019-2020. Methods This was a retrospective analysis of an electronic database. We obtained the number of visits and the ages, main complaints, triage codes, discharge diagnoses and outcomes of patients who accessed the PED from the 1 October 2020 to 28 February 2021. These were compared to the same period in 2019-2021. Results Visits fell by 52%, from 10982 in 2019-2020 to 5317 in 2020-2021 (p < 0.0001). The reductions were 52% in neonates, 58% in infants, 53% in toddlers, 61% in preschool children, 48% in school children and 46% in adolescents. Non-urgent and urgent triage codes declined. Respiratory and gastrointestinal infections fell by 72% and 71% respectively. Injuries declined by 42%, mainly among adolescents. Accidental intoxication, psychiatric symptoms and substance or alcohol abuse declined by 24%, 33% and 64%. Hospital admissions reduced by 8% and admissions to intensive care fell by 29%. Conclusion During the first winter of pandemic social distancing visits to an Italian PED fell by 52%, with higher reductions in younger children and infants, and hospital admissions fell by 8%.

A 7-year-old girl with a longstanding history of recurrent pneumonia, arthritis and unsteady gait was finally diagnosed with ataxia-telangiectasia. The classical bulbar conjunctival telangiectasia was present and helped to make the diagnosis.

To investigate the coping strategies of a group of adolescents with somatic symptom disorder compared to non-somatic symptom disorder peers during the COVID-19 related lockdown.This cross-sectional study is the second part of a previously published study showing an improved trend in depression and anxiety in a group of patients with somatic symptom disorder compared to non-somatic symptom disorder peers. An anonymous semi-structured survey was distributed to two groups of Italian adolescents to measure the impact of quarantine on their daily life and coping strategies.We recruited 115 adolescents, 58 (50.4%) mean age 15.3, with a recent diagnosis of somatic symptom disorder and 57 (49.6%) mean age 15.8, control peers.The aim of this study was to detect differences in coping strategies and relationships with parents and peers, during the lockdown period in a group of adolescents with somatic symptom disorder and low disease burden when compared with a non-somatic symptom disorder group.The relationship with parents significantly worsened in 4 (6.9%) of adolescents with somatic symptom disorder compared to 12 (21.1%) adolescents in the non-somatic symptom disorder group (p = 0.048). The relationship with peers significantly improved in 13 (22.4%) of adolescents with symptom disorder versus 3 (5.3%) of peers of the non- somatic symptom disorder group (p = 0.013).Adolescents with somatic symptom disorder with a low burden of physical symptoms experienced less deterioration in their relationships with parents and peers than the non-somatic symptom disorder group.

Adolescents with mental health disorders are a high-risk population and problems during COVID-19 lockdowns have included increasing, widespread anxiety, fear, anger and uncertainty. Somatic symptom disorder (SSD) is characterised by disproportionate thoughts, feelings and behaviours about physical symptoms associated with the distress and disruption of everyday functioning. SSD accounts for 15-25% of adolescent mental health cases in primary care paediatric settings,3 and 8.6% of non-traumatic adolescent pain in emergency departments.4.

MED13-related disorder is a new neurodevelopmental disorder recently described in literature, which belongs to the group of CDK8-kinase module genes-associated conditions. It is characterized by variable intellectual disability and/or developmental delays, especially in language. Autism spectrum disorder (ASD), attention deficit hyperactivity disorder (ADHD), eye or vision problems, hypotonia, mild congenital hearth abnormalities and dysmorphisms have been described among individuals with MED13 mutations. We report the case of a 13-year-old girl who received a previous clinical diagnosis of Kabuki syndrome (KS) without mutations in classic KS genes. After a whole exome sequencing (WES) analysis a de novo missense mutation in MED13 (c.C979T; p.Pro327Ser) was found. This variant has been once described in literature as accountable for a novel neurodevelopmental disorder. The aim of this report is to improve clinical delineation of MED13-related condition and to explore differences and similarities between KS spectrum and MED13-related disorders.

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. 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. Several studies investigated the efficacy of topical clobetasol for immuno-related mucositis, but little is known about its pharmacokinetics and side effects. In this report, we have reviewed the literature, defining a maximum putative dose of clobetasol mucosal gel to avoid Cushing syndrome.

A 13-year-old boy was admitted for a 4-day history of fever, malaise, sore throat and cough, treated with amoxicillin for 2 days. Physical examination revealed sparse targetoid cutaneous lesions, stomatitis with vesicles, blisters and mild conjunctival hyperaemia (figure 1). Blood tests showed an increase in erythrocyte sedimentation rate (92 mm/hour; normal value

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A 15-year-old boy was admitted with a history of cytopenia (white blood cell count 3.170/μm, platelets 90.000/μm) associated with splenomegaly, found during investigations for recurrent mild jaundice due to Gilbert’s syndrome.He was in good general health, without systemic symptoms; therefore, the leading causes of asymptomatic splenomegaly were excluded. Coagulation, liver tests and abdomen ultrasound (US) were normal, showing a hepatopetal portal flow to the colour-Doppler. There was no sign of haemolysis on haematology investigations. The C reactive protein, immune globulins levels and erythrocyte sedimentation rate were normal, excluding both an infective and an immune regulation disorder. We excluded the haematological malignancy and lymphoproliferative disorders through a peripheral blood smear and a bone marrow biopsy.His history was remarkable for neonatal sepsis, which required umbilical venous catheter during hospitalisation in a neonatal intensive care unit (NICU). The patient follow-up was interrupted for a while, probably due to his good health condition.At age 17 years, the child accessed our emergency department. for a minor trauma to the limbs, and his physical examination was unremarkable, except for the splenomegaly. We repeated the abdomen US, with colour flow Doppler (figure 1).Figure 1B-mode shows 1.4 cm of maximum calibre of portal vein at hilus with slightly perihilar hyperechogenicity; colour and power Doppler US shows preserved hepatopetal flow and PSV of 41 cm/s (normal range 20–40 cm/s). PSV, peak systolic velocity; US, ultrasound.QuestionsWhat is the most likely diagnosis?Portal vein obstructionGaucher diseaseAutoimmune sclerosing cholangitisLeukemic hepatic infiltrationWhat is the gold standard imaging for diagnosis?Abdomen Doppler USContrast-enhanced CTCT without contrastUltrasound-based elastographyHow should this child be managed?Upper gastrointestinal endoscopyBeta-blocker therapyLeft-mesenteric portal vein bypass (Meso-Rex bypass)Transjugular intrahepatic porto-systemic shuntAnswers can be found on page 02.

ObjectiveThis study aims to explore the attitude of adolescents with chronic diseases toward social media exposure, focusing in particular on Facebook.DesignCross-sectional study.SettingAn 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.PatientsWe recruited 212 adolescents (aged between 13 and 24 years) with a diagnosis of inflammatory bowel disease, coeliac disease, diabetes mellitus type 1 and cystic fibrosis.ResultsTwo hundred and seven of the 212 (97.6%) expressed the need of sharing their illness experience with friends, 201 out of 212 (94.8%) usually searched information on the internet to find new therapies and to discover their prognosis. One hundred and forty-nine out of 212 adolescents (70.3%) perceived dependence on their parents as the most negative aspect of having a chronic disease, and 200 out of 212 (94.3%) were looking for friends with the same disease on Facebook. Two hundred and ten out of 212 (99.1%) did not want their doctors or nurse on their social media platforms. During the active disease periods, the time spent with social media increased from an average of 5 to 11 hours.ConclusionsThis descriptive analysis focused on the Facebook impact on chronic disease perception among affected adolescents. It showed that they used to spend an increased amount of time on this platform during disease flare-up and highlighted their wish of keeping doctors and nurses away from their social dimension.

Internet influences our communication, social and work interactions, entertainment, and many other aspects of life. Even if the original purpose was to simplify our lives, an excessive and/or maladaptive use of it may have negative consequences. The dopamine transporter (DAT1) gene was studied in relation to addictions, including excessive use of the Internet. The crucial role of DAT1 was previously underlined in modulating emotional aspects, such as affiliative behaviors. The present research follows a new approach based on cross-correlation between (de)methylation levels in couples of CpG loci, as previously shown. We investigated the possible relationships between Internet addiction, impulsivity, quality of attachment, DAT1 genotypes (from the 3&prime, untranslated region (UTR) variable number of tandem repeats (VNTR) poly-morphism), and the dynamics of methylation within the 5&rsquo, UTR of the DAT1 gene. From a normative sample of 79 youths, we extrapolated three subgroups a posteriori, i.e., one &ldquo, vulnerable&rdquo, with high Internet Addiction Test (IAT) scores (and high Barrat Impulsivity Scale (BIS) scores, n = 9) and two &ldquo, controls&rsquo, &rsquo, with low BIS scores and 10/10 vs. 9/x genotype (n = 12 each). Controls also had a &ldquo, secure&rdquo, attachment pattern, while genotypes and attachment styles were undistinguished in the vulnerable subgroup (none showed overt Internet addiction). We found a strongly positive correlation in all groups between CpG2 and CpG3. An unsuspected relationship between the 3&rsquo, UTR genotype and a 5&rsquo, UTR intra-motif link was revealed by CpG5&ndash, CpG6 comparison. The negative correlation between the CpG3&ndash, CpG5 positions was quite significant in the control groups (both with genotype 10/10 and with genotype 9/x), whereas a tendency toward positive correlation emerged within the high IAT group. In conclusion, future attention shall be focused on the intra- and inter-motif interactions of methylation on the CpG island at the 5&prime, UTR of DAT1.

Aim - To evaluate the cardiopulmonary function in children returning to play sports after mild or asymptomatic SARS-CoV-2 infection. - Methods - This is a consecutive case series conducted at the Institute for Maternal and Child Health, Trieste, Italy. Paediatric patients who accessed the Institute for cardiologic and pneumological evaluation before the return to play competitive sports were recruited. According to the Italian Sports Medical Federation recommendations, echocardiogram, electrocardiogram, treadmill ECG test and pulmonary function tests were performed. - Results - 240 patients (aged 8-17 years old) were recruited. Among these, 233 children were considered for the final analysis. Out of 233, 147 (63.1%) had a mild symptomatic form of SARS-CoV-2 infection, while 86 (36.9%) were asymptomatic. The main referred symptoms were fever (n = 68, 46.3%), headache (n = 42, 28.6%), anosmia (n = 40, 27.2%), rhinitis (n = 34, 23.1%), ageusia (n = 32, 21.8%), cough (n = 22, 15%), asthenia (n = 21, 14.3%), arthralgia-myalgia (n = 20, 13.6%), sore throat (n = 14, 9.5%) and gastrointestinal symptoms (n = 9, 6.1%). No child presented evidence of cardiopulmonary function impairment after an average time of 76.1 days (SD 39.2) from SARS-CoV-2 swab positivity and a median of 68 days (IQ1 49, IQ3 98). - Conclusion - This study suggests that the diagnostic yield of cardiopulmonary tests before returning to play sports may be very low in the absence of specific symptoms.