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Key words: Churg-strauss syndrome, Eosinophilic granulomatosis with polyangiitis in childhood, Vasculitis, Asthma, Hypereosinophilia Background - Eosinophilic granulomatosis with polyangiitis, formerly known as Churg-Strauss syndrome, is an extremely rare systemic vasculitis in the paediatric population. The hallmarks of eosinophilic granulomatosis with polyangiitis are a long history of asthma and peripheral eosinophilia with eosinophilic inflammation that may involve several organs. Findings - The paper reports the clinical characteristics, courses, and outcomes of the four patients diagnosed with eosinophilic granulomatosis with polyangiitis at IRCCS Burlo Garofolo (Trieste, Italy) from 1996 to 2015. The mean age at diagnosis was 11.5 years. All the patients presented a history of asthma and peripheral eosinophilia at diagnosis. 3/4 of the children presented upper airway and pulmonary disease. Skin and heart involvement was present in half of the patients, whereas gastrointestinal and neurological symptoms were reported in 25% of the cases. When performed, tissue biopsy revealed eosinophilic inflammation in all the cases. Anti-neutrophil cytoplasmic antibodies were negative in 66% patients. One young child died shortly after presentation, one remitted after immunosuppressive treatment and two patients needed low-dose corticosteroid therapy to maintain the remission. Conclusion - Comparison with an updated review of the series and cases of childhood-onset eosinophilic granulomatosis with polyangiitis reported in the literature showed similar demographic characteristics, clinical features and outcomes. Cardiac disease represents the poorer prognostic factor, leading to the 60% of the deaths reported.

Mouhamed, a 7-year-old boy of African origin, presented with progressive fatigue and difficulty in walking. He was never treated with vitamin D supplementation. The evaluation of his calcium-phosphorus metabolism revealed a myopathy related to severe rickets. Therefore, he was treated with high-dose vitamin D3 and myopathy and fatigue progressively resolved. Vitamin D plays a crucial role in the calcium-phosphorus metabolism, by acting on enterocytes, osteoclasts and renal tubule. Vitamin D deficiency is defined when the 25OHD value is less than 20 ng/ml. In order to guarantee the assumption of the minimum daily dose of vitamin D, it is recommended to start vitamin D3 supplementation in all newborns and infants in their first year of life, regardless of the feeding modality. Exposure to the sun is essential for the activation of vitamin D, so dark-skinned children and mothers or those little exposed to the sun should start vitamin D3 supplementation. Vitamin D3 should also be supplemented in children with cerebral palsy and in patients treated with anti-epileptic drugs. Other conditions at risk of vitamin D deficiency are inflammatory bowel disease, celiac disease, cystic fibrosis, obesity, liver failure, cholestasis and vegetarian or vegan diets. Classic signs of rickets are the rickety rosary, the widening of the wrist and the arching of the tibia. Severe hypocalcaemia secondary to vitamin D deficiency can occur with dilated cardiomyopathy or convulsions, especially in dark-skinned infants. Vitamin D deficiency should be considered in children with progressive myopathy or muscular weakness, especially in dark-skinned ones or in those poorly exposed to the sun for cultural or religious reasons.

Introduction: The present study aimed at evaluating Italian epidemiological trends of pediatric inflammatory bowel diseases (IBD) over the period 2009–2018. Materials and methods: Data from 1969 patients enrolled in the Italian Society of Pediatric Gastroenterology, Hepatology and Nutrition Registry, by 49 pediatric IBD centers throughout the country, were analyzed, comparing three different time intervals (2009–2012, 2013–2015, 2016–2018). Results: The number of new IBD diagnoses ranged from 175 to 219 per year, evenly distributed over the examined period of time. From 2009 to 2018, the minimal incidence ranged from 1.59 to 2.04 /105 inhabitants aged < 18 years, with an overall slight predominance of ulcerative colitis (UC) over Crohn's disease (CD) (ratio: 1.1). Mean diagnostic delay was 6.8 months for CD and 4.1 months for UC, with a significant reduction for CD when comparing the three-time intervals (p =0.008). The most frequent disease locations according to the Paris classification were ileocolonic for CD (41.3%) and pancolitis for UC (54.6%). Conclusions: The minimal incidence rate in Italy seems to have stabilized over the last two decades, even if it has increased when compared to previous reports. UC is still slightly more prevalent than CD in our country. Diagnostic delay significantly decreased for CD, reflecting an improved diagnostic capacity.

Altered state of consciousness in infants can be the sole sign of a compromised intestinal blood flow. The article reports the case of an infant with inconsolable crying and lethargy with an omphalomesenteric duct remnant causing small bowel volvulus.

Background Adult patients with both inflammatory bowel disease (IBD) and celiac disease (CeD) have peculiar phenotypic features. This study aimed at describing the characteristics and natural history of children with both IBD and CeD. Methods This was a case-control study based on a national registry. Cases included children diagnosed with both IBD and CeD. Two matched IBD controls without CeD, and 2 matched CeD controls were selected for each case. Inflammatory bowel disease phenotype and natural history, comprising growth and pubertal development, were compared between groups. Results Forty-nine (1.75%) patients with IBD and CeD were identified out of 2800 patients with IBD. Compared with patients with IBD alone, patients with IBD and CeD presented more frequently with autoimmune diseases (odds ratio, 2.81; 95% CI, 0.97–8.37; P = 0.04). Ileocolonic localization (46.1% vs 73.1%), treatment with azathioprine (46.2% vs 71.2%), and anti-TNF biologics (46.2% vs 69.2%) were less common in patients with Crohn’s disease and CeD than in patients with Crohn’s disease alone. Patients with ulcerative colitis and CeD had an increased risk of colectomy despite similar medical treatments compared with patients with ulcerative colitis alone (13.0% vs 0%). Pubertal delay was more common in patients with IBD and CeD compared with patients with IBD alone (14.9% vs 3.2%; odds artio, 5.24; 95% CI, 1.13–33.0; P = 0.02) and CeD alone (14.9% vs 1.1%; P = 0.002). Conclusions Children with IBD and CeD may have peculiar features with a higher risk for autoimmune diseases, colectomy, and pubertal delay compared with IBD alone.

AimDescribe the use of the pediatric emergency department of a tertiary-level children’s hospital in Italy by adolescents. MethodsThis retrospective study was based on the medical records of patients aged 13 to 17 years, who accessed the pediatric emergency department of the Institute for Maternal and Child Health IRCCS Burlo Garofolo, from 1 January to 31 December 2018. For each patient, we reported: age, gender, triage category, chief complaint, diagnosis, admission status, and hospital ward of destination. The primary outcome was to describe the use of the department in terms of leading causes of access, diagnoses, and outcomes. ResultsDuring the study period, 3062 adolescents accessed to the department, for a total of 3895 unscheduled visits. The principal causes of access were trauma (45.3%) and organic diseases (38.8%). 249 adolescents (6.4%) had mental health problems. One-hundred-forty-two adolescents (3.6%) were admitted to the hospital, 54 of whom to the neuropsychiatric ward, for mental health problems. Compared to younger patients entering the department, adolescents were admitted to the neuropsychiatric ward more frequently (p

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Objectives Parents have a central role in the management of children with inflammatory bowel disease (IBD). Alterations in parental psychological well-being may affect the patient's health-related quality of life (HRQoL). This study aimed to evaluate the correlation between maternal and paternal distress, anxiety, depression and pain catastrophizing and the HRQoL of patients with IBD. Methods Children with IBD ages 8 to 18 years and their parents were prospectively recruited. Children answered questionnaires on HRQoL while parents completed an assessment of distress, anxiety, depression, and pain catastrophizing. Univariate and multivariate regression models analysis were used to evaluate correlations between parental measures and patient's HRQoL and between the factors related to children health and parental psychological suffering. Results One hundred patients (45 Crohn disease, 55 ulcerative colitis), 90 mothers and 62 fathers were enrolled. Parents had high levels of distress while anxiety, depression, and pain catastrophizing levels were relatively low. Parental distress had the most substantial correlation with children's HRQoL and was associated with patients' disease activity and recent flares. On multivariate regression analysis, parental factors explained less than 20% of the variance in the children's HRQoL scores. Mothers suffered from psychological alterations more frequently than fathers, but the parental inter-rater agreement was strong in regards to distress and anxiety. Conclusions Parental distress is high and correlates with the HRQoL of children with IBD. Interventions aimed at evaluating and managing parental distress should be considered during the management of children with IBD.

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