BackgroundBehçet’s disease is a rare multi-systemic inflammatory disease with unknown etiology which involves principally oral and genital mucosa, skin and eyes. Average age at onset of the disease is about 25-30 years, but it may be diagnosed before the age of 16. It is not very rare in Italy, even though there are limited data concerning epidemiology. Aim of this study is to describe the baseline data of an Italian cohort of patients with as having BD or probable BD.MethodsWe described the baseline data of the first national epidemiological study on children coming from 16 Italian Pediatric Rheumatologic Centers diagnosed by the treating physicians as having Behçet’s Disease. Data on demographic characteristics, clinical features and therapy were collected. We then compared our findings to those of international pediatric cohort studies and also retrospectively evaluated the ability to diagnose BD using ISG, ICBD and, for the first time, the new PEDBD criteria.ResultsThe study included 110 patients (62 M, 48F). Average age at onset was 8.34±4.11 years. The frequencies of signs/symptoms were: recurrent oral aphtosis 94.5%, genital ulcers 33.6%, ocular 43.6%, gastrointestinal 42.7%, musculoskeletal 42.7%, neurological 30.9% and vascular involvement 10%. Thirty-two patients (29.1%) fulfilled ISG, 78 (70.9%) ICBD, 50 (45.5%) PEDBD criteria and 31 (28%) didn’t fulfill any of them. The most frequently used treatments were colchicine and corticosteroids followed by immunosuppressants. Four patients received biologic therapy (anti TNF-α and anti-IL-1) to treat severe organ involvement.ConclusionsRecurrent oral aphtosis was the most frequent clinical manifestation, followed by ocular involvement. Gastrointestinal lesions were more frequent in Italy than in non-European countries as opposed to genital ulcers. Skin, ocular and vascular manifestations had a higher frequency in males and genital ulcers in females. Constitutional symptoms were present in 44.5% and recurrent fever in one third of our population.

Food protein-induced allergic proctocolitis (FPIAP) is a condition characterized by inflammatory changes in the distal colon in response to one or more foreign food proteins because of immune-mediated reactions. FPIAP prevalence estimates range widely from 0.16% in healthy children and 64% in patients with blood in stools. In clinical practice, FPIAP is diagnosed when patients respond positively to the elimination of a suspected triggering food allergen. Nevertheless, significant proportions of infants get misdiagnosed with IgE mediated allergy and undergo unnecessary dietary changes. Diagnosis is based on clinical symptoms, a good response to an allergen-free diet and the recurrence of symptoms during the “allergy challenge test”. Sometimes clinical features may be non-specific and the etiology of rectal bleeding in childhood may be heterogeneous. Therefore, it is crucial to exclude a variety of other possible causes of rectal bleeding in the pediatric age group, including infection, anal fissure, intestinal intussusception and, in infants, necrotizing enterocolitis and very early onset inflammatory bowel disease. The diagnostic workup includes in those cases invasive procedures such as sigmoidoscopy and colonoscopy with biopsies. The high prevalence of FPIAP contrasts with the lack of known information about the pathogenesis of this condition. For this reason and due to the absence of a review of the evidence, a literature review appears necessary to clarify some aspects of allergic colitis. The aim of the review is to fill this gap and to lay the foundations for a subsequent evidence-based approach to the condition.

The aim of this study was to assess the impact of the COVID-19 pandemic and population lockdown on pediatric ED consultations. Methods: A cross-sectional study on pediatric emergency department consultations before and during the current COVID-19 pandemic (March-May 2019 vs. March-May 2020) was performed in two hospitals in the Campania region (Southern Italy) [i.e., Salerno University Hospital (Salerno) and Pediatric Regional Referral Emergency Hub "AORN Santobono-Pausillipon" (Naples)]. Results: 29,368 consecutive ED pediatric patients (13,430 females; mean age ± SD = 5.4 ± 4.7 years) were seen in March-May 2019 and 9,133 (4,494 females; mean age ± SD = 5.9 ± 4.2 years) in March-May 2020. Resuscitation/emergency and urgent care pediatric ED consultations were 1,388 (4.7%, 95% CI 4.5-4.9) in the 2019 trimester, while they were 648 (7.1%, 95% CI 6.6-7.6) in the 2020 trimester (p < 0.01). Mean pediatric ED daily consultations were 326.3 (95% CI 299.9-352.7) in the considered period of 2019 and 101.4 (95%CI 77.9-124.9) in the same period of 2020 (p < 0.001). COVID-19 nasal swabs were performed for 385 children; of those, six resulted positive and four of them were hospitalized. Conclusions: This work provides a unique snapshot of the pediatric EDs demands in the era of COVID-19. We witnessed a significant reduction of non-urgent health care demands during the pandemic but an increase of more severe urgent cases. The COVID-19 pandemic and the following lockdown unveiled the inappropriateness of the majority of pediatric ED consultations. Nevertheless, the current scenario highlighted the need for appropriate and timely clinical evaluations in the pediatric primary care to tackle late and more severe diagnoses in EDs.

This is the first epidemiologic study on Kawasaki disease incidence in the country of Italy. Figures are in line but slightly higher than those reported for other European countries. What is known: • Kawasaki disease is more common in Asian populations. • European incidence data are scarce. What is new: • Epidemiological data in Italy show similar incidence, albeit slightly higher, than in other European countries. • Incidence data slightly rose in the recent past.

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BackgroundKawasaki disease (KD) is a systemic vasculitis with an acute and self-limited course. The incidence of KD differs widely among ethnic groups and is higher in the Asian population. In Italy, no recent data are available. Our purpose is to define the epidemiology of Kawasaki disease in the years 2008–2013 in children aged < 14 years in the Italian regions of Tuscany and Emilia Romagna through administrative data.MethodsWe studied the epidemiology of KD in the years 2008–2013 in children 0–14 years old resident in Tuscany and in Emilia Romagna regions using hospital ICD-9 discharge codes with a thorough data cleaning for duplicates.ResultsThe distribution of the KD patients across ages was similar for the two regions with a peak in the second year of life. When considering data of the two regions together, the rate of incidence was 17.6 for 100,000 children under 5 years. For both Regions the incidence rose slightly during the study period and had a seasonal distribution, with higher incidence in spring and winter.ConclusionThis is the first Italian study performed through the use of administrative data. Figures are in line but slightly higher than those published in other European countries.

Background Intestinal Ganglioneuromatosis (IG) is a rare disorder of the enteric nervous system. In pediatric age it is often associated with genetic syndromes such as Neurofibromatosis 1 (NF1), multiple endocrine neoplasia type 2B (MEN2B) and Cowden syndrome (PTEN mutation), and ganglioneuromas (GNs) may be sometimes the first sign of the disease. Isolated GNs are rare and sporadic. Clinical symptom vary and depend on the size and on the location of the GNs. This disorder affects intestinal motility and it, consequently, causes changes in bowel habits, abdominal pain, occlusive symptoms and rarely lower gastrointestinal bleeding secondary to ulceration of the intestinal mucosa. On the other hand, patients can remain asymptomatic for many years. Case presentation We describe a 9-year-old boy referred to our emergency department for right lower quadrant abdominal pain. No familial history for gastrointestinal disorders. No history of fever or weight loss. At physical examination, he had diffused abdominal pain. Abdominal ultrasonography showed a hypoechoic formation measuring 41.8 mm by 35 mm in the right lower quadrant of the abdomen. Routine blood tests were normal, but fecal occult blood test was positive. Abdominal TC confirmed the hypodense formation, of about 5 cm in transverse diameter, in the right hypochondrium that apparently invaginated in the caecum-last ileal loop. Colonoscopy showed in the cecum an invaginated polypoid lesion of the terminal ileal loop. Laparoscopic resection of the polypoid lesion was performed. Histological diagnosis of the large neoplasm observed in the terminal ileum was diffuse ganglioneuromatosis. NF1, RET and PTEN gene tests resulted negative for specific mutations. At the 1 year follow-up, the patient presented good general condition and blood tests, fecal occult blood test, esophagogastroduodenoscopy, colonoscopy and MR-enterography were negative. Conclusions Only few cases are reported in literature of IG in pediatric age. Although rare, the present case suggests that this disorder must be taken in consideration in every patient with GI symptoms such as abdominal pain, constipation, lower intestinal bleeding, in order to avoid a delayed diagnosis.

Skin ulcers are a dangerous and uncommon complication of vasculitis. We describe the case of a teenager suffering from Systemic Lupus Erythematosus with digital ulcer resistant to conventional therapy, treated successfully with Hyperbaric Oxygen Therapy. The application of hyperbaric oxygen, which is used for the treatment of ischemic ulcers, is an effective and safe therapeutic option in patients with ischemic vasculitic ulcers in combination with immunosuppressive drugs. Further studies are needed to evaluate its role as primary therapy for this group of patients.