Incidence and Prevalence of Multisystem Inflammatory Syndrome in Children (MIS-C) in Southern Italy.

Multisystem inflammatory syndrome in children (MIS-C) is a pediatric hyperinflammatory syndrome related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection whose epidemiology is not very well known at present. The objective of the study was to better understand the incidence of MIS-C in the Apulia region in southern Italy. Our primary goal was to estimate the incidence of newly identified cases of MIS-C in children aged 0-18 years, during a period of six months, encompassing the second pandemic wave. We also analyzed the characteristics of our cohort in terms of clinical features, treatment, and outcomes. The cumulative incidence of MIS-C was 3.27 per 100,000 residents between 0 and 18 years of age. In our cohort, gastrointestinal, mucocutaneous, and cardiac involvement were the most common clinical features. With our step-up approach to therapy, no patients required intensive care unit (ICU) admission and no cardiac sequelae after 6 months of onset were found in echocardiograms. Conclusion: Our epidemiological study of MIS-C in southern Italy showed unexpectedly overlapping figures with other US studies.

Canakinumab efficacy and long-term tocilizumab administration in tumor necrosis factor receptor-associated periodic syndrome (TRAPS).

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autosomal dominantly inherited autoinflammatory disease caused by mutations in the TNFRSF1A gene. Treatment is aimed at preventing acute disease attacks, improving quality of life, and preventing long-term complications such as systemic reactive amyloidosis. Biologic agents have significantly improved TRAPS management. In particular, interleukin 1 (IL-1) inhibition either with the recombinant IL-1 receptor antagonist anakinra or with the human IgG1 anti-IL-1ß monoclonal antibody canakinumab has recently shown to induce a prompt and stable disease remission. Conversely, the successful experience with IL-6 inhibition is nowadays limited to a single patient. Anyway, introduction of new treatment options for patients requiring a lifelong therapy is desirable. We describe two TRAPS patients (son and father) successfully treated with canakinumab and tocilizumab, respectively. In particular, we highlight the clinical and laboratory efficacy as well as the good safety profile of tocilizumab during a 42-month follow-up period.

Efficacy of adalimumab in young children with juvenile idiopathic arthritis and chronic uveitis: a case series.

BACKGROUND: Juvenile idiopathic arthritis is a relatively common chronic disease of childhood, and is associated with persistent morbidity and extra-articular complications, one of the most common being uveitis. The introduction of biologic therapies, particularly those blocking the inflammatory mediator tumor necrosis factor-α, provided a new treatment option for juvenile idiopathic arthritis patients who were refractory to standard therapy such as non-steroidal anti-inflammatory drugs, corticosteroids and/or methotrexate. CASE PRESENTATIONS: The first case was a 2-year-old girl with juvenile idiopathic arthritis and uveitis who failed to respond to treatment with anti-inflammatories, low-dose corticosteroids and methotrexate, and had growth retardation. Adalimumab 24 mg/m2 every 2 weeks and prednisone 0.5 mg/kg/day were added to methotrexate therapy; steroid tapering and withdrawal started after 1 month. After 2 months the patient showed good control of articular and ocular manifestations, and she remained in remission for 1 year, receiving adalimumab and methotrexate with no side effects, and showing significant improvement in growth. Case 2 was a 9-year-old boy with an 8-year history of juvenile idiopathic arthritis and uveitis that initially responded to infliximab, but relapse occurred after 2 years off therapy. After switching to adalimumab, and adjusting doses of both adalimumab and methotrexate based on body surface area, the patient showed good response and corticosteroids were tapered and withdrawn after 6 months; the patient remained in remission taking adalimumab and methotrexate. The final case was a 5-year-old girl with juvenile idiopathic arthritis for whom adalimumab was added to methotrexate therapy after three flares of uveitis. The patient had two subsequent episodes of uveitis that responded well to local therapy, but was then free of both juvenile idiopathic arthritis and uveitis symptoms, allowing methotrexate and then adalimumab to be stopped; the patient remained in drug-free remission. CONCLUSION: This report includes the first published case of the use of adalimumab in a child aged <3 years. Our clinical experience further supports the use of biologic therapy for the management of juvenile idiopathic arthritis and uveitis in children as young as two years of age.

Juvenile polyp.

The most frequent clinical presentation of intestinal polyps is painless rectal bleeding. Although colonoscopy is the modality of choice for the diagnosis of intestinal polyps, sonography may be the first diagnostic tool in the evaluation of children with bloody stools. Ultrasonography has the advantage of being safe and noninvasive.

Lung ultrasound characteristics of community-acquired pneumonia in hospitalized children.

BACKGROUND: The diagnosis of community-acquired pneumonia (CAP) is based mainly on the patient's medical history and physical examination. However, in severe cases a further evaluation including chest X-ray (CXR) may be necessary. At present, lung ultrasound (LUS) is not included in the diagnostic work-up of pneumonia. AIM: To describe the ultrasonographic appearance of CAP at presentation and during the follow-up. METHODS: A total of 102 patients with clinical signs and symptoms suggesting pneumonia, who underwent a clinically driven CXR, were evaluated by LUS on the same day. LUS signs of pneumonia included subpleural lung consolidation, B-lines, pleural line abnormalities, and pleural effusion. The diagnostic gold standard was the ex-post diagnosis of pneumonia made by two independent experienced pediatricians on the basis of clinical presentation, CXR and clinical course following British Thoracic Guidelines recommendations. RESULTS: A final diagnosis of pneumonia was confirmed in 89/102 patients. LUS was positive for the diagnosis of pneumonia in 88/89 patients, whereas CXR was positive in 81/89. Only one patient with normal LUS examination had an abnormal CXR, whereas 8 patients with normal CXR had an abnormal LUS. LUS was able to detect pleural effusion resulting from complicated pneumonia in 16 cases, whereas CXR detected pleural effusion in 3 cases. CONCLUSIONS: LUS is a simple and reliable imaging tool, not inferior to CXR in identifying pleuro-pulmonary alterations in children with suspected pneumonia. During the course of the disease, LUS allows a radiation-free follow-up of these abnormalities.

Lung ultrasound in bronchiolitis: comparison with chest X-ray.

The diagnosis of bronchiolitis is based mainly on the patient's medical history and physical examination. However, in severe cases, a further evaluation including chest X-ray (CXR) may be necessary. At present, lung ultrasound (LUS) is not included in the diagnostic work-up of bronchiolitis. This study aimed to compare the diagnostic accuracy of LUS and CXR in children with bronchiolitis, and to evaluate the correlation between clinical and ultrasound findings. Only patients with a diagnosis of bronchiolitis, who had undergone a CXR, were enrolled in the study. Fifty-two infants underwent LUS and CXR. LUS was also performed in 52 infants without clinical signs of bronchiolitis. LUS was positive for the diagnosis of bronchiolitis in 47/52 patients, whereas CXR was positive in 38/52. All patients with normal LUS examination had a normal CXR, whereas nine patients with normal CXR had abnormal LUS. In these patients, the clinical course was consistent with bronchiolitis. We found that LUS is a simple and reliable tool for the diagnosis and follow-up of bronchiolitis. It is more reliable than CXR, can be easily repeated at the patient's bedside, and carries no risk of irradiation. In some patients with bronchiolitis, LUS is able to identify lung abnormalities not revealed by CXR. Furthermore, there is a good correlation between clinical and ultrasound findings. Given the short time needed to get a US report, this technique could become the routine imaging modality for patients with bronchiolitis.

Comparison of esophageal pH and multichannel intraluminal impedance testing in pediatric patients with suspected gastroesophageal reflux.

BACKGROUND: Multichannel intraluminal impedance (MII) is a pH-independent method of assessing gastroesophageal reflux. AIM: To evaluate the diagnostic accuracy of MII-pH as compared with conventional pH monitoring in detecting reflux events (REs) and symptom association in different age groups. METHODS: : A prospective direct comparison of 2 diagnostic techniques on 291 consecutive patients referred for suspected gastroesophageal reflux disease. Sensitivity and diagnostic accuracy of MII-pH versus pH monitoring and symptom association were measured. RESULTS: MII-pH detected 13631 REs, 6260 (46%) of which were nonacid. The prevalence of weakly acid refluxes in the 24 hours and postprandial period as well as the proximal extension of refluxate were significantly greater in infants as compared with children (P < 0.001, P < 0.001, and P < 0.01, respectively). The diagnostic accuracy of combined MII-pH in revealing all RE and acid RE were significantly higher in infants as compared with children (92% vs 82%, P < 0.01 and 83% vs 76%, P < 0.04, respectively). The addition of MII to conventional pH monitoring significantly increases the diagnostic yield of symptom association analysis in revealing an association between atypical symptoms and refluxes irrespective of age, whereas in studying typical symptoms it was true only for infants. CONCLUSIONS: Addition of MII to conventional pH monitoring significantly increases the diagnostic yield in detecting REs, prevalently in infants, and in revealing an association between refluxes and symptoms, prevalently respiratory ones and in infants group.