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Objective To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV) and the 12-month outcomes in children with AAV. Methods Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis. The primary outcome measure was achievement of disease remission (Pediatric Vasculitis Activity Score [PVAS] of 0) at 12 months with a corticosteroid dosage of

Objective To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). Methods The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e-entry) cohort, with the data censored to November 2015. The EMA algorithm was used to uniquely distinguish children with MPA from children with GPA, whose diagnoses had been classified according to both adult- and pediatric-specific criteria. Descriptive statistics were used for comparisons. Results In total, 231 of 440 patients (64% female) fulfilled the classification criteria for either MPA (n = 48) or GPA (n = 183). The median time to diagnosis was 1.6 months in the MPA group and 2.1 months in the GPA group (ranging to 39 and 73 months, respectively). Patients with MPA were significantly younger than those with GPA (median age 11 years versus 14 years). Constitutional features were equally common between the groups. In patients with MPA compared to those with GPA, pulmonary manifestations were less frequent (44% versus 74%) and less severe (primarily, hemorrhage, requirement for supplemental oxygen, and pulmonary failure). Renal pathologic features were frequently found in both groups (75% of patients with MPA versus 83% of patients with GPA) but tended toward greater severity in those with MPA (primarily, nephrotic-range proteinuria, requirement for dialysis, and end-stage renal disease). Airway/eye involvement was absent among patients with MPA, because these GPA-defining features preclude a diagnosis of MPA within the EMA algorithm. Similar proportions of patients with MPA and those with GPA received combination therapy with corticosteroids plus cyclophosphamide (69% and 78%, respectively) or both drugs in combination with plasmapheresis (19% and 22%, respectively). Other treatments administered, ranging in decreasing frequency from 13% to 3%, were rituximab, methotrexate, azathioprine, and mycophenolate mofetil. Conclusion Younger age at disease onset and, perhaps, both gastrointestinal manifestations and more severe kidney disease seem to characterize the clinical profile in children with MPA compared to those with GPA. Delay in diagnosis suggests that recognition of these systemic vasculitides is suboptimal. Compared with adults, initial treatment regimens in children were comparable, but the complete reversal of female-to-male disease prevalence ratios is a provocative finding.

Objective To quantify the impact of inflammatory brain diseases in the pediatric population on health-related quality of life, including the subdomains of physical, emotional, school and social functioning. Methods This was a multicenter, observational cohort study of children (

Background Sepsis and severe focal infections represent a substantial disease burden in children admitted to hospital. We aimed to understand the burden of disease and outcomes in children with life-threatening bacterial infections in Europe. Methods The European Union Childhood Life-threatening Infectious Disease Study (EUCLIDS) was a prospective, multicentre, cohort study done in six countries in Europe. Patients aged 1 month to 18 years with sepsis (or suspected sepsis) or severe focal infections, admitted to 98 participating hospitals in the UK, Austria, Germany, Lithuania, Spain, and the Netherlands were prospectively recruited between July 1, 2012, and Dec 31, 2015. To assess disease burden and outcomes, we collected demographic and clinical data using a secured web-based platform and obtained microbiological data using locally available clinical diagnostic procedures. Findings 2844 patients were recruited and included in the analysis. 1512 (53·2%) of 2841 patients were male and median age was 39·1 months (IQR 12·4–93·9). 1229 (43·2%) patients had sepsis and 1615 (56·8%) had severe focal infections. Patients diagnosed with sepsis had a median age of 27·6 months (IQR 9·0–80·2), whereas those diagnosed with severe focal infections had a median age of 46·5 months (15·8–100·4; pInterpretation Mortality in children admitted to hospital for sepsis or severe focal infections is low in Europe. The disease burden is mainly in children younger than 5 years and is largely due to vaccine-preventable meningococcal and pneumococcal infections. Despite the availability and application of clinical procedures for microbiological diagnosis, the causative organism remained unidentified in approximately 50% of patients.

An experimental study has been conducted to evaluate the feasibility of a current mode gamma radiation dosimeter, consisting of a commercial PIN photodiode as a radiation sensor, and a custom made auto-ranging electrometer for real-time measurement of the PIN photodiode’s response under radiation exposure. The radiation induced direct current response for single PIN photodiodes with different active areas, as well as for multiple PIN photodiodes connected in parallel, has been investigated. Three types of commercial silicon PIN photodiodes have been chosen for evaluation - S1223, BPW34, and PS100-6-CER2 PIN. During the experiment, five samples have been tested - three samples made of single PIN photodiodes (one sample of each photodiode type) and two samples formed by connecting multiple photodiodes in parallel (two BPW34 photodiodes in parallel and four BPW34 photodiodes in parallel). The samples have been irradiated with a 60Co gamma ray source and the relations between the induced photocurrent and the dose rate, and between the accumulated charge and the absorbed dose, have been determined. For measuring the photodiodes response, a custom made auto-ranging electrometer controlled by a personal computer, and capable of measuring direct currents from 50 pA to 10 mA with relative error less than 2.5%, has been used. Obtained results have shown very good linearity between the dose rate and the induced photocurrent for dose rates ranging from 0.93 Gy/h to 67 Gy/h. Also, very good linearity has been observed between the accumulated charge and the absorbed dose for all tested samples, within the investigated range of absorbed doses from 472 mGy to 3.3 Gy. On the basis of the obtained results, a simple model has been derived, enabling the estimation of the photodiode’s current response as a function of the dose rate and the photodiode’s geometry (active area and depletion layer width). [Projekat Ministarstva nauke Republike Srbije, br. 43011]

We report on a 4-year-old boy admitted to our Institute because of fever (39°C) persisting for 8 days. Physical examination showed bilateral conjunctivitis, strawberry tongue, lip fissures, enlarged cervical lymph nodes, and polymorphous rash, followed by finger and toe desquamation (see Figure I in the online-only Data Supplement). Diagnosis of Kawasaki disease was made. According to the Kobayashi risk score, our patient had 8 points, predicting treatment unresponsiveness of 71.4%.1 Over the next 2 weeks, the symptoms of acute phase persisted despite treatment with intravenous immunoglobulin, as well as retreatment with intravenous immunoglobulin and methyl-prednisolone boluses. In week 4 of the disease, he was given infliximab in a single dose of 5 mg/kg. He became afebrile, and his inflammatory parameters normalized after treatment with infliximab. An initial echocardiography examination performed immediately after the admission showed no specific changes. The second echocardiographic …

We report on pediatric patient with clinical and laboratory evidence of pancreatitis at onset of atypical Kawasaki disease (KD). In KD pancreatic inflammation was described previously, but clinical pancreatitis was rarely reported and its true incidence is unknown. In febrile pediatric patients suspected to have KD, but not fulfilling classical diagnostic criteria, signs of pancreatic inflammation may help in making correct diagnosis. Further analysis of cases with atypical KD developing pancreatitis may reveal if signs of pancreatic inflammation can be used as supportive diagnostic finding.