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An abstract of the conference paper "Therapeutic approaches for the treatment of new onset and flared juvenile systemic lupus erythematosus with active renal disease: an international multicenter PRINTO study," by S. Pasic, and colleagues, is presented.

An abstract of the conference paper "JIA affected sibling pairs present high correlation for ANA and ILAR category," by T. Herlin, and colleagues, is presented.

Background: Acute pain is a common symptom in children of all ages, and is associated with a variety of conditions. Despite the availability of guidelines, pain often remains underestimated and undertreated. Paracetamol and ibuprofen are the most commonly used drugs for analgesia in Pediatrics. Multimodal pain management by using a combination of paracetamol and ibuprofen results in greater analgesia., Methods: An investigation using the Nominal Group Technique was carried out between May and August 2022. Two open (non-anonymous) questionnaires were consecutively sent to a Board of ten clinicians to understand their opinions on the use of the oral paracetamol and ibuprofen association. Answers were examined in a final meeting where conclusions were drawn., Results: The board achieved a final consensus on a better analgesic power of paracetamol and ibuprofen in fixed-dose combination as compared to monotherapy, without compromising safety. Strong consensus was reached on the opinion that the fixed-dose combination of paracetamol and ibuprofen may be a useful option in case of inefficacy of one or other drug as monotherapy, especially in case of headaches, odontalgia, earache, and musculoskeletal pain. The use of the fixed combination may be also considered suitable for postoperative pain management., Conclusions: The use of the fixed-dose combination may represent advantage in terms of efficacy and safety, allowing a better control of the dose of both paracetamol and ibuprofen as monotherapy, thus minimizing the risk of incorrect dosage. However, the limited evidence available highlights the need for future well designed studies to better define the advantages of this formulation in the various therapeutic areas., (© 2023. The Author(s).)

Abstract: School Active Breaks are short bouts of physical activity (5-15 minutes) conducted by appropriately trained teachers and delivered during or between curricular lessons. They are a good strategy to counteract sedentary behaviors, and a growing body of evidence shows that they can represent also a tool to promote and improve health, school wellbeing and academic achievements. On 19 February 2022, the Working Group on Movement Sciences for Health of the Italian Society of Hygiene, Preventive Medicine and Public Health organized an Awareness Day on the effectiveness, usefulness and feasibility of School Active Breaks, opened to teachers, educators, school leaders, pediatricians, personnel from Departments of Prevention and Public Health and Health Policy-makers. During the event, the testimonies about the experiences already carried out in Italy showed that School Active Breaks are an effective intervention that each school can easily include in its educational offer and apply in any context.

Background: COVID-19 was declared a pandemic on 11 March 2020 and unprecedented containment measures were taken to limit its spreading. These exceptional measures may have an impact on the mental health of the population., Objectives: We hypothesize that children with the rheumatological chronic disease might be a population at higher risk of psychological distress., Design: This study evaluated the psychological effect of social distancing in pediatric rheumatological patients and its possible correlation with disease relapse., Setting: Pediatric patients diagnosed with juvenile idiopathic arthritis (JIA) were included in the study., Subjects and Methods: They completed the Multidimensional Anxiety Scale for Children (MASC-2) to assess anxious symptoms, the Children Depression Inventory Self Report (CDI-2 SR) for depression symptoms and a semi-structured questionnaire to collect their activities. Their parents completed the Children Depression Inventory 2 Parent (CDI-2 P)., Results: The MASC-2 test showed high values in anxiety factors. The CDI-2 SR test showed high values for Ineffectiveness and Functional Problems. The CDI-2 P showed high values for Emotional Problems factor. All the patients with high level of depression were females. The result is that those who take therapy were younger, have reduced values in Functional Problems and in Social Anxiety. 47% of the sample had disease relapse., Conclusions: During the COVID-19 period of isolation, in patients with JIA, there was an increase in anxious and depressive symptoms in female adolescents, a tendency to disease relapse, despite generally good compliance to therapy. Those under treatment were younger and showed reduced values in Functional Problems, Social Anxiety, and Humiliation/Rejection., Limitations: Limited number of patients to whom questionnaires could be distributed.

The overwhelming success of tyrosine kinase inhibitor (TKI) therapy in chronic myeloid leukemia (CML) patients has opened a discussion among medical practitioners and the lay public on the real possibility of pregnancy and conception in females and males with CML. In the past 10 years this subject has acquired growing interest in the scientific community and specific knowledge has been obtained "from bench to bedside". Embryological, pharmacological, and pathophysiological studies have merged with worldwide patient databases to provide a roadmap to a successful pregnancy and birth in CML patients. Male conception does not seem to be affected by TKI therapy, since this class of drugs is neither genotoxic nor mutagenic, however, caution should be used specially with newer drugs for which little or no data are available. In contrast, female patients should avoid TKI therapy specifically during the embryonic stage of organogenesis (5-12 weeks) because TKIs can be teratogenic. In the last 15 years, 41 pregnancies have been followed in our center. A total of 11 male conceptions and 30 female pregnancies are described. TKI treatment was generally terminated as soon as the pregnancy was discovered (3-5 weeks), to avoid exposure during embryonic period and to reduce the risk of needing treatment in the first trimester. Eleven pregnancies were treated with interferon, imatinib or nilotinib during gestation. Nilotinib plasma levels in cord blood and maternal blood at delivery were studied in 2 patients and reduced or absent placental crossing of nilotinib was observed. All of the patients were managed by a multidisciplinary team of physicians with obligatory hematological and obgyn consultations. This work provides an update on the state of the art and detailed description of pregnancy management and outcomes in CML patients.

Background: SARS-CoV2 infection may present at onset with cutaneous manifestations as chilblains, pernio-like lesions characterized by rapid onset, itching, pain and tenderness and quick improvement with re-warming as similarly observed in primary acrocyanosis. The purpose of the present study was to detect in a single institution series of pediatric patients, during COVID-19 pandemic, the prevalence of acrocyanosis compared to previous period and an eventual correlation with SARS-Cov2 (Severe Acute Respiratory Syndrome Coronavirus 2) infection or other secondary etiologies of this disorder., Methods: We retrospectively analyzed the prevalence of pediatric patients with acrocyanosis between January 2020 and July 2021, compared to the same period of previous year. All patients were investigated with capillaroscopies, clinical and laboratory texts. Those patients referred to our Institution, during the Covid-19 pandemic were also examined for SARS-CoV2 serologies to find out an eventual specific correlation with this secondary potential etiology., Results: During the first wave of Covid-19 pandemic we observed an increased prevalence of this manifestation. The analysis showed that the higher prevalence of patients with acrocyanosis, in this period, was not related to SARS-Cov2 infection., Conclusions: The acrocyanosis in pediatric patients is rarely associated with rheumatological diseases, being more frequently a primary disorder and strictly related to a sedentary lifestyle.

In Italy, during the second epidemic wave of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), rapid antigenic (Ag) test at point-of-care (POCT) station were employed to quickly evaluate large numbers of swabs. We collected data of all children who underwent the Ag test in our hospital. All positive patients were recalled to perform reverse transcription polymerase chain reaction. A total of 2133 tests were collected over 1 month. Clinical data of 1941 children (median age = 3.7 years) were analyzed: 1343 (69.2%) patients complained of symptoms, 594 (30.6%) had a history of close contact with SARS-CoV-2-positive individuals. Among symptoms reported, acute rhinitis was the most frequent (67.9%), followed by cough (42.6%) and fever (31.5%). Among all tests, 95.8% resulted negative, 4.2% positive: 37/89 were confirmed. In confirmed cases, fever (56.2% vs 32.2%; P = .041) and gastrointestinal symptoms (18.8% vs 6.25%; P = .041) were significantly more frequent compared with negative children. The use of POCT for Ag test seems appropriate for SARS-CoV-2 screening in the pediatric population. In children, fever and gastrointestinal symptoms may constitute red flags of SARS-CoV-2.

Aim of these revised recommendations for the general management of Kawasaki disease is to encourage its prompter recognition and warrant the most appropriate therapy, based on ascertained scientific data, raising awareness of the complications related to misdiagnosis or delayed treatment. A set of 20 synthetic operative statements is herein provided, including the definition of Kawasaki disease, its protean presentations, clinical course and seminal treatment modalities of all disease phases. The application of these recommendations should improve prognosis of Kawasaki disease and prevent the progression to permanent vascular abnormalities, thereby diminishing morbidity and mortality.

Background: Although epidemic, novel coronavirus (SARS-CoV-2) infection rarely affects the pediatric population. However, in the last months, an increasing number of Italian pediatricians have reported the occurrence of erythema pernio-like in children following a flu-like syndrome, after the outbreak of SARS-CoV-2 infection., Objective: The study aims to report cutaneous manifestation of SARS-CoV-2 infection in 4 adolescents., Methods: Observational study reporting 4 adolescents with skin lesions, 2 weeks after the occurrence of a flu-like syndrome., Results: Fourteen days after a flu-like syndrome, adolescents exhibited skin lesions to toes and feet. These lesions were rounded, with blurred limits, with a 5-15 mm diameter, red-violaceous- bluish colored, sometimes resulting in bullous lesions in correspondence of the central or apical portion, which tended to be covered with blackish crusts after evolution. Lesions were also accompanied by pain of variable intensity and evolved within two or three weeks to self-resolution without particular sequelae. In two patients, pharyngeal and nasal swabs were negative for SARSCoV- 2 infection, at the presentation of skin lesions. After 2 months from clinical manifestations, patients were tested positive for SARS-CoV-2 antibodies., Conclusions: In the pediatric and adolescent population, the occurrence of an erythema pernio-like after a flu-like syndrome could be associated with SARS-CoV-2 infection., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Severe Acute Respiratory Syndrome - Coronavirus - 2 (SARS-CoV-2) and its related Coronavirus Disease - 19 (COVID-19) has become a health emergency worldwide. The medical community has been concerned since the beginning of the outbreak about the potential impact of COVID-19 in children, especially in those with underlying chronic diseases. Fortunately, COVID-19 has been reported to be less severe in children than in adults. However, epidemiologic and clinical data are scarce. Children show unique features of SARS-CoV-2 involvement that may account for the low rate of infection and death in this age group. The purpose of this review is to summarize the most relevant evidence of COVID-19 in children highlighting similarities and differences with adults., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Objectives: To present the results of a Delphi consensus survey among Italian paediatric and adult rheumatologists on transitional care (TC) of young people (YP) with juvenile idiopathic arthritis (JIA)., Methods: A taskforce of 27 paediatric and adult rheumatologists evaluated the applicability of the 2016 EULAR/PReS recommendations for TC to the Italian rheumatology practice and healthcare system and formulated additional country-specific statements aimed to increase their suitability. After a two-round discussion, applicability of EULAR/PReS recommendations and agreement with newly-proposed statements were voted on a 0-10 scale (where 0 = no applicability/agreement and 10 = total applicability/agreement). A mean level of agreement ≥8 was deemed acceptable., Results: The consensus threshold was reached for only 4 of the 12 EULAR/PReS recommendations and for 25 of the 27 country-specific statements. Poor agreement with EULAR/PReS recommendations was mostly explained by paucity of centres in Italy that possess both paediatric and adult rheumatologists, disagreement about optimal time of transition start and de nition of transition coordinator, diversity between paediatric and adult clinimetric assessments, and lack of administrative and financial support., Conclusions: This consensus initiative represents an important step forward toward the establishment of a nationwide TC network for YP with JIA in Italy. The main goals established for the future are the identification of adult rheumatology centres that are willing to participate in the TC process, the education of adult rheumatology teams on childhood-onset rheumatic diseases and transition issues, and the increased awareness of public healthcare authorities and other stakeholders about the importance of good-quality TC.

Acute mastoiditis (AM) is the most common complication of acute otitis media (AOM) and is one of the most severe acute bacterial diseases in infants and children. In some geographic areas, the incidence of AM is increasing, and the causative role of some bacterial pathogens could be greater than previously thought. In this paper, the results of a study that evaluated the epidemiology and microbial etiology of paediatric AM in Umbria, which is a region of central Italy, are reported. This is a retrospective study of patients aged 0-14 years with AM admitted to the pediatric wards of the hospitals of Umbria, Italy, between June 1 and September 30 in four consecutive years (2015-2018). A total of 108 children were enrolled. The prevalence of AM in males during the four years of analysis was significantly higher than that in females at 63% (95% confidence intervals [CI]: 0.54-0.72). The most frequently affected age groups were 5-9 years (45.4%) and 10-14 years (31.5%), with statistically significant differences in comparison with children aged <1 year (5.6%, 95% CI: 0.01-0.10) and 1-4 years (17.6%, 95% CI: 0.10-0.25). In most cases (64, 59.3%), AM was associated with spontaneous tympanic membrane perforation (STP). The culture of the middle ear fluid revealed the presence of Pseudomonas aeruginosa in 56 cases (51.6%). The mean incidence rates of pediatric AM in Umbria during the study increased significantly with time, as it was 18.18/100,000 children/year in 2015-2016 and 29.24/100,000 children/year in 2017-2018 (CI difference: +2.5 - +19.9, p < 0.05). The incidence rates of Pseudomonas aeruginosa detection in pediatric AM associated with STP significantly increased with time. The incidence was 6.06/100,000 children/year in 2015-2016 and 18.61/100,000 children/year in 2017-2018 (CI difference: +6.1 - +19.0, p < 0.001). This study demonstrated the high and increasing incidence of AM in the Umbria region during the summer months and the frequent detection of P. aeruginosa as an etiologic agent of the disease in the presence of STP. Confirmation of these results with a larger study population, in different settings, and throughout the whole year is needed to define the first-line approach of AM with STP in pediatrics.

This second part of practical Guidelines related to Kawasaki disease (KD) has the goal of contributing to prompt diagnosis and most appropriate treatment of KD resistant forms and cardiovascular complications, including non-pharmacologic treatments, follow-up, lifestyle and prevention of cardiovascular risks in the long-term through a set of 17 recommendations.Guidelines, however, should not be considered a norm that limits the treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient's condition, and disease severity or individual complications.

The primary purpose of these practical guidelines related to Kawasaki disease (KD) is to contribute to prompt diagnosis and appropriate treatment on the basis of different specialists' contributions in the field. A set of 40 recommendations is provided, divided in two parts: the first describes the definition of KD, its epidemiology, etiopathogenetic hints, presentation, clinical course and general management, including treatment of the acute phase, through specific 23 recommendations.Their application is aimed at improving the rate of treatment with intravenous immunoglobulin and the overall potential development of coronary artery abnormalities in KD. Guidelines, however, should not be considered a norm that limits treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient's condition, and disease severity or complications.

The family name of author Francesco La Torre was incorrect in the published article. The correct family name should read as La Torre F.

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Italian language.The reading comprehension of the questionnaire was tested in 10 JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents.The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the 3 Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, test-retest reliability, and construct validity (convergent and discriminant validity).A total of 1296 JIA patients (7.2% systemic, 59.5% oligoarticular, 21.4% RF negative polyarthritis, 11.9% other categories) and 100 healthy children, were enrolled in 18 centres. The JAMAR components discriminated well healthy subjects from JIA patients except for the Health Related Quality of Life (HRQoL) Psychosocial Health (PsH) subscales. All JAMAR components revealed good psychometric performances.In conclusion, the Italian version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Background: Data from routine clinical practice are needed to further define the efficacy and safety of biologic medications in children with juvenile idiopathic arthritis (JIA). The aim of this analysis was to investigate the disease status, reasons for discontinuation and adverse events in Italian JIA patients treated with etanercept (ETN)., Methods: In 2013, all centers of the Italian Pediatric Rheumatology Study Group were asked to make a census of patients given ETN after January 2000. Patients were classified in three groups: group 1 = patients still taking ETN; group 2 = patients discontinued from ETN for any reasons; group 3 = patients lost to follow-up while receiving ETN. All three groups received a retrospective assessment; patients in group 1 also underwent a cross-sectional assessment., Results: 1038 patients were enrolled by 23 centers: 422 (40.7%) were in group 1, 462 (44.5%) in group 2, and 154 (14.8%) in group 3. Median duration of ETN therapy was 2.5 years. At cross-sectional assessment, 41.8% to 48.6% of patients in group 1 met formal criteria for inactive disease, whereas 52.4% of patients in group 2 and 55.8% of patients in group 3 were judged in clinical remission by their caring physician at last visit. A relatively greater proportion of patients with systemic arthritis were discontinued or lost to follow-up. Parent evaluations at cross-sectional visit in group 1 showed that 52.4% of patients had normal physical function, very few had impairment in quality of life, 51.2% had no pain, 76% had no morning stiffness, and 82.7% of parents were satisfied with their child's illness outcome. Clinically significant adverse events were reported for 27.8% of patients and ETN was discontinued for side effects in 9.5%. The most common adverse events were new onset or recurrent uveitis (10.2%), infections (6.6%), injection site reactions (4.4%), and neuropsychiatric (3.1%), gastrointestinal (2.4%), and hematological disorders (2.1%). Ten patients developed an inflammatory bowel disease and 2 had a malignancy. One patient died of a fulminant streptococcal sepsis., Conclusions: Around half of the patients achieved complete disease quiescence under treatment with ETN. The medication was overall well tolerated, as only one quarter of patients experienced clinically significant adverse events and less than 10% had treatment discontinued for toxicity.

Objectives: Polyarteritis nodosa (PAN) is a rare vasculitis in childhood and poor information is known about its long-term outcome. Our aim was to describe the clinical features, at onset and during the disease course, of childhood-onset PAN and identify a potential correlation with persistent organ damage and worse outcome in a cohort of paediatric patients with a confirmed diagnosis of PAN., Methods: A retrospective collection of demographic and clinical data of 52 Caucasian children diagnosed with PAN, fulfilling the EULAR/PRES diagnostic criteria, recruited from eight paediatric rheumatologic centres and one transition unit, was performed. A statistical correlation was made between clinical involvement at onset or during the overall disease course and patients' final outcome., Results: Data from 52 patients (31 males, 21 females) were collected: their mean age at onset was 7.9 years (median 6.3) and mean follow-up period was 6.2 years (median 5.4). At the last follow-up visit, 27 patients (51.9%) were off therapy in clinical remission, 17 (32.7%) were in clinical remission while on medication, and 6 (11.6%) had a persistent or relapsing disease course. Two patients (3.8%) deceased because of severe cerebral involvement. Cranial nerve palsy during the disease course was significantly correlated with a worse prognosis (p=0.011). The presence of nephrogenic hypertension at onset and seizures during the disease course were significantly associated with the development of irreversible organ damage (p= 0.040 and 0.011, respectively)., Conclusions: Childhood PAN is a severe disease with substantial risk of long-term morbidities. In our cohort of patients the worst outcome was significantly correlated with renal and neurological involvement.

Polyarteritis or panarteritis nodosa (PAN) is a necrotizing, focal segmental vasculitis that affects predominantly medium-sized arteries in many different organ systems. It is extremely rare in childhood. Involvement of the oral mucosa at diagnosis is uncommon in PAN. Here, we report a case of a pediatric patient with tongue necrosis.