Familial Mediterranean Fever, a monogenic autoinflammatory disease secondary to MEFV gene mutations in the chromosome 16p13, is characterized by recurrent self-limiting attacks of fever, arthritis, ...aphthous changes in lips and/or oral mucosa, erythema, serositis. It is caused by dysregulation of the inflammasome, a complex intracellular multiprotein structure, commanding the overproduction of interleukin 1. Familial Mediterranean Fever can be associated with other multifactorial autoinflammatory diseases, as vasculitis and Behçet disease.Symptoms frequently start before 20 years of age and are characterized by a more severe phenotype in patients who begin earlier.Attacks consist of fever, serositis, arthritis and high levels of inflammatory reactants: C-reactive protein, erythrocyte sedimentation rate, serum amyloid A associated with leucocytosis and neutrophilia. The symptom-free intervals are of different length.The attacks of Familial Mediterranean Fever can have a trigger, as infections, stress, menses, exposure to cold, fat-rich food, drugs.The diagnosis needs a clinical definition of the disease and a genetic confirmation. An accurate differential diagnosis is mandatory to exclude infective agents, autoimmune diseases, etc.In many patients there is no genetic confirmation of the disease; furthermore, some subjects with the relieve of MEFV mutations, show a phenotype not in line with the diagnosis of Familial Mediterranean Fever. For these reasons, diagnostic criteria were developed, as Tel Hashomer Hospital criteria, the "Turkish FMF Paediatric criteria", the "clinical classification criteria for autoinflammatory periodic fevers" formulated by PRINTO.The goals of the treatment are: prevention of attacks recurrence, normalization of inflammatory markers, control of subclinical inflammation in attacks-free intervals and prevention of medium and long-term complications, as amyloidosis. Colchicine is the first step in the treatment; biological drugs are effective in non-responder patients.The goal of this paper is to give a wide and broad review to general paediatricians on Familial Mediterranean Fever, with the relative diagnostic, clinical and therapeutic aspects.
This review aims to evaluate the role of Virtual Reality (VR) in cognitive rehabilitation of different neurological diseases, and the accessibility to healthcare systems providing this type of ...treatment.
Studies performed between 2003 and 2017 and fulfilling the selected criteria were found on PubMed, Scopus, Cochrane and Web of Sciences databases. The search combined the terms VR rehabilitation with different neurological disease.
Our findings showed that neurological patients performed significant improvement in many cognitive domains (executive and visual-spatial abilities; speech, attention and memory skills) following the use of VR training.
This review supports the idea that rehabilitation through new VR tools could positively affect neurological patients' outcomes, by boosting motivation and participation so to get a better response to treatment. In particular, VR can be used to enhance the effects of conventional therapies, promoting longer training sessions and a reduction in overall hospitalization time.
The neural, the endocrine, and the immune systems are studied as distinct districts in physiological and pathological settings. However, these systems must be investigated with an integrative ...approach, while also considering that therapeutic agents, such as glucocorticoids, can induce a reversible or irreversible change of this homeostasis. Children and adolescents affected by rheumatic diseases frequently need treatment with corticosteroids, and the treatment must sometimes be continued for a long time. In the biological era, the treat-to-target strategy allowed a real revolution in treatment, with significant steroid dose sparing or, in many patients, steroid treatment withdrawal. In this review, the impact of glucocorticoids on endocrine, immune, and neurologic targets is analyzed, and the crosstalk between these systems is highlighted. In this narrative review, we explore the reasoning as to why glucocorticoids can disrupt this homeostasis, we summarize some of the key results supporting the impact of glucocorticoids treatment on endocrine, immune, and neurologic systems, and we discuss the data reported in the international literature.
To identify the clinical characteristics, reasons for use and response to treatment with anakinra in a series of patients with Kawasaki Disease (KD).
A retrospective chart review of patients treated ...with anakinra for KD diagnosed according to the AHA criteria. We compared clinical, biological and echocardiographic characteristics of KD before and after anakinra use. We analysed reasons for use of anakinra, and compared treatment regimens used in 7 European KD referral centres.
Eight boys and 3 girls with treatment-refractory KD, aged 4 months to 9 years old, received at least 2 different KD treatments prior to anakinra, which was given on mean at 25 days after disease onset (8 to 87 days). The main reasons for use of anakinra were clinical and biological inflammation, progression of coronary dilatations, and severe myocarditis with cardiac failure. Doses of anakinra ranged from 2 to 8 mg/kg and duration varied from 6 to 81 days. Efficacy of anakinra was judged in terms of fever resolution (100%), decrease of CRP (100%), and in terms of its effect on coronary artery dilatation Z scores, which decreased in 10/11 patients and increased in one who died suddenly of pericardial hemorrhage.
Anakinra used late in the disease course led to a rapid and sustained improvement in clinical and biological inflammation. Our retrospective analysis did show neither a striking nor a rapid decrease of coronary dilatations and we cannot determine if anakinra itself had an effect on coronary artery dimensions.
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.
This study aimed to examine the effect of cognitive priming linked to the COVID-19 pandemic, through state anxiety and personal need for structure, on teachers’ tendency toward sustainability and ...teachers’ tendency toward a conservative socio-economic vision. We involved a sample of 984 Italian teachers, and by manipulating the saliency of the COVID-19 pandemic, we found that the saliency of the COVID-19 pandemic positively impacted state anxiety and that state anxiety impacted teachers’ tendency toward sustainability both directly and indirectly through the mediational role of the personal need for structure. Finally, we found that state anxiety only indirectly through the personal need for structure impacted teachers’ tendency toward a conservative socio-economic vision.
There is mounting evidence on the existence of a Pediatric Inflammatory Multisystem Syndrome-temporally associated to SARS-CoV-2 infection (PIMS-TS), sharing similarities with Kawasaki Disease (KD). ...The main outcome of the study were to better characterize the clinical features and the treatment response of PIMS-TS and to explore its relationship with KD determining whether KD and PIMS are two distinct entities.
The Rheumatology Study Group of the Italian Pediatric Society launched a survey to enroll patients diagnosed with KD (Kawasaki Disease Group - KDG) or KD-like (Kawacovid Group - KCG) disease between February 1st 2020, and May 31st 2020. Demographic, clinical, laboratory data, treatment information, and patients' outcome were collected in an online anonymized database (RedCAP®). Relationship between clinical presentation and SARS-CoV-2 infection was also taken into account. Moreover, clinical characteristics of KDG during SARS-CoV-2 epidemic (KDG-CoV2) were compared to Kawasaki Disease patients (KDG-Historical) seen in three different Italian tertiary pediatric hospitals (Institute for Maternal and Child Health, IRCCS "Burlo Garofolo", Trieste; AOU Meyer, Florence; IRCCS Istituto Giannina Gaslini, Genoa) from January 1st 2000 to December 31st 2019. Chi square test or exact Fisher test and non-parametric Wilcoxon Mann-Whitney test were used to study differences between two groups.
One-hundred-forty-nine cases were enrolled, (96 KDG and 53 KCG). KCG children were significantly older and presented more frequently from gastrointestinal and respiratory involvement. Cardiac involvement was more common in KCG, with 60,4% of patients with myocarditis. 37,8% of patients among KCG presented hypotension/non-cardiogenic shock. Coronary artery abnormalities (CAA) were more common in the KDG. The risk of ICU admission were higher in KCG. Lymphopenia, higher CRP levels, elevated ferritin and troponin-T characterized KCG. KDG received more frequently immunoglobulins (IVIG) and acetylsalicylic acid (ASA) (81,3% vs 66%; p = 0.04 and 71,9% vs 43,4%; p = 0.001 respectively) as KCG more often received glucocorticoids (56,6% vs 14,6%; p < 0.0001). SARS-CoV-2 assay more often resulted positive in KCG than in KDG (75,5% vs 20%; p < 0.0001). Short-term follow data showed minor complications. Comparing KDG with a KD-Historical Italian cohort (598 patients), no statistical difference was found in terms of clinical manifestations and laboratory data.
Our study suggests that SARS-CoV-2 infection might determine two distinct inflammatory diseases in children: KD and PIMS-TS. Older age at onset and clinical peculiarities like the occurrence of myocarditis characterize this multi-inflammatory syndrome. Our patients had an optimal response to treatments and a good outcome, with few complications and no deaths.
A 9-year-old boy with petechiae on the legs and abdominal pain was unsuccessfully treated with steroids. He was admitted to our hospital for the onset of fever, ecchymosis, and arthralgia. Skin ...lesions suggested vasculitis, but they were not typical of Henoch–Schönlein purpura. He showed ecchymosis of the scrotal bursa, diffusion of petechiae to the trunk and arms, vomiting, severe abdominal pain, oliguria with hyponatremia, hypoalbuminemia, low C3 levels, high levels of creatinine, blood urea nitrogen, and tubular enzymes, proteinuria, and glycosuria. The urinary sediment showed macrohaematuria, and hyaline and cellular casts. Ultrasound showed polyserositis. He was treated with intravenous furosemide, albumin, and methylprednisolone. He underwent colonoscopy and gastroscopy because of development of acute pancreatitis and severe anaemia. Typical lesions of Henoch–Schönlein purpura were observed in the small intestine and colon mucosa. He received three high doses of methylprednisolone, followed by intravenous cyclophosphamide. A dramatic and persistent response was observed after these doses. A single high dose of cyclophosphamide is appropriate in Henoch–Schönlein purpura with acute renal failure and severe pancreatitis that are non-responsive to high-dose steroids.
•Stroke represents the leading cause of disability in the industrialized world.•Post-stroke impairment interferes with the QoL of the patient and caregiver.•Robot-assisted hand training allow to ...perform practical tasks with a VR setting.•Hand robotic plus VR-based training may amplify the functional outcome achievement.
Robot-assisted hand training adopting end-effector devices results in an additional reduction of motor impairment in comparison to usual care alone in different stages of stroke recovery. These devices often allow the patient to perform practical, attentive, and visual-spatial tasks in a semi-virtual reality (VR) setting. We aimed to investigate whether the hand end-effector robotic device AmadeoTM could improve cognitive performance, beyond the motor deficit, as compared to the same amount of occupational treatment focused on the hand. Forty-eight patients (aged 54.3 ± 10.5 years, 62.5% female) affected by either ischemic or hemorrhagic stroke in the chronic phase were enrolled in the study. The experimental group (EG) underwent AmadeoTM robotic training, while the control group (CG) performed occupational therapy involving the upper limb. Patients were assessed at the beginning and at the end of the rehabilitation protocol using a specific neuropsychological battery, as well as motor function tests. The EG showed greater improvements in different cognitive domains, including attentive abilities and executive functions, as well as in hand motor function, as compared to CG. Our study showed that task-oriented VR-based robotic rehabilitation enhanced not only motor function in the paretic arm but also global and specific cognitive abilities in post-stroke patients. We may argue that the hand robotic plus VR-based training may provide patients with an integration of cognitive and motor skill rehabilitation, thus amplifying the functional outcome achievement.
Based on the life design paradigm and career construction adaptation model and on recent directions from the perspective of sustainable and inclusive career guidance, the study aimed at examining the ...relationship between career adaptability, the tendency to consider systemic challenges to attain sustainable development, and state personal and social hope and their role on the tendency to invest in higher education. The analyses carried out involving 416 Italian high school students found that career adaptability and the tendency to consider systemic challenges in order to attain sustainable development were directly and indirectly, through state personal and social hope, related to the tendency to invest in higher education. The results obtained allowed to provide new contributions to extend results previously described by the life design approach in career development issues and provided useful suggestions for preventive career interventions.
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