Development and Preliminary Validation of an Electromyography-Scoring Protocol for the Assessment and Grading of Muscle Involvement in Patients With Juvenile Idiopathic Inflammatory Myopathies.

Abstract Introduction We performed a pilot study in order to investigate the feasibility of an electromyography (EMG)-scoring protocol for the assessment of disease activity in juvenile idiopathic inflammatory myopathies (JIIM). Methods Children with JIIM followed up in a tertiary-level care center underwent standardized clinical, laboratory, and EMG assessment. An EMG-scoring protocol was devised by a consensus panel including a pediatric neurophysiologist and two pediatric rheumatologists, based on a combined score obtained as the sum of (1) the presence of denervation signs (fibrillation potentials) and (2) motor unit remodeling (mixed pattern of short- and long-duration motor unit action potentials). The EMG-scoring protocol was then validated following the Outcome Measures in Rheumatoid Arthritis Clinical Trials filter for outcome measures in rheumatology and the consensus-based standards for the selection of health measurement instruments methodology. Results Thirteen children (77% females) were included in the study, with a median age of 10 years (interquartile range: 7-17 years) and median disease duration of 11.8 months (interquartile range: 2.1-44.5). A total of 39 EMG examinations were evaluated. A strong positive association between a standardized tool for muscle strength assessment and the combined score was observed. No significant associations were found with both creatine kinase and erythrocyte sedimentation rate levels. Discussion Our EMG-scoring protocol is the first standardized and reproducible tool for the neurophysiologic evaluation and grading of muscle involvement in patients with JIIM and could provide relevant additional information in the assessment and follow-up of these rare conditions

Lower Limb Rehabilitation in Juvenile Idiopathic Arthritis using Serious Games

Patients undergoing physical rehabilitation therapy must perform series of exercises regularly over a long period of time to improve, or at least not to worsen, their condition. Rehabilitation can easily become boring because of the tedious repetition of simple exercises, which can also cause mild pain and discomfort. As a consequence, patients often fail to follow their rehabilitation schedule with the required regularity, thus endangering their recovery. In the last decade, video games have become largely popular and the availability of advanced input controllers has made them a viable approach to make physical rehabilitation more entertaining while increasing patients motivation. In this paper, we present a framework integrating serious games for the lower-limb rehabilitation of children suffering from Juvenile Idiopathic Arthritis (JIA). The framework comprises games that implement parts of the therapeutic protocol followed by the young patients and provides modules to tune, control, record, and analyze the therapeutic sessions. We present the result of a preliminary validation we performed with patients at the clinic under therapists supervision. The feedback we received has been overall very positive both from patients, who enjoyed performing their usual therapy using video games, and therapists, who liked how the games could keep the children engaged and motivated while performing the usual therapeutic routine

Serious Games for Wrist Rehabilitation in Juvenile Idiopathic Arthritis

Rehabilitation is a painful and tiring process involving series of exercises that patients must repeat over a long period. Unfortunately, patients often grow bored, frustrated, and lose motivation making rehabilitation less effective. In the recent years video games have been widely used to implement rehabilitation protocols so as to make the process more entertaining, engaging and to keep patients motivated. In this paper, we present an integrated framework we developed for the wrist rehabilitation of patients affected by Juvenile Idiopathic Arthritis (JIA) following a therapeutic protocol at the Clinica Pediatrica G. e D. De Marchi. The framework comprises four video games and a set modules that let the therapists tune and control the exercises the games implemented, record all the patients actions, replay and analyze the sessions. We present the result of a preliminary validation we performed with four poliarticular JIA patients at the clinic under the supervision of the therapists. Overall, we received good feedback both from the young patients, who enjoyed performing known rehabilitation exercises using video games, and therapists who were satisfied with the framework and its potentials for engaging and motivating the patients

Corticosteroid Treatment in Sydenham Chorea: A 27-Year Tertiary Referral Center Experience

Objective: The purpose of this study was to investigate the effectiveness of corticosteroid therapy for children suffering from Sydenham chorea (SC). Methods: The design of the study was observational, retrospective and conducted at the single center of the Rheumatology Unit of Policlinic Hospital of Milan, Italy, from May 1995 to May 2022. All data about the patients were collected from medical records. Results: From a total of 59 patients enrolled in the study (44 females and 15 males; median age 9.3 years, range 7.4–10.6 years), 49 were eligible for primary outcome analysis (10 patients were excluded due to incomplete data). Overall, 75% of patients received steroid therapy, while the remaining cases were treated with symptomatic drugs, including neuroleptics and antiseizure drugs. We found that the duration of chorea was significantly shorter in patients treated with corticosteroids in comparison to those receiving symptomatic treatment (median time: 31 vs. 41 days, p = 0.023). Additionally, patients with arthritis at the onset of the disease had a longer duration of chorea than those without arthritis (median time 90.5 vs. 39 days, p = 0.02). We also found that chorea recurred in 12% of the patients and seemed to be linked to a younger age at onset (p = 0.01). Conclusions: The study suggests that corticosteroid therapy can lead to a faster resolution of SC when compared to neuroleptics and antiseizure drugs treatment

Paediatric acute rheumatic fever in developed countries: Neglected or negligible disease? Results from an observational study in Lombardy (Italy)

Introduction: Acute Rheumatic Fever (ARF) is a multisystemic disease that results from an autoimmune reaction due to group A streptococcal infection. The disease affects predominantly children aged 5 to 15 years and although its incidence in developed Countries declined since the early 1900s, to date there is a paucity of data that confirm this epidemiological trend. Objective: The study aimed to assess the burden of ARF in term of hospitalization and to describe the characteristics of acute rheumatic fever (ARF) in the paediatric population of Lombardy. Study design: The study was carried out by analyzing hospital discharge records of patients resident of Lombardy and aged 0–17 years old who, from 2014 to 2016, were hospitalized with the diagnosis of ARF. The following variables have been studied: age, sex, municipality of residence, date of diagnosis of each patient, hospital of admission, and presentation of the disease. Results: From 2014 to 2016, 215 patients were found to meet the inclusion criteria and diagnosed as affected from Acute Rheumatic Fever. The rate of hospitalization showed a slightly increasing trend from 3.42 in 2014 to about 5.0 in 2016. Moreover, ARF presented a typical seasonal trend with lower cases in the autumn and a peak of hospitalization in the spring. Conclusion: To date, ARF seems to be a rare but not negligible disease in southern central European countries, and in Lombardy we estimated an annual hospitalization rate of 4.24 cases per 100,000 children. The increasing trend found in our study suggests that the burden of the disease could be reduced by involving multidisciplinary health professionals who, in addition to the paediatrician of free choice, would promote evidence based medicine management of the disease during all its clinical phases

Fever of Unknown Origin in Children: The Challenge of History Taking

A fever of unknown origin (FUO) in children is usually described as a fever of at least 8 days duration with no apparent diagnosis after initial investigations, including taking medical history and preliminary laboratory assessment. Infectious diseases are the most common cause of FUO, followed by rheumatologic and neoplastic conditions. In this report, we present a case of a 15-year-old Caucasian boy with a silent past medical history, who presented at our Pediatric ER department with a three-day history of fever, fatigue, and abdominal pain with diarrhoea. Initial laboratory testing and microbiological work-up were non-significant. At hospital admission, a broad infectious diagnostic work-up was pursued, including serologies and polymerase-chain-reaction (PCR) for CMV, EBV, HAV, Parvovirus, Toxoplasma gondii and Adenovirus, all negative. Given mild splenomegaly and linfadenopathy, systemic Juvenile Idiopathic Arthritis (s-JIA) was suspected, as well as Multi-inflammatory Syndrome in Children (MIS-C), but the patient did not meet their main diagnostic criteria. Malignancy was ruled out by a negative bone marrow fine-needle aspiration cytology and whole-body PET-CT scan. On hospital day 8, Brucella was identified on a new set of blood cultures and a combined antibiotic therapy was started with IV Gentamicin plus per os Doxycycline. The patient’s general conditions rapidly improved, and both fever and diarrhoea resolved. A reassessment of the patient’s medical history before discharge revealed exposure to unpasteurized soft cheese in the weeks prior to the onset of symptoms. This case underlines the importance of taking a complete medical history, as well as a full diagnostic work-up to unveil unusual infectious etiologies behind FUO. After the preliminary negative microbiological tests, a connective tissue disease was ruled out (i.e. lack of cutaneous or articular involvement), as well as malignancy, which led to a closer evaluation for infection and the diagnosis of Brucellosis

Lower Limb Rehabilitation in Juvenile Idiopathic Arthritis using Serious Games

Patients undergoing physical rehabilitation therapy must perform series of exercises regularly over a long period of time to improve, or at least not to worsen, their condition. Rehabilitation can easily become boring because of the tedious repetition of simple exercises, which can also cause mild pain and discomfort. As a consequence, patients often fail to follow their rehabilitation schedule with the required regularity, thus endangering their recovery. In the last decade, video games have become largely popular and the availability of advanced input controllers has made them a viable approach to make physical rehabilitation more entertaining while increasing patients motivation. In this paper, we present a framework integrating serious games for the lower-limb rehabilitation of children suffering from Juvenile Idiopathic Arthritis (JIA). The framework comprises games that implement parts of the therapeutic protocol followed by the young patients and provides modules to tune, control, record, and analyze the therapeutic sessions. We present the result of a preliminary validation we performed with patients at the clinic under therapists supervision. The feedback we received has been overall very positive both from patients, who enjoyed performing their usual therapy using video games, and therapists, who liked how the games could keep the children engaged and motivated while performing the usual therapeutic routine