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essing the reporting quality of physical activity programs in randomized controlled trials for the management of juvenile idiopathic arthritis using three standardized essment tools | Pediatric Rheumatology | Full Text

The reporting quality of physical activity (PA) programs in randomized controlled trials (RCTs) for the management of juvenile idiopathic arthritis (JIA) remains unknown. This study aimed to ess and compare the reporting quality of PA programs in RCTs for the management of JIA using three difference standardized essment tools, and to describe the elements that were similar and different between these tools. A systematic search was conducted for moderate-to high-quality RCTs of PA programs in JIA, published up until January 2019. Two reviewers independently included 10 RCTs and scored the reporting quality of PA programs using the following tools: Consensus on Exercise Reporting Template (CERT) checklist, Consensus on The utic Exercise Training (CONTENT) scale, and Template for Intervention Description and Replication (TIDieR) checklist. Results showed that reporting of PA programs in 10 moderate- to high-quality RCTs for JIA management remains incomplete. The average reporting quality (± standard deviation) for all RCTs combined was moderate for the three standardized essment tools with 70.8 (±14.3)% for the TIDieR checklist, 53.2 (±20.2)% for the CERT checklist, and 70.0 (±18.9)% for the CONTENT scale. Despite some overlap, the three standardized essment tools (TIDieR, CERT, CONTENT) included different elements resulting in different scores. All tools ess elements linked to PA programs (provider, location, timing, personalization and adherence), but the CERT checklist includes other essential elements (e.g., additional resources, motivational strategies, adverse events). The lack of complete reporting of PA programs in RCTs for the management of JIA and the variation in scores and essed elements among standardized essment tools show the need to improve reporting. Using the most comprehensive standardized tool (i.e., the CERT) and providing accessible supplemental information on PA programs may improve the reporting quality of PA programs in RCTs and help reproduce PA programs in research and clinical practice.

essing the reporting quality of physical activity programs in randomized controlled trials for the management of juvenile idiopathic arthritis using three standardized essment tools

Abstract

Background

Recurrent Henoch-Schönlein Purpura with bullous rash and pulmonary nodules | Pediatric Rheumatology | Full Text

Henoch-Schönlein purpura (HSP) is the most common vasculitis of childhood. It has a characteristic rash described as palpable purpura that most frequently affects the distal lower extremities and ocks. HSP rarely presents with bullous rash nor pulmonary nodules. We present a novel case of a 12-years-old female with recurrent pediatric HSP with a combination of the rare manifestations of bullous rash and pulmonary nodules. She initially presented with the bullous rash, chest pain, cough, and abdominal pain. Patient was successfully treated with intravenous pulse corticosteroids followed by a high dose oral corticosteroid taper, with resolution of the bullous rash and pulmonary nodules. The rare manifestations of scarring bullous rash and pulmonary nodules can be presenting features of pediatric HSP, the combination of which has not been previously reported. The treatment of intravenous corticosteroid resolved patient’s abdominal symptoms, rash and pulmonary nodules.

Recurrent Henoch-Schönlein Purpura with bullous rash and pulmonary nodules

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Nephrological disorders and neurological involvement in pediatric primary Sjogren syndrome:a case report and review of literature | Pediatric Rheumatology | Full Text

Sjögren syndrome (SS) is a rare disease in pediatrics, and little attention has been paid to the clinical feature in these patients. To date, there are few cases concern about neurological and nephrological disorders in childhood Sjögren syndrome. We describe a case of Sjögren syndrome in a 12-year-old who developed neurological disorders and interstitial nephritis and review the literature currently available on this topic. A 12-year-old was admitted to our hospital for arthritis and glucosuria. She was required to do labial gland and renal biopsy, because the positive for anti-nuclear antibody and anti-Sjögren syndrome B (anti-SSB) antibody. Then the biopsy was performed revealing the lymphocytic infiltrate in the small area and renal tubular interstitial damage,thus the diagnosis of Sjögren syndrome with tubular interstitial damage was made. Three months later, she presented again with headache, fever, nausea, vomiting and was recovered without drug therapy. Based on the patient’s medical history, laboratory and imaging examination, and treatment, we speculate that the disorders of the nervous system were caused by the Sjögren syndrome. The has stable renal function and no residual nervous system damage in the next 1.5 years, but she underwent low dose prednisone therapy because of persistent renal glucosuria. Nephrological disorders and neurological involvement are rare manifestations of Sjögren syndrome in children, and rarely presented as the initial symptoms. It should be suspected in children presenting with unexplained renal diseases, neurological abnormalities, or unexplained fever. Although there is no guidelines on the diagnosis and treatment of children Sjögren syndrome are currently available, early recognition and the appropriate treatment of renal damage and neurologic involvement would improve prognosis and prevent complications.

Nephrological disorders and neurological involvement in pediatric primary Sjogren syndrome:a case report and review of literature

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The ociation of MEFV gene mutations with the disease risk and severity of systemic juvenile idiopathic arthritis | Pediatric Rheumatology | Full Text

Systemic juvenile idiopathic arthritis (sJIA) has many clinical features overlapping with familial Mediterranean fever (FMF), which is caused by mutations in MEFV gene. And FMF patients were easily misdiagnosed as sJIA in China. So we speculate that MEFV is critical genetic background for sJIA and influences patients’ severity. In this study, we aim to figure out whether MEFV mutations are risk factor for the occurrence of sJIA and to study the ociation of MEFV mutations with disease severity of sJIA patients. The present study includes 57 sJIA children and 2573 healthy controls. Odd ratio with 95% confidence interval based on allelic frequency of MEFV mutations or variants was used to evaluate their contribution to sJIA susceptibility. Meta- ysis was then performed to reach comprehensive conclusion. All included sJIA patients were grouped by presence and number of MEFV mutations. Clinical data and indicators of disease severity were compared among different groups. Multiple linear regression method was used to find out whether the number of MEFV variants is ociated with the severity of sJIA. Kaplan-Meier curves and log rank test were used to estimate the probability of the first relapse. The MEFV mutations of our subjects predominantly existed in exons 2 and 3. No significant difference was found in allelic frequency between sJIA children and healthy controls. Meta- ysis demonstrated that p.M694V/I was a risk factor for sJIA (pooled OR: 7.13, 95% CI: 3.01–16.89). The relative period of activity was significantly lower in the one mutation group than those with more than one mutation (p = 0.0194). However, no relevance was found in multiple linear regression models. The mutation p.M694V/I in MEFV might be a risk factor for sJIA. SJIA patients carrying more than one heterozygous mutation in MEFV tend to be more severe than those containing only one, but studies in other cohort of patients need to be performed to validate it.

The ociation of MEFV gene mutations with the disease risk and severity of systemic juvenile idiopathic arthritis

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Type 1 interferonopathy presenting as juvenile idiopathic arthritis with interstitial lung disease: report of a new phenotype | Pediatric Rheumatology | Full Text

STING- ociated vas pathy with onset in infancy (SAVI) is a type 1 interferonopathy manifesting as a pulmonary and vascular syndrome resulting from gain-of-function mutations in TMEM173, the gene encoding STING. Familial reports in the literature are sparse. We report a case series of SAVI in a three generation kindred, with a phenotype of interstitial lung disease (ILD) and rheumatoid factor positive polyarticular juvenile idiopathic arthritis (JIA). Current and historical medical records were reviewed for clinical and laboratory information. Whole blood from cases 1 and 2, plus stored appendicectomy tissue from case 3, underwent DNA sequencing of the TMEM173 gene. Peripheral blood RNA was obtained from cases 1 and 2 for functional essment of the TMEM173 mutation. DNA sequencing identified the same heterozygous TMEM173 mutation (c.463G > A; p.Val155Met) in all three cases, consistent with a diagnosis of the autosomal dominant condition SAVI. Functional essment of this mutation identified a prominent interferon signature which was confirmed on repeat testing. SAVI presented in this family as ILD with early onset juvenile rheumatoid arthritis. This condition should be considered in all rheumatoid arthritis patients with early-onset ILD and in all JIA patients with ILD.

Type 1 interferonopathy presenting as juvenile idiopathic arthritis with interstitial lung disease: report of a new phenotype

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