IntroductionDown's Arthropathy (DA) was first reported in the literature in 1984. Crude estimates suggest higher incidence and prevalence rates of DA compared with Juvenile Idiopathic Arthritis ...(JIA), (JIA prevalence 1/1000, estimated DA prevalence 8.7/ 1000). Despite this fact, there remains a paucity of data on this condition. DA is rarely recognised at onset, and remains underdiagnosed. As a direct consequence children with DA are presenting with significant joint damage and disability at diagnosis.ObjectivesPerform a musculoskeletal examination on children with Trisomy 21 (T21) aged 0-20 yearsMethodsChildren with T21 were invited to attend a screening clinic. Screening involved completion of a health questionnaire and a comprehensive musculoskeletal examination. DA cases detected were investigated and managed as per normal clinical practice. Data on a convenience sample of 33 newly diagnosed children with JIA was collected to create a comparison group.Results503 children with T21 have been screened for DA, 22 new cases have been diagnosed. In total, we now have 33 children attending our centre with DA (combining cases attending pre-dating the start of the study). This suggests the point prevalence of DA in Ireland is 18-21/1000. The majority of children presented with a polyarticular pattern of disease. 88% of the DA cohort had small joint involvement of the hands, significantly higher than that observed in the JIA comparison group. Erosive changes were reported on X-Ray in 29.2% of the DA cohort (9.5% in the JIA Cohort). Methotrexate-associated nausea was a significant barrier to treatment with this DMARD in DA. There was a significant delay in diagnosis of DA, 1.7 years v 0.7 years in the JIA cohort.ConclusionChildren with T21 are at increased risk of developing arthritis. There is a lack of awareness of this risk among health care professionals and the general public at large. This almost certainly contributes to poor recognition of the disease and a delay in diagnosis. The predominant pattern of disease is polyarticular small joint arthritis. Treatment with standard protocols used in JIA is complicated by drug-associated side effects in children with T21. Our study has raised a number of questions. Future research to accurately define this disease and identify best practice with regards to treatment would be invaluable. We advocate that all children with T21 should have annual musculoskeletal examination as part of their health surveillance programme.
The antioxidant capacity of 22 kinds of fruits was measured by the developed electron spin resonance (ESR) method based on Cu.sup.2+ sensor. Cu.sup.2+ is reduced to Cu.sup.+ by the antioxidants in ...the fruits, and the remaining Cu.sup.2+ was determined by ESR and UV-Vis spectroscopy. Cu.sup.2+ can give an ESR signal whereas Cu.sup.+ cannot, and the loss of the ESR signal was used to quantify the antioxidant capacity of various fruits. The results were shown as vitamin C equivalent antioxidant capacity (VCEAC). The VCEAC values obtained by ESR and UV-Vis methods ranged from 24.23 to 688.61 mg/100 g and from 24.12 to 677.79 mg/100 g, respectively. Cupric ion reducing antioxidant capacity (CUPRAC) and 1,1-diphenyl-2-picryl-hydrazyl (DPPH) methods were employed for comparison. Based on Pearson's correlation test, the results obtained by CUPRAC and DPPH methods were both significantly correlated with these obtained by the present method, which indicated that the novel method was reliable. Total phenolic content for all kinds of fruits was measured with the Folin-Ciocalteu reagent, and VCEAC values obtained by the ESR method were significantly correlated with total phenolic contents.
This study investigates the role of CD4 super(+)CD25 super(+) regulatory T cells during the clinical course of juvenile idiopathic arthritis (JIA). Persistent oligoarticular JIA (pers-OA JIA) is a ...subtype of JIA with a relatively benign, self-remitting course while extended oligoarticular JIA (ext-OA JIA) is a subtype with a much less favorable prognosis. Our data show that patients with pers-OA JIA display a significantly higher frequency of CD4 super(+)CD25 super(bright) T cells with concomitant higher levels of mRNA FoxP3 in the peripheral blood than ext-OA JIA patients. Furthermore, while numbers of synovial fluid (SF) CD4 super(+)CD25 super(bright) T cells were equal in both patient groups, pers-OA JIA patients displayed a higher frequency of CD4 super(+)CD25 super(int) T cells and therefore of CD4 super(+)CD25 super(total) in the SF than ext-OA JIA patients. Analysis of FoxP3 mRNA levels revealed a high expression in SF CD4 super(+)CD25 super(bright) T cells of both patient groups and also significant expression of FoxP3 mRNA in the CD4 super(+)CD25 super(int) T cell population. The CD4 super(+)CD25 super(bright) cells of both patient groups and the CD4 super(+)CD25 super(int) cells of pers-OA JIA patients were able to suppress responses of CD25 super(neg) cells in vitro. A markedly higher expression of CTLA-4, glucocorticoid-induced TNFR, and HLA-DR on SF CD4 super(+)CD25 super(bright) T regulatory (Treg) cells compared with their peripheral counterparts suggests that the CD4 super(+)CD25 super(+) Treg cells may undergo maturation in the joint. In correlation with this mature phenotype, the SF CD4 super(+)CD25 super(bright) T cells showed an increased regulatory capacity in vitro compared with peripheral blood CD4 super(+)CD25 super(bright) T cells. These data suggest that CD4 super(+)CD25 super(bright) Treg cells play a role in determining the patient's fate toward either a favorable or unfavorable clinical course of disease.
Juvenile idiopathic arthritis (JIA) is a collective term for pediatric inflammatory arthritis of unknown etiology, which presents diverse clinical and imaging findings. The pathogenesis is complex; ...however, most cases stem from an autoimmune mechanism. Herein we provide a short review of imaging findings of JIA. Imaging assessment begins with plain radiography demonstrating joint swelling, periarticular osteopenia, and juxtaarticular bone erosion. Bone erosion occurs later in JIA. Instead, aberrant epimetaphyseal growth often gives the first clue to the diagnosis. US and MRI can demonstrate the details of the synovium, cartilage, and subchondral bone. JIA is subdivided into oligoarthritis, polyarthritis (rheumatoid factor-negative and positive), psoriatic arthritis, enthesitis-related arthritis, and systemic JIA. Awareness of the different clinical characteristics, pathogenic background, and prognosis of each subtype facilitates a more advanced, imaging-based diagnosis. Unlike the other types, systemic JIA is an autoinflammatory disease accompanied by inflammatory cytokinemia and systemic symptoms stemming from aberrant activation of the innate immunity. Other autoinflammatory diseases, both monogenic (e.g., NOMID/CINCA) and multifactorial (e.g., CRMO), are also discussed.
New therapeutic strategies for juvenile idiopathic arthritis (JIA) have evolved within the past ten years, and as a result, an update of the 2011 recommendations of the German management guidelines ...was initiated.
A systemic literature review was performed, overarching principles were proposed and pre-selected via an online survey followed by two multidisciplinary consensus conferences. Pharmacological and non-pharmacological treatments were discussed, statements were proposed and ultimately agreed upon by nominal group technique (NGT).
12 overarching therapeutic principles, as well as 9 recommendations on pharmacological and 5 on non-pharmacological treatments for JIA were agreed upon.
This report summarizes the recent update of the interdisciplinary, consensus-based German guidelines on the management of JIA. The multi- and interdisciplinary participation of all caregivers was central for this patient-focused update. With these guidelines, physicians can choose an evidence-based approach, which allows better tailored treatment in this vulnerable cohort of children and adolescents.
•Evidence-based recommendations are crucial for children and adolescents with JIA.•Therapy guidelines for JIA have been updated in a multidisciplinary setting.•Non-pharmacological interventions may be essential in reaching therapeutic targets.
Chronic inflammatory conditions, such as juvenile idiopathic arthritis, are associated with growth failure. Growth failure appears to be correlated with both the effects of inflammation and negative ...effects of glucocorticoids (used as therapeutic option) on the growth hormone axis and locally on the growth plate and bone metabolism. In the last decade, the introduction of biologics has changed the disease course regarding consequences and outcomes. Anyway in some cases, treatment with biologics has failed in restoring normal growth in patients with juvenile idiopathic arthritis; in contrast, several studies have reported improved height velocity and growth rate in patients with juvenile idiopathic arthritis treated with growth hormone. This study aimed to evaluate the impact of growth hormone treatment on the growth and pubertal development in juvenile idiopathic arthritis patients through a narrative review of the literature over the last four decades.
Adolescents with juvenile-onset autoimmune inflammatory rheumatic diseases (AIIRD) could be at-risk for disease flare secondary to SARS-CoV-2 infection or to withholding anti-inflammatory therapy. ...While vaccination can protect against COVID-19, safety and immunogenicity data regarding anti-SARS-CoV-2 vaccines among adolescents with AIIRD are limited. This international, prospective, multicentre study evaluated the safety and immunogenicity of the BNT162b2 anti-SARS-CoV-2 vaccine among adolescents and young adults with juvenile-onset AIIRD, 80% of whom are on chronic immunomodulatory therapy.
Vaccine side effects, disease activity, and short-term efficacy were evaluated after 3 months in 91 patients. Anti-spike S1/S2 IgG antibody levels were evaluated in 37 patients and 22 controls, 2-9 weeks after the second dose.
Ninety-one patients and 40 healthy controls were included. Safety profile was good, with 96.7% (n = 88) of patients reporting mild or no side-effects, and no change in disease activity. However, 3 patients had transient acute symptoms: 2 following the first vaccination (renal failure and pulmonary haemorrhage) and 1 following the second dose (mild lupus flare vs viral infection). Seropositivity rate was 97.3% in the AIIRD group compared with 100% among controls. However, anti-S1/S2 antibody titres were significantly lower in the AIIRD group compared with controls (242 ± 136.4 vs 387.8 ± 57.3 BAU/ml, respectively; p< 0.0001). No cases of COVID-19 were documented during the 3-month follow-up.
Vaccination of juvenile-onset AIIRD patients demonstrated good short-term safety and efficacy, high seropositivity rate, but lower anti-S1/S2 antibody titres compared with healthy controls. These results should encourage vaccination of adolescents with juvenile-onset AIIRD, even while on immunomodulation.
This volume is an extended dialogue between the internationally acclaimed Chinese filmmaker Jia Zhangke and film scholar Michael Berry in which Jia offers a comprehensive first-hand account of his ...life, art, and approach to filmmaking.
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