An update on mesenchymal tumors of the head and neck

The purpose of this review is to offer a brief reappraisal of several soft tissue neoplasms germane to the head and neck region. Specifically, this paper is intended to draw attention to the recently described low-grade sarcoma with neural and myogenic features. In addition, other tumors that are largely specific to the head and neck region, including nasopharyngeal angiofibroma, sinonasal hemangiopericytoma and spindle cell/pleomorphic lipoma, will be reviewed in light of recent advances in our understanding of their pathophysiology and diagnosis.     

注射用依那西普联合骨痹汤治疗幼年脊柱关节病的临床观察

目的观察注射用依那西普联合骨痹汤治疗幼年脊柱关节病(JSp A)的临床疗效。方法将77例JSp A患者随机分为2组,治疗组40例应用注射用依那西普联合骨痹汤治疗,对照组37例单纯应用柳氮磺吡啶肠溶片治疗。2组均治疗12周,观察2组治疗前、治疗2周后及治疗12周后红细胞沉降率(ESR)、C反应蛋白(CRP)、血小板计数(PLT)及视觉模拟评分(VAS)变化,比较2组疗效。结果 2组治疗过程中ESR、CRP、PLT及VAS评分均逐步降低,治疗2周后比较差异无统计学意义(P0.05),但治疗12周后,2组均较本组治疗前降低(P0.05),且治疗组各项观察指标较对照组降低更明显(P0.05)。治疗组缓解率55.0%、总有效率97.5%,对照组缓解率27.0%、总有效率81.1%,2组缓解率、总有效率比较差异均有统计学意义(P0.05),治疗组疗效优于对照组。结论注射用依那西普联合骨痹汤治疗JSp A,疗效显著,安全性好。     

Sulphasalazine in the treatment of pauciarticular-onset juvenile chronic arthritis

Summary Sulphasalazine in a dose of 50 mg/kg/day was administered to ten patients with pauciarticular-onset juvenile chronic arthritis (JCA), with active disease not adequately controlled by nonsteroidal anti-inflammatory drugs (NSAID). The treatment was initiated with 1/4 of this dose and increased by weekly increments of 250–500 mg until the total dose was reached. In all patients sulphasalazine was the first disease-modifying agent tried. Among nine of the ten patients there was significant improvement in all clinical scores, including the number of active joints and the severity grading (tenderness and limitation of motion). Within 3 months of sulphasalazine therapy the laboratory measurements revealed marked improvement in the erythrocyte sedimentation rate (ESR) and haemoglobin values. One patient, in whom the ESR and haemoglobin were normal at onset, had no change in clinical scores. Transient skin rash and elevated liver enzyme levels developed in one patient. These preliminary data suggest that sulphasalazine is an effective and safe second-line agent in the management of pauciarticular-onset JCA. More trials with this drug are needed, including double blind, to study efficacy and safety of sulphasalazine in JCA.     

Evidence-based use of methotrexate in children with rheumatic diseases: a consensus statement of the Working Groups Pediatric Rheumatology Germany (AGKJR) and Pediatric Rheumatology Austria

Juvenile idiopathic arthritis (JIA) is the most common diagnosis in children and adolescents with rheumatic disorders. In many children and adolescents, JIA is successfully treated with non-steroidal anti-inflammatory drugs (NSAID) and physiotherapy. However, in a significant number of cases the disease is resistant to this therapy, and treatment with second line disease-modifying antirheumatic drugs (DMARDs) is required. Methotrexate (MTX) is frequently referred to as first-choice second-line agent for the treatment of JIA. To increase drug safety, the Working Groups for Children and Adolescents with Rheumatic Diseases in Germany (AGKJR) and Pediatric Rheumatology Austria have initiated the formulation of evidence-based recommendations. Evidence is based on consensus expert meetings, a MEDLINE search with the key words Methotrexate and juvenile arthritis limited to age 0–18 years, standard textbooks and review articles, data from the central registry of the German Research Center for Rheumatic Diseases (Deutsches Rheumaforschungszentrum Berlin DRFZ), experience with MTX in adults with rheumatoid arthritis (RA), and recommendations of the German Society of Rheumatology (DGRh). Based on these data, evidence and recommendations are graded, and evidence-based recommendations for the use of MTX in children and adolescents with rheumatic disease are presented.Section Pharmacotherapy of the Working Group Pediatric Rheumatology Germany and Austria: I. Foeldvari; J.P. Haas, A. Haeffner, D. Hobusch,G. Horneff, A. Hospach, R. Keitzer, G. Klaus, M. Metzler, H. Michels, T. Niehues, I. Pilz, M. Sailer Höck, M. Schöntube, L. Schuchmann, K. Schumacher, H.W. Seyberth, E. Siemers, A. Urban, E. Weißbarth-Riedl. Working Group Pediatric Rheumatology North-Rhine-Westfalia: S. Benseler, G. Bürk, S. Fahl, I. Foeldvari, D. Föll, M. Frosch, G. Ganser, S. Kastner, I. Kleine, E. Lainka, K. Mönkemöller, J. Neubert, U. Neudorf, T. Niehues, J. Roth, S. Seeliger, N. Wagner, R. Wieland, H. Winowski.     

Proxy‐reported health‐related quality of life of patients with juvenile idiopathic arthritis: The pediatric rheumatology international trials organization multinational quality of life cohort study

Objective

To investigate the proxy‐reported health‐related quality of life (HRQOL) and its determinants in patients with juvenile idiopathic arthritis (JIA).

Methods

In this multinational, multicenter, cross‐sectional study, HRQOL of patients with JIA was assessed through the Child Health Questionnaire (CHQ) and was compared with that of healthy children of similar age from the same geographic area. Potential determinants of HRQOL included demographic data, physician's and parent's global assessments, measures of joint inflammation, Childhood Health Assessment Questionnaire (CHAQ), and erythrocyte sedimentation rate.

Results

A total of 6,639 participants (3,324 with JIA and 3,315 healthy) were enrolled from 32 countries. The mean ± SD physical and psychosocial summary scores of the CHQ were significantly lower in patients with JIA than in healthy children (physical: 44.5 ± 10.6 versus 54.6 ± 4.0, P < 0.0001; psychosocial: 47.6 ± 8.7 versus 51.9 ± 7.5, P < 0.0001), with the physical well‐being domain being most impaired. Patients with persistent oligoarthritis had better HRQOL compared with other subtypes, whereas HRQOL was similar across patients with systemic arthritis, polyarthritis, and extended oligoarthritis. A CHAQ score >1 and a pain intensity rating >3.4 cm on a 10‐cm visual analog scale were the strongest determinants of poorer HRQOL in the physical and psychosocial domains, respectively.

Conclusion

We found that patients with JIA have a significant impairment of their HRQOL compared with healthy peers, particularly in the physical domain. Physical well‐being was mostly affected by the level of functional impairment, whereas the intensity of pain had the greatest influence on psychosocial health.     

幼年特发性关节炎诊疗规范

幼年特发性关节炎(JIA)临床上常见亚型包括全身型JIA、少关节型/多关节型JIA和幼年脊柱关节炎。本病无特异性诊断指标, 需与感染性疾病和恶性病相鉴别。全身型JIA起病多急骤, 病情进展快, 易合并巨噬细胞活化综合征而危及生命。儿童风湿科医生对JIA的诊断及治疗经验仍不足, 规范化诊疗水平有待进一步提高。中华医学会风湿病学分会组织有关专家, 在借鉴国内外诊疗规范和分类标准的基础上, 制定本规范, 旨在规范JIA各亚型及全身型JIA合并巨噬细胞活化综合征的诊断和治疗方案, 以降低致死率和严重并发症的发生率, 从而改善患儿预后。     

Juvenile Xanthogranuloma–A Rare Cause of Unilateral Orbital Swelling in an Elderly Patient

Objective: To report the clinical and pathologic features of an elderly patient with a unilateral orbital swelling and proptosis caused by Juvenile Xanthogranuloma diagnosed and confirmed by orbital biopsy.

Design: Interventional case report.

Participants: One patient.

Intervention: Steroids (Medrol dose pack) and radiation.

Main Outcome Measures: Unusual clinical presentation and pathological features of Juvenile Xanthogranuloma in the orbit.

Conclusions: Juvenile Xanthogranuloma affecting one orbit is very rare with unilateral involvement in an elderly patient. Steroids and radiation therapy were very effective in treatment and provided impressive results.