Papular xanthomas and erosive arthritis in a 3 year old girl, is this a new MRH variant?

abstract：UNLABELLED: BACKGROUND:As patients with juvenile idiopathic arthritis (JIA) progress into adulthood, long-term outcome is determined by disease activity, physical and psychosocial development. Decreased aerobic capacity may play a critical role in health-related outcomes in JIA, since it has been linked with cardiovas...

journal_title：Pediatric rheumatology online journal

authors： van Pelt PA,Takken T,van Brussel M,de Witte M,Kruize AA,Wulffraat NM

更新日期：2012-08-20 00:00:00

abstract：BACKGROUND:The usefulness of musculoskeletal ultrasonography (MSUS) in paediatric population is limited by lack of reference values. One of such parameters is hip joint capsule thickness, postulated as an early measure for synovitis. However, the joint capsule is hardly a distinguished structure from slit synovial cavi...

journal_title：Pediatric rheumatology online journal

authors： Żuber Z,Owczarek A,Sobczyk M,Migas-Majoch A,Turowska-Heydel D,Sternal A,Michalczak J,Chudek J

更新日期：2017-01-31 00:00:00

abstract：BACKGROUND:Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in childhood and a major cause of morbidity among children with pediatric rheumatic diseases. The management of JDM is very heterogeneous. The JDM working group of the Society for Pediatric Rheumatology (GKJR) aims to define consensus- a...

journal_title：Pediatric rheumatology online journal

authors： Hinze CH,Oommen PT,Dressler F,Urban A,Weller-Heinemann F,Speth F,Lainka E,Brunner J,Fesq H,Foell D,Müller-Felber W,Neudorf U,Rietschel C,Schwarz T,Schara U,Haas JP

更新日期：2018-06-25 00:00:00

abstract：:Following publication of the original article [1], we have been notified that the colour representation of the graph is not correct in Figure 6 legend. ...

journal_title：Pediatric rheumatology online journal

authors： Lee WF,Wu CY,Yang HY,Lee WI,Chen LC,Ou LS,Huang JL

更新日期：2020-02-18 00:00:00

abstract：BACKGROUND:There are few studies on radiographic outcome after long-term disease duration in juvenile idiopathic arthritis (JIA). We wanted to evaluate 29-year radiographic outcome in hands/wrists and predictors of damage in patients with long-term active JIA. METHODS:Patients diagnosed from 1980 to 1985, who had acti...

journal_title：Pediatric rheumatology online journal

authors： Selvaag AM,Kirkhus E,Törnqvist L,Lilleby V,Aulie HA,Flatø B

更新日期：2017-04-11 00:00:00

abstract：BACKGROUND:Fibrodysplasia ossificans progressiva (FOP) is the most catastrophic form of heterotopic ossification, due to ongoing intracellular signaling through the bone morphogenic protein pathway. The paroxysmal appearance of inflammatory lumps and elevated inflammatory markers during flares, suggest that FOP is an a...

journal_title：Pediatric rheumatology online journal

authors： Haviv R,Moshe V,De Benedetti F,Prencipe G,Rabinowicz N,Uziel Y

更新日期：2019-12-21 00:00:00

abstract：PURPOSE:We investigated the etiology of acute hepatitis in three children with systemic Juvenile Idiopathic Arthritis (sJIA) taking Interleukin-1 receptor antagonist (IL1RA). METHODS:Laboratory and clinical data for three children with sJIA diagnosed at ages 13 months to 8 years who developed acute hepatitis during tr...

journal_title：Pediatric rheumatology online journal

authors： Canna S,Frankovich J,Higgins G,Narkewicz MR,Nash SR,Hollister JR,Soep JB,Dragone LL

更新日期：2009-12-22 00:00:00

abstract：BACKGROUND:Juvenile Idiopathic Arthritis (JIA) may cause significant impairment in health-related quality of life (HrQoL), despite effective therapies. The aim of this study was to assess HrQoL during first-year treatment in patients with new-onset polyarticular JIA, and to compare treatment strategies. METHODS:In ACU...

journal_title：Pediatric rheumatology online journal

authors： Tarkiainen M,Tynjälä P,Vähäsalo P,Kröger L,Aalto K,Lahdenne P

更新日期：2019-12-16 00:00:00

abstract：BACKGROUND:Early diagnosis and treatment of Juvenile Idiopathic Arthritis (JIA) is essential to optimize outcomes. Wait times (WTs) to consultation with a pediatric rheumatologist consultation is a Canadian quality measure, with benchmarks set at 7 days for systemic JIA (sJIA) and 4 weeks for other JIA categories. In t...

journal_title：Pediatric rheumatology online journal

authors： Barber CEH,Barnabe C,Benseler S,Chin R,Johnson N,Luca N,Miettunen P,Twilt M,Veeramreddy D,Shiff NJ,Schmeling H

更新日期：2020-03-06 00:00:00

abstract：BACKGROUND:Dystrophic calcifications may occur in patients with J uvenile Idiopathic Inflammatory Myopathy (JIIM) as well as other connective tissue and metabolic diseases, but a reliable method of measuring the volume of these calcifications has not been established. The purpose of this study is to determine the feasi...

journal_title：Pediatric rheumatology online journal

authors： Ibarra M,Rigsby C,Morgan GA,Sammet CL,Huang CC,Xu D,Targoff IN,Pachman LM

更新日期：2016-11-29 00:00:00

abstract：BACKGROUND:Kawasaki disease (KD) is an acute febrile systemic vasculitis that affects small and medium blood vessels. Intensified treatments for the most severely affected patients have been proposed recently, and the early identification of KD patients at high risk for coronary artery aneurysms (CAA) is crucial. Howev...

journal_title：Pediatric rheumatology online journal

authors： Chbeir D,Gaschignard J,Bonnefoy R,Beyler C,Melki I,Faye A,Meinzer U

更新日期：2018-07-18 00:00:00

abstract：BACKGROUND:Deficiency of interleukin-36 receptor antagonist (DITRA) is a life threatening monogenic autoinflammatory disease caused by loss of function mutations in the IL36RN gene. Affected patients develop recurrent episodes of generalized pustular psoriasis (GPP) with systemic inflammation and fever. We here review ...

journal_title：Pediatric rheumatology online journal

authors： Hospach T,Glowatzki F,Blankenburg F,Conzelmann D,Stirnkorb C,Müllerschön CS,von den Driesch P,Köhler LM,Rohlfs M,Klein C,Hauck F

更新日期：2019-07-08 00:00:00

abstract：BACKGROUND:Transition guidelines and recommendations for developing countries are limited and best transition practices in young patients with chronic medical conditions have been poorly examined. This study evaluates transition practices from pediatric to adult rheumatology care in Brazil. METHODS:Practicing pediatri...

journal_title：Pediatric rheumatology online journal

authors： Anelli CG,Amorim ALM,Osaku FM,Terreri MT,Len CA,Reiff A

更新日期：2017-05-30 00:00:00

abstract：: Autoimmunity is often observed among individuals with primary immune deficiencies; however, the frequency and role of autoimmunity in Schimke immuno-osseous dysplasia (SIOD) has not been fully assessed. SIOD, which is caused by mutations of SMARCAL1, is a rare autosomal recessive disease with its prominent features b...

journal_title：Pediatric rheumatology online journal

authors： Zieg J,Krepelova A,Baradaran-Heravi A,Levtchenko E,Guillén-Navarro E,Balascakova M,Sukova M,Seeman T,Dusek J,Simankova N,Rosik T,Skalova S,Lebl J,Boerkoel CF

更新日期：2011-09-13 00:00:00

abstract：: Bullous systemic lupus erythematosus is a rare blistering condition with a distinctive combination of clinical, histological and immunopathologic features that together constitute a unique bullous disease phenotype. It is often associated with autoimmunity to type VII collagen. Here we report a child who presented wi...

journal_title：Pediatric rheumatology online journal

authors： Kumar S,Agarwal I

更新日期：2007-11-21 00:00:00

abstract：BACKGROUND:Relapsing periodic arthritis is a general term used for a group of diseases with recurring and periodic nature, in which the joints are intermittently involved. The aim of this study is to evaluation of the possible relationship between MEFV gene mutations in intermittent arthritis of children which has recu...

journal_title：Pediatric rheumatology online journal

authors： Salehzadeh F,Barak M,Nematdoust Haghi R

更新日期：2019-06-06 00:00:00

abstract：BACKGROUND:Paediatric rheumatology service in Sub-Sahara African is virtually not available as there is a shortage of paediatric rheumatologists and other rheumatology health professionals. We aim to describe the clinical spectrum and the frequencies of paediatric rheumatic diseases (PRDs) in Lagos State University Tea...

journal_title：Pediatric rheumatology online journal

authors： Olaosebikan BH,Adelowo OO,Animashaun BA,Akintayo RO

更新日期：2017-01-31 00:00:00

abstract：PURPOSE:To present the case of a 14-year-old boy with clinical and radiographic features of pseudorheumatoid chondrodyspalsia with additional, potentially serious, cervical malformations. METHODS:Detailed clinical and radiological examinations were undertaken with emphasis on the usefulness of 3D-CT scanning. RESULTS...

journal_title：Pediatric rheumatology online journal

authors： Al Kaissi A,Ben Chehida F,Ben Ghachem M,Grill F,Klaushofer K

更新日期：2007-09-27 00:00:00

abstract：:Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease in Canada and is characterized by a clinical syndrome of episodic inflammatory symptoms. Traditionally, the disease is defined by autosomal recessive inheritance of MEFV gene variants, yet FMF also not uncommonly manifests in indi...

journal_title：Pediatric rheumatology online journal

authors： Westwell-Roper C,Niemietz I,Tucker LB,Brown KL

更新日期：2019-05-14 00:00:00

abstract：BACKGROUND:Idiopathic juvenile osteoporosis (IJO) is a rare condition of poorly understood etiology and pathophysiology that affects otherwise healthy children. This condition is characterized clinically by bone pain and vertebral fractures; spontaneous recovery is observed after puberty in the majority of cases. Altho...

journal_title：Pediatric rheumatology online journal

authors： Bacchetta J,Wesseling-Perry K,Gilsanz V,Gales B,Pereira RC,Salusky IB

更新日期：2013-02-19 00:00:00

abstract：: Recent recognition of the markedly high prevalence of temporomandibular joint (TMJ) arthritis in children with juvenile idiopathic arthritis (JIA) coupled with the significant morbidity associated with TMJ damage has prompted increased interest in both the clinical and pathological aspects of TMJ arthritis. This revi...

journal_title：Pediatric rheumatology online journal

authors： Ringold S,Cron RQ

更新日期：2009-05-29 00:00:00

abstract：BACKGROUND:Periodic fever syndromes (PFS) are an emerging group of autoinflammatory disorders. Clinical overlap exists and multiple genetic analyses may be needed to assist diagnosis. We evaluated the diagnostic value of a 5-gene sequencing panel (5GP) in patients with undiagnosed PFS. METHODS:Simultaneous double stra...

journal_title：Pediatric rheumatology online journal

authors： De Pieri C,Vuch J,De Martino E,Bianco AM,Ronfani L,Athanasakis E,Bortot B,Crovella S,Taddio A,Severini GM,Tommasini A

更新日期：2015-04-10 00:00:00

abstract：BACKGROUND:Anti-TNF (Tumor necrosis factor) therapy is effective in treating pediatric patients with refractory rheumatic disease. There is however a concern that anti-TNF usage may increase the risk of malignancy. Reports on specific types of malignancy in this patient population have been emerging over the past decad...

journal_title：Pediatric rheumatology online journal

authors： Okihiro A,Hasija R,Fung L,Cameron B,Feldman BM,Laxer R,Schneider R,Silverman E,Spiegel L,Yeung RSM,Tse SML

更新日期：2018-03-14 00:00:00

abstract：BACKGROUND:Withholding live-attenuated vaccines in patients using interleukin (IL)-1 or IL-6 blocking agents is recommended by guidelines for both pediatric and adult rheumatic diseases, since there is a risk of infection in an immune suppressed host. However, this has never been studied. This retrospective, multicente...

journal_title：Pediatric rheumatology online journal

authors： Jeyaratnam J,Ter Haar NM,Lachmann HJ,Kasapcopur O,Ombrello AK,Rigante D,Dedeoglu F,Baris EH,Vastert SJ,Wulffraat NM,Frenkel J

更新日期：2018-03-21 00:00:00

abstract：BACKGROUND:Juvenile idiopathic arthritis (JIA) is an autoimmune disease characterized by persistent chronic arthritis. Disease risk is believed to be influenced by both genetic and environmental factors. It is well established that the PTPN22 single nucleotide polymorphism (SNP) rs2476601 is associated with JIA suscept...

journal_title：Pediatric rheumatology online journal

authors： Goulielmos GN,Chiaroni-Clarke RC,Dimopoulou DG,Zervou MI,Trachana M,Pratsidou-Gertsi P,Garyfallos A,Ellis JA

更新日期：2016-04-23 00:00:00

abstract：BACKGROUND:An increased concentration of plasma homocysteine (Hcy) has toxic effects on vascular endothelium. This seems to be a risk factor of cardiovascular disease, premature stroke and venous thrombosis. The risk is higher in coincidence with other factors like chronic diseases and familiar hypercholesterolemia. Th...

journal_title：Pediatric rheumatology online journal

authors： Gonçalves M,D'Almeida V,Guerra-Shinohara EM,Galdieri LC,Len CA,Hilário MO

更新日期：2007-04-02 00:00:00

abstract：BACKGROUND:Patient recruitment can be very challenging in paediatric studies, especially in relatively uncommon conditions, such as juvenile idiopathic arthritis (JIA). However, involving children and young people (CYP) in the design of such trials could promise a more rapid trajectory towards making evidence-based tre...

journal_title：Pediatric rheumatology online journal

authors： Sherratt FC,Roper L,Stones SR,McErlane F,Peak M,Beresford MW,Foster H,Ramanan AV,Rooney M,Baildam E,Young B

更新日期：2018-12-04 00:00:00

abstract：BACKGROUND:Juvenile Idiopathic Arthritis (JIA) is the most common cause of non-infectious joint inflammation in children. Synovial inflammation results in pain, swelling and stiffness. Animal and adult human studies indicate that localized joint-associated inflammation may produce generalized changes in pain sensitivit...

journal_title：Pediatric rheumatology online journal

authors： Cornelissen L,Donado C,Kim J,Chiel L,Zurakowski D,Logan DE,Meier P,Sethna NF,Blankenburg M,Zernikow B,Sundel RP,Berde CB

更新日期：2014-09-06 00:00:00

abstract：BACKGROUND:No standard treatment protocol exists for Kawasaki disease (KD) after spontaneous defervescence that does not use intravenous immunoglobulin (IVIG). Moreover, some cases present an indolent course and later develop coronary artery lesions (CALs). We aimed herein to assess the short-term prognosis of KD after...

journal_title：Pediatric rheumatology online journal

authors： Takahashi T,Sakakibara H,Morikawa Y,Miura M

更新日期：2015-11-04 00:00:00

abstract：BACKGROUND:Juvenile Dermatomyositis is a rare, potentially life-threatening condition with no known cure. There is no published literature capturing how children and young people feel about their condition, from their perspective. This study was therefore unique in that it asked children and young people what is it lik...

journal_title：Pediatric rheumatology online journal

authors： Livermore P,Gray S,Mulligan K,Stinson JN,Wedderburn LR,Gibson F

更新日期：2019-06-18 00:00:00