Unusual presentation of childhood Systemic Lupus Erythematosus.

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：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: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:Familial chilblain lupus (FCL) is a rare, chronic form of cutaneous lupus erythematosus, which is characterized by painful bluish-red inflammatory cutaneous lesions in acral locations. Mutations in TREX1, SAMHD1 and STING have been described in FCL patients. Less than 10 TREX1 mutation positive FCL families ...

journal_title：Pediatric rheumatology online journal

authors： Yi C,Li Q,Xiao J

更新日期：2020-04-15 00:00:00

abstract：BACKGROUND:Kawasaki disease (KD) is the most common pediatric systemic vasculitides of unknown etiology. Recent clinical studies led to reappraisal of the usefulness of initial combination therapy of intravenous immunoglobulin (IVIG) plus a corticosteroid for patients with severe KD. However, the molecular mechanisms u...

journal_title：Pediatric rheumatology online journal

authors： Inoue T,Murakami S,Matsumoto K,Matsuda A

更新日期：2020-10-06 00:00:00

abstract：BACKGROUND:The United States pediatric rheumatology workforce is committed to a mission of providing children access to pediatric rheumatology care. With a limited number and distribution of pediatric rheumatologists, telemedicine has been proposed as one way to meet this mission, yet the adoption of this modality has ...

journal_title：Pediatric rheumatology online journal

authors： Bullock DR,Vehe RK,Zhang L,Correll CK

更新日期：2017-07-11 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：BACKGROUND:Acquired complement deficiency can occur in the setting of autoimmune syndromes, such as systemic lupus erythematosus (SLE), with very low or, occasionally, undetectable C3 levels. Based on inherited complement defects, patients with transiently low complement may be at similar risk for serious bacterial inf...

journal_title：Pediatric rheumatology online journal

authors： Al-Shaikhly T,Hayward K,Basiaga ML,Allenspach EJ

更新日期：2020-09-24 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:There are controversies regarding the accuracy of the tuberculin skin test (TST) and methods based on the production of interferon gamma by sensitized T cells for the diagnosis of latent tuberculosis infection (LTBI) in pediatrics and immunosuppressed patients. Our objectives are to study TST and ELISPOT/T. ...

journal_title：Pediatric rheumatology online journal

authors： Sztajnbok F,Boechat NL,Ribeiro SB,Oliveira SK,Sztajnbok DC,Sant'Anna CC

更新日期：2014-05-22 00:00:00

abstract：OBJECTIVES:To examine the prescribing habits of NSAIDs among pediatric medical and surgical practitioners, and to examine concerns and barriers to their use. METHODS:A sample of 1289 pediatricians, pediatric rheumatologists, sports medicine physicians, pediatric surgeons and pediatric orthopedic surgeons in the United...

journal_title：Pediatric rheumatology online journal

authors： Levy DM,Imundo LF

更新日期：2010-02-04 00:00:00

abstract：BACKGROUND:JIA-associated uveitis (JIAU) is a serious, sight-threatening disease with significant long-term complications and risk of blindness, even with improved contemporary treatments. The MIWGUC was set up in order to propose specific JIAU activity and response items and to validate their applicability for clinica...

journal_title：Pediatric rheumatology online journal

authors： Foeldvari I,Klotsche J,Simonini G,Edelsten C,Angeles-Han ST,Bangsgaard R,de Boer J,Brumm G,Torrent RB,Constantin T,DeLibero C,Diaz J,Gerloni VM,Guedes M,Heiligenhaus A,Kotaniemi K,Leinonen S,Minden K,Miranda V,Miser

更新日期：2019-10-01 00:00:00

abstract：BACKGROUND:CANDLE syndrome (an acronym for Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature) is a recently described rare autosomal recessive disorder charaterized by systemic autoinflammation. Clinical manifestations include presentation in the first year of life, episodes of fever ...

journal_title：Pediatric rheumatology online journal

authors： Boyadzhiev M,Marinov L,Boyadzhiev V,Iotova V,Aksentijevich I,Hambleton S

更新日期：2019-05-02 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：: Takayasu arteritis (TA) is a rare chronic inflammatory disease of the aorta and its major branches. It is seen predominantly in females during the second and third decades of life, although it can occur in childhood. The aetiology of TA remains unknown. To date, familial cases of TA have been considered rare; however...

journal_title：Pediatric rheumatology online journal

authors： Morishita KA,Rosendahl K,Brogan PA

更新日期：2011-02-02 00:00:00

abstract：BACKGROUND:The pathologic diagnosis of isolated myocarditis without pericardial involvement is uncommonly encountered in systemic onset Juvenile Idiopathic Arthritis (soJIA). CASE:An eleven year-old boy with soJIA died suddenly while being treated with the interleukin 1 (IL-1) receptor inhibitor, anakinra. His autopsy...

journal_title：Pediatric rheumatology online journal

authors： Zeft AS,Menon SC,Miller D

更新日期：2012-04-10 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：BACKGROUND:Recently preliminary ultrasonography (US) definitions, in B mode, for normal components of pediatric joints have been developed by the OMERACT US group. The aim of the current study was to include Doppler findings in the evaluation and definition of normal joint features that can be visualized in healthy chi...

journal_title：Pediatric rheumatology online journal

authors： Collado P,Windschall D,Vojinovic J,Magni-Manzoni S,Balint P,Bruyn GAW,Hernandez-Diaz C,Nieto JC,Ravagnani V,Tzaribachev N,Iagnocco A,D'Agostino MA,Naredo E,OMERACT ultrasound subtask force on pediatric.

更新日期：2018-04-10 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: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 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:To test clinical findings associated with early temporomandibular joint (TMJ) arthritis in comparison to the current gold standard contrast enhanced magnetic resonance imaging (MRI) in children with juvenile idiopathic arthritis (JIA). METHODS:Seventy-six consecutive JIA patients were included in this study...

journal_title：Pediatric rheumatology online journal

authors： Keller H,Müller LM,Markic G,Schraner T,Kellenberger CJ,Saurenmann RK

更新日期：2015-12-09 00:00:00

abstract：BACKGROUND:Juvenile Fibromyalgia (JFM) is characterized by chronic widespread musculoskeletal pain and approximately 40% of children and adolescents with JFM also suffer from benign joint hypermobility (HM). It is not currently known if the presence of HM affects the pain experience of adolescents with JFM. The objecti...

journal_title：Pediatric rheumatology online journal

authors： Ting TV,Hashkes PJ,Schikler K,Desai AM,Spalding S,Kashikar-Zuck S

更新日期：2012-06-15 00:00:00

abstract：BACKGROUND:To investigate the prevalence of cumulative organ damage among Egyptian children with juvenile-onset systemic lupus erythematosus (jSLE) and the relationships between the organ damage and the demographic data, clinical variables, and disease activity. METHODS:A total of 148 patients with jSLE have been foll...

journal_title：Pediatric rheumatology online journal

authors： Salah S,Lotfy HM,Mokbel AN,Kaddah AM,Fahmy N

更新日期：2011-12-09 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：: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: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：: There is a debate whether post-streptococcal reactive arthritis (PSRA) is a separate entity or a condition on the spectrum of acute rheumatic fever (ARF). We believe that PSRA is a distinct entity and in this paper we review the substantial differences between PSRA and ARF. We show how the demographic, clinical, gene...

journal_title：Pediatric rheumatology online journal

authors： Uziel Y,Perl L,Barash J,Hashkes PJ

更新日期：2011-10-20 00:00:00

abstract：BACKGROUND:Juvenile idiopathic arthritis (JIA) is the most common pediatric rheumatic disease and a leading cause of childhood disability. The objective of this study was to characterize the PK, safety, and taste acceptability of tofacitinib in patients with JIA. METHODS:This Phase 1, open-label, multiple-dose (twice ...

journal_title：Pediatric rheumatology online journal

authors： Ruperto N,Brunner HI,Zuber Z,Tzaribachev N,Kingsbury DJ,Foeldvari I,Horneff G,Smolewska E,Vehe RK,Hazra A,Wang R,Mebus CA,Alvey C,Lamba M,Krishnaswami S,Stock TC,Wang M,Suehiro R,Martini A,Lovell DJ,Pediatric Rheu

更新日期：2017-12-28 00:00:00

abstract：:Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is auto...

journal_title：Pediatric rheumatology online journal

authors： Clarke SL,Sen ES,Ramanan AV

更新日期：2016-04-27 00:00:00