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The authors describe a 5-year-old girl who developed a Noonan syndrome-like disorder as a result of the CBL c.1194CG/p.His398Gln variant, including headache, papilledema, intracranial hypertension, hyperproteinorrhachia, leucorrhachia, and brain inflammation and vasculitis with CD3 positive lymphocyte infiltration. The patient responded partially to corticosteroids, acetazolamide, and ventriculoperitoneal valve placement. The serum cytokine profile revealed persistently elevated levels of IL-1 RA, IL-2R alpha, IL-6, IL-18, MCP-1, and MCP-3. Cyclophosphamide was used as a bridge to allogeneic hematopoietic stem cell transplantation in this case.

Objectives: To evaluate electrocardiogram markers to predict coronary involvement in patients with Kawasaki disease by assessing measures of ventricular repolarization parameters on the 12-lead electrocardiogram. Study design: This cross-sectional study included 180 Spanish and Japanese patients ≤14 years of age with Kawasaki disease, with or without coronary involvement, from 2011 to 2016. We manually measured the Tp-Te/QT ratio and QTc interval (with Bazett's formula) in 12-lead electrocardiogram in the acute and recovery period and explored their potential association with coronary involvement. Results: No association was found between Tp-Te/QT ratio obtained manually in V5 and V6 leads and coronary involvement in the acute (V5:0.25 [IQR, 0.21-0.27] vs 0.25 [IQR, 0.20-0.27], P = .80; V6:0.24 [IQR, 0.21-0.27] vs 0.25 [IQR, 0.20-0.27], P = .86) or the recovery (V5: 0.23 [IQR, 0.20-0.25] vs 0.23 [IQR, 0.19-0.25], P = .68; V6: 0.23 [IQR, 0.20-0.25] vs 0.23 [IQR, 0.17-0.25], P = .50) period. By contrast, QTc in V5 and V6 was significantly lower in patients with Kawasaki disease and coronary involvement in the acute period (V5: 378 ms [IQR, 364-395 ms] vs 390 ms [IQR, 371-411 ms], P = .04; V6: 377 ms [IQR, 364-392 ms] vs 390 ms [IQR, 371-410 ms], P = .01). A QTc interval of

The Juvenile Arthritis Multidimensional Assessment Report (JAMAR) is a new parent/patient-reported outcome measure that enables a thorough assessment of the disease status in children with juvenile idiopathic arthritis (JIA). We report the results of the cross-cultural adaptation and validation of the parent and patient versions of the JAMAR in the Colombian Spanish language. The reading comprehension of the questionnaire was tested in ten JIA parents and patients. Each participating centre was asked to collect demographic, clinical data and the JAMAR in 100 consecutive JIA patients or all consecutive patients seen in a 6-month period and to administer the JAMAR to 100 healthy children and their parents. The statistical validation phase explored descriptive statistics and the psychometric issues of the JAMAR: the three Likert assumptions, floor/ceiling effects, internal consistency, Cronbach's alpha, interscale correlations, and construct validity (convergent and discriminant validity). A total of 22 JIA patients (9.1% systemic, 27.3% RF-negative polyarthritis, 36.4% enthesitis-related arthritis, 27.2% other categories) were enrolled in the paediatric centre of Bogota. All JAMAR components revealed good psychometric performances. In conclusion, the Colombian Spanish version of the JAMAR is a valid tool for the assessment of children with JIA and is suitable for use both in routine clinical practice and clinical research.

Introduction: Kawasaki disease (KD) is an acute self-limited systemic vasculitis relatively common in childhood. The etiology of KD is still unknown, although clinical, laboratory and epidemiological features suggest an infectious origin or trigger. Differences on incidence between countries have been related to specific genetic factors, ethnicity, country of birth and some other sociocultural and environmental factors. We present a population-based study on incidence of KD in Catalonia (Spain), focusing on differences between patients in rural and non-rural areas of the region. Methods: Observational population-based study including all Pediatric Units in Catalan hospitals, between 2004 and 2014. A 12-month (March 2013–March 2014) prospective collection of new cases of KD was carried out to determine the incidence of KD. The rest of the data was retrieved retrospectively. Results: Data from 399 patients over the 10-year study period was analyzed. Among the total KD patients, 353 (88.5%) lived in non-rural areas and 46 (11.5%) in rural areas. It was found that there is a significant difference (p

Objective. To evaluate the efficacy and safety of canakinumab treatment in active hyperimmunoglobulinemia D with periodic fever syndrome (HIDS). Methods. This was a 3-part, open-label study with a 6-month treatment period (P1; 300 mg or 4 mg/kg q6w), a 6-month withdrawal period (P2), and a 24-month treatment period (P3). The primary endpoint was reduction in frequency of attacks during treatment period compared with the historical period (HP; period in which patients did not receive drugs other than NSAIDs and/or steroids). Results. All nine patients completed P1 and P2, whereas only eight completed P3. All patients achieved a complete response during P1, and only two required dose adjustments. The median number of attacks per patient decreased from 5 (3–12) during HP to 0 (0–2) during P1. During P2, seven out of nine patients flared with a median of 110 days (62-196) after the last canakinumab dose. Laboratory parameters were normalized at Day 15 and remained at normal levels throughout the study. The blood transcriptome assessed during P1 showed upregulated interferon and myeloid-related inflammatory responses in untreated patients compared to healthy controls, which rapidly decreased following canakinumab injection, reaching levels comparable to those of healthy controls. At least one adverse event (AE) was detected in all nine patients; most of them being mild in intensity, with infections being the most frequent AE. Serious AEs were reported in four patients. Conclusions. This study demonstrated the efficacy and safety of canakinumab treatment to control active HIDS and to suppress inflammatory transcriptional responses. This article is protected by copyright. All rights reserved.

Juvenile idiopathic arthritis (JIA) is a complex rheumatic disease with both autoimmune and autoinflammatory components. Recently, familial cases of systemic-onset JIA have been attributed to mutations in LACC1/FAMIN. We describe three affected siblings from a Moroccan consanguineous family with an early-onset chronic, symmetric and erosive arthritis previously diagnosed as rheumatoid factor (RF)-negative polyarticular JIA. Autozygosity mapping identified four homozygous regions shared by all patients, located in chromosomes 3, 6 (n:2) and 13, containing over 330 genes. Subsequent whole exome sequencing identified two potential candidate variants within these regions (in FARS2 and LACC1/FAMIN). Genotyping of a cohort of healthy Moroccan individuals (n: 352) and bioinformatics analyses finally supported the frameshift c.128_129delGT mutation in the LACC1/FAMIN gene, leading to a truncated protein (p.Cys43Tyrfs*6), as the most probable causative gene defect. Additional targeted sequencing studies performed in patients with systemic-onset JIA (n:23) and RF-negative polyarticular JIA (n: 44) revealed no pathogenic LACC1/FAMIN mutations. Our findings support the homozygous genotype in the LACC1/FAMIN gene as the defect underlying the family here described with a recessively inherited severe inflammatory joint disease. Our evidences provide further support to the involvement of LACC1/FAMIN deficiency in different types of JIA in addition to the initially described systemic-onset JIA., The authors would like to thank the CRG Genomics Unit for assistance with whole exome sequencing. This work has been partially funded by: CERCA Programme/Generalitat de Catalunya (JIA, XE, SO), the PERIS program of the Generalitat de Catalunya grant SLT002/16/00310 (RR), the Spanish Ministry of Economy and Competitiveness co-financed by European Regional Development Fund (ERDF) grant SAF2015-68472-C2-1-R (JIA), the Instituto de Salud Carlos III/Transnational Research Projects on Rare Diseases (JIA) grant AC15/00027, the Spanish Society of Pediatric Rheumatology (JIA), the Secretaria d’Universitats i Recerca del Departament d’Economia grant 2009-SGR-1502 (XE) and the European Union Seventh Framework Programme (FP7/2007-2013) grant agreement no. 262055 (XE). We also acknowledge support of the Spanish Ministry of Economy and Competitiveness (MEIC) to the EMBL partnership, ‘Centro de Excelencia Severo Ochoa’.

Mevalonate Kinase Deficiency (MKD) is a rare monogenic autoinflammatory disorder (AID) with autosomal recessive inheritance caused by mutations in the MVK gene. It includes hyperimmunoglobulinemia D syndrome (HIDS) and mevalonic aciduria (a severe form). Patients have recurrent inflammatory attacks with high fever, gastrointestinal symptoms, lymphadenopathy, splenomegaly, arthralgia, rash, pharyngitis, aphtosis and constitutional complaints. Heightened understanding of molecular mechanisms in monogenic autoinflammatory disorders has provided tools for targeted treatment. HIDS is an extrinsic inflammasomopathy and is responsive to anti-IL-1 therapies, such as the recombinant IL-1-receptor antagonist anakinra, the monoclonal antibody against IL-1b canakinumab (CAN), and the recombinant IL-1R fusion protein rilonacept. Areas covered: CAN is a human monoclonal anti-IL-1β antibody that binds with high affinity and neutralizes the activity of IL-1 β. Both observational registries and some case reports have seemed promising in the efficacy of CAN in the HIDS treatment. Two clinical trials have corroborated CAN as an effective and safe drug. Expert commentary: CAN is effective and safe for the treatment of HIDS patients. Some data suggest these patients may need higher dosage or shorter dosing interval than other AIDs, to achieve and maintain complete clinical and laboratory response. Reported adverse events were mild, most often non-complicated infections.

Systemic connective tissue disorders are characterized by the presence of autoantibodies and multiorgan system involvement. Juvenile systemic lupus erythematosus with or without associated antiphospholipid syndrome; juvenile dermatomyositis; sclerodermiform syndromes, including systemic and localized sclerodermas and eosinophilic fasciitis; mixed connective tissue disease; and Sjogren syndrome are the disorders that affect children most frequently. Diagnosis is difficult, because the clinical presentation of patients is diverse, from mild to severe disease. In addition, all organs may be affected. However, a variety of imaging techniques are now available to investigate rheumatic disease in children. These imaging modalities offer the potential for earlier diagnosis and improved assessment of therapeutic response. This article reviews the main connective tissue disorders that affect children, highlighting their key imaging features on images acquired with different diagnostic imaging modalities and correlating these features with clinical and pathologic findings, when available. ©RSNA, 2019.

ObjectiveTo report 2 patients with anti–myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis who were initially misdiagnosed with small vessel primary CNS vasculitis.MethodsReview of symptoms, MRI and neuropathologic features, and response to treatment. MOG antibodies were determined in serum and CSF using a cell-based assay.ResultsSymptoms included fever, headache, and progressive mental status changes and focal neurologic deficits. CSF studies revealed lymphocytic pleocytosis, and both patients had abnormal brain MRIs. Brain biopsy samples showed prominent lymphocytic infiltration of the wall of small vessels; these findings initially suggested small vessel CNS vasculitis, and both patients were treated accordingly. Although 1 patient had a relapsing-remitting course not responsive to cyclophosphamide, the other one (also treated with cyclophosphamide) did not relapse. Retrospective assessment of serum and CSF demonstrated MOG antibodies in both cases, and review of biopsy specimens showed absence of fibrinoid necrosis (a pathologic requirement for small vessel CNS vasculitis).ConclusionsAnti–MOG-associated encephalitis can be mistaken for small vessel CNS vasculitis. This is important because the diagnosis of anti–MOG-associated encephalitis does not require brain biopsy and can be established with a serologic test.

Aparició en l'adult; Malalties autoinflamatòries; Variants de baixa penetrància Aparición en el adulto; Enfermedades autoinflamatorias; Variantes de baja penetrancia Adult onset; Autoinflammatory diseases; Low-penetrance variants Background: Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autosomal-dominant autoinflammatory disease caused by mutations in the TNFRSF1A gene. R92Q, a low-penetrance variant, is usually associated with a milder TRAPS phenotype than structural or pathogenic mutations. No studies differentiating R92Q-related disease in patients with pediatric and adult onset have been performed to date. Objective: To analyze clinical features and disease outcomes in patients diagnosed with TRAPS associated with R92Q variant and to investigate differences between patients with pediatric and adult disease onset. Methods: A retrospective review of patients with R92Q-related disease from four reference centers for autoinflammatory diseases was performed. Clinical and laboratory features, family history of autoinflammatory diseases, treatments received, and outcomes during follow-up were recorded and separately analyzed in pediatric and adult patients. Our results were included in the analysis with other reported pediatric and adult R92Q-related disease series. Results: Our series encompassed 18 patients (9 females and 9 males) with R92Q variant. In 61% of patients, disease onset occurred during infancy and in 39%, during adulthood, with a median diagnostic delay of 5 years and a follow-up of 5.4 years. A positive family history of autoinflammatory disease was detected in 28% of patients. All patients presented with febrile recurrent episodes. Other common symptoms included arthralgia/arthritis (61%), myalgia (39%), asthenia/fatigue (44%), abdominal pain (39%), headache (33%), odynophagia (33%), skin rash (28%), and chest pain (22%). During attacks, 80% of patients increased acute phase reactants levels. No patient had developed amyloidosis during the study period. At the end of follow-up, 28% of patients were asymptomatic and treatment free, 50% were receiving non-steroidal anti-inflammatory drugs or glucocorticoids on demand, and 22% were being treated with biologic agents. When differences between pediatric and adult patients were globally analyzed, adults tended to have longer attacks duration and presented more frequently with chest pain and headache, while abdominal pain, vomiting, cervical adenitis, and pharyngitis predominated in pediatric patients. No differences in outcomes and treatment requirements were observed in both age groups. Conclusion: This study has contributed to characterize R92Q-related disease by identifying trends in disease phenotypes depending on the age at disease onset. This study has been supported by Ministerio de Economía y Competitividad (SAF 14/57708-R) and co-funded by Fondo Europeo de Desarrollo Regional (FEDER), Unión Europea, and Una manera de hacer Europa (JH-R).

The term juvenile idiopathic arthritis (JIA) encompasses a group of arthritides of unknown cause that begin before 16 years of age and last for at least 6 weeks. Temporomandibular joint (TMJ) involvement has been described in up to 87% of children with JIA and has been associated with all JIA subtypes. TMJ involvement is difficult to detect clinically. In one study, 71% of JIA patients with active TMJ synovitis were asymptomatic and 63% had normal physical examination results. Moreover, the main growth center of the mandible is located in the condyle, separated from the joint space by only a thin layer of fibrocartilage. This makes mandibular growth vulnerable to arthritic changes, eventually resulting in facial asymmetry and retrognathia. Therefore, early detection and treatment of TMJ arthritis are paramount to preserving motility and preventing deformity. As clinical symptoms and physical examination results are not good markers of TMJ involvement, imaging plays a key role in diagnosis and treatment monitoring. Magnetic resonance imaging is the technique of choice for the study of TMJ arthritis. It has the advantages of displaying both soft tissue and bone, is the most sensitive technique for detecting acute synovitis, and is the only one able to demonstrate bone marrow edema. In addition, it allows the assessment of changes to the joint over time and evaluation of the effectiveness of therapeutic interventions. ©RSNA, 2017.

To evaluate the efficacy and safety of canakinumab treatment in active hyperimmunoglobulinemia D with periodic fever syndrome (HIDS).This was a 3-part open-label study with an initial 6-month treatment period in which patients with HIDS (n = 9) received canakinumab subcutaneously at a dose of 300 mg (or 4 mg/kg for those weighing ≤40 kg) every 6 weeks (period 1 [P1]), followed by a 6-month withdrawal period (period 2 [P2]), and then a 24-month extension treatment period with canakinumab at the same dose (period 3 [P3]). The primary end point was reduction in the frequency of attacks during treatment periods as compared to the historical period (HP; defined as the period in which patients did not receive drugs other than nonsteroidal antiinflammatory drugs and/or steroids).All 9 patients completed P1 and P2, whereas only 8 patients completed P3. All patients achieved a complete response during P1, and only 2 required dose adjustments. The number of attacks per patient decreased from a median of 5 (range 3-12) during the HP to a median of 0 (range 0-2) during P1. During P2, 7 of 9 patients experienced a disease flare within a median of 110 days (range 62-196) after the last canakinumab dose. Laboratory findings were normalized by day 15 of treatment and remained at normal levels throughout the study. Analysis of blood transcriptome profiles, assessed during P1, showed up-regulated levels of interferon and myeloid-related inflammatory responses in untreated patients compared to healthy controls, and these rapidly decreased following canakinumab injection, reaching levels comparable to those of healthy controls. At least 1 adverse event (AE) was detected in all 9 patients. Most of the AEs were mild in intensity, with infections being the most frequent AE. Serious AEs were reported in 4 patients.The results of this study demonstrate the efficacy and safety of canakinumab treatment to control active HIDS and to suppress inflammation-related transcriptional responses.

Treatment with tumor necrosis factor α inhibitors is a risk factor for tuberculosis (TB). Despite previous treatment with isoniazid for latent TB, a 9-year-old girl with juvenile idiopathic arthritis developed disseminated TB after changing therapy with etanercept to adalimumab and after new contact with a smear-positive relative. Genotyping strain matches and susceptibility to isoniazid make reinfection more likely than reactivation in our patient.

The antiphospholipid syndrome (APS) is an acquired thrombophilic disorder characterized by the presence of autoantibodies to a variety of phospholipids and phospholipid-binding proteins. Clinical manifestations range from being asymptomatic to having imminently life-threatening events. Catastrophic antiphospholipid syndrome (CAPS) occurs in

Familial cold autoinflammatory syndrome, Muckle-Wells syndrome (MWS), and chronic, infantile, neurological, cutaneous and articular (CINCA) syndrome are dominantly inherited autoinflammatory diseases associated to gain-of-function NLRP3 mutations and included in the cryopyrin-associated periodic syndromes (CAPS). A variable degree of somatic NLRP3 mosaicism has been detected in approximate to 35% of patients with CINCA. However, no data are currently available regarding the relevance of this mechanism in other CAPS phenotypes. Objective To evaluate somatic NLRP3 mosaicism as the disease-causing mechanism in patients with clinical CAPS phenotypes other than CINCA and NLRP3 mutation-negative. Methods NLRP3 analyses were performed by Sanger sequencing and by massively parallel sequencing. Apoptosis-associated Speck-like protein containing a CARD (ASC)-dependent nuclear factor kappa-light chain-enhancer of activated B cells (NF-kappa B) activation and transfection-induced THP-1 cell death assays determined the functional consequences of the detected variants. Results A variable degree (5.5-34.9%) of somatic NLRP3 mosaicism was detected in 12.5% of enrolled patients, all of them with a MWS phenotype. Six different missense variants, three novel (p.D303A, p.K355T and p.L411F), were identified. Bioinformatics and functional analyses confirmed that they were disease-causing, gain-of-function NLRP3 mutations. All patients treated with anti-interleukin1 drugs showed long-lasting positive responses. Conclusions We herein show somatic NLRP3 mosaicism underlying MWS, probably representing a shared genetic mechanism in CAPS not restricted to CINCA syndrome. The data here described allowed definitive diagnoses of these patients, which had serious implications for gaining access to anti-interleukin 1 treatments under legal indication and for genetic counselling. The detection of somatic mosaicism is difficult when using conventional methods. Potential candidates should benefit from the use of modern genetic tools.

Kawasaki disease (KD) is an acute self-limited systemic vasculitis relatively common in childhood. In Japan, last published survey shows an incidence up to 239.6/105 children

Chronic anterior uveitis affects 10-30 % of patients with juvenile idiopathic arthritis (JIA) and is still a cause of blindness in childhood. In most patients it is asymptomatic, bilateral, and recurrent, so careful screening and early diagnosis are important to obtain the best long-term prognosis. The treatment of chronic uveitis associated with JIA is challenging. Initial treatment is based on topical steroids and mydriatic drops. Methotrexate is the most common first-line immunomodulatory drug used. For refractory patients, biologicals, mainly the anti-tumor-necrosis-factor (TNF) drugs adalimumab and infliximab, have been revealed to be effective and have changed the outcome for these patients. Collaboration between pediatric rheumatologists and ophthalmologists is important for the successful diagnosis and treatment of patients with uveitis associated with JIA.

The aims of the study were to assess efficacy and safety of TNF-alpha antagonists (anti-TNF) in a cohort of patients with juvenile idiopathic arthritis (JIA) who began treatment under 4 years old and to assess relapse rate after methotrexate and/or anti-TNF withdrawal. We made a retrospective charts review of our non-systemic JIA patients treated with anti-TNF under 4 years of age between January 2006 and April 2013. Demographics, epidemiologic, clinical, laboratory data and rate of relapse after treatment withdrawal due to clinical remission were collected. Efficacy and safety end points included side effects (SE) and time to achieve clinical remission. We included 27 patients, 23 received etanercept and 4 adalimumab with a median age of 3.01 (range 0.88-3.97) years at anti-TNF beginning and 1.94 (range 0.18-5.44) and 2.39 (range 0.18-7.24) years of treatment and follow-up, respectively. All patients had previously received disease-modifying antirheumatic drugs at optimal dose. Nineteen patients reached clinical remission on treatment in a median time of 9.1 (range 6.23-21.17) months. Four of those relapsed during treatment. Six developed mild SE, mostly mild infections. No serious SE were described. Eleven patients who reached clinical remission relapsed after treatment withdrawal. None achieved clinical remission off treatment. Most patients reached clinical remission on anti-TNF. In our cohort of patients, etanercept and adalimumab were safe, with mostly mild infections and no serious SE. We observed a high relapse rate during treatment withdrawal.

Objective.To assess whether blockade of tumor necrosis factor-α (TNF-α) influences surgical wound healing in a normal mouse experimental model.Methods.Wound healing time course and degree of surgical wound collagenization were measured by morphological techniques and digital image analysis in 80 male SWISS-OF1 mice (40 received subcutaneous etanercept at a dose of 0.1 mg/25g/ at −7, 0, 7, and 14 days).Results.No significant differences were observed between treated and untreated animals in wound healing, re-epithelialization, or formation of inflammatory infiltrate or granulation tissue at days 7, 15, or 20 after surgery. At 20 days, the collagen area was larger in treated versus untreated mice (109029 ± 28489 μm2 vs 79305 ± 19798 μm2, p = 0.026, Mann-Whitney U test).Conclusion.Surgical wounds showed a higher degree of collagenization at 20 days in etanercept-treated versus untreated mice, with no differences in the time course of wound healing. These data suggest that biological therapies to block TNF-α do not affect wound healing and do not need to be suspended during the perioperative period.

Background Temporomandibular joint (TMJ) is affected in approximately 80% of Juvenile Idiopathic Arthritis (JIA) patients, usually asymptomatic. Magnetic Resonance Imaging (MRI) with contrast remains as the gold standard for its diagnosis. Main objectives of management are to control active synovitis and prevent chronic damage but nowadays there is not a consensus on how to treat these patients. TMJ arthroscopy seems to be a non-invasive technique that allows direct examination and local treatment. Objectives To review our JIA patients who have been treated with TMJ arthroscopy because of local active synovitis. Assess clinical and MRI with contrast outcome after 6 and 12 months. Methods We made a retrospective charts review of our JIA patients in whom TMJ arthroscopy had been done. Clinical symptoms and signs were evaluated as pain, maximum interincisal opening, mouth opening deviation and/or presence of microretrognatia. MRI with contrast of TMJ was done before and after arthroscopy. Activity and damage was evaluated with Cron etal score. Arthroscopy was made under general anesthesia and nasotracheal intubation. We used a 1.9 mm glass lens arthroscope for small joints. If synovitis was detected local electrocoagulation was done. We made local infiltration with 1 ml of triamcinolone hexacetonide and sodic hialuronidate. Results 12 patients were included. Clinical symptoms and imaging score improved after arthroscopy in all patients. We have not complications. Not local relapse have been identified. Conclusions TMJ arthroscopy is a complex technique that allows direct joint examination. Electrocoagulation produces a synovial scar that reduce relapse with low index of complications. This technique seems to be promising in patients with TMJ active synovitis despite of systemic treatment. References Stoll ML, Sharpe T, Beukelman T, Good J, Young D, Cron RQ. Risk factors for temporomandibular joint arthritis in children with juvenile idiopathic arthritis. J Rheumatol 2012;39(9)1880-87 Vaid YN, Dunnavant FD, Royal SA, Beukelman T, Stoll ML, Cron RQ. Imaging of the temporomandibular joint in juvenile idiopathic arthritis. Arthritis Care Res 2014;66(1):47-54 Disclosure of Interest None declared

between onset of the disease and diagnostic was 7.2±5.3 days. Ethnic distribution was: Caucasian 279 patients(69.9%), North African 26 (6.5%), Amerindian 21 (5.2%), Asian 14 (3.5%) and Sub-Saharan 4 (1%). Ethnicity was not available in 55 (13.8%) patients. Distribution of classical manifestations for KD was: fever in 100% of patients, changes in extremities 40.3% (desquamation in 31% of them), exanthema 84.2%, conjunctival injection 79.7%, changes in lips and oral cavity 55.6% and lymphadenopathy 28.8%. Other clinical findings reported were: sterile pyuria in 80(20%) patients, nausea and vomiting in 96(24%), abdominal pain in 85(21.3%), gallbladder distention in 14 (3.5%), transaminase elevation in 120(30%), jaundice in 21(5.1%), irritability in 118(29.5%), aseptic meningitis in 16(4%), sensorineural hearing loss in 2 patients, uveitis in 11(2.7%) and arthritis or arthralgia in 55(13.8%). Cardiologic findings were: perivascular brightness of the coronary wall in 42(10.5%) patients, pericarditis in 9(2.3%), myocarditis in 4(1%), mitral regurgitation in 28 (7%) and CA in 53 patients(13.3%), 26(49%) of them disappearing before the 2 nd month after the onset of KD. 4 patient had giant CA. Intravenous immunoglobulin (IVIG) was administered in 389(97.5%) patients with response to the 1 st dose in 332(83.2%). Day of IVIG administration was 7.5±3.1. Other treatment plans were: 2 nd (69% response) and 3 rd IVIG doses, oral or iv corticosteroids and abciximab (administered in 3 of the patients with giant CA). 97.7% of patients received anti-platelet dose aspirin in the convalescent phase. Conclusion