Joan Calzada-Hernández

Incidence and clinical features of kawasaki disease in Catalonia (Spain)

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

Disseminated Tuberculosis Resulting From Reinfection in a Pediatric Patient Sequentially Treated With Etanercept and Adalimumab

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.

Pediatric cryoglobulinemic vasculitis successfully managed with rituximab

Cryoglobulinemia is a small and medium vessel vasculitis due to deposit of immune-complexes containing cryoglobulins. It could be associated with malignancy, viral infections, and rheumatic diseases (RamosCasals, Stone, Cid, & Bosch, 2012). Rituximab, anti-CD20 monoclonal antibody, has demonstrated to be a good clinical option for severe cryoglobulinemic vasculitis in adult patients (Ferri et al., 2011; Terrier et al., 2012). A 10-year-old girl presented with palpable purpura and skin ulcers at lower limbs was transferred to our center for ongoing care. There was neither family nor personal background of autoimmune disorders. She had a history of intermittent fever, abdominal pain, vomits, and paresthesia on lower limbs. She appeared cachectic. Skin ulcers of lower limbs were present with severe involvement on both heels (Figure 1a,b). Leukocytoclastic vasculitis without small vessel thrombosis had been detected on skin biopsy. A series of laboratory investigations were performed detecting cryocrit composed by monoclonal immunoglobulin (Ig) M kappa and polyclonal IgG with rheumatoid factor (RF) activity (180 UI/L). With clinical suspicious of cryoglobulinemic vasculitis, studies were extended to rule out potential infectious, neoplastic, and systemic etiologies (Table 1). We detected isolated antinuclear antibodies (1/160) with no extractable nuclear antigen specificity. Neurological assessment demonstrated mononeuritis of the right peroneal

Efficacy and safety of TNF-alpha antagonists in children with juvenile idiopathic arthritis who started treatment under 4 years of age

The experience in the use of tumour necrosis factor (TNF) antagonists in children below 4 years is limited, although there are some trials in the literature which support safety and efficacy under this age.

Prospective analysis of Kawasaki disease cases in Catalonia (Spain) from March 2013 to March 2014.

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 <5 years old (yo). In Madrid (Spain) a retrospective study with no well defined reference area showed an incidence of 15.1/105 children <5yo.

Diagnosis of tuberculosis infection in pediatric patients treated with inhibitors of the tumour necrosis factor alpha. A multicenter national study comparing tuberculin skin test and igra tests

In the last years, inhibitors of tumor necrosis factor alpha (antiTNFα) have been a major advance in the treatment of many rheumatic diseases and inflammatory bowel disease, also in the pediatric patient. However, antiTNFα use is associated with an increased risk of serious infections, including tuberculosis (TB). In adults, the new interferon γ release assays (IGRA) tests for the diagnosis of TB infection appear to show better sensitivity and specificity than the tuberculin skin test (TST) in these patients. Data in children are still very scarce. We have previously reported a latent tuberculosis infection (LTI) prevalence rate of 1.4% (95%CI: 0-2.9) in children on antiTNFα treatment, which is similar to that reported in healthy pediatric population studies in Spain. LTI was diagnosed in 3 adolescent girls (out of 221 patients) in whom QTF tested positive, while TST was positive in only one of them.

Patient With iDEND Syndrome–Related Mutation

Mutations on the ATP-sensitive K+ (KATP) channel are the most common cause of permanent neonatal diabetes. Heterozygous gain-of-function mutations in KCNJ11 and ABCC8, encoding respectively for the Kir6.2 and sulfonylurea receptor 1 (SUR1) subunit of the KATP channel, account for the majority of cases of permanent neonatal diabetes (1). Kir6.2 is expressed in the pancreatic β-cell, but also in skeletal muscle, peripheral nerves, and especially in the brain. Specific KCNJ11 mutations also are associated with developmental delay and epilepsy, known as developmental delay, epilepsy, and neonatal diabetes (DEND) syndrome, or in milder forms, intermediate DEND (iDEND). Sulfonylureas improve KATP channel function in the pancreatic β-cell and also in neuronal and muscle cells, and could potentially play a role in the treatment and/or prevention of such neurologic manifestations. Our patient has a KCNJ11 mutation associated with iDEND syndrome and was successfully switched to glyburide early. The patient …

PReS-FINAL-1014: Role of MHC class I overexpression on muscle biopsy of patients with juvenile dermatomyositis

Juvenile Dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in childhood, a systemic vasculopathy that usually affects skin and skeletal muscle but also can affect gastrointestinal tract and other organs. Diagnosis is based on Bohan and Peters criteria and the goals of treatment include control of skin and muscle symptoms and prevention of disease complications. Stepwise aggressive treatment decreases JDM activity and improves long-term outcome. Muscle involvement in JDM can be assessed by electromyography (EMG), magnetic resonance imaging (MRI) and/or muscle biopsy. Muscle biopsy assesses the presence of lymphocytic inflammatory infiltrate, perifascicular atrophy, muscle fibers necrosis and it allows studying the overexpression of major histocompatibility complex (MHC) class I in the sarcolemma and sarcoplasm of the muscle cell. In healthy muscles, there is no MHC class I expression but in inflammatory myopathies there is a distinctive and generalized overexpression, not limited to the affected areas, it appears before the inflammatory infiltrate occurs and it is not modified by immunosuppressive treatment.

PReS-FINAL-2265: Tuberculosis in pediatric patients who are receiving anti-TNF agents

Adult patients receiving anti-TNFα treatment are at increased risk for developing tuberculosis (TB). Few data have been published in the pediatric population.