Cholecystitis and nephrotic syndrome complicating Epstein-Barr virus primary infection.

Epstein-Barr virus (EBV) infection results in a spectrum of clinical manifestations. The host immune response to EBV plays a key role in the extent and degree of clinical features, which in children under 4 years of age are usually mild, non-specific and self-limiting. A 2-year-old boy in whom no known immune disorder could be found presented with acute acalculous cholecystitis, renal dysfunction with massive proteinuria, ascites, pleural effusion, minimal peripheral oedema and a severe systemic inflammatory response. Improvement occurred after initiation of corticosteroids and antiviral treatment with gancyclovir. In severely symptomatic or complicated EBV infection, a primary immunodeficiency must be suspected. If a primary immunodeficiency has been ruled out, the correct management of severe EBV infection in the immunocompetent host remains controversial.

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

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.

Non-systemic juvenile idiopathic arthritis outcome after reaching clinical remission with anti-TNF-α therapy: a clinical practice observational study of patients who discontinued treatment.

TNF-alpha-blocking agents (anti-TNF) used in juvenile idiopathic arthritis (JIA) are well established; however, time to withdraw is unclear. Neither prolonged nor tapering treatment seems to influence risk of relapse. Our aim was to assess relapse percentage after anti-TNF withdrawal of our non-systemic JIA patients after reaching clinical remission. A retrospective review of our non-systemic JIA patients in whom anti-TNF had been withdrawn due to inactive disease was achieved, between December 2000 and November 2011. We analyzed percentages of relapse according to JIA categories and antinuclear antibodies (ANA) positivity. n = 18 patients were included. Eighty-two percentage of patients relapsed after treatment withdrawal, and mean time to relapse was 3.04 months (SD 2.03). The percentage of relapse after anti-TNF discontinuation in the main JIA category was 88 % of negative rheumatoid factor polyarticular JIA and 80 % of persistent oligoarticular JIA. We did not find significant statistical differences according to ANA positivity (9 of 14 were ANA positive), and mean time to relapse (days) was 85.0 (SD 69.4) for ANA-positive versus 102.4 (SD 47.7) for ANA-negative patients (p = NS). Relapse percentage following anti-TNF discontinuation was high (82 %) and occurred within the first 3 months after it. No relationship regarding JIA subtype and ANA positivity was found.

Clinical risk factors are more relevant than respiratory viruses in predicting bronchiolitis severity.

BACKGROUND: The role of respiratory viruses in the pathogenesis of bronchiolitis was re-evaluated with the use of molecular methods such as PCR for virus detection. Whether specific viruses or the classical clinical risk factors are more important in determining severe bronchiolitis is not well established. AIM: To analyze the specific viruses and clinical variables that can predict severe bronchiolitis at admission. METHODS: Nasopharyngeal aspirates were prospectively collected from 484 children <12 months admitted to the pediatrics ward or PICU at Universitary Hospital Sant Joan de Déu (Barcelona, Spain) for bronchiolitis from October 2007 to October 2008. Clinical and demographic data were collected. Sixteen respiratory viruses were studied using PCR. Severity was assessed with a bronchiolitis clinical score (BCS). RESULTS: Four hundred ten infants that tested positive for respiratory viruses were analyzed. Mixed viral infections did not increase the severity of the disease. Rhinovirus was associated with severe BCS in univariate analysis (P = 0.041), but in the multivariate logistic regression including viruses and clinical data only bronchopulmonary dysplasia (OR 7.2; 95% CI 1.2-43.3), congenital heart disease (OR 4.7; 95% CI 1.1-19.9), prematurity (OR 2.6; 95% CI 1.3-5.1), and fever (OR 1.8, 95% CI 1.1-3.1) showed statistical significance for predicting severe BCS. CONCLUSIONS: Classical clinical risk factors have more weight in predicting a severe BCS in infants with acute bronchiolitis than the involved viruses.