Familial Mediterranean fever (FMF) is the most common inherited periodic fever syndrome characterized by recurrent episodes of serositis. Recently, a few studies have suggested that FMF is related to increased risk of atherosclerosis. Mean platelet volume (MPV) is a marker of platelet activation. Larger platelets are associated with increased atherosclerosis risk. The aim of the study is to evaluate levels of MPV in pediatric FMF patients during and between attacks. The study consisted of 48 patients during an attack (group 1), 63 patients in attack-free period (at least 2 weeks after an attack, group 2), and 49 healthy controls (group 3). Erythrocyte sedimentation rate, C-reactive protein, white blood cell count, platelet count (PLT), and MPV levels were retrospectively recorded from the computerized patient database. Mean platelet volume was significantly lower in FMF patients during attack than in attack-free period (p = 0.00); however, there was no difference among attack-free patients and healthy controls (p = 0.38). The mean platelet counts of FMF patients during attack were higher than the healthy controls (p = 0.02). There was an inverse correlation between MPV and mean PLT in the attack-free period (r = -446, p = 0.01). This study suggests that an early atherosclerosis marker, MPV, is not elevated in pediatric FMF patients on colchicine treatment.

Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children. Gastrointestinal (GI) bleeding is one of the major complications affecting one third of the cases which may cause serious morbidity. Platelet volume directly correlates with the platelet function and activation. Small platelets have lower functional capabilities than larger ones. The aim of this retrospective study was to evaluate levels of mean platelet volume (MPV) in patients with HSP compared with healthy controls and to investigate the relationship between MPV and gastrointestinal bleeding. The study consisted of 43 HSP patients (male/female = 25/18, mean age = 6.2 +/- 2.6 years) and 27 age-matched healthy children (male/female = 14/13, mean age = 6.9 +/- 2 years) as control group. HSP patients had significantly lower MPV levels than healthy controls (7.5 +/- 0.8 vs. 7.9 +/- 0.5, p = 0.027). Thirteen of 43 patients had gastrointestinal bleeding. MPV was significantly lower in patients with GI bleeding than patients without bleeding (7.0 +/- 0.8 vs. 7.7 +/- 0.6, p = 0.01). Platelet counts, white blood cell counts, and C-reactive protein levels were significantly higher in patients with GI bleeding when compared to patients without GI bleeding (p = 0.03, p = 0.004, and p = 0.03, respectively). This study suggests that low MPV may contribute to GI bleeding in HSP.

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of childhood rheumatic diseases, especially systemic onset juvenile idiopathic arthritis (SoJIA). We report a 4-year-old girl with probable SoJIA who presented with MAS. She did not respond to pulse methyl prednisolone and Cyclosporine A (CsA). She also failed to respond to intravenous immunoglobulin (IVIG) therapy. Etanercept was started, based on the observation of increased serum levels of tumor necrosis factor-alpha (TNF-alpha) in patients with MAS. Her condition improved following etanercept, suggesting that etanercept might have a therapeutic role in resistant MAS.

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