Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis of childhood. Gastrointestinal (GI) bleeding is one of the major complications of HSP. The blood neutrophil-to-lymphocyte ratio (NLR) is identified as a potentially useful marker of clinical outcome in inflammatory diseases. NLR may be a useful biomarker of GI bleeding in children with HSP, which has a neutrophil-dominated inflammation. The aim of this study was to evaluate NLR in patients with HSP and to investigate the relationship with GI bleeding. The study consisted of 63 HSP patients and 38 age- and sex-matched healthy children. C-reactive protein, white blood cell count, platelet count, mean platelet volume (MPV), hemoglobin level, and NLR were evaluated. Logistic regression analysis and receiver operating characteristic (ROC) analysis were used to determine the variables associated with GI bleeding. NLR and MPV were the only two indicators associated with GI bleeding in HSP in logistic regression analysis. The area under the ROC curve analysis indicated that NLR could be a more efficient potential predictor of GI bleeding in HSP when compared to MPV. This study suggested that higher NLR might predict GI bleeding in HSP.

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.

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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.

Acid Ceramidase Deficiency (Farber disease, FD) is an ultra-rare Lysosomal Storage Disorder that is poorly understood and often misdiagnosed as Juvenile Idiopathic Arthritis (JIA). Hallmarks of FD are accumulation of ceramides, widespread macrophage infiltration, splenomegaly, and lymphocytosis. The cytokines involved in this abnormal hematopoietic state are unknown. There are dozens of ceramide species and derivatives, but the specific ones that accumulate in FD have not been investigated. We used a multiplex assay to analyze cytokines and mass spectrometry to analyze ceramides in plasma from patients and mice with FD, controls, Farber patients treated by hematopoietic stem cell transplantation (HSCT), JIA patients, and patients with Gaucher disease. KC, MIP-1α, and MCP-1 were sequentially upregulated in plasma from FD mice. MCP-1, IL-10, IL-6, IL-12, and VEGF levels were elevated in plasma from Farber patients but not in control or JIA patients. C16-Ceramide (C16-Cer) and dhC16-Cer were upregulated in plasma from FD mice. a-OH-C18-Cer, dhC12-Cer, dhC24:1-Cer, and C22:1-Cer-1P accumulated in plasma from patients with FD. Most cytokines and only a-OH-C18-Cer returned to baseline levels in HSCT-treated Farber patients. Sphingosines were not altered. Chitotriosidase activity was also relatively low. A unique cytokine and ceramide profile was seen in the plasma of Farber patients that was not observed in plasma from HSCT-treated Farber patients, JIA patients, or Gaucher patients. The cytokine profile can potentially be used to prevent misdiagnosis of Farber as JIA and to monitor the response to treatment. Further understanding of why these signaling molecules and lipids are elevated can lead to better understanding of the etiology and pathophysiology of FD and inform development of future treatments.