QT and JT dispersion in children with familial mediterranean fever

Objectives: This study aims to determine QT dispersion and JT dispersion, and their relationship with conventional echocardiography values in a group of children with familial Mediterranean fever (FMF). Patients and methods: The study included 48 FMF patients (26 males, 22 females, mean age 11.10±3.42 years; range 5 to 18 years) as the FMF patients and 31 healthy children (17 males, 14 females, mean age 9.61±2.83 years; range 5 to 17 years) as the healthy controls. Electrocardiography and conventional echocardiography were performed on the FMF patients and healthy controls. Both groups were evaluated with a standard 12-lead electrocardiography. QT, JT and RR distances were measured in both groups. The corrected QT (QTc) and corrected JT (JTc) were calculated. QTcd and corrected JT dispersion (JTcd) were detected. Results: There was no statistically significant difference between the FMF patients and healthy controls in terms of RR, QT, QTd, QTcd, JT, JTc, JTd, and JTcd measurements and echocardiography parameters. QTc value was higher in the FMF patients than the healthy controls. Conclusion: QTc value indicates increased ventricular sensitivity and is an important marker of cardiovascular mortality. It has an important effect on sudden cardiac death and arrhythmia. Our study results suggest that electrocardiographic monitoring may be useful in patients with FMF

Cardiovascular disease risk assessment in patients with familial Mediterranean fever related renal amyloidosis

Chronic inflammation and proteinuria is a risk factor for cardiovascular disease (CVD) in patients with chronic kidney diseases and rheumatologic disorders. Our aim was to investigate the CVD events (CVDEs) and survival between the patients with FMF-related AA amyloidosis and glomerulonephropathies (GN) to define possible predictors for CVDEs. A prospective follow-up study with FMF-amyloidosis and glomerulonephropathy (GN) was performed and patients were followed for CVDEs. Flow-mediated dilatation (FMD), FGF-23, serum lipid, hsCRP levels, BMI and HOMA were assessed. A Cox regression analysis was performed to evaluate the risk factors for CVDEs. There were 107 patients in the FMF-amyloidosis group and 126 patients with GN group. Forty-seven CVDEs were observed during the 4.2-years follow up; all 28 patients in the FMF-amyloidosis group and 14/19 patients with GN developed CVDEs before the age of 40 (p = 0.002). CVD mortality was 2.8 times higher (95% CI 1.02–7.76) in patients with FMF-amyloidosis. Across both groups, FMD and FGF23 (p < 0.001) levels were independently associated with the risk of CVDEs. Patients with FMF-amyloidosis are at increased risk of early CVDEs with premature mortality age. FGF 23, FMD and hsCRP can stratify the risk of early CVD in patients with FMF-related AA amyloidosis

Nummular keratopathy in a patient with Hyper-IgD Syndrome

<p>Abstract</p> <p>Purpose</p> <p>To report a case of recurrent nummular keratitis in a pediatric patient with Hyperimmunoglobulinemia D syndrome.</p> <p>Methods</p> <p>A retrospective chart review.</p> <p>Results</p> <p>A 14-year-old boy with Hyperimmunoglobulinemia D syndrome (HIDS) presented with photophobia and ocular irritation concomitant with disease exacerbation. He was found on exam to have significant nummular keratitis, which responded to a short course of topical steroids. Despite acute response to local immunosuppression, the patient had several recurrent attacks and eventually developed a large corneal scar and decreased vision. After initiation of infliximab therapy his ocular sequelae improved dramatically and his vision returned to 20/20.</p> <p>Conclusion</p> <p>One possible form of end-organ damage associated with HIDS is vision threatening nummular keratopathy.</p

{\phi}^4 Solitary Waves in a Parabolic Potential: Existence, Stability, and Collisional Dynamics

We explore a {\phi}^4 model with an added external parabolic potential term. This term dramatically alters the spectral properties of the system. We identify single and multiple kink solutions and examine their stability features; importantly, all of the stationary structures turn out to be unstable. We complement these with a dynamical study of the evolution of a single kink in the trap, as well as of the scattering of kink and anti-kink solutions of the model. We see that some of the key characteristics of kink-antikink collisions, such as the critical velocity and the multi-bounce windows, are sensitively dependent on the trap strength parameter, as well as the initial displacement of the kink and antikink