Assessment of cardiac function in absence of congenital and acquired heart disease in patients with Down syndrome.

BACKGROUND: Extra genetic material in patients with Down syndrome (DS) may affect the function of any organ system. We evaluated cardiac functions using conventional tissue Doppler and two-dimensional speckle tracking echocardiography in patients with DS in the absence of congenital and acquired heart disease in patients. METHODS: A total of 115 patients with DS between 6 and 13 years of age with clinically and anatomically normal heart and 55 healthy children were included in this cross-sectional study. DS was diagnosed by a karyotype test. Patients with mosaic type were not included in this study. Systolic and diastolic functions were evaluated by echocardiography. RESULTS: Pulsed waved Doppler transmitral early/late inflow velocity (E/A), tissue Doppler mitral annular early/late diastolic peak velocity (Ea/Aa), transtricuspid E/A and tricuspid valve annulus Ea/Aa, pulmonary venous Doppler systolic/diastolic (S/D) wave ratio were lower in patients with Down syndrome than in the control group (P=0.04, P=0.001, P<0.05, P<0.001, P<0.001, respectively). Mitral and tricuspid annular Ea were lower in patients with DS (P<0.001). The right and left ventricular myocardial performance indexes were higher in patients with DS than in the controls (P<0.01). They had significantly higher left ventricular mass, ejection fraction, the mitral annular plane systolic excursion values. However, the Down syndrome group compared with the controls had a lower strain values examined by two-dimensional longitudinal speckle-tracking strain echocardiography. CONCLUSION: These findings suggest conventional tissue Doppler and two-dimensional longitudinal speckletracking strain echocardiography were useful methods of investigating ventricular function and identifying a higher incidence of biventricular dysfunction in patients with Down syndrome compared with the healthy controls.

Cardi-O-Fix duct occluder versus Amplatzer duct occluder for closure of patent ductus arteriosus.

BACKGROUND: We sought to investigate the safety, efficacy, and follow-up results of percutaneous patent ductus arteriosus (PDA) closure using the novel Cardi-O-Fix duct occluder (CDO), a device similar to but less expensive than the Amplatzer duct occluder (ADO). We also aimed to compare these two devices in terms of results. METHODS: Between March 2005 and May 2012, 167 patients diagnosed with moderate-to-large PDA underwent transcatheter closure. ADO was used in 56 (33.5%) patients with a mean age of 8.1 ± 11.9 years (3.6 months-56 years), whereas CDO was used in 111 (66.5%) patients with a mean age of 12.6 ± 14.6 years (4.8 months-63 years). RESULTS: The narrowest PDA diameter, the used device diameter, procedure time, fluoroscopy time, and residual shunt rates were similar between the two groups. Procedural success rate was 100% in both groups. Although the residual shunt rate was higher in the CDO group immediately after the procedure, the difference was not statistically significant (12.6 vs. 8.9%; P = 0.3). There was no statistically significant difference between groups at discharge and during follow-up. No deaths occurred in any of the groups, and there were no differences in complication rates during the short- and mid-term follow-up periods (CDO 7/111 vs. ADO 5/56; P = 0.5 π). CONCLUSION: The CDO can be used for PDA closure because of its safety, effectiveness, and simplicity in use. It is available in bigger sizes and can be used in patients with large defects. According to our short- and mid-term findings, the results it yields are similar to those of the ADO; thus, it may be the preferred choice owing to its low cost and large size variability.

Economic impact of juvenile idiopathic arthritis and familial Mediterranean fever.

The aim of the study was to determine the economical impact of juvenile idiopathic arthritis (JIA) and familial Mediterranean fever (FMF) in Turkey. A total of 100 patients (69 F/31 M) with JIA and 100 with FMF (68 F/32 F) who were consecutively seen in the outpatient clinic of the pediatric rheumatology department at Cerrahpasa Medical School between August 2008 and January 2009 were studied. Cost data were collected through a questionnaire filled out by the parents. The mean age (JIA: 11 ± 5 years; FMF:12 ± 4 years) and mean disease duration (JIA:5 ± 3 years; FMF: 4 ± 3 years) of the patients were similar. JIA patients were assigned to 5 subtypes (polyarticular: n = 45, oligoarticular: n = 30, systemic onset: n = 13, psoriatic: n = 6, and enthesopathy-related JIA: n = 6). Forty-nine percent of the patients with JIA were treated with anti-TNF drugs and 61% with DMARDs. All patients with FMF were using colchicine. The total annual cost of JIA (3,994 ± 4,101) was considerably higher than that of FMF (162 ± 77) (P < 0.001). Medication fee was the major determinant of total costs in both diseases constituting 85% in JIA and 39% in FMF. Among the subtypes of JIA, total annual costs were the highest among patients with polyarticular type (6,045 ± 4,078). Medications especially anti-TNF drugs were the major contributor among all determinants of costs in JIA. The low costs of health care system and prominent changes in the health care policies for the last 5 years in Turkey might have played role in our findings.