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Objective: Cardiac implantable electronic devices (CIED) have been increasingly used in recent years. The incidence of infection has ranged from 0.5 % to 12% in the literature. The purposes of this study was to investigate the frequency of CIED infection and to find the causes of infection. Patients and methods: Totally, 211 patients with CIED infection were retrospectively evaluated. For each patient, all the following data were recorded; age, sex, CIED type, accompanying diseases, complete blood count, serum biochemistry, echocardiographic findings and whether first implantation or replacement. In addition, wound culture, antibiotic regime, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), glomerular filtration rate (GFR) and anticoagulation test results were recorded in infected patients. Results: CIED infection was detected in 18 of the 211 patients (8.5%). 15 cases developed infection following the first implantation (10%), and 3 cases after replacement (5%). Infection was detected within 60 days in 5 patients. CIED pocket cultures were positive in 12 patients (66.7%). 13 of 18 infected devices were removed (72.2%). Diabetes mellitus [Odds Ratio, OR: 4.56 (1.449-14.408)] (p=0.010), male sex [OR: 3.84 (1.034-14.232)] (p=0.045) and increasing age [OR: 0.96 (0.932-0.998)] (p=0.038) were found as significant independent variables on development of CIED; but, pacemaker, implantable cardioverter defibrillator and cardiac resynchronization were not independent variables [OR: 1.66 (0.469-5.929)] (p=0.43). Conclusions: Increasing age, male sex, diabetes mellitus were related to increased frequency of CIED infections. Identification of comorbid conditions prior to CIED implantations may be important in reducing risk of CIED infections. [ABSTRACT FROM AUTHOR]

Ewing sarcoma family of tumor is the second most common bone tumor in children and young adults, after osteosarcoma. Different chemotherapeutic regimens are used in treatment of relapsed/recurrent Ewing sarcoma. Promising results were achieved with combination of irinotecan and temozolomide. Our case was a 13-year-old female with relapsed Ewing sarcoma and after 12 cycles of irinotecan (20 mg/m2/10 days) and temozolomide (100mg/m2/5 days) treatment she had not any complaints, and her radiological lesions remained stable for 66 months. Irinotecan and temozolomide have been used successfully in treatment of relapsed/recurrent Ewing sarcoma, but there is a need for randomized prospective studies with larger patient groups for establishing the efficacy of this treatment protocol. [ABSTRACT FROM AUTHOR]

Wilson disease (WD) is an autosomal recessive disorder of copper transport caused by mutations in the ATP7B gene that encodes a P-type copper ATPase, ATP7B. In WD, a mutated dysfunctional ATP7B leads to a progressive accumulation of Cu in the liver and brain. Clinically, WND shows considerable phenotypic variability including fulminant hepatic failure, hemolysis, chronic liver disease, such as hepatitis and cirrhosis, and neuro-psychiatric disease with or without hepatic involvement. An 18 -year-old female patient who has the diagnosis of Wilson 's disease was referred from outside center for genetic counseling. The mutations p.M1169T was identified in the homozygous form. [ABSTRACT FROM AUTHOR]

MELAS (Mitochondrial Encephalopathy Lactic Acidosis and Stroke like episodes) is a multisystemic muscle disease. Clinical findings are presented with myopathy, eye findings, sensorineural hearing loss, epilepsy, headache, stroke, endocrinopathies. The mutations responsible for the disease are A3243G, T3271C, C3093G, A3252G, C3256T, A3260G, T3291C, T3308C, A13514G, respectively. In this article, six patients with MELAS from a family with different symptoms A3243G mutation are presented to draw attention to the clinical manifestations of the disease. [ABSTRACT FROM AUTHOR]