Search

INTRODUCTION: Main purpose of this study is to evaluate the effects of some genetic modifiers; especially by correcting the imbalance between α/non α chains on clinical severity of Turkish β Thalassemia patients. MATERIALS and METHODS: Eighty-four patients diagnosed as β Thalassemia Major (TM) or β Thalassemia Intermedia (TI) were recruited from pediatric hematology clinics of Dr. Behçet Uz Children's Hospital and Tepecik State Hospital. The clinical and demographic characteristics (including β Thalassemia mutations) of patients were retrospectively reviewed from patients' records. Genetic analysis of patients for Xmn1 Polymorphism and α thalassemia mutations were done at Aegean University, Faculty of Medicine Department of Medical Genetics. RESULTS: Xmnn1 (+/+) genotype, Xmn1 (+/-) genotype, α thalassemia gene deletion, mild β+ alleles were found in 10,1,5,6 of TI patients respectively. One or more positive genetic modifiers were found in 21 patients out of 26 TI (80,8%) patients. Xmnn1 (+/+) genotype, Xmn1 (+/-) genotype, α thalassemia gene deletion, mild β+ alleles were found in 1, 3, 4,5 of TM patients respectively. One or more positive genetic modifiers were found in 11 patients out of 58 TM (18,9%) patients. In the study, all TI patients having β0 mutations had Xmn1 (+/+) genotype. CONCLUSIONS: This study showed that in cases of TI with β+ mutations most common genetic modifier factors are the type of mutation and presence of α thalassemia, while in cases of β0 mutations the most common genetic modifier is the presence of Xmn1 (+/+) genotype. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Dr. Behcet Uz Children's Hospital is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The article focuses on a study on use of acetylsalicylic acid leading to the Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome. Topics discussed include use of hydantoinc resulting in DRESS syndrome, stimulation of the syndrome with reactivation of herpes viruses by factors like human herpes virus 6, and use of drugs such as allopurinol and sulfonamids leading to DRESS syndrome.

Copyright of Journal of Pediatric Infection / Çocuk Enfeksiyon Dergisi is the property of Journal of Pediatric Infection / Cocuk Enfeksiyon Dergisi and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Pediatric cancer patients have an increased risk of potentially life-threatening fungal infections such as Candida parapsilosis, associated with long-term CVADs. The Infectious Diseases Society of America (IDSA) guidelines on Candida catheter-related bloodstream infections recommend systemic antifungal therapy and catheter removal. In this study, we focused on our experience with antifungal failure due to totally implanted catheter-associated C. parapsilosis bloodstream infections. We investigated cases leading to port removal in pediatric malignancy patients and the associated patient outcomes. In the first phase of the study, a retrospective chart review was performed to collect patient information, including primary disease; time from hospitalization to port-related candidemia; antifungal drug choice; and the time at which port removal occurred. During the second phase, antifungal susceptibility tests for C. parapsilosis were performed in our microbiology laboratory. All patients had fevers and were neutropenic at the time of candidemia diagnosis. The mean duration between the first isolation of Candida parapsilosis from the port samples to the port removal was 9.75 ± 5.29 days for 11 patients. Patient fevers lasted for a mean time of 16.22 ± 6.51 days. The median recovery duration from fever after CVC removal was four days (range 2-12 days). The median duration for achieving negative blood cultures, following antifungal treatment was 18 days (range 10-27 days). Our data favored the removal of catheters in the presence of ongoing fever, as suggested by the guidelines, independent of the chosen antifungal treatment. Future studies with large samples are needed to evaluate the effects of catheter removal on mortality rates and patient outcomes. [ABSTRACT FROM AUTHOR]

The aim of this study was to investigate the efficacy of a 21-day schedule of liposomal amphotericin B compared to pentavalent antimony salts in the treatment of patients during a first episode of visceral leishmaniasis. In this study, 17 cases of visceral leishmaniasis admitted to Behçet Uz Children's Hospital between January 2005 and April 2012 were reviewed retrospectively. The study group was composed of 11 males (64.7%) and 6 females (35.3%). One group included 11 patients who were treated with pentavalent antimony salts, sodium stibogluconate or meglumine antimoniate, intramuscularly for 28 days. The second group was treated with amphotericin B intravenously at a dosage of 3 mg/kg on days 1-5, 10 and 21 (a cumulative dose of 21 mg/ kg/day). While pentavalent antimony salts were found to increase biochemical and hematological findings, liposomal amphotericin B was responsible for rapid recovery in fever and shorter hospital stay. As a result, our study shows the advantages of both medications independent of their costs. [ABSTRACT FROM AUTHOR]