The effect of measurement area size on the reliability of myocardial iron load measurement in cardiac magnetic resonance imaging examinations of thalassemia patients.

Background/aim: The aim of this study was to evaluate the intraobserver and interobserver reliability of cardiac T2* MRI measurements in different region of interest (ROI) sizes.

Materials And Methods: Cardiac T2* MRIs of 24 thalassemia major patients were evaluated. Two different ROI sizes were used for measurement.

β2-Microglobulin, Neutrophil Gelatinase-Associated Lipocalin, and Endocan Values in Evaluating Renal Functions in Patients with β-Thalassemia Major.

Chronic anemia, transfusion-associated iron deposition, and chelating agents lead to renal impairment in β-thalassemia (β-thal) patients. The present study aimed to determine the most reliable and practical method in assessing and predicting renal injury in β-thal major (β-TM) patients. Therefore, we assessed the predictive values of urine β2-microglobulin (β2-MG) and neutrophil gelatinase-associated lipocalin (NGAL) levels, their ratios to urine creatinine, and serum endocan level.

Retrospective evaluation of children with immune thrombocytopenic purpura and factors contributing to chronicity.

Objective: Immune thrombocytopenic purpura (ITP) is the most common cause of acquired thrombocytopenia children. The aim of this retrospective study is to describe presenting features and clinical characteristics of ITP and evaluate clinical course, treatment modalities, and complications and determine the effects of preceding infection history, age, gender, treatment modality, and admission platelet count on chronicity.

Method: Two hundred and eleven patients who were diagnosed ITP and followed-up in Department of Pediatric Hematology, Ankara Children Hematology Oncology Education and Research Hospital between January 2008 and September 2012 were included.

The Incidence of Ototoxicity in Patients Using Iron Chelators.

Objective: In this study, we aimed to detect the incidences of ototoxicity in patients with hemoglobinopathies taking deferoxamine (DFO), deferiprone, and deferasirox using the National Cancer Institute (NCI) Common Terminology Criteria for Adverse Events (CTCAE) scale to obtain more objective data.

Materials And Methods: Fifty-five transfusion-dependent patients were evaluated in this study. The NCI CTCAE scale was used to assess ototoxicity levels.

Prophylactic acyclovir and preemptive ganciclovir to prevent cytomegalovirus disease in children after hematopoietic stem cell transplant.

Objectives: The purpose of this study was to evaluate the effectiveness of acyclovir prophylaxis and preemptive ganciclovir treatment in preventing cytomegalovirus disease in children who underwent hematopoietic stem cell transplant.

Materials And Methods: We reviewed the clinical records of 66 children (36 boys, 30 girls; mean age, 9 ± 5 y; age range, 2-20 y) who underwent hematopoietic stem cell transplant at Ankara Children's Hematology and Oncology Hospital, Bone Marrow Transplantation Unit, between April 2010 and March 2012.

Results: In these 66 children, 61 children (92.

Treatment of Priapism with Automated Red Cell Exchange and Hyperbaric Oxygen in an 11-year-old Patient with Sickle Cell Disease.

Priapism affects up to 50% of all males with sickle cell disease, and there is no standard treatment. Delayed and unsuccessful treatment leads to corporal fibrosis and impotence. It is therefore necessary to determine the best treatment methods for this complication in order to offer effective interventions to all affected patients.

Unilateral exudative retinal detachment as the sole presentation of relapsing acute lymphoblastic leukemia.

Ocular findings are rarely the initial symptom of leukemia, although up to 90% of all leukemia patients have fundus changes during the course of the disease. Herein we report a relapsing acute lymphoblastic leukemia patient with thesole presentation of sudden visual loss and exudative retinal detachment. An 8-year-old boy with acute lymphoblasticleukemia developed sudden visual loss during his first remission period.

Adjuvant corticosteroid therapy in hepatosplenic candidiasis-related iris.

Candida infections are the most frequent infections in neutropenic patients. Hepatosplenic candidiasis (HSC) is a part of disseminated Candida infection that occurs most commonly in patients with hematologic malignancies treated with chemotherapy and requires protracted antifungal therapy. During invasive mycosis with rapid resolution of immunosuppression, immune reconstitution inflammatory syndrome (IRIS) which mimics treatment failure, drug toxicity or breakthrough infections may occur.

Deferasirox therapy in children with Fanconi aplastic anemia.

Thirty-nine children with Fanconi aplastic anemia (FAA) have been followed up in our center between January 2008 and November 2010. Eight of these children (20%) with a transfusional iron overload had been undergoing deferasirox treatment during the study period. In the English literature, transfusional iron overload and the use of an iron chelator in children with FAA has not yet been evaluated.

The role of tumor necrosis factor-alpha -308 G/A and transforming growth factor-beta 1 -915 G/C polymorphisms in childhood idiopathic thrombocytopenic purpura.

Objective: To increase our understanding of the etiology of idiopathic thrombocytopenic purpura (ITP) some cytokine gene polymorphisms were analyzed for susceptibility to the disease. The aim of this study was to investigate the role of tumor necrosis factor-alpha (TNF-α) -308 G/A and transforming growth factor-beta 1 (TGF-β1) -915 G/C polymorphisms in the development and clinical progression of childhood ITP.

Methods: In all, 50 pediatric patients with ITP (25 with acute ITP and 25 with chronic ITP) and 48 healthy controls were investigated via LightCycler® PCR analysis for TNF-α -308 G/A and TGF-β1 -915 G/C polymorphisms.

Serum sickness-like syndrome after immunoglobulin M-enriched polyclonal immunoglobulin.

Medication reactions, infectious etiologies, graft vs. host disease, serum sickness, and serum sickness-like reaction are the most common conditions that cause skin fever and rashes in immunosuppressed patients. In addition to this long list of diseases, severity of the primary disease and deterioration in the patient's health status can make the diagnosis difficult.

A different interaction between parathyroid hormone, calcitriol and serum aluminum in chronic kidney disease; a pilot study.

Background/aims: Aluminum (Al) is an ingredient of a variety of foodstuffs and medications as well as of domestic water supplies. The patients with chronic kidney disease (CKD) are more susceptible to bone toxicity of Al. The aim of the study was to investigate the interactions between serum Al, parathyroid hormone (PTH) and active vitamin-D in CKD.

High dose calcitriol in osteopetrorickets.

Osteopetrorickets is a rare autosomal recessive disorder of osteoclast function characterized by abnormally dense bone and failure of resorption of calcified cartilage. Rickets is a paradoxical complication of osteopetrosis, resulting from the inability of the osteoclasts to maintain a normal calcium-phosphorus balance in the extracellular fluid. We report a patient with an unusual case of infantile osteopetro-rickets who was admitted with anterior fontanel bulging and was treated with haploidentical bone marrow transplantation.

Relation of soluble endothelial protein C receptor and cytokines after allogeneic hematopoietic stem cell transplantation.

Aim: The objective of this study was to elucidate the effects of tumor necrosis factor α (TNF-α), interleukin 1β (IL-1β), interleukin 2 (IL-2), interleukin 6 (IL-6), and interleukin 8 (IL-8) on the expression of soluble endothelial protein C receptor (sEPCR) in the pathogenesis of thrombotic complications after hematopoietic stem cell transplantation (HSCT).

Methods: The relationship between plasma concentrations of proinflammatory cytokines (TNF-α, IL-1β, IL-2, IL-6, and IL-8) and sEPCR was evaluated in 32 consecutive allogeneic hematopoietic stem cell-transplanted patients prior to conditioning regimen and randomly once between +5 and +30 days after transplantation and compared these results with 20 healthy controls.

Results: Soluble endothelial protein C receptor levels did not indicate any significant difference between pre- and posttransplantation period, and sEPCR levels showed a significantly negative correlation between IL-6 and IL-8 (sEPCR and IL-6, r = -.

An autoimmune lymphoproliferative syndrome initially diagnosed as Evans syndrome.

Autoimmune lymphoproliferative syndrome (ALPS) is a rare childhood disorder characterized by chronic non-malignant lymphoproliferation and autoimmunity. Patients with ALPS frequently exhibit episodic and intermittent, severe autoimmune- induced hemolytic anemia, thrombocytopenia or combined cytopenias. The co-occurrence of immune-mediated cytopenias, autoimmune thrombocytopenia and autoimmune hemolytic anemia is also known as Evans syndrome.