A5083866058- Platelet Disorders and Treatments
- Immunodeficiency and Autoimmune Disorders
- Blood Coagulation and Thrombosis Mechanisms
- Hemophilia Treatment and Research
- Blood disorders and treatments
- Immune Cell Function and Interaction
- Blood properties and coagulation
- Venous Thromboembolism Diagnosis and Management
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Autoimmune and Inflammatory Disorders Research
- Acute Lymphoblastic Leukemia research
- Porphyrin Metabolism and Disorders
- Cystic Fibrosis Research Advances
- Acute Myeloid Leukemia Research
- Hemoglobinopathies and Related Disorders
- Neurological and metabolic disorders
- Eosinophilic Disorders and Syndromes
- Childhood Cancer Survivors' Quality of Life
- Folate and B Vitamins Research
- Pharmacological Effects and Toxicity Studies
- Respiratory viral infections research
- Gastrointestinal Bleeding Diagnosis and Treatment
- Brucella: diagnosis, epidemiology, treatment
- Chronic Myeloid Leukemia Treatments
- Blood groups and transfusion
Necmettin Erbakan University
2016-2025
Selçuk University
2009-2017
Ege University
2017
Ankara Fizik Tedavi ve Rehabilitasyon Eğitim ve Araştırma Hastanesi
2016
Cukurova University
2015
Istanbul University
2015
Ankara University
2015
Akdeniz University
2015
Konya Food and Agriculture University
2012-2013
Pediatric Nephrology of Alabama
2013
Granulocytic sarcoma (GS), or myeloid chloroma, is a tumoral mass containing myeloblasts and immature granulocytes in an anatomic site other than the bone marrow. GS very rare children with acute promyelocytic leukemia (APL). This case report presents of manifesting as solitary mass. A 15-year-old female presented left knee pain. Complete blood count biochemistry were normal. No blasts early granulocytic elements observed peripheral smear. Magnetic resonance imaging (MRI) revealed 4x4-cm...
Chediak-Higashi syndrome (CHS) is caused by autosomal recessive mutations in LYST, resulting enlarged lysosomal compartments multiple cell types. CHS patients display oculocutaneous albinism and may develop life-threatening hemophagocytic lymphohistiocytosis (HLH). While NK cell-mediated cytotoxicity has been reported to be uniformly defective, variable defects T observed. The latter linked the degree of HLH susceptibility. Since discrepancies cell- cellular might result from differences...
Abstract Background With more than 3.6 million Syrian refugees Turkey hosts the world's largest number of Syrians. Considering morbidity, mortality, and healthcare spending, cancer is one leading health economic burden for patients systems. However, very limited information available in scientific literature to understand characteristics countries hosting refugees. The aim present study evaluate demographic clinical characteristics, treatment outcome living Konya, Turkey. Methods We...
Inborn errors of IFN-γ immunity can underlie tuberculosis (TB). We report three patients from two kindreds without EBV viremia or disease but with severe TB and inherited complete ITK deficiency, a condition associated that renders immunological studies challenging. They have CD4+ αβ T lymphocytopenia concomitant expansion CD4−CD8− double-negative (DN) Vδ2− γδ lymphocytes, both displaying unique CD38+CD45RA+T-bet+EOMES− phenotype. Itk-deficient mice recapitulated an the DN lymphocyte...
Abstract Background CD 3 chain expression defects including gamma, epsilon, delta, and zeta subunits, are autosomal recessive inherited severe combined immunodeficiencies ( SCID ). The phenotype is usually T ‐ B + NK with lymphopenia where the clinical findings may be mild 3γ) or 3δ, ε, ζ) owing to underlying molecular defect. There limited information about disease in literature. Methods Here, we present two siblings from non‐consanguineous family autoimmunity E vans syndrome, autoimmune...
Purpose To determine the prevalence and pattern of ocular involvement in children with leukemia at time diagnosis. Methods The data patients who underwent complete ophthalmic examination diagnosis between January 2005 December 2014 were retrospectively reviewed. Demographic data, type leukemia, findings, blood parameters, duration follow-up analyzed. Results A total 185 (111 male 74 female) included study, a median age 6.0 years (range 0.5-18.0 years) 36.0 months 0.5-108.0 months). Ocular...
Severe congenital neutropenia is a rare disease, and autosomal dominantly inherited ELANE mutation the most frequently observed genetic defect in registries from North America Western Europe. However, eastern countries where consanguineous marriages are common, recessive forms might be more frequent.Two hundred sixteen patients with severe 28 different pediatric centers Turkey were registered.The was HAX1 (n = 78, 36.1%). A heterozygous detected 29 (13.4%) our cohort. Biallelic mutations of...
The patients with CD3γ deficiency can present different clinical findings despite having the same homozygous mutation. We report three new CD3gamma-deficient siblings from a consanguineous family combined T-B+NK+ immunodeficiency and their variable cellular phenotypes mutation of CD3G gene (c.80-1G>C). also re-evaluate previously reported non-consanguineous two median age at diagnosis was 11 years (14 months-20 years). found all five to display autoimmunity: autoimmune thyroiditis (n = 5),...
Posterior reversible encephalopathy syndrome is characterized by hypertension, seizure, headache, clouding of consciousness, and visual disturbance, diagnosed in the presence typical lesions on magnetic resonance imaging. We retrospectively evaluated five patients who were as having posterior followed up Meram Medical Faculty, Pediatric Intensive Care Hematology wards, between January 2010 2014. reviewed demographic clinical data, neuroimaging findings. The primary diseases subjects included...
Objective: Immune thrombocytopenia (ITP) is a rare autoimmune disease and hematologic disorder characterized by reduced platelet counts that can result in significant symptoms, such as bleeding, bruising, epistaxis, or petechiae.The thrombopoietin receptor agonist eltrombopag (EPAG) second-line agent used to treat chronic ITP purpura adults children. Materials Methods:The present retrospective study evaluated the efficacy, safety, side effects of EPAG treatment pediatric patients with acute...
Glanzmann’s thrombasthenia (GT) is an inherited disorder of platelet aggregation, characterized by qualitative and quantitative defect on αIIbβ3 integrin (GpIIb/IIIa), resulting in lifelong bleeding tendency due to defective plug formation. The αIIb gene (ITGA2B) β3 (ITGB3) are closely located at chromosome 17q21.31-32. ITGA2B consist 30 exons encoding α chain, whereas ITGB3 has 15 β chain. Until now, according the Human Gene Mutation Database (HGMD), 138 mutations 101 have been identified....
This study aimed to define the status of juvenile myelomonocytic leukemia (JMML) patients in Turkey terms time diagnosis, clinical characteristics, mutational studies, course, and treatment strategies.Data including laboratory characteristics strategies JMML were collected retrospectively from pediatric hematology-oncology centers Turkey.Sixty-five children with diagnosed between 2002 2016 18 institutions throughout enrolled study. The median age at diagnosis was 17 months (min-max: 2-117...
Heparin-induced thrombocytopenia (HIT) is a well described side effect of heparin therapy. A 12-year-old boy developed deep-vein thrombosis. Risk factors for initial thrombosis are antiphospholipid syndrome and heterozygous mutation prothrombin G20210A. Anticoagulant therapy with warfarin 12 months was effective, but discontinuation after resulted in recurrence Unfractionated (UFH) initiated during the acute period, heparin-induced developed. Transition from UFH to fondaparinux successful...
Essential thrombocythemia (ET) is an extremely rare childhood disorder characterised by clonal expansion of megakaryocytic lineage in bone marrow, leading to a persistent increase the number circulating thrombocytes and thus increased risk for thrombotic haemorrhagic events. The molecular mechanisms ET are not fully understood. Most children with have JAK2 V617F somatic mutation; however, another mutation, involving W L or K substitution at Mpl codon 515, was reported small proportion adult...
Objective: This study aimed to observe the preventive effect of prophylactic treatment on joint health in people with hemophilia (PwH) and investigate importance integration ultrasonographic examination into clinical radiological evaluation joints. Materials Methods:This national, multicenter, prospective, observational included male patients aged ≥6 years diagnosis moderate or severe A B from 8 centers across Turkey between January 2017 March 2019.Patients were followed for 1 year 5 visits...
The plasminogen (Plg) protein is the inactive proenzyme form of plasmin that dissolves fibrin thrombi by a process called fibrinolysis. It has been shown homozygous or compound-heterozygous deficiency this major cause rare inflammatory disease affecting mainly mucous membranes found in different body sites. In study, five individual Turkish patients and nine families with type 1 Plg were investigated for PLG gene mutations. All coding regions mutations screened using denaturing high-pressure...
Abstract Background In patients with acute lymphoblastic leukemia (ALL), the risk of thromboembolism increases due to hemostatic changes secondary primary disease and treatment‐related factors. this multicenter study, we aimed research frequency central nervous system (CNS) thrombosis occurring during treatment, hereditary acquired factors, clinical laboratory features thrombosis, treatment approaches, thrombosis‐related mortality morbidity rates in pediatric ALL patients. Procedure...
Severe combined immunodeficiency (SCID) is an inborn errors of immunity (IEI) disorder characterized by impairment in the development and function lymphocytes could be fatal if not treated with hematopoietic stem cell transplant first 2 years life. There are various diagnostic criteria for SCID among different primary societies. We retrospectively evaluated clinical laboratory findings 59 patients followed up diagnosis at our clinic over past 20 order to develop algorithm that would help...
Although splenic abnormalities are common in patients with lupus, spontaneous rupture of spleen is extremely rare.A 15-year-old boy new-onset Evans syndrome subsequently diagnosed as systemic lupus erythematosus developed during the course his illness. Despite severe thrombocytopenia, he was managed conservatively gradual regression hematoma without further complication.Splenic may occur spontaneously erythematosus. We conclude that conservative treatment be preferred especially...