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Öğe Cardiac Tamponade may be the First Symptom of Leukemia(Informa Healthcare, 2014) Karabel, Musemma; Soker, Murat; Kelekci, Selvi; Karabel, Duran; Yel, Servet; Bilici, Meki[Abstract Not Available]Öğe The Clinical Findings and Prophylactic Treatment in Children with Factor X Deficiency(Informa Healthcare, 2013) Karabel, Musemma; Soker, Murat; Yildirim, Aysen Turedi; Oymak, Yesim; Kelekci, Selvi; Karabel, DuranFactor X (FX) is the component of both extrinsic and intrinsic coagulation cascade and is the first enzyme of the common pathway which results in thrombus. Congenital FX deficiency (FXD) is an extremely rare coagulation defect. In this study, we aimed to investigate the clinical and laboratory data of the patients diagnosed with FXD. The files of the 15 patients (7 female, 8 male) diagnosed and followed up for FXD within the last 4 years were evaluated retrospectively. The mean age of the patients was 29 months (min-max: 1-144 months). The most presenting complaints were easy bruisability (n=8; 53%) and epistaxis (n=8; 53%). FX levels were <1% in six patients, 1-5% in four patients, and >5% in five patients. Heparin added-Protrombin Complex was used for prophlaxy (n=11; 73%). Any treatment-related complication was not observed. Heparin-added PCC can be used safely for effective prophlaxy. We suggest that family history is important when considering prophlaxy and in patients with life-threatening bleeding or with FXD sibling the prophlaxy should be introduced in the early course.Öğe The Effects of Pomegranate and Carvacrol on Methotrexate-Induced Bone Marrow Toxicity in Rats(Canadian Soc Clinical Investigation, 2014) Sen, Velat; Bozkuri, Mehtap; Soker, Sevda; Ece, Aydin; Gunes, Ali; Uluca, Unal; Soker, MuratPurpose: The aim of this study was to evaluate the e ff ects of pomegranate (PMG) extract and carvacrol (CARV) on methotrexate (MTX)- induced oxidative stress and bone marrow toxicity. Methods: Wistar albino rats (32 rats) were divided into four groups (n=8): Group 1 was control; Group 2 was given a single intraperitoneal injection of methotrexate (20 mg/kg); Group 3 was treated with carvacrol (73 mg/kg i.p.) one day before MTX (20 mg/kg i.p.) injection; and, Group 4 received a single dose of MTX (20 mg/kg i.p) while PMG was administered orally for seven days at 225 mg/kg. After animals were euthanized, blood samples were taken to evaluate hematological parameters and oxidative stress. In addition, the femur was cropped and bone marrow was extracted for examination. Results: White blood cell count, hemoglobin, hematocrit and platelet count were found to be decreased in the MTX group, but these changes were prevented in the groups that received CARV and PMG. Furthermore, decreased bone marrow cellularity was found in the groups treated with MTX, whereas the PMG and CARV groups had cellularity similar to controls. Strikingly, oxidative stress increased in the MTX group, but was ultimately decreased in the rats that received the antioxidants PMG and CARV. Conclusion: Carvacrol and PMG were found to be protective against methotrexateinduced oxidative bone marrow damage. Use of these antioxidants, in combination with chemotherapeutics, may help to reduce some adverse effects of methotrexate.Öğe Electron Microscopic and Histological Evaluation of the Levator Muscle in Patients with Congenital Blepharoptosis(Sci Printers & Publ Inc, 2016) Soker, Sevda; Cakmak, Sevin; Nergiz, Yusuf; Deveci, Engin; Gul, Mehmet; Soker, Murat; Akkus, MuratOBJECTIVE: To examine the levator muscle in patients with congenital blepharoptosis and to investigate the relationship between those findings and age, gender, and degree of blepharoptosis. STUDY DESIGN: The levator muscles of 13 congenital ptosis patients who had undergone levator muscle resection were examined by light and electron microscopy. During the preoperative period the ptosis amount, levator function, tear function, Bell's phenomenon, and jaw-wink phenomenon were evaluated. RESULTS: There were 9 (69.2%) male and 4 (30.8%) female cases, with an average age of 10.61 +/- 4.77 years (range, 4-19). The quality and quantity of the levator muscle fibrils were assessed by histological examination. There was no relationship detected between histological features of levator palpebralis superior muscle and patients' age and gender (p < 0.05). Patients with a weak levator palpebralis superior muscle had fatty degeneration histologically. Higher levator palpebralis superior muscle function correlated with decreased fatty degeneration and increased skeletal muscle fibrils, but these findings were not statistically significant. There was a reduction in the diameter of muscle fibers and nucleus deformation when muscle function was examined in patients under low-magnification electron microscope. There were lipid droplets of various sizes in some sections, and the mitochondria were seen in mitochondrial crystalline structure depending on cytopathies. In some sections of this group there was large mitochondria and edema formation due to an increase in the mitochondrial matrix. CONCLUSION: More ultrastructural studies in larger populations are needed to support the relationship between structure and function of levator palpebralis superior muscle in patients with congenital blepharoptosis.Öğe Hereditary Spherocytosis: Evaluation of 68 Children(Springer India, 2015) Konca, Capan; Soker, Murat; Tas, Mehmet Ali; Yildirim, RukenTo determine the clinical and hematologic features of 68 children with hereditary spherocytosis (HS). In this retrospective study, we analyzed recorded information of 68 HS patients diagnosed between March 1997 and March 2007, including clinical manifestations at admission, gender, median age at diagnosis, family history, hematologic and biochemical data, patient management, complications, median age of splenectomy, and median follow-up time. Sixty-eight patients with HS (36 male and female) were investigated. The median age at diagnosis was 5.6 years (range 3 months to 18 years). Twenty-seven (39.7 %) had parents with consanguineous marriages, and 20 (29.4 %) had parents with first-degree consanguinity. Predominant clinical manifestations at admission were anemia in 59 patients (86.76 %), splenomegaly in 49 (72.05 %), and jaundice in 33 (48.52 %). Patients were classified as mild, moderate, or severe in 29.4, 61.7, and 8.8 % of patients, respectively. Five patients (7.3 %) underwent splenectomy. Major complications of HS were hemolytic, aplastic, and megaloblastic crises and cholelithiasis in 7 (10.2 %), 1 (1.4 %), 7 (10.2 %), and 6 (8.8 %) of patients, respectively. There were no deaths during follow-up. HS should be considered in evaluating possible diagnoses in patients with hemolytic anemia. In this study, the clinical course of patients with HS was relatively benign, with low proportions of patients having splenectomized and aplastic crises.Öğe Intracranial Haemorrhage in Factor X Deficiency: Report of Five Cases and Review of Literature(Wiley, 2020) Soker, Murat; Uzel, Hulya[Abstract Not Available]Öğe MRI-based evaluation of the factors leading to pituitary iron overload in patients with thalassemia major(Masson Editeur, 2016) Cetincakmak, Mehmet Guli; Hattapoglu, Salih; Menzilcioglu, Sait; Alan, Bircan; Uluca, Unal; Ucar, Ahmet; Soker, MuratAim: Given the lack of studies evaluating pituitary iron overload in patients with thalassemia major, we used magnetic resonance imaging (MRI) to evaluate these patients and the factors affecting the disease process. Materials and methods: The 84 patients with beta-thalassemia major who were included in this study were referred to our clinic for cardiac and hepatic T2* MRI. T2*-weighted images of the pituitary gland, heart, and liver were obtained using a 1.5-tesla MRI unit and a multi-echo gradient-echo sequence. Associations between pituitary T2*, cardiac T2*, hepatic T2*, pituitary height, serum ferritin (SF) level, patient age, and other demographic findings were assessed. Results: Pituitary T2* values correlated with hepatic TZ values, cardiac T2* values, SF level, and patient age (P <= 0.001, 0.001, 0.001, 0.01, respectively) but not with pituitary height (P=0.76). Pituitary and cardiac T2* values were lower in the subset of patients who underwent splenectomy (P=0.046 and P= 0.002, respectively). Conclusion: Pituitary iron overload rapidly increases during puberty and in this study correlated with cardiac and hepatic T2* values, patient age, SF level, and liver size, but not with the height of the pituitary. Pituitary iron overload also increases following splenectomy. Together, these findings indicate that numerous factors contribute to pituitary iron overload. (C) 2016 Elsevier Masson SAS. All rights reserved.Öğe Neurological presentations of nutritional vitamin B12 deficiency in 42 breastfed infants in Southeast Turkey(Tubitak Scientific & Technological Research Council Turkey, 2011) Taskesen, Mustafa; Yaramis, Ahmet; Katar, Selahattin; Gozu, Ayfer; Pirinccioglu, Ayfer Gozu; Soker, MuratAim: Nutritional vitamin B12 deficiency is common in developing and underdeveloped countries and has a wide variety of neurological presentations. The aim of this study was to evaluate the neurological characteristics and laboratory results of infants with vitamin B12 deficiency. Materials and methods: A total of 42 infants were included in this study. All patients were evaluated for clinical, physical, and neurological abnormalities, and an attempt was made to obtain short-term neurologic follow-up. Results: Of 42 patients, 24 (57%) were boys and 18 (43%) were girls. The average age at diagnosis was 13.04 +/- 5.68 months. Most of these infants were breastfed only and born from mothers with inadequate animal-derived protein consumption. Hypotonia (100%), anorexia (92.8%), neurodevelopmental (85.7%), and social (80.9%) retardation were the most present symptoms in all infants. Conclusion: Severe neurological and hematological findings may be found in children with vitamin B12 deficiency. Early diagnosis and treatment is crucial in cases of hematological complications and neurologic impairment. Neurologic impairment may be irreversible if the diagnosis is delayed beyond 12 months. We think that dietary management, such as nutritional support with vitamin B12 for the mothers during pregnancy and complementary food for infants, may prevent the neurological deficits and neurodevelopmental retardation.Öğe Reduced bone mineral density in children and adolescents with hemophilia from east Turkey(Wiley-Blackwell, 2016) Soker, Murat; Aktar, Fesih; En, Velat; Uzel, Hulya; Akpolat, Veysi; Soker, Sevda[Abstract Not Available]Öğe Urinary early kidney injury molecules in children with beta-thalassemia major(Taylor & Francis Ltd, 2015) Sen, Velat; Ece, Aydin; Uluca, Unal; Soker, Murat; Gunes, Ali; Kaplan, Ibrahim; Tan, IlhanBackground: The aim of this study was to investigate novel urinary biomarkers including N-acetyl-beta-D-glucosaminidase (NAG), neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), and liver-type fatty acid binding protein (L-FABP) in children with beta-thalassemia major (beta-TM). Materials and methods: Totally, 52 patients (29 boys, 23 girls) with beta-TM and 29 healthy controls (3-17 years) were included. Various demographic characteristics and blood transfusions/year, disease duration, and chelation therapy were recorded. Serum urea, creatinine, electrolytes, and ferritin and urinary creatinine, protein, calcium, phosphorus, sodium, potassium, and uric acid in first morning urine samples were measured and estimated glomerular filtration rate (eGFR) was calculated. Routine serum and urinary biochemical variables, urinary NAG to Creatinine (U-NAG/Cr), U-NGAL/Cr, UKIM-1/Cr, and UL-FABP/Cr ratios were determined. Results: Patients had similar mean serum urea, creatinine and eGFR levels compared with controls (p > 0.05 for all). The mean urinary protein to creatinine (U-Protein/Cr) ratio was significantly higher in patients compared to the healthy subjects (0.13 +/- 0.09 mg/mg and 0.07 +/- 0.04 mg/mg, respectively; p < 0.001). Significantly increased U-NAG/Cr (0.48 +/- 0.58 vs. 0.23 +/- 0.16, p = 0.026) and U-NGAL/Cr (22.1 +/- 18.5 vs. 11.5 +/- 6.17, p = 0.01) ratios were found in beta-TM patients compared with healthy controls. However, no differences were found in serum and urinary electrolytes or UKIM-1/Cr and UL-FABP/Cr ratios between patients and controls (p > 0.05). Significant correlations were found between urinary biomarkers and urinary electrolytes (p < 0.05). Conclusions: Our results suggest that urinary NAG and NGAL may be considered to be reliable markers to monitor renal injury in beta-TM patients.Öğe Urinary levels of early kidney injury molecules in children with vitamin B12 deficiency(Soc Argentina Pediatria, 2016) Gunes, Ali; Aktar, Fesih; Tan, Ilhan; Soker, Murat; Uluca, Unal; Balik, Hasan; Mete, NuriyeThe aim of this study was to investigate urine early kidney injury molecules, including human kidney injury molecule-1 (KIM-1), liver-type fatty-acid binding protein (L-FABP), N-acetyl-b-D-glucosaminidase A (NAG), and neutrophil gelatinase-associated lipocalin (NGAL) in children with vitamin B12 (cobalamin) deficiency (CD). Twelve children with vitamin B12 deficiency and 20 healthy matched controls were included. Hematologic parameters, serum urea, creatinine (Cr), electrolytes, B12 and folate levels were recorded. Estimated glomerular filtration rate (eGFR) was calculated. Urine protein, electrolytes, and urinary early markers were measured. Patients with CD had significantly higher urine electrolyte/Cr ratios (p < 0.05). Significantly higher urinary KIM-1/Cr, L-FABP/Cr, NAG/Cr and NGAL/Cr were found in CD group (p < 0.05). Significant negative correlations were found between levels of serum B12 and urinary markers in the patients (p < 0.05). Increased urinary kidney injury molecules and electrolytes in children with B12 deficiency suggest a possible subclinical renal dysfunction, which cannot be determined by conventional kidney function tests.