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Öğe Anxiety, depression and quality of life in patients with beta thalassemia major and their caregivers(E-Century Publishing Corp, 2014) Yengil, Erhan; Acipayam, Can; Kokacya, Mehmet Hanifi; Kurhan, Faruk; Oktay, Gonul; Ozer, CahitMental health and health related quality of life is commonly affected in patients with chronic problems and their caregivers. In the present study, it was aimed to assess depression and anxiety in patients with beta thalassemia major (BTM) and in their caregivers; and to evaluate effects of these disorders on quality of life. The study was carried out in a district Hereditary Hemoglobinopathy Center and included 88 patients with BTM and 63 of their caregivers. All subjects were assessed using Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI) and Short Form-36 (SF-36) by a trained psychiatry resident via face-to-face interview. The BDI scores were 17 or above in 20.5% of the patients with BTM and 28.6% of their caregivers (P = 0.248). Of the patients with BTM, there were mild anxiety symptoms in 19.3%, while moderate and severe anxiety symptoms in 14.8% and 4.5%, respectively. Anxiety levels were similar between the patients with BTM and their caregivers (P = 0.878). It was found that BDI and BAI scores were negatively correlated to scores of physical health and mental health components of SF-36 in patients with BTM and their caregivers. In linear regression analysis, it was seen that depression affected physical and mental health of the patients with BTM and their caregivers regardless from anxiety. BTM leads an increase in the frequency of depression and anxiety in both patients and their caregivers, and affects negatively physical and mental components of quality of life.Öğe Hemoglobin SE disease in Hatay, in the southern part of Turkey(Mdpi, 2015) Acipayam, Can; Oktay, Gonul; Ilhan, Gul; Curuk, Mehmet AkifDouble heterozygosity for hemoglobin (Hb) E and S, known as HbSE disease, is a rare, clinically benign condition involving mild hemolysis. Only 25 cases have been reported to date. The current literature generally associates HbSE with a benign clinical course, although vaso-occlusive complications have been reported. Although only single case reports were previously available, we have observed 20 cases of HbSE and wish to report them. We examined the records of patients presenting to our hemoglobinopathy center in 2001-2013. High performance liquid chromatography (HPLC) was used for hematological assessment of blood samples with ethylenediaminetetraacetic acid. Eight patients were male and 12 female. Mean hemoglobin electrophoresis values were Hb Al: 6.3%, Hb E 34.5%, HbS: 59.5% and Hb F: 1.9%. Three patients (15%) were symptomatic with vaso-occlusive crisis and one had cerebral stroke. These were siblings. The patient with cerebral stroke was using hydroxyurea. The incidence of HbSE disease is rising due to population admixtures and racial intermarriages. Increased numbers of cases of HbSE have been detected after premarital hemoglobinopathy screening in the Antakya and Cukurova regions of Turkey. The aim of this study was to report large numbers of patients with HbSE diagnosed through the routine HPLC method. The secondary aim was to emphasize that severe vaso-occlusive crisis such as infarction symptoms could be seen in HbSE.Öğe Lipoprotein Subfraction Profile and HDL-Associated Enzymes in Sickle Cell Disease Patients(Springer Heidelberg, 2013) Ozturk, Oktay H.; Can, Yesim; Yonden, Zafer; Motor, Sedat; Oktay, Gonul; Kaya, Hasan; Aslan, MutayAlthough hypocholesterolemia is a reported finding in sickle cell disease (SCD), low-density lipoprotein (LDL)/high-density lipoprotein (HDL) subfractions and HDL-associated enzymes have not been determined in SCD patients. Blood was collected from 38 hemoglobin (Hb)A volunteers and 45 homozygous HbSS patients who had not received blood transfusions in the last 3 months. Serum lipids were measured by automated analyzer while LDL and HDL subfraction analysis was done by continuous disc polyacrylamide gel electrophoresis. Serum levels of cholesteryl ester transfer protein (CETP), lecithin-cholesterol acyltransferase (LCAT), apolipoprotein B (apoB) and apolipoprotein A-1 (apoA-I) were determined by enzyme-linked immunosorbent assay (ELISA). Total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C) and high-density lipoprotein cholesterol (HDL-C) levels were significantly decreased, while TG levels were significantly increased in SCD patients compared to controls. A significant decrease in intermediate-density lipoprotein (IDL)-C, IDL-B, IDL-A and LDL-1 fractions were seen in SCD patients, while no significant difference was observed in small dense LDL particles. A significant decrease was seen in HDL-large, HDL-intermediate and HDL-small fractions in SCD patients versus controls. Levels of LCAT and ApoA-1 protein measured in SCD patients were significantly lower while no significant difference was observed in CETP and ApoB protein levels compared to controls. The reduction observed in LDL- and HDL-C in SCD patients was reflected as significantly decreased IDL, LDL-1 and HDL-subfractions. Decreased HDL subfractions may possibly lead to the reduced ApoA-1 and LCAT protein levels observed in SCD patients.Öğe Mean platelet volume can predict cerebroyascular events in patients with Sickle Cell Anemia(Professional Medical Publications, 2015) Celik, Tanju; Unal, Sule; Ekinci, Ozalp; Ozer, Cahit; Ilhan, Gul; Oktay, Gonul; Arica, VefikObjective: The purpose of this study was to determine the impact of mean platelet volume (MPV) on the frequency and severity of vaso-occlusive and cerebrovascular events in patients with sickle cell anemia (SCA). Methods: The 238 cases diagnosed with SCA were evaluated retrospectively with respect to the occurrence of painful crisis for the previous year. The incidence, severity and type of the vaso-occlusive crises of the patients with SCA between March 2010 and March 2011 were recorded. The last MPV values in patients who were free of erythrocyte transfusion for the last three months and who had no current vaso-occlusive crises were evaluated. All the patients were grouped according to the frequency of the crises for the previous year preceding the data collection. Group 1: 1 to 3 crises, Group 2: 4 to 5 and Group 3: 6 or more crises annually. Results: In accordance with the results obtained during the evaluation of the cases diagnosed with sicklecell anemia, MPV value was found to be significantly higher in patients with cerebrovascular events. Also MPV values increased with increasing incidence of the crises (1=0.297) (p=0.001). Conclusion: One of the contributing factors for this clinical heterogeneity may be related to the MPV values in patients with sickle cell anemia. The higher MPV values may be an early predictor of future cerebrovascular events in patients with sickle cell anemia and may require close follow-up and additional measures.Öğe Neuroimaging Findings in Pediatric Patients with Thalassemia Major(Mdpi, 2022) Akbas, Yilmaz; Aydin, Sultan; Tuncer, Gokcen Oz; Koker, Alper; Coban, Yasemin; Oktay, Gonul; Yeral, HakanBackground: Cranial magnetic resonance imaging (MRI) studies about iron accumulation in children with thalassemia major are quite limited. Aim: This study aimed to detect neurological findings with cranial MRIs in the pediatric patients with thalassemia major who did not develop any neurological complications. Materials and Methods: Pediatric patients with thalassemia major who followed in the Pediatric Hematology Unit between 1 July 2017 and 1 January 2019 were included in the study. The patients underwent cranial MRI scans. Results: A total of 30 patients were included. The median age was 15 (range from 4-18) years old. We found that 7 patients had a splenectomy and 19 of the remaining 23 patients had splenomegaly. In addition, 13 of the patients had hepatomegaly, 10 had skeletal deformities, and 17 had growth retardation. The mean ferritin level was 3772.3 +/- 2524.8. We detected various pathologies on cranial MRI images of 10 (33.3%) patients. In 3 of these patients, millimeter-sized ischemia-compatible lesions were found in the cerebral white matter, which did not fit any arterial area, and 5 patients had hyperintense lesions in the basal ganglia. Conclusion: Our study is valuable since 1/3 of our pediatric patients with thalassemia major were detected with intracranial pathology.Öğe Nocturnal enuresis in sickle cell disease and thalassemia major: associated factors in a clinical sample(Springer Japan Kk, 2013) Ekinci, Ozalp; Celik, Tanju; Unal, Sule; Oktay, Gonul; Toros, Fevziye; Ozer, CahitIn this study, we aimed to investigate the prevalence and associated factors of nocturnal enuresis in sickle cell disease (SCD) and thalassemia major (TM) patients in a single center from Turkey. One hundred and six patients, 51 (48.1 %) with TM and 55 (51.9 %) with SCD, and 80 age-matched healthy controls were included in the study. Semi-structured interviews were conducted with the caregivers of pediatric and adult patients. The interview included questions on nocturnal enuresis and psychosocial variables. Patients' hospital files were reviewed to search for disease-related factors. Twenty-eight of the patients (26.4 %) and three (3.7 %) of the controls had nocturnal enuresis. Younger age, TM diagnosis, family history of nocturnal enuresis and family problems were found to be more frequent in patients with nocturnal enuresis. Among the patients with SCD, frequencies of hospitalization and painful crises were found to be higher in those with enuresis. According to the binary logistic regression analysis, diagnosis of TM (p = 0.031, OR = 0.262) and younger age (p = 0.005, OR = 0.869) were found to be independent risk factors for nocturnal enuresis in the patient group. Nocturnal enuresis is a common problem in children and young adults with TM and SCD. Associated factors in both conditions will be clarified with future studies.Öğe Spectrum of a-thalassemia mutations including first observation of - -FIL deletion in Hatay Province, Turkey(Academic Press Inc Elsevier Science, 2013) Celik, Muhammet Murat; Gunesacar, Ramazan; Oktay, Gonul; Duran, Gulay Gulbol; Kaya, HasanAlpha thalassemia (alpha-thal) is one of the most common genetic disorders in the world. It is characterized by the absence or reduced expression of alpha-globin genes. The frequency of alpha-thal mutations in the province of Hatay in South Turkey is unknown. Therefore, in the present study, we aimed to investigate the spectrum of alpha-thal mutations in this province. Three hundred and nine patients were tested for alpha-thal mutations by using reverse dot blot hybridization technique and nine different mutations were detected in 97 of them. Among the 9 different mutations found, the most frequent mutations were the -alpha(3.7) (43.81%), -alpha 2(-5nt) (6.70%), - -(MED) (5.67%) and alpha 2(Poly) (A2) (2.57%). In the present study, - -(FIL) mutation was detected in a patient for the first time in Turkey. Our results indicated that alpha-thal mutations are highly heterogeneous and -alpha(3.7) is the most prevalent mutation in Hatay province of South Turkey. In addition, - -(FIL) mutation was detected in a patient for the first time in Turkey. This new finding may contribute to the establishment of a national mutation database and genetic counseling. (C) 2013 Elsevier Inc. All rights reserved.Öğe Very high levels of C-reactive protein should alert the clinician to the development of acute chest syndrome in sickle cell patients(Medi+World Int, 2014) Acipayam, Can; Kaya, Sadik; Helvaci, Mehmet Rami; Ilhan, Gul; Oktay, GonulPurpose: Acute chest syndrome (ACS) is associated with both inflammation and tissue ischemia. C-reactive protein (CRP) is a marker of systemic inflammation. The aim of this study was to determine if a relationship exists between CRP and severe ACS. Methods: Forty-three patients with painful crises (range: 4-18 years, mean: 11.4 years) hospitalized between 2012 and 2014, consisting of 23 patients with ACS and 20 patients without ACS (uncomplicated vaso-occlusive crisis) were recruited into this study. Retrospective data were obtained directly from inpatient medical records. ACS was defined as a new pulmonary infiltrate on chest radiograph after admission and before discharge. CRP was measured using a BN II Nephelometer. Results: Mean length of hospital stay of ACS patients was 9.9 days (range 7-18 days) while that of patients without ACS was 5.2 days (range 2-10 days), (p=0.001). In 91% of the ACS cases, ACS developed within the first 72 hours, while the remaining 9% cases were admitted for vaso-occlusive crises but subsequently developed ACS during their hospital stay on the 5th to 7th days. CRP levels on admission were significantly higher in patients with ACS than those without ACS (p=0.001). C onclusion: We investigated CRP in relation to ACS in children with sickle cell disease (SCD). Elevated CRP was determined in all ACS patients with SCD. CRP may be a superior diagnostic marker and herald severe ACS in individuals with SCD.
