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Öğe Acute chest syndrome in severity of sickle cell diseases(E-Century Publishing Corp, 2014) Helvaci, Mehmet Rami; Acipayam, Can; Aydogan, Akin; Akkucuk, Seckin; Oruc, Cem; Gokce, CumaliBackground: Sickle cell diseases (SCDs) are chronic inflammatory processes on capillary level. We tried to understand whether or not there are some positive correlations between acute chest syndrome (ACS) and severity of SCDs. Methods: All patients with the SCDs were taken into the study. Results: The study included 337 cases (167 females). There were 15 patients (4.4%) with the ACS. The mean ages were similar in both groups (29.4 versus 29.7 years in the ACS group and other, respectively, P > 0.05). The female ratios were similar in both groups, too (60.0% versus 49.0%, respectively, P > 0.05). Additionally, prevalences of associated thalassemia minors were similar in them (66.6% versus 65.5%, respectively, P > 0.05). Smoking was higher in the ACS group (20.0% versus 13.9%), but the difference was nonsignificant (P > 0.05). Although the mean white blood cell count and hematocrit value of peripheric blood were higher in the ACS group, the mean platelet count was lower in them, but the differences were nonsignificant again (P > 0.05 for all). On the other hand, although the painful crises per year, tonsilectomy, priapism, ileus, digital clubbing, pulmonary hypertension, rheumatic heart disease, cirrhosis, stroke, and mortality were higher in the ACS group, the difference was only significant for the stroke (P < 0.05), probably due to the small sample size of the ACS group. Conclusion: SCDs are chronic destructive processes on capillaries iniatiating at birth, and terminate with early organ failures in life. Probably ACS is one of the terminal consequences of the inflammatory processes that may indicate shortened survival in such patients.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 Autosplenectomy in severity of sickle cell diseases(E-Century Publishing Corp, 2014) Helvaci, Mehmet Rami; Acipayam, Can; Davran, RamazanBackground: We tried to understand whether or not there is an association between prevalence of autosplenectomy and severity of sickle cell diseases (SCDs). Methods: All SCDs patients with red blood cell (RBC) transfusions of less than 50 units in their lives were put into the first group and 50 units or higher were put into the second group. Results: The study included 316 patients (155 females). There were 224 cases (70.8%) in the first group and 92 cases (29.1%) in the second group (p<0.001). The male ratio was significantly higher in the second group (64.1% versus 45.5%, p<0.001). Although both the white blood cell and platelet counts were higher in the second group, there was a significant difference in platelet counts (p=0.005), and this was probably due to the small sample sizes. Although the prevalence of autosplenectomy was significantly higher in the first group (56.2% versus 45.6%, p<0.05), the mean number of painful crises per year, digital clubbing, chronic obstructive pulmonary disease (COPD), leg ulcers, stroke, chronic renal disease (CRD) and coronary heart disease (CHD) were significantly higher in the second groups (p<0.05 for all). Conclusion: In contrast to the lower prevalence of autosplenectomy, the mean number of painful crises per year, digital clubbing, COPD, leg ulcers, stroke, CRD, and CHD were significantly higher in the second group. So there may be an inverse relationship between prevalence of autosplenectomy and severity of SCDs, and spleen may act as a chronic inflammatory focus as a filter of blood for these abnormally hard RBCs.Öğe Duodenal perforation: an unusual complication of sickle cell anemia(African Field Epidemiology Network-Afenet, 2014) Acipayam, Can; Aldic, Guliz; Akcora, Bulent; Celikkaya, Mehmet Emin; Askar, Hasan; Dorum, Bayram AliDuodenal perforation in childhood is a rare condition with a high mortality rate if not treated surgically. Primary gastroduodenal perforation is frequently associated with peptic ulcer and exhibits a positive family history. Helicobacter pylorus is the most significant agent. Secondary gastroduodenal perforation may be a finding of specific diseases, such as Crohn disease, or more rarely may be associated with diseases such as cystic fibrosis or sickle cell anemia. A 14-year-old boy presented with abdominal and back pain. The patient was operated on for acute abdomen and diagnosed with duodenal perforation. Helicobacter pylorus was negative. There was no risk factor to account for duodenal perforation other than sickle cell anemia. Surgical intervention was successful and without significant sequelae. Duodenal perforation is a rare entity described in patients with sickle cell anemia. To our knowledge, this is the first report of duodenal perforation in a patient sickle cell anemia.Öğe The Effectiveness of Enteral Nutrition Support in the Growth of Children Patients with Cancer(Derman Medical Publ, 2016) Acipayam, Can; Bayram, Ibrahim; Yilmaz, Sema; Sezgin, Gulay; Ozkan, Ayse; Tanyeli, AtilaAim: The purpose of this study was to assess, through anthropometric and biochemical parameters, the positive effect on growth of enteral nutrition support in children with cancer receiving chemotherapy. Material and Method: Forty-three consecutive patients newly diagnosed with pediatric malignant disease and receiving intensive chemotherapy were included. Twenty-six patients received an enteral nutrition formula. Seventeen control patients did not receive enteral nutrition formula. Anthropometric parameters (weight, height, body mass index, triceps, subscapular and suprailiac skinfold thickness), serum albumin, prealbumin and transferrin levels and lipid profiles were measured at time of diagnosis and the 3rd month. Results: At the end of 3 months we determined a marked increase in subscapular and suprailiac skin fold thicknesses in the enteral nutrition group compared to at time of diagnosis (p= 0.01 and p= 0.014, respectively). Prealbumin and albumin values increased considerably after 3 months of enteral nutrition formula support (p= 0.005 and p= 0.006, respectively). Weight percentile increment was determined (69.2% of patients) in the enteral nutrition group compared to at time of diagnosis. At 3 months, albumin and suprailiac skinfold thicknesses values were higher in the enteral nutrition group as compared to controls (p= 0.012 and p= 0.017, respectively). There were no significant changes in anthropometric and biochemical parameters in the control group at the end of treatment compared to at time of diagnosis. Discussion: This study demonstrates an improvement in anthropometric and biochemical parameters in children with cancer receiving an enteral nutrition formula.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 Orbital compression syndrome complicated by epidural hematoma and wide cephalohematoma in a patient with sickle cell disease(Mosby-Elsevier, 2014) Ilhan, Nilufer; Acipayam, Can; Aydogan, Fusun; Atci, Nesrin; Ilhan, Ozgur; Coskun, Mesut; Daglioglu, Mutlu CihanOrbital wall infarctions resulting in orbital and epidural hematomas are rare manifestations of sickle cell disease (SCD). We report orbital compression syndrome associated with an epidural hematoma and wide cephalohematoma in a 15-year-old boy with SCD. An infarcted orbital bone was observed on magnetic resonance imaging and three-phase bone scintigraphy with Technetium-99m methylene diphosphonate. The patient recovered completely without surgical intervention at the end of the fourth week. Prompt diagnosis and proper management are critical for complete recovery.Öğe Portal Vein Thrombosis in a Chirrotic Patient with Immune Thrombocytopenic Purpura During Eltrombopag Treatment(Derman Medical Publ, 2016) Ilhan, Gul; Acipayam, CanPortal vein thrombosis (PVT) is a rare but serious complication in liver cirrhosis. Eltrombopag is a new, second generation agent used for immune thrombocytopenic purpura (ITP). It may cause thrombotic events. PVT has been rarely reported as a life threatening complication in some cirrhotic patients during eltrombopag using. We presented 63 years old cirrhotic and immune thrombocytopenic patient who had PVT after eltrombopag.Öğe Relationship between depression, anxiety, quality of life and vaso-occlusive crisis in adolescents with sickle cell disease(Acta Medica Mediterranea, 2014) Ozer, Cahit; Yengil, Erhan; Acipayam, Can; Kokacya, Mehmet HanifiAims: To explore the relationship between frequency of pain episodes and depression, anxiety, and health related quality of life (HRQOL) in adolescents with sickle cell disease (SCD). Materials and methods: Ten males and twelve females, between the ages of 12 and 19, selected during admittance to pediatric hematology outpatients of city hospital with a vaso-occlusive crisis in the period between September 2012 and February 2013, were included in the study. Beck Depression (BDI) and Anxiety Inventories (BAI), and Short Form Health Survey (SF-36) were used to evaluate depression, anxiety and HRQOL. Results: Nine (40.9%) of the adolescents with SCD had BDI scores higher or equal to 17. In the whole group, 3 (13.6%) patients did not have anxiety, while 14 (63.6%) of them had mild and 4 (18.2%) had moderate anxiety. Physical and mental components of HRQOL were not correlated to BDI and BAI scores. Patients with frequent pain episodes (>10 annually) were found to have tendency to depression and anxiety while their mental and physical components of HRQOL were lower than patients with infrequent pain episodes (<5 annually). Conclusion: Depression and anxiety in adolescents with SCD was found relatively high, while Physical and Mental components of HRQOL were relatively low. However, Physical and Mental Health of the patients was not affected from depression and anxiety presence. Pain episode frequency might be the main determinant of higher depression and anxiety and lower HRQOL component scores.Öğe RELATIONSHIP BETWEEN DEPRESSION, ANXIETY, QUALITY OF LIFE AND VASO-OCCLUSIVE CRISIS IN ADOLESCENTS WITH SICKLE CELL DISEASE(Carbone Editore, 2014) Ozer, Cahit; Yengil, Erhan; Acipayam, Can; Kokacya, Mehmet HanifiAims: To explore the relationship between frequency of pain episodes and depression, anxiety, and health related quality of life (HRQOL) in adolescents with sickle cell disease (SCD). Materials and methods: Ten males and twelve females, between the ages of 12 and 19, selected during admittance to pediatric hematology outpatients of city hospital with a vaso-occlusive crisis in the period between September 2012 and February 2013, were included in the study. Beck Depression (BDI) and Anxiety Inventories (BAI), and Short Form Health Survey (SF-36) were used to evaluate depression, anxiety and HRQOL. Results: Nine (40.9%) of the adolescents with SCD had BDI scores higher or equal to 17. In the whole group, 3 (13.6%) patients did not have anxiety, while 14 (63.6%) of them had mild and 4 (18.2%) had moderate anxiety. Physical and mental components of HRQOL were not correlated to BDI and BAI scores. Patients with frequent pain episodes (>10 annually) were found to have tendency to depression and anxiety while their mental and physical components of HRQOL were lower than patients with infrequent pain episodes (<5 annually). Conclusion: Depression and anxiety in adolescents with SCD was found relatively high, while Physical and Mental components of HRQOL were relatively low. However, Physical and Mental Health of the patients was not affected from depression and anxiety presence. Pain episode frequency might be the main determinant of higher depression and anxiety and lower HRQOL component scores.Öğe Screening of Glucose-6-Phosphate Dehydrogenase Deficiency in Cord Blood(Derman Medical Publ, 2014) Acipayam, Can; Orhaner, Betul Biner; Karal, YaseminAim: Glucose-6-phosphate dehydrogenase deficiency is an important factor in etiology of pathologic neonatal jaundice. The aim of this study was to indicate the significance of screening glucose-6-phosphate dehydrogenase deficiency in the cord blood of neonates and the frequency of this deficiency in the etiology of neonatal hyperbilirubinemia. Material and Method: The study was performed consecutive 1015 neonates were included. Five hundred fifty six (54.8%) of them were male and 459 (45.2%) were female. The following parameters were recorded: Gender, birth weight, birth height, head circumference and gestational age. The glucose-6-phosphate dehydrogenase level of neonates were measured with quantitative method in cord blood. Also, hemoglobine, hematocrite, red blood cell count and blood group were measured. The following parameters were recorded in cases with jaundice: exchange transfusion, phototherapy, physiologic and pathologic jaundice, peak bilirubin day, maximum bilirubin level, total bilirubin level at the first day of jaundice, beginning time of jaundice. Results: Enzyme deficiency was detected in 133 (13.1%) of neonates and 76 (57%1 of them were male, 57 (43%)were female. Significant difference was detected in low glucose-6-phosphate dehydrogenase enzyme level with jaundice group for total bilirubin level at the first day of jaundice, maximum total bilirubin level and pathologic jaundice (p<0.05). Discussion: The ratio of glucose-6-phosphate dehydrogenase deficiency was found in Edirne in this study and this ratio was higher than other studies conducted in our country. For this reason, glucose-6-phosphate dehydrogenase enzyme level in cord blood of neonates should be measured routinely and high risk neonates should be followed up for hyperbilirubinemia and parents should be informed in our region.Öğe Tonsilectomy in sickle cell diseases(E-Century Publishing Corp, 2015) Helvaci, Mehmet Rami; Gokce, Cumali; Davran, Ramazan; Acipayam, Can; Akkucuk, Seckin; Ugur, MustafaBackground: We tried to understand whether or not there are lowered prevalences of terminal consequences of sickle cell diseases (SCDs) with tonsilectomy. Methods: All cases with SCDs were taken into the study. Results: The study included 334 patients (164 females). There were 27 cases with tonsilectomy and 307 cases without. The mean ages, female ratios, and prevalences of associated thalassemia minors and smoking were similar in both groups (P>0.05 for all). Although the white blood cell and platelet counts of peripheric blood were higher in patients without tonsilectomy, the mean hematocrit value was lower in them, but the differences were nonsignificant probably due to the small sample size of the tonsilectomy group (P>0.05 for all). Similarly, although the painful crises per year, digital clubbing, leg ulcers, pulmonary hypertension, chronic obstructive pulmonary disease, rheumatic heart disease, avascular necrosis of bone, cirrhosis, stroke, and mortality were higher in cases without tonsilectomy, the differences were nonsignificant probably due to the same reason again (P>0.05 for all). Conclusion: There may be an inverse relationship between prevalence of tonsilectomy and severity of SCDs, and the tonsils may act as chronic inflammatory foci accelerating the chronic endothelial damage all over the body in such patients.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.
