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    Gender differences in severity of sickle cell diseases in non-smokers
    (Professional Medical Publications, 2013) Helvaci, Mehmet Rami; Ayyildiz, Orhan; Gundogdu, Mehmet
    Objective: To find out gender differences in severity of sickle cell diseases (SCDs) in non-smokers. Methods: Three groups of SCDs patients on the basis of red blood cell (RBC) transfusions were included. Less than 10 units in their lives were kept in Group-1, Ten units of higher in Group-2 and 50 units or higher as the Third Group. Patients with a history of using one pack of cigarettes -year or above were excluded. Results: The study included 269 patients. Mean ages of the groups were similar (28.4, 28.5, and 28.9 years, respectively). Prevalences of cases without any RBC transfusion in their lives were 7.2% and 3.7% in females and males, respectively (p < 0.05). Prevalences of cases without any painful crisis were 13.8% and 6.0% in females and males, respectively (p < 0.001). There was progressive increase according to mean painful crises, clubbing, chronic obstructive pulmonary disease (COPD), leg ulcers, stroke, chronic renal disease (CRD), pulmonary hypertension, and male ratio from the first towards the third groups (p < 0.05, nearly for all). Mean ages of mortal cases were 29.1 and 26.2 years in females and males, respectively (p > 0.05). Conclusion: The higher painful crises per year, digital clubbing, COPD, leg ulcers, stroke, CRD, pulmonary hypertension, and male ratio of the third group, lower male ratio of patients without any RBC transfusion, lower male ratio of patients without any painful crisis, lower mean ages of male SCDs patients with mortality, and longer overall survival of females in the world could not be explained by well known strong atherosclerotic effects of smoking alone, instead it may be explained by the dominant role of male sex in life.
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    Left renal atrophy in sickle cell diseases
    (Medi+World Int, 2016) Helvaci, Mehmet Rami; Davran, Ramazan; Davarci, Mursel; Muftuoglu, Orhan Ekrem; Pocock, Lesley
    Background: We tried to understand whether or not there is a difference in occurrence of renal atrophy between the left and right sides in sickle cell diseases (SCDs). Methods: All patients with SCDs were enrolled into the study. Results: The study included 311 patients (153 females). There were seven cases (2.2%) with left renal atrophy against one case (0.3%) with right renal atrophy (p<0.001). Associated thalassemias were detected in 44.0% and splenomegaly in 12.5% of the patients. There was digital clubbing in 6.4%, chronic obstructive pulmonary disease in 4.8%, leg ulcers in 12.8%, stroke in 7.0%, chronic renal disease in 8.6%, pulmonary hypertension in 11.8%, cirrhosis in 3.5%, coronary heart disease in 8.0%, and exitus in 5.7% of the patients. Conclusion: Renal atrophy is significantly higher on the left side in SCDs. Splenomegaly induced flow disorders in left renal vessels, structural anomalies of the left renal vein including nutcracker syndrome and passage behind the aorta, and possibly the higher arterial pressure of left kidney due to the shorter distance to heart as an underlying cause of endothelial damage induced atherosclerosis, may be some of the possible causes. Because of the higher prevalences of left varicocele probably due to drainage of left testicular vein into the left renal vein, high prevalences of associated thalassemias with SCDs as a cause of splenomegaly, and tissue ischemia and infarctions induced edematous splenomegaly in early lives of the SCDs cases, splenomegaly induced flow disorders of left renal vein may be the most significant cause among them.
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    Red blood cell supports in severe clinical conditions in sickle cell diseases
    (Medi+World Int, 2016) Helvaci, Mehmet Rami; Atci, Nesrin; Ayyildiz, Orhan; Muftuoglu, Orhan Ekrem; Pocock, Lesley
    Background: Sickle cell diseases (SCDs) are accelerated atherosclerotic processes. We tried to understand whether or not there is a prolonged survival with the increased number of red blood cells (RBC) transfusion in the SCDs. Methods: As one of the significant endpoints of the SCDs, cases with chronic obstructive pulmonary disease (COPD) and without, were collected into the two groups. Results: The study included 428 patients (221 males). There were 71 patients (16.5%) with COPD. Mean age was significantly higher in the COPD group (32.8 versus 29.8 years, P=0.005). Male ratio was significantly higher in the COPD group, too (78.8% versus 46.2%, P<0.001). Smoking (35.2% versus 11.4%, P<0.001) and alcohol (7.0% versus 1.9%, P<0.01) were also higher among the COPD cases. Beside these, priapism (14.0% versus 3.0%, P<0.001), HCV RNA positivity (2.7% versus 0.5%, P<0.05), cirrhosis (8.4% versus 3.3%, P<0.05), leg ulcers (23.9% versus 12.0%, P<0.01), digital clubbing (25.3% versus 6.7%, P<0.001), coronary heart disease (23.9% versus 13.7%, P<0.05), chronic renal disease (15.4% versus 7.0%, P<0.01), stroke (16.9% versus 8.1%, P<0.01), and mean transfused RBC units in their lives (63.8 versus 33.0, P=0.003) were all higher among the COPD cases. This was probably due to the higher number of transfused RBC units; the mean age of mortality was also higher in the COPD group, significantly (38.3 versus 30.4 years, P=0.04). Conclusion: SCDs are chronic catastrophic processes on vascular endothelium terminating with accelerated atherosclerosis induced endorgan failures in early years of life. RBC supports in severe clinical conditions probably prolong survival of the patients.