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Gender differences in severity of sickle cell diseases in non-smokers
Mehmet Rami Helvaci1, Orhan Ayyildiz2, Mehmet Gundogdu3
1Mehmet Rami Helvaci, MD, Assoc. Prof. of Internal Medicine, Medical Faculty of the Mustafa Kemal University, Antakya, Turkey.
Insights
This study found that males with sickle cell disease (SCD) experience more severe symptoms and complications than females, regardless of smoking status. These findings suggest a significant role of sex in SCD progression and outcomes.
Area of Science:
- Hematology
- Genetics and Heredity
- Clinical Medicine
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with significant morbidity and mortality.
- Gender-based differences in disease severity and progression are increasingly recognized across various chronic conditions.
- Investigating these differences in SCD, particularly in non-smokers, is crucial for understanding disease pathophysiology and tailoring treatments.
Purpose of the Study:
- To investigate potential gender differences in the severity of sickle cell disease (SCD) among non-smoking patients.
- To explore how red blood cell (RBC) transfusion history correlates with gender and disease severity indicators.
Main Methods:
- A cohort of 269 non-smoking sickle cell disease patients was categorized into three groups based on cumulative red blood cell (RBC) transfusion history (Group 1: <10 units, Group 2: 10+ units, Group 3: 50+ units).
- Data collected included prevalence of RBC transfusions, painful crises, and various clinical complications such as clubbing, chronic obstructive pulmonary disease (COPD), leg ulcers, stroke, chronic renal disease (CRD), and pulmonary hypertension.
- Statistical analyses were performed to compare gender-specific outcomes across the transfusion groups.
Main Results:
- Females had a higher prevalence of no RBC transfusions (7.2% vs. 3.7%) and no painful crises (13.8% vs. 6.0%) compared to males (p<0.05 and p<0.001, respectively).
- A progressive increase in the prevalence of painful crises, clubbing, COPD, leg ulcers, stroke, CRD, pulmonary hypertension, and a higher male ratio was observed from Group 1 to Group 3 (p<0.05 for most).
- Mean age at mortality was similar between genders (29.1 years for females vs. 26.2 years for males, p>0.05).
Conclusions:
- The study indicates that males with SCD experience more severe disease manifestations and complications compared to females, even in the absence of smoking.
- The observed disparities in disease severity, transfusion requirements, and complication rates suggest a significant influence of biological sex on SCD pathogenesis.
- These findings highlight the need to consider gender as a critical factor in managing sickle cell disease and potentially in future therapeutic strategies.
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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