June 2021 -
Volume 14 Issue 1

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Original Contributon

Original Contributon

Improving the outcome of Sickle Cell disease patients in a resource limited setting Sudan Sickle Cell Anemia Center (SSCAC): a promising and developing experience
Alam Eldin Musa Mustafa, Osman Abelgadir Osman, Niemat Mohammed Tahir
[pdf]
[Abstract]
DOI: 10.5742/MEJIM2020.93792.


A much higher prevalence of chronic obstructive pulmonary disease in males with sickle cell diseases even in the absence of smoking and alcohol
Mehmet Rami Helvaci, Emin Maden, Atilla Yalcin, Orhan Ekrem Muftuoglu, Abdulrazak Abyad,
Lesley Pocock
[pdf]
[Abstract]
DOI: 10.5742/MEJIM2021.93793.

Pulmonary hypertension may not have an atherosclerotic background in sickle cell diseases
Mehmet Rami Helvaci, Zeki Arslanoglu, Atilla Yalcin, Orhan Ekrem Muftuoglu, Abdulrazak Abyad, Lesley Pocock
[pdf]
[Abstract]
DOI: 10.5742/MEJIM2021.93794.

Review

Frailty : Update on Diagnosis Evaluation and Management: Part 1
Abdulrazak Abyad, Sonia Ouali Hammami
[pdf]
[Abstract]
DOI: 10.5742/MEJIM2021.93795

Case Study

Questioning and prying into botulinum toxin after aesthetic treatment
Ebtisam Elghblawi
[pdf]
[Abstract]
DOI: 10.5742/MEJIM2021.93796

 

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Chief Editor:
Ahmad Husari MD FCCP D'ABSM
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Publisher:
Lesley Pocock
medi+WORLD International
AUSTRALIA
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Journal Edition - September 2021, Volume 14, Issue 2

Sickle cell anemia versus sickle cell diseases in adults
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Mehmet Rami Helvaci (1)
Hasan Yilmaz (2)
Atilla Yalcin (1)
Orhan Ekrem Muftuoglu (1)
Abdulrazak Abyad (3)
Lesley Pocock (4)

(1) Specialist of Internal Medicine, MD
(2) Specialist of Ear, Nose, and Throat Diseases, MD
(3) Middle-East Academy for Medicine of Aging, MD
(4) medi-WORLD International

Corresponding author:
Prof Dr Mehmet Rami Helvaci,
07400, ALANYA, Turkey
Phone: 00-90-506-4708759
Email: mramihelvaci@hotmail.com

Received July 2021. Accepted August 2021. Published September 1, 2021.Please cite this article as: Helvaci MR et al. Acute chest syndrome may not have an atherosclerotic background in sickle cell diseases. Middle East J Intern Med 2021; 14(2): 10- 19. DOI: 10.5742/MEJIM2020.93797.


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ABSTRACT

Background: We tried to understand prevalence and clinical severity of sickle cell anemia (SCA) alone or sickle cell diseases (SCD) with associated alpha- or beta-thalassemias in adults.

Methods: All adults with the SCA or SCD were studied.

Results: The study included 441 patients (215 females). The prevalence of SCA was significantly lower than the SCD in adults (29.0% versus 70.9%, p<0.001). The mean age and female ratio were similar in the SCA and SCD groups (31.2 versus 30.5 years and 52.3% versus 47.2%, p>0.05 for both, respectively). The mean body mass index was similar in both groups, too (21.5 versus 21.7 kg/m2, p>0.05, respectively). On the other hand, the total bilirubin value of the plasma was higher in the SCA, significantly (5.7 versus 4.4 mg/dL, p= 0.000). Whereas the total number of transfused units of red blood cells in their lives was similar in the SCA and SCD groups (43.6 versus 37.1 units, p>0.05, respectively).

Conclusion: The SCA alone and SCD are severe inflammatory processes on vascular endothelium particularly at the capillary level, and terminate with an accelerated atherosclerosis and end-organ failures in early years of life. The relatively suppressed hemoglobin S synthesis in the SCD secondary to the associated thalassemias may decrease sickle cell-induced chronic endothelial damage, inflammation, edema, fibrosis, and end-organ failures. The lower prevalence of the SCA in adults and the higher total bilirubin value of the plasma in them may indicate the relative severity of hemolytic process, vascular endothelial inflammation, and hepatic involvement in the SCA.

Key words: Sickle cell anemia, sickle cell diseases, thalassemias, chronic endothelial damage, atherosclerosis, end-organ failure, metabolic syndrome

 

 




 

 

 

 

 

 
 


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