scholarly journals High prevalence of Plasmodium falciparum pfcrt K76T mutation in children with sickle cell disease at a tertiary hospital in north-western Tanzania

2014 ◽  
Vol 16 (4) ◽  
Author(s):  
Stella Mongella ◽  
Nizar Enweji ◽  
Naizihjwa Mnong'one ◽  
Mercy Minde ◽  
Erasmus Kamugisha ◽  
...  



2012 ◽  
Vol 14 (3) ◽  
Author(s):  
Stephen E. Mshana ◽  
Alfred Meremo ◽  
Benson R. Kidenya ◽  
Rodrick Kabangila ◽  
Johannes Kataraihya


Hematology ◽  
2014 ◽  
Vol 2014 (1) ◽  
pp. 425-431 ◽  
Author(s):  
Kenneth I. Ataga ◽  
Elizabeth S. Klings

Abstract The increased survival of patients with sickle cell disease (SCD) into adulthood is associated with an increased incidence of multiorgan dysfunction and a progressive systemic and pulmonary vasculopathy. The high prevalence of an elevated tricuspid regurgitant jet velocity and its association with an increased risk of death in adult patients is well established. However, there has been controversy regarding the prevalence of pulmonary hypertension (PH) and its association with mortality in SCD. Multiple recently published reports demonstrate that PH as diagnosed by right heart catheterization is common in adult SCD patients, with a prevalence of 6%–11%. Furthermore, PH is associated with an increased risk of death in SCD patients. In this chapter, we provide evidence for the high prevalence of PH in SCD and its association with mortality and make recommendations for its evaluation and management. Finally, we provide the rationale for screening for this life-threatening complication in adult patients with SCD.



Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3184-3184
Author(s):  
Murtadha K. Al-Khabori ◽  
Anil Pathare ◽  
Khalil Al Farsi ◽  
Mohammed Al Huneini ◽  
Salam Alkindi

Abstract Abstract 3184 Background: Osteonecrosis (ON) of the femoral and humeral heads is frequently seen in patients with sickle cell disease (SCD). Earlier studies reported a high prevalence of thrombophilia in patients with ON. Aims: To study the prevalence of thrombophilia in patients of SCD with ON. Methods: Case records of SCD patients with ON were retrospectively reviewed for protein S, protein C, and anti-thrombin deficiency, along with activated protein C resistance (APCR). Results: A total of sixty-three patients were identified, 35 of whom were males, with a median age of 21 years (range 15to 46). Median haemoglobin, total white cell count and platelet counts were 10 g/dL (range 7.7to 13.3), 7.5 ×109/L (range 3.4 to 16.7) and 302 ×109/L (range 72 to 1101) respectively. Twenty-eight patients were on hydroxyurea. Thrombophilia testing showed that 29% (95% confidence interval: 17–40), 47% (95% CI: 29–64) and 79% (95% CI: 65–93) of the patients had low levels of functional (<60 unit/dl), total antigenic (<70 unit/dl) and free antigenic (<70 unit/dl) protein S respectively, while 21% (95% CI: 10–31) and 67% (95% CI: 46–87) had low levels of functional (<70 unit/dl) and antigenic (<70 unit/dl) protein C respectively. In addition, 14% (95% CI: 5–23) and 22% (95% CI: 0–56) of the patients had low levels of functional (<80 unit/dl) and antigenic (<80 unit/dl) anti-thrombin levels respectively. Only 2% (95% CI: 0–5) of these patients had an abnormally low APCR (APCR ratio ≤2.3). Summary/Conclusions: Patients with SCD and ON have a high prevalence of thrombophilia. These results indicate that a prospective study with more detailed thrombophilia work-up, along with confirmatory DNA analysis, as well as a study of the role of prophylactic anticoagulation in such patients is highly warranted. Disclosures: Pathare: Sultan Qaboos University: Employment, Research Funding. Alkindi:Sultan Qaboos University: Employment, Research Funding.



2007 ◽  
Vol 29 (5) ◽  
pp. 334-337 ◽  
Author(s):  
Stephen C. Nelson ◽  
Beverly B. Adade ◽  
Elizabeth A. McDonough ◽  
Kristin L. Moquist ◽  
Jane M. Hennessy


Author(s):  
Shada Murshed Alharbi ◽  
Jawad Hussain Alshaiti ◽  
Jamila Mofareh Ghazwani ◽  
Afia Mofareh Ghazwani ◽  
Nawaf Mohammed Abushelwah ◽  
...  

The prevalence rates of hereditary anemias in Saudi Arabia are remarkably high when compared to other countries. For instance, estimates show that the prevalence of thalassemia constitutes one of the highest rates globally. Furthermore, it has been demonstrated that epidemiology significantly differs between the different regions across the Kingdom. Therefore, many epidemiological investigations were conducted. In this context, it has been demonstrated that the prevalence of thalassemia ranges from 0.4% to 5.9% in the Northern and Eastern regions, respectively. In the present literature review, we have discussed the different findings of epidemiological studies that studied the epidemiology of hereditary anemias in Saudi Arabia. We mainly discussed the epidemiology of glucose-6-phosphate dehydrogenase deficiency (G6PD), sickle cell disease, and thalassemia. Recent evidence indicates that the trends of β-thalassemia are significantly decreasing over the past years. On the other hand, it has been demonstrated that the prevalence trends of sickle cell disease is constant over the past years. G6PD is also highly prevalent in Saudi Arabia. However, recent evidence is lacking in the literature and needs to be updated by future investigations. Consanguineous marriage has been reported to be an important risk factor for the high prevalence of β-thalassemia and sickle cell disease across the Kingdom.



2020 ◽  
Vol 19 (1) ◽  
Author(s):  
Richard O. Mwaiswelo ◽  
William Mawala ◽  
Per O. Iversen ◽  
Mariane de Montalembert ◽  
Lucio Luzzatto ◽  
...  




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