scholarly journals Vitamin D level, lipid profile, and vitamin D receptor and transporter gene variants in sickle cell disease patients from Kurdistan of Iraq

Author(s):  
Abdalla Hussein Hama ◽  
Ebrahim Shakiba ◽  
Zohreh Rahimi ◽  
Mehran Karimi ◽  
Hadi Mozafari ◽  
...  
2015 ◽  
Vol 05 (01) ◽  
pp. 24-33
Author(s):  
E. Leila Jerome Clay ◽  
Alison Motsinger-Reif ◽  
Janelle Hoskins ◽  
Lindsay Veit ◽  
Ali Calikoglu ◽  
...  

2021 ◽  
Author(s):  
Abdalla Hussein Hama ◽  
Ebrahim Shakiba ◽  
Zohreh Rahimi ◽  
Mehran Karimi ◽  
Hadi Mozafari ◽  
...  

Abstract Background. Sickle cell disease patients are susceptible to the development of vitamin D deficiency. Vitamin D through binding to vitamin receptor (VDR) exerts its function and affects gene transcription in target tissues. Also, VDR variants could affect bone mineral density. Methods. In a case-control study 101 sickle cell disease patients including 61 SS, 39 S/β-thalassemia, and 1 SD along with 110 healthy individuals from Kurdistan of Iraq were studied. The lipid profile, vitamin D level, FokI, and TaqI variants of VDR and group-specific component (GC) were detected using the standard enzymatic method, the immunodiagnostic systems limited EIA kit and PCR-RFLP methods, respectively. Results. Around 93 and 82% SS and S/β thalassemia patients, respectively had vitamin D deficiency compared to 83% healthy individuals. Severe vitamin D deficiency (<10 ng/ml) was detected in 78.7% of SS patients. Plasma levels of total cholesterol, HDL-C, and LDL-C in SCD patients were significantly lower compared to controls. Vitamin D levels were negatively correlated to TG and positively correlated to total cholesterol and HDL-C. The frequencies of the C allele of FokI were 81.7 (p=0.003), 80.2 (p=0.034), and 84.6% (p=0.011) in all SCD, SS, and SS/βthal patients, respectively compared to 69.1% in controls. However, no significant difference was detected by comparing the frequencies of VDR TaqI and GC polymorphisms between SCD patients and controls.Conclusion. In the present study we found hypocholesterolemia, high prevalence of VDR FokI C allele, and low vitamin D level among children and adults with SCD patients from Kurdistan of Iraq.


2018 ◽  
Vol 2018 ◽  
pp. 1-7 ◽  
Author(s):  
Mona Hamdy ◽  
Niveen Salama ◽  
Ghada Maher ◽  
Amira Elrefaee

Lower levels of vitamin D have been documented in many patients with sickle cell disease (SCD), but data are still inconclusive regarding the association between vitamin D deficiency (VDD) and the occurrence or the severity of various SCD complications. Our study aimed to detect the prevalence of vitamin D deficiency among Egyptian patients with SCD and to associate it with the clinical course of the disease. We measured the level of 25-hydroxy vitamin D in 140 children (age from 4.3 to 15.5years), 80 patients with SCD and 60 controls using enzyme-linked immunosorbent assay. Vitamin D was deficient in 60% of SCD compared to 26.7% of controls. Severe VDD was significantly higher in SCD patients than controls. Patients were divided into 2 groups; Normal group (32 patients) and Deficient group (48 patients). There were statistically significant differences between the 2 groups regarding their age, height percentile, the presence of clinical jaundice, and osseous changes (P values 0.043, 0.024, 0.001, and 0.015, respectively). Hemoglobin and hematocrit values were significantly lower in Deficient group (P values 0.022 and 0.004, respectively) while the levels of aspartate aminotransferase, lactate dehydrogenase, and total and indirect bilirubin were significantly higher in the same group (P values 0.006, 0.001, 0.038, and 0.016, respectively). The frequency of blood transfusions, hospitalization, and vasoocclusive crisis previous year as well as the history of bone fracture and recurrent infections proved to be significantly higher in Deficient group. These findings suggest that VDD may play a role in the pathogenesis of hemolysis and other complication of SCD. Vitamin D monitoring and supplementation in patients with SCD should be implemented as a standard of care to potentially improve health outcomes in these affected patients.


2012 ◽  
Vol 159 (2) ◽  
pp. 211-215 ◽  
Author(s):  
Ifeyinwa Osunkwo ◽  
Thomas R. Ziegler ◽  
Jessica Alvarez ◽  
Courtney McCracken ◽  
Korin Cherry ◽  
...  

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4857-4857
Author(s):  
Samip Master ◽  
Shajadi Patan ◽  
Shashank Cingam ◽  
Runhua Shi ◽  
Richard Preston Mansour

Abstract Introduction: The chronic pain in sickle cell disease (SCD) arises from chronic bone damage as a consequence of bone marrow infraction during vaso-occulsive events. There are certain barriers to adequate pain management in adult patients with SCD, namely, limited knowledge among the clinicians, inadequate assessment, concerns about addiction, and biases against opioid use. We did retrospective analysis to investigate the average pain medication needed by adult patient with SCD. We also did analysis to see if there was a relation between plasma vitamin D level and amount of pain medication needed. Methods: We take care of approximately 300 active adult SCD at Hematology clinic at our institute. We did a retrospective analysis of 458 adult patients with SCD seen at our clinic between 2001 and 2016. We collected data on type of SCD, plasma 25 -hydroxyvitamin d level and amount of opioid pain medication in mg. To get uniform units of opiates, we converted all the different opiates into morphine. Results: The average morphine dose in a 24 hours period needed to manage chronic pain in an adult patient with SS type was 84 mg with standard deviation of 72, for SC type was 60 mg with standard deviation of 72 and for sickle beta thal type was 72 mg with standard deviation of 71. There were 4 patients with SS with hereditary persistence of hemoglobin F and average opiate dose in them was 84 mg. We obtained vitamin d level on 223 patients and out of them, 47 had vitamin d level of <4.2 ng/ml (lowest level reportable by our lab). We also found negative correlation between amount of pain medication and vitamin D level. The spearman correlation coefficient was -0.2 and p value was <0.01. Conclusion: Because we were unable to find any previous reports of the correlation of vitamin D levels and opiate use in an adult population with SCD, we believe that this is the first study to report this correlation. It also provides a rough estimate regarding average amount of opiates that an adult patient with SCD needs. This correlation between Vitamin D levels and opiate requirement supports our current practice of screening all patients for Vitamin D deficiency using 25hydroxy vitamin D levels and treating all patients who are found to be deficient. We do not know if this Vitamin D replacement will reduce pain, bone health or the amount of opiate medication needed in the future. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1385-1385
Author(s):  
Jin Han ◽  
Santosh L. Saraf ◽  
Taimur Abbasi ◽  
Xu Zhang ◽  
Robert E. Molokie ◽  
...  

Abstract Background: Vitamin D deficiency (VDD) is highly prevalent among patients with sickle cell disease (SCD). Although little is known about the risk factors for VDD in SCD, it has been shown that VDD is associated with chronic pain, bone fragility, and pulmonary function in SCD. In this study we investigated the potential clinical predictors for VDD in patients with SCD. Method: In a retrospective, cross-sectional analysis, a total of 167 adults with SCD treated at the University of Illinois Medical Center with a baseline 25-hydroxy vitamin D (25-OHD) measurement were screened. Clinical variables were recorded from a clinic visit at least four weeks from a vaso-occlusive pain episode or red blood cell transfusion. Statistical association between 25-OHD and other clinical parameters were investigated. Results: After stratifying the patients based on 25-OHD levels, we observed the median age was significantly younger in patients with lower 25-OHD levels (Table 1). When analyzing different age groups by Kruskal Wallis analysis, 25-OHD levels were significantly elevated in patients ≥ 40 years old (Figure 1). When using Spearman correlation analysis, the 25-OHD levels as a continuous variable positively correlated with increasing age (p<0.001); they also showed a significant negative relationships with creatinine clearance, total bilirubin, platelet count, and white blood cell count (Table 2). Using ordinal logistic regression, age was an independent predictor of 25-OHD levels, as a three-categorical variable, in SCD (OR 0.55, 95% CI: 0.38 – 0.81; p =0.002) after adjusting for gender, creatinine clearance, and vitamin D supplementation (Table 3), which means that younger patients has higher chance of VDD. In the patients with VDD (25-OHD <20 ng/mL), weekly supplementation with oral ergocalciferol (50,000 units for twelve weeks) substantially improved 25-OHD levels (9.9 vs 23.7 ng/mL, p<0.0001, N=24). During a median of 40-month follow-up (range 0 to 96 months), thirteen patients died, but the log rank test or multivariate Cox regression analysis failed to show statistical significance between 25-OHD levels and mortality after adjusting ESRD and baseline vitamin D supplementation, likely due to a short follow-up period and a small sample size. Summary: Lower 25-OHD levels were associated with younger age in patients with SCD, especially patients younger than 40 years old. One possible explanation is that lower 25-OHD levels may be linked to higher mortality in SCD, but future research is needed to clarify the association between VDD and mortality in SCD. Disclosures No relevant conflicts of interest to declare.


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