scholarly journals Disease Severity Scores and Haemogram Parameters in Nigerian Sickle Cell Disease Patients

2015 ◽  
Vol 06 (06) ◽  
Author(s):  
Emmanuel Okocha ◽  
Emmanuel Onwubuya
Keyword(s):  
Sickle Cell Disease ◽  
Disease Severity ◽  
Sickle Cell ◽  
Cell Disease ◽  
Severity Scores ◽  
Disease Severity Scores

Does Living Closer to a Medical Care Center Matter in Sickle Cell Disease?

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 875-875
Author(s):  
Jude C Jonassaint ◽  
Charles R. Jonassaint ◽  
Charlene M. Flahiff ◽  
Andrea Ball ◽  
Soheir S. Adam ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Disease Severity ◽  
Sickle Cell ◽  
Employment Status ◽  
Medical Center ◽  
Tertiary Care ◽  
Cell Disease ◽  
Severity Scores ◽  
Disease Severity Scores

Abstract Many of the tertiary care hospitals in North Carolina (NC) are often frequented by patients who have to travel a long distance, given the rural nature of the state. Nearly one half of the adult sickle cell disease (SCD) patients seen at the Duke Comprehensive Sickle Cell Center (DCSCC) come from areas further than a 1-hr drive. The current study aims to determine whether geographical proximity to a comprehensive medical center is associated with SCD outcomes, as indicated by severity score, hospitalization frequency, and quality of life. Methods: Two hundred and two patients who primarily receive their SCD disease-related care from DCSCC were enrolled in the study. The sample included 101 males and 101 females, aged 20–69 years (mean=35.6), with SCD disease (SS: n=135; SC: n=47; other: n=20), and level of education ranging from 4–18 years (mean=13.1). Patients lived an average of 50.4 miles (median: 38.6, range:0.4 to 383) from DCSCC. Linear regressions, controlling for age and SCD diagnosis, were used to test associations between continuous variables. Severity scores measuring end organ damage were determined as previously described (Afenyi-Annan et al. 2008), and frequency of hospitalizations over the past 2 years was determined by self-report and medical record review. To measure mental and physical quality of life (QoL) domains, patients were administered the SF36 QoL scale. Hydroxyurea (HU) and opiate pain therapies were also recorded. Patients were considered to be on opiates if they had used opiates daily for a period of thirty days in the previous 12 months. Results and Discussion: Living closer to Duke had a statistically significant association with higher disease severity scores (β = −0.17, p=0.01). Moreover, proximity to Duke was associated with higher frequency of hospitalizations (β = −0.23, p=0.002). These associations were not modified by gender, employment status or education. Medication use did not account for the association between proximity and disease severity, or proximity and frequency of hospitalizations. The mental domain scores of self-reported QoL correlated negatively with hospitalizations (r= −0.18, p=0.02), whereas the physical domain score negatively correlated with both disease severity (r= −0.19, p <0.01) and hospitalizations (r = −0.26, p <0.01). However, proximity to DCSCC was not associated with the mental or the physical QoL domain. Therefore, it is unlikely that patients move closer to Duke due to higher perceived severity of illness and related medical needs. Conclusion: Patients who live closer to our tertiary care comprehensive center had higher disease severity scores and more hospitalizations over a two year period than patients who live farther away. Neither age, disease diagnosis, gender, employment status, education, nor HU and/or opiate medication use accounted for the negative association between proximity to DCSCC and disease outcomes. On the other hand, distance from DCSCC did not affect patients’ quality of life. The cross-sectional nature of the current study makes it difficult to determine causality. However, it is possible that patients who live close to a major medical center rely more on health system availability as a means to managing their disease, while those living further away rely on self-care at home or adhere to long-term medical regimens. Health care providers may need to focus on developing practice guidelines that encourage and empower patients to take a more active role in their medical care and be less dependent on their healthcare providers to decrease frequency of hospitalization and, perhaps decrease the progression of their disease.

scholarly journals Serum levels of copper, zinc and disease severity scores in sickle cell disease patients in Benin City, Nigeria

2019 ◽  
Vol 19 (3) ◽  
pp. 2798-2805
Author(s):  
Mathias A Emokpae ◽  
Emmanuel B Fatimehin ◽  
Progress A Obazelu
Keyword(s):  
Disease Severity ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Serum Levels ◽  
Serum Copper ◽  
Clinical State ◽  
Cell Disease ◽  
Severity Scores ◽  
Copper Zinc ◽  
Disease Severity Scores

Background: Micronutrient deficiency is recognized in sickle cell anaemia (SCA) but it is not known for certain whether changes in zinc, copper and copper-to-zinc ratio are associated with Sickle cell disease severity scores. Objective: To compare serum levels of copper, zinc and copper-to-zinc ratio in SCA subjects with control group and correlate the variables with objective disease severity scores. Methods: Serum copper and zinc were determined in 100 SCA patients and 50 controls using kits supplied by Centronic, Germany. Unpaired Students’t-test was used to compare the variables between SCA patients in steady clinical state, vaso-occlusive crisis and controls, while Spearman correlation coefficient was used to associate the parameters with disease severity scores. Results: Serum copper level was higher (P=0.008) in SCA patients than controls, while serum zinc level was lower (P<0.001) in SCA patients than controls. The copper/zinc ratio was higher (P<0.001) in SCA patients than controls. Significantly higher (P<0.001) copper and lower (P<0.001) zinc levels were observed in patients in vaso-occlusive crisis than in steady clinical state. Zinc correlated inversely (r=-0.2743; P=0.006) while copper-to-zinc ratio correlated positively with disease severity scores. Conclusion: Copper-to-zinc ratio may be an indicator of disease severity in SCA patients.Keywords: Copper/zinc ratio, disease severity score, sickle cell anaemia.

The Vitamin D Receptor Gene and Disease Severity in Sickle Cell Anemia

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 2122-2122
Author(s):  
E. Leila Jerome Clay ◽  
Alison Motsinger-Reif ◽  
Janelle Hoskins ◽  
Lindsay Veit ◽  
David A. Barrow ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Vitamin D ◽  
Sickle Cell Disease ◽  
Disease Severity ◽  
Sickle Cell ◽  
Severity Score ◽  
Validation Cohort ◽  
Cell Disease ◽  
Discovery Cohort ◽  
Severity Scores

Abstract Abstract 2122 Vitamin D is important in multiple aspects of health, including the cardiovascular, immune and skeletal systems and its effects are mediated through the vitamin D receptor (VDR). The systems affected by vitamin D are also perturbed by sickle cell disease (SCD). Vitamin D deficiency is common in SCD, but its contribution to disease manifestations is not yet known. In normal populations, vitamin D has been shown to be associated with hypertension and vascular pathology. That association may be particularly relevant to the inflammatory / endothelial damage seen in sickle cell disease. We have used clinical and laboratory data to create separate inflammatory and vaso-occlusive severity scores. Our hypothesis is that specific VDR polymorphisms are associated with disease severity in sickle cell disease. DNA specimens from 1141 study participants in the NIH-funded Silent Infarct Transfusion (SIT) trial were used. In this multi-center international trial, the participants were children ages 4 to 13 years of age with SCD who were screened for the presence of silent cerebral infarction and had demographic and clinical data collected, as well as samples for a biologic repository for a self-renewing source of DNA. An initial 570 samples served as the discovery cohort. The subsequently enrolled 530 individuals formed our validation cohort. We evaluated 79 single nucleotide polymorphisms (SNP) in the VDR gene and three associated genes, CYP27B1, VD binding protein, retinoid X receptor, and tagging SNPs from the African American population from Hapmap. The discovery cohort individuals had VDR haplotype information from a prior genome-wide association study (GWAS), and analysis for additional VDR-related SNPs was performed using a specifically designed Sequenom assay. The validation cohort was analyzed for SNPs that were significant in the discovery cohort. Phenotype data was obtained from the demographic and clinical information of the participants, and was used to create the severity scores. The vaso-occlusive score includes: number of hospitalizations for pain, number of hospitalizations for acute chest, and avascular necrosis. The inflammatory severity score includes: priapism, transient ischemic attacks (TIA), silent cerebral infarct, systolic and diastolic blood pressure, transcranial doppler velocity, white count and baseline hemoglobin. The overall severity score includes all of the inflammatory and vaso-occlusive variables. To derive the scores, the variables were transformed into quartiles. Each individual subject was assigned values of 1, 2, 3, or 4 for each variable with 1 representing lowest severity and 4, the highest. In addition, in concert with prior analyses of the SIT data, the variable for number of hospitalizations for pain was used alone as a severity measure. The severity scores were not normally distributed and were not totally continuous distributions, so the Kruskal-Wallis test was used in association analysis. To look for complex genetic models including potential gene-gene interactions for prediction of disease severity, the Multifactor Dimensionality Reduction (MDR) method was utilized, with repeat analyses performed for each severity score. By univariate analysis, no associations were found between any of the VDR associated SNPs and the 3 severity scores. Using MDR in the discovery cohort, one SNP, rs7965281, was found to be associated with the inflammatory severity score. It remained significant after correcting for multiple comparisons with permutation analysis. In the validation cohort, rs7965281 was tested for association with each of the severity scores. There was no association with the inflammatory or the vaso-occlusive severity score but a trend towards association with the overall severity score (p= 0.08). All the SNPs were tested for association with the variable, number of hospitalizations for pain using regression analysis. Two additional SNPs, rs7855881 and rs34312136 were found to be nominally significant (p=0.01 and p=0.04 respectively). Rs7965281 shows a trend as well with p=0.06. In the literature, rs7965281 is associated with reduced risk for cutaneous melanoma in a large population based study as well as with blood pressure in a British population. Further work in our validation cohort, including MDR analysis for gene-gene interaction using the 3 significant SNPs remains to be done and this may provide further direction in future research. Disclosures: No relevant conflicts of interest to declare.

Nutrient Insufficiencies/Deficiencies in Children With Sickle Cell Disease and Its Association With Increased Disease Severity

2016 ◽  
Vol 63 (6) ◽  
pp. 1060-1064 ◽  
Author(s):  
David J. Martyres ◽  
Abi Vijenthira ◽  
Nick Barrowman ◽  
Sydney Harris-Janz ◽  
Christine Chretien ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Disease Severity ◽  
Sickle Cell ◽  
Cell Disease

scholarly journals The Polymorphisms in the Vitamin D Receptor Gene and Disease Severity in Sickle Cell Disease

2015 ◽  
Vol 05 (01) ◽  
pp. 24-33
Author(s):  
E. Leila Jerome Clay ◽  
Alison Motsinger-Reif ◽  
Janelle Hoskins ◽  
Lindsay Veit ◽  
Ali Calikoglu ◽  
...  
Keyword(s):  
Vitamin D ◽  
Sickle Cell Disease ◽  
Disease Severity ◽  
Sickle Cell ◽  
Vitamin D Receptor ◽  
Receptor Gene ◽  
Vitamin D Receptor Gene ◽  
Cell Disease

scholarly journals Effects of Delayed Pubertal Development, Nutritional Status, and Disease Severity on Longitudinal Patterns of Growth Failure in Children With Sickle Cell Disease

2007 ◽  
Vol 61 (5, Part 1) ◽  
pp. 607-613 ◽  
Author(s):  
Babette S Zemel ◽  
Deborah A Kawchak ◽  
Kwaku Ohene-Frempong ◽  
Joan I Schall ◽  
Virginia A Stallings
Keyword(s):  
Sickle Cell Disease ◽  
Nutritional Status ◽  
Disease Severity ◽  
Sickle Cell ◽  
Pubertal Development ◽  
Growth Failure ◽  
Cell Disease ◽  
Longitudinal Patterns

scholarly journals New concepts in assessing sickle cell disease severity

1998 ◽  
Vol 58 (1) ◽  
pp. 61-66 ◽  
Author(s):  
John-John B. Schnog ◽  
Leroy R. Lard ◽  
Robert A. Rojer ◽  
Fey P. L. Van der Dijs ◽  
Frits A. J. Muskiet ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Disease Severity ◽  
Sickle Cell ◽  
Cell Disease ◽  
New Concepts

Prevalence of ocular abnormalities in relation to sickle cell disease severity among children in South-western, Nigeria

2020 ◽  
pp. 112067212095761
Author(s):  
Oluwatoyin I. Oladimeji ◽  
Oluwagbemiga O. Adeodu ◽  
Oluwatoyin H. Onakpoya ◽  
Samuel A. Adegoke
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Disease Severity ◽  
Sickle Cell ◽  
The Body ◽  
University Teaching ◽  
Teaching Hospitals ◽  
Cell Disease ◽  
Cross Sectional ◽  
Ocular Abnormalities

Introduction: Sickle cell disease (SCD) ranks high among genetic disorders worldwide. It is characterised by repeated vaso-occlusion with resultant end-organ damage. This process can occur in all vascular beds in the body, including ocular blood vessels and may cause irreversible blindness in advanced stages. Little is known of the relationship between the prevalence of ocular abnormalities among children with SCD and their disease severity. Methods: A descriptive cross-sectional study was carried out at the Paediatric Haematology Clinics and the Eye Centre of the Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife. Children with SCD in steady state were recruited from the Haematology Clinics and examined for ocular abnormalities at the Eye Centre of the hospital. The subjects SCD severity grade was determined using a previously validated scoring system. Results: One hundred and twenty (120) children aged 5 to 15 years were examined. Of these, 72 had one or more ocular abnormalities giving the prevalence of ocular abnormalities among them to be 60.0%. Though a higher proportion of children with moderate disease, 23 (65.7%) of 35, compared to those with mild disease, 49 (57.6%) of 85 had ocular abnormalities, this difference was not statistically significant, p = 0.412. Conclusion: Ocular abnormalities among Nigerian children with SCD are common even in steady-state, but not significantly associated with disease severity. Periodic screening for ocular abnormalities should thus be done on them irrespective of disease severity.

Serum Ferritin and Severity Scores in Sickle Cell Disease Patients in Nnewi (South East Nigeria)

2016 ◽  
Vol 11 (2) ◽  
pp. 1-7
Author(s):  
E Okocha ◽  
E Onwubuya ◽  
C Osuji ◽  
G Ahaneku ◽  
U Okonkwo ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Cell Disease ◽  
Severity Scores
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