A Comparison Of Sickle Cell Anemia Between Patients From Nigeria and The United States

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 997-997
Author(s):  
Santosh L. Saraf ◽  
Bamidele O. Tayo ◽  
Titilola S. Akingbola ◽  
Babatunde L. Salako ◽  
Jennifer Layden ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Blood Cell ◽  
Sickle Cell ◽  
White Blood Cell ◽  
Sickle Cell Anemia ◽  
The United States ◽  
Medical Attention ◽  
Acute Chest Syndrome ◽  
The University ◽  
The U.S

Abstract Sickle cell anemia (SCA) is among the most common monogenetic diseases worldwide and environmental and genetic factors are thought to contribute to variations in clinical presentation. We conducted a study to compare clinical and laboratory features between 214 SCA patients from the University of Ibadan, Nigeria and 200 SCA patients from the University of Illinois at Chicago, U.S. between the ages of 11 and 30 years old. Laboratory and clinical data were collected from clinic visits and comparisons between the two cohorts were adjusted for age, gender, and hydroxyurea use. SCA patients from Nigeria had lower median body weight, height, body mass index, systolic blood pressure, mean arterial pressure and pulse pressure (Table 1). Proportions of hydroxyurea use and vaccinations for streptococcus pneumoniae and influenza A were lower in SCA patients from Nigeria. Hematological indices showed that SCA patients from Nigeria had lower median values for hemoglobin F (HbF) percent, hemoglobin concentration and mean corpuscular volume but platelet and white blood cell counts were not significantly different compared to SCA patients from the U.S. In the white blood cell differential, lower granulocyte counts and higher lymphocyte and monocyte counts were observed in SCA patients from Nigeria. The proportion of patients with a history of ≥ 3 vaso-occlusive pain episodes requiring medical attention per year were similar between the Nigerian and U.S. cohorts but the proportions with histories of stroke, acute chest syndrome and > 5 lifetime transfusions were lower in the Nigerian versus U.S. cohort. On logistic regression analysis, male gender was independently associated with vaso-occlusive crises ≥ 3 per year in the Nigerian cohort (OR 1.9, 95% CI 1.1 – 3.4, p=0.04) and lower HbF concentration was associated with this pain category in the U.S. cohort (Hb F 1% decrement OR 1.1, 95% CI 1.0 – 1.1, p=0.02). A higher white blood cell count was independently associated with stroke history in both the Nigerian and U.S. cohorts (Nigeria: OR 1.1, 95% CI 1.0 - 1.3, p=0.03; US: OR 1.2, 95% CI 1.1 – 1.3, p=0.003). Additionally, a lower HbF concentration was independently associated with stroke history in the Nigerian cohort (Hb F 1% decrement OR 2.5, 95% CI 0.2 – 0.9, p=0.03). The lower height and low proportions of vaccination among the Nigerian SCA patients point to the need for improved nutrition and vaccination strategies, and the low utilization of hydroxyurea therapy highlights the need to investigate the appropriateness of this agent in the African context. On the other hand, the higher values for BMI and blood pressure in the UIC compared to Nigerian SCA patients parallel similar findings in non-sickle cell cohorts and raises the possibility that life-style factors may contribute to the higher rate of stroke in the UIC cohort. Whether the lower rate of complications in general in the Nigerian SCA patients reflects higher early mortality versus favorable genetic or environmental factors deserves further investigation. In summary, our study found both differences and similarities between patients with SCA from Nigeria and the U.S. and a better understanding of the genetic and environmental contributions may help guide future therapies for SCA in both settings. Disclosures: No relevant conflicts of interest to declare.

Risk Factors for Intracranial Hemorrhage in Children with Sickle Cell Anemia.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1657-1657
Author(s):  
John J. Strouse ◽  
Michael R. DeBaun ◽  
James F. Casella
Keyword(s):  
Risk Factors ◽  
Blood Pressure ◽  
Ischemic Stroke ◽  
Steady State ◽  
Sickle Cell ◽  
Intracranial Hemorrhage ◽  
Sickle Cell Anemia ◽  
Small Sample ◽  
Acute Chest Syndrome ◽  
Corticosteroid Administration

Background: Intracranial hemorrhage (ICH) is an uncommon, but devastating, complication of sickle cell disease (SCD) with mortality from 30 to 65%. Most reported cases are in adults; little is known about children. Proposed risk factors include previous ischemic stroke, aneurysms, low steady-state hemoglobin, high steady-state leukocyte count, acute chest syndrome, and hypertransfusion. Methods: Retrospective case-control study designed to characterize and evaluate risk factors for ICH among children with SCD age < 19 years hospitalized at Johns Hopkins Children’s Center from January 1979 to March 2004. Cases had SCD and ICH (intraparenchymal (IPH), subarachnoid (SAH), or intraventricular (IVH) hemorrhage confirmed by neuroimaging or analysis of cerebrospinal fluid; traumatic subdural and epidural hemorrhages were excluded). Controls had SCD and ischemic stroke (focal neurological deficits with corresponding cerebral infarcts by neuroimaging). Both were identified by searching the hospital discharge database using ICD-9 codes for acute stroke and SCD and reviewing the Division of Pediatric Hematology’s records. ACS was defined as a new pulmonary infiltrate and two of the following: chest or rib pain, dyspnea, fever, tachypnea, grunting, nasal flaring, or retractions. Blood pressure was adjusted for age, sex, and hemoglobin genotype. Results: We identified 7 cases (mean age=11.2 years, range 2 to 16 years) and 9 controls (mean age 6.2 years, range 2 to 8 years). As expected, cases were significantly older than controls (p<0.01). All cases and controls had sickle cell anemia. Cases presented with impaired mental status (5/7), bradycardia (5/7), headache (4/7), and emesis (3/7). They often had multiple sites of hemorrhage (5/7) and died during the initial hospitalization (4/7). Five had IPH involving the frontal, parietal, and/or temporal lobes (2 of the patients with IPH also had SAH, 1 had IVH and 1 had both SAH and IVH). Two additional patients had SAH (one also with IVH). Most cases and controls had elevated systolic blood pressure at the time of stroke (4/7 cases, 8/9 controls). Cases had lower steady-state hemoglobin (mean±SE 7.1±0.3 g/dl vs. 7.7±0.4 g/dl), lower steady-state blood pressures (systolic 104±9 vs. 117±5 mm Hg, diastolic 50±5 vs. 61±5 mm Hg) and higher steady-state leukocyte counts (16,590±2823/ul vs. 13,851±2184/ul) than controls, but these differences were not statistically significant. Mean hemoglobin concentration was increased 2.8 g/dl (39.9%) from steady-state at the time of stroke in cases and was unchanged in controls (p=0.08). Other events in the two weeks before ICH associated with increased odds of ICH included transfusion (simple in 5 cases, erythrocytapheresis in 1), ACS (3 cases), and corticosteroid administration (high-dose dexamethasone for ACS in 2, stress doses for possible adrenal insufficiency in 1). Conclusions: In this group of children with SCD, ICH was associated with antecedent events including transfusion and possibly corticosteroids. Mortality was similar to that of adults with SCD and ICH. Limitations of this study include the small sample size and the retrospective design. The contribution of antecedent events to ICH in children with SCD deserves further evaluation. Odds Ratios of Intracranial Hemorrhage For Events in the Last 14 Days Event Odds Ratio (95% CI) P-value Transfusion 48 (1.8-2469) <0.01 ACS 6 (0.3-33) 0.26 Corticosteroids ∞ (1.3- ∞) 0.06

Determinants of Inter-Procedure Interval in Patients with Sickle Cell Anemia Enrolled a Chronic Red Blood Cell Exchange Program

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4284-4284
Author(s):  
Alecia C. Nero ◽  
Theresa Nguyen Kinard ◽  
Beverley Adams-Huet ◽  
Karen Matevosyan ◽  
Ravindra Sarode
Keyword(s):  
Blood Cell ◽  
Multiple Regression ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Small Sample ◽  
Blood Type ◽  
Acute Chest Syndrome ◽  
Intractable Pain ◽  
Post Procedure

Abstract Background: Sickle cell anemia (SCA) is a genetically inherited disorder that can lead to severe sequelae in any organ system. The most clinically devastating complications, e.g. stroke, are managed with chronic red blood cell (RBC) transfusions as curing the underlying SCA is often not an option. Chronic RBC exchange (RBCx), is an effective alternative to simple transfusions and often performed every 4-5 weeks. Isovolemic Hemodilution-Red Blood Cell Exchange (IHD-RBCx) is a modified RBCx method used at UT Southwestern Medical Center (UTSW) and increases the time interval between transfusions, i.e. inter-procedure interval (IPI), up to 9 weeks while maintaining the desired hemoglobin S targets at safe levels. This minimizes the overall lifetime procedures and blood exposures. The standard operating procedure for IHD-RBCx is published (Matevosyan et al, 2012). However, we observed IPI variability between patients in this chronic IHD-RBCx program. We sought to analyze hematologic characteristics in a cohort of adult SCA patients that may be associated with this noted variability. Methods: This IRB approved cross-sectional study evaluated medical and blood bank records of adult patients with SCA, i.e. hemoglobin SS or Sβ0 thalassemia genotypes, undergoing chronic IHD-RBCx at UTSW adult hospital sites between January 1, 2012 and December 31, 2013. Excluded participants were those with autoimmune disease, non-compliance with IHD-RBCx leading to delays > 7 days, or use of hydroxyurea or steroids within four months of the study period. Patients with < 5 exchanges were excluded as several procedures are needed to determine baseline IPIs. The primary indication for IHD-RBCx was secondary stroke prevention. Three subjects had alternative indications (i.e. recurrent severe acute chest syndrome that failed hydroxyurea therapy, intractable pain and severe anemia ineligible for iron chelation due to end-stage renal disease). Median values during the 2 year study were determined for the IPI, laboratory, and RBC properties for use in subsequent analyses. Correlation and multiple regression analyses were performed to determine variables predictive of IPI. Results: Twenty-four SCA patients in this chronic IHD-RBCx transfusion program met inclusion criteria of which 14 (58.3%) were female with age range 18-41 years. Three subjects had incomplete data. The median number of packed RBC (pRBC) units per IHD-RBCx was 8 (range: 6-11 units). The median volume per unit of pRBCs was 304.5 mL (range: 298.8-326.5 mL) with average age of the pRBCs 5.8 days ± 0.7 days. The median IPI was 55.8 days (range: 36.0-65.5 days). The strongest correlation with IPI was the change from the mid to post-procedure hemoglobin and hematocrit values, r=0.56, p=0.005 and r=0.57, p=0.003, respectively. Participants with documented direct antiglobulin test positivity (n=7), compared to those who remained negative (n=16), had 4.9 days increased IPI. This difference was not statistically significant, p=0.19. ABO differences in blood type also did not show statistical significant differences. However, A+ subjects had a 58 day interval compared to 51, 52, 53 days for B+, O+, O- respectively (Figure). In multiple regression models, log blood urea nitrogen (BUN) and post-procedure white blood cell count (WBC) were found to be independent predictors of IPI, beta=-11.3, p=0.04 and -2.1, p=0.03 respectively, with adjusted R2=0.31. Conclusions: Chronic IHD-RBCx effectively treats severe SCA complications. Discontinuing transfusions lead to repeat stroke events or the recurrence of disease manifestations making chronic transfusion programs an indefinite therapy. Outside of the expected correlation between the IPI and hemoglobin/hematocrit, WBC and BUN were found to have negative associations with IPIs. The decrease in WBCs is likely due to the direct clearing effect by the operating system. The association between BUN and IPI may imply relationship with renal function. Interestingly, association with creatinine was not statistically significant. The study is limited by the small sample size. Future studies of similar cohorts should prospectively evaluate target mechanisms which may lead to interventions that can minimize lifetime blood exposures given the growing demands for continued RBCx in severe SCA. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Arterial blood pressure in adult Nigerians with sickle cell anemia

2010 ◽  
Vol 56 (3) ◽  
pp. 326-331 ◽  
Author(s):  
N.I. Oguanobi ◽  
B.J.C. Onwubere ◽  
O.G. Ibegbulam ◽  
S.O. Ike ◽  
B.C. Anisiuba ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Arterial Blood Pressure ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Arterial Blood

Identifying Top Predictors of Change in Noncalcified Coronary Burden in Psoriasis by Machine Learning Over 1-Year

2021 ◽  
pp. 247553032110007
Author(s):  
Eric Munger ◽  
Amit K. Dey ◽  
Justin Rodante ◽  
Martin P. Playford ◽  
Alexander V. Sorokin ◽  
...  
Keyword(s):  
Machine Learning ◽  
Blood Pressure ◽  
Blood Cell ◽  
Cell Count ◽  
White Blood Cell Count ◽  
White Blood Cell ◽  
Severity Index ◽  
Blood Cell Count ◽  
Psoriasis Area Severity Index ◽  
Severity Index Score

Background: Psoriasis is associated with accelerated non-calcified coronary plaque burden (NCB) by coronary computed tomography angiography (CCTA). Machine learning (ML) algorithms have been shown to effectively identify cardiometabolic variables with NCB in cross-sectional analysis. Objective: To use ML methods to characterize important predictors of change in NCB by CCTA in psoriasis over 1-year of observation. Methods: The analysis included 182 consecutive patients with 80 available variables from the Psoriasis Atherosclerosis Cardiometabolic Initiative, a prospective, observational cohort study at baseline and 1-year using the random forest regression algorithm. NCB was assessed at baseline and 1-year from CCTA. Results: Using ML, we identified variables of high importance in the context of predicting changes in NCB. For the cohort that worsened NCB (n = 102), top baseline variables were cholesterol (total and HDL), white blood cell count, psoriasis area severity index score, and diastolic blood pressure. Top predictors of 1-year change were change in visceral adiposity, white blood cell count, total cholesterol, c-reactive protein, and absolute lymphocyte count. For the cohort that improved NCB (n = 80), the top baseline variables were HDL cholesterol related including apolipoprotein A1, basophil count, and psoriasis area severity index score, and top predictors of 1-year change were change in apoA, apoB, and systolic blood pressure. Conclusion: ML methods ranked predictors of progression and regression of NCB in psoriasis over 1 year providing strong evidence to focus on treating LDL, blood pressure, and obesity; as well as the importance of controlling cutaneous disease in psoriasis.

Association of plasma CD40L with acute chest syndrome in sickle cell anemia

Cytokine ◽  
2017 ◽  
Vol 97 ◽  
pp. 104-107 ◽  
Author(s):  
Vanessa Tonin Garrido ◽  
Laura Sonzogni ◽  
Siana Nkya Mtatiro ◽  
Fernando F. Costa ◽  
Nicola Conran ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Acute Chest Syndrome

Clinical epidemiology of individuals with Sickle cell anemia using Hydroxyurea at Muhimbili National Hospital, Dar Es Salaam, Tanzania

2020 ◽  
Vol 31 (1) ◽  
pp. 106-119
Author(s):  
Elisha Osati ◽  
Edward Kija ◽  
Florence Urio ◽  
Magdalena Lyimo ◽  
Siana Nkya ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Severe Pain ◽  
Clinical Epidemiology ◽  
Fetal Hemoglobin ◽  
Dar Es Salaam ◽  
Acute Chest Syndrome ◽  
Muhimbili National Hospital ◽  
National Hospital

Background: The pathophysiology of sickle cell disease (SCD) is complex and involves nitric oxide depletion, increased inflammation/adhesion molecules and vaso-occlusion in addition to the chronic hemolytic anemia. This pathophysiology results in systemic clinical complications including recurrent episodes of severe pain, stroke, acute chest syndrome (ACS) and an increased susceptibility to infection. SCD severity varies among individuals and fetal hemoglobin (HbF) is known as a major modulator of the disease. To date, hydroxyurea (HU) is a known intervention that acts by increasing HbF in individuals with SCD. The increase in HbF reduces the risk of ‘sickling’ events and improves clinical outcomes. This is the first study on the use of HU in individuals with SCA in Tanzania.Methods: A case-control study to determine the proportion, indications, clinical and laboratory outcomes of SCD patients with HU use was conducted at Muhimbili National Hospital in Dar Es Salaam, Tanzania.Results: Forty-two patients with Sickle cell anemia (SCA) on HU treatment and 32 patients with SCA not on HU treatment were enrolled. The proportion of HU use by individuals with SCA at Muhimbili National Hospital was 10 per 1000. The mean HbF % was 9.8 ± 2.4 vs 6.2 ±1.4 for controls (P <0.001). Thirty (71.4%) were enrolled for HU treatment due to central nervous system (CNS) events, frequent painful crises 11(26.2%) and recurrent anemia 1(2.4%). Thirty-two SCA patients (76.2%) reported improvements after being on HU for at least six months. Of these, 91% reported no history of severe pain that required hospitalizations since they started HU. Twenty patients (66.7%) out of those with CNS events reported not to have experienced convulsions after HU initiation.Conclusions: HbF was higher in patients who were on HU and had positive correlation with clinical outcomes. Further clinical trials are required to evaluate more effects of HU use among SCA individuals in Tanzania. Keywords: Sickle cell anemia, HU, Fetal hemoglobin, Tanzania.

Prevalence of Red Blood Cell Alloimmunization Among Beta-Thalassemia and Sickle Cell Anemia Patients: a Study from Saudi Arabia

2021 ◽  
Vol 67 (10/2021) ◽  
Author(s):  
Raed Felimban ◽  
Ahmed Alsharyufi ◽  
Jasem Aljehani ◽  
Ahmed Sahlool ◽  
Hamead Aljabri ◽  
...  
Keyword(s):  
Saudi Arabia ◽  
Blood Cell ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Red Blood Cell ◽  
Beta Thalassemia
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