Monocyte Chemotactic Protein-1 Is Associated with Microvascular Abnormalities and Serum Ferritin Concentrations in Sickle Cell Disease Patients

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3255-3255
Author(s):  
Ralph Green ◽  
Joshua W Miller ◽  
Sandra L Samarron ◽  
Xin Lin ◽  
Anthony T Cheung ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Severity Index ◽  
Computer Assisted ◽  
Cell Disease ◽  
Iron Stores ◽  
Monocyte Chemotactic Protein ◽  
Chemotactic Protein

Abstract Abstract 3255 Introduction: Microvascular abnormalities underlie much of the morbidity observed in sickle cell disease (SCD). Adherence of sickle erythrocytes and leukocytes to the microvascular endothelium leads to vaso-occlusion and end organ ischemia/reperfusion injury. Monocyte chemotactic protein-1 (MCP-1) is a chemokine involved in the recruitment of monocytes to sites of endothelial activation or injury and thus contributes to the pathophysiology of vascular disease. In SCD patients, circulating MCP-1 concentrations are elevated during both steady state and vaso-occlusive crisis. However, the relationship between MCP-1 and microvascular abnormalities in SCD patients has not been assessed. In the present study, we measured a panel of circulating cytokines including serum MCP-1 and hematological biomarkers including serum ferritin in SCD patients who were being monitored by computer-assisted intravital microscopy (CAIM) to assess severity of microvasculopathy. Methods: The study included 31 steady state SCD patients (22 females, 9 males) ranging in age from 4–61 years. MCP-1 concentrations were measured by Luminex multiple analyte profiling. Ferritin concentrations were measured by chemiluminometric immunossay. The conjunctival microvasculature was assessed using CAIM. A severity index on a scale of 0–15 was calculated as described previously (Cheung et al, Am J Hematol 85:899, 2010) to quantify the degree of microvasculopathy observed among the patients. Results: The mean ± SD MCP-1 concentration was 445 ± 253 pg/ml (range: 122–1180 pg/ml), mean ± SD ferritin concentration was 1675 ± 1883 ng/ml (range 18–5825 ng/ml), and mean ± SD severity index was 5.0 ± 2.5 (range: 0–9). By multiple regression analysis, with controlling for age and gender, MCP-1 was directly correlated with severity index (p = 0.046), and serum ferritin was directly correlated with MCP-1 (p = 0.019). Conclusions: Monocyte recruitment to the site of endothelial injury involves chemotactic signaling mediated in part by MCP-1, levels of which rise in SCD. Our results indicate that MCP-1 is associated with and may directly contribute to the microvasculopathy observed in SCD patients. Furthermore, we find that MCP-1 levels are correlated with ferritin concentrations. Ferritin reflects body iron stores, and an increase of iron stores in SCD resulting from chronic blood transfusion as well as increased iron absorption associated with hemolysis may thus lead to monocyte/macrophage recruitment to the vascular endothelium and contribute to vasculopathy. Measurements of MCP-1 and ferritin may also serve as surrogate biomarkers of microvasculopathy severity and inflammation. Disclosures: Green: Emisphere - Consultancy : Consultancy; Teva Pharmaceuticals - expert testimony: Consultancy.

Serum Ferritin a Predictor of Iron Overload in Patients with Thalassemia and Sickle Cell Disease?.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3789-3789 ◽  
Author(s):  
Zahra Pakbaz ◽  
Roland Fischer ◽  
Richard Gamino ◽  
Ellen B. Fung ◽  
Paul Harmatz ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Iron Concentration ◽  
Cell Disease ◽  
Liver Iron Concentration ◽  
Liver Iron ◽  
Iron Stores ◽  
Significant Difference

Abstract Introduction: Monitoring iron overload by serum ferritin in patients with hemosiderosis is still a routine practice although its limitations are widely studied and well known. Using non-invasive liver iron assessment by quantitative MRI or by biomagnetic liver susceptometry (BLS) with SQUID biomagnetometers would be the better alternative, however, these methods are available at only a few centers worldwide. Objective: To determine the relationship between serum ferritin (SF) and liver iron concentration (LIC), measured by BLS at CHRCO, in patients with different types of hemosiderosis. Methods and Patients: A total of 97 patients with thalassemia (TM: 3 to 52 y, 54% females) and 39 patients with sickle cell disease (SCD: 5 to 49 y, 60% female) were prospectively assessed for LIC and SF. Both tests were performed within 2 weeks of each other. Most patients with TM and SCD were chronically transfused, while 10 b-thalassemia intermedia (TI), 5 HbE/β-thalassemia (HbE), and 5 SCD patients were not on transfusion programs. LIC was measured by LTc SQUID biosusceptometer system (Ferritometer®, Model 5700, Tristan Technologies, San Diego, USA) under the standardized Hamburg-Torino-Oakland protocol. A non-parametric test (U-test) was utilized to analyze differences between SF and LIC data. Results: In chronically transfused TM and SCD patients, the median SF and LIC were very similar (Table I). In TI&HbE patients, ferritin results were disproportionately low with respect to LIC. In order to improve prediction of iron stores by SF, the SF/LIC ratio was calculated. There was a significant difference between the median ratios of the two groups of transfused and non- transfused thalassemia patients, 0.82 vs. 0.32 [μg/l]/[μg/gliver], respectively (p < 0.01). In SCD patients the ratio is significantly (p < 0.01) higher. Conclusion: Present data confirm ferritin to be a poor predictor of liver iron stores both in sickle cell disease and thalassemia. Relying only on ferritin to monitor iron overload in patients with hemosiderosis can be misleading, especially, in sickle cell disease and non-transfused thalassemia patients. Taking into account disease specific ferritin-LIC relations, could improve the prediction of iron stores. However, assessment of liver iron stores is the ultimate method to initiate and adjust chelation treatment in order to avoid progressive organ injury. Table I. Median values and ranges ( − ) of serum ferritin (SF) and liver iron concentration (LIC) in transfused (Tx) and non-transfused (non-Tx) hemosiderosis patients. Patient group n SF μg/l] LIC [mg/gliver ] SF:LIC Thalassemia Tx 82 1721 (209–8867) 3424 (364–7570) 0.82 (0.3–1.8) TI &HbE non-Tx 15 766 (52–2681) 2174 (226–5498) 0.32 (0.1–1.4) SCD Tx 34 2757 (400–9138) 1941 (518–6670) 1.2 (0.6–3.3)

Microvascular abnormalities in sickle cell disease: a computer-assisted intravital microscopy study

Blood ◽  
2002 ◽  
Vol 99 (11) ◽  
pp. 3999-4005 ◽  
Author(s):  
Anthony T. W. Cheung ◽  
Peter C. Y. Chen ◽  
Edward C. Larkin ◽  
Patricia L. Duong ◽  
Sahana Ramanujam ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Intravital Microscopy ◽  
Computer Assisted ◽  
Red Cell ◽  
Cell Disease ◽  
Computer Assisted Image ◽  
Conjunctival Vessels ◽  
Painful Crisis

The conjunctival microcirculation of 18 homozygous sickle cell disease (SCD) patients during steady-state, painful crisis, and postcrisis conditions was recorded on high-resolution videotapes using intravital microscopy. Selected videotape sequences were subsequently coded, frame-captured, studied, and blindly analyzed using computer-assisted image analysis protocols. At steady-state (baseline), all SCD patients exhibited some of the following morphometric abnormalities: abnormal vessel diameter, comma signs, blood sludging, boxcar blood flow phenomenon, distended vessels, damaged vessels, hemosiderin deposits, vessel tortuosity, and microaneurysms. There was a decrease in vascularity (diminished presence of conjunctival vessels) in SCD patients compared with non-SCD controls, giving the bulbar conjunctiva a “blanched” avascular appearance in most but not all SCD patients during steady-state. Averaged steady-state red cell velocity in SCD patients was slower than in non-SCD controls. During painful crisis, a further decrease in vascularity (caused by flow stoppage in small vessels) and a 36.7% ± 5.2% decrease in large vessel (mostly venular) diameter resulted. In addition, the conjunctival red cell velocities either slowed significantly (6.6% ± 13.1%; P &lt; .01) or were reduced to a trickle (unmeasurable) during crisis. The microvascular changes observed during crisis were transient and reverted to steady-state baseline after resolution of crisis. When combined, intravital microscopy and computer-assisted image analysis (computer-assisted intravital microscopy) represent the availability of a noninvasive tool to quantify microvascular abnormalities in vascular diseases, including sickle cell disease. The ability to identify and relocate the same conjunctival vessels for longitudinal studies uniquely underscores the applicability of this quantitative real-time technology.

Lower Ferritin Concentrations Are Associated with Decreased Hemolysis in Sickle Cell Disease Children without Iron Overload.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 2571-2571
Author(s):  
Oswaldo L Castro ◽  
Mehdi Nouraie ◽  
Lori Luchtman-Jones ◽  
Xiaomei Niu ◽  
Caterina Minniti ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Deficiency ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Research Funding ◽  
Iron Status ◽  
Red Cell ◽  
Cell Disease ◽  
Lower Serum ◽  
Iron Stores

Abstract Abstract 2571 Poster Board II-548 The role of iron in the pathophysiology of sickle cell disease (SCD) is complex and not fully understood. Iron overload is associated with disease severity primarily because multiple transfusions are linked to a severe SCD clinical course. Additionally, hemolysis, also associated with disease severity, increases iron absorption. Iron deficiency decreases red cell MCHC, which lowers Hb S polymerization and thus may improve the clinical manifestations of SCD. Such a hypothesis is supported by our recent observation of a homozygous SCD adult with iron deficiency anemia and a very low hemolytic rate that increased dramatically with iron supplementation. This experience and similar case reports from the literature led us to examine the relationship of ferritin levels with hemolysis and other laboratory and clinical parameters in a group of non-iron overloaded children with sickle cell disease. All subjects in this analysis were enrolled in a prospective study of the prevalence and significance of pulmonary hypertension in children with SCD (PUSH). Because of the known association of high serum ferritin with multiple transfusions and with a severe clinical course in this and other SCD populations, we excluded children who had ferritin concentrations of 242 ng/ml or higher. This cut-off value is 3 SDs above the geometric mean of the ferritin concentrations in a group of 42 age, sex, and ethnicity matched control children without SCD. Hence the group of sickle cell children with ferritin levels of < 242 ng/ml should include only those with iron deficiency or with normal iron stores. The table shows correlations between serum ferritin (natural log) and age, hematologic, iron status, and hemolytic parameters, including a previously described hemolytic component derived by principal component analysis from reticulocyte count, LDH, AST, and bilirubin. In this group of non-iron overloaded SCD children and adolescents (median age 12 y, range 3–20 y), lower serum ferritin was related to higher serum transferrin and to lower serum iron and MCV, documenting that serum ferritin was reflective of iron status. Hemolytic parameters such as reticulocyte count and the hemolytic component were significantly lower with lower ferritin levels. In multivariable analysis these relationships remained statistically significant (P for MCV and ferritin: 0.003, P for hemolytic component and ferritin: 0.044) even after correcting for alpha-thalassemia, which is known to also lower MCV and hemolysis, and for markers of inflammation (WBC) and liver disease (ALT), which could increase the ferritin level regardless of iron stores. Ferritin was significantly lower in older subjects, probably as a result of growth-related red cell mass expansion in the presence of marginal iron stores. Our results thus suggest that low iron stores are independently associated with decreased hemolysis. Low hemolysis is likely to be beneficial in SCD by reducing hemolysis-related vasculopathy, which in adult SCD patients predicts an increased risk of pulmonary hypertension, leg ulcers, priapism, and death. Whether iron status per se plays a role in the pathogenesis of SCD vasculopathy is not known. In non-SCD adults, decreasing iron stores by frequent blood donation has beneficial effects on endothelial function and cardiovascular disease even within the normal range for iron stores. Hence, lowering iron stores could benefit SCD subjects by an additional, hemolysis-independent mechanism. Therapeutic iron depletion is not an option for children because of their need for adequate iron stores for optimal physical and neuro-psychological development. However, carefully controlled studies should be considered to reduce iron stores and so decrease the hemolytic rate in adults with SCD. It may be possible to achieve levels of iron reduction that lower hemolysis but do not worsen the anemia: in our study subjects, low iron stores were not associated with increased anemia and the red cell counts were actually higher with lower ferritin levels. Disclosures: Gordeuk: TRF Pharma: Research Funding; Merck: Research Funding; Biomarin pharmaceutical company: Research Funding; Novartis: Speakers Bureau.

Noninvasive Assessment of Liver Fibrosis in Patients with Sickle Cell Disease

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 3406-3406
Author(s):  
Emma Drasar ◽  
Emer Fitzpatrick ◽  
Kate Gardner ◽  
Moji Awogbade ◽  
Anil Dhawan ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Liver Fibrosis ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Negative Correlation ◽  
Liver Dysfunction ◽  
Cell Disease ◽  
Non Invasive ◽  
Conjugated Bilirubin

Abstract Complications affecting multiple organs, including the liver, contribute to early mortality in patients with sickle cell disease (SCD). Earlier diagnosis and monitoring of "sickle hepatopathy", preferably using non-invasive methods, would enable earlier intervention and therefore, potentially a better outcome for patients. In many non-SCD chronic liver diseases, fibrosis stage is the most important predictor of morbidity and mortality. Two non-invasive methods of assessing liver fibrosis are used: Transient Elastography (TE, Echosens) and Enhanced Liver Fibrosis Score™ (ELF, iQUR). TE detects fibrosis using ultrasound and low frequency elastic waves with a propagation velocity directly related to the stiffness of the liver. The ELF score examines the balance between matrix deposition and degradation using levels of three proteins analysed using a patented formula. We report a prospective study correlating liver function tests (LFTs) with the level of liver fibrosis (as ascertained by TE and ELF) in a cohort of patients with SCD. Ethical approval for this study was obtained from the NHS ethics committee (11/LO/0005) and patients were consecutively recruited in the steady-state sickle cell clinic at Kings' College Hospital, London, during 2012. Patients were excluded if they had viral hepatitis or were pregnant. Clinical (transfusion and hydroxyurea therapy), imaging (liver iron concentration, LIC) and laboratory data were collected during steady-state. TE and ELF were performed on 194 patients. The patients ranged from 17-72 years (mean age 35 years); 78 (40%) were male, 134 (68%) had HbSS or HbSb0 (SCA), 48 (26%) Hb SC, and the remainder, Hb Sβ+ (excluded due to low numbers). Statistical analysis was undertaken in IBM SPSS version 20. There was significant correlation between both TE and ELF with age, when corrected for sickle genotype (TE β = 0.19, p = 0.006 and ELF β = 0.2, p = 0.005) (Figure). Patients with SCA had significantly higher TE results and mean ELF scores than those with HbSC (TE, 6.8 vs 5.3, p<0.0001 and ELF, 9.2 vs 8.6 p <0.0001) (Table). In SCA patients, TE correlated significantly with all serum LFTs (Albumin R = -0.35 p<0.0001, AST R = 0.44 p<0.0001, ALP R = 0.29 p<0.0001, GGT R = 0.40 p<0.0001, conjugated bilirubin R = 0.26 p = 0.004). Positive correlation was found with LDH (R = 0.24 p = 0.004) and negative correlation with Hb (R= -0.25 p = 0.002). In the Hb SC group, TE correlations were weaker for AST (R = 0.39 p = 0.004), ALP (R = 0.30 p = 0.03), WBC (R = 0.39 p = 0.02) and reticulocyte count (R = 0.35 p = 0.01). All markers of iron loading correlated with TE values, when corrected for sickle genotype (serum ferritin β = 0.25, p <0.0001, total top-up units β = 0.22, p = 0.001, total units transfused β = 0.25, p <0.0001 and LIC β = 0.32, p = 0.046). In SCA patients, ELF score correlated with serum LFTs (Albumin R = -0.30 p<0.0001, AST R = 0.39 p<0.0001, ALP R = 0.25 p = 0.003, GGT R = 0.28 p = 0.001, conjugated bilirubin R = 0.36 p<0.0001). Positive correlation was seen with LDH (R = 0.26 p = 0.002) and negative correlation with Hb (R = -0.25 p = 0.004). Negative correlation was also seen with HbF levels (R = -0.24 p = 0.01). In the HbSC group, there were no significant correlations between ELF and serum LFTs. However associations were seen between ELF score and LDH (R = 0.40 p = 0.004) and Hb level (R = -0.31 p = 0.01). ELF score correlated with serum ferritin (β = 0.25 p <0.0001), and total blood transfusion units (β = 0.24 p = 0.001). These data show significant levels of liver dysfunction in our SCD population using both TE and ELF score which correlated significantly with abnormal LFTs and markers of hemolysis and iron overload. The role of TE and ELF in monitoring liver dysfunction in SCD needs to be further validated, preferably with longitudinal, and if possible histological data. Table 1. Range and mean TE results and ELF score for whole cohort and subgroups. Whole cohort n = 194 (%) SCA n = 134 (%) Hb SC n = 48 (%) p value FibroScan results (kPa) Mean (range) 6.3 (2.0 - 21.3) 6.8 (2.0 - 21.3) 5.3 (2.0 - 16.0) < 0.0001 None/mild (0-7.65) 153 (79) 98 (73) 43 (90) Moderate (7.66-13.00) 33 (17) 29 (22) 4 (8) Severe (≥13.01) 8 (4) 7 (5) 1 (2) ELF score Mean (range) 9.1 (7.1 - 11.6) 9.2 (7.1 - 11.6) 8.6 (7.5 - 10.3) <0.0001 None/mild (≤9.7) 164 (84) 107 (79) 46 (96) Moderate (9.8 - 11.2) 27 (14) 24 (19) 2 (4) Severe (≥11.3) 3 (2) 3 (2) 0 (0) Disclosures No relevant conflicts of interest to declare.

scholarly journals Neutrophil gelatinase–associated lipocalin as a biomarker of nephropathy in sickle cell disease

2021 ◽  
Author(s):  
Rajaa Marouf ◽  
Adekunle D. Adekile ◽  
Hadeel El-Muzaini ◽  
Rasha Abdulla ◽  
Olusegun A. Mojiminiyi
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Roc Curve ◽  
Screening Tests ◽  
Cell Disease ◽  
Urine Ngal ◽  
Neutrophil Gelatinase Associated Lipocalin ◽  
Plasma Ngal ◽  
Neutrophil Gelatinase

AbstractSickle cell nephropathy (SCN) develops via altered hemodynamics and acute kidney injury, but conventional screening tests remain normal until advanced stages. Early diagnostic biomarkers are needed so that preventive measures can be taken. This study evaluates the role of neutrophil gelatinase–associated lipocalin (NGAL) as a biomarker of SCN in steady state and vaso-occlusive crisis (VOC). In this case-control study, 74 sickle cell disease (SCD) patients (37 in steady state and 37 in VOC) and 53 control subjects had hematological and biochemical measurements including plasma and urine NGAL. Univariate and logistic regression analyses were used to find the associations between variables. The receiver operating characteristic (ROC) curve was used to determine the diagnostic performance characteristics of plasma and urine NGAL for detection of VOC. Plasma and urine NGAL, urine microalbumin:creatinine ratio, and urine protein:creatinine ratio were significantly higher in VOC. Microalbuminuria was present in 17.1% steady state and 32.0% VOC patients. Microalbuminuria showed significant correlations with age, plasma NGAL, WBC, and hemolytic parameters. Area under the ROC curve for plasma NGAL was 0.69 (95%CI = 0.567–0.813; p = 0.006) and 0.86 (95%CI = 0.756–0.954; p < 0.001) for urine NGAL. Urine NGAL cut-off value of 12.0 ng/mL had 95% sensitivity and 65% specificity. These results confirm the presence of nephropathy during VOC and suggest that plasma and urine NGAL would be useful in the identification of SCN. Urine NGAL should be used as the screening biomarker, and patients with VOC and urine NGAL > 12.0 ng/mL should be selected for aggressive management to prevent progression of renal damage.

scholarly journals Effects of Genotypes and Treatment on Oxygenscan Parameters in Sickle Cell Disease

Cells ◽  
2021 ◽  
Vol 10 (4) ◽  
pp. 811
Author(s):  
Camille Boisson ◽  
Minke A. E. Rab ◽  
Elie Nader ◽  
Céline Renoux ◽  
Celeste Kanne ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Cell Disease ◽  
Oxygen Gradient ◽  
Acute Complication ◽  
Adults And Children ◽  
The Difference

(1) Background: The aim of the present study was to compare oxygen gradient ektacytometry parameters between sickle cell patients of different genotypes (SS, SC, and S/β+) or under different treatments (hydroxyurea or chronic red blood cell exchange). (2) Methods: Oxygen gradient ektacytometry was performed in 167 adults and children at steady state. In addition, five SS patients had oxygenscan measurements at steady state and during an acute complication requiring hospitalization. (3) Results: Red blood cell (RBC) deformability upon deoxygenation (EImin) and in normoxia (EImax) was increased, and the susceptibility of RBC to sickle upon deoxygenation was decreased in SC patients when compared to untreated SS patients older than 5 years old. SS patients under chronic red blood cell exchange had higher EImin and EImax and lower susceptibility of RBC to sickle upon deoxygenation compared to untreated SS patients, SS patients younger than 5 years old, and hydroxyurea-treated SS and SC patients. The susceptibility of RBC to sickle upon deoxygenation was increased in the five SS patients during acute complication compared to steady state, although the difference between steady state and acute complication was variable from one patient to another. (4) Conclusions: The present study demonstrates that oxygen gradient ektacytometry parameters are affected by sickle cell disease (SCD) genotype and treatment.

scholarly journals Is there a role for selective vasodilation in the management of sickle cell disease?

Blood ◽  
1988 ◽  
Vol 71 (3) ◽  
pp. 597-602 ◽  
Author(s):  
GP Rodgers ◽  
MS Roy ◽  
CT Noguchi ◽  
AN Schechter
Keyword(s):  
Blood Flow ◽  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Microvascular Obstruction ◽  
Controlled Study ◽  
Control Group ◽  
Cell Disease ◽  
Retinal Arteriolar

Abstract To test the hypothesis that microvascular obstruction to blood flow at the level of the arteriole may be significant in individuals with sickle cell anemia, the ophthalmologic effects of orally administered nifedipine were monitored in 11 steady-state patients. Three patients with evidence of acute peripheral retinal arteriolar occlusion displayed a prompt reperfusion of the involved segment. Two other patients showed fading of retroequatorial red retinal lesions. Color vision performance was improved in six of the nine patients tested. The majority of patients also demonstrated a significant decrease in the amount of blanching of the conjunctiva which reflects improved blood flow to this frequently involved area. Such improvements were not observable in a control group of untreated stable sickle cell subjects. These findings support the hypothesis that inappropriate vasoconstriction or frank vasospasm may be a significant factor in the pathogenesis of the microvascular lesions of sickle cell disease and, further, that selective microvascular entrapment inhibition may offer an additional strategy to the management of this disorder. We believe a larger, placebo-controlled study with nifedipine and similar agents is warranted.

Acute phase proteins and interleukins in steady state sickle cell disease

2009 ◽  
Vol 61 (1) ◽  
pp. 49-54 ◽  
Author(s):  
Konstantinos L. Bourantas ◽  
Georgios N. Dalekos ◽  
Alexandres Makis ◽  
Aristidis Chaidos ◽  
Stavroula Tsiara ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Acute Phase ◽  
Acute Phase Proteins ◽  
Cell Disease

scholarly journals Serum ferritin and total units transfused for assessing iron overload in adults with sickle cell disease

2012 ◽  
Vol 157 (5) ◽  
pp. 645-647 ◽  
Author(s):  
Emma Drasar ◽  
Nisha Vasavda ◽  
Norris Igbineweka ◽  
Moji Awogbade ◽  
Marlene Allman ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Iron Overload ◽  
Sickle Cell ◽  
Serum Ferritin ◽  
Cell Disease

scholarly journals Normal Non-HDL Cholesterol, Low Total Cholesterol, and HDL Cholesterol Levels in Sickle Cell Disease Patients in the Steady State: A Case-Control Study of Tema Metropolis

2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Richard K. D. Ephraim ◽  
Patrick Adu ◽  
Edem Ake ◽  
Hope Agbodzakey ◽  
Prince Adoba ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Steady State ◽  
Sickle Cell Disease ◽  
Total Cholesterol ◽  
Sickle Cell ◽  
Density Lipoprotein ◽  
Hdl Cholesterol ◽  
Lipoprotein Cholesterol ◽  
Cell Disease ◽  
Cross Sectional

Background.Abnormal lipid homeostasis in sickle cell disease (SCD) is characterized by defects in plasma and erythrocyte lipids and may increase the risk of cardiovascular disease. This study assessed the lipid profile and non-HDL cholesterol level of SCD patients.Methods.A hospital-based cross-sectional study was conducted in 50 SCD patients, in the steady state, aged 8–28 years, attending the SCD clinic, and 50 healthy volunteers between the ages of 8–38 years. Serum lipids were determined by enzymatic methods and non-HDL cholesterol calculated by this formula: non-HDL-C = TC-HDL-C.Results.Total cholesterol (TC) (p=0.001) and high-density lipoprotein cholesterol (HDL-C) (p<0.0001) were significantly decreased in cases compared to controls. The levels of non-HDL-C, low-density lipoprotein cholesterol (LDL-C), and triglyceride (TG) were similar among the participants. The levels of decrease in TC and HDL were associated with whether a patient was SCD-SS or SCD-SC. Systolic blood pressure and diastolic blood pressure were each significantly associated with increased VLDL [SBP,p=0.01, OR: 0.74 (CI: 0.6–0.93); DBP,p=0.023, OR: 1.45 (CI: 1.05–2.0)].Conclusion.Dyslipidemia is common among participants in this study. It was more pronounced in the SCD-SS than in SCD-SC. This dyslipidemia was associated with high VLDL as well as increased SBP and DBP.

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