Hospitalization for Acute Pain in Sickle Cell Disease: Changes in Clinical Parameters and Factors Predicting Hospital Discharge and Re-Admission

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 3662-3662
Author(s):  
Mehdi Nouraie ◽  
Debra L Weiner ◽  
Mariana Hildesheim ◽  
Ward Hagar ◽  
Oswaldo L Castro ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Hospital Stay ◽  
Sickle Cell ◽  
Research Funding ◽  
Ischemia Reperfusion ◽  
Serum Lactate Dehydrogenase ◽  
Index Hospitalization ◽  
Cell Disease ◽  
Readmission Risk

Abstract Introduction: Acute vaso-occlusive crisis (VOC) is the hallmark clinical complication of sickle cell disease (SCD). VOC involves several mechanisms, including rigid erythrocytes, adhesive blood cells, activated coagulation, activated and dysregulated endothelium, inflammation, and ischemia-reperfusion injury. Surprisingly few data document clinical and laboratory markers that change from steady state to VOC and during the hospital stay, and that predict subsequent rehospitalization. We characterized such markers in this study. Methods: Data were collected as part of a clinical trial of nitric oxide as potential treatment for VOC (DeNOVO). Participants, age ≥ 10 years requiring hospitalization for VOC, were recruited from 11 academic medical centers at the time of VOC. Length of hospital stay (LOS), change in visual analogue scale (VAS) pain score, and hospital readmission within 30 days were study outcomes. Steady state laboratory values were available in a subgroup of patients for comparison. Differences between admission and steady state laboratory measures and changes in clinical and laboratory parameters from hospital admission to discharge were analyzed by Wilcoxon signed-rank test. Multivariate linear and logistic regression analyses were used to identify the independent predictors of LOS and 30-day readmission risk. Results: 150 recruited patients had a median (interquartile range; IQR) age of 24 (17-33) years; 50% were female, and 91% had HbSS genotype. The median VAS score at time of admission was 7.7 cm which subsided to 3.3 cm at time of discharge (P <0.001). Serum lactate dehydrogenase concentrations (LDH) and neutrophils counts increased from steady state to the time of the VOC (P ≤ 0.001) and then decreased during the hospital stay (P ≤ 0.01) (Figure). The median (IQR) LOS was 3.5 (1.9-6.0) days. Longer LOS was predicted by higher admission VAS and serum alkaline phosphatase level, as well as higher increase in respiratory rate during hospital stay (P ≤ 0.006) (Table). The rate of 30-day hospital/ED readmission was 24%. Longer LOS was associated with lower risk of readmission (OR = 0.65, 95% CI: 0.50-0.86 per day). Other risk factors for readmission were higher serum creatinine at discharge (OR = 5.54, 95% CI: 1.12-27.51 per mg/dL), greater increase in serum alanine aminotransferase (OR = 1.03, 95%CI: 1.01-1.06 per unit increase) and greater cumulative opioid doses (OR = 1.25, 95%CI: 1.11-1.49 per 100 mg) during the index hospitalization. Conclusion: VOC is associated with neutrophilia and increased LDH, consistent with inflammation and hemolysis and/or tissue injury, although of insufficient magnitude to be used as diagnostic criteria. The magnitude of pain and marker of apparent bone involvement predict longer LOS. SCD patients with VOC carry a high burden of readmission risk, which is characterized by higher opioid utilization and earlier discharge from the index hospitalization. These concepts can shape models, especially to reduce readmission rate, to be evaluated in prospective clinical trials. *First two authors contributed equally to this work. Figure Figure. Table Table. Disclosures Lanzkron: NKT: Research Funding; Selexys: Research Funding; Prolong: Research Funding; PCORI: Research Funding; NHLBI: Research Funding; HRSA: Research Funding; GBT: Consultancy; Pfizer: Research Funding.

scholarly journals Blood mononuclear cell gene expression profiles characterize the oxidant, hemolytic, and inflammatory stress of sickle cell disease

Blood ◽  
2004 ◽  
Vol 104 (1) ◽  
pp. 270-280 ◽  
Author(s):  
Maria L. Jison ◽  
Peter J. Munson ◽  
Jennifer J. Barb ◽  
Anthony F. Suffredini ◽  
Shefali Talwar ◽  
...  
Keyword(s):  
Gene Expression ◽  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Expression Profiles ◽  
Ischemia Reperfusion ◽  
Transcriptional Analysis ◽  
Heme Oxygenase 1 ◽  
Specific Gene ◽  
Cell Disease

Abstract In sickle cell disease, deoxygenation of intra-erythrocytic hemoglobin S leads to hemoglobin polymerization, erythrocyte rigidity, hemolysis, and microvascular occlusion. Ischemia-reperfusion injury, plasma hemoglobin-mediated nitric oxide consumption, and free radical generation activate systemic inflammatory responses. To characterize the role of circulating leukocytes in sickle cell pathogenesis we performed global transcriptional analysis of blood mononuclear cells from 27 patients in steady-state sickle cell disease (10 patients treated and 17 patients untreated with hydroxyurea) compared with 13 control subjects. We used gender-specific gene expression to validate human microarray experiments. Patients with sickle cell disease demonstrated differential gene expression of 112 genes involved in heme metabolism, cell-cycle regulation, antioxidant and stress responses, inflammation, and angiogenesis. Inducible heme oxygenase-1 and downstream proteins biliverdin reductase and p21, a cyclin-dependent kinase, were up-regulated, potentially contributing to phenotypic heterogeneity and absence of atherosclerosis in patients with sickle cell disease despite endothelial dysfunction and vascular inflammation. Hydroxyurea therapy did not significantly affect leukocyte gene expression, suggesting that such therapy has limited direct anti-inflammatory activity beyond leukoreduction. Global transcriptional analysis of circulating leukocytes highlights the intense oxidant and inflammatory nature of steady-state sickle cell disease and provides insight into the broad compensatory responses to vascular injury.

scholarly journals Clinical and Laboratory Predictors of 30-Day Hospital Readmission Risk in Adult Patients with Sickle Cell Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2384-2384
Author(s):  
Seyed Mehdi Nouraie ◽  
Melissa Saul ◽  
Enrico M Novelli ◽  
Gregory J. Kato ◽  
Mark T Gladwin
Keyword(s):  
Health Care ◽  
Liver Disease ◽  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Hospital Stay ◽  
Sickle Cell ◽  
Hospital Readmission ◽  
Adult Patients ◽  
Cell Disease ◽  
Readmission Risk

Abstract Introduction: Thirty-day readmission risk is widely accepted as an indicator of quality of care. Sickle cell disease (SCD) has one the highest hospital readmission risk with a wide variation between different studies. In recent studies, older age, insurance status, and systemic complications including sepsis, renal and liver disease increased the risk of readmission whereas blood transfusion reduced the risk. During hospital stay, patients experience a variety of changes in their symptoms and laboratory measures. Evidence on the role of these changes on readmission risk is limited. In the current study, we aimed to assess the clinical and laboratory predictors of 30-day readmission risk in SCD adult patients in a tertiary health care system. Methods: Medical record discharge abstract files which cover visits for the SCD patients at the University of Pittsburgh Medical Center (UPMC) were extracted from electronic health records. Laboratory test results were obtained for each admission and were linked to discharge data. For each admission ICD 9/10 codes were used to identify the comorbidities. Blood transfusion information was recorded during each the admission. Natural language processing was used to extract medical concepts from chest X-ray and CT scan reports during patient's admission. Acute chest syndrome/pneumonia were identified from a combination of ICD codes and radiologic reports. For each laboratory value, a single rate of change (trajectory) was calculated with a random coefficient model from any measures during the hospital stay. Rate of changes were categorized to negative and positive trajectory. We used Generalized Estimating Equations models to assess predictors of 30-day readmission risk including the relationship between negative trajectory of any laboratory values duration the admission. Results: During January 2010 to May 2016, data for 2,108 hospital admissions in 173 SCD unique adult patients (median age of 32, 57% female and 59% SS genotype) were extracted. Risk of 30-day readmission was 41.2%. Older age (P <0.001) but not genotype (P = 0.8) predicted a lower readmission risk. Blood transfusion reduced readmission risk by 15% (Figure a). This effect was more significant in younger age (P for interaction with age = 0.045, Figure b). The most common discharge diagnoses were chronic pulmonary heart disease (23%), acute chest/pneumonia (22%), chronic renal disease (12%) and chronic liver disease (7%). Trajectory of neutrophil and WBC count changes were negative in 85% and 78% of admissions, respectively. These values were 67% for hemoglobin, 71% for creatinine and 46% for platelet count. During the period of hospital stay, decline in WBC (OR = 0.47, P = 0.030), neutrophil count (OR = 0.37, P = 0.023) or creatinine (OR = 0.40, P = 0.004) was associated with lower readmission risk. Conclusions: These results support that cardiopulmonary comorbidities are unexpectedly common in adult SCD patients. Blood transfusion in younger SCD patient reduced the readmission risk. Renal complications and leukocytosis in these patients contributed to health care utilization. Using advanced predictive models can help us to define patients who are at higher risk of readmission and generate strategies to reduce hospital readmission. Disclosures No relevant conflicts of interest to declare.

scholarly journals High-Throughput Mirna Analysis Suggests Pro-Inflammatory Profile in Sickle Cell Disease

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 1077-1077
Author(s):  
Matthew Cannon ◽  
Sarah Glass ◽  
Sidney Smith ◽  
Melanie Heinlein ◽  
Rosa Lapalombella ◽  
...  
Keyword(s):  
Chronic Pain ◽  
Steady State ◽  
Sickle Cell Disease ◽  
Red Blood Cells ◽  
Sickle Cell ◽  
Blood Cells ◽  
Research Funding ◽  
Red Cell ◽  
Cell Disease ◽  
Acute Crisis

Abstract BACKGROUND: Mature circulating red blood cells, though devoid of a nucleus, have been shown to contain an abundance of miRNAs. Further, it has been shown that sickle cell patient-derived RBCs have a dramatic difference in miRNA content than normal RBCs. Given that a range of miRNAs are involved in the regulation of immunity, including the release of inflammatory mediators, we hypothesize that miRNAs enriched in circulating red blood cells function to prolong the inflammatory state in sickle cell disease. Further, we hypothesize that these miRNAs can be used as biomarkers for use in the clinic to predict crisis and differentiate acute versus chronic pain. Exploring this miRNA enrichment in circulating red blood cells in sickle cell patients will provide practical insight for the inflammation state and will inform characteristics of patients who may need greater care in the clinic. METHODS: Twenty steady state patients were recruited and categorized according to their chronic pain status and crisis frequency per year. Whole blood was drawn during routine visits to the OSU Wexner Medical Center Hematology Clinic. Additionally, whole blood was drawn from five patients either in acute pain crisis (recruited prior to crisis) or within a few days of crisis. Samples were subject to double gradient centrifugation and red cells were resuspended in Trizol and cryopreserved. MiRNAs were isolated from red cell Trizol suspensions using a commercial isolation kit (QIAGEN Cat#217004). Isolated miRNAs were then subject to a NanoString Human miR (v3) expression assay. Differential expression analysis was conducted to compare miRNAs with at least 1.5 fold difference (p = 0.05) between steady state and acute crisis. Target prediction and GO ontology analysis was performed for statistically significant miRNAs using DIANA Tools mirPath v3. Follow-up qPCRs were performed using TaqMan Advanced miRNA cDNA Synthesis Kit (Cat#A28007) and TaqMan Advanced miRNA Assays (Cat#A25576) to validate the decreased expression of miRNAs. Additional qPCRs were performed using TaqMan Gene Expression Assays (Cat#4331182) to investigate mRNA regulatory effects of significant miRNAs in the total red cell population. Western blots were also performed to investigate regulatory effects of these miRNAs at the protein level. RESULTS & CONCLUSION: Comparison of RBC miRNA profiles from patients during acute crisis to those in steady state shows several significantly decreased (>1.5 fold) miRNAs in crisis. Among these miRs we have found previously uncharacterized miRNAs, hsa-miR-2116-5p and hsa-miR-302d-3p. DIANA tools miRNA analysis software predicts these miRNAs to be involved in regulation of cell-to-cell adhesion pathways through gene transcripts such as Protocadherin Beta 6 (PCDHB6) and Neural Cell Adhesion Molecule 2 (NCAM2). Interestingly, inspection of miRNA predicted targets that fall under significant GO terms also predicts several individual miRNAs to regulate inflammatory response and nociceptive signaling gene transcripts like A20 (TNFAIP3) and Cathepsin S (CTSS). Validation of these miRNAs was performed via qPCR for 5 out of the 6 significantly decreased miRNAs. Of the 5 miRNAs tested, hsa-miR-2116-5p, hsa-miR-302d-3p, and hsa-miR-1246 were validated as having decreased expression in acute crisis patients compared to steady state. qPCRs were then performed to probe for miRNA based regulation of top predicted target mRNA transcripts. Both CTSS and TNFAIP3 showed increased expression of mRNA transcripts in acute crisis patient red cells as compared to steady state. Next, western blot analysis was performed on red cell protein lysate. Interestingly, this analysis revealed a pattern in activated CTSS expression that was independent of acute crisis. Steady state patients reporting chronic pain showed increased activated CTSS compared to those without chronic pain. Activated CTSS was not found in red cell lysates from three normal, non-SCD donors. Taken together, these results suggest that red blood cells may play a larger role in inflammation and pain responses in sickle cell disease than previously thought. Further these results suggest activated CTSS as a potential biomarker for differentiating chronic pain in patients. Follow-up studies are underway to further stratify and investigate these findings. Disclosures Desai: University of Pittsburgh: Research Funding; Ironwood: Other: Adjudication Committee; NIH: Research Funding; FDA: Research Funding; Selexy/Novartis: Research Funding; Pfizer: Research Funding.

Exhaled Carbon Monoxide as a Marker of Hemolysis In Transgenic Mouse Models of Sickle Cell Anemia.

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1642-1642
Author(s):  
John D Belcher ◽  
Julia Nguyen ◽  
Julie Furne ◽  
Julie Vineyard ◽  
Joan Denise Beckman ◽  
...  
Keyword(s):  
Carbon Monoxide ◽  
Sickle Cell Disease ◽  
Mouse Models ◽  
Normal Control ◽  
Sickle Cell ◽  
Research Funding ◽  
Cell Activation ◽  
Ischemia Reperfusion ◽  
Cell Disease ◽  
Free Heme

Abstract Abstract 1642 Sickle cell disease is characterized by recurring episodes of hemolysis and painful vasoocclusion, which leads to ischemia-reperfusion injury and organ damage. Recently, the roles of heme-induced oxidative stress, nitric oxide consumption, endothelial cell activation, and inflammation in sickle cell disease have been recognized, in part because of the development of transgenic murine models of this condition. These mouse models provide insights into the pathophysiology of human sickle cell disease and the development of new therapeutic approaches. However, hemolytic rates and disease severity vary considerably between mouse models. Ferrous (Fe2+) hemoglobin (Hb) is easily oxidized in circulation to ferric (Fe3+) Hb, which readily releases free heme. Free heme is degraded by heme oxygenase which produces carbon monoxide (CO) as a byproduct. Endogenous CO production has been used as a measure of in vivo hemolysis in humans and mice, but there have been no reports of endogenous CO production in sickle mouse models. VCO was measured in sickle mouse models using a previously described rebreathing technique t (Levitt et al. J Lab Clin Med 113:241-247). Expired CO levels were highest in Hb-S knock-in Townes-SS sickle mice, almost 7-fold higher than normal control Townes-AA mice (p<0.05). Expired CO also was significantly (p<0.05) higher in HbS-BERK mice compared to normal control HbA-BERK. Injection of phenylhydrazine or hemin into normal C57BL/6 mice also raised expired CO levels 24 hours after injection (p<0.05). Expired CO levels were not significantly higher than controls in the New York, S+S-Antilles, het-BERK and Townes-AS models. These data confirm that hemolytic rates differ markedly between models. Additional data will be presented on the effects of inhaled CO on expired CO and the hemolytic rate in the Townes-SS model. Disclosures: Belcher: Sangart: Consultancy, Research Funding. Vercellotti:Sangart: Consultancy, Research Funding.

scholarly journals Complement Activation during Vaso-Occlusive Pain Crisis in Pediatric Sickle Cell Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 858-858
Author(s):  
Justin J Yoo ◽  
Sara H Graciaa ◽  
Jayre A Jones ◽  
Zoey Zuo ◽  
Connie M Arthur ◽  
...  
Keyword(s):  
Steady State ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Complement Activation ◽  
Research Funding ◽  
Cell Disease ◽  
Intravascular Hemolysis ◽  
Acute Complications ◽  
Steady State Levels ◽  
Pain Crisis

Abstract Background: Sickle cell disease (SCD) affects millions of individuals worldwide with substantial morbidity and mortality. The sickle hemoglobin (HbS) polymerizes upon deoxygenation, causing rigid and adhesive red blood cells (RBCs), triggering vascular occlusion, greatly shortened RBC lifespan, and chronic hemolysis. Amongst acute complications in SCD, vaso-occlusive pain crisis (VOC) is the leading cause of hospitalization, with supportive care being the primary approach to management. We and others have recently demonstrated important contributions of complement to the pathophysiology of SCD. When the complement pathway (CP) is activated during SCD crises, inhibition at C5 using eculizumab, has been successful in treating various acute complications in SCD (Chonat et al, Haematologica). In this study, we prospectively analyzed the extent of CP activation among children with SCD presenting with VOC. Methods: Patients aged 0-21 years old managed at Children's Healthcare of Atlanta with homozygous sickle cell (SS) or S beta zero thalassemia genotypes were enrolled in an IRB-approved research study. Inclusion criteria included those requiring intravenous opioids for VOC, and excluded those with chronic pain, &gt;6 VOC admission in the previous 12 months or on chronic transfusions. Blood samples were collected within 48 hours of VOC presentation, and steady-state levels were obtained at a 4-week clinic follow-up. Data was analyzed using a paired t-test, and receiver operator characteristic (ROC) curves were generated comparing intra-person complement levels during acute VOC versus respective steady-state levels. Results: Sixty-four patients have been enrolled thus far, of which 43 (67%) had steady-state samples collected. The majority of patients (90.5%) have SS genotype with a mean (SD) patient age of 14.15 (4.68) years. Fifty-three (84.1%) patients reported taking hydroxyurea (HU). Fifty-nine (93.7%) patients had at least one VOC admission in the past 12 months, with an average of 2.98 (1.67) VOC admissions. Pain Score reported on 55 patients averaged 4.93 (4.78) on a pain scale of 0 to 10. Mean values during VOC and steady-state of hemoglobin (Hb) were 8.12 and 9.01 g/dL, platelet count 431 and 511, and lactate dehydrogenase (LDH) 549 and 483 U/L, respectively. Seventeen patients had complement work-up performed during acute and steady-state, and 4 of them had additional samples collected during subsequent VOC. Complement protein levels C3, C4, C5, properdin, factor B, and complement regulatory proteins factor H and I were unremarkable during VOC and steady-state. However, complement activation markers, specifically anaphylatoxins C3a, C5a and Bb were significantly elevated during VOC compared to steady-state (see Table 1) suggesting activation of alternative CP during VOC (see Table 1 and Figure 1A-C). Terminal complement complex (C5b9) was not statistically different between VOC and steady-state (Figure 1D, red dotted lines signify normal ranges). Remarkably, patients who re-presented with acute VOC exhibited similar increases in their C3a/C5a (Figure 1E-F), substantiating the increases related to their VOC. Hemoglobin and LDH (Figure 1G-H) were similarly significant, suggestive of intravascular hemolysis. Three (7.1%) patients developed acute chest syndrome, two of whom experienced respiratory failure requiring intensive care management, and all exhibited significant CP activation. The area under the curve (AUC) of the ROC curve was analyzed to determine the ability of complement biomarkers to differentiate VOC from steady-state. Based on the AUC of these biomarkers, complement anaphylatoxins C3a and C5a exhibited the highest AUC of 0.76 and 0.87, respectively. Discussion: To our knowledge, this is the first prospective and comprehensive evaluation of CP in patients with SCD during VOC and steady-states. These preliminary findings suggest CP activation is present in a large proportion of patients during VOC, with increased activation of alternative and common CP, associated with intravascular hemolysis. Minimal increase in C5b9 could be explained by a significant proportion (&gt; 80%) of our patients being on HU therapy, similar to prior data (Roumenina et al, AJH). Specifically, C3a/C5a, along with other biomarkers, could not only predict disease activity in patients during VOC, but provide pharmacological targets in VOC, which need further validation. Figure 1 Figure 1. Disclosures Stowell: Alexion: Consultancy; Argenx: Speakers Bureau; Grifols: Speakers Bureau. Chonat: Alexion: Consultancy, Research Funding; Agios: Consultancy, Research Funding; Novartis: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; Takeda: Consultancy, Research Funding.

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 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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