scholarly journals Improvement in hemolysis and pulmonary arterial systolic pressure in adult patients with sickle cell disease during treatment with hydroxyurea

2009 ◽  
Vol 84 (8) ◽  
pp. 529-531 ◽  
Author(s):  
Matthew Olnes ◽  
Amy Chi ◽  
Carissa Haney ◽  
Rose May ◽  
Caterina Minniti ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Systolic Pressure ◽  
Adult Patients ◽  
Cell Disease ◽  
Arterial Systolic Pressure ◽  
Pulmonary Arterial Systolic Pressure ◽  
Pulmonary Arterial

Chronic Kidney Disease and Mild Elevations in Pulmonary Arterial Systolic Pressure Are Associated with Increased Mortality in Adult Patients with Sickle Cell Disease.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1539-1539
Author(s):  
Daniel Lorch ◽  
Daniel Spevack ◽  
Jane Little
Keyword(s):  
Chronic Kidney Disease ◽  
Sickle Cell Disease ◽  
Kidney Disease ◽  
Sickle Cell ◽  
Survival Data ◽  
Conflicts Of Interest ◽  
Large Population ◽  
Systolic Pressure ◽  
Cell Disease ◽  
Pulmonary Arterial

Abstract Abstract 1539 Poster Board I-562 Background Pulmonary arterial hypertension (PAH) has recently been shown to be an important predictor of prognosis in sickle cell disease (SCD). We studied this association in a large population of adults with SCD. Methods and Results 215 patients with sickle cell syndromes in whom echoes had been performed, 113 as outpatients and 102 as inpatients, were identified from our database. Clinical characteristics were analyzed, and survival data were extracted from a national mortality registry. At the time of examination, subjects were 39 ± 14 years old, with hematocrits (Hcts) of 23.6 ± 4.4. 78 out of 213 patients who had undergone echoes had PAH (PASP ≥ 40 mmHg) on echocardiogram. Those patients were older (41 ± 15 vs 37 ± 13 years, p=0.03), had higher creatinine values (1.3 vs 0.9 mg/dL, p=0.01) higher serum alkaline phosphatase levels (172.2 ± 23.2 vs 104.5 ± 5.5 U/L, p=.0058), and were more often taking anti-hypertensive medications (38 vs 17%, p<0.001). During a median follow-up of 7.8 years [interquartile range (IR); 3.9, 10.0] there were 30 deaths. In a multivariate Cox proportional hazards model, PAH (hazard ratio (HR) 11.3; 95% CI 2.7, 47.9), chronic kidney disease (CKD) (HR 17.0; 95% CI 3.7, 79.1, defined as an estimated glomerular filtration rate, eGFR, of <60 cc/min/1.73 m2) and hyperbilirubinemia (HR 13.1; 95% CI 2.0, 83.9) independently predicted mortality after adjustment for inpatient/outpatient status, age, sex, blood counts, measurement of chamber size and ventricular function on echocardiogram, oxygen saturation, history of transfusion and background medical therapy. The association of PAH and mortality was not modified by inpatient/outpatient status (p=0.6). Conclusion In a community-based academic center, we found that mild elevation in PASP on echocardiogram, whenever obtained (see figure), depressions in eGFR (see figure), and hyperbilirubinemia were highly predictive for mortality in sickle cell disease. It is unclear whether these relationships are causal or an epiphenomenon of severe underlying disease. Hemolysis (lower Hcts, higher Lactate dehydrogenase levels), implicated in other studies, was not prominent in the PAH cohort. Disclosures No relevant conflicts of interest to declare.

Quality Metrics and Health Care Utilization for Adult Patients with Sickle Cell Disease

2019 ◽  
Vol 111 (1) ◽  
pp. 54-61 ◽  
Author(s):  
Monica Ter-Minassian ◽  
Sophie Lanzkron ◽  
Alphonse Derus ◽  
Elizabeth Brown ◽  
Michael A. Horberg
Keyword(s):  
Health Care ◽  
Sickle Cell Disease ◽  
Health Care Utilization ◽  
Sickle Cell ◽  
Quality Metrics ◽  
Adult Patients ◽  
Care Utilization ◽  
Cell Disease

Abstract 65: Anemia-related Heart Failure in Sub-saharan African Sickle Cell Disease Patients

2017 ◽  
Vol 121 (suppl_1) ◽  
Author(s):  
Adebayo C Atanda ◽  
Yahya Aliyu ◽  
Oluwafunmilayo Atanda ◽  
Aliyu Babadoko ◽  
Aisha Suleiman ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Hemoglobin Level ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Jet Velocity ◽  
Sub Saharan

Introduction: Anemia has been implicated in heart failure. Existing literatures, involving predominantly African-Americans, suggests that Sickle Cell Disease (SCD) maybe linked to various cardiovascular complications including pulmonary hypertension and left venticular dysfunction. Peculiarly, our study involves exclusively Sub-Saharan population. Method: We conducted a cross sectional observational study of 208 hydroxyurea-naive consecutive SCD patients aged 10-52 years at steady state and 94 healthy non-matched controls who were studied in an out patient clinic in Sub-Saharan Africa. SCD patients were required to have electrophoretic or liquid chromatography documentation of major sickling phenotypes. Control group was required to have non-sickling phenotypes. Cardiac measurements were performed with TransThoracic Echo according to American Society of Echocardiography guidelines. Hemoglobin level was also obtained. Results: Hemoglobin level in SCD group (8.5+/- 1.5) was significant (P<0.001) compared to control (13.8+/- 1.7). Although SCD group had significantly higher values of left ventricular (LV) size, there was no qualitative evidence of LV dysfunction. SCD group had higher values of Ejection Fraction but not statistically significant. There was no evidence of LV wall stiffening to impair proper filling in SCD group, with the ratio of early to late ventricular filling velocities, E/A ratio elevated (1.7+/-0.4 compared to 1.6+/- 0.4; P=0.010). Right ventricular systolic pressure was determined using the formula of 4x Tricuspid Reugurgitant jet (TRV) square as an indirect measurement of Pulmonary arterial systolic pressure. SCD patients had significantly higher mean±SD values for tricuspid regurgitant jet velocity than did the controls (2.1±0.6 vs. 1.8±0.5; p= 0.001). Within the SCD group, there was no clear pattern of worsening diastolic function with increased TRV. Furthermore, E/A had a significant positive relationship with jet velocity in bivariate analysis (R=0.20; P=0.013). Conclusions: We were unable to demonstrate existence of anemia-associated left ventricular dysfunction in Sub-Saharan African with SCD. Further studies is required to highlight the reason behind this finding.

scholarly journals Instruments to Measure Perceptions in the Emergency Department Provider-Patient with Sickle Cell Disease Interaction: Findings of an Integrative Review from a Ph.D. Project

2020 ◽  
Vol 14 (1) ◽  
pp. 263-284
Author(s):  
Aindrea B. Maddray ◽  
Shannon M. Phillips
Keyword(s):  
Emergency Department ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Reliability And Validity ◽  
Adult Patients ◽  
Integrative Review ◽  
Cell Disease ◽  
Related Stigma ◽  
Health Related ◽  
Adequate Reliability

Background & Purpose: The purpose of this review is to examine instruments that measure providers' perceptions of adult patients with Sickle Cell Disease (SCD), examine instruments that measure adult patients with SCD perceptions of providers' behaviors, and determine optimal instruments to use in evaluating the perceptions of Emergency Department (ED) providers and adult patients with SCD of one another's behaviors after an interaction in the ED. Methods: An integrative review was conducted searching EBSCOhost and PubMed databases using the keywords: measure [OR] measure* [OR] assess* [OR] scale [OR] survey [OR] tool [AND] stigma* [OR] stereotype [OR] prejudice [OR] bias [OR] perception [OR] attitude [OR] discrimination [OR] racism [OR] behavior [AND] interaction [OR] relationship [OR] communication [AND] sickle cell. Initial search located 256 articles, but only 15 articles were included in the final review. Results: Fifteen articles reporting six instruments were reviewed. Four instruments evaluated a provider’s perceptions of patients with SCD behaviors, and two instruments evaluated how patients with SCD perceived provider behaviors. The two patient-focused instruments and three provider-focused instruments were found to be adequately reliable and valid according to the Psychometric Grading Framework (PGF). Conclusions: The findings suggest that the General Perceptions About Sickle Cell Disease Patients Scale would be an optimal instrument to evaluate ED providers' perceptions of adult patients with SCD behaviors. One patient-focused instrument, The Sickle Cell Health-Related Stigma Scale (SCD-HRSS), reported adequate reliability and validity but was not specific to measuring the patient's perceptions of ED providers' behaviors, nor was it administered in the ED environment. The SCD-HRSS Doctors subscale has potential adaptability for use in measuring patients with SCD perceptions of ED provider behaviors in the ED environment.

scholarly journals Efficacy of Allogeneic Hematopoietic Cell Transplantation (HCT) in Sickle Cell Disease: Results of a Systematic Review/Meta-Analysis

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2106-2106
Author(s):  
Madiha Iqbal ◽  
Tea Reljic ◽  
Ernesto Ayala ◽  
Hemant S. Murthy ◽  
Ambuj Kumar ◽  
...  
Keyword(s):  
Systematic Review ◽  
Sickle Cell Disease ◽  
Clinical Outcomes ◽  
Sickle Cell ◽  
Pediatric Patients ◽  
Medical Literature ◽  
Meta Analysis ◽  
Adult Patients ◽  
Cell Disease ◽  
Allogeneic Hct

Background: Sickle cell disease (SCD) is an inherited hemoglobinopathy which affects over 300,000 children born each year worldwide. In spite of improvement in supportive care in recent years, there is still a lack of effective treatment options. SCD leads to debilitating and cyclic episodes of erythrocyte sickling with progressive organ injury, contributing to lifetime morbidity and shortened life expectancy. Allogeneic HCT (allo-HCT) is a potentially curative therapy for SCD because engraftment is associated with resolution of the clinical phenotype of the disease and abrogation of its complications. Medical literature on allo-HCT for SCD is largely limited to children. Recent studies have evaluated the efficacy of allo-HCT in the adult population. Here, we conduct a systematic review/meta-analysis to assess the totality of evidence pertaining to the efficacy (or lack thereof) of allo-HCT in children and adults. Materials and methods: We performed a comprehensive search of the medical literature using PubMed/Medline, EMBASE and Cochrane library on July 3rd, 2019. We extracted data on clinical outcomes related to benefits (overall [OS] and disease free/event free survival [EFS/DFS]) and harms (non-relapse mortality [NRM] and graft failure [GF]), independently by two authors. Our search strategy identified 1001 references but only 30 studies (n= 1995 patients) were included in this systematic review/meta-analysis. We also performed a sub analysis on clinical outcomes for studies that included only pediatric patients (defined as <18 years) and those in patients ≥18 years of age. Results: Median age for patients enrolled in all the studies was at 10 years. Recurrent veno-occlusive crises represented the most common indication for allo-HCT followed by acute chest syndrome and stroke; nevertheless, most patients had more than one indication. Matched related donors (MRD) were the most common donor source (93%). Bone marrow was the most common source of hematopoietic stem cells (77%). Majority of patients underwent conditioning with myeloablative regimens (77%). Pooled OS rates (n=29 studies, 1681 patients) after allogeneic HCT was 95% (95%CI=93-96%) with low heterogeneity (I2=6.4%) among included studies (Figure 1). Pooled EFS/DFS rates (n=29 studies, 1894 patients) post-allografting was 90% (95%CI=87-93%) with moderate heterogeneity (I2=54%). Pooled NRM rates from 30 studies (1995 patients) was 4% (95%CI=2-6%) with low heterogeneity (I2=29.4%). Pooled GF rates from 28 studies (1851 patients) was 4% (95%CI=2-6%) with moderate heterogeneity (I2=55%). A subset analysis specifically for pediatric patients (n= 11 studies, 1009 patients, median age at 9.7 years) showed a pooled OS rate of 96% (95%CI=94-97%) with low heterogeneity (I2=0%); and for adult patients (n=3 studies, 51 patients, median age at 33.4 years) the pooled OS was 94% (95%CI=80-100%) with moderate heterogeneity (I2=52%). Pooled EFS/DFS for pediatric patients (n= 11 studies, 1009 patients) was at 89 %( 95%CI=84-93%) with moderate heterogeneity (I2=55.1%); and for adult patients (n=2 studies, 30 patients) was at 95% (95%CI=83-100%) with high heterogeneity (I2=96.5%). Pooled NRM from 10 studies with pediatric patients (281 patients) was at 6 % (95%CI=3-10%) with low heterogeneity (I2=0%); and from 3 studies with adult patients (51 patients) was at 1% (95%CI=0-7%) with low heterogeneity (I2=15.1%). Pooled GF from 10 studies with pediatric patients (281 patients) was at 3 % (95%CI=1-7%) with moderate heterogeneity (I2=40%); and from 2 studies with adult patients (30 patients) was at 5% (95%CI=0-17%) with high heterogeneity (I2=95.4%). Conclusions: The results of our systematic review/meta-analysis show excellent OS, EFS/DFS in children and adults undergoing allo-HCT with pooled OS rates exceeding 90%. The main limitation to offering an allo-HCT in SCD remains the availability of a suitable donor as 85% of patients meeting criteria do not have a MRD. We anticipate that with emergence of haploidentical transplantation the number of allo-HCT will increase in the future. GF remains a significant concern in this population and future studies should focus on novel immune suppression strategies to help reduce GF. Disclosures Kharfan-Dabaja: Pharmacyclics: Consultancy; Daiichi Sankyo: Consultancy.

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