Laparoscopic cholecystectomy in adult patients with beta-thalassemia or sickle cell disease

2005 ◽  
Vol 19 (12) ◽  
pp. 1668-1669 ◽  
Author(s):  
G. Marakis ◽  
T. E. Pavlidis ◽  
K. Ballas ◽  
S. Rafailidis ◽  
A. Sakantamis
Keyword(s):  
Laparoscopic Cholecystectomy ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Adult Patients ◽  
Beta Thalassemia ◽  
Cell Disease

Laparoscopic cholecystectomy for cholelithiasis in children with sickle cell disease

2017 ◽  
Vol 10 (4) ◽  
pp. 320-326 ◽  
Author(s):  
Yousef Al Talhi ◽  
Bader Hamza Shirah ◽  
Muteb Altowairqi ◽  
Yasmin Yousef
Keyword(s):  
Laparoscopic Cholecystectomy ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease

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

scholarly journals A Universal Approach to Correct Various HBB Gene Mutations in Human Stem Cells for Gene Therapy of Beta-Thalassemia and Sickle Cell Disease

2017 ◽  
Vol 7 (1) ◽  
pp. 87-97 ◽  
Author(s):  
Liuhong Cai ◽  
Hao Bai ◽  
Vasiliki Mahairaki ◽  
Yongxing Gao ◽  
Chaoxia He ◽  
...  
Keyword(s):  
Stem Cells ◽  
Gene Therapy ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Gene Mutations ◽  
Beta Thalassemia ◽  
Human Stem Cells ◽  
Cell Disease ◽  
Universal Approach

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.

Hypercoagulability in Sickle Cell Disease and Beta-Thalassemia

2008 ◽  
Vol 8 (7) ◽  
pp. 639-645 ◽  
Author(s):  
Sylvia Singer ◽  
Kenneth Ataga
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Beta Thalassemia ◽  
Cell Disease
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