The global burden of pulmonary hypertension in sickle cell disease: a systematic review and meta-analysis

2016 ◽  
Vol 95 (11) ◽  
pp. 1757-1764 ◽  
Author(s):  
B. M. Musa ◽  
N. A. Galadanci ◽  
M. Coker ◽  
S. Bussell ◽  
M. H. Aliyu
Keyword(s):  
Systematic Review ◽  
Pulmonary Hypertension ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Meta Analysis ◽  
Global Burden ◽  
Cell Disease

scholarly journals The global burden of sickle cell disease in children under five years of age: a systematic review and meta-analysis

2018 ◽  
Vol 8 (2) ◽  
Author(s):  
Elizabeth Wastnedge ◽  
Donald Waters ◽  
Smruti Patel ◽  
Kathleen Morrison ◽  
Mei Yi Goh ◽  
...  
Keyword(s):  
Systematic Review ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Meta Analysis ◽  
Global Burden ◽  
Cell Disease ◽  
Children Under Five ◽  
Under Five

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.

scholarly journals Sickle cell disease, sickle trait and the risk for venous thromboembolism: a systematic review and meta-analysis

2018 ◽  
Vol 16 (1) ◽  
Author(s):  
Jean Jacques Noubiap ◽  
Mazou N. Temgoua ◽  
Ronni Tankeu ◽  
Joel Noutakdie Tochie ◽  
Ambroise Wonkam ◽  
...  
Keyword(s):  
Systematic Review ◽  
Venous Thromboembolism ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Meta Analysis ◽  
Cell Disease

scholarly journals Iron stores in pregnant women with sickle cell disease: a protocol for a systematic review and meta-analysis

BMJ Open ◽  
2019 ◽  
Vol 9 (9) ◽  
pp. e026497 ◽  
Author(s):  
Desmond Aroke ◽  
Benjamin Momo Kadia ◽  
Tsi Njim
Keyword(s):  
Systematic Review ◽  
Sickle Cell Disease ◽  
Iron Deficiency ◽  
Pregnant Women ◽  
Sickle Cell ◽  
Iron Deficiency Anaemia ◽  
Meta Analysis ◽  
Cochrane Library ◽  
Cell Disease ◽  
Deficiency Anaemia

IntroductionSickle cell disease (SCD) is the most common inherited disease worldwide. The greatest disease burden is seen in sub-Saharan Africa. Early diagnosis and improved care of people living with SCD have led to an increase in the number of women with SCD reaching the reproductive age. Iron deficiency anaemia remains the most common cause of anaemia in pregnancy, affecting 51%–63% of pregnancies in Africa. However, the unavailability of guidelines on supplementation of iron in this pregnant subpopulation often leaves clinicians in a fix. We propose to conduct the first systematic review and possibly a meta-analysis on the prevalence, associated factors and maternal/fetal outcomes of iron deficiency anaemia among pregnant women with SCD.Methods and analysisWe will search the following electronic databases for studies on the iron status of pregnant women with SCD: PubMed, MEDLINE, EMBASE, Google Scholar, African Journals Online, African Index Medicus, Popline and the Cochrane Library. After the selection of eligible studies from the search output, review of full text, data extraction and data synthesis will be performed. Studies obtained from the review shall be evaluated for quality, risk of bias and heterogeneity. Appropriate statistical methods shall be used to pool prevalence estimates for matching studies globally and in subpopulations. This protocol has been reported as per the 2015 guidelines of the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols.Ethics and disseminationThere is no requirement for ethical approval as the proposed study will use published data. The findings of this study will be published in a peer-reviewed journal and will be presented at conferences.Trial registration numberCRD42018109803.

scholarly journals Adverse maternal and perinatal outcomes in pregnant women with sickle cell disease: systematic review and meta-analysis

Blood ◽  
2015 ◽  
Vol 125 (21) ◽  
pp. 3316-3325 ◽  
Author(s):  
E. Oteng-Ntim ◽  
D. Meeks ◽  
P. T. Seed ◽  
L. Webster ◽  
J. Howard ◽  
...  
Keyword(s):  
Systematic Review ◽  
Sickle Cell Disease ◽  
Pregnant Women ◽  
Sickle Cell ◽  
Meta Analysis ◽  
Perinatal Outcomes ◽  
Cell Disease

scholarly journals The Role of Hydroxyurea to Prevent Silent Stroke in Sickle Cell Disease: Systematic Review and Meta-Analysis

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 2373-2373
Author(s):  
Carla Hasson ◽  
Lisa Veling ◽  
Juan Felipe Rico ◽  
Rahul Mhaskar
Keyword(s):  
Systematic Review ◽  
Sickle Cell Disease ◽  
Adverse Events ◽  
Sickle Cell ◽  
Observational Studies ◽  
Assessment Tool ◽  
Conflicts Of Interest ◽  
Meta Analysis ◽  
Blood Transfusions ◽  
Cell Disease

Abstract Chronic blood transfusions are standard of care for stroke prevention in sickle cell disease. We evaluated hydroxyurea's efficacy in preventing silent stroke. We searched for randomized controlled trials (RCTs) and observational studies on Pubmed, CENTRAL, Embase, and Web of Science without using language/time limits. Eligible studies compared hydroxyurea with transfusions or observation to prevent silent stroke. This systematic review adheres to the Cochrane guidelines. Data were pooled using random effects model using STATA to perform meta-analysis. Methodological quality of RCTs was investigated using the Cochrane risk of bias assessment tool, while observational studies were assessed using the Downs & Black Checklist for Study Quality. One RCT enrolling 121 participants was included. There were no strokes, silent strokes, or deaths reported in either arm. There was no difference between hydroxyurea versus chronic blood transfusions (RR 1.52, 95% CI 0.57 to 4.02, P = 0.39) for adverse events. We included 10 observational studies, with 361 participants receiving hydroxyurea. There were no deaths attributed to hydroxyurea. Approximately 1% (I2 = 48.67%, 95% CI 0.0 to 0.05, 314 participants, seven comparisons) of patients receiving hydroxyurea had stroke. Approximately 18% (I2 = 91.37%, 95% CI 0.03 to 0.4, 266 participants, six comparisons) of the hydroxyurea patients had silent stroke. Approximately 24% (I2 = 88.54%, 95% CI 0.02 to 0.57, 91 participants, four comparisons) of the hydroxyurea patients had adverse events attributed to hydroxyurea. Our findings suggest that hydroxyurea is safe and may prevent silent stroke and stroke. More high-quality studies are needed. Disclosures No relevant conflicts of interest to declare.

scholarly journals Association between sickle cell disease and dental caries: a systematic review and meta-analysis

Hematology ◽  
2020 ◽  
Vol 25 (1) ◽  
pp. 309-319
Author(s):  
Hui Yue ◽  
Xinxin Xu ◽  
Qin Liu ◽  
Xiaozhi Li ◽  
Wei Jiang ◽  
...  
Keyword(s):  
Systematic Review ◽  
Sickle Cell Disease ◽  
Dental Caries ◽  
Sickle Cell ◽  
Meta Analysis ◽  
Cell Disease

scholarly journals Pulmonary complications for women with sickle cell disease in pregnancy: systematic review and meta-analysis

Thorax ◽  
2020 ◽  
Vol 75 (7) ◽  
pp. 568-575 ◽  
Author(s):  
Sivarajini Inparaj ◽  
Mickey Buckingham ◽  
Laura Oakley ◽  
Paul T Seed ◽  
Sebastian Lucas ◽  
...  
Keyword(s):  
Systematic Review ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Maternal Morbidity ◽  
Meta Analysis ◽  
Pulmonary Complications ◽  
Morbidity And Mortality ◽  
Cell Disease ◽  
Maternal Morbidity And Mortality ◽  
In Pregnancy

BackgroundSickle cell disease (SCD) is a multisystem disease characterised by vaso-occlusive crisis, chronic anaemia and a shorter lifespan. More patients with SCD are living till reproductive age and contemplating pregnancy. Pulmonary complications in pregnancy are significant causes of maternal morbidity and mortality but yet this has not been systematically quantified. A systematic review and meta-analysis were conducted to quantify the association between SCD and pulmonary complications in pregnancy.MethodsMEDLINE, EMBASE, Web of Science, Cochrane and Maternity and Infant Care databases were searched for publications between January 1998 and April 2019. Observational studies involving at least 30 participants were included. Random-effects models were used for statistical meta-analysis.FindingsTwenty-two studies were included in the systematic review and 18 in the quantitative analysis. The meta-analysis included 3964 pregnancies with SCD and 336 559 controls. Compared with women without SCD, pregnancies complicated by SCD were at increased risk of pulmonary thromboembolism (relative risk (RR) 7.74; 95% CI 4.65 to 12.89). The estimated prevalence of acute chest syndrome and pneumonia was 6.46% (95% CI 4.66% to 8.25%), with no significant difference between the HbSS and HbSC genotypes (RR 1.42; 95% CI 0.90 to 2.23).InterpretationThis meta-analysis highlighted a strong association between SCD and maternal pulmonary complications. Understanding the risks of and the factors associated with pulmonary complications would aid preconceptual counselling and optimal management of the condition in pregnancy, thereby reducing associated maternal morbidity and mortality.PROSPERO registration numberCRD42019124708.

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