Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis

Abstract Background and purpose Silent cerebral infarcts (SCIs) are the most common neurological complication in children and adults with sickle cell disease (SCD). In this systematic review, we provide an overview of studies that have detected SCIs in patients with SCD by cerebral magnetic resonanc...

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Main Authors: Maite E. Houwing, Rowena L. Grohssteiner, Marjolein H. G. Dremmen, Ferdows Atiq, Wichor M. Bramer, Anne P. J. de Pagter, C. Michel Zwaan, Tonya J. H. White, Meike W. Vernooij, Marjon H. Cnossen
Format: Article
Language:English
Published: BMC 2020-12-01
Series:BMC Medicine
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Online Access:https://doi.org/10.1186/s12916-020-01864-8
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author Maite E. Houwing
Rowena L. Grohssteiner
Marjolein H. G. Dremmen
Ferdows Atiq
Wichor M. Bramer
Anne P. J. de Pagter
C. Michel Zwaan
Tonya J. H. White
Meike W. Vernooij
Marjon H. Cnossen
author_facet Maite E. Houwing
Rowena L. Grohssteiner
Marjolein H. G. Dremmen
Ferdows Atiq
Wichor M. Bramer
Anne P. J. de Pagter
C. Michel Zwaan
Tonya J. H. White
Meike W. Vernooij
Marjon H. Cnossen
author_sort Maite E. Houwing
collection DOAJ
description Abstract Background and purpose Silent cerebral infarcts (SCIs) are the most common neurological complication in children and adults with sickle cell disease (SCD). In this systematic review, we provide an overview of studies that have detected SCIs in patients with SCD by cerebral magnetic resonance imaging (MRI). We focus on the frequency of SCIs, the risk factors involved in their development and their clinical consequences. Methods The databases of Embase, MEDLINE ALL via Ovid, Web of Science Core Collection, Cochrane Central Register of Trials via Wiley and Google Scholar were searched from inception to June 1, 2019. Results The search yielded 651 results of which 69 studies met the eligibility criteria. The prevalence of SCIs in patients with SCD ranges from 5.6 to 80.6% with most studies reported in the 20 to 50% range. The pooled prevalence of SCIs in HbSS and HbSβ0 SCD patients is 29.5%. SCIs occur more often in patients with the HbSS and HbSβ0 genotype in comparison with other SCD genotypes, as SCIs are found in 9.2% of HbSC and HbSβ+ patients. Control subjects showed a mean pooled prevalence of SCIs of 9.8%. Data from included studies showed a statistically significant association between increasing mean age of the study population and mean SCI prevalence. Thirty-three studies examined the risk factors for SCIs. The majority of the risk factors show no clear association with prevalence, since more or less equal numbers of studies give evidence for and against the causal association. Conclusions This systematic review and meta-analysis shows SCIs are common in patients with SCD. No clear risk factors for their development were identified. Larger, prospective and controlled clinical, neuropsychological and neuroimaging studies are needed to understand how SCD and SCIs affect cognition.
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spelling doaj.art-bf5dbc08a2204ce6967ebba2efb69a742022-12-21T23:21:50ZengBMCBMC Medicine1741-70152020-12-0118111710.1186/s12916-020-01864-8Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysisMaite E. Houwing0Rowena L. Grohssteiner1Marjolein H. G. Dremmen2Ferdows Atiq3Wichor M. Bramer4Anne P. J. de Pagter5C. Michel Zwaan6Tonya J. H. White7Meike W. Vernooij8Marjon H. Cnossen9Department of Pediatric Haematology and Oncology, Erasmus MC – Sophia Children’s HospitalDepartment of Pediatric Haematology and Oncology, Erasmus MC – Sophia Children’s HospitalDepartment of Pediatric Radiology, Erasmus MC – Sophia Children’s HospitalDepartment of Haematology, Erasmus MCMedical Library, Erasmus MCDepartment of Pediatric Haematology and Oncology, Erasmus MC – Sophia Children’s HospitalDepartment of Pediatric Haematology and Oncology, Erasmus MC – Sophia Children’s HospitalDepartment of Child and Adolescent Psychiatry, Erasmus MC – Sophia Children’s HospitalDepartment of Radiology and Nuclear Medicine, Erasmus MCDepartment of Pediatric Haematology and Oncology, Erasmus MC – Sophia Children’s HospitalAbstract Background and purpose Silent cerebral infarcts (SCIs) are the most common neurological complication in children and adults with sickle cell disease (SCD). In this systematic review, we provide an overview of studies that have detected SCIs in patients with SCD by cerebral magnetic resonance imaging (MRI). We focus on the frequency of SCIs, the risk factors involved in their development and their clinical consequences. Methods The databases of Embase, MEDLINE ALL via Ovid, Web of Science Core Collection, Cochrane Central Register of Trials via Wiley and Google Scholar were searched from inception to June 1, 2019. Results The search yielded 651 results of which 69 studies met the eligibility criteria. The prevalence of SCIs in patients with SCD ranges from 5.6 to 80.6% with most studies reported in the 20 to 50% range. The pooled prevalence of SCIs in HbSS and HbSβ0 SCD patients is 29.5%. SCIs occur more often in patients with the HbSS and HbSβ0 genotype in comparison with other SCD genotypes, as SCIs are found in 9.2% of HbSC and HbSβ+ patients. Control subjects showed a mean pooled prevalence of SCIs of 9.8%. Data from included studies showed a statistically significant association between increasing mean age of the study population and mean SCI prevalence. Thirty-three studies examined the risk factors for SCIs. The majority of the risk factors show no clear association with prevalence, since more or less equal numbers of studies give evidence for and against the causal association. Conclusions This systematic review and meta-analysis shows SCIs are common in patients with SCD. No clear risk factors for their development were identified. Larger, prospective and controlled clinical, neuropsychological and neuroimaging studies are needed to understand how SCD and SCIs affect cognition.https://doi.org/10.1186/s12916-020-01864-8Sickle cell diseaseSilent cerebral infarctionStrokeMagnetic resonance imaging
spellingShingle Maite E. Houwing
Rowena L. Grohssteiner
Marjolein H. G. Dremmen
Ferdows Atiq
Wichor M. Bramer
Anne P. J. de Pagter
C. Michel Zwaan
Tonya J. H. White
Meike W. Vernooij
Marjon H. Cnossen
Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis
BMC Medicine
Sickle cell disease
Silent cerebral infarction
Stroke
Magnetic resonance imaging
title Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis
title_full Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis
title_fullStr Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis
title_full_unstemmed Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis
title_short Silent cerebral infarcts in patients with sickle cell disease: a systematic review and meta-analysis
title_sort silent cerebral infarcts in patients with sickle cell disease a systematic review and meta analysis
topic Sickle cell disease
Silent cerebral infarction
Stroke
Magnetic resonance imaging
url https://doi.org/10.1186/s12916-020-01864-8
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