SynthesisSystematic reviews2020
Screening for sickle cell disease in newborns: a systematic review.
Synthesis in Systematic reviews, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
10 citing papers in PubMed, 22 citations in OpenAlex.
- The phenotypes of sickle cell disease: strategies to aid the identification of undiagnosed patients in the Italian landscape.Italian journal of pediatrics · 2025Review
- Findings supporting neonatal screening for sickle cell disease: an observational study in Senegal.Frontiers in pediatrics · 2025Article
- Newborn Screening for Six Primary Conditions in a Clinical Setting in Morocco.International journal of neonatal screening · 2024Article
- Determination of birth prevalence of sickle cell disease using point of care test HemotypeSC™ at Rundu Hospital, Namibia.BMC pediatrics · 2024Article
- The value-based price of transformative gene therapy for sickle cell disease: a modeling analysis.Scientific reports · 2024Article
- Multi-Organ Morphological Findings in a Humanized Murine Model of Sickle Cell Trait.International journal of molecular sciences · 2023Article
- Hematological and molecular analyses of the HbS allele among the Sudanese population.The Journal of international medical research · 2022Article
- Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018.International journal of general medicine · 2022Article
- Building research capacity for sickle cell disease in Africa: Lessons and challenges from establishing a birth cohort in Tanzania.Frontiers in pediatrics · 2022Article
- Role of Primary Care in Bridging Gaps in the Health Care System for Vulnerable Children in the United States of America: A Sickle Cell Disease Case Study.Journal of primary care & community healthArticle
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundSickle cell disease (SCD) is an inherited autosomal recessive disorder caused by the replacement of normal haemoglobin (HbA) by mutant Hb (sickle Hb, HbS). The sickle-shaped red blood cells lead to haemolysis and vaso-occlusion. Especially in the first years of life, patients with SCD are at high risk of life-threatening complications. SCD prevalence shows large regional variations; the disease predominantly occurs in sub-Saharan Africa. We aimed to systematically assess the evidence on the benefit of newborn screening for SCD followed by an earlier treatment start.
methodsWe systematically searched bibliographic databases (MEDLINE, EMBASE, Cochrane Databases, and the Health Technology Assessment Database), trial registries, and other sources to identify systematic reviews and randomised controlled trials (RCTs) or non-randomised trials on newborn screening for SCD. The last search was in 07/2020. Two reviewers independently reviewed abstracts and full-text articles and assessed the risk of bias of the studies included. Data were extracted by one person and checked by another. As meta-analyses were not possible, a qualitative summary of results was performed.
resultsWe identified 1 eligible study with direct evidence: a Jamaican retrospective study evaluating newborn screening for SCD followed by preventive measures (prevention of infections and education of parents). The study included 500 patients with SCD (intervention group, 395; historical control group, 105). Although the results showed a high risk of bias, the difference between the intervention and the control group was very large: mortality in children decreased by a factor of about 10 in the first 5 years of life (0.02% in the intervention group vs. 0.19% in the control group, odds ratio 0.09; 95% confidence interval [0.04; 0.22], p < 0.001).
conclusionThe results are based on a single retrospective study including historical controls. However, the decrease of mortality by a factor of 10 is unlikely to be explained by bias alone. Therefore, in terms of mortality, data from this single retrospective study included in our systematic review suggest a benefit of newborn screening for SCD (followed by preventive measures) versus no newborn screening for SCD (weak certainty of conclusions).
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.