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The effects of nutritional supplementation for children and adolescents with sickle cell disease: a systematic review and meta-analyses

2025-04Journal article Open access
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datacite.subject.sdg03:Saúde de Qualidade
dc.contributor.authorOrsi, Bruna C.
dc.contributor.authorGorski, Daniela
dc.contributor.authorKrul, Naila E.
dc.contributor.authorWiens, Astrid
dc.contributor.authorBrito, Miguel
dc.contributor.authorTonin, Fernanda
dc.contributor.authorPontarolo, Roberto
dc.date.accessioned2025-03-03T14:40:54Z
dc.date.available2025-03-03T14:40:54Z
dc.date.issued2025-04
dc.description.abstractBackground & aims: Sickle cell disease (SCD), a neglected chronic genetic blood disorder that severely impacts the pediatric population, often leading to premature death, is associated with compromised nutritional status. This study aimed to evaluate the effect of nutritional supplementation on SCD-related complications. Methods: A systematic review with searches in PubMed, Scopus, and Web of Science was performed. Randomized controlled trials (RCT) assessing diet or supplements as a complementary therapy for children and adolescents with SCD were included (PROSPERO: CRD42024532369). The data for outcomes of interest (efficacy, safety) were pooled using pairwise and network meta-analyses with ranking (p-score) analysis. The results were presented as odds ratio or mean differences with 95 % confidence intervals (NMAstudio2.0). Results: Twenty RCTs were included (2002–2023) (n = 2058), analyzing 9 dietary supplements on different regimens. All patients use hydroxyurea as an active treatment. Supplementation with fatty acids (n = 3 studies) and l-arginine (n = 4) presented higher efficacy and safety, significantly improving pain intensity, vaso-occlusive crises (VOC), and inflammation when compared to usual care/placebo (p < 0.05). Vitamin D3 (n = 6) at different dosages may reduce respiratory complications and length of hospital stay, yet further studies are needed to confirm its significant effects. Evidence is limited and of poor quality regarding the effects of add-on vitamin A (n = 2), magnesium sulfate (n = 2), and zinc (n = 4) for this population. Conclusions: The complementary use of certain supplements (fatty acids, l-arginine, vitamin D3) can enhance the management of VOC and improve patients' physiological functions. These supplements are often affordable and can contribute towards the reduction of opioid use and shorten patients' hospital stays - especially in low/middle-income countries where resources are scarce. Although further studies are needed to refine these findings (e.g., appropriate doses/regimens), practical guidelines and decision-makers may benefit from updated evidence.eng
dc.description.sponsorshipThis work was partially supported by the Coordenação de Aperfeiçoamento de Pessoal de Nível Superior- Brasil (CAPES)Finance Code 001, by the Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) and by FCT/MCTES (UIDB/05608/2020 and UIDP/05608/2020) e H&TRC. Funders have no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, nor approval of the manuscript; decision to submit the manuscript for publication.
dc.identifier.citationOrsi BC, Gorski D, Krul NE, Wiens A, Brito M, Tonin FS, et al. The effects of nutritional supplementation for children and adolescents with sickle cell disease: a systematic review and meta-analyses. Clin Nutr. 2025;47:157-68.
dc.identifier.doi10.1016/j.clnu.2025.02.016
dc.identifier.issn0261-5614
dc.identifier.urihttp://hdl.handle.net/10400.21/21625
dc.language.isoeng
dc.peerreviewedyes
dc.publisherElsevier
dc.relation.hasversionhttps://www.sciencedirect.com/science/article/pii/S0261561425000512
dc.relation.ispartofClinical Nutrition
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.subjectNutrition
dc.subjectSickle cell disease
dc.subjectDietary supplements
dc.subjectChildren
dc.subjectAdolescents
dc.subjectSystematic review
dc.subjectMeta-analysis
dc.subjectFCT_UIDB/05608/2020
dc.subjectFCT_UIDP/05608/2020
dc.titleThe effects of nutritional supplementation for children and adolescents with sickle cell disease: a systematic review and meta-analyseseng
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage168
oaire.citation.startPage157
oaire.citation.titleClinical Nutrition
oaire.citation.volume47
oaire.versionhttp://purl.org/coar/version/c_970fb48d4fbd8a85
person.familyNameBrito
person.familyNameTonin
person.givenNameMiguel
person.givenNameFernanda
person.identifier.ciencia-id231F-F341-7E93
person.identifier.ciencia-idD01C-C700-9411
person.identifier.orcid0000-0001-6394-658X
person.identifier.orcid0000-0003-4262-8608
person.identifier.ridA-7970-2016
person.identifier.ridO-2050-2017
person.identifier.scopus-author-id35224551000
person.identifier.scopus-author-id56085115800
relation.isAuthorOfPublication4252d8e0-800c-4d67-8b13-0b711d860669
relation.isAuthorOfPublication61ded30e-ecec-4b3e-b953-2293e080ebdd
relation.isAuthorOfPublication.latestForDiscovery4252d8e0-800c-4d67-8b13-0b711d860669

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