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Methodological Quality and Content Analysis of Clinical Practice Guidelines for Fever Management in Children With Sickle Cell Disease

In brief

Fever guidelines for sickle-cell children average below 50% quality and lack disease-specific focus

A systematic review identified only eight clinical practice guidelines addressing acute fever in pediatric sickle cell disease, and none were dedicated to the condition; the overall quality averaged 46% (range 25-67%). This gap highlights the urgent need for rigorously developed, disease-specific guidance to standardize care and reduce infection-related mortality.

Journal
Pediatric blood & cancer (Q1)
Published
4 August 2026
Study design
Practice guideline / consensus
Evidence level
Level 1, High (CEBM 1c)
Authors
Elspeth Bittle, Tea Reljic, Ambuj Kumar, Jane S Hankins, Sheena Mukkada
PMID
42549946
DOI
10.1002/1545-5017.70529

Why clinicians should know about it

Abstract

Infection/sepsis remains the number one cause of death among children under the age of 5 with sickle cell disease (SCD). Although clinical practice guidelines (CPGs) are an important tool for standardizing evidence-based care, the quantity and quality of existing CPGs for managing acute fever in children with SCD is unknown. We conducted a systematic review of CPGs published in the last 10 years addressing acute fever management in children with SCD. A PubMed search was conducted using standard search filters for CPGs, pediatrics, fever, and SCD. Additionally, a manual search of article citations, professional societies and consortia websites, and inquiries with experts was performed. CPGs were appraised by two reviewers using the Appraisal of Guidelines for Research and Evaluation II (AGREE II) instrument [http://www.agreetrust.org]. Results are summarized using descriptive statistics. The systematic and manual searches yielded 839 articles; 8 CPGs met inclusion criteria, but none were dedicated SCD acute fever CPGs. Geographic representation included France, India, Italy, Nigeria, sub-Saharan Africa, the United Kingdom, and the United States. The mean overall quality score was 46% (range 25%-67%). Rigorously developed CPGs focused on managing acute fever in children with SCD are lacking. This work highlights the need to generate a high-quality evidence-based CPG for managing acute fever in pediatric SCD.

Abstract as published, via PubMed.

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For healthcare professionals. The summary is generated by AI from the published abstract, and the evidence level is assigned automatically from the study design on the Oxford CEBM hierarchy. Neither is medical advice. Read the full paper before changing practice.