Key Findings
- Purpose
To characterize body mass index (BMI) distribution and associated clinical factors in individuals with sickle cell disease across diverse global settings. - Population
532 patients with SCD from the multinational CASiRe cohort (United States, Italy, and Ghana) spanning pediatric and adult care. - Headline Result
- Underweight affected 9.5% of pediatric/adolescent patients and 17.2% of adults, highest in severe genotypes (SS/SB0Thal) and in Ghana.
- Overweight/obesity reached 26.8% in adults (vs 14% pediatrics), peaking at 49% in HbSC and 32% among individuals receiving hydroxyurea.
- BMI distribution varied by country, age, genotype severity, and treatment exposure, consistent with an evolving nutritional phenotype in SCD.
- Why It Matters
SCD care has historically focused on growth failure and undernutrition; clinicians now encounter a dual nutritional burden that may influence cardiometabolic risk, organ complications, and long-term outcomes. - Evidence Gaps & Limitations
Cross-sectional design limits causal inference; dietary intake, socioeconomic contributors, and longitudinal metabolic outcomes were not evaluated.
Source: Journal of Sickle Cell Disease — “International variations in body mass index within the CASiRe global cohort of sickle cell disease patients”
Regulatory & Guideline Watch
Current SCD guidelines emphasize organ-specific complications such as renal and cardiopulmonary disease, but lack SCD-specific obesity assessment and management protocols, despite obesity emerging as a risk factor alongside persistent undernutrition.