Related Experiment Video
Updated: Aug 21, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Underweight children older than 5 years with sickle cell anemia are at risk for early mortality in a low-resource
Lauren J Klein1, Shehu Umar Abdullahi2, Safiya Gambo3
1Department of Pediatrics, D. Brent Polk Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Monroe Carell Jr. Children's Hospital at Vanderbilt, Nashville, TN.
Insights
Underweight status in children with sickle cell anemia (SCA) is a significant risk factor for mortality. Early identification of underweight children with SCA can help screen for those at higher risk of death.
Area of Science:
- Pediatrics
- Hematology
- Nutritional Science
Background:
- Undernutrition is a known risk factor for mortality in young children.
- Its role in mortality for older children and adults with sickle cell anemia (SCA) is less understood.
- Sickle cell anemia (SCA) affects children globally, with significant impact in regions like Nigeria.
Purpose of the Study:
- To test the hypothesis that underweight is associated with mortality in school-aged children (5-12 years) with SCA.
- To identify nutritional status as a potential risk factor for mortality in this vulnerable population.
- To evaluate the utility of underweight as a screening tool for mortality risk in children with SCA.
Main Methods:
- Secondary analysis of data from the Primary Prevention of Stroke in Children with Sickle Cell Disease in Nigeria trial.
- Classification of nutritional status using World Health Organization growth references: underweight (weight-for-age z score), stunting (height-for-age z score), and wasting (body mass index z score).
- Pooled analysis to assess the association between weight-for-age z score and mortality hazard.
Main Results:
- Children with SCA who died during the study had a lower mean weight-for-age z score compared to survivors.
- A lower weight-for-age z score was significantly associated with an increased hazard of death.
- Underweight participants (weight-for-age z score <-1) exhibited a higher probability of death during follow-up.
Conclusions:
- Underweight status is a previously unrecognized risk factor for early mortality in school-aged children with SCA in Nigeria.
- Nutritional assessment, specifically identifying underweight, can serve as a simple screening method for children with SCA at risk of death.
- Findings highlight the critical interplay between nutrition and mortality in pediatric SCA, emphasizing the need for nutritional interventions.
Abstract:
Undernutrition is a risk factor for under-5 mortality and is also postulated to be a risk factor for mortality in older children and adults with sickle cell anemia (SCA). We tested the hypothesis that underweight is associated with mortality in children aged 5 to 12 years with SCA. We performed a secondary analysis of participants in the Primary Prevention of Stroke in Children with Sickle Cell Disease in Nigeria trial, a double-blind, parallel-group randomized controlled trial for low-dose or moderate-dose hydroxyurea in children with abnormal transcranial Doppler velocities and a comparison group of participants with nonelevated transcranial Doppler velocities in northern Nigeria. Nutritional status was classified as underweight (weight-for-age z score), stunting (height-for-age z score), and wasting (body mass index z score) using the World Health Organization growth reference. The mean weight-for-age z score was lower in children who died during the study than in those who survived. Otherwise, the baseline characteristics of children who died during the study were not significantly different from those of the children who survived. A pooled analysis of participants demonstrated that a lower weight-for-age z score was associated with an increased hazard of death. Underweight participants (weight-for-age z score <-1) had a greater probability of death during follow-up than those who were not underweight. Underweight status in school-aged children with SCA is a previously unrecognized risk factor for early mortality in Nigeria and can be easily applied to screen children at risk for death. This trial was registered at www.clinicaltrials.gov as #NCT02560935.
Related Concept Videos
Anorexia Nervosa
Symptoms and Physical Effects
Individuals with anorexia nervosa commonly exhibit extreme...
Bone Marrow Sampling and Transplants
The transplant begins with high doses of chemotherapy and radiation treatment, which aim to destroy...
Chronic Kidney Disease II: Clinical Manifestations
Relative Risk
Immunodeficiency Diseases
There are three main causes of immunodeficiency...
Disorders of Erythrocytes
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
On the other...

