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Developmental screening in young children with sickle cell disease. Results of a cooperative study
W C Wang1, R Grover, D Gallagher
1St. Jude Children's Research Hospital, Memphis, Tennessee.
Insights
Young children with sickle cell disease generally show normal development before age 3. Older children may exhibit developmental deficits, potentially due to ischemic damage, warranting cautious interpretation of screening tests.
Area of Science:
- Pediatric Health
- Hematology
- Developmental Pediatrics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder impacting child development.
- Early assessment of developmental trajectories in children with SCD is crucial for timely intervention.
Purpose of the Study:
- To evaluate the developmental status of young children diagnosed with sickle cell disease.
- To identify potential developmental delays or abnormalities in relation to SCD.
Main Methods:
- The Denver Developmental Screening Test (DDST) was administered to 344 children under 6 years old.
- Testing was conducted by trained examiners across 12 institutions participating in the Cooperative Study of Sickle Cell Disease (CSSCD).
Main Results:
- Most children (90.4%) scored normal on the DDST; 1.5% had abnormal scores.
- Questionable or abnormal (Q/A) scores were significantly more frequent in children aged 3-5 years compared to younger children (12.6% vs. 3.8%, P = 0.002).
- No correlation was found between DDST results and specific sickle cell genotypes.
Conclusions:
- Development appears relatively normal in children with SCD before age 3.
- Increased Q/A scores in older children suggest potential neurodevelopmental impairments, possibly linked to ischemic events.
- The DDST is a screening tool and results require careful clinical interpretation, especially in the context of SCD.
Purpose:
The goal of the study was to assess development in young children with sickle cell disease as part of the Cooperative Study of Sickle Cell Disease (CSSCD).
Patients And Methods:
The Denver Developmental Screening Test (DDST) was administered to children younger than 6 years at 12 participating institutions of the CSSCD. Trained examiners administered tests to 344 children.
Results:
Tests were scored as normal in 90.4%, questionable in 6.4%, and abnormal in 1.5%; 1.7% of children were considered untestable. There was no relationship between DDST results and sickle cell genotype. Questionable and abnormal (Q/A) scores were more common in children ages 3-5 years than in younger children (12.6% versus 3.8%; P = 0.002).
Conclusions:
Because the DDST is a screening test, it should be interpreted cautiously. However, the more numerous Q/A scores in our "older" group agree with the findings of recent reports of neuropsychological impairment in school-age children with sickle cell disease. Our data suggest that development is relatively normal before age 3 years; deficits seen in older children may reflect subsequent ischemic insults.