An exploratory study of physiological correlates of neurodevelopmental delay in infants with sickle cell anaemia
Alexandra M Hogan1, Fenella J Kirkham, Mara Prengler
1Institute of Child Health, University College London, London, UK. a.hogan@soton.ac.uk
Insights
Infants with sickle cell anaemia (SCA) show increased risk of neurodevelopmental delay. This delay is linked to SCA
Area of Science:
- Neuroscience
- Pediatrics
- Hematology
Background:
- Sickle cell anaemia (SCA) is a genetic blood disorder with potential systemic complications.
- Early identification of neurodevelopmental issues in infants with SCA is crucial for timely intervention.
- Existing research on early neurodevelopmental outcomes in SCA is limited.
Purpose of the Study:
- To investigate the risk of neurodevelopmental delay in infants with SCA.
- To determine if neurodevelopmental delay is associated with SCA pathology.
- To explore early indicators of cognitive impairment in SCA infants.
Main Methods:
- Longitudinal assessment of 28 infants (14 SCA, 14 controls) using the Bayley Infant Neurodevelopmental Screener (BINS) at 3, 9, and 12 months.
- Recording of Transcranial Doppler (TCD) velocity and pulse oximetry (SpO2) in SCA infants and controls.
- Collection of haemoglobin values from SCA infants.
Main Results:
- SCA infants consistently scored higher for neurodevelopmental delay risk compared to controls across all assessment ages.
- A significant increase in moderate-to-high BINS risk scores was observed between 3 and 9 months in SCA infants.
- At 9 months, BINS scores negatively correlated with TCD velocity and positively with haemoglobin levels in SCA infants.
Conclusions:
- Infants with SCA may face a higher risk of neurodevelopmental delay than previously recognized.
- SCA pathology, indicated by TCD velocity and haemoglobin levels, appears associated with early neurodevelopmental outcomes.
- This study highlights the need for further research into the early precursors of cognitive impairment in SCA.
Abstract:
This study aimed to investigate whether infants with sickle cell anaemia (SCA) are at risk of neurodevelopmental delay, and whether any delay is associated with SCA pathology. Twenty-eight infants (14 SCA; 14 age- and ethnic-similar controls) were assessed longitudinally with the Bayley Infant Neurodevelopmental Screener (BINS) at 3, 9 and 12 months. Transcranial Doppler (TCD) and pulse oximetry (SpO2) measures were recorded longitudinally in SCA infants, and a subgroup of controls. Haemoglobin values were obtained from SCA infants. At each age, SCA infants obtained BINS scores indicative of greater risk of neurodevelopmental delay compared with controls. The number of moderate-high BINS risk scores increased significantly between 3 and 9 months. At 9 months BINS raw scores correlated negatively with TCD velocity and positively with haemoglobin. This exploratory study suggests that SCA infants may be at greater risk of neurodevelopmental delay than previously considered, and may provide the impetus for further research into the very early precursors of cognitive impairment.
