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Sickle cell retinopathy in Jamaican children: further observations from a cohort study
J F Talbot1, A C Bird, G H Maude
1Medical Research Council Laboratories, University of the West Indies, Kingston, Jamaica.
Insights
Peripheral retinal vessel closure is common in children with sickle cell disease (SCD), affecting up to 90% by age 12. Lower hemoglobin and weight in SS disease, and high mean cell volume in SC disease, are associated with vessel closure.
Area of Science:
- Ophthalmology
- Hematology
- Pediatrics
Background:
- Sickle cell disease (SCD) is a genetic blood disorder with significant systemic complications.
- Retinopathy is a known complication of SCD, but its progression in pediatric populations requires further characterization.
Purpose of the Study:
- To investigate the prevalence and risk factors of peripheral retinal vessel closure in children with SCD.
- To assess the incidence of proliferative retinopathy in this cohort.
Main Methods:
- Serial retinal examinations and fluorescein angiography/angioscopy were conducted in children aged 5-13 years with SCD.
- A cohort study design with 1229 patient-years of observation was employed.
- Matched pair analysis identified risk factors for complete vessel closure.
Main Results:
- Peripheral retinal vessel closure increased from approximately 50% at age 6 to 90% by age 12 in children with SS and SC genotypes.
- In SS disease, complete closure correlated with lower total and fetal hemoglobin and lower weight.
- In SC disease, high mean cell volume and low platelet count were associated with closure risk.
- Proliferative retinopathy was rare, observed in only one child.
Conclusions:
- Peripheral retinal vessel closure is highly prevalent in pediatric SCD patients.
- Specific hematological parameters and anthropometrics are associated with retinopathy severity in different SCD genotypes.
- The low incidence of proliferative retinopathy suggests that early detection and management of vessel closure may be effective.
Abstract:
Serial retinal examinations were performed in children aged 5 years and older and fluorescein angiography/angioscopy in children 6 years and older participating in a cohort study of sickle cell disease. There were 1229 patient years of observation among 389 children aged 5-13 years. Peripheral retinal vessel closure was present in approximately 50% of children with SS and SC genotypes at age 6 years and increased to affect 90% of children by age 12 years. A matched pair analysis, comparing groups with minimal and complete closure, indicated that complete closure was associated with significantly lower total haemoglobin and fetal haemoglobin levels and significantly lower weight in SS disease, whereas in SC disease the risk factors appeared to be high mean cell volume and low platelet count. Proliferative retinopathy was rare, occurring only once in an 8-year-old boy with SC disease, despite 592 patient years of observation in children over this age.