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Purpose <p>To analyze clinical, laboratory, and Doppler vascular parameters in pediatric sickle cell disease (SCD) patients and identify correlations and predictive factors for sickle cell retinopathy (SCR) and proliferative SCR (PSR).</p> Methods <p>A retrospective study included pediatric SCD patients screened for SCR between December 2023 and August 2024. Systemic, transcranial-cervical Doppler, and ophthalmologic evaluations were performed. Correlation analyses explored relationships between clinical, laboratory, and ophthalmologic parameters and SCR. Logistic regression identified predictive factors for SCR and PSR.</p> Results <p>We included 172 eyes from 86 pediatric SCD patients (mean age: 11.1 years; 57% male). SCR was diagnosed in 20 patients (23.3%), including 15 with non-proliferative (NPSR) and 5 with PSR. SCR correlated significantly with higher vaso-occlusive crises (VOC) frequency (ρ = 0.379, <i>p</i> &lt; 0.001), lower fetal hemoglobin (HbF) (ρ = -0.363, <i>p</i> = 0.001), older age (<i>r</i> = 0.295, <i>p</i> = 0.006), and glucose-6-phosphate dehydrogenase (G6PD) deficiency (ρ = 0.428, <i>p</i> &lt; 0.001). Doppler evaluations showed reduced velocities associated with SCR (<i>p</i> &lt; 0.05). Logistic regression identified G6PD deficiency (OR = 8.34, <i>p</i> = 0.014), increased VOC (OR = 2.22, <i>p</i> = 0.011), older age (OR = 1.26, <i>p</i> = 0.04), and lower HbF (OR = 0.89, <i>p</i> = 0.047) as predictors of SCR. An age cut-off of 11.5 years yielded 65% sensitivity and 64% specificity.</p> <p>For PSR, significant correlations included SC genotype (<i>r</i> = 0.728, <i>p</i> &lt; 0.001) and higher hemoglobin (Hb) (<i>r</i> = 0.518, <i>p</i> = 0.019). Lower hydroxyurea doses were linked to PSR (<i>r</i> = -0.548, <i>p</i> = 0.012). Hb levels predicted PSR, with a 9.2&#xa0;g/dL cut-off (80% sensitivity, 86% specificity).</p> Conclusion <p>Early detection of SCR is crucial in pediatric SCD patients. Key risk factors include older age (cut-off 11.5 years), higher VOC frequency, G6PD deficiency, and lower HbF for SCR, and SC genotype, reduced hydroxyurea doses, and higher Hb (cut-off 9.2&#xa0;g/dL) for PSR. Regular ophthalmologic screening and targeted management may help prevent vision loss and improve clinical outcomes.</p>

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Sickle cell retinopathy in pediatric patients: exploring correlations and predictive factors

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年8月23日

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  • Nuno Rodrigues Alves,
  • Catarina Barão,
  • Patrícia Barros Silva,
  • Inês Ludovico,
  • Lívio Costa,
  • Eduardo Silva,
  • Rita Anjos,
  • Rita Serras Pereira

摘要

Purpose

To analyze clinical, laboratory, and Doppler vascular parameters in pediatric sickle cell disease (SCD) patients and identify correlations and predictive factors for sickle cell retinopathy (SCR) and proliferative SCR (PSR).

Methods

A retrospective study included pediatric SCD patients screened for SCR between December 2023 and August 2024. Systemic, transcranial-cervical Doppler, and ophthalmologic evaluations were performed. Correlation analyses explored relationships between clinical, laboratory, and ophthalmologic parameters and SCR. Logistic regression identified predictive factors for SCR and PSR.

Results

We included 172 eyes from 86 pediatric SCD patients (mean age: 11.1 years; 57% male). SCR was diagnosed in 20 patients (23.3%), including 15 with non-proliferative (NPSR) and 5 with PSR. SCR correlated significantly with higher vaso-occlusive crises (VOC) frequency (ρ = 0.379, p < 0.001), lower fetal hemoglobin (HbF) (ρ = -0.363, p = 0.001), older age (r = 0.295, p = 0.006), and glucose-6-phosphate dehydrogenase (G6PD) deficiency (ρ = 0.428, p < 0.001). Doppler evaluations showed reduced velocities associated with SCR (p < 0.05). Logistic regression identified G6PD deficiency (OR = 8.34, p = 0.014), increased VOC (OR = 2.22, p = 0.011), older age (OR = 1.26, p = 0.04), and lower HbF (OR = 0.89, p = 0.047) as predictors of SCR. An age cut-off of 11.5 years yielded 65% sensitivity and 64% specificity.

For PSR, significant correlations included SC genotype (r = 0.728, p < 0.001) and higher hemoglobin (Hb) (r = 0.518, p = 0.019). Lower hydroxyurea doses were linked to PSR (r = -0.548, p = 0.012). Hb levels predicted PSR, with a 9.2 g/dL cut-off (80% sensitivity, 86% specificity).

Conclusion

Early detection of SCR is crucial in pediatric SCD patients. Key risk factors include older age (cut-off 11.5 years), higher VOC frequency, G6PD deficiency, and lower HbF for SCR, and SC genotype, reduced hydroxyurea doses, and higher Hb (cut-off 9.2 g/dL) for PSR. Regular ophthalmologic screening and targeted management may help prevent vision loss and improve clinical outcomes.