PATIENT-RELATED FACTORS AND CLINICAL MANAGEMENT OUTCOMES OF RETINOBLASTOMA IN CALABAR, NIGERIA.

Background: Retinoblastoma is curable in industrialized countries. However, it is associated with mortality in resource-poor nations due to disparities and poor access to eye care. Aim was to determine the relationships between patient-related factors and clinical outcomes of Retinoblastoma management in a tertiary hospital in Nigeria. Materials and methods: This was a retrospective study of all children who were diagnosed and treated for Retinoblastoma from January 2017 through December 2022. Information obtained from their records included biosocioeconomic data, symptoms, lag time from initial symptoms, staging, treatment and outcome (dead or alive). Results: Fifty-three patients, aged 6 to 88 months on first hospital presentation were recruited. There were 29(54.7%) females and 20(37.7%) patients died. Parental low socioeconomic class, rural residence and poor nutrition occurred more in those that survived, though not significantly (p>0.05). Median(interquartile) age at diagnosis [24(18-36) months, p=0.005] and lag time [13(6-20) months, p=0.274] were low in the survived group. Bilateral Retinoblastoma (20.8%,p=0.002), brain metastasis (22.6%,p<0.001), IRSS IV (18.9%,p=0.01) and relapse (34%,p<0.001) occurred more among the patients that died. The overall survival (OS) was 22(11.77-32.23) months with 1-year OS of 63%. Treatment with only chemotherapy [HR 4.76(95%CI:1.726-13.128)], incomplete chemotherapy [HR 5.61(95%CI:1.271-24.741)], relapse [HR 5.98(95%CI:1.376-25.983)] and eye surgery after 3 chemotherapy cycles [HR 8.22(95%CI:1.087-62.239)] were predictors of mortality. Conclusion: Early presentation of retinoblastoma especially of advanced and bilateral disease may lead to improved survival if chemotherapy and eye surgery are appropriately performed. Routine screening and immediate referral of retinoblastoma particularly in rural areas are recommended.

Prolonged QTc Interval in Nigerian Children with Sickle Cell Anemia.

Prolonged QTc interval, a risk factor for ventricular arrhythmia, occurs in sickle cell anemia. The aim of this study was to determine the risk of prolonged QTc interval and its relationship with vaso-occlusive painful crises (VOCs) and follow-up steady-state in the same children with sickle cell anemia. This prospective cohort study enrolled 38 subjects, aged 5-17 years. History of bone pain and examination were obtained during VOC and steady-state. Assessment of QTc interval was with 12-lead electrocardiography. The QTc interval value >0.440 seconds was taken as prolonged. Median (interquartile range) of QTc interval was higher during VOC [0.447 (0.438-0.459) seconds] than during steady-state [0.435 (0.417-0.440) seconds]. Risk of prolonged QTc interval was higher during VOC (68.4%) than in steady-state (21.1%) with relative risk of 3.250 [95% confidence interval (CI) = 1.692-6.241]. Prolonged QTc interval was likely to occur [area under curve (AUC) = 0.759, p<0.001] during VOC with 68.4% sensitivity, 78.9% specificity and at cutoff point of 0.441 seconds. Prolonged QTc interval negatively correlated with packed cell volume (PCV) during VOC [rs (36) = -0.14, p = 0.387]. Binary logistics of the combined effect of PCV and gender on QTc interval showed that during VOC, males were more likely to have prolonged QTc [odds ratio (OR): 1.337 (95% CI: 0.327-5.464; p = 0.686]. Children with sickle cell anemia, particularly males, were three-times more likely to have prolonged QTc interval during VOC when QTc interval was >0.441 seconds. Routine electrocardiography may help to identify those with QTc intervals above this threshold for prompt cardiac-oriented management.