Background: Outcome data for neuroblastoma in sub-Saharan Africa are minimal, whereas poor outcome is reported in low-and middle-income countries. A multi-institutional retrospective study across South Africa was undertaken to determine outcome. pediatric oncology units were included. Kaplan-Meier curves and Cox regression models were employed to determine two-year survival rates and to identify prognostic factors.Results: Data from 390 patients were analyzed. The median age was 39.9 months (range, 0-201 months). The majority presented with stage 4 disease (70%). The main chemotherapy regimens were OPEC/OJEC (44.8%), St Jude NB84 protocol (28.96%), and Rapid COJEC (22.17%).Only 44.4% had surgery across all risk groups, whereas only 16.5% of high-risk patients received radiotherapy. The two-year overall survival (OS) for the whole cohort was 37.6%: 94.1%, 81.6%, and 66.7%, respectively, for the very-low-risk, low-risk, and intermediate-risk groups and 27.6% for the high-risk group (P < 0.001, 95% CI). The median survival time for the whole group was
Four children are described, (three black and one white, two boys and two girls) with type A postaxial polydactyly. All four of them, in addition, had either a partial or complete atrioventricular septal defect (AVSD). None of these children had associated major malformations. Minor anomalies were observed (e.g., two patients with hypersegmentation of the sternal segments, one patient with undescended testes, one patient with hypoplastic lumbar vertebra, and one patient with a degree of craniofacial abnormality). Chromosome analysis was carried out for three of the four patients, and was normal in all of them. It is suggested that there is a specific association between type A postaxial polydactyly and the AVSD found in each of these patients. This picture does not conform to, but bears some resemblance to, the Ellis-van Creveld syndrome.
Fluorosis of the primary dentition was determined in 146 children living in a hot, dry African village with a fluoride concentration in the drinking water of 0.98-2.7 ppm. The prevalence of fluorosis was compared using DEAN's (8) and THYLSTRUP & FEJERSKOV's (14) indices. The prevalence varied according to tooth type, being highest in maxillary second molars (25%) and lowest in mandibular central incisors (4.6%). The two scoring systems produced almost identical prevalences of fluorosis but the severity scores varied. Generally THYLSTRUP & FEJERSKOV's index scores were one score higher than DEAN's. The THYLSTRUP & FEJERSKOV index is recommended for future studies.
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