Linda Wainwright

Hepatocellular carcinoma and liver tumors in South African children: a case for increased prevalence.

The high regional incidence of hepatocellular carcinoma (HCC) in South Africa also may be present in children of the region, although the link to hepatitis B (HBV) appears less clear. The objective of this study was to assess the incidence and probable causes of HCC in South African children.

Kaposi sarcoma in South African children

The incidence of Kaposis sarcoma (KS) in sub‐Saharan Africa, increased tens of times since the onset of the AIDS epidemic. There is, however, very little literature concerning the clinical features of this disease, its management and outcome in HIV‐positive children in Africa. This study describes retrospectively the clinical presentation of the malignancy, its management and outcome, in a series of HIV‐positive children.

Infection with human immunodeficiency virus-1 (HIV) among children with cancer in South Africa†

HIV increases the risk of certain cancers known, or thought, to have an underlying infectious etiology; the impact on the risk of cancer in children is less clear.

Paroxysmal nocturnal hemoglobinuria arising from Fanconi anemia.

The progress of a female child with African type Fanconi anemia that evolves in time into paroxysmal nocturnal hemoglobinuria is described. Modern diagnostic methods are used to confirm this process. A discussion of possible mechanisms ensues.

The Saint Siluan warning signs of cancer in children: Impact of education in rural South Africa

Two thirds of children with cancer never reach a specialist centre for treatment in South Africa. The majority of those who present have advanced disease. A campaign was undertaken to educate the public and the primary health workers on the Saint Siluan early warning signs of cancer in children. There was a statistically significant increase in the number of new patients referred in the 6 years following the campaign (P = 0.001), but did not succeed in achieving the referral of patients at earlier stages of disease. This list of the warning signs appears to be useful in promoting awareness of cancer. Pediatr Blood Cancer 2011;56:314–316.

A 20-Year Retrospective Review of Retinoblastoma at Two Tertiary Academic Hospitals in Johannesburg, South Africa

Aim: This paper presents a 20-year review of retinoblastoma in Johannesburg, South Africa, aiming to better characterize the disease in this sub-Saharan setting. Methods: The study represents a retrospective case series of retinoblastoma patients presenting to Charlotte Maxeke Johannesburg Academic Hospital and Chris Hani Baragwanath Academic Hospital between January 1, 1992, and December 31, 2011. Results: The total number of cases identified was 282, with 245 meeting the study inclusion criteria. Retinoblastoma comprised 6.9% of the total pediatric oncology presentations; 65.3% were unilateral, 34.3% bilateral, and 0.4% trilateral. The overall male-to-female ratio was 1.08. The mean age at presentation overall was 32.6 months (median 28.0), in the unilateral group 39.4 months (median 33.0), and in the bilateral group 19.7 months (median 17.0). The mean delay to presentation overall was 7.0 months (median 4.0). The most frequent presenting symptoms were leukocoria (37.1%) and proptosis (34.7%). The distribution of disease stages at presentation (International Retinoblastoma Staging System) was 1.6% stage 0, 24.1% stage I, 27.8% stage II, 16.3% stage III, and 25.3% stage IV. 26.5% defaulted care. The 5-year Kaplan-Meier survival estimate was 57.7% overall. Conclusion: This study shows that delayed presentation and refusal of therapy remains a significant barrier to effective treatment in this African setting.