Prevalence of Poverty and Hunger at Cancer Diagnosis and Its Association with Malnutrition and Overall Survival in South Africa.

Many South African children live in poverty and food insecurity; therefore, malnutrition within the context of childhood cancer should be examined. Parents/caregivers completed the Poverty-Assessment Tool (divided into poverty risk groups) and the Household Hunger Scale questionnaire in five pediatric oncology units. Height, weight, and mid-upper arm circumference assessments classified malnutrition. Regression analysis evaluated the association of poverty and food insecurity with nutritional status, abandonment of treatment, and one-year overall survival (OS). Nearly a third (27.8%) of 320 patients had a high poverty risk, associated significantly with stunting (p = 0.009), food insecurity (p < 0.001) and residential province (p < 0.001) (multinomial regression). Stunting was independently and significantly associated with one-year OS on univariate analysis. The hunger scale was significant predictor of OS, as patients living with hunger at home had an increased odds ratio for treatment abandonment (OR 4.5; 95% CI 1.0; 19.4; p = 0.045) and hazard for death (HR 3.2; 95% CI 1.02, 9.9; p = 0.046) compared to those with food security. Evaluating sociodemographic factors such as poverty and food insecurity at diagnosis is essential among South African children to identify at-risk children and implement adequate nutritional support during cancer treatment.

Nosocomial outbreak of hepatitis B virus infection in a pediatric hematology and oncology unit in South Africa: Epidemiological investigation and measures to prevent further transmission.

BACKGROUND: Hospital-acquired hepatitis B virus (HBV) infection has been well described and continues to occur worldwide. Recent nosocomial outbreaks have been linked to unsafe injection practices, use of multi-dose vials, and poor staff compliance with standard precautions. This report describes a nosocomial outbreak that occurred in a pediatric hematology and oncology unit of a large academic hospital, the epidemiological investigation of the outbreak, and preventive measures implemented to limit further in-hospital transmission. METHODS: Outbreak investigation including contact tracing and HBV screening were initially carried out on all patients seen by the unit during the same period as the first three cases. Routine screening for the entire patient population of the unit was initiated in February 2013 when it was realized that numerous patients may have been exposed. RESULTS: Forty-nine cases of HBV infection were confirmed in 408 patients tested between July 2011 and October 2013. Phylogenetic analysis of the HBV preC/C gene nucleotide sequences revealed that all tested outbreak strains clustered together. Most (67%) patients were HBeAg positive. The cause of transmission could not be established. Preventive measures targeted three proposed routes. HBV screening and vaccination protocols were started in the unit. CONCLUSIONS: The high number of HBeAg positive patients, together with suspected lapses in infection prevention and control measures, are believed to have played a major role in the transmission. Measures implemented to prevent further in-hospital transmission were successful. On-going HBV screening and vaccination programs in pediatric hematology and oncology units should become standard of care.

Malignancies in South African children with HIV.

OBJECTIVES: In 2008 the South African Children's Cancer Study Group decided to review the epidemiology, management, and chemotherapy response of HIV-positive children with malignancy. METHODS: This is a retrospective analysis of data collected from the records of HIV-positive children diagnosed with malignancy at 7 university-based pediatric oncology units serving 8 of the 9 provinces in South Africa. RESULTS: Two hundred eighty-eight HIV-positive children were diagnosed with 289 malignancies between 1995 and 2009. Age at diagnosis ranged from 17 days to 18.64 years; median 5.79 years. Of the 220 HIV-associated malignancies, there were 97 Kaposi sarcomas, 61 Burkitt lymphomas, 47 other B-cell lymphomas including 2 primary central nervous system lymphomas, 12 Hodgkin lymphomas, and 3 leiomyosarcomas. Sixty-nine patients presented with non-AIDS-defining malignancies. More than 80% presented with advanced disease. Most patients (76.7%) were naive to antiretroviral therapy; 22.2% did not have access because it only became available in public hospitals in 2004. One hundred ninety-seven children were treated with curative intent; 91 received palliative care due to advanced malignancy and/or advanced HIV disease. Nearly one third had coexisting pathology, mostly tuberculosis. Overall survival for the whole group was 33.7%, but was 57.8% for those treated with antiretroviral therapy and chemotherapy. CONCLUSIONS: The study shows more Kaposi sarcoma and fewer primary central nervous system lymphomas among HIV-positive children than that is reported in the developed world, but confirms a higher incidence of non-Burkitt B-cell lymphoma than in HIV-negative children. The high number of non-AIDS-defining malignancies underscores the prevalence of HIV-AIDS in South Africa. The overall survival should improve with universal access to antiretrovirals and earlier diagnosis.

Stem Cell Transplant in Immune-deficiency-associated Vaccine-derived Poliovirus.

Patients with severe primary immunodeficiency are at risk for complications from live-attenuated vaccines. Here, we report a case of a vaccine-associated paralytic polio and Bacille Calmette-Guérin disease in a 6-month-old girl with severe combined immunodeficiency resulting from homozygous recombinant activating gene 1 deficiency. The patient was successfully treated with intravenous immunoglobulins and oral pocapavir for poliovirus, and antimycobacterial therapy for regional Bacille Calmette-Guérin disease, allowing stem cell transplant. Following transplantation, poliovirus type 3 with 13 mutations was detected from cerebrospinal fluid but not from stool, indicating ongoing viral evolution in the central nervous system despite pocapavir treatment. Clinical improvement and immune reconstitution allowed the patient to be successfully discharged with no further detection of poliovirus.

Reporting Incidences of Neuroblastoma in Various Resource Settings.

PURPOSE: The incidences of neuroblastoma (NB) differ significantly between various resource settings because of varying quality of cancer registries and underdiagnoses. This study aimed to evaluate current regional variations as reported by international cancer registries and the theoretical and reported differences in international NB incidences and to evaluate South Africa (SA) as a case for variable reporting. METHODS: A comprehensive literature review on registries reporting on NB was performed to construct incidence tables. The SEER Program incidence of 10.5/million children was used to calculate the expected number of NB cases for each country. Registry data of NB cases between 2000 and 2016 were requested from The South African National Cancer registry (SA-NCR) and the South African Children's Tumour Registry (SACTR) for comparison and to perform a probabilistic linkage study. RESULTS: Internationally, incidences varied between -97.1% and +80% compared with the SEER program. SA under-reported NB cases by an estimated 74.2%. Between 2000 and 2016, the SA-NCR reported between 23 and 51 cases/year, whereas the SACTR reported between 18 and 57 cases/year for the same period. The incidence reported by the SA-NCR varied between 1.5 and 2.8/million children under 15-year per year, whereas the SACTR reported 1.74-2.6 cases/million children. Both registries reported incidences less than high-income country. A probabilistic record linkage of the two registries resulted in a combined incidence of 2.9 cases/million children. CONCLUSION: As with most low- and middle-income countries, SA has either a lower incidence or underdiagnoses of NB cases. The reasons for under-reporting are not clear, but can be due to undiagnosed NB cases with spontaneous regression, missed possible cases because of lack of autopsies, and diagnosed cases not recorded in registries.

The Utility of Metabolic Parameters on Baseline F-18 FDG PET/CT in Predicting Treatment Response and Survival in Paediatric and Adolescent Hodgkin Lymphoma.

Lymphoma is the third most common paediatric cancer. Early detection of high-risk patients is necessary to anticipate those who require intensive therapy and follow-up. Current literature shows that residual tumor avidity on PET (Positron Emission Tomography) following chemotherapy corresponds with decreased survival. However, the value of metabolic parameters has not been adequately investigated. In this retrospective study, we aimed to evaluate the prognostic value of metabolic and other parameters in paediatric and adolescent Hodgkin lymphoma. We recorded tMTV (total Metabolic Tumor Volume), TLG (Total Lesion Glycolysis), and SUVmax (maximum Standard Uptake Value) on baseline PET, as well the presence of bone marrow or visceral involvement. HIV (human immunodeficiency virus) status and baseline biochemistry from clinical records were noted. All patients received stage-specific standard of care therapy. Response assessment on end-of-treatment PET was evaluated according to the Deauville criteria. We found that bone marrow involvement (p = 0.028), effusion (p < 0.001), and treatment response (p < 0.001) on baseline PET, as well as HIV status (p = 0.036) and baseline haemoglobin (p = 0.039), were significantly related to progression-free survival (PFS), whereas only effusion (p = 0.017) and treatment response (p = 0.050) were predictive of overall survival (OS). Only baseline tMTV predicted treatment response (p = 0.017). This confirms the value of F-18 FDG PET/CT (Fluoro-deoxy-glucose Positron Emission Tomography/Computed Tomography) in prognostication in paediatric and adolescent Hodgkin lymphoma; however, further studies are required to define the significance of metabolic parameters.

Endocrine profiling in patients with Fanconi anemia, homozygous for a FANCG founder mutation.

BACKGROUND: Fanconi anemia (FA) is phenotypically diverse, hereditary condition associated with bone marrow failure, multiple physical abnormalities, and an increased susceptibility to the development of malignancies. Less recognized manifestations of FA include endocrine abnormalities. International discourse has highlighted that these abnormalities are widespread among children and adults with FA. To date there has been no systematic study that has evaluated the endocrine abnormalities in a cohort of patients with FA, homozygous for a founder mutation (c.637_643del (p.Tyr213Lysfs*6)) in FANCG. The objectives of the study were to evaluate endocrine gland function in patients with FA of a single FA genotype, and to determine the frequency and nature of endocrine abnormalities in this group. METHODS: Cross-sectional, descriptive study of 24 South African patients of African ancestry with FA (homozygous for a FANCG founder mutation). Outcomes measured included growth, pubertal status, growth hormone axis screening, thyroid gland function, glucose and insulin metabolism and bone age (BA). RESULTS: Endocrine dysfunction was present in 70.8% (17 of 24), including abnormal insulin-like growth factor 1 (IGF-1)/insulin-like growth factor-binding protein 3 (IGFBP-3) in 25.0% (6 of 24), insulin resistance in 41.7% (10 of 24), abnormal thyroid function in 16.7% (4 of 24) and short stature in 45.8% (11 of 24). No abnormalities of glucose metabolism were identified. Abnormal pubertal status was seen in three males (12.5%). Abnormal BAs were present in 34.8% (8 of 23). CONCLUSION: Endocrine abnormalities occur at a high frequency in patients with FA, homozygous for a FANCG founder mutation, similar to other FA cohorts. Our data are specific to FA patients with a single genotype, and therefore provide the first genotype-phenotype information on endocrine abnormalities in South African patients, homozygous for a FANCG founder mutation. Recommendations regarding endocrine screening in this patient subgroup are made, including, but not limited to, baseline testing of thyroid function, fasted insulin and glucose, and IGF-1 and IGFBP-3.

Torsion of a bifid omentum as a rare cause of acute abdomen: a case report.

BACKGROUND: Omental torsion is a rare and very unusual cause of acute abdominal pain. If often mimics other acute pathologies and it is very difficult to diagnose preoperatively, which can lead to deterioration of the patient. It is seldom reported in the literature. CASE PRESENTATION: We report a well-documented case of a 67-year-old white woman who complained about abdominal pain, which was slowly increasing in severity. She had no previous abdominal interventions. An abdominal ultrasound showed multiple gallstones. At laparoscopy, free hemorrhagic fluid was seen and further exploration showed torsion of the right part of her omentum. A partial omentectomy was performed. Her postoperative course was uneventful. CONCLUSIONS: Omental torsion is a rare cause of abdominal pain. Primary omental torsion is seldom reported in the literature. Blood examinations are frequently normal. Abdominal ultrasound and computed tomography can exclude other pathologies. Exploration remains the preferred diagnostic and therapeutic modality. Surgeons should include the diagnosis of omental torsion in their differential diagnosis of acute abdominal pain.

Investigating hepatitis B immunity in patients presenting to a paediatric haematology and oncology unit in South Africa.

BACKGROUND: Hepatitis B is an important public health concern in South Africa (SA). The hepatitis B virus (HBV) vaccine was introduced into the South African Expanded Programme on Immunisation (EPI-SA) in 1995. There is no 'catch-up' programme in place. The duration of protection after hepatitis B vaccination in the SA population is unknown. Waning of vaccine-induced immunity leaves people at risk of acquiring hepatitis B infection in settings where the prevalence of infection is high and horizontal transmission is likely. OBJECTIVE: To assess immunity to HBV in patients at presentation to a paediatric haematology and oncology unit. METHODS: An audit of hepatitis profiles was done of all new patients seen in the unit from January 2012 to December 2013. Patients were classified as immune (antibody levels to hepatitis B surface antigen (anti-HBs) >100 mIU/ml), low immune (anti-HBs 10 - 100 mIU/ml) and not immune (anti-HBs <10 mIU/ml). RESULTS: Of the 210 patients included (median age 6.5 years), 84 (40.0%) had no immunity to hepatitis B despite presumed vaccination as part of the EPI schedule. Six patients tested positive for hepatitis B core antibody (anti-HBc), consistent with previous infection. No patients had active hepatitis B infection (hepatitis B surface antigen-positive). Most human immunodeficiency virus (HIV)-infected patients were not immune to HBV (80.0%). CONCLUSION: A significant number of children in SA are not immune to hepatitis B despite vaccination being part of the EPI-SA. Combined passive-active immunisation should be considered for all oncology patients in settings where exposure to HBV is possible. Consideration should also be given to offering booster vaccination to the population as a whole.