Although high and variable frequencies of sickle cell trait
Although high and variable frequencies of sickle cell trait in different ethnic groups in Uganda have been reported previously, those data were from around 70 years ago and were based on small sample sizes. Thus, the findings of Ndeezi and colleagues provide a contemporary overview that is nationally representative. While the genesis of the S mutation is well recognised as a secondary factor to the endemicity of malaria and natural selection, the time is ripe to begin to address the genesis of new variants and their importance.
The children assessed in US3 were enrolled in the Early Infant Diagnosis programme for HIV screening and did not provide consent to be tested for sickle cell disease, a genetic disorder. Furthermore, the results were communicated to the congo red but optimum clinical care could not be assured. The ethical justification for this study could be debated, as it is unlikely that it would have been permissible in most developed countries, where the use of residual neonatal dried blood spots for research purposes has been challenged. Nevertheless, the Ugandan Government and Ministry of Health deserve commendation for making an important decision in favour of public health. This study provides an excellent example of a meaningful integration of screening programmes to provide useful data for health planning that could be emulated by other countries in sub-Saharan Africa, where sickle cell is highly prevalent and early infant diagnosis programmes are in place. Much too often in these countries, ministries of health partner with foreign donors to run several parallel programmes that are expensive and sometimes counterproductive.
23 (0·5%) of 5080 children with HIV assessed by Ndeezi and colleagues also had sickle cell disease, compared with 693 (0·8%) of 92 024 who were HIV negative. This finding suggests early mortality in children with comorbid sickle cell disease and HIV, and should be studied further. Several case reports suggest that sickle cell disease slows the progression of HIV, for which several hypotheses have been proposed that might offer new leads for HIV treatments. Almost all these reports, however, involved patients with horizontal infection. What happens in infants who acquire infection vertically and have sickle cell disease remains unclear and warrants exploration, for which there cannot be a better natural platform than where both disorders are highly prevalent. The introduction of early diagnosis of sickle cell disease in African countries would provide a unique opportunity to assess the interaction between this disorder and HIV.
The time for a worldwide concerted effort to control sickle cell disease is long overdue. Although technical support can be provided by partners in developed countries, as was the case in US3, the ultimate and long-term care of patients remains the prerogative of the host country. African countries and leaders, therefore, must own up to this responsibility, improve funding allocation for supporting care, and use appropriate media to promote public awareness and strategies to reduce incidence.
Finally, the goal of preventing early death in people with sickle cell disease in sub-Saharan Africa goes well beyond neonatal screening. In Brazil, such screening has not been associated with a substantial decline in early mortality. Thus, while early diagnosis is logical, what is more important is to provide good care afterwards, otherwise a neonatal screening programme could easily become another way of increasing the existing pool of people known to be affected by sickle cell disease.
Local transmission of Zika virus (ZIKV) in the Americas was first confirmed in February, 2014, on Easter Island. In May, 2015, 16 patients from the Brazilian states of Bahia and Rio Grande do Norte were found to be ZIKV-positive. 22 countries and territories have subsequently identified autochthonous transmission within the region (). Until recently, ZIKV infection was only associated with mild symptoms (headache, rash, joint pain, conjunctivitis) but a possible link between ZIKV infection during pregnancy and subsequent birth defects (most notably microcephaly) was identified in November, 2015. Approaching 4000 cases of suspected ZIKV-related microcephaly have arisen in Brazil alone where initial figures suggest between 440 000 and 1·3 million people have been infected so far. Concern in the region is escalating, with public health authorities in Colombia, Ecuador, El Salvador, and Jamaica all issuing an unprecedented health notice warning their residents to avoid pregnancy until 2018.