Sickle-cell disorders: limits of descriptive epidemiology

Abstract

The global mapping of sickle haemoglobin (HbS) allele frequencies in neonates at a fine-grained 5×5 km resolution, undertaken by Frédéric Piel and colleagues 1 Piel FB Patil AP Howes RE et al. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimates. Lancet. 2012; (Published online Oct 25.)http://dx.doi.org/10.1016/S0140-6739(12)61229-X PubMed Google Scholar in The Lancet, uses a new approach extended from work on malaria (with which haemoglobin variation is closely associated 2 Piel FB Patil AP Howes RE et al. Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nat Commun. 2010; 1: 104 Crossref PubMed Scopus (302) Google Scholar , 3 Firdous N Gibbons S Modell B Falling prevalence of beta-thalassaemia and eradication of malaria in the Maldives. J Community Genet. 2011; 2: 173-189 Crossref PubMed Scopus (16) Google Scholar ), and thus adds a powerful new instrument to the haemoglobin research community's armamentarium. The contribution is multifaceted: updating the literature review to 2010; applying a rigorous method including use of the global rural urban mapping project (GRUMP); and publication of results in a prestigious journal, which highlights the position of sickle-cell disorders as a global health concern. 4 WHO59th World Health Assembly resolution WHA59.20 on sickle cell anaemia. http://apps.who.int/gb/ebwha/pdf_files/WHA59-REC1/e/Resolutions-en.pdf Google Scholar Although the coverage is currently limited to HbS, its extension, in time, to other alleles that interact with HbS to cause sickle-cell disorders (principally haemoglobin C and β-thalassaemias) will greatly enrich the outputs. Global epidemiology of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and population estimatesHbS will have an increasing effect on public health systems. Our estimates can help countries and the international community gauge the need for appropriate diagnoses and genetic counselling to reduce the number of neonates affected. Similar mapping and modelling methods could be used for other inherited disorders. Full-Text PDF Open AccessBoosting services for people with sickle-cell diseaseThe Igbo people of Nigeria called them Ogbanje—malevolent children destined to be lazy and die and be reborn repeatedly. Particular families were plagued by such children who usually died before their 10th birthday. Belief in Ogbanje is centuries old, but a little more than 100 years ago, a less ethereal explanation for the Ogbanje was found. Walter Clement Noel was of African descent and living in America. He had anaemia and chest pains, and in 1910 doctors Ernest Irons and James Herrick looked at his blood under a microscope. Full-Text PDF