Sickle cell disease

FB Piel, MH Steinberg, DC Rees - New England Journal of …, 2017 - Mass Medical Soc
Sickle cell disease is caused by an alteration in a single DNA base, but its clinical
manifestations are influenced by other genes and behavioral and environmental factors …

Sickle-cell disease

DC Rees, TN Williams, MT Gladwin - The Lancet, 2010 - thelancet.com
Sickle-cell disease is one of the most common severe monogenic disorders in the world.
Haemoglobin polymerisation, leading to erythrocyte rigidity and vaso-occlusion, is central to …

World distribution, population genetics, and health burden of the hemoglobinopathies

TN Williams, DJ Weatherall - Cold Spring …, 2012 - perspectivesinmedicine.cshlp.org
Although information about the precise world distribution and frequency of the inherited
hemoglobin disorders is still limited, there is no doubt that they are going to pose an …

Human genetics and malaria resistance

SN Kariuki, TN Williams - Human genetics, 2020 - Springer
Malaria has been the pre-eminent cause of early mortality in many parts of the world
throughout much of the last five thousand years and, as a result, it is the strongest force for …

The pathogenesis of Plasmodium falciparum malaria in humans: insights from splenic physiology

PA Buffet, I Safeukui, G Deplaine… - Blood, The Journal …, 2011 - ashpublications.org
Clinical manifestations of Plasmodium falciparum infection are induced by the asexual
stages of the parasite that develop inside red blood cells (RBCs). Because splenic …

The spleen and sickle cell disease: the sick (led) spleen

V Brousse, P Buffet, D Rees - British journal of haematology, 2014 - Wiley Online Library
The spleen has a combined function of immune defence and quality control of senescent or
altered red cells. It is the first organ injured in sickle cell anaemia (SCA) with evidence of …

Sickle cell disease: Reducing the global disease burden

J Mburu, I Odame - International journal of laboratory …, 2019 - Wiley Online Library
Sickle cell disease has been largely an invisible global health issue, especially in regions of
high incidence mainly due to lack of awareness among both the local health policy makers …

Life-threatening infectious complications in sickle cell disease: a concise narrative review

D Ochocinski, M Dalal, LV Black, S Carr, J Lew… - Frontiers in …, 2020 - frontiersin.org
Sickle cell disease (SCD) results in chronic hemolytic anemia, recurrent vascular occlusion,
insidious vital organ deterioration, early mortality, and diminished quality of life. Life …

Crises in sickle cell disease

EM Novelli, MT Gladwin - Chest, 2016 - Elsevier
In spite of significant strides in the treatment of sickle cell disease (SCD), SCD crises are still
responsible for high morbidity and early mortality. While most patients initially seek care in …

[HTML][HTML] Burden of sickle cell trait and disease in the Uganda Sickle Surveillance Study (US3): a cross-sectional study

G Ndeezi, C Kiyaga, AG Hernandez… - The Lancet Global …, 2016 - thelancet.com
Background Sickle cell disease contributes substantially to mortality in children younger
than 5 years in sub-Saharan Africa. In Uganda, 20 000 babies per year are thought to be …