Background Sickle cell disease (SCD) is commonly encountered in Africa and Middle Eastern countries. The causative mutation in the gene encoding the hemoglobin subunit 尾 (HBB) leads to various genotypic variants of the disease. This results in varied phenotypes, with a spectrum of complications, ...
Sickle cell syndromes, also collectively referred to as sickle cell disease (SCD), are generic terms for a group of chronic inherited disorders of hemoglobin structure in which the affected individual inherits two mutant globin genes (one from each parent), at least one of which is always the ...
Sickle cell disease (SCD), is an autosomal recessive hemoglobin disorder that results from the replacement of glutamate for valine at the sixth amino acid of the beta-globin chain. This replacement results in the formation of hemoglobin S (HbS) tetramers that accumulate during oxidative stress, t...
Sickle cell disease can be caused by multiple genetic variants. Most estimates of sickle cell disease burden account only for the homozygous HbSS variant, which excludes mortality and morbidity caused by the HbSC variant or thalassaemia. Inclusion of HbSC and thalassaemia would add a further 130 ...
In sickle cell anaemia, blood is also chronically low in oxygen. This lack of oxygen-rich blood can damage nerves and organs, including one’s kidneys, liver, and spleen and can be fatal. Kidney injury in sickle cell disease is referred to as sickle cell nephropathy. Sickle cell nephropathy...
Conversely, sickle cell disease occurs when an individual inherits 2 abnormal sickle genes (SS). There are different types of sickle cell disease, including hemoglobin SS (HbSS), hemoglobin SC (Hb SC), and hemoglobin S beta-thalassemia. These hemoglobin types cause a vaso-occlusive crisis; ...
P. (1983), Abnormalities of Serum Transcobalamins in Sickle Cell Disease (HbSS) in Black Africa. Scandinavian Journal of Haematology, 30: 135–140. doi: 10.1111/j.1600-0609.1983.tb01459.x Author Information 1 Laboratory of Medical Biochemistry, Faculty of Medicine, University of Nancy, 54500 ...
Malaria is fatal in homozygous sickle cell (HbSS) disease. In a cross‐sectional s... JR Aluoch - 《Tropical Medicine & International Health Tm & Ih》 被引量: 59发表: 2010年 Sickle-cell disease of Africans in Kenya. This investigation is an attempt to explain the anomaly that whereas ...
Universal newborn screening, in combination with early intervention for affected infants, has nearly eliminated early childhood mortality due to sickle cell disease (SCD; HbSS) in high-income developed countries1–3. In con- trast, a majority of children born with SCD in low-income developing ...
Life expectancy in HbSS from a multicenter study in the USA in 1994 was estimated at 42 for men and 48 for women,8 and 95% of children survive to adulthood.9 The effect of sickle cell disease on infection Impaired splenic function The spleen has a key role in the increased susceptibility...