000			01313cam a2200169 4500
001			NMDX7173
008			120401t2007 xxu||||| |||| 00| 0 eng d
100			_aRoberts-Harewood, M.
240			_aPractitioner
245			_aProphylaxis crucial in sickle cell disease
260			_c2007
500			_aNMUH Staff Publications
500			_a251
520			_a<span style="font-size: 10pt;">Sickle cell disease comprises a heterogeneous family of disorders characterised by the tendency of erythrocytes to 'sickle' when deoxygenated. It is an autosomal recessive condition that occurs when sickle haemoglobin (HbS), encoded by the gene Betas, combines with another abnormal haemoglobin gene. The most common form is homozygous sickle cell disease (SS), commonly known as sickle cell anaemia. The Betas gene can also interact with a thalassaemia genes and other structurally abnormal haemoglobins, such as HbC, found in parts of Ghana, and HbDPunjab, found in parts of India, to produce other forms of sickle cell disease - SC and SD respectively. The sickle gene is widely distributed throughout Central and Southern Africa, the Eastern Mediterranean and the Indian subcontinent.</span>
856			_uhttps://www.ncbi.nlm.nih.gov/pubmed/17784665
856			_uhttp://search.proquest.com/publication/40532?
999			_c76162 _d76162