title: Care of patients with haemoglobin abnormalities: history and biology.
abstract: Haemoglobinopathies refer to a range of genetically inherited disorders of red blood cell haemoglobin and include sickle cell disorders and thalassaemias. They occur most commonly in populations whose ancestors come from Africa, Asia, Mediterranean Islands, and the Middle and Far East. Haemoglobin (Hb) abnormalities (or haemoglobinopathies) are caused by (i) abnormalities of the protein structure; (ii) imbalanced globin chain production owing to reduced rate of synthesis of normal a or b globin chains; or (iii) a combination of the two. This article will focus on the biological basis of sickle cell disorders and will discuss the history and pathology of the conditions.
authors.affiliation: Department of Nursing, Institute of Health and Community Studies, Bournemouth University.
authors.forename: Ahmed D
authors.initials: AD
authors.lastname: Khattab
authors.forename: Barry
authors.initials: B
authors.lastname: Rawlings
authors.forename: Ibtisam S
authors.initials: IS
authors.lastname: Ali
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications / Q000453:epidemiology / Q000235:genetics* / Q000628:therapy*; D002423:Cause of Death; D002648:Child; D005260:Female; D005838:Genotype; D006454:Hemoglobins / Q000737:chemistry; D006801:Humans; D007238:Infarction / Q000209:etiology; D007674:Kidney Diseases / Q000209:etiology; D008017:Life Expectancy; D008297:Male; D024802:Nurse's Role; D010375:Pedigree; D011317:Priapism / Q000209:etiology; D013154:Spleen / Q000098:blood supply; D020521:Stroke / Q000209:etiology; D013789:Thalassemia / Q000150:complications / Q000453:epidemiology / Q000235:genetics* / Q000628:therapy*; D006113:United Kingdom / Q000453:epidemiology; D014918:Wills
chemical_list: D006454:Hemoglobins