Sickle-cell disease Wikipedia

Description: Sickle-cell disease is a general term for a group of genetic disorders caused by sickle hemoglobin (Hgb S or Hb S). In many forms of the disease, the red blood cells change shape, usually looking much like that of a banana, upon deoxygenation because of polymerization of the abnormal sickle hemoglobin. This process damages the red blood cell membrane, and can cause the cells to become stuck in blood vessels. This deprives the downstream tissues of oxygen and causes ischemia and infarction. The disease is chronic and lifelong. Individuals are most often well, but their lives are punctuated by periodic painful attacks. In addition to periodic pain, there may be damage of internal organs, such as stroke. Lifespan is often shortened with sufferers living to an average of 40 years. Sickle-cell disease occurs more commonly in people (or their descendants) from parts of the world, such as sub-Saharan Africa, where malaria is or was common. Sickle-cell disease can occur in any individual of any color or ethnicity, however. Contents 1 Types and terminology 2 Signs and symptoms 2.1 Vaso-occlusive crises 2.2 Other sickle-cell crises 2.3 Complications 3 Diagnosis 4 Pathophysiology 5 Genetics 5.1 Inheritance 5.1.1 Examples 6 Treatment 6.1 Febrile illness 6.2 Painful (vaso-occlusive) crises 6.3 Acute chest crises 6.4 Hydroxyurea 6.5 Future treatments 7 Situation of carriers 8 History 9 See also 10 Notes 11 References 12 External links