Monday, January 24, 2011

Short History of Sickle Cell Anemia

Sickle cell anemia is a blood disease. The genetic basis of sickle cell anemia is central to the history of medical genetics.

No one knows exactly when sickle cell anemia was first reported. Ancient people did not now the cause of sickle cell anemia. However, they saw children suffering from painful disease.

Sickle cell anemia is more common in Africa than on any other continent.

The existence of sickle anemia has long been suspected in ancient Egypt among mummies with severe anemia. DNA was extracted form dental samples (of Egyptian Predynastic mummies 3200 BC), indicating the presence of sickle cell anemia.

The first account of sickle cell disease in Western medical literature is attributed to James Herrick, a prominent American cardiologist from Chicago.

In 1910 he observed sickle shape red cells in the blood of a dental student, Clement Noel a 20 years old from Grenada who suffered from chronic hemolytic anemia.

The student complaint was complex: shortness of breath, dizziness, headaches, leg ulcers, severe stomach pain and anemia. Dr. Herrick had never seen a case like it before.

The red blood cells in the blood sample were obviously abnormal. Instead of their ordinary disk-like shape, they were distorted into long, sickle shaped structure.

In 1940s. scientists labeled sickle cell anemia as the first ‘molecular disease’.

The researchers learned more details about sickle cell anemia and how to it distorted red blood cell but cure failed to appear. By early 1970s, sickle cell anemia was called “the neglected diseases.”

In 1947 James V. Neel, scientist from University of Rochester, concluded that with respect to the mode of inheritance sickle cell anemia was a homozygous state and sickle cell trait the heterozygous state for a genetic character that had yet to be define.

Linus Pauling proposed that sickling reflected an abnormality of the hemoglobin molecule, based on the observation of Irving Sherman, then a medical student, that under the polarizing microscope sickle cells, induced by deoxygenation, exhibited birefringence.
Short History of Sickle Cell Anemia

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