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reference materials

Sickle Cell Anemia

key information

source: Orphanet

year: 2021

summary/abstract:

Epidemiology:
Sickle cell anemia (SCA) is the most common form of SCD. Worldwide, it is estimated that there are over 400,000 newborns with sickle cell anemia. The birth prevalence varies according to region, with prevalence greatest in regions affected by holoendemic malaria. In Europe, the pooled birth prevalence is 1/2,300, although this varies between countries.

Clinical Description:
The disease does not manifest during fetal life or up to the first three months of life due to the presence of high levels of fetal hemoglobin. Clinical manifestations evolve with age and are extremely variable between individuals and at different times. In addition to anemia and bacterial infections, VOAs cause hyperalgic focal ischemia (and sometimes infarction) when they occur in the abdomen, chest or skeleton. Over the course of time, VOAs may compromise the integrity of tissues or organs.

 

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