Sickle cell trait

There are an estimated 100 million individuals with hemoglobin S worldwide.

Defined as the inheritance of the single copy of the sickle mutation that results from a single base pair substitution in the gene encoding the beta-globulin chain of hemoglobin.

In the United States there are more than 3 million carriers of hemoglobin S.

In the US approximately 8% of blacks and 0.5% of Hispanics and 0.2% of Caucasians carry hemoglobin S.

Carriers of hemoglobin S are generally asymptomatic and have a normal life span.

Renal manifestations of the most reported complications and include impaired urinary concentration, asymptomatic hematuria, and papillary necrosis.

Carriers of hemoglobin S are at increased risk for splenic infarction, exertional rhabdomyoslysis and renal medullary cancers.

The risk of exercise-related sudden death is 10-30 times higher among hemoglobin S carriers then among non-carriers.

The risk of exercise related sudden death in sickle trait carriers is increased by heat exposure, dehydration and intense exercise.

Patients have more than 50% normal hemoglobin and they are essentially asymptomatic, except under unusual circumstance.

Among African Americans associated with increased chronic kidney disease, decline in eGFR, and albuminuria (Naik RP et al).

Compared with noncarriers of the sickle cell trait, they have a 1.8 odds for  the incident chronic kidney disease, a1..3 fold odds of GFR decline greater than 3 mL per minute per 1.73 m² and a 1.9 fold odds of albuminuria.


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