Hepatobiliary Complications

BackgroundBiliary tract abnormalities are common in people with SCD in general and in those with HbSS in particular. These abnormalities include cholelithiasis, acute cholecystitis, biliary…

Acute Anemia

Background Nearly all people with SCD have chronic anemia, but individual baseline hemoglobin values vary widely depending upon hemoglobin genotype (HbSS, HbSC, HbSf-thalassemia, HbSp0-thalassemia), current…

Splenic Sequestration

BackgroundSplenic sequestration is defined as sudden enlargement of the spleen and reduction in hemoglobin concentration by at least 2 g/dL below the baseline value. It…

Acute Chest Syndrome

Background ACS is one of the most common and serious acute complications ofSCD. It is the second most frequent reason for hospitalization in children and…

Acute Stroke

Background Stroke is one of the most common and devastating complications of SCD. In the absence of primary stroke prevention, approximately 10 percent of children…

Multisystem Organ Failure

Background Multisystem organ failure (MSOF) is a severe and life-threatening complication usually associated with a VOC and characterized by failure of the lungs, liver, and/or…

Acute Ocular Conditions

Background In persons with SCD, acute ocular complications may occur secondary to trauma, infection, vaso-occlusive episodes leading to occlusion of the eye vasculature, or progression…

Priapism

BackgroundPriapism is a sustained, unwanted painful erection lasting 4 or more hours. Stuttering priapism is the occurrence of multiple self-limited episodes of shorter duration (<4…

Vaso-Occlusive Crisis

A VOC is the hallmark acute complication for persons with SCD and manifests as acute severe pain. Although VOCs are typically associated with excruciating pain…

Fever

Background People with SCA have an increased risk of severe bacterial infection, resulting primarily from reduced or absent splenic function. By 2 or 3 months…