CCRN Exam Prep - Pediatric Critical Care

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Which of the following disorders is often the earliest clinical syndrome seen in children who have sickle cell anemia?
  1. Hand and foot disease
  2. Acute sickle dactylitis
  3. Rickets
  4. Paget’s bone disease
Explanation

Answer − B − The disorder often the earliest clinical syndrome seen in children with sickle cell anemia is acute sickle dactylitis. Also called hand-foot syndrome, it is often the earliest clinical syndrome seen in children with sickle cell anemia. There is painful, symmetrical swelling of the hands and feet.

Acute sickle dactylitis, or hand-foot syndrome, is a painful and sometimes debilitating complication of sickle cell disease. Sickle cell disease is an inherited blood disorder characterized by the presence of abnormal hemoglobin S (HbS) in red blood cells. This abnormal hemoglobin can cause the red blood cells to become rigid and crescent- or sickle-shaped, leading to reduced blood flow and oxygen delivery to tissues.

In acute sickle dactylitis, the small blood vessels in the hands and feet become blocked by sickled red blood cells, resulting in inflammation, pain, and swelling in the fingers, toes, or both. This condition is more common in children with sickle cell disease and typically occurs between the ages of 6 months and 5 years.

Symptoms of acute sickle dactylitis may include:

  • Swelling and redness in the hands, feet, fingers, or toes
  • Severe pain in the affected areas
  • Fever
  • Warmth over the inflamed area

 

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