Acute chest syndrome - sickle cell disease

Case contributed by Dr Miriam Leiderer

Presentation

Patient of sub-saharan ethnicity presenting with acute onset chest, back and leg pain and shortness of breath

Patient Data

Age: 13 years
Gender: Male

On admission

X-ray

Chest x-ray on admission shows slightly increased density in the left lower zone.

Bilateral opacities and left lower lobe atelectasis with silhouetting of the left diaphragm.

Day 3, under therapy

X-ray

Markedly improved ventilation bilaterally.

Day 10, follow-up

X-ray

Opacities and left lower lobe atelectasis have resolved completely.

Case Discussion

This case shows acute chest syndrome in a child with sickle cell disease. The patient presented with a pain crisis and increasing respiratory symptoms. On day two, an exchange transfusion was initiated, after which the patient recovered.

Other than blood transfusions, treatment of acute chest syndrome includes broad-spectrum antibiotics, bronchodilators, and supportive care. 

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