Clinical Presentation and Diagnosis of SCD General

• Indentified by neonatal screening before 2 months of age Symptoms

• Painful vasoocclusive crises are the hallmark of SCD

• Dactylitis (hand-foot syndrome) before age 1 year

• May develop infarction of the spleen, liver, bone marrow, kidney, brain, and lungs

• Gallstones

• Priapism in males

• Slow healing lower extremity ulcers after trauma or infection

• Weakness, fatigue Signs

• Chronic hemolytic anemia is common

• Enlargement of spleen, and heart

• Scleral icterus Laboratory Tests

• Hgb 7 to 10 g/dL (70-100 g/L or 4.3-6.2 mmol/L)

• Low HgF and increased reticulocytes, platelets, and WBCs

• Presence of sickled cells on blood smear (see Fig. 68-2)

• Neonatal screening: hemoglobin electrophoresis, isoelectric focusing, or DNA analysis

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