Serum alpha-hydroxybutyrate dehydrogenase levels in children with sickle cell disease.

Serum alpha-hydroxybutyrate dehydrogenase (alpha-HBDH) levels were determined in 28 children with homozygous sickle cell disease (SCD) during the steady state, crisis, and postcrisis periods. Serum alpha-HBDH levels were also studied in 20 patients with infection. In patients during the steady state, serum alpha-HBDH levels were significantly above control values. During the vaso-occlusive crisis there was a significant further increase in the alpha-HBDH levels. In patients with infection, however, there was no significant increase in serum alpha-HBDH levels. These findings clearly indicate that the level of serum alpha-HBDH in patients with SCD permits differentiation between vaso-occlusive crises, infection, and spurious symptoms.