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

G Karayalcin, P Lanzkowsky, A B Kari

    The American Journal of Pediatric Hematology/Oncology
    |January 1, 1981
    PubMed

    Insights

    Serum alpha-hydroxybutyrate dehydrogenase (alpha-HBDH) levels help distinguish sickle cell disease (SCD) crises from infections. Elevated alpha-HBDH indicates a crisis, while normal levels suggest infection in SCD patients.

    Area of Science:

    • Biochemistry
    • Hematology
    • Pediatrics

    Background:

    • Sickle cell disease (SCD) is a genetic blood disorder with significant health complications.
    • Differentiating between vaso-occlusive crises and infections is crucial for effective SCD management.
    • Serum biomarkers can aid in diagnosing acute complications in SCD.

    Purpose of the Study:

    • To investigate serum alpha-hydroxybutyrate dehydrogenase (alpha-HBDH) levels in children with sickle cell disease (SCD).
    • To determine if alpha-HBDH levels can differentiate between steady state, vaso-occlusive crisis, and infection in SCD patients.

    Main Methods:

    • Serum alpha-HBDH levels were measured in 28 children with homozygous SCD during steady state, crisis, and postcrisis.
    • Alpha-HBDH levels were also assessed in 20 patients with infection.
    • Levels were compared to control values and between different clinical states.

    Main Results:

    • Children with SCD in the steady state showed significantly elevated alpha-HBDH levels compared to controls.
    • A further significant increase in alpha-HBDH levels was observed during vaso-occlusive crises.
    • Patients with infection did not exhibit a significant increase in serum alpha-HBDH levels.

    Conclusions:

    • Serum alpha-HBDH levels are significantly elevated in SCD patients during steady state and crisis.
    • Elevated alpha-HBDH levels can effectively differentiate vaso-occlusive crises from infections in SCD.
    • This biomarker aids in accurate diagnosis and management of SCD complications.

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