Akenzua G I, Ihongbe J C, Asemota H N
Institute of Child Health College of Medical Sciences, University of Benin.
Afr J Med Med Sci. 1992 Dec;21(2):13-7.
To assess the value of alpha-hydroxybutyrate dehydrogenase (alpha-HBDH) in the diagnosis of painful crisis (PC) of sickle cell anaemia (SCA), we studied plasma enzyme levels in 55 children with HbSS and 21 control subjects with haemoglobin genotype AA. In 21 children with SCA, mean plasma alpha-HBDH was 373.8 +/- 113.5 micrograms/l during PC and during steady state in 34 children, it was 341.2 +/- 103.4 micrograms/l. These values were significantly higher than that of 128 +/- 19.5 micrograms/l obtained in control subjects. However, the difference between mean plasma alpha-HBDH levels in SCA children in PC and in steady state was 32.6 micrograms/l, t = 1.095; P < 0.2. There was no correlation between alpha-HBDH levels and reticulocyte counts (r = 0.0856; t = 0.4565; 0.7 < P < 0.6). The high levels of alpha-HBDH in patients with SCA is probably due to chronic haemolysis and not marrow infarction. Therefore, alpha-HBDH is of doubtful value in the diagnosis of painful crisis.
为评估α-羟丁酸脱氢酶（α-HBDH）在镰状细胞贫血（SCA）疼痛性危象（PC）诊断中的价值，我们研究了55例HbSS患儿和21例血红蛋白基因型为AA的对照受试者的血浆酶水平。21例SCA患儿在PC期间血浆α-HBDH平均水平为373.8±113.5微克/升，34例处于稳定状态的患儿血浆α-HBDH平均水平为341.2±103.4微克/升。这些值显著高于对照受试者的128±19.5微克/升。然而，SCA患儿在PC期和稳定期的血浆α-HBDH平均水平差异为32.6微克/升，t = 1.095；P < 0.2。α-HBDH水平与网织红细胞计数之间无相关性（r = 0.0856；t = 0.4565；0.7 < P < 0.6）。SCA患者α-HBDH水平升高可能是由于慢性溶血而非骨髓梗死。因此，α-HBDH在疼痛性危象的诊断中价值存疑。
