Duchenne型PMDにおける血清α-Hydroxybutyrate Dehydrogenase(HBD)について

抄録

Measurement of serum a-hydroxybutyrate dehydrogenase (HBD) and lactate dehydrogenase (LD) activities was performed in 68 boys (age 7-19) hospitalized in Suzuka-Byoin National Sanatorium with Duchenne's type progressive muscular dystrophy.
Elevated serum HBD activities (>300u./ml) were found in 43 cases out of 68(63.2%) and the enzyme levels were of roughly inverse proportion to the stages of the diseases; the more chronic the disease, and the more advanced the loss of muscle mass, the lower the serum HBD activities. The mean HBD value of 33 cases belonging to stages V · VI was 206.9u./ml, which is a little lower than the upper normal limit of HBD.
The difference of the mean values between stage I · II, III · IV, and V · VI was significant but that between stage V · VI and VII · VIII was not, (P<0.005)
In this study1 there was neither increase in the HBD/LD ratio above the upper normal limit nor any significant difference between the mean values of each stage group of the disease. This is contrary to the previous findings of high ratio. (Elliott and Wilkinson) The difference may be derived from the different methods of mesurement but the fact there was no significant difference between the mean values of each stage group may suggest that no important meanings are given to the HBD/LD ratio in progressive muscular dystrophy.