???

????????????????????????1??????????????????????????????????????????????
?????????????????????????????????????????????????????3??????????????????????????????

??????????????????
B???????????
???
???

???????
??
??
???
?

??????????????????????

?????????????????????
??
?????,????????,(??)??,?????,???75%??????DIC???
????
?????
??.
?????????????

(?)? ???????
1.??? ???????????????????????????
2.???? ????(placental sulfatase deficiency)????Ryan 1980?????16-OH-DHEAS????????????
3.?M ?????k?????????????
4.? ???????????
()
?????????????????????????
???5????????????36????????????????????????42?????????
1.?? ???????????????????
2.?? ??????????????...

??2????????8????????????????????????????????????????
???
?????????????????????????????w????????????????????????????????????????????????????????????????
?
??
?????????????????56?56????????????????????????

,1.?,42??.??,????,????,????,???.??,??,???,???,??,??42???.
2.?,6~8??,???,??.4?,??.???????,??,??3...

1.?2.???????3.?????????4.
????????2000???5000
????????????????????