?????????????????????? Thalassemia

1
?????????????????????????????????????????????????
???????????????????????????????????????

• ??? ???????? ???????
• ????????????? 7
• ?????????????? ???????????????

2
?????????????????????? Thalassemia

1. ??????????????????????????
2. ??????????????????????????????

3
?????????????????????????????

• Health education
• ????????????????????????????
• ???????????????????????
• ?? PND
• ?????????????????????????????????
  ??????????????????????

4
??????????????????????????????

• ??????????????????????????????????????????????????
  ???????????????
• ???????????????? MCH Board ??????????????????????
• ??????????????????????????????????????????????????
  ??????????????
• ?????????????????????????
• ?????????????????????????????????????
• ????????????????????????????????? OF, DCIP
• ????????????????????????????? ??.??. (??. 2544)
• ??????????????????????

5
?????????????????????????????????????
??????????????????????
???????????

• ???????????????????????????????????????? ???
  ???.
• ????????????????????

6
?????????????????????????????????????
??????????????????????
???.

• ????????????????????
• Screening test ???????? OF, DCIP
• ??????????? Screening test ve ????????????
• ????????????????????????????????????????
  ??.??????

7
?????????????????????????????????????
??????????????????????
??.??????

• ??????????????????????
• Screening test ???????????? ???.
• ???? confirm ???? Electrophoresis
  ???Microclolum
• ???????????????????? PCR ??? ??.??.
  (??????????? ? - thal trait ???????)
• ?? PND ??????? Amniocentesis
• ????????????????? ??.??. ????????????
  ?????????????? ????????????????? ????????
• Terminate of pregnancy ??????????????????

8
Clinical Guideline for Thalassemia Prevention and
Control
First ANC ???? OF, DCIP/ Hct, MCV
??????????????? ??????????????????

ve
-ve
-ve
????????????? ???? OF, DCIP/ Hct, MCV
ve
-ve
????????????? Hb typing
ve
-ve
Counseling ????????? (?-thal ??????? PCR ???
??.??.)
Refer
normal
?? PND
abnormal
ANC ???????
Therapeutic Abortion
9
????????????????????????????????? ??.??????
ANC
CBC
OF/ DCIP
Positive
Electro phoresis
WARD / OPD
CBC
HbA2,E
hemolysate
???.
?????? / ??.?????
CBC
?????????????
OF/ DCIP
Positive
CBC Hb, Hct, MCV, Rbc morphology
10
??????????????????????
?? Hct OF DCIP MCV Hb Typing
???? - ve - ve gt80 AA2, A2 lt 3.5
? - trait ve - ve lt80 AA2, A2 gt 3.5
E trait - ve ve lt80,gt80 AE, E 25 35
? - trait ve - ve lt80 AA2, A2 lt 3.5
EE ve ve lt80 ,gt80 A2E, E gt 80
HbH disease ve ve/- ve lt80 AA2H
? - thal ve ve lt80 A2F, A2FA
?/E - thal ve ve lt80 EF, EFA
?????????????????????? 1. gene ??????? PCR
??? AF ???? CVS ???? Cord blood
2. Hb Typing ???
cord blood
11
?????????????????????????

• ?1 trait ?1 trait Hb
  Barts hydrops fetalis
• - trait ? - trait
  ? - Thalassemia
• - trait HbE Trait ? -
  Thalassemia HbE

12
??????????????????????????????????????????????????
?????? ???????????????
?????????? (??) ????????????(???) ???????????(???) ????????? (???) ??????????????????(???) ???????????????? (???)
2536-2538 5254 1373 15 0 0
2539 2133 804 13 3 2
2540 1185 745 11 2 2
2541 1743 658 3 0 0
2542 1694 699 5 1 1
2543 1702 781 9 2 2
13
??????????????????????????????????????????????????
?????? ??????????????? ???????????????????????????
??????????????????? ??????? ?? 2544
?????????? (??) ????????????(???) ????????????(???) ??? (???) ??????????(???) ????????? (???) ??????????????????(???) ???????????????? (???)
2544 1684 350 (20.78) 5 190 (53.52) 10 1 1
2545 1473 308 (20.90) 7 176 (55.87) 15 2 2
2546 1305 283 (21.69) 5 164 (56.94) 12 0 0
2547 1474 272 (18.45) 5 170 (61.73) 9 0 0
2548 1471 312 (21.21) 4 185 (58.54) 13 3 3
2549 1552 370 (23.94) 5 206 (54.49) 22 5 4
2550 ??.-??. 1355 280 (20.66) 2 166 (58.86) 21 4 2
14
??????????????????????????????????????

• 1. ????????????????????????? ???. ????? ???.
  ??????
• 2. ??????????????????????
• 3. ???????????????????????????
• 4. ?????????? ?????????????????????????????????

15
??????????????? ????? ?????????????????????????

???????????????????????????????????????????????
??????? ??????????? ??????????????????? (???) ????????????????(??) ??????????????????????????????? (???) ??????????????? ????? (???) ?????????????????????? (???)
Hydrop fetalis 1,250 0 0 12,000 15,000,000
? - thal major 625 10 6,250 9,400 58,750,000
?/E - thal 3,250 30 97,500 4,950 48,262,000
HbH disease 7,000 60 420,000 500 21,000,000
??? 12?125 - 523,750 - 143,012,500
16
??????????????????????????????????????????????????
??????? ???????????????
????????(??) ???????? ???????? ?????????????????????? ????????????????? ????????????????? ????????? ????????????? ????????????? ??????????? (???) ?? PND????????????? ????????????????
????????(??) ??? GA GA lt 16 wk ?????????????????????? ??? GA GA lt 16 wk ????????? ??? GA GA lt 16 wk ??????????? (???) ?? PND????????????? ????????????????
2547 1474 1091 (74.01 ) 1474 (100 ) 272 (18.45 ) 214 (14.51 ) 5 (0.33 ) 201 (72.56 ) 168 (78.50 ) 9 0 0
2548 1471 1131 (78.88 ) 1471 (100 ) 312 (21.21 ) 209 (14.20 ) 4 (0.27 ) 199 (62.97 ) 167 (79.90 ) 13 3 3
2549 1552 1197 (77.12 ) 1552 (100 ) 370 (23.84 ) 220 (14.17 ) 5 (0.32 ) 237 (63.20 ) 181 (82.27 ) 22 5 4
2550 (?.?.-?.?.) 1355 1090 (80.44 ) 1355 (100 ) 280 (20.66 ) 197 (14.53 ) 2 (0.14 ) 215 (76.24 ) 165 (83.75 ) 21 4 2