. 12 QT: , , , . , - QT 25% , , .
12. - QT
QT | |||||
LQT-JLN1 | KCNQ1 | α- Kv7.1 | IKs | ||
LQT-JLN2 | KCNE1 | MinK β- Kv7.1 | IKs | ||
LQT1 | KCNQ1 | α- Kv7.1 | IKs | ||
LQT2 | KCNH2 | α- Kv11.1 | IKr | ||
LQT3 | SCN5A | α- Nav1.5 | INa | ||
LQT4 | ANK2 | B | INa,K INcx | ||
LQT5 | KCNE1 | MinK β- Kv7.1 | IKs | ||
LQT6 | KCNE2 | MIRP1 β- Kv7.1 | IKr | ||
LQT7 | KCNJ2 | α- Kir2.1 | IK1 | ||
LQT8 | CACNA1c | α- L- Cav1.2 | ICa,L | ||
: JLN , LQT, QT QT; -; - |
QT: (RomanoWard), (Jervell and Lange-Nielsen), (AndresenTawil) (Timothy).
- (RomanoWard), QT, , () , . 90% QT 1- (QT1), 2- (QT2) 3- (QT3) , (. 13, . 31).
13. QT.
QT1 | QT2 | QT3 | |
KCNQ1 | KCNH2 | SCN5A | |
IKs | IKr | INa | |
, | , | ST | |
, TdP | , | , (), | ( , ) |
QT | ( ) | ||
β- | ( 80%) | ( 50%) | ( ) |
QT I | |||
: QT QT; TdP Torsade de Pointes; |
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. 31. QT: () T QT1; () T- QT2; () T- , ST- QT3.
QT1 , KCNQ1, α- , IKs, . IKs QT . QT1 TdP (. 32) . QT1 (. . 31).
. 32. Torsade de Pointes ( ).
QT2 KCNH2, α- Kv11.1, IKr. QT2 TdP , . . QT2 , (. . 31).
QT3 , SCN5A, α- , , Na+ . TdP QT3 , . , , QT. ST (. . 31).
- ( ), , QT . KCNQ1 KCNE2, - Kv7.1, IKs.
, QT U, TdP, . 60% KCNJ2, α- Kir2.1, IK1, . 40% . , , (, , , , , ), - , . - , , , . 50% de novo
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QT, CACNA1c, α- CaV1.2. QT QTc ( 700 ), ( 2,5 ). 60% [ , , ] ( II 2:1). ( , ), , , ( , ), (). - , de novo.