??????? ????????? ??????? ???????? ???? ????? PFIC

Progressive Familial Intrahepatic Cholestasis (PFIC)-  ?? ??? ???? ????? ????? ????? ??? ??? ??????? ????? ?????? ??? ??? ??????? ?????? ???? ?? ???? ???????? ?????? ?????? ?????? ????? ???????? ???? ?? ???????. ??? ??? ?????? ????? ??? 3 ????? ????? ????? ?? ????? ?????? ???? ??????? ????? ?? ???????? ??????? ???????? ??????? ?????? ???????? ????? ?????? ??????? ??????????? ???????.

???? ???????? ?? ?????? ?????? ??? ????? PFIC ????? ?????? ?????? ????? ???? ??????? ?????? (??? ?? ??? ??????????? ?????? ???????)? ???? ?????? ?????? ??????? ????? ????? ?? ??????. ????? ??? ??????? ?????? ????? ?? ????? ??? ??? ?????? ????? ???? ?????? ??? ???? ????? ????? ???? ????? ?????? ?????.

??????? ????????? ??????? ???????? ???? ????? PFIC- ?? ????? ??? ????? ?????? ?? ?????????? ???????? ???????? ???? ????? ??? ????? ????????? ????? ?? ????? ?? ??????? ???????? ??? ????? ????? ?????? ???  (??????? - ???? ????? ???? ?? ???? ?????)

??? ??????? ???? PFIC ??? ????? ????????? ????? ????? ??? ??????? ????????? ???? ?????? ??? ???? ??? ???? ??????? ????????? ?? ????? ????? ??????? ??? ???? ????. ??????? ???? ???????? (???????????) ?????? ??????? ????? ?? ?????? ??? ????? ?? ???? ??????? ??? ??????? ??????? ????? ??????? ??? ???????????? A, D, E, k ?????? ????. 

???? ??????? ??? ??????? ?????????? ????? ?????? ?????? ?????? ???? ?? ???? ????? ????????? ?????????? ??? ??? ??????? ????? ????? ?????? ??????? ?????? ??? ?????.
??????? (?????? ????? ????? ???????) ????? ??? ????? ??????????? ?? ???? ????????.