Tus Sau: Clyde Lopez
Hnub Kev Tsim: 26 Lub Xya Hli Ntuj 2021
Hloov Hnub: 1 Lub Kaum Ib Hli Ntuj 2024
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Congenital fibrinogen tsis txaus - Tshuaj Kho Mob
Congenital fibrinogen tsis txaus - Tshuaj Kho Mob

Congenital fibrinogen deficiency yog ib qho tsis tshua muaj tshwm sim, muaj pub los ntawm cov ntshav tsis txhaws feem ntau. Nws cuam tshuam cov protein hu ua fibrinogen. Qhov protein no xav tau rau cov ntshav txhaws.

Tus kab mob no yog vim muaj qhov txawv txav. Fibrinogen cuam tshuam nyob ntawm seb cov noob caj noob ces:

  • Thaum cov noob qes uas dhau los ntawm ob leeg niam txiv, ib tus neeg yuav muaj tag nrho cov tsis muaj fibrinogen (afibrinogenemia).
  • Thaum cov noob qes tsis dhau los ntawm ib tug niam txiv, ib tus neeg yuav muaj ib qib fibrinogen tsawg dua (hypofibrinogenemia) los yog muaj teeb meem nrog kev ua haujlwm ntawm fibrinogen (dysfibrinogenemia). Qee zaum, ob qho teeb meem fibrinogen no tuaj yeem tshwm sim hauv tib tus neeg.

Cov neeg uas tsis muaj fibrinogen tag nrho yuav muaj cov tsos mob nram qab no:

  • Bruising tau yooj yim
  • Los ntshav los ntawm txoj hlab ntaws menyuam tom qab yug los
  • Los ntshav hauv cov nqaij mos
  • Los ntshav hauv hlwb (tsawg tsawg)
  • Los ntshav hauv cov pob qij txha
  • Los ntshav ntau tom qab raug mob lossis raug phais
  • Looj taub tsis nres yooj yim

Cov neeg muaj ntshav tsawg ntawm cov fibrinogen los ntshav tsawg zuj zus thiab los ntshav tsis ntau heev. Cov uas muaj teeb meem nrog kev ua haujlwm ntawm fibrinogen feem ntau tsis muaj tsos mob.


Yog tias koj tus kws kho mob xav tias muaj qhov teeb meem no, koj yuav tsum kuaj sim kom paub meej tias hom mob no puas loj npaum li cas.

Kev Xeem muaj xws li:

  • Los ntshav sijhawm
  • Fibrinogen kuaj thiab reptilase sij hawm los xyuas cov fibrin thiab qib zoo
  • Qee lub sij hawm thromboplastin lub sij hawm (PTT)
  • Lub sijhawm Prothrombin (PT)
  • Thrombin lub sijhawm

Cov kev kho mob hauv qab no tuaj yeem siv los rau lub sijhawm los ntshav lossis npaj rau kev phais:

  • Cryoprecipitate (cov khoom ntshav uas muaj cov tshuaj fibrinogen thiab lwm cov ntshav txha)
  • Fibrinogen (RiaSTAP)
  • Ntshav (feem ua kua ntawm cov ntshav uas txhaws tau ntshav)

Cov neeg muaj tus mob no yuav tsum txhaj tshuaj tiv thaiv kab mob siab B. Muaj ntau lub txhab ntshav yuav ua rau koj muaj feem kis mob siab.

Kev los ntshav ntau yog hom mob no. Lub sijhawm uas mob no yuav hnyav, lossis ua rau neeg tuag taus. Kev los ntshav hauv lub hlwb yog qhov ua kom tuag taus rau cov neeg uas muaj tus kabmob no.

Teeb meem yuav suav nrog:


  • Cov ntshav txhaws nrog kev kho mob
  • Kev txhim kho ntawm cov tshuaj tiv thaiv (inhibitors) rau fibrinogen nrog kev kho mob
  • Lub plab zom mov txhaws ntshav
  • Kev Txij Nkawm
  • Kev tawg ntawm spleen
  • Kho kho tej qhov txhab zoo

Hu rau koj tus kws khomob lossis nrhiav kev khomob kub ntxhov yog tias koj los ntshav ntau dhau.

Qhia koj tus kws phais mob ua ntej koj yuav phais yog tias koj paub lossis xav tias koj muaj teeb meem ntshav tsis zoo.

Qhov no yog ib qho muaj mob. Tsis muaj kev paub txog kev tiv thaiv.

Afibrinogenemia; Cov Hypofibrinogenemia; Dysfibrinogenemia; Qhov tsis muaj kuv tsis muaj peev xwm

Gailani D, Wheeler AP, Neff AT. Tsis tshua muaj qhov coagulation qhov tsis txaus. Hauv: Hoffman R, Benz EJ, Silberstein LE, li al, eds. Hematology: Qhov Ntsiab Cai thiab Kev Coj UaCov. 7 tus ed. Philadelphia, PA: Elsevier; 2018: chap 137.

Ragni MV. Hemorrhagic mob: coagulation yam tsis. Hauv: Goldman L, Schafer AI, eds. Goldman-Cecil TshuajCov. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016: chap 174.

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