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The Role of the C1-Esterase Inhibitor in HAE
Daim Duab: The Role of the C1-Esterase Inhibitor in HAE

C1 esterase inhibitor (C1-INH) yog ib qho protein nyob hauv cov kua hauv koj cov ntshav. Nws tswj hwm cov protein hu ua C1, uas yog ib feem ntawm kev ua kom tiav.

Qhov kev sib txuam ua ke yog ib pab muaj ze li ntawm 60 cov protein nyob hauv ntshav ntshav lossis ua pa ntawm qee cov qe ntshav. Lub cev sib xyaw ua haujlwm nrog koj lub cev tiv thaiv kab mob los tiv thaiv lub cev los ntawm kev kis kab mob. Lawv kuj pab tshem cov cell tuag thiab cov khoom tawm txawv teb chaws. Nws muaj cuaj lub ntsiab protein ntxiv. Lawv tau sau npe C1 txog C9. Tsis tshua muaj txiaj ntsig, tib neeg tuaj yeem txais qee yam tsis txaus ntawm qee cov protein ntxiv. Cov neeg no ntxim rau ib co kab mob los yog autoimmune ntshawv siab.

Tsab ntawv xov xwm no tham txog kev ntsuas uas raug ntsuas los ntsuas ntsuas C1-INH hauv koj cov ntshav.

Xav tau cov ntshav ntxiv. Qhov no feem ntau tau ris ntshav hlab ntsha. Cov txheej txheem hu ua venipuncture.

Tsis muaj kev npaj tshwj xeeb yog xav tau.

Thaum cov koob tau tso rau hauv cov ntshav, qee cov neeg hnov ​​mob me ntsis. Lwm tus yuav hnov ​​tsuas yog tus pleev qhov muag lossis qhov quav. Tom qab ntawd, yuav muaj qee qhov throbbing.


Tej zaum koj yuav xav tau qhov kev ntsuas no yog tias koj muaj cov tsos mob ntawm tus mob caj dab lossis tau mob angioedema. Ob qho tib si ntawm kev sib deev mob los ntawm cov qib siab C1-INH.

Qhov ua tiav cov tseem ceeb kuj tseem ceeb rau kev kuaj kab mob autoimmune, xws li kab mob lupus erythematosus.

Cov kab ke tus nqi yuav txawv me ntsis ntawm ntau lub chaw soj nstuam. Koj tus neeg saib xyuas kev noj qab haus huv tseem yuav ntsuas cov qib kev ua haujlwm zoo ntawm koj C1 esterase inhibitor. Tham nrog koj tus kws kho mob txog cov ntsiab lus ntawm koj cov kev kuaj sim.

Qib C1-INH qis kawg tuaj yeem ua rau qee yam mob angioedema. Mob hlwb (Angioedema) ua rau lub ntsej muag o, mob caj pas thiab tus nplaig sai sai. Nws tseem yuav ua rau ua pa nyuaj. O kev mob nyob rau hauv txoj hnyuv thiab mob plab kuj tuaj yeem tshwm sim. Muaj ob hom kev tiv thaiv mob angioedema uas tshwm sim los ntawm theem qis ntawm C1-INH. Kab mob siab ntsig los yog mob rau cov menyuam thiab cov neeg hluas hnub nyoog qis dua 20 xyoo. Cov mob angioedema twb pom nyob rau cov neeg laus dua 40 xyoo. Tus neeg mob uas muaj tus mob angioedema feem ntau kuj yuav muaj lwm yam mob xws li mob qog nqaij hlav lossis mob kis mob hlwb.


Kev phom sij cuam tshuam nrog kev tso ntshav tawm ntawm lub cev me ntsis, tab sis yuav suav nrog:

  • Los ntshav ntau
  • Tsaus muag lossis hnov ​​nkaus lub teeb
  • Hematoma (ntshav ntau nyob hauv qab daim tawv nqaij)
  • Kev kis tus kab mob (muaj kev pheej hmoo me me txhua lub sijhawm ntawm daim tawv tawg)

C1 inhibiting qhov zoo tshaj yuav; C1-INH

  • Kuaj ntshav

Cicardi M, Aberer W, Banerji A, li al. Kev faib tawm, kev kuaj mob, thiab mus kom ze rau kev kho cov mob vwm: daim ntawv tshaj tawm los ntawm Hereditary Angioedema International Ua Haujlwm Pab Pawg. Kev ua xuaCov. Xyoo 2014; 69 (5): 602-616. PMID: 24673465 www.ncbi.nlm.nih.gov/pubmed/24673465.

Leslie TA, Greaves MW. Kab mob los yog kab mob rau hau. Hauv: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, eds. Kev Kho Mob Ntawm Cov tawv nqaij: Cov Tshuaj Txhuam Kho Kom ZooCov. Thib 5 ed. Philadelphia, PA: Elsevier; 2018: chap 101.

Zanichelli A, Azin GM, Wu MA, li al. Kev kuaj mob, chav kawm, thiab kev tswj hwm ntawm tus mob angioedema hauv cov neeg mob uas tau nrhiav C1-inhibitor tsis muaj peev xwm. J Kev Cuam Tshuam Clin Immunol PractCov. 2017; 5 (5): 1307-1313. PMID: 28284781 www.ncbi.nlm.nih.gov/pubmed/28284781.


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