Kab mob hemorrhagic telangiectasia

Hereditary hemorrhagic telangiectasia (HHT) yog ib qho muaj keeb ntawm cov hlab ntsha tuaj yeem ua rau ntshav ntau dhau.

HHT tau dhau los ntawm cov tsev neeg nyob rau hauv tus qauv kev tshwm sim. Qhov no txhais tau tias qhov txawv txav yog xav tau los ntawm ib tug niam txiv thiaj li yuav tau tus kabmob.

Cov kws tshawb fawb tau txheeb pom plaub caj hlav neeg muaj feem hauv tus mob no. Txhua cov keeb no pom tias nws tseem ceeb rau cov hlab ntshav tau tsim kho kom raug. Kev hloov pauv hauv ib qho ntawm cov noob caj noob ces yog lub luag haujlwm rau HHT.

Cov neeg muaj HHT tuaj yeem txhim kho cov hlab ntsha tsis zoo hauv ntau thaj chaw ntawm lub cev. Cov hlab ntsha no hu ua arteriovenous malformations (AVMs).

Yog tias lawv nyob ntawm daim tawv nqaij, lawv raug hu ua telangiectasias. Cov chaw sib xws muaj xws li daim di ncauj, tus nplaig, pob ntseg, thiab ntiv tes. Cov ntshav ntsha mus tsis zoo kuj tuaj yeem txhim kho lub hlwb, lub ntsws, lub siab, lub plab hnyuv, lossis lwm thaj chaw.

Cov tsos mob ntawm tus mob no suav nrog:

• Nquag cov ntswg rau cov menyuam yaus
• Los ntshav hauv lub plab zom mov (GI), suav nrog cov ntshav hauv cov quav, lossis cov quav dub lossis dub.
• Qaug dab lossis tsis paub dab tsi, mob me me (los ntshav los rau hauv lub hlwb)
• Ua tsis taus pa
• Tsau daim siab
• Lub plawv tsis ua hauj lwm
• Kev mob ntshav los ntawm cov ntshav tsawg

Tus kws kho mob yuav kuaj nws lub cev thiab nug txog koj cov tsos mob. Tus kws kho mob uas paub zoo muaj peev xwm kuaj pom telangiectases thaum kuaj lub cev. Muaj ntau tsev neeg keeb kwm ntawm tus mob no.

Kev Xeem muaj xws li:

• Tso ntshav kuaj ntshav
• Kuaj ntshav
• Kev ntsuam xyuas lub plawv hu ua echocardiogram
• Endoscopy, uas siv lub koob yees duab me me txuas rau ntawm txoj hlab nyias kom ntsia hauv koj lub cev
• MRI los kuaj txog AVMs hauv lub hlwb
• CT los yog ultrasound scans txhawm rau kuaj AVMs hauv lub siab

Kev kuaj txog keeb kwm muaj los mus saib rau hloov pauv hauv cov genes cuam tshuam nrog tus mob no.

Cov kev kho mob yuav suav nrog:

• Kev phais mob txhawm rau kho ntshav los ntawm qee qhov chaw
• Kev Siv Hluav Taws Xob (cov ntaub so ntswg hluav taws xob nrog hluav taws xob) lossis laser phais kom kho tau cov ntswg los yog hnyav
• Endovascular embolization (txhaj tshuaj los ntawm txoj hlab me me) los kho cov ntshav tsis zoo hauv lub hlwb thiab lwm qhov ntawm lub cev

Qee tus neeg siv tshuaj kho estrogen, tuaj yeem txo cov ntshav tawm ntu. Hlau kuj tseem tuaj yeem muab tau yog tias muaj ntshav ntau ntau, ua rau muaj ntshav khiav tawm. Zam txhob siv cov tshuaj ntshav-ua ntshav. Qee cov tshuaj uas cuam tshuam rau kev tsim kho cov hlab ntshav tau kawm txog kev kho mob yav tom ntej.

Qee tus neeg yuav tsum siv tshuaj tua kab mob ua ntej ua haujlwm los yog hniav phais. Cov tib neeg mob ntsws AVM yuav tsum zam kev sib tw (duav) txhawm rau txhawm rau tiv thaiv kev mob decompression (khoov duav). Nug koj tus kws kho mob seb koj yuav tsum tau ceev faj dab tsi ntxiv.

Cov peev txheej no tuaj yeem muab cov ntaub ntawv qhia ntxiv ntawm HHT:

• Cov Chaw Tiv Thaiv thiab Tiv Thaiv Kab Mob - www.cdc.gov/ncbddd/hht
• Kho HHT - curehht.org
• Koom haum Lub Koom Haum rau Cov Tsis Txaus Ntseeg - rarediseases.org/rare-diseases/hereditary-hemorrhagic-telangiectasia

Cov neeg muaj tus mob no tuaj yeem nyob lub neej tag nrho, nyob ntawm seb qhov twg hauv lub cev AVMs nyob.

Cov teeb meem no tuaj yeem tshwm sim:

• Lub plawv tsis ua hauj lwm
• Ntshav siab hauv lub ntsws (mob ntsws)
• Muaj ntshav sab hauv
• Ua tsis taus pa
• Mob stroke

Hu rau koj tus kws kho mob yog tias koj lossis koj tus menyuam muaj ntswg los ntshav lossis lwm yam tsos mob ntawm tus mob no.

Kev tawm tswv yim rau caj ces yog pom zoo rau cov khub niam txiv uas xav muaj menyuam thiab cov uas muaj keeb kwm tsev neeg ntawm HHT. Yog tias koj muaj tus mob no, kev kho mob tuaj yeem tiv thaiv qee yam mob stroke thiab lub plawv tsis ua haujlwm.

HHT; Osler-Weber-Rendu syndrome; Osler-Weber-Rendu kab mob; Rendu-Osler-Weber syndrome

• Kev kaw lus hauv lub cev
• Arteries ntawm lub paj hlwb

Brandt LJ, Aroniadis OC. Vascular cuam tshuam ntawm txoj hnyuv plab hnyuv. Hauv: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger thiab Fordtran's Gastrointestinal thiab Kab Mob SiabCov. 10 tus ed. Philadelphia, PA: Elsevier Saunders; 2016: chap 37.

Cappell MS, Lebwohl O. Kab Mob Hemorrhagic telangiectasia Hauv: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, eds. Kev Kho Mob Ntawm Cov tawv nqaij: Cov Tshuaj Txhuam Kho Kom ZooCov. 4 tug ed. Philadelphia, PA: Elsevier Saunders; Xyoo 2014: chap 98.

McDonald J, Pyeritz RE. Kab mob hemorrhagic telangiectasia. Hauv: Adam MP, Ardinger HH, Pagon RA, li al, eds. GeneReviews [Is Taws Nem]Cov. Seattle, WA: University of Washington, Seattle; Xyoo 1993-2019. Hloov kho dua Lub Ob Hlis 2, 2017. Nkag Mus Siv Lub Tsib Hlis 6, 2019.