Kev Cais Lub Qe Ntshav Bullosa

Kev mob ntsws o ua paug (eB) yog ib pab muaj mob ua kom tawv nqaij sawv hlwv tom qab raug mob me. Nws kis hauv tsev neeg.

Muaj plaub yam tseem ceeb ntawm EB. Lawv yog:

• Cov tshuaj tiv thaiv phaum mob (epidermolysis bullosa)
• Kev nthuav dav bullosa simplex
• Hemidesmosomal epidermolysis bullosa
• Hlaus epidermolysis bullosa

Lwm hom EB uas tsis tshua muaj neeg hu ua epidermolysis bullosa acquita. Daim ntawv no nthuav dav tom qab yug. Nws yog lub cev tsis muaj zog, txhais tau tias lub cev tawm tsam nws tus kheej.

EB tuaj yeem sib txawv los ntawm me me rau neeg tuag taus. Daim foos me ua rau o ntawm daim tawv nqaij. Daim ntawv tuag ua rau lwm cov kabmob. Feem ntau ntawm cov mob no pib thaum yug los lossis sai sai tom qab. Nws tuaj yeem nyuaj los txheeb xyuas qhov tseeb hom EB uas ib tus neeg muaj, txawm hais tias cov cim cim tshwj xeeb tam sim no muaj rau feem ntau.

Kev nrhiav tsev neeg keeb kwm yog qhov pheej hmoo. Txoj kev pheej hmoo loj dua yog tias niam txiv muaj tus mob no.

Ua raws li daim ntawv ntawm EB, cov tsos mob tuaj yeem suav nrog:

• Alopecia (plaub hau poob)
• Cov hlwv ncig lub qhov muag thiab taub ntswg
• Cov hlwv nyob rau hauv lossis ib ncig ntawm lub qhov ncauj thiab lub qa, ua rau muaj teeb meem noj lossis nqos nyuaj
• Cov hlwv ua pa rau ntawm daim tawv nqaij raws li kev raug mob me lossis kev hloov kub, tshwj xeeb tshaj yog ntawm taw
• Blistering uas yog nyob rau thaum yug
• Cov teeb meem xws li hniav lwj
• Quaj quaj, hnoos, lossis lwm yam teeb meem ua pa
• Cov pob me me dawb ntawm daim tawv nqaij raug mob yav tas los
• Ntsia tsis tau lossis rhuav tshem cov ntsia hlau

Koj tus neeg saib xyuas kev noj qab haus huv yuav saib koj daim tawv nqaij los kuaj mob EB.

Kev ntsuam xyuas uas siv los ua pov thawj rau kev kuaj mob muaj xws li:

• Kev tshuaj ntsuam caj ces
• Nqaij xyoob tawv nqaij
• Kev ntsuam xyuas tshwj xeeb ntawm cov tawv nqaij hauv qab lub tshuab tsom

Kev kuaj tawv nqaij yuav raug siv los txheeb xyuas daim foos EB.

Lwm yam kev xeem uas yuav muaj xws li:

• Kuaj ntshav rau anemia
• Kab lis kev cai los kuaj xyuas kev kis kab mob yog tias qhov txhab kho tsis zoo
• Mob sab hauv siab lossis sab sau GI yog tias muaj cov tsos mob suav nrog teeb meem nqos

Feem ntau ntawm cov kev loj hlob yuav raug kuaj xyuas feem ntau rau tus menyuam uas muaj EB.

Lub hom phiaj ntawm kev kho mob yog tiv thaiv cov blisters los ntawm kev tsim thiab zam ntawm cov teeb meem. Lwm txoj kev kho mob yuav yog nyob ntawm seb nws mob tsis zoo npaum li cas.

TSEV zov

Ua raws li cov lus qhia no hauv tsev:

• Saib xyuas koj daim tawv kom zoo kom tiv thaiv kev kis kab mob.
• Ua raws li koj tus kws kho mob cov lus qhia yog tias thaj chaw blistered dhau los ua cov nyom lossis nyoos. Koj yuav tau siv tshuaj tua kab mob tsis tu ncua thiab siv tshuaj pleev cov tshuaj pleev rau qhov chaw zoo. Koj tus kws kho mob yuav qhia rau koj paub yog tias koj xav tau cov ntaub qhwv los yog hnav khaub ncaws, thiab yog tias muaj, siv hom twg.
• Koj yuav tsum siv cov tshuaj steroid qhov ncauj rau lub sijhawm luv luv yog tias koj muaj teeb meem nqos. Koj yuav tsum tau noj tshuaj yog tias koj kis qhov kab mob candida (poov xab) hauv lub qhov ncauj lossis caj pas.
• Saib xyuas koj lub qhov ncauj kom zoo thiab mus soj ntsuam hniav tas li. Nws yog qhov zoo tshaj plaws mus ntsib kws kho kaus hniav uas tau ntsib kev kho cov neeg mob EB.
• Noj zaub mov zoo. Thaum koj muaj kev raug mob rau ntau cov tawv nqaij, koj yuav xav tau cov calories ntau thiab cov protein kom pab koj cov tawv nqaij zoo. Xaiv cov khoom noj muag muag thiab zam rau txiv ntoo, tej daim, thiab lwm yam zaub mov yog tias koj muaj qhov ncauj tawm hauv koj lub qhov ncauj. Tus neeg noj zaub mov zoo tuaj yeem pab koj noj zaub mov kom zoo.
• Ua kev tawm dag zog ib ce tawm dag zog lub cev qhia koj los pab ua kom koj cov pob qij txha thiab cov leeg ua tau txawb.

NTU

Kev phais mob los kho cov mob no suav nrog:

• Daim tawv nqaij pleev rau hauv qhov chaw uas cov kiav txhab tob
• Dilation (nthuav dav) ntawm txoj hlab pas yog tias muaj qhov nqaim nqaim
• Kho ntawm tes deformities
• Kev tshem tawm ntawm txhua tus kabmob muaj pob qog nqaij hlav (hom mob qog nqaij hlav) uas nthuav tawm

LWM YAM KEV KHO

Lwm yam kev kho mob rau tus mob no yuav suav nrog:

• Cov tshuaj noj uas tiv thaiv lub cev tsis muaj zog yuav raug siv rau daim ntawv autoimmune ntawm tus mob no.
• Yuav kawm txog Protein thiab gene thiab kev siv cov tshuaj interferon.

Qhov kev txiav txim siab nyob ntawm qhov mob hnyav heev.

Tus mob ntawm thaj chaw blistered muaj ntau.

Cov hom ntawv me ntawm EB txhim kho nrog lub hnub nyoog. Cov foos txaus ntshai heev ntawm EB muaj qhov kev phom sij heev.

Hauv cov ntawv hnyav, caws pliav tom qab hlwv daim ntawv tuaj yeem ua rau:

• Kev ua haujlwm tsis meej (piv txwv li, ntawm cov ntiv tes, lauj tshib, thiab hauv caug) thiab lwm yam kev txawv
• Nqos teeb meem yog tias lub qhov ncauj thiab txoj hlab pas cuam tshuam
• Fiv ntiv tes thiab ntiv taw
• Muaj tsawg kev mus ncig los ntawm qhov caws pliav

Cov teeb meem no tuaj yeem tshwm sim:

• Mob Kev Plab
• Txo lub neej ncua rau cov mob hnyav ntawm cov mob
• Txoj hlab pas nqaim
• Teeb meem qhov muag, suav nrog dig muag
• Kev kis mob, suav nrog sepsis (mob rau hauv cov ntshav lossis cov ntaub so ntswg)
• Poob kev ua haujlwm ntawm txhais tes thiab taw
• Nqaij ua tej leeg
• Tus kab mob pheej ncig
• Kev noj zaub mov tsis zoo los ntawm kev noj zaub mov nyuaj, ua rau tsis ua kom tiav
• Squamous cell mob cancer

Yog koj tus minyuam muaj hlwv hlwv sai li sai tau tom qab yug los, hu rau koj tus kws kho mob. Yog tias koj muaj tsev neeg muaj keeb kwm ntawm EB thiab npaj yuav muaj menyuam, koj yuav xav muaj kev pab tawm tswv yim txog caj ces.

Kev tawm tswv yim rau caj ces tau raug pom zoo rau cov niam txiv yav tom ntej uas muaj tsev neeg keeb kwm ntawm ib hom kab mob epidermolysis bullosa.

Thaum cev xeeb tub, ib qho kev sim hu ua chorionic villus sampling siv tau los ntsuas tus menyuam. Rau cov khub niam txiv muaj qhov pheej hmoo kis me nyuam nrog EB, kev kuaj mob tuaj yeem ua tiav thaum hnub pib 8 txog 10 ntawm cev xeeb tub. Tham nrog koj tus kws kho mob.

Txhawm rau tiv thaiv daim tawv nqaij ua paug thiab sawv hlwv, hnav ntaub qhwv ncig ntawm thaj chaw raug mob xws li lauj tshib, hauv caug, pob taws thiab pob tw. Zam kev sib tw kis las.

Yog tias koj muaj EB nrhiav thiab nyob ntawm cov tshuaj steroids ntev dua 1 lub hlis, koj yuav xav tau calcium thiab vitamin D tshuaj ntxiv. Cov tshuaj pab no yuav pab tiv thaiv kom tsis txhob txha (pob txha nyias).

EB; Kev Sib Tw Qeb Hauv Tshuaj; Kab mob Dystrophic epidermolysis bullosa; Hemidesmosomal epidermolysis bullosa; Mob Weber-Cockayne; Kev nthuav dav bullosa simplex

• Kev mob kis tus kabmob bullosa, hom dystrophic
• Kev tua kab mob phaus mob kabmob ntawm tes, dystrophic

Denyer J, Pillay E, Clapham J. Cov Txheej Txheem Zoo Tshaj Plaws rau daim tawv nqaij thiab mob nqaij zoo nyob rau hauv kev tua kab mob Bullosa: Kev Pom Zoo Thoob Ntiaj TebCov. London, UK: Thoob Ntiaj Teb Sib Txawv; Xyoo 2017.

Zoo nkauj, J-D, Mellerio JE. Hauv: Bolognia JL, Schaffer JV, Cerroni L, eds. Lub cev ntaj ntsugCov. 4 tug ed. Philadelphia, PA: Elsevier Saunders; 2018: chap 32.

Habif TP. Vesicular thiab bullous kab mob. Hauv: Habif TP, ed. Soj ntsuam kho cov tawv nqaijCov. 6 tus ed. Philadelphia, PA: Elsevier; 2016: chap 16.