Dab tsi yog mob epidermolysis bullosa, tshwm sim thiab kho mob

Zoo Siab

• Cov tsos mob tseem ceeb
• Ua rau muaj mob rau cov ntawv qib siab
• Dab tsi yog cov hom
• Yuav kho tus mob li cas
• Thaum xav tau kev phais mob
• Dab tsi los ua kom tsis txhob muaj npuas
• Yuav ua li cas thiaj hnav khaub ncaws
• Dab tsi yog cov nyom

Daim tawv nqaij mob ua paug yog cov caj ces ntawm cov tawv nqaij uas ua rau muaj cov hlwv ntawm daim tawv nqaij thiab cov qog ua kua, tom qab ib qho kev sib txhuam los yog qhov mob me uas tuaj yeem tshwm sim los ntawm kev voos daim khaub ncaws ntawm daim tawv nqaij lossis, yooj yim, los ntawm kev tshem tawm pab band, piv txwv li. Qhov xwm txheej no tshwm sim vim kev hloov caj ces kis tau los ntawm niam txiv mus rau lawv cov menyuam, uas ua rau muaj kev hloov pauv ntawm cov khaubncaws sab nraud povtseg thiab cov khoom tshwm sim hauv daim tawv nqaij, xws li keratin.

Cov tsos mob thiab cov tsos mob ntawm tus kab mob no tau txuas nrog cov tsos mob ntawm lub ntsej muag mob ntawm daim tawv nqaij thiab ntawm qee qhov ntawm lub cev, thiab tej zaum txawm tshwm ntawm lub qhov ncauj, xib teg thiab lub xib taws ntawm taw. Cov tsos mob no sib txawv raws hom thiab qhov mob ntawm cov kabmob epidermolysis bullosa, tab sis feem ntau lawv mob dhau sijhawm.

Kev kho mob rau kev mob khaub thuas feem ntau yog txhawb kev saib xyuas, xws li tswj kom muaj zaub mov txaus thiab hnav khaub ncaws tawv nqaij. Ib qho ntxiv, tseem tab tom tshawb fawb kom ua cov hloov pob txha caj qaum rau cov neeg uas muaj tus mob no.

Cov tsos mob tseem ceeb

Cov tsos mob tseem ceeb ntawm kev muaj mob epidermolysis yog:

• Blistering ntawm daim tawv nqaij ntawm qhov tsawg txhuam;
• Cov hlwv pom tshwm hauv sab hauv thiab qhov muag txawm tias;
• Kho cov tawv nqaij nrog qhov zoo nkauj thiab dawb me ntsis;
• Ntsia cov nyom;
• Cov plaub hau thinning;
• Kev txo tawm hws lossis ua hws ntau dhau.

Ua raws li kev mob hnyav ntawm cov tawv nqaij mob loj heev, kev txom nyem ntawm cov ntiv tes thiab ntiv taw kuj tseem yuav tshwm sim, ua rau deformities. Txawm hais tias yog tus yam ntxwv ntawm tus mob epidermolysis, lwm yam kab mob tuaj yeem ua rau pom cov hlwv ntawm daim tawv nqaij, xws li mob herpes simplex, epidermolytic ichthyosis, qog ua rau mob qog ntshav thiab ua qog ua paug. Paub tias dab tsi yog thaus thab plaub thiab yog dab tsi kho.

Ua rau muaj mob rau cov ntawv qib siab

Kev kis mob rau hauv lub cev yog tshwm sim los ntawm kev hloov caj ces ntawm niam txiv mus rau menyuam, thiab tej zaum yuav muaj kev ntseeg ntau, uas leej niam leej txiv muaj tus kabmob kis, lossis kev tsis ua haujlwm, uas leej niam thiab leej niam muaj tus kabmob kis tab sis tsis muaj tus cim ntawm cov cim lossis tsos mob ntawm tus kab mob.

Cov menyuam yaus uas muaj cov txheeb ze nyob ze nrog tus kabmob lossis nrog tus kabmob muaj kabmob sib kis tau zoo yog yug los ntawm hom mob no, yog li yog tias cov niam txiv paub tias lawv muaj tus kab mob no los ntawm kev kuaj keeb kwm, kev qhuab qhia caj ces yog qhia. Saib dab tsi yog kev sab laj txog mob caj ces thiab yuav ua li cas.

Dab tsi yog cov hom

Daim tawv nqaij mob tob tob tuaj yeem faib ua peb hom raws li txheej txheej ntawm daim tawv nqaij uas ua rau cov hlwv, xws li:

• Daim tawv nqaij tsis yooj yim: blistering tshwm sim nyob rau txheej sab saud ntawm daim tawv nqaij, hu ua epidermis, thiab nws yog qhov tshwm sim rau lawv tshwm sim ntawm tes thiab taw. Hauv hom no nws yog qhov pom pom cov ntsia hlau nchav thiab tuab thiab cov hlwv tsis kho sai;

• Cov tshuaj tiv thaiv phaum mob (epidermolysis bullosa): blisters nyob rau hauv hom no tshwm sim vim qhov tsis xws luag tawm ntawm hom V | Kuv collagen thiab tshwm sim nyob rau hauv txheej sab saum toj kawg nkaus ntawm daim tawv nqaij, hu ua dermis;
• Hlaus epidermolysis bullosa: tus cwj pwm los ntawm kev tsim cov hlwv vim muaj kev sib cais ntawm thaj av nruab nrab ntawm txheej txheem sab saum toj thiab nruab nrab ntawm daim tawv nqaij thiab qhov no, tus kab mob tshwm sim los ntawm kev hloov hauv cov noob txuas nrog rau cov kab mob dermis thiab cov kabmob av, xws li Laminin 332.

Kinder lub syndrome kuj yog ib hom kev mob sib kis ntawm cov plaub hau, tab sis nws yog qhov tsis tshua muaj thiab koom nrog txhua txheej ntawm daim tawv nqaij, ua rau muaj qhov tsis zoo ntawm lub cev. Tsis hais hom mob twg los xij, nws tseem ceeb heev uas yuav tsum nco ntsoov tias kev kis mob rau cov nqaij mos tsis kis, yog li, nws tsis kis ntawm ib tus neeg mus rau lwm tus los ntawm kev sib chwv nrog rau daim tawv nqaij mob.

Yuav kho tus mob li cas

Tsis muaj kev kho tshwj xeeb rau tus mob epidermolysis bullosa, thiab nws yog ib qho tseem ceeb heev kom muaj kev sab laj nrog kws kho mob dermatologist kom txheeb xyuas qhov xwm txheej ntawm daim tawv nqaij thiab zam kev cuam tshuam, xws li kev kis mob, piv txwv.

Cov kev kho mob rau tus kab mob no muaj cov kev txhawb nqa, xws li hnav khaub ncaws hnav thiab tiv thaiv qhov mob thiab, ntawm qee kis, tsev kho mob yuav tsum ua rau hnav khaub ncaws tsis huv, tsis muaj kab mob me, kom cov tshuaj muaj cai ncaj qha mus rau txoj hlab ntshav, li tshuaj tua kab mob hauv rooj plaub ntawm kev kis, thiab kom tso cov hlwv ntawm daim tawv nqaij. Txawm li cas los xij, qee qhov kev tshawb fawb tau raug tsim los ua cov kev hloov pauv ntawm qia rau hauv kev kho tus mob dystrophic bullous epidermolysis.

Tsis zoo li cov hlwv uas ua los ntawm kub nyhiab, hlwv tshwm sim los ntawm epidermolysis bullosa yuav tsum tau xuas nrig nrog ib rab koob tshwj xeeb, siv cov ntaub ua kom tsis muaj zog, tiv thaiv nws los ntawm kev sib kis thiab ua rau daim tawv nqaij txuas ntxiv. Tom qab tso dej tawm, nws yog ib qho tseem ceeb kom thov siv khoom, xws li tshuaj tsuag antibacterial, los tiv thaiv mob sib kis.

Thaum xav tau kev phais mob

Kev phais mob ntxiv yog feem ntau qhia rau ntawm rooj plaub uas nti nti los ntawm npuas ua rau lub cev txav lossis ua rau lub cev puas tsuaj uas ua rau lub neej tsis zoo. Muaj qee kis, kev phais mob kuj tseem siv tau los ua kom tawv nqaij, tshwj xeeb tshaj yog ntawm cov qhov txhab uas tau siv sijhawm ntev los kho.

Dab tsi los ua kom tsis txhob muaj npuas

Txij li thaum tsis muaj txoj kev kho tau, kev kho tsuas yog ua los pab txo cov tsos mob thiab txo qhov muaj mob tawm tshiab. Thawj kauj ruam yog kom muaj qee qhov kev saib xyuas hauv tsev, xws li:

• Hnav cov khaub ncaws paj rwb, zam kev ntawm cov xov hluavtaws;
• Tshem cov cim npe ntawm txhua cov khaub ncaws;
• Cov ris tsho hauv qab tig kom sab kom tsis txhob muaj kev sib txuam ntawm daim tawv nqaij;
• Coj cov khau uas lub teeb thiab dav dav kom yooj yim hnav yooj yim khi nkawm thom khwm;
• Ua tib zoo saib thaum siv phuam tom qab da dej, maj mam nias ntawm daim tawv nqaij nrog phuam mos;
• Thov Vaseline hauv kev nplua nuj ua ntej tshem tawm kev hnav ris tsho thiab tsis txhob yuam nws txoj haujlwm tshem tawm;
• Yog tias cov khaub ncaws nws kawg lo rau ntawm daim tawv nqaij, tawm ntawm thaj chaw ntub dej kom txog thaum cov khaub ncaws xoob tawm ntawm daim tawv nqaij ib leeg;
• Npog cov qhov txhab nrog ib tus hnav khaub ncaws tsis nplaum thiab nrog cov ntaub nyias nyias xoob;
• Pw nrog thom khwm thiab hnab looj tes kom tsis txhob raug mob uas tuaj yeem tshwm sim thaum pw tsaug zog.

Ntxiv rau, yog tias muaj tawv nqaij khaus, tus kws kho mob yuav sau ntawv siv corticosteroids, xws li prednisone lossis hydrocortisone, ua rau mob tawv nqaij thiab txo cov tsos mob, tsis txhob khawb qhov tawv nqaij, ua kom muaj qhov txhab tshiab. Nws kuj yog qhov yuav tsum tau ceev faj thaum da dej, zam qhov ua kom dej kub dhau.

Daim ntawv thov ntawm botox ntawm txhais ko taw zoo li yuav ua tau rau kev tiv thaiv cov hlwv hauv thaj av no, thiab gastrostomy tau qhia thaum tsis tuaj yeem noj zaub mov kom zoo yog tias tsis muaj cov hlwv hauv lub qhov ncauj lossis txoj hlab pas.

Yuav ua li cas thiaj hnav khaub ncaws

Kev hnav khaub ncaws yog ib feem ntawm cov kev coj ua niaj hnub ntawm cov neeg uas muaj qhov tsis zoo thiab cov kev hnav ris tsho no yuav tsum ua nrog kev saib xyuas nws thiaj li txhawb nqa kho kom zoo, txo qis kev sib txhuam thiab tiv thaiv kom tsis txhob los ntshav ntawm daim tawv nqaij, rau qhov no nws yog ib qho tseem ceeb uas siv cov khoom tsis zoo ntawm daim tawv nqaij. , uas yog, uas tsis muaj kua nplaum uas txuas nrog zoo heev.

Txhawm rau hnav cov qhov txhab uas muaj ntau qhov zais cia, nws yog ib qho tseem ceeb uas yuav tsum siv cov ris tsho ua los ntawm cov khoom ua npuas polyurethane, vim tias lawv nqus cov kua thiab pab tiv thaiv cov kab mob me.

Hauv qhov xwm txheej uas qhov mob twb qhuav lawm, nws raug nquahu kom siv kev hnav khaub ncaws hydrogel, vim tias lawv pab tshem tawm cov tawv nqaij tuag thiab daws qhov mob, khaus thiab tsis xis nyob hauv thaj chaw. Cov ris tsho hnav ris tsho yuav tsum tau kho nrog cov leeg los yog cov tawv nqaij elastic, nws tsis pom zoo kom siv cov nplaum rau ntawm daim tawv nqaij.

Dab tsi yog cov nyom

Piv txwv hais tias qhov uas mob rau daim tawv nqaij tuaj yeem ua rau qee qhov mob hnyav xws li kis tau tus mob, vim tias qhov tsim teeb meem tawm ntawm daim tawv nqaij ua rau muaj kev cuam tshuam cov kab mob thiab cov kab mob hu ua fungi. Hauv qee qhov xwm txheej loj dua, cov kab mob no nkag mus rau ntawm daim tawv nqaij ntawm tus neeg muaj mob rau cov nqaij tawv tuaj yeem ncav cuag cov ntshav thiab kis mus rau lwm tus lub cev, ua rau mob sepsis.

Cov neeg muaj mob epidermolysis bullosa kuj tseem tuaj yeem noj zaub mov hauv qhov tsis zoo, uas tshwm sim los ntawm cov hlwv hauv qhov ncauj lossis los ntshav, los ntshav los ntawm qhov txhab. Qee qhov teeb meem ntawm kaus hniav, xws li caries, tuaj yeem tshwm sim, vim tias ob sab hauv ob sab hauv qhov ncauj tsis huv heev hauv cov neeg muaj tus kab mob no. Tsis tas li, qee yam ntawm cov kab mob epidermolysis bullosa nce ntxiv kev pheej hmoo ntawm tus neeg muaj mob qog nqaij hlav.