Npauj Npaim Me Nyuam, tom qab lub npe zoo nkauj no, yog qhov mob uas tib neeg tsis tuaj yeem xav txog. Vim li cas kuv thiaj hais li ntawd? Nws lub npe tag nrho yog muaj keeb kwm kab mis uas muaj keeb heev epidermolysis bullosa, thiab daim tawv nqaij yog li tsis yooj yim li cov tis ntawm tus npauj npaim. Tam sim no ua raws kuv kom nkag mus rau hauv lub ntiaj teb ntawm tus menyuam yaus.
Dab tsi yog cov tshuaj ntsuab Epidermolysis bullosa?
Epidermolysis bullosa (EB) yog cov kab mob tawv nqaij tsis tshua muaj tus kab mob caj dab tom qab kev sib txhuam me ntsis tom qab kev sib txhuam me me lossis kev sib tsoo. Tus neeg mob tawv nqaij yog qhov tsis zoo li tus npauj npaim tus tis, mob me ntsis tuaj yeem ua rau mob tawv nqaij thiab cov tawv nqaij thiab tsis tuaj yeem kho tau rau lub neej.
Heredily Epidermololysis bullosa tshwm sim?
EB tsis yog kab mob kis tau, nws tshwm sim los ntawm kev ua txhaum caj ces. Peb cov tawv nqaij tau muab faib ua cov epidermis thiab dermis, uas tau sib khi ua ke los ntawm cov kua nplaum "(protein). Muaj teeb meem nrog cov "kua nplaum" ntawm EB cov neeg mob, uas ua rau txheej txheem tawv nqaij cais tau yooj yim, yog li cov hlwv yog tsim. Lub hauv paus ua rau muaj teeb meem nrog "kua nplaum" yog tias qee cov noob muaj kev sib hloov.
EB tau muab faib ua plaub pawg:
Epidermolysis yig bulosa yooj yim (bs):
Intraepidermal Bluab los ntawm Keratin tsis xws luag, nruj blisters nrog ib lub cheeb ntawm 0.5-2.0 cm, thiab intraiteplermal bluab yog tshwm sim los ntawm keratin tsis xws luag. Tsis muaj nti tau sab laug tom qab kho, tab sis pigmentation lossis hypopigmentation yuav nyob twj ywm.
Dystrophic Epidermolysis bulosa (deb):
Ntaus Vii collagen tsis txaus ua rau kev puas tsuaj ntawm dermal - zoo li "yam ntxwv zoo tom qab kho tau rau txhais tes.
Junctional Epidermolysis Bulosa (Jeb):
Kev ua kom tuag taus yog tshwm sim los ntawm Laminin tsis txaus, nrog cov hlwv uas dav, bullae, thiab kev yaig tam sim no thaum yug los.
Ntau qhov system koom tes: Tej zaum yuav nrog kev puas tsuaj rau lub pa ua pa thiab zom plab zom mov
Kindler Syndrome:
Ib txoj kev phaesotype tshwm sim los ntawm qhov tsis xws luag nyob rau hauv ib multiproteIin complex
Tej zaum yuav cuam tshuam rau cov epidermis, txheej ntshiab, thiab SubloMina Densa, nrog cov tawv nqaij Attendy thiab Telangiectasia nrog hnub nyoog
Soj ntsuam tshwm sim:
Mechanically gragile, nro bullae, thiab erosive bullae, thiab erosive crusts yog cov muaj ntau yam ntawm txhua yam ntawm cov tshuaj lom b.
Cov tawv nqaij ua kom tawv nqaij nce ntxiv, thiab txawm tias kev sib txhuam me me tuaj yeem ua rau kev sib txhuam me me tuaj yeem ua rau tawv nqaij delamination, feem ntau hauv feeb, tab sis kev tawv ncauj, thiab crusting. Lwm tus epithelia kuj tseem yuav raug cuam tshuam, suav nrog lub conjunctiva, xees kab ke, zom (tshwj tsis yog plab), thiab kev ua haujlwm plab), thiab kev ua me ntsis), thiab kev ua me me), thiab kev ua me me), thiab kev ua me me), thiab kev ua haujlwm plab), thiab kev ua haujlwm plab), thiab kev ua me ntsis), thiab kev ua me me), thiab kev ua haujlwm plab), thiab kev ua me ntsis), thiab kev ua haujlwm plab), thiab kev ua me ntsis), thiab kev ua me nyuam tsawg), thiab kev ua me me), thiab kev ua haujlwm plab), thiab kev ua me me), thiab kev ua me ntsis), thiab kev ua me me), thiab kev ua me me), thiab kev ua me me), thiab kev ua me me), thiab kev ua me me), thiab kev ua haujlwm plab), thiab kev ua me nyuam muaj menyuam. Xws li lub vev xaib tshwj xeeb muaj ntau dua hauv dystrophic eb thiab tsis tshua muaj ntau hauv Jeb. Bullae, kev yaig, mob plab, thiab / lossis nti nti.
Kho:
Txog tam sim no, tseem tsis muaj kev kho ua tiav rau cov kab mob Epidermolysis muaj cov kev kho mob thiab kev tswj hwm tuaj yeem txhim kho cov tsos mob thiab txhim kho lub neej zoo. Tam sim no, lub chaw kuaj mob tseem ceeb yog cov txhab txhab txhab nyiaj kho mob, tiv thaiv, thiab cov tsos mob.
Daim tawv nqaij ua haujlwm:
Mam noo kho cov khaub ncaws yuav siv tau rau cov kev txhab kev kho mob: hlwv tuaj yeem raug purterured thiab drained nrog steract muaj kev mob thiab tiv thaiv microbial txoj hnyuv. Cov tawv nqaij tawg tuaj yeem them nrog tshwj xeeb tsis yog- cov khoom noj khoom haus zoo nkauj thiab lipid -}} bolloid hnav khaub ncaws. Hloov cov hnav khaub ncaws txhua hnub thiab siv cov khaub ncaws tshwj xeeb (xws li Silicone Gel Silicone mesh / ultra- nyias nyias nyias nyias cov khaub ncaws nyias nyias). Silicone gel txo cov dej evaporation los ntawm lub cev thaiv cov nyhuv lub cev, tswj hwm qhov sib npaug ntawm collagen synthesis thiab kev tsis zoo, txwv tsis pub tshaj tawm ntawm hom I Collagen; Txo cov qog nqaij necrosis qhov tseem ceeb- (tnf {{}) qhia, txo txoj hlab ntaub ntawm cov kev txiav txim electrostatic. Kev tshawb fawb RCTION
