Epidermolysis ng'ombe

Epidermolysis bullosa (EB) ndi gulu lamavuto momwe matuza a khungu amapangika pambuyo povulala pang'ono. Zimaperekedwa m'mabanja.

Pali mitundu inayi yayikulu ya EB. Ali:

• Dystrophic epidermolysis bullosa
• Epidermolysis bullosa simplex
• Hemidesmosomal epidermolysis bullosa
• Kuphatikizana kwa epidermolysis bullosa

Mtundu wina wosowa wa EB umatchedwa epidermolysis bullosa acquisita. Fomuyi imayamba pambuyo pobadwa. Ndi matenda omwe amabwera chifukwa cha autoimmune, omwe amatanthauza kuti thupi limadzivulaza lokha.

EB imatha kusiyanasiyana kuyambira zazing'ono mpaka zakupha. Mawonekedwe ang'onoang'ono amayambitsa kuphulika kwa khungu. Mawonekedwe owopsa amakhudza ziwalo zina. Mitundu yambiri yamtunduwu imayamba pobadwa kapena posachedwa. Kungakhale kovuta kuzindikira mtundu weniweni wa EB womwe munthu ali nawo, ngakhale zilembo zamtunduwu zilipo kwa ambiri.

Mbiri ya banja ndiyomwe ili pachiwopsezo. Chiwopsezo chimakhala chachikulu ngati kholo lili ndi vutoli.

Kutengera mawonekedwe a EB, zizindikilo zimatha kuphatikiza:

• Alopecia (kutayika tsitsi)
• Matuza kuzungulira maso ndi mphuno
• Matuza mkati kapena mozungulira pakamwa ndi pakhosi, zomwe zimayambitsa mavuto akudya kapena kuvutikira
• Matuza pakhungu chifukwa chovulala pang'ono kapena kusintha kwa kutentha, makamaka kumapazi
• Kuphulika komwe kumakhalapo pakubadwa
• Mavuto amano monga kuwola kwa mano
• Kulira mofuula, kutsokomola, kapena mavuto ena opuma
• Ziphuphu zazing'ono zoyera pakhungu lomwe lidavulala kale
• Kutaya msomali kapena misomali yopunduka

Wothandizira zaumoyo wanu ayang'ana khungu lanu kuti mupeze EB.

Mayeso omwe amagwiritsidwa ntchito kutsimikizira matendawa ndi awa:

• Kuyesedwa kwachibadwa
• Khungu lakhungu
• Kuyesedwa kwapadera kwa zitsanzo za khungu pansi pa microscope

Mayeso apakhungu atha kugwiritsidwa ntchito kuzindikira mawonekedwe a EB.

Mayesero ena omwe angachitike ndi awa:

• Kuyezetsa magazi magazi m'thupi
• Chikhalidwe chofufuza matenda a bakiteriya ngati mabala akulephera bwino
• Pamwamba endoscopy kapena GI pamwambapa ngati zizindikilo zikuphatikiza kumeza mavuto

Kukula kwa msinkhu kumayang'aniridwa nthawi zambiri kwa mwana yemwe ali ndi EB kapena akhoza kukhala nayo.

Cholinga cha chithandizo ndikuteteza matuza kupanga ndi kupewa zovuta. Mankhwala ena amatengera kukula kwa vutoli.

KUSAMALIRA KWA PANSI

Tsatirani malangizo awa kunyumba:

• Samalirani bwino khungu lanu kuti mupewe matenda.
• Tsatirani malangizo a omwe akukuthandizani ngati madera omwe ali ndi matuza aphulika kapena aiwisi. Mungafunike chithandizo cham'madzi cham'madzi nthawi zonse ndikugwiritsa ntchito mafuta opha maantibayotiki m'malo ngati mabala. Wopezayo amakudziwitsani ngati mukufuna bandeji kapena chovala, ndipo ngati ndi choncho, ndi mtundu wanji womwe mungagwiritse ntchito.
• Mungafunike kugwiritsa ntchito mankhwala amlomo a steroid kwakanthawi kochepa ngati mukumeza mavuto. Muyeneranso kumwa mankhwala mukalandira matenda a candida (yisiti) pakamwa kapena pakhosi.
• Samalirani thanzi lanu lakumlomo ndikupita kukayezetsa mano nthawi zonse. Ndibwino kuti muwone dotolo wamankhwala yemwe amadziwa bwino kuchitira anthu omwe ali ndi EB.
• Idyani chakudya chopatsa thanzi. Mukakhala ndi vuto lalikulu pakhungu, mungafunike mafuta owonjezera komanso mapuloteni othandizira khungu lanu. Sankhani zakudya zofewa ndipo pewani mtedza, tchipisi, ndi zakudya zina zokhazokha ngati muli ndi zilonda mkamwa. Katswiri wazakudya akhoza kukuthandizani pazakudya zanu.
• Kodi kuchita masewera olimbitsa thupi kukuwonetsani kuti muthandizire kulumikizana ndi minofu yanu.

KUGWIDWA

Kuchita opaleshoni yothandizira izi kungaphatikizepo:

• Ankalumikiza khungu m'malo momwe zilonda zakuya
• Kuchulukitsa (kukulira) kwa kum'mero ​​ngati pali kuchepa
• Kukonza zopindika m'manja
• Kuchotsa squamous cell carcinoma (mtundu wa khansa yapakhungu) yomwe imayamba

CHithandizo CHINA

Mankhwala ena amtunduwu atha kukhala:

• Mankhwala omwe amaletsa chitetezo cha mthupi atha kugwiritsidwa ntchito ngati mawonekedwe amthupi mwawo.
• Mapuloteni ndi mankhwala amtundu komanso kugwiritsa ntchito mankhwala osokoneza bongo a interferon akuwerengedwa.

Maganizo ake amatengera kukula kwa matendawa.

Matenda a madera omwe ali ndi matuza ndiofala.

Mitundu yofatsa ya EB imakula ndikukula. Mitundu yayikulu kwambiri ya EB imakhala ndiimfa yayikulu kwambiri.

M'mitundu yayikulu, mabala pambuyo pa matuza angayambitse:

• Zowonongeka kwa mgwirizano (mwachitsanzo, zala, zigongono, ndi mawondo) ndi zina zopunduka
• Kumeza mavuto ngati mkamwa ndi kummero zakhudzidwa
• Zosakanizidwa zala ndi zala
• Kuyenda pang'ono kuchokera ku zipsera

Zovuta izi zitha kuchitika:

• Kuchepa kwa magazi m'thupi
• Kuchepetsa moyo wautali chifukwa cha zovuta zamatendawo
• Kuchepetsa kwa Esophageal
• Mavuto amaso, kuphatikiza khungu
• Kutenga, kuphatikizapo sepsis (matenda m'magazi kapena minofu)
• Kutaya ntchito m'manja ndi m'mapazi
• Kusokonekera kwa minofu
• Matenda a Periodontal
• Kuperewera kwa zakudya m'thupi kwakukulu komwe kumadza chifukwa chodyetsa zovuta, kumabweretsa kulephera kukula
• Khansa yapakhungu lama cell squamous

Ngati khanda lanu likuphulika atangobadwa, itanani omwe akukuthandizani. Ngati muli ndi mbiri ya banja la EB ndipo mukufuna kukhala ndi ana, mungafune kukhala ndi upangiri wa majini.

Upangiri wa chibadwa umalimbikitsidwa kwa oyembekezera makolo omwe ali ndi mbiri yabanja yamtundu uliwonse wa epidermolysis bullosa.

Pakati pa pakati, mayeso otchedwa chorionic villus sampling atha kugwiritsidwa ntchito kuyesa mwana. Kwa maanja omwe ali pachiwopsezo chachikulu chokhala ndi mwana yemwe ali ndi EB, kuyezetsa kumatha kuchitika sabata ya 8 mpaka 10 ya mimba. Lankhulani ndi omwe amakupatsani.

Pofuna kupewa kuwonongeka kwa khungu ndi kuphulika, valani malo ozungulira ovulala monga zigongono, mawondo, akakolo, ndi matako. Pewani masewera olankhulana nawo.

Ngati muli ndi EB acquisita ndipo muli ndi ma steroids kwa nthawi yoposa mwezi umodzi, mungafunike calcium ndi vitamini D zowonjezera. Zowonjezera izi zitha kuthandiza kupewa kufooka kwa mafupa (kupewetsa mafupa).

EB; Kuphatikizana kwa epidermolysis bullosa; Dystrophic epidermolysis bullosa; Hemidesmosomal epidermolysis bullosa; Matenda a Weber-Cockayne; Epidermolysis bullosa simplex

• Epidermolysis bullosa, yotchuka kwambiri
• Epidermolysis bullosa, wosasintha

Wotsutsa J, Pillay E, Clapham J. Malangizo Apamwamba Othandizira Khungu ndi Kusamalira Mabala ku Epidermolysis Bullosa: Mgwirizano Wapadziko Lonse. London, UK: Mabala Amayiko Onse; 2017.

Zabwino, JD, Mellerio JE. Mu: Bolognia JL, Schaffer JV, Cerroni L, olemba. Matenda Opatsirana. Wolemba 4. Philadelphia, PA: Elsevier Saunders; 2018: mutu 32.

Khalani TP. Matenda opatsirana komanso oopsa. Mu: Habif TP, mkonzi. Matenda Opatsirana Matenda. Lachisanu ndi chimodzi. Philadelphia, PA: Elsevier; 2016: chap 16.