Ibhabhathane syndrome: epidermolysis bullosa

Epidermolysis bullosa (EB), okanye njengoko ibizwa ngokuba, ibhabhathane syndrome - oku kuphazamiseka ilifa ezinqabileyo kakhulu. Aba bantwana ibizwa ngokuba abantwana amabhabhathane. Ukuba le igama sisaba nemibutho umdla, esi sifo ayikho nezamkelekileyo tsakisaka. Enyanisweni, izigulane epidermolysis bullosa bachithe ubomi babo iphela isohlwayo esibuhlungu. Isizathu - ulusu lwabo, ethe ngamaphiko butterfly.

Ngelishwa, esi sifo "ibhabhathane syndrome" ayikwazi iintsholongwane. Ngenxa enokuba kubhaliwe kumgaqo ngokunzulu kweli kwinqanaba yemfuza. abantwana abanjalo kufuneka inkathalelo eyodwa: bume ethambileyo ngokupheleleyo, impahla yokunxiba kunye nezihlangu. ERashiya, esi sifo esinganyangekiyo akakho zifakwe kuluhlu izifo kakhulu ezinqabileyo. Noko ke, kukho ingxowa-mali ekhethekileyo ukunceda abantwana Butterfly "uBella."

Qho ngonyaka imali eninzi kuyanikelwa-mali, ukuba ngandlel 'ukunceda abantwana abaye babe sifo "ibhabhathane syndrome". Iifoto izigulane kunokubonwa kweli nqaku, yaye uncwadi zonyango zenzululwazi. Nangona kunjalo, ulwazi epidermolysis bullosa ukuba ifumene ukusasazwa efanelekileyo kumazwe eRussia ico.

Izifo "ibhabhathane syndrome" ayaziwa kuluntu jikelele. Bambalwa Unga ukulibiza igama layo inzima - epidermolysis bullosa. Noko ke, aba bantwana baye bonke phezu amazwe eRussia ico. Ngalinye izigulane kunye nengxaki efanayo: oogqirha zasekuhlaleni abayazi indlela ntoni, ukuba uphethwe "malunga", kwaye usoloko ethembekile iintlobo zesi sifo. Kwaye ngamanye amaxesha unina abusithelise aba bantwana, ekhokelela ekufeni kwabo ngenxa kukhathalelo engafanelekanga.

ibhabhathane syndrome luphawulwa imbonakalo yabantu irhashalala kwiqamza. Kuxhomekeka amaqamza ezi ntlobo zilandelayo Epidermolysis Bullosa (EB):

1. Simple. Oku kwakheka kwi kwiindawo aphezulu ulusu. Isikhumba sikhula ezintendeni kunye iintende zeenyawo. Amaqamza ukuze kwakheke nkqu emlonyeni.
2. Border. Kubonakala kwi isiphambuka dermal-epidermal. Xa amaqamza kakhulu sifo kuvela iindonga kuvuleke iminxunya, amathumbu kunye iphecana genitourinary.
3. Dystrophic. Amaqamza kuvela ngamadolo, izandla, iingalo, inwebu yangaphakathi, inokuyichaphazela kuvuleke iminxunya, kungabangela iingxaki ngokukhula kunye nesondlo. Olu hlobo linophawu Fusion leminwe neenzwane.

Syndrome ibhabhathane - ziziphumo lokusekwa ngokungafanelekanga ye kwiproteni eluswini, okanye ukungabikho kwalo. Kwakhona unobangela sifo inokuba into evele gene kuguquka.

Ekuzalweni, isikhumba umntwana n'ekiweebwayo njengoko eluswini inyoka. Kakade ke, oku kubangela ngumbala kukothuka kwi unina. Abaninzi nje ukwala abantwana abagulayo isekhona kwiwadi kubeleka. Kodwa ke amathuba okusinda umntwana nesi sifo epidermolysis bullosa kwikhaya abantwana kuyinto zero. Uninzi lwabantwana wafa ukhathalelo engalunganga. Ngapha koko, aba bantwana kufuneka ukusingatha ngenyameko.

Lelona xesha linzima izigulane - oku phezulu yobudala ukuya kwiminyaka emi-3. Ngokuthe ngcembe, nangononophelo ekunene, isikhumba coarsens. A abantwana abancinane xa uzama ukuze igaqele zonke amadolo kunye nezandla abamhlophe. isikhumba kunye amaxolo off, ebangela intlungu kakhulu. abantwana abanjalo nkqu kunyikima ngezandla njengomntu oqhelekileyo. Njengoko ekhula, baza ngokuthe ngcembe bafunda ukuzikhusela ngokwabo. Xa ndandineminyaka engama-3 yobomi kuba lula.

Noko ke, izigulana kunye kakhulu lwe Epidermolysis Bullosa nazo imihla ngemihla ubuhlungu obukhulu ukurhawuzelela, nto leyo ayiyi kunceda nokuba antihistamines. Ukongeza, emva kweentsuku ezintathu, kufuneka banyamezele zokubopha eziyimfuneko kunye buhlungu. Aba bantwana, njengazo zonke ezinye abagulela ukufa, izinto ezikhethekileyo. Banamehlo, yinto eningenakukholwa yiyo, mhlawumbi kulo intlungu engathethekiyo, thina lungenakuqondeka.