Matenda Urbach-Vite akuti: zizindikiro, chithandizo, madokotala ananena zawo

Disease Urbach-Vite - ndi osowa kwambiri majini matenda, opatsirana mu autosomal unsembe recessive. Chifukwa cha matenda - ndi kusintha pa khromozomu yoyamba ECM1 mbewu. Mu mabuku a zamankhwala pali dzina lina matendawa - lipoid proteinosis.

History of anapeza

Mlandu matenda, amene zizindikiro zogwirizana ndi matenda Urbach-Vite, anawalemba mu 1908. Iwo anafotokoza pulofesa Swiss ndi audiologist Friedrich Siebenmann. Mu 1925 ndi dermatologist Swiss ndi histologist Iogann Misher anafotokoza atatu milandu ofanana.

Komabe, mwalamulo ndi osowa recessive majini matenda zalembedwa yekha mu 1929 ndi dermatologist ndi Urbach otolagingologom Vita. Iwo Erich Urbach otchedwa kudwala lipoid proteinosis, thangwi iye atawira kuti zokhudzana ndi vutoli wa zamadzimadzi ndi mapuloteni madipoziti mu zimakhala za thupi.

Popeza kutsegula wakhala anawerengedwa malinga ndi zinthu zosiyanasiyana kuchokera milandu mazana atatu ndi mazana anayi a matenda padziko lonse.

zizindikiro

Mwa zizindikiro ambiri matenda - totupa padziko zikope monga papules (iwonso zilipo pa Luba ndi khosi), wodwala akukhala kusasa mawu, pali kuchepetsa mphamvu regenerative wa khungu, mucosa thickening ndi induration ya ubongo minofu m'dera mwakanthawi. The chizindikiro otsiriza ndi owopsa chifukwa kungachititse kuti zosatha Lobe khunyu.

matenda oyamba kumaonekera detstkom zaka (zaka 3-7). Zinali ndiye kuti pali totupa pakhungu, ndi hoarseness.

Tikumbukenso kuti zizindikiro ndi osokoneza ndiponso mwina wosiyana kwambiri wodwalayo mtima. Monga matenda Urbach-Vite imafalikira autosomal recessive, zilibe kuonekera ngati genotype wa allele yachibadwa lalikulu. Nthawi zambiri anthu sadziwa n'komwe kuti ali onyamula jini ZOOPSA. Ena TV ndi matenda osowa alibe zizindikiro. Ndipo pokhapokha ngati makolo anu onse ali jini udindo proteinosis lipoid, matenda kuonekera mbewu.

diagnostics

matenda Urbach-Vite ndi kovuta kuti apezeke chifukwa zizindikiro si konsekonse. Pankhani ya totupa (papules) pa zikope za, papular totupa pakhungu wa khosi ndi pamphumi matenda umaphatikiza kuchezera dermatologist.

Pakuti amaganiziridwa matenda Urbach-Vite anatumizidwa Kujambula popanda Mosiyana amene angazindikire kudzikundikira kashiamu mu thupi. Komabe, chizindikiro ichi si kuti lipoid proteinosis. Calcification ndi khalidwe la nsungu simplex encephalitis ndi.

A mbali yaikulu pa matenda a masewero kafukufuku chibadwa. Kuti limachititsa kudziwa motsimikiza ngati pali ECM1 jini kusintha, ndipo ngati ndi choncho, ngati masinthidwe anachita kutsogolera kwa matenda Urbach-Vite.

chithandizo

Ngakhale kuti proteinosis lipoid chatsegulidwa kwa nthawi yaitali, ndi matenda osowa, monga ambiri kuvutika majini sangakhoze anachiritsidwa. Komabe, zochitika atsopano m'munda wa sayansi kulola kuchepetsa zizindikiro zambiri za matendawa.

Choncho, azimuth zabwino unkachitika pamene kulandira "heparin" ndi "dimethyl sulfoxide" Ngakhale milandu onse. Dongosolo Oral wa Mlingo yaing'ono "dimethylsulfoxide" (Dimexidum) chifukwa katundu odana ndi yotupa mankhwala, kuchepetsa dermotropic matenda mawonetseredwe.

Ntchito "D-penicillamine" ankakhalanso bwino, koma osati ankagwiritsa ntchito. Mwalamulo ndi milandu odwala "Tigasonom" imene ambiri analamula kuti psoriasis.

Popeza kashiamu mafunsidwe mu zimakhala ubongo kungachititse kuti m'badwo wa nyemba owonjezera magetsi ndi matenda a khunyu, odwala Kwalamulidwa anticonvulsants ( "Metindion" "ethosuximide").

kaonedwe

Urbach-Vite matenda ndi mankhwalawa monga ulamuliro, si owopsa. Anthu odwala matenda akhoza moyo wautali ndiponso wodzaza moyo. Koma sitiyenera kunyalanyaza dokotala maulendo zonse. Chifukwa cha thickening wa Kakhungu mucous zina osauka airway kutsekeka. odwala amenewa kubwezeretsa patency wa airway ali wofunikila tracheotomy kapena ntchito mpweya woipa laser.

Pa nthawi imene madipoziti kashiamu kuyambitsa khunyu khunyu, m'pofunika kuona katswiri-epileptolog amene amasankha chithandizo choyenera, malingana ndi kuopsa kwa zizindikiro.