Syndrome "pebaldizm": chii, hunokonzera, zviratidzo, kuongorora chirwere uye kurapwa

Imwe siyana ruvara muviri zvirwere - isingawanzowanikwi chirwere "pebaldizm". Gnozdnym hwokuratidza atindivare bvudzi romusoro wake. Izvi hazvisi mhembwewo zvizorwa urema, asi chiratidzo vakawanda zvirwere zvakakomba.

Munyaya ino tichakurukurei muudzame "pebaldizm". Zvinokonzera, zviratidzo, chirwere, kurapwa, zvichange kurondedzerwa.

Zvinokonzera bvudzi chibi

Zvaipesana ruvara muviri chinhu depigmentation - ruvara kurasikirwa, hyperpigmentation - amplification staining.

The bvudzi ruvara ruvara kuva ukoshi 2 - eumelinin, pheomelanin. Ivo vari cortex bvudzi. Eumelanin anoita bvudzi dema uye shava pheomelanin - tsvuku-tsvuku. Tikafunga pigments ava nomumwe nhamba uye kubata pamwe mhepo rinopa bvudzi imwe ruvara. Hair ruvara ari majini chiite.

chirwere "pebaldizm" kakawanda sei kuitika, chii chirwere ichi?

In kutyora melanocytes, ruvara rwacho-synthesizing bvudzi, graying nezera hwapedyo. Kana mamiriro rinotanga muduku - ichi uchapupu nemagene chirwere, kana zvirwere zvakakomba.

Hosha umo achitambura pigmentary Exchange

Nei "pebaldizm" urwere, chii kutsauka ichi?

Zvinotevera inoti vari kukura kutyora melanocytes:

• pebaldizm mune kure chimiro;
• albinism;
• pebaldizm pamwe chirwere ziso, nzeve, ganda, tsinga, zvetsandanyama nemapfupa hurongwa.

zvekuchipatara mufananidzo

Sei kuratidza pacharo "pebaldizm" urwere, zvinorevei?

The kwebvudzi ruvara muchimiro nechitarisiko jena curl they mumusoro anogona kuitika kubva pakuzvarwa kusvikira vayaruka.

Nokukurumidza pane kutyora ruvara muviri, asi zvikuru kuti chikonzero kwakanyanya nemigumisiro.

Kure atindivare curl they pamwe chikamu atindivare pahuma yako. Pamwe kuonekwa foci pamusoro hyperpigmentation, t. E. Gnozdnogo achiipa ganda pamberi pechiso mudumbu, dzakatendereka, makumbo, muchipfuva, maoko kukura kubva 1 mm kusvika 6 cm.

Chirwere dzokuvanda shure pebaldizmom

Pane guru boka zvirwere uye anomalies okukura, kubatanidza pamwe bleaching bvudzi chakakura kukuvadza nhengo uye hurongwa. Undiagnosed zvokurwara achachengetedza upenyu mwana uye vabereki vazopa taisireva.

Waardenburg-Klein urwere

The chirwere chiri autosomal chikuru mufananidzo nhaka, kureva. E. The chete kazhinji kunoitika muna vakomana nevasikana. The majini urema inogona nhaka kubva kana mubereki.

Kuwedzera bleaching bvudzi tambo zvinochengetwa rwevanhu uye ocular zvinoratidza chirwere.

Ocular zviratidzo chirwere ichi:

• hypoplasia of eyelashes;
• pfumbu nzvimbo pamusoro yomukati nevagari vayo;
• yakamanikana ziso mutswe;
• Meso siyana wechitema;
• hyperopia;
• nevakawanda munzvimbo dzokuzororera dzaiva meso.

Common zvingaratidza kuva kunosiyana inosiyana zvakaoma kwazvo:

• kukwana albinism;
• paupamhi nasal zambuko;
• nomumhu;
• anomalies kwemeno;
• matsi-mutism kana yoga kusanzwa;
• anomalies of zvomukati;
• kushandura kukura dehenya.

Akapiwa kuti pakutanga kwokuomerwa chirwere uye rinowanika shoma pebaldizmom chete vanofanira firimu kuti asava zvakakomba zvokurwara. Ndizvo piebaldism.

Wolfe kuti urwere

Kure heterochromia, t. E. A akasiyana ziso ruvara riri kunyanya kuwanikwa mhuka. Muvanhu, kakawanda pakati yose ndimo 1%.

Famous vanhu vane zvakasiyana ziso ruvara: Mila Kunis ( "Oz The Great uye Wamasimba", "Bloodlines", "Jupiter Ascending"), British mutambi Dzheyn Seymur, zvakanakisisa kuzivikanwa kuti firimu "Live uye Regai Die", Devid Boui - dombo British singer, kunyora nziyo, mugadziri, mutsindo injiniya, yokudhirowa, mutambi.

Kure heterochrony chirwere, uye akabatana pebaldizmom chirwere inonzi Wolfe, uye kunoita kuti munhu kukura nokukurumidza kusanzwa.

Kusiyanisa chirwere ichi chinofanira kuva retinitis pigmentosa - ruvara epithelium dystrophy ziso. Kazhinji musimboti mukurwara ichi - retinitis pigmentosa pasina ruvara.

Amai mwanasikana pamwe chirwere kuti "pebaldizm"

The chirwere recessive, nevakadzi chromosome-chokuita nhaka. Mukadzi inopfuura majini mwanakomana wake, uye ane chirwere. Vanasikana vevanhu ava mutano, kana vane mai wakasimba. Kwaizova chete kuoneka pebaldizm.

Kana mai akanaka akaora, asi ari mutakuri Gene, mwana achava kurwara.

Rokuongorora maperembudzi uchishandisa ophthalmoscopy, iro rinotaura zviri fundus pigmentation mavara akafanana dzakafusiwa.

Gipomelanoz Ito

The chirwere hwapedyo kubva pakuberekwa. Tenderai vana aroorane zvakaenzana maitikiro. Not nekusimba nhaka hutachiwana kubva vabereki vevana, asi chirwere anogara pamwe vakaremara pakati zvomukati.

The chirwere chinoitwa akaora pahwaro zvinotevera zviratidzo:

• kuoneka ganda migove amelanotic noshamisa chimiro curls, twemazaya echando;
• On the Palms, plantar hukawanika uye mucous membranes kuchinja kwakadaro regai kuitika;
• mune zvenyama munharaunda bleached bvudzi chechitima - pebaldizm;
• zera depigmentation nzvimbo havazodi kuonekwa;
• kukuvara tsinga hwagara akaratidza retardation mupfungwa, convulsive zvirwere, ungaderedza tsandanyama matauriro;
• zvokurwara ziso pachena strabismus kunosiyana inosiyana nekuomarara;
• akaperekedza scoliosis, chipfuva deformation migumo muna 25% nyaya;
• aine kakawanda 10% pamwe mwoyo kuremara.

Kusiyanisa chirwere ichi kubva zvakafanira mugariro inonzi Urinary ruvara. Vane zvekuchipatara zviratidzo zvakafanana, asi achangobva nhaka.

ruvara incontinence

Ko isingawanzowanikwi chirwere ari inoparadzirwa "pebaldizm"?

Maperembudzi chakasvitswa kubva mai nomwanasikana. Zvichienderana kwokuomerwa ari kurapwa mufananidzo kunoratidzwa anotevera zviratidzo:

• Skin upundu ari muchimiro rinoparadza refu iyo, pashure kuomesa uye kubviswa crusts siya zvikamu pigmentation. Kwemakomo vane undulating nzira pamwe periodic nechitarisiko ganda masero matsva. Kubva izvi runofarira kuibvisa mwana anorasikirwa ruvara uye yava nani.
• Dysplasia uye zino configuration, zvipikiri, bvudzi depigmentation sezvo alopecia nemhanza.
• Chetsinga Zvirwere zvinozarurwa kunonoka psycho-kutaura kukura, vakawedzera intracranial kumanikidzwa, nezvipusha, kusimba maoko nemakumbo.
• Ziso Zvirwere dzinotsauka uye pachena strabismus, nystagmus, tsanga, akaderedza chiratidzo, upofu.

Gristselli urwere

Ichi boka zvirwere zvinogona kuitika muna matatu siyana, chikuru zvakafanana kuvapo rakabuda ruvara makwapa akasvibirika bvudzi romusoro wake. Zvinotevera Matafura dziripo:

• Chetsinga nepfungwa pamwe hypopigmentation.
• The kupfuura ngozi Chirwere umo "pebaldizm" chirwere pamwe havachanyatsogoni hematopoiesis. Clinical kuratidzwa: mwero nefivha, kutenda mashura zvapambira uye kurapwa. Kuchinyanya Fivha zvishoma nezvishoma kunowedzera, anobatana wakakurisa chiropa, chirwatata, iyo rutapudzo pamusoro erythrocytes, leukocytes, maplatelet, fibrinogen muropa. The yokupedzisira rinobatanidzwa dzinotsanagura tsinga.
• hypopigmentation ganda uye bvudzi kunosiyana zvakaoma kwazvo, iyo kusvikira zvino zvakabuda pasina kuparadzwa masero eropa uye tsinga. Nokuti nenguva zvokuongorora chirwere ichi ngozi vanofanira kutanga hematologist dermatologist, genetics. Iva nechokwadi kufungawo kuvapo mhuri zvinetso siyana ruvara muviri, kuti kuongorora kuti asava Gene kunotouraya.

Yokukurumidzira ngozi nehosha nokuda mukuru kakawanda tichibatanidza pamwe chirwere cheDown syndrome, Edwards, Turner.

Ziva depigmentation ganda uye bvudzi kunogona pakuzvarwa, asi kuti varatidze pamberi Turner kuti chirwere unogona kuva munhu kukura chete kana vanozvisanangurira nomumhu, lymphostasis, mwoyo chirwere, BP, underdeveloped mammary omumuviri, yakaderera kubva munyatso, kuzvitsinhanisa yomunyika sikarudzi connective jira. Intelligence pavarwere izvi zvinenge asingadi kutambura, background manzwiro ari inenge nguva dzose akakwidziridzwa, pane zvimwe zvakanaka euphoria, rinotsanangura yakanaka evanhu kwadzakabva wevarwere izvi.

mhedziso

Muvanhu, majini pakati dzose pigmentation ganda anogara kuzivikanwa pachinhu majini anokonzera kutyora ruvara muviri.

The chikuru zvinoreva nezvinetso pigmentation ndechokuti vari zvezvinangwa ngozi pamusoro chromosomal chirwere, vakawanda vachiberekwa malformations.

Chero kutyora ganda ruvara muviri, mucous membranes, bvudzi vanofanira kubvunza nyanzvi. Takurukura "pebaldizm" syndrome. Chii, zvakava pachena.