Syndrome "pebaldizm": hore na ke eng e le hore a, matšoao, phumano le ho kalafo

E mong tse fapa-fapaneng metabolism mafu pigment - seoelo lefu "pebaldizm". Gnozdnym tšoauoa ka discoloration tsa moriri hloohong. Sena ha se le seng kotsi setlolo sekoli, empa e le letšoao la palo e khōlō ea mafu a tebileng.

A sehloohong sena a re hlahlobeng ka ho qaqileng "pebaldizm". Sesosa, matšoao, phumano, kalafo, le bona ba tla a hlalosa.

Lisosa tsa moriri tera h

Tlōlo ea metabolism pigment ke depigmentation - pigment tahlehelo, hyperpigmentation - kholiso staining.

Moriri 'mala pigment ba le boleng 2 - eumelinin, pheomelanin. Ba ka cortex ea moriri. Eumelanin etsang moriri ba batsho le ba sootho pheomelanin - bosehla khubelu. Ha e kopana le ka mebala tsena palong motho le sebelisana le moea neha moriri 'mala o itseng. Moriri 'mala e liphatsa tsa lefutso e etselitsoe.

lefu "pebaldizm" Ke hangata hakae ke etsahala, se lefu lena?

Ka tlōla melanocytes eo, pigment-synthesizing moriri, graying le lilemo develops. Ha boemo ba qala ka le monyenyane - sena ke bopaki ba lefu ea liphatsa tsa lefutso, kapa mafu a kotsi.

Mafu a eo ho eona le bothata pigmentary phapanyetsano

Ke hobane'ng ha ho "pebaldizm" lefu, se kheloha ee?

E re e latelang di ho ntshetsa pele ka ho tlōla melanocytes ho:

• pebaldizm foromo ka thōko;
• albinism;
• pebaldizm kopantswe le lefu leihlo, tsebe, letlalo, tsamaiso ea methapo, musculoskeletal tsamaiso.

setshwantsho ea tleleniki

Moo e bontša ka boeona e "pebaldizm" lefu, e bolela eng?

Tahlehelo ea pigment moriri ka sebōpeho sa ponahalo ea le phomola hamonate tšoeu hloohong ka etsahala ho tloha tsoalo ho ba motho e moholo.

Kapele ho na le ke ho tlōla metabolism pigment, e tebileng le ho feta ka liphello sesosa boima.

Ka thōko discoloration phomola hamonate ho kopantswe le karolo e itseng ea discoloration ka phatla ea hao. Tsamaea le ponahalo ea foci ea hyperpigmentation, 't. E. Gnozdnogo ntshofala tsa letlalo holim pel'a mpeng, lirope, meomo, sefubeng, matsoho litekanyo ho tloha 1 limilimithara ho cm, 6.

Lefu nanarela ka mor'a pebaldizmom

Ho na le sehlopha se seholo sa mafu a le anomalies ya ntshetsopele, kopantseng hammoho le soeufala moriri makala tsenyo e tebileng le tsamaiso ea. Undiagnosed bolwetse tla boloka bophelo ba ngoana le batsoali ho fana e ngata mathata.

lefu Waardenburg-Klein

Lefu lena le ke autosomal paterone ke 'muso o hlaheletseng oa lefa, ke hore,. E. ho tšoanang, hangata e le hlaha ka bashanyana le banana. Sekoli liphatsa tsa lefutso li ka li futsitseng ho motsoali oa ka.

Ho phaella tabeng ea ho soeufala tsa palesa moriri ke lemohiloeng kakaretso le ocular liponahatso tsa lefu lena.

matšoao a Ocular lefu lena:

• hypoplasia tsa eyelashes ea;
• libakeng tse ea bohlooho ka lintši ka hare;
• moqotetsane leihlo slit;
• fapaneng mahlo 'mala;
• hyperopia;
• pharaletseng aroloa ka mahlo.

matšoao a tloaelehileng le sebelisoang malapeng ba ka 'na ba sa tšoaneng likhato tsa ho tiea:

• leeme albinism;
• sephara nasal borokho;
• lekgutshwanyane seemo;
• anomalies meno;
• ba sa utloeng litsebeng-mutism kapa ka thōko a sa utloe litsebeng;
• anomalies tsa litho tsa ka hare;
• fetola boholo ba lehata la.

Fuoa hore qalong le ho tiea tsa lefu lena le hlaha ka feela bonyane pebaldizmom hloka ho screened ho qhelela ka thōko bolwetse tebileng. Ke seo piebaldism.

lefu Wolfe e

Ka thōko heterochromia, 't. E. A fapaneng leihlo' mala o hangata e fumanoang ka liphoofolo. Ka batho, e leng maqhubu a har'a baahi bohle ba e 1%.

Famous batho ba nang le fapaneng leihlo mala: kaganaa Kunis ( "Oz The Great le Matla", "Bloodlines", "Jupiter Ascending"), British actor Dzheyn Seymur, best tsebahala ka filimi "Live le re E-shoa", Devid Boui - British lefika sebini, mongoli oa lipina, mohlahisi, moenjiniere e phetseng hantle, moetsi oa litšoantšo, motšoantšisi.

Ka thōko heterochrony ke lefu, 'me hammoho le pebaldizmom syndrome, e bitswang Wolfe,' me e etsa hore e botholo potlako eketseha.

Ho phapang lefu lena e lokela ho ba le retinitis pigmentosa - pigment epithelium dystrophy leihlo. Hangata e mothofatso tsela ea lefu lena - retinitis pigmentosa ntle pigment.

'Mè le morali oa ka lefu la "pebaldizm"

Lefu lena le ke lefa la recessive, chromosome-amahanngoa basali. Mosali e mong e ntse e feta phatsa ea lefutso ho mora oa hae, 'me o na le lefu le itseng. Barali ba batho bana ba phetseng hantle, haeba ba ne ba ba le 'mè ka bophelo bo botle. E ne e tla ba pontšo feela ea pebaldizm.

Haeba 'mè e ka bophelo bo botle ea bongaka, empa ke microcyteme ba phatsa ea lefutso e, ngoana o tla ba kula.

Ho hlahloba lefu lena le ho sebedisa ophthalmoscopy, eo e tsebahatsa le matheba fundus pigmentation joaloka tšoèlè-jele.

Gipomelanoz Ito

Lefu lena le hlaha ho tloha tsoalong. Ba utloa bohloko bana ba sexes bobeli le maqhubu a lekanang. Ha proven phetiso lefa le tsoang ho batsoali ho bana, empa lefu lena le kamehla ho kopantswe le likoli congenital tsa litho tsa ka hare.

fumanoe e entsoeng ea bongaka ka lebaka la matšoao a latelang:

• hlaha ka letlalo amelanotic likarolo tloaelehang ka curls foromo, snowflakes;
• Ka palmar e, bokaholimo plantar le lera la mucous liphetoho tse joalo ha li etsahala;
• sebakeng se ya ntango seteishene toa moriri - pebaldizm;
• lilemo depigmentation libakeng tse ba tlase bonahalang;
• senya tsamaiso ea methapo kamehla e bonahatsa kholofalo kelellong, mafu convulsive, fokotseha mesifa molumo;
• bolwetse ea leihlo e bonahale strabismus sa tšoaneng likhato tsa ho tiea;
• a tsamaea le scoliosis, sefubeng deformation lipheletsong ka 25% ea linyeoe;
• le maqhubu a ya 10% ho kopantswe le likoli pelo.

Ho phapang lefu lena ho tloha boemong bo hlokahalang e bitswang pigment mosese. Ba na le tse tšoanang le matšoao litleleniki tsa, empa sa tsoa se futsitseng.

pigment incontinence

E le lefu ka seoelo e fetisoa "pebaldizm"?

Lefu lena le e fetisoa ho tloha ho 'm'a ho morali. Ho itšetlehile ka ho tiea tsa setshwantsho ea tleleniki e bontšoa ke matšoao a latelang:

• Letlalo lekhopho ka mokgwa wa hlobolisa telele eo, ka mor'a hore ho omisa le ho tloswa ea crusts siea likarolo pigmentation. Foqoha ha ba le 'nete hore maqhubu le ponahalo ea nako le nako ya lisele tse ncha letlalo. From tsena ngoana merero e mebe le nonofo ya mmele ba felloa ke pigment le ba lerootho.
• Dysplasia le leino phetolo, manala, moriri depigmentation ka alopecia le lefatla.
• mafu a methapo ea kutlo li bonahatsoa tsoarelloa tsehetso ea hlabollo-puo ntshetsopele, e ile ea eketseha intracranial khatello, sethoathoa, bofokoli matsohong le maotong.
• maloetse leihlo ha boa tsitsa le bonahatsoa strabismus, nystagmus, lera, fokotswa pono, bofofu.

lefu Gristselli

Sena ke sehlopha sa maloetse a hore ka etsahala ka mefuta e meraro, ho tšoana ka sehloohong ke ho ba teng ha likoatipa discolored tsa moriri hloohong. The lipontšo tse latelang di a fumaneha:

• mafu a methapo ea kutlo le hypopigmentation.
• The mofuta kotsi ka ho fetisisa ea lefu leo ho lona "pebaldizm" lefu hammoho le phytoteratology hematopoiesis. Clinical liponahatso: e leka-lekaneng le feberu, phahametseng tlhaho le kalafo. Tiisetso feberu butle-butle eketsa, o kopanela tlōlong atolosoa sebete, spleen, phokotsong ea erythrocytes, leukocytes, li-platelet, fibrinogen maling. qetellang e amehang le thulaganyo ea tsamaiso ea methapo ea kutlo.
• hypopigmentation tsa letlalo 'me moriri oa sa tšoaneng ho tiea, tseo ho fihlela nakong eo o tsoela pele ntle ho timetsoa ha lisele tsa mali le tsamaiso ea methapo. Bakeng sa fumanoe nakong ea lefu lena le kotsi ba lokela ho batla keletso ea ngaka ena dermatologist, liphatsa tsa lefutso. Ba bonnete ba hore ho nahana ka hore ho na le lelapa la mafu a tse fapa-fapaneng tsa pigment metabolism, ho itlhahloba ho qhelela ka thōko liphetoho liphatseng tsa lefutso phatsa ea lefutso.

Metsotso e jele babeli le kotsing ea lefu ka lebaka la maqhubu a phahameng kopantseng ka Down syndrome, Edwards, Turner.

Hlwaya depigmentation tsa letlalo 'me moriri oa ka u be ho tsoalo, empa ho theha ho ba teng ha lefu Turner e ka ba joaloka motho ea hōla feela ha tšoauoa lekgutshwanyane seemo, lymphostasis, lefu la pelo, khatello e phahameng ea mali, underdeveloped litsoelesa mammary, le tlaase boemong ba ho nipple ka, tloswa ea litho tse ka hare genital connective lesela. Bohlale ka bakuli bana hoo e batlang e ha e utloa bohloko, semelo sa feto-fetoha e hoo e ka bang kamehla bo phahameng, ho na le ba bang ba nyakallo bo nepahetseng, e hlalosang e ntle tlwaetsa ea sechaba ea bakuli tsena.

bofello

Ho batho, phatsa ea lefutso ea pigmentation tlwaelehileng tsa letlalo ke kamehla e ka sehloohong mabapi le phatsa ea lefutso le boikarabelo bakeng sa tlōlo ea metabolism pigment.

Se boleloang ke tsa motheo tsa mathata a ka pigmentation e le hore ba ba batshwai kotsi ya lefu chromosomal, malformations multiple congenital.

Leha e le tlōlo ea metabolism letlalo pigment, lera la mucous, moriri o lokela ho batla keletso ho setsebi. Re tšohlile "pebaldizm" lefu. ke eng ho eona, ho ile ha hlaka.