Syndrome "pebaldizm": waxa ay tahay, keena, calaamadaha, iyo daaweynta

Mid ka mid ah noocyo kala duwan oo ka mid ah xanuunada-shiid Midabka - syndrome dhif ah "pebaldizm". Gnozdnym lagu gartaa midab of timaha madaxiisa ku. Tani ma aha cillad aan dhib la isku qurxiyo, laakiin calaamad u ah tiro badan oo ah cudurro halis ah.

In this article aan tixgelin doonna si faahfaahsan "pebaldizm". Sababaha, calaamadaha, cudurka, daaweynta, waxaa sidoo kale lagu tilmaamay doonaa.

Sababaha bleach timaha

Xadgudubka shiid Midabka waa depigmentation a - khasaaraha Midabka, hyperpigmentation - xayd-weyneeye.

Midabka The midabka timaha leeyihiin qiimo ah 2 - eumelinin, pheomelanin. Waxay yihiin in kiliyaha ee timaha. Eumelanin dhigaysa timo madow iyo brown pheomelanin - huruud ah-cas. isku dhafan oo ka mid ah pigments in tirada shakhsi iyo in la dhexgalo oo la hawada siinayaa timaha midabka gaar ah. Midabka timaha waxaa hiddo qorsheeyay.

syndrome "pebaldizm" Intee baan marar badan dhacdo, waa maxay cudurka this?

In xadgudub ah melanocytes, timaha Midabka-synthesizing ah, graying da'da yeesho. Haddii xaalad bilaabmin da 'yar ah - taasi waa caddeyn ah cudur hidde, ama cudurro halis ah.

Cudurada taas oo u xanuunsadaa sarrifka pigmentary

Waa maxay sababta ay jirto a "pebaldizm" syndrome, waa maxay weecasho this?

The waddamada soo socda ayaa soo koraya xadgudub ku ah melanocytes ah:

• pebaldizm qaab go'doon,
• albinism;
• pebaldizm weheliso cudurada isha, dhegta, maqaarka, habdhiska, nidaamka muruqyada.

sawir clinical

Sidee laftiisa "pebaldizm" syndrome teedu, maxay ka dhigan tahay?

Guuldaradii ugu of Midabka timaha qaab muuqaalka Laabid cad on madaxa dhici karaan laga bilaabo dhalashada ilaa qaangaarnimada.

The si degdeg ah waxaa jira xadgudub shiid Midabka, ka sii daran cawaaqib Jidka culus.

Laabid midab go'doon weheliso qayb ka mid ah midab on foolkaaga. Weheliyay muuqaalka kore ee Axdigu ee hyperpigmentation, t. E. Gnozdnogo madoobaato maqaarka dushiisa hore ee caloosha, bowdyaha, lugaha, xabadka, gacmaha dhinac laga bilaabo 1 mm ilaa 6 cm.

Cudurka danbaysay pebaldizmom

Waxaa jira kooxo badan oo cudurro iyo cillado horumarka, wada isku sharraxin timo bleaching xubnaha dhaawac halis ah iyo nidaamka. cudurada baarin wuu dhawri doonaa nolosha ilmaha iyo waalidku ay u bixiyaan dhibaato badan.

Waardenburg-Klein syndrome

Cudurku waa autosomal Kudayasho xukuma dhaxalka, E. ie. Kaasu wuxuu inta badan ku dhacaa gabdhaha iyo wiilasha. iin The hidde la iska dhaxli karo waalidka midkood.

Waxa intaa dheer in bleaching ee qaybood timaha waa daliilka guud iyo indhaha la fiirsanayo cudurka.

calaamadaha indhaha ah ee cudurka this:

• hypoplasia of xoqa,
• meelaha cirro on the sunnayaasha gudaha,
• dillaac isha cidhiidhi ah;
• indhaha midabo kala duwan leh;
• hyperopia;
• indhaha si ballaaran u dhaxeyso.

Calaamadaha caadiga ah waxaa laga yaabaa in darajo darraanta kala duwan:

• albinism qayb ahaan;
• buundada sanka ballaadhan,
• laakiinse sarajooggiisu gaaban;
• cillado ee ilkaha;
• kuwii dhegahala'aana wax-mutism ama lumista maqalka ee go'doonka ah;
• cillado oo ka mid ah xubnaha gudaha,
• beddeli size ee qalfoofka.

Marka la eego in marka hore darnaanta cudurka iyo dhacdaa pebaldizmom ugu yar kaliya u baahan tahay in la baaro si looga saaro cudurada halis ah. Taasi waxa ay piebaldism.

Wolfe ayaa syndrome

Go'doon E. A midabka indhaha kala duwan heterochromia, t. Waxaa inta badan laga helaa xayawaanka. In aadanaha, inta jeer ee dadka oo dhan waa 1%.

Caanka ah dadka la midabka indhaha kala duwan: Mila Kunis ( "Oz The Great iyo awood leh", "Bloodlines", "Jupiter oo kor u kacaya"), Atariishada British Dzheyn Seymur, wanaagsan ee loo yaqaan for filimka "Live oo ha dhinta", Devid Boui - dhagax weyn British Fanaanada, Xisbi, soo saare, injineer dhawaaqa, artist, actor.

heterochrony go'doon waa cudur, iyo isku dhafan oo syndrome pebaldizmom yeedhay Wolfe, iyo keenaysaa in culeys dhakhso u kordhaya.

Si aad u kala cudurkan la pigmentosa retinitis waa in - isha Midabka Dystrophy epithelium. Inta badan muuqashadii Dabcan cudurka this - pigmentosa retinitis aan Midabka.

Hooyo iyo gabadh la syndrome ee "pebaldizm"

Cudurku waa dhaxalkii recessive, koromosoomyada-lala haweenka. Qof dumar ah oo ag maraaba wuu hiddo u qaaday inuu wiilkiisii, oo wuxuu haysto oo cudur. Gabdhaha nimankan caafimaad, haddii ay hooyo caafimaad. Waxa ay noqon doontaa muujinta kaliya ee pebaldizm.

Haddii hooyadu waa caafimaad caafimaad, laakiin waa side of hiddo, ilmahaagu wuxuu noqon doonaa bukay.

Si loo ogaado cudurka isticmaalaya ophthalmoscopy, taas oo cadaynaysa baraha midabka fundus sida aboor cunay.

Gipomelanoz Ito

Cudurku wuxuu ku yeesho laga bilaabo dhalashada. reer labada jinsiba leh inta jeer ee loo siman yahay daaya. Ma la xaqiijiyay gudbinta dhaxlo waalidka farcankaaga, laakiin cudurka waxa had iyo jeer lagu daro cilladaha lagu dhasho oo ka mid ah xubnaha gudaha.

Waxaa cudurka lagu sameeyey caafimaad ku saleysan oo ka mid ah calaamadaha soo socda:

• muuqan on maqaarka qaybo amelanotic yaab leh in Suul foomka, barafka xashka ah;
• On palmar ah, meelaha plantar iyo xub-axaleedka isbedel sida ma dhici;
• gobolka temporal saldhigga timaha bleached - pebaldizm;
• meelaha depigmentation da'da yar noqdaan la arki karo;
• waxyeello ku habka dareemayaasha jirka ayaa had iyo jeer u muuqday dhimaal maskaxeed, xanuunada maskaxda la marsiiyo, hoos u muruq,
• cudurada of strabismus ku cad isha degrees darraanta kala duwan;
• qalooca la socday, xabadka jirka lahayo ku in 25% ka mid ah xaaladaha,
• la soo noqnoqoshada ah 10% ay weheliso wadno xanuun.

Si aad u kala cudurkan xaalad lagama maarmaanka u yeedhay Midabka kaadida. Waxay leeyihiin calaamado clinical la mid ah, laakiin dhawaan dhaxlay.

Midabka saxaarada

Ma syndrome dhif ah loo kala qaado "pebaldizm"?

cudurka waxaa soo gudbiyo hooyada gabadhii. Iyadoo ku xiran darnaanta sawirka caafimaad oo lagu muujiyey by calaamadaha soo socda:

• Finan maqaarka ah qaab strips dheer oo, ka dib markii lagu qalajiyo iyo saarista qalalin tago qaybo midabka. Mandheera leeyihiin dabcan ah dhireedku leh muuqaalka kore wareegsan unugyada cusub maqaarka. From kuwaas oo ilmaha la suulaan koriinkedu lumin Midabka iyo yaraaday.
• Kobac la'aanta iyo ilig qaabeynta, ciddiyaha, depigmentation timo alopecia xiiro.
• xanuunada dareemayaasha waxaa lagu muujiyey horumarinta dhac psycho-hadalka, cadaadis intracranial kordhay, qalal, daciifnimo in gacmaha iyo lugaha.
• xanuunada Eye waa deganayn oo Cad strabismus, nystagmus, caadka, aragga hoos, indho la'aan.

Gristselli syndrome

Tani waa koox ka mid ah cudurrada ku dhici kara saddex foomamka, isku mid ah ugu weyn waa joogitaanka jaamaha discolored oo timaha madaxiisa ku. The qaab soo socda waxaa laga heli karaa:

• xanuunada dareemayaasha la hypopigmentation.
• Nooca ugu halista badan ee cudurka, taas oo "pebaldizm" syndrome weheliyaan hematopoiesis oo yaraada. daliilka Clinical: qandho dhexdhexaad ah, paranormal iyo daaweynta. qandho Lixaadka si tartiib tartiib ah u kordhiyaa, biiray beerka ku ballaadhisay, caajisnimo ah, hoos u erythrocytes, leukocytes, platelets, fibrinogen ee dhiigga. dambe waxaa ku lug leh nidaamka habka dareemayaasha jirka.
• hypopigmentation oo ka mid ah maqaarka iyo timaha darraanta kala duwan, taas oo ilaa markaas soo baxa oo aan burburka unugyada dhiigga iyo habka dareemayaasha jirka. Waayo, cudurka waqtigii cudurkan khatarta ah inay la tashadaan dermatologist hematologist ah, genetics. U hubso inay tixgeliyaan jiritaanka qoyska ka mid ah xanuunada kala duwan shiid Midabka, falanqaynta si looga saaro Isbedelo hiddo.

Degdeg ah iyo khatarta cudurka ay sabab u tahay inta jeer ee sare lagu daro Down syndrome, Edwards, Turner.

Aqoonso depigmentation oo ka mid ah maqaarka iyo timaha waxay noqon kartaa marka uu dhasho, laakiin in la dhiso joogitaanka Turner ayaa syndrome noqon kartaa sida qof kor u sii kordhaya oo keliya marka lagu calaamadeeyay dherer gaaban, lymphostasis, cudurka wadnaha, cadaadiska dhiigga ee sareeya, qanjirada caanaha dib dhacsan, meel hoose oo ibta naaska, la bedelay xubnaha taranka gudaha connective maro. Sirdoonka ee bukaanka, kuwaas oo ku dhawaad ma ku xanuunsado, niyadda asalka waxaa had iyo jeer Qolol, waxaa jira qaar ka mid ah rayn wanaagsan, oo uu sharaxayaa ah la qabsashada bulshada wanaagsan bukaannada kuwan.

gunaanad

In aadanaha, hiddo midabka caadiga ah ee maqaarka had iyo jeer waa adag ee la xiriirta hidda mas'uulka ka ah xadgudubka shiid Midabka.

Macnaha aasaasiga ah ee dhibaatooyinka la midabka waa in ay yihiin calamado khatar cudurka koromosoomyada, curyaanimo badan ee lagu dhasho.

xadgudub kasta oo shiid Midabka maqaarka, xub-axaleedka, timaha waa in la tasho dhakhtar takhasus u leh. Waxaan ka fikiray "pebaldizm" syndrome. Waa maxay, waxaa u cadaatay.