Syndrome "pebaldizm": oko kukuthi, ibangela, iimpawu, isifo kwaye afumane unyango

Enye iindidi ebala ukuphazamiseka ukutya - onge syndrome "pebaldizm". Gnozdnym luphawulwa kombala iinwele zentloko yakhe. Esi siphene ingozi izithambiso, kodwa iliphawu inani elikhulu kwezifo.

Kweli nqaku siza kuqwalasela ngokweenkcukacha "pebaldizm". Oonobangela, iimpawu, uxilongo, unyango, nazo ziya kuchazwa.

Izinto ezibangela bleach kweenwele

Ukophulwa sombala lokugaya kukho ibala - ilahleko sakhi, hyperpigmentation - yokukhulisa bala.

Iinwele Umbala sombala nexabiso-2 - eumelinin, pheomelanin. Ngokuba ngumonzakalo iinwele. Eumelanin yenza iinwele ezimnyama kunye brown pheomelanin - otyheli-ebomvu. Indibaniselwano yezi zombala kwenani ngamnye kunye nobudlelwane kunye umoya unika iinwele umbala ethile. Hair mbala izakhi ihlelwa.

syndrome "pebaldizm" Kukangaphi zenzeka njani, yintoni na kwesi sifo ndinaso?

Xa waphula umthetho melanocytes, iinwele mbala-synthesizing, Ukungaboni kunye nobudala kuphuhliswa. Ukuba imeko iqala eselula - oku bubungqina sisifo yemfuza, okanye izifo kakhulu.

Izifo apho ophethwe exchange pigmentary

Kutheni kukho "pebaldizm" syndrome, yintoni na le kutenxa?

Ithi zilandelayo asakhulayo waphula umthetho melanocytes:

• pebaldizm ngohlobo ekwanti;
• albino;
• pebaldizm kudityaniswe isifo samehlo, iindlebe, ulusu, luvo, inkqubo musculoskeletal.

picture clinical

Indlela ukuthi kuziveza "pebaldizm" syndrome, oko kuthetha ntoni?

Ilahleko sombala iinwele ngohlobo legqwetha curl emhlophe phezu kwentloko kunokwenzeka ukususela ekuzalweni ukuya ebudaleni.

Phuthuma kukho lunyhasho sombala ukutya, kokukhona kakhulu iziphumo unobangela onzima.

ukoma kombala Asihlali edityaniswe inxalenye kombala kwamehlo akho. Ehamba ukubonakala nakujoliso olutsha lwe hyperpigmentation, t. E. Gnozdnogo mnyama kwesikhumba kumphezulu phambi esiswini, namathanga ebronzi, imilenze, isifuba, iingalo imilinganiselo ukususela 1 mm ukuya 6 cm.

Isifo yoko pebaldizmom

Kukho iqela elikhulu izifo kunye mgaqweni zophuhliso, edibanisa kunye mhlophe iinwele amalungu umonakalo omkhulu kunye neenkqubo. ngezifo akubonakali kuwugcina umphefumlo womntwana kwaye abazali ukuba ahambise engxakini.

Waardenburg-Klein syndrome

Esi sifo i autosomal indlela olongamileyo lilifa, okt. E. I esifanayo badla eyenzeka amakhwenkwe kunye namantombazana. I siphene lwemfuzo kungaba njengelifa nokuba umzali.

Ukongeza sokukhanyisa of imicu neenwele ibonwa bebona jikelele kunye ocular esi sifo.

iimpawu ocular yale sifo:

• hypoplasia le ngeenkophe;
• iindawo ingwevu iintshiyi ongaphakathi;
• noqhoqhoqho amehlo elimxinwa;
• amehlo anemibala eyahlukeneyo;
• hyperopia;
• ngokubanzi zishiyane amehlo.

iimpawu ezifanayo kusenokuba izidanga sandla ezahlukeneyo:

• albino ubuso;
• ibhulorho ebanzi ngeempumlo;
• omfutshane ngesithomo;
• mgaqweni amazinyo;
• isithulu-mutism okanye ilahleko ezindlebeni ekwanti;
• mgaqweni amalungu zangaphakathi;
• utshintsha ubungakanani Amaza.

Ngenxa yokuba ekuqaleni yengxinano sifo yaye kwenzeka pebaldizmom ubuncinane kuphela kufuneka kujongisiswa ecaleni ngezifo kakhulu. Nantso into piebaldism.

Wolfe engqondweni

Asihlali heterochromia, t. E. A iliso eyahlukileyo umbala ikakhulu lifumaneka kwizilwanyana. Xa abantu, rhoqo yonke labantu yi-1%.

Famous abantu umbala ngeliso ezahlukeneyo: Mila Kunis ( "Oz The Great Nonamandla", "ahlobene", "Jupiter ukwenyuka"), ongumdlali waseBritani Dzheyn Seymur, eyona nto idume ngayo lo mboniso "Live Let Die", Devid Boui - rock British imvumi, umbhali, umvelisi, injineli ephilileyo, igcisa, weqonga.

heterochrony Asihlali sisifo, kwaye zidityaniswe kunye syndrome pebaldizmom ekuthiwa Wolfe, kwaye bukhokelela ukungeva nkqi sandayo.

Ukuze ukohlula esi sifo kufuneka abe retinitis pigmentosa - epithelium muscular amehlo. Amaxesha amaninzi uqobo ekuhambeni kwalo sifo - retinitis pigmentosa ngaphandle ebaleni.

Umama kunye nentombi kunye syndrome of "pebaldizm"

Esi sifo ilifa sofuzo, enxulumene chromosome-yabafazi. Umfazi eyipasile sofuzo unyana wakhe, yena abe sisifo. Zintombi kula madoda zisempilweni, ukuba abe nomama esempilweni. Bekuya kuba imbonakaliso kuphela pebaldizm.

Ukuba unina uphilile na ngokwezonyango, kodwa inaso esi sofuzo, umntwana lowo uya kuba uyagula.

Ukufumanisa isifo usebenzisa ophthalmoscopy, nto leyo ebonisa iindawo fundus nombala ngathi edliwe ngamanundu.

Gipomelanoz incito

Esi sifo sibangelwa kwasekuzalweni. Bayekeni abantwana ngokwesini zombini rhoqo ngokulinganayo. Not ukuzabalaza transmission ilifa kubazali kwinzala, kodwa ke esi sifo isoloko kudityaniswe bekhubazekile amalungu zangaphakathi.

Uxilongo senziwa ngokwezonyango ngokwesiseko ezi mpawu zilandelayo:

• ivele kwi eluswini izabelo amelanotic engaqhelekanga e curls fomu, iqhwa;
• Kwi palmar, imiphezulu plantar kunye imifinya yangaphakathi olo tshintsho azenzeki;
• kummandla nezenyama station iinwele eyome - pebaldizm;
• iindawo ubudala lonke ibala eyahluklleyo kangako;
• umonakalo luvo belisoloko wabonakalaliswa ukukhubazeka engqondweni, ukuphazamiseka convulsive, sinciphise kwezihlunu;
• ngezifo iso ibonakale ezingafani sandla strabismus;
• zokufota wahamba, isifuba deformation iziphelo ngo-25% yamatyala;
• nge frequency ka-10% kudityaniswe abaneziphene ngentliziyo.

Ukuze ukohlula esi sifo kwi imeko eziyimfuneko ekuthiwa ebala womchamo. Bona uneempawu ezifana lwezonyango, kodwa kutshanje ilifa.

sombala ngumchamo

Xana a syndrome enqabileyo isasazeka "pebaldizm"?

Esi sifo sisasazwa ukusuka kumama kwintombi. Kuxhomekeka ebukhulwini yomfanekiso lonyango Ubonakala mpawu zilandelayo:

• Irhashalala ngohlobo imicwe elide, emva omiswe kunye nokususwa soma ushiye izabelo nombala. Mlilo ziye ikhosi lihlangana kunye ukubonakala ngamaxesha iiseli ezintsha ulusu. Ukusuka kwezi ziza ekukhuleni komntwana kuphulukana kombala uze ube luthuthu.
• Dysplasia nezinyo uqwalaselo, izikhonkwane, ibala iinwele njengoko alopecia zibe ziinkqayi.
• zizigulo zemithambo kubonakaliswa uphuhliso ezilibazisekileyo ngokwasengqondweni-ntetho, ukwanda koxinzelelo intracranial, ukuxhuzula, ubuthathaka iingalo kunye nemilenze.
• ukuphazamiseka ngeso iyahlozinga kwaye zibonakale strabismus, nystagmus, cataract, kwamehlo, ukungaboni.

Gristselli syndrome

Eli liqela izifo ezinokwenzeka ngeendlela ezintathu, lo ukufana iphambili ubukho namabala olumbhatshileyo iinwele zentloko yakhe. Ezi iziboniso zilandelayo ziyafumaneka:

• zizigulo zemithambo nge hypopigmentation.
• Uhlobo ingozi sifo apho "pebaldizm" syndrome kunye hematopoiesis kakuhle. obubonakala Clinical: ngcathu fever, nobani kunye nonyango. ngcembe ngamandla fever kwandisa, uzibandakanya isibindi banzi, i udakada, ukuncitshiswa erythrocytes, leukocytes, iiplatelets, lwefibrinogen egazini. Le yokugqibela inxaxheba kwiinkqubo ze-luvo.
• hypopigmentation esikhumba neenwele sandla ezahlukahlukeneyo, nto leyo kude koko ngaphandle intshabalalo weeseli zegazi kunye luvo. Kuba uxilongo kwangethuba esi sifo ezinobungozi kufuneka ukuba bajonge wesikhumba segazi, imizila yemfuza. Qiniseka ukuba bakuthathele ingqalelo ubukho losapho zezigulo ezahlukeneyo mbala ukutya, uhlalutyo ukwahlula zofuzo.

Wokuphuthuma nobungozi sisifo ngenxa rhoqo edibanisa kunye Down syndrome, Edwards, Turner.

Chonga lonke ibala esikhumba neenwele unokuba ekuzalweni, kodwa ukumisa ubukho Turner engqondweni kunokuba abe ngumntu phezulu ikhula kuphela xa kumakishwa omfutshane ngesithomo, lymphostasis, isifo sentliziyo, uxinzelelo oluphezulu lwegazi, namadlala ngokwaneleyo mammary, isikhundla sezantsi kwesikhundla ingono, endaweni ngamalungu angaphakathi obuni nokuqina nendwangu. Intelligence kwezi zigulane phantse awehlelwa, imvelaphi isimo phantse lonke eziphakamileyo, kukho abathile imincili HIV, nto leyo echaza lithathelwe elungileyo zentlalo kula izigulane.

isiphelo

Xa abantu, isakhi of nombala okuqhelekileyo ulusu usoloko ku lawula ngokunxulumene gene onoxanduva nokuhluthwa mbala ukutya.

Intsingiselo ezisisiseko iingxaki kunye nombala kukuba markers eziyingozi isifo chromosomal, malformations ezininzi bekhubazekile.

Nayiphi na impatho ukutya yesikhumba sakhi, inwebu yangaphakathi, iinwele kufuneka bajonge yincutshe. Siye sahlolisisa "pebaldizm" syndrome. Yintoni na, kwacaca.