Syndrome "pebaldizm": ukuthi kuyini, kubangela, izimpawu, ukuxilongwa kanye nokwelashwa

Omunye ezihlukahlukene sombala umzimba iziyaluyalu - ezingavamile syndrome "pebaldizm". Gnozdnym libhekene ichaza ukushintsha kombala ikhanda lakhe nezinwele zakhe. Lokhu akulona iphutha yezimonyo ongenangozi, kodwa uphawu inani elikhulu izifo ezingathi sína kwababhemayo.

Kulesi sihloko sibheka ngokuningiliziwe "pebaldizm". Izimbangela, izimpawu, uphethwe, ukuphathwa, uyophinde kuchazwe.

Izimbangela izinwele bleach

Ukwephula sombala umzimba kuyinto depigmentation - sombala ukulahlekelwa, hyperpigmentation - okhulisa staining.

Izinwele umbala sombala zinenani 2 - eumelinin, pheomelanin. Ziyakwazi cortex izinwele. Eumelanin kwenza izinwele ezimnyama nsundu pheomelanin - ophuzi-obomvu. Inhlanganisela yalawo zombala kunombolo ngabanye kanye nokuxhumana emoyeni unikeza izinwele umbala ethile. Umbala izinwele is zofuzo ihlelwa.

syndrome "pebaldizm" Kukangaki zenzeka, Siyini ngempela lesi sifo?

Ephula melanocytes, izinwele sombala-Kuhlanganisa, graying ngeminyaka eba. Uma isimo siqala esemncane - lokhu kuwubufakazi isifo esidluliselwa ngofuzo, noma izifo ezingathi sína kwababhemayo.

Izifo lapho ehlupheka pigmentary lokushintshisana

Kungani kunesidingo "pebaldizm" syndrome, siyini lesi ukuphambuka?

Lithi elandelayo bayatfutfuka ephula melanocytes:

• pebaldizm ngefomu angawodwa;
• albino;
• pebaldizm kuhlangene isifo samehlo, indlebe, isikhumba, isimiso sezinzwa, uhlelo zemisipha.

emitholampilo isithombe

Kanjani ukuthi kuziveza "pebaldizm" syndrome, kusho ukuthini?

Ukulahlekelwa izinwele sombala ngesimo ukubukeka zinyakaze ezimhlophe ekhanda singenzeka kusukela ekuzalweni ebudaleni.

Ukushesha kukhona ukwephulwa sombala umzimba, i-lubucayi kakhudlwana nemiphumela imbangela esinzima ngokwengeziwe.

Gqwa ukushintsha kombala zinyakaze kuhlanganiswe ingxenye ukushintsha kombala phakathi kwamehlo akho. Ehamba ukubukeka foci of hyperpigmentation, t. E. Gnozdnogo ukuhwalala kwesikhumba phezu phambi esiswini, namathanga, imilenze, esifubeni, izingalo Ubukhulu kusuka 1 mm kuya ku-6 cm.

Izifo yokuquba ngemuva pebaldizmom

Kukhona iqembu elikhulu izifo kanye anomalies yentuthuko, ngokuhlanganisa kanye amakhorali izinwele sina umonakalo kwezitho izinhlelo. zokugula akubonakali lizovikela ukuphila kwengane nabazali ukuletha asemrarweni.

Waardenburg-Klein syndrome

Lesi sifo kuyinto autosomal iphethini evelele ifa, isb. E. efanayo kaningi kwenzeka abafana namantombazane. Isici zofuzo kungenziwa njengefa kusukela noma umzali.

Ngaphezu bleaching izinwele imicu zenzo ziwukubonakaliswa waphawula jikelele kanye ocular lesi sifo.

izimpawu Ocular yalesi sifo:

• hypoplasia we eyelashes;
• izindawo empunga amashiya kwangaphakathi;
• ewumngcingo iso umqhewu;
• amehlo anemibala ehlukene;
• hyperopia;
• esilinganayo kabanzi amehlo.

izimpawu ezivamile kungenzeka lamadigri kobukhulu obuhlukahlukene:

• albino esinqunyiwe;
• ezibanzi ibhuloho acinene;
• onesiqu esifushane;
• anomalies amazinyo;
• abayizithulu-mutism noma ezicezile ubuthulu;
• anomalies kwezitho zangaphakathi;
• ushintshe usayizi Yekhanda.

Njengoba sazi ukuthi ekuqaleni kobukhulu isifo futhi kwenzeka pebaldizmom ubuncane kuphela kudingeka ukuba bahlolwe ukubekela eceleni zokugula sina. Yilokho piebaldism.

Wolfe Syndrome

Gqwa heterochromia, t. E. Iso ezahlukene umbala ikakhulu etholakala ezilwaneni. Kubantu, imvamisa phakathi lonke labantu 1%.

Famous abantu ngamehlo ezahlukene umbala: Mila Kunis ( "Oz Omkhulu futhi Onamandla", "Bloodlines", "i-Jupiter Ukwenyuka"), actress saseBrithani uJane Seymour, waziwa kakhulu ifilimu "Live Ake Die", David Bowie - rock British umculi, wengoma, umkhiqizi, unjiniyela umsindo, umculi, umlingisi.

heterochrony gqwa kuyisifo, futhi lihambisana pebaldizmom syndrome ngokuthi Wolfe, futhi kuholela ubuthulu eyanda ngokushesha kangaka.

Ukuze uhlukanise lesi sifo kufanele kube ne retinitis pigmentosa - sombala epithelium sokuwohloka iso. Ngokuvamile samuntu Yiqiniso yalesi sifo - retinitis pigmentosa ngaphandle sombala.

Umama nendodakazi sifo of "pebaldizm"

Lesi sifo ifa esikhubazayo, abesifazane-chromosome-exhumene sika. A intombi idlula isakhi sofuzo indodana yakhe, futhi unesifo. Nina madodakazi akwa-la madoda enempilo, uma ube umama ophile kahle. Kungaba kuphela ukubonakaliswa pebaldizm.

Uma umama okunempilo emtholampilo, kodwa othwala isakhi sofuzo, umntwana kuyoba ogulayo.

Ukuxilonga izifo usebenzisa ophthalmoscopy, okuyinto ikhomba izindawo fundus lesikhumba like zidliwe inundu.

Gipomelanoz Ito

Lesi sifo sibangelwa lokuzalwa. Vumelani abantwana besilisa nabesifazane nge imvamisa alinganayo. Hhayi efakazelwe ukudluliswa lezinceku kubazali enzalweni, kodwa lesi sifo ngaso sonke isikhathi kuhlanganiswe ukukhubazeka azalwa naso we izitho zangaphakathi.

Imiphumela yaloku kuhlola okungenhla senziwa emtholampilo ngesisekelo zimpawu ezilandelayo:

• ivele isikhumba izingxenye amelanotic yinqaba ngefomu curls, amakhekheba eqhwa;
• On the Palmar, evele plantar kanye nolwelwesi lwamafinyila ushintsho olunjalo azenzeki;
• esifundeni temporal of bleached izinwele esiteshini - pebaldizm;
• yobudala depigmentation izindawo ziphelelwa ebonakalayo;
• ukulimala isimiso sezinzwa ubelokhu wabonakaliswa retardation engqondo, iziyaluyalu convulsive, kwehle imisipha ithoni;
• zokugula esweni strabismus manifest lamadigri kobukhulu obuhlukahlukene;
• ukugobeka komhlandla ephelezelwa, esifubeni ukusonteka yesiqephu e-25% amacala;
• nge imvamisa 10% kuhlanganiswe ukukhubazeka inhliziyo.

Ukuze uhlukanise lesi sifo kusukela isimo ezidingekayo ngokuthi sombala urinary. Sinenkosi izimpawu zokwelashwa ezifanayo, kodwa muva nje ngofuzo.

sombala incontinence

Ingabe a syndrome ezingavamile idluliselwa "pebaldizm"?

Lesi sifo idluliselwa kusuka kumama kuya indodakazi. Kuye kobukhulu isithombe emtholampilo livezwa zimpawu ezilandelayo:

• Ukuqubuka kwesikhumba ngesimo emapheshana eside okuyinto, emva omiswe nokususwa soma ushiye izingxenye lesikhumba. Izintaba-mlilo ziqhume kufanele inkambo inamagquma nge ukubukeka gqwa amangqamuzana amasha isikhumba. Kulezi iziza nokuvuthwa ingane ulahlekelwe sombala futhi iyaphaphatheka.
• Dysplasia izinyo ukumisa, izinzipho, nezinwele depigmentation njengoba Alopecia nobumpandla.
• iziyaluyalu kwemizwa ziyabonakala imisiwe ukuthuthukiswa psycho-enkulumweni, waqinisa intracranial, ukuquleka, ubuthaka ezingalweni kanye nasemilenzeni.
• Eye iziyaluyalu kukhona strabismus engazinzile nokuzivelelayo, nystagmus, ale nkinga, umbono encishisiwe, ubumpumputhe.

Gristselli syndrome

Lena leqembu lezifo ezingaba zenzeka amafomu ezintathu, ukufana eyinhloko khona nezimagqabhagqabha ezishintshe umbala izinwele ekhanda lakhe. I ekhombisa alandelayo ayatholakala:

• iziyaluyalu kwemizwa ne hypopigmentation.
• Uhlobo oluvame kakhulu isifo lapho "pebaldizm" syndrome kuhambisana hematopoiesis kahle. ukubonakaliswa Clinical: imfiva ngokulinganisela, Paranormal kanye nokwelashwa. Ukugxila imfiva kancane kancane kwandisa, ijoyina isibindi lakhulisiwe, ubende, ukuncishiswa erythrocyte, leukocyte, izinhlayiya zegazi fibrinogen egazini. Lesi sakamuva ehilelekile izinqubo ezithile zesimiso sezinzwa.
• hypopigmentation kwesikhumba futhi izinwele kobukhulu obuhlukahlukene, okuyinto kuze kube yileso sikhathi wabe ngaphandle ukubhujiswa egazi kanye nesimiso sezinzwa. Ukuze kuhlonzwe esifike ngesikhathi lesi sifo esiyingozi kufanele uthintane wesikhumba eyisazi, izakhi zofuzo. Qiniseka acabangele khona zomkhaya kwempilo sombala umzimba, ukuhlaziya ukubekela eceleni zofuzo.

Ukuphuthuma ingozi yesifo ngenxa high imvamisa ngokuhlanganisa ke abane-Down syndrome, Edwards, Turner.

Thola depigmentation ka lishonile kunesikhumba futhi uboya kungaba ngesikhathi sokuzalwa, kodwa ukusungula khona Turner Syndrome kungaba njengoba umuntu ekhula kuphela uma imakwe onesiqu esifushane, lymphostasis, isifo senhliziyo, umfutho wegazi ophakeme, ampofu nezindlala mammary, isikhundla esiphansi ingono, esikhundleni yezitho zangaphakathi zangasese zezinzwa indwangu. Intelligence kulezi iziguli cishe akusho ahlupheke, isimo ngemuva cishe njalo iyokhushulwa, kukhona ezinye kwesasa omuhle, echaza lithathelwe ezinhle zomphakathi salezi ziguli.

isiphetho

Kubantu, isakhi sofuzo of lesikhumba evamile kwesikhumba uhlale evelele maqondana isakhi sofuzo esiyimbangela yalesi ukuhlukumeza sombala umzimba.

Mayelana nencazelo eyisisekelo yegama izinkinga nge lesikhumba siwukuthi bangamalungu nakomaka eziyingozi yesifo chromosomal, kombungu esizalweni amaningi.

Noma ikuphi ukwephulwa umzimba isikhumba lesi sakhi, nolwelwesi lwamafinyila, izinwele kufanele uthintane onguchwepheshe. Sesihlole "pebaldizm" syndrome. Kuyini, kwaba sobala.