IWardenarden Syndrome ihlelwe yimpawu kwiintlobo ezine
I-Waardenburg syndrome yintlupheko yezofuzo ezibonakaliswa yimimandla eqhelekileyo ye- albinism yolusu kunye neenwele. Olunye uhlobo lwe-Waardenburg syndrome luvelisa nesisithulu sentsholongwane (izithulu ekuzalweni). Le ngxaki ikholelwa ukuba iya kuba yi-2-3% yazo zonke iimeko zokuzalwa zesithulu.
I-Waardenburg syndrome ichaphazela bonke abesilisa nabesifazane kunye nabantu bazo zonke iintlanga.
Kuqikelelwa ukuba kwenzeka kwi-1 yabantu abangama-42,000.
Iimpawu kunye neNdidi
I-Waardenburg syndrome iyahlula zibe ziindidi, ngokusekelwe kwiimpawu. Abantu ngabanye bayabonwa ngokuba ne- Waardenburg syndrome hlobo 1 ukuba banayo i-2 enkulu okanye 1 enkulu kunye ne-2 imigaqo engundoqo kwizintlu ezingezantsi.
Imigaqo ephakamileyo:
- Ukuzalwa isisithulu okanye ukuva ukuva ( ukulahlekelwa kwintlungu yokuzalwa kwengqondo) (58% yabantu)
- Amehlo enqabileyo yesafire ebomvu okanye amehlo ahlukeneyo kumbala omhlophe
- Ukutshilwa kweenwele ezimhlophe ebunzini
- Ilungu lentsapho elikhawuleza elinamayeza eWardenburg
- Ingxenyeni yangaphakathi yelihlo elahlekile kwicala (i-dystopia canthorum)
Imilinganiselo encinci:
- Iipatches zokukhanya okanye isikhumba esimhlophe
- Amacwecwe aphakama phakathi kobuso
- Ukungaqhelekanga kwamanzi
- Ukulungiswa kwangaphambili kweenwele (ngo-30 ubudala)
I-Waardenburg syndrome hlobo 2 ichazwa ngokuba nayo yonke impawu yohlobo 1 ngaphandle kwedystopia canthorum. I-77% yabantu abanohlobo lwe-2 banokulahlekelwa kwindlebe.
Uhlobo lwe-Waardenburg syndrome hlobo 3 , okanye i-Klein-Waardenburg syndrome, lufana nohlobo lwe-1 kodwa lubandakanya izivumelwano ze-muscle.
Uhlobo lwe-Waardenburg syndrome uhlobo lwe-4 , okanye i-Waardenburg-Shah syndrome, lufana nohlobo lwe-1 kodwa lubandakanya isifo seHirschsprung (i-malformation).
Ukuxilongwa
Iintsana ezizalwe nge-Waardenburg syndrome zinokuba neenwele kunye nolusu oluguquka kunye nokulahlekelwa kwindlebe. Nangona kunjalo, ukuba iimpawu zibuhlungu, i-Waardenburg syndrome inokuthi ingabonakaliswa ukuba ilungu lentsapho lifumaneke kwaye zonke iintsapho ziyahlolwa.
Iimvavanyo zokuvakala eziqhelekileyo zingasetyenziswa ukufumanisa ukulahlekelwa kwindlebe.
Unyango
Izimpawu ezahlukeneyo ze-Waardenburg syndrome zivela kubantu abahlukeneyo, kwintsapho efanayo. Abanye abantu abayi kufuna unyango ngelixa abanye badinga ukuhlinzwa okanye iso okanye ezinye izinto ezingaqhelekanga. Akukho zondlo ezikhethekileyo okanye imingcele yemisebenzi iyadingeka, kwaye i-Waardenburg syndrome ayisoloko isichaphazela ingqondo.
Ukululekwa ngeGenesis
Ngenxa yendlela indlela i-Waardenburg syndrome ezuze ngayo (i-autosomal ephezulu) kwiintlobo 1 no-2, umntu ochaphazelekayo unethuba elingama-50% ekukhulelweni ngamnye komntwana ochaphazelekayo. Ekubeni iimpawu ziyahluka, akukho ndlela yokuchaza ukuba umntwana ochaphazelekayo uya kuba neempawu ezinzima okanye ezinzima kunomzali wakhe. Ifa leentlobo ezi-3 kunye ne-4 ziyinkimbinkimbi, kodwa ukululekwa ngezofuzo kunokunceda ukuhlola ingozi yokudlula i-Waardenburg syndrome kumntwana.
> Imithombo:
> Bason, L. (2002). IWardenarden syndrome. eMedicine.
> Dourmishev AL, Dourmishev LA, Schwartz RA, et al. IWardenarden syndrome. Int J Dermatol 1999; 38: 656.
Ehlelwe nguRichard N. Fogoros, MD