Izimpawu Nezokwelapha ze-SBMA
Isifo se-Kennedy, esaziwa nangokuthi u-spinal bulbar muscular atrophy noma i-SBMA, isifo esiyilungu lezinzwa. Isifo se-Kennedy sithinta amangqamuzana akhethekile e-nerve alawula ukunyakaza kwe-muscle (ikakhulukazi, ama-neurons aphansi), okubangela ukuhamba kwemisipha eminingi yezingalo nemilenze. Kuphinde kuthinte izinzwa ezilawula imisipha ye-bulbar, elawula ukuphefumula, ukugwinya nokukhuluma.
I-Kennedy isifo singabangela futhi i-androgen (i-hormone yamadoda) ukungabi namuntu okwehliswayo okubangela amabele akhulisiwe emadodeni, ukunciphisa ukuzala, kanye ne-atrophy ye-testicular.
Isifo se-Kennedy sibangelwa ukukhubazeka kwezakhi zofuzo kwi-X (isifazane) i-chromosome. Njengoba abantu besilisa bane-X kuphela ye-chromosome, banesifo esibucayi kakhulu. Amantombazane, anama-chromosome amabili amabili, angathatha isisindo esingenasici kwenye i-chromosome eyodwa ye-X, kodwa enye i-chromosome evamile ye-X inciphisa noma ifihle izimpawu zesifo. Isifo se-Kennedy silinganiselwa ukuthi senzeke kubantu abangu-1 ku-40,000 emhlabeni jikelele.
Izimpawu ze-Kennedy Disease
Ngokwesilinganiso, izimpawu ziqala kubantu abaneminyaka engama-40 kuya kwengu-60. Izimpawu zihamba kancane, futhi zingabandakanya:
- Ubuthakathaka kanye nama-muscle cramps ezandleni nasemilenzeni
- Ubuthakathaka bomzimba, ubuso bomlomo nolimi. Isikhumba singase siphume noma sigwedeze, futhi izwi lingase libe yinto engcono kakhulu.
- Ukuqothula kwemisipha encane engabonakala ngaphansi kwesikhumba.
- Ukuthuka noma ukuthuthumela ngezikhundla ezithile. Izandla zingathuthumela uma zizama ukuthatha noma ukubamba okuthile.
- Ubumbulu noma ukulahleka kwenzwa phezu kwezinye izindawo zomzimba.
Isifo se-Kennedy singaba neminye imiphumela emzimbeni, kufaka phakathi:
- I-Gynecomastia , okusho ukukhuliswa kwezicubu zesifuba kubesilisa
- I-Testicular Atrophy, lapho izitho zomzimba zesilisa zinciphisa ngesayizi futhi zilahlekelwa umsebenzi.
Ukuhlonza Isimo
Kunezinkinga eziningi ze-neuromuscular ezinempawu ezifana nesifo se-Kennedy, ngakho-ke ukuhlukunyezwa noma ukuhlolwa kwengqondo kungase kube okuvamile.
Ngokuvamile, abantu abanesifo se-Kennedy bacatshangwa ngephutha ukuthi bane- amyotrophic lateral sclerosis (i-ALS, noma isifo sikaLou Gehrig). Kodwa-ke, i-ALS, kanye nezinye izifo ezifanayo, azibandakanyi ukuphazamiseka kwe-endocrine noma ukulahlekelwa kwenzwa.
Ukuhlolwa kofuzo kungase kuqinisekiswe ukuthi i-Kennedy disease defect ikhona kwi-X chromosome. Uma ukuhlolwa kofuzo kulungile, akukho ezinye izivivinyo okudingeka zenziwe uma ukuxilongwa kungenziwa kusuka ekuhlolweni kofuzo kuphela.
Ukwelashwa kwe-SBMA
Isifo se-Kennedy noma i-SBMA asithinti isikhathi sokuphila, ngakho-ke ukwelashwa kugxile ekulondolozeni umsebenzi womzimba womuntu wonke kulo lonke impilo yakhe ngokusebenzisa ezinye izinhlobo zokwelashwa ezilandelayo:
- Ukwelapha ngokomzimba
- Ukwelapha emsebenzini
- Ukwelashwa kwenkulumo
Lezi zinhlobo zokwelapha zibalulekile ekugcineni amakhono omuntu kanye nokuzivumelanisa nokuqhubeka kwesifo. Imishini ephathekayo efana nokusetshenziswa kwamagundane noma izihlalo zabakhubazekile ngamandla angasiza ukugcina ukuhamba nokuzimela.
Ukululekwa nge-Genetic
Isifo se-Kennedy sihlotshaniswa ne-X (esifazane) ye-chromosome, ngakho-ke uma owesifazane ethwala isakhi esingenasici, amadodana akhe anethuba elingamaphesenti angama-50 anesifo esiyinkinga kanye namadodakazi akhe anethuba elingama-50% lokuba ngumthwali.
Abababa abakwazi ukudlulisela isifo seKennedy emadodaneni abo. Amadodakazi abo, kodwa, azoba yiziphathamandla zezakhi zofuzo.
Imithombo:
I-Barkhaus, i-PE (2003). Isifo se-Kennedy.
I-Kennedy's Disease Association. "Iyini Izifo ZakaKennnedy?"