Yintoni isifo sika-Alport, iimpawu kunye nendlela yokunyanga
Umxholo
Isifo iAlport syndrome sisifo esinqabileyo semfuza esibangela ukonakala okuqhubekayo kwimithambo yegazi encinci ekwiglomeruli yezintso, ukuthintela ilungu ukuba lingakwazi ukucoca igazi ngokuchanekileyo kwaye libonakalise iimpawu ezinjengegazi kumchamo kunye nokwanda kwenani leprotheni. kuvavanyo lwegazi.
Ukongeza kokuchaphazela izintso, le syndrome nayo inokubangela iingxaki ekuveni okanye ekuboneni, njengoko ithintela ukuveliswa kweprotein ebalulekileyo ekusebenzeni kwamehlo neendlebe.
Isifo i-Alport's syndrome asinalo unyango, kodwa unyango luyanceda ekunciphiseni iimpawu kunye nokulibazisa ukukhula kwesi sifo, ukuthintela ukusebenza kwezintso ekuchaphazelekeni.
Iimpawu eziphambili
Iimpawu eziqhelekileyo zesifo sika-Alport zibandakanya:
- Igazi kumchamo;
- Igazi elonyukayo;
- Ukudumba kwemilenze, amaqatha, iinyawo nobuso.
Ukongeza, kukho iimeko apho ukuva nokubona kuchaphazeleka sesi sifo, kubangela ubunzima bokuva nokubona.
Ukuba asiwathathi amanyathelo okhuseleko, esi sifo sinokuqhubela phambili ukusilela kwezintso okungapheliyo kwaye sidinga ukufakwa kwidayisi okanye ukufakelwa kwezintso
Yintoni ebangela isifo
Isifo iAlport's syndrome sibangelwa lutshintsho kwimfuza oluqulathe imiyalelo yokuveliswa kweprotein eyaziwa ngokuba luhlobo IV collagen. Olu hlobo lwe-collagen luyinxalenye ye-glomeruli yezintso kwaye, ke, xa ingekho, imithambo yegazi kule mimandla iyonzakala kwaye iyaphilisa, iphazamisa ukusebenza kwezintso.
Ngokunjalo, le collagen ikwakhona ezindlebeni nasemehlweni kwaye, ke, utshintsho kula malungu lunokuvela ngokuhamba kwexesha.
Indlela yokuqinisekisa ukuxilongwa
Akukho luvavanyo luchongiweyo lokufumanisa isifo sika-Alport, ke ugqirha unokuyalela iimvavanyo ezininzi, ezinje ngovavanyo lomchamo, uvavanyo lwegazi okanye i-biopsy yezintso ukubona ukuba lukhona na utshintsho olunokubangela isifo.
Unyango lwenziwa njani
Unyango lwe-Alport's syndrome lwenziwa ngenjongo yokunciphisa iimpawu, kuba akukho ndlela ithile yonyango. Yiyo loo nto kuyinto eqhelekileyo ukusebenzisa amayeza kuxinzelelo lwegazi kunye nokuchama, ukuze uxinzelelo lwegazi kunye nokuthintela ukwanda kokulimala kwezintso.
Ukongeza, kuyacetyiswa ukuba kugcinwe ukutya okunetyuwa encinci ukuthintela ukusebenza kwezintso okugqithisileyo. Nantsi indlela yokugcina ukutya kolu hlobo.
Kwiimeko ezinzima kakhulu, apho izintso zichaphazeleke kakhulu kwaye akukho kuphucuka kwiimpawu, kunokuba yimfuneko ukuqala nge-dialysis okanye ukufakelwa kwezintso.