Kuza kwenzeka ntoni ukuba amahlwili egazi ayenzeka ngaphakathi kwemithambo yegazi emincinci kwizintso zakho, ethintela ukuhamba kwegazi ukuya kwizintso zakho? Kuyoyikisa ukucinga ngako, akunjalo? Yiloo nto kanye esithetha ngayo namhlanje. Oku kubizwa ngokuba yi-Atypical Hemolytic Uremic Syndrome, okanye i-aHUS ngamafutshane. Nangona ingenakunyangwa, inokulawulwa kakuhle ngonyango olufanelekileyo . Ngoko masifunde ngakumbi ngayo.
Yintoni umahluko phakathi kwe-HUS engaqhelekanga kunye ne-HUS eqhelekileyo?
Usenokuba ukhe weva ngemeko ebizwa ngokuba yi-HUS. Siyibiza ngokuba yi-"HUS eqhelekileyo." Idla ngokubangelwa lusulelo lwebhaktheriya olufana ne-E. coli, olubangela urhudo. Kodwa imeko esithetha ngayo namhlanje, i-aHUS, yahlukile.
Le meko, ebizwa ngokuba yi-aHUS, inokwenzeka nakweyiphi na iminyaka. Kubantwana abancinci, ichaphazela abafana namantombazana ngokulinganayo. Nangona kunjalo, njengoko bekhula, abafazi banamathuba amaninzi okufumana esi sifo. Oku kungenxa yokuba ukukhulelwa kunokuba sisizathu esikhulu sesifo. Akuqhelekanga kangangokuba ezinye izifundo zibonisa ukuba ngabantu ababini kuphela kwisigidi abanesifo.
Kutheni i-aHUS iphuhla?
Ngamafutshane, abantu abaninzi abaphuhlisa i-aHUS banotshintsho lwemfuza, okanye utshintsho lwemfuza, emizimbeni yabo. Cinga ngayo ngokungathi kukho 'iqhosha' emzimbeni wakho elinokubangela esi sifo. Kodwa ukuba neqhosha elivuliweyo akuzibangeli isifo ngokuzenzekelayo. Lifuna into engaphandle kwalo ukuze 'livulwe', okanye lisebenze. Ezi sizibiza ngokuba 'zii-triggers'.
Izinto eziphambili ezibangela oku:
- Ukukhulelwa
- Iimeko ezithile ezisulelayo
- Umhlaza
- Amanye amayeza
- Ukufakelwa amalungu omzimba
- Ezinye izifo ezihlala ixesha elide (ezifana ne-systemic sclerosis okanye i-malignant hypertension)
Okumangalisayo kukuba, malunga nesiqingatha sezigulane ze-aHUS, oogqirha abanayo inguqu yemfuza abanokuyifumana. Sibiza iimeko ezinjalo ngokuthi "yi-idiopathic," oko kuthetha ukuba unobangela awaziwa. Abaphandi bakholelwa ukuba le meko ibangelwa yinguqu yemfuza engekaziwa.
Zithini iimpawu?
Iimpawu ze-aHUS zihlala zibonakala ngequbuliso emva kokuba kwenzeke into ebangela ukuba kubekho isifo. Ukudumba kunokuba ncinci okanye kube nzima. Kwiimeko ezincinci, izintso zisenokungachaphazeleki kakhulu, kodwa kwiimeko ezinzima, zinokubangela ukuba izintso zingaphumeleli .
Iimpawu onokuzibona kwizigaba zokuqala:
- Iintlungu zomzimba kunye nokudinwa kakhulu
- Ukuziva ungaphili kakuhle
Esi sisifo esiqhubekayo. Oku kuthetha ukuba iimpawu ziya zisiba mandundu ngokuhamba kwexesha, ingakumbi ukuba azifunyaniswanga kwangethuba. I-aHUS ichaphazela kakhulu izintso zakho. Nangona kunjalo, ezinye izitho zomzimba, ezifana nengqondo, isibindi, imiphunga kunye nentliziyo, nazo zinokuchaphazeleka kukuvaleka kwemithambo yegazi.
| Amanqaku aphambili ekufuneka aqwalaselwe xa kuchongwa isifo | Uvavanyo kunye noko bakufunayo |
|---|---|
| Iimpawu ezi-3 ezifunwa ngugqirha | 1. I-Hemolytic anemia: Iiseli ezibomvu zegazi zitshatyalaliswa ngokukhawuleza kunokuba ziveliswa. 2. Ukuncipha kweeplatelets: Ukuncipha kwenani leeseli ezinceda ukujiya kwegazi. 3. Iingxaki zezintso: Ukungasebenzi kakuhle kwezintso. |
| Uvavanyo |
|
Indlela yokuxilonga esi sifo?
Ukuba akukho mntu kusapho lwakho owakha waba nesi sifo ngaphambili, kunokuba nzima kancinci ukufumanisa i-aHUS. Ngokwesiqhelo, ingcali ye-nephrologist okanye i-hematologist yeyona mntu ufanelekileyo ukwazi ngale nto. Baza kunika ingqalelo ekhethekileyo kwizinto ezikhankanyiweyo kwitheyibhile engentla baze bakuthumelele uvavanyo olufunekayo.
Ziziphi iindlela zonyango?
Ngethamsanqa, kukho amayeza amabini avunyiweyo e-aHUS.
- `I-Eculizumab (iSoliris)`
- `Ravulizumab-cwcz (Ultomiris)`
Zombini ezi zinto ziphantsi kodidi lwamayeza abizwa ngokuba yi-'monoclonal antibodies.' Ngamafutshane, ezi ziiproteni ezenziwe ngokwenziwa ezisebenza njenge-antibodies zendalo zomzimba wethu. Zilawula inxalenye yenkqubo yomzimba yokuzikhusela kwaye ziyeke ukujiya kwegazi.
Iyeza elibizwa ngokuba yi-``Eculizumab`` linokunyusa inani lee-platelet kunye neeseli ezibomvu zegazi egazini lakho. Ukuba unyango luqalwa ngokukhawuleza , eli yeza linokubuyisela umonakalo othile kwizintso.
Zombini ezi ziyobisi zinikwa ngenaliti. Zinokubangela iziphumo ebezingalindelekanga ezincinci, ezifana nentloko ebuhlungu, iimpawu ezifana nomkhuhlane, isisu esibuhlungu, kunye neentlungu zomzimba. Okubaluleke kakhulu, kukho umngcipheko wosulelo olunzima, olufana ne-meningitis (isifo se-meningococcal), xa usebenzisa ezi ziyobisi. Ngoko ke ugqirha wakho uya kukuxelela ukuba ufumane inaliti yoko, kwaye angakunika nee-antibiotics ukuthintela usulelo.
Ukongeza kula mayeza, unyango lwe-plasma lungasetyenziswa. Oku kuquka ukunika isigulana i-plasma (inxalenye engamanzi egazini) ethathwe kumntu ophilileyo (i-plasma infusion) okanye ukususa i-plasma yesigulana size siyitshintshe nge-plasma ephilileyo (i-plasma exchange).
Ukuba izintso aziphenduli nakweyiphi na kwezi ndlela zonyango, ukhetho lokugqibela kukusebenzisa i-kidney dialysis okanye ukufakelwa kwezintso .
Iingxaki ezinokubakho kunye neengxamiseko
Ukongeza ekungasebenzi kakuhle kwezintso, i-aHUS inokubangela ezinye iingxaki.
| Iingxaki ezinokubakho ze-aHUS | |
|---|---|
| |
| Ixesha lokuya kwi-ETU yesibhedlele (iYunithi yoNyango oluNgxamisekileyo) ngoko nangoko | |
| |
Ukuphila nesi sifo kunye nempilo yengqondo
Kuqhelekile ukuziva udandathekile kwaye uxhalabile xa uphila nesifo esifana nesi ixesha elide. Asikuko ukuba unetyala. Yiyo loo nto kubalulekile ukuba ukhathalele impilo yakho yengqondo kunye nempilo yakho yomzimba .
- Cela uncedo kwiqela lakho lezonyango: Oogqirha, abahlengikazi, kunye nabasebenzi bezentlalo banokukukhokela kwiindawo apho unokufumana khona inkxaso yengqondo oyifunayo.
- Bona umcebisi: Ukuthetha nomcebisi onelayisenisi kunokukunceda unciphise umthwalo wakho kwaye kwakha amandla engqondo owadingayo ukuze ukwazi ukumelana nale meko.
- Zikhathalele: Lala ngokwaneleyo. Fumana iindlela zokunciphisa uxinzelelo. Yitya ukutya okulungele izintso. Zilolonge kangangoko unako. Ezi zinto zinokuba nefuthe elikhulu kwimpilo-ntle yakho iyonke.
- Thetha nabantu obathandayo: Ukuthetha ngale nto nosapho kunye nabahlobo kuya kukunika isiqabu esikhulu.
Umyalezo Wokuya Ekhaya
- I-aHUS yimeko engaqhelekanga kodwa enzulu ebangelwa yimbangela yemfuza kunye nesizathu sangaphandle esifana nokukhulelwa kunye nosulelo.
- Oku kwenzeka ikakhulu xa igazi liqhekeka kwimithambo yegazi emincinci yezintso nakwezinye izitho zomzimba.
- I-anemia, iiplatelets ezisezantsi, kunye nokusebenza kakubi kwezintso zezona mpawu ziphambili.
- Nangona esi sifo singenakunyangwa ngokupheleleyo, ngokuxilongwa kwangethuba kunye nonyango olufanelekileyo, sinokulawulwa kakuhle, iingxaki zinokuthintelwa, kwaye ubomi obuqhelekileyo bunokukhokelwa.
- Ukuba unazo ezi mpawu okanye unamathandabuzo malunga noku, bona ugqirha wakho ngokukhawuleza ukuze akunike icebiso.
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