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Uyazazi yini lezi zifo zezinso ezingavamile? (I-Atypical Hemolytic Uremic Syndrome - aHUS)

Uyazazi yini lezi zifo zezinso ezingavamile? (I-Atypical Hemolytic Uremic Syndrome - aHUS)

Yini ebingenzeka uma amahlule egazi ehlangana ngaphakathi kwemithambo yegazi emincane ezinso zakho, evimba ukugeleza kwegazi ezinso zakho? Kuyesabeka ukucabanga ngakho, akunjalo? Yilokho kanye esikhuluma ngakho namuhla. Lokhu kubizwa ngokuthi i-Atypical Hemolytic Uremic Syndrome, noma i-aHUS ngamafuphi. Nakuba kungelapheki, kungaphathwa kahle ngokwelashwa okufanele . Ngakho-ke ake sifunde kabanzi ngakho.

Uyini umehluko phakathi kwe-HUS engavamile kanye ne-HUS ejwayelekile?

Kungenzeka ukuthi uke wezwa ngesimo esibizwa ngokuthi i-HUS. Sisibiza ngokuthi "i-HUS ejwayelekile." Ngokuvamile sibangelwa ukutheleleka ngamagciwane njenge-E. coli, okubangela uhudo. Kodwa isimo esikhuluma ngaso namuhla, i-aHUS, sihlukile.

Lesi simo, esibizwa ngokuthi i-aHUS, singavela kunoma yimuphi ubudala. Ezinganeni ezincane, sithinta abafana namantombazane ngokulinganayo. Kodwa-ke, njengoba bekhula, abesifazane banamathuba amaningi okuthola lesi sifo. Lokhu kungenxa yokuthi ukukhulelwa kungaba yimbangela enkulu yalesi sifo. Akuvamile kangangokuthi ezinye izifundo ziphakamisa ukuthi abantu ababili kuphela kwabayisigidi abanalesi sifo.

Kungani i-aHUS ithuthuka?

Kalula nje, abantu abaningi abathola i-aHUS banoshintsho lwezakhi zofuzo, noma ukuguquka kwezakhi zofuzo, emizimbeni yabo. Cabanga ngakho njengokungathi kukhona 'inkinobho' emzimbeni wakho engabangela lesi sifo. Kodwa ukuba nje naleyo nkinobho ayibangeli lesi sifo ngokuzenzakalelayo. Kudinga okuthile okungaphandle kwayo ukuze 'kuvulwe', noma kusebenze. Lokhu sikubiza ngokuthi 'izimbangela'.

Izinto ezibangela lokhu kungaba:

  • Ukukhulelwa
  • Izimo ezithile ezithelelanayo
  • Umdlavuza
  • Eminye imithi
  • Ukufakelwa isitho somzimba
  • Ezinye izimo zezokwelapha zesikhathi eside (njenge-systemic sclerosis noma i-malignant hypertension)

Ngokumangalisayo, cishe engxenyeni yeziguli ze-aHUS, odokotela abanalo ushintsho lwezakhi zofuzo abangaluthola. Sibiza izimo ezinjalo ngokuthi "idiopathic," okusho ukuthi imbangela ayaziwa. Abacwaningi bakholelwa ukuthi lesi simo sibangelwa ushintsho lwezakhi zofuzo olungakatholakali.

Ziyini izimpawu?

Izimpawu ze-aHUS zivame ukuvela ngokuzumayo ngemva kokuba kuvele into ebangela ukuvuvukala. Ukuvuvukala kungaba kuncane noma kube kubi kakhulu. Ezimweni ezincane, izinso zingase zingathinteki kakhulu, kodwa ezimweni ezimbi kakhulu, zingaholela ekuhlulekeni kwezinso .

Izimpawu ongase uzizwe ezigabeni zokuqala:

  • Ubuhlungu bomzimba nokukhathala okukhulu
  • Ukuzizwa ungaphilile kahle

Lesi yisifo esiqhubekayo. Lokhu kusho ukuthi izimpawu ziya ziba zimbi ngokuhamba kwesikhathi, ikakhulukazi uma zingatholakali kusenesikhathi. I-aHUS ithinta kakhulu izinso zakho. Kodwa-ke, ezinye izitho zomzimba, njengobuchopho, isibindi, amaphaphu, nenhliziyo, nazo zingathinteka ukuvaleka kwemithambo yegazi.

Amaphuzu abalulekile okufanele uwacabangele ekuxilongweni Ukuhlolwa kanye nalokho abakufunayo
Izimpawu ezintathu udokotela azifunayo

1. I-Hemolytic anemia: Amaseli abomvu egazi abhujiswa ngokushesha kunalokho akhiqizwayo.

2. Ukwehla kwama-platelet: Ukwehla kwenani lamaseli asiza ukujiya kwegazi.

3. Izinkinga zezinso: Ukungasebenzi kahle kwezinso.

Ukuhlolwa
  • Inani eliphelele legazi (i-CBC): Ukulinganisa amazinga e-platelet kanye namaseli abomvu egazi.
  • Ukuhlolwa kwe-eGFR: Ukubona ukuthi izinso zisebenza kahle kangakanani.
  • Ukuhlolwa kwe-ADAMTS13: Ukuqinisekisa ukuthi i-TTP ayisona isifo esifana ne-aHUS.
  • Ukuhlolwa kwezakhi zofuzo: Ukubona ukuthi kukhona yini ukuguquka kwezakhi zofuzo okubangela lesi sifo.

Ungasithola kanjani lesi sifo?

Uma kungekho muntu emndenini wakho oke waba nalesi sifo ngaphambili, kungaba nzima ukuthola i-aHUS. Ngokuvamile, udokotela wezinzwa noma isazi segazi ngumuntu ongcono kakhulu ukwazi ngalokhu. Bazonaka kakhulu izici ezibalwe etafuleni elingenhla bese bekudlulisela ukuze uthole izivivinyo ezidingekayo.

Yiziphi izindlela zokwelapha?

Ngenhlanhla, kunemithi emibili evunyelwe ye-aHUS.

  • `I-Eculizumab (i-Soliris)`
  • `Ravulizumab-cwcz (Ultomiris)`

Zombili lezi ziyingxenye yemithi ebizwa ngokuthi 'ama-antibody e-monoclonal.' Kalula nje, lezi zingamaprotheni enziwe ngokwenziwa asebenza njengama-antibody emvelo omzimba wethu. Alawula ingxenye yesimiso somzimba sokuzivikela futhi avimbele ukujiya kwegazi.

Umuthi obizwa ngokuthi "i-Eculizumab" ungandisa inani lama-platelet namaseli abomvu egazini lakho. Uma ukwelashwa kuqalwa ngokushesha okukhulu , lo muthi ungalungisa ngisho nomonakalo othile ezinso.

Yomibili le mithi inikezwa ngomjovo. Ingabangela imiphumela emibi emincane, njengekhanda elibuhlungu, izimpawu ezifana nomkhuhlane, ukucasuka kwesisu, kanye nobuhlungu bomzimba. Okubaluleke kakhulu, kunengozi yokutheleleka okukhulu, njenge-meningitis (isifo se-meningococcal), lapho uthatha le mithi. Ngakho udokotela wakho uzokutshela ukuthi uthole umjovo walokho, futhi angakunikeza nama-antibiotic ukuvimbela ukutheleleka.

Ngaphezu kwale mithi, ukwelashwa nge-plasma nakho kungasetshenziswa. Lokhu kuhilela ukunikeza isiguli i-plasma (ingxenye yegazi ewuketshezi) ethathwe kumuntu ophilile (ukufakwa kwe-plasma) noma ukususa i-plasma yesiguli bese kufakwa i-plasma enempilo (ukushintshana kwe-plasma).

Uma izinso zingasabeli kunoma yikuphi kwalokhu kwelashwa, inketho yokugcina iwukuhlanzwa kwezinso noma ukufakelwa izinso .

Izinkinga ezingaba khona kanye nezimo eziphuthumayo

Ngaphezu kokwehluleka kwezinso, i-aHUS ingabangela ezinye izinkinga.

Izinkinga ezingaba khona ze-aHUS
  • I-anemia ye-Hemolytic
  • Ukwehla kwama-platelet (i-Thrombocytopenia)
  • Umfutho wegazi ophakeme
  • Isifo senhliziyo noma ukuhlaselwa yinhliziyo
  • Ikhanda elibuhlungu
  • Umbono ophindwe kabili
  • Ukukhubazeka kobuso
  • Ukuquleka
  • Isikhathi sokuya esibhedlela i-ETU (i-Emergency Treatment Unit) ngokushesha

    • Ukuchama okuncishisiwe kakhulu noma okuyekile ukuchama
    • Ukudideka, ukuqonda okushintshile
    • Ukuvuvukala okukhulu komzimba
    • Ubunzima bokuphefumula

    Ukuphila nalesi sifo kanye nempilo yengqondo

    Kuvamile ukuzizwa ucindezelekile futhi ukhathazekile uma unesifo esifana nalesi isikhathi eside. Akulona iphutha lakho. Yingakho kubalulekile ukunakekela impilo yakho yengqondo kanye nempilo yakho yomzimba .

    • Cela usizo eqenjini lakho lezokwelapha: Odokotela, abahlengikazi, kanye nezisebenzi zezenhlalakahle bangakuqondisa ezindaweni lapho ungathola khona ukwesekwa kwengqondo okudingayo.
    • Bona umeluleki: Ukukhuluma nomeluleki onelayisensi kungasiza ekunciphiseni umthwalo wakho futhi kwakha amandla engqondo owadingayo ukuze ubhekane nalesi simo.
    • Zinakekele: Lala ngokwanele. Thola izindlela zokunciphisa ukucindezeleka. Yidla ukudla okuhambisana nezinso. Zivocavoce ngangokunokwenzeka. Lezi zinto zingaba nomthelela omkhulu enhlalakahleni yakho iyonke.
    • Khuluma nabathandekayo bakho: Ukukhuluma ngalokhu nomndeni nabangane kuzokunikeza impumuzo enkulu.

    Umlayezo Wokuya Nawe Ekhaya

    • I-aHUS yisimo esingavamile kodwa esibucayi esibangelwa imbangela yofuzo kanye nesizathu sangaphandle njengokukhulelwa nokutheleleka.
    • Lokhu kwenzeka kakhulu lapho kwakheka amahlule egazi emithanjeni yegazi emincane yezinso nezinye izitho zomzimba.
    • Izimpawu eziyinhloko yi-anemia, ama-platelet aphansi, kanye nokusebenza kwezinso okungasebenzi kahle.
    • Nakuba lesi sifo singelapheki ngokuphelele, ngokuxilongwa kusenesikhathi kanye nokwelashwa okufanele, singalawulwa kahle, izinkinga zingavinjelwa, futhi impilo evamile ingaphila.
    • Uma unalezi zimpawu noma ungabaza ngalokhu, bona udokotela wakho ngokushesha ukuze akunike iseluleko.

    i-aHUS, i-Atypical Hemolytic Uremic Syndrome, Isifo Sezinso, Amahlule Egazi, Ukwehluleka Kwezinso, Isazi Sezinso, Isazi Segazi, i-Eculizumab
    ⚠️ Important: The medical articles and information on Nirogi Lanka are for general awareness only, and are by no means a substitute for professional medical advice, diagnosis, or treatment. For any medical problem you have, consult a qualified physician immediately.

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    Awekho amazwana athunyelwe okwamanje. Faka amazwana akho lapha okokuqala ngqa.

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    Uyazazi yini lezi zifo zezinso ezingavamile? (I-Atypical Hemolytic Uremic Syndrome - aHUS)

    Uyazazi yini lezi zifo zezinso ezingavamile? (I-Atypical Hemolytic Uremic Syndrome - aHUS)

    Yini ebingenzeka uma amahlule egazi ehlangana ngaphakathi kwemithambo yegazi emincane ezinso zakho, evimba ukugeleza kwegazi ezinso zakho? Kuyesabeka ukucabanga ngakho, akunjalo? Yilokho kanye esikhuluma ngakho namuhla. Lokhu kubizwa ngokuthi i-Atypical Hemolytic Uremic Syndrome, noma i-aHUS ngamafuphi. Nakuba kungelapheki, kungaphathwa kahle ngokwelashwa okufanele . Ngakho-ke ake sifunde kabanzi ngakho.

    Uyini umehluko phakathi kwe-HUS engavamile kanye ne-HUS ejwayelekile?

    Kungenzeka ukuthi uke wezwa ngesimo esibizwa ngokuthi i-HUS. Sisibiza ngokuthi "i-HUS ejwayelekile." Ngokuvamile sibangelwa ukutheleleka ngamagciwane njenge-E. coli, okubangela uhudo. Kodwa isimo esikhuluma ngaso namuhla, i-aHUS, sihlukile.

    Lesi simo, esibizwa ngokuthi i-aHUS, singavela kunoma yimuphi ubudala. Ezinganeni ezincane, sithinta abafana namantombazane ngokulinganayo. Kodwa-ke, njengoba bekhula, abesifazane banamathuba amaningi okuthola lesi sifo. Lokhu kungenxa yokuthi ukukhulelwa kungaba yimbangela enkulu yalesi sifo. Akuvamile kangangokuthi ezinye izifundo ziphakamisa ukuthi abantu ababili kuphela kwabayisigidi abanalesi sifo.

    Kungani i-aHUS ithuthuka?

    Kalula nje, abantu abaningi abathola i-aHUS banoshintsho lwezakhi zofuzo, noma ukuguquka kwezakhi zofuzo, emizimbeni yabo. Cabanga ngakho njengokungathi kukhona 'inkinobho' emzimbeni wakho engabangela lesi sifo. Kodwa ukuba nje naleyo nkinobho ayibangeli lesi sifo ngokuzenzakalelayo. Kudinga okuthile okungaphandle kwayo ukuze 'kuvulwe', noma kusebenze. Lokhu sikubiza ngokuthi 'izimbangela'.

    Izinto ezibangela lokhu kungaba:

    • Ukukhulelwa
    • Izimo ezithile ezithelelanayo
    • Umdlavuza
    • Eminye imithi
    • Ukufakelwa isitho somzimba
    • Ezinye izimo zezokwelapha zesikhathi eside (njenge-systemic sclerosis noma i-malignant hypertension)

    Ngokumangalisayo, cishe engxenyeni yeziguli ze-aHUS, odokotela abanalo ushintsho lwezakhi zofuzo abangaluthola. Sibiza izimo ezinjalo ngokuthi "idiopathic," okusho ukuthi imbangela ayaziwa. Abacwaningi bakholelwa ukuthi lesi simo sibangelwa ushintsho lwezakhi zofuzo olungakatholakali.

    Ziyini izimpawu?

    Izimpawu ze-aHUS zivame ukuvela ngokuzumayo ngemva kokuba kuvele into ebangela ukuvuvukala. Ukuvuvukala kungaba kuncane noma kube kubi kakhulu. Ezimweni ezincane, izinso zingase zingathinteki kakhulu, kodwa ezimweni ezimbi kakhulu, zingaholela ekuhlulekeni kwezinso .

    Izimpawu ongase uzizwe ezigabeni zokuqala:

    • Ubuhlungu bomzimba nokukhathala okukhulu
    • Ukuzizwa ungaphilile kahle

    Lesi yisifo esiqhubekayo. Lokhu kusho ukuthi izimpawu ziya ziba zimbi ngokuhamba kwesikhathi, ikakhulukazi uma zingatholakali kusenesikhathi. I-aHUS ithinta kakhulu izinso zakho. Kodwa-ke, ezinye izitho zomzimba, njengobuchopho, isibindi, amaphaphu, nenhliziyo, nazo zingathinteka ukuvaleka kwemithambo yegazi.

    Amaphuzu abalulekile okufanele uwacabangele ekuxilongweni Ukuhlolwa kanye nalokho abakufunayo
    Izimpawu ezintathu udokotela azifunayo

    1. I-Hemolytic anemia: Amaseli abomvu egazi abhujiswa ngokushesha kunalokho akhiqizwayo.

    2. Ukwehla kwama-platelet: Ukwehla kwenani lamaseli asiza ukujiya kwegazi.

    3. Izinkinga zezinso: Ukungasebenzi kahle kwezinso.

    Ukuhlolwa
    • Inani eliphelele legazi (i-CBC): Ukulinganisa amazinga e-platelet kanye namaseli abomvu egazi.
    • Ukuhlolwa kwe-eGFR: Ukubona ukuthi izinso zisebenza kahle kangakanani.
    • Ukuhlolwa kwe-ADAMTS13: Ukuqinisekisa ukuthi i-TTP ayisona isifo esifana ne-aHUS.
    • Ukuhlolwa kwezakhi zofuzo: Ukubona ukuthi kukhona yini ukuguquka kwezakhi zofuzo okubangela lesi sifo.

    Ungasithola kanjani lesi sifo?

    Uma kungekho muntu emndenini wakho oke waba nalesi sifo ngaphambili, kungaba nzima ukuthola i-aHUS. Ngokuvamile, udokotela wezinzwa noma isazi segazi ngumuntu ongcono kakhulu ukwazi ngalokhu. Bazonaka kakhulu izici ezibalwe etafuleni elingenhla bese bekudlulisela ukuze uthole izivivinyo ezidingekayo.

    Yiziphi izindlela zokwelapha?

    Ngenhlanhla, kunemithi emibili evunyelwe ye-aHUS.

    • `I-Eculizumab (i-Soliris)`
    • `Ravulizumab-cwcz (Ultomiris)`

    Zombili lezi ziyingxenye yemithi ebizwa ngokuthi 'ama-antibody e-monoclonal.' Kalula nje, lezi zingamaprotheni enziwe ngokwenziwa asebenza njengama-antibody emvelo omzimba wethu. Alawula ingxenye yesimiso somzimba sokuzivikela futhi avimbele ukujiya kwegazi.

    Umuthi obizwa ngokuthi "i-Eculizumab" ungandisa inani lama-platelet namaseli abomvu egazini lakho. Uma ukwelashwa kuqalwa ngokushesha okukhulu , lo muthi ungalungisa ngisho nomonakalo othile ezinso.

    Yomibili le mithi inikezwa ngomjovo. Ingabangela imiphumela emibi emincane, njengekhanda elibuhlungu, izimpawu ezifana nomkhuhlane, ukucasuka kwesisu, kanye nobuhlungu bomzimba. Okubaluleke kakhulu, kunengozi yokutheleleka okukhulu, njenge-meningitis (isifo se-meningococcal), lapho uthatha le mithi. Ngakho udokotela wakho uzokutshela ukuthi uthole umjovo walokho, futhi angakunikeza nama-antibiotic ukuvimbela ukutheleleka.

    Ngaphezu kwale mithi, ukwelashwa nge-plasma nakho kungasetshenziswa. Lokhu kuhilela ukunikeza isiguli i-plasma (ingxenye yegazi ewuketshezi) ethathwe kumuntu ophilile (ukufakwa kwe-plasma) noma ukususa i-plasma yesiguli bese kufakwa i-plasma enempilo (ukushintshana kwe-plasma).

    Uma izinso zingasabeli kunoma yikuphi kwalokhu kwelashwa, inketho yokugcina iwukuhlanzwa kwezinso noma ukufakelwa izinso .

    Izinkinga ezingaba khona kanye nezimo eziphuthumayo

    Ngaphezu kokwehluleka kwezinso, i-aHUS ingabangela ezinye izinkinga.

    Izinkinga ezingaba khona ze-aHUS
    • I-anemia ye-Hemolytic
    • Ukwehla kwama-platelet (i-Thrombocytopenia)
    • Umfutho wegazi ophakeme
    • Isifo senhliziyo noma ukuhlaselwa yinhliziyo
  • Ikhanda elibuhlungu
  • Umbono ophindwe kabili
  • Ukukhubazeka kobuso
  • Ukuquleka
  • Isikhathi sokuya esibhedlela i-ETU (i-Emergency Treatment Unit) ngokushesha

    • Ukuchama okuncishisiwe kakhulu noma okuyekile ukuchama
    • Ukudideka, ukuqonda okushintshile
    • Ukuvuvukala okukhulu komzimba
    • Ubunzima bokuphefumula

    Ukuphila nalesi sifo kanye nempilo yengqondo

    Kuvamile ukuzizwa ucindezelekile futhi ukhathazekile uma unesifo esifana nalesi isikhathi eside. Akulona iphutha lakho. Yingakho kubalulekile ukunakekela impilo yakho yengqondo kanye nempilo yakho yomzimba .

    • Cela usizo eqenjini lakho lezokwelapha: Odokotela, abahlengikazi, kanye nezisebenzi zezenhlalakahle bangakuqondisa ezindaweni lapho ungathola khona ukwesekwa kwengqondo okudingayo.
    • Bona umeluleki: Ukukhuluma nomeluleki onelayisensi kungasiza ekunciphiseni umthwalo wakho futhi kwakha amandla engqondo owadingayo ukuze ubhekane nalesi simo.
    • Zinakekele: Lala ngokwanele. Thola izindlela zokunciphisa ukucindezeleka. Yidla ukudla okuhambisana nezinso. Zivocavoce ngangokunokwenzeka. Lezi zinto zingaba nomthelela omkhulu enhlalakahleni yakho iyonke.
    • Khuluma nabathandekayo bakho: Ukukhuluma ngalokhu nomndeni nabangane kuzokunikeza impumuzo enkulu.

    Umlayezo Wokuya Nawe Ekhaya

    • I-aHUS yisimo esingavamile kodwa esibucayi esibangelwa imbangela yofuzo kanye nesizathu sangaphandle njengokukhulelwa nokutheleleka.
    • Lokhu kwenzeka kakhulu lapho kwakheka amahlule egazi emithanjeni yegazi emincane yezinso nezinye izitho zomzimba.
    • Izimpawu eziyinhloko yi-anemia, ama-platelet aphansi, kanye nokusebenza kwezinso okungasebenzi kahle.
    • Nakuba lesi sifo singelapheki ngokuphelele, ngokuxilongwa kusenesikhathi kanye nokwelashwa okufanele, singalawulwa kahle, izinkinga zingavinjelwa, futhi impilo evamile ingaphila.
    • Uma unalezi zimpawu noma ungabaza ngalokhu, bona udokotela wakho ngokushesha ukuze akunike iseluleko.

    i-aHUS, i-Atypical Hemolytic Uremic Syndrome, Isifo Sezinso, Amahlule Egazi, Ukwehluleka Kwezinso, Isazi Sezinso, Isazi Segazi, i-Eculizumab
    ⚠️ Important: The medical articles and information on Nirogi Lanka are for general awareness only, and are by no means a substitute for professional medical advice, diagnosis, or treatment. For any medical problem you have, consult a qualified physician immediately.

    💬 Comments (0)

    Awekho amazwana athunyelwe okwamanje. Faka amazwana akho lapha okokuqala ngqa.

    Engeza amazwana akho

    Sicela ubale: 2 + 5 =