Luyini lolu hlobo olungavamile lwe-anemia nezinkinga zezinso? (I-Atypical Hemolytic Uremic Syndrome - aHUS) Ake sixoxe!

Ungacabanga ukuthi bekuzokwenzekani uma uhlelo lwethu lokuzivikela, uhlelo lokuzivikela, esikhundleni sokusivikela ezifweni, luqala ukuhlasela imizimba yethu? Leso yisimo esibi, kodwa esingajwayelekile, esizokhuluma ngaso namuhla yi-aHUS, noma i-Atypical Hemolytic Uremic Syndrome . Lokhu kwenzeka lapho imithambo yethu yegazi yonakala, kwakheka amahlule amancane egazi, futhi ukugeleza kwegazi ezithweni ezibalulekile njengezinso kuncipha. Lesi simo sivame ukubangelwa ukuguquka kwezakhi zofuzo .

Odokotela bavame ukukuxilonga ukuthi une-aHUS uma unazo zonke lezi zimo ezintathu ndawonye:

• I-Microangiopathic hemolytic anemia : Lokhu kusho ukuthi amangqamuzana akho abomvu egazi abhujiswa ngokushesha kakhulu, ngakho umzimba wakho awukwazi ukwenza amangqamuzana amasha ukuze uwathathe indawo yawo.
• I-Thrombocytopenia : Lokhu ukwehla kwenani lama-platelet, okusiza ukugoba kwegazi.
• Ukulimala kwezinso okubukhali : Lolu uhlobo lokwehluleka kwezinso, kodwa ngezinye izikhathi kungaguqulwa.

Zonke lezi zimo ezintathu zingavela ngesikhathi esisodwa, noma zingaba zimbi kakhulu ngezinye izifo.

Uyini umehluko phakathi kwe-HUS ejwayelekile nale aHUS?

Esikhathini esidlule, lesi simo esibizwa ngokuthi i-HUS (Hemolytic Uremic Syndrome) sahlukaniswa saba izinhlobo ezimbili eziyinhloko.

• I-HUS ejwayelekile (i-HUS ehlobene nohudo): Lokhu kuvame ukuhambisana nohudo. Kubangelwa uhlobo lwebhaktheriya elibizwa ngokuthi i-E. coli . Abanye abantu bayibiza nangokuthi i-STEC-HUS .
• I-aHUS esikhuluma ngayo namuhla (i-HUS engavamile): Lolu akulona uhlobo lwesifo sohudo olubonakala. Esikhathini esiningi, cishe ingxenye yabantu abathola i-aHUS, ibangelwa ushintsho lwezakhi zofuzo, ukuguquka kwezakhi zofuzo . Odokotela ngezinye izikhathi babiza lesi simo ngokuthi i-complement-mediated thrombotic microangiopathy (CM-TMA) .

Yiziphi izimbangela ezithile ze-aHUS?

Kalula nje, akuwona wonke umuntu onoguquko lwezakhi zofuzo oluzothuthukisa i-HUS. Ngokuvamile, ibangelwa, noma ibhebhethekiswe, ezinye izimo zempilo. Cabanga ngalezi zinto:

• Isikhathi sokukhulelwa
• Umdlavuza
• Izifo ezahlukahlukene
• Imithi ethile, ikakhulukazi imithi yokwelapha ngamakhemikhali , imithi yokucindezela amasosha omzimba , imithi yokunciphisa igazi , imithi yokuvimbela inzalo ngomlomo , kanye neminye imithi yokulwa nokuvuvukala .

Ziyini izimpawu ze-aHUS?

Manje ake sibheke ukuthi yiziphi izimpawu eziboniswa umuntu one-aHUS. Ungase ube neziningana zalezi, noma ungase ube nezimpawu ezikhula kancane kancane.

• Ukuzizwa ukhathele ngaso sonke isikhathi (Ukukhathala)
• Isikhumba esikhanyayo (Umbala ophuzi)
• Isicanucanu noma ukuhlanza
• Ukwehla kokukhishwa komchamo
• Igazi emchameni
• Umfutho wegazi ophezulu (i-Hypertension)
• Ubunzima bokuphefumula (i-Dyspnea)
• Ukuvuvukala
• Ukudideka , ukulahlekelwa yinkumbulo
• Imfiva

Iningi labantu alinazo zonke lezi zimpawu ngesikhathi esisodwa. Ngezinye izikhathi zivela kancane kancane. Ungase uzizwe sengathi ubulokhu uhlushwa okuthile isikhashana, kodwa awukwazi ukuthola ukuthi kuyini. Lezi zimpawu zezinzwa , yilokho ukudideka engikushilo ekuqaleni, azenzeki kuwo wonke umuntu, akuvamile kangako.

Yini ngempela ebangela i-aHUS?

Imbangela eyinhloko ye-aHUS ushintsho ku-DNA yethu (ukuguqulwa kwezakhi zofuzo) , noma ukwakheka kwama-autoantibodies ahlasela amangqamuzana ethu ngokumelene 'namaprotheni ahambisanayo' ohlelweni lwethu lokuzivikela komzimba . Lezi zinguquko zezakhi zofuzo zingazuzwa kubazali bethu, noma zingenzeka ngokungahleliwe. Lezi zinguquko zezakhi zofuzo zithinta umsebenzi wamaprotheni abizwa ngokuthi 'izici ezihambisanayo' .

Cabanga ngalezi 'zinto ezihambisanayo' njengamasosha amancane asiza amasosha ethu omzimba. Njengokudla okunongiwe, lawa maprotheni athola amagciwane ayingozi (njengamabhaktheriya namagciwane) bese ewamaka ukuze amanye amangqamuzana omzimba 'awadle'. Ezivame ukuthinteka ku-aHUS yizinto ezihambisanayo H, I, 3, kanye no-B.

Isibonelo, umsebenzi we-complement factor H ukuvikela amangqamuzana anempilo emzimbeni wethu nokuvimbela ezinye izinto ezihambisanayo ukuthi zingasebenzi uma zingadingeki. Manje cabanga ukuthi kwenzekani uma le complement factor H ingasebenzi kahle (okungukuthi, uma kukhona ukuguquka kwezakhi zofuzo ze-CFH) ? Amangqamuzana ethu awatholi isivikelo asidingayo. Nansi-ke izinto ezenzekayo:

• Amangqamuzana ethu omzimba ahlasela futhi alimaze ngephutha amangqamuzana angaphakathi kwemithambo yegazi yethu (amangqamuzana e-endothelial) .
• Ama-platelet aqoqana eduze kwalomonakalo bese enza ihlule legazi. Lokhu kuvimbela imithambo yegazi.
• Lokhu kunciphisa ukugeleza kwegazi ezithweni zethu zomzimba, okubangela ukuthi zilimale futhi umsebenzi wazo wehle. Izinso yizona ezithinteka kakhulu.
• Lawa mahlule egazi alimaza namanye amaseli egazi adlula kuwo, okubangela isimo esibizwa ngokuthi i-hemolytic anemia .

Wazi kanjani ukuthi une-aHUS?

Udokotela uvame ukuxilonga i-aHUS ngokusekelwe ezimpawu zakho kanye nemiphumela yokuhlolwa okuhlola inani legazi lakho kanye nendlela izitho zakho ezisebenza ngayo. Uzokwenza nokuhlolwa ukuqinisekisa ukuthi awunazo ezinye izifo ezibangela izimpawu ezifanayo (njenge-STEC-HUS eshiwo ngenhla).

Yiziphi izivivinyo ezisetshenziswa ukuxilonga i-aHUS?

Kungadingeka wenze izivivinyo ezinjengalezi:

• Ukuhlolwa kwegazi: I-Complete Blood Count (CBC) , ukuhlolwa kokusebenza kwezinso, njll.
• Ukuhlolwa kwezakhi zofuzo : Lokhu kuhlola ukuguquka kwezakhi zofuzo.
• Ukuhlolwa kwendle : Hlola izifo ezifana ne-E. coli.
• Ukuhlolwa kwezinso : Ukuthatha ingxenye encane yezinso ukuze iyohlolwa.
• Ukuhlolwa kwezithombe : njenge- ultrasound noma i-CT scan .

Iphathwa kanjani i-aHUS?

Ukwelashwa kwe-aHUS kuncike embangela eyinhloko kanye nobunzima besimo. Uma unesimo se-aHUS esingesibi kakhulu, udokotela wakho angakuqapha futhi akunikeze ukunakekelwa okusekelayo , njengokumpontshelwa igazi noma imithi yokucindezela kwegazi okuphezulu, uma kudingeka.

Kodwa-ke, uma une-aHUS enzima, ungadinga ukwelashwa okufana nalokhu:

• Ukushintshaniswa kwe-plasma yokwelapha : Kulokhu, ingxenye ye-plasma yegazi iyasuswa bese kunikezwa i-plasma entsha.
• I-Rituximab noma eminye imithi esetshenziselwa ukwelapha izifo ezihlasela amasosha omzimba.
• I-Dialysis : Indlela yokuhlanza igazi lapho izinso zingasebenzi kahle.
• Ukufakelwa kwezinso : Lokhu kwenziwa kuphela ezimweni ezinzima kakhulu.

Kulabo abanezinguquko zezakhi zofuzo 'ezihambisanayo' okukhulunywe ngazo ngenhla, odokotela bavame ukusebenzisa umuthi obizwa ngokuthi i-eculizumab ukwelapha i-aHUS. I-Eculizumab isebenza ngokuvimbela amaprotheni athile ahambisanayo, ivimbele amasosha omzimba wakho ekulimazeni imithambo yegazi yakho.

Ingabe ikhona imiphumela emibi yokwelashwa?

Uma usebenzisa i-Eculizumab , ungase uvikeleke kancane emithini yokugomela i -meningococcal kanye ne-pneumococcal . Ngakho-ke, uma kungenzeka, udokotela wakho angakweluleka ukuthi uthole le mithi yokugomela ngaphambi kokuqala i-Eculizumab .

Ikusasa liphetheni ngomuntu one-aHUS?

Uma lesi simo sitholakala futhi selashwa kusenesikhathi, izimo ezifana nokulimala kwezinso okubangelwa yi-aHUS zingaphathwa ngempumelelo. Ungase ube nezikhathi zokululama ngaphandle kwezimpawu. Kodwa-ke, uma kukhona okunye okubangela i-aHUS futhi, ingaba yingozi empilweni futhi.

Esikhathini esidlule, cishe ingxenye, noma ama-50%, abantu abane-aHUS bahlushwa isifo sezinso sesigaba sokugcina (ESKD) , okusho ukuthi izinso zabo azisebenzi kahle ngokuphelele.

Kodwa kunezindaba ezinhle! Izifundo ezintsha zibonisa ngokusobala ukuthi ingozi ye-ESKD inciphile kakhulu kubantu abane-aHUS ngenxa yokwelashwa kwe- Eculizumab .

Iyini ingozi yokufa ngenxa ye-aHUS?

Amacala abucayi e-aHUS, njengokwehluleka kwezinso noma ukulimala kwezinye izitho zomzimba, abucayi kakhulu. Ezimweni ezinjalo ezibucayi, kunengozi yokufa engaba ngu-15%.

Ingabe i-aHUS ingavinjelwa?

Ngenxa yokuthi ivame ukubangelwa izinguquko zofuzo, i-aHUS ngokuvamile ayinakuvinjelwa. Kodwa-ke, uma usengozini yokuthola isifo esibi kakhulu, ungase ukwazi ukugwema ezinye zezinto ezisibangelayo .

Ngizinakekela kanjani?

Uma utholakale une-aHUS, khuluma nodokotela wakho ngezindlela zokugwema izinto ezingenza isimo sibe sibi kakhulu. Kubalulekile futhi ukwazi izimpawu zesimo esibi kakhulu.

Kufanele ngimbone nini udokotela?

Uma ubulokhu uzizwa ukhathele noma ukhathele isikhathi eside – njengokungathi unesifo esingamahlalakhona esingapheli – khuluma nodokotela wakho. Futhi, khuluma nganoma yiziphi ezinye izimpawu oye waba nazo muva nje, njengokushintsha kwemikhuba yakho yokugeza.

Kufanele ngiye nini e-Emergency Treatment Unit (ETU) ?

Uma unalezi zimpawu ezinzima, hamba uye ekamelweni eliphuthumayo ngokushesha:

• Ukuphuma komchamo kuncishiswe kakhulu.
• Ukudideka noma ukushintsha kwengqondo.
• Ukuvuvukala okukhulu.
• Ubunzima obukhulu bokuphefumula.

Yimiphi imibuzo okufanele ngiyibuze udokotela?

Kungaba usizo ukubuza udokotela wakho imibuzo efana nale:

• Isimo sami sibi kangakanani?
• Yini eyabangela lokhu ukuba kuqhubeke?
• Ngingakwazi yini ukuvimbela izimo ezinjengalezi esikhathini esizayo?
• Yiziphi izimpawu okufanele ngiziqaphele?
• Kufanele ngize nini ukukubona futhi?

Sivame kangakanani lesi simo esibizwa ngokuthi i-aHUS?

I-aHUS ayivamile kakhulu.Isimo sezokwelapha. Sithinta cishe umuntu oyedwa kubantu abayizinkulungwane ezingamakhulu amahlanu. Sivame kakhulu kubantu abadala kunasezinganeni.

Okokugcina, izinto okufanele uzikhumbule

Ukuthola ukuthi wena noma ingane yakho ninesimo sofuzo kungaba yinto eshintsha impilo. I-aHUS yisimo esibucayi esingabangela amahlule egazi futhi sonakalise izitho zomzimba. Kodwa-ke, ukuthuthukiswa kwamuva komuthi obizwa ngokuthi i-eculizumab kuthuthukise kakhulu izinga lokusinda kwabantu abane-aHUS. Uma utholakale une-aHUS, khuluma nodokotela wakho ngezindlela zokugwema izimbangela nokunciphisa ingozi yokugula okungathí sina. Angakusiza uqonde ukuthi yini okudingeka uyenze ukuze uhlale uphilile.

i- aHUS, isifo sezinso, amahlule egazi, isimiso somzimba sokuzivikela, izinguquko zofuzo, i-anemia, i-Eculizumab