Singakhuluma ngesifo esingavamile esibizwa ngokuthi i-C3G (Complement 3 Glomerulopathy), esithinta izinso?

Ingabe ngezinye izikhathi uzwa igazi emchameni wakho, noma ukuvuvukala emilenzeni yakho? Noma uzizwa ukhathele ngaso sonke isikhathi? Lokhu ngezinye izikhathi kungaba ngenxa yenkinga ngezinso zakho. Namuhla sizokhuluma ngesimo esingavamile kodwa esibaluleke kakhulu esithinta izinso. Leso yi-C3G (C Three G) noma i-``Complement 3 Glomerulopathy``.

Kuyini i-C3G (i-Complement 3 Glomerulopathy)?

Kalula nje, i-C3G iyiqembu lezifo ezithinta indlela izinso zakho ezisebenza kahle ngayo. Uyazi, izinso zifana nezihlungi ezimbili emizimbeni yethu. Umsebenzi wazo oyinhloko ukuhlunga igazi, ukususa imfucuza engadingekile namanzi angaphezulu, nokwenza umchamo.

I-"Complement 3" iyiprotheni ekhethekile egazini lethu. Ibaluleke kakhulu ohlelweni lokuzivikela olulwa nezifo emizimbeni yethu. I-"Glomerulopathy" iwukulimala kwe-glomeruli, okuyimithambo yegazi emincane ngaphakathi kwezinso, efana nama-meshes amancane. Lawa ma-glomeruli yiwo ahlunga igazi.

Khona-ke i-C3G iyisifo esihilela iphrotheni ethi `complement 3`, elimaza i-`glomeruli` futhi iphazamise ukusebenza kwezinso. Esikhathini esidlule, okungukuthi, ngaphambi kuka-2013, odokotela babiza lokhu ngokuthi ``Membranoproliferative glomerulonephritis (MPGN) uhlobo I, II noma III`` noma ``Mesangioproliferative glomerulonephritis``. Kodwa manje leli qembu lezifo libizwa ngokuthi i-C3G.

Ingabe zikhona izinhlobo ze-C3G?

Yebo, kunezinhlobo ezimbili eziyinhloko ze-C3G. Odokotela banquma lezi zinhlobo ezimbili ngokuthatha ingxenye encane yezinso (siyibiza ngokuthi 'i-kidney biopsy ') bese beyibheka ngaphansi kwe-microscope, ngokusekelwe emonakalweni nasekuvuvukeni kwe-'glomeruli'.

• Isifo se-Dense deposit (DDD): Esasaziwa ngaphambilini ngokuthi uhlobo lwe-MPGN II, lesi simo sibonakala ngokwakheka kwama-deposit aminyene, afana neribhoni ku-glomerular basement membrane (GBM), ulwelwesi lwe-glomeruli. Sivame ukuhlasela izingane kanye nabantu abadala abasebasha abaseminyakeni yabo yama-20.

• `C3 glomerulonephritis` (C3GN): Ngaphambilini yayibizwa ngokuthi `MPGN uhlobo I` noma `III`. Kulokhu, ama-deposit aminyene njengalawo abonakala ku-DDD awabonakali. Lokhu kuthinta kakhulu abantu abangaphezu kweminyaka engama-30 ubudala.

Ingabe i-C3G iyisifo esingamahlalakhona?

Yebo, i-C3G iyisifo esingamahlalakhona. Lokhu kusho ukuthi akuyona into ezokhula ngokuzumayo futhi ibe ngcono, futhi umonakalo wezinso unganda ngokuhamba kwesikhathi. Ngakho-ke, kubaluleke kakhulu ukuqaphela lokhu.

Ivame kangakanani le C3G?

Empeleni lesi yisifo esingavamile kakhulu.Odokotela balinganisela ukuthi lokhu kwenzeka kubantu ababili kuya kwabathathu kubantu abayikhulu. Ngakho-ke kungenzeka ukuthi awuzange uzwe okuningi ngakho. Kodwa yize kungavamile, kubalulekile ukukuqaphela.

Ziyini izimpawu zesifo se-C3G?

Manje ake sibone ukuthi ziyini izimpawu zalesi sifo se-C3G. Zombili lezi zinhlobo zinezici eziningana ezifanayo, ezibalulekile ukuziqaphela:

• Igazi emchameni ( i-hematuria ): Ngezinye izikhathi umchamo ungase ubonakale ubomvu, noma kungaba inani elincane elingabonakala kuphela ngaphansi kwe-microscope.
• Iphrotheni eningi emchameni (i-proteinuria): Lokhu kungabangela ukuthi umchamo ubonakale unamagwebu.
• Ukuchama okuncane kunokuvamile (i-oliguria).
• Ukwehla kwamazinga amaprotheni egazini (i-hypoalbuminemia).
• Ukuvuvukala (i-edema): Ikakhulukazi ezandleni, emaqakaleni nasezinyaweni. Cabanga nje, uma uvuka ekuseni futhi izinyawo zakho zizizwa zivuvukile, kungaba yinto efana nale.
• Umfutho wegazi ophezulu (umfutho wegazi ophakeme).
• Ukutheleleka okuphindaphindiwe.
• Ukuzizwa ukhathele kakhulu ('Ukukhathala'): Hhayi ukukhathala nje kuphela, kodwa nomuzwa wokukhathala kungakhathaliseki ukuthi ulala kangakanani.
• Abanye abantu bangase babe nezimo ezifana ne -gout .

Zombili lezi zimpawu zingaholela ekuhlulekeni kwezinso . Ukwehluleka kwezinso nakho kungabangela izimpawu ezifana nalezi:

• Ngihlala ngikhathele.
• Isicanucanu, ukuhlanza.
• Ubunzima bokunquma, ukuphelelwa yithemba.
• Ukuvuvukala komzimba.
• Ukuchama okuningi kunokujwayelekile (lokhu kungabonakala ngezinye izikhathi ezigabeni zokuqala zokwehluleka kwezinso).

Ngaphezu kwezinso, i-C3G ingabangela nezinye izimpawu. Lezi zimpawu zifaka:

• Izinkinga zokubona: Ukubona kungaphazamiseka ngenxa yokuqongelela kwamaprotheni kanye ne-calcium deposits (drusen), ikakhulukazi kuma-macula.
• I-Lipodystrophy: Lesi yisimo lapho amafutha elahleka khona ezindaweni ezithile zomzimba (ikakhulukazi ebusweni nasezithweni).

Kungani le C3G yakhiwe?

Kulungile, manje ake sibone ukuthi kungani le C3G yakheka. Lokhu kuyinkimbinkimbi kancane, kodwa ngizokuchaza kalula. Lokhu kwenzeka lapho "uhlelo lokuhambisana" emzimbeni wethu lungasebenzi kahle. Lolu "hlelo lokuhambisana" luyiqoqo lamaprotheni egazini lethu. Lokhu kuyingxenye yesimiso sethu sokuzivikela komzimba. Lokhu kusiza ekulweni nezinto ezifana namagciwane namagciwane avela ngaphandle.

Okwenzekayo kumuntu one-C3G ukuthi lolu 'hlelo lokugcwalisa' lusebenza kakhulu, noma alulawuleki. Bese iphrotheni ethi 'complement 3 (C3)' yonakala. Lezi zingcezu zephrotheni ethi C3 eyonakele zibhajwa ku-'glomeruli'. Bese kuba nzima ukuthi i-'glomeruli' ihlunge igazi futhi yenze umchamo. Uma ingelashwa, lo monakalo ungaqhubeka futhi uholele ekuhlulekeni kwezinso.

Ngokuvamile, kunzima ukukhomba isizathu esiqondile sokuthi kungani "lolu hlelo lokuncoma" luba yinto engajwayelekile. Kodwa ngezinye izikhathi:

• I-C3G ingavela ngenxa yezinguquko ezakhiweni zethu zofuzo (ukuguqulwa kwezakhi zofuzo ezihlobene nohlelo lokugcwalisa, njenge-``C3`` kanye ne-``CFH```).
• Isifo segazi esibizwa ngokuthi ``i-Monoclonal gammopathy`` singalimaza nezinso ngokungaqondile, siphazamise ukusebenza kohlelo ``lokuphelelisa``, futhi sibangele ukwakheka kwe-C3G. Le ``monoclonal gammopathy`` yisimo esithinta amaseli e-plasma emnkantsheni wethu wamathambo.
• Abacwaningi bezokwelapha basola ukuthi into ebizwa ngokuthi "ama-autoantibodies" nayo ingadlala indima. "Ama-Autoantibodies" ngamaprotheni akhiqizwa amasosha omzimba ethu ahlasela ngephutha izicubu zethu ezinempilo. Kodwa-ke, awekho ucwaningo olwanele lokusho ngokuqinisekile ukuthi la "ma-autoantibodies" ayimbangela ye-C3G.

Ubani othinteka kakhulu yilesi sifo se-C3G?

Lesi sifo se-C3G singakhula kunoma ubani. Sithinta kokubili amadoda nabesifazane ngokulinganayo. Isilinganiso seminyaka yokuxilongwa sicishe sibe yiminyaka engama-23. Kodwa-ke, uhlobo lwe-`DDD` esikhulume ngalo ekuqaleni lungavela lusemncane kunohlobo lwe-`C3GN`.

Yiziphi izinkinga ezingaba khona ze-C3G?

Inkinga enkulu neyingozi kakhulu ukuthi cishe abantu abangu-50% abane-C3G, noma oyedwa kwababili, bazoqala ukwehluleka kwezinso zingakapheli iminyaka eyi-10 bexilongiwe. Yilokhu okufanele sonke sikwesabe kakhulu.

Isifo se-C3G sitholakala kanjani?

Udokotela uzokwenza izivivinyo eziningana ukuhlola impilo yezinso zakho nokuthi zisebenza kahle kangakanani. Lokhu kungafaka:

• Ukuhlolwa kwegazi: Kuthathwa isampula encane yegazi emthanjeni osengalweni yakho bese ilabhorethri ihlola ukuthi amaprotheni akho ahambisanayo ajwayelekile noma ukuthi kukhona yini imfucuza eningi egazini lakho (isib. i-creatinine).
• `Isilinganiso Sokuhlunga Kwe-Glomerular Esilinganiselwe (eGFR): Lolu ukuhlolwa okulinganisa ukuthi izinso zakho zihlunga kahle kangakanani igazi lakho, ngokusekelwe enanini le-`serum creatinine` noma i-`cystatin C` kusampula yegazi. Lokhu kungakunikeza umbono omuhle wokuthi izinso zakho zisebenza kahle kangakanani.
• Ukuhlolwa komchamo: Kuzodingeka uchamele enkomishini ekhethekile bese unikeza udokotela wakho isampula. Ilebhu izohlola iphrotheni noma igazi emchameni, okungabonakala kuphela ngaphansi kwe-microscope.
• I-biopsy yezinso: Yilokhu okushiwoyoIndlela engcono kakhulu yokuxilonga isifo se-C3G ngokuqinisekile iwukusebenzisa i-biopsy. Kulokhu, udokotela uthatha ingxenye encane yezicubu ezinso lakho esebenzisa inalithi encane noma ukunqunywa okuncane bese eyihlola elebhu. Uma lesi sampula siqukethe izingcezu zephrotheni ye-C3 eyonakele ku-glomeruli yakho, kungaba yisifo se-C3G. Le biopsy ingakutshela nokuthi yiluphi uhlobo lwe-C3G onalo (i-DDD noma i-C3GN), ngokusekelwe ekutheni unazo yini lezo zinhlayiya eziqinile noma cha.

Ingabe i-C3G ingalapheka ngokuphelele?

Ngeshwa, okwamanje alikho ikhambi lesifo se-C3G. Kodwa ungakhathazeki, odokotela nabacwaningi bayaqhubeka nokufunda ngalesi sifo futhi basebenza ukuthola izindlela zokwelapha ezintsha. Sithemba ukuthi kuzofika ukwelashwa okungcono esikhathini esizayo.

Yiziphi izindlela zokwelapha i-C3G?

Okwamanje azikho izindlela zokwelapha ezithile ezivunyelwe yi-U.S. Food and Drug Administration (FDA) ye-C3G. Kodwa-ke, odokotela bangakunikeza izindlela zokwelapha ezahlukahlukene kanye nokunakekelwa okusekelayo ukuze banciphise ukuqhubeka kwe-C3G, balawule izimpawu, futhi banciphise ukulimala kwezinso. Lokhu kufaka phakathi:

• Imithi yokucindezela kwegazi: Imithi efana ne-angiotensin-converting enzyme (ACE) inhibitors kanye ne-angiotensin II receptor blockers (ARBs) yehlisa ingcindezi ngaphakathi kwe-glomeruli. Lokhu kunciphisa inani lamaprotheni emchameni futhi kunciphisa ukucindezeleka ezinso.
• Imithi yokwehlisa i-cholesterol: Udokotela wakho angase akunike imithi efana nama-statin ukuze wehlise i-cholesterol futhi unciphise umonakalo ezinso zakho.
• Izinguquko ekudleni: Uchwepheshe wezokudla kwezinso uzokusiza ukuthi wakhe uhlelo lokudla olukufanele futhi olukusiza ukuthi ugcine isisindo esinempilo. Ukunciphisa ukudla kwakho usawoti (i-sodium), amafutha agcwele, kanye ne-cholesterol kungasiza ekunciphiseni ukulimala kwezinso.
• Ama-Corticosteroids: Aziwa nangokuthi "ama-steroids," lawa yimithi yokulwa nokuvuvukala esebenza ngokuvimba amasosha omzimba ekuhlaseleni ama-glomeruli akho.
• I-Eculizumab: Lona umuthi we-antibody we-monoclonal osetshenziselwa ukwelapha izifo zegazi ezingavamile. Ungasiza ekuthuthukiseni ukusebenza kwezinso ngokunciphisa amaprotheni emchameni nokwandisa izinga lokuhlunga kwe-glomerular kwezinye iziguli ze-C3G.
• I-Dialysis: Cishe abantu abangu-50% abane-C3G kuthiwa banenkinga yokwehluleka kwezinso. I-Dialysis yilapho izinso zihluleka khona, futhi umsebenzi okufanele wenziwe yizinso (ukuhlanza igazi, ukususa imfucuza, kanye noketshezi oluningi) wenziwa ngosizo lomshini.
• Ukufakelwa kwezinso: Lokhu kungenye indlela yokwelapha ukwehluleka kwezinso. Udokotela ohlinzayo ususa izinso zakho ezingasebenzi kahle bese ezifaka esikhundleni sazo izinso eziphilile ezivela kumnikeli.

Uma une-`DDD` noma i-`C3GN` futhi une-`monoclonal gammopathy`, udokotela wakho uzoncoma ukuhlolwa okwengeziwe ukuze ahlole ezinye izimo eziyisisekelo.

Iyini inhloso yesifo se-C3G?

Cishe ingxenye yeziguli ezine-C3G iba nokwehluleka kwezinso zingakapheli iminyaka eyi-10 zixilongiwe. Okwamanje akukho ikhambi lokwehluleka kwezinso. Kodwa-ke, ngokuxilongwa okunembile nokwelashwa okusheshayo, ungaphila impilo ende nenempilo ngaphandle kwezinguquko ezinkulu. Ngakho-ke, kubalulekile ukuhlala uzolile futhi ulandele iseluleko sikadokotela wakho.

Ingabe isifo se-C3G singavinjelwa?

Ngeshwa, okwamanje ayikho indlela yokuvimbela isifo se-C3G, njengoba sivame ukubangelwa yizinto ezingaphezu kwamandla ethu.

Ngizinakekela kanjani?

Uma une-C3G, udokotela wakho uzokwakha uhlelo lokwelashwa olungcono kakhulu kuwe. Lokhu kungafaka phakathi imithi, ukunakekelwa okusekelayo, kanye nezinguquko zendlela yokuphila. Uhlelo lwakho lokwelashwa lungafaka okulandelayo, futhi kubalulekile ukulandela lezi zinyathelo:

• Thatha umuthi wakho njengoba nje udokotela wakho ekuyalele. Ungaphuthelwa ngisho usuku olulodwa.
• Ukushintsha ukudla kwakho, ikakhulukazi ukunciphisa usawoti, uwoyela, noshukela, nokudla izithelo nemifino eminingi.
• Gwema ukusebenzisa imithi engeyona i-steroidal elwa nokuvuvukala (ama-NSAID) (isib., imithi yobuhlungu efana ne-ibuprofen ne-diclofenac) ngaphandle kweseluleko sikadokotela, ngoba ingalimaza izinso futhi isheshise ukwehluleka kwezinso.
• Ukunakekela impilo yakho yengqondo. Ukuba nesifo esingavamile kangaka kungakwenza uzizwe unesizungu futhi wesaba. Futhi, ukwazi ukuthi i-C3G ingabangela ukwehluleka kwezinso kwandisa ingozi yakho yokuthola ukucindezeleka. Khuluma nodokotela wakho ngempilo yakho yengqondo bese ucela ukwelulekwa nokwelashwa uma kudingeka. Buza udokotela wakho ngamaqembu okusekela abantu abane-C3G. Ukuhlangana nabanye abantu abane-C3G kungasiza ekunciphiseni isizungu nokwabelana ngolwazi.

Kufanele ngimbone nini udokotela?

Uma unezimpawu ze-C3G, ikakhulukazi izinguquko emchameni wakho (ogcwele igazi, onegwebu, omncane), ukuvuvukala, noma ukukhathala okukhulu, bona udokotela ngokushesha. Lokhu kungaba izimpawu zokuthi izinso zakho azisebenzi kahle.

Uma kutholakale ukuthi une-C3G, udokotela wakho uzokudlulisela kuchwepheshe wezifo zezinso (udokotela wezinso). Nguye ozokunikeza uhlelo lokwelapha olungcono kakhulu.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Uma ubona udokotela, ungangabazi ukubuza imibuzo efana nale:

• Ngingazi kanjani ukuthi ngine-C3G?
• Uma ngingenayo i-C3G, yisiphi esinye isifo engingaba naso?
• Uhlobo luni lwe-C3G enginalo (i-DDD noma i-C3GN)?
• Yikuphi ukwelashwa ongincomayo? Ingabe kukhona imiphumela emibi?
• Yiziphi izinguquko okufanele ngizenze ekudleni kwami?
• Ucabanga ukuthi izinso zami zizohluleka ukusebenza? Yini engingayenza ukuze ngivimbele lokho ukuthi kungenzeki?
• Ngingambona yini udokotela wezinzwa?

Ekugcineni, kufanele ngithi...

I-C3G yisifo esingavamile nesiyinkimbinkimbi esithinta amayunithi amancane okuhlunga ezinso. Uma une-C3G, ungase ubhekane nezinguquko emchameni wakho, ukuvuvukala, ubuhlungu bamalunga, izinkinga zokubona, kanye nokukhathala.

Ungase ube nemizwa ehlukahlukene njengoba wamukela lokhu kuxilongwa futhi ujwayela indlela i-C3G ezothinta ngayo impilo yakho. Ungakhathazeki, awuwedwa. Zinike isikhathi nendawo yokufunda nge-C3G kanye nezinketho zakho zokwelashwa. Ukufunda ngezinketho zakho nokuthi yini ongayilindela kungakusiza ukubhekana nemizwa yakho ngendlela enempilo nephumelelayo. Odokotela bakhona ukukunikeza ulwazi nesiqondiso. Uma unemibuzo, udinga iseluleko noma ukwesekwa, khuluma nabo. Khumbula, ngokuxilongwa kusenesikhathi kanye nokwelashwa okufanele, ungalawula lesi sifo futhi uphile impilo enhle.

I- C3G, isifo sezinso, i-complement 3, i-glomerulopathy, ukwehluleka kwezinso, iphrotheni yomchamo, i-dialysis