Singathetha ngesifo esingaqhelekanga esibizwa ngokuba yi-C3G (Complement 3 Glomerulopathy), esichaphazela izintso?

Ngaba ngamanye amaxesha uva igazi kumchamo wakho, okanye ukudumba emilenzeni yakho? Okanye uziva udiniwe ngalo lonke ixesha? Oku ngamanye amaxesha kunokubangela ingxaki kwizintso zakho. Namhlanje siza kuthetha ngemeko engaqhelekanga kodwa ebaluleke kakhulu echaphazela izintso. Oko kukuthi i-C3G (C Three G) okanye i-``Complement 3 Glomerulopathy``.

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

Ngamafutshane, i-C3G liqela lezifo ezichaphazela indlela ezisebenza ngayo izintso zakho. Uyazi, izintso zifana nezihluzo ezibini emizimbeni yethu. Umsebenzi wazo ophambili kukucoca igazi, ukususa inkunkuma engeyomfuneko kunye namanzi agqithisileyo, nokwenza umchamo.

I-"Complement 3" yiproteni ekhethekileyo egazini lethu. Ibaluleke kakhulu kwinkqubo yomzimba yokulwa nezifo emizimbeni yethu. I-"Glomerulopathy" kukonakala kwe-glomeruli, eziyimithambo yegazi emincinci ngaphakathi kwezintso, efana nee-meshes ezincinci. Ezi glomeruli zezona zihluza igazi.

Emva koko i-C3G sisifo esibandakanya iproteni ethi `complement 3`, eyonakalisa i-`glomeruli` kwaye iphazamise ukusebenza kwezintso. Ngaphambili, oko kukuthi, ngaphambi kowama-2013, oogqirha babebiza le ``Membranoproliferative glomerulonephritis (MPGN) uhlobo I, II okanye III`` okanye ``Mesangioproliferative glomerulonephritis``. Kodwa ngoku olu qela lwezifo lubizwa ngokuba yi-C3G.

Ngaba kukho iintlobo ze-C3G?

Ewe, zimbini iintlobo eziphambili ze-C3G. Oogqirha bafumanisa ezi ntlobo zimbini ngokuthatha iqhekeza elincinci lezintso (silibiza ngokuba yi-'kidney biopsy' ) baze balijonge phantsi kwe-microscope, ngokusekelwe kumonakalo kunye nokudumba kwe-'glomeruli'.

• Isifo seDense deposit (DDD): Ngaphambili esaziwa ngokuba yiMPGN type II, esi sifo sibonakala ngokwenziwa kwee-dense, ezifana neribhoni kwi-glomerular basement membrane (GBM), ulwelo lwe-glomeruli. Sidla ngokuchaphazela abantwana kunye nabantu abadala abaselula abakwiminyaka yabo yama-20.

• `C3 glomerulonephritis` (C3GN): Ngaphambili yayibizwa ngokuba yi-`MPGN type I` okanye `III`. Kule meko, iindawo ezixineneyo ezifana nezo zibonwa kwi-DDD azibonakali. Oku kuchaphazela kakhulu abantu abangaphezu kweminyaka engama-30 ubudala.

Ngaba i-C3G sisifo esingapheliyo?

Ewe, i-C3G sisifo esingapheliyo. Oku kuthetha ukuba asiyonto eza kuvela ngequbuliso ize ibe ngcono, kwaye umonakalo kwizintso unokwanda ngokuhamba kwexesha. Ke ngoko, kubaluleke kakhulu ukuba uqaphele oku.

Ixhaphake kangakanani le C3G?

Esi sisifo esingaqhelekanga ngokwenene.Oogqirha baqikelela ukuba oku kwenzeka kubantu ababini ukuya kwabathathu kwikhulu lamawaka. Ngoko ke kusenokwenzeka ukuba awuzange uve okuninzi ngako. Kodwa nangona kunqabile, kubalulekile ukuba uqaphele.

Zithini iimpawu zesifo se-C3G?

Ngoku makhe sibone ukuba zeziphi iimpawu zesi sifo se-C3G. Zombini ezi ntlobo zineempawu ezininzi ezifanayo, ezibalulekileyo ukuziqonda:

• Igazi kumchamo ( i-hematuria ): Ngamanye amaxesha umchamo ungabonakala ubomvu, okanye usenokuba ngumlinganiselo omncinci onokubonwa kuphela nge-microscope.
• Iprotheyini engaphezulu kumchamo (i-proteinuria): Oku kunokubangela ukuba umchamo ubonakale ngathi unamagwebu.
• Ukuchama kancinci kunesiqhelo (i-oliguria).
• Ukwehla kwamanqanaba eeprotheyini egazini (hypoalbuminemia).
• Ukudumba (i-edema): Ingakumbi ezandleni, emaqatheni nasezinyaweni. Khawucinge nje, ukuba uvuka kusasa iinyawo zakho ziziva zidumbile, inokuba yinto efana nale.
• Uxinzelelo lwegazi oluphezulu (uxinzelelo lwegazi oluphezulu).
• Usulelo oluphindaphindayo.
• Ukudinwa kakhulu (`Ukudinwa`): Akupheleli nje ekudinweni, kodwa kukuziva udiniwe nokuba ulala kangakanani na.
• Abanye abantu banokuba neemeko ezifana ne -gout .

Zombini ezi mpawu zinokubangela ukungasebenzi kakuhle kwezintso . Ukungasebenzi kakuhle kwezintso kunokubangela iimpawu ezifana nezi:

• Ndihlala ndidiniwe.
• Isicaphucaphu, ukuhlanza.
• Ubunzima bokwenza isigqibo, ukuphelelwa lithemba.
• Ukudumba komzimba.
• Ukuchama kakhulu kunesiqhelo (oku ngamanye amaxesha kunokubonwa kwizigaba zokuqala zokungasebenzi kakuhle kwezintso).

Ukongeza kwizintso, i-C3G inokubangela nezinye iimpawu. Ezi ziquka:

• Iingxaki zokubona: Ukubona kunokuphazamiseka ngenxa yokuqokelelwa kweeproteni kunye ne-calcium deposits (drusen), ingakumbi kwi-macula.
• I-Lipodystrophy: Le yimeko apho amafutha alahleka kwiindawo ezithile zomzimba (ingakumbi ebusweni nakwimilenze).

Kutheni le C3G yenziwe?

Kulungile, ngoku makhe sibone ukuba kutheni le C3G yenziwe. Oku kunzima kancinci, kodwa ndiza kukuchaza ngokulula. Oku kwenzeka xa ``inkqubo yokuncedisa`` emizimbeni yethu ingasebenzi kakuhle. Le ``nkqubo yokuncedisa`` yingqokelela yeeproteni egazini lethu. Le yinxalenye yenkqubo yethu yokuzikhusela. Oku kunceda ukulwa nezinto ezifana neebhaktheriya kunye neentsholongwane ezivela ngaphandle.

Okwenzekayo kumntu one-C3G kukuba le `nkqubo yokuncedisa` iyasebenza kakhulu, okanye ayilawuleki. Emva koko iproteni ye-`complement 3 (C3)` yonakele. Ezi ziqwenga zeproteni ye-C3 ezonakeleyo zinamathela kwi-`glomeruli`. Emva koko kuba nzima kwi-`glomeruli` ukucoca igazi nokwenza umchamo. Ukuba ayinyangwa, lo monakalo unokuqhubeka kwaye ukhokelele ekungasebenzini kwezintso.

Amaxesha amaninzi, kunzima ukuchonga isizathu esichanekileyo sokuba le "nkqubo yokuncoma" ingabi nantsingiselo. Kodwa ngamanye amaxesha:

• I-C3G inokukhula ngenxa yotshintsho kwiijini zethu (iinguqu kwiijini ezinxulumene nenkqubo yokongeza, njenge-``C3`` kunye ne-``CFH```).
• Isifo segazi esibizwa ngokuba yi-``Monoclonal gammopathy`` sinokonakalisa izintso ngokungathanga ngqo, siphazamise ukusebenza kwenkqubo ye-``complement``, kwaye sibangele ukwakheka kwe-C3G. Le ``monoclonal gammopathy`` yimeko echaphazela iiseli zeplasma kwithambo lethu.
• Abaphandi bezonyango bacinga ukuba into ebizwa ngokuba yi-"autoantibodies" nayo inokudlala indima. "Autoantibodies" ziiproteni eziveliswa yinkqubo yethu yokuzikhusela komzimba ezihlasela ngempazamo izicubu zethu eziphilileyo. Nangona kunjalo, akukho phando lwaneleyo lokutsho ngokuqinisekileyo ukuba ezi "autoantibodies" ziyimbangela ye-C3G.

Ngubani ochaphazeleka kakhulu sesi sifo se-C3G?

Esi sifo se-C3G sinokuvela nakubani na. Sichaphazela amadoda nabafazi ngokulinganayo. Ubudala obuqhelekileyo bokuxilongwa bumalunga neminyaka engama-23. Nangona kunjalo, uhlobo lwe-`DDD` esilukhankanyileyo ngaphambili lunokubonakala lusemncinci kunohlobo lwe-`C3GN`.

Ziziphi iingxaki ezinokubakho ze-C3G?

Eyona ngxaki iphambili neyona iyingozi kukuba malunga nama-50% abantu abane-C3G, okanye omnye kwabababini, baya kukhubazeka kwezintso kwiminyaka eli-10 emva kokufunyaniswa ukuba banayo le ngxaki. Le yinto esifanele siyoyike kakhulu.

Isifo se-C3G sifunyanwa njani?

Ugqirha uza kwenza uvavanyo oluninzi ukujonga impilo yezintso zakho kunye nokuba zisebenza kakuhle kangakanani na. Ezi zingabandakanya:

• Uvavanyo lwegazi: Kuthathwa isampuli yegazi encinci kumthambo osengalweni yakho kwaye ilebhu ihlola ukuba iiproteni zakho ezincedisayo ziqhelekile na okanye kukho inkunkuma engaphezulu (umz. i-creatinine) egazini lakho.
• `Izinga lokuHluza eliQikelelweyo leGlomerular (eGFR): Olu luvavanyo olulinganisa indlela izintso zakho ezihluza ngayo igazi lakho, ngokusekelwe kubungakanani be-`serum creatinine` okanye `cystatin C` kwisampulu yegazi. Oku kunokukunika umbono olungileyo wokuba izintso zakho zisebenza kakuhle kangakanani.
• Uhlalutyo lomchamo: Kuya kufuneka uchamele kwikomityi ekhethekileyo uze unike ugqirha wakho isampuli. Ilebhu iya kujonga iproteni okanye igazi kumchamo, elinokubonwa kuphela ngemakroskopu.
• I-biopsy yezintso: Yile ntoEyona ndlela ilungileyo yokufumanisa isifo se-C3G ngokuyi-biopsy. Kule nto, ugqirha uthatha iqhekeza elincinci lesicubu sezintso zakho esebenzisa inaliti encinci okanye umngxuma omncinci aze alivavanye kwilebhu. Ukuba le sampulu iqulethe iziqwenga zeprotheyini ye-C3 eyonakeleyo kwi-glomeruli yakho, isenokuba sisifo se-C3G. Le biopsy inokukuxelela nokuba loluphi uhlobo lwe-C3G onalo (i-DDD okanye i-C3GN), ngokusekelwe ekubeni unazo na ezo depositi ziqinileyo okanye awunazo.

Ngaba i-C3G inganyangeka ngokupheleleyo?

Ngelishwa, okwangoku akukho nyango lwesifo se-C3G. Kodwa ungakhathazeki, oogqirha nabaphandi bayaqhubeka nokufunda esi sifo kwaye basebenza ukufumana unyango olutsha. Sinethemba lokuba unyango olungcono luya kuza kwixesha elizayo.

Ziziphi iindlela zonyango ze-C3G?

Okwangoku akukho nyango luthile oluvunyiweyo yi-U.S. Food and Drug Administration (FDA) ye-C3G. Nangona kunjalo, oogqirha banokunika uluhlu lweendlela zonyango kunye nonyango oluxhasayo ukuze kuncitshiswe ukuqhubela phambili kwe-C3G, kulawulwe iimpawu, kwaye kuncitshiswe umonakalo wezintso. Ezi ziquka:

• Amayeza oxinzelelo lwegazi: Amayeza afana ne-angiotensin-converting enzyme (ACE) inhibitors kunye ne-angiotensin II receptor blockers (ARBs) anciphisa uxinzelelo ngaphakathi kwi-glomeruli. Oku kunciphisa ubungakanani beproteni kumchamo kwaye kunciphisa uxinzelelo kwizintso.
• Amayeza okwehlisa i-cholesterol: Ugqirha wakho unokukunika amayeza afana nee-statins ukunciphisa i-cholesterol nokunciphisa umonakalo kwizintso zakho.
• Utshintsho kwindlela yokutya: Ingcali yokutya kwezintso iya kukunceda uphuhlise isicwangciso sokutya esifanelekileyo kuwe kwaye sikuncede ugcine ubunzima obusempilweni. Ukunciphisa ukutya kwakho ityuwa (i-sodium), amafutha agcweleyo, kunye ne-cholesterol kunokunceda ukunciphisa umonakalo wezintso.
• IiCorticosteroids: Ezi zikwaziwa ngokuba zii-"steroids," ezi ziziyobisi ezilwa nokuvuvukala ezisebenza ngokuthintela inkqubo yomzimba yokuzikhusela ekuhlaseleni i-glomeruli yakho.
• I-Eculizumab: Eli liyeza le-antibody elisetyenziswa ukunyanga iingxaki zegazi ezingaqhelekanga. Linokunceda ukuphucula ukusebenza kwezintso ngokunciphisa iiproteni kumchamo kunye nokunyusa izinga lokuhluza i-glomerular kwezinye izigulane ze-C3G.
• I-Dialysis: Malunga nama-50% abantu abane-C3G kuthiwa bane-kidney failure. I-Dialysis kuxa izintso zingasebenzi, kwaye umsebenzi omele wenziwe zizintso (ukucoca igazi, ukususa inkunkuma, kunye nolwelo olugqithisileyo) wenziwa ngoncedo lomatshini.
• Ukufakelwa kwezintso: Olu lolunye unyango lokungasebenzi kakuhle kwezintso. Ugqirha wotyando ususa izintso zakho ezingasebenzi kakuhle aze azifake endaweni yazo izintso eziphilileyo ezivela kumntu onikelayo.

Ukuba une-`DDD` okanye `C3GN` kwaye une-`monoclonal gammopathy`, ugqirha wakho uya kucebisa uvavanyo olongezelelweyo ukuze ajonge ezinye iimeko ezingaphantsi.

Iyintoni imbonakalo yesifo se-C3G?

Malunga nesiqingatha sezigulane ezine-C3G ziba nokusilela kwezintso kwiminyaka eli-10 emva kokufunyaniswa kwazo. Okwangoku akukho nyango lokusilela kwezintso. Nangona kunjalo, ngokuxilongwa ngokuchanekileyo kunye nonyango olukhawulezileyo, ungaphila ubomi obude nobunempilo ngaphandle kotshintsho olukhulu. Ke ngoko, kubalulekile ukuhlala uzolile kwaye ulandele iingcebiso zikagqirha wakho.

Ngaba isifo se-C3G singathintelwa?

Ngelishwa, okwangoku akukho ndlela yokuthintela isifo se-C3G, njengoko sidla ngokubangelwa zizinto ezingaphaya kwamandla ethu.

Ndingazinyamekela njani?

Ukuba une-C3G, ugqirha wakho uza kuyila isicwangciso sonyango esifanelekileyo kuwe. Oku kungabandakanya amayeza, unyango oluxhasayo, kunye notshintsho kwindlela yokuphila. Isicwangciso sakho sonyango singabandakanya oku kulandelayo, kwaye kubalulekile ukulandela la manyathelo:

• Sela amayeza akho njengoko ugqirha wakho ekuyalele. Ungaphoswa nalusuku olunye.
• Ukutshintsha indlela otya ngayo, ingakumbi ukunciphisa ityuwa, ioyile, kunye neswekile, kunye nokutya iziqhamo kunye nemifuno emininzi.
• Kuphephe ukusebenzisa amayeza angengo-steroidal anti-inflammatory (NSAIDs) (umz., iintlungu ezifana ne-ibuprofen kunye ne-diclofenac) ngaphandle kwengcebiso kagqirha, kuba anokonakalisa izintso kwaye akhawulezise ukungasebenzi kakuhle kwezintso.
• Ukunyamekela impilo yakho yengqondo. Ukuba nesifo esingaqhelekanga kunokukwenza uzive unesizungu kwaye woyika. Kwakhona, ukwazi ukuba i-C3G inokubangela ukungasebenzi kakuhle kwezintso kwandisa umngcipheko wokuba noxinzelelo. Thetha nogqirha wakho malunga nempilo yakho yengqondo kwaye ufumane iingcebiso kunye nonyango ukuba kuyimfuneko. Buza ugqirha wakho malunga namaqela enkxaso kubantu abane-C3G. Ukudibana nabanye abantu abane-C3G kunokunceda ukunciphisa isizungu kunye nokwabelana ngamava.

Ndifanele ndimbone nini ugqirha?

Ukuba uneempawu ze-C3G, ingakumbi utshintsho kumchamo wakho (onegazi, onamagwebu, ongaphantsi), ukudumba, okanye ukudinwa kakhulu, bona ugqirha ngoko nangoko. Ezi zinokuba ziimpawu zokuba izintso zakho azisebenzi kakuhle.

Ukuba ufunyaniswe une-C3G, ugqirha wakho uza kukuthumela kwingcali yezifo zezintso (ingcali ye-nephrologist). Nguye oza kukunika isicwangciso sonyango esifanelekileyo.

Ndingayibuza yiphi imibuzo ugqirha wam?

Xa ubona ugqirha, ungathandabuzi ukubuza imibuzo efana nale:

• Ndingaqiniseka njani ukuba ndine-C3G?
• Ukuba andinayo i-C3G, sesiphi esinye isifo endinokuba naso?
• Loluphi uhlobo lwe-C3G endinalo (i-DDD okanye i-C3GN)?
• Loluphi unyango olundicebisayo? Ngaba kukho naziphi na iziphumo ebezingalindelekanga?
• Zeziphi iinguqu endifanele ndizenze kwindlela enditya ngayo?
• Ucinga ukuba izintso zam ziya kungasebenzi? Ndingenza ntoni ukuthintela oko ukuba kungenzeki?
• Ndingambona ugqirha wezifo zengqondo?

Okokugqibela, ndimele ndithi...

I-C3G sisifo esingaqhelekanga nesintsonkothileyo esichaphazela iiyunithi ezincinci zokucoca kwizintso. Ukuba une-C3G, unokufumana utshintsho kumchamo wakho, ukudumba, iintlungu zamalungu, iingxaki zokubona, kunye nokudinwa.

Usenokuba neemvakalelo ezahlukeneyo njengoko usamkela esi sifo kwaye uhlengahlengisa indlela i-C3G eza kuyichaphazela ngayo ubomi bakho. Ungakhathazeki, awuwedwa. Zinike ixesha nendawo yokufunda nge-C3G kunye neendlela zonyango lwakho. Ukufunda ngeendlela zakho kunye noko unokukulindela kunokukunceda ujongane neemvakalelo zakho ngendlela enempilo nenemveliso. Oogqirha bakhona ukuze banike ulwazi kunye nesikhokelo. Ukuba unemibuzo, ufuna iingcebiso okanye inkxaso, thetha nabo. Khumbula, ngokuxilonga kwangoko kunye nonyango olufanelekileyo, ungalawula esi sifo kwaye uphile ubomi obuhle.

I- C3G, isifo sezintso, i-complement 3, i-glomerulopathy, ukungasebenzi kakuhle kwezintso, iproteni yomchamo, i-dialysis