Ingabe le phrotheni ye-M iyinkinga nakwezinso zakho? (I-Monoclonal Gammopathy of Renal Significance - MGRS) Ake sixoxe ngalokhu!

Sonke siyazi ukuthi izinso zethu ziyisitho esibaluleke kakhulu esenza umsebenzi waso buthule. Kodwa-ke, ngezinye izikhathi lezi zinso zingagula buthule. Namuhla sizokhuluma ngesifo esibangela isifo sezinso, esingase sibonakale siyinkimbinkimbi uma uzwa igama, kodwa kulula ukuliqonda. Lokhu kubizwa ngokuthi i-MGRS (i-Monoclonal Gammopathy of Renal Significance) . Nakuba igama lide kancane, ungakhathazeki, masiliqonde kalula.

Kuyini i-MGRS kalula?

Kulungile, ake sibheke ukuthi iyini i-MGRS. Kalula nje, i-MGRS iyiqembu lezimo ezilimaza izinso. Lo monakalo uvela ngoba amanye amangqamuzana e-plasma emzimbeni wethu, noma amaseli e-B (lawa ayizinhlobo zamangqamuzana egazi amhlophe), enza amakhophi amaningi kakhulu awo, noma ama-clone . Cabanga ngakho njengomshini wokukopisha owenza amakhophi amaningi ephepha elifanayo.

Amaseli e-plasma akha ngale ndlela akhiqiza uhlobo lwe -antibody olubizwa ngokuthi i-M protein . Lawa maprotheni e-M ahlala ezithweni ezahlukahlukene zomzimba wethu, ikakhulukazi izinso, bese eqala ukuzilimaza.

Into ebalulekile ukuthi i-MGRS ayiwona umdlavuza. Kodwa-ke, inani elincane labantu abane-MGRS lingagcina lithole umdlavuza, njenge- multiple myeloma . Ngakho-ke kubalulekile ukuqaphela lokhu.

Ingabe zikhona izinhlobo ze-MGRS?

Yebo, kunezinhlobo ezingaphezu kweshumi nambili zezimo ze-MGRS. Lezi zihlukaniswa ngokwengxenye yezinso ethinta iphrotheni ye-M. Kunezinhlobo ezintathu eziyinhloko:

1. Izifo ze-Glomerular

Uhlobo oluvame kakhulu lwe-MGRS yi -glomerulopathies . Lokhu kulimaza imithambo yegazi emincane ngaphakathi kwezinso. Kufana nama-meshes esihlungini samanzi. Ezinye zezifo eziwela kulesi sigaba zifaka:

• I-amyloidosis yezinso. Lokhu kufaka phakathi izinhlobo ezibizwa ngokuthi i-AL amyloidosis, i-AH amyloidosis, kanye ne-AHL amyloidosis.
• I-glomerulonephritis ye-cryoglobulinemic (CG).
• I-Fibrillary glomerulopathy (FGN).
• I-immunotactoid glomerulopathy (ITG).
• Izifo ze-glomerular ezihlobene namaprotheni e-monoclonal. Lokhu kufaka phakathi izimo ezibizwa ngokuthi 'uchungechunge olukhanyayo' kanye 'nezifo zokubekwa kwe-heavy chain'.

2. Izifo ze-Tubulointerstitial

Lezi zifo zithinta amapayipi (amapayipi) athwala izakhamzimba kanye noketshezi oluhlungiwe yizinso lubuyele egazini, kanye nezicubu ezizungezile. Ezinye izibonelo zifaka:

• I-Fanconi syndrome yochungechunge olukhanyayo (LCFS).
• I-Light chain proximal tubulopathy (LCPT) engenamakristalu.
• I-histiocytosis egcina amakristalu.

3. Izimo ezithinta imithambo yegazi ngaphakathi kwezinso (izilonda zemithambo yegazi ngaphakathi kwezinso)

Lezi zimo zithinta imithambo yegazi ezinso. Isibonelo yi- atypical hemolytic uremic syndrome (aHUS) . Lokhu kwenzeka lapho imithambo yegazi emincane ezinso ivinjelwa amahlule egazi.

Ivame kangakanani i-MGRS?

I-MGRS empeleni iyisimo esingavamile kakhulu . Kulinganiselwa ukuthi ithinta abantu abangaphansi kuka-1%. Ivame kakhulu kubantu abaneminyaka engaphezu kuka-60 .

Ziyini izimpawu ze-MGRS?

Izimpawu ze-MGRS zingahluka kuye ngohlobo lwe-MGRS onayo. Ezinye izimpawu ezivamile zifaka:

• Ukuzizwa ukhathele kakhulu (ukukhathala) .
• Ukuvuvukala emilenzeni engezansi, emaqakaleni, noma ezinyaweni. Ngezinye izikhathi isisu, izandla, noma ubuso nakho kungavuvukala.
• I-Nephrotic syndrome iyenzeka. Lokhu kubonakala ngokukhishwa kwamaprotheni emchameni kanye namazinga aphezulu e-cholesterol egazini.
• Umchamo onegwebu noma onensipho . Lokhu kungaba uphawu lwephrotheni emchameni (i-proteinuria).
• Ukuchama njalo kuyisidingo sokuchama njalo.
• Igazi emchameni (i-hematuria) noma umchamo ophenduka ube pinki.
• Isicanucanu nokuhlanza.
• I-POEMS syndrome . Lesi futhi yisimo esingavamile.
• Ukuba ndikindiki, ukuqaqamba, noma ubuthakathaka ezithweni (lezi yizimpawu ze-neuropathy).
• Ukushintsha kombala wesikhumba (amabala akhanyayo noma amnyama).
• Ubunzima bokuphefumula (ukuphelelwa umoya).

Yini ebangela i-MGRS?

Imbangela eyinhloko ye-MGRS ukulimala kwezinso okubangelwa amaprotheni e-M ashiwo ngenhla. Amaprotheni e-M awuhlobo lwe-antibody olungasebenzi kahle, futhi olukhiqizwa ngendlela engafanele. Enziwa ngama-clones (amakhophi) amaseli e-plasma alimele, uhlobo lweseli elimhlophe legazi, noma amangqamuzana e-B.

Abanye abantu bangaba nala ma-clone amaseli e-plasma enza amaprotheni e-M ngaphandle kokulimaza noma yiziphi izitho zomzimba. Lesi simo sibizwa ngokuthi i-Monoclonal Gammopathy of Undetermined Significance (MGUS) . Kodwa-ke, kubantu abane-MGRS, lawa maprotheni e-M ayakhelana ezinso futhi azilimaze.

Iyini imbangela eyinhloko ye-MGRS?

Ochwepheshe abakakazi kahle ukuthi yini ebangela i-MGRS. Izinkinga zokuzivikela komzimba , izinguquko zofuzo , izifo, kanye nezici zemvelo konke kucatshangwa ukuthi kunomthelela kulokhu.

Yiziphi izinkinga ezingaba khona ngenxa ye-MGRS?

Lawa ma-clone e-plasma cell kanye ne-MGRS angabangela ukulimala kwezinso, okungaholela ezinkingeni ezinkulu. Ezinye zalezi zifo zifaka:

• Isifo sezinso esingamahlalakhona (CKD) .
• Isifo sezinso (izinso) sesigaba sokugcina .
• Ukwehluleka kwezinso .
• Ukuthuthuka kube ezinye izifo. Kwabanye abantu, inani lama-clone e-plasma cell liyanda kakhulu kangangokuthi lingakhula libe uhlobo lomdlavuza wamangqamuzana amhlophe egazi obizwa ngokuthi i-multiple myeloma (MM) . I-MGRS ingakhula ibe eminye imidlavuza, njenge- Waldenström macroglobulinemia , i-chronic lymphocytic leukemia (CLL) , noma i-B-cell lymphoma .

I-MGRS ihlolwa kanjani?

Udokotela angaqinisekisa i-MGRS ngokwenza i-biopsy yezinso . Lokhu kuhilela ukuthatha ingxenye encane yezicubu ezinso lakho bese eyihlola iphrotheni ye-M. Bangase futhi bahlole amasampula egazi lakho, umchamo, noma umongo wamathambo.

Ingabe ikhona indlela yokwelapha i-MGRS?

Yebo, kunezindlela zokwelapha i-MGRS. Odokotela bayayelapha ngemithi efana ne -chemotherapy yomdlavuza. Le mithi ibulala amangqamuzana anenkinga noma iwavimbe ekwenzeni amakhophi engeziwe. Eminye imithi ingasiza ekunciphiseni ukuvuvukala. Umgomo oyinhloko wokwelashwa ukugcina izinso zakho zisebenza futhi uzivimbele ekulimaleni.

Kwezinye izimo ze-MGRS, odokotela bangase benze nokufakelwa kwe-stem cell . Lokhu kushintsha amaseli akho alimele ngamaseli anempilo. Uma izinso zakho zonakele kakhulu, ungase udinge ne-dialysis noma ukufakelwa kwezinso .

Imithi ethile kanye nokwelashwa okusetshenziselwa i-MGRS

Uhlobo lomuthi owunikezwayo luzoncika ohlotsheni lwe-MGRS onayo. Nazi ezinye izindlela zokwelapha:

• Ama-antibodies e-monoclonal - isib. i-rituximab, i-daratumumab.
• Ama-proteasome inhibitors - isib. i-bortezomib, i-carfilzomib, i-ixazomib.
• Imithi yokwelapha umzimba - isib. i-thalidomide, i-lenalidomide, i-pomalidomide.
• Ama-alkylating agents - isibonelo, i-cyclophosphamide, i-bendamustine, i-melphalan.
• Ama-corticosteroids - isibonelo i-prednisone, i-dexamethasone.
• Ukufakelwa kweseli le-stem le-Autologous.

Iyini imiphumela emibi yokwelashwa?

Ukwelashwa okufana ne-chemotherapy kungabangela imiphumela eminingi engemihle, ngezinye izikhathi ngisho nokulimala kwezitho zomzimba. Yingakho odokotela bekhetha ukwelashwa ngokucophelela ukuze bathole inzuzo enkulu kukho futhi banciphise ingozi yemiphumela emibi kakhulu. Eminye yemiphumela emibi ongase ube nayo ihlanganisa:

• I-anemia ( ukuntuleka kwegazi).
• Ukopha.
• Ukuqunjelwa.
• Uhudo.
• Ngikhathele.
• Ingozi eyengeziwe yokutheleleka ngamagciwane, amagciwane kanye nesikhunta.
• Ukudla akunambitheki.
• Isicanucanu nokuhlanza.

Ingabe i-MGRS ingavinjelwa?

Ngenxa yokuthi asazi kahle ukuthi yini ebangela i-MGRS, okwamanje ayikho indlela ethile yokuyivimbela.

Yini ongayilindela uma une-MGRS?

Nakuba i-MGRS iyisimo esingelapheka, ngokuvamile ayipheli ngokuphelele. Ingaphinde ivele ngemva kokwelashwa. Uma izinso zakho zonakele kakhulu, ungase udinge ukufakelwa izinso.

Cishe abantu abangu-18% abane-MGRS kamuva bazoba nomdlavuza obizwa ngokuthi i-multiple myeloma. Ngakho-ke, udokotela wakho uzokuqapha njalo ngamasampula egazi nomchamo, njengoba lokhu kungatholakala kusenesikhathi uma kwenzeka.

Ungaphila isikhathi esingakanani ne-MGRS?

Ukuthi ungaphila isikhathi esingakanani ne-MGRS kuncike ohlotsheni lwe-MGRS onayo, ubukhali bayo, nokuthi usabela kanjani ekwelashweni. Abantu abanomonakalo omncane wezinso banombono ongcono kunabantu abanomonakalo omkhulu. Isibonelo, kolunye ucwaningo, abantu abane-amyloidosis ehlotshaniswa ne-MGRS baphila iminyaka engaba yi-10 ngemva kokuxilongwa. Kodwa abantu abangenayo i-amyloidosis baphila isikhathi eside kakhulu. Futhi, abantu abasabela kahle ekwelashweni banemiphumela emihle kakhulu.

Ngizinakekela kanjani?

Qiniseka ukuthi ulandela noma yiziphi izincomo udokotela wakho akunikeza zona zokunakekela impilo yezinso zakho ekhaya. Lokhu kungafaka phakathi ukunciphisa izinto ezifana notshwala, nokulawula izici eziyingozi zesifo sezinso, njengomfutho wegazi ophakeme nesifo sikashukela. Nakuba lezi zinto zingeke zelaphe ngqo i-MGRS, zingasiza ekunciphiseni ingozi yokulimala okuqhubekayo kwezinso. Udokotela wakho angakusiza futhi ukuthi uphathe imiphumela emibi yokwelashwa.

Kufanele ngimbone nini udokotela?

Uma unezimpawu zenkinga yezinso, njengokuvuvukala, umchamo onegwebu noma opinki, ukuchama njalo, noma ukuchama okuncane kakhulu, qiniseka ukuthi ubona udokotela.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Kungaba usizo ukubuza udokotela wakho le mibuzo:

• Yiziphi izindlela zokwelapha ezitholakalayo zohlobo lwe-MGRS enginalo?
• Yini engingayenza ukuze ngivimbele ukulimala kwezinso?
• Yiziphi izimpawu ezintsha noma eziya ziba zimbi okufanele ngiziqaphele?

Okokugcina, into ebaluleke kakhulu (Umyalezo Wokuya Ekhaya)

I-MGRS yisimo esibi kakhulu esingalimaza izinso ngendlela engaba yingozi empilweni. Kodwa khumbula, akuwona wonke umuntu one-MGRS ozoba nomdlavuza. Udokotela wakho uzokuqapha njalo futhi athathe izinyathelo zokuhlonza nokwelapha noma yiziphi izinkinga ezintsha ngokushesha okukhulu . Ukwelashwa okusha sekwenze kwaba nokwenzeka ukuthuthukisa ikhwalithi yempilo yakho nokunciphisa ingozi yakho yezifo ezingathi sína. Ngakho-ke ungesabi, landela iseluleko sikadokotela wakho, futhi ubhekane nalesi simo.

` I-MGRS, i-Monoclonal Gammopathy yokubaluleka kwezinso, Isifo sezinso, Amaprotheni e-M, Amaseli e-Plasma, i-Kidney Biopsy, i-Nephrotic Syndrome, i-Multiple Myeloma