Isifo seMedullary Cystic

-Delile

• Izinhlobo ze-MCKD
• Izimbangela ze-MCKD
• Izimpawu ze-MCKD
• Ukuhlola nokuhlonza i-MCKD
• Qedela ukubalwa kwegazi
• Ukuhlolwa kwe-BUN
• Ukuqoqwa komchamo
• Ukuhlolwa kwegazi kwe-creatinine
• Uric acid ukuhlolwa
• Ukuhlolwa komchamo
• Ukuhlola ukuhlolwa
• Isidumbu
• Yelashwa kanjani i-MCKD?
• Izinkinga zesikhathi eside ze-MCKD
• Uyini umbono we-MCKD?

Siyini isifo se-medullary cystic kidney?

Isifo sezinso seMedullary cystic (MCKD) yisimo esingajwayelekile lapho amasaka amancane, agcwele uketshezi abizwa ngama-cysts enkabeni yezinso. Ukuqubuka kwenzeka nakuwo amashubhu ezinso. Umchamo uhamba kuma-tubules kusuka ezinso nangohlelo lokuchama. Isibazi sibangela ukuthi la ma-tubules angasebenzi kahle.

Ukuze uqonde i-MCKD, kuyasiza ukwazi kancane ngezinso zakho nokuthi zenzani. Izinso zakho izitho ezimbili ezimise ubhontshisi ezilingana nesibhakela esivaliwe. Zitholakala ngapha nangapha komgogodla wakho, eduze naphakathi komhlane wakho.

Izinso zakho ziyahlunga futhi zihlanze igazi lakho - nsuku zonke, cishe amalitha wegazi angama-200 angena ezinso zakho. Igazi elihlanzekile libuyela esimisweni sakho sokujikeleza kwegazi. Imikhiqizo kadoti noketshezi oluthe xaxa kuba umchamo. Umchamo uthunyelwa esinyeni futhi ekugcineni ususwe emzimbeni wakho.

Umonakalo odalwe yi-MCKD uholela ekutheni izinso zikhiqize umchamo ongagxilile ngokwanele. Ngamanye amagama, umchamo wakho unamanzi kakhulu futhi awunalo inani elifanele lokungcola. Ngenxa yalokhu, uzogcina uchama ngendlela engamanzi kakhulu kunokujwayelekile (i-polyuria) njengoba umzimba wakho uzama ukukhipha yonke imfucuza eyengeziwe. Futhi lapho izinso zikhiqiza umchamo omningi kakhulu, khona-ke amanzi, isodiyamu, namanye amakhemikhali abalulekile alahleka.

Ngokuhamba kwesikhathi, i-MCKD ingaholela ekuhlulekeni kwezinso.

Izinhlobo ze-MCKD

I-juvenile nephronophthisis (NPH) ne-MCKD zihlobene kakhulu. Zombili lezi zimo zidalwa wuhlobo olufanayo lokulimala kwezinso futhi kuholele kwizimpawu ezifanayo.

Umehluko omkhulu yiminyaka yokuqala. I-NPH ivame ukuvela phakathi kweminyaka yobudala eyi-10 kuye kwengama-20, kuyilapho i-MCKD isifo sokuqala kwabantu abadala.

Ngaphezu kwalokho, kukhona ama-subsets amabili e-MCKD: uhlobo lwesibili (ngokujwayelekile luthinta abantu abadala abaneminyaka engama-30 kuya kwengama-35) bese uthayipha u-1 (uvamise ukuthinta abantu abadala abaneminyaka engama-60 kuya kwengama-65).

Izimbangela ze-MCKD

Kokubili i-NPH ne-MCKD yizimo ezivelele kakhulu zofuzo. Lokhu kusho ukuthi udinga kuphela ukuthola isakhi kusuka kumzali oyedwa ukuze uthuthukise lesi sifo. Uma umzali enofuzo, ingane inethuba elingamaphesenti angama-50 lokulithola nokuthuthukisa isimo.

Ngaphandle kweminyaka yokuqala, omunye umehluko omkhulu phakathi kwe-NPH ne-MCKD ukuthi ubangelwa ukukhubazeka okuhlukile kofuzo.

Ngenkathi sigxile kwi-MCKD lapha, okuningi esikuxoxayo kuyasebenza naku-NPH.

Izimpawu ze-MCKD

Izimpawu ze-MCKD zibukeka njengezimpawu zezinye izimo eziningi, okwenza kube nzima ukwenza ukuxilongwa. Lezi zimpawu zifaka:

• ukuchama ngokweqile
• imvamisa eyandayo yokuchama ebusuku (nocturia)
• umfutho wegazi ophansi
• ubuthakathaka
• izifiso zikasawoti (ngenxa yokulahleka ngokweqile kwe-sodium kusuka ekuchameni okwandisiwe)

Njengoba lesi sifo siqhubeka, ukwehluleka kwezinso (okwaziwa nangokuthi isifo sezinso sokugcina) kungahle kuvele. Izimpawu zokwehluleka kwezinso zingafaka okulandelayo:

• ukulinyazwa noma ukopha
• ukukhathala kalula
• ama-hiccups ajwayelekile
• ikhanda
• izinguquko kumbala wesikhumba (ophuzi noma onsundu)
• ukulunywa kwesikhumba
• ukuqunjelwa kwemisipha noma ukudikiza
• isicanucanu
• ukulahlekelwa umuzwa ezandleni noma ezinyaweni
• ukuhlanza igazi
• indle enegazi
• ukuncipha komzimba
• ubuthakathaka
• isithuthwane
• izinguquko esimweni sengqondo (ukudideka noma ukuqapha okushintshiwe)
• ikhoma

Ukuhlola nokuhlonza i-MCKD

Uma unezimpawu ze-MCKD, udokotela wakho angalawula izivivinyo eziningi ezahlukahlukene ukuqinisekisa ukutholakala kwakho. Ukuhlolwa kwegazi nomchamo kuzoba okubaluleke kakhulu ekuboneni i-MCKD.

Qedela ukubalwa kwegazi

Isibalo segazi esiphelele sibheka izinombolo zakho zizonke zamaseli abomvu egazi, amangqamuzana amhlophe egazi, nama-platelets. Lokhu kuhlola kubheka i-anemia nezimpawu zokutheleleka.

Ukuhlolwa kwe-BUN

Ukuhlolwa kwegazi urea nitrogen (BUN) kubheka inani le-urea, umkhiqizo wokuwohloka kwamaprotheni, ophakanyiswayo lapho izinso zingasebenzi kahle.

Ukuqoqwa komchamo

Ukuqoqwa komchamo wamahora angama-24 kuzoqinisekisa ukuchama ngokweqile, kubhalwe phansi ivolumu kanye nokulahleka kwama-electrolyte, futhi kukalwe imvume ye-creatinine. Ukuvunyelwa kwe-creatinine kuzoveza ukuthi izinso zisebenza kahle yini.

Ukuhlolwa kwegazi kwe-creatinine

Kuzokwenziwa ukuhlolwa kwe-creatinine yegazi ukubheka izinga lakho le-creatinine. I-Creatinine ingumkhiqizo wemfucumfucu wamakhemikhali okhiqizwa imisipha, ehluzwa emzimbeni izinso zakho. Lokhu kusetshenziselwa ukuqhathanisa izinga le-creatinine yegazi nemvume yezinso ye-creatinine.

Uric acid ukuhlolwa

Ukuhlolwa kwe-uric acid kuzokwenziwa ukuhlola amazinga e-uric acid. I-Uric acid ikhemikhali elenziwa lapho umzimba wakho wephula izinto ezithile zokudla. I-uric acid iphuma emzimbeni ngomchamo. Amaleveli e-uric acid ngokuvamile aphezulu kubantu abane-MCKD.

Ukuhlolwa komchamo

Kuzohlolwa umchamo ukuhlaziya umbala, amandla adonsela phansi athile, kanye ne-pH (acid noma i-alkaline) amazinga womchamo wakho. Ngaphezu kwalokho, indle yakho yomchamo izobhekwa igazi, amaprotheni, nokuqukethwe kwamaseli. Lokhu kuhlolwa kuzosiza udokotela ekuqinisekiseni ukuxilongwa noma ukukhipha ezinye izinkinga ezikhona.

Ukuhlola ukuhlolwa

Ngokungeziwe ekuhlolweni kwegazi nomchamo, udokotela wakho angaphinde oda i-CT esiswini / sezinso. Lokhu kuhlolwa kusebenzisa i-X-ray imaging ukubona izinso nengaphakathi lesisu. Lokhu kungasiza ukukhipha ezinye izimbangela zezimpawu zakho.

Udokotela wakho angafuna futhi ukwenza i-ultrasound yezinso ukubona ngeso lengqondo ama-cysts ezinsweni zakho. Lokhu ukuthola ukuthi umonakalo wezinso ungakanani.

Isidumbu

Ku-biopsy yezinso, udokotela noma omunye uchwepheshe wezempilo uzosusa ucezu oluncane lwezicubu zezinso ukuze aluhlole elebhu, ngaphansi kwesibonakhulu. Lokhu kungasiza ukukhipha ezinye izimbangela zezimpawu zakho, kufaka phakathi ukutheleleka, imali engavamile, noma ukusikeka.

I-biopsy ingasiza nodokotela wakho ukuthi anqume isigaba sesifo sezinso.

Yelashwa kanjani i-MCKD?

Alikho ikhambi le-MCKD. Ukwelashwa kwalesi simo kuqukethe ukungenelela okuzama ukunciphisa izimpawu nokunciphisa ukuqhubeka kwesifo.

Esigabeni sokuqala sesifo, udokotela wakho angancoma ukuthi wandise ukudla kwakho oketshezi. Kungadingeka ukuthi uthathe isengezo sikasawoti ukugwema ukuphelelwa amandla kwamanzi.

Njengoba lesi sifo siqhubeka, ukwehluleka kwezinso kungaba nomphumela. Uma lokhu kwenzeka, kungadingeka ukuthi wenze i-dialysis. I-Dialysis yinqubo lapho umshini ususa imfucuza emzimbeni izinso ezingasakwazi ukukuhlunga.

Yize i-dialysis iyindlela yokwelapha esekela impilo, abantu abanezinso ezihlulekayo bangakwazi nokufakelwa izinso.

Izinkinga zesikhathi eside ze-MCKD

Izinkinga ze-MCKD zingathinta izitho nezinhlelo ezahlukahlukene. Lokhu kufaka phakathi:

• i-anemia (insimbi ephansi egazini)
• ukuncipha kwamathambo, okuholela ekuqhekekeni
• ukuminyanisa kwenhliziyo ngenxa yokwakhiwa koketshezi (i-cardiac tamponade)
• izinguquko kushukela metabolism
• ukuhluleka kwenhliziyo
• ukwehluleka kwezinso
• izilonda esiswini nasemathunjini
• ukopha ngokweqile
• umfutho wegazi ophakeme
• ukungazali
• izinkinga zokuya esikhathini
• ukulimala kwemizwa

Uyini umbono we-MCKD?

I-MCKD iholela ekugcineni kwesifo sezinso - ngamanye amazwi ukwehluleka kwezinso kuzokwenzeka ekugcineni. Ngaleso sikhathi, kuzodingeka ukuthi ube nokufakelwa kwezinso noma wenze i-dialysis njalo ukuze ugcine umzimba wakho usebenza kahle. Khuluma nodokotela wakho ngezinketho zakho.