Isifo seMedullary Cystic

Umxholo

• Iindidi zeMCKD
• Unobangela weMCKD
• Iimpawu zeMCKD
• Ukuvavanywa nokuchongwa kweMCKD
• Gcwalisa ubalo lwegazi
• Uvavanyo lweBUN
• Ukuqokelelwa komchamo
• Uvavanyo lwe-creatinine yegazi
• Uric acid uvavanyo
• Uhlalutyo lomchamo
• Ukujonga iimvavanyo
• Uqhaqho
• Iphathwa njani iMCKD?
• Ubunzima bexesha elide beMCKD
• Ithini imbonakalo yeMCKD?

Yintoni isifo se-cystic kidney?

Isifo sesifo sezintso seMedullary (MCKD) yimeko enqabileyo apho iisaka ezincinci, ezizaliswe lulwelo ezibizwa ngokuba zii-cysts kumbindi wezintso. Ukuqhawuka kwenzeka nakwiitubules zezintso. Ukuhamba komchamo kwimibhobho ukusuka kwizintso kunye nenkqubo yokuchama. Ukunqumka kubangela ukuba ezi tubules zingasebenzi kakuhle.

Ukuze uyiqonde iMCKD, kuyanceda ukwazi kancinci malunga nezintso zakho kunye nezinto abazenzayo. Izintso zakho ngamalungu amabini amile okombotyi malunga nobungakanani benqindi elivaliweyo. Zibekwe macala omabini omqolo wakho, kufutshane kumbindi womqolo wakho.

Izintso zakho zihluza kwaye zicoca igazi lakho - yonke imihla, malunga neekota zegazi ezingama-200 zidlula kwizintso zakho. Igazi elicocekileyo libuyela kwinkqubo yakho yokujikeleza kwegazi. Iimveliso zenkunkuma kunye nolwelo olongezelelekileyo luba ngumchamo. Umchamo uthunyelwa kwisinyi kwaye ekugqibeleni ususwe emzimbeni wakho.

Umonakalo obangelwe yi-MCKD ukhokelela kwizintso ukuvelisa umchamo ongagxili ngokwaneleyo. Ngamanye amagama, umchamo wakho unamanzi kakhulu kwaye awunalo inani elifanelekileyo lenkunkuma. Ngenxa yoko, uyakuphela uchama indlela engamanzi ngaphezu kwesiqhelo (polyuria) njengoko umzimba wakho uzama ukulahla yonke inkunkuma eyongezelelweyo. Kwaye xa izintso zivelisa umchamo omninzi, emva koko amanzi, isodiyam, kunye nezinye iikhemikhali ezibalulekileyo ziyalahleka.

Ixesha elingaphezulu, i-MCKD inokukhokelela kukusilela kwezintso.

Iindidi zeMCKD

I-nephronophthisis yolutsha (i-NPH) kunye ne-MCKD zihambelana kakhulu. Zombini iimeko zibangelwa luhlobo olufanayo lomonakalo wezintso kwaye zikhokelela kwiimpawu ezifanayo.

Umahluko omkhulu yiminyaka yokuqalisa. I-NPH ihlala ivela phakathi kweminyaka eli-10 ukuya kwengama-20, ngelixa iMCKD sisifo sokuqala kwabantu abadala.

Ukongeza, zimbini iiseti ezisezantsi zeMCKD: uhlobo 2 (ichaphazela abantu abadala abaneminyaka engama-30 ukuya kwengama-35) kwaye uchwetheze uhlobo 1 (ichaphazela abantu abadala abaneminyaka engama-60 ukuya kuma-65).

Unobangela weMCKD

Zombini i-NPH kunye neMCKD ziimeko eziphambili ze-autosomal. Oku kuthetha ukuba ufuna kuphela ukufumana ufuzo kumzali omnye ukuze uphuhlise ingxaki. Ukuba umzali une-gene, umntwana unethuba lama-50 lokufumana kwaye aphuhlise imeko.

Ngaphandle kweminyaka yobudala, omnye umahluko omkhulu phakathi kwe-NPH kunye neMCKD kukuba zibangelwa ziziphene zemfuza ezahlukileyo.

Ngelixa sigxile kwi-MCKD apha, uninzi lwezinto esixoxa ngazo ziyasebenza nakwi-NPH ngokunjalo.

Iimpawu zeMCKD

Iimpawu ze-MCKD zijongeka njengeempawu zezinye iimeko, zisenza ukuba kube nzima ukwenza isifo. Ezi mpawu zibandakanya:

• ukuchama kakhulu
• ukwanda rhoqo kokuchama ebusuku (nocturia)
• uxinzelelo lwegazi oluphantsi
• ubuthathaka
• Iminqweno yetyuwa (ngenxa yokulahleka kakhulu kwe-sodium ekunyukeni kokuchama)

Njengoko isifo siqhubeka, ukusilela kwezintso (okwaziwa ngokuba sisifo se-renal). Iimpawu zokusilela kwezintso zingabandakanya oku kulandelayo:

• ukugruzuka okanye ukopha
• ukudinwa ngokulula
• ii-hiccups rhoqo
• intloko ebuhlungu
• utshintsho kumbala wesikhumba (omthubi okanye omdaka)
• ukurhawuzelela ulusu
• ukuxinana kwezihlunu okanye ukujijeka
• isicaphucaphu
• ukulahlekelwa yimvakalelo ezandleni okanye ezinyaweni
• ukugabha igazi
• ilindle elinegazi
• ukuhla ukusinda
• ubuthathaka
• ukuxhuzula
• utshintsho kwimeko yengqondo (ukudideka okanye ukulumkisa okutshintshileyo)
• ikhoma

Ukuvavanywa nokuchongwa kweMCKD

Ukuba uneempawu ze-MCKD, ugqirha wakho unokuyalela inani leemvavanyo ezahlukeneyo ukuqinisekisa isifo sakho. Uvavanyo lwegazi kunye nomchamo luya kuba lolona lubalulekileyo ekuchongeni iMCKD.

Gcwalisa ubalo lwegazi

Inani elipheleleyo legazi lijonga amanani akho eeseli ezibomvu zegazi, iiseli ezimhlophe zegazi kunye neeplatelets. Olu vavanyo lukhangela i-anemia kunye neempawu zosulelo.

Uvavanyo lweBUN

Uvavanyo lwe-urea nitrogen (BUN) lwegazi lukhangela isixa se-urea, imveliso eyaphukileyo yeprotein, ephakanyisiweyo xa izintso zingasebenzi kakuhle.

Ukuqokelelwa komchamo

Ingqokelela yomchamo yeeyure ezingama-24 iya kuqinisekisa ukuchama okugqithisileyo, uxwebhu lwevolumu kunye nelahleko yee-electrolyte, kwaye ulinganise ukucinywa kwe-creatinine. Ukucaciswa kwe-creatinine kuya kutyhila ukuba ingaba izintso zisebenza ngokufanelekileyo.

Uvavanyo lwe-creatinine yegazi

Uvavanyo lwe-creatinine lwegazi luyakwenziwa ukujonga inqanaba lakho lokuyila. I-Creatinine yimveliso yenkunkuma yeekhemikhali eveliswa yimisipha, ethi ihluzwe iphume emzimbeni zizintso zakho. Oku kusetyenziselwa ukuthelekisa inqanaba lokudala kwegazi kunye nokususwa kwezintso kwe-creatinine.

Uric acid uvavanyo

Uvavanyo lwe-uric acid luya kwenziwa ukujonga amanqanaba e-uric acid. IUric acid yikhemikhali eyenziweyo xa umzimba wakho wophula izinto ezithile zokutya. Uric acid idlula emzimbeni ngomchamo. Amanqanaba e-uric acid ahlala ephezulu kubantu abane-MCKD.

Uhlalutyo lomchamo

Uvavanyo lwe-urinalysis luya kwenziwa ukuhlalutya umbala, ubunzima obuthile, kunye ne-pH (i-asidi okanye i-alkaline) amanqanaba omchamo wakho. Ukongeza, i-sediment yakho yomchamo iya kujongwa kwigazi, iprotheni, kunye nomxholo weseli. Olu vavanyo luya kunceda ugqirha ekuqinisekiseni ukuxilongwa okanye ukulawula ezinye iingxaki ezinokwenzeka.

Ukujonga iimvavanyo

Ukongeza kuvavanyo lwegazi kunye nomchamo, ugqirha wakho unokuyalela isisu / sezintso i-CT scan. Olu vavanyo lusebenzisa i-X-ray imaging ukubona izintso nangaphakathi kwesisu. Oku kunokunceda ukulawula ezinye izizathu ezinokubangela iimpawu zakho.

Ugqirha wakho unokufuna ukwenza i-kidney ultrasound ukujonga ngee-cysts kwizintso zakho. Oku kukufumanisa ubungakanani bomonakalo wezintso.

Uqhaqho

Kwi-biopsy yezintso, ugqirha okanye enye ingcali yezempilo iyakususa iqhekeza elincinci lezicubu zezintso ukuyihlola elebhu, phantsi kwemicroscope. Oku kunokukunceda ukulawula ezinye izizathu ezinokubangela iimpawu zakho, kubandakanya usulelo, iidiphozithi ezingaqhelekanga, okanye ukonakala.

I-biopsy inokunceda ugqirha wakho amisele inqanaba lesifo sezintso.

Iphathwa njani iMCKD?

Akukho lunyango lweMCKD. Unyango lwale meko lubandakanya ungenelelo oluzama ukunciphisa iimpawu kunye nokucothisa ukuqhubela phambili kwesi sifo.

Kumanqanaba okuqala esi sifo, ugqirha wakho unokucebisa ukuba wandise ulwelo lwakho. Unokucelwa ukuba uthathe isongezelelo setyuwa ukunqanda ukomisa.

Njengoko isifo siqhubeka, ukusilela kwezintso kunokubangela. Xa oku kusenzeka, unokucelwa ukuba wenze idialysis. I-Dialysis yinkqubo apho umatshini esusa inkunkuma emzimbeni engenakucoca izintso.

Nangona i-dialysis yonyango olugcina ubomi, abantu abanezintso ezingaphumeleliyo banokukwazi ukufakelwa izintso.

Ubunzima bexesha elide beMCKD

Iingxaki zeMCKD zinokuchaphazela amalungu kunye neenkqubo ezahlukeneyo. Oku kubandakanya:

• i-anemia (intsimbi esezantsi egazini)
• buthathaka kwamathambo, kukhokelela ekuqhekekeni
• Ukunyanzelwa kwentliziyo ngenxa yokwakheka kolwelo (itamponade yentliziyo)
• utshintsho kwimetabolism yeswekile
• ukusilela kwentliziyo
• ukusilela kwezintso
• izilonda esiswini nasemathumbu
• ukopha kakhulu
• igazi elonyukayo
• ukungachumi
• iingxaki zokuya exesheni
• umonakalo wemithambo-luvo

Ithini imbonakalo yeMCKD?

I-MCKD ikhokelela kwisigaba sokugqibela sesifo sezintso - ngamanye amagama ukusilela kwezintso kuya kwenzeka ekugqibeleni. Ngelo xesha, kuya kufuneka ukuba ube nokufakelwa kwezintso okanye ungene kwi-dialysis rhoqo ukuze ugcine umzimba wakho usebenza ngokufanelekileyo. Thetha nogqirha wakho malunga nokukhetha kwakho.