ʻO ka maʻi Cystic Medullary

Anter

• Nā ʻano o MCKD
• Nā kumu o MCKD
• Nā ʻōuli o MCKD
• Ke hoʻāʻo nei a me ka hōʻoia ʻana iā MCKD
• Helu piha koko
• Hōʻike BUN
• ʻOhi mimi
• Hōʻaʻo hana kolo
• Hōʻike ʻakika Uric
• Urinalysis
• Nā hoʻāʻo kiʻi
• ʻO Biopsy
• Pehea e mālama ʻia ai ʻo MCKD?
• Nā pilikia lōʻihi loa o MCKD
• He aha ka manaʻo no MCKD?

He aha ka maʻi maʻi maʻi cystic medullary?

ʻO ka maʻi maʻi maʻi maʻi maʻi maʻi (MCKD) kahi ʻano laha ʻole kahi e piha ai nā ʻeke piha piha i ka wai i kapa ʻia he mau cist ma waenakonu o nā puʻupaʻa. Kū ka scarring i loko o nā tubula o nā puʻupaʻa. Hele ka mimi i loko o nā tubula mai ka puʻupaʻa a ma o ka ʻōnaehana urinary. Hoʻokumu ka scarring i kēia mau tubule i mea pono ʻole.

I mea e maopopo ai iā MCKD, kōkua ia e ʻike iki e pili ana i kāu mau puʻupaʻa a me nā mea a lākou e hana ai. ʻO kāu mau puʻupaʻa he ʻelua ʻano o ke ʻano o ka pī e pili ana i ka nui o ka lima i paʻa. Aia lākou ma nā ʻaoʻao o kou iwikuamoʻo, kokoke i ka waena o kou kua.

Kānana kou hoʻomaʻemaʻe a hoʻomaʻemaʻe i kou koko - i kēlā me kēia lā, ma kahi o 200 kaata o ke koko e hele i waena o kāu mau puʻupaʻa. Hoʻi ke koko maʻemaʻe i kāu ʻōnaehana holo. Luku i nā huahana a me ke keu wai i mimi. Hoʻouna ʻia ka mimi i ka puhā a lawe hope ʻia mai kou kino.

ʻO ka poino i hana ʻia e MCKD alakaʻi i nā puʻupaʻa e hana i ka mimi ʻaʻole i lawa ka noʻonoʻo. I nā huaʻōlelo ʻē aʻe, wai loa kāu mimi a nele i ka nui o ka ʻōpala. A ʻo kahi hopena, e hoʻopau ʻoe i ka mimiki ala ʻoi aku ka nui o ka wai ma mua o ka maʻamau (polyuria) i ka hoʻāʻo ʻana o kou kino e hoʻopau i nā ʻōpala keu. A ke hana nā puʻupaʻa i ka nui o ka mimi, a laila nalowale ka wai, sodium, a me nā kemikino koʻikoʻi ʻē aʻe.

Ma hope o ka hala ʻana o ka manawa, hiki i ka MCKD ke alakaʻi i ka hōʻino kīwī.

Nā ʻano o MCKD

Pili loa ka nephronophthisis ʻōpio (NPH) a me MCKD. Hoʻokumu ʻia nā kūlana ʻelua e ka ʻano like o ka hōʻino ʻana o ka hakuʻala a hopena i nā ʻōuli like.

ʻO ka ʻokoʻa nui ka makahiki o ka hoʻomaka. Kū pinepine ka NPH ma waena o nā makahiki 10 a 20, ʻoiai ʻo MCKD kahi maʻi maʻi makua.

Hoʻohui ʻia, aia ʻelua mau papa o MCKD: ʻano 2 (pili pinepine i nā mākua 30 a 35 mau makahiki) a me ka ʻano 1 (pili pinepine i nā mākua 60 a 65 mau makahiki).

Nā kumu o MCKD

ʻO NPH a me MCKD nā kūlana genetomy autosomal. ʻO kēia ke kumu e kiʻi ai ʻoe i ka ʻāpana mai hoʻokahi makua e hoʻomohala i ka maʻi. Inā loaʻa i ka makua kahi moʻokūʻauhau, loaʻa i kahi keiki ke 50 pakeneka o ka loaʻa ʻana iā ia a hoʻomohala ʻana i ke ʻano.

Ma waho aʻe o ka makahiki o ka hoʻomaka, ka ʻokoʻa nui ʻē aʻe ma waena o NPH a me MCKD ma muli o nā hemahema o nā ʻokoʻa like ʻole.

ʻOiai mākou e kia i MCKD ma aneʻi, ʻo ka hapa nui o nā mea a mākou e kūkākūkā ai e pili pū me NPH.

Nā ʻōuli o MCKD

ʻO nā ʻōuli o MCKD e like me nā ʻōuli o nā ʻano ʻano ʻē aʻe, e paʻakikī ana e hana i kahi hōʻailona. Loaʻa kēia mau ʻōuli:

• urination nui loa
• mahuahua alapine (frequency) o ka urination i ka po (nocturia)
• haʻahaʻa haʻahaʻa haʻahaʻa
• nāwaliwali
• kuko ka paʻakai (ma muli o ka lilo nui o ka sodium mai ka hoʻonui ʻana o ka urination)

Ke holo nei ka maʻi, hiki i ka hopena o ke kīkole (ʻo ia hoʻi i kapa ʻia ʻo ka hopena o ka pae hope loa. Hiki i nā ʻōuli o ke kōpaʻa ʻole ke hoʻopili i kēia mau mea:

• ʻūpā a hoʻokahe paha
• luhi wale
• hiccups pinepine
• ʻeha poʻo
• nā loli i ka ʻili o ka ʻili (melemele a i ʻole brown)
• ʻili o ka ʻili
• ʻūpī mākī a wili paha
• nausea
• nalo o ka manaʻo i nā lima a i ʻole nā ​​wāwae
• luaʻi koko
• noho noho koko
• Kaumaha emi
• nāwaliwali
• ka hopu ʻana
• nā loli i ke kūlana noʻonoʻo (huikau a i ʻole ka makaʻala i hoʻololi ʻia)
• koma

Ke hoʻāʻo nei a me ka hōʻoia ʻana iā MCKD

Inā loaʻa iā ʻoe nā ʻōuli o MCKD, hiki i kāu kauka ke kauoha i kekahi mau hoʻokolohua like ʻole e hōʻoia i kāu hōʻailona. ʻO nā hoʻokolohua koko a me ka mimi ka mea nui loa no ka hoʻomaopopo ʻana iā MCKD.

Helu piha koko

Nānā kahi helu piha o ke koko i kāu helu holoʻokoʻa o nā hunaola ʻulaʻula, nā hunaola keokeo, a me nā platelet. Nānā kēia hōʻike no ka anemia a me nā hōʻailona o ka maʻi.

Hōʻike BUN

Nānā ka hoʻāʻo urea nitrogen (BUN) i ka nui o ka urea, kahi huahana breakdown o ka protein, i hoʻokiʻekiʻe ʻia ke hana ʻole nā ​​puʻupaʻa.

ʻOhi mimi

ʻO kahi hōʻiliʻili mimi he 24 mau hola e hōʻoia i ka nui o ka urination, e kākau i ka leo a me ka nalo ʻana o nā electrolytes, a ana i ka ʻae kope. E hōʻike ka cleinine clearance inā e holo pono nā puʻupaʻa.

Hōʻaʻo hana kolo

E hana ʻia kahi ho'āʻo creatinine koko e nānā i kāu pae creatinine. ʻO Creatinine kahi huahana neoneo i hana ʻia e nā mākala, i kānana ʻia i waho o ke kino e kou mau puʻupaʻa. Hoʻohana ʻia kēia e hoʻohālikelike i ke kiʻekiʻe o ka creatinine koko me ka hoʻomaʻemaʻe ʻana o ka haku mele.

Hōʻike ʻakika Uric

E hana ʻia kahi hōʻike waikawa uric e nānā i nā pae waikawa uric. ʻO ka waikawa Uric kahi kemika i hana ʻia i ka wā e wāwahi ai kou kino i kekahi mau meaʻai. Holo ka acid acid mai loko o ke kino ma o ka mimi. Kūlana kiʻekiʻe nā pae o ka uric acid i ka poʻe i loaʻa iā MCKD.

Urinalysis

E hana ʻia kahi urinalysis e kālailai i ke kala, ka umekaumaha kikoʻī, a me nā pae pH (acid a alkaline paha) o kāu mimi. Hoʻohui ʻia, e nānā ʻia kāu sediment mimi no ke koko, ka protein, a me nā ʻike o ke kelepona. E kōkua kēia hoʻokolohua i ke kauka i ka hōʻoia ʻana i kahi hōʻailona a i ʻole ka hoʻoholo ʻana i nā ʻano maʻi ʻē aʻe.

Nā hoʻāʻo kiʻi

Ma waho o nā hoʻokolohua koko a me ka mimi, kauoha paha kāu kauka i kahi scan CT ʻōpū / hakuʻala. Hoʻohana kēia hōʻike i ke kiʻi kiʻi X e ʻike ai i nā puʻupaʻa a ma loko o ka ʻōpū. Hiki i kēia ke kōkua i ka pale aku i nā kumu kūpono ʻē aʻe o kāu mau hōʻailona.

E makemake paha kāu kauka e hana i kahi ultrasound kidney e ʻike pono ai i nā koʻi ma kāu mau puʻupaʻa. ʻO kēia e hoʻoholo ai i ka nui o ka hōʻino ʻana o ka hakuʻala.

ʻO Biopsy

I loko o kahi biopsy hakuʻala, e wehe ke kauka a i ʻole ʻoihana olakino ʻē aʻe i kahi ʻāpana puʻupaʻa liʻiliʻi e nānā iā ia i kahi lab, ma lalo o kahi microscope. Hiki i kēia ke kōkua i ke kāpae ʻana i nā kumu kūpono ʻē aʻe o kāu mau ʻōuli, e like me nā maʻi, nā waihona ʻē, a i ʻole ka scarring.

Hiki i kahi biopsy ke kōkua i kāu kauka e hoʻoholo i ke kahua o ka maʻi maʻi maʻi.

Pehea e mālama ʻia ai ʻo MCKD?

ʻAʻohe mea ola no MCKD. ʻO ka mālama ʻana no ke ʻano he mea hoʻopili e hoʻāʻo e hōʻemi i nā ʻōuli a lohi i ka holomua o ka maʻi.

I nā wā mua o ka maʻi, hiki i kāu kauka ke koi aku e hoʻonui i kāu lawe ʻana i nā wai. Koi ʻia paha ʻoe e lawe i kahi pāʻakai paʻakai e hōʻalo ai i ka make wai.

Ke holo nei ka maʻi, hiki i ka hopena kīnā ʻole ke hopena. Ke hiki mai kēia, koi ʻia ʻoe e hana i ka dialysis. ʻO Dialysis kahi hana e hemo ai ka mīkini i nā ʻōpala mai ke kino i hiki ʻole i nā puʻupaʻa ke kānana hou aku.

ʻOiai he lāʻau ola ke mālama ʻana i ka dialysis, hiki i ka poʻe me ke kīkino ʻole ke hoʻowalewale i kahi transplan kidney.

Nā pilikia lōʻihi loa o MCKD

Hiki i nā pilikia o MCKD ke hoʻololi i nā ʻōnaehana like ʻole a me nā ʻōnaehana. Pākuʻi ʻia kēia mau mea:

• anemia (haʻahaʻa hao i loko o ke koko)
• nāwaliwali o nā iwi, a hiki i ka haki
• kaomi o ka puʻuwai ma muli o ke kahe o ka wai (tamponade cardiac)
• nā hoʻololi i ka kō kō
• puʻuwai puʻuwai kongestive
• hakuʻala ʻole
• ulcer i loko o ka ʻōpū a me ka ʻōpū
• kahe nui ke koko
• kahe koko kiʻekiʻe
• infertility
• pilikia menstrual
• pōʻino nerve

He aha ka manaʻo no MCKD?

Alakaʻi ʻo MCKD i ka maʻi hōʻino pae hope loa - i nā huaʻōlelo ʻē aʻe e kū ka hopena kō ʻole i ka hopena. I kēlā manawa, pono ʻoe e loaʻa i kahi transplan kidney a i ʻole hana i ka dialysis maʻamau i mea e mālama pono ai i kou kino. E kamaʻilio me kāu kauka e pili ana i kāu koho.