He aha kēia mau pōhaku puʻupaʻa āu e ulu nei? (Nā Pōhaku Cystine) E aʻo maʻalahi kākou e pili ana iā lākou!

Ua hoʻokomo ʻia ʻoe i ka haukapila me nā pōhaku puʻupaʻa a me ka ʻeha hiki ʻole ke hoʻomanawanui ma kou kua, ʻōpū haʻahaʻa, a ʻaoʻao paha? A i ʻole ua lohe paha ʻoe i kekahi āu i ʻike ai i loaʻa i kēlā pilikia? He ʻike ʻeha maoli nā pōhaku puʻupaʻa. Hiki ke hana ʻia kēia mau pōhaku ma nā ʻano like ʻole. I kēia lā e kamaʻilio mākou e pili ana i kahi ʻano pōhaku puʻupaʻa i ʻoi aku ka kakaikahi, akā hiki ke hoʻopilikia inā ʻaʻole mālama pono ʻia, ʻoi aku hoʻi i waena o ka poʻe ʻōpio, i kapa ʻia ʻo nā pōhaku cystine .

He aha kēia mau pōhaku cystine?

I ka ʻōlelo maʻalahi, he ʻano pōhaku puʻupaʻa nā pōhaku cystine. He mau puʻupuʻu paʻakikī nā pōhaku puʻupaʻa e hana ʻia i ka wā e hōʻiliʻili ai nā kemika like ʻole i loko o kou mimi, e like me nā ʻiliʻili liʻiliʻi. Hana ʻia nā pōhaku cystine i kahi kemika i kapa ʻia ʻo cystine . ʻO kēia cystine kahi waikawa amino i loaʻa i loko o ko mākou kino. ʻO ka mea maʻamau, ʻo ka mea e hana ʻia, ʻo ia ka hoʻihoʻi ʻia ʻana o kēia cystine i loko o ke kino e nā puʻupaʻa. Akā i kekahi manawa, ke hana pono ʻole kēia, hōʻiliʻili ka cystine i loko o ka mimi a hana i nā pōhaku.

He nui pinepine kēia mau pōhaku cystine. No laila, i kekahi manawa pono ke ʻoki kino e wehe iā lākou e pale aku i ka hōʻino ʻana i nā puʻupaʻa a i ʻole ke ala mimi. ʻO ka mea kaumaha, hiki i ka poʻe he nui i loaʻa nā pōhaku cystine ke loaʻa i kēia mau pōhaku i nā manawa he nui i ko lākou ola. Akā, mai hopohopo, aia nā ala e mālama ai i kēia kūlana me ka ʻole o ke ʻoki kino. No ka laʻana, hiki iā ʻoe ke kāohi i kēia kūlana ma ka hoʻololi ʻana i kāu meaʻai a i ʻole ka hoʻohana ʻana i nā lāʻau lapaʻau e kaulike i ka pH o kāu mimi.

No laila he aha ka Cystinuria?

He maʻi hoʻoilina kakaʻikahi ʻo Cystinuria . ʻO kēia ka wā e hōʻiliʻili ai kahi kemika i kapa ʻia ʻo cystine i loko o kou mimi. Ma kahi o hoʻokahi o ka ʻumi tausani o ka poʻe i loaʻa i kēia maʻi. Inā loaʻa iā ʻoe ka cystinuria, pilikia kou kino i ka hoʻoheheʻe ʻana i ka cystine. ʻO kēia ka wā e hōʻiliʻili ai a hana i nā pōhaku cystine. Hiki iā Cystinuria ke hoʻoulu iā ʻoe e hoʻomohala i nā pōhaku puʻupaʻa i nā manawa he nui i kou ola.

Hoʻokumu ʻia kēia e kahi ʻano gene i kapa ʻia (autosomal recessive gene) . ʻO ia hoʻi, inā loaʻa i kahi keiki kēia gene mai ka makuahine a me ka makuakāne, e loaʻa i ke keiki ka cystinuria. He maʻi kēia no ke ola holoʻokoʻa a ʻaʻole hiki ke ho'ōla loa ʻia. Eia nō naʻe, hiki ke hoʻomalu maikaʻi ʻia me ka mālama kūpono .

Pehea ka kakaikahi o nā pōhaku puʻupaʻa cystine?

Ke hoʻohālikelike ʻia me nā ʻano pōhaku puʻupaʻa ʻē aʻe, ʻike wale ʻia nā pōhaku cystine i 1% a 2% o nā hihia. He mea kākaʻikahi loa ia. Akā, ʻo ka mea kupanaha, 6% a 8% o nā pōhaku puʻupaʻa i nā keiki he mau pōhaku cystine . ʻO ka ʻoiaʻiʻo, 80% o ka poʻe i loaʻa nā pōhaku cystine e hoʻomohala i kā lākou pōhaku mua ma mua o ka 20 o ko lākou mau makahiki.Eia nō naʻe, hiki ke hoʻomohala i hoʻokahi a ʻoi aku paha o kēia mau pōhaku i kēlā me kēia makahiki.

He aha nā hōʻailona o nā pōhaku cystine?

Ua like loa nā hōʻailona o nā pōhaku cystine me nā pōhaku puʻupaʻa ʻē aʻe. Hoʻomaka wale ʻoe e ʻike i nā hōʻailona ke hana ʻia kahi pōhaku.

Hiki ke hana ʻia nā mea e like me kēia:

• He ʻeha ʻoi ma kekahi ʻaoʻao o ke kino (kua) .
• Ke ʻeha nei ma ka ʻāpana groin a i ʻole ka ʻōpū lalo.
• Ke ʻike nei i ke koko i loko o ka mimi .
• Ka luaʻi ʻana a me ka ʻeha o ka ʻōpū (nausea).
• ʻEha i ka wā e mimi ai (hana kēia i ka wā e kokoke ana ka pōhaku i ka ʻōpū mimi).

I kekahi manawa, hiki i kahi pōhaku cystine ke paʻa a hoʻoulu i kahi maʻi ʻaʻai mimi (UTI) . No ka mea, ʻoi aku ka nui o nā pōhaku cystine ma mua o nā pōhaku ʻē aʻe, ʻoi aku ka nui o ko lākou hoʻoulu ʻana i ia mau pilikia.

No ke aha i hana ʻia ai kēia mau pōhaku cystine?

E like me kā mākou i ʻōlelo ai ma mua, ʻoi aku ka nui o ka ulu ʻana o nā pōhaku cystine i nā poʻe me kahi maʻi kakaikahi i kapa ʻia ʻo cystinuria . Hoʻokumu ʻia ʻo Cystinuria e nā mutations i loko o nā genes SLC3A1 a me SLC7A9 . ʻO kēia kūlana ke kumu o kou kino ʻaʻole hiki ke hoʻohana pono i ka amino acid cystine. A laila kūkulu ʻia a hana i nā pōhaku. Hiki i kēia mau pōhaku ke noho ma nā wahi āpau o kou ala mimi, e like me kou ʻōpū mimi a i ʻole nā ​​​​puʻupaʻa.

I ka ʻōlelo maʻalahi, he pilikia hoʻoilina kēia. ʻO ia hoʻi, ʻaʻole na ʻoe ia hewa.

He aha nā mea i hana ʻia ai nā pōhaku cysteine?

Hana ʻia nā pōhaku Cystine me ka cystine . E like me kā mākou i ʻōlelo ai ma mua, he waikawa amino ka cystine. He ʻano protein nā waikawa Amino. Kōkua lākou i ko mākou kino e hoʻoponopono i nā ʻiʻo, kūkulu i nā ʻiʻo, a kaua i nā maʻi. Loaʻa i ka poʻe me ka cystinuria nā pilikia me ko lākou mau puʻupaʻa i ka omo ʻana a i ʻole ka hoʻohana ʻana i kēia waikawa amino.

He aha nā pilikia e hiki mai ana mai nā pōhaku cystine?

He maʻi ola ka Cystinuria. Hiki i kāu kauka ke kōkua iā ʻoe e mālama i ke kūlana me nā lāʻau lapaʻau, nā hoʻololi ʻana i ka meaʻai, a me ke ʻoki kino paha. Eia nō naʻe, ʻoi aku ka nui o kou loaʻa ʻana o nā pōhaku cystine i kou ola a pau. Hiki i kēia mau pōhaku ke ʻeha loa i ko lākou hala ʻana.

Hiki i nā pōhaku Cystine ke hana i nā pilikia e like me:

• Nā maʻi puʻupaʻa .
• Ka paʻa ʻana o ka ureter (ka ʻōmole e lawe ai i ka mimi mai nā puʻupaʻa a i ka ʻōpū mimi).
• Nā Maʻi ʻAla Mimi (UTI) .
• Ka pōʻino o nā puʻupaʻa.

Pehea e ʻike ai inā he mau pōhaku cystine kou?

E ʻike kāu kauka i nā pōhaku cystine ma ka hoʻolohe ʻana i kāu mau hōʻailona a lawe i kāu mōʻaukala olakino piha, me ka ʻike inā ua loaʻa i kekahi o kou ʻohana kēia mau maʻi. Eia kekahi, hiki iā ʻoe ke hana i kēia mau mea:

• Nā hoʻāʻo kiʻi , e like me nā scan ultrasound a me nā scan CT , a me nā hoʻāʻo mimi.
• Ke hoʻouna ʻana i kahi pōhaku i kahi keʻena hoʻokolohua no ka hoʻāʻo ʻana. Inā hiki iā ʻoe, e hōʻiliʻili i kahi pōhaku i puka i waho me kāu mimi, a hiki i kāu kauka ke hoʻouna iā ia i kahi keʻena hoʻokolohua no ka nānā ʻana. Ma kēlā ala, hiki iā lākou ke ʻike pono i ke ʻano o ka kemika i hana ʻia ai ka pōhaku.

Pehea e mālama ʻia ai ka cystinuria?

ʻO ka mea nui loa i ka mālama ʻana i nā pōhaku cystine, ʻo ia ka hoʻāʻo ʻana e pale iā lākou mai ka hoʻokumu ʻia ʻana ma kahi mua . ʻO kekahi o nā ala maikaʻi loa e hana ai i kēia, ʻo ia ka hoʻemi ʻana i ka nui o ka cystine i loko o kāu mimi. E hoʻohana paha kāu kauka i ka hui pū ʻana o nā lāʻau lapaʻau a me nā hoʻololi ʻai e kōkua i ka hōʻemi ʻana i ka nui o nā pōhaku āu e hana ai. Inā ʻaʻole nā ​​​​lāʻau lapaʻau a me nā hoʻololi ʻai e pale i ka hoʻokumu ʻia ʻana o nā pōhaku, a inā nui loa nā pōhaku, ʻo ke ʻoki ʻana kekahi koho.

Lāʻau lapaʻau

Inā manaʻo kāu kauka e kōkua ka inu ʻana i ka nui o ka wai i ka hemo ʻana o ka pōhaku ma kona iho, hiki iā ia ke kuhikuhi i ka lāʻau ʻeha ma luna o ka counter (OTC) no ka ʻeha. Hiki iā lākou ke kuhikuhi i nā lāʻau lapaʻau e like me tiopronin a me d-penicillamine e kōkua i ka pale ʻana i ka hoʻokumu ʻia ʻana o ka pōhaku i ka wā e hiki mai ana.

Hoʻolālā ʻAi

Hiki i kāu kauka ke aʻo aku iā ʻoe e hoʻololi i kāu mau ʻano ʻai e pale aku i ke kūkulu ʻia ʻana o nā pōhaku. ʻO kekahi o kēia mau mea:

• ʻO ka inu ʻana i ka wai he nui. Hiki i kēia ke kōkua i ka hoʻoheheʻe ʻana i nā pōhaku cystine. Hiki i kāu kauka ke ʻōlelo iā ʻoe e inu i 96 auneke (ma kahi o 2.8 lita) o ka wai i ka lā, inu i hoʻokahi kīʻaha wai i kēlā me kēia hola, a inu paha i ka wai ke ala ʻoe i ka pō.
• E kaupalena i ka nui o ka sodium (paʻakai) āu e ʻai ai. ʻO ia hoʻi, pono ʻoe e hoʻōki i ka ʻai ʻana i nā meaʻai hau a i ʻole nā ​​meaʻai i loko o ka pahu. ʻAʻole wale ka paʻakai a mākou e hoʻokomo ai i ka laiki a mākou e ʻai ai, akā aia pū kekahi paʻakai huna i nā mea e like me nā pickles, pickles, sauces, nā meaʻai i hoʻopaʻa ʻia, a me nā meaʻai i kūʻai ʻia ma ka hale kūʻai. No laila pono ʻoe e makaʻala i kēlā.
• ʻO ka protein holoholona , ​​ʻo ia hoʻi ka ʻai ʻana i ka ʻiʻo liʻiliʻi a ma kahi o ka ʻai ʻana i ka protein mea kanu. No ka laʻana, hiki iā ʻoe ke hoʻohui i nā mea e like me ka chickpeas, lentils, a me ka soy i kāu papaʻai ma kahi o ka ʻiʻo a me ka iʻa.

ʻoki kino

Hiki ke hōʻino ʻia nā pōhaku nui inā ʻaʻole lākou e hele ʻia ma ke ala mimi. Hiki i kēia mau pōhaku ke ʻeha loa a hiki ke ālai i ke kahe ʻana o ka mimi mai ke kino. Aia kekahi mau ʻano hana e hiki ai i kāu kauka ke hoʻohana e wehe i kahi pōhaku:

• Hoʻokomo ʻia kahi kāmela liʻiliʻi a maʻalahi hoʻi i loko o kou urethra, ma o ka ʻōpū mimi a me ka urethra, e ʻimi i ka pōhaku a wehe iā ia (ureteroscopy) .
• Hana ʻia kahi ʻoki liʻiliʻi ma ka ʻili o kou kua, hoʻokomo ʻia kahi paipu ma loko o ia mea i loko o ka puʻupaʻa, a laila wehe ʻia ka pōhaku.(nephrolithotomy percutaneous).

Pehea ʻoe e ʻike ai inā ua holomua ka lāʻau lapaʻau?

Hiki i kāu kauka ke kauoha i kahi urinalysis i kēlā me kēia manawa e nānā i kāu mau pae cystine. E kōkua kēia hoʻāʻo iā ia e hoʻoholo inā e hana ana kāu hoʻolālā lapaʻau a ʻaʻole paha.

Hiki ke hoʻoheheʻe ʻia nā pōhaku cystine?

ʻAe, hiki ke hele aku kahi pōhaku cystine. Akā, pono ia e nānā pono a me ka mālama mau ʻana . Ma kahi o ka hoʻoheheʻe loa ʻana, hiki ke hoʻolilo iā ia i mea liʻiliʻi a maʻalahi hoʻi e hele aku.

He aha nā meaʻai e kōkua ai me ka cystitis?

Ua hōʻike ʻia e ka noiʻi hiki i ka poʻe me ka cystinuria ke pōmaikaʻi mai ka hana ʻana i nā hoʻololi ʻana i ka meaʻai. ʻO kekahi o kēia mau mea e komo pū ana me ka inu ʻana i ka wai hou aku, ka ʻai ʻana i nā huaʻai a me nā mea kanu hou aku, a me ka ʻai ʻana i ka meaʻai haʻahaʻa i ka paʻakai. He mea maikaʻi loa nā hua citrus e like me nā ʻalani a me nā lemona, nā mea kanu ʻōmaʻomaʻo, a me nā lau lau nahele.

Hiki ke pale ʻia nā pōhaku cystine?

He maʻi hoʻoilina ka Cystinuria, no laila ʻaʻole hiki ke pale ʻia. Eia nō naʻe, ma ka hahai ʻana i ka hoʻolālā lapaʻau a kāu kauka e hāʻawi ai iā ʻoe , hiki iā ʻoe ke pale i ke kūkulu ʻia ʻana o nā pōhaku cystine . ʻO ia hoʻi, hiki iā ʻoe ke kāohi i ke kūkulu ʻia ʻana o nā pōhaku.

He mea hoʻoweliweli anei ke cystinuria i ke ola?

ʻAʻole kēia he mea hoʻoweliweli i ke ola . ʻAʻole loaʻa i ka hapa nui o ka poʻe nā maʻi koʻikoʻi e like me ka hāʻule ʻana o nā puʻupaʻa. ʻO ka mea nui loa, ʻo ia ke hana pū me kāu kauka e ʻimi i kahi lāʻau e kūpono iā ʻoe. Pane maikaʻi ka hapa nui o ka poʻe i ka lāʻau. Hiki nō hoʻi ke loaʻa kahi lāʻau i ʻole e hōʻino i nā puʻupaʻa, ka ʻōpū mimi, a i ʻole ke ala mimi.

He aha ka manaʻolana no nā pōhaku sistine i ka wā e hiki mai ana?

Hiki i nā pōhaku cystine ke hana i nā manawa he nui i kou ola. Inā loaʻa iā ʻoe ka cystinuria, pono ʻoe e hoʻomau i ka hana ʻana i nā hoʻololi i ka meaʻai a lawe i nā lāʻau lapaʻau e pale aku i ka hana ʻana o nā pōhaku cystine. E nānā kāu kauka iā ʻoe a e hōʻoia e mālama pono ana ʻoe i nā pōhaku cystine hou e hana ʻia. ʻO kēia ke pale aku i ka hōʻino ʻana i kou ala mimi, nā puʻupaʻa, a me ka ʻōpū mimi.

Āhea wau e ʻike ai i ke kauka?

Inā loaʻa iā ʻoe kekahi mau hōʻailona o ka pōhaku puʻupaʻa, e ʻike pono i ke kauka . Eia kekahi o nā hōʻailona maʻamau:

• ʻEha ma ka ʻōpū, ʻaoʻao o ke kua, a i ʻole ka ʻūhā.
• Ka luaʻi a me ka luaʻi.
• ʻEha i ka wā e mimi ai.

He maʻi ola ka Cystinuria e hoʻoulu ai i nā pōhaku cystine. E hana pū me kāu kauka e hoʻomohala i kahi hoʻolālā lapaʻau e pale aku i ka hoʻokumu ʻana o nā pōhaku. Ma muli o ke ʻano genetic, makemake paha ka poʻe me ka cystinuria e noʻonoʻo i ke aʻoaʻo genetic inā lākou e noʻonoʻo nei e hoʻomaka i kahi ʻohana.Hiki nō hoʻi ke kōkua i ka loaʻa ʻana. ʻO ka nūhou maikaʻi, hiki ke hoʻomalu ʻia kēia ʻano me ka lāʻau lapaʻau a ʻaʻole ia e hoʻopilikia i ka hāʻule ʻana o nā puʻupaʻa a i ʻole ka hōʻino nui ʻana i ke ala mimi.

Nā mea nui e hoʻomanaʻo (Leka Lawe-Home)

ʻAe, e hōʻuluʻulu kākou i kekahi o nā mea e pono ai ʻoe e hoʻomanaʻo mai nā mea a mākou i kamaʻilio ai:

• ʻO nā pōhaku Cystine kahi ʻano pōhaku puʻupaʻa i hoʻokumu ʻia ma muli o kahi kūlana genetic i kapa ʻia ʻo cystinuria .
• Hiki i kēia mau mea ke nui loa a hiki ke hana ʻia i nā manawa he nui i ka wā e ola ana.
• Hiki i nā hōʻailona ke komo pū me ka ʻeha nui, ke koko i loko o ka mimi, a me ka luaʻi ʻana .
• ʻO ka mālama ʻana, ʻo ia ka inu ʻana i ka nui o ka wai, ka hoʻēmi ʻana i ka paʻakai a me ka protein holoholona, ​​a inā pono, nā lāʻau lapaʻau a me ke ʻoki kino .
• ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia ka cystinuria, me ka mālama kūpono, hiki ke kaohi ʻia ke kūkulu ʻia ʻana o nā pōhaku a hiki ke alakaʻi ʻia i kahi ola maʻamau.
• Inā loaʻa iā ʻoe nā hōʻailona o nā pōhaku puʻupaʻa , mai hoʻonele i ka manawa a e ʻike i ke kauka.

E ʻoluʻolu e hoʻomaopopo he ʻike wale nō kēia ʻike a ʻaʻole pono e lawe ʻia ma ke ʻano he ʻōlelo aʻoaʻo lapaʻau. He mea maikaʻi mau ke nīnau aku i ke kauka no kekahi mau hopohopo olakino.

Nā pōhaku puʻupaʻa, nā pōhaku cystine, cystinuria, nā pōhaku mimi, nā maʻi puʻupaʻa, nā maʻi hoʻoilina, nā maʻi ʻaʻai mimi