Ko te cystine te take o ngā kōhatu whatukuhu? (Ngā Kōhatu Cystine) Kia mōhio tātou ki tēnei!

He mea whakamataku ngā kōhatu whatukuhu i a tātou katoa, tika? Kua rongo pea koe mō taua mamae. Engari i mōhio koe he rerekē ngā momo kōhatu whatukuhu? I tēnei rā ka kōrero tātou mō tētahi momo kōhatu onge engari he mea nui rawa atu. Ko ngā kōhatu cystine tēnā. Kia tirohia tātou he aha i puta ai, he aha ngā tohu, me ngā mea ka taea te mahi mō ēnei.

He aha ngā kōhatu cystine?

He momo kōhatu whatukuhu anō hoki ngā kōhatu cystine. He mārō, he rite ki te kōhatu ngā kōhatu whatukuhu, he mea hanga nā te kohikohinga o ngā matū maha i roto i te mimi. He matū e kiia nei ko te cystine te mea hanga o ngā kōhatu cystine . He waikawa amino nui te cystine mō tō tātou tinana. Hei whakamārama, he wāhanga iti noa iho o ngā pūmua e āwhina ana ki te hanga uaua me te whakatika i ngā kiko o roto i tō tātou tinana.

I te nuinga o te wā, ka mimiti anōtia tēnei cystine e ō tātou whatukuhu ki roto i te tinana. Engari i ētahi tāngata, kāore tēnei tukanga e mahi tika. Kātahi ka kohia te cystine kāore e taea te mimiti ki roto i te mimi, ā, i roto i te wā, ka hanga he kōhatu. Ka taea e ēnei kōhatu cystine te tipu nui ake i ētahi atu kōhatu whatukuhu. Nō reira, i ētahi wā me tango mā te pokanga hei whakamutu i te kino o ngā whatukuhu, o te ara mimi rānei.

Nā, he aha te Cystinuria?

Ko te tino take o ēnei kōhatu cystine he mate ira e kiia nei ko te cystinuria . He mate onge tēnei, he mate tuku iho. Ki te whai koe i tēnei mate, kāore e taea e tō tinana te whakarewa tika i tētahi matū e kiia nei ko te cystine. Nō reira ka kohia ki roto i tō mimi, ka hanga kōhatu.

Nā te mea he mate ira tēnei, me riro i te tamaiti te ira mai i tōna whaea me tōna pāpā kia whanake ai . Tērā pea ka whanakehia he kōhatu cystine e te tangata whai mate nei i ngā wā maha puta noa i tōna oranga.

He pēhea te noa o tēnei mate? He aha ngā tohu?

Ehara tēnei i te mea e pā ana ki te katoa, he mea onge noa iho. I te nuinga o te wā, ki te tangohia e koe te 1, te 2 rānei o roto i te 100 tāngata e pāngia ana e ngā kōhatu whatukuhu, kotahi, e rua rānei o rātou ka pāngia e ngā kōhatu cystine. Engari he mea miharo, he maha ake ngā wā ka kitea tēnei āhuatanga i roto i ngā tamariki me ngā taiohi . Tata ki te 80% o te hunga e pāngia ana e ngā kōhatu cystine ka pāngia e tā rātou kōhatu tuatahi i mua i te 20 tau te pakeke. Heoi, ka taea e ēnei kōhatu te hanga i ngā tau katoa.

Mō ngā tohu, he tino ōrite ki ērā o ētahi atu kōhatu tākihi. Kāore e kitea ngā tohu kia puta rā anō he kōhatu.

I ētahi wā, ka taea e ēnei kōhatu cystine te nui rawa atu ka aukati i te ara mimi, ā, ka puta he mate urutā (UTI) .

He aha ngā raruraru ka puta?

He mate mō te wā roa te Cystinuria, ā, ki te kore e whakahaerea tika, ka pā mai pea ngā raruraru. He mea nui kia mahi tahi koe me tō tākuta ki te whakahaere i tēnei mate.

Ko ngā raruraru pea ko:

• Ngā mate tākihi: Ka ārai pea ngā kōhatu i te rere o te mimi, ā, ka taea hoki e ngā iroriki te uru ki roto i ngā tākihi.
• Te aukati i te mimi: Ka āraia pea te ngongo e kawe ana i te mimi mai i te whatukuhu ki te mimi pūkahukahu (ureter) e te kōhatu.
• Ngā mate urutā auau o te ara mimi (UTI).
• Te pakaru o te whatukuhu tuturu:Ki te kore e rongoatia mō te wā roa, ka heke pea te mahi a te whatukuhu.

Me pēhea te tātaritanga a te tākuta i tēnei? He aha ngā maimoatanga?

Mena he rite ō tohu ki ngā kōhatu tākihi, ka whakahaerehia e tō tākuta ētahi whakamātautau hei whakatau mena he kōhatu cystine ēnei.

Ngā tikanga tātaritanga

• Te pātai atu ki a koe mō ētahi atu kōrero: Ka pātai te tākuta mō ō tohumate, me te mea kua pāngia tētahi o tō whānau e ngā raruraru ōrite.
• Ngā whakamātautau: Tērā pea ka tonoa he whakamātautau mimi me ngā whakamātautau whakaahua pēnei i te matawai ultrasound , te matawai CT rānei.
• Te whakamātautau i te kōhatu: Mena kua puta mai he kōhatu i roto i tō mimi, ka taea te tuku ki te taiwhanga hei whakamātautau kia kitea ai te momo kōhatu. Koinei anake te huarahi hei whakaū 100% he kōhatu cystine.

Ngā tikanga maimoatanga

Ko te whāinga matua o te rongoā i ngā kōhatu cystine he ārai i te hanganga o ngā kōhatu hou, me te tango atu i ngā kōhatu o mua. Mā tō tākuta e whakawhanake he mahere rongoā e tika ana māu.

• Ngā huringa o te āhua noho me te kai
• Inumia he nui te wai: Koinei te mea nui rawa atu. Tērā pea ka kī atu tō tākuta kia inu koe i te iti rawa i te 3-4 rita wai i te rā. Ko te tikanga me waiho hei tikanga māu te inu i tētahi karāhe wai i ia hāora. Kaua e miharo mēnā ka ara ake koe i te pō ki te inu wai. Ka whakawaiwai te wai i tō mimi, ā, ka whakaiti i te kohikohinga o te cystine.
• Te whakahaere tote (konutai): Whakaitihia te nui o te tote ka tāpirihia e koe ki tō kai. Āta karohia ngā kai pēnei i ngā kai kēne, ngā kai tio, ngā kai tukatuka rānei, he nui te tote.
• Te whakaiti i te pūmua kararehe: Ka taea e koe te whakaiti i te kai pūmua kararehe pēnei i te mīti, te ika me ngā hua manu, ka arotahi ki te pūmua tipu.

• Ngā rongoā

Mena kei te mamae koe nā te kōhatu, ka tohua e tō tākuta he rongoā whakamamae mamae. Hei tāpiri, he rongoā motuhake hei āwhina i te aukati i te hanganga o ngā kōhatu cystine hou. Ko te Tiopronin me te d-penicillamine ēnei rongoā. Ka āwhina ēnei ki te whakarewa i te cystine. Me tango noa ēnei rongoā i raro i te tirotiro a te tākuta .

• Pokanga

Mena he tino nui ngā kōhatu, e aukati ana i te ara mimi, e mamae nui ana rānei, me tango pea mā te pokanga.

• Ureteroscopy: Ka tukuna he kāmera iti, ngāwari ki raro i te urethra hei kimi i te kōhatu, hei wawahi rānei kia mongamonga, hei tango katoa rānei.
• Te Nephrolithotomy ā-kiri:He tikanga e tapahia ana te kiri o te tuara, ā, ka tukuna he ngongo mā roto i taua ngongo ki roto i te whatukuhu hei tango i te kōhatu. He tikanga tēnei mō ngā kōhatu tino nunui.

Me pēhea koe e noho ai i roto i tēnei āhuatanga? He mea hei wehi māu?

He mate mō te wā roa te Cystinuria. Engari ki te tika te whakahaere, kāore he mea e manukanuka ai. Kāore e tino mōrearea te mate. Ki te tika te rongoā, kāore e tino piki haere ki ngā mate kino pērā i te ngoikore o te whatukuhu.

Hei aroturuki i te angitu o te maimoatanga, ka whakahaerehia e te tākuta he whakamātautau mimi i ia wā hei tirotiro i te taumata o te cystine i roto i te mimi. Mena e tika ana, ka whakatikatikaina te mahere maimoatanga. I te mea he mate ira tēnei, he mea pai pea te rapu tohutohu ira mena kei te whakaaro koe ki te whakatū whānau.