He aha ēnei kōhatu whatukuhu e puta ake ana i a koe? (Ngā Kōhatu Cystine) Me ako ngāwari tātou mō ēnei!

Kua whakaurua koe ki te hōhipera mō ngā kōhatu tākihi me te mamae e kore e taea te whakaroa i tō tuara, i tō puku o raro, i tō taha rānei? Kua rongo rānei koe mō tētahi e mōhio ana koe kua pāngia e tēnei raruraru? He wheako tino mamae ngā kōhatu tākihi. Ka taea e ēnei kōhatu te hanga i roto i ngā momo huarahi maha. I tēnei rā ka kōrero tātou mō tētahi momo kōhatu tākihi he onge ake, engari ka pā mai he raruraru mēnā kāore e rongoatia tika, inā koa i waenga i ngā taiohi, e kiia nei ko ngā kōhatu cystine .

He aha ēnei kōhatu cystine?

He māmā noa iho, he momo kōhatu tākihi ngā kōhatu cystine. He puranga mārō ngā kōhatu tākihi ka puta mai i te wā e kohia ai ngā matū maha i roto i tō mimi, pērā i ngā kirikiri ririki. He matū e kiia nei ko te cystine te mea hanga ai ngā kōhatu cystine . He waikawa amino tēnei cystine e kitea ana i roto i ō tātou tinana. Ko te tikanga, ko te mea e tupu ana ko te mimiti anō o tēnei cystine ki roto i te tinana e ngā tākihi. Engari i ētahi wā, ina kore e tika te mahi, ka kohia te cystine ki roto i te mimi ka hanga kōhatu.

He nui rawa ēnei kōhatu cystine. Nō reira, i ētahi wā ka hiahiatia he pokanga hei tango i a rātou hei ārai i te kino o ngā whatukuhu, o te ara mimi rānei. Ko te mea pouri, he maha ngā tāngata e pāngia ana e ngā kōhatu cystine ka pāngia pea e ēnei kōhatu i ngā wā maha i roto i ō rātou oranga. Engari kaua e manukanuka, he huarahi hei rongoā i tēnei mate me te kore pokanga. Hei tauira, ka taea e koe te whakahaere i tēnei mate mā te whakarerekē i tō kai , mā te whakamahi rānei i ngā rongoā hei whakataurite i te pH o tō mimi.

Nā, he aha te Cystinuria?

He mate onge, he mate tuku iho te Cystinuria . Koinei te wā ka puta he matū e kiia nei ko te cystine i roto i tō mimi. Tata ki te kotahi i roto i te tekau mano tāngata he mate pēnei. Mena he cystinuria tōu, ka uaua ki tō tinana te whakarewa i te cystine. Koinei te wā ka puta he kōhatu cystine, ka hanga. Ka taea e te Cystinuria te whakawhanake i ngā kōhatu tākihi i ngā wā maha puta noa i tō oranga.

Nā tētahi momo ira e kīia nei ko te (ira autosomal recessive) tēnei i hua mai. Ko te tikanga, ki te riro mai i te tamaiti tēnei ira i te whaea me te pāpā, ka pāngia te tamaiti e te cystinuria. He mate tēnei mō te wā katoa, ā, kāore e taea te whakaora katoa. Heoi, ka taea te whakahaere pai mā te maimoatanga tika .

E hia te onge o ngā kōhatu whatukuhu cystine?

Ki te whakatauritea ki ētahi atu momo kōhatu tākihi, ka puta noa ngā kōhatu cystine i roto i te 1% ki te 2% o ngā take. He tino onge tēnā. Engari he mea miharo, 6% ki te 8% o ngā kōhatu tākihi i roto i ngā tamariki he kōhatu cystine . Inaa, 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 te whakawhanake i tētahi, i ētahi rānei o ēnei kōhatu i ngā tau katoa.

He aha ngā tohu o ngā kōhatu cystine?

He tino rite ngā tohu o ngā kōhatu cystine ki ērā o ētahi atu kōhatu tākihi. Ka tīmata noa te rongo i ngā tohu ina puta he kōhatu.

Tērā pea ka tupu ēnei mea:

• He mamae wero nui i te taha kotahi o tō tinana (tuara) .
• Te mamae i te rohe o te hope, i te puku o raro rānei.
• Te kitenga i te toto i roto i te mimi .
• Te ruaki me te mamae o te puku (whakapaipai).
• Te mamae i te wā e mimi ana (ka puta tēnei ina tata te kōhatu ki te pūhā).

I ētahi wā, ka piri te kōhatu cystine ka puta he mate mimi (UTI) . Nā te mea he nui ake ngā kōhatu cystine i ētahi atu kōhatu, he nui ake te tūponotanga ka puta ēnei raruraru.

He aha i puta ai ēnei kōhatu cystine?

E ai ki tā mātou i kōrero ai i mua ake nei, ko ngā kōhatu cystine te mea tino tūpono ka puta i roto i te hunga whai mate onge e kiia nei ko te cystinuria . Ko te Cystinuria ka puta mai i ngā whakarerekētanga ira o ngā ira SLC3A1 me SLC7A9 . Nā tēnei mate ka kore e taea e tō tinana te whakamahi tika i te waikawa amino cystine. Kātahi ka hanga kōhatu. Ka taea e ēnei kōhatu te noho ki ngā wāhi katoa o tō ara mimi, pērā i tō pūkahukahu, i ō whatukuhu rānei.

He raruraru ira tēnei. Arā, ehara i te mea nāu te he.

He aha ngā mea i hangaia ai ngā kōhatu cysteine?

He mea hanga ngā kōhatu cystine i te cystine . E ai ki tā mātou i kōrero ai i mua ake nei, he waikawa amino te cystine. He momo pūmua ngā waikawa amino. Ka āwhina i te tinana ki te whakatika i ngā kiko, ki te hanga uaua, me te whawhai ki ngā mate. He raruraru ki te hunga whai cystinuria te mimiti, te whakamahi rānei i tēnei waikawa amino e ō rātou whatukuhu.

He aha ngā raruraru ka pā mai i te kōhatu cystine?

He mate mō te wā roa te Cystinuria. Ka taea e tō tākuta te āwhina i a koe ki te whakahaere i te mate mā te rongoā, te whakarerekē i te kai, me te pokanga pea. Heoi, he nui ake te tūponotanga ka pāngia koe e ngā kōhatu cystine puta noa i tō oranga. Ka tino mamae ēnei kōhatu i a rātou e puta ana.

Ka taea e ngā kōhatu cystine te whakararu i ngā raruraru pēnei i:

• Ngā mate pukupuku .
• Te aukati i te ureter (te ngongo e kawe ana i te mimi mai i ngā whatukuhu ki te pūkahukahu).
• Ngā Mate Pukupuku o te Ara Mimi (UTI) .
• Te kino o te whatukuhu.

Me pēhea koe e mōhio ai mēnā he kōhatu cystine tōu?

Mā tō tākuta e tātaritanga ngā kōhatu cystine mā te whakarongo ki ō tohumate me te tango i tō hītori hauora katoa, tae atu ki te mea kua pāngia tētahi o tō whānau e ēnei āhuatanga. Hei tāpiri, ka taea pea e koe te mahi i ēnei e whai ake nei:

• Ngā whakamātautau whakaahua , pērā i ngā matawai ultrasound me ngā matawai CT , me ngā whakamātautau mimi.
• Te tuku i tētahi kōhatu ki tētahi taiwhanga hei whakamātautau. Ki te taea, kohia tētahi kōhatu kua puta mai i roto i tō mimi, ā, ka taea e tō tākuta te tuku atu ki tētahi taiwhanga hei tātaritanga. Pērā ai, ka taea e rātou te kimi i te matū e hangaia ana te kōhatu.

Me pēhea te rongoā mō te cystinuria?

Ko te mea nui rawa atu i te rongoā i ngā kōhatu cystine ko te ngana ki te aukati i te hanganga o aua kōhatu i te tuatahi . Ko tētahi o ngā huarahi pai ki te mahi i tēnei ko te whakaiti i te nui o te cystine i roto i tō mimi. Tērā pea ka whakamahia e tō tākuta he huinga rongoā me ngā huringa kai hei āwhina i te whakaiti i te maha o ngā kōhatu ka hangaia e koe. Mena kāore ngā rongoā me ngā huringa kai e aukati i te hanganga o ngā kōhatu, mena he tino nui ngā kōhatu, ko te pokanga tētahi atu kōwhiringa.

Rongoā

Ki te whakaaro tō tākuta mā te inu nui i te wai ka āwhina i te tango i te kōhatu i a ia anō, ka tohua pea e ia he rongoā mamae kore-tohu (OTC) mō te mamae. Ka tohua pea hoki e ia he rongoā pēnei i te tiopronin me te d-penicillamine hei āwhina i te aukati i te hanganga o ngā kōhatu a muri ake nei.

Mahere Kai

Tērā pea ka tohutohu tō tākuta kia whakarerekē koe i ō tikanga kai hei ārai i te hanganga o ngā kōhatu. Ko ētahi o ēnei ko:

• Te inu i te nui o te wai. Ka āwhina tēnei ki te whakarewa i ngā kōhatu cystine. Tērā pea ka kī atu tō tākuta kia inu koe i te 96 aune (tata ki te 2.8 rita) o te wai i te rā, kia inu rānei i tētahi karāhe wai i ia hāora, kia inu wai rānei ina ara ake koe i te pō.
• Whakawhāitihia te nui o te konutai (tote) e kainga ana e koe. Ko te tikanga me mutu te kai i ngā kai tio, i ngā kai kēne rānei. Ehara i te mea ko te tote anake ka tāpirihia e tātou ki te raihi e kainga ana e tātou, engari he tote huna anō hoki kei roto i ngā mea pēnei i te pīkara, te pīkara, te ranu, ngā kai kua tākaihia, me ngā kai kua hokona mai i te toa. Nō reira, me tūpato koe ki tēnā.
• Te pūmua kararehe , arā, te kai iti iho i te mīti, me te kai i te pūmua tipu. Hei tauira, ka taea e koe te tāpiri i ngā mea pēnei i te pīni kikī, te rēneti, me te pīni soya ki tō kai hei utu mō te mīti me te ika.

Pokanga

Ka pakaru pea ngā kōhatu nunui mēnā kāore e tukuna mā te ara mimi. Ka tino mamae ēnei kōhatu, ā, ka ārai i te rere o te mimi mai i te tinana. He maha ngā tikanga ka taea e tō tākuta te whakamahi hei tango i te kōhatu:

• Ka tukuna he kāmera iti, ngāwari ki roto i tō urethra, mā roto i te pūkahukahu me te urethra, hei kimi i te kōhatu, hei tango atu (ureteroscopy) .
• Ka tapahia he kōhao iti ki te kiri o tō tuara, ka whakaurua he ngongo mā roto ki roto i te whatukuhu, ā, ka tangohia te kōhatu.(te pokanga tango toto mā te kiri).

Me pēhea koe e mōhio ai mēnā kei te angitu te maimoatanga?

Tērā pea ka tonoa e tō tākuta he tātaritanga mimi i ia wā hei tirotiro i ō taumata cystine. Mā tēnei whakamātautau ia e āwhina ki te whakatau mēnā kei te whai hua tō mahere maimoatanga.

Ka taea te whakarewa i ngā kōhatu cystine?

Ae, ka taea te tuku i te kōhatu cystine. Engari me āta aroturuki me te rongoā tonu . Engari i te memeha rawa, ka taea te whakaiti, me te ngāwari ake te tuku.

He aha ngā kai e āwhina ana ki te cystinuria?

Kua whakaatuhia e ngā rangahau he pai ngā hua mō te hunga whai cystinuria mā te whakarerekē i ō rātou kai. Ko ētahi o ēnei ko te inu wai, te kai i ngā hua me ngā huawhenua, me te kai i ngā kai iti te tote. He tino pai ngā hua citrus pērā i te karaka me te rēmana, ngā huawhenua matomato, me ngā rau rau.

Ka taea te ārai i ngā kōhatu cystine?

He mate tuku iho te Cystinuria, nō reira kāore e taea te ārai. Heoi, mā te whai i te mahere maimoatanga a tō tākuta , ka taea e koe te ārai i te hanganga o ngā kōhatu cystine . Arā, ka taea e koe te whakahaere i te hanganga o ngā kōhatu.

He mea mōrearea te mate i te cystinuria?

Kāore tēnei e tino mōrearea ki te ora . Ko te nuinga o te tangata kāore e pāngia e ngā mate kino pēnei i te ngoikore o te whatukuhu. Ko te mea nui ko te mahi tahi me tō tākuta ki te kimi i tētahi maimoatanga e pai ana mōu. He pai te urupare a te nuinga o te tangata ki te maimoatanga. Ka taea hoki te kimi i tētahi maimoatanga kāore e kino ki ngā whatukuhu, ki te pūkahukahu, ki te ara mimi rānei.

He aha te tirohanga ā-muri mō ngā kōhatu sistine?

Ka taea e ngā kōhatu cystine te hanga i ngā wā maha i roto i tō oranga. Mena he cystinuria tōu, me huri tonu koe i tō kai me te tango rongoā hei ārai i te hanganga o ngā kōhatu cystine. Ka tirotirohia koe e tō tākuta, ā, ka whakarite kia tika te rongoā i ngā kōhatu cystine hou e puta mai ana. Hei ārai i te kino o tō ara mimi, ō whatukuhu, me tō pūkahukahu.

Āhea ahau me toro atu ki te tākuta?

Mena kei a koe ētahi tohu o te kōhatu whatukuhu, me toro atu ki te tākuta . Anei ētahi o ngā tohu e tino kitea ana:

• Te mamae i roto i te puku, i te taha o te tuara, i te hope rānei.
• Whakapairuaki me te ruaki.
• Te mamae i te wā e mimi ana.

He mate mō te wā roa te Cystinuria e puta ai ngā kōhatu cystine. Mahi tahi me tō tākuta ki te whakawhanake i tētahi mahere maimoatanga hei ārai i te hanganga o ngā kōhatu. Nā te mea he mate ira te mate, ka hiahia pea te hunga whai cystinuria ki te whai whakaaro ki te tohutohu ira mēnā kei te whakaaro rātou ki te whakatū whānau.He mea āwhina anō hoki te whiwhi i tēnei mate. Ko te rongo pai, ka taea te whakahaere i tēnei mate mā te rongoā, ā, kāore e pāngia ai te whatukuhu e te ngoikore, e te kino rānei ki te ara mimi.

Ngā mea tino nui hei maumahara (Karere hei kawe ki te kāinga)

Āe, me whakarāpopoto e tātou ētahi o ngā mea hei maumahara māu mai i ngā mea kua kōrerohia e tātou:

• Ko ngā kōhatu cystine he momo kōhatu whatukuhu e puta mai ana i tētahi āhuatanga ira e kiia nei ko te cystinuria .
• Ka tino nui ēnei, ā, ka puta mai i ngā wā maha i te roanga o te oranga.
• Ko ētahi o ngā tohu ko te mamae nui, te toto i roto i te mimi, me te ruaki .
• Ko te maimoatanga ko te inu i te nui o te wai, te whakaiti i te tote me te pūmua kararehe, ā, ki te tika, ko ngā rongoā me te pokanga .
• Ahakoa kāore e taea te whakaora katoa i te cystinuria, mā te maimoatanga tika, ka taea te whakahaere i te hanganga o ngā kōhatu, ā, ka taea te noho i te oranga noa.
• Ki te puta mai ngā tohu o te kōhatu whatukuhu , kaua e whakaroa, me toro atu ki te tākuta.

Kia mōhio mai, he mea hei whakamōhio noa iho ēnei kōrero, kaua e whakaarohia he tohutohu hauora. He mea pai tonu te toro atu ki te tākuta mō ngā āwangawanga hauora katoa.

Ngā kōhatu tākihi, ngā kōhatu cystine, cystinuria, ngā kōhatu mimi, ngā mate tākihi, ngā mate ira, ngā mate mimi