Kei te pāngia koe e ngā puku e tata ana ki ō hononga? Me kōrero tātou mō te Tumoral Calcinosis!

Kei te ahua pēhia koe e te puku, e te wāhi mārō rānei i raro i te kiri e tata ana ki tētahi hononga pēnei i tō tuke, pakihiwi, turi rānei? Kāore pea e mamae ina pēhia, engari he nui rawa. He mea noa te mataku ina kite koe i tētahi mea pēnei. Engari ehara i te mea he mate pukupuku kino katoa ēnei puku. I tēnei rā ka kōrero tātou mō tētahi āhuatanga e whakaatu ana i ēnei tohu, engari ehara i te mate pukupuku, ā, he onge noa iho engari he mea nui kia mōhiotia. Koia te 'Tumoral Calcinosis' .

He aha te mate pukupuku Calcinosis?

He māmā noa iho, he mate ira te tumoral calcinosis. Koinei te wā e tino teitei rawa ai te taumata o te matū e kiia nei ko te phosphate i roto i tō toto. Ko te Phosphate he matūriki hiko, he ion rānei, kei roto ko te kohuke phosphorus. Nō reira, i roto i te tangata e pangia ana e te tumoral calcinosis, ka kohia te phosphorus me te konupūmā i roto i te tinana, ka puta he puku i raro i te kiri, inā koa i te taha o ngā hononga.

He mea nui te whākōkī me te konupūmā mō te hanga i ngā niho me ngā wheua hauora, kaha. Engari ina tino teitei rawa, ka tīmata te puta o ngā tipu kore-matepukupuku (pai), arā, ehara i te mea morearea, engari he rite ki ngā pukupuku, ki roto i ngā kiko. Koinei te mea e kiia nei e tātou ko te matepukupuku calcinosis.

He ingoa anō tēnei mō tēnei?

Āe, ko te ingoa hauora katoa mō tēnei mate ko 'Hyperphosphatemic Familial Tumoral Calcinosis' (HFTC) . He ingoa roa, kāore? Ka karangatia hoki e ētahi tākuta ko 'Teutschlaender disease'. Hei maumahara ki a Otto Teutschlaender, he tākuta Tiamana i ako i tēnei mate mai i te tekau tau 1930 ki te tekau tau 1950.

He aha te tikanga o tēnei ingoa hauora roa?

Me wehewehe tēnei ingoa kia rua, kia mārama ai? He māmā ake tēnā.

• Hyperphosphatemic: Ko te tikanga he tiketike te taumata phosphate i roto i te toto.
• Whānau: Ko te tikanga he mate tēnei e pā ana ki ngā whānau, ā, he mea tuku iho.
• Puku: E whakamahia ana tēnei kupu nā te mea he rite te āhua o ēnei tipu rite ki te puku ki ngā puku matepukupuku.
• Te mate pāwera: Ko te tikanga ka whakatakotoria te konupūmā ki roto i ngā kiko o ō uaua me te pūnaha musculoskeletal.

Kua mārama koe ki te tikanga o taua ingoa roa inaianei?

He pēhea te pānga o te Tumoral Calcinosis ki te tinana?

Ko ēnei puku, e kiia nei ko te tumoral calcinosis , ka puta pinepine i raro i te kiri, e tata ana ki ngā hononga . Ka taea e rātou te tupu i roto i ngā hononga neke atu i te kotahi, engari ka taea hoki e rātou te tupu i roto i ngā hononga maha. Ko ngā wāhi tino kitea ko:

• Ngā hononga hope
• Ngā tuke
• Ngā pakihiwi
• Raro
• Ngā hononga o te ringaringa
• E pā ana ki te taurakira
• Rohe kauae

He pēhea te noa o tēnei āhuatanga?

Kāore i te tino mōhiotia e ngā tohunga hauora te tokomaha o ngā tāngata e pāngia ana e tēnei mate pukupuku. Nā te mea he tino onge tēnei mate , he mea tino noa i roto i ngā tāngata o Āwherika me te Rawhiti o Waenganui. Ka pāngia e te tāne me te wahine.

He aha ngā momo matua o te Tumoral Calcinosis?

Ko te momo tino noa o tēnei ko te Hyperphosphatemic Familial Tumoral Calcinosis, i kōrerohia e tātou i mua ake nei. Hei tāpiri, kei reira ētahi atu momo onge:

• Te mate Hyperphosphatemia-hyperostosis (HHS): He āhuatanga tēnei e hua ai te tipu nui o te wheua me te whanaketanga o ngā whara wheua ehara i te matepukupuku.
• Normophosphatemic tumoral calcinosis: He maha ngā wā ka kitea tēnei momo i roto i te hunga e pāngia ana e te ngoikore o te whatukuhu. Ka taea e ia te whakaputa i tētahi āhuatanga e kiia nei ko te hyperparathyroidism, he repe parathyroid e kaha rawa ana te mahi.

He aha te take o te mate pukupuku Calcinosis?

He mate pākia te mate pukupuku calcinosis e tukuna iho ana . Tata ki te hautoru o te hunga whai mate pēnei he huringa, he whakarerekētanga rānei, kei roto i tō rātou ira FGF23. Ka āwhina te ira FGF23 i ō tātou pūtau wheua ki te hanga i tētahi pūmua e kiia nei ko te fibroblast growth factor (FGF) 23. Kei ētahi tāngata anō hoki tēnei mate nā ngā huringa o ngā ira GALNT3 me KL. Ka whakahaere ēnei ira i te whakaputanga o te FGF23. Ko te hunga whai mate pukupuku calcinosis nomophosphatemic he whakarerekētanga kei roto i te ira SAMD9.

I te nuinga o te wā, ka tohutohu te pūmua `FGF23` i ō tātou whatukuhu ki te nui o te phosphate hei mimiti anō mai i te toto. Engari ina puta he whakarerekētanga ira, ka hē tēnei tukanga. Kātahi ka mimiti anō ngā whatukuhu i te nui atu o te phosphate i te mea e tika ana. I te nuinga o te wā, ka tukuna e ngā whatukuhu te phosphate nui rawa atu ki roto i te mimi. Engari i roto i te mate pukupuku calcinosis, ka herea tēnei phosphate ki te konupūmā, ka hanga he pukupuku i roto i ngā kiko ngohengohe.

Ko wai kei te mōrearea ki te whakawhanake i tēnei mate?

He mate tuku iho ā-ira te mate pukupuku calcinosis. He uaua ki te mārama ki tēnei, engari i ngā kupu māmā noa iho, kia pāngia ai koe e te mate, me riro i a koe te whakarerekētanga ira mai i ō mātua e rua. Tērā pea ko ō mātua te hunga kawe i te whakarerekētanga ira, engari kāore pea i a rātou te mate.

Mena he kawe mate ngā mātua e rua, ka pā ki ia tamaiti te tūponotanga e whai ake nei:

• Kotahi i roto i te whā te tūponotanga kāore he ira kua whakarerekētia (kāore he mōrearea ki te pāngia e te mate).
• Kotahi i roto i te whā te tūponotanga ka pāngia koe e te mate pukupuku calcinosis.
• Kotahi i roto i te rua te tūponotanga ka noho hei kaikawe engari kāore e pāngia e te mate.

He aha ngā tohu o te Tumoral Calcinosis?

Nā tēnei āhuatanga ka puta ngā puku rerekē te rahi i te taha o tētahi, neke atu rānei o ngā hononga. He maha ngā wā ka puta mai ēnei puku i te tamarikitanga, i te taiohitanga rānei . Heoi, ka taea hoki te whanake i roto i ngā pēpi me ngā kaumātua. I ētahi wā, he torutoru noa iho ngā puku ka puta i roto i te oranga. He maha rānei ngā puku ka puta i te wā kotahi.

Tērā pea ka pāngia koe e ēnei tohu:

• Te uaua ki te neke, ki te piko rānei i te hononga e pāngia ana kia tika.
• He puku mārō, kāore he mamae (tērā pea ka paku mamae ētahi puku ki te pā atu).
• Te mamae o te uaua me te angaanga.

Me pēhea te tautuhi i te mate pukupuku Calcinosis?

He tākuta tohunga te taote wheua e tohunga ana ki ngā raruraru wheua me ngā hononga. Ka tirohia koe e ia, ka pātai atu mō ō tohumate. Kātahi ka mahia e ia tētahi, ētahi rānei o ngā hihi-X e whai ake nei hei rapu i ēnei pukupuku me ētahi atu raruraru wheua:

• Whakamātautau hihi-X `(X-ray)`
• Matawai CT
• Matawai MRI `(MRI)`

He aha atu ngā āhuatanga e whakaatu ana i ngā tohu rite ki te Tumoral Calcinosis?

He mea tino nui tēnei. Nā te mea he mea nui kia tika te tautuhi i te mate . Ko te take he maha atu ngā mate e whakaatu ana i ngā tohu rite, engari he kino ake. Ko ētahi o ēnei mate ko:

• Ngā pukupuku kiko ngohengohe pai me ngā pukupuku kiko ngohengohe kino
• Te myonecrosis konupūmā (he pukupuku o ngā putunga konupūmā i roto i ngā uaua o te waewae)
• Te uaua konupūmā (ngā putunga konupūmā me te uaua konupūmā)
• Calcinosis circumscripta (ngā putunga konupūmā i roto i te kiri me ngā kiko o ngā ringa me ngā waewae)
• Te mate pāwera o te ngoikore o te whatukuhu (ngā putunga konupūmā i roto i te hunga e whiwhi ana i te dialysis)
• Calcinosis universalis (te whakatakotoranga o ngā tote konupūmā ki roto i te kiri, ngā kiko, ngā uaua me ngā uaua)
• Ngā mate hononga-kiko
• Kauti
• Myositis ossificans (te uaua e huri ana hei wheua)
• Osteosarcoma me te sarcoma (matepukupuku wheua)
• Ko te chondromatosis synovial (ngā pona cartilage i roto i ngā hononga)

Titiro? Ahakoa he rite ngā tohu, he rerekē rawa te pūtake. Koia te mea nui kia mōhiotia te āhua o te mate.

He aha ngā maimoatanga kore-pokanga?

Tērā pea ka whakaitihia e tō tākuta te nui o te phosphate i roto i tō toto.Tērā pea ka tohua he rongoā here-phosphate. Ina whakakotahitia ēnei rongoā here-phosphate me te rongoā e kiia nei ko te acetazolamide (e whakamahia whānuitia ana mō te mate haurangi me te glaucoma), ka taea e rātou te aukati i te kaha o te tinana ki te mimiti i te phosphorus. Me kai pea koe i tētahi kai iti te phosphorus .

He aha te kai iti te whākōkī?

Mena he mate pukupuku tōu, ka tūtohu pea tō tākuta i tētahi kai iti te whākōkī. Kei roto i te mīti, te ika, me ngā hua miraka te whākōkī. Ka tāpirihia hoki te whākōkī ki ētahi atu kai hei mea tiaki, hei mea kakara rānei.

Ngāna kia whakaitihia ngā kai me ngā inu he nui te phosphorus, pērā i:

• Pia, kōra, tī hukapapa me ngā inu tiakarete.
• Ngā momo tiakarete me te karamere.
• Ngā kai miraka (miraka, tīhi, aihikirīmi, miraka kawa ehara i te Kariki, hupa kirīmi).
• Ngā kai moana pērā i te tio me te tātini, me ngā whekau o roto pērā i te ate kau me te ate heihei.
• Ngā mīti tukatuka (hōtiti wera, mīti tina, pēkana, hōtiti).
• Ngā hua i hangaia mai i te parani oati.

He pai ake te kōrero ki tō tākuta, ki tō tohunga kai rānei mō tēnei mea mō ētahi atu kōrero.

He aha ngā maimoatanga pokanga?

Tērā pea me pokanga koe hei tango i ngā pukupuku nunui rawa atu, ā, kāore e taea te mahi tika. Heoi anō, he nui ake te tūponotanga ka tipu anō ēnei pukupuku . Tērā pea ka tangohia e tō tākuta tētahi tauira o te kiko kua tangohia, ā, ka mahia he koiora hei whakaū i te mate.

He aha ngā raruraru ka pā mai i te Tumoral Calcinosis?

He tino onge te pānga o tēnei āhuatanga ki ō oko toto, ki tō roro rānei, ka piki ake ai tō tūponotanga ki ngā raruraru hauora nui pērā i te mate ngakau me te whiu.

Hei tāpiri, ka taea hoki e tēnei āhuatanga te whakaputa i ngā āhuatanga e whai ake nei:

• Ngā whakapūkara o te kornea, ngā rārangi angioid rānei (ngā rārangi whero) i roto i te kornea o ngā kanohi.
• Ngā raruraru niho.
• Te tipu nui rawa o te wheua (hyperostosis).
• Te mumura i roto i ngā wheua roa (diaphysis) i roto i ō waewae, i ō ringa rānei.

Ka taea te ārai i te mate pukupuku Calcinosis?

Mena kei a kōrua ko tō hoa te whakarerekētanga ira e puta ai te mate pukupuku, ka taea e koe te kōrero ki tētahi kaitohutohu ira mō ngā huarahi hei whakaiti i te tūponotanga o te tukunga iho o te whakarerekētanga ira ki ō tamariki. Ka whiriwhiri ētahi i tētahi tikanga e kiia nei ko te Preimplantation Genetic Diagnosis (PGD) . Kei roto i tēnei ko te tautuhi i ngā kukune kāore i te whakarerekētanga ira. Kātahi ka whakamahia e tō tākuta tētahi tikanga e kiia nei ko te In Vitro Fertilization (IVF) hei whakawhiti i ngā kukune hauora ki roto i te pūnaha whakaputa uri o te wahine. Mā tēnei tikanga, e kore tō tamaiti e whiwhi i te whakarerekētanga ira e puta ai te mate.

He aha te tirohanga mō te tangata e pāngia ana e te Tumoral Calcinosis?

Ko te nuinga o te hunga e pāngia ana e te mate pukupuku calcinosis he ora tonu, he ora pukumahi . Kāore i te maha ngā raruraru hauora nui e pā mai ana i tēnei mate. Heoi, me toro atu koe ki tō tākuta i ia wā, i ia wā, kia tirohia ai mēnā ka hoki mai anō.

Āhea ahau me toro atu ki te tākuta?

Tirohia te tākuta mēnā kei a koe ēnei tohu:

• Te hekenga o te whānuitanga o te nekehanga i roto i te hononga.
• Te mamae o ngā hononga, te mamae o te wheua, te mamae rānei o ngā uaua.
• Te putanga mai o tētahi pukupuku, he puku rānei i raro i te kiri.

He aha taku e pātai atu ai ki taku tākuta?

Ka taea e koe te pātai atu ki te tākuta i ngā pātai penei:

• He aha i pā mai ai te mate pukupuku calcinosis ki ahau?
• He aha te maimoatanga pai rawa atu mōku?
• Me pokanga ahau?
• He aha ngā momo kai me whakaiti e au?
• Me āwangawanga ahau mō ngā raruraru?
• Me whakamātautau ira te toenga o tōku whānau?

Ka taea te kī he mate pukupuku te mate pukupuku o te pukupuku?

Ahakoa te kupu "tumoral" i roto i te ingoa, ko ngā pukupuku e puta mai ana i te tumoral calcinosis ehara i te mea he mate pukupuku . Kāore he mōrearea o ēnei pukupuku ka kino. Nō reira kaua e manukanuka.

He pono he ahua whakararuraru, he whakamataku hoki ngā pukupuku i raro i te kiri nā te mate pukupuku calcinosis. Engari mō te nuinga o te tangata, kāore ēnei pukupuku e puta he raruraru nui. Ka taea e te rongoā te whakaheke i te taumata o te phosphorus i roto i te toto. Ka tūtohu pea tō tākuta kia kite koe i tētahi tohunga kai hei āwhina i a koe ki te kai i tētahi kai iti-phosphorus. Mena he mamae ō hononga, ō wheua, ō uaua rānei e whakawhāiti ana i tō nekehanga, kōrero ki tō tākuta. Ahakoa ka taea te tango i ngā pukupuku mā te pokanga, kia maumahara ka taea e rātou te hoki mai anō.

Karere hei Maumahara (Ngā Mea hei Mahara)

Āe, me whakarāpopoto tātou i ngā kōrero tino nui i kōrerohia e tātou i tēnei rā mō te Tumoral Calcinosis:

• He mate onge tēnei, he mate tuku iho. Mā te pikinga o te taumata o te phosphate i roto i te toto ka honoa rātou ki te konupūmā hei hanga i ngā puku tata ki ngā hononga.
• Ehara ēnei pukupuku i te matepukupuku .
• He maha ngā wā kāore ēnei e mamae, engari ka taea e rātou te whakararuraru i te nekehanga o ngā hononga.
• Kei roto i te maimoatanga ngā rongoā whakaiti phosphate, he kai iti te phosphate, ā, ki te tika, he pokanga.
• Ki te kite koe i tētahi puku pēnei, kaua e mataku, engari me rapu tohutohu hauora. He mea tino nui te tautuhi tika i tō mate.
• He tokomaha te hunga e ora noa ana, e ora kaha ana me tēnei mate.

Nō reira, ki te mea kei a koe, kei tētahi rānei e mōhio ana koe, he pai ake te toro atu ki tētahi tākuta mō ētahi tohutohu.

Te mate pukupuku calcinosis, ngā whēkau hononga, te phosphate, te konupūmā, ngā mate ira, ngā pona kiri, ngā mate wheua