Ka nui rawa atu ētahi wāhanga o te tinana? Me kōrero tātou mō te Proteus Syndrome

Kua kite, kua rongo rānei koe i tētahi tangata e tipu nui haere ana ōna wāhanga tinana – hei tauira, he ringa, he waewae rānei – ā, he nui rawa atu i ērā atu? Mēnā rānei ka matotoru ake te kiri i ētahi wāhi, ka rite ki ngā puku rerekē? He tino onge tēnei mate. I tēnei rā ka kōrero tātou mō tētahi mate he ahua rerekē, engari he mea nui kia mōhio te katoa. Ko te ingoa o tēnei mate ko te Proteus Syndrome.

He aha te Mate Proteus?

He kupu ngāwari noa iho, he mate ira tino onge te Proteus Syndrome. Ka nui rawa te tipu o ngā wheua, te kiri, ngā whekau o roto, ngā kiko rānei o tō tinana. He mea nui, he koretake te nuinga o tēnei tipu. Ko te tikanga he rerekē te pānga ki ngā taha matau me ngā taha maui o te tinana. Tērā pea he nui ake tētahi taha, engari he noa pea tētahi taha.

Ko "Proteus" te ingoa o tēnei, i reira tētahi atua Kariki onamata ko "Proteus" tōna ingoa, ka taea e ia te huri ki tētahi āhua. Ka huri anō hoki tēnei mate i te āhua o te tinana, nō reira i tapaina ai.

Kāore he tohu o tēnei āhua i te nuinga o te wā e whakaatuhia ana e te pēpi whānau hou. Ka tīmata tēnei whanaketanga rerekē i waenga i te 6 me te 18 marama te pakeke . Kātahi ka tere haere i roto i ngā tau 10 tuatahi o te oranga, ā, ka kaha ake te kino i a rātou e pakeke haere ana. Haunga ngā panonitanga o te āhua tinana, ka pāngia pea ētahi tāngata e ngā raruraru pūnaha io. Ka nui ake hoki te mōrearea o te hunga whai mate Proteus ki te toto totoka me ngā pukupuku kore-matepukupuku .

Ko wai ka pāngia e tēnei? He pēhea te noa o tēnei?

Ka pāngia te tangata katoa e te mate Proteus, engari kua whakaatuhia e ētahi rangahau he nui ake te kitea o tēnei mate i roto i ngā tāne i ngā wāhine.

He tino onge tēnei mate . E pāngia ana e te iti iho i te kotahi i roto i te miriona tāngata puta noa i te ao. Nā te mea he tino onge, ā, nā te mea he maha atu anō ngā mate e whakaatu ana i te tipu koretake, he uaua ki te tautuhi tika. Nō reira, he uaua ki te kī e hia ngā tāngata e pāngia ana e tēnei mate. E whakapono ana ngā tākuta kāore ētahi tāngata i tautuhia, ā, kua hē te tautuhi i ētahi. Heoi, e whakaponohia ana he iti iho i te kotahi rau tāngata puta noa i te ao e pāngia ana e tēnei mate.

He aha ngā tohu? Me pēhea te mōhio ki tēnei?

Ko ngā tohu tuatahi o te mate Proteus ka puta i waenga i te 6 me te 18 marama te pakeke . Koinei te wā ka tīmata ai te tauira tipu kore taurite i whakahuatia i mua ake nei. Ka rerekē te tauira o tēnei tipu me tōna kaha i ia tangata, i ia tangata. Ka pā ki tētahi wāhanga o te tinana, tae atu ki ngā wheua, te kiri, ngā whekau, me ngā kiko. Ka tere te whanake o te mate i roto i ngā tau tekau tuatahi o te oranga.

### Ngā huringa o ngā wheua:

Tērā pea ka tipu rerekē ngā wheua i roto i ō ringaringa, ō waewae, tō angaanga, me tō tuaiwi.

• Ka taea e ngā ringa me ngā waewae te tipu ki ngā roa tino rerekē . Whakaarohia he roa ake tētahi ringa i tētahi, he rerekē rānei te roa o ō waewae.
• Ka puta pea te piko nui o te tuaiwi (scoliosis) .
• I roto i te wā, ka kore pea ngā hononga e mahi tika i tēnei tipu rerekē.

### Ngā huringa kiri:

Ka taea e te mate Proteus te whakaputa i ngā tipu rerekē o te kiri.

• I ētahi wāhi, ka mātotoru te kiri, ka ara ake, ka hanga he puku miro e rite ana ki te mata o te roro. Ka kiia tēnei he nevus kiko honohono cerebriform .
• Ka kitea ēnei i ngā kapu o ngā waewae , engari i ētahi wā ka puta ki ngā ringa.

He tino ahurei tēnei momo puku kiri, kāore e kitea i roto i tētahi atu mate hauora.

### Ngā uaua toto me te kiko ngako:

• Tērā pea he tipu rerekē o ō uaua toto, arā, ko ngā kapiripiri toto me ngā uaua toto.
• Ka taea e te kiko ngako te tupu nui rawa, ka nui rawa te ngako e kohia ana ki ngā wāhi pērā i te puku, ngā ringa, me ngā waewae.
• He maha ngā wā, ka puta ake ngā pukupuku ngako (lipomas) ehara i te matepukupuku.

### Ngā raruraru o te pūnaha io:

Ka pāngia hoki ētahi tāngata e te mate Proteus e ngā raruraru ki te pūnaha io.

• Ngā hauātanga hinengaro.
• Ngā mate haurangi `(Ngā hopukanga)`.
• Te ngaronga o te tirohanga.

### Ngā panonitanga o te āhua o te kanohi:

Ka taea e te mate Proteus te whakaputa i ētahi āhuatanga motuhake o te kanohi.

• He mata roa.
• Te āhua nei kei te piko haere ngā kokonga o waho o ngā kanohi.
• He ihu papatahi, he pongaponga whānui.
• He rite ki te mea kua puare tō waha.

He aha ngā raruraru mōrearea ka puta mai i tēnei?

Ko te raruraru tino morearea o te mate Proteus ko te deep vein thrombosis (DVT), he toto totoka i roto i ngā uaua hohonu . He maha ngā wā ka pā te DVT ki ngā uaua hohonu o ngā waewae, o ngā ringa rānei, ka mamae, ka pupuhi hoki.

Ki te haere tēnei `DVT` mā roto i te toto ki ngā pukahukahu, ka puta he āhuatanga morearea e kiia nei ko te `Pulmonary Embolism`. Ko te pulmonary embolism te tino take o te mate i roto i te hunga e pāngia ana e te mate Proteus.

He pēhea te whanaketanga o te Mate Proteus? He mea ira mai?

Ko te take o tēnei he panonitanga (whakarerekētanga) i roto i te ira e kiia nei ko te `AKT1`.. Ka āwhina te ira `AKT1` ki te whakahaere i te tipu me te wehenga o te pūtau. Ina whakarerekētia tēnei ira, ka ngaro te kaha o te pūtau ki te whakahaere i tōna tipu. Ka puta te tipu me te wehenga rerekē o te pūtau. Koinei te take o te tipu nui rawa e kitea ana i roto i te mate Proteus.

Ko te mea nui , ehara te mate Proteus i te mate tuku iho. Ka puta tēnei whakarerekētanga ira i muri i te whakatōtōtanga o te kukune, e kiia nei ko te whakarerekētanga tinana . Ka puta noa tēnei whakarerekētanga ira i roto i tētahi pūtau kotahi o te kukune e whanake ana i te tīmatanga o te hapūtanga. I te tipu me te wehewehe o tēnei pūtau kua whakarerekētia, ka puta tēnei whakarerekētanga i roto i ētahi pūtau, ko ētahi kāore. Ka kiia tēnei ko te whakarerekētanga ira mosaic . He rite ki tētahi pikitia i hangaia ki ngā wahi rerekē o ngā tae. Nā te mea ka pā noa te whakarerekētanga ira `AKT1` ki ētahi pūtau o te tinana, ka pā noa te mate ki tētahi wāhanga o te tinana.

Me pēhea te tautuhi a ngā tākuta i tēnei?

Ka whakahaerehia e tō tākuta he whakamātautau tinana hei tautuhi i te mate Proteus. Ka whakamahia hoki e rātou he rārangi arowhai motuhake o ngā tohu e pā ana ki te mate. Kia whakaarohia ai e te tākuta tēnei tautuhi, me toru ngā tohu noa e kitea ana. Ko ēnei:

• Te tohatoha o te mosaic : Ko te tikanga tēnei kua horapa ngā tipu puta noa i te tinana. He tipu ētahi wāhanga o te tinana, ko ētahi kāore.
• Te puta noa mai i ētahi wā : Ko te tikanga kāore he tangata kē atu o tō whānau e pāngia ana e ēnei tohu.
• Te ahunga whakamua : Ko te tikanga tēnei, i roto i te wā, kua tino rerekē te āhua o ngā wāhanga tinana e pāngia ana nā te tipu nui rawa atu.

Hei tāpiri atu ki tēnei, ka rapu tō tākuta i ētahi atu tohu motuhake. Hei tātaritanga mō te mate Proteus, me whai tohu motuhake koe mai i ia kāwai kei runga i te rārangi arowhai a te tākuta.

### He aha ngā momo whakamātautau e mahia ana?

Kāore te whakarerekētanga ira e puta ai te mate Proteus i roto i ngā pūtau katoa o te tinana. Nō reira, he uaua pea te whakamātautau ira. Nā te mea kāore pea te whakarerekētanga ira i roto i te tauira toto, he iti noa iho rānei te nui o te kitea.

Heoi, ka taea e ngā tākuta te kite i tēnei ira kua whakarerekētia mā te tango i tētahi tauira iti o tō kiko kua pāngia (biopsy) me te whakamātautau (whakamātautau DNA) . Ka whakamahia te DNA mai i taua tauira hei kite i te ira kua whakarerekētia.

He rongoā kei reira mō tēnei? Ka taea te whakaora?

Engari, kāore he rongoā mō te mate Proteus . Ko te arotahi o te maimoatanga he whakahaere i ō tohumate. Me mahi tahi koe me tētahi rōpū tohunga hei āwhina i a koe me ō hiahia hauora takitahi.

• Taote pokanga wheuaHe rongoā mō ngā raruraru o ō wheua. He maha ngā maimoatanga hei whakaiti i te tipu nui o te wheua i roto i ngā ringaringa, ngā waewae, me ngā maihao. Ka taea hoki e rātou te whakatika i ngā raruraru o te tuaiwi me ngā hononga. I mua i tētahi pokanga, ka arotakehia koe e te tākuta toto hei aromatawai i tō mōrearea o te toto totoka.
• Me toro atu pea koe ki tētahi tākuta kiri mō te nui rawa o te hinu kiri me ngā panoni kiri. Tērā pea ētahi raruraru e hiahiatia ana he maimoatanga tonu, he maimoatanga auau hoki. Mō ētahi atu raruraru, ka whai pea tō tākuta i tētahi huarahi "tāria, tirohia". Arā, ka aroturukihia e rātou tō āhua i mua i te tūtohutanga i tētahi maimoatanga motuhake.

Ko ētahi atu kaiwhakarato hauora ka whai wāhi pea ki tō tiaki ko:

• He tohunga rongoā whakaora (Taonga rongoā tinana)
• He tākuta e tohunga ana ki ngā mate manawa (Pulmonologist)
• Kaiwhakaora tinana
• Kaiwhakaora mahi
• He tangata e hanga ana i ngā hu me ngā tautoko waewae motuhake (Pedorthist)

Nō nā tata nei anō i kitea ai e ngā kaipūtaiao te ira e puta ai te mate Proteus, ka ārahi pea tēnei ki ngā whanaketanga nui i roto i te whanaketanga o ngā rongoā hou me ētahi atu maimoatanga.

He ara hei ārai i tēnei?

Kāo, kāore e taea te ārai i te mate Proteus. Nā te mea he mate ira tēnei. Ko te whakarerekētanga ira e hua ai tēnei he mea tūpono i te whanaketanga o te kukune. Ehara i te mea i puta mai i tētahi mea i mua atu, i te wā rānei e hapu ana. Nō reira kaua e manukanuka ki tēnā.

Kei te pēhea te roa o te oranga?

Ko te roanga o te oranga o te tangata e pāngia ana e te mate Proteus e whakawhirinaki ana ki ngā wāhanga o te tinana e pāngia ana me te kaha o te mate. Ka taea e ngā raruraru te whakararu i te ora , ā, he nui ake te tūponotanga ka mate i te mate tonu. Ko ēnei raruraru ka uru atu ki te deep vein thrombosis (DVT), te pulmonary embolism, me te mate pukupuku. Ko te pulmonary embolism te tino take o te mate i roto i te hunga e pāngia ana e te mate Proteus.

I te katoa, tata ki te 25% o te hunga e pāngia ana e te mate Proteus ka mate i mua i te 22 tau te pakeke. Heoi, ko te hunga he ngawari ngā tohu he pai te roa o te oranga me te whai hua o te maimoatanga.

Me pēhea te noho tahi me te Mate Proteus?

He tino whakarihariha, he tino uaua hoki te noho tahi me ngā panonitanga tinana e puta mai ana i te mate Proteus. He mea nui kia ako koe me tō whānau i ngā mea katoa e taea ana mō tēnei mate.Mā te kōrero ki ētahi atu tāngata e pāngia ana e tēnei mate, me te whakarongo ki ō rātou wheako, ka āwhina i a koe kia mōhio kāore koe i te mokemoke, ā, kei reira ētahi e mārama ana ki a koe. Pātai atu ki tō tākuta mō ngā rauemi hei āwhina i a koe ki te whakahaere me te aro atu ki tō āhua.

He wheako whakamataku, he wheako mamae hoki te kitenga kei a koe, kei tāu tamaiti rānei tētahi mate ira onge. He tino uaua pea mēnā ka puta he huringa nui ki te tinana i roto i te mate. Nō reira, he mea nui kia kimihia he tākuta ka taea te tātari tika i te mate Proteus me te whakatū i tētahi rōpū tohunga. Mēnā he maimoatanga tika, he pai te roa o te oranga o te nuinga o te tangata. He mea nui anō hoki kia kimihia he rōpū tāngata ka taea te tautoko i a koe i a koe e anga atu ana ki tēnei tātaritanga uaua.

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

• He tino onge te mate Proteus ira, e tohuhia ana e te tipu nui rawa, kore taurite o ētahi wāhanga o te tinana.
• Ehara tēnei i te mea tuku iho . Nā te whakarerekētanga ira matapōkere e puta ana i te wā o te kukune.
• Ka rerekē rawa ngā tohu i ia tangata, i ia tangata.
• Ahakoa kāore he rongoā katoa , he maha ngā maimoatanga hei whakahaere i ngā tohu.
• He mea tino nui kia rapu tautoko mai i tētahi tīma tākuta motuhake , kia mōhio hoki ki ngā āhuatanga o te mate.
• He mea nui kia mōhio ki ngā raruraru pēnei i te deep vein thrombosis (DVT) me te pulmonary embolism (Pulmonary Embolism) hei tiaki i ngā oranga.
• Mena kei te pāngia koe, tō whanaunga rānei e tēnei mate, kaua e wareware ki te kaha e ahu mai ana i te tautoko hinengaro me ngā rōpū tautoko .

Ko tāku tumanako ka whai hua ēnei mōhiohio ki a koe. Mēnā he āwangawanga āu, kaua e wareware ki te rapu tohutohu hauora.

` Te Mate Proteus, Ngā Mate Ira, Te Whanaketanga Rerekē, Ira AKT1, DVT, Te Pukupuku Pūkahukahu, Ngā Mate Kiri, Te Whanaketanga Wheua, Ngā Mate Onge