Ka kata ohorere rānei tāu tamaiti i ētahi wā, kāore he take? Kāore e taea te aukati i taua kata, pērā i te ngangau? Kei te whakaatu rānei tāu tamāhine, tāu tama rānei i ngā tohu o te pakeketanga wawe? He mea noa ki a koe, hei mātua, te mataku me te manukanuka ina kite koe i ēnei mea. I tēnei rā kei te kōrero tātou mō tētahi mate roro tino onge, engari he mea nui kia mōhiotia, ka puta ai ēnei tohu.
He aha rawa te Hypothalamic Hamartoma (HH)?
He kupu ngāwari noa iho, he tipu puku ehara i te matepukupuku e tupu ana i roto rawa i te roro, i roto i tētahi wāhanga tino nui e kiia nei ko te hypothalamus, he rite te rahi ki te aramona.
Whakaarohia te hypothalamus hei pokapū whakahaere matua o tō tātou tinana. He mahi nui tāna ki te pupuri i te taurite o ngā mea katoa (homeostasis), e hono ana i te pūnaha homoni me te pūnaha io o tō tātou tinana.
Ko ētahi o ngā mea e whakahaerehia ana e te hypothalamus ko:
- Te toto totoka
- Te pāmahana tinana
- Ngā kare ā-roto o te ahotea, te manukanuka
- Te hiakai me te matewai
- Te tukunga o ngā homoni mai i te repe pituitary
- Ngā huringa moe me te ara ake
Ka tīmata te whanaketanga o tēnei pukupuku HH i te wā e noho tonu ana te tamaiti i roto i te kōpū . I te whanaketanga o te roro o te tamaiti, ka tipu haere tēnei pukupuku. Kaua e manukanuka, kāore e horapa ki ētahi atu wāhanga o te roro, ki ētahi atu okana rānei o te tinana. I te nuinga o te wā, kotahi anake te pukupuku, engari he tino onge ka maha.
Ko te mea pai rawa atu, kei reira ngā maimoatanga pai hei whakahaere i ngā tohumate e puta mai ana i tēnei mate.
He aha ngā tohu o tēnei mate?
Kāore ēnei tohu e ōrite mō ia tamaiti, ā, ka rerekē pea i waenga i tētahi tamaiti me tētahi atu. Heoi, e toru ngā rōpū matua o ngā tohu ka kitea.
1. Te pakeketanga wawe
Koinei tētahi o ngā tohu matua o tēnei mate. I roto i ngā kupu māmā, ka tīmata te tinana o te tamaiti ki te pakeke i mua noa atu i te pakeketanga noa. Ka kiia tēnei ko te "Central Precocious Puberty". Ka puta tēnei nā te mea ka tukuna e te roro ngā tohu hei whakaputa i ngā homoni ira tangata i te wā tino wawe.
Ko te tikanga, mehemea ka whakaatuhia e te kōtiro ngā tohu o te pakeketanga i mua i te 8 tau te pakeke, me te tama i mua i te 9 tau te pakeke, me āwangawanga tātou.
| Ngā āhuatanga e kitea ana i roto i ngā kōtiro | Ngā āhuatanga e kitea ana i roto i ngā tama |
|---|---|
| Te whanaketanga o ngā ū | Te hōhonutanga/te pāorooro o te reo |
| Te tupu o ngā huruhuru i roto i ngā kēkē me ngā wāhi tūmataiti | Te tupu o ngā huruhuru i te mata, i ngā kēkē, me ngā wāhi tūmataiti |
| Te paheketanga (te paheketanga) | Te whakanui ake i ngā porotiti me te ure |
| Te hanganga o te hakihaki | Te hanganga o te hakihaki |
2. Ngā mate hopuhopu
He maha hoki ngā mate hopuhopu. He momo hopuhopu motuhake anō hoki kei roto i tēnei mate. Ka kiia ko te hopuhopu Gelastic .
Ko te hopukanga gelastic he mahi hiko rerekē i roto i te roro e puta ai te kata kāore e taea te whakahaere, kāore he take mārama. He rite ki te kata nui.
Ehara tēnei kata i te mea harikoa. Tērā pea he torutoru hēkona noa iho te roa, engari ka taea te puta i ētahi wā i te rā, ā, i ētahi wā kino, he maha ngā wā i te hāora. I te nuinga o te wā, ka tīmata tēnei momo hopuhopu i mua i te kotahi tau te pakeke o te tamaiti . Engari ka heke haere i muri i te 10 tau te pakeke.
Hei tāpiritanga, ka kitea anō hoki ētahi atu momo hopuhopu.
- Ngā hopukanga atonic (ngā whakaekenga taka): Te ngaronga ohorere o te mana whakahaere o te tinana, te ngoikore o ngā uaua, me te hinga ki te whenua.
- Ngā haurangi dacrystic: Te tangi kore e taea te whakahaere, te tangi tonu me te kore take, he rite ki te kata.
- Ngā hopukanga tonic-clonic: E mōhio ana tātou katoa, he hopukanga nui e ngaro ai te māramatanga me te oho o te tinana.
3. Ngā Tohu Pūnaha Io me ngā Huringa Whanoke
I te mea ko te hypothalamus tētahi wāhanga o te roro e āwhina ana ki te whakahaere i ō tātou kare ā-roto me ō tātou whanonga, ka pā te HH ki ngā whanonga, te wairua, me te kaha ako o te tamaiti. Ko ngā raruraru ka puta ko:
- Te uaua ki te aro me te hihiko rawa (Attention-deficit/hyperactivity disorder - ADHD)
- Whanoke kino, whanoke `(Te mate whanonga)`
- Hauātanga hinengaro
- Ngā mate hinengaro
- Mate ātete-whakahē
- Whēkenga tauhohe
- Ngā raruraru mahara poto
He momo hematoma hypothalamic kei reira?
Āe, e rua ngā momo matua o tēnei pukupuku, i runga i tōna taunga i te hypothalamus me ngā tohu e pā mai ana.
1. Te whara o roto i te hypothalamus: I tēnei momo, kei muri i te hypothalamus te pukupuku. Ka whakararuraru tēnei i te mahi hiko o te roro, ka nui ake te tūponotanga o ngā mate hopuhopu me ngā raruraru whanonga .
2. Te whara parahypothalamic (te whara pedunculated): Kei mua o te hypothalamus te pukupuku i konei. Koinei te tohu matua e kitea ana i te wā o te pakeketanga wawe .
Tērā pea ka pāngia ētahi tamariki e ngā momo e rua.
He aha te take o tēnei āhuatanga?
Kāore anō kia kitea he take motuhake mō tēnei . Ā, kāore anō kia kitea he huarahi hei ārai. I te nuinga o te wā, ka puta noa mai. Ko te tikanga, ehara i te mea tuku iho. He iti rawa te tūponotanga ka pāngia tāu tamaiti e tēnei mate nā te mea kei roto i tō whānau tētahi o rātou.
Engari kua kitea i roto i ngā rangahau hou kei roto i te ira e kīia nei ko te `GLI3` ētahi huringa i roto i te kiko o ēnei pukupuku. Kei te haere tonu ngā rangahau mō tēnei.
Kua kitea hoki he hononga pea tō te mate ira e kiia nei ko te "Pallister-Hall syndrome (PHS)" ki te HH.
Me pēhea te tautuhi tika i tēnei mate?
Tērā pea ka tonoa e tō tākuta he matawai MRI (Magnetic Resonance Imaging) o tō roro hei whakaū i te mate. Ka taea e te MRI te whakaputa i ngā whakaahua tino mārama, taipitopito hoki o te roro.
Me hoatu pea he rongoā whakamoe māmā ki ngā tamariki nohinohi kia kore ai rātou e nekeneke i te wā o te MRI, nā te mea ka kore e mārama ngā whakaahua o te matawai mēnā ka neke rātou.
Mena ka pāngia tāu tamaiti e ngā mate haurangi, ka tūtohutia hoki kia whakamātauria he whakamātautau EEG (Electroencephalogram) . Mā tēnei ka whakamātau i te mahi hiko o te roro.
Hei tāpiritanga, ka taea te whakahaere whakamātautau io hei ine i ngā pūkenga whakaaro, ako, me te mahara o te tamaiti, ā, ka taea hoki te whakahaere whakamātautau toto hei tirotiro i ngā taumata homoni. He tino āwhina ngā mōhiohio mai i ēnei whakamātautau ki te whakamahere maimoatanga.
Me pēhea te rongoā?
E rua ngā whāinga matua o te rongoā i te HH. Ko tētahi he whakahaere i te pakeketanga wawe, ko tētahi he whakahaere i ngā mate haurangi.
- Mō te pakeketanga wawe: Ka whakamahia ngā rongoā mō tēnei. Ka hoatu ngā agonists homoni tuku-gonadotropin hei werohanga ia marama. Mā tēnei ka whakahaere i te tukunga o ngā homoni, ā, ka whakahaere hoki i ngā tohu o te pakeketanga. Ina tae te tamaiti ki te tau e tika ana kia puta ai te pakeketanga, ka mutu te rongoā e te tākuta.
- Mō ngā mate hopuhopu: Kāore ngā 'mate hopuhopu Gelastic' i kōrerotia e tātou i mua ake nei e pai te urupare ki ngā rongoā ārai hopuhopu paerewa. Nō reira , he maha ngā wā ka hiahiatia he pokanga, he tikanga motuhake rānei .
Kei raro nei ētahi o ngā tikanga matua e whakamahia ana hei tango, hei whakangaro rānei i te hematoma hei whakahaere i ngā haurangi.
| Tikanga maimoatanga | He whakamārama ngāwari noa iho |
|---|---|
| Te pokanga maripi Gamma | Ehara tēnei i te pokanga tūturu. Kāore te kiri e tapahia. Ka arohia he hihi kaha (irahiko) ki te wāhi o te pukupuku roro, ā, ka whakangaromia. |
| Te tango i te kiri (pokanga tango) | Ka tangohia e te taote pokanga te pukupuku mā te whakatuwhera i te angaanga (craniotomy), mā te hanga rānei i tētahi kōhao iti ki te angaanga, kātahi ka whakauru i tētahi taputapu pēnei i te endoscope. |
| Te tango i te taiaho stereotactic | He tikanga iti rawa te whakaekenga tēnei. Ka tukuna he hihi laser mā roto i te taura muka whatu hei whakangaro i te pukupuku. |
| Te whakawera wera stereotactic | Ka whakamahia e tēnei tikanga te wera hei whakangaro i te pukupuku. Ka whakaurua ngā taputapu angiangi mā te tumuaki, ā, ka whakamahanatia ki ngā ngaru reo irirangi hei whakangaro i te kiko pukupuku. |
Hei tāpiritanga, ka taea te whakarato i te tautoko mātauranga motuhake, te whakaora whanonga, me te rongoā i te kura hei aro atu ki ngā raruraru whanonga me te ako a te tamaiti.
Āhea koe me toro atu ki te tākuta?
Hei whaea, hei pāpā rānei, ko koe te mea tino mōhio ki tāu tamaiti. Nō reira, ki te kite koe i ētahi huringa, i ētahi tohu rerekē rānei i roto i tāu tamaiti, kaua e warewarehia . Ki te pāngia tāu tamaiti e ētahi o ēnei tohu e whai ake nei, rapua tonutia he tohutohu hauora.
- Ngā haurangi: Ina koa ka kata noa iho, kāore he take.
- Ngā tohu o te pakeketanga wawe: E ai ki tā tātou i kōrero ai i mua ake nei, ko ngā mea pēnei i te tupu o te makawe me ngā panonitanga o te reo.
- Ngā raruraru ako: te kore e taea te arotahi, ngā huringa o te mahara.
- Ngā huringa whanonga: Te riri mō ngā mea iti, te huri hei pukuriri.
- Ngā koretake o ngā homoni: te matewai nui, ngā huringa o te hiakai, ngā huringa ohorere o te kare ā-roto.
Tērā pea ka tino mataku koe ina kitea e koe he HH tō tamaiti. Engari kia maumahara, he tino whai hua ngā maimoatanga mō tēnei. Kōrero ki tō tākuta, ā, whiriwhiria te maimoatanga e pai ana mō tāu tamaiti.
Karere Tangohia ki te Kāinga
- Ko te Hypothalamic Hamartoma (HH) he pukupuku onge, ehara i te matepukupuku ka puta i roto i te roro i te whānautanga.
- Ko ngā tohu matua e rua o tēnei ko ngā haurangi gelastic me te pakeketanga wawe.
- Ko te whakamātautau matua e whakamahia ana hei tautuhi i te mate ko te matawai MRI o te roro.
- Ka taea te whakahaere angitu i te pakeketanga o te pakeketanga mā te rongoā, te pokanga haurangi, ētahi atu tikanga motuhake rānei.
- Mena he iti noa iho tō whakapae kei tō tamaiti ēnei tohu, me toro atu ki tētahi tākuta tamariki, ki tētahi tākuta roro rānei i mua i te wā.











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