Kei te puta mai ngā puku rerekē i te tinana o tāu tamaiti? He tohu pea tēnei mō ngā mate pukupuku lymphatic matatini (CLA)!

I ētahi wā ka tino mataku tātou ina kite tātou i ngā huringa iti i roto i te tinana o ā tātou tamariki, kāore? Ina koa mēnā kāore tātou i te tino mōhio he aha aua mea. I tēnei rā ka kōrero tātou mō tētahi mate onge engari he mea nui kia mōhiotia. Ko te ingoa o tēnei ko ngā Complex Lymphatic Anomalies , arā, ko te (CLA) te poto. I tapaina anō tēnei e ētahi i mua ko (Lymphangiomatosis) . Nō reira, tera pea ka rongo koe i ēnei ingoa e rua.

Tuatahi, me titiro tātou, he aha ngā Āhuatanga Kino o te Rōpū Rewharewha Matatini (CLA)?

Ki te whakarapopototanga, ko ngā Āhuatanga Lymphatic Matatini (CLA) he āhuatanga onge engari he āhua whānau tonu pea, e puta ai he pukupuku pai i roto i te tamaiti e kiia nei ko ngā malformations lymphatic, lymphangiomas rānei, i roto i te pūnaha lymphatic o te tamaiti. Ka taea e ēnei pukupuku te tipu ki roto i ngā wheua, i ngā kiko honohono, me ētahi atu okana o te tamaiti, ka pā kino.

Whakaarohia, ahakoa kei a tā tātou tamaiti tēnei mate i te whānautanga mai, i te nuinga o te wā ka tīmata te puta o ngā tohu i a rātou e pakeke haere ana, i muri i ētahi tau pea. Koia te take i ētahi wā kua roa rawa te wā ki te kite i tēnei.

Nā, he aha tēnei Pūnaha Lymphatic?

Āe, kei te whakaaro pea koe he aha te pūnaha lymphatic. He kupu ngāwari noa iho, he pūnaha parepare whakamiharo i roto i tō tātou tinana. He rite ki ngā hōia o tō tātou whenua. Ka āwhina tēnei pūnaha lymphatic i tō tātou tinana ki te ārai i ngā mate me te whawhai ki a rātou mēnā ka pā mai. He wāhanga nui o tō tātou pūnaha ārai mate.

Ko te pūnaha lymphatic he mea hanga mai i tētahi whatunga o ngā ngongo iti e kiia nei ko ngā oko lymphatic, ngā repe pērā i ngā kōpū lymphatic, me ngā whekau pērā i te whēkau. He rite ēnei oko lymphatic ki ngā ngongo iti. Ka kohia e rātou te wai lymph mai i ngā kiko o te tinana, ka tātarihia, ka horoia, kātahi ka whakahokia ki te toto. He mahi whakamiharo tēnei?

E rua ngā momo CLA: Ko te Motuhake me te Pūnaha.

Ka taea te wehewehe i ngā Āhuatanga Matatini o te Lymphatic ki ngā momo matua e rua:

1. Ngā CLA Motuhake: Koinei te wā e herea ana ngā pukupuku ki tētahi wāhi o te tinana o te tamaiti. Hei tauira, ko te kōhao uma anake ka pā ki a rātou. I tēnei wā, ko ngā pukahukahu me ngā kiko ngohengohe o te uma (mediastinum) te mea tino pāngia.

2. Ngā CLA Pūnaha: I tēnei wā, ka puta ēnei pukupuku ki ngā wāhi maha o te tinana o te tamaiti, pērā i ngā wheua, te uma, me te puku.

Nō reira, ka rerekē pea ngā tohu me ngā maimoatanga i runga i te wāhi e pāngia ana.

He aha ngā momo matua o ngā Āhuatanga Matatini o te Lymphatic (CLA)?

E whā ngā momo matua o te CLA. Ahakoa he rite tonu ngā tohu o ia momo, he rerekē ngā āhuatanga. Ā, he rerekē anō hoki ngā whakarerekētanga ira e puta ai ēnei.

• Te Āhuatanga Whānui o te Lymphatic (GLA):I te nuinga o te wā ka pā tēnei āhuatanga ki ngā wāhanga rerekē o te tinana o te tamaiti. Hei tauira, ka puta ēnei pukupuku i roto i ngā wheua, te whēkau, te ate, ngā pukahukahu, me ngā kiko ngohengohe o te uma (mediastinum).
• Kaposiform Lymphangiomatosis (KLA): Koinei te momo CLA tino kino, tino tere hoki te horapa. I roto i te KLA, ka nui haere ngā oko toto lymphatic e kawe ana i te wai lymph puta noa i te tinana o te tamaiti. Mā tēnei ka uru atu, ka kino hoki ngā okana, ngā wheua, me ngā kiko e karapoti ana i a rātou.
• Te Āhuatanga Kino o te Pūnaha Rewharewha o te Pūnaha Rewharewha Matua (CCLA): He nui ake hoki ngā oko toto lymph o ngā tamariki whai CCLA. Heoi, i roto i te CCLA, ko te nuinga o ēnei oko toto nui kei roto i te tinana. Ka pā pea tēnei ki te pūnaha nakunaku, ā, ka kore e taea e te tamaiti te tango tika i te wai lymph mai i te tinana.
• Mate Gorham-Stout (GSD): E mōhiotia ana ko te mate a Gorham, ka memeha te nui o te wheua i tēnei āhuatanga nā te hanganga o ngā oko toto lymphatic i roto i ngā wheua. E kiia ana hoki ko te "mate wheua ngaro." Ka pā ki tētahi wheua. Heoi, ko ngā tamariki whai GSD te nuinga o te wā ka ngaro te wheua i roto i ngā rara, te angaanga, te wheua kaki, me te tuaiwi kaki.

He pēhea te noa o tēnei āhuatanga CLA?

He tino onge ngā mate pukupuku lymphatic matatini. Nā te mea kāore i te tino kitea, he uaua pea ki ngā tākuta te tautuhi tika i a rātou. Nō reira, ki te puta ngā tohu, he mea nui kia toro atu koe ki tētahi tohunga i te wā e taea ai.

He aha te take o tēnei CLA?

Kei te rapu tonu ngā tākuta me ngā kairangahau i te pūtake o te CLA. Heoi, e kī ana ngā rangahau hou ka puta pea tēnei mate i ngā whakarerekētanga ira tuku iho e tukuna ana mai i ngā mātua ki ngā tamariki, me ngā whakarerekētanga ira tinana e puta ana i muri i te whānautanga. Ko te tikanga he hononga ira.

Ko wai kei te mōrearea ki te whakawhanake i te CLA?

Ka pāngia te tangata e ngā mate pūkahukahu matatini. Ina koa, ka puta ngā tohu o tēnei mate i te mutunga o te tamarikitanga, i te taiohitanga rānei. Nō reira, ahakoa kei te whānau mai te mate, tera pea ka roa te wā ka puta mai ngā tohu.

Nā, he aha ngā tohu o te CLA?

Ka rerekē pea ngā tohu i ia tamaiti e pāngia ana e tēnei mate. Waihoki, ka rerekē ngā tohu i runga i te pūnaha okana e pāngia ana me te kaha o tōna mate. I te nuinga o te wā, ka puhoi te whanaketanga o tēnei mate. Nō reira, kāore pea tātou e aro nui ki ētahi o ngā tohu, ka whakaaro rānei tātou he mate kē atu ēnei.

Mena ka pāngia te pūnaha manawa o tāu tamaiti:

I tēnei wā, ka puta pea ngā tohu rite ki te mate huango.

• Te maremare tonu.
• Te uaua ki te manawa (dyspnea).
• He wheowheo ( he tangi whiowhio ) i te wā e manawa ana.

Mena ka pāngia te pūnaha angaiwi o tāu tamaiti:

• Te mamae o ngā wheua, o ngā hononga rānei.
• Ahakoa he hinganga iti noa iho, ka whati pea ngā wheua .
• Te ngoikore o ngā peka.
• Te mamae, te ngoikore rānei i puta mai i te pēhanga o te io .

Mena ka pāngia te pūnaha nakunaku o tāu tamaiti:

• Te pupuhi o te puku me te mamae o te puku .
• Te ngoikore me te ngenge tonu.
• Te korere.
• Kāore he reka o te kai.
• Whakapairuaki me te ruaki.
• Te ngaronga taumaha mō te kore take.

Mena ka pāngia te pūnaha mimi o tāu tamaiti:

• Te toto i roto i te mimi (hematuria).
• Te mamae o te taha.
• Te toto toto tiketike i roto i ngā tamariki.

Mena kei tō tamaiti tētahi, ētahi rānei o ēnei tohu, rapua he tohutohu hauora. Ehara ēnei i te tohu e pā ana ki te CLA, engari he mea nui kia whakamātauria.

Me pēhea te tātaritanga a ngā tākuta i tēnei mate CLA?

Nā te mea he mate onge te CLA, ā, he rerekē ngā tohu, he uaua pea ki ngā tākuta te tautuhi i te mate tuturu. Nā te mea he maha ngā wā ka pāngia ngā pukahukahu me ngā wheua o ngā tamariki, ka tono pea te tākuta o tāu tamaiti i ngā whakamātautau pēnei i:

• Pūnaha tirotiro manawa: Hei tirotiro mō te mate pukupuku, ētahi atu āhuatanga rerekē rānei i roto i ngā ara manawa.
• Whakamātautau mahi a ō pukahukahu: Inehia te pai o te mahi a ō pukahukahu.
• MRI o te tinana katoa: Hei tirotiro i ngā mea pēnei i ngā whekau kua pāngia, ngā whara wheua, te wai rānei i roto i te puku.
• Ngā hihi-X: Hei tirotiro anō i ngā wheua e ahua whakapaetia ana, e whara rānei i runga i te MRI. Ka taea hoki e ngā hihi-X o te uma te rapu i ngā āhuatanga rerekē o ngā pukahukahu.

I ētahi wā ka tangohia e ngā tākuta he tauira iti o ngā kōpū lymph me te kiko (biopsy) ka tirohia i raro i te karuārai. Ahakoa ka taea e tēnei whakamātautau te tautuhi i te CLA, i ētahi wā ka puta he raruraru, pērā i te kohinga wai lymph huri noa i ngā pukahukahu (chylothorax). Nā tēnei, he maha ngā wā ka whakawhirinaki ngā tākuta ki ngā whakamātautau iti ake te whakaekenga hei whakatau i te mate.

Me pēhea te rongoā mō te CLA?

Nā te mea ka pāngia e te CLA ngā wāhanga maha o te tinana o te tamaiti, ka mahi tahi ngā tākuta, arā, ka whakamahia e rātou he huarahi maha-mātauranga hei whakarite i te maimoatanga e tika ana mō te tamaiti. I te rongoā i te CLA, ko te arotahi matua ko te whakahaere i ngā tohu o te tamaiti. Hei mahi i tēnei, ka taea e koe te mahi i ngā mea penei:

• Te honohono wheua: Mō te pakaru o te wheua i roto i ngā āhuatanga pēnei i te GSD.
• He kai nui te pūmua, he kai werohanga ā-uaua rānei (kai parenteral katoa, TPN rānei).
• Sclerotherapy: Hei whakaiti, hei whakakore rānei i ngā pukupuku, i ngā oko toto lymph rānei.
• Pokanga: Hei tango i ngā pukupuku, hei whakauru rānei i ngā ngongo iti (shunt) hei whakahaere i te rere o te wai lymph.
• Te rongoā irahiko, te rongoā matepukupuku rānei: Hei whakaiti i ngā pukupuku kore-matepukupuku (ka whakamahia anake ēnei i ngā wā tino kino, e morearea ana ki te ora).

Nō nā tata nei, kua whakamahia hoki ngā rongoā kua arotahi ki ngā whakarerekētanga ira e puta ai tēnei mate. Kua whakaatuhia hoki te whai huatanga o ēnei rongoā hei rongoā tuatahi mō te CLA.

Ka taea te ārai i ngā Āhuatanga Kino o te Lymphatic (CLA)?

Kāore, kāore . Ka puta te CLA i te wā e hapu ana, ā, kāore i te mōhiotia te tino take. Nō reira, kāore he mea e taea e koe te mahi hei ārai i tō pēpi kei pāngia e tēnei mate.

He aha ngā raruraru ka pā mai i te CLA?

Ka taea e te CLA te whakararu i tāu tamaiti mō:

• Ascites: He kohi wai i roto i te puku.
• Ngā whati wheua.
• Chylothorax me Chylopericardium: He kohinga wai miraka (chyle) kei roto te wai lymph me te ngako i roto i te kiriuhi e hipoki ana i ngā pukahukahu (pleura) me te pēke e karapoti ana i te ngākau (pericardium).
• Te hinga o te pukahukahu/te pneumothorax.
• Te ngoikore o te ate.
• Pararutiki.
• Te rerenga toto o te ngākau.
• Te rerenga toto i roto i te pleural me te edema pūkahukahu cardiogenic.

Ka tino kino pea ētahi o ēnei raruraru, nō reira he mea nui kia mōhio koe ki ngā tohu me te whai i ngā tohutohu a tō tākuta.

He aha te heke mai mō te tangata whai CLA?

Kāore he rongoā mō ngā Āhuatanga Kino o te Lymphatic (CLA). Ko te heke mai o tāu tamaiti kei runga i ngā pūnaha tinana e pāngia ana me te kaha o ngā tohu.

Ko ngā rerenga wai i roto i te kiri te tino take o te mate i waenga i te hunga whai CLA. Heoi, mā te maimoatanga me te whakahaere tika, ka taea te āwhina i te tamaiti kia ora i te oranga noa.

Āhea ahau me toro atu ki te tākuta?

Mena kei tō tamaiti tētahi o ēnei tohu, me toro atu ki te tākuta tonu:

• Te mamae i roto i te puku, i te whekau rānei.
• Mena he toto kei roto i te mimi.
• Te maremare tonu, te wheezing, te uaua rānei ki te manawa.
• Te whakapairuaki, te ngenge, te ngaronga taumaha rānei kāore i te mārama.

Mena kei a koe tētahi o ēnei mea, me toro atu ki te tākuta ināianei tonu.

He aha taku e pātai atu ai ki te tākuta?

Ka hiahia pea koe ki te pātai atu ki te tākuta o tāu tamaiti i ēnei pātai:

• Ko ēhea ngā wāhanga o te tinana o taku tamaiti kua pāngia e tēnei mate?
• He aha ngā kōwhiringa maimoatanga e wātea ana mō taku tamaiti?
• Me pokanga taku tamaiti?
• Me mōhio ahau ki ngā tohu o ngā raruraru?

He mea tino nui kia pātaihia ēnei pātai, kia mārama ai hoki ki te āhuatanga.

Hei whakamutunga, me kī atu ahau...

Ina kitea e koe kei te pāngia tō tamaiti e te CLA (lymphangiomatosis), he mea noa te mataku me te manukanuka mō te pānga ki tōna hauora me tōna heke mai. Tērā pea he tokomaha o tātou kāore anō kia rongo i tēnei mate i mua. Ā, kāore he mea e taea e koe te mahi hei ārai i tēnei.

Ko te taahiraa tuatahi ki te āwhina i tāu tamaiti ko te mōhio ki tēnei mate, ōna tohu, me ngā maimoatanga. Tirohia he tākuta e tohunga ana ki ngā mate o te pūnaha lymphatic. Ka taea e rātou te whakautu i ō pātai me te whakarato i te tiaki hauora motuhake e hiahiatia ana e tāu tamaiti.

Ahakoa kāore he rongoā mō te CLA, mā te maimoatanga me te whakahaere tika ka āwhina i tāu tamaiti kia ora harikoa, kia ora hauora hoki. Nō reira, kia kaha, kia kaha hoki te mahi mō tāu tamaiti.

Ngā Āhuatanga Matatini o te Lymphatic, Lymphangiomatosis, Ngā Mate Pūnaha Lymphatic, Ngā Puku Tamariki, Osteomalacia, Ngā Tohu o te Pūkahukahu, Ngā Mate Tamariki Onge