He raruraru tā tāu tamaiti ki ngā oko toto waikura i roto i ōna whekau? (Te Lymphangiectasia Whekau Tuatahi) Me kōrero tātou mō tēnei!

Kua kite koe i ētahi wā ka pupuhi noa ngā waewae me ngā ringa o tāu tamaiti? He maha rānei āna amuamu mō te mamae o te puku? Kei te heke tōna taumaha, engari kāore i te piki ake? Tērā pea ka whakaaro koe he tohu noa ēnei kāore tātou e aro nui ana ki tēnā. Heoi, kei muri i ēnei, tera pea he mate onge engari he tino āwangawanga e kiia nei ko te 'Primary Intestinal Lymphangiectasia' (PIL) . He ahua whakamataku te ingoa, kāore? Kaua e manukanuka. Me kōrero tātou mō tēnei mea i roto i ngā taipitopito me te tino māmā i tēnei rā.

He aha te 'Lymphangiectasia Puku Tuatahi'?

He kupu ngāwari noa iho tēnei, he mate onge tēnei e pā ana ki te pūnaha nakunaku o ā tātou tamariki, inā koa ko te whekau iti. E mōhio ana koe, ko te nuinga o ngā kai e kainga ana e tātou ka nakunakuhia, ā, ko ngā matūkai kei roto ka mimitihia ki roto i te tinana i roto i tēnei whekau iti.

I tēnei āhua (PIL), kua pakaru te pūnaha ngongo iti e kiia nei ko ngā 'uaua lymph' i roto i te whekau iti o tāu tamaiti, ā, kāore e mahi tika. He whatunga tino pai ēnei oko lymph o ngā awaawa e horapa ana puta noa i tō tātou tinana. He rite ki tētahi atu 'pūnaha paipa' i roto i tō tātou tinana. Ko tā rātou mahi matua he kohikohi i ngā wai me ngā pūmua e kohia ana i roto i ō tātou kiko (e kiia ana tēnei wai ko te 'lymph') ka whakahokia ki te pūnaha tohanga toto.

Engari i roto i te tamaiti e pāngia ana e te PIL, ka pupuhi ēnei oko toto waikura, ka 'tātari'. Ko te mea ka tupu, i te kore e mimiti i ngā matūkai me ngā pūmua mai i ēnei oko toto waikura ki roto i te tinana, ka turuturu anō ki roto i ngā whekau. He rite ki te wai e turuturu ana mai i tētahi paipa turuturu.

Ina turuturu mai ngā mea pēnei, e rua ngā raruraru matua ka ara ake:

1. Te iti o ngā taumata pūmua: He mea nui te pūmua ki te tinana. Nō reira, ina turuturu ēnei ki roto i ngā whekau, kāore e nui te pūmua e whiwhihia ana e te tinana. Ka pupuhi pea te tinana katoa. Ka kiia hoki tēnei āhuatanga ko te 'protein-losing enteropathy' i roto i ngā kupu hauora.

2. Ngā ngoikoretanga o te kai: Haunga te turuturu o te wai, ka ngaro hoki ngā huaora me ngā kohuke nui. Ki te kore tēnei e tiakina tika, ka pāngia te tamaiti e ngā ngoikoretanga o te kai.

(PIL) te nuinga o te wā ka pā ki ngā tamariki nohinohi me ngā taiohi. I te nuinga o te wā ka kitea i mua i te 3 tau te pakeke. Heoi, ka pā pea ki ngā pakeke me ngā pēpi i roto i te kōpū. Kāore anō kia kitea he rongoā mōna. Heoi, ka taea e ngā tākuta te whakahaere i ngā tohu me te whakaiti i te turuturu me te pupuhi mā te whakamahi i tētahi mahere kai me ngā rongoā motuhake. Ka taea hoki e rātou te hoatu ki te tinana ngā huaora me ngā kohuke e hiahiatia ana e ia.

He maha atu anō ngā ingoa e huaina ana mō tēnei āhuatanga (PIL):

• Te lymphangiectasia o te puku
• Te mate pukupuku o te roro
• Te mate a Waldmann

He aha ngā tohu o tēnei āhuatanga (PIL)?

Ko te tohu matua me te tohu tino kitea o te PIL ko te pupuhi nā te pupuri wai. Ka kiia tēnei e ngā tākuta he lymphedema . Tērā pea ka kite tuatahi koe i tēnei i ngā waewae me ngā kapu o tāu tamaiti. I te nui haere o tēnei wai, ka tīmata pea te pupuhi o te mata, o ngā ringa, tae atu ki te wāhi ira tangata o tāu tamaiti.

Whakaarohia, ina ara ake tāu tamaiti i te ata, he noa iho ōna waewae, engari i te roanga o te rā, i te ahiahi, kua pupuhi rawa ōna waewae, kāore e taea te mau hu. Koinei te mea ka tupu i tēnei take.

Hei tāpiritanga, tera pea he wai kei te ngākau o te tamaiti (e kiia nei ko te pericarditis ), he wai kei roto i te uma (e kiia nei ko te pleural effusion ), he wai rānei kei roto i te puku (e kiia nei ko te ascites, ko te abdominal effusion rānei) .

Anei ētahi atu tohu:

• Mamae puku
• Whakapairuaki me te ruaki
• Mate puku (korere )
• Te ngenge tonu (Fatigue)
• Te whakahekenga taumaha
• Ahakoa te nui o te kai e kainga ana, kāore e piki te taumaha (nā te kore o te tinana e mimiti tika i ngā matūkai, e kiia ana tēnei ko te 'malabsorption' )

Mena kei tō tamaiti tētahi, ētahi rānei o ēnei tohu, he pai ake te rapu tohutohu hauora.

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He raruraru nui kei te pā mai i te mate (PIL)?

Ae, ahakoa he tino onge, ka taea e te PIL te pupuhi nui i te tinana katoa o te tamaiti. Ka kiia tēnei ko te 'anasarca' . He mate kino tēnei ka taea te whakamate i te ora. Nō reira, he mea nui kia mōhio ki ngā tohu.

He aha te take mō tēnei āhuatanga (PIL)?

Ko tēnei āhuatanga (PIL) i puta mai i tētahi āhuatanga rerekē o ngā awa lymphatic o te pēpi. Ko ēnei awa lymphatic, e ai ki te kōrero i mua ake nei, he pūnaha ngongo e kawe ana i ngā mea pēnei i te ngako mai i te whekau iti o te pēpi ki te ate me ētahi atu wāhanga o te tinana.

Nā tēnei āhuatanga rerekē ka āraia ngā oko toto waikura. Kātahi ka kī i te wai waikura, ka pupuhi ngā oko toto, ā, ka pakaru, ā, ka kohia ngā mea o roto i aua oko toto ki roto i te whekau nui o te tamaiti (koroni).

Heoi anō, kāore anō ngā tohunga hauora kia tino mōhio he aha i puta ai tēnei mate. Koinei te raruraru.

He ira tuku iho tēnei? (He ira tuku iho te lymphangiectasia puku?)

He tino onge te puta o ngā pūrongo mō ētahi mema o te whānau kotahi e pāngia ana e te mate. Heoi, kāore anō kia tino mārama ngā kairangahau mēnā he mate ira tēnei. Kei te haere tonu ngā rangahau mō tēnei.

Me pēhea te tautuhi i tēnei mate (PIL)? (Tautuhinga)

I te nuinga o te wā, ko te mea tuatahi ka mahia e te tākuta ko te tirotiro tinana i tō pēpi me te pātai atu ki a koe mō ngā tohu o tō pēpi. I ētahi wā, ka taea e te ultrasound prenatal , e mahia ana i te wā e noho tonu ana te pēpi i roto i te kōpū, te whakaatu i ngā tohu o te pupuhi (edema) i ngā waewae o te pēpi, ka taea ai e koe te whakaaro he aha te mea e tupu ana.

I muri i te whānautanga o te pēpi, ka tirohia e te tākuta te PIL mā te whakamahi i tētahi taputapu motuhake e kiia nei ko te endoscope hei tirotiro i roto i ngā whekau o te pēpi. He ngongo ngāwari tēnei me te kāmera e piri ana ki roto. Ka whakamoea te pēpi i raro i te rongoā whakamoe i te wā o tēnei whakamātautau, kia kore ai rātou e mamae. Nō reira kaua e manukanuka.

I tēnei endoscopy, ka tangohia e te tākuta ngā wahi iti o te kiko (e kiia ana ko te biopsy ) mai i ētahi wāhi o te whekau o te tamaiti. Kātahi ka tirohia ēnei tauira kiko e te tohunga mate pukupuku , he tākuta e tohunga ana ki te tirotiro i te kiko, i raro i te karuārai hei tirotiro mō te pupuhi rerekē o ngā oko toto lymph.

Hei tāpiritanga, ka taea e ngā whakamātautau toto te whakatau i te nui o te pūmua i roto i te tinana. Ka taea hoki te tango tauira paru hei tirotiro mēnā kei te turuturu te wai mai i ngā pona lymph ki roto i te whekau iti.

He aha ngā maimoatanga pai rawa atu mō te (PIL)?

I te rongoā i te PIL, ko te mea nui ko te whakarerekē i te kai a te tamaiti. Koinei te patu matua ki te PIL. Whakaarohia he rite ki te whakatakoto i tētahi 'mono' hei aukati i te turuturu o ngā matūkai.

He maha ngā tākuta e tohu ana i tētahi kai iti te ngako, nui te pūmua.

He uaua te nakunaku i te ngako. Nō reira, he uaua ki te ngongo tika i te ngako e te puku turuturu o tō pēpi. Nō reira, tera pea kei roto i tō kai hou tētahi momo ngako motuhake e kiia nei ko ngā 'medium-chain triglycerides (MCT).' Ko ēnei (MCT) he momo ngako e ngāwari ana te ngongo e te tinana. Waihoki, mā te tāpiri atu i te pūmua ki tō kai ka taea te whakanui ake i te nui o ngā matūkai ka ngongohia e tō tinana.

I te mea kua ngaro ētahi o ngā matūkai, ka tūtohu pea te tākuta i ngā tāpiringa huaora me ngā kohuke hei whakarite kei te whiwhi te tinana o te tamaiti i ngā mea e hiahiatia ana e ia.

Hei tāpiritanga, ka tohua pea e te tākuta ngā rongoā pēnei i ēnei hei āwhina i ngā āhuatanga rerekē o te mate (PIL):

• Ngā rongoā diuretic (e kiia ana ko ngā pire wai): Ka mahi ēnei mā te whakaiti i te nui o te wai tāpiri e kohia ana i roto i te tinana nā te turuturu o te pūmua.
• Ngā rongoā rite ki te somatostatin (pērā i te octreotide): Ka āwhina ēnei rongoā ki te whakaiti i te turuturu o ngā matū mai i te pūnaha lymphatic.

Ētahi atu kōwhiringa maimoatanga:

• Pokanga: Mena kāore ētahi atu maimoatanga e whai hua, ā, he tino onge, ka tūtohu pea ngā tākuta kia pokangahia te wāhanga mate o te whekau o tāu tamaiti.
• Ētahi atu maimoatanga pea: Kei te haere tonu ngā rangahau mō tēnei. Kei te whakamatautauria ētahi rongoā hou, pērā i te sirolimus, mō te PIL. Tērā pea he āwhina ēnei i ētahi wā, engari kāore anō kia whakamahia whānuitia.

He aha te matapae mō te āhuatanga (PIL)?

E ai ki te kōrero i mua ake nei, ehara te (PIL) i te mate e kore e taea te rongoā katoa. Heoi, me piki ake ngā taumata pūmua i roto i te toto o te tamaiti i roto i ētahi wiki i muri i te tīmatanga o te maimoatanga. Heoi, me kai te tamaiti i tētahi kai iti-ngako me te tango i ngā tāpiringa kai mō te toenga o tōna oranga.

Ahakoa he wero tēnei, ki te whakahaeretia tika, ka taea e te tamaiti te noho i tētahi oranga noa. Ko te mea nui ko te whai i ngā tohutohu a te tākuta me te tiaki i te kai a te tamaiti.

Ka taea te ārai i te āhuatanga (PIL)?

I te mea kāore anō kia mōhiotia e ngā kairangahau te pūtake o tēnei mate (PIL), kāore anō kia kitea he huarahi hei ārai i tēnei mate i tēnei wā.

Āhea te wā me kite taku tamaiti i te tākuta?

Ki te kite koe i te pupuhi i ngā waewae, i ngā tapuwae rānei o tāu tamaiti, i tētahi atu rānei o ngā tohu o te PIL kua whakahuatia ake nei, he pai ake te kawe i tāu tamaiti ki te tākuta tonu. Ko te wawe o te kitenga, ko te wawe hoki o te tīmatanga o te maimoatanga.

He aha ngā pātai me pātai atu e au ki te tākuta o taku tamaiti?

Mena he PIL tāu tamaiti, he maha pea āu pātai hei pātai atu ki tō tākuta. Anei ētahi o aua pātai:

• He aha te kai e tino pai ana mō tēnei mate (PIL)?
• He aha ngā tāpiringa me hoatu e au ki taku tamaiti?
• He aha atu anō taku e taea ai te whakahaere pai ake i ngā tohumate o taku tamaiti?
• He tino kino tēnei āhuatanga (PIL)?
• He aha ngā raruraru me tūpato ahau?

Kaua e wehi ki te pātai i ēnei pātai. Kōrerohia ō āwangawanga ki tō tākuta.

Hei whakamutunga, ngā mea hei maumahara (Karere hei tango ki te kāinga)

Ina tīmata koe ki te pāngia e ngā tohu o te Primary Intestinal Lymphangiectasia, ka uaua pea māu me ō tākuta te mārama ki te āhua o te mate. He tino pōhēhē, he tino whakapoauau hoki te wheako. Heoi, ka taea e ō tākuta te mahi tahi me koe ki te āwhina i a koe ki te kimi i te take.

Waimarie, mā te kai iti-ngako me ngā tāpiringa kai, ka taea te whakahaere i ngā tohumate o tāu tamaiti i roto i te wā. Pātai atu ki te tākuta o tāu tamaiti he aha atu ngā mea ka taea e koe te mahi hei āwhina i tāu tamaiti ki te whakahaere i tēnei mate. He mea tino nui tō aroha, tō tautoko, me tō aro ki tāu tamaiti i tēnei haerenga.

Te lymphangiectasia whekau tuatahi, PIL, te aukati lymphatic, ngā mate puku i roto i ngā tamariki, te pupuhi waewae, te korenga o te pūmua, ngā mate pūnaha nakunaku