He pilikia anei kāu keiki me nā kīʻaha lymph i loko o kona ʻōpū? (Primary Intestinal Lymphangiectasia) E kamaʻilio kākou e pili ana i kēia!

Ua ʻike paha ʻoe i ka pehu ʻana o nā wāwae a me nā lima o kāu keiki liʻiliʻi i kekahi manawa? A i ʻole ke hoʻopiʻi pinepine nei ʻo ia no ka ʻeha ʻōpū? Ke emi nei kona kaumaha me ka ʻole o ka piʻi ʻana o ke kaumaha? Manaʻo paha ʻoe he mau hōʻailona maʻamau kēia a mākou e nānā ʻole ai. Eia naʻe, ma hope o kēia mau mea, aia paha kahi maʻi kakaikahi akā hopohopo maoli i kapa ʻia ʻo 'Primary Intestinal Lymphangiectasia' (PIL) . He mea weliweli iki ke kani o ka inoa, ʻeā? Akā, mai hopohopo. E kamaʻilio kākou e pili ana i kēia me nā kikoʻī a me ka maʻalahi loa i kēia lā.

He aha ke ʻano o ka 'Primary Intestinal Lymphangiectasia'?

I ka ʻōlelo maʻalahi, he maʻi laha ʻole kēia e kū mai ana i loko o ka ʻōnaehana hoʻoheheʻe o kā mākou mau keiki, ʻo ia hoʻi ka ʻōpū liʻiliʻi. Ua ʻike ʻoe, ua hoʻoheheʻe ʻia ka hapa nui o ka meaʻai a mākou e ʻai ai a ua komo nā meaola i loko o ke kino i loko o kēia ʻōpū liʻiliʻi.

I kēia manawa, ma kēia kūlana (PIL), ua hōʻino ʻia ka ʻōnaehana paipu liʻiliʻi i kapa ʻia ʻo 'nā kīʻaha lymph' i loko o ka ʻōpū liʻiliʻi o kāu keiki a ʻaʻole lākou e hana pono. ʻO kēia mau kīʻaha lymph he pūnaewele maikaʻi loa o nā kahawai i hoʻolaha ʻia ma ko mākou kino. Ua like ia me kekahi 'ʻōnaehana paipu' ʻē aʻe i loko o ko mākou kino. ʻO kā lākou hana nui ka hōʻiliʻili ʻana i nā wai a me nā protein e hōʻiliʻili ana i loko o kā mākou mau ʻiʻo (kapa mākou i kēia wai 'lymph') a hoʻihoʻi iā lākou i ka ʻōnaehana hoʻopuni koko.

Akā i loko o ke keiki me PIL, pehu kēia mau kīʻaha lymph a 'leak'. ʻO ka mea e hana ʻia ana, ma kahi o ka omo ʻana i nā meaʻai a me nā protein mai kēia mau kīʻaha lymph i loko o ke kino, leak hou lākou i loko o nā ʻōpū. Ua like ia me ka wai e leak ana mai kahi paipu leak.

Ke liki nā mea e like me kēia, kū mai nā pilikia nui ʻelua:

1. Nā pae protein haʻahaʻa: ʻO ka protein kahi mea e hana ana i kahi hana koʻikoʻi loa i loko o ke kino. No laila, ke kahe kēia mau mea i loko o nā ʻōpū, ʻaʻole lawa ka protein i ke kino. Hiki i kēia ke hoʻoulu i ke kino holoʻokoʻa e pehu. Kāhea pū mākou i kēia ʻano ʻo 'protein-losing enteropathy' ma nā ʻōlelo lapaʻau.

2. Nā hemahema meaʻai: Me kēlā wai e kahe ana, nalowale pū nā huaora a me nā minelala koʻikoʻi. Inā ʻaʻole mālama pono ʻia kēia, hiki i ke keiki ke loaʻa i nā hemahema meaʻai like ʻole.

(PIL) ka mea maʻamau e pili ana i nā keiki ʻōpio a me nā ʻōpio. Hoʻomaopopo pinepine ʻia ma mua o 3 mau makahiki. Eia nō naʻe, hiki i kekahi manawa ke hoʻopilikia i nā pākeke a me nā keiki i loko o ka ʻōpū. ʻAʻohe lāʻau lapaʻau no ia mea. Eia nō naʻe, hiki i nā kauka ke kāohi i nā hōʻailona a hōʻemi i ka leakage a me ka pehu ma ka hoʻohana ʻana i kahi papaʻai kūikawā a me nā lāʻau lapaʻau. Hiki iā lākou ke hāʻawi i ke kino i nā huaora a me nā minelala e pono ai.

Ua kapa ʻia kēia kūlana (PIL) e kekahi mau inoa ʻē aʻe:

• ʻO ka lymphangiectasia ʻōpū
• ʻO ka Lymphangiectasia
• Maʻi Waldmann

He aha nā hōʻailona o kēia maʻi (PIL)?

ʻO ka hōʻailona nui a maʻamau o PIL ka pehu ʻana ma muli o ka paʻa ʻana o ka wai. Kāhea nā kauka i kēia lymphedema . E ʻike mua paha ʻoe i kēia ma nā wāwae a me nā wāwae o kāu keiki. I ka hōʻiliʻili ʻana o kēia wai, e hoʻomaka paha ka pehu ʻana o ka maka o kāu keiki, nā lima, a me ka ʻāpana genital.

E noʻonoʻo, i ke ala ʻana o kāu keiki i ke kakahiaka, ua maʻamau ko lākou mau wāwae, akā i ka hala ʻana o ke ao, i ke ahiahi, ua pehu loa ko lākou mau wāwae a ʻaʻole hiki iā lākou ke komo i nā kāmaʻa. ʻO ia ka mea e hana ʻia ma kēia hihia.

Eia kekahi, loaʻa paha i ke keiki ka wai a puni ka puʻuwai (i kapa ʻia ʻo pericarditis ), ka wai i loko o ka umauma (i kapa ʻia ʻo pleural effusion ), a i ʻole ka wai i loko o ka ʻōpū (i kapa ʻia ʻo ascites a i ʻole ka abdominal effusion) .

Eia kekahi mau hōʻailona ʻē aʻe:

• ʻEha ʻōpū
• Ka luaʻi a me ka luaʻi
• Ka maʻi ʻōpū (ʻeha ʻōpū )
• Manaʻo luhi i nā manawa a pau (Fatigue)
• Ka emi ʻana o ke kaumaha
• ʻAʻole e piʻi ke kaumaha me ka nānā ʻole i ka nui o ka meaʻai āu e ʻai ai (ʻo kēia ma muli o ke kino ʻaʻole i omo pono i nā meaola, i kapa ʻia ʻo 'malabsorption' )

Inā loaʻa i kāu keiki kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​ʻoi aku ka maikaʻi o ka ʻimi ʻana i ke kōkua lapaʻau.

Aia kekahi mau pilikia koʻikoʻi o ke kūlana (PIL)?

ʻAe, ʻoiai he mea kakaikahi loa, hiki i ka (PIL) ke hoʻoulu i ke kino holoʻokoʻa o ke keiki e pehu nui. Ua kapa ʻia kēia ʻo 'anasarca' . He kūlana koʻikoʻi kēia e hiki ke hoʻoweliweli i ke ola. No laila, he mea nui loa ka makaʻala i nā hōʻailona.

He aha ke kumu o kēia kūlana (PIL)?

Hoʻokumu ʻia kēia kūlana (PIL) e kahi ʻano ʻē i nā kahawai lymphatic o ka pēpē. ʻO kēia mau kahawai lymphatic, e like me ka mea i ʻōlelo ʻia ma mua, he ʻōnaehana o nā paipu e lawe ana i nā mea e like me ka momona mai ka ʻōpū liʻiliʻi o ka pēpē i ke akepaʻa a me nā ʻāpana ʻē aʻe o ke kino.

ʻO kēia ʻano ʻē ke kumu e ālai ʻia ai nā kīʻaha lymph. A laila, hoʻopiha lākou me ka wai lymph, pehu nā kīʻaha, a i ka hopena e pohā lākou, a hōʻiliʻili nā mea o loko o kēlā mau kīʻaha i loko o ka ʻōpū nui o ke keiki (colon).

Eia nō naʻe, ʻaʻole maopopo i nā loea lapaʻau ke kumu o kēia maʻi. ʻO ia ka pilikia.

He ʻano hoʻoilina anei kēia? (He ʻano hoʻoilina anei ka lymphangiectasia ʻōpū?)

He mea kākaʻikahi loa, ua loaʻa nā hōʻike e pili ana i kekahi mau lālā o ka ʻohana hoʻokahi i hoʻopilikia ʻia e ke kūlana. Eia nō naʻe, ʻaʻole maopopo i nā mea noiʻi inā he kūlana hoʻoilina kēia. Ke hana hou ʻia nei nā noiʻi hou aku ma kēia.

Pehea e ʻike ʻia ai kēia maʻi (PIL)? (Diagnosis)

ʻO ka hapa nui o ka manawa, ʻo ka mea mua a ke kauka e hana ai, ʻo ia ke nānā kino i kāu pēpē a nīnau iā ʻoe e pili ana i nā hōʻailona o kāu pēpē. I kekahi manawa, hiki i kahi ultrasound prenatal , i hana ʻia ʻoiai ke pēpē i loko o ka ʻōpū, ke hōʻike i nā hōʻailona o ka pehu (edema) i nā wāwae o ka pēpē, hiki ke hāʻawi iā ʻoe i kahi manaʻo o ka mea e hana nei.

Ma hope o ka hānau ʻana o ke pēpē, e nānā ke kauka no ka PIL ma ka hoʻohana ʻana i kahi mea hana kūikawā i kapa ʻia he endoscope e nānā i loko o nā ʻōpū o ke pēpē. He ʻōmole palupalu kēia me kahi kāmela i hoʻopili ʻia iā ia. E hoʻomoe ʻia ke pēpē ma lalo o ka anesthesia i ka wā o kēia hoʻāʻo, no laila ʻaʻole lākou e ʻeha. No laila, mai hopohopo.

I loko o kēia endoscopy, lawe ke kauka i nā ʻāpana liʻiliʻi o ka ʻiʻo (i kapa ʻia he biopsy ) mai kekahi mau wahi o ka ʻōpū o ke keiki. A laila, nānā kekahi pathologist , he kauka loea i ka nānā ʻana i nā ʻiʻo, i kēia mau laʻana ʻiʻo ma lalo o kahi microscope e nānā i kekahi pehu kūpono ʻole o nā kīʻaha lymph.

Eia kekahi, hiki i nā hoʻāʻo koko ke hoʻoholo i ka nui o ka protein i loko o ke kino. Hiki ke lawe ʻia kahi hāpana ʻōpala e ʻike ai inā e kahe ana ke wai mai nā lymph nodes i loko o ka ʻōpū liʻiliʻi.

He aha nā lāʻau lapaʻau maikaʻi loa no (PIL)?

I ka mālama ʻana i ka PIL, ʻo ka mea nui loa, ʻo ia ka hoʻololi ʻana i ka ʻai a ke keiki. ʻO kēia ka mea kaua nui e kūʻē i ka PIL. E noʻonoʻo e like me ke kau ʻana i kahi 'plug' e hoʻōki i ka liki ʻana o nā meaʻai.

He nui nā kauka e kuhikuhi ana i ka papaʻai haʻahaʻa momona a kiʻekiʻe i ka protein.

He mea paʻakikī iki ka ʻeli ʻana i ka momona. No laila, he mea paʻakikī i ka ʻōpū leaka o kāu pēpē ke omo pono i ka momona. No laila, hiki i kāu meaʻai hou ke komo pū me kahi ʻano momona kūikawā i kapa ʻia ʻo 'medium-chain triglycerides (MCTs).' ʻO kēia mau (MCTs) kahi ʻano momona i maʻalahi ke omo ʻia e ke kino. Eia kekahi, ʻo ka hoʻohui ʻana i ka protein hou aʻe i kāu meaʻai hiki ke hoʻonui i ka nui o nā meaola a kou kino e omo ai.

Ma muli o ka nalowale ʻana o kekahi mau meaʻai, hiki i ke kauka ke ʻōlelo aku i nā mea hoʻopihapiha huaora a me nā mineral e hōʻoia i ka loaʻa ʻana o ke kino o ke keiki i ka mea e pono ai.

Eia kekahi, hiki i ke kauka ke kuhikuhi i nā lāʻau lapaʻau e like me kēia e kōkua ai i nā ʻano like ʻole o ke kūlana (PIL):

• ʻO nā diuretics (i kapa ʻia hoʻi he mau pila wai): Hana kēia ma ka hoʻēmi ʻana i ka nui o ka wai keu e hōʻiliʻili ana i loko o ke kino ma muli o ka liki ʻana o ka protein.
• Nā analog Somatostatin (e like me octreotide): Kōkua kēia mau lāʻau lapaʻau i ka hōʻemi ʻana i ka leakage o nā mea mai ka ʻōnaehana lymphatic.

Nā koho lapaʻau ʻē aʻe:

• ʻO ke ʻoki kino: Inā ʻaʻole kōkua nā lāʻau ʻē aʻe, a he kakaikahi loa, hiki i nā kauka ke ʻōlelo i ke ʻoki kino e wehe i ka ʻāpana maʻi o ka ʻōpū o kāu keiki.
• Nā lāʻau lapaʻau ʻē aʻe: Ke hoʻomau nei ka noiʻi ʻana i kēia. Ke hoʻāʻo ʻia nei kekahi mau lāʻau lapaʻau hou, e like me sirolimus, no PIL. He mea kōkua paha kēia i kekahi mau hihia, akā ʻaʻole i hoʻohana nui ʻia.

He aha ka wānana no ke kūlana (PIL)?

E like me ka mea i ʻōlelo ʻia ma mua, ʻaʻole hiki ke ho'ōla loa ʻia ka (PIL). Eia nō naʻe, pono e piʻi aʻe nā pae protein i loko o ke koko o ke keiki i loko o kekahi mau pule o ka hoʻomaka ʻana o ka lāʻau. Eia nō naʻe, pono ke keiki e ʻai i ka meaʻai haʻahaʻa-momona a lawe i nā mea hoʻohui meaʻai no ke koena o kona ola.

ʻOiai he pilikia kēia, inā mālama pono ʻia, hiki i ke keiki ke ola maʻamau. ʻO ka mea nui loa, ʻo ia ka hahai ʻana i nā ʻōlelo a ke kauka a mālama i ka ʻai a ke keiki.

Hiki ke pale ʻia ke kūlana (PIL)?

ʻOiai ʻaʻole ʻike ka poʻe noiʻi i ke kumu o kēia kūlana (PIL), ʻaʻohe ala e pale aku ai i kēia kūlana i kēia manawa.

Āhea e pono ai i kaʻu keiki ke ʻike i ke kauka?

Inā ʻike ʻoe i ka pehu ʻana ma nā wāwae o kāu keiki, nā wāwae, a i ʻole kekahi o nā hōʻailona o PIL i ʻōlelo ʻia ma luna, ʻoi aku ka maikaʻi o ka lawe koke ʻana i kāu keiki i ke kauka. ʻO ka wā mua e ʻike ʻia ai, ʻo ka wā koke e hoʻomaka ai ka lāʻau.

He aha nā nīnau e pono iaʻu e nīnau aku i ke kauka o kaʻu keiki?

Inā loaʻa i kāu keiki ka PIL, nui paha kāu mau nīnau e nīnau aku ai i kāu kauka. Eia kekahi o lākou:

• He aha ka meaʻai kūpono loa no kēia maʻi (PIL)?
• He aha nā mea hoʻohui e pono iaʻu e hāʻawi aku i kaʻu keiki?
• He aha hou aʻe kaʻu e hana ai e hoʻokele maikaʻi i nā hōʻailona o kaʻu keiki?
• He koʻikoʻi anei kēia kūlana (PIL)?
• He aha nā pilikia e pono iaʻu e makaʻala ai?

Mai makaʻu e nīnau i kēia mau nīnau. E kūkākūkā i kou mau hopohopo me kāu kauka.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai (Leka Lawe-Home)

Ke hoʻomaka mua ʻoe e ʻike i nā hōʻailona o ka Primary Intestinal Lymphangiectasia, hiki ke paʻakikī iā ʻoe a me kāu mau kauka ke hoʻomaopopo i ka mea e hana nei. Hiki ke lilo i mea huikau a huikau loa. Eia nō naʻe, hiki i kāu mau kauka ke hana pū me ʻoe e kōkua iā ʻoe e ʻike i ke kumu.

ʻO ka mea pōmaikaʻi, me ka ʻai haʻahaʻa momona a me nā mea hoʻohui meaʻai, hiki ke mālama ʻia nā hōʻailona o kāu keiki i ka hala ʻana o ka manawa. E nīnau i ke kauka o kāu keiki i nā mea ʻē aʻe āu e hiki ai ke hana e kōkua i kāu keiki e mālama i kēia kūlana. He mea waiwai nui kou aloha, kākoʻo, a me kou nānā ʻana i kāu keiki i kēia huakaʻi.

`ʻO ka lymphangiectasia ʻōpū mua, PIL, ke ālai ʻana o ka lymphatic, nā maʻi ʻōpū i nā keiki, ka pehu ʻana o nā wāwae, ka nele o ka protein, nā maʻi ʻōnaehana hoʻoheheʻe