Ke ulu nei paha nā puʻupuʻu ʻē i ke kino o kāu keiki? He hōʻailona paha kēia o nā Complex Lymphatic Anomalies (CLA)!

I kekahi manawa, makaʻu loa mākou ke ʻike mākou i nā loli liʻiliʻi i ke kino o kā mākou mau keiki, ʻeā? ʻOi loa aku inā ʻaʻole mākou i ʻike pono i kēlā mau mea. I kēia lā, e kamaʻilio mākou e pili ana i kahi kūlana kakaʻikahi akā koʻikoʻi loa e makaʻala. Ua kapa ʻia kēia ʻo Complex Lymphatic Anomalies , a i ʻole (CLA) no ka pōkole. Ua kapa pū kekahi poʻe iā ia (Lymphangiomatosis) ma mua. No laila, lohe paha ʻoe i kēia mau inoa ʻelua.

ʻO ka mea mua, e nānā kākou, he aha nā Complex Lymphatic Anomalies (CLA)?

I ka ʻōlelo maʻalahi, ʻo Complex Lymphatic Anomalies (CLA) he ʻano laha ʻole akā hiki ke hānau ʻia kahi e ulu ai i kahi keiki nā ʻōpū benign i kapa ʻia ʻo lymphatic malformations a i ʻole lymphangiomas i loko o ka ʻōnaehana lymphatic o ke keiki. Hiki i kēia mau ʻōpū ke ulu i loko o nā iwi o ke keiki, nā ʻiʻo pili, a me nā ʻōpū ʻē aʻe, e hōʻino ana.

E noʻonoʻo wale, ʻoiai inā loaʻa i kā mākou keiki kēia maʻi i ka wā e hānau ʻia ai lākou, ʻo ka hapa nui o ka manawa e hoʻomaka ai nā hōʻailona e ʻike ʻia i ka wā e ʻelemakule iki ai lākou, malia paha he mau makahiki ma hope. ʻO ia ke kumu i kekahi manawa ua lohi loa ka ʻike ʻana i kēia.

No laila, he aha kēia ʻōnaehana Lymphatic?

ʻAe, ke noʻonoʻo nei paha ʻoe i ke ʻano o ka ʻōnaehana lymphatic. I ka ʻōlelo maʻalahi, he ʻōnaehana pale kupaianaha ia i loko o ko mākou kino. Ua like ia me nā koa ma ko mākou ʻāina. Kōkua kēia ʻōnaehana lymphatic i ko mākou kino e pale aku i nā maʻi a hakakā iā lākou inā hana lākou. He ʻāpana koʻikoʻi ia o kā mākou ʻōnaehana pale.

Ua hana ʻia ka ʻōnaehana lymphatic me kahi pūnaewele o nā ʻōmole liʻiliʻi i kapa ʻia he mau kīʻaha lymphatic, nā glands e like me nā lymph nodes, a me nā ʻōpū e like me ka spleen. Ua like kēia mau kīʻaha lymphatic me nā ʻōmole liʻiliʻi. Hōʻiliʻili lākou i ka wai lymph mai nā ʻiʻo o ke kino, kānana iā ia, hoʻomaʻemaʻe iā ia, a laila hoʻihoʻi iā ia i ke koko. ʻAʻole anei kēia he hana kupaianaha?

ʻElua ʻano o CLA: Isolated a me Systemic.

Hiki ke hoʻokaʻawale ʻia nā ʻano Lymphatic paʻakikī i ʻelua mau ʻano nui:

1. Nā CLA i hoʻokaʻawale ʻia: ʻO kēia ka manawa e kaupalena ʻia ai nā puʻupuʻu i hoʻokahi wahi o ke kino o ke keiki. No ka laʻana, hiki iā lākou ke hoʻopilikia wale i ka lua o ka umauma. I kēia hihia, ʻo nā māmā a me nā ʻiʻo palupalu o ka umauma (mediastinum) ka mea i hoʻopilikia nui ʻia.

2. Nā CLA ʻōnaehana: I kēia hihia, hiki i kēia mau puʻupuʻu ke kū ma nā wahi he nui ma ke kino o ke keiki, e like me nā iwi, ka umauma, a me ka ʻōpū.

Ma kēia ʻano, ʻokoʻa paha nā hōʻailona a me nā lāʻau lapaʻau ma muli o ka wahi i hoʻopilikia ʻia.

He aha nā ʻano nui o nā Complex Lymphatic Anomalies (CLA)?

ʻEhā ʻano nui o CLA. ʻOiai he like nā hōʻailona o kēlā me kēia ʻano, he mau kūlana ʻokoʻa lākou. A ʻokoʻa nō hoʻi nā hoʻololi ʻana o ka gene e hoʻokumu ai iā lākou.

• ʻAnomaly Lymphatic Laulā (GLA):Hoʻopilikia pinepine kēia ʻano maʻi i nā ʻāpana like ʻole o ke kino o ke keiki. No ka laʻana, hiki i kēia mau puʻupuʻu ke loaʻa i nā iwi, ka spleen, ke akepaʻa, nā māmā, a me nā ʻiʻo palupalu o ka umauma (mediastinum).
• ʻO Kaposiform Lymphangiomatosis (KLA): ʻO kēia ke ʻano CLA koʻikoʻi loa a laha wikiwiki hoʻi. I loko o KLA, hoʻonui ʻia nā kīʻaha lymphatic e lawe ana i ka wai lymph ma ke kino o ke keiki. ʻO kēia ke kumu e komo ai lākou a hōʻino i nā ʻōpū, nā iwi, a me nā ʻiʻo a puni lākou.
• ʻAnomaly Lymphatic Conduction Central (CCLA): Loaʻa i nā keiki me CCLA nā kīʻaha lymph i hoʻonui ʻia. Eia nō naʻe, i ka CCLA, aia kēia mau kīʻaha lymph i hoʻonui ʻia ma ke kino. Hiki i kēia ke hoʻopilikia i ka ʻōnaehana hoʻoheheʻe a pale i ke keiki mai ka hoʻokahe pono ʻana i ka wai lymph mai ke kino.
• ʻO ka maʻi Gorham-Stout (GSD): ʻIke ʻia hoʻi ʻo ka maʻi ʻo Gorham, ʻo kēia ʻano ke kumu o ka nui o ka iwi e heheʻe ma muli o ke kūkulu ʻia ʻana o nā kīʻaha lymphatic i loko o nā iwi. Ua kapa ʻia hoʻi ʻo ia ʻo "ka maʻi iwi nalowale." Hiki iā ia ke hoʻopilikia i kekahi iwi. Eia nō naʻe, ʻo nā keiki me GSD e ʻike pinepine i ka nalowale o ka iwi ma nā iwi ʻaoʻao, ke poʻo, ka iwi ʻāʻī, a me ka iwi kuamoʻo.

Pehea ka maʻamau o kēia kūlana CLA?

He ʻano laha ʻole nā ​​​​​​Anomalies Lymphatic Complex. No ka mea, ʻaʻole lākou i maʻamau, hiki i kekahi manawa ke paʻakikī i nā kauka ke ʻike pololei iā lākou. No laila, inā loaʻa iā ʻoe nā hōʻailona, ​​​​he mea nui loa e ʻike koke i kahi loea.

He aha ke kumu o kēia CLA?

Ke hoʻāʻo nei nā kauka a me nā mea noiʻi e ʻike i ke kumu o CLA. Eia nō naʻe, ua hōʻike ʻia nā noiʻi hou e hiki ke hana ʻia ke kūlana e nā hoʻololi ʻana o nā gene hoʻoilina i hoʻoili ʻia mai nā mākua a i nā keiki, a me nā hoʻololi ʻana o nā gene somatic e hana ʻia ma hope o ka hānau ʻana. ʻO ia hoʻi, ua pili ia i ka genetics.

ʻO wai ka mea e pilikia ana i ka hoʻomohala ʻana i ka CLA?

Hiki i nā ʻAnomalies Lymphatic Complex ke hoʻopilikia i kekahi. ʻO ke kikoʻī, ʻike pinepine ʻia nā hōʻailona o kēia ʻano i ka wā kamaliʻi a i ʻole ka wā ʻōpio. No laila, inā loaʻa ke ʻano i ka wā hānau, hiki ke lōʻihi ka manawa e hōʻike ai nā hōʻailona.

No laila, he aha nā hōʻailona o CLA?

Hiki ke ʻokoʻa nā hōʻailona mai kekahi keiki a i kekahi keiki me kēia maʻi. Eia kekahi, ʻokoʻa nā hōʻailona ma muli o ka ʻōnaehana okana i hoʻopilikia ʻia a me ke koʻikoʻi o ia mea. ʻO ka hapa nui o ka manawa, lohi ka holomua o kēia maʻi. No laila, ʻaʻole paha mākou e nānā nui i kekahi o nā hōʻailona, ​​a i ʻole manaʻo paha mākou ua loaʻa lākou ma muli o kekahi maʻi ʻē aʻe.

Inā pilikia ka ʻōnaehana hanu o kāu keiki:

I kēia manawa, hiki ke ʻike ʻia nā hōʻailona like me ka hānō.

• ʻŪhū mau.
• Ka paʻakikī o ka hanu ʻana (dyspnea).
• He kani hū ( he kani ʻūlū ) i ka wā e hanu ana.

Inā pilikia ka ʻōnaehana iwi o kāu keiki:

• ʻEha i nā iwi a i ʻole nā ​​hono.
• ʻOiai he hāʻule iki wale nō hiki ke hana i nā haki .
• ʻO ka mae ʻana o nā lālā.
• ʻEha a nāwaliwali paha i hoʻokumu ʻia e ke kaomi ʻana o nā aʻalolo .

Inā pilikia ka ʻōnaehana hoʻoheheʻe o kāu keiki:

• ʻO ka pehu ʻana o ka ʻōpū a me ka ʻeha o ka ʻōpū .
• Anemia a me ka luhi mau.
• Ka maʻi pākī.
• ʻAʻohe ʻono o ka meaʻai.
• Ka luaʻi a me ka luaʻi.
• Ka pohō kaumaha me ke kumu ʻole.

Inā pilikia ka ʻōnaehana mimi o kāu keiki:

• Ke koko i loko o ka mimi (hematuria).
• ʻEha ʻaoʻao .
• Ke koko kiʻekiʻe i nā keiki.

Inā loaʻa i kāu keiki kekahi a ʻoi aku paha o kēia mau hōʻailona, ​​e ʻoluʻolu e ʻimi i ke kōkua lapaʻau. ʻAʻole kēia he mau hōʻailona e pili pono ana iā CLA, akā he mea nui e hoʻāʻo ʻia.

Pehea e ʻike ai nā kauka i kēia kūlana CLA?

No ka mea he maʻi laha ʻole ʻo CLA a ʻokoʻa nā hōʻailona, ​​​​hiki i kekahi manawa ke paʻakikī i nā kauka ke ʻike pololei i ke kūlana. Ma muli o ka hoʻopilikia pinepine ʻana i nā māmā a me nā iwi o nā keiki, hiki i ke kauka o kāu keiki ke kauoha i nā hoʻokolohua e like me:

• Bronchoscopy: No ka nānā ʻana i ka maʻi a i ʻole nā ​​​​​​mea ʻē aʻe i loko o nā alaloa.
• Hoʻāʻo ʻana i ka hana o ka puʻuwai: E ana i ka maikaʻi o ka hana ʻana o kou mau māmā.
• MRI o ke kino holoʻokoʻa: E nānā i nā mea e like me nā ʻōpū i hoʻopilikia ʻia, nā ʻeha iwi, a i ʻole ke wai i loko o ka ʻōpū.
• Nā kukuna X: No ka nānā hou ʻana i nā iwi i ʻike ʻia he kānalua a ʻeha paha ma ka MRI. Hiki i nā kukuna X o ka umauma ke nānā i nā mea ʻē i loko o nā māmā.

I kekahi manawa, lawe nā kauka i kahi laʻana liʻiliʻi o nā lymph nodes a me nā ʻiʻo (biopsy) a nānā iā ia ma lalo o kahi microscope. ʻOiai hiki i kēia hoʻāʻo ke ʻike maopopo i ka CLA, hiki iā ia ke hana i nā pilikia i kekahi manawa, e like me ka hōʻiliʻili ʻana o ka wai lymph a puni nā māmā (chylothorax). Ma muli o kēia, hilinaʻi pinepine nā kauka i nā hoʻokolohua liʻiliʻi ʻole e hana i kahi hōʻailona.

Pehea e mālama ʻia ai ʻo CLA?

No ka mea hiki i ka CLA ke hoʻopilikia i nā wahi he nui o ke kino o ke keiki, hana pū nā kauka, ʻo ia hoʻi, hoʻohana lākou i kahi ala multidisciplinary e hoʻolālā i ka lāʻau lapaʻau kūpono no ke keiki. I ka mālama ʻana iā CLA, ʻo ke kumu nui ka kaohi ʻana i nā hōʻailona o ke keiki. No ka hana ʻana i kēia, hiki iā ʻoe ke hana i nā mea e like me:

• Hoʻopili iwi: No ka hōʻino ʻana o ka iwi ma nā kūlana e like me GSD.
• ʻO ka ʻai kiʻekiʻe-protein a i ʻole ka meaʻai intravenous (total parenteral nutrition a i ʻole TPN).
• Sclerotherapy: E hoʻēmi a hoʻopau paha i nā ʻōpū a i ʻole nā ​​​​kīʻaha lymph.
• ʻO ke ʻoki kino: E wehe i nā puʻupuʻu a i ʻole e hoʻokomo i nā ʻōmole liʻiliʻi (shunt) e hoʻomaʻamaʻa i ke kahe ʻana o ka wai lymph.
• ʻO ka lāʻau radiation a i ʻole chemotherapy: No ka hoʻēmi ʻana i nā ʻōpū noncancerous (hoʻohana wale ʻia kēia mau mea i nā hihia koʻikoʻi loa a hoʻoweliweli i ke ola).

I kēia mau lā iho nei, ua hoʻohana ʻia nā lāʻau lapaʻau i kuhikuhi ʻia e kuhikuhi ana i nā hoʻololi genetic e hoʻoulu ai i kēia kūlana. Ua hōʻike ʻia hoʻi kēia mau lāʻau lapaʻau he pono ia ma ke ʻano he lāʻau lapaʻau laina mua no CLA.

Hiki ke pale ʻia nā Complex Lymphatic Anomalies (CLA)?

ʻO ka mea pōʻino, ʻaʻohe . Loaʻa ka CLA i ka wā hāpai, a ʻaʻole ʻike ʻia ke kumu pololei. No laila, ʻaʻohe mea hiki iā ʻoe ke hana e pale aku i kāu pēpē mai ka loaʻa ʻana o kēia maʻi.

He aha nā pilikia e hiki ke loaʻa mai ka CLA?

Hiki i ka CLA ke hoʻopilikia i kāu keiki no:

• Ascites: Hōʻiliʻili ʻana o ka wai i loko o ka ʻōpū.
• Nā haki iwi.
• ʻO Chylothorax a me Chylopericardium: He hōʻiliʻili o kahi wai waiū (chyle) i loaʻa ka wai lymph a me ka momona i loko o ka membrane e uhi ana i nā māmā (pleura) a i ʻole ka ʻeke a puni ka puʻuwai (pericardium).
• Pūmāmā hiolo / pneumothorax.
• Ka hemahema o ke akepaʻa.
• Ka maʻi lolo.
• Ka hoʻokahe ʻana o ka puʻuwai.
• ʻO ka effusion pleural a me ka edema pulmonary cardiogenic.

Hiki i kekahi o kēia mau pilikia ke koʻikoʻi loa, no laila he mea nui e makaʻala i nā hōʻailona a hahai i nā ʻōlelo a kāu kauka.

He aha ka wā e hiki mai ana o kekahi me CLA?

ʻAʻohe lāʻau lapaʻau no nā Complex Lymphatic Anomalies (CLA). Aia ka wā e hiki mai ana o kāu keiki i nā ʻōnaehana kino i hoʻopilikia ʻia a me ke koʻikoʻi o nā hōʻailona.

ʻO nā pleural effusions ke kumu nui o ka make i waena o ka poʻe me CLA. Eia nō naʻe, me ka mālama pono ʻana a me ka hoʻokele ʻana, hiki ke kōkua ʻia kahi keiki e ola i kahi ola maʻamau.

Āhea wau e ʻike ai i ke kauka?

Inā loaʻa i kāu keiki kekahi o kēia mau hōʻailona, ​​e ʻike koke i ke kauka:

• ʻEha i loko o ka ʻōpū a i ʻole nā ​​naʻau.
• Inā he koko i loko o ka mimi.
• ʻŪhū mau, hanu ʻana, a paʻakikī paha ka hanu ʻana.
• Ka luaʻi, ka luhi, a i ʻole ka pohō kaumaha i wehewehe ʻole ʻia.

Inā loaʻa iā ʻoe kekahi o kēia mau mea, e ʻoluʻolu e ʻike koke i ke kauka.

He aha kaʻu e nīnau aku ai i ke kauka?

Hiki paha iā ʻoe ke nīnau i ke kauka o kāu keiki i nā nīnau e like me kēia:

• He aha nā ʻāpana o ke kino o kaʻu keiki i hoʻopilikia ʻia e kēia maʻi?
• He aha nā koho lapaʻau i loaʻa no kaʻu keiki?
• Pono anei kaʻu keiki i ke ʻoki kino?
• Pono anei au e makaʻala i nā hōʻailona o nā pilikia?

He mea nui loa e nīnau i kēia mau nīnau a loaʻa kahi ʻike maopopo o ke kūlana.

ʻO ka mea hope loa, pono wau e ʻōlelo ...

Ke ʻike ʻoe ua loaʻa i kāu keiki ka CLA (lymphangiomatosis), he mea maʻamau ke makaʻu a hopohopo e pili ana i ka hopena o ia mea i ko lākou olakino a me ka wā e hiki mai ana. He nui o kākou i lohe ʻole paha i kēia maʻi ma mua. ʻAʻohe mea hiki iā ʻoe ke hana e pale aku ai.

ʻO ka hana mua i ke kōkua ʻana i kāu keiki, ʻo ia ka ʻike pono e pili ana i kēia maʻi, kona mau hōʻailona, ​​a me nā lāʻau lapaʻau. E ʻike i ke kauka loea i nā maʻi ʻōnaehana lymphatic. Hiki iā lākou ke pane i kāu mau nīnau a hāʻawi i ka mālama olakino kikoʻī e pono ai kāu keiki.

ʻOiai ʻaʻohe lāʻau lapaʻau no CLA, hiki i ka mālama pono ʻana ke kōkua i kāu keiki e ola i kahi ola hauʻoli a olakino. No laila, e noho ikaika a hana i ka mea maikaʻi loa no kāu keiki.

Nā ʻAnomalies Lymphatic Paʻakikī , Lymphangiomatosis, Nā Maʻi ʻŌnaehana Lymphatic, Nā Puʻupuʻu Kamaliʻi, Osteomalacia, Nā Hōʻailona Pulmonary, Nā Maʻi Kakaikahi o ke Kamaliʻi