Nā ʻano / langerhans / hoʻomanawanui / langerhans-lapaʻau-pdq

Langerhans Cell Histiocytosis lapaʻau (®) - Mana Mana Manaʻo

ʻO ka ʻike pili e pili ana iā Langerhans Cell Histiocytosis (LCH)

NA MANA KUNI

• He ʻano maʻi ʻaʻai ʻo Langerhans cell histiocytosis i hiki ke hōʻino i nā mea a i ʻole e hoʻokumu i nā leona i hoʻokahi a i ʻole nā ​​wahi i loko o ke kino.

• ʻO ka mōʻaukala ʻohana o ka maʻi ʻaʻai a i ʻole ka loaʻa ʻana o kahi makua i hōʻike ʻia i kekahi mau kemika e hoʻonui paha i ka makaʻu o LCH.

• Aia nā hōʻailona a me nā hōʻailona o ka LCH ma kahi o ke kino.

• Ili a me nā kui

• Waha

• Iwi

• Nā pūnana lymph a me kāu

• ʻŌnaehana Endocrine

• Maka

• ʻŌnaehana ʻōnaehana waena (CNS)

• ʻO ke ake a me ka palapū

• Lung

• ʻŌiwi iwi

• ʻO nā hoʻāʻo e nānā ana i nā ʻōnaehana a me nā ʻōnaehana o ke kino kahi e kū ai ka LCH e hoʻohana ʻia e ʻike pono i ka LCH.

• Kū kekahi mau kumu i ka wānana (kahi o ke ola ʻana) a me nā koho lapaʻau.

He ʻano maʻi ʻaʻai ʻo Langerhans cell histiocytosis i hiki ke hōʻino i nā mea a i ʻole e hoʻokumu i nā leona i hoʻokahi a i ʻole nā ​​wahi i loko o ke kino.

He maʻi ʻaʻai laha ʻole ʻo Langerhans cell histiocytosis (LCH) i hoʻomaka i nā hunaola LCH. ʻO nā pūnaewele LCH kahi ʻano o ka huna dendritic e hakakā ai i ka maʻi. I kekahi manawa aia nā hoʻololi (hoʻololi) i nā pūnaewele LCH ke ʻano. Hoʻopili kēia i nā hoʻololi ʻana o nā gen BRAF, MAP2K1, RAS a me ARAF. Hiki i kēia mau hoʻololi ke ulu i nā pūnaewele LCH a hoʻonui koke. ʻO kēia ke kumu o ke kūkulu ʻana o nā pūnaewele LCH i kekahi mau ʻāpana o ke kino, kahi e hiki ai iā lākou ke hōʻino i nā mea a i ʻole nā ​​liona.

ʻAʻole ʻo LCH kahi maʻi o nā pūnaewele Langerhans e kū maʻamau i ka ʻili.

LCH hiki i kēlā me kēia makahiki, akā ʻike pinepine ʻia i nā keiki ʻōpio. ʻOkoʻa ka mālama ʻana i ka LCH i nā keiki mai ka mālama ʻana iā LCH i nā mākua. ʻO ka mālama ʻana iā LCH i nā keiki a me ka mālama ʻana iā LCH i nā mākua i wehewehe ʻia i nā ʻāpana ʻokoʻa o kēia hōʻuluʻulu manaʻo.

ʻO ka mōʻaukala ʻohana o ka maʻi ʻaʻai a i ʻole ka loaʻa ʻana o kahi makua i hōʻike ʻia i kekahi mau kemika e hoʻonui paha i ka makaʻu o LCH.

ʻO nā mea āpau e hoʻonui i kou makaʻu i ka loaʻa ʻana i kahi maʻi i kapa ʻia he kumu weliweli. ʻO ka loaʻa ʻana o kahi mea pilikia ʻaʻole ia he kumu e loaʻa ʻoe i ka maʻi ʻaʻai. ʻaʻohe o nā kumu pilikia ʻaʻole ia he kumu ʻaʻole ʻoe e loaʻa i ka maʻi ʻaʻai. E kamaʻilio me kāu kauka inā manaʻo ʻoe ua makaʻu paha ʻoe.

ʻO nā kumu pilikia no ka LCH me kēia mau mea:

• Loaʻa i kahi makua i hōʻike ʻia i kekahi mau kemika.

• Loaʻa i kahi makua i hōʻike ʻia i ka hao, ka granite, a i ʻole ka lepo lāʻau i ka wahi hana.

• ʻO kahi moʻolelo ʻohana o ka maʻi ʻaʻai, me LCH.

• Loaʻa i ka mōʻaukala pilikino a i ʻole ka moʻolelo ʻohana o ka maʻi thyroid.

• Loaʻa i nā maʻi ma ke ʻano hānau.

• Puhi paka, ʻoi aku i ka poʻe ʻōpio.

• ʻO ka Hispanic.

• ʻAʻole ka lāʻau ʻaukā ma ke ʻano kamaliʻi.

Aia nā hōʻailona a me nā hōʻailona o ka LCH ma kahi o ke kino.

ʻO LCH a i ʻole nā ​​kumu ʻē aʻe i loaʻa ai nā hōʻailona a me nā ʻōuli ʻē aʻe. E nīnau me kāu kauka inā loaʻa iā ʻoe a ʻo kāu keiki paha kekahi o kēia mau mea:

Ili a me nā kui

Hiki i ka LCH ma nā pēpē ke hoʻopili i ka ʻili wale nō. I kekahi mau hihia, ʻoi aku ka maikaʻi o ka LCH ʻili wale nō i mau pule a i ʻole mau mahina a lilo i ʻano i kapa ʻia ʻo Lisy multisystem ʻoi loa ka pilikia.

I nā pēpē, nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i ka ʻili e hoʻopili pū paha:

• ʻO ke kahe ʻana o ka ʻili i like paha me "cap cradle".

• Ke kolo nei i nā kolamu o ke kino, e like me ka kuʻekuʻe o loko a i ʻole ka perineum.

• ʻO ka ʻili i hoʻāla ʻia, ʻeleʻele a poni paha i nā wahi āpau o ke kino.

I nā keiki a me nā mākua, nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i ka ʻili a me nā kui ke komo pū:

• ʻO ke kahe ʻana o ka ʻili i like paha me ka dandruff.

• Hoʻokiʻekiʻe, ʻulaʻula a ʻeleʻele paha, hū ka iwi i ka ʻūhā, ʻōpū, kua, a i ʻole ka umauma, ʻeha a ʻeha paha.

• Nā puʻupuʻu a i ʻole nā ​​ulcer ma ka ʻili.

• Ulcer ma hope o nā pepeiao, ma lalo o ka umauma, a i ʻole ka ʻūhū.

• Nā manamana lima i hāʻule a i ʻole nā ​​discolored groove e holo i ka kui.

Waha

ʻO nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i ka waha e hoʻopili pū paha:

• Gums pehu.

• Nā ʻeha ma luna o ka waha o ka waha, ma loko o nā papalina, a i ʻole i ke alelo a i ʻole nā ​​lehelehe.

Nā niho i lilo i kaulike ʻole a hāʻule paha i waho.

Iwi

ʻO nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i ka iwi:

• ʻO ka ʻōhū a i ʻole ka puʻupuʻu ma luna o ka iwi, e like me ka iwi poʻo, iwi iwi, iwi ʻaoʻao, pelvis, ʻōiwi, iwi ʻūhā, iwi luna o luna, kuʻe lima, kumu maka, a iwi paha a puni ka pepeiao.

• ʻO ka ʻeha ma kahi e pehu ai a he puʻupuʻu paha ma luna o kahi iwi.

ʻO nā keiki me nā liona LCH i loko o nā iwi a puni nā pepeiao a i ʻole nā ​​maka he makaʻu nui no ka maʻi diabetes insipidus a me nā maʻi ʻōnaehana waena.

Nā pūnana lymph a me kāu

ʻO nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i nā lymph node a i ʻole thymus paha i loko:

• Nā pehu lymph pehu.

• Hī pilikia.

• ʻO ka maʻi vena cava kiʻekiʻe loa. Hiki i kēia ke hoʻoulu i ka hanu, pilikia i ka hanu ʻana, a me ka pehu ʻana o ka maka, ka ʻāʻī, a me nā lima kiʻekiʻe.

ʻŌnaehana Endocrine

ʻO nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i ka pituitary gland hiki ke hoʻopili ʻia:

• Insipidus diabetes. Hiki i kēia ke hana i ka make wai ikaika a me ka urination pinepine.

• Hoʻonui lohi.

• ʻO ka wā ʻōpio a i ʻole ka hopena o ka wā ʻōpio.

• Kaumaha nui loa.

ʻO nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i ka thyroid e hoʻopili pū paha:

• Pehu thyroid.

• Hypothyroidism. Hiki i kēia ke kumu i ka luhi, ka ʻole o ka ikaika, ka makaʻala i ke anuanu, ka paʻa paʻa ʻana, ka ʻili maloʻo, ka lauoho lahilahi, nā pilikia hoʻomanaʻo, ka hoʻopilikia ʻana i ka noʻonoʻo, a me ke kaumaha. I nā pēpē, hiki i kēia ke kumu i ka lilo o ka makemake ʻole a ʻānai ʻia i ka meaʻai. I nā keiki a me nā ʻōpio, hiki i kēia ke kumu i nā pilikia o ka lawena, ka loaʻa ʻana o ke kaumaha, ka lohi o ka ulu ʻana, a me ka wā ʻōpio.

• Hī pilikia.

Maka

ʻO nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e hoʻopili i ka maka e hoʻopili pū paha:

• Nā pilikia ʻike.

ʻŌnaehana ʻōnaehana waena (CNS)

ʻO nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i ka CNS (lolo a me ka iwi kuamoʻo)

• Ka nalowale o ke kaulike, nā neʻe ʻana o ke kino i hoʻohui ʻole ʻia, a me ka pilikia e hele wāwae.

• Pilikia i ka ʻōlelo ʻana.

• Pilikia ka ʻike ʻana.

• Poʻo poʻo poʻo.

• Hoʻololi i ka lawena a i ʻole ke ʻano.

• Nā pilikia hoʻomanaʻo.

Hoʻokumu ʻia kēia mau hōʻailona a me nā ʻōuli e nā leona i ka CNS a i ʻole ka CNS neurodegenerative Syndrome.

ʻO ke ake a me ka palapū

ʻO nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i ke ake a i ʻole ka ʻāpala e komo pū paha:

• ʻO ka ʻōhū ʻana i loko o ka ʻōpū i hoʻokumu ʻia i ka hoʻonui ʻia o ka wai.

• Hī pilikia.

• Melemele o ka ʻili a keʻokeʻo o nā maka.

• Kāwili.

• Pākuʻi maʻalahi a hoʻokahe paha.

• Luhi nui loa.

Lung

ʻO nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i ka akemāe pū paha:

• Pulupā naha. Hiki i kēia ʻano ke hōʻeha i ka umauma a i ʻole ke kūiki, ka pilikia i ka hanu ʻana, ka luhi, a me ke kala bluish i ka ʻili.

• Hoʻopilikia i ka hanu ʻana, keu hoʻi i nā mākua puhipaka.

• Mū maloo.

• ʻEha ʻeha.

ʻŌiwi iwi

ʻO nā hōʻailona a i ʻole nā ​​hōʻailona o ka LCH e pili ana i ka momona o ka iwi.

• Pākuʻi maʻalahi a hoʻokahe paha.

• Fiva.

• Nā maʻi pinepine.

ʻO nā hoʻāʻo e nānā ana i nā ʻōnaehana a me nā ʻōnaehana o ke kino kahi e kū ai ka LCH e hoʻohana ʻia e ʻike pono i ka LCH.

Hiki ke hoʻohana i nā hoʻokolohua a me nā kaʻina hana e ʻike ai (loaʻa) a ʻike ʻia ka LCH a i ʻole nā ​​kumu i hoʻokumu ʻia e LCH:

• ʻO ka hoʻāʻo kino a me ka mōʻaukala olakino: kahi hōʻike o ke kino e nānā i nā hōʻailona laulā o ke olakino, e like me ka nānā ʻana i nā hōʻailona o ka maʻi, e like me nā puʻupuʻu a i ʻole nā ​​mea ʻē aʻe e ʻano ʻē. E lawe ʻia kahi moʻolelo o nā ʻano olakino o ka mea maʻi a me nā maʻi i hala a me nā mālama ʻana.

• Nānā Neurological: He mau nīnau a me nā hoʻāʻo e nānā i ka lolo, nā ʻaluna, a me nā hana o ke aʻalolo. Nānā ka hōʻike i ke kūlana noʻonoʻo o ke kanaka, ka hoʻonohonoho ʻana, a me ka hiki ke hele maʻamau, a me ka maikaʻi o ka hana o nā mākala, nā manaʻo, a me nā reflexes. Kāhea ʻia paha kēia he neuro exam a i ʻole he neurologic exam.

• Helu piha ke koko (CBC) me ka ʻokoʻa: kahi kaʻina hana e huki ʻia ai kahi hāpana o ke koko a nānā ʻia no kēia mau mea:

• ʻO ka nui o ka hemoglobin (ka protein i hāpai i ka oxygen) i loko o nā hunaola ʻulaʻula.

• ʻO ka ʻāpana o ka hāpana koko i hana ʻia i nā hunaola ʻulaʻula.

• ʻO ka helu a me ke ʻano o nā hunaola keokeo.

• ʻO ka helu o nā hunaola ʻulaʻula a me nā platelet.

• Nā aʻo kemika koko: kahi hana e nānā ʻia ai kahi hāpana koko e ana i ka nui o kekahi mau mea i hoʻokuʻu ʻia i loko o ke kino e nā meaola a me nā mea i loko o ke kino. ʻO kahi mea maʻamau (ʻoi aʻe a i ʻole haʻahaʻa ma mua o ka maʻamau) o kahi mea hiki ke lilo i hōʻailona o ka maʻi.

• Hōʻoia i ke ʻano o ke akepaʻa : He hoʻāʻo koko e ana i nā pae o ke koko o kekahi mau mea i hoʻokuʻu ʻia e ke ake. ʻO kahi pae kiʻekiʻe a haʻahaʻa paha o kēia mau mea i hiki ke lilo i hōʻailona o ka maʻi i ke ake.

• ʻO ka hoʻāʻo ʻana o ka ʻāpana BRAF: kahi hoʻokolohua hoʻokolohua kahi e hoʻāʻo ʻia ai kahi ʻāpana o ke koko a i ʻole nāʻaʻaʻa no kekahi mau hoʻololi i ka gen BRAF.

• ʻO Urinalysis: kahi hoʻāʻo e nānā i ke kala o ka mimi a me nā mea o loko, e like me ke kō, protein, ʻulaʻula, a me nā ʻula koko keʻokeʻo.

• ʻO ka hoʻāʻo hoʻowahāwahā wai: ʻO kahi hoʻāʻo e nānā i ka nui o ka mimi i hana ʻia a inā e noʻonoʻo ʻia ke hāʻawi ʻia i kahi wai liʻiliʻi a hāʻawi ʻole paha. Hoʻohana ʻia kēia hōʻike e ʻike pono ai i ka maʻi diabetes insipidus, a ʻo LCH paha ka hopena.

• ʻO ka ʻiʻo o ka iwi iwi a me ka biopsy: Ka lawe ʻia ʻana o ka iwi iwi a me kahi ʻāpana o ka iwi ma ka hoʻokomo ʻana i kahi nila kuha i loko o ka iwi ʻūʻū. ʻIke kahi pathologist i ka momona o ka iwi a me ka iwi ma lalo o kahi microscope e nānā i nā hōʻailona o LCH.

ʻIʻini iwi a me ka biopsy. Ma hope o ka helu ʻia ʻana o kahi ʻili o ka ʻili, hoʻokomo ʻia kahi nila o ka iwi iwi i ka iwi ʻūhā o ka mea maʻi. Lawe ʻia nā laʻana o ke koko, ka iwi, a me ka iwi o ka iwi no ka ninaninau ʻana ma lalo o kahi microscope.

E hana ʻia paha kēia mau hoʻāʻo ma ke kiko i lawe ʻia.

• Immunohistochemistry: ʻO kahi hoʻokolohua hoʻokolohua e hoʻohana ai i nā anti-antibodies e nānā ai no kekahi antigens (markers) i kahi laʻana o kaʻiʻo o ka mea maʻi Hoʻopili pinepine ʻia nā mea ʻino i kekahi enzyme a i ʻole ka waihoʻoluʻu fluorescent. Ma hope o ka hoʻopili ʻana o nā mea ʻume i kekahi antigen kikoʻī i ka laʻana kiko, hoʻōla ʻia ka ʻimene a me ka kala, a laila ʻike ʻia ka antigen ma lalo o kahi microscope. Hoʻohana ʻia kēia ʻano hoʻāʻo e kōkua i ke kuhi ʻana i ka maʻi ʻaʻai a kōkua e haʻi i kekahi ʻano maʻi ʻaʻai mai kekahi ʻano maʻi ʻaʻai.

• Kahe uila ʻili pūnaewele. Hoʻopili ʻia nā hunaola mai kahi hāpana o ke koko o ka mea maʻi, ka iwi o ka iwi, a i ʻole nā ​​mea ʻē aʻe me ka pena wai fluorescent, waiho ʻia i loko o kahi wai, a laila hele kekahi i hoʻokahi manawa i ke kaola o ke kukui. Hoʻokumu ʻia nā hopena o ka hoʻāʻo e pili ana i ka hopena o nā hunaola i hoʻohuehue ʻia me ka pena fluorescent i ke kaola o ke kukui.

• ʻImi iwi: kahi hana e nānā ai inā aia ka hoʻokaʻawale wikiwiki ʻana i nā hunaola i ka iwi. Hoʻokomo ʻia kahi mea liʻiliʻi loa o nā mea radioactive i loko o ke aa a hele i waena o ke kahe o ke koko. Hōʻiliʻili ka mea lekiō i nā iwi me ka maʻi ʻaʻai a ʻike ʻia e kahi scanner.

ʻImi iwi. Hoʻokomo ʻia kahi mea liʻiliʻi o ka radioactive i ke aa o ke keiki a hele i loko o ke koko. Hōʻiliʻili ka mea lekiō i nā iwi. Ke moe nei ke keiki ma kahi pākaukau e heʻe ma lalo o ka scanner, ʻike ʻia ka mea radioactive a hana ʻia nā kiʻi ma ka papapihi kamepiula.

• X-ray: Kākā x o nā mea a me nā iwi i loko o ke kino. ʻO ka x-ray kahi ʻano kukuna ikehu e hiki ke hele ma waena o ke kino a ma ke kiʻi ʻoniʻoni, e hana ana i kiʻi o nā wahi i loko o ke kino. I kekahi manawa hana ʻia kahi ana iwi. He hana kēia e x-ray i nā iwi āpau i ke kino.

• CT scan (CAT scan): Kahi hana e hana ai i ka moʻo o nā kiʻi kikoʻī o nā wahi i loko o ke kino, i lawe ʻia mai nā ʻaoʻao ʻokoʻa. Hana ʻia nā kiʻi e kahi kamepiula i hoʻopili ʻia i kahi mīkini x-ray. Hoʻokomo ʻia paha ke kala i loko o ke aa a i ʻole ale ʻia e kōkua i nā ʻōpū a i ʻole nā ​​kino i hōʻike hou aku me ka maopopo. Kāhea ʻia kēia kaʻina hana he tomography i helu ʻia, kamepiula tomography, a i ʻole he tomography axial computerized.

ʻO ka scan tomography compute (CT) o ka ʻōpū. Moe ka mea maʻi ma ka papaʻaina e paheʻe i loko o ka mīkini CT, e lawe nei i nā kiʻi x-ray o loko o ke kino.

• MRI (kiʻi resonance ʻume mākenēki): kahi hana e hoʻohana ai i ka magnet, nā nalu lekiō, a me kahi kamepiula e hana i kahi moʻo o nā kiʻi kikoʻī o nā wahi i loko o ke kino. Hoʻokomo ʻia kahi mea i kapa ʻia ʻo gadolinium i kahi aa koko. Hōʻiliʻili ka gadolinium a puni nā pūnaewele LCH i hōʻike ʻia lākou i ka mālamalama i ke kiʻi. Kāhea ʻia kēia kaʻina hana he kiʻi nukelea kiʻi resonance (NMRI).

ʻO ke kiʻi ʻana o ka resonance ʻume mākenēki (MRI) o ka ʻōpū. Moe ke keiki ma ka papaʻaina e paheʻe i ka scanner MRI, e paʻi ana i nā kiʻi o loko o ke kino. Kōkua ka pale ma luna o ka ʻōpū o ke keiki e hoʻomaʻemaʻe i nā kiʻi.

• PET scan (positron emission tomography scan): kahi hana e loaʻa ai nā pūnaewele tumora i loko o ke kino. Hoʻokomo ʻia kahi liʻiliʻi o ka glucose radioactive (kō) i loko o kahi aa koko. Hoʻopili ka scanner PET a puni ke kino a hana i kahi kiʻi o kahi e hoʻohana ʻia ai ka glucose i ke kino. ʻOi aku ka mālamalama o nā hunaola Tumor i ke kiʻi no ka mea ʻoi aku ka nui o kā lākou hana a lawe i nā glucose ma mua o ka hana ʻana o nā hunaola maʻamau.

Positron emission tomography (PET) scan NineManga.com Moe ke keiki ma ka papaʻaina e heʻe ai ka scanner PET. Hoʻomaha ke poʻo a me ke kaula keʻokeʻo e kōkua i ke keiki e moe mau. Hoʻokomo ʻia kahi liʻiliʻi o ka glucose radioactive (kō) i ka aa o ke keiki, a hana kahi scanner i kiʻi i kahi e hoʻohana ʻia ai ka glucose i ke kino. Hōʻike ʻia nā hunaola maʻi ʻālohilohi i ke kiʻi no ka mea lawe lākou i ka glucose ma mua o ka hana ʻana o nā hunaola maʻamau.

• ʻO ka hoʻāʻo ʻana o Ultrasound: kahi kaʻina hana e kipaku ʻia ai nā nalu kani ikaika (ultrasound) mai nā aʻa a i ʻole nā ​​ʻola a hana i nā leo. Hoʻokumu nā leo i kahi kiʻi o nā kino o ke kino i kapa ʻia he sonogram. Hiki ke paʻi ʻia ke kiʻi e nānā ma hope.

ʻO ke ultrasound ʻōpū. Hoʻouna ʻia kahi transducer ultrasound i kahi kamepiula ma luna o ka ʻōpū. Hoʻopiʻi ka transducer ultrasound i nā nalu kani i nā mea kūloko a me nā aʻa e hana i nā leo e hana i kahi sonogram (kiʻi kamepiula).

• Hōʻike hana pulmonary (PFT): kahi hoʻāʻo e ʻike i ke ʻano o ka hana o nā māmā. Ana ʻia ka nui o ka ea e hiki ai i nā māmā ke hoʻopaʻa a pehea ka wikiwiki e neʻe ai ka ea i loko a i waho o nā māmā. Ana ʻia hoʻi ka nui o ka oxygen e hoʻohana ʻia ai a pehea e hāʻawi ʻia ai ke kalapona kalapona i ka hanu ʻana. Kāhea ʻia kēia mea ʻo ka hoʻāʻo hana lung.

• ʻO Bronchoscopy: kahi hana e nānā ai i loko o ka trachea a me nā ala nui i loko o ka māmā no nā wahi kūpono ʻole. Hoʻokomo ʻia kahi bronchoscope ma o ka ihu a waha paha i loko o ka trachea a me nā akemāmā. ʻO ka bronchoscope kahi mea lahilahi, like me ka Tube me ke kukui a me nā aniani no ka nānā ʻana. Loaʻa paha iā ia kahi mea hana e hemo i nā mea kiko kiko, i nānā ʻia ma lalo o kahi microscope no nā hōʻailona o ka maʻi ʻaʻai.

• Endoscopy: Kahi hana e nānā ai i nā ʻokena a me nā ʻaʻa i loko o ke kino e nānā ai no nā wahi kūpono ʻole i ka gastrointestinal tract a i ʻole nā ​​ake māmā. Hoʻokomo ʻia kahi endoscope ma o kahi ʻoki (ʻoki) i ka ʻili a i ʻole wehe i ke kino, e like me ka waha. ʻO ka endoscope kahi mea lahilahi, e like me ka Tube me kahi kukui a me nā aniani no ka nānā ʻana. Loaʻa paha iā ia kahi mea hana e hemo ai i nā laʻana a i ʻole nā ​​lymph node Eia kekahi laʻana, i nānā ʻia ma lalo o kahi microscope no nā hōʻailona o ka maʻi.

• ʻO Biopsy: Ka lawe ʻia ʻana o nā pūnaewele a i ʻole nāʻaʻaʻa i hiki iā lākou ke ʻike ma lalo o kahi microscope e kahi pathologist e nānā ai i nā pūnaewele LCH. No ke kuhikuhi ʻana i ka LCH, kahi biopsy o ka iwi, ka ʻili, nā lymph node, ke ake, a me nā wahi ʻē aʻe o ka maʻi e hana ʻia ai.

Kū kekahi mau kumu i ka wānana (kahi o ke ola ʻana) a me nā koho lapaʻau.

ʻOi aku ka maikaʻi o ka LCH i loko o nā lālā e like me ka ʻili, nā iwi, nā pūnana lymph, a i ʻole ka pituitary gland me ka lapaʻau ʻana a ua kapa ʻia ʻo "low-risk". ʻOi aku ka paʻakikī o ka mālama ʻana i ka LCH i ke kīkala, ke ake, a me ka iwi iwi a ua kapa ʻia ʻo "high-risk".

Pili ka wānana a me nā koho lapaʻau i kēia mau mea:

• Ehia mau makahiki o ka mea maʻi ke ʻike ʻia me LCH.

• ʻO nā ʻōiwi a ʻino paha nā ʻōnaehana e hoʻopili ʻia e LCH.

• Ehia mau ʻōnaehana a i ʻole nā ​​ʻōnaehana o ke kanesa e hoʻopili ai.

• ʻIke ʻia ka maʻi ʻaʻai i ke akepaʻa, ka palu, ka iwi iwi, a i ʻole kekahi mau iwi i ka iwi poʻo.

• Pehea ka pane koke ʻana o ka maʻi ʻaʻai i ka lapaʻau mua.

• Inā paha aia kekahi mau hoʻololi i ka moʻokūʻauhau BRAF.

• Inā ua hōʻoia wale ʻia ka maʻi ʻaʻai a ua hoʻi mai paha (recurred).

I nā pēpē a hiki i hoʻokahi makahiki, hiki i ka LCH ke hele me ka ʻole o ka lapaʻau.

Nā kahua o LCH

NA MANA KUNI

• ʻAʻohe ʻōnaehana hoʻonohonoho no Langerhans cell histiocytosis (LCH).

• Hoʻokumu ʻia ka mālama ʻana i ka LCH ma kahi e loaʻa ai nā hunaola LCH i loko o ke kino a inā paha he haʻahaʻa ʻo LCH a i ʻole kahi makaʻu nui.

• LCH hou

ʻAʻohe ʻōnaehana hoʻonohonoho no Langerhans cell histiocytosis (LCH).

ʻO ka nui a hoʻolaha paha o ka maʻi ʻaʻai e wehewehe pinepine ʻia ma ke ʻano he pae. ʻAʻohe ʻōnaehana hoʻonohonoho no LCH.

Hoʻokumu ʻia ka mālama ʻana i ka LCH ma kahi e loaʻa ai nā hunaola LCH i loko o ke kino a inā paha he haʻahaʻa ʻo LCH a i ʻole kahi makaʻu nui.

Hōʻike ʻia ʻo LCH ma ke ʻano he maʻi maʻi ʻōnaehana a i ʻole multisystem maʻi, kaukaʻi ʻia i ka nui o nā ʻōnaehana kino e hoʻopili ʻia.

• LCH ʻōnaehana hoʻokahi: Loaʻa ʻo LCH ma kekahi ʻāpana o kahi ʻola a i ʻole ʻōnaehana kino a i ʻole ma mua o hoʻokahi ʻāpana o kēlā ʻoka a ʻōnaehana kino paha. ʻO ka iwi ka wahi nui loa no ka LCH e loaʻa ai.

• Multisystem LCH: Kū ʻo LCH i ʻelua a ʻoi paha o nā ʻola a i ʻole nā ​​ʻōnaehana o ke kino a i ʻole hoʻolaha ʻia i loko o ke kino. ʻOi aku ka maʻamau o Multisystem LCH ma mua o ka LCH pūnaewele hoʻokahi.

Hiki i ka LCH ke hoʻopili i nā lālā haʻahaʻa a i ʻole nā ​​kūlana kiʻekiʻe:

• ʻO nā lālā haʻahaʻa haʻahaʻa me ka ʻili, ka iwi, nā akemāmā, nā pūnana lymph, nā ʻōpū o ka gastrointestinal, ka pituitary gland, ka thyroid gland, ka thymus, a me ka ʻōnaehana waena (CNS).

• ʻO nā mea ʻāpilikia kiʻekiʻe me ke akepaʻa, ka iwi, a me ka iwi iwi.

LCH hou

ʻO LCH hou ka maʻi ʻaʻai i hoʻi hou (hoʻi mai) ma hope o ka mālama ʻia ʻana. E hoʻi paha ka maʻi ʻaʻai ma ka wahi like a i ʻole nā ​​ʻāpana ʻē aʻe o ke kino. Kū pinepine ia i ka iwi, nā pepeiao, ka ʻili, a i ʻole ka pituitary gland. Hoʻomaʻa pinepine ʻo LCH i ka makahiki ma hope o ka hoʻōki ʻana i ka lāʻau. Ke ala hou ʻo LCH, kapa ʻia paha ia he reactivation.

Ka Nohie Manaʻo Oponopono Laulima no LCH

NA MANA KUNI

• Aia nā ʻano lapaʻau like ʻole no nā mea maʻi me ka Langerhans cell histiocytosis (LCH).

• Pono nā kamaliʻi me ka LCH e hoʻomaʻamaʻa ʻia e kā lākou hui mālama ola i loea i ka mālama ʻana i ka maʻi ʻaʻa kamaliʻi.

• Hoʻohana ʻia nā ʻano ʻeiwa o ka mālama maʻamau.

• ʻO Chemotherapy

• ʻOki kino

• Lapaʻau hoʻomākala

• Hoʻoikaika Photodynamic

• Immunotherapy

• Pahuhopu kau ʻia

• Laau lāʻau ʻē aʻe

• Ke kuʻi ʻōnaʻi ʻōnaeho

• Nānā

• Ke hoʻāʻo ʻia nei nā ʻano lapaʻau hou i nā hoʻokolohua hoʻokolohua.

• ʻO ka mālama ʻana no ka Langerhans cell histiocytosis ka mea e hopena ai i nā hopena ʻaoʻao.

• Makemake paha nā mea maʻi e noʻonoʻo e pili ana i ka hoʻokolokolo ʻana.

• Hiki i nā mea maʻi ke komo i nā hoʻokolohua hoʻokolohua ma mua, i ka wā, a ma hope paha o ka hoʻomaka ʻana i kā lākou lapaʻau.

• Ke kū nei ka lapaʻau ʻana o LCH, hiki mai paha nā leona hou a i ʻole hoʻi e hoʻi mai nā ʻino kahiko.

• Pono paha nā hoʻokolohua ukali.

Aia nā ʻano lapaʻau like ʻole no nā mea maʻi me ka Langerhans cell histiocytosis (LCH).

Loaʻa nā ʻano lapaʻau like ʻole no nā mea maʻi me LCH. Kūlana maʻamau kekahi mau hōʻailona (ʻo ka lāʻau i hoʻohana ʻia i kēia manawa), a ʻo kekahi e hoʻāʻo ʻia nei i nā hoʻokolohua hoʻokolohua. ʻO kahi hoʻokolohua hoʻokolohua lapaʻau kahi noiʻi noiʻi i manaʻo ʻia e kōkua i ka hoʻomaikaʻi ʻana i nā mālama ʻana i kēia manawa a i ʻole loaʻa ka ʻike e pili ana i nā mālama hou no nā maʻi me ka maʻi ʻaʻai. Ke hōʻike nā hoʻokolohua hoʻokolohua ʻoi aku ka maikaʻi o kahi lapaʻau hou ma mua o ka maʻamau maʻamau, lilo paha ka lāʻau hou i ka lapaʻau maʻamau. Ke hiki, pono nā mea maʻi e komo i ka hoʻokolohua hoʻokolohua i mea e loaʻa ai nā ʻano lapaʻau hou no LCH. ʻO kekahi mau hoʻokolohua hoʻokolohua e hāmama wale i nā mea maʻi i hoʻomaka ʻole i ka lāʻau.

Ke mālama ʻia nei nā hoʻokolohua hoʻokolohua ma nā wahi he nui o ka ʻāina. Loaʻa nā ʻike e pili ana i nā hoʻokolohua hoʻokolohua mau mai ka pūnaewele NCI. Ke koho nei i ka lāʻau kūpono kūpono he hoʻoholo ia e pili pono i ka mea maʻi, ʻohana, a me ka hui mālama olakino.

Pono nā kamaliʻi me ka LCH e hoʻomaʻamaʻa ʻia e kā lākou hui mālama ola i loea i ka mālama ʻana i ka maʻi ʻaʻa kamaliʻi.

E mālama ʻia ka lapaʻau ʻana e kahi oncologist pediatric, he kauka i loea i ka mālama ʻana i nā keiki me ka maʻi ʻaʻai. Hana ka oncologist pediatric me nā mea lawelawe olakino pediatric ʻē aʻe i loea i ka mālama ʻana i nā keiki me LCH a i loea i kekahi mau wahi o ka lāʻau. Pākuʻi ʻia kēia mau mea i lalo:

• Kahuna Pediatrician.

• Kauka ʻōpio.

• Hematologist Pediatric.

• Oncologist uila.

• Neurologist.

• Endocrinologist.

• ʻO ka loea kahu maʻi Pediatric.

• Lapaʻau hoʻōla.

• ʻOlogistlapa psychologist.

• Limahana limahana.

Hoʻohana ʻia nā ʻano ʻeiwa o ka mālama maʻamau.

ʻO Chemotherapy

ʻO Chemotherapy kahi lāʻau lapaʻau maʻi maʻi e hoʻohana i nā lāʻau e hōʻoki i ka ulu ʻana o nā hunaola maʻi ʻaʻai, ma ka luku ʻana i nā hunaola a i ʻole ke kāpae ʻana iā lākou mai ka hoʻokaʻawale. Ke lawe ʻia ka chemotherapy e ka waha a i ʻole injected i loko o kahi aa koko a i ʻole nā ​​mākala, komo nā lāʻau i ke kahe o ke koko a hiki ke kiʻi i nā hunaola maʻi ʻōpio i loko o ke kino (chemotherapy ʻōnaehana). Ke kau pololei ʻia ka chemotherapy ma luna o ka ʻili a i ʻole i loko o ka wai cerebrospinal, kahi ʻokana, a i ʻole he kino o ke kino e like me ka ʻōpū, hoʻopili nui nā lāʻau i nā hunaola maʻi ʻaʻai i loko o kēlā mau wahi (chemotherapy āpana).

Hāʻawi ʻia ʻo Chemotherapy e ka injection a i ʻole ka waha a i ʻole noi ʻia i ka ʻili e mālama iā LCH.

ʻOki kino

Hiki ke hoʻohana ʻia ke kaha ʻoki e wehe i nā liona LCH a me kahi liʻiliʻi o nā mea olakino kokoke. ʻO Curettage kahi ʻano o ke kaha ʻana e hoʻohana ana i ka curette (kahi mea ʻoi, mea hana kīʻaha) e ʻānai ai i nā pūnaewele LCH mai ka iwi.

Ke loaʻa nā ʻino ʻino a ʻino nui paha, hiki ke hoʻoneʻe ʻia ke kino holoʻokoʻa a kāpae ʻia me kahi akepaʻa olakino olakino a i ʻole puʻupuʻu mai kahi mea hāʻawi.

Lapaʻau hoʻomākala

ʻO ka radiation radiation kahi lāʻau maʻi maʻi maʻi e hoʻohana i nā kukuna x nui ikaika a i ʻole nā ​​ʻano radiation e hoʻomake i nā hunaola maʻi ʻaʻai a mālama ʻole paha i ka ulu ʻana. Hoʻohana ka radiation radiation kūwaho i kahi mīkini ma waho o ke kino e hoʻouna i ka radiation i kahi o ke kino me ka maʻi ʻaʻai. Hāʻawi ʻia paha ʻo Ultraviolet B (UVB) radiation radiation ma ka hoʻohana ʻana i kahi kukui kūikawā e kuhikuhi ana i ka radiation i nā ʻili LCH ʻili.

Hoʻoikaika Photodynamic

ʻO Photodynamic therapy kahi lāʻau lapaʻau i hoʻohana i ka lāʻau lapaʻau a me kekahi ʻano o ke kukui laser e pepehi i nā hunaola maʻi ʻaʻai. ʻO kahi lāʻau e hana ʻole a hiki i ka wā e hōʻike ʻia i ka mālamu e hoʻopili ʻia i loko o kahi aa koko. Hōʻiliʻili ka lāʻau i nā mea maʻi ʻaʻai ma mua o nā hunaola maʻamau. No ka LCH, pili ke kukui kukuna i ka ʻili a lilo ka lāʻau i mea hana a make i nā hunaola maʻi ʻaʻai. Hoʻomaʻamaʻa ka lāʻau Photodynamic i ka hōʻemi iki ʻana i nā mea olakino ʻO nā mea maʻi i loaʻa ka photodynamic therapy ʻaʻole pono e hoʻolōʻihi i ka manawa i ka lā.

I kekahi ʻano o ka photodynamic therapy, i kapa ʻia ʻo psoralen a me ultraviolet A (PUVA) therapy, loaʻa i ka mea maʻi ka lāʻau i kapa ʻia psoralen a laila ultraviolet A kuhikuhi ʻia ka radiation i ka ʻili.

Immunotherapy

ʻO ka Immunotherapy kahi lapaʻau e hoʻohana i ka ʻōnaehana pale maʻi o ka mea maʻi e hakakā i ka maʻi ʻaʻai. Hoʻohana ʻia nā mea i hana ʻia e ke kino a i hana ʻia paha i kahi keʻena hoʻokolohua e hoʻonui, kuhikuhi, a hoʻihoʻi hou i nā pale kūlohelohe o ke kino i ka maʻi ʻaʻai. Kāhea ʻia kēia ʻano lapaʻau maʻi ʻaʻai a biotherapy a i ʻole biologic therapy. Aia kekahi ʻano o ka immunotherapy:

• Hoʻohana ʻia ʻo Interferon e mālama iā LCH o ka ʻili.

• Hoʻohana ʻia ʻo Thalidomide e mālama iā LCH.

• Hoʻohana ʻia ka immunoglobulin intravenous (IVIG) e mālama iā CNS neurodegenerative Syndrome.

Pahuhopu kau ʻia

ʻO ka therapy target kahi ʻano lapaʻau e hoʻohana i nā lāʻau a i ʻole nā ​​mea ʻē aʻe e hoʻouka aku i nā hunaola maʻi ʻaʻai. Hiki i nā mea hoʻomākaukau pahuhopu ke hōʻemi i ka hōʻeha i nā hunaola maʻamau ma mua o ka chemotherapy a i ʻole radiation radiation. Aia kekahi mau ʻano o ka hoʻomōhala hoʻomāka ʻia.

• Hoʻopili nā mea kāohi Tyrosine kinase i nā hōʻailona pono e ulu ai nā tumors. ʻO nā mea kālai lāʻau Tyrosine kinase i hoʻohana ʻia e mālama iā LCH me kēia mau mea:

• Hoʻopau ʻo Imatinib mesylate i nā hunaola koko mai ka huli ʻana i nā hunaola dendritic i lilo i mau pūnaʻi maʻi maʻi

• Pākuʻi nā mea pale BRAF i nā protein i pono no ka ulu ʻana o ka cell a make paha i nā hunaola maʻi ʻaʻai. ʻIke ʻia ka ʻāpana BRAF i kahi palapala mutated (hoʻololi ʻia) i kekahi LCH a me ke ālai ʻana iā ia e kōkua i ka mālama ʻana i nā hunaola maʻi ʻaʻai.

• ʻO Vemurafenib a me dabrafenib nā mea pāpālina BRAF i hoʻohana ʻia e mālama iā LCH.

• Hoʻohana ka monoclonal antibody therapy i nā mea pale i hana ʻia i loko o ke keʻena hoʻokolohua mai kahi ʻano hoʻokahi o nā pūnaewele immune immune. Hiki i kēia mau lāʻau ʻino ke hoʻomaopopo i nā mea ma nā hunaola maʻi ʻaʻai a i ʻole nā ​​mea maʻamau e kōkua ai i nā hunaola maʻi ʻaʻai. Hoʻopili nā mea ʻino i nā mea a pepehi i nā hunaola maʻi ʻaʻai, ālai i ko lākou ulu ʻana, a i ʻole mālama iā lākou mai ka hoʻolaha ʻana. Hoʻohana wale ʻia lākou a i ʻole lawe i nā lāʻau, toxins, a i ʻole radioactive pono i nā hunaola maʻi ʻaʻai. Hāʻawi ʻia nā antibodies Monoclonal e ka hoʻokomo.

• ʻO Rituximab kahi antibody monoclonal i hoʻohana ʻia e mālama iā LCH.

Laau lāʻau ʻē aʻe

ʻO nā lāʻau ʻē aʻe i hoʻohana ʻia e mālama iā LCH me kēia mau mea:

• Hoʻohana ʻia ʻo Steroid therapy, e like me prednisone, e mālama i nā liona LCH.

• Hoʻohana ʻia ka lāʻau Bisphosphonate (e like me pamidronate, zoledronate, a i ʻole alendronate) e mālama i nā liona LCH o ka iwi a hoʻemi i ka ʻeha o ka iwi.

• ʻO nā lāʻau anti-inflammatory he lāʻau lapaʻau (e like me pioglitazone a me rofecoxib) i hoʻohana mau ʻia e hōʻemi i ke kuni, ka pehu ʻana, ka ʻeha, a me ka ʻulaʻula. Hiki ke hāʻawi pū ʻia nā lāʻau anti-inflammatory a me chemotherapy e mālama i nā mākua me ka iwi LCH.

• ʻO Retinoids, e like me isotretinoin, nā lāʻau e pili ana i ka wikamina A i hiki ke hoʻolohi i ka ulu ʻana o nā pūnaewele LCH i loko o ka ʻili. Lawe ʻia nā retinoids e ka waha.

Ke kuʻi ʻōnaʻi ʻōnaeho

ʻO ka transplant cell kahi hana e hāʻawi ai i ka chemotherapy a me ka hoʻololi ʻana i nā hunaola koko e luku ʻia e ka lāʻau LCH. Lawe ʻia nā hunaola ʻōpala (ʻaʻaʻa ʻole o ke koko) mai ke koko a i ʻole ka iwi o ka mea maʻi a i ʻole he mea hāʻawi a hoʻopaʻa ʻia a mālama ʻia. Ma hope o ka pau ʻana o ka chemotherapy, hoʻoheheʻe ʻia nā pūnaewele i mālama ʻia a hoʻihoʻi ʻia i ka mea maʻi ma o ka hoʻopili ʻana. E ulu kēia mau hunaola i (a hoʻihoʻi) i nā huna koko o ke kino.

Nānā

Ke nānā pono nei i ka nānā ʻana i ke kūlana o ka mea maʻi me ka ʻole o ka hāʻawi ʻana i nā lāʻau a hiki i ka wā e ʻike ʻia ai nā hōʻailona a i ʻole nā ​​loli.

Ke hoʻāʻo ʻia nei nā ʻano lapaʻau hou i nā hoʻokolohua hoʻokolohua.

Loaʻa nā ʻike e pili ana i nā hoʻokolohua hoʻokolohua mai ka pūnaewele NCI.

ʻO ka mālama ʻana no ka Langerhans cell histiocytosis ka mea e hopena ai i nā hopena ʻaoʻao.

No ka ʻike e pili ana i nā hopena i hoʻomaka i ka wā o ka mālama ʻana no ka maʻi ʻaʻai, ʻike i kā mākou ʻaoʻao ʻaoʻao ʻaoʻao.

ʻO nā hopena ʻē aʻe mai ka mālama ʻana i ka maʻi ʻaʻai e hoʻomaka ma hope o ka mālama ʻana a hoʻomau no nā mahina a i ʻole mau makahiki e kapa ʻia nā hopena hopena. Hiki i nā hopena hope loa o ka mālama ʻana i ka maʻi ʻaʻai ke hoʻopili i kēia mau mea:

• Hoʻonui lohi a ulu.

• Lohe hoʻolohe.

• Nā pilikia iwi, niho, ake, a me nā akemāmā.

• Nā loli i ke ʻano, ka manaʻo, ke aʻo ʻana, ka noʻonoʻo ʻana, a me ka hoʻomanaʻo ʻana paha.

• ʻO nā maʻi ʻelua, e like me leukemia, retinoblastoma, Ewing sarcoma, lolo a me ka maʻi ʻaʻai ate.

E mālama a kāohi ʻia paha kekahi mau hopena hopena. He mea nui e kamaʻilio pū me nā kauka o kāu keiki e pili ana i nā hopena o ka mālama ʻana i ka maʻi ʻaʻai i kāu keiki. (E ʻike i ka hōʻuluʻulu e pili ana i nā hopena hope o ka mālama ʻana no ka maʻi ʻaʻai kamaliʻi no ka ʻike hou aku.)

Nui nā mea maʻi me ka multisystem LCH i hopena hopena ma muli o ka mālama ʻana a i ʻole ka maʻi ponoʻī. Loaʻa pinepine i kēia mau maʻi i nā pilikia olakino lōʻihi e hoʻopili i ko lākou ola.

Makemake paha nā mea maʻi e noʻonoʻo e pili ana i ka hoʻokolokolo ʻana.

No kekahi mau mea maʻi, ke komo nei i ka hoʻokolohua hoʻokolohua ke koho lapaʻau maikaʻi loa paha. ʻO nā hoʻokolohua hoʻokolohua kahi ʻāpana o ka noiʻi ʻana o ka maʻi ʻaʻai. Hana ʻia nā hoʻokolohua hoʻokolohua e ʻike inā palekana a maikaʻi a maikaʻi paha nā hoʻomaʻamaʻa maʻi ʻaʻai ma mua o ka maʻamau maʻamau.

ʻO ka hapa nui o nā hana maʻamau no ka maʻi ʻaʻai i kau ʻia ma nā hoʻokolohua hoʻokolohua mua. ʻO nā mea maʻi i komo i ka hoʻokolokolo hoʻokolohua hiki ke loaʻa i ka lāʻau maʻamau a i ʻole i waena o nā mea mua i loaʻa kahi lāʻau hou.

ʻO nā mea maʻi i komo i nā hoʻokolohua hoʻokolohua kōkua pū kekahi i ka hoʻomaikaʻi ʻana i ke ʻano o ka mālama ʻana i ka maʻi ʻaʻai i ka wā e hiki mai ana. ʻOiai ʻaʻole nā ​​alakaʻi hoʻokolohua e alakaʻi i nā hoʻoponopono hou maikaʻi, pane pinepine lākou i nā nīnau nui a kōkua i ka neʻe i mua o ka noiʻi.

Hiki i nā mea maʻi ke komo i nā hoʻokolohua hoʻokolohua ma mua, i ka wā, a ma hope paha o ka hoʻomaka ʻana i kā lākou lapaʻau.

Hoʻopili wale kekahi mau hoʻokolohua hoʻokolohua i nā mea maʻi i loaʻa ʻole ka lapaʻau. ʻO nā hoʻokolohua ʻē aʻe e hōʻoia i nā mālama ʻana no ka poʻe maʻi i loaʻa ʻole ka maikaʻi o ka maʻi ʻaʻai. Aia kekahi mau hoʻokolohua hoʻokolohua e hoʻāʻo i nā ala hou e hōʻoki i ka maʻi ʻaʻai mai ka hoʻi hou ʻana (hoʻi ʻana) a hōʻemi paha i nā hopena o ka mālama ʻana i ka maʻi ʻaʻai.

Ke mālama ʻia nei nā hoʻokolohua hoʻokolohua ma nā wahi he nui o ka ʻāina. Hiki ke ʻike ʻia ka ʻike e pili ana i nā hoʻokolohua hoʻokolohua e kākoʻo ʻia e NCI ma ka pūnaewele ʻimi noiʻi hoʻokolohua a NCI. Hiki ke loaʻa nā hoʻokolohua hoʻokolohua i kākoʻo ʻia e nā hui ʻē aʻe ma ka pūnaewele ClinicalTrials.gov.

Ke kū nei ka lapaʻau ʻana o LCH, hiki mai paha nā leona hou a i ʻole hoʻi e hoʻi mai nā ʻino kahiko.

ʻOi aku ka maikaʻi o nā maʻi he nui me ka LCH me ka lāʻau. Eia nō naʻe, i ka pau ʻana o ka mālama ʻana, hiki ke ʻike ʻia nā liona hou a i ʻole hoʻi e hoʻi nā lehele kahiko. Kapa ʻia kēia he reactivation (recurrence) a hiki i loko o hoʻokahi makahiki ma hope o ka hoʻōki ʻana i ka lāʻau. ʻO nā mea maʻi me ka maʻi multisystem e loaʻa hou kahi reactivation. ʻO nā pūnaewele maʻamau o ka hoʻoulu hou ʻana he iwi, pepeiao, a ʻili paha. Hiki ke ulu i ka insipidus diabetes. ʻO nā pūnaewele liʻiliʻi o ka hoʻoulu hou ʻana e komo pū me nā lymph node, ka iwi iwi, ka iwi, ka ate, a me ka akemāmā. Hiki i kekahi mau mea maʻi ke ʻoi aku ma mua o hoʻokahi reactivation i loko o kekahi mau makahiki.

Pono paha nā hoʻokolohua ukali.

Ma muli o ka makaʻu o ka hoʻoulu hou ʻana, pono e nānā ʻia nā mea maʻi LCH no nā makahiki he nui. ʻO kekahi o nā hoʻokolohua i hana ʻia e kuhikuhi iā LCH e hana hou ʻia paha. ʻO kēia e ʻike ai i ke ʻano o ka maikaʻi o ka mālama ʻana a inā he mau liona hou. Pili pū paha kēia mau hoʻāʻo:

• ʻImi kino.

• Hōʻike Neurological.

• ʻImi ultrasound.

• MRI.

• CT scan.

• Scanner PET.

ʻO nā hoʻokolohua ʻē aʻe e pono ai e hoʻopili ʻia:

• ʻO ka hoʻāʻo ʻana o ka lāʻau Brain i hoʻāla i ka pane (BAER) hōʻike: kahi hoʻāʻo e ana i ka pane o ka lolo i ke kaomi ʻana i nā kani a i ʻole kekahi mau kani.

• Hōʻike hana pulmonary (PFT): kahi hoʻāʻo e ʻike i ke ʻano o ka hana o nā māmā. Ana ʻia ka nui o ka ea e hiki ai i nā māmā ke hoʻopaʻa a pehea ka wikiwiki e neʻe ai ka ea i loko a i waho o nā māmā. Ana ʻia hoʻi ka nui o ka oxygen e hoʻohana ʻia ai a pehea e hāʻawi ʻia ai ke kalapona kalapona i ka hanu ʻana. Ua kapa ʻia kēia he hoʻokolohua hana māmā.

• ʻO ke kukui x: kahi x-ray o nā mea a me nā iwi i loko o ka umauma. ʻO ka x-ray kahi ʻano kukuna ikehu e hiki ke hele ma waena o ke kino a ma ke kiʻi ʻoniʻoni, e hana ana i kiʻi o nā wahi i loko o ke kino.

Hiki i nā hopena o kēia mau hoʻāʻo ke hōʻike inā ua loli kou ʻano a i ʻole inā ua hoʻi hou ke kanesa. Kapa ʻia kēia mau hoʻāʻo i kekahi manawa nā hoʻokolohua ukali a i ʻole nā ​​loiloi. Hoʻoholo ʻia nā hoʻoholo e pili ana inā e hoʻomau, hoʻololi, a kū paha i ka lāʻau ʻana ma nā hopena o kēia mau hoʻāʻo.

Ka mālama ʻana i ka LCH Low-Risk i nā Keiki

Ma keia Pauku

• Nā Liona ʻili

• ʻO nā leona i nā iwi a i ʻole nā ​​ʻohana haahaa haʻahaʻa ʻē aʻe

• Nā ʻionslelo CNS

No ka ʻike e pili ana i nā lāʻau i helu ʻia ma lalo, e ʻike i ka ʻaoʻao Overtion Option Overview.

Nā Liona ʻili

Hoʻomaʻamaʻa ʻia i nā ʻili o ka ʻili ʻo Langerhans cell histiocytosis (LCH):

• Nānā.

Ke hiki mai nā huhū nui, ʻeha, ulceration, a i ʻole ke kahe ʻana o ke koko, hiki i ka mālama ʻana ke hoʻopili me kēia:

• Pākuʻi steroid.

• Hāʻawi ʻia ʻo Chemotherapy e ka waha a i ʻole ka ʻaʻa.

• Pili ʻo Chemotherapy i ka ʻili.

• ʻO Photodynamic therapy me psoralen a me ultraviolet A (PUVA) therapy.

• ʻO UVB radiation radiation.

ʻO nā leona i nā iwi a i ʻole nā ​​ʻohana haahaa haʻahaʻa ʻē aʻe

ʻO ka hoʻomaʻamaʻa ʻana i nā liona iwi LCH o ka wā kamaliʻi i mua, nā ʻaoʻao, a i hope paha o ka iwi poʻo, a i ʻole i kekahi iwi ʻē aʻe e pili pū paha.

• ʻOki kino (curettage) me ka ʻole a me ka ʻole o ka steroid therapy.

• ʻO ka radiation radiation dosis haʻahaʻa no nā leona e hoʻopili i nā mea kokoke.

Hana ʻia nā liona LCH o ke keiki hou i ʻike ʻia i loko o nā iwi a puni nā pepeiao a i ʻole nā ​​maka e hana ʻia e hoʻohaʻahaʻa i ka makaʻu o ka maʻi diabetes insipidus a me nā pilikia lōʻihi lōʻihi. Hiki ke hoʻopili ʻia i ka mālama ʻana:

• ʻO Chemotherapy a me steroid therapy.

• ʻOki kino (curettage).

ʻO ka mālama ʻana i nā liona LCH o ke kuamoʻo a i ʻole ka iwi ʻūhā e pili pū me:

• Nānā.

• Pāpaʻi pāhawewe haʻahaʻa.

• ʻO Chemotherapy, no nā liona i hohola aʻe mai ka iwi kuamoo i nāʻiʻo kokoke.

• ʻO ke kaha e hoʻoikaika ai i ka iwi nāwaliwali ma ka hoʻopaʻa ʻana a i ʻole ka hoʻopili ʻana ʻana i nā iwi.

Hoʻomaʻamaʻa ʻia i nā ʻāpana iwi ʻelua a ʻoi paha:

• ʻO Chemotherapy a me steroid therapy.

Hoʻohui ʻia ʻelua a ʻoi aku paha nā ʻeha iwi i hui pū ʻia me nā ʻili o ka ʻili, nā liona node lymph, a i ʻole insipidus diabetes hiki ke hoʻopili ʻia:

• Chemotherapy me a i ʻole me ka ʻole steroid therapy.

• Hoʻoikaika Bisphosphonate.

Nā ʻionslelo CNS

Hoʻomaʻamaʻa ʻia nā liona o ke ʻōnaehana LCH kikowaena liʻiliʻi (CNS):

• Chemotherapy me a i ʻole me ka ʻole steroid therapy.

Hoʻomaopopo ʻia ka mālama ʻana i ka LCH CNS neurodegenerative Syndrome:

• Hoʻopaʻa kaulike ʻia me nā mea hōʻoki BRAF (vemurafenib a i ʻole dabrafenib).

• ʻO Chemotherapy.

• Hoʻopaʻa kaulike ʻia me kahi antibody monoclonal (rituximab).

• Hoʻomaʻo Retinoid.

• Immunotherapy (IVIG) me ka ʻole o ka chemotherapy.

E hoʻohana i kā mākou ʻimi hoʻokolohua hoʻokolohua e ʻike i nā hoʻokolohua hoʻokolohua maʻi maʻi i kākoʻo ʻia e NCI e ʻae nei i nā mea maʻi. Hiki iā ʻoe ke ʻimi i nā hoʻāʻo e pili ana i ke ʻano o ka maʻi ʻaʻai, ka makahiki o ka mea hoʻomanawanui, a kahi e hana ʻia ai nā hoʻokolokolo. Loaʻa nā ʻike laulā e pili ana i nā hoʻokolohua hoʻokolohua.

Lapaʻau o kiʻekiʻe-kamepiula LCH ma Children

No ka ʻike e pili ana i nā lāʻau i helu ʻia ma lalo, e ʻike i ka ʻaoʻao Overtion Option Overview.

Hoʻomaʻamaʻa ʻia nā liona maʻi LCH multisystem maʻi hou i ʻike ʻia i ke kōkō, ke ake, a i ʻole ka iwi iwi a me kahi hui ʻē aʻe a i ʻole pūnaewele paha e komo pū:

• ʻO Chemotherapy a me steroid therapy. Hiki ke hāʻawi ʻia nā ʻoi aku ʻoi aku ma mua o hoʻokahi lāʻau chemotherapy a me steroid therapy i nā mea maʻi i pane ʻole nā ​​tumors i ka chemotherapy mua.

• Hoʻopaʻa kaulike (vemurafenib).

• ʻO ke kuʻi akepaʻa no nā mea maʻi me ka hōʻino nui o ke ake.

• ʻO kahi hoʻokolohua hoʻokolohua e hoʻonohonoho pono i ka mālama ʻana o ka mea maʻi ma muli o nā hiʻohiʻona o ka maʻi ʻaʻai a pehea e pane ai i ka lāʻau.

• ʻO kahi hoʻokolohua hoʻokolohua o ka chemotherapy a me ka steroid therapy.

Lapaʻau o Recurrent, Refractory, a me Progressive Childhood LCH i nā Keiki

No ka ʻike e pili ana i nā lāʻau i helu ʻia ma lalo, e ʻike i ka ʻaoʻao Overtion Option Overview.

ʻO ka LCH hou he kanesa ʻaʻole hiki ke ʻike ʻia no kekahi manawa ma hope o ka lapaʻau ʻana a laila hoʻi mai. ʻO Refractory LCH kahi maʻi ʻaʻai e maikaʻi ʻole me ka lāʻau. ʻO ka LCH holomua ka maʻi ʻaʻai e hoʻomau e ulu i ka wā o ka lāʻau.

Hiki ke hoʻopili ʻia i ka hoʻomaʻamaʻa ʻana i ka LCH kūmau hoʻi, refactory, a i ʻole holomua holomua.

• Chemotherapy me a i ʻole me ka ʻole steroid therapy.

• Hoʻoikaika Bisphosphonate.

Hoʻomaopopo ʻia ka hoʻomaʻamaʻa ʻana i ka Lisy multisystem kiʻekiʻe ʻoi loa o ka hopena:

• ʻO ka chemotherapy kiʻekiʻe loa.

• Hoʻopaʻa kaulike (vemurafenib).

• Ke kuʻi ʻōnaʻi ʻōnaeho.

Ke aʻo ʻia nei no nā LCH o ka wā kamaliʻi pinepine, refactory, a i ʻole holomua.

• ʻO kahi hoʻokolohua hoʻokolohua e hoʻonohonoho pono i ka mālama ʻana o ka mea maʻi ma muli o nā hiʻohiʻona o ka maʻi ʻaʻai a pehea e pane ai i ka lāʻau.

• ʻO kahi hoʻokolohua hoʻokolohua e nānā i kahi laʻana o ka tumo o ka mea maʻi no kekahi ʻano loli. ʻO ke ʻano o ka hoʻomālama ʻia e hāʻawi ʻia i ka mea maʻi e pili ana i ka ʻano o ka loli.

Lapaʻau o LCH i nā mākua

Ma keia Pauku

• Hoʻomaʻo i ka LCH o ka Lung i nā mākua

• Ka mālama ʻana i ka LCH o ka iwi i nā mākua

• Ka mālama ʻana i ka LCH o ka ʻili i nā mākua

• Lapaʻau o Single-System a Multisystem LCH i nā mākua

No ka ʻike e pili ana i nā lāʻau i helu ʻia ma lalo, e ʻike i ka ʻaoʻao Overtion Option Overview

ʻO ka Langerhans cell histiocytosis (LCH) i nā mākua e like me ka LCH i nā keiki a hiki ke hana i nā mea like a me nā ʻōnaehana e like me nā kamaliʻi. Hoʻopili kēia i ka endocrine a me nā ʻōnaehana waena, ka ate, ka palu, ka iwi iwi, a me nā ʻōpū o ka gastrointestinal. I nā mākua, ʻike pinepine ʻia ʻo LCH i ka māmā me he maʻi ʻōnaehana hoʻokahi. LCH i ka māmā pinepine pinepine i nā ʻōpio ʻōpio puhipaka. Loaʻa pinepine ka LCH mākua i ka iwi a me ka ʻili.

E like me nā keiki, pili nā hōʻailona a me nā hōʻailona o ka LCH ma kahi e loaʻa ai i loko o ke kino. E ʻike i ka ʻikepili ʻike laulā no nā hōʻailona a me nā hōʻailona o LCH.

ʻO nā hoʻāʻo e nānā ana i nā ʻōnaehana a me nā ʻōnaehana o ke kino kahi e hana ʻia ai ka LCH e hoʻohana ʻia e ʻike (loaʻa) a hōʻike iā LCH E ʻike i ka ʻikepili ʻikepili ākea no nā hoʻokolohua a me nā kaʻina hana i hoʻohana ʻia e ʻike pono i ka LCH.

I ka poʻe mākua, ʻaʻohe nui o ka ʻike e pili ana i ke ʻano o ka hoʻomaʻamaʻa maikaʻi loa. I kekahi manawa, hiki mai ka ʻike mai nā hōʻike o ke kuhi, ka mālama ʻana, a me ka ukali ʻana o hoʻokahi mākua a i ʻole kahi hui liʻiliʻi o nā mākua i hāʻawi ʻia i ke ʻano o ka lapaʻau.

Hoʻomaʻo i ka LCH o ka Lung i nā mākua

Hoʻomaʻamaʻa ʻia ka LCH o ka māmā i nā mākua:

• Ke haʻalele nei i ka puhi puaʻa no nā mea maʻi āpau e puhipaka. Eʻoi aku ka maikaʻi o ka hōʻino ʻana o ka maʻi i ka manawa ma ka poʻe maʻi ʻaʻole i haʻalele i ka puhipaka. I nā mea maʻi i haʻalele i ka puhipaka, maikaʻi paha ka pohō o ka nāha a ʻoi aku paha ka maikaʻi o ka hele ʻana o ka manawa.

• ʻO Chemotherapy.

• Ke lawe aku i nā maʻi no nā mea maʻi me ka hōʻino nui o ka hanu.

I kekahi manawa e hele aku ʻo LCH o ka māmā a ʻaʻole e ʻoi aku ka maikaʻi inā ʻaʻole mālama ʻia.

Ka mālama ʻana i ka LCH o ka iwi i nā mākua

ʻO ka mālama ʻana no ka LCH e pili ana i ka iwi wale nō i nā mākua ke hoʻopili pū ʻia.

• ʻO ke kaha me me ka ʻole o ka steroid therapy.

• Chemotherapy me a i ʻole me ka hoʻowalewale ʻana o ka radiation radiation.

• Lapaʻau hoʻomākala.

• Hoʻoikaika Bisphosphonate, no ka ʻehaʻeha o ka iwi koʻikoʻi.

• Nā lāʻau anti-inflammatory me chemotherapy.

Ka mālama ʻana i ka LCH o ka ʻili i nā mākua

ʻO ka mālama ʻana no ka LCH e hoʻopili wale i ka ʻili i nā mākua.

• ʻOki kino.

• ʻO Steroid a i ʻole nā ​​lāʻau lapaʻau ʻē aʻe i hoʻopili ʻia a i ʻole injected i loko o ka ʻili.

• ʻO Photodynamic therapy me psoralen a me ultraviolet A (PUVA) pāhawewe.

• ʻO UVB radiation radiation.

• ʻO Chemotherapy a i ʻole immunotherapy i hāʻawi ʻia e ka waha, e like me methotrexate, thalidomide, hydroxyurea, a i ʻole interferon.

• Hiki ke hoʻohana ʻia ka Retinoid therapy inā ʻaʻole maikaʻi ʻo nā ʻili o ka ʻili me nā lāʻau ʻē aʻe.

ʻO ka mālama ʻana no ka LCH e pili ana i ka ʻili a me nā ʻōnaehana o ke kino i nā mākua.

• ʻO Chemotherapy.

Lapaʻau o Single-System a Multisystem LCH i nā mākua

ʻO ka mālama ʻana i ka ʻōnaehana hoʻokahi a me ka maʻi multisystem i nā mākua i pili ʻole i ka hū, ka iwi, a me ka ʻili e komo pū paha:

• ʻO Chemotherapy.

• Hoʻolālā pahuhopu (imatinib, a vemurafenib).

No ka ʻike hou aku e pili ana i nā hoʻāʻo LCH no nā mākua, e ʻike i ka pūnaewele Histiocyte SocietyExit Disclaimer.

E hoʻohana i kā mākou ʻimi hoʻokolohua hoʻokolohua e ʻike i nā hoʻokolohua hoʻokolohua maʻi maʻi i kākoʻo ʻia e NCI e ʻae nei i nā mea maʻi. Hiki iā ʻoe ke ʻimi i nā hoʻāʻo e pili ana i ke ʻano o ka maʻi ʻaʻai, ka makahiki o ka mea hoʻomanawanui, a kahi e hana ʻia ai nā hoʻokolokolo. Loaʻa nā ʻike laulā e pili ana i nā hoʻokolohua hoʻokolohua.

E aʻo hou aʻe e pili ana iā Langerhans Cell Histiocytosis

No ka ʻike hou aku mai ka National Cancer Institute e pili ana i ka mālama ʻana iā Langerhans cell histiocytosis, e ʻike i kēia:

• Computers Tomography (CT) Scans a me ka maʻi ʻaʻai

• Photodynamic Therapy no ka maʻi ʻaʻai

• Immunotherapy e mālama i ka maʻi ʻaʻai

• ʻO nā Therapy Cancer i kau ʻia

• ʻO nā Transplant Cell-Blood Stem

No ka ʻike hou aku i ka maʻi ʻaʻai kamaliʻi a me nā kumuwaiwai maʻi ʻaʻai ʻē aʻe, e ʻike i ka mea aʻe:

• E pili ana i ka maʻi ʻaʻai

• Nā maʻi ʻōpio

• Huli ʻo Cure no ka hōʻole ʻana i nā maʻi ʻaʻai keiki

• Nā hopena hope loa o ka mālama ʻana no ka maʻi ʻaʻai kamaliʻi

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