Langerhans histiocytosis pūtau: Take me te rongoā

Langerhans pūtau - ma pūtau toto e he wahi o te pūnaha ārai mate, me te i roto i ngā āhuatanga noa whai wāhi nui i roto i te tiaki i te tinana ki wheori, huakita me ētahi atu microorganisms kino. E kitea ratou i roto i te kiri, taikiri, kōateate, wheua wheua me pūkahukahu.

He aha te mea Langerhans pūtau histiocytosis?

I roto i tenei mate, e mohiotia ana ko histiocytosis X mua, tīmata ngā pūtau Langerhan ki proliferate i roto i te tauine pathological. Engari o tiaki te tinana, pā nohanga nui o enei pūtau te tapatahi o te kopa, me te ara whakangaro ratou. mamae te nuinga maha i nga wheua, pūkahukahu me te ate. Ahakoa tenei tini pūtau he nui i roto i te noa ki te mate pukupuku, e kore te nuinga o ngā kairangahau e whakapono puka histiocytosis X o te mate pukupuku. Ko te āhuatanga matua differentiating i roto i tenei horopaki, whakaae te hanganga noa o ngā pūtau pukuriri, me te anake te raruraru ko to ratou tupu vitiviti. I tēnei wā, Langerhans pūtau histiocytosis (i roto i ngā tamariki, kia tuhia te reira, he reira atu noa atu i roto i ngā pakeke) Kei te mohio mate o te pūnaha ārai mate, ai pūtau ārai tini hohoro rawa, ka meinga mumura me te kino ki te kikokiko e karapoti ana.

taea ki te whakawhanake i roto i tetahi wahi o te tinana, me te i roto i tetahi okana tenei mate ko te. Ka taea e ara puta te reira i roto i te maha o ngā wāhi rerekē te wā kotahi. I roto i te nuinga o te wā, Heoi, ka pā te māuiuitanga pūnaha maha o te tinana anake i roto i ngā tamariki raro i te rua tau o te tau. E kitea pāpaka mokemoke i roto i rua tamariki me ngā pakeke.

take

Ko reira unknown tonu he aha i reira ko te māuiuitanga taua. Heoi, kua arotahi aivanaa i runga i te whakaū o te whakapae e whai ake nei: te nuinga pea he ahua o te mea noa i roto i te taiao, pea he mate viral e homai whanaketanga ki te whanaketanga o disorders meinga tauhohenga rawa kaha i te pūnaha ārai mate. Heoi, ko te pūtake tika o te mate i te kau i raro ki te tāutu. Ko ngā kotahi kitea mohiotia: tata tūroro katoa ki te mate paetata i roto i te pūkahukahu e kaha mua ranei kaipaipa hikareti. Otiia, ara, ki te te tohatoha papatipu o te kai paipa i roto i te hapori, ko te Langerhans rawa onge histiocytosis. Ko te take ngā te whakamahi o hikareti i roto i te rārangi o ngā āhuatanga mōrearea rite kahore, no te mea te mea maha reira he mate, me te e kore e pā te pūkahukahu. Ko te whakapae tino pea ko e te pānga kino o ifi tini mohio o ngā pūtau Langerhans i roto i te tūroro ki te predisposition takoto ira onge ki reira. E ai ki tetahi atu ariā, te take pakiaka o te mate e takoto i roto i te tumu disorders pūnaha ārai mate.

horapa

Ko te onge whakaaro he mate, me te puta i roto i pā ana ki kotahi take mō 250 mano tamariki me kotahi take ia miriona pakeke. Ahakoa kia puta te tohu i tetahi tau, ai tino maha pāngia i roto i ngā tamariki me ngā taiohi, me 70% o te wā puta i roto i te tāutu histiocytosis tau ki 17 tau. I roto i te rōpū pakeke whakaotinga o tūroro, ara tatauranga i roto i te tau i tetahi ki te e toru nga tau.

ahua o te mate

Langerhans histiocytosis pūtau i roto i ngā tamariki me ngā pakeke whai wāhi 3 puka whakaaro i mua pathologies motuhake. Te reira i granuloma eosinophilic, mate Henda - Shyullera - mate Karaitiana me Letterer - Siwa.

tohu

Ka taea e te mate faaite mai ano e te āhuatanga e whai ake nei:

• mamae tamau me te tetere i roto i nga wheua, rawa i roto i tou ringa waewae ranei;
• he kapiti i roto i te wheua, rawa, ki te kore te mea mua e te whara whaiaro ranei mua e te marama, pupuhi iti;
• ngā āputa nui i waenganui i niho i roto i te wā i reira kua horapa te Langerhans histiocytosis pūtau i roto i ngā tamariki ki te wheua o te kauae;
• mate taringa rere taringa ranei, ki te mea kua pāngia te mate nga wheua o te angaanga tata te taringa;
• ponana kiri preferentially paetata i runga i nga papa i te tumuaki ranei;
• lymphadenopathy;
• whakarahi ate, i roto i ētahi wā kia haere tahi e tohu o okana pōrearea (kōwhaitanga o te kiri, me kanohi (jaundice) ranei whakaeminga pathological o wai i roto i te puku - ascites);
• exophthalmos - te displacement o te 'Afio mua histiocytosis tika ki te localization o nga kanohi;
• hāhā o te manawa, me te mare, pūkahukahu ki he pāpaka;
• tupu puhoi me urination nui e tika ana ki te tāne i hormonal.

tohu iti noa o te mate ko:

• kirika;
• mate taimaha;
• kārangirangi;
• te ngoikore ki te pupuri i te taimaha me te pupuri i te tinana i roto i te āhua.

tātaringa

Mai (kore he faaiteraa Whakaahua o tenei mate he tirohanga tino) Langerhans histiocytosis pūtau he tino onge, me te whakahuatia e tohu e te hunga ngāwari pohehe mo nga tohu o maha atu mate, maha he matatini tātaritanga, me te e wa. Ko te aha pea ka he tākuta ui koe ngā pātai e pā ana ki ētahi atu mate ki atu tōtika rerekëtanga ki te histiocytosis tikanga.

Te tūhuratanga tātaritanga tuatahi ko te whakamātautau tinana noa, i roto i nei aromatawai i te tākuta te hauora whānui, me te utu aro ngā ki aua wāhi i reira puta nga tohu o te mate. Ki te ite te manawanui mamae i roto i te wheua amuamu o huamo i te wheua ranei, kia whakahau i te tākuta he paerewa X-hihi, me te wheua scintigraphy. X-hihi whakakitea whati wahi i huaina pāpaka lytic, me scintigraphy ka whakatau i te rohe o te whara te wahi takitahi tamata te wheua ki ora.

Tikanga computed Tomography (CT) e whakamahia ana i roto i te ako o te hoki me te papatoiake i roto i te wā i reira hae te rata e ko te reira i reira kia kia whitiki o huringa haamou. A, no te e Me tohu tohu o ate pōrearea, whakamātautau toto. Ki te mea i reira he tohu o te kino pūkahukahu, i roto i te wā ranei te wahi i te mātanga i runga i te pūtake o te whakamātautau hauora tuatahi hae e kia whakaatu Langerhans pūtau histiocytosis reira i roto i te pūkahukahu, whakahau X-hihi computed ranei tomography o te pouaka. CT scan o te matenga i mahi ki te aromatawai i te āhua o te roro. Ka taea hoki te hangarau hou hi'ohi'oi, PET (positron tukunga tomography) kia whai hua.

Ki te whakapae koe he histiocytosis Langerhans pūtau i roto i ngā tamariki (kite whakaahua. I te timatanga o te tuhinga) kia tika unuhanga, rite he anake te tikanga ki te whakaū i te tātaritanga pōhēhē tenei. I roto i tenei tikanga, te tango i te tākuta he tauira iti o te kiko wheua ranei ki te kia ako i roto i te taiwhanga. I roto i te nuinga o te wā, tangohia te tauira o te rauemi koiora te i ngā wāhi i reira paetata tika Langerhans histiocytosis pūtau: wheua, kiri me taikiri. Ka taea e whakamātautau toto te āwhina whakatau pānga ahakoa te te hinu wheua wheua.

roanga

Ko reira uaua ki te matapae pēhea te hohoro te mate me pehea e faaite mai i te reira iho. I roto i ētahi wā, te māuiuitanga riro i tahaki, a e kore e rapua e te wawaotanga hauora. Tenei puta ka paetata te ofatiraa i roto i te kotahi rohe (i roto i te wheua kiri ranei), ka mutu te kai hikareti me te kino pūkahukahu ki te pau hikareti ranei. Heoi, i te reira e tupu ano e he mate te mate; Ka taea te tohu mate i roto i aua wā, ina e whawhai whakaeminga o ngā pūtau tomuri proliferating i te wa ano he maha o whēkau rerekē wahi tinana ranei. Ko te āhua o te hauora o te hikareti tīmata ki te whakapai ake anake, no te momi.

ārai

Mai te take o maumaui tonu te mea ngaro, pera tae noa kahore he mehua ārai whai hua. Heoi, i homai e pāngia nga pūkahukahu e anake i te pā ki te paowa hikareti, hoatu ake he ma'unimā taea te kite i rite te histiocytosis ārai paetata i roto i te pūkahukahu.

maimoatanga

Ki te mea he tātaritanga whakapumautia o "histiocytosis o ngā pūtau Langerhans" te manawanui, ka whakawhirinaki te maimoatanga i runga i te pakeke me te whānuitanga o te pakaru.

Ki te kitea pīari te i roto i te kotahi anake pūnaha o te tinana, ka whakahau i te tākuta corticosteroids, i roto i ngā "Prednisolone". I tua atu, kia whai koe ki te mamae i te akoranga o te hahau. I tua atu, i te wāhi kua pā o wheua nekehia atu e tīwani - haro. Ki te hāngai ngā pūtau pathologically accrescent i roto i tetahi rohe o te kiri, e taea te ora te mate na roto i te uhi i te pua nani rohe hauota pā - kaihoko hahau. Kei te whakamahia hoki te reira "Methotrexate". faaterehia iraruke te i roto i te wā i reira te mate wheua (hei tauira, i roto i te waewae o runga ranei i roto i te tuaiwi) arahina ki te vaivai o te kongakonga kōhiwi katoa. pokanga Musculo-kirihou i runga i te tuaiwi - hononga - e tūtohu ka wheua tapepe i roto i te rohe waha.

Whānui (nahanaha) Langerhans histiocytosis pūtau - he huru nui atu taumaha. Hoki hoki e taea te tono maimoatanga o hahau, Heoi, kei te tino iti tona whai huatanga whakaritea ki te therapy ki lesion kotahi. Ahakoa te korenga i ngā tohunga te whai huatanga o maha raau taero, kua kore ano i kitea maimoatanga tino pai hauora. Otiia, i roto i te tikanga ki te aroturuki i te whanaketanga o te mate kia whakahau koutou tākuta tetahi o nga tikanga e whai ake nei:

• "Vinblastine";
• "Etoposide";
• "Mercaptopurine";
• "Cladribine";
• "Cytarabine";
• "Methotrexate".

I roto i ētahi wā, tūtohu kaiwhakaako ate transplantation, wheua wheua, pūkahukahu ranei, engari i roto i kairangahau whakaaro i runga i te tino pai transplantation okana i roto i te tūroro ki te histiocytosis kore kua ano i tae.

A, no te ki te kite i te tākuta?

Kia tino ki te whakapā atu ki tō tākuta tamariki tiaki tuatahi ki te whakapae koe Langerhans histiocytosis pūtau i roto i ngā tamariki. tohu Ocular, mamae huamo i roto i te wheua ranei, rashes kiri o ko nga tohu o tenei mate onge. Ki te kia kōrero tetahi o enei tohu māuiuitanga ranei i roto i ngā pakeke i te rata.