Mate Erdheim - he aha te māuiuitanga?

Ahakoa te meka e te whakawhanake rongoā tere, e kore i maha mate i ako ra ano te mutunga. i whakaahuatia etahi o ratou e kore e pera i mua. Na reira, e kore raraunga i runga i te tahi mau pathologies i tenei wā, ko te nui. He tauira ko te mate Erdheim. he tuwhera mamae anake i roto i te rau tau XX. Ko ia he mate onge, na reira he kore taea ki te puta ki te ako hohonu o tenei mate. Ko te take, me te pathogenesis o disorders unknown ki te mutunga. Otiia, i reira e rave rahi ariā o te mate. He mātātoa te ako katoa o ratou. Ki te rā, 500 anake ngā take i mohio o te mate ao. Mai whakaaro māuiuitanga te onge, e kore te mea tonu ngāwari ki te tāutu i.

He aha te mea te mate Erdheim?

Hoki te wā tuatahi i runga i tenei mate, ka mohiotia te reira i roto i te tau 1930. I whakatuwheratia te reira i pūtaiao William Chester me tona kaiwhakaako - Hakopa Erdheim. mahi tahi ratou ki runga ki te ako o tenei mate. Na te maha i huaina te mate te syndrome Erdheim Chester. E ai ki nga raraunga, i whakahaere ki te kohikohi i runga i te tau, he nui ake noa i roto i nga tangata i te māuiuitanga. I roto i te nuinga o te wā, te mate tuatahi faaite ake i roto i te tau o 50 tau. Otiia, i reira e rave rahi take o atu tahumaero i roto i ngā tamariki. rerekē Tohu o mate. Nuinga o ngā wā, i te mau faaiteraa haumanu e whai ake nei puta: mate wheua, disorders neurological me te mate huka insipidus. Mate (syndrome) Erdheim-Chester āhuatanga e kookuhutanga o ngā kopa tinana histiocytes nelangergansovymi. no enei pūtau ki te wāhanga o te pūnaha ārai mate. Tikanga, E ratou he mahi tiaki. Ko reira he histiocytes uri roa ho'i i, rite ki te hua pā ratou whekau ngā i roto i tenei māuiuitanga.

mate Erdheim: tohu mate

kia rerekē te pikitia haumanu o tenei syndrome onge. Tei te reira i runga i nei i patua whēkau e histiocytes. I roto i te tata wā katoa mate Erdheim whakakitea e huringa i roto i te kōhiwi, pūnaha io, me te kiri. I roto i nga tohu o tenei mate e taea te taratahi maumaui e whai ake:

• kiriuhi wheua Osteosclerosis. Ko reira i roto i te nuinga o te tūroro tenei faaiteraa. Otiia, i roto i te nuinga o te wā, tenei tohu e kore whakakorikoria nga tūroro. he wahi anake o te iwi e mate ana i tenei mate, amuamu o mamae i roto i te pae o te lesion.
• Exophthalmos. Tenei tohu mate whakawhanake rite ki te hua o te hinga o te wāhi kei muri i te 'Afio. Hoki tini o histiocytes taea meinga kōpeketanga o te karere whatu me te uaua. Na reira, i roto i te tahi mau tūroro i reira he he tohumate rite diplopia. korerotia te hekenga i roto i te acuity ataata ētahi tūroro.
• kookuhutanga Histiocytic o nga whekau endocrine. Faaiteraa Ko te whanaketanga o te mate huka insipidus (matewai, polyuria), whakararuraru pūkoro.
• Hurihiko me uteronefroz. Ēnei tohu whakawhanake rite ki te hua o te kōpeketanga o kiko histiocytic o nga whatukuhu, me ureters.
• Ko te hinganga o te pūnaha Cardiovascular me pūkahukahu.
• Xanthelasma (ngako) i runga i te kamo me te xanthoma. Ka taea te Kei Neoplasms katoa i runga i te tinana.

Tātaritanga Erdheim syndrome

he uaua parenga mate Erdheim-Chester no o tona takanga onge me te tokomaha nui o mau faaiteraa e taea puta ki ētahi atu pathologies. Nuinga o ngā wā, kia whakarongo ki te huinga o ngā tohu pēnei i proptosis me te mamae i roto i te wheua, me te te whanaketanga āta o te matewai me te polyuria. Ka taea te kitea aronga o te kookuhutanga i X-hihi o wahi, Angaanga. Ano, ka whakapae te mate te, whakamana te unuhanga te tinana e e ite kookuhutanga rerekē. I tua atu, i mahi i te reira fundus whakamātautau, CT ko MRI o te wāhi retroperitoneal. Ki te reira te kawea i roto i te mau faaiteraa waihakihaki unuhanga o ngā wāhi pathological (xanthomas). Ka taea e whakau i te tātaritanga me te whakamātautau ira.

Methods mō te rongoā mate Erdheim

Hei timata therapy kia tonu i muri tātaritanga: mate Erdheim. heke maimoatanga o tenei mate i te kau e ki e tuhituhia "interferon". Tei horopeta medicament i runga i te rahi o te aronga o te kookuhutanga. Mai tata tono tarukino "vemurafenib", i te mea he inhibitor o te protooncogene mutant. I tua atu, i runga i te haumanu, therapy symptomatic. Tei mate waitohunga ki runga ki tona tere o tītohu rere. E whakaaro Exophthalmos me te hinga o te pūnaha romahā tohu kino.