Erdheim maʻi - He aha ka pathology?

I loko nō o ka mea i laau lapaau ua ulu koke, he nui maʻi i ole, ua aʻo a hiki i ka hopena. Ua ho'ākāka 'ia ma kekahi o ia i laila, i ka wā kahiko. No laila,ʻikepili ma kekahi pathologies ma keia kahua mea,ʻaʻole lawa. Lakou i mea hoike o ka Erdheim maʻi. Popilikia Ua hamama wale nō i loko o ka XX kenekulia. He mea pohihihi keia maʻi, no laila,ʻaʻole nō he manawa kūpono e hoomau aku i ka hohonu study o keia maʻi. Nā kumu a me pathogenesis o kāna hana i ike ole ia a hiki i ka hopena. Aka hoi, eia kekahi mau theories o ka maʻi. A pau o ia i 'Imi? Anoee. I ka lā, 500 wale ikeia hihia o ka maʻi nā hualoaʻa a. Mai pathology ua noʻonoʻo pohihihi keia, ka mea, he mama i ka hōʻike,ʻaʻole e mau ana.

He aha ka ka maʻi Erdheim?

No ka mea manawa mua ma luna o kēia maʻi, ka mea iʻikeʻia i loko o 1930. Ua Ua weheʻepekema William Honolulu a me kona mau kumu - Iakoba Erdheim. Lakou hana a pau ma luna o ka hoʻopaʻa 'ana i kēia maʻi. No laila, i ka maʻi ua pinepine kapaʻia ka maʻi pale Erdheim Honolulu. E like me nā ikepili, a e hoʻokele e ohi i ma luna o nā makahiki, ka pathology mea oi, he pono ole i loko o nā kānaka. Ma ka hapanui o na hihia, ka maʻi hoike mai ai ia mua ia iho i loko o ka makahiki o 50 makahiki. Aka hoi, eia kekahi mau hihia o ka morbidity i waena o na keiki. Symptoms o ka maʻi ĸokoĸa ana nä pane. Loa pinepine, i ka i kēia lapaʻau hoikeana ana: iwi maʻi, neurological kāna hana a me ka mimikō insipidus. Maʻi (maʻi pale) Erdheim-Honolulu wehewehe aku ma ka infiltration o kekahi mau kino nā'aʻaʻa nelangergansovymi histiocytes. Mau keena e pili ana i na eiiiiiaiou o ka'ōnaehana paleʻea nenoaiu. Maʻamau, ka mea, i ka aiiay kuleana pili i. Akā, he mea unmotivated nā'ōlelo histiocytes, like me ka hopena ka mea, he aha ka mea likeʻole nā loko o keia pathology.

Erdheim maʻi: maʻi symptoms

Ka lapaʻau kiʻi o kēia ke nānā maʻi pale i e okoa. Ua hilinaʻi nui ma luna i i luku ai nā loko ma histiocytes. Ma aneane pau hihia Erdheim maʻi hoikeia aku ai e loli i loko o ka iwi, ko mākou hopohopo nenoaiu a me ka ili. Mawaena o na symptoms o keia maʻi hiki ke kaʻawale kēia 'aʻe' ana:

• Osteosclerosis periosteum. Keia hoike ana ka makana i loko o ka hapa nui o nā mea maʻi. Aka hoi, ma ka loa hoopii, keia symptom 'aʻole i hoopilikia i ka hoʻomanawanuiʻana. Wale i ka hapa o ke kanaka e loaa ana mai i kēia maʻi, complains o kaʻeha i loko o ke kahua o ka lesion.
• Exophthalmos. Kēia maʻi symptoms hoʻomōhala like me ka hopena o ke pio ana o ka wa aia ma ke kua o ka'ōnohi a poʻopoʻo. Nō hoʻi ka hoʻonuiʻana o nā histiocytes hiki i mīkini'opihia o ka optic nā aʻalolo a me ka Muscle. No laila, i loko o kekahi nā mea maʻi he mea he symptom like diplopia. Kekahi e hoomanawanui i hai mai i ka emi ma ka pilikia makapō acuity.
• Histiocytic infiltration o ka endocrine nā loko. Hoike ana o ka ulu ana o mimikō insipidus (makewai, polyuria), metabolic kāna hana.
• Hydro a me uteronefroz. Mau symptoms hoʻomōhala like me ka hopena o ka mīkini'opihia o histiocytic'aʻaʻa o nā puʻupaʻa, a ureters.
• I ka make ana o ka mea'ōnaehana mānowai koko'ōnaehana a me ia maʻiʻaʻai.
• Xanthelasma (momona) ma luna o na lihilihi a me ka xanthoma. Neoplasms hiki ke kai piha a pau ma luna o ke kino.

ʻike, Erdheim maʻi pale

Manaʻo pü 'Erdheim-Honolulu maʻi mea paʻakikī no ka mea, o kona pohihihi keia hanana a me ka nui helu o ka hoike ana i hiki ke hana me nā pathologies. Loa pinepine, e hoʻolohe i kekahi hui malu pu ana o symptoms e like me proptosis a me kaʻeha i loko o ka iwi, e like me ka pono me ka holo ana o ka makewai, a polyuria. Foci o infiltration hiki ke 'akomi i ka X-kukuna o ka lala, iwipoo. Nō hoʻi, i ka wa a ka maʻi ua kēia, ua hana i ka biopsy kino i eʻike piʻi'āno'ē infiltration. Eia hou, e hana fundus hookolokolo, HI a me MRI o ka retroperitoneal hakahaka. Inā he ua lawe mai i ka biopsy cutaneous hoikeana o pathological wahi (xanthomas). Ke hoopaa i kaʻike, a me ka aaiaoe ninaninau.

Nā Papahana no ka hana maikaʻi mai Erdheim

E hoʻomaka Inc e e koke ma hopeʻike,: Erdheim maʻi. ua hoemiia lapaʻau o keia maʻi ma ka manawa i kuhikuhi "interferon". medicament mahele lāʻau hilinaʻi nui ma luna o ka nui o ka foci o infiltration. Mai nei pili lāʻau "vemurafenib", a mea he inhibitor o ka mutant protooncogene. Eia hou, ke kaumaha ma muli o ka lapaʻau, symptomatic Inc. Maʻi prognosis hilinaʻi nui ma luna o kona uku o ka peleʻike manaʻo. Exophthalmos a me ke pio ana o ka ÷ naehana hanu'ōnaehana i manaoia noho lakou malaila hoailona.