Matenda Erdheim - zimene kudwala?

Ngakhale kuti mankhwala kusiyana mofulumira, matenda ambiri afufuza mpaka kumapeto. Ena a iwo anafotokoza osati kale. Choncho, deta pa ena pathologies panthawi imeneyi si kokwanira. Chitsanzo ndi matenda Erdheim. Mavuto ndi lotseguka kokha m'zaka za m'ma XX. Iye ndi matenda osowa, choncho palibe kuthekera kwa chitani kuphunzira kwakuya kwa matenda. Zimayambitsa ndi pathogenesis cha matenda osadziwika mapeto. Komabe, pali maganizo ambiri a matenda. Onsewo zikukangalika kuphunzira. Pakali pano, 500 kokhaku milandu matenda padziko lonse. Popeza kudwala imatengedwa zina si kophweka kuzindikira.

Kodi matenda Erdheim?

Kwa nthawi yoyamba pa matenda ichi, anadziwika mu 1930. Iwo linatsegulidwa asayansi William Chester ndi mphunzitsi wake - Yakobo Erdheim. Iwo ankagwira ntchito limodzi pa kuphunzira za matenda. Choncho matenda limatchedwa syndrome Erdheim Chester. Malinga ndi kafukufuku, amene anatha kusonkhanitsa kwa zaka zambiri, ndi kudwala ndi ambiri mwa anthu. Nthawi zambiri, matenda oyamba kumaonekera ndi zaka 50. Komabe, pali milandu yambiri ya odwala mwa ana. Zizindikiro za matenda zosiyanasiyana. Nthawi zambiri izi mawonetseredwe matenda zimachitika: matenda a mafupa, matenda a ubongo ndi shuga insipidus. Matenda (matenda) Erdheim-Chester yodziwika ndi kusalola thupi zimakhala zosiyanasiyana nelangergansovymi histiocytes. Maselo amenewa a zigawo za chitetezo cha m'thupi. Bwinobwino, iwo ali ndi ntchito yake yoteteza. Koma pali unmotivated kubalana histiocytes, chifukwa iwo bwanji ziwalo zosiyanasiyana kudwala izi.

matenda Erdheim: zizindikiro matenda

Chithunzi matenda a syndrome zosowazi angakhale osiyana. Izo zimatengera chimene ziwalo anawapha. ndi histiocytes. Pafupifupi milandu onse matenda Erdheim anaonetsa kusintha mu mafupa, dongosolo mantha ndi khungu. Mwa zizindikiro za matenda akhoza olekanitsidwa kuyan'anila zotsatirazi:

• Osteosclerosis periosteum. mawonetseredwe Izi alipo ambiri odwala. Komabe, nthawi zambiri, chizindikiro ichi si kusokoneza anthu odwala. Only gawo la anthu odwala matenda, anadandaula ululu mu malo a chotupa cha.
• Exophthalmos. zizindikiro matenda kukhala chifukwa cha kugonjetsedwa kwa danga ili kumbuyo mwana wa m'diso la. Komanso kuchuluka kwa histiocytes zingachititse psinjika za mitsempha chamawonedwe ndi minofu ya. Choncho, odwala ena pali chisonyezo monga diplopia. Odwala ena lipoti kuchepa acuity zithunzi.
• Histiocytic kusalola ziwalo endocrine. Mawonetseredwe ndi chitukuko cha matenda a shuga insipidus (ludzu, polyuria), matenda kagayidwe kachakudya.
• Hydro ndi uteronefroz. Zizindikiro zimenezi kukhala chifukwa cha psinjika minofu histiocytic a impso ndi ureters.
• Kugonjetsedwa kwa dongosolo mtima ndi mapapo.
• Xanthelasma (mafuta) pa zikope ndi xanthoma. Neoplasms akhoza ili yonse pa thupi.

syndrome matenda Erdheim

Akuganiza Erdheim-Chester matenda ndi zovuta chifukwa cha pakuwonekera kwake kawirikawiri ndi ambiri maonekedwe kumene kungachitike ndi pathologies ena. Nthawi zambiri, ayenera kulabadira osakaniza zizindikiro monga proptosis ndi ululu mafupa, komanso mwapang'onopang'ono ludzu, polyuria. Foci ya kusalola angathe kuonedwa ndi X-ray wa miyendo achigaza. Komanso, pamene matenda ndi achifwamba, ndi biopsy imagwiridwa matupi amene anthu nthenda kusalola. Komanso, anachita fundus bwinobwino CT ndi Kujambula wa danga retroperitoneal. Ngati pali ikuchitika ndi biopsy mawonetseredwe cutaneous m'madera pathological (xanthomas). Otsimikiza matenda ndi Kupenda ndi chibadwa.

Njira zochepetsera matenda Erdheim

Kuyamba mankhwala ayenera yomweyo pambuyo matenda: matenda Erdheim. Chithandizo cha matenda pa nthawi yafupika wakuuzani "interferon". medicament mlingo zimadalira kukula kwa foci wa kusalola. Popeza posachedwapa ntchito mankhwala "vemurafenib", chomwe ndi woletsa wa protooncogene ZOOPSA. Komanso, malinga ndi matenda, mankhwala symptomatic. Disease madokotala ananena zawo kumadalira mlingo wake zodabwitsa otaya. Exophthalmos ndi kugonjetsedwa kwa dongosolo kupuma amaonedwa zizindikiro akumane.