Erdheim isifo - yeka zokugula?

Naphezu kweqiniso lokuthi imithi okhula ngokushesha, izifo eziningi ezingazange wafunda kuze kube sekupheleni. Ezinye zazo zazibizwa akunjalo kudala. Ngakho-ke, i-data esekhadini ezinye pathologies kulesigaba akwanele. Isibonelo salokhu isifo Erdheim. Ukuhlupheka ivulelwe kuphela ekhulwini XX. He is isifo ezingavamile, ngakho akukho kungenzeka ukuqhubekela isifundo esijulile yalesi sifo. Izimbangela pathogenesis ka iziyaluyalu ezingaziwa kuze kube sekupheleni. Noma kunjalo, kukhona eyeza nezazi zezinkanyezi ezikhuluma eziningana lesi sifo. Bonke ukukhuthalele wafunda. Kuze kube manje, 500 kuphela amacala eyaziwayo izifo emhlabeni wonke. Njengoba zokugula kubhekwa ezingavamile, akulula ngaso sonke isikhathi ukuthola ukuthi iyini.

Uyini isifo Erdheim?

Ngokokuqala ngqá kule isifo, yabe seyaziwa ngo-1930. Lalivulwe ososayensi uWilliam Chester futhi uthisha wakhe - Jakobe Erdheim. Basebenza ndawonye esifundweni yalesi sifo. Ngakho lesi sifo ngokuvamile okubizwa ngokuthi syndrome Erdheim Chester. Ngokusho idatha, okuyinto wakwazi ukuqoqa eminyakeni, lo zokugula landile amadoda. Ezimweni eziningi, ukugula kuqala kuzibonakalisa kweminyaka eyi-50. Noma kunjalo, kukhona amacala amaningi yokugula kwezingane. Ngezimpawu zesifo ziyahlukahluka. Ngokuvamile, izenzo zokwelapha ezilandelayo zenzeka: isifo ithambo, iziyaluyalu zezinzwa sikashukela insipidus. Izifo (syndrome) Erdheim-Chester libhekene kokuziphatha ezicutshini zomzimba ahlukahlukene histiocytes nelangergansovymi. La mangqamuzana basonta izingxenye kwesimiso somzimba sokuzivikela ezifweni. Ngokuvamile, basuke babe umsebenzi zokuzivikela. Kodwa kukhona unmotivated ukukhiqizwa kabusha histiocytes, ngenxa ezisithintayo izitho ezihlukahlukene kule egunjini lokuhlolwa kwezifo.

Erdheim isifo: izimpawu isifo

Isithombe basemtholampilo yilesi sifo ezingavamile zingase zihluke. Kuya ngekutsi umuntfu izitho ahlaselwa histiocytes. Cishe kuzo zonke izimo Erdheim isifo owabonakaliswa izinguquko amathambo, isimiso sezinzwa futhi isikhumba. Phakathi izimpawu zalesi sifo zingase isehlane ukwephulwa elandelayo:

• Osteosclerosis periosteum. Lokhu kubonakaliswa kuyoboniswa nguMbusi elikhona iningi iziguli. Noma kunjalo, ezimweni eziningi, le uphawu akusho ziphazamisa iziguli. Kuphela ingxenye kubantu abaphethwe yilesi sifo, ekhononda ka ubuhlungu indawo ye lesion.
• Exophthalmos. Lokhu izimpawu isifo ukuthuthukisa ngenxa kokunqotshwa isikhala elitholakala ngemva yeso. Futhi ekwandeni histiocytes kungabangela kokucindezelwa we emsipheni optic futhi imisipha. Ngakho-ke, kwezinye iziguli kukhona uphawu njengoba diplopia. Ezinye iziguli kubika ukwehla acuity ezibukwayo.
• kokuziphatha Histiocytic kwezitho endocrine. Izenzo ukuthuthukiswa sikashukela insipidus (ukoma, polyuria), disorder umzimba.
• Isikhungo samanzi kanye uteronefroz. Lezi zimpawu ukuthuthukisa ngenxa yokucinana kwezicubu histiocytic we nezinso ureters.
• Ukunqotshwa kwe-uhlelo senhliziyo nemithambo yegazi kanye namaphaphu.
• Xanthelasma (amafutha) ku Isivalo kanye xanthoma. Neoplasms kungenziwa akhiwe wonke umzimba.

Sifo Erdheim syndrome

Umsolwa Erdheim-Chester isifo kunzima ngenxa ivela khona ezingavamile futhi inani elikhulu ukubonakaliswa ukuthi singenzeka nezinye pathologies. Ngokuvamile, kufanele sinake inhlanganisela izimpawu ezifana proptosis kanye nobuhlungu amathambo, kanye zavela kancane kancane ngenxa yokoma futhi ingabe polyuria. Foci ka kokuziphatha kungatholakala by X-ray kwamalungu omzimba, ugebhezi. Futhi, lapho isifo okusolwa, a biopsy is nemizimba uhlangabezana kokuziphatha normal kwenziwa. Ngaphezu kwalokho, kwenziwa fundus luhlolo, CT nama-MRI isikhala retroperitoneal. Uma kukhona Kwenziwa a ukubonakaliswa biopsy cutaneous izindawo sokugembula (xanthomas). Can uqinisekise uphethwe nge luhlolo zofuzo.

Izindlela zokwelapha isifo Erdheim

Ukuze uqale ukwelashwa kufanele kube ngokushesha ngemva uphethwe: Erdheim isifo. Ukwelashwa yalesi sifo okwamanje kuyehla kube enquma "interferon". umthamo nomuthi incike ubukhulu foci of kokuziphatha. Njengoba muva isicelo izidakamizwa "vemurafenib", okuyinto i inhibitor we protooncogene asabekayo. Ngaphezu kwalokho, kuye ngokuthi lwasemtholampilo, ukwelashwa lingaze. Izifo Izibikezelo kuxhomeke izinga yayo ukugeleza imihlola. Exophthalmos futhi ukunqotshwa umgudu wokuphefumula ezibhekwa izimpawu ezingezinhle.