Isifo Erdheim - yeka zokugula?

Nangona isibakala sokuba iyeza likhula ngokukhawuleza, izifo ezininzi azikhange kufundwe de kube sekupheleni. Abanye babo ezingachazwanga kudala. Ngoko ke, idatha kwezinye ezimbi kweli nqanaba akwanelanga. Umzekelo isifo Erdheim. Imbandezelo ivuleleke kuphela kwinkulungwane XX. Yena sisifo esingaqhelekanga, ngoko akukho kungenzeka ukuba bangadlulela ukufunda nzulu kwesi sifo. Oonobangela kunye Pathogenesis na ukuphazamiseka ezingaziwayo ukuya ekupheleni. Noko ke, kukho iingcamango ezininzi zesi sifo. Bonke bephela ngenkuthalo efunda. Ukuza kuthi ga ngoku, kuphela 500 amatyala ezaziwayo izifo emhlabeni. Ekubeni ngezifo kucingwa ezinqabileyo, akusoloko kulula ukubona.

Yintoni isifo Erdheim?

Kuba okokuqala kule sifo, oko kwazeka ngo-1930. Kuye kwavulwa izazinzulu William Chester kunye notitshala wakhe - uJacob Erdheim. Basebenza kunye kwi sifundo kwesi sifo. Ngoko ke esi sifo idla ngokuba syndrome Erdheim Chester. Ngokutsho data, nelawula ukuqokelela kule minyaka, lo ngezifo ixhaphake kakhulu ngabantu. Kwiimeko ezininzi, isigulo ubonisa kuqala ngokwayo xa eneminyaka engama-50. Noko ke, kukho iimeko ezininzi lezifo phakathi kwabantwana. Iimpawu zesifo ziyahluka. Amaninzi, wabonakala lwezonyango ezilandelayo: isifo bone, zizigulo zemithambo kunye seswekile insipidus. Isifo (syndrome) Erdheim-uChester luphawulwa afunxwe izicwili omzimba ezahlukeneyo histiocytes nelangergansovymi. Ezi seli kuba iinxalenye kwamajoni omzimba. Ngokuqhelekileyo, banalo zikhusela. Kodwa kukho histiocytes ukuzala unmotivated, ngenxa yoko ezichaphazela amalungu ezahlukeneyo kule ngezifo.

isifo Erdheim: iimpawu zesifo

Lo mfanekiso kwezonyango yale syndrome enqabileyo inokuba nomahluko. Kuxhomekeke apho amalungu ahlaselwa histiocytes. Phantse Kwiimeko zonke izifo Erdheim kuboniswa utshintsho kuphahla, luvo nolusu. Phakathi iimpawu zesi sifo bodwa ephule zilandelayo:

• Osteosclerosis periosteum. Oku kuzibonakalisa eqhutywa uninzi lwezigulane. Nakuba kunjalo, kwiimeko ezininzi, le uphawu akuthethi ukuphazamisa izigulane. Kuphela isabelo sabantu kwesi sifo, ikhalazela of intlungu kwisiza sisilonda.
• Exophthalmos. Esi sifo Iimpawu ukuphuhlisa ngenxa ngokoyiswa kwisithuba esemva lweliso lakho. Kwakhona kwaba histiocytes kunokubangela ucinezelo le-luvo optic kunye esihlunwini. Ngoko ke, kwezinye izigulane kukho uphawu njengoko diplopia. Ezinye izigulane ingxelo ukuhla acuity olubonakalayo.
• nokungena Histiocytic amalungu yonyango. Iziphiwo na uphuhliso seswekile insipidus (unxano, polyuria), ukuphazamiseka metabolic.
• Ulungiselo uteronefroz. Ezi mpawu ukuphuhlisa ngenxa ucinezelo yethishyu histiocytic ye izintso ureters.
• Ekubulaleni kwenkqubo sentliziyo kunye nemiphunga.
• Xanthelasma (amafutha) kwi iinkophe kunye xanthoma. Neoplasms kungenziwa akhiwe bonke phezu komzimba.

Sifo Erdheim syndrome

Usola isifo Erdheim-uChester kunzima ngenxa ivela ezinqabileyo kwaye inani elikhulu ukwenza izinto ezinokwenzeka kunye nezinye izinto ezimbi. Amaninzi, kufuneka sinikele ingqalelo indibaniselwano iimpawu ezifana proptosis kunye neentlungu emathanjeni, kwakunye nophuhliso ngokuthe ngcembe lunxano kunye polyuria. Nakujoliso olutsha of nokungena kungabonwa yi-X-reyi amalungu omzimba, yokakayi. Kwakhona, xa isifo kurhanelwa, a biopsy olwenziwa imizimba ukuba bahlangabezana nokungena engaqhelekanga. Ngaphezu koko, wenza fundus yoviwo, CT kunye MRI of isithuba retroperitoneal. Ukuba kukho i kubonakaliswa biopsy cutaneous lwemimandla zophendlo lwezifo (xanthomas) okwenziwa. Ngaba uqinisekise uxilongo kunye uviwo yemfuza.

Zokufuya ekunyangeni isifo Erdheim

Ukuze uqale unyango kufuneka ngoko nangoko emva kokuxilongwa: isifo Erdheim. Unyango lwesi sifo ngalo mzuzu liye lancitshiswa emisela "interferon". ithamo medicament kuxhomekeke kubungakanani nakujoliso olutsha of nokungena. Ekubeni kutshanje basebenzisa iziyobisi "vemurafenib", nto leyo inhibitor le protooncogene njengesi. Ukongeza, ngokuxhomekeke lwezonyango, unyango nomqondiso. Disease abane kuxhomekeke ekujikelezeni kwayo iziganeko kwemali. Exophthalmos kunye ngokoyiswa isixokelelwano sokuphefumla zijongwa imiqondiso ezimbi.