Disease Rendu-Osler matenda: matenda ndi mankhwala

Osler-Rendu matenda - Maonekedwe a capillaries yaing'ono magazi (arterioles kapena venules). N'zochititsa chidwi kuti ndondomeko imeneyi lilibe khalidwe yotupa. Nthawi zambiri, matenda akuwonetseredwa monga Intaneti ndi mtima (kapena asterisks). Koma kuwonongeka mtima kumachitika osati padziko khungu. Komanso zizindikiro kumachitika pa nembanemba mucous ziwalo zina (bronchus, mphuno, pakamwa, chikhodzodzo).

Disease Rendu-Osler: Clinic

Kwa nthawi yoyamba matenda likutchedwa mbadwa ya France - Lui Mari Rendu. Malizitsani chithunzi cha matenda operekedwa katswiri British William Osler. Information ndi kuyang'anila wodzilemekeza Frederika Vebera. Anapeza kuti nthendayo tinatengera. Mwana zokwanira adzalandira jini zosalongosoka kholo la. Malinga syndrome zowawa mofanana anyamata ndi atsikana. Pa chitukuko cha yankho lake majini izi: woyamba ndi udindo endoglin, asaaonerera pa ulimi wa glycoprotein lapansi. Gulu lachiwiri amatanthauza kusintha kukula china. Pamene zipangizo zawo kusintha kutaya elasticity, pali zopindika mafupa awo, kuvutika mu kapangidwe ka maselo za indofeliyamu. Dziwani kuti matenda Rendu-Osler (mwiniwakeyo haemangiomatosis) - chikhalidwe yosowa ndi chikhalidwe cha chiyambi chake si nthawi zonse bwino. Kuchuluka kwa matenda ali motere: zingamvekere 1 munthu pa 5,000.

Zizindikiro zoyambirira za matenda

Monga ulamuliro, kwa zaka 6 osati zizindikiro ziwonekera wa matenda (ngakhale matenda Rendu-Osler ndi cholowa). Akafike msinkhu winawake (zaka 6-10) pali zizindikiro choyamba. Pa khungu kapena nembanemba mucous ziwalo zina Tingaone zikamera angiomas (i.e., mtima zotupa). Chifukwa, kupunduka wa capillaries ndi mitsempha akhoza kupangidwa chofiira (kapena buluu kulocha) mawanga kuti magazi. Disease Rendu-Osler-Weber ndi amakhala ndi mitengo otsika clotting magazi. Chifukwa, magazi mulole (monga microtraumas zotsatira). Nthawi zina chizindikiro chabe kuti zingasonyeze pamaso pa matenda, ndi nosebleeds. Komanso atsikana angakhale katundu msambo. Chifukwa cha pafupipafupi magazi akufotokozera magazi m'thupi, mlingo hemoglobin kugwa.

siteji ya matenda

Disease Rendu-Osler-Weber ali magawo angapo. Pakuti aliyense wa iwo amakhala ndi zizindikiro zina. Mu magawo oyambirira a zotupa mtima ang'ono, ndi mawonekedwe a mawanga yaing'ono. The wapakatikati amakhala ndi digiri ya mitsempha kangaude, "akangaude". Nodular mawonekedwe - Maonekedwe a chofiira (kuzungulira kapena chowulungika) tinatake tozungulira kuti protrude pamwamba pa millimeters ochepa. Iwo ndi awiri a pakati pa 5 ndi 7 millimeters. Pamene deta maphunziro zofuna tcheru kukhala wotumbululuka (mitundu). Kenako lembani ndi magazi.

Gulu la mitundu ya matenda

Monga tanenera kale, zingakhale za cholowa Rendu-Osler matenda (matenda chithunzi mawonetseredwe Tingaone m'nkhani ino), amene kuchokera makolo. Ngati matenda imeneyi amadwala ndi bambo ndi mayi anga, mwana ali matenda kudzachitika mu mawonekedwe kwambiri. Palinso ayi milandu, amene uka chifukwa masinthidwe a. Malingana ndi kumene akatswiri chotupa kusiyanitsa mtundu wa m'mphuno (mphuno umawawukhira), pharyngeal, cutaneous (mwina magazi madera ena a khungu). mawonekedwe choipidwa amakhala ndi magazi kuchokera ziwalo. Lusoli anaona ndi mtundu wosanganiza, kumene kumakhudza onse khungu ndi ziwalo.

Disease Rendu-Osler (ICD-10 kachidindo): mfundo zoopsa pa matenda

Choyamba, waukulu chiopsezo Chinthu ndi chibadwa. Komanso, matenda kungakhale mankhwala anatengedwa ndi mkazi wapakati pa miyezi itatu yoyambirira. matenda osakhala opatsirana anasamutsa nthawi imeneyi. Kungakhale magazi izi: zovala akusisita pa malo zowonongeka, kuthamanga kwa magazi, chakudya olakwikawo. Chosowa kufufuza mfundo zofunika chakudya kuti angathe kulimbitsa mitsempha - ndicho chimodzi mwa zifukwa kufooka kwawo. Izi ndi zoona makamaka kumbali ya zamasamba.

njira matenda

Chiphunzitso matenda matenda a matenda Osler-Rendu zochokera mosamala thanzi la wodwalayo. Kawirikawiri pa nkhope, khungu, mucous kuwona telangiectasia. Izi mapangidwe mtundu ofiira omwe zinthu padziko khungu. zopindika otere pabwino ziwalo mkati. Tiyenera wamba magazi mayeso amene angasonyeze kukhalapo kwa magazi m'thupi. kusanthula amuzolengedwa limachititsa kuti azindikire co-matenda. Chimaphatikizanso mayeso mkodzo. Ngati pali zimapezeka maselo ofiira, n'zotheka kunena kuti impso osauka magazi, kapepala mkodzo. The katswiri amachititsa mndandanda wa mayesero. Tsinani chitsanzo limachititsa kuweruza kukha magazi subcutaneous. Pakuti mayeso khungu pansi pa phewa kutigwira kwa kanthawi. Mfundo zimenezi amapereka ndiponso oyang'anira mayeso (ndi superimposed pa mkono kwa mphindi zochepa). Ngati pali kukayikitsa matenda Rendu-Osler, matenda si popanda wozengereza mayesero pa liwiro clotting magazi, magazi nthawi (yopuma chala). thupi M'mimbamo anayendera ntchito endoscope. Ngati zizindikiro kunja ndi kwina, ndiye kukhala yopangidwa tomography. Njira imeneyi zimathandiza inu kuona zakusokonenezedwa mu ziwalo mkati.

mankhwala a matenda

matenda Rendu-Osler chithandizo, lili motere. Choyamba, ichi ndi mankhwala ndiwofatsa. Kwenikweni umenewu chagona mu ntchito zoletsa ya ulimi wothirira pamwamba kuti umawawukhira. mankhwala awa ziletsa resorption wa magazi aumirira ndi magazi asiye kutuluka choncho. Monga mmene kumapeto, mankhwala ena sizithandiza. Nthawi zambiri, opaleshoni chofunika. Pa msonkhano anachotsa gawo zowonongeka chombocho. Iwo akhoza m'malo prosthesis (ngati n'koyenera). Pamene nosebleeds cryocautery amapereka zotsatira zabwino. Kugwiritsa asafe, mazira cholandirira gawo. Sakhala ogwira ndi ntchito laser kwa cauterization. Mu zina, timadzi mankhwala.

Magazi (gemokomponentnaya mankhwala)

Nthawi kumene matenda Rendu-Osler amachititsa lalikulu magazi imfa, amafuna kugwiritsa ntchito magazi. Chakuchepa zigawo zikuluzikulu udindo clotting, amapanga kwa kuikidwa plasma (mwatsopano mazira). mankhwala amagwiritsidwa ntchito zinthu zimene zingawononge moyo wa munthu. Komanso mu Zikatero, mungafunike ndi kuikidwa maselo othandiza magazi kuundana mwiniwake. Pa nthawi imene wodwalayo mawonekedwe choopsa cha magazi m'thupi (pamlingo wochepa kwambiri wa hemoglobin) kapena kusowa kwa magazi chikomokere (imfa ya kutsitsimuka chifukwa chosowa mpweya ku ubongo), kuikidwa maselo ofiira kuchokera mwiniwake magazi. Nthawi zambiri iwo anamasulidwa mapuloteni anapeza padziko.

zosokonezeka zotheka matendawa

Disease Rendu-Osler matenda, pathogenesis chitukuko ndi chosowa mankhwala zingachititse kuti chiwerengero cha zoyipa. Choyamba, kukhala magazi m'thupi. Chochititsa chidwi magazi imfa kungachititse kuti chikomokere magazi ochepa. Munthu mu nkhani iyi anakomoka ndi sakulabadira zikuchitika kunja. N'zothekanso isanayambike ziwalo. Matendawa akhoza kuyambitsa khungu (kwa magazi mu diso). State wa ziwalo chatsitsa kwambiri. kuopsa mbali ya matenda angakhale kukha mwazi muubongo. Komabe, undiagnosed matenda ndi mankhwala woyenera zimathandiza kusunga thanzi ndi moyo wa odwala.

zodzitetezera

miyeso chachikulu kupewa zofunika patsogolo vutoli wapezeka. Mabanja anthu mu chimodzi kapena zingapo ndi matenda Rendu-Osler, ayenera kuonana ndi zachibadwa a. Pali milandu kumene kuimilidwa ndi kupewa pathupi mwana. Komanso muyenera kuganizira za asilikali a chitetezo cha thupi. kanthu zabwino adzakhala ndi nezi zonse mu msewu, kuumitsa kwa thupi. kudya zakudya zopatsa nthanzi ndi chakudya chamagulu n'kofunika nayenso.

kupewa Secondary ndi mayeso zonse ndi akatswiri. cholinga chake ndi kuzindikira mwamsanga matenda. Ngati matenda wakhala matenda, mankhwala ayenera kukhala yake ndi wathunthu. Ngati ndi kotheka, opaleshoni wodwala ayenera kufunsa munthu panthawiyi, dokotala. Izi adzalola kuteteza munthu ku matenda pa opaleshoni (lalikulu magazi). Wodwala aliyense ndi achibale ake ayenera kuuzidwa pa mmene thandizo kwa magazi (onse mkati ndi kunja). Ndi bwino kuchita kuikidwa zigawo zikuluzikulu magazi mwiniwake pokha pokha kufunikanso kwambiri.