Disease Rendu-Osler chirwere: kuongorora chirwere uye kurapwa

Osler-Rendu chirwere - ndiyo pakuonekwa diki ropa capillaries (arterioles kana venules). Zvinokosha kuti izvi hazvirevi kuva anogumbura hunhu. Kazhinji, chirwere kunoratidzwa kuva vascular network (kana asterisks). Asi vascular kukuvadzwa kunoitika kwete chete pamusoro pechiso ganda. Uyewo Zviratidzo kungaitwa mucous membranes mamwe nhengo (bronchus, mhuno, muromo, dundira).

Disease Rendu-Osler: Clinic

Kokutanga hosha chinorondedzerwa chizvarwa France - Lui Mari Rendu. Pedzisa mufananidzo chirwere apa nyanzvi British William Osler. Information uye zvakawanika vaisanganisa Frederika Vebera. It Vakawana kuti hosha iri nhaka. Mwana zvakakwana nhaka chakaremara Gene nomubereki. Maererano chirwere vanotambura zvakafanana vakomana nevasikana. Nokuti kukura mhinduro yake majini zvinotevera: wokutanga ndiye anokonzera endoglin, anodzora kweShanduro glycoprotein. chikamu The yechipiri inoreva rokuchinja kukura chinhu. Kana avo mutation midziyo vanorasikirwa elasticity, pane urema zviri majoini dzavo, abnormalities marongerwo masero endothelial. Zvinokosha kucherechedza kuti Rendu-Osler chirwere (neakangwara haemangiomatosis) - isingawanzowanikwi ezvinhu, uye marwiro kwayakatangira haisi pachena nguva dzose. Kuwanda chirwere ndeinotevera: zvinokanganisawo 1 munhu pa 5,000.

Zviratidzo chirwere rokutanga

Kazhinji, kusvikira 6 makore kwete pachena zviratidzo chirwere (kunyange Rendu-Osler chirwere chacho chichigarwa nhaka). Paaizosvika rimwe zera (makore 6-10) pane zviratidzo vokutanga. On ganda kana mucous membranes dzimwe nhengo zvinogona kuona chidzoke angiomas (i.e., vascular anomera). Somugumisiro, zvikanganiso capillaries nemidziyo ropa zvinogona kuumbwa tsvuku (kana tint yebhuruu) mavara kuti ropa. Disease Rendu-Osler-Weber uye kunoratidzwa yakaderera dziine kweropa. Somugumisiro, ropa angabvuma (kunyange Somugumisiro microtraumas). Dzimwe nguva chete chiratidzo kuti zvingaratidza kuvapo kwezvirwere, muri nosebleeds. Uyewo, vasikana vanogona kuva rinorema kumwedzi. Pamusana kakawanda ropa hwapedyo kupererwa, kuti Chizenga pamwero rinokundwa.

pavaipfuura chirwere

Disease Rendu-Osler-Weber ane nzendo dzakawanda. Nokuti mumwe nomumwe wavo kunoratidzwa zvimwe zviratidzo. In nzendo vascular maronda vokutanga ndivo diki, vane chimiro mavara duku. The zvenguva inozivikanwa nokuva mwero dandemutande mutsinga, "madandaude". Nodular fomu - ndiyo pakuonekwa tsvuku (kunhivi kana denderedzwa) nodules kuti protrude pamusoro pemvura iri millimeters shoma. Vane dhayamita inobva 5 kusvika 7 millimeters. Apo mashoko rokudzidzisa kufurirwa-inonyatsoziva kuva hapana (mhando dzose). Ipapo kuzadza neropa.

Kupatsanura siyana chirwere

Sezvambotaurwa, kungava kuti enhaka Rendu-Osler chirwere (Photo chirwere kuratidzwa zvinogona kuonekwa munyaya ino), icho chinopfuudzwa kubva kuvabereki. Kana urwere ichi kutambura uye baba, naamai vangu, mwana ane chirwere kuchaitika nenzira zvikuru chimiro. Kunewo nguva dziri kure nyaya, izvo simuka nokuda mumwe mutation. Zvichienderana apo lesion nyanzvi kusiyanisa mhando nasal (mhino bleeds), pharyngeal, cutaneous (anogona ropa zvimwe nzvimbo ganda). Visceral chimiro kunoratidzwa ropa nhengo dzomukati. Inogonawo kuonekwa uye zvakavhenganiswa mhando, hunobata zvose ganda uye nhengo dzomukati.

Disease Rendu-Osler (ICD-10 romutemo): zvinokonzera njodzi pa kuongorora chirwere

Chokutanga, chikuru ngozi iri yedzinza. Uyewo, chirwere kungakonzera mishonga kutorwa mukadzi pamuviri ari wokutanga mitatu. Kunaka uye zvirwere Kutamiswa panguva iyi. Kungakonzera ropa zvinotevera: nguvo vachidzipukuta pamusoro nzvimbo dzaparadzwa, BP, asina kudzikama kudya. Kushaya zvinokosha zvisaririra zvinhu zvokudya anogona kusimbisa midziyo ropa - ndicho chimwe zvikonzero utera hwavo. Izvi ndezvechokwadi kunyanya vatsigiri kudya miriwo.

nzira nechirwere

Pfungwa kunotoda chirwere pakati Osler-Rendu chirwere kwakavakirwa kuongorora mamiriro murwere. Kazhinji kumeso, ganda romusoro, mucous anogona kuona telangiectasia. inotungamirwa uku yakatsvuka, izvo kuita pamusoro ganda pamusoro. urema kwakadaro kunogona akaiswa pamusoro zvomukati. Tinoda nevakawanda kuongororwa ropa kuti anogona kuratidza kuvapo kupererwa neropa. Nemakemikari kuongorora chinoita kuti vakwanise kuona co-utachiona uye zvirwere. It kunosanganisirawo weti bvunzo. Kana paine zviri kuwanikwa masero matsvuku eropa, zvinokwanisika kutaura kuti kusimukira itsvo ropa, Urinary turakiti. Mazvikokota anoitisa yaitevedzana nemiedzo. Nzwara mharidzo runoita kutonga subcutaneous hemorrhages. Nokuti bvunzo ganda pasi bondo manikidzira kwekanguva. mashoko akadaro anopa uye joko bvunzo (riri seari pasi honokono kwemaminitsi mashoma). Kana paine fungidziro pamusoro Rendu-Osler chirwere, chirwere haisi pasina kuita bvunzo kumhanya kweropa, kubuda ropa kwenguva refu (hwatsvuka chigunwe). muviri mhango akaongorora kushandisa endoscope. Kana kwokunze zviratidzo hatisi, zvino kuva computed tomography. Nzira iyi inobvumira kuti kuona chero kutyorwa mu zvomukati.

pakurapa chirwere

Rendu-Osler kurapwa chirwere, anogovera sezvinotevera. Chekutanga, iri kuchinja kurapa. Chaizvoizvo Its iri kushandisa FIs pamusoro pemvura kudiridza kuti bleeds. mishonga izvi tunodzivisa resorption ropa clot uye kumisa kubuda kweropa saka. Sezvo tsika inoratidza zvimwe zvinodhaka hadzibatsiri. Kazhinji, kuvhiya kunodiwa. Pamusangano akabvisa hunenge chikamu mudziyo. Inogona kutsiva prosthesis (kana zvakakodzera). Kana nosebleeds cryocautery anopa Somugumisiro akanaka. Kushandisa mvura senitrogen, mudziyo nechando mugove. Zvishoma chinobatsira kushandisa Laser nokuda cauterization. Mune zvimwe zviitiko, hormone kurapa.

Ropa (gemokomponentnaya kurapa)

Muzviitiko apo Rendu-Osler chirwere kunokonzera guru kurasikirwa neropa, zvinoda kushandiswa yezvipo ropa. Kushayiwa yenhengo anokonzera pakugwamba, inoumba kuti kuisirwa plasma (yakanaka muchando). kurapa iri inoshandiswa ezvinhu kuti zvingakanganisa upenyu hwevanhu. Uyewo Pakadaro, ungada kuisira anyatsoronga maplatelet. Muzviitiko apo murwere ane kwazvo muchimiro kupererwa neropa (yakaderera chaizvo kwevari Chizenga) kana kuuya neta neta chaanoziva (kurasikirwa pfungwa nokuda kushayikwa okisijeni kuuropi), kuisirwa matsvuku masero kubva muropa anyatsoronga. Kazhinji vanenge vasunungurwa mapuroteni nechepamusoro.

Possible yematambudziko chirwere

Disease Rendu-Osler chirwere, ari pathogenesis okukura uye kushaya kunobudirira kurapwa kunogona kuita kuti vakawanda zvinhu zvisingafadzi. Chokutanga, kutanga kupererwa neropa. Rinokosha kurasikirwa neropa kungaita kuti neta neta pakoma. Murume uyu nyaya azheuka uye haateereri zviwane. Zvinogonekawo kutanga rutivi rwomuviri. The chirwere kungaita kuti upofu (nokuti ropa kupinda the retina). State of zvomukati kuipa yekudyidzana. Inokuvadza divi Migumisiro chirwere kunogona uropi ropa. Zvisinei, undiagnosed noutachiona uye ruzivo kurapa rinobvumira kuponesa utano uye upenyu varwere.

matanho

Primary matanho kudzivirira zvinodiwa pamberi dambudziko iri dzakaonekwa. Mhuri umo mumwe kana kupfuura nhengo nechirwere Rendu-Osler chirwere, vanofanira kubvunza munhu geneticist. Pane zviitiko apo kurumbidzwaka uku Kurega yokubereka mwana. Uyewo vanofanira kuva nehanya kuchengeteka mauto muviri. chiito Good achava dzose famba mumugwagwa, kuomesa muviri. Kudya zvinovaka muviri ane mwero zvinodyiwa zvakakodzera.

Secondary kudzivirira nguva dzose bvunzo kubudikidza unyanzvi. Chinangwa charo ndiwo vasabatwa chirwere mangwanani. Kana urwere kwave nechirwere, kurapwa kunofanira yakakodzera uye kwakakwana. Kana zvakakodzera, kuvhiya murwere unofanira hazvo watarisa hematologist. Izvi zvichaita kuti kudzivirira munhu yematambudziko pakuvhiya (zvikuru ropa). Mumwe murwere nehama dzake dzinofanira kupiwa mashoko sei kuti kupa rubatsiro rwekutanga kuti ropa (zvose zvemukati uye kwokunze). Zvinokurudzirwa kuita kuisira anyatsoronga ropa zvinoriumba chete vanenge zvokuora kunofanirawo.