Disease Rendu-Osler izifo: isifo kwaye afumane unyango

isifo Osler-Rendu - kuyinto ukuvela emithanjeni ukopha encinane (arterioles okanye venules). Kuyinto ebalulekileyo ukuba le nkqubo ayinayo umlinganiswa arhabaxa. Kwiimeko ezininzi, esi sifo lubonakala njenge networks nemithambo (okanye asterisks). Kodwa umonakalo semithambo kwenzeka kuphela kwi ngaphezulu elufeleni. Kwakhona iimpawu esinokubakho kwiinwebu nezinye izigqeba (bronchus, impumlo, umlomo, isinye).

Disease Rendu-Osler: Clinic

Ngokuba lixesha lokuqala sifo uchazwa inzalelwane France - Lui Mari Rendu. Gqibezela umfanekiso sifo enikiweyo yingcali yaseBritani William Osler. Ulwazi kunye nemigqaliselo yongezwe Frederika Vebera. Kwaye kwafumaniseka ukuba esi sifo njengelifa. Umntwana ngokwaneleyo ukuba ilifa gene ezinesiphene umzali. Ngokutsho syndrome ubunzima ngokulinganayo zombini amakhwenkwe kunye namantombazana. Ukuze uphuhliso impendulo yakhe zofuzo zilandelayo: owokuqala unoxanduva endoglin, ulawula imveliso ye glycoprotein. Udidi lwesibini ibhekisa into yokukhula aguqulayo. Xa iimpahla zazo ukuguquka ngamandla okubuyela kwimo, kukho iziphene kwamalungu, ukungaqheleki xa isakhiwo iiseli endothelial. Kubalulekile ukuphawula ukuba isifo Rendu-Osler (haemangiomatosis olusasaziweyo) - imeko enqabileyo, kunye nohlobo imvelaphi yayo akusoloko kucacile. Ukuxhaphaka sifo zezi zilandelayo: ichaphazela umntu 1 5,000 ngamnye.

Iimpawu zokuqala sifo

Njengomgaqo, ukuya kutsho kwiminyaka 6 iimpawu zibonakale kwesi sifo (nangona isifo Rendu-Osler yi ilifa). Emva kokuba iminyaka ethile (iminyaka 6-10) kukho imiqondiso yokuqala. Esikhumbeni okanye kwiinwebu namanye amalungu kungabonwa le angiomas kokuhluma (ngamanye amazwi, nangezilonda nemithambo). Ngenxa yoko, iziphene ngcanjana nempahla igazi kuyilwa obomvu (okanye ithinti blue) amabala ukuba exesheni. Disease Rendu-Osler-Weber kwaye luphawulwa amazinga aphantsi igazi ekunqandeni. Ngenxa yoko, ukopha kungase (njengoko a microtraumas ngenxa). Maxa wambi kuphela uphawu asenokubonisa ubukho sifo, kuba nokopha. Kwakhona, amantombazana kuba sexesheni enzima. Ngenxa ukopha rhoqo uvela anaemia, inqanaba haemoglobin uyawa.

Inqanaba lesi sifo

Disease Rendu-Osler-Weber na izigaba ezininzi. Ngokuba elowo kubo luphawulwa iimpawu ezithile. Kumanqanaba okuqala izilonda nemithambo zincinane, babe uhlobo amabala ezincinane. I-intermediate iphawulwa ukuba iqondo emithanjeni zesigcawu, "izigcawu". ifomu Nodular - kuyinto ukuvela obomvu (ngeenxa okanye elimbhoxo) maqhuqhuva ukuba ezivelileyo ngaphezu komhlaba a ngemilimitha ezimbalwa. Zinelungelo sangqa omise ukusuka-5 ukuya ku 7. Xa data zemfundo uxinzelelo-onovakalelo ube luthuthu (zonke iindidi). Emva koko ke bhala igazi.

Ucalulo lweendlela sifo

Njengoko sele kukhankanyiwe, kusenokuba zamafa sifo Rendu-Osler (obubonakala isifo ifoto kunokubonwa kweli nqaku), apho sivela kubazali. Ukuba le syndrome ubunzima kunye notata, kunye nomama wam, umntwana lowo sifo liya kwenzeka uhlobo kakhulu ngakumbi. Kukho iimeko manqapha, leyo kuvela ngenxa kuguquka. Kuxhomekeka apho iingcali sisilonda esidumbileyoesingaphakathi ukwahlula uhlobo ngeempumlo (impumlo ukopha), pharyngeal, cutaneous (inokuba ophela iindawo ezithile kwesikhumba). ifomu Visceral luphawulwa nokopha emazikweni zangaphakathi. Kusenokwenzeka kwakhona eziqwalaselwa uhlobo engumxube, nto leyo ichaphazela zombini ulusu kunye nezibilini.

Disease Rendu-Osler (ICD-10 ikhowudi): ubungozi xa kufunyaniswa isifo

Kuqala kwinto zonke, umngcipheko into eyinhloko yofuzo. Kwakhona, esi sifo kungabangela amayeza ezithathwe umfazi okhulelweyo kwiinyanga ezintathu zokuqala. izifo ezingakhuselekanga ezosulelayo yagqithiselwa ngeli thuba. Kungabangela ukopha zilandelayo: impahla ngokuhlikihla kwimimandla ezonakeleyo, uxinzelelo oluphezulu lwegazi, ngokunciphisa ingafanelekanga. Ukunqongophala izinto zokulanda umkhondo zibalulekile ukutya ukuba unako ukomeleza imithambo yegazi - loo nto esinye sezizathu kubuthathaka babo. Oku kuyinyaniso ngokukodwa abaxhasi imifino.

iindlela isifo

Ukusekwa uxilongo lwezonyango sifo Osler-Rendu ngokusekelwe zoviwo ngemeko yesigulana. Ngokuqhelekileyo phezu kobuso, Nentloko enesihlwitha yohambayo, yangaphakathi ungabona telangiectasia. Le ukuyilwa umbala obomvu, eba phezu esikhumbeni. iziphene ezilolu hlobo ibekwe enyama yangaphakathi. Sifuna uvavanyo lwegazi eziqhelekileyo kubonisa ubukho kwegazi. Uhlalutyo ezinto kwenza kube lula ukubona intsebenziswano-usulelo kunye nezifo. Kwakhona ibandakanya uvavanyo lomchamo. Xa kuthe kwafunyanwa iiseli ezibomvu, kunokwenzeka ukuba bathi kwizintso asakhasayo ukopha, womchamo. Yingcali uqhuba uthotho lweemvavanyo. Aliqhawule isampuli yenza kube lula ukuba agwebe hemorrhages ongama. Kuba uvavanyo ulusu phantsi ukulicudisa collarbone okwethutyana. Olunjalo ulwazi inikeza kunye nokulawula test (ukuba enyanzeliswa phezu zengalo okwemizuzu embalwa). Ukuba kukho urhano lwesifo Rendu-Osler, uphethwe yini ngaphandle zokwenza uvavanyo kwisantya igazi ekunqandeni, ukopha ubude bexesha (breaks umnwe). umzimba uvuleke zihlolwe usebenzisa mbhobho wokuhlola. Ukuba iimpawu zangaphandle abangekhoyo, woba Itomography ngekhompyutha. Le ndlela ivumela ukuba ubone naziphi na ukunyhashwa kwi izibilini.

unyango lwesifo

unyango lwesifo Rendu-Osler, ubonelela ngolu hlobo lulandelayo. Okokuqala, lo kwanonyango endala. kubume bayo siyifumana ukusetyenziswa inhibitors of okunkcenkceshela ocinga ukuba ukopha. Ezi amayeza zinqanda resorption le Ihlwili igazi ukunqanda ukopha njalo. Njengoko yokusebenza ibonisa, amanye amachiza angasebenzi. Amaxesha amaninzi, utyando iyafuneka. Ngexesha lentlanganiso Ndiyikhuphe kwee tu into eyonakeleyo le nqanawa. Kunokuba endaweni afakelweyo (ukuba kuyimfuneko). Xa nokopha cryocautery inika nemiphumo emihle. Ukusebenzisa nitrogen engamanzi, ukudla isitya isabelo. ngempumelelo Ngaphantsi ke ukusetyenziswa laser for cauterization. Kwezinye iimeko, kwanonyango hormone.

Igazi (therapy gemokomponentnaya)

Kwiimeko apho sifo Rendu-Osler ibangela ukopha enkulu, ifuna ukusetyenziswa ezinikelwe igazi. Ukuswela components anoxanduva kwamahlwili, yenza ngenxa ukutofelwa plasma (fresh zibambene). Olu nyango isetyenziswa iimeko ezisongela ubomi babantu. Kwakhona Kwiimeko ezinjalo, kusenokufuneka ukuba ukutofelwa ziiplatelets kubalizi. Kwiimeko apho isigulana umntu kakhulu lwe-anaemia (amanqanaba asezantsi kakhulu mpilo) okanye eze isiqaqa zobuthathaka begazi (ilahleko osezingqondweni ngenxa yokunqongophala kwe-oksijini engqondweni), ukutofelwa iiseli ezibomvu exhasanayo igazi. Ngokufuthi kokuba bekhululwe kwiiproteni safumaneka kumphezulu.

iingxaki kwesi sifo

isifo Isifo Rendu-Osler, lo Pathogenesis kophuhliso kunye nokungabikho unyango olusebenzayo kungakhokelela eziliqela izinto ezimbi. Okokuqala, ukuphuhlisa anemia. ukopha ebalulekileyo kunokukhokelela isiqaqa igazi. Le ndoda kule meko zingqondweni aze akaziphenduli ezenzeka ngaphandle. Kwakhona ekuqaleni ekukhubazekeni kunokwenzeka. Esi sifo kukhokelela kubumfama (ngokuba ukopha kungena retina). State of nezibilini kuguga kakhulu. iziphumo ebezingalindelekanga eziyingozi isifo inokuba legazi ebuchotsheni. Noko ke, usuleleko kungafunyaniswa kwaye unyango ofanelekileyo ikuvumela ukugcina impilo kunye nobomi izigulane.

amanyathelo othintelo

amanyathelo Primary zokuthintela ziyafuneka ngaphambi kokuba ingxaki ibanjiwe. Iintsapho apho amalungu elinye okanye ngaphezulu kwafunyaniswa ukuba izifo Rendu-Osler, kufuneka udibane nge zofuzo u. Kukho iimeko apho ingcebiso ayeke ekhulelwe umntwana. Kwakhona kufuneka sibe nenkxalabo malunga imikhosi ukhuseleko umzimba. inyathelo Good ziya iin- rhoqo esitratweni, lukhuni komzimba. ngokudla kunye ukutya okunazo nako kuyimfuneko.

Uthintelo Secondary i iimviwo rhoqo ziingcali. Injongo yawo lo ekufumanekeni kusekwangoko isifo. Ukuba sifo sele unesifo, unyango kufuneka ngexesha nangokupheleleyo. Ukuba kuyimfuneko, utyando isigulane kufuneka nakanjani kubuzisa segazi. Oku kuya kuvumela ukukhusela umntu iingxaki ngexesha utyando (nokopha). Ngasinye isigulane kunye nezalamane zakhe kufuneka banikwe ulwazi malunga nendlela ukunika uncedo lokuqala ukuba ukopha (olwangaphakathi nolwangaphandle). Kucetyiswa ukuba wenze ukutofelwa yeekhomponenti igazi kuphela kwiimeko kokokunyanzelwa kakhulu.