Hemophilia - zvanzi chirwere? Sei uye chii vari zviratidzo hemophilia?

For vasingazivi zvikuru vanhu hemophilia - vanonzi chirwere youmambo, ziva nezvazvo chete munhoroondo vanoti, izvozvo vakatambura Tsarevich Alexei. Nemhaka yokushaya ruzivo, vanhu kazhinji vanofunga kuti vanhuwo havagoni kurwara ne hemophilia. Zvinofungidzirwa kuti zvinokanganisawo chete zvizvarwa zvekare. Mafungiro akafanana vagara kuti "revatongi" gout. Zvisinei, kana gout - Power chirwere, uye zvino zvinogona pasi munhu, kuti hemophilia - nhaka chirwere, uye kuwana chero mwana, ane madzitateguru aiva nechirwere.

Chii Hemophilia?

In vanhu, hosha inonzi "mvura neropa." Zvechokwadi, ayo maumbirwo pathological, uye saka hausi kukwanisa clot. The kuchangotanga muvare - uye nekudeura ropa zvakaoma kurega. Zvisinei, ndiro kuratidzwa kwokunze. Zvikuru kwazvo zvemukati kuitika majoini, dumbu, itsvo. Ropa mavari anogona kunzi kunyange pasina kunze simba uye vari migumisiro ngozi.

Nokuti ropa coagulation mutoro gumi dzakananga mapuroteni kuti kunofanira kuvapo muropa panguva chaicho yevasungwa. Hemophilia hosha chirwere mune chiitiko mumwe mapuroteni izvi vasipo zvachose kana aripo mune kukwana yevasungwa.

mhando hemophilia

In mushonga, pane mhando nhatu chirwere.

1. Hemophilia A. Inonzi Kusavapo kana kushaya coagulation chinhu VIII. Vakawanda chimiro chirwere ndiko, maererano nenhamba, 85 muzana zvose nyaya. Paavhareji, mumwe mwana ane 10 000 inongova murwere hwakadaro hemophilia.
2. Hemophilia B. Kana vakachengeta nezvinetso chinhu pasi nhamba IX. Kunoonekwa sezvo zvikuru zvisingawanzoitiki; nayo ngozi achirwara ari katanhatu kuvatumwa munyaya nezvechisarudzo A.
3. Hemophilia C achishayiwa chinhu nhamba XI. Miti iyi akasiyana: nayo kunoratidzwa vose varume nevakadzi. Uyezve, kazhinji achirwara-Ashkenazi vaJudha (izvo vakawanda hakusi chaiwo chero zvirwere vachiwanzoti vari dzakawanda uye zvakafanana "makagadzirira" kune marudzi ose, marudzi uye mapoka emadzinza). With kuratidzwa hemophilia uyewo akagogodza kubva zvinonakisa kunotoda mufananidzo, saka mumakore achangopfuura, rakundwa kubva mazita hemophilia.

Zvinofanira kucherechedzwa kuti mumwe pavatatu mhuri chirwere ichi kunoitika (kana chirwere) kekutanga, inova yakavhundutsa vabereki vasina kugadzirira.

Nei chirwere kunoitika?

Muvambi wayo anova vachiberekwa hemophilia Gene, riri pamusoro X chromosome. Carrier ari munhukadzi, akati iye haasi murwere, asi kuti pangava kakawanda nosebleeds, kumuremera kumwedzi, kana kupfuura zvishoma nezvishoma kuporesa maronda diki (semuenzaniso, pashure pamusoro kudzura mazino). The jini recessive, saka kurwara, havasi vose vane mai - kuva mutakuri chirwere. Kazhinji, mukana iri vakaparadzira 50:50. It anowedzera kana mhuri ine uyewo baba. Girls uyewo vari vatakuri jini fanira.

Sei hemophilia - chirwere varume

Sezvambotaurwa, kune hemophilia jini recessive uye wanamatira kuti mashoko okutodza, anotaurwa so X. In vakadzi, okutodza izvi zviviri. Kana imwe Gene akadaro akauraya, zviri kurukutika uye chechipiri woutsinye, kureva, saka kuti kunongova mutakuri musikana, kuburikidza icho chinopfuudzwa hemophilia, asi pacharo anoramba utano. Kunenge kuti pamwe pamuviri X chromosome anogona zvine runoenzanirana Gene. Zvisinei, panguva paumbwe zvibereko zvavo kweropa maitiro (uye ichi ndepana kota pamuviri vhiki) zvinova unsustainable, uye pane kwatangwa pamuviri (kusvodza). Sezvo kutanga kungava nokuda kwezvikonzero zvakasiyana-siyana, kazhinji hapana zvidzidzo samoabortnogo haana kuitwa, saka kuti nhamba iri nyaya iyi kwete.

Chimwe chinhu - varume. Vane yechipiri X chromosome asipo, achitsinhanisa Y. Hukuru "XXX" harisi, saka kana munhu recessive anoratidza pacharo, rwunotanga kuyerera chirwere pane ayo Isimba rakadini rinogona kubudiswa mamiriro. Zvisinei, nokuti mashoko okutodza ichiri maviri, mukana zvakadaro Kugadzirwa rangano chaizvo hafu pasinei nezvipingamupinyi zvose.

Zviratidzo hemophilia

Ivo kungazviratidza pakuzvarwa, kana kuMudzidzisi chinokonzera mumuviri zviri zvachose ndisipo, uye anogona kuita pacharo akanzwa chete pamusoro nguva kana pane kushayikwa kwayo.

1. Ropa pasina zvikonzero pachena. Kazhinji mwana anozvarwa aine mitaro ropa kubva mhino, maziso, guvhu, uye zvakaoma kumisa kubuda kweropa.
2. Hemophilia (mufananidzo uri dzinoratidza), kunoratidza pacharo kupinda hombe kuzvimba mavanga pamusoro zvachose vasingakoshi zvazvinoita (somuenzaniso, munwe kuvafurira).
3. Akadzokorora ropa kufanana kare aporeswa ronda.
4. Inosimudzira mhuri ropa: mhuno, mazino kunyange Kukwesha mazino ako.
5. Ropa kupinda majoini.
6. ropa iri weti uye mutsvina yadzo.

Zvisinei, "zviratidzo" vakadaro hadzishandi anoratidza zviri pamusoro hemophilia. Somuenzaniso, nosebleeds zvingaratidza kusava nesimba kwemasvingo midziyo ropa, ropa riri weti - nokuti renal zvirwere uye pachituru - denda. Saka zviri chokwadi kuti zvimwe tsvakurudzo.

Kuziva hemophilia

Kuwedzera kudzidza yokurapwa nhoroondo murwere uye kuongororwa yake zvakasiyana siyana vanachiremba zvidzidzo rabhoritari nemiedzo. Panzvimbo yokutanga vakatsunga nokuvapo muropa zvose coagulation zvinoita vavo yevasungwa. Kugadza nguva zvaida kuti coagulation ropa mharidzo. Kazhinji miedzo aya pamwe uye tsvakurudzo DNA. Kutsunga zvingava zvakakodzera kuti zvakarurama kuongorora chirwere:

• thrombin nguva;
• vakavhengana Paarismängud;
• prothrombin indekisi;
• uwandu fibrinogen.

Dzimwe nguva akumbira uye mamwe unyanzvi ruzivo. Zvechokwadi, vakagadzirira pamwe yakakodzera zvokushandisa, kwete chipatara ose, saka pamwe vakafungira hemophilia inotumirwa ropa yezvirwere.

The chirwere, icho pamwe hemophilia (mufananidzo)

The kupfuura hunhu hemophilia - ndiko articular kubuda ropa. The dzokurapa zita - gemoartroz. Inokudziridza asi nokukurumidza, kunyange zvazvo vakawanda yaingowanikwa varwere zvikuru hemophilia. Ivo ropa majoini kuitika pasina zvokunze simba, nomuzvarirwo. Nyoro zvakatsamwisa gemoartroza kwaida kukuvara. Majoini vanokanganiswa zvikurukuru nevaya vari kushushikana mupfungwa, kureva, mabvi, hudyu uye pristopnye. Second mumutsetse - papfudzi vanovatevera - kugokora. Zviratidzo zvokutanga pachena gemoartroza vanotova mwana-gore masere. Nokuda articular maronda wevarwere vakawanda vawane akaremara.

Vanoshupika nhego: itsvo

Hemophilia chirwere kunowanzokonzera nechitarisiko ropa weti. Inonzi hematuria; anogona mberi zvakanaka, kunyange chiratidzo yasara anovhundutsa. About hafu haematuria achabatsirwa kwenguva refu kurwadziwa. Kakawanda renal colic kunokonzerwa ropa clots kukuvadza ureter. The anowanzodanwa pavarwere vane hemophilia pyelonephritis, akanyatsotevera nokuda kakawanda chiitiko - hydronephrosis, uye munguva ichangopfuura akatora rirambe richibvira kuoma dzimwe nhengo dzomuviri. Mabatirwo renal chirwere kuoma nemitemo vamwe pamusoro mishonga zvinodhaka: chinhu chinowedzera liquefaction ropa, hazvigoni kushandiswa.

hemophilia kurapwa

Zvinosuruvarisa hemophilia - hosha isingarapiki kuti zvaanoripirwa upenyu hwevanhu. Kwete kufunga imwe nzira yaunogona kumanikidza muviri kubereka zvinodiwa mapuroteni, kana hazvina kuita kubva pakuberekwa. Zvisinei, kufambira mberi mune zvokurapa ano kuti tikwanise kuramba muviri panguva pamwero panguva iyo vanhu hemophilia, kunyanya mu kwete kwazvo, zvingaita dzinenge dzose kuvapo. Kuti adzivise ropa uye hematoma zvinoda kugara aizomupa zvinogadzirisa asipo pakugwamba zvinhu. Vari kure ropa revanhu vaipa uye vairima mari remhuka. Administration zvinodhaka ane zvechigarire hwaro sechinhu preventative uye dzokurapwa kana kuuya oparesheni kana kukuvara.

In zvakafanana, risingagwambi vanofanira kuramba kumbotambudzika mumuviri kurapa kuramba kwemajoini utano. Kana zvakare guru, zvinova ngozi hematoma chiremba anoita kuvhiyiwa kuti dzibviswe.

Sezvo zvaida ropa zvinodhaka akaita pachishandiswa ropa, hemophilia chirwere kunowedzera ngozi kuti nemavhairasi chiropa, cytomegalovirus utachiona, herpes, uye - vakaipisisa - HIV. Pasina mubvunzo, vaipa vose kuedzwa kuti vakachengeteka ropa ravo, asi kuti pave hapana.

vawana hemophilia

Kazhinji, hemophilia ndiyo nhaka. Zvisinei, pane dzimwe nhamba kana kwakaratidzwa mukati vakuru, kare pasina nhoroondo izvozvo. Sezvineiwo, zviitiko zvakadaro apo neapo - imwe kana mbiri vanhu miriyoni paminiti. Most kuwana chirwere, kuva vanopfuura makore 60. Muzviitiko zvose zviri vawana haemophilia - munhu worudzii A. Zvinokosha kuti zvikonzero iyo akasvika, vakawana isingasviki hafu varwere. Pakati nekenza izvi, akatora vamwe mishonga, autoimmune chirwere, kashoma chaizvo - pathological, pamwe panowanikwa chakakomba, mushakabvu pamuviri. Nei vamwe akarwara, aigona haangasimbiswi n'anga.

Victorian chirwere

nyaya yokutanga vawana zvirwere kunotsanangurwa Muenzaniso Queen Victoria. Kwenguva refu zvaionekwa chete nerudzi rwayo, kubva kunyange wake, kana anoda kusvika hafu yezana remakore pashure hemophilia vakadzi haana kuchengetwa. Zvisinei, mugore rechimakumi maviri remakore, nokuuya nhamba iri kutsvaka chirwere youmambo, mambokadzi rakasiyana haagoni kunzi: hemophilia, yakabuda pashure pokuberekwa, asiri enhaka, hakunei pabonde kurwara.