Hemophilia - chii ichocho, uye kuti ungadzivisa sei zvinetso zvakaoma?

Kunzwisisa hemophilia - kuti izvi zvakakosha, nokuti chirwere ichi chinonyanyisa. Izvi zvinokonzerwa nehupenyu. Chirwere ichi chakaipa kwazvo: zvinokosha kuti chizive pane imwe nzvimbo yekutanga yekusimudzirwa, kuitira kuti usaunza mamiriro ezvinhu kumamiriro ezvinhu akaoma.

Hemophilia - chii ichocho?

Kuti unzwisise kuti chirwere ichi chii, zvinoreva izwi rinoshandiswa richabatsira. Kubva mururimi rwechiGiriki, "haima" zvinoreva "ropa," uye "philia" zvinoreva "kusvuta." Ichi chirwere chinoratidzwa nechepasi blood coagulability. Vanotambura kubva kuvanhu, asi pane zvimwe (rudzi C). Vanowana ropa risina kuvhara kubva kuna amai: ndivo vanotakura chirwere chacho uye vanoendesa "bhonasi" kuvana vavo. Nhoroondo yacho ine mazita evakadzi vakuru avo vakanga vachitakura hemophilia gene. Somuenzaniso, Queen Victoria uye Duchess of Hesse.

Hemophilia inogarwa sei?

Ijeni rehutano huno huri kuwedzerwa. Inowanikwa mu X chromosome. Nhaka yejeni rehutano inobatanidza nepabonde. Kuti uite kutanga kuratidza chirwere chacho, unoda maviri X-chromosomes ane mutation. Uyu i "chikadzi" chevakadzi. Varume vane X uye Y chromosomes. Zvisinei, pakuita zvinhu zvose zvinoitika zvakasiyana. Apo mukadzi anova nepamuviri nemusikana ane zvirwere zviviri zvemukati X chromosomes, kupera pamuviri kunoitika kwemavhiki mana. Izvi zvinokonzerwa nekuti iyo inosimudzira fetus haigoneki.

Musikana anogona kuberekwa ane mutating imwe yechromosome. Muchiitiko ichi, chirwere chacho pachacho hachizviratidzi pachedu: iyo inowanikwa yakawanda gene haina kuisa chikonzero chekukonzera chirwere chacho. Nhaka ye hemophilia inogona kuonekwa kune vakomana. Mumuviri wenyama, hapana chinotakura geni muY chromosome, uye X-set nechinamutsiriro chiri kukurumidza kukura. Nokuda kwechikonzero ichi, vakomana vanogara nhaka iyi chirwere, uye hemophilia ine tsika yakawanda.

Ndeipi inopisidzira kuipa kwekuvhara ropa?

Izvi zvinokonzera hutano zvine ngozi. Kubudirira kwematambudziko anotevera hakushamisiki kuwirirana nechigarire chechirwere. Iyi ndiyo ngozi iyo yakaipa coagulability yeropa:

1. Icho chinokonzera musasa we placenta munguva yekudzivirira kwekupedzisira.
2. Inogona kukonzera kubuda ropa munguva yekushanda.
3. Dental extraction, chirwere chokuvhiya uye kukuvara kwakakomba kunokonzera kurasikirwa kweropa.

Hemophilia - Species

Kuvhara ropa kunodzivirira kwemuviri. Mune izvi, fibrinogens, platelets uye plasma zvinhu zvinotora chikamu chinoshanda. Kushayikwa kweimwe yezvinhu izvi kunokonzera kuvhiringidzwa mukushanda kweiyo yose coagulation system. Zvichienderana nezvisina kukwana, idzi marudzi e hemophilia anozivikanwa:

• A;
• B;
• C.

Maererano nehuwandu hwekudzivirira kwechirwere chechirwere ichi, madhigirii aya echirwere anosiyaniswa:

1. Zviri nyore - nekubuda ropa kunowanzoitika. Mukuwedzera, ivo vakaderera-zvakanyanya.
2. Pakati-yakakomba - nokuti inoratidzirwa kusinganzwisisiki kwekuratidzwa kwezviratidzo.
3. Yakanyanya-kana, somuenzaniso, kusagadzikana kwemazi muropa muchecheche, izvi zvinoratidzwa nekubuda ropa kubva mumudonzvo wembilical, kuvapo kwehematomas pamusoro, melena nezvimwe zvakadaro. Mukukura, chirwere chakadaro hachina ngozi kudarika kubva paucheche.

Hemophilia A

Nokuti rudzi urwu rwechirwere runoratidzika nehuwandu hwehutachiona hwemagetsi e-globulin - chikonzero VIII. Urwu urwere hwehutano hunofungidzirwa sekuru uye hunojairika. Iyo inowanikwa mune 85% yezviitiko apo murwere ane hemophilia gene. Urwu rudzi rwechirwere runofambidzana nekubuda ropa kwakanyanya. Nokuda kwechikonzero ichi zvakakosha kunzwisisa: hemophilia - chii ichocho uye chii chakazara.

Chimwe chikamu chechirwere ichi ndiko kuputsa kwechikamu che plasma che hemostasis. Muchirevo chisinganzwisisiki, kubuda ropa hakuiti nguva dzose mushure mekukuvara. Ichi ndechokuti platelet uye zvikamu zvevascular zvinoshanda zvakanaka. Mushure memusi iwoyo mushure mokugamuchira kukuvara, kubuda ropa kunogona kutanga, izvo zvakaoma kumira. Dambudziko iri harigoni kugadziriswa rimwe zuva.

Hemophilia B

Zita rechipiri rechirwere ichi ndiKristumass chirwere. Chirwere ichi chinoratidzwa nekushaikwa kwechikonzero IX. Iyi enzyme inobatanidza inoshandisa Stuart-Prower yakaoma. Urwu rudzi rweHemophilia muvana runowanikwa mu20% yezviitiko zvine chirwere chekushaya ropa coagulability. Utano hwakadaro hunoonekwa mune 1 kubva pavana 30 000 vachangoberekwa.

Hemophilia C

Iyi subspecies ine kliniki yake yekuratidzwa. Izvo zvakasiyana chaizvo neyechirwere chechirwere chechirwere. Iyi subspecies inotorerwa kubva kuhuwandu hwemazuva ano. Nokuda kwechikonzero ichi zvakakosha kunzwisisa hemophilia - chii ichocho. Iine chinhu chakasiyana cheicho chisingaitiki mune mamwe marudzi ezvirwere. Hemophilia yakadaro mumadzimai inowanikwa kakawanda sevarume. Izvi zvinokonzerwa nekuti kuchinjwa kwegene kunoitwa nechimwe chinhu chikuru. Iko kunewo kugona kwenyika munyika. Ichi chirwere chinokanganisa vaJudha veAshkenazi kakawanda.

Zviratidzo zvehemophilia

Mune zvirwere zvemhando A neB, imwe yekliniki yemifananidzo. Muchiitiko ichi, kushaya ropa kwezviratidzo zvinotevera:

1. Kuvapo kwezvivako zvakasiyana-siyana pamuviri. Vanoratidzika kunyange mushure mokunge bruise ruduku. Hematomas akadaro ane kuparadzirwa kwakasiyana-siyana. Apo kuomerera pane nzvimbo yekupwanya kune marwadzo akasimba.
2. Mumakore makumi mapfumbamwe nemakumi matatu ematambudziko, hemarthrosis inowanikwa. Nhengo yacho inopisa, inopisa, uye ganda riri pamusoro paro rinoputika. Nekudzokorora kwemafupa, osteoarthritis inotanga kukura. Panguva imwe chete iyo inogona kuva nekuputika kwemapfupa eplavic uye vertebral column, atrophy yemasumbu, kududzirwa kwetsoka.
3. Kushambadzira kunoitika mushure mekuvhiyiwa, kuputswa, kuiswa kwezino uye zvimwe kukuvadzwa.
4. Mu5% yezviitiko (zvinowanzoitika kune varwere vari vaduku) kune chirwere chakashata .
5. Hemorrhage mu mesentery - inosangana nemarwadzo akaoma uye zvimwe zviratidzo zvakafanana nekliniki ye peritonitis.
6. Mumakumi maviri ematatu, hematuria inoonekwa. Izvi zvinogona kuendeswa mberi nekurwiswa kwechirwere colic uye kukonzera kukura kwe pyelonephritis.
7. Kuumbwa kwechirwere , kunokonzerwa nemafuta makuru ehematomas.
8. Kubuda ropa mumakumbo, iyo inowanikwa mu8% yezviitiko kune avo vanotambura nehurombo husina kukwana. Mucheka mutsvuku unowanzosangana neutera uye chizuru.

Zviratidzo zvekusava neropa kuvhara mu hemophilia Rudzi rweC in varwere vazhinji vanoratidzirwa zvishoma kana kusaratidzwa zvachose. Vamwe vanhu vanobuda ropa, menorrhagia uye kuonekwa kwemavhumu pamuviri. Kunyange mumhuri imwe, iyo nhengo dzayo dzinotambura nechirwere ichi, kuoma kwechipatara zviratidzo zvakasiyana zvikuru. Zvisinei, kusiyana nezvirwere zvezvikwata A neB, hemophilia C zvinokonzera kupisa mumuviri zvisingawanzoitiki. Hemarthroses inowanzoitika mumamiriro ezvinhu ekutsamwa kukuru kwezvipindi.

Zvakaipa coagulability yeropa - chii chaunofanira kuita?

Kana uine chiratidzo chimwe chechirwere, iwe unofanira kubva uchitsvaka rubatsiro rwechiremba. Kuti asimbise kuongororwa, chiremba achapa murwere kuongororwa kunosanganisira nzira idzi dzeb laboratory:

• kutsunga kwehuwandu hwekugadzirisa zvinhu zviripo muropa;
• prothrombin index;
• kutsunga nguva iyo iyo kuvhara ropa kuchiitika;
• mixt;
• iyo yehutrogen muropa nezvakadaro.

Hemophilia inoonekwa isingabatsiri - kurapwa kwechirwere ichi kwakaderedzwa kusvika pakurapwa kwehutano. Mukuwedzera, kwete basa rakanyanya rakapiwa kudya. Munguva yekuwedzera kwechirwere ichi, kurapa kwayo kunofanira kuitwa muchipatara chechipatara chechipatara. Nyanzvi dzekliniki yakadai dzinoziva hemophilia - chii chinorwara. Murwere akaonana nomurwere anofanira kuva ne "Book of the patient" naye. Ichi chinyorwa chinotevera chine ruzivo pamusoro peboka revanhu rega, iro Rh factor. Inoratidzawo kuoma kwechirwere ichi.

Nzira yei kumira ropa neropa rakanyanya coagulability?

Kana munhu ane hemophilia, anofanirwa kunyatsochenjerera. Iwe haugoni kutora zvishoma nezvoutano hwako! Nheyo dzemitemo munyaya iyi ndeyotevera:

1. Kana mavanga acho akadzika, unofanirwa kuputira uye kutungamira mushonga wokurapa. Uyezve, mazaya anofanira kushandiswa kune yakakanganiswa (haifaniri kuuya kusangana neganda, saka rinofanira kunge rakaputirwa necheputi chepurasi kana tambo tsvuku vasati vashandise).
2. Mune maronda uye maronda anogona kuita mavhambe makuru. Pakati pavo, ropa rinopfuta. Nemhaka yezvipfeko zvakadaro, maronda anowedzera. Kuti udzivise izvi, unofanirwa kubvisa zvakananga "mavhinga". Uyezve, nzvimbo ino inofanira kushambidzwa nechinangwa chepenicillin. Mushure maizvozvi, bhanhire rakapetwa ne hemostatic yakagadzirwa rinoshandiswa paganda. Izvi zvinokurumidza kukuvadza kuporesa.

Mishonga nehurombo husingawaniki coagulability

Therapy ye hemophilia inoratidzika seyiyi:

1. Murwere anokonzerwa nekushayikwa kwezvinhu. Nhamba yenzira dzakadaro dzinogona kusiyana kubva 4 kusvika ku8 pazuva.
2. Ipai majekiseni ezvigadziriswa zvakaitwa pamusana pe plasma.
3. Kuti abvise kubva mumuviri wevarwere zvirwere zvinoratidzira, chiremba anokwanisa kupa plasmapheresis .
4. Hereditary hemophilia inopa kukonzera kwegadziriro yeglucose, Reamberin kana Polyglucin.

Ne hemarthroses, kuvharwa kwebhegi rekubatanidza kunowedzerwa. Kupisa ropa rine zviri mukati maro uye kuwedzerwa kwaro nekugadzirira mahomoni kunoitwa. Pasina kukanganisa, chikwata chakagadzikana chinofanira kunge chisina kukwana panguva yerapi. Mune ramangwana, mushure mekugadzirisa, kurapwa kwepanyama muviri uye physiotherapeutic manipulations zvinorayirwa.

Ndezvipi zvingaidya nehurombo husina kuvhara ropa?

Chikafu chinokonzera simba pamamiriro evarwere. Kudzivirira kuitika kwekubuda ropa, varwere vane hemophilia vanofanira kuwedzera kudya kwavo nezvokudya zvakadaro:

• Mafuta emhuka kubva (batare, marori, kamu);
• chiropa (ubongo, itsvo, chiropa);
• buckwheat porridge;
• majiki ehuputi (blueberry, bruariberi, bheriberry nevamwe);
• bhanana nemango;
• tsvuku tsvuku, radish, red cabbage nemimwe miriwo;
• walnuts;
• nyemba, mapea, soya uye dzimwe nyemba;
• dill, parsley, basil;
• white bread.