Hemophilia - ichi ndi chiyani matenda? Kodi zimapatsirana motani ndipo zizindikiro za hemophilia ndi ziti?

Kwa anthu ambiri osadziŵa, hemophilia ndi omwe amatchedwa matenda achifumu, amadziwa za mbiriyi okha: amanena kuti Tsarevich Alexei anavutika nazo. Chifukwa cha kusowa nzeru, anthu amakhulupirira kuti anthu wamba sangathe kudwala ndi hemophilia. Pali lingaliro lomwe limakhudza obadwa akale okha. Maganizo omwewo kwa nthawi yaitali anali "gout". Komabe, ngati gout ndi matenda, ndipo munthu aliyense akhoza kuthandizidwa ndi izo tsopano, hemophilia ndi matenda obadwa nawo, ndipo mwana aliyense yemwe ali ndi matenda a makolo ake akhoza kuchipeza.

Kodi hemophilia ndi chiyani?

Kwa anthu, matendawa amatchedwa "magazi amadzi". Ndipo ndithudi, chiwerengero chake ndi matenda osokoneza bongo, omwe amatha kusinthasintha. Chotsitsa pang'ono - ndi kutuluka kwa magazi n'kovuta kuima. Komabe, izi ndi mawonetseredwe akunja. Zowopsa kwambiri, zimachitika m'magulu, m'mimba, impso. Kuwonongeka kwa magazi mwa iwo kungayambitse ngakhale popanda kukhudzidwa kunja ndikukhala ndi zotsatira zoopsa.

Mapuloteni khumi ndi awiri apadera amavomereza kuti coagulability ya magazi , yomwe imayenera kupezeka m'magazi ena. Matenda a hemophilia amapezeka ngati chimodzi mwa mapuloteni amenewa sichipezeka kwathunthu kapena chilibe chiwerengero chokwanira.

Mitundu ya hemophilia

Mu mankhwala, pali mitundu itatu ya matendawa.

1. Hemophilia A. Icho chimayambitsidwa chifukwa chosoweka kapena kusowa kwa chigawo cha VIII. Mtundu wambiri wa matendawa, malinga ndi chiwerengero, ndi 85 peresenti ya matenda onsewa. Pafupifupi, mwana mmodzi pa 10,000 ali odwala ndi hemophilia.
2. Hemophilia B. Kwa iye, pali mavuto ndi chiwerengero cha IX. Amadziwika kuti ndi osowa kwambiri: chiopsezo chodwala chimakhala kasanu ndi chimodzi kusiyana ndi chiwerengero cha A.
3. Hemophilia C. Palibe chinthu china XI. Mitundu imeneyi ndi yodabwitsa: ndi yapadera kwa amuna ndi akazi. Komanso, nthawi zambiri Ayuda a Ashkenazi amadwala (omwe kawirikawiri amakhala osagwirizana ndi mavuto alionse: nthawi zambiri amitundu komanso "amamvetsera" mitundu yonse, mitundu ndi mafuko onse). Mawonetseredwe a hemophilia C amachotsedwanso kunja kwa chithunzi chachikulu, choncho m'zaka zaposachedwa achotsedwa pa mndandanda wa hemophilia.

Ndikoyenera kudziwa kuti mu magawo atatu a mabanja matendawa amapezeka (kapena amapezeka) kwa nthawi yoyamba, yomwe imapweteka makolo osakonzekera.

Nchifukwa chiyani matendawa amapezeka?

Wolakwa wake ndi jeni la congenital hemophilia, yomwe ili pa X chromosome. Wonyamulirayo ndi mkazi, ndipo iye mwiniyo sali wodwala, pokhapokha atakhala ndi magazi ochulukirapo, amakhalanso ndi msambo kapena amachiritsa mabala abwino (pang'onopang'ono, atachotsa dzino). Jini ndiloperewera, kotero sikuti aliyense amene mayi ake ndi wothandizira matendawa akudwala. Kawirikawiri mwayiwu umaperekedwa 50:50. Zimatuluka ngati abambo akudwala m'banja. Atsikana amakhala osamalira jini mosalephera.

Chifukwa chake hemophilia ndi matenda ammuna

Monga tanenera kale, jini ya hemophilia imakhala yambiri ndipo imagwirizanitsidwa ndi chromosome, yomwe imatchedwa X. Amayi ali ndi chromosomes iwiri. Ngati wina akukhudzidwa ndi jini, imakhala yofooka ndi yowonjezereka ndi yachiwiri, yomwe imakhala yaikulu kwambiri, kotero kuti msungwanayo amangokhala chonyamulira chomwe chimapititsa hemophilia, koma amakhalabe wathanzi. Zikuoneka kuti panthawi yomwe mayiyo ali ndi pakati, ma X-chromosome akhoza kukhala ndi jini lofanana. Komabe, pamene mwanayo amadziwika yekha (ndipo izi zimachitika pa sabata lachinayi la mimba), imakhala yosasintha, ndipo mimba yokhayokha imapezeka (kutuluka padera). Popeza chochitika ichi chikhoza kuyambitsidwa ndi zifukwa zosiyana, kawirikawiri palibe kafukufuku wongodzivulaza, kotero palibe chiwerengero cha nkhaniyi.

Chinthu china ndi amuna. Iwo alibe kachilombo kachiwiri ka chromosome, amalowetsedwa ndi Y. Palibe "X", koma ngati chodziwikacho chimaonekera, ndiye kuti chimayambira ndondomeko ya matendawo, osati nthawi yake. Komabe, popeza akadakali ma chromosomes awiri, mwayi wa chitukuko choterocho ndi chimodzimodzi mwa mwayi.

Zizindikiro za hemophilia

Zitha kuwonetseredwa ngakhale pa kubadwa kwa mwana, ngati chinthu chofanana m'thupi sichikusowapo, ndipo chingamveke patapita nthawi ngati pali vuto.

1. Kutaya magazi pokhapokha kulibe zifukwa zomveka. Kawirikawiri mwana amabadwa ndi ziboda zamagazi kuchokera m'mphuno, maso, mphukira, ndipo zimakhala zovuta kusiya magazi.
2. Hemophilia (zithunzi zikuwonetsa izi) zikudziwonetsera ngati kupanga mapangidwe akuluakulu a chiwonongeko chochuluka kuchokera ku zotsatira zosafunikira kwenikweni (mwachitsanzo, kukakamizira chala).
3. Kutuluka magazi kuchokera ku bala limene likuwoneka kuti lachiritsidwa.
4. Kuchulukanso kwa magazi m'mimba: mphuno, m'mphuno ngakhale pamene mukutsuka mano.
5. Kuwonongeka kwa magazi m'magulu.
6. Njira zamagazi mumkodzo ndi zinyenyeswazi.

Komabe, "zizindikiro" zotero sizikutanthauza kuti ali ndi haemophilia. Mwachitsanzo, nosebleeds ikhoza kuyankhula za kufooka kwa makoma a mitsempha ya magazi, magazi mu mkodzo - matenda a impso, ndi muchitetezo - pa chilonda. Choncho, maphunziro oonjezera amafunika.

Kuzindikira kwa hemophilia

Kuphatikiza pa kuphunzira anamnesis wodwalayo ndi kuyesedwa kwake ndi akatswiri osiyanasiyana, kafukufuku wa labotale akuchitika. Choyamba, kukhalapo kwazigawo zonse m'magazi ndi ndondomeko zawo zimatsimikiziridwa. Nthaŵi yomwe idatengere kuti yikani nyemba ya magazi imayikidwa. Kawirikawiri, mayeserowa amaphatikizidwa ndi kuphunzira DNA. Kuti mudziwe zambiri, zingakhale zofunikira kufotokozera:

• Thrombin nthawi;
• Osokonezeka;
• Prothrombin index;
• Mitundu ya fibrinogen.

Nthawi zina deta yapadera kwambiri imapemphedwa. N'zoona kuti si chipatala chilichonse chomwe chili ndi zipangizo zoyenera, choncho zimatumizidwa ku labotale ya magazi ndi kukayikira kwa hemophilia.

Matenda ophatikizidwa ndi hemophilia (chithunzi)

Hemophilia yowonjezereka ndi magazi omwe amagawidwa. Dzina lachipatala ndi hemoarthrosis. Zimayamba mofulumira, ngakhale kuti zimakhala zovuta kwambiri kwa odwala omwe ali ndi mitundu yambiri ya hemophilia. Iwo ali ndi magazi m'magazi opanda mphamvu zowoneka kunja, mwadzidzidzi. Mitundu yofewa chifukwa cha kukhumudwa kwa hemoarthrosis, kukhumudwa kumafunika. Mankhusu amakhudzidwa choyamba ndi omwe akuvutika maganizo, ndiko, bondo, ntchafu ndi chitsa. Wachiwiri pambali pake - mapewa, pambuyo pawo - goli. Zizindikiro zoyamba za hemoarthritisi zimawoneka kale ali ndi ana a zaka 8. Chifukwa cha kuvulala, odwala ambiri amalemala.

Chiwalo choopsa: impso

Matenda a hemophilia nthawi zambiri amachititsa maonekedwe a magazi mu mkodzo. Amatchedwa hematuria; Zimatha kuyenda mopweteka, ngakhale kuti chizindikirocho chimasokonezabe. Pafupifupi theka la ma hematuria akuphatikizapo ululu wowawa kwambiri. Kawirikawiri, colic yamphongo imayamba chifukwa chokankhira magazi m'magetsi. Ambiri mwa odwala omwe ali ndi hemophilia ndi pyelonephritis, amatsatiridwa ndi kuchuluka kwa zochitika - hydronephrosis, ndipo malo otsiriza amakhala ndi capillary sclerosis. Chithandizo cha matenda onse a impso ndi chovuta ndi zoletsa zina pa mankhwala: palibe chomwe chimathandiza kuchepetsa magazi, sangagwiritsidwe ntchito.

Kuchiza kwa hemophilia

Mwamwayi, hemophilia ndi matenda osachiritsika omwe amayenda ndi munthu moyo wake wonse. Osati kutulukira koma ndi njira yomwe mungapangire thupi kutulutsa mapuloteni oyenera, ngati sakudziwa kuchita izo kuchokera kubadwa. Komabe, zopindulitsa zamankhwala zamakono zimalola kukhala ndi thupi pamlingo umene odwala omwe ali ndi haemophilia, makamaka mu mawonekedwe oopsa kwambiri, angakhale ndi moyo wamba. Pofuna kuteteza kuvulaza ndi kutaya mwazi kumafuna kuyanjanitsa nthawi zonse zothetsera zifukwa zosowa. Iwo ali kutali ndi magazi a anthu opereka ndi zinyama zoperekedwa kuti apereke zopereka. Kuyamba mankhwala osokoneza bongo kumakhala kosatha monga njira zothandizira komanso zothandizira pakagwa opaleshoni kapena kupsinjika.

Mofananamo, odwala omwe ali ndi hemophilia ayenera nthawi zonse kukhala ndi physiotherapy kuti apitirize kugwira ntchito. Pankhani yochuluka kwambiri, amakhala oopsa, opaleshoni ya opaleshoni yopanga mafupa opanga opaleshoni kuti awachotse.

Popeza kuikidwa kwa mankhwala opangidwa pogwiritsa ntchito magazi opereka magazi, matenda a hemophilia amachititsa kuti munthu adziwe ngati ali ndi kachilombo ka HIV, matenda a cytomegalovirus, herpes komanso, makamaka, kachilombo ka HIV. Mosakayikira, onse opereka ndalama amayesedwa kuti azitetezedwa mwazi wawo, koma palibe amene angapereke chitsimikiziro.

Anapeza hemophilia

Nthaŵi zambiri, hemophilia imachotsedwa. Komabe, pali ziwerengero zina za milandu pamene zimawonekera kwa akuluakulu omwe sanakhalepo kale. Mwamwayi, milandu yotereyi ndi yosavuta kwambiri - munthu mmodzi kapena awiri pa milioni. Ambiri amadwala, pokhala ndi zaka zoposa 60. Nthawi zonse, hemophilia imapezedwa ndi mitundu yosiyanasiyana ya A. Ndizodabwitsa kuti zifukwa zomwe zinkawonekera zinapezeka mu oposa theka la odwala. Zina mwa izo, matenda opatsirana khansa, kumwa mankhwala ena, matenda a autoimmune, kawirikawiri - matenda, matenda aakulu, kutenga mimba mochedwa. Chifukwa chake ena adadwala, madokotala sakanatha kukhazikitsidwa.

Matenda achigonjetso

Nkhani yoyamba ya matenda omwe akupezeka ikufotokozedwa mu chitsanzo cha Mfumukazi Victoria. Kwa nthawi yaitali, iwo ankawoneka kuti ndiwo okhawo a mtundu wawo, popeza kale, ngakhale pafupi theka la zana pambuyo pa hemophilia amachitikira mwa akazi. Komabe, m'zaka za zana la makumi awiri ndi awiri, pakufika kwa chiŵerengero cha kulandira matenda a chifumu, mfumukazi yodabwitsayi silingaganizidwe: hemophilia, yomwe inabweranso pambuyo pobadwa, si nthano, sichidalira kugonana kwa odwala.