Hemophilia - ndi chiyani, ndi momwe mungapewe mavuto aakulu?

Kumvetsa hemophilia - kuti izi ndi zofunika kwambiri, chifukwa matendawa ndi osayenera. Matendawa ndi olowa. Matendawa ndi owopsa kwambiri. Ndikofunika kuti tidziwone poyambirira pa chitukuko, kuti tisabweretse vutoli.

Hemophilia - ndi chiyani?

Kuti mumvetse zomwe matendawa ali, tanthauzo la mawu ogwiritsidwa ntchito lidzakuthandizani. Kuchokera ku Chigriki, "haima" amatanthawuza "magazi," ndi "philia" amatanthawuza "kuledzera." Matendawa ndi otsika magazi. Amakumana ndi amuna, koma pali zosiyana (mtundu C). Amapezako magazi oipa kuchokera kwa mayi: iye ndi wonyamulira wa matendawa ndipo amamupatsa ana ake "bonasi". Mbiriyi ili ndi mayina a akazi akulu omwe anali kunyamula jeni la hemophilia. Mwachitsanzo, Mfumukazi Victoria ndi Duchess ya Hesse.

Kodi haemophilia imachokera bwanji?

Gulu la matendawa ndilopitirira. Ipezeka mu X chromosome. Cholowa cha jini lachibadwa chimagwirizanitsidwa ndi kugonana. Kuti muyambe kuwonetsa matendawa, mukufunikira ma X-chromosomes ndi kusintha. Uyu ndi "wokhala" wamkazi. Amuna ali ndi chromosomes X ndi Y. Komabe, pakuchita zonse zimachitika mosiyana. Mayi akakhala ndi mimba ndi mtsikana yemwe ali ndi ma chromosomes a mutation awiri, kuperewera kwa padera kumachitika kwa milungu iwiri. Izi zili choncho chifukwa chakuti mwana wosabadwayo sangathe kugwira ntchito.

Mtsikana akhoza kubadwa ndi chromosome imodzi yosinthika. Pankhaniyi, matendawa sadziwonetsera okha: jini yathanzi labwino sichimayambitsa matendawa. Cholowa cha hemophilia chikhoza kuwonedwa mwa anyamata. Mu thupi lachibadwidwe, palibe jini yochulukirapo mu Y chromosome, ndipo X-yokhala ndi kusintha kwabwino kumakula. Pa chifukwa chimenechi, anyamata amatha kulandira matendawa, ndipo hemophilia imakhala ndi khalidwe lopitirira malire.

Kodi n'chiyani chimayambitsa magazi oipa?

Matendawowa ndi owopsa kwambiri. Kuwonekera kwa mavuto omwe akutsatidwa kumakhala kosagwirizana kwambiri ndi siteji ya matendawa. Izi ndizoopsa kwambiri coagulability ya magazi ndi:

1. Zimayambitsa mphamvu ya placenta kumapeto kwa mimba.
2. Zingayambitse magazi ambiri panthawi ya kuvutika.
3. Kuchuluka kwa mano, opaleshoni ndi kuvulala kwakukulu zimayambitsa magazi.

Hemophilia - Mitundu

Kutseka magazi ndi chitetezo cha thupi. Pogwiritsa ntchito izi, fibrinogens, platelets ndi zinthu za plasma zimatenga mbali yogwira ntchito. Kuperewera kwa chimodzi mwa zinthu zimenezi kumayambitsa chisokonezo pa ntchito yonse ya coagulation. Malingana ndi chinthu chosowa, mitundu iyi ya hemophilia ndi yosiyana:

• A;
• B;
• C.

Malingana ndi kukula kwa matenda, matendawa amasiyana kwambiri:

1. Zovuta - ndi kutuluka kwa magazi kumachitika kawirikawiri. Komanso, iwo ndi otsika kwambiri.
2. Zomwe zili pakati - chifukwa zimadziwika mozama kwambiri za mawonetseredwe otentha.
3. Zowonjezera - ngati, mwachitsanzo, kutayika kochepa kwa magazi mwa mwana wakhanda, izi zimawonetseredwa ndi kutuluka magazi kuchokera mu umbilical, kukhalapo kwa mahematusi pamutu, melena ndi zina zotero. Pamene munthu akukula, matenda oterowo ndi oopsa kwambiri kuyambira ali mwana.

Hemophilia A

Pakuti mtundu uwu wa matenda umakhala ndi vuto la antihemophilic globulin - chinthu VIII. Mtundu umenewu umatengedwa kuti ndi wamtengo wapatali komanso wofala kwambiri. Amapezeka m'thupi la 85% pamene wodwalayo ali ndi jini ya hemophilia. Matenda amtunduwu amatsatiridwa ndi magazi ambiri. Pa chifukwa ichi nkofunika kumvetsetsa: hemophilia - ndi chiyani ndi zomwe zikuphatikizapo.

Mbali yapadera ya matendawa ndi kuphwanya gawo la plasma la hemostasis. Mwachidule, magazi samapezeka nthawi yomweyo atangodzivulaza. Ichi ndi chifukwa chakuti mapapu ndi mitsempha yambiri imagwira bwino. Pambuyo pa tsiku lomwelo atalandira chovulala, magazi ochulukirapo angayambe, omwe ndi ovuta kwambiri kuima. Vuto ili silikhoza kuthetsedwa tsiku limodzi.

Hemophilia B

Dzina lachiwiri la matendawa ndi Christmass matenda. Matendawa ali ndi kusowa kwa factor IX. Chipangizochi chimayambitsa Stuart-Prower. Mtundu uwu wa haemophilia kwa ana umapezeka 20% mwa matenda omwe amapezeka ndi ofooka a magazi coagulability. Matenda oterewa amapezeka m'modzi mwa anyamata okwana 30,000.

Hemophilia C

Ma subspecies awa ali ndi mawonetseredwe ake enieni. Ndizosiyana kwambiri ndi mtundu wamtundu wa matenda. Ma subspecies amenewa amachotsedwa ngakhale ku zochitika zamakono. Pa chifukwa ichi nkofunika kumvetsa hemophilia - ndi chiyani. Zili ndi mbali zosiyana siyana zomwe sizikuchitika mu matenda ena. Hemophilia yotere mwa akazi imapezeka nthawi zambiri monga amuna. Izi zili choncho chifukwa chakuti majini amatha kupititsa patsogolo. Palinso chidziwitso cha dziko lonse. Matendawa amakhudza Ayuda a Ashkenazi nthawi zambiri.

Zizindikiro za hemophilia

Mu matenda a mitundu A ndi B, chithunzi chofanana chachipatala. Pachifukwa ichi, zizindikiro zosasamala za magazi ndi izi:

1. Kukhalapo kwa zizindikiro zosiyanasiyana pamthupi. Amawonekere ngakhale pambuyo povunda pang'ono. Mankhwala oterewa ali ndi magawo osiyanasiyana. Pogwiritsa ntchito kuvulaza pali ululu waukulu.
2. Pa ma 80%, hemarthrosis amapezeka. Mgwirizanowo umakhala wotentha, wotentha, ndipo khungu likuwombera. Modziwa mobwerezabwereza, matenda a osteoarthritis amayamba kukula. Panthaŵi imodzimodziyo pangakhale kupotuka kwa mafupa a m'mimba ndi chigoba cha m'mimba, kupweteka kwa minofu, kupotoza kwa mapazi.
3. Kupuma kwa magazi kumene kumachitika atatha opaleshoni, kudula, kuchotsa dzino komanso kuvulala kwina.
4. Pafupipafupi 5% (makamaka pa odwala ali aang'ono) pali kupweteka koopsa .
5. Kuwonongeka kwa magazi m'thupi - kumaphatikizapo ululu waukulu ndi zizindikiro zina zofanana ndi kuchipatala cha peritonitis.
6. Pa 20%, milandu ya hematuria imapezeka. Matendawa angaperekedwe ndi zida za coal colic ndikupanga chitukuko cha pyelonephritis.
7. Mapangidwe a chiphuphu , opwetekedwa ndi zimphona zazikulu.
8. Kutuluka kwa m'mimba, komwe kumawoneka mu 8% mwa anthu omwe amavutika ndi coagulation. Zingwe zamtundu uliwonse zimakhala ndi zofooka ndi chizungulire.

Zizindikiro za magazi osauka mu mtundu wa hemophilia C ambiri odwala amalephera kufotokozedwa kapena osayesedwa konse. Anthu ena ali ndi magazi, mimba komanso maonekedwe a matupi. Ngakhale m'banja limodzi, omwe mamembala awo amavutika ndi matendawa, kuuma kwa mawonetseredwe a chipatala amasiyana kwambiri. Komabe, mosiyana ndi matenda a mitundu A ndi B, hemophilia C mwadzidzidzi kuchepa kwa minofu mu minofu ndizosowa kwambiri. Matenda a hemorrose amangochitika pokhapokha ngati akugwedezeka kwambiri.

Kuphatikizana kolakwika kwa magazi - choti uchite chiyani?

Ngati muli ndi chizindikiro chimodzi cha matenda, muyenera nthawi yomweyo kupeza thandizo lachipatala. Kuti atsimikizire kuti akudwala matendawa, dokotalayo amapereka mankhwala kwa wodwala kuti apeze njira zoterezi:

• kutsimikiza kuchuluka kwa zinthu zomwe zimagwiritsidwa ntchito m'magazi;
• prothrombin index;
• kudzipereka kwa nthawi yomwe magazi akutha ;
• miyambo;
• mlingo wa fibrojeni m'magazi ndi zina zotero.

Hemophilia imaonedwa kuti ndi yopanda malire - chithandizo cha matendawa chachepetsedwa kukhala mankhwala ochiritsira. Kuonjezera apo, osati gawo lapang'ono lomwe laperekedwa ku zakudya. Panthawi ya kuchulukitsidwa kwa matendawa, mankhwala ake ayenera kuchitidwa kuchipatala cha chipatala. Akatswiri a kliniki yoteroyo amadziwa hemophilia - ndi matenda otani. Wodwala yemwe wamuuza wodwalayo ayenera kukhala ndi "Bukhu la wodwala" naye. Bukuli likuphatikizapo zambiri zokhudza gulu la magazi a anthu, Rh factor. Amasonyezanso kuopsa kwa matendawa.

Mmene mungayimire magazi ndi osauka magazi coagulability?

Munthu akakhala ndi hemophilia, ayenera kusamala kwambiri. Inu simungakhoze kutenga mopepuka za thanzi lanu! Makhalidwe abwino pa nkhaniyi ndi awa:

1. Ngati mabalawo akuya, muyenera kusuntha ndi kutsata mankhwala othandizira. Kuwonjezera pamenepo, ayezi ayenera kugwiritsidwa ntchito pa malo owonongeka (sayenera kugwirizana ndi khungu, choncho ayenera kukulunga ndi thaulo lamapepala kapena chopukutira chapamwamba asanagwiritse ntchito).
2. M'londa ndi zong'onoting'ono zingapange ma cloti akuluakulu. Mwa iwo, magazi amafuula. Chifukwa cha zotchinga zoterozo, mabala akuwonjezeka. Pofuna kupewa izi, muyenera kuchotsa mosamala "zilonda". Komanso, dera limeneli liyenera kutsukidwa ndi yankho la penicillin. Pambuyo pake, bandeji yopangidwa ndi hemostatic ikugwiritsidwa ntchito pakhungu. Izi zimafulumizitsa machiritso ovulaza.

Mankhwala ndi osauka magazi coagulability

Mankhwala a hemophilia amawoneka ngati awa:

1. Wodwala ali jekeseni ndi zinthu zosasoweka. Chiwerengero cha njira zoterezi chingasinthe kuyambira 4 mpaka 8 patsiku.
2. Perekani jekeseni wa zokonzekera pamaziko a plasma.
3. Kuchotsa mthupi la wodwalayo kuti zikhale ndi zizindikiro zothandizira, dokotala akhoza kupereka plasmapheresis .
4. Hereditary hemophilia imayambitsa vuto la shuga, Reamberin kapena Polyglucin.

Pogwiritsa ntchito zikwama zamtunduwu, kutuluka kwa thumbalo kumagwiranso ntchito. Kutsekemera kwa magazi m'magawo ake ndi kupindulitsa kwake ndi makonzedwe a hormonal akuchitika. Popanda kulephera, chiwalo chokhudzidwacho chiyenera kuchepetsedwa panthawi ya mankhwala. M'tsogolomu, atatha kukonzanso, maphunziro ochiritsira thupi ndi physiotherapeutic manipulations akulamulidwa.

Kodi kudya ndi kusasaka magazi?

Zakudya zabwino zimakhudza kwambiri vuto la odwala. Pofuna kupewa kutuluka kwa magazi, odwala omwe ali ndi hemophilia ayenera kulimbikitsa zakudya zawo ndi chakudya:

• Mafuta a nyama (mafuta, mafuta anyama, kirimu);
• chiwindi (ubongo, impso, chiwindi);
• phula la buckwheat;
• zipatso za violet mtundu (buluu, buluu, mabulosi akuda ndi ena);
• nthochi ndi mango;
• tsabola wofiira, radish, kabichi wofiira ndi masamba ena;
• walnuts;
• nyemba, nandolo, soya ndi nyemba zina;
• katsabola, parsley, basil;
• mkate woyera.