Incazelo jikelele yalesi sifo
Kuyisifo esingajwayelekile segazi lapho kutholakala khona izinkinga zokuvala igazi.
Izinhlobo ze-hemophilia
I-Hemophilia yisifo sofuzo lapho kuguquka khona isakhi esisodwa se-X chromosome. Ngokuya ngokuthi isiphi isakhi sofuzo esiguqukile, kuhlukaniswa uhlobo oluhlukile lwesifo. IHemophilia inezinhlobo ezintathu: A, B, C.
- Uhlobo A - awekho amaprotheni akhethekile egazini: i-antihemophilic globulin, factor VIII yokujiya kwegazi. Lokhu kukhubazeka kofuzo kwenzeka kwiziguli ezingama-85% futhi kubhekwa njengokujwayelekile.
- Uhlobo B - i-factor IX ayisebenzi ngokwanele, ngenxa yokuthi inqubo yokwakhiwa kwepulagi yesibili yokuvala iphazamisekile.
- Thayipha C - I-hemophilia yalolu hlobo ivela ngenxa yenani elinganele le-XI coagulation factor. Uhlobo C luhlobo olungajwayelekile, oluthinta kakhulu ama-Ashkenazi amaJuda. Bobabili abesifazane nabesilisa bangagula ngayo. Okwamanje, lolu hlobo lukhishiwe ekuhlukanisweni ngenxa yokuthi izimpawu zalo zehluke kakhulu ezinhlotsheni ezimbili zokuqala.
Izimbangela zalesi sifo
Isizathu esiyinhloko sokuthuthukiswa kwe-hemophilia kubhekwa njengesici sokuzalwa.
Amacala ayivelakancane ngokweqile “hemophilia okuzenzakalelayo“. Uvela ngokuzumayo, yize umndeni ubengenaso lesi sifo phambilini. Kamuva, leli fomu le-hemophilia lidluliselwa ngokwesiko - ezingeni lofuzo. Ochwepheshe bezokwelapha abakazitholi izizathu eziqondile zokuthuthukiswa kwalesi sifo. Bakholelwa ukuthi lokhu ukuguqulwa okusha kofuzo.
Amathuba okuzuza njengefa hemophilia
Kakhulukazi abesilisa baphathwa yilesi sifo. Lesi sifo sidluliselwa kubulili besilisa ngendlela eyeqisayo (exhunywe kwi-X chromosome). Uma indodana izuze njengefa i-X - chromosome yomama, ayitholi i-hemophilia. Abesifazane banikezwa indima yokuba "umqhubi" noma umphathi, kepha kungahle kube nokuhlukile. Uma ubaba egula nge-hemophilia, futhi umama ethwala, khona-ke abazali abanjalo bangaba nentombazane ehlushwa yilesi sifo. Sekukonke, cishe amacala angama-60 abhalisiwe emhlabeni lapho amantombazane ehlushwa yi-hemophilia, futhi ayengabathwali.
Kunezinhlobo ezi-3 zokudluliswa kwe-hemophilia
- 1 Umama uphatha ufuzo, kepha ubaba uyindoda enempilo. Kulokhu, imiphumela emi-4 iyenzeka, ngamathuba angama-25%. Indodana enempilo noma indodakazi enempilo, indodana egulayo noma indodakazi ethwele ingase izalwe.
- 2 Umama uphilile, ubaba ugula yi-hemophilia. Kulesi simo, wonke amadodana azoba nempilo, nawo onke amadodakazi abe ngabathwali.
- 3 Umama uthwala isakhi sofuzo, nobaba uyagula. Kulokhu okuhlukile, kungaba nemiphumela emi-4: indodana enempilo, indodakazi egulayo, indodana egulayo, noma indodakazi yenethiwekhi. Umphumela ngamunye unamandla alinganayo.
Izimpawu ze-hemophilia
Ku-hemophilia, kuphuma ukopha ngokweqile, okwenzeka ngezikhathi ezithile ngokuhlala okuhlukile ngesikhathi sokulimala okuhlukahlukene, izinqubo zokwelashwa (ikakhulukazi imijovo yemisipha kanye nokungenelela kokuhlinzwa), nalapho kukhishwa amazinyo.
Izinsini noma ukopha ngamakhala kunzima kakhulu ukuyeka. Futhi, ukopha okuzenzakalelayo kungaqala.
Ngokulimala okuncane kanye nemihuzuko, kwakhiwa i-hematoma enkulu.
Isici esiyinhloko se-hemophilia ukuphuma kwegazi ngaphakathi - i-hemarthrosis. Lapho zivela ejoyintini, ukuhamba kahle kuphazamisekile, ukuvuvukala kwenzeka. Konke lokhu kuhambisana nobuhlungu obukhulu. Ngemuva kokuphuma kokuqala okunjalo, igazi elikujoyinti liyazincibilika lodwa nokusebenza kwejoyini kuyabuyiselwa. Kodwa ngokuphindaphinda, amahlule akha ku-capsule ehlangene ne-cartilage, embozwe izicubu ezixhuma. I-Ankylosis iqala ngenxa yalezi zinqubo.
Esinye isibonakaliso esibalulekile ku-hemophilia yisimo sokopha sekwephuzile, nokubambezeleka. Ukopha akuvuli ngokushesha ngemuva kokulimala, kepha ngemuva kwesikhathi esithile. Lokhu kungenxa yokuthi ekuqaleni ukopha kumiswa ngama-platelet, ukwakheka kwawo okungashintshiwe. Kungathatha kusuka emahoreni ayisithupha kuye kwayi-6 ngaphambi kokuba kuvuleke ukuphuma kwegazi - konke kuya ngobukhulu besifo.
Kusengaba khona igazi emchameni noma endleni. Isibonakaliso esinzima kakhulu nesibulalayo se-hemophilia ukuphuma kwegazi emgogodleni noma ebuchosheni.
Ezinganeni, i-hemophilia ingathatha isikhathi eside ukukhula. Lokhu kusebenza ezinganeni ezinceliswa ibele ngumama. Ngempela, obisini lwebele kunezinto ezikhethekile ezisekela ikhono legazi lokugcwala ngokujwayelekile. Ngakho-ke, uma umama encelisa ingane isikhathi eside, izimpawu zokuqala zizovela kamuva.
Amafomu we-hemophilia
Ngokuya ngobukhulu besifo, izinhlobo ezi-3 ze-hemophilia ziyabonakala.
- RџS•Rё ubumnene Ukopha kwe-hemophilia kwenzeka kuphela ngesikhathi sokuhlinzwa noma ngemuva kokuhlukumezeka okukhulu. Egazini, i-coagulation factor iqukethe inani elingu-5-25%.
- RџS•Rё inkambo elinganiselayo i-hemophilia clotting factor egazini iqukethe izinga elingu-1 kuye ku-5%. Izimpawu zokuqala zomtholampilo zingatholakala zisencane. Kuleli fomu, ukuphuma kwegazi kuhambisana nokulimala okulingene, futhi ngemivimbo emincane kuvela ama-hematomas abucayi.
- RџS•Rё ifomu elibi izifo, igazi liqukethe i-coagulation factor engaphansi kuka-1%. Kulokhu, i-hemophilia izibonakalisa ezinyangeni zokuqala zokuphila - ngesikhathi sokudabuka, ukuphuma kwegazi kuyavuleka, futhi emizameni yokuqala yokuhamba, kuvela ama-hematomas abukhali futhi abanzi (ngesikhathi sokukhasa, ngenxa yokubanjwa yinoma yiziphi izinto noma ukuwa).
Iziguli ezaziwayo kanye nabathwali be-hemophilia
INdlovukazi uVictoria ithathwa njengomthwali odume kakhulu we-hemophilia emlandweni. Ngaphezu kwalokho, akwaziwa ngokuthembekile ukuthi ngaziphi izizathu waba yilokho. Phela, ngaphambi kwalokhu, akekho noyedwa emndenini owayegula ngalesi sifo. Kunezinguqulo ezi-2.
Owokuqala uyavuma ukuthi ubaba wakhe kungenzeka ukuthi wayengomunye umuntu owayehlushwa i-hemophilia, hhayi uMbusi waseKent Edward Augustus. Kepha abukho ubufakazi obubhaliwe.
Ngakho-ke, kubekwe inguqulo yesibili - uVictoria unokuguquka kwezakhi zofuzo. Okungukuthi, wayenehlobo “lokuzenzakalelayo” le-hemophilia. Futhi ngokwesimiso esivamile, i-hemophilia yatholwa yindodana yakhe - uMbusi wase-Albany, uLeopold nabanye abazukulu nabazukulu.
I-hemophilia ibhekwa njengesifo samakhosi. Lokhu kuchazwa iqiniso lokuthi phambilini, ngenxa yokulondolozwa kwesihloko, imishado nezihlobo eziseduze yayivunyelwe. Ngakho-ke, amathuba okuba nezingane ezigulayo enkantolo akhule kakhulu.
Inganekwane ye-hemophilia
Kukhona inkolelo yokuthi umuntu one-hemophilia angaphuma igazi aze afe uma kulimala okuncane esikhunjeni. Lesi sitatimende asilona iqiniso, futhi kubantu abanjalo, ukuklwebheka nokusikeka okuncane akulona ingozi yokufa.
Izingozi ukungenelela kokuhlinzwa, ukukhipha amazinyo, ukugoma nokopha okuzenzekelayo emisipheni nasemithanjeni yegazi, okwenzeka ngenxa yezindonga zemithambo ezibuthakathaka ezigulini.
Ukudla okunempilo kwe-hemophilia
Nge-hemophilia, azikho iziqondiso eziqinile zokudla okufanele zilandelwe.
Ukubuyisa igazi nokwenza ngcono ukujiya, kuyadingeka ukudla ukudla okuqukethe usawoti we-phosphorus, calcium, amavithamini A, B, C, D. Into ebaluleke kakhulu ukuhlinzeka umzimba ngenani likavithamini K. elidingekayo i-vitamin coagulation (coagulation).
UVitamin K ungatholakala ngokudla isipinashi, ulethisi, u-anyanisi, izaqathe, ubhanana, ugarlic, ukhukhamba, utamatisi, amapheya, ama-apula, iklabishi (ikakhulukazi i-broccoli, iklabishi elimhlophe, ukholifulawa), upelepele oshisayo, ubhontshisi, isikhuphasha seqanda, ushizi, uwoyela webhotela , i-oats, i-turnip tops, isilimo esidliwayo esinamagatsha anamanzi.
Ukwenza ngcono isimo segazi, khuphula i-hemoglobin, uqinise izindonga zemithambo yegazi namazinga we-cholesterol aphansi, kubalulekile ukufaka ekudleni kwesibindi, inhlanzi enamafutha, izithelo ezisawolintshi, amantongomane, ipomegranate, ukwatapheya, ama-beet, ujusi we-cranberry, uju , iphalishi le-buckwheat, isanqante, i-apula kanye namajusi wesithombo sebhitrudi…
Ijusi le-beet kufanele liphuzwe lihlanjululwe ngesanqante noma ijusi le-apula. Okokuqala, kufanele ihlanjululwe ku-1 kuye ku-1, bese unciphisa kancane kancane ukuxubusha futhi wandise ukugcwala kwejusi le-beet.
Futhi, ungaphuza amanzi ahlanziwe, itiye eliluhlaza, itiye ngama-currants, i-viburnum noma ama-raspberries, i-rosehip decoction.
Imithi yendabuko ye-hemophilia
Ukuze zivimbele ukopha, iziguli zingathatha ukukhishwa kwembewu yamagilebhisi, ama-decoctions we-dioecious nettle, i-yarrow, i-astragalus, i-sophora yaseJapan, isikhwama somalusi, i-arnica, i-coriander, i-witch hazel, impande ye-dandelion. Lezi zitshalo zokwelapha zizosiza ekuqiniseni izindonga zemithambo yegazi, zikhulise ukujiya kwegazi futhi zithuthukise ikhwalithi yegazi.
Kufanele uthathe i-aspirin elinganiselwe neminye imithi yokwehlisa igazi, engadala ukopha.
Ukudla okuyingozi nokulimazayo kwe-hemophilia
- ukudla okunamafutha (iqukethe inani elikhulu le-cholesterol, ngaphezu kwalokho, amafutha avimba ukumuncwa kwe-calcium, futhi kubalulekile ekulinganiseni kwamaselula);
- izitsha ezithosiwe, ezinosawoti, ezibhemayo (lokhu kudla kuqukethe izinto eziguqula ukwakheka kwegazi kungabi ngcono, yingakho izinguquko ezimbi zenzeka emzimbeni wonke ngokuzayo);
- utshwala, i-sweet soda, iziphuzo zamandla (zonakalisa futhi zinciphise amaseli egazi, yingakho igazi lingakwazi ukubhekana nemisebenzi yalo);
- ukudla okusheshayo, amafutha e-confectionery, imikhiqizo eseqediwe, ukudla okusheshayo, ukugcina ukudla okusemathinini, amasoseji, amasoso nezinongo, kanye nokudla okunezithasiselo zokudla ezihlukahlukene (le "mikhiqizo" yakha izinhlanganisela ezinzima amangqamuzana egazi angakwazi ukuzisebenzisa ukuze ondle umzimba womuntu. , kodwa wona ngokwawo ahlanganisa ubuthi emzimbeni ngalezi zinto eziyingozi ze-ballast).
Qaphela!
Abaphathi abanacala nganoma yimuphi umzamo wokusebenzisa imininingwane oyinikiwe, futhi akuqinisekisi ukuthi ngeke ikulimaze wena uqobo. Izinto zokwakha azinakusetshenziselwa ukunquma ukwelashwa nokwenza ukuxilongwa. Njalo thintana nodokotela wakho onguchwepheshe!