I-Hemophilia - yintoni na, kunye nendlela yokuphepha iingxaki ezinzima?

Qondisisa i-hemophilia-ukuba oku kubaluleke kakhulu, kuba le nto isifo sinokungahambi. Le ntsholongwane ifa. Esi sifo sinobungozi: kubalulekile ukuyichonga kwisigaba sokuqala sophuhliso, ukwenzela ukuba ungayifaki imeko kwiimeko ezibucayi.

Hemophilia - yintoni na?

Ukuqonda oko oku kugula, intsingiselo yegama elisetyenzisiweyo liya kunceda. Ukususela kwisiGrike, elithi "haima" lithetha "igazi," kunye "nephilia" lithetha "umlutha." Esi sifo sibonakala nge-coagulability ephantsi yegazi. Baxhatshazwa ngabantu, kodwa kukho ukungafani (uhlobo C). Bafumana i-blood clotting embi kumama: nguye umthwali wesifo kwaye udlulisela "ibhonasi" koonyana bakhe. Imbali iqulethe amagama amabhinqa aphezulu abathwala i-hemophilia gene. Ngokomzekelo, uKumkanikazi waseVictoria kunye neDuchess yaseHesse.

I-haemophilia ilifa njani?

Imfuza ye-pathology iyagqitha. Itholakala kwi X chromosome. Ilifa le-gene pathological linxulumene nesondo. Ukuze uqale ukubonakalisa isifo, udinga ezimbini i-X-chromosomes ngokuguquka. Lo ngumfazi "obekwe". Amadoda nawo anama-chromosomes e-X nee-Y. Nangona kunjalo, ekusebenzeni konke kwenzeka ngokuhlukileyo. Xa ibhinqa likhulelwe intombazana enesibini eguquguqukayo i-chromosomes X, ukukhulelwa kwesisu kuqhutyelwa iiveki ezi-4. Oku kubangelwa kukuba i-fetus ekhulayo ayikwazi ukusebenza.

Intombazana inokuzalwa iguqule i-chromosome enye. Kule meko, esi sifo ngokwaso asibonakali: i-gene ephilileyo ayiyifuni ngokusesikweni isisombululo sesifo. Ifa le-hemophilia lingabonwa kubafana. Emzimbeni womzimba, akukho geni ephezulu kwi-Y chromosome, kwaye i-X-set with the change is intensively developing. Ngenxa yoko, amakhwenkwe azuze esi sifo, kwaye i-hemophilia inomlinganiselo ogqithiseleyo.

Yintoni ebeka ingozi kwigazi elibi?

Le meko yentsholongwane iyingozi kakhulu. Ukuvela kweengxaki ezilandelayo zidibaniswa ngokungenakulinganiswa kunye nesigaba sesifo. Le yingozi kangakanani i-coagulability embi yegazi:

1. Ikhupha i-detache ye-placenta ekukhulelweni kwexesha elide.
2. Ingabangela ukuphuma kwegazi ngexesha lomsebenzi.
3. Utyando lwamazinyo, imisebenzi yokuhlinzwa kunye nokulimala kanobom kuphazamisa ukulahleka kwegazi.

Hemophilia - Iindidi

Ukuqhawula ngegazi kuyindlela yokukhusela yomzimba. Kule nkqubo, i-fibrinogens, iiplatelets kunye neeplasma zizinto zithatha inxaxheba. Ukusilela kwesinye sezi zinto kubangela ukuphazamiseka ekusebenzeni kwenkqubo yonxibelelwano. Ngokuxhomekeke kwizinto ezingekhoyo, ezi ntlobo ze-hemophilia ziyahlukana:

• A;
• B;
• C.

Ngokwezinga lokubandezeleka kwekhosi yesi sifo, ezi zi-degrees of pathology ziyahluka:

1. Kulula - kunye nokuphuma kwegazi kwenzeka ngokungaqhelekanga. Ukongezelela, bancinci kakhulu.
2. Ubukhulu-obukhulu-kuba bubonakaliswe ubunzima obunomlinganiselo obonakalisiweyo obonakalisiweyo.
3. Ubunzima - ukuba, ngokomzekelo, i-coagulation ephosakeleyo yegazi kwintsana, oku kubonakaliswa ngokuphuma kwintambo yomlomo, ubukho beempawu ze-hematom ekhanda, melena njalonjalo. Xa usukhulile, isifo esinjalo asiyingozi nakakhulu kunasebuntwaneni.

Hemophilia A

Kuba olu hlobo lwesifo lubonakaliswe ngophotho lwe-antihemophilic globulin - into yeVIII. Olu hlobo lwesifo luqwalaselwa njengesiqhelo kunye nolona luqhelekileyo. Kufunyanwe kwi-85% yamatyala xa isiguli sinomdlavuza we-hemophilia. Olu hlobo lwesifo luhamba kunye nokuphuma kwegazi. Ngenxa yeso sizathu kubalulekile ukuqonda: i-hemophilia - yintoni na into ehambelana nayo.

Into ehlukile yalolu hlobo lwesifo kukuphulwa kwinqanaba le-plasma ye-hemostasis. Ngendlela elula, ukuphuma kwamanzi akusoloko kwenzeka emva kokulimala. Oku kungenxa yokuba iiplatelet kunye nezigaba ze-vascular zisebenza kakuhle. Emva komhla omnye emva kokufumana ukulimala, ukuphuma kwegazi okunzima kunokuqala, okunzima kakhulu ukuyeka. Le ngxaki ayikwazi ukusombulwa ngosuku olunye.

Hemophilia B

Igama lesibini lesi sifo sisifo se-Christmass. Esi sifo sibonakaliswe kukusilela kwe-IX. Le nxalenye ye-enzyme isebenza kwi-Stuart-Prower ubunzima. Olu hlobo lwe-hemophilia kubantwana lwenzeka kwi-20% yamatyala kunye nokuxilongwa kobuthakathaka begazi. Olu hlobo lentsholongwane luboniswa ku-1 kwabantwana abangama-30 000 abasanda kuzalwa.

Hemophilia C

Ezi ziqununu zinezibonakaliso zaso. Kuhluke kakhulu kwindlela yokudala yesifo. Ezi nkqutyana zitshintshwe ngaphandle kwoluhlu lwamanje. Ngenxa yoko kubalulekile ukuqonda i-hemophilia-yintoni na. Inomlinganiselo ohlukile owenzeki kwezinye iintlobo zesifo. I-hemophilia enjalo kubasetyhini ibonwa rhoqo njengabantu. Oku kubangelwa ukuba i-gene transfer isenziwa yinto ebalulekileyo. Kukho ukuphakama kobuzwe kwiimeko zezilwanyana. Esi sifo sichaphazela ama-Ashkenazi amaYuda kaninzi.

Iimpawu ze-hemophilia

Kwizifo zeentlobo ze-A kunye neB, umfanekiso ochanekileyo weklinikhi. Kule meko, iimpawu zokungafihli kwegazi zilandelayo zilandelayo:

1. Ubukho bee-hematomas ezahlukeneyo emzimbeni. Ziyabonakala nangemva kwesikhuhlane esincinci. Ezi zinyango ziba neendlela ezininzi zokuhambisa. Xa ugxininisa kwindawo yokuqhwala kukho intlungu enamandla.
2. Kwi-80% yamatyala, i-hemarthrosis ivela. Isibambano sitshisa, sitshisa, kwaye ulusu lukhuni. Ngokuphindaphindiweyo, i-osteoarthritis iqala ukuphuhlisa. Ngxeshanye kunye nalo kukho ukukhawuleka kwamathambo e-pelvic kunye nekholenda ye-vertebral, i-atrophy of muscles, deformation of feet.
3. Ukuqhekeza okwenzeka emva kokuhlinzwa, ukusika, ukuchithwa kwezinyo kunye nezinye ukulimala.
4. Kwi-5% yamatyala (ngokuphindaphindiweyo kwizigulane ezisebuncinci) kukho isifo esibuhlungu .
5. Ukuphazamiseka kwimizimba kwintsimi - ihamba neentlungu ezibi kunye nezinye iimpawu ezifana neklinikhi ye-peritonitis.
6. Kwi-20% yamatyala, i-hematuria iyabonwa. Le meko yentsholongwane iya kuhamba kunye nokuhlaselwa kwe-coal renal kwaye kubangele uphuhliso lwe-pyelonephritis.
7. Ukubunjwa kweengqungquthela , ezibangelwa zizidumbu ezinkulu.
8. Ukuphuma kwintsholongwane, okubonakalayo kwi-8% yamatyala kulabo abahluphekayo kwi-coagulation engafanelekanga. Amanqatha amnyama ahlala ehamba kunye nobuthathaka kunye neentlanzi.

Izibonakaliso zokungena kwegazi nge-hemophilia uhlobo C kwizigulane ezininzi zibonakaliswe ngokuthe tye okanye zingabonakali nhlobo. Abanye abantu baye baphala ngamanzi, i-menorrhagia kunye nokubonakala kwemivimbo emzimbeni. Kwaye kwintsapho enye, amalungu ayo abandezelekayo kwesi sifo, ubunzima beembonakaliso zekliniki ziyahluka kakhulu. Nangona kunjalo, ngokungafani nezifo zeentlobo ze-A kunye ne-B, i-hemophilia C ngokukhawuleza i-hemorrhages kwiimisipha ayinqabile. I-hemorthroses zenzeka kuphela kwimeko yokuxhatshazwa kwamalungu.

Ukungasebenzi ngokubi kwegazi - ntoni na?

Ukuba ubuncinane uphawu olunye lwesifo, kufuneka ufune ngokukhawuleza uncedo lwezokwelapha. Ukuqinisekisa ukuxilongwa, ugqirha uya kumisela isigulane uviwo olubandakanya iindlela zelabhoratri:

• ukuzimisela kwemali yezinto zokuhlala kunye negazi;
• Iprothrombin index;
• ukuzimisela kwexesha apho i- clotting yegazi yenzeka khona;
• mixtra;
• kwinqanaba le-fibrogen egazini njalo njalo.

I-Hemophilia ithathwa njengengenanto - unyango lwesi sifo luyancitshiswa ekubeni unyango lwezonyango. Ukongezelela, akukho nxaxheba encinci inikwe isondlo. Ngethuba lexesha lokuxhatshazwa kwesi sifo, unyango lwayo kufuneka lwenziwe esibhedlele kwiziko lempilo. Iingcali zecliniki enjalo ziyazi i-hemophilia-yintoni i-pathology. Isigulane esiye sadibana nesigulane kufuneka sibe ne "Incwadi yesigulane" naye. Olu phepha luqulethe ulwazi malunga neqela legazi legazi, iR Rh yayo. Kwakhona kubonisa ubunzima besifo.

Indlela yokumisa igazi nge-coagulability yegazi?

Xa umntu ene-hemophilia, kufuneka aqikelele. Awukwazi ukuthatha lula impilo yakho! Imigaqo yokuziphatha kulo mzekelo ilandelayo:

1. Ukuba amanxeba azinzulu, kufuneka uhlawule kwaye uphathe unyango oluthathe indawo. Ukongeza, iqhwa kufuneka lisetyenziswe kwindawo eyonakalisiweyo (akufanele idibane kunye nesikhumba, ngoko kufuneka ihlanganiswe ngetayileni yecawa okanye ithawula elincinci ngaphambi kokusetyenziswa).
2. Kwisilonda kunye nemijelo ingenza amaqhezu amakhulu. Kuzo, igazi liyavutha. Ngenxa yamanqatha anjalo, amanxeba akhula. Ukuze ugweme oku, kufuneka uwasuse ngokucophelela "izibonda". Ngaphezulu, le ndawo kufuneka ihlanjwe ngesisombululo se-penicillin. Emva koko, i-bandage ebanjwe ngumbumba we-hemostatic isetyenziswe elukhumbeni. Oku kukhawuleza ukuphilisa isilonda.

Amayeza kunye ne-coagulability yegazi

Ulwaphulo lwe-hemophilia lubonakala kanje:

1. Isigulane sijojowe ngezinto zokulahleka ezingekho. Inani leenkqubo zinokuhluka ukusuka kwi-4 ukuya kwi-8 ngosuku.
2. Nika iilenki zamalungiselelo ezenziwe nge plasma.
3. Ukususa kwiimpawu zomzimba zesigulane kwiimeko zokugulisa, ugqirha unokumisela i-plasmapheresis .
4. I-hemophilia yeHereditary inikezela isisombululo se-glucose, iReamberin okanye i-Polyglucin.

Nge-hemarthroses, ukugqitywa kwesikhwama sokubambisana kwandiswa. Ukuphefumlelwa kwezinto ezinokugawula igazi kunye nokucebisa kwayo kunye nokulungiswa kwama-hormone kwenziwa. Ngokuqinisekileyo, inyathelo elichaphazelekayo kufuneka lingenakunyuswa ngexesha lonyango. Kwixesha elizayo, emva kokuvuselelwa, ukuqeqeshwa okusemzimbeni kunye ne-physiotherapeutic manipulations zichazwe.

Yintoni eyokutya kunye negazi elibi?

Isondlo sinomphumo onamandla kwimeko yezigulane. Ukuthintela ukuvela kwegazi, izigulane ezine-hemophilia kufuneka zicebise ukutya kwazo ngokutya okunjalo:

• amafutha evela kwizilwanyana (ibhotela, isinqumelo, ukhilimu);
• isibindi (ubuchopho, izintso, isibindi);
• ibhakede;
• amajikijolo ombala we-violet (blueberry, blueberry, blackberry kunye nabanye);
• iibhanana kunye nemango;
• pepper, obomvu, iklabishi ebomvu kunye neminye imifuno;
• i-walnuts;
• iimbotyi, iifista, ii-soybean nezinye iimbotyi;
• i-dill, i-parsley, i-basil;
• isonka esimhlophe.