I-hemophilia ezinjeni ukuswela amahlwili okuholela ekopha ngokweqile. Kumele kuthathwe ezinye izindlela zokugwema ukulimala enjeni, ngokumpontshelwa kudingekile uma kwenzeka kuphuma igazi kaningi noma ngesikhathi sokuhlinzwa uma kunesidingo.
I-hemophilia yisifo esizuzwe njengefa kuthinta iningi lezinja zesilisa, ngokuba ngabesifazane abathwali balesi sikhubekiso sezakhi zofuzo ngakho-ke abadlulisa lesi sifo.
Izinhlobo
Ezinye izinhlobo zithambekele kakhulu kunabanye ukuthola lesi sifo, futhi izinja ezithintekile kufanele zisuswe enzalweni yazo lapho zikhona noma zisazi nje. Kumele sikwazi lokho futhi Kube khona amacala emakati, yize iningi lalokhu kutholakala ukuthi kutholakala ukuthi kunesifo.
Kunezinhlobo ezimbili ze-hemophilia ezinjeni, uhlobo lwe-A no-B hemophilia, konke kuncike kufuzo oluthintekayo. Ukungasebenzi kahle kwegazi kubangela ukopha, kube nzima ukuyiyeka. Kwenzeka okufanayo nakubantu abahlushwa yiyo, kulokhu amadoda athinteka kakhulu.
Yiziphi izimpawu nezimpawu ezikhona ku-hemophilia?
Izimpawu zomtholampilo ziyahlukahluka futhi azidingi ukwelashwa okujwayelekile, ngaphandle kokulimala okulandelwa ukopha. Kwezinye izimo izimpawu kungenzeka ukuthi azikho nhlobo. Izinhlobo ezinzima kakhulu zenzeka zisencane kakhulu, futhi izinhlobo ezinzima kakhulu ze-hemophilia lapho usumdala zihlala zinesibikezelo esibi.
Izimpawu ze-hemophilia ezinjeni ziyahlukahluka, ukopha okuphindaphindayo, ukopha ngaphansi kwesikhumba, ukulinyazwa ngesikhathi sokujova umjovo wokugoma, ukopha ngesikhathi sokukhula kwamazinyo, ukopha emakhaleni, ubukhona begazi esitokisini noma emchameni, njll. Izimpawu ezinganakwa, ikakhulukazi uma zibandakanya izitho zangaphakathi, isibonelo umgudu wokuphefumula, zibeka impilo yesilwane engcupheni.
Uma kusolwa i-hemophilia, udokotela wezilwane uzosebenzisa izivivinyo ezithile ukunquma nokucacisa ukuxilongwa. Kukhona nesifo, la Isifo se-von Willebrand, eyenzeka ikakhulukazi ohlotsheni lwe-Doberman futhi ifana ne-hemophilia.
Yikuphi ukwelashwa okufanele kakhulu kwe-hemophilia ezinjeni? Alikho ikhambi le-hemophilia. Kwezinye izimo udokotela wezilwane uzoncoma ukuphathwa kwama-coagulants noma ukwelashwa okususelwa ku-vitamin K ukuzama ukulwa nemiphumela yalesi sifo.
Ngaphambi kwanoma iluphi uphawu lokugula enjeni yethu, kufanele siye kudokotela wezilwane ngokushesha okukhulu. Lokhu kuzokunikeza ukuxilongwa kwakho futhi kunqume izindlela zokwelashwa ezikhonjiswe kakhulu ngesilwane. Ngenja ye-hemophiliac, kuzodingeka ukuthatha izinyathelo zokuzivikela empilweni yansuku zonke, empeleni, zizoqapha ukuze zingazilimazi. Ukunakwa kufanele futhi kunikezwe ezinye izinja noma izilwane ukuze kungabikho ukuthi zingaba sengozini yokulunywa noma ukuklwebheka.
Luyini uhlobo lwe-hemophilia ezinjeni?
Uhlobo A hemophilia luhlobo lwakudala kakhulu ezinjeni. Kungenxa yokushoda kwe-coagulation factor (factor VIII) futhi kuholela ekopha okungatheni noma okuphawulekayo esilwaneni esincane. I-hemophilia yisifo esizuzwe njengefa futhi ikakhulukazi nge-chromosome yezocansi (X). Ngakho-ke, ikakhulukazi abesilisa abahlushwa yilesi sifo.
Ngaphezu kwemvelaphi yefa, i-hemophilia A nayo ingaba ngumphumela woshintsho «okuzenzekelayo»Kwi-chromosome yezocansi. Kulokhu, abazali abanayo i-chromosome "engajwayelekile" ngakho-ke abasidlulisi lesi sifo, noma kunjalo, lesi simo asivamile. Ngokunikwa ubunjalo besifo, kunconywa ukuthi izilwane ezithintekile zingazalanga.
Ziyini izimpawu zomtholampilo ezinjeni?
Zingahluka kakhulu, kepha okuvame kakhulu ukuphuma noma ukulimaza. Ezinye izinja ezithintekile kungenzeka zingabi nazo izimpawu zomtholampilo nganoma yisiphi isikhathi empilweni yazo. Ezimweni eziningi, izimpawu azicaci futhi azidingi ukwelashwa okukhethekile, ngaphandle kwalapho kwenzeka ukulimala okulandelwa ukopha.
Ngakolunye uhlangothi, izinhlobo ezinzima kakhulu zenzeka zisencane. Ukopha kungazenzakalela kunoma yisiphi isitho, izimpawu zomtholampilo zizoncika kakhulu endaweni okuyo. Uma ukopha kwenzeka emajoyintini noma emisipheni, kungahle kube nokukhubazeka. Zingavela futhi ngesikhathi sokulahleka kwamazinyo obisi (Izinyanga ezine kuya kweziyi-4). Igazi elisha lingabe seligobhoza liphuma emlonyeni womdlwane.
Ukopha kungaba khona kuphela ngesikhathi sokuhlinzwa (ngokwesibonelo, ukuthena) futhi kungabukeka njenge-hematoma noma i-scrotum esinqunyiwe. Ukopha okuyingozi kakhulu yilokho okubandakanya izitho zangaphakathi futhi izimpawu zomtholampilo zivame ukukhungatheka noma zinganakwa, okubeka impilo yenja engcupheni futhi kungenziwa ngenxa yokuthi amaplatelets aphansi. Ukuxilongwa kwe-hemophilia A kusekelwe Ukuzimisela kwe-clotting factor VIII egazini lenja. Ukuqedwa kokuhlolwa okuphelele kwe-coagulation kungafaki ezinye izimbangela ezivame kakhulu zokopha njenge-anticoagulant rodenticide poisoning.
Okwamanje alukho umuthi othize wokulwa ne-hemophilia A ezinjeni. Ukwelashwa ngakho-ke ikakhulukazi kunenkambiso. Kwezinye izimo, ukumpontshelwa igazi kungadingeka ekuphumeni kwegazi okukhuluNgenxa yalesi sizathu, kunconywa ukuthi wenze iqembu legazi lanoma iyiphi inja ene-hemophilia A.
Ucwaningo olwenziwa e-United States nguDavid A. Wilcox ngo-2013 lwabika ukwelashwa kwezinja ezintathu ezine-hemophilia A enamandla ngokujova i-vector yegciwane (...othwala isakhi sofuzo se-Factor VIII esinesici) kumaseli akho egazi. Izinja ezimbili kwezintathu zikwazile ukukhiqiza i-factor VIII eyanele ukunqanda ukopha izinyanga ezingama-30. Lo msebenzi kungenzeka uholele ekusetshenzisweni okusebenzayo kubantu nasezinjeni esikhathini esizayo esiseduze.
Yiziphi izindlela zokuvimbela okufanele zisetshenziswe?
Ezinjaneni ezine-hemophilia kunjalo kubalulekile ukuvimbela ukuphuma kwegazi. Inja kumele ihlale endaweni lapho kuncipha khona ingozi yokulimala, ngaphandle kokudlala ngokungazelelwe nezinye izinja, amakati noma izingane. Ngaphambi kwanoma yikuphi ukubonisana (ngisho nomjovo owodwa), udokotela wezilwane kufanele aziswe ngaso sonke isikhathi.
Inja yesilisa enesifo segazi isenofuzo olungalungile ku-X chromosome yayo. Akufanele izale kabushaNgaphandle kwalokho zonke izingane zazo zizophatha isakhi sofuzo esingalungile (kodwa ngeke zigule). Enjeni yesilisa, isakhi sofuzo esingalungile sivela kunina (ngaphandle kwesimo esingajwayelekile sokuguquka okuzenzekelayo). Ngakho-ke, kuzodingeka ukuthi ususe umama ekukhiqizeni futhi ulandele yonke inzalo yakhe. Kuzoba khona amadodana athile agulayo kanti amanye amantombazane azalelwa ngokwesivumelwano.