Isifo iVon Willebrand: siyini, singasikhomba kanjani nokuthi kwenziwa kanjani ukwelashwa

-Delile

• Izimpawu eziyinhloko
• Kunjani ukuxilongwa
• Yelashwa kanjani
• Ukwelashwa ngesikhathi sokukhulelwa
• Ingabe ukwelashwa akulungile enganeni?

Isifo sikaVon Willebrand noma i-VWD yisifo sofuzo nesofuzo esivezwa ukwehla noma ukungabikho komkhiqizo weVon Willebrand factor (VWF), onendima ebalulekile kwinqubo yokujiya. Ngokwesichibiyelo, isifo sika-von Willebrand singahlukaniswa sibe yizinhlobo ezintathu eziyinhloko:

• Thayipha 1, lapho kuncipha khona kancane ekukhiqizeni i-VWF;
• Thayipha 2, lapho into ekhiqizwayo ingasebenzi khona;
• Thayipha i-3, lapho kunokuntuleka okuphelele kwe-Von Willebrand factor.

Lesi sici sibalulekile ukukhuthaza ukunamathela kweplatelet ku-endothelium, kunciphise futhi kumise ukopha, futhi kuthwala i-factor VIII ye-coagulation, okubalulekile ukuvikela ukonakala kweplatelet ku-plasma futhi kuyadingeka ukuze kusebenze i-factor X nokuqhubeka kwe-cascade. ukwakha ipulaki leplatelet.

Lesi sifo siwufuzo kanye nofuzo, okusho ukuthi, singadluliselwa phakathi kwezizukulwane, noma kunjalo, singatholakala futhi lapho umuntu esemdala lapho umuntu enohlobo oluthile lwesifo sokuzivikela komzimba noma umdlavuza, ngokwesibonelo.

Isifo sikaVon Willebrand asinalo ikhambi, kepha ukulawula, okumele kwenziwe impilo yonke ngokuyalelwa ngudokotela, uhlobo lwesifo nezimpawu ezethulwayo.

Izimpawu eziyinhloko

Izimpawu zesifo sikaVon Willebrand zixhomeke ohlotsheni lwalesi sifo, kodwa-ke, okuvame kakhulu kufaka phakathi:

• Ukopha njalo nokuhlala isikhathi eside emakhaleni;
• Ukopha okuphindaphindiwe ezinsinini;
• Ukopha ngokweqile ngemuva kokusikwa;
• Igazi esitokisini noma emchameni;
• Ukulimala njalo ezingxenyeni ezahlukahlukene zomzimba;
• Ukwanda kokugeleza kokuya esikhathini.

Imvamisa, lezi zimpawu ziba nzima kakhulu ezigulini ezinesifo sohlobo lwe-von Willebrand sohlobo lwe-3, njengoba kunokushoda okukhulu kweprotheni elawula ukujiya.

Kunjani ukuxilongwa

Ukutholakala kwesifo sikaVon Willebrand kwenziwa ngokuhlolwa kwelabhorethri lapho kutholakala khona ukutholakala kweVWF neplasma factor VIII, ngaphezu kokuhlolwa kwesikhathi sokugaya kanye nenani lamaplatelet ajikelezayo. Kujwayelekile ukuthi ukuhlolwa kuphindwe izikhathi ezi-2 kuya kwezi-3 ukuze kutholakale ukuxilongwa okuyikho kwalesi sifo, kugwenywe imiphumela engeyinhle.

Ngoba kuyisifo sofuzo, ukwelulekwa ngezakhi zofuzo ngaphambi noma ngesikhathi sokukhulelwa kunganconywa ukubheka ubungozi bokuthi ingane izalwe inalesi sifo.

Mayelana nokuhlolwa kwelabhoratri, amazinga aphansi noma ukungabi khona kwe-VWF ne-factor VIII ne-aPTT ethathe isikhathi eside kuvame ukukhonjwa.

Yelashwa kanjani

Ukwelashwa kwesifo sikaVon Willebrand kwenziwa ngokuyalelwa ngudokotela wezifo zegazi kanye nokusetshenziswa kwama-antifibrinolytics, okwazi ukulawula ukopha kumlomo womlomo, impumulo, ukopha kanye nezinqubo zamazinyo, kuyanconywa.

Ngaphezu kwalokho, kungakhonjiswa ukusetshenziswa kwe-Desmopressin noma i-Aminocaproic acid ukulawula i-coagulation, ngaphezu kwe-Von Willebrand factor concentrate.

Ngesikhathi sokwelashwa, kuyalulekwa ukuthi abantu abanesifo i-Von Willebrand bagweme izimo eziyingozi, njengokuzijwayeza kwezemidlalo eyedlulele nokudla i-aspirin nezinye izidakamizwa ezingezona ukuvuvukala, njenge-Ibuprofen noma i-Diclofenac, ngaphandle kwezeluleko zezokwelapha.

Ukwelashwa ngesikhathi sokukhulelwa

Abesifazane abanesifo sikaVon Willebrand bangakhulelwa okujwayelekile, ngaphandle kwesidingo semithi, noma kunjalo, lesi sifo singadluliselwa ezinganeni zabo, ngoba siyisifo sofuzo.

Kulezi zimo, ukwelashwa kwalesi sifo ngesikhathi sokukhulelwa kwenziwa kuphela izinsuku ezi-2 kuya kwezingu-3 ngaphambi kokubeletha nge-desmopressin, ikakhulukazi lapho ukulethwa kungengxenye yokuhlinzwa, futhi ukusetshenziswa kwalo muthi ukulawula ukopha nokonga impilo yowesifazane kubalulekile. Kubalulekile futhi ukuthi lo muthi usetshenziswe kuze kube yizinsuku eziyi-15 ngemuva kokubeletha, njengoba amazinga e-factor VIII ne-VWF ehla futhi, engcupheni yokuphuma kwegazi langemva kokubeletha.

Kodwa-ke, lokhu kunakekelwa akudingeki ngaso sonke isikhathi, ikakhulukazi uma amazinga we-factor VIII evame ukuba ngama-40 IU / dl noma ngaphezulu. Yingakho kubalulekile ukuxhumana ngezikhathi ezithile nodokotela wegazi noma udokotela wababelethisi ukuqinisekisa isidingo sokusetshenziswa kwemithi nokuthi ngabe kukhona yini ubungozi kowesifazane nasenganeni.

Ingabe ukwelashwa akulungile enganeni?

Ukusetshenziswa kwemithi ehlobene nesifo sikaVon Willebrand ngesikhathi sokukhulelwa akulimazi ingane, ngakho-ke kuyindlela ephephile. Kodwa-ke, kuyadingeka ukuthi ingane yenze ukuhlolwa kofuzo ngemuva kokuzalwa ukuze kuqinisekiswe ukuthi inesifo noma cha, futhi uma kunjalo, ukuqala ukwelashwa.