Isifo seVon Willebrand: siyintoni, siyichonga njani kwaye senziwa njani unyango

Umxholo

• Iimpawu eziphambili
• Njani na isifo
• Unyango lwenziwa njani
• Unyango xa ukhulelwe
• Ngaba unyango alulunganga emntwaneni?

Isifo sikaVon Willebrand okanye i-VWD sisifo semfuza kunye nelifa elibonakaliswa kukuhla okanye ukungabikho kwemveliso yeVon Willebrand factor (VWF), enendima ebalulekileyo kwinkqubo yokujiya. Ngokwesilungiso, isifo sika-von Willebrand sinokuhlelwa kwiindidi ezintathu eziphambili:

• Uhlobo lwe-1, apho kukho ukuhla kwenxalenye yemveliso yeVWF;
• Uhlobo lwe-2, apho into evelisiweyo ingasebenzi;
• Uhlobo lwe-3, apho kukho ukusilela okupheleleyo kweVon Willebrand factor.

Le nto ibalulekile ukukhuthaza ukubambelela kweplatelet kwi-endothelium, ukunciphisa kunye nokuyeka ukopha, kwaye iphethe into VIII yokuqina, kubalulekile ukuthintela ukonakala kweplatelet kwiplasma kwaye kuyimfuneko ekusebenzeni kwento X kunye nokuqhubekeka kwe-Cascade. ukwenza iplagi yeplatelet.

Esi sifo sinemfuza kunye nelifa, oko kukuthi, sinokudlula phakathi kwezizukulwana, nangona kunjalo, sinokufunyanwa ngexesha lomntu omdala xa umntu enesifo esithile somzimba okanye umhlaza, umzekelo.

Isifo sikaVon Willebrand asinanyango, kodwa ulawulo, ekufuneka lwenziwe kubomi bonke ngokhokelo lukagqirha, uhlobo lwesifo kunye neempawu ezizisiweyo.

Iimpawu eziphambili

Iimpawu zesifo sikaVon Willebrand zixhomekeke kuhlobo lwesifo, nangona kunjalo, esona siqhelekileyo siquka:

• Ukopha rhoqo kunye nexesha elide;
• Ukuphindaphinda ukopha kwiintsini;
• Ukopha kakhulu emva kokusikwa;
• Igazi esitulweni okanye kumchamo;
• Ukutyumza rhoqo kwiindawo ezahlukeneyo zomzimba;
• Ukwanda kokuhamba sexesheni.

Ngokwesiqhelo, ezi mpawu ziba qatha kwizigulana ezine-von Willebrand yohlobo lwesifo se-3, njengoko kukho ukusilela okukhulu kweprotein elawula ukujiya.

Njani na isifo

Ukuchongwa kwesifo sikaVon Willebrand kwenziwa ngeemvavanyo zaselebhu apho kuvavanywa khona ubukho beVWF kunye neplasma factor VIII, ukongeza kuvavanyo lwexesha lokopha kunye nenani leeplatelets ezijikelezayo. Kuqhelekile ukuba uvavanyo luphindwe kabini ukuya kuma-3 amaxesha ukuze kufunyaniswe isifo, kuthintelwe iziphumo ezingezizo.

Kuba sisifo semfuza, ukucebisa ngemfuza ngaphambi okanye ngexesha lokukhulelwa kunokucetyiswa ukujonga umngcipheko wokuba umntwana azalwe enesi sifo.

Ngokunxulumene neemvavanyo zaselebhu, amanqanaba asezantsi okanye ukungabikho kwe-VWF kunye ne-factor VIII kunye ne-aPTT yexesha elide zihlala zichongwa.

Unyango lwenziwa njani

Unyango lwesifo sikaVon Willebrand lwenziwa ngokukhokelwa ngugqirha wegazi kunye nokusetyenziswa kwe-antifibrinolytics, ekwaziyo ukulawula ukopha kumlomo womlomo, impumlo, ukopha kunye neenkqubo zamazinyo, zihlala zicetyiswa.

Ukongeza, ukusetyenziswa kwe-Desmopressin okanye i-Aminocaproic acid yokulawula i-coagulation kungabonakaliswa, ukongeza kwi-Von Willebrand factor concentrate.

Ngexesha lonyango kuyacetyiswa ukuba abantu abanesifo iVon Willebrand bayaziphepha iimeko ezinobungozi, ezinje ngokwenza imidlalo egqithileyo kunye nokutya i-aspirin kunye nezinye iziyobisi ezingezizo ezokulwa nokudumba, ezinjengeIbuprofen okanye iDiclofenac, ngaphandle kwengcebiso yezonyango.

Unyango xa ukhulelwe

Abasetyhini abanesifo sikaVon Willebrand banokukhulelwa okuqhelekileyo, ngaphandle kwesidingo samayeza, nangona kunjalo, esi sifo sinokudluliselwa ebantwaneni babo, kuba sisifo semfuza.

Kule meko, unyango lwesifo ngexesha lokukhulelwa lwenziwa kuphela kwiintsuku ezi-2 ukuya kwezi-3 ngaphambi kokubeleka nge-desmopressin, ngakumbi xa ukuhanjiswa kungokokuhlinzwa, kunye nokusetyenziswa kweli yeza ukulawula ukopha kunye nokugcina ubomi bomfazi kubalulekile. Kukwabalulekile ukuba eli chiza lisetyenziswe ukuya kuthi ga kwiintsuku ezili-15 emva kokubeleka, njengoko amanqanaba e-VIII kunye ne-VWF ehla kwakhona, kunye nomngcipheko wokopha emva kokubeleka.

Nangona kunjalo, olu khathalelo alusoloko luyimfuneko, ngakumbi ukuba amanqanaba e-VIII ahlala engama-40 IU / dl okanye nangaphezulu. Kungenxa yoko le nto kubalulekile ukuba unxibelelane ngamaxesha athile nesazi ngegazi okanye ugqirha wezokubelekisa ukuqinisekisa isidingo sokusetyenziswa kwamayeza nokuba ngaba kukho umngcipheko kubo bobabini abasetyhini kunye nosana.

Ngaba unyango alulunganga emntwaneni?

Ukusetyenziswa kwamayeza ahambelana nesifo sikaVon Willebrand ngexesha lokukhulelwa akumonzakalisi umntwana, kwaye ke yindlela ekhuselekileyo. Nangona kunjalo, kubalulekile ukuba umntwana enze uvavanyo lofuzo emva kokuzalwa ukuze aqinisekise ukuba unesifo okanye hayi, kwaye ukuba kunjalo, ukuqala unyango.