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Ukopha okuBomi esichengeni kwii-Blood Thinners okanye kwi-Hemophilia

Amanani kaxakeka ayohluka ngokwamazwe. Eli phepha lisebenzisa u-911, inani laseMelika naseKhanada. Amanye amazwe amaninzi asebenzisa i-112, 999, okanye elinye inani. Fumana inombolo yelizwe lakho.

Xa umntu othatha imithi ye-blood thinners (** i-anticoagulants okanye i-antiplatelet drugs ) okanye ehlala enesifo sokopha ( I-Hemophilia A, i-Hemophilia B, okanye i-von Willebrand Disease ) unobungozi, ukuwa, intloko, okanye ukopha kwangaphakathi, amaxesha amisiweyo e-clotting ayisebenzi. Ngenxa yokuba isakhono somzimba sokwenza umnatha wefibrin ozinzileyo okanye iplagi yeplatelet ivalwe ngokwekhemikhali okanye ayikho ngokwemfuza, kwanokukrazuka okuncinci kwesikhumba sentloko, ukopha, okanye iqhuma elibonakala lincinci entloko kunokuqhubela phambili ukuya ekuphumeni okubulalayo okanye ukuxinezeleka kwengqondo. Uncedo lokuqala lufuna ** uxinzelelo olwandisiweyo, olungathinteliyo oluthe ngqo (ubuncinci i-15 ukuya kwi-30 imizuzu eqhubekayo ngaphandle kokukroba), elawulwa yi-** World Federation of Hemophilia (WFH) "FACTOR FIRST / Treat First, Scan Second" umgaqo **, kunye nesaziso esiphuthumayo esibhedlele kunye ** negama elichanekileyo kunye nexesha lokugqibela lokulungiselela i-dose yexesha kwi-blood thinner thinner i-conductors I ambulance ide yafika.

Kwi-Hemophilia, FUZA INTO YOKUQALA NGOKUNGOKO NGAMVA phambi kokuba ulinde ii-X-reyi okanye i-CT scan.

Ukuba umntu one-Hemophilia A, i-Hemophilia B, okanye i-von Willebrand Disease unokulimala entloko, intamo okanye umqala, ukulimala komqolo, ukwenzakala kwesisu, okanye ukopha okukhulu kwamalungu / imisipha, owona mgaqo ubalulekileyo wokusinda kwi-World Federation of Hemophilia (WFH) kunye neNational Bleeding Disorders (WFH) kunye neNational Bleeding Disorders (ACNDF) faka i-clotting factor ye-emergency clotting factor ye-emergency concentrate ekhaya okanye kwi-ambulensi NGOKUNGOKO, kwaye basoloko bezisa i-vials zabo zasekhaya kunye ne-Emergency Care Letter esibhedlele. Ungaze uvumele isebe likaxakeka ukuba lishiye isigulana se-hemophilia sihleli kwigumbi lokulinda okanye silinde iziphumo ze-CT scan yentloko phambi kokuba kufakwe into; ukunyusa amanqanaba e-clotting factor ukuya kwi- \(80\%\text{ ukuya ku-}100\%\) ngokukhawuleza kuphelisa ubuchopho obubulalayo okanye ukopha kwangaphakathi ngelixa kulungiselelwa iskeni.

Yenza oku ngoku

  1. Faka uxinzelelo oluqhubekayo, oluqinileyo oluthe ngqo kulo nakuphi na ukuphuma kwegazi kwangaphandle kwi-15 epheleleyo ukuya kwimizuzu engama-30 ngaphandle kokuphakamisa i-gauze ukuze uhlolisise. Cofa phantsi ngamandla ngqo kwindawo yokopha usebenzisa i-gauze eyinyumba, i-hemostatic dressing (efana ne-kaolin okanye i-chitosan gauze), okanye ilaphu elisongiweyo elicocekileyo. Musa ukuphakamisa ilaphu rhoqo kwimizuzu emi-2 ukubona ukuba kuyekile ukopha na: kwisigulana esikwizithinteli-gazi okanye esinehemophilia, ukuphakamisa iphedi kukrazula iplagi eethe-ethe eyenza iplagi kwaye usete iwotshi yokopha ibe ngunothi. Ngobomi obusongela ukopha kwelungu elingayi kuyeka ngoxinzelelo oluthe ngqo, faka i-windlass tourniquet \(5\text{ ukuya }8\text{ centimeters}\) (\(2\text{ ukuya }3\text{intshi}\)) ngaphezu kwenxeba (bona ukopha).
  2. Fowunela inombolo yakho kaxakeka yasekuhlaleni ngokukhawuleza ukopha okukhulu kwangaphandle, nayiphi na intloko / intamo / isifuba / ukwenzakala kwesisu, okanye iimpawu zokopha kwangaphakathi. Cofa u-911 eUnited States naseKhanada, 112 kulo lonke i-European Union, 999 eUnited Kingdom, okanye ubone zonke amanani kaxakeka. Xela ngokucacileyo i-dispatcher ** igama elichanekileyo leyeza ** (umzekelo, "Apixaban," "Rivaroxaban," "Dabigatran," "Warfarin," okanye "Clopidogrel") okanye **ingxaki yokopha ngokuchanekileyo ** ("I-Hemophilia Enzima A ene-inhibitors").
  3. Ukuba isigulana sineHemophilia okanye i-von Willebrand Disease kwaye sinogxininiso lwekhaya, faka ithamo likaxakeka NGOKU. Landela iprotocol yesigulana yeHemophilia Treatment Centre (HTC) engxamisekileyo ukuze uhlengahlengise kwaye ufakele Factor VIII, Factor IX, von Willebrand factor concentrate(okanye i-Factor*) okanye i-Factor***(okanye i-Factor*) ngokukhawuleza lawula intranasal desmopressin [DDAVP] ukuba imiselwe i-Hemophilia A epholileyo okanye i-Type 1 von Willebrand isifo). Ukupakisha **2 ukuya kwii-3 iibhokisi ezongezelelweyo zogxininiso lwasekhaya lwesigulane ** kwisikhwama esifakwe kwi-ambulensi, njengoko izibhedlele zoluntu zihlala zingagcini yonke into ethile okanye imveliso ye-inhibitor.
  4. Ukuba isigulana esinegazi okanye esinehemophilia sibethe intloko yaso (nokuba kancinci sineempawu zero), yiya kwiSebe likaNgxamiseko ngoko nangoko ukuze ufumane i-Head CT scan. I-Intracranial hemorrhage ngoyena nobangela uphambili wokufa kwi-hemophilia kunye nezigulane ezine-anticoagulated. Ingqondo ecothayo ye-venous iyopha (subdural hematoma) ihlala inexesha elithi "ikhefu elicacileyo" apho umntu eziva elungile kangange-100 leepesenti iiyure ezininzi emva kokungqubeka ngentloko kwikhabhathi okanye ukutyibilika emgangathweni, ngaphambi kokuba ozele ngequbuliso, agabhe, kwaye abandezeleke ngokubulala ubuchopho.
  5. Bhala ixesha elichanekileyo lewotshi ipilisi yokugqibela ye-blood thinner yaginywa (okanye i-injection yokugqibela engaphantsi kwe-subcutaneous inikezelwe). Oogqirha besibhedlele kunye noosokhemisti bafuna iyure echanekileyo yedosi yokugqibela ** ye-apixaban, i-rivaroxaban, i-edoxaban, i-dabigatran, i-enoxaparin, okanye i-warfarin yokukhetha kunye ne-dose ye-proversal antithrombin-Factoral echanekileyo I-Complex Concentrate [4F-PCC], i-Andexanet alfa, i-Idarucizumab, okanye iProtamine).
  6. Gcina isigulana silele sithe tyaba, sishushu phantsi kweengubo ezomileyo, yaye akukho nto ithethwayo ngomlomo (NPO). I-Hypothermia (ubushushu bomzimba obungaphantsi kwe-\(35^\circ\text{C}\) okanye i-\(95^\circ\text{F}\)) ikhubaza nayiphi na iplatelet ne-enzyme eseleyo. Musa ukunika ukutya, amanzi, okanye naziphi na izithomalalisi zentlungu ze-NSAID (ungaze unike i-aspirin, ibuprofen, okanye i-naproxen).

Uvavanyo olungxamisekileyo kunye nesigqibo sokubuyisela umva ukopha

Sebenzisa le tshati yeklinikhi ukulungelelanisa ukulawulwa kwangoko ukopha ekhaya, ukutshintshwa kwezinto, kunye nokusebenza kwe-antidote esibhedlele.

flowchart TD
    Start["Umothuko, Impembelelo yeNtloko, okanye ukopha okuSebenzayo<br/>kwiSigulana kwi-Blood Thinners<br/>OKANYE ne-Hemophilia / von Willebrand Disease"] --> SiteCheck{"Inyathelo loku-1: Jonga iMimandla yeNgozi ye-Anatomical<br/>1. YIPHI na intloko, ubuso, iliso, intamo, umqala okanye ukwenzakala komqolo?<br/>2. Isifuba, umqolo, isisu, i-pelvis, okanye intlungu yehip/groin?<br/>3. Ukugabha igazi, ilindle elimnyama, okanye igazi lokukhohlela?<br/>4. Ukopha kwenxeba eliphuma ngaphandle okanye elingalawulekiyo?"}

    SiteCheck -->|"EWE: Nasiphi na isi-1 esikhoyo (Nokuba iqhuma lentloko libonakala lincinci)"| LifeThreat["UKOPHA OKUSEMNGENI OKANYE UMNGCIPHEKO OMKHULU:<br/>Fowunela 911 / 112 / 999 Ngoko nangoko"]

    SiteCheck -->|"HAYI: Wopha ngeempumlo, ukopha emlonyeni, ukusikwa kancinci,<br/>okanye ukopha kwamalungu okukwadwa/imisipha"| LocalCare["I-Hemostasis ekujoliswe kuyo + i-Triage engxamisekileyo:<br/>- Uxinzelelo oluqhubekayo oluthe ngqo 15-30 mins (AKUKHO PEEKING)<br/>- Tranexamic acid (TXA) yokuhlamba umlomo / ipilisi ukuba imiselwe<br/>- R.I.C.E. + Ukungeniswa kweFactor ngoko nangoko kukopha komdibaniso/kwemisipha"]

    LifeThreat --> DisorderType{"Inyathelo lesi-2: Ngaba isigulana si<br/>neHemophilia / vWD OKANYE sithatha i-Blood Thinners?"}

    DisorderType -->|"Isifo sokopha esizuzwa njengelifa:<br/>Hemophilia A, Hemophilia B, okanye vWD"| FactorFirst["UMGAQO A: 'UMBONO KUQALA' (Nyanga Phambi kokuSkena)<br/>1. Ukufakela i-dose engxamisekileyo ye-Factor VIII / IX / vWF / rFVIIa NGOKU <br/> (Ithagethi ye-80-100% yomsebenzi wentloko / intamo / i-GI / i-trauma enkulu) <br/> 2. Pakisha iibhokisi zeemeko zekhaya zesigulana + Ileta kaNgxamiseko ye-HTC<br/>3. Faka uxinzelelo oluqhubekayo oluthe ngqo / i-tourniquet ukuba ukopha kwangaphandle"]

    DisorderType -->|"I-Prescription Blood Thinner:<br/>Warfarin, DOAC (Apixaban/Rivaroxaban/Dabigatran),<br/>Heparin/Enoxaparin, okanye iClopidogrel/Ticagrelor"| ReversalPath["UMGAQO B: ISILUMKISO SOKUBUYISWA KOMTHETHO<br/>1. Uxinzelelo oluqhubekayo oluthe ngqo (i-hemostatic gauze) / i-tourniquet<br/>2. Chonga INGCACISO yegama lechiza + INGCACISO yeyure yedosi yokugqibela<br/>3. Isilumkiso se-EMS kunye ne-Hospital Pharmacy ukulungiselela i-IV Antidote: <br/> - Warfarin -> 4-Factor PCC + IV Vitamin K <br/> - Dabigatran -> Idarucizumab (Praxbind) <br/> - Apixaban / Rivaroxaban -> Andexanet alfa C okanye 4-Factor PC"]

    LocalCare --> PersistCheck{"Ngaba ukopha kuyaqhubeka kwimizuzu engama-20 ukuya kwengama-30<br/>OKANYE kukudumba kwamalungu/izihlunu kwinyonga, ithanga, ithole, okanye umphambili?"}
    PersistCheck -->|"EWE"| LifeThreat

Icandelo 1: Ulawulo olungxamisekileyo lokopha kumyalelo kagqirha we-Blood Thinners (Anticoagulants & Antiplatelets)

Izigidi zabantu abadala zisela yonke imihla amayeza okulwa ne-antithrombotic fibrillation ye-atrial, i-deep vein thrombosis, i-pulmonary embolism, iivalvu zentliziyo, okanye i-coronary artery stents. Ukwazi **loluphi udidi lwechiza ** isigulana esithathayo kubalulekile kuba iklasi nganye ibhloka inyathelo elahluke ngokupheleleyo lokujika kwaye lifuna i-antidote ethile yesibhedlele.

I-Anticoagulant eBanzi kunye ne-Antiplatelet Reversal Table (ACC / ASH / ISTH Guidelines)

Udidi lwaMayeza & naMagama enziwe ngabenzi / eeBrand Njani Ibhloka Ukuvala Isiqingatha sobomi (Ihlala ixesha elide kangakanani iyasebenza) ISibhedlele esiKhethekileyo sokuBuyisa uBuyiselo loNyango oluNgxamisekileyo Ulwazi olubalulekileyo lwe-Pre-Hospital ukunika iParamedics
Abachasi beVithamin K (VKAs):
Warfarin (Coumadin, Jantoven, Marevan), Acenocoumarol, Phenprocoumon
Iibhloko zesibindi se-Vitamin K-exhomekeke kwi-clotting **Iizinto II, VII, IX, kunye ne-X **. ** Ixesha elide kakhulu: i-36 kwiiyure ze-72 ** (ingozi yokuphuma kwegazi ihlala kwi-3 kwiintsuku ze-5 emva kokuyeka iipilisi). ** I-4-Factor Prothrombin Complex Concentrate (4F-PCC, Kcentra/Beriplex/Octaplex)** inikwe i-IV (ibuyisela i-INR ku **15 imizuzu ) ** PLUS nge-Intravenous Vitamin K (\(5\text{ ukuya }10\text{ mg}\)). (IPlasma eNqandisiweyo eNtsha [FFP] kuphela ukuba iPCC ayifumaneki.) Ingxelo yakutshanje **inqanaba lovavanyo lwegazi lwe-INR ** ukuba liyaziwa, idosi yemihla ngemihla, kwaye kutheni isigulane sithatha i-warfarin (umzekelo, i-valve yentliziyo yomatshini vs. AFib).
I-Thrombin eNgqo (Factor IIa) Inhibitor (DOAC):
Dabigatran (Pradaxa)
Ngqo ubophelela kwaye iibhloko esebenzayo Thrombin (Factor IIa). ** I-12 kwiiyure ze-17 ** (ukuya kwii-24 ukuya kwii-36 + iiyure kwisifo sezintso). Idarucizumab (Praxbind): Iqhekeza le-antibody le-monoclonal elenziwe ngumntu (\(5\text{gram}\) IV) eyenza i-dabigatran ingasebenzi ngokupheleleyo phakathi imizuzu. (I-Hemodialysis inokucoca i-dabigatran ngaphandle kwegazi.) Ingxelo ** iyure echanekileyo ye-capsule yokugqibela iginywe ** kwaye ingaba isigulane sinesifo esingapheliyo sezintso. (Qaphela: I-Dabigatran ayinakugcinwa ngaphandle kwe-blister/ibhotile yayo yoqobo.)
I-Direct Factor Xa Inhibitors (DOACs):
Apixaban (Eliquis), Rivaroxaban (Xarelto), Edoxaban (Savaysa/Lixiana), Betrixaban
Ngqo ubophelela kunye neebhloko zivuliwe Factor Xa. **8 kwiiyure ze-15 ** (ixesha elide kwizintso okanye ukukhubazeka kwesibindi). Andexanet alfa (Andexxa / Ondexxya) (recombinant decoy Factor Xa protein) OKANYE idosi ephezulu ye-4-Factor PCC (\(25\text{ ukuya }50\text{ units/kg}\)). (Amalahle avulwe ngomlomo anganikwa kwi-ER ukuba ipilisi ibiginywe kwiiyure ezi-2.) Iyure echanekileyo yepilisi yokugqibela ibalulekile: I-Andexanet alfa dosing (idosi ephantsi vs. idosi ephezulu) ixhomekeke ngqo kumandla e-milligram kunye nokuba ipilisi yokugqibela yathathwa \(<8\text{ hours}\) okanye \(\ge 8\text{ hours}\) ago.
IHeparin (Itofwa):
Enoxaparin (Lovenox/Clexane), Dalteparin, IHeparin engaqhawukiyo, Fondaparinux
Yenza i-antithrombin inhibit ** Factor Xa kunye neFactor IIa **. **I-Enoxaparin: iiyure ezi-4 ukuya kwezi-7 ** (isitofu esingaphantsi kwesikhumba). Iprotamine sulfate inikwe ngokucothayo IV (ibuyisela umva i-100% ye-heparin engaqhawukiyo kunye ne- - 60% ukuya kwi-70% ye-enoxaparin). Ingxelo ** idosi ye-milligram echanekileyo kunye nexesha lewotshi ** yesitofu sokugqibela solusu lwesisu.
Amachiza eAntiplatelet (P2Y12 Inhibitors & Aspirin):
Clopidogrel (Plavix), Prasugrel (Effient), Ticagrelor (Brilinta), Aspirin
Ikhubaza ngokusisigxina igazi ** iiplatelets** zabo bonke ubomi be-7-to-10-day (engenakuguquleka ngaphandle kwe-ticagrelor). Isiphumo sithatha iintsuku ezi-5 ukuya kwezili-10 de umongo wethambo wenze iiplatelet ezintsha kraca. ** I-Desmopressin (i-DDAVP) **, ** i-Tranexamic Acid (TXA) **, kwaye ijoliswe ** I-Platelet Transfusion ** i-bleeds yokuhlinzwa * (Qaphela: i-Ticagrelor ijikeleza egazini kwaye ivimba iiplatelet ezixutywe nge-24h; i-antidote yophando yi-bentracimab) *. Xelela oogqirha ukuba isigulane sine- **yamva nje i-coronary artery drug-eluting stent ** (ibekwe phakathi kwe-3 yokugqibela kwiinyanga ze-12), njengoko ukumisa ii-antiplatelet ezimbini kunomngcipheko we-stent clotting.

Umthetho othi "Libazisekile ukopha" emva kweNqanaba lentloko kwi-Blood Thinners

Esinye sezizathu eziqhelekileyo zokufa okunokuthintelwa kubantu abadala asebekhulile bathatha ** i-apixaban, i-rivaroxaban, i-dabigatran, i-warfarin, okanye i-clopidogrel ** kukuwa kwenqanaba elincinci lomhlaba okanye ukubethelwa kwentloko ngomnyango wemoto, i-shelf, okanye udonga apho umntu ** engalahlekelwa zingqondo kwaye enentloko ye-zero emva koko . - Kutheni lo ingumgibe: Ngaphakathi kokakayi, uxinzelelo oluncinci olusezantsi imithambo evala imithambo yolula ukunqumla isithuba esiphakathi kobuchopho kunye ne-dura mater eyomeleleyo yangaphandle. Ukungcangcazela kancinci kukrazula omnye wale mithanjana mincinci. Kumntu one-clotting eqhelekileyo, i-vein itywina ngemizuzu emi-2. Kumntu okwigazi elincinci, umthambo uphuma kancinci kancinci wehla ngethontsi kangangeeyure ** ezi-3 ukuya kwezi-24. - IProtokholi eSinyanzeliso: Imigaqo-nkqubo yeklinikhi evela kwi-American College of Emergency Physicians (ACEP) kunye ne-UK NICE (NG232 Isikhokelo sokulimala kweNtloko) sicebisa ukuba *nabani na othatha i-anticoagulant okanye i-P2Y12 ye-antiplatelet drug ogcina impembelelo yentloko ethe ngqo kufuneka aqhube intloko yesibhedlele ngokukhawuleza i-CT scan ngaphakathi kweeyure eziqhelekileyo, nangona engazange ahlolwe ngokufanelekileyo - Kwakhona, nangona intloko yokuqala ye-CT scan icacile ngokupheleleyo, umntu omdala onokwethenjelwa kufuneka aqaphele isigulane kwixesha elizayo ** i-24 ukuya kwiiyure ze-72 ** ukuze ** ukulibaziseka kwe-intracranial hemorrhage ** (intloko entsha, ukulala okungavamile, ukudideka, ingxaki yokulinganisela, okanye ukuhlanza).

Icandelo 2: Ulawulo olungxamisekileyo lweHemophilia A, iHemophilia B, kunye neVon Willebrand Disease

Ukuqonda Iziphazamiso Zokopha Esizizuze Njengelifa

  1. I-Hemophilia A (i-Classic Hemophilia): Ukunqongophala kofuzo okunxulumene ne-X I-Factor VIII (8). Ihlelwa ngokwesiseko senqanaba lomsebenzi egazini:
  2. Kumandundu (\(<1\%\) Umsebenzi weFactor VIII): Ukopha okuzenzekelayo kumalungu, izihlunu ezinzulu, kunye namalungu nangaphandle kokwenzakala okwaziwayo.
  3. Ephakathi (\(1\%\text{ ukuya }5\%\) umsebenzi): Ukopha kakhulu emva kwamaqhuma amancinane, ukukruneka, okanye umsebenzi wamazinyo.
  4. Iphakathi (\(5\%\text{ ukuya }40\%\) umsebenzi): Ukopha kakhulu ngokwenzakala okanye utyando.
  5. I-Hemophilia B (iSifo seKrisimesi): Ukunqongophala kwehlwili I-Factor IX (9). Ngoklinikhi iyafana neHemophilia A kubunzima bokopha, kodwa ifuna iFactor IX concentrate (ukunika iFactor VIII kwisigulane seHemophilia B ayenzi nto).
  6. Izigulana kwi-Emicizumab (Hemlibra) okanye nge-"Factor Inhibitors":
  7. I-Emicizumab (Hemlibra): Izigulana ezininzi ezine-Hemophilia A ngoku zithatha inaliti ye-antibody yeveki okanye yenyanga engaphantsi kwe-subcutaneous ebizwa ngokuthi emicizumab elinganisa i-Factor VIII ukuthintela ukopha mihla le. Umgaqo obalulekileyo wonxunguphalo: I-Emicizumab ibonelela ngokhuseleko olusisiseko olulingana noqikelelo lwe-\(10\%\text{ ukuya }15\%\) factor factor, enganelanga ukumisa ukopha okukhulu kokwenzakala okanye ukopha kwengqondo. Isigulana esikwi-emicizumab esinokwenzakala entloko okanye ukopha okukhulu sisafuna i-intravenous Factor VIII ngoko nangoko (okanye i-recombinant Factor VIIa ukuba zine-inhibitors).
  8. Isilumkiso esiBalulekileyo kwiZigulana ze-Inhibitor kwi-Emicizumab (Ungaze unike i-High-Dose aPCC / FEIBA): Ukuya kuthi ga kwi-30 yepesenti ye-Hemophilia eyingozi kakhulu yezigulane zenza amajoni omzimba omzimba (inhibitors) atshabalalisa i-Factor VIII emiselweyo. Ngexesha lokuphumelela ukopha kwisigulane esithatha i-*emicizumab **, unyango olungxamisekileyo olukhethiweyo yi recombinant activated Factor VII (rFVIIa, NovoSeven / Sevenfact). Iidosi eziphezulu okanye eziphindaphindiweyo ze-Activated Prothrombin Complex Concentrate (aPCC / FEIBA \(>100\text{ U/kg/day}\)) kufuneka ziphetshwe ngokungqongqo kwizigulane kwi-emicizumab, kuba ukudibanisa kubangela i-blood clots eyintlekele kunye i-thrombotic microangiopathy (TMA).
  9. Von Willebrand Disease (vWD): Esona sifo sixhaphakileyo esophayo kumadoda nabasetyhini, esibangelwa kukunqongophala okanye isiphene ku von Willebrand Factor (vWF), i-molecular "glue" enceda iiplatelet zincamathele kwiindonga zemithambo yegazi ezikrazukileyo zize zithwale iFactor VIII egazini. Ngokuqhelekileyo kubangela kakhulu ** ukopha kwe-mucosal ** (i-nosebleeds engalawulekiyo, i-hemorrhage enzima yokuya exesheni, ukopha kwentsini, kunye nokopha kwesisu), elawulwa ngokukhawuleza nge-**tranexamic acid **, ** desmopressin (DDAVP) ** (kwizigulane eziphendulayo ze-1), okanye i-intravenous vWF / Factor-Factor-PIIs, i-Wii Vonvendi).

"IiNdawo Ezintlanu Zokopha Ezibulalayo" kwiHemophilia (Apho Ukudumba Kubulala Phambi Kokuba Kubonwe Igazi)

Kwi-hemophilia, olona gazi lubulalayo lopha alubonakali ngokupheleleyo ngaphandle ngenxa yokuba igazi lihlala ngaphakathi kwimingxuma yomzimba evaliweyo okanye iindawo eziqinileyo zobuso. Nyanga iintlungu okanye ukudumba kuyo nayiphi na kwezi ndawo zintlanu ze-anatomical njenge--100%-iNqanaba leNqanaba eliNgxamisekileyo eliBomi eliTyalayo*:

Indawo yokopha iLethal Iimpawu ezisisilumkiso (Iintlungu zihlala zifika kwiiyure eziphambi kokudumba okubonakalayo) Kutheni ibulala ngokukhawuleza okanye ikhubazeke ngokusisigxina Inyathelo likaNgxamiseko ngoko nangoko ("Into yokuqala")
1. I-Intracranial / Central Nervous System (Intloko kunye noMnqonqo) Naluphi na ukubetha kwentloko, intloko, ukulala, ukucaphuka (kwiintsana / abantwana), isicaphucaphu, intamo eqinile, intlungu emva, okanye ukubetha komlenze. Ukakhayi kunye nomjelo womqolo awunako ukwanda; ukopha kucinezela i-brainstem okanye umnqonqo kwimizuzu ukuya kwiiyure. Faka iFactor ngoko nangoko kwi \(80\%\text{ ukuya }100\%\) umsebenzi NGAPHAMBI kokulinda iCT scan; fowunela 911/112/999.
2. Umqala, iNtamo, uMgangatho woMlomo, okanye uLwimi (Retropharyngeal) Umqala obuhlungu emva kokukhwehlela, umsebenzi wamazinyo, okanye i-tonsillitis; ukuvuvukala phantsi kwesilevu okanye entanyeni; ilizwi elibi; ukuvuza; ingxaki yokuginya. Ukwandisa i-hematoma kwizicubu ezithambileyo zentamo okanye ulwimi lutyhala umoya uvale ngokupheleleyo, kubangele ukufutha ngokukhawuleza. Factor Factor kwangoko \(80\%\text{ ukuya }100\%\); fowunela 911 / 112 / 999; gcina umonde uhleli ** nkqo**; lungiselela ukhuseleko lomoya kwangethuba.
3. I-Iliopsoas Muscle Bleed (I-Hip eNzulu / isithuba seRetroperitoneal) **I-groin enzulu, isisu esisezantsi, okanye intlungu ephantsi **; **i-hip ebanjwe igobile (i-flexed) kwaye ayikwazi ukulungisa umlenze **; ukuba ndindisholo okanye ukubetha phambi kwethanga (i-femoral nerve compression). Isithuba se-retroperitoneal iliopsoas sinokubamba ** 1 ukuya kwiilitha ze-3 zegazi ** (okubangela ukothuka kwe-hemorrhagic) ngelixa utyumza i-nerve ye-femoral kunye nokubangela ukukhubazeka komlenze we-quadriceps ngokusisigxina. Factor Factor kwangoko \(80\%\text{ ukuya }100\%\); gcina ukuphumla okungqongqo kwibhedi kunye ne-hip esekelwe kwimiqamelo (ungaze unyanzelise umlenze ngqo); isithuthi esisiwa esibhedlele.
4. Umphambili okanye iCalf Muscle Compartment (Compartment Syndrome) I-Tight, rock-hard, ukuvuvukala okubuhlungu kwi-forearm okanye emlenzeni ophantsi; iintlungu ezimandundu xa iminwe okanye iinzwane zoluliwe zisenza; iminwe okanye iinzwane ezirhabaxa/ezibandayo. Ukopha ngaphakathi kwi-sheath ye-muscle eqinile ikhupha ukuhamba kwegazi kunye nokutyumza i-median, i-ulnar, okanye i-tibial nerves (bona ukutyumza ukulimala kunye ne-compartment syndrome). Factor Factor ngoko nangoko kwi \(50\%\text{ ukuya }100\%\) (ukumisa ukopha nge factor kudla ngokuthintela imfuno yotyando fasciotomy); phakamisa kancinci ukuya kwinqanaba lentliziyo; musa ukubopha ngokuqinileyo.
5. Ukopha kweGastrointestinal okanye uMchamo Ukugabha igazi elibomvu okanye izinto ezimnyama "zekofu-yomhlaba"; izitulo ezimnyama ze-tarry (melena); okanye igazi elibomvu eliqaqambileyo / amahlwili kumchamo (hematuria). Ukulahlekelwa okukhulu kwegazi kwangaphakathi kwizilonda zesisu okanye imithwalo ye-mucosal. (Qaphela: Kwi-hematuria yezintso ezingenantlungu, SUKU sebenzisa i-tranexamic acid, njengoko amahlwili akwi-ureter ebangela ukuvaleka kwezintso kakhulu.) Faka iFactor kwangoko; fowunela 911/112/999 ukopha kweGI (bona ukopha emathunjini).

Ukuqaphela i-"Target Joint" Aura kunye nokusebenzisa i-R.I.C.E. (Plus Factor)

Xa ukuphuma kwegazi kuqala ngaphakathi kwedolo, i-ankle, okanye i-elbow joint (hemarthrosis), abantu abane-hemophilia baziva ngokucacileyo "ukubetha, ukuxubha, ukufudumala, okanye i-aura eqinile" ngaphakathi kwelungu ** 1 ukuya kwiiyure ze-2 ngaphambi kokuba ** i-joint ibonakala idumbile okanye ishushu kumntu ongaphandle. - Soloko ukholelwa ukuba isigulana siziva "ukugquma": Ukufaka i-clotting factor ngexesha laloo aura yokuntlithwa kwangoko kuthintela ukopha phambi kokuba intlala itshatyalaliswe. - Dibanisa i-factor infusion ekhawulezileyo kunye ne-R.I.C.E.: Rest the joint (akukho bunzima), sebenzisa I ce esongelwe ngelaphu imizuzu eyi-15 / 15 imizuzu, sebenzisa ngobumnene Cinkxaso yoxinzelelo ukuba iyanyamezelwa, kwaye Eiphakamise imilenze.

Inyathelo ngenyathelo lokulawula ukopha emzimbeni kwamanxeba angaphandle kunye ne-nosebleeds

Ingaba isigulane sikwi-apixaban, i-warfarin, okanye isifo se-von Willebrand, sebenzisa ezi ndlela ezikhethekileyo ukulawula ukopha kwangaphandle ngelixa ukuguqulwa kwezonyango kuqhubeka:

1. Ukusikwa kolusu lwangaphandle kunye nokuRhutywa: Imizuzu engama-20 yoMthetho woxinzelelo olungaqhawukanga

  1. Sebenzisa i-Hemostatic Gauze ukuba Iyafumaneka: Ukuba ikiti yakho yoncedo lokuqala iqulethe isinxibo esine-hemostatic esifakwe kaolin (QuikClot Combat Gauze / Bleeding Control Dressing), chitosan (Celox / ChitoGauze), okanye i-oxidized regenerated regenerated cellulose, ngqo kwipakethe ye-cellulose ephumayo okanye uyicinezele. Izinxibo zeChitosan zisebenza ngentlawulo ye-electrostatic kwiiseli ezibomvu zegazi naxa izinto zokujiyisa zesigulana zithintelwe ziincindi zegazi okanye ihemophilia.
  2. ** Cinezela phantsi ngamandla ngezandla zozibini kwaye uqalise isibali-xesha kangangemizuzu engama-20 egcweleyo:**
  3. Musa ukusebenzisa i-dabbing elula. Ncamisa ubunzima bomzimba wakho ngesithende sesandla sakho ngqo enxebeni (ngaphandle kokuba kukwenzakala kwamehlo okanye kukuvuleka kokakayi).
  4. Ukuba igazi lintywila kwiphedi yokuqala yeyasuyasu: SUKUYIKHUPHISA ipadi egcwele igazi. Ukuyisusa kukrazula iplagi eyenza iplatelet. Gcoba isibini esomileyo se-gauze ngqo phezulu kweyokuqala kwaye ucinezele ngakumbi enye imizuzu eyi-15 ukuya kwe-20.
  5. Usifaka nini isicelo seTourniquet yeLimb: Ukuba igazi elibomvu elikhanyayo likhupha okanye lidibanisa ngokukhawuleza kwinxeba okanye kwinxeba lomlenze kwaye uxinzelelo oluthe ngqo alukwazi ukuyilawula, sebenzisa i-windlass tourniquet yorhwebo \(5\text{ ukuya }8\text{ centimeters}\) (\(2\text{ ukuya}3\text{intshi}\)) de ubhale lonke ixesha lokuphuma kwegazi kwinxeba, bhala lonke ixesha lokuphuma kwegazi. umtya, kwaye ungaze uwukhulule phambi kokuba ufike esibhedlele (bona ukopha).

2. Iimpumlo eziMandundu (Epistaxis) kwi-Blood Thinners okanye kwi-vWD / Hemophilia

I-nosebleeds lelona gumbi likaxakeka liqhelekileyo lokutyelela izigulana ezisebenzisa i-anticoagulant kunye nezo zinesifo se-von Willebrand. Landela ngqo I-nosebleeds protocol kunye nexesha elongeziweyo: 1. Hlala ume nkqo kwaye ungqiyame PHAMBILI kancinci esinqeni (ungaze ujongise intloko ngasemva, ekhuphela igazi emqaleni lingene esiswini kunye nendlela yomoya, ibangele ukugabha kwegazi). 2. Vuthela impumlo ngobunono kanye ukususa amahlwili axengaxengayo, afana nejeli angekaguquguquki, kwaye ukuba une-over-the-counter oxymetazoline (\(0.05\%\)) impumlo yokutshiza (Afrin) okanye umyalelo isisombululo se-tranexamic acid (TXA), fafaza izitshizi ezi-2 kwimpumlo yokopha okanye ubeke i-xxxxxxxxxxxia phambi kwempumlo). 3. Jina amaphiko athambileyo, anamazantsi empumlo ngokuqinileyo ngokuchasene neseptum kangangemizuzu engama-20 ukuya kwengama-30 eqhubekayo ngewotshi ngaphandle kokuyeka kube kanye ukujonga. 4. Bamba ipakethe yomkhenkce ukunqumla ibhulorho yempumlo nezidlele, kwaye ufunxe kwityhubhu yomkhenkce (ukupholisa uphahla lomlomo kuthintela i-palatine kunye nemithambo yeempumlo). 5. Uya nini kwiSebe likaNgxamiseko: Ukuba i-nosebleed isavuza ngokusebenzayo emva kwe-20 ukuya kwimizuzu engama-30 yokucofa ngokuqhubekayo*, ukuba igazi ligalela ngasemva komqala (impumlo yangasemva), okanye ukuba umntu uziva enesiyezi, uluthuthu, okanye intliziyo ekhawulezayo.

3. Ukopha ngomlomo, kwiintsini, okanye ngolwimi: I-Tranexamic Acid (TXA) kunye neSingxobo seTea eManzi

  • Ukopha emlonyeni, kwiintsini, okanye emva kokukhutshwa kwezinyo kwisigulane esinokuphazamiseka kwegazi okanye kwi-anticoagulants, ** i-antifibrinolytic therapy ** kunye ne *Tranexamic Acid (TXA, Lysteda / Cyklokapron) ** okanye ** i-Aminocaproic Acid (i-Amicar) * * ibaluleke kakhulu kuba amathe omntu axutywe ngegazi le-clops ( i-ssolve enzyme yendalo)
  • Ukuba iyalelwe, swishing \(10\text{mL}\) of 4.8% okanye 5% tranexamic acid solution ngobunono emlonyeni 2 imizuzu (okanye ukutyumza \(500\text{mg}\) TXA tablet in \(10\text{mL}\)wamanzi ukwenza intlama/ukuhlamba umlomo) uzinzisa amahlwili omlomo ngokumangalisayo.
  • Ekhaya, ukuluma ngokuqinileyo kwimizuzu engama-20 ukuya kwengama-30 kwi-** epholileyo, imanziswe iti yeti emnyama** esongelwe kumaleko omnye we-gauze ebhityileyo inceda ukopha okucothayo kwi-gum socket kuba yendalo tannic acid kumagqabi eti amnyama ikhuthaza i-vasoconstriction yendawo kunye neprotein precipitation.

Isikripthi sonxibelelwano esichanekileyo se-911 / 112 / 999 kunye neSibhedlele sokuThatha iSibhedlele

Ngenxa yokuba amagumbi onxunguphalo oluntu ahlala evavanya izigulana ngokubona ilahleko yegazi yangaphandle, kufuneka ubize ngokucacileyo "I-Anticoagulant Reversal" okanye "iProtocol ye-Hemophilia Factor First" kwigama lokuqala:

  • Isigulana esikuMgangatho weGazi Thinners:

    *"Ndidinga i-ambulensi [Idilesi echanekileyo]. Isigulane sineminyaka engu-[Ubudala] ubudala [indoda / ibhinqa] ethatha igazi elincinci [Apixaban / Rivaroxaban / Dabigatran / Warfarin / Clopidogrel] kwaye uye wabandezeleka [igazi elingalawulekiyo / ukuwa kunye nempembelelo yentloko / igama lokugqibela, i-dose ye-X Me]. Ixesha]

  • Isigulana esineHemophilia okanye i-von Willebrand Disease:

    *"Ndifuna i-ambulensi [Eyona dilesi] yesigulane esine- [I-Hemophilia Emandundu A / I-Hemophilia B / von Willebrand Disease / I-Hemophilia ene-Inhibitors]. I-100 yepesenti yomsebenzi PHAMBI kokulinda ii-CT scans [sele sizifakile / sizise] iibhotile [zakhe] zasekhaya ze [Igama elichanekileyo leBrand] kunye nencwadi yeprotocol [yakhe] yeZiko loNyango lweHemophilia.

Uluhlu lokukhangela ibhegi esibhedlele xa kungxamisekile ukopha

  • **Kwizigulane ezine-Hemophilia / vWD: Zisa ZONKE iibhokisi zasekhaya ze-clotting factor concentrate, i-vials diluent, iinaliti zokubuyisela kwakhona, kunye ne-infusions ye-infusion ** (nokuba uya kwisibhedlele esikhulu, ukuza nokubonelela kwakho kuthintela ukulibaziseka kwekhemesti yeeyure ezi-2 ngelixa isibhedlele si-odola imveliso yakho ethile).
  • Igosa lesigulana "Ikhadi leNkathalelo yeNgxaki yokuBopha / i-HTC Triage Letter" (edwelisa inqanaba lesiseko senqanaba, isimo se-inhibitor, i-formula ye-dosing engxamisekileyo esekelwe kubunzima, kunye nenombolo ye-24 yeyure kwi-call ye-hematologist yefowuni).
  • Kwizigulane ze-Blood Thinner: Ezona bhotile zepilisi zazo zonke ii-anticoagulants kunye ne-antiplatelet, kunye nexesha elichanekileyo lewotshi yokuginywa kwepilisi yokugqibela.
  • Eyona misebenzi yezintso yakutsha nje (i-creatinine/eGFR) kunye neziphumo zelebhu ye-INR ukuba ziyafumaneka kwi-app yesigulana.

Thatha ikhosi

Ukuthatha uncedo lokuqala oluqinisekisiweyo kwaye Yeka ikhosi ye-Bleed evela kwi-Red Cross, i-American College of Surgeons Misa inkqubo ye-Bleed, i-St John Ambulance, okanye umboneleli wendawo ikunika ukuqeqeshwa kwezandla ekupakisheni amanxeba, isicelo se-hemostatic gauze, kunye nokusetyenziswa kwe-windlass tourniquet esindisa ubomi ngexesha lokuphuma kwegazi okukhulu.

Imibuzo edla ngokubuzwa

Ukuba umntu othatha i-Eliquis (i-apixaban), i-Xarelto (i-rivaroxaban), okanye i-warfarin ibetha intloko yabo ngokukhawuleza kwaye ivakalelwa yinto eqhelekileyo ngokupheleleyo, ngaba bafuna ngokwenene i-CT scan yesibhedlele?

Ewe. Kwizigulana ezithatha i-blood thinners ezichazwe ngugqirha, kwanentloko ethambileyo ngaphandle kokulahlekelwa zingqondweni inokukrazula umthambo omncinci woxinzelelo olusezantsi kumphezulu wobuchopho. Ngenxa yokuba i-blood thinner ithintela oko kuvuza kuncinane ekutywinweni, igazi linokuphuma kancinane kuKhakhayi kwiiyure ezi-3 ukuya kwezingama-24 (i-subdural hematoma) ibe neempawu zero ekuqaleni ("ikhefu le-lucid") ngaphambi kokubangela isiqaqa nokufa ngesiquphe; intloko engxamisekileyo yesibhedlele i-CT scan ibamba ukopha ngelixa isenokubuyiselwa umva ngee-antidotes emithanjeni.

Uthetha ukuthini umthetho othi "Factor First" kwimo engxamisekileyo yehemophilia?

I-World Federation ye-Hemophilia (WFH) kunye ne-National Bleeding Disorders Foundation (NBDF) igunyazisa ukuba nanini na xa umntu one-hemophilia ehlaselwa entloko, ukudumba kwentamo, intlungu enzulu yesisu / i-groin, okanye ukwenzakala okukhulu, i-clotting factor concentrate kufuneka ifakwe ngokukhawuleza ukuphakamisa amanqanaba e-factory ukuya kwi-80 ukuya kwi-80 ukuya kwi-X-ray, ukulinda ukuhlolwa kwe-X-ray. intloko CT scan. I-infusing factor ithatha imizuzu kuphela, ayinamngcipheko ukuba akukho gazi likhoyo, kwaye inqanda ukopha okubulalayo kwangaphakathi okanye kwengqondo ngexesha leyure elithathayo ukugqiba umfanekiso wesibhedlele.

Kutheni i-groin enzulu okanye intlungu ye-hip (i-iliopsoas bleed) ithathwa njengengxakeko esongela ubomi kwi-hemophilia?

I-iliopsoas bleed iyenzeka ngaphakathi kwesihlunu esikhulu esihamba ukusuka kumqolo osezantsi ukuya kwi-pelvis ukuya kwi-hip. Ngenxa yokuba esi sithuba esinzulu se-retroperitoneal sifihlwe ngaphakathi emzimbeni, akukho kugruzuka kubonakala eluswini, kodwa ukuya kuthi ga kwi-1 ukuya kwi-3 ilitha yegazi kunokugalela kwi-sheath ye-muscle, ebangela ukothuka kwe-hemorrhagic esongela ubomi ngelixa ityumza i-nerve ye-femoral kwaye ibangela ukukhubazeka okusisigxina kwethanga. Uphawu oluyisilumkiso lweklasiki luyi-groin okanye intlungu ephantsi edibeneyo kunye nokubamba i-hip bent (i-flexible) kwaye ayikwazi ukulungisa umlenze.

Kutheni kufuneka ndibambe uxinzelelo oluthe ngqo kwi-15 ukuya kwimizuzu engama-30 epheleleyo ngaphandle kokuphakamisa i-gauze ukujonga ukuba ukopha kuyekile?

Kumntu othatha i-anticoagulants okanye ophila nengxaki yokopha, ukwenza ihlwili lokuqala elibuthathaka kuthatha ixesha elide kunesiqhelo kwimizuzu emi-3 ukuya kwemi-5. Ngalo lonke ixesha uphakamisa i-gauze pad ukuze "ukrobe" enxebeni, ukrazula imisonto eethe-ethe yefibrin kunye neplagi yeplatelet kudonga lwenqanawa ekrazukileyo, uqalise kwakhona ukopha okukhulu ukusuka ekuqaleni. Gcina uqinile, uxinzelelo olungaphazamisekiyo kwi-pad ubuncinane ubuncinane be-15 kwimizuzu ye-30 ngewashi, kwaye ukuba igazi lihamba, beka i-pad yesibini ngqo phezulu kweyokuqala ngaphandle kokuyisusa.

Imithombo ijongiwe

  • I-World Federation of Hemophilia (WFH): Izikhokelo ze-WFH zoLawulo lwe-Hemophilia, uHlelo lwe-3rd (i-Srivastava et al., I-Haemophilia, i-"Factor First" i-triage yongxamiseko kunye namanqanaba okujoliswe kuwo kwi-intracranial, retropharyngeal, kunye ne-iliopsoas hemorrhage).
  • ** I-National Bleeding Disorders Foundation (i-NBDF, eyayisakuba yiNational Hemophilia Foundation, eUnited States) iBhunga leNgcebiso yezoNyango kunye neNzululwazi (MASAC):** *Uxwebhu lwe-MASAC lwe-275 / uLawulo lweSebe eliNgxamisekileyo loLawulo lwabantu abane-Hemophilia kunye nezinye iziphazamiso zokopha * (kubandakanywa nezilumkiso zokhuseleko kwi-emicizuma/FEBAI kunye ne-aPCC/FEBAI).
  • ** IKholeji yaseMelika yeCardiology (ACC, eUnited States): ** *2020 iNgcali yeSigqibo seNgcaciso yeNgcaciso yeNgcaciso yoLawulo lwe-Bleeding kwiZigulana kwi-Oral Anticoagulants * (Tomaselli et al., * I-Journal ye-American College of Cardiology *, iiprothokholi zokuguqula i-4F-PCC, i-idarucizumat kunye ne-alxaxanet).
  • ** I-American Society ye-Hematology (ASH), i-International Society kwi-Thrombosis kunye ne-Haemostasis (ISTH), i-NHF, kunye ne-WFH: ** * Izikhokelo ze-2021 malunga nokuxilongwa kunye noLawulo lwe-von Willebrand Disease * (* I-Blood Advances *, James et al. / Connell et al.).
  • Iziko leSizwe lezeMpilo kunye nokuGqwesa kweNkathalelo (i-NICE, e-United Kingdom): *Ukulimala kweNtloko: Uvavanyo kunye noLawulo lwangethuba * (I-NICE Guideline NG232, i-neuroimaging criteria yezigulane ezichasene ne-anticoagulated).

Izikhokelo ezinxulumeneyo