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Te whakaheke toto i runga i te whakaheke toto, i te Hemophilia ranei

He rereke nga nama ohorere i ia whenua. Ka whakamahia e tenei wharangi te 911, te nama mo te United States me Canada. He maha nga whenua e whakamahi ana i te 112, 999, tetahi atu nama ranei. Kimihia te nama mo to whenua.

Ki te tango te tangata e tango ana i nga rongoa whakaheke toto (anticoagulants, raau antiplatelet ranei) ka ora ranei me te mate whakaheke toto (Hemophilia A, Hemophilia B, he mate von Willebrand ranei) ka pangia e te tapahi, te hinga, te mahunga o te mahunga, te toto o roto ranei, karekau e tika te wa e pa ana ki te toto. Na te mea ko te kaha o te tinana ki te hanga i te kawa fibrin umau, i te mono platelet ranei ka aukati matū, karekau ranei i te ira, ahakoa he iti te whiu o te mahunga, te ihu, me te ahua ngawari o te mahunga ka taea te ahu whakamua ki te exsanguination mate, ki te paheketanga roro ranei. Ko te awhina tuatahi e tika ana ** kia roa, kia kaha te pehanga tika (i te iti rawa ki te 15 ki te 30 meneti haere tonu me te kore e tiro)**, e whakahaeretia ana e te World Federation of Hemophilia (WFH) "FACTOR FIRST / Treat First, Scan Second" ture, me te whakamohiotanga tonu ki te hohipera me te **ingoa tika me te wa horopeta whakamutunga o te whakaheke toto i mua i te whakarite i te roopu ohotata i mua i te whakarite i te reanga ohotata i mua i a raatau mahi. ka tae mai.

I roto i te Hemophilia, INFUSE CLOTTING FACTOR I mua i te tatari mo nga hihi X, CT scan ranei.

Mena he tangata whai Hemophilia A, Hemophilia B, he mate kino von Willebrand ranei ka pangia e te mahunga, te kaki, te korokoro ranei e pupuhi ana, te whara o muri, te mamae o te puku, te kaha toto ranei o nga hononga/iuaua, ko te ture tino nui mo te oranga mai i te World Federation of Hemophilia (WFH) me te National Bleeding Disorders Foundation (FIRST/MASCTOR) I roto i te FIRST/FACTOR. Ko te tauwehe whawhati whawhati tata o te manawanui ka aro ki te kainga, ki roto ranei i te waka tūroro TONU, me te kawe tonu i a ratou oko take kaainga me te Reta Tiaki Ohotata ki te hohipera. Kaua rawa e tukua he tari whawhati tata ki te waiho i te turoro hemophilia e noho ana i roto i te rūma tatari, e tatari ana ranei mo te mahunga CT scan i mua i te whakauru i te take; ko te whakanui ake i nga taumata tauwehe katoo ki te \(80\%\text{ ki te }100\%\) ka mutu tonu te mate o te roro mate, te toto o roto ranei i te wa e whakaritea ana nga tirotiro.

Mahia tenei inaianei

  1. Hoatu tonu te pehanga tika ki nga toto o waho mo te katoa 15 ki te 30 meneti me te kore e hiki te kaikawe ki te tirotiro. Me pehi tika ki runga i te waahi whakaheke toto ma te whakamahi i te karaehe horomata, he kakahu hemostatic (pērā i te kaolin, te kawa chitosan ranei), he kakahu pokekore ranei. Kaua e hiki te kakahu ia 2 meneti ki te kite mena kua mutu te toto: i roto i te tangata turoro kei te whakaheke toto, kei te mate hemophilia ranei, ma te hikina te papa ka haea te mono karekau, ka whakahoki ano i te karaka toto ki te kore. Mo te whakaheke toto karekau e mutu i runga i te pehanga tika, whakamahia he karaehe ** tourniquet** \(5\text{ ki }8\text{ centimeters}\) (\(2\text{ to }3\text{inches}\)) i runga ake i te patunga (tirohia whakaheke toto).
  2. Waea wawe ki to nama whawhati tata mo te kaha o te toto o waho, te mahunga/kaki/uma/te puku, he tohu ranei o te toto o roto. Waea 911 i Amerika me Kanata, 112 puta noa i te Kotahitanga o Europi, 999 i te United Kingdom, tirohia katoa ranei tau ohorere. Korerohia ki te kaituku te ingoa rongoa tika (hei tauira, "Apixaban," "Rivaroxaban," "Dabigatran," "Warfarin," ranei "Clopidogrel") ranei te te mate toto tika ("Hemophilia A me te aukati").
  3. Mēnā he mate Hemophilia, von Willebrand rānei te tūroro, ā, kei tōna ringa te aro nui ki te kāinga, whakatōhia te horopeta ohotata NEI. A pee i te kawa ohorere o te Pokapū Tiaki Hemophilia (HTC) ki te whakahou me te whaowhia Factor VIII, Factor IX, von Willebrand factor concentrate, or recombinant Factor VIIa (na tonu) recombinant Factor VIIa (na mo te whakahaere tonu) [DDAVP] mena kua tohua mo te mate Hemophilia A ngawari, Momo 1 von Willebrand mate ranei). Whakaohia 2 ki te 3 pouaka taapiri o te kakano i te kainga o te manawanui ki roto i te peeke kiriweti hei kawe i roto i te waka tūroro, i te mea he maha nga wa karekau nga whare hauora hapori e penapena i nga tohu tohu, hua aukati ranei.
  4. Mehemea ka pa te mahunga o te turoro i runga i te whakaheke toto, i te hemophilia ranei (ahakoa he tohu kore noa), haere tonu ki te Tari Ohotata mo te tirotiro i te Upoko CT. Ko te whakaheke toto te take matua o te mate o te hunga mate hemophilia me te hunga mate anticoagulated. He puhoi te toto o te roro venous (subdural hematoma) i te nuinga o te waa he "maramatanga" ka 100 paiheneti te pai o te tangata mo etahi haora i muri i te tukinga o tona mahunga ki runga i te rūnanga, ka paheke ranei ki te papa, i mua i te momoe ohorere, te ruaki, me te mate o te roro mate.
  5. Tuhia te wa tika o te karaka i horomia ai te pire whakamutunga o te angiangi toto (me te werohanga i raro i te kiri whakamutunga ranei i tukuna). Ko nga taote o te hohipera me nga kai rongoa e hiahia ana ki te haora tika o te horopeta whakamutunga o te apixaban, rivaroxaban, edoxaban, dabigatran, enoxaparin, warfarin ranei hei whiriwhiri me te horopeta tika i te whakaurunga whakamuri-Fadoctor-PC, [F] Andexanet alfa, Idarucizumab, Protamine ranei).
  6. Kia takoto papatahi te manawanui, kia mahana i raro i nga paraikete maroke, kia kaua rawa he mea ma te waha (NPO). Ko te mate werawera (te mahana o te tinana kei raro i te \(35°\text{C}\) \(95°\text{F}\)) ka pararutiki ahakoa te mahi o te pereti me te enzyme e mau tonu ana. Kaua e hoatu he kai, he wai, he rongoa NSAID ranei (kaua rawa e hoatu he aspirin, ibuprofen, naproxen ranei).

Te whakamatautau ohorere me te whakaheke toto te rakau whakatau

Whakamahia tenei mahere rerenga haumanu ki te whakarite i te whakahaerenga o te whakaheke toto ki te kainga, te whakakapi i te take, me te whakahohenga rongoa rongoa a te hohipera.

flowchart TD
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    SiteCheck -->|"ĀE: 1 kei reira (Ahakoa he ahua iti te mahunga)"| LifeThreat["TE TOTO WHAKATAU ORA, TE TRAUMA WHAKAMAHI KI TE ORA:<br/>Waea 911 / 112 / 999 Inamata"]

    SiteCheck -->|"KO: Te ihu, ka toto te waha, te tapahi iti,<br/>te whakaheke toto ranei nga hononga/uauaua"| LocalCare["Ko te Hemostasis kua whakaritea + Te Whakatairanga Tere:<br/>- Tonu tonu te pehanga tika 15-30 mins (KORE PIPE)<br/>- Tranexamic acid (TXA) mouthwash/pill if prescribed<br/>- R.I.C.E. + Infusion Factor Inamata mo te toto o te hononga/iuaua"]

    LifeThreat --> DisorderType{"Hipanga 2: Kei te turoro he<br/>Hemophilia / vWD, ka tango ranei i te Toto Thinners?"}

    DisorderType -->|"He mate whakaheke toto:<br/>Hemophilia A, Hemophilia B, vWD ranei"| FactorFirst["KAAWAA A: 'TUA TUATAHI' (Tapahia i mua i te Matawai)<br/>1. Infuse horopeta whawhati tata o Factor VIII / IX / vWF / rFVIIa NOW<br/> (Target 80-100% mahi mo te mahunga/kaki/GI/tukino nui)<br/>2. Paohia nga pouaka take kaainga a te manawanui + HTC Emergency Reta<br/>3. Hoatuhia te pehanga tika tonu / tourniquet mena ka rere te toto o waho"]

    DisorderType -->|"Tuhinga Toto Toto:<br/>Warfarin, DOAC (Apixaban / Rivaroxaban / Dabigatran),<br/>Heparin / Enoxaparin, Clopidogrel / Ticagrelor ranei"| ReversalPath["KAWA B: WHAKAARO WHAKAARO ANTICOAGULANT<br/>1. Te pehanga tika tonu (hemostatic gauze) / tourniquet<br/>2. Tautuhia te ingoa tarukino TONU + haora TONU o te horopeta whakamutunga<br/>3. Kia mataara wawe a EMS & Hospital Pharmacy ki te whakarite IV Antidote:<br/> - Warfarin -> 4-Factor PCC + IV Huaora K<br/> - Dabigatran -> Idarucizumab (Praxbind)<br/> - Apixaban / Rivaroxaban -> Andexanet alfa or 4-Factor PCC"]

    LocalCare --> PersistCheck{"Kei te haere tonu te toto i mua i te 20-30 min<br/>RĀNEI kei te pupuhi nga hononga/uaua i te hope, te huha, te kuao kau, te ringaringa ranei?"}
    PersistCheck -->|"Ae"| LifeThreat

Wāhanga 1: Te whakahaere ohorere o te whakaheke toto i runga i te whakahaunga Nga Whakapai Toto (Anticoagulants & Antiplatelets)

E hia miriona nga pakeke e tango ana i nga rongoa antithrombotic ia ra mo te mate o te atrial fibrillation, te thrombosis uaua hohonu, te pulmonary embolism, te reera o te ngakau miihini, te stent arteri coronary ranei. Ko te mohio ko tehea momo raau taero ka tangohia e te manawanui he mea tino nui na te mea ka aukati ia karaehe tetahi waahanga rerekee o te whakaheke toto me te tono rongoa rongoa motuhake mo te hohipera.

Te Ripanga Whakamuri Aroturuki Matawhānui me te Ripanga Whakamuri (ACC / ASH / ISTH Aratohu)

Karaehe rongoa me nga Ingoa Generic / Waitohu Me pehea te aukati i te whakakao Hawhe-ora (E hia te roa e noho hohe ana) Tauwhāiti i te Hohipera Whakamuri Whakamuri Ohotata Nga korero nui i mua i te hohipera ki te hoatu ki nga Paramedics
** Huaora K Antagonists (VKAs):
Warfarin** (Coumadin, Jantoven, Marevan), Acenocoumarol, Phenprocoumon
Ka aukati i te whakahiato ate o te Huaora K-tiaki i te katinga Tauwehe II, VII, IX, me X. He roa rawa: 36 ki te 72 haora (ka noho te mate toto 3 ki te 5 ra i muri i te whakamutu i nga pire). 4-Factor Prothrombin Complex Concentrate (4F-PCC, Kcentra/Beriplex/Octaplex) hoatu IV (whakamuri INR i roto i 15 meneti) PLUS Intravenous Huaora K (\(5\text{ ki }10\text{ mg}\)). (Te Plasma Frozen Hou [FFP] mena karekau te PCC i te waatea.) Ripoata hou taumata whakamatautau toto INR ki te mohiohia, te horopeta o ia ra, me te take e tango ai te manawanui i te warfarin (hei tauira, te waro ngakau miihini me te AFib).
Torombin Tika (Factor IIa) Inhibitor (DOAC):
Dabigatran (Pradaxa)
Ka here tika, ka poraka i te kaha Thrombin (Factor IIa). 12 ki te 17 haora (tae atu ki te 24 ki te 36+ haora i te mate whatukuhu). Idarucizumab (Praxbind): He kongakonga antibody monoclonal i hangaia e te tangata (\(5\text{ karamu}\) IV) ka tino whakakore i te dabigatran i roto i nga meneti. (Ka taea hoki e te hemodialysis te tātari te dabigatran mai i te toto.) Ripoata haora tika o te kapopa whakamutunga i horomia me te mea he mate whatukuhu roa te manawanui. (Tuhipoka: Kare e taea te penapena a Dabigatran ki waho o tana putea pepa.)
Nga Kaitautoko Xa Tikanga (DOAC):
Apixaban (Eliquis), Rivaroxaban (Xarelto), Edoxaban (Savaysa/Lixiana), Betrixaban
Ka herea tika, ka whakahohea nga poraka Factor Xa. 8 ki te 15 haora (he roa ake i roto i te whatukuhu, ate ngoikore ranei). Andexanet alfa (Andexxa / Ondexxya) (te whakahiato whakahiato Factor Xa pūmua) ** RĀNEI 4-Factor PCC te horopeta teitei (\(25\text{ ki }50\text{ units/kg}\))**. (Ka taea te hoatu waro whakahohe-waha ki te ER mena ka horomia te pire i roto i te 2 haora.) He mea tino nui te haora tika o te pire whakamutunga: Ko te horopeta a Andexanet alfa (he horopeta iti me te horopeta teitei) ka whakawhirinaki tika ki te kaha milligrame me te mea i tangohia te pire whakamutunga \(<8\text{ hours}\) or \(\ge 8\text{ hours}\) ago.
Heparins (Ka werohia):
Enoxaparin (Lovenox/Clexane), Dalteparin, Heparin Whakakorea, Fondaparinux
Whakahohe ai i te antithrombin hei aukati i te Factor Xa me Factor IIa. Enoxaparin: 4 ki te 7 haora (i werohia i raro i te kiri). Protamine sulfate kia ata hoatu IV (ka whakatakahia te 100% o te heparin karekau me te -60% ki te 70% o te enoxaparin). Ripoata ** te horopeta mirikaramu tika me te wa karaka** o te werohanga kiri o te kopu whakamutunga.
Taukino Antiplatelet (P2Y12 Inhibitors & Aspirin):
Clopidogrel (Plavix), Prasugrel (Effient), Ticagrelor (Brilinta), Aspirin
Ka whakakorea nga toto ** platelets** mo te roanga o te oranga 7-ki-10-ra (kaore e taea te huri engari te ticagrelor). Ka roa te paanga 5 ki te 10 ra kia hanga e te hinu wheua he pereti hou. ** Desmopressin (DDAVP) **, ** Tranexamic Acid (TXA) **, me te whai i te ** Platelet Transfusion ** mo te whakaheke toto (Tuhipoka: Ka huri te Ticagrelor i roto i te toto me te aukati i nga pereti whakawhiti mo te 24h; ko te antidote rangahau he bentracimab). Korero atu ki nga taote mena he taapiri whakamomori i te ngakau korokoro o mua tata nei (i tuuhia i roto i nga marama 3 ki te 12 kua hipa), na te mea ko te aukati i nga antiplatelet e rua ka tupono ki te stent clotting.

Ko te Ture "Kua Taarua Te Toto Roo" Whai muri i te Mahunga o te Upoko i runga i nga Whakangao Toto

Ko tetahi o nga tino take o te mate ka taea te aukati i roto i nga pakeke e tango ana i te apixaban, rivaroxaban, dabigatran, warfarin, clopidogrel ranei he iti te hinganga o te whenua, he tukituki ranei te mahunga ki te kuaha waka, papa, pakitara ranei kei reira te tangata kaore e ngaro te mahara, kare he mahunga o muri noa. - He aha te take he mahanga tenei: I roto i te angaanga, he iti te pehanga iti ** nga uaua piriti** ka toro atu ki te mokowhiti i waenga i te mata roro me te dura mater o waho. Ka haea tetahi o enei uaua iti na te oho ngawari. I roto i te tangata he totika noa, ka hiri te uaua i roto i te 2 meneti. I roto i te tangata i runga i te toto angiangi, ka rere haere te uaua i ia topata mo 3 ki te 24 haora. - Kawa Whakahau: Ko nga kaupapa here haumanu mai i te American College of Emergency Physicians (ACEP) me UK NICE (NG232 Head Injury Guideline) e kii ana ** ko te tangata e tango ana i te rongoa anticoagulant, P2Y12 antiplatelet ranei e mau tonu ana te pa ki te upoko, me uru wawe ki te tirotiro CT mo te upoko o te hōhipera i roto i nga haora, ahakoa kare ano ratou i paopao ki waho* - Ano, ahakoa he tino marama te karapa tuatahi o te upoko CT, me titiro tetahi pakeke pono ki te manawanui mo te **24 ki te 72 haora e whai ake nei ** mo te whakaroa te whakaheke toto i roto i te cranial (he mahunga hou, he moe rerekee, he rangirua, he raruraru toenga, he ruaki ranei).

Wāhanga 2: Te whakahaere ohorere o Hemophilia A, Hemophilia B, me te mate von Willebrand

Te Maramatanga ki nga Whakamatea Toto

  1. Hemophilia A (Classic Hemophilia): He ngoikoretanga ira ira hono-X o te toto o te toto Factor VIII (8). Ka whakarōpūhia ma te taumata mahi i roto i te toto:
  2. Tino (\(<1\%\) Te mahi Tauwehe VIII): Te whakaheke toto ki roto i nga hononga, nga uaua hohonu, me nga whekau ahakoa kaore i mohiotia he mamae.
  3. Whakaahua (\(1\%\text{ ki te }5\%\) mahi): He nui te toto i muri i te paheketanga iti, te wiwi, te mahi niho ranei.
  4. Maamaa (\(5\%\text{ ki te }40\%\) mahi): He nui te toto me te whara, te pokanga ranei.
  5. Hemophilia B (Mai Kirihimete): Te ngoikoretanga o te totoka Factor IX (9). He rite tonu ki te Hemophilia A i te kaha o te whakaheke toto, engari me hiahia te aro o Factor IX (ko te hoatu Factor VIII ki te turoro Hemophilia B kaore he mahi).
  6. Ko nga turoro kei runga i te Emicizumab (Hemlibra) me nga "Tauwehe Tauwehe" ranei:
  7. Emicizumab (Hemlibra): He maha nga turoro me te Hemophilia A ka tango i te werohanga antibody ia wiki, ia marama ranei, e kiia nei ko emicizumab e whai ana i te Factor VIII hei aukati i nga toto o ia ra. Ture ohorere tino nui: Ka whakarato a Emicizumab i te whakamarutanga turanga e rite ana ki te \(10\%\kuputuhi{ ki te }15\%\) mahi tauwehe, KORE e ranea ki te aukati i te mamae nui o te toto, te toto roro ranei. Ko te manawanui kei runga i te emicizumab e whara ana ma te mahunga, he toto nui ranei me hiahia tonu kia werohia te Factor VIII (mehemea kei a raatau nga aukati).
  8. Whakatupato Nui mo nga Turoro Kaitiaki i runga i te Emicizumab (Kaua rawa e Homai aPCC / FEIBA): Tae atu ki te 30 paiheneti o nga turoro Hemophilia A kino ka whakawhanake i nga paturopi aukati (kaiwhakapae) e whakangaro ana i te Paerewa VIII kua whakauruhia. I te wa o te pakarutanga o te toto i roto i te turoro aukati e tango ana i te emiziumab, ko te maimoatanga ohorere e whiriwhiria ko Recombinant activated Factor VII (rFVIIa, NovoSeven / Sevenfact). ** Me tino karohia nga pota teitei o te Prothrombin Complex Concentrate Whakahohe (aPCC / FEIBA \(>100\text{ U/kg/ra}\)) i nga turoro kei runga i te emicizumab**, na te mea ka pa mai te whakakotahitanga o te toto toto me te thrombotic microangiopathy (TMA).
  9. Von Willebrand Disease (vWD): Ko te mate whakaheke toto e tino kitea ana i roto i nga tane me nga wahine, na te ngoikoretanga, te koha ranei i roto i te von Willebrand Factor (vWF), te "glue" ngota e awhina ana i nga pereti kia piri ki nga pakitara o nga oko toto kua pakaru me te kawe i te Factor VIII ki roto i te toto. I te nuinga o te wa ka puta he te toto mucosal (nga ihu kore e taea te whakahaere, te whakaheke toto nui o te paheketanga, te toto kapia, me te toto o te gastrointestinal), ka whakahaerea ma te ** waikawa tranexamic**, desmopressin (DDAVP) (i nga turoro Momo 1 urupare), i te urunga ranei vWF/Factor VIII concentrate(Pure VIII concentrate) ranei.

Ko "E rima nga waahi whakaheke toto" i roto i te Hemophilia (Kei te mate te pupuhi i mua i te kitenga o te toto)

I roto i te hemophilia, ko nga toto tino mate kare e kitea mai i waho na te mea ka kuhu te toto ki roto i nga rua o te tinana kati, ki nga waahanga whaanui ranei. Whakaorangia te mamae, te pupuhi ranei i roto i tetahi o enei wahanga tinana e rima hei 100%-Factor-Level-Moumanawa-Whakamate Ohotata:

Rohe Toto Whakamate Nga Tohu Whakatupato (He maha nga wa ka tae mai te mamae i mua i te ahua o te pupuhi) He aha te mea ka tere te mate, te mate mokemoke ranei Mahi Ohotata Inamata ("Factor First")
1. Intracranial / Pūnaha Nervous Puku (Upoko me te Hiwi) Te mahunga o te mahunga, te mahunga, te moe, te pukuriri (i nga kohungahunga/tamariki), te nausea, te kaki maro, te mamae o muri, te ngongo o te waewae. Kaore e taea e te angaanga me te hiku te whanui; ka kopeke te toto i roto i nga meneti ki nga haora. Infuse Factor tonu ki te \(80\%\text{ to }100\%\) mahi i mua i te tatari mo te CT scan; waea 911 / 112 / 999.
2. Te korokoro, te kaki, te papa o te waha, te arero ranei (Retropharyngeal) Te mamae o te korokoro i muri i te maremare, te mahi niho, te tonsillitis ranei; te pupuhi i raro i te kauae, i te kaki ranei; reo maru; drooling; raruraru horomia. Ko te toronga o te hematoma i roto i nga kopa ngohe o te kaki, o te arero ranei ka panaia te ararere kia kati rawa, ka mate ohorere. Infuse Factor tonu ki te \(80\%\text{ ki }100\%\); waea 911 / 112 / 999; kia manawanui ki te noho ** tu tika**; whakarite mo te whakamarumaru moata i te huarahi rererangi.
3. Iliopsoas Muscle Bleed (Deep Hip / Retroperitoneal Space) Te mamae hohonu, te puku o raro, te mamae o raro ranei; kua piko te huha (he piko) kaore e taea te whakatika i te waewae; te ngongo, te ngongo ranei i te mua o te huha (femoral nerve compression). Ka taea e te mokowā iliopsoas retroperitoneal te pupuri 1 ki te 3 rita o te toto (ka puta te ohorere o te hemorrhagic) i te wa e kuru ana te nerve femoral me te parapara tonu o nga waewae quadriceps. Infuse Factor tonu ki te \(80\%\text{ ki }100\%\); kia mau ki te okiokinga mo te moenga me te tautoko o te hope i runga i nga urunga (kaua rawa e tohe te waewae kia tika); kawe ki te hōhipera.
4. Puawai Ouaua kuao kau ranei (Waihanga Syndrome) Te pupuhi, te toka-pakeke, te pupuhi mamae i roto i te ringa o raro, i te waewae ranei; te mamae nui ina totoro nga maihao, matimati ranei; te ngongo, te koma/maeke maihao, matimati ranei. Ko te toto i roto i te pukoro uaua ka koowaowaotia te rere o te toto totoka, ka kuru i nga uaua o waenga, ulnar, tibial ranei (tirohia whara kuru me te mate wehenga). Tukuhia te Factor ki te \(50\%\text{ ki te }100\%\) (ko te aukati i te toto me te tauwehe he maha nga wa e kore ai e mate te mahi fasciotomy); whakanekehia iti ki te taumata ngakau; kaua e takai pai.
5. Te Toto Puawai, Puta Mimi ranei Te ruaki i te toto whero, i nga mea pouri "kawhe-papa" ranei; kumete tarry pango (melena); he toto whero kanapa ranei i roto i te mimi (hematuria). He nui te mate o roto mai i te mate whewhe puku, i nga oko mucosal ranei. (Tuhipoka: Mo te hematuria whatukuhu kore mamae, KAUA e whakamahi i te waikawa tranexamic, na te mea ka pakaru nga whatukuhu i roto i te ureter.) Factor Infuse tonu; waea 911 / 112 / 999 mo nga toto GI (tirohia toto gastrointestinal).

Te mohio ki te "Target Joint" Aura and Applying R.I.C.E. (Tauake Tāpiri)

Ka timata te toto i roto i te turi, i te pona, i te hononga tuke ranei (hemarthrosis), ka rongo te tangata whai hemophilia i te "tetere, te pupuhi, te mahana, te aura maro" kei roto i te hononga 1 ki te 2 haora i mua i te ahua tetere, te wera ranei o te hononga ki te tangata o waho. - Me whakapono i nga wa katoa ki te "pupuhi" o te manawanui: Ko te whakauru i te take o te whakaheke toto i te wa o taua aura oho moata ka mutu te toto i mua i te pakaru o te kirikiri. - Whakakotahitia te whaowhia take tonu me te R.I.C.E.: Rest te hononga (kaore he taumahatanga), pania Ice takai ki te kakahu mo te 15 meneti i runga / 15 meneti ka mutu, tono marie C tautoko whakakoi ki te pai, ka Ewhakaekehia te peka.

Te whakahaere i te whakaheke toto tinana mo nga patunga o waho me te ihu

Ahakoa kei runga te manawanui i te apixaban, warfarin, he mate von Willebrand ranei, whakamahia enei tikanga motuhake hei whakahaere i te toto o waho i te wa e haere tonu ana nga mahi whakaora:

1. Nga Tapahanga Kiri o waho me nga Taahitanga: Te Ture Peehanga 20-Minute Kore I pakaru

  1. Whakamahia te Hemostatic Gauze mena kei te waatea: Mena kei roto i to kete awhina tuatahi he whakakapi hemostatic kua whakapouritia ki te kaolin (QuikClot Combat Gauze / Whakapakari Toto Toto), chitosan (Celox / ChitoGauze), ** ranei ** cellulose kua whakahouhia te waiora**, whakakoohia, pehia tika ranei ki runga i te toto. Ko nga kakahu o te Chitosan ka mahi ma te utu hiko i runga i nga toto toto whero ahakoa ka araia e te hunga mate nga mea whakaheke toto e te whakaheke toto, te hemophilia ranei.
  2. Pehia ki raro ma nga ringa e rua ka timata i te taima mo te 20 meneti katoa:
  3. Kaua e whakamahi i te whakamaarama marama. Whakapaihia to taumaha ma te rekereke o to ringa ki runga i te patunga (mehemea he whara kanohi, he whati angaanga ranei).
  4. Mena ka uru te toto ki roto i te papa kakano tuatahi: KAUA e tango i te papa kua rukuhia ki te toto. Ma te tango i te reira ka pakaru te mono platelet. Patupatuahia te whariki maroke tuarua ki runga ake o te tuatahi ka pehi kaha ake mo etahi atu 15 ki te 20 meneti.
  5. Ahea e tono ai i te Tirohanga Mahi: Mena kei te pupuhi mai te toto whero kanapa ranei mai i te marutanga o te ringa, o te waewae ranei, a ka kore e taea e te pehanga tika te whakahaere, whakamahia he hikoi hikoi arumoni \(5\text{ to }8\text{ centimeters}\) (\(2\text{ to }3\text{inches}\)) kei runga ake o te patunga, mirohia te mahanga, me te tuhi kia mutu te wa. Kaua e wewete i mua i te taenga ki te hohipera (tirohia whakaheke toto).

2. Te Ihu Tino (Epistaxis) i runga i nga Whakaiti Toto, i roto ranei i te vWD / Hemophilia

Ko nga toto ihu te nuinga o nga haerenga mo nga ruuma whawhati tata mo nga turoro e mau ana i nga anticoagulants me te hunga whai mate von Willebrand. Whaia te tika kawa nosebleeds me te wa roa: 1. E noho tika, ka piko ki mua ki mua i te hope (kaua e whakamuri te mahunga ki muri, ka heke te toto ki te korokoro ki roto i te puku me te ara rererangi, ka ruaki te toto). 2. Me pupuhi marietia te ihu katahi ki te whakakore i nga koroke karekau e rite ana ki te tiēre, a ki te mea kei a koe i runga i te kaute oxymetazoline (\(0.05\%\)) te rehu nasal (Afrin) te whakahaunga ranei te wairewa tranexamic acid (TXA), rehu kia 2 nga rehu ki roto i te pongaihu toto ki roto i te pongaihu metazoline (ranei ka pania he miro oxygent ranei ki mua. o te pongaihu). 3. Kopihia nga parirau ngoikore, kikokiko o raro o te ihu ki te septum mo te 20 ki te 30 meneti haere tonu i te karaka kaua e tuku kotahi ki te tirotiro. 4. Purihia he kete hukapapa puta noa i te piriti o te ihu me nga paparinga, ka ngotea ki runga i te poraka tio (ma te whakamatao i te tuanui o te waha ka whakatiki i nga uaua o te ihu me te ihu). 5. Ahea haere ai ki te Tari Ohotata: Mena kei te kaha tonu te pupuhi o te ihu i muri i te 20 ki te 30 meneti o te kookiri tonu, mena kei te rere te toto ki te tuara o te korokoro (ihu ihu o muri), ka rongo ranei te tangata kei te ngenge, te koma, te ngakau tere ranei.

3. Te Puke-waha, Te Kapia, te Arero Te Tototanga: Te Waikawa Tranexamic (TXA) me te Tikanga Peeke Tii Makuku

  • Mo te toto i roto i te waha, i nga kapia, i muri ranei i te tangohanga niho i roto i te turoro he mate whakaheke toto, i runga ranei i nga anticoagulants, whakapakari antifibrinolytic me te Tranexamic Acid (TXA, Lysteda/Cyklokapron) ranei Aminocaproic Acid (Amicar) he mea tino nui na te mea he mea nui te huka toto o te tangata (i roto i te toto)
  • Mēnā kua tohua, kohia te \(10\text{ mL}\) o 4.8%, 5% ranei te wairewa waikawa tranexamic ki roto i te waha mo te 2 meneti (me te kuru ranei i te \(500\text{ mg}\) TXA papa i roto i te \(10\text{ mL}\) o te wai ki te hanga whakapiri/waha waha) ka tino whakapumau i nga kopa-waha.
  • I te kainga, ka ngau kaha mo te 20 ki te 30 meneti i runga i te pueke tii pango me te makuku kua takai ki te paparanga kotahi o te kakani angiangi ka awhina i te whakaheke toto kapia na te mea ko te ** waikawa tannic** i roto i nga rau tii pango ka whakatairanga i te whakaheke toto me te rerenga pūmua.

He tuhinga korero tika mo te 911 / 112 / 999 me te Hohipera Triage

I te mea he maha nga wa e whakamatautauria ai e nga ruma ohorere a te hapori nga turoro ma te mate toto o waho, me tino tono koe "Anticoagulant Reversal" ranei "Hemophilia Factor First Protocol" i te kupu tuatahi:

  • Mo te Turoro kei runga i nga Tohu Toto Toto:

    "Kei te hiahia ahau ki te waka tūroro i [Wāhitau Tika]. Ko te manawanui he [Tane/wahine]-tau-tau [tane/wahine] e tango ana i te toto kikokore [Apixaban / Rivaroxaban / Dabigatran / Warfarin / Clopidogrel] a kua mate [he toto kore e taea te whakahaere / he hinga me te paanga o te upoko / ruaki toto]. te whiwhi tari whawhati tata o te hōhipera ka hiahia te manawanui ki te whakahoki i te anticoagulant i roto tonu i te toto [4-Factor PCC / Andexanet alfa / Idarucizumab]."

  • Mo te Turoro kei Hemophilia, he mate von Willebrand ranei:

    "Kei te hiahia ahau ki te waka tūroro i [Wāhitau Tika] mo te tūroro ki te [Hemophilia A / Hemophilia B / von Willebrand mate / Hemophilia with Inhibitors]. Kua mate [ia] [he mahunga / te pupuhi kaki / he iliopsoas hohonu kua toto / he mamae nui]. I raro i te World Federation of Hemophilia im 100 paihēneti mahi i mua i te tatari mo nga tirotiro CT tātaritanga kua [kua mauria mai] [ana] oko kaainga o te [Ingoa Tohu Tika] me [tana] reta kawa whawhati tata o te Whare Tiaki Hemophilia."

He rarangi arowhai peeke-hohipera mo nga mate ohorere

  • Mo nga turoro Hemophilia / vWD: Mauria mai nga pouaka KATOA o te kakano tauwehe totoka, ipu waimeha, ngira whakatikatika, me nga taonga whaowhia (ahakoa ki te haere ki te hohipera nui, ma te kawe mai i a koe ake taonga ka aukati i te roanga o te rongoa rongoa mo te 2 haora i te wa e ota ana te hohipera i to hua motuhake).
  • Ko te mana o te manawanui "Kaari Tiaki Ohotata o te toto / HTC Triage Letter" (e whakarārangihia ana te taumata tauwehe turanga, te mana aukati, te tauira whakangao whawhati tata-taimaha, me te nama waea a te tohunga hematologist 24-haora.
  • Mo nga turoro Whakapai Toto: Ko nga ipu pire tuturu o nga anticoagulants me nga antiplatelets katoa, me te wa tika i horomia ai te pire whakamutunga.
  • Ko te nuinga o nga mahi whatukuhu hou (creatinine/eGFR) me nga hua taiwhanga INR mena kei te waatea i runga i te taupānga tomokanga manawanui.

Me whai akoranga

Ko te tango i tetahi awhina tuatahi whaimana me te Whakamutua te akoranga Toto mai i te Ripeka Whero, te American College of Surgeons Stop the Bleed programme, St John Ambulance, tetahi kaiwhakarato rohe ranei e tuku whakangungu-a-ringa ki a koe mo te whakakakahu i nga patunga, te tono kawa hemostatic, me te whakamahi i te hikoi hau e whakaora ana i nga oranga i te wa o te whakaheke toto nui.

Pātai auau

Mena he tangata e tango ana i a Eliquis (apixaban), Xarelto (rivaroxaban), warfarin ranei ka tukituki noa tona mahunga me te ahua tino noa, kei te tino hiahia ratou ki te tirotiro CT hōhipera?

Ae. I nga turoro e tango ana i nga rongoa whakaheke toto, ahakoa te mahunga o te mahunga me te kore e ngaro te mohio ka haea tetahi uaua iti-penehanga piriti i runga i te mata o te roro. Na te mea ka aukati te angiangi o te toto kia kore e hiri, ka tere te rere o te toto ki roto i te angaanga i roto i te 3 ki te 24 haora (he hematoma subdural) kaore he tohu i te tuatahi ("te waahi marama") i mua i te paheketanga o te koma me te mate; ka hopukina e te upoko o te hōhipera CT scan te toto i te mea ka taea tonu te huri me nga rongoa rongoa.

He aha te tikanga o te ture "Factor First" i roto i te ohorere hemophilia?

Ko te World Federation of Hemophilia (WFH) me te National Bleeding Disorders Foundation (NBDF) e whakahau ana i nga wa katoa ka pa te tangata whai hemophilia i te whara o te mahunga, te pupuhi o te kaki, te mamae hohonu o te puku, te mamae nui ranei, me whakauaua tonu te kakano o te toto ki te piki ake i te taumata tauwehe ki te 80 ki te 100 paiheneti-whakamatautau i mua i te tirotiro a te taote, te CT ranei. He meneti noa iho te taunga whaowhia, karekau he tupono mena karekau he toto, ka mutu te whakaheke toto mate o roto, roro ranei i roto i te haora ki te whakaoti i nga whakaahua o te hohipera.

He aha te take i kiia ai he mate ohotata ki te mate hemophilia te mamae hohonu, te mamae hope ranei (he iliopsoas te toto)?

Ka puta te toto o te iliopsoas i roto i te uaua nui e rere ana mai i te tuara o raro ma te pelvis ki te hope. Na te mea kei te hunahia tenei waahi retroperitoneal hohonu i roto i te tinana, kaore e kitea he maru i te kiri, engari tae atu ki te 1 ki te 3 rita o te toto ka rere ki roto i te pukoro uaua, ka puta te ohorere o te hemorrhagic i te wa e pakaru ana te nerve femoral me te parapara tonu o te huha. Ko te tohu whakatupato matarohia ko te mamae o te tuara o raro me te pupuri i te huha piko (whakapiko) me te kore e taea te whakatika i te waewae.

He aha te take me pupuri e au te pehanga tika mo te 15 ki te 30 meneti katoa me te kore e hiki te kaikawe ki te tirotiro mena ka mutu te toto?

I roto i te tangata e tango ana i nga anticoagulants, e noho ana ranei me te mate whakaheke toto, ka roa ake te hanga o te katinga ngoikoretanga i te 3 ki te 5 meneti. I nga wa katoa ka hikitia e koe te papa kaini ki te "titiro" ki te patunga, ka werohia e koe nga miro fibrin pakarukaru me te mono pereti i te pakitara ipu kua pakaru, ka timata ano te toto nui mai i te pakaru. Kia mau tonu te pehanga i runga i te papa mo te iti rawa 15 ki te 30 meneti i te karaka, a ki te mimiti te toto, tuu tika te papa tuarua ki runga o te papa tuatahi me te kore e tangohia.

Kua tirohia nga puna

  • World Federation of Hemophilia (WFH): WFH Guidelines for the Management of Hemophilia, 3rd Edition (Srivastava et al., Haemophilia, "Factor First" whawhati tata whawhati tata me nga taumata tauwehe mo te intracranial, retropharyngeal, and iliopsoas hemorrhage).
  • National Bleeding Disorders Foundation (NBDF, National Hemophilia Foundation i mua, United States) Medical and Scientific Advisory Council (MASAC): *MASAC Document 275 / Emergency Department Whakahaere o nga Tangata me te Hemophilia me etahi atu mate whakaheke toto * (tae atu ki nga whakatupato haumaru mo te emicizumab me te aPCC / FEIBA).
  • American College of Cardiology (ACC, United States): 2020 ACC Expert Consensus Decision Pathway on Management of bleeding in Patients on Oral Anticoagulants (Tomaselli et al., Journal of the American College of Cardiology, reversal protocols for 4F-PCC, and idarucizumanetb).
  • American Society of Hematology (ASH), International Society on Thrombosis and Haemostasis (ISTH), NHF, and WFH: 2021 Nga Aratohu mo te Whakatairanga me te Whakahaere o te mate von Willebrand * ( Toto Haerenga *, James et al. / Connell et al.).
  • National Institute for Health and Care Excellence (NICE, United Kingdom): Mahunga Mahunga: Aromatawai me te Whakahaere Moata (NICE Guideline NG232, neuroimaging paearu mo nga turoro anticoagulated).

Nga kaiarahi e pa ana