ACE-Inhibitor Kutupa Lilime ndi Cholowa Angioedema Airway Emergency¶

Kutupa kofulumira, kopanda ululu kwa milomo, lilime, pansi pakamwa, kapena m'ng'oma popanda ming'oma kapena kuyabwa ndi ngozi yowopsa yowopsa chifukwa cha kuchuluka kwa bradykinin m'malo motulutsa histamine. Wodwala akamamwa mankhwala othamanga kwambiri a magazi omwe amathera ndi "-pril" (monga lisinopril, enalapril, kapena ramipril) kapena wodwala matenda a Angioedema (HAE) amakula kwambiri, mankhwala oletsa anaphylaxis monga epinephrine, antihistamines, ndi corticosteroids nthawi zambiri amalephera kuletsa kutupa. Ngati inu kapena munthu wina wapafupi nanu akukula pang'onopang'ono lilime, kuvutika kumeza malovu, mawu osamveka a "mbatata yotentha", kapena kupuma mokweza, imbani foni nambala yanu yadzidzidzi nthawi yomweyo. manambala angozi. Onaninso ma protocol okhudzana ndi ndege mu thupi lawo siligwirizana ndi anaphylaxis, epiglottitis, croup, ndi kutsekeka kwapamwamba kwa mpweya,ndi abscess peritonsillar ndi angina Ludwig.

Chitani izi tsopano¶

1. Imbani foni nambala yanu yadzidzidzi nthawi yomweyo (911 ku United States ndi Canada, 112 kudutsa European Union, 999 ku United Kingdom) ngati kutupa kulikonse kungakhudze lilime, mkamwa wofewa, uvula, pansi pakamwa, khosi, kapena mawu. Osadziyendetsa nokha kuchipatala panthawi yotupa mkamwa kapena pakhosi, chifukwa msewu wapamtunda ukhoza kutsekedwa mkati mwa galimoto yoyenda mkati mwa mphindi imodzi.
2. Perekani mankhwala opulumutsira a Hereditary Angioedema (HAE) nthawi yomweyo ngati wodwala wanyamula. Ngati munthuyo akudziwa kuti HAE kapena wapeza vuto la C1-esterase inhibitor, muthandizeni kudzibaya jekeseni wa subcutaneous ikatibant (30 milligrams pakhungu la m'mimba) kapena lowetsani intravenous kapena plasma recombinseantried mu icatibant (C1-INH)** kapena ecallantide pompano. Kuchedwa kulikonse kwa mphindi 15 popereka chithandizo cha bradykinin kumapangitsa kuti edema ya laryngeal ikule.
3. Gwiritsirani ntchito epinephrine auto-injector ngati chomwe chimayambitsa kutupa sichidziwika kapena ngati ming'oma, kuyabwa, kapena kupuma kulipo. Ngakhale kuti angioedema ya bradykinin-mediated simayankha modalirika ku epinephrine, oima pafupi sangathe nthawi zonse kudziwa histamine anaphylaxis kusiyana ndi bradykinin angioedema m'mphindi zoyamba za vuto. Kupereka intramuscular epinephrine mkati mwa ntchafu yakunja ndikotetezeka komanso kopulumutsa moyo ngati kutupa kumakhala kosagwirizana ndi anaphylaxis.
4. *Mukhazikitseni wodwalayo mowongoka kapena kutsamira patsogolo pang'ono pomuika katatu. Musamakakamize munthu wotupa lilime kapena mmero kuti agone chagada. Mphamvu yokoka imakokera lilime lokulirapo, lolemera kumbuyo ku khoma lakumbuyo kwa pharyngeal likakhala pamwamba, ndikutsekereza kutuluka kwa mpweya nthawi yomweyo. Perekani kapu kapena thaulo kuti athe kudontha kapena kulavulira malovu m'malo moyesa kumeza.
5. Letsani mapiritsi onse a ACE-inhibitor kuthamanga kwa magazi, sacubitril-valsartan, mankhwala a shuga a gliptin, ndi mankhwala a estrogen nthawi yomweyo. Mubweretsereni mabotolo a mapiritsi enieni a wodwala (makamaka lisinopril, enalapril, ramipril, benazepril, perindopril, captopril, kapena quinapril paramedical mwachindunji kwa dokotala wachipatala).
6. Odwalayo asamachite chilichonse ndi pakamwa (NPO) ndipo pewani kukhudza kapena kufufuza kumbuyo kwa mmero. Musapatse mapiritsi oletsa kutulutsa madzi m'kamwa, madzi, kapena mapiritsi ophwanyidwa kuti ameze lilime kapena pharynx ndi kutupa, chifukwa kutsamwitsidwa ndi kupuma kungayambitse laryngospasm yakupha.
7. Alert paramedics ndi ogwira ntchito zadzidzidzi mwamsanga kuti mukukayikira kuti bradykinin-mediated angioedema imafuna kukonzekera koyambirira kwa fiberoptic intubation. Auzeni gulu lotsitsimula momveka bwino ngati wodwalayo atenga ACE inhibitor kapena ali ndi HAE kotero kuti akatswiri ochita opaleshoni amakonzekera kusinthasintha kwa fiberoptic bronchoscope ndi lilime la opaleshoni loletsa pakamwa pakamwa pakamwa.

Mtengo wosankha: Kutsika kwa milomo, lilime, ndi angioedema wapanjira¶

flowchart TD
    Start["Kutupa Kwambiri kwa Milomo, Lilime, Nkhope, kapena Pakhosi"] --> AirwayCheck{"Yang'anani Mbendera Zofiira za Airway: Kusintha kwa mawu, kudontha, kupuma pang'ono, kupuma pang'ono, kapena lilime / pansi pakamwa pakamwa?"}

    AirwayCheck -->|"INDE: Kutsekereza Ndege Kuyandikira"| CallEMS["1. Imbani 911/112/999 Nthawi yomweyo. 2. Khalani oleza mtima ndi kutsamira kutsogolo. 3. Perekani mankhwala opulumutsa a HAE (icatibant / C1-INH) ngati amadziwika HAE, kapena Epinephrine ngati sichikudziwika chifukwa chake."]

    AirwayCheck -->|"AYI: Kutupa kwa milomo kapena nkhope kumangotupa"| MediatorCheck{"Unikani Njira: Ming'oma (urticaria), kuyabwa kwambiri, kapena kuyamba mwadzidzidzi mutatha kudya, kuluma, kapena maantibayotiki?"}

    CallEMS --> HospitalPrep["Alert Hospital Triage: Konzekerani Fiberoptic Nasotracheal Intubation Yoyamba Kugalamuka + Kukhazikitsa Kawiri Pathreyi ya Cricothyrotomy"]

    MediatorCheck -->|"INDE: Ming'oma ndi Kuyabwa Kulipo"| HistaminePath["HISTAMINE-MEDIATED (MAST CELL) ANGIOEDEMA / ANAPHYLAXIS: 1. Injecting IM Epinephrine (0.3 mpaka 0.5 mg lateral ntchafu). 2. Perekani H1/H2 antihistamines + corticosteroids. 3. Kuyang'ana kwa dipatimenti yazadzidzi za biphasic reaction."]

    MediatorCheck -->|"AYI: Kutupa kosapweteka kapena kolimba kosayabwa POPANDA ming'oma"| DrugHistory{"Yang'anani Mankhwala & Mbiri Yaumwini / Banja"}

    DrugHistory -->|"Kutenga Lisinopril, Enalapril, Ramipril, Sacubitril-Valsartan, kapena DPP-4 Inhibitor"| ACEIPath["ACE-INHIBITOR / BRADYKININ ANGIOEDEMA: 1. Itanani EMS ku Dipatimenti ya Emergency Airway monitoring. 2. Siyani mpaka kalekale ACE inhibitor. 3. Epinephrine/steroids osagwira ntchito; ganizirani Fresh Frozen Plasma kapena TXA mukuyenda movutikira kwambiri."]

    DrugHistory -->|"Mbiri ya Hereditary Angioedema (HAE), mbiri yabanja, kapena kupweteka kwa m'mimba kobwerezabwereza"| HAEPath["KUSINTHA KWA CHOALOWA KAPENA KUPEZEKA KWA C1-INH (HAE): 1. Perekani Subcutaneous Icatibant (30 mg) kapena IV C1-Esterase Inhibitor mwamsanga. 2. Mayendedwe kupita ku dipatimenti yazadzidzidzi ngati pali vuto lililonse pakamwa, chilankhulo, kapena laryngeal."]

Pathophysiology: Bradykinin motsutsana ndi histamine mu kutupa kwapamwamba kwa mpweya¶

Kumvetsetsa chifukwa chake matenda ena otupa lilime lalikulu amayankha pakangopita mphindi zochepa ku epinephrine pomwe ena amapitilira kutupa ngakhale kuti amwedwa mobwerezabwereza epinephrine, antihistamines, ndi steroids amafunika kufufuza njira ziwiri zosiyana za biochemical zomwe zimayang'anira kufalikira kwa mitsempha.

The histamine ndi mast cell pathway (matupi angioedema)¶

Mu classic matupi awo sagwirizana angioedema, allergen monga peanut protein, njuchi venom, kapena penicillin cross-links immunoglobulin E (IgE) antibodies pa mast cell ndi basophils. M'mphindi zochepa chabe, maselowa amataya histamine, leukotrienes, tryptase, ndi prostaglandin mu minofu yozungulira. Histamine imamangiriza ku H1 ndi H2 zolandilira pa post-capillary venules, kumayambitsa kufiira, kuyabwa kwambiri, ndi mafunde okwera (** urticaria kapena ming'oma **). Chifukwa mast cell degranulation imayimitsidwa ndi kukondoweza kwa beta-2 adrenergic ndi alpha-1 vasoconstriction, intramuscular epinephrine imayimitsa mwachangu edema yolumikizana ndi histamine, pomwe antihistamines ndi steroids zimalepheretsa kuyambiranso.

Njira ya kallikrein-kinin ndi bradykinin (non-allergic angioedema)¶

Mu bradykinin-mediated angioedema, mast cell ndi histamine amachita ziro. Wolakwayo ndi bradykinin, peptide ya vasoactive ya amino-amino acid yopangidwa ndi kallikrein-kinin contact system. Pamene bradykinin imamanga ku bradykinin B2 receptors pa vascular endothelium, imayambitsa kutulutsidwa kwa nitric oxide, prostacyclin synthesis, ndi phosphorylation ya vascular endothelial cadherin (VE-cadherin). Izi zimatsegula zolumikizana zolimba pakati pa ma cell a endothelial, zomwe zimapangitsa kuti madzi a m'magazi atsanulire m'kati mwa lilime, larynx, nkhope, malekezero, kapena khoma lamatumbo.

Chifukwa bradykinin salimbikitsa kuyabwa kwa histamine kapena ma cell a dermal mast cell, bradykinin-mediated angioedema imatulutsa ming'oma ndi ziro kuyabwa. Khungu kapena lilime limawoneka lotumbululuka kapena lowoneka bwino, limakhala lolimba kapena lolimba m'malo moyabwa, ndipo limakhazikika pakadutsa maola awiri mpaka 12. Chifukwa bradykinin imagwira ntchito mwachindunji pa B2 endothelial receptors, diphenhydramine, methylprednisolone, ndi epinephrine sangathe kutseka ma endothelial osagwirizana.

Momwe ACE inhibitors amapangira kawopsedwe ka bradykinin¶

Nthawi zambiri, bradykinin imakhala ndi theka la moyo wa masekondi pafupifupi 15 chifukwa imayimitsidwa mwachangu ndi Angiotensin-Converting Enzyme (ACE, yomwe imatchedwanso kininase II) ndi aminopeptidase P (APP), yokhala ndi cleavage yosunga ndi klzzwxh:001211 klzzwxh:0014- Wodwala akamamwa ACE inhibitor (lisinopril, enalapril, ramipril, benazepril, quinapril, perindopril, captopril, fosinopril, kapena trandolapril), mankhwalawa amalepheretsa ACE kutembenuza angiotensin I kukhala angiotensin II, yomwe nthawi imodzi imalepheretsa mankhwala a bradys Pkinisal pathology. - Mu 0.1 mpaka 0.7 peresenti ya odwala onse, ndi ** 1.5 mpaka 5 peresenti ya odwala akuda ndi a ku Africa ** (omwe nthawi zambiri amanyamula ma polymorphisms amtundu wa aminopeptidase P kapena neprilysin), ma enzymes osunga zobwezeretsera amalephera kuchotsa bradykinin akuwunjikana. - Miyezo ya Bradykinin imakwera ka 5 mpaka 10 m'matumbo a m'kamwa, mosankha kulunjika lilime, milomo, malo achilankhulo, uvula, ndi larynx.

Momwe Hereditary Angioedema (HAE) ndi Kuperewera kwa C1-Inhibitor kumayambitsa kuwukira¶

Mu Hereditary Angioedema (HAE), odwala amatengera kusintha kwakukulu kwa autosomal (kapena kusintha kwadzidzidzi mu 25 peresenti ya milandu) kukhudza C1-esterase inhibitor (C1-INH) , serine protease inhibitor yopangidwa ndi chiwindi. C1-INH ndiye brake yoyamba yomwe imalepheretsa kutsegulidwa Factor XIIa ndi plasma kallikrein. Pamene magwiridwe antchito a C1-INH amatsika pansi pa 50 peresenti yanthawi zonse: 1. Kuvulala kwakung'ono, ntchito zamano, kupsinjika, matenda a virus, kapena ma estrogens amayambitsa Factor XII kukhala Factor XIIa. 2. Factor XIIa Yosasankhidwa imasintha prekallikrein kukhala plasma kallikrein yogwira ntchito, yomwe imaphwanya kininogen (HMWK) yolemera kwambiri ya mamolekyu kuti itulutse mafunde akulu a bradykinin. 3. Pali mitundu itatu ya chibadwa ya HAE: - ** HAE Type I (85 peresenti): ** Pang'ono C1-INH antigenic puloteni mlingo, otsika C1-INH ntchito yogwira ntchito, ndi otsika wothandizira **C4 **. - ** HAE Type II (15 peresenti): ** Mapuloteni amtundu wa C1-INH achibadwa kapena okwera, otsika kwambiri a C1-INH, ndi otsika ** C4 **. - HAE yokhala ndi Normal C1-INH (yosowa): Normal C1-INH ndi C4, yokhala ndi masinthidwe mu Factor XII (F12), plasminogen (PLG), angiopoietin-1 (ANGPT1), kapena kininogen-1 (KNG1) yoyambitsidwa kwambiri ndi estrogen kapena mimba. 4. Acquired Angioedema (AAE): Imayamba pambuyo pa zaka 40 ndi **palibe mbiri ya banja **, yoyendetsedwa ndi B-cell lymphoproliferative disorders (lymphoma, CLL, kapena MGUS) kapena autoantibodies yomwe imadya C1-INH ndi **C1q ** imathandizira mapuloteni.

Kuyerekeza kwathunthu: Histamine vs ACE-inhibitor vs. Hereditary Angioedema¶

Misampha yovuta kwambiri mu ACE-inhibitor angioedema¶

Msampha 1: Kungoganiza kuti mankhwala omwe amwedwa mosamala kwa zaka sizingakhale chifukwa¶

Limodzi mwamalingaliro olakwika owopsa omwe odwala komanso asing'anga amalingalira ndikungoganiza kuti kusamvana kapena kusagwirizana ndi mankhwala kumachitika sabata yoyamba yoyambira kulandira mankhwala atsopano. Ndi ACE-inhibitor angioedema, pomwe pafupifupi 50 peresenti ya milandu imachitika mkati mwa masiku 30 oyamba a lisinopril kapena enalapril, maperesenti 50 otsalawo amapezeka miyezi, zaka, kapena kupitilira zaka khumi wodwala atamwa mapiritsi omwewo tsiku lililonse popanda vuto. Kugunda kwachiwiri, monga ntchito yaing'ono ya mano, kachilombo ka HIV pamwamba pa kupuma, kupwetekedwa mtima kwa uvula, kuyambitsa mankhwala osakanikirana, kapena kuchepa kwa ma enzymes okhudzana ndi ukalamba, mwadzidzidzi kumapereka chidziwitso kwa wodwalayo pamlingo wa bradykinin pambuyo pa zaka 8 za chithandizo chokhazikika.

Msampha 2: Mankhwala a Synergistic omwe amachulukitsa chiopsezo cha ACE-inhibitor angioedema¶

Kuphatikizika kwina kwa mankhwala nthawi imodzi kumalepheretsa ma enzymes angapo omwe amaphwanya bradykinin, zomwe zimapangitsa kuti lilime lowopsa komanso kutupa kwam'mphuno kukhale koopsa: 1. Sacubitril-Valsartan (mankhwala a Entresto / ARNI): Sacubitril amaletsa neprilysin (neutral endopeptidase). Kutenga sacubitril-valsartan nthawi imodzi ndi ACE inhibitor kumayambitsa kuwonongeka kwa bradykinin. Mawu Azachipatala: Nthawi yokwanira yosamba maola 36 imafunika pakati pa mlingo womaliza wa ACE inhibitor ndi mlingo woyamba wa sacubitril-valsartan (ndi mosemphanitsa). Kuphatikiza apo, sacubitril-valsartan imatsutsana ndi aliyense yemwe ali ndi mbiri yakale ya ACE-inhibitor angioedema. 2. DPP-4 Inhibitors ("Gliptins" a Type 2 Diabetes): Mankhwala monga sitagliptin, saxagliptin, linagliptin, ndi alogliptin amatsekereza dipeptidyl peptidase-4 (DPP-4), enzyme yoyamba yosunga zobwezeretsera yomwe imawononga bradykinin ndi chinthu P pamene ACE yatsekedwa. Kuonjezera gliptin kwa wodwala yemwe atenga kale lisinopril kapena ramipril kumawonjezera chiopsezo cha bradykinin angioedema ndi katatu mpaka kanayi. 3. mTOR Inhibitors (Sirolimus ndi Everolimus mu Odwala Opatsira Organ): Omwe amawasinthitsa amatenga sirolimus kapena everolimus pamodzi ndi ACE inhibitor amakumana ndi ziwopsezo zazikulu za lingual angioedema chifukwa cha kusokonezeka kwa endothelial chotchinga. 4. Recombinant Tissue Plasminogen Activator (Alteplase / Tenecteplase for Acute Stroke): Wodwala sitiroko yemwe amwa ACE inhibitor alandira mtsempha wa thrombolysis (tPA), kupanga kwa plasmin kumatsegula mwachindunji kuphulika kwa kallikrein-bradykinin. Mpaka 5 peresenti ya odwala sitiroko omwe ali pa ACE inhibitors amayamba kukhala orolingual angioedema mkati mwa mphindi 30 mpaka 120 atalandira IV alteplase, nthawi zambiri asymmetric ndi oipitsitsa kumbali ya ischemic brain hemisphere. 5. Nonsteroidal Anti-Inflammatory Drugs (NSAIDs): Ibuprofen, naproxen, ndi aspirin amatha kuyambitsa kusintha kwa mast-cell leukotriene kapena kukulitsa kutupa kwa ACE-inhibitor.

Msampha 3: Zenera loyimitsa pambuyo posiya (mpaka masabata 6)¶

Wodwala akadwala ACE-inhibitor angioedema, sayenera kumwa mankhwala aliwonse omwe ali mu kalasi ya ACE-inhibitor kwa moyo wake wonse** (kusintha kuchokera ku lisinopril kupita ku enalapril kapena ramipril kungayambitsenso zomwezo chifukwa ndi kalasi lonse la enzymatic zotsatira, osati kusagwirizana ndi kapangidwe kake ka mankhwala). Ngakhale ACE inhibitor itayimitsidwa kwathunthu, milingo ya kininase ya minofu imatenga masabata kuti iyambikenso; odwala amakhalabe pachiwopsezo cha kutupa kwa milomo kapena lilime nthawi zonse kwa **mpaka masabata 4 mpaka 6 pambuyo pa mapiritsi awo omaliza **. Kusintha kwa Angiotensin Receptor Blocker (ARB) monga losartan kapena valsartan kuyenera kuchitidwa mosamala ndi dokotala pambuyo pa kuthetsa kwathunthu, monga kusintha pang'ono kapena kubwereza kwa angioedema (pafupifupi 2 mpaka 8 peresenti) kumawonedwa panthawi ya kusintha.

Kukhazikika kwa lilime ndi airway angioedema: Gulu la Ishoo¶

Mu dipatimenti yazadzidzidzi, madokotala amawunika malo enieni a kutupa m'kamwa ndi mmero m'malo modalira pulse oximetry yokha. Pulse oximetry (\(SpO_2\)) ndi chizindikiro chakumapeto koopsa pakutsekeka kwa mpweya: Wodwala yemwe ali ndi 90 peresenti yotsekeka m'phuno amakhala ndi mpweya wabwino wa 98 peresenti pogwira ntchito mwaukali kuti akoke mpweya kudzera pamphuno ya pinhole, ndipo amangogwetsa \(SpO_2\) masekondi asanapume ndi hypoxic ya mtima wonse.

Magulu a ndege zadzidzidzi amagwiritsa ntchito Ishoo Clinical Staging System kuti adziwe yemwe angawonedwe ku chipatala chachipatala ndi omwe amafunikira kuvomerezedwa mwamsanga ku Intensive Care Unit (ICU) ndi kudzutsidwa modzidzimutsa:

Chifukwa chiyani standard sequence intubation (RSI) imatha kufa mu lingual and laryngeal angioedema¶

Pazidzidzi zambiri zachipatala zomwe zimafuna chubu chopumira, madokotala odzidzimutsa amachita ** Rapid Sequence Intubation (RSI) ** popereka mankhwala osokoneza bongo (monga etomidate kapena ketamine) ndi neuromuscular paralyzing drug (monga rocuronium kapena succinylcholine), kuyembekezera masekondi a 45 kwa apnea pakamwa, ndi kuika pakamwa pa lablango.

Mu Stage III kapena Stage IV angioedema ya lilime kapena larynx, RSI yokhazikika yokhala ndi neuromuscular paralysis ndiyo chifukwa chachikulu cha "Cannot Intubate, Cannot Oxygenate" (CICO) kumangidwa kwa mtima: 1. Kutayika kwa Pharyngeal Muscle Tone pa Kufa ziwalo: Ngakhale kuti wodwala yemwe ali ndi lilime lalikulu kapena kutupa kwapang'onopang'ono amakhala maso ndikukhala mowongoka, amagwiritsa ntchito minofu yapakhosi, nsagwada, ndi pharynx kuti atsegule kanjira kakang'ono ka mamilimita atatu. Munthu wopuwala akayamba kugwira ntchito ndipo wodwalayo agonekedwa pansi, kamvekedwe ka minofu kamatha ndipo lilime lolemera ndi makwinya a aryepiglottic amagwera pamodzi. 2. Kulephera kwa Oral Direct ndi Video Laryngoscopy: Lilime lakutsogolo ndi pansi pakamwa pakamwa patupa mpaka kuwirikiza kawiri kapena katatu, minofuyo imakhala yolimba komanso yosagwirizana. Pali ziro chipinda m'kamwa kuika muyezo Macintosh kapena kanema laryngoscope tsamba, ndipo lilime sangathe wothinikizidwa mu submandibular danga kuona zingwe mawu. 3. Kulephera kwa Supraglottic Airways (LMA) ndi Bag-Valve-Mask Ventilation: Kukankhira mpweya kuchokera pamwamba ndi chigoba cha bag-valve kumapangitsa kutupa kwa epiglotti kutsika, pamene Laryngeal Mask Airway (LMA) siingathe kulambalala lilime lalikulu kapena kukweza minyewa yotupa.

Muyezo wagolide: Awake fiberoptic nasotracheal intubation yokhala ndi "kukhazikitsa kawiri"¶

Ngati njira yodutsa mpweya iyenera kukhala yotetezedwa kwambiri ndi ACE inhibitor kapena HAE angioedema: - Musungeni wodwala kukhala choongoka ndikupumira mwachisawawa. - Konzani "Kukhazikitsa Kawiri": Musanakhudze mphuno kapena mmero ndi scope, dotolo kapena dotolo amalemba cricothyroid nembanemba pakhosi ndi cholembera cha opaleshoni, amatsuka khungu ndi chlorhexidine, jekeseni lidocaine wa m'deralo pamwamba pa nembanemba, ndikutsegula **scalpel-bougie-cricothyroid-tube bedi *corotomy. - Ikani m'mphuno ndi m'phuno: Vasoconstrictor (oxymetazoline) ndi atomized local anesthetic (4 peresenti ya lidocaine) amagwiritsidwa ntchito pamphuno ndi pharynx yomveka bwino kuti asagwedezeke kapena laryngospasm. - Galamukani Kusinthasintha kwa Fiberoptic Nasotracheal Intubation: Kudutsa kachingwe kakang'ono kosinthika ka fiberoptic bronchoscope kodzaza ndi endotracheal chubu (\(6.0\text{ mpaka }7.0\text{mm}\) m'mimba mwake) kupyola **mphuno (njira ya nasotracheal)**kudutsa m'kamwa modutsa pansi, kulowera mkati modutsa pakamwa. mawu asanapatsidwe mankhwala ogonetsa kapena opuwala.

Chithandizo chamankhwala chadzidzidzi chomwe chilipo: Momwe mungaletsere kutupa kwa bradykinin¶

1. Chithandizo choyamba chopulumutsa cha Hereditary Angioedema (HAE) ndi Kuperewera kwa C1-INH¶

Maupangiri a International WAO/EAAC (World Allergy Organisation / European Academy of Allergy and Clinical Immunology) amalamula kuti wodwala aliyense yemwe ali ndi HAE azinyamula milingo iwiri yamankhwala opulumutsira omwe akufunidwa nthawi zonse ndikulandira chithandizo pachizindikiro choyambirira cha vuto lililonse la m'kamwa, kumaso, laryngeal, kapena m'mimba:

• Icatibant (Firazyr): Chosankha chopanga bradykinin B2 receptor antagonist chomwe chimatchinga mwachindunji bradykinin kuchoka kumangiriza kupita ku endothelial B2 receptors. Kuperekedwa ngati jekeseni 30-milligram (\(3\text{ mL}\)) imodzi pakhungu la m'mimba (kalingo wa ana amatengera kulemera kwake kuchokera pa \(10\mawu{mpaka 30\mawu{mg}\)). Odwala ndi achibale amaphunzitsidwa kubaya jekeseni kunyumba ambulansi isanafike. Mlingo wobwereza ukhoza kuperekedwa pambuyo pa maola 6 ngati kuli kofunikira (kuchuluka kwa 3 m'maola 24). Imayimitsa kutupa mkati mwa *15 mpaka 30 mphindi **.
• Plasma-Derived C1-Esterase Inhibitor Concentrate (Berinert, Cinryze): Imalowa m'malo mwa protein ya C1-INH yamunthu yomwe ikusowa mwachindunji, kutseka Factor XIIa ndi plasma kallikrein. Amalowetsedwa mtsempha wapakati pa mlingo wotengera kulemera kwa \(20\text{ International Units pa kilogalamu}\) (\(20\text{IU/kg}\)) kupitirira mphindi 5 mpaka 10.
• Recombinant Human C1-Esterase Inhibitor (Ruconest): Amayendetsedwa mtsempha wamagazi pa \(50\mawu{IU/kg}\) (mpaka \(4,200\text{IU}\)); contraindicated odwala odziwika kalulu ziwengo.
• Ecallantide (Kalbitor): Chosankha plasma kallikrein inhibitor chovomerezedwa ku United States kwa odwala azaka 12 ndi kupitilira apo; kuperekedwa ngati 30 mamiligalamu kudzera mu majekeseni atatu a \(1\text{mL}\) (\(10\text{mg}\)) ndi dokotala yemwe ali ndi zida zothana ndi chiwopsezo cha 3 mpaka 4 peresenti ya anaphylaxis.

2. Njira zadzidzidzi za mzere wachiwiri pamene C1-INH kapena Icatibant palibe¶

Mzipatala zakumidzi kapena zoletsa ACE-inhibitor angioedema pomwe ma biologics a HAE sapezeka, madokotala azadzidzidzi amagwiritsa ntchito zida ziwiri zomwe zimapezeka kwambiri: - Tranexamic Acid (TXA, \(1,000\text{mg}\) IV kupitirira mphindi 10): Imaletsa kutembenuka kwa plasminogen kukhala plasmin, kuchedwetsa kaphatikizidwe ka Factor XII ndi kallikrein-bradykinin. - Plasma Yozizira Yatsopano (FFP, 2 units [\(10\text{ to }15\text{mL/kg}\)] IV): Madzi a m'magazi opereka ali ndi ntchito yachilengedwe C1-esterase inhibitor komanso yogwira kininase II (ACE)* ndi aminopeptidase P yopanga ma enzymes aceady incucukinini omwe amayamba nthawi yomweyo angioedema. (Mu HAE, FFP imagwiritsidwa ntchito pokhapokha ngati C1-INH ndi icatibant palibe).

Kuzindikira kuukira kwa Angioedema m'mimba (Kupewa opaleshoni yosafunikira)¶

Ngakhale kuti angioedema ya ACE-inhibitor nthawi zambiri imayambitsa edema ya m'mimba, kuukira kwa Angioedema (HAE) kumakhudza m'mimba mwa odwala oposa 80 peresenti. - Kutupa kwa matumbo a submucosal kumalepheretsa lumen ya m'mimba ndikutulutsa malita amadzimadzi mu peritoneal cavity (acute ascites), kumayambitsa kupweteka kwa 10/10, kusanza kosalekeza, kutsekula m'mimba, kutsekula m'mimba, ndi hypovolemic shock. - ** Msampha Wopanga Opaleshoni: ** Chifukwa chakuti wodwalayo ali ndi mimba yofewa komanso maselo oyera a magazi okwera (kuchokera ku hemoconcentration) ndi ** zero zotupa zowoneka za nkhope panthawi yachiwonongeko , mpaka gawo limodzi mwa magawo atatu a odwala HAE osadziwika amakumana ndi laparotomies yofufuza kapena appendectomies zosafunikira. - Mmene Mungasiyanitse: Mosiyana ndi bacterial peritonitis (onani appendicitis ndi peritonitis), vuto la m'mimba la HAE nthawi zambiri limakhala lopanda kutentha thupi kwambiri, limawonetsa gawo lofananira ** khoma lamatumbo likukhuthala (mawonekedwe a "ndalama zandalama") ndi ma ascites pa CT, ndipo amatha pakadutsa mphindi 30 mpaka 90 za icatibant kapena intravenous C1-INH.

Zomwe zimayambitsa kuukira kwa HAE ndi pre-prophylaxis¶

Odwala omwe ali ndi HAE kapena Acquired C1-Inhibitor Deficiency ayenera kukonzekera zoyambitsa zamakina ndi mahomoni zomwe zimayambitsa Factor XII: 1. ** Kuchotsa Mano, Opaleshoni Yam'kamwa, ndi Intubation (Chiwopsezo Chachikulu): ** Popanda chithandizo chisanachitike, ** 20 mpaka 40 peresenti ya odwala HAE amayamba kuopseza moyo wa laryngeal edema mkati mwa maola 4 mpaka 48 pambuyo pochotsa dzino **. Odwala ayenera kulandira *mankhwala anthawi yochepa (\(1,000\text{ mpaka }2,000\text{IU}\) kapena \(20\text{IU/kg}\) ya C1-INH concentrate yopangidwa ndi plasma) maola 1 mpaka 6 musanachite opaleshoni iliyonse ya mano, opareshoni ya m'kamwa, endoscopy 4 pa bedi, kapena mu intuba. 2. Mamankhwala a Estrogen: Kulera kwapakamwa kophatikizana ndi m'malo mwa mahomoni a estrogen kumakweza Factor XII ndikuyambitsa kuukira; Zosankha za progestin-only kapena zopanda mahomoni ndizokonda. 3. ACE Inhibitors: Zotsutsana kwambiri ndi odwala onse a HAE.

Nambala zangozi zachigawo ndi ndondomeko za bungwe¶

Mukamapempha thandizo pakatupa lilime, milomo, kapena mmero, fotokozani momveka bwino kuti wodwala ali ndi acute upper airway angioedema:

• United States ndi Canada: Imbani 911 kuti mupeze ambulansi ya Advanced Life Support (ALS). Pansi pa **FDA **, ** AAAAI **, ndi ** US HAEA ** ndondomeko, zibweretseni kunyumba za icatibant, Berinert, Cinryze, kapena Ruconest mu ambulansi kotero kuti opaleshoni kapena madokotala odzidzimutsa angathe kupereka mankhwala a wodwalayo mwamsanga.
• United Kingdom: Imbani 999 (kapena 112). Pansi pa ** NHS **, ** Resuscitation Council UK **, ndi ** BSACI ** malangizo, ogwira ntchito ku ambulansi amapereka odwala C1-INH kapena icatibant ndi kuchenjeza Dipatimenti Yodzidzimutsa yomwe ikulandira ndi "Airway Standby" kuyitana kwa opaleshoni ya opaleshoni ndi ENT.
• European Union: Imbani 112. WAO ndi EACI malangizo amalimbikitsa kunyamula Khadi la Emergency ku Europe la HAE lofotokoza kulemera kwa C1-INH (\(20\text{ IU/kg}\) IV) ndi icatibant (\(30\text{mg}\) SC) dosing.
• Australia ndi New Zealand: Imbani 000 ku Australia kapena 111 ku New Zealand ndikutsatira ASCIA angioedema action plan.
• India ndi Asia-Pacific: Imbani 112 ku India, 119 ku Japan ndi South Korea, kapena 995 ku Singapore. Kumene C1-INH kapena icatibant sizikusungidwa, dziwitsani dokotala zadzidzidzi za intravenous Tranexamic Acid (\(1\text{g}\) IV) ndi Fresh Frozen Plasma (\(2\text{mayunitsi}\) IV) ndondomeko zopulumutsira.

Mwaona manambala angozi kwa manambala onse otumizira mayiko.

Mafunso omwe amafunsidwa pafupipafupi¶

Chifukwa chiyani lisinopril kapena ramipril zitha kuyambitsa lilime kutupa mwadzidzidzi nditatha kumwa mosatekeseka kwa zaka zisanu?¶

ACE-inhibitor angioedema sichiwopsezo chambiri cha chitetezo chamthupi; ndi zotsatira za biochemical zomwe zimachitika chifukwa chotsekereza enzyme ya ACE yomwe imawononga bradykinin. Mukhoza kumwa lisinopril, enalapril, kapena ramipril kwa zaka zambiri pamene ma enzymes osunga zobwezeretsera sangagwirizane ndi chilolezo cha bradykinin, mpaka kachigawo kakang'ono kakang'ono ngati chimfine, ntchito ya mano, kupwetekedwa mtima, kapena kuwonjezera mankhwala a shuga kapena odana ndi kutupa kumagonjetsa ma enzymes osungira ndikuyambitsa kutupa kwa lilime.

Kodi ndiyenera kugwiritsabe ntchito EpiPen ngati lilime la wina likutupa ndipo sindikudziwa ngati ndi ziwengo kapena ACE inhibitor?¶

Inde, ngati chomwe chimayambitsa lilime kapena kutupa kwa mmero sichidziwika kapena chingakhale chosagwirizana, jekeseni epinephrine mkati mwa ntchafu mwamsanga mutangoyimba 911, 112, kapena 999. kunyamulabe kupita kuchipinda chodzidzimutsa nthawi yomweyo.

Kodi ndingasinthe kuchoka pa lisinopril kupita ku inhibitor ina ya ACE monga enalapril kapena ramipril nditatupa milomo kapena lilime?¶

Ayi, simuyenera kumwanso mankhwala aliwonse amtundu wa ACE-inhibitor. Chifukwa mankhwala onse omwe amatha "-pril" amalepheretsa enzyme yomweyi (kininase II / ACE), kusintha kuchokera ku lisinopril kupita ku enalapril kapena ramipril kumakhala ndi chiopsezo chachikulu choyambitsa vuto lakupha laryngeal; dokotala wanu ayenera kukusinthirani ku gulu lina la mankhwala monga calcium channel blocker kapena kufufuza mosamala angiotensin receptor blocker (ARB).

Kodi madotolo amayesa bwanji Hereditary Angioedema ngati milomo, lilime, kapena m'mimba mwanga ukutupa popanda ming'oma?¶

Katswiri wa immunologist amalamula kuti ayezedwe katatu m'magazi: **kuwonjezera mlingo wa C4** (kuyesa koyezetsa bwino kwambiri, kochepera pa 95 peresenti ya odwala a HAE Type I ndi II), **C1-esterase inhibitor (C1-INH) antigenic protein level**, ndi **C1-esterase inhibitor (C1-INH) (C1-INH) fufuzani mulingo wa ntchito** kuti mupeze ntchito* ya angioedema* mwa akuluakulu). Kudumpha pafupipafupi pakhungu kapena mapanelo a IgE sangathe kuzindikira HAE.

Chifukwa chiyani matenda otupa lilime ambiri a ACE-inhibitor amayamba pakati pausiku kapena m'mawa?¶

Kugona mopanda phokoso kumawonjezera kuthamanga kwa venous hydrostatic m'mutu ndi m'khosi, pomwe cortisol yausiku imatsika pakati pausiku ndi 4:00 AM komanso kugwedezeka kosawoneka bwino kwa mkamwa wofewa ndi lilime chifukwa cha kukokoloka kumapangitsa kuti bradykinin madzimadzi azituluka m'kamwa lotayirira.

Kodi ndi nthawi yotani yochira pamene ACE-inhibitor kapena HAE kutupa lilime kufika kuchipatala?¶

Popanda mankhwala opulumutsira a bradykinin, kutupa kwa ACE-inhibitor kapena HAE kumakulirakulira kwa maola 12 mpaka 24 ndipo kumatenga maola 48 mpaka 72 kuti athetse, motero odwala omwe ali ndi intubation nthawi zambiri amakhala pachipinda chothandizira mpweya ku ICU kwa masiku awiri mpaka 4 mpaka kuyezetsa kwakhafu kutsimikizira kuti larynx yafota. Pamene subcutaneous icatibant kapena mtsempha wa C1-INH waperekedwa msanga, kupita patsogolo kumasiya mkati mwa mphindi 30 ndipo kutupa kumatha mkati mwa maola 4 mpaka 12.

Maupangiri ogwirizana¶

• Thupi lawo siligwirizana ndi anaphylaxis
• Epiglottitis, croup, stridor, ndi kutsekeka kwapamwamba kwa mpweya
• Kutupa kwa peritonsillar, angina ya Ludwig, ndi matenda a m'khosi
• Kutsekeka ndi kutsekereza mpweya wothandizira woyamba

Magwero ndi malangizo ovomerezeka¶

• World Allergy Organization (WAO) ndi European Academy of Allergy and Clinical Immunology (EAACI): The International WAO/EAACI Guideline for the Management of Hereditary Angioedema (Maurer et al., Allergy 2022; kukhazikitsa pa-kufunika icatibant, plasma-derived/pre-procedural/pre-procedural standard, C.
• American Academy of Emergency Medicine (AAEM) ndi American College of Emergency Physicians (ACEP, United States): Clinical Practice Statement and Consensus Guidelines on the Evaluation and Management of Bradykinin-Mediated and ACE-Inhibitor Induced Angioedema in the Emergency Department (Ishoosteroids of antihistaging, awastamineke fibre, antihibitor, awastamine, antihibitor, antihibitor, antihibitor, ndi fumbi. ndondomeko).
• United States Hereditary Angioedema Association (US HAEA) Medical Advisory Board: 2020 Guidelines for the Management of Hereditary Angioedema (Busse et al., Journal of Allergy and Clinical Immunology: In Practice).
• American Heart Association (AHA) ndi American Stroke Association (ASA, United States): Malangizo a Early Management of Patients With Acute Ischemic Stroke (kasamalidwe ka acute orolingual bradykinin angioedema yoyambitsidwa ndi mtsempha wa mtsempha wa alteplase/tenecteplase mwa odwala pa ACE inhibitors).
• British Society for Allergy and Clinical Immunology (BSACI, United Kingdom) ndi Resuscitation Council UK: Chitsogozo cha Management of C1-Inhibitor Deficiency and Acute Upper Airway Angioedema.