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Case Presentation. Case. Points from case ? When to give Epi pen to patients with allergic Rxn’s / angioedema Documentation ( how to RTN to ER ?) Admission criteria for allergic Rxn’s ( wouldn’t help with this case) Beware bowel edema as manifestation of allergic Rxn
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Case • Points from case • ? When to give Epi pen to patients with allergic Rxn’s/ angioedema • Documentation ( how to RTN to ER ?) • Admission criteria for allergic Rxn’s ( wouldn’t help with this case) • Beware bowel edema as manifestation of allergic Rxn • Earlier airway intervention • ?surgical
Angioedema • Angioedema is characterized by painless, nonpruritic, nonpitting, and well-circumscribed areas of edema due to increased vascular permeability
Angioedema- Location • most apparent in the head and neck, including the face, lips, floor of the mouth, tongue, and larynx, but edema may involve any portion of the body • may involve the gastrointestinal tract, leading to intestinal wall edema
Angioedema- Pathophysiology • This deficiency in functioning C1-INH leads to autoactivation of the complement system and release of kininlike mediators, resulting in edema of the subcutaneous or submucosal tissues C’ Pathway (-) C1-INH (-) kallikrein high molecular weight–kinogenbradykinin
Angioedema - Classification • 1) Hereditary angioedema (HAE) • 2) Acquired angioedema (AAE) • 3) Angioedema associated with allergic reactions (which is often associated with urticaria) • 4) Angioedema secondary to medications • ACE / ARB • 5) Idiopathic angioedema
Angioedema- HAE • C1 Esterase Inhibitor Deficiency • 3 Types • 1) Low levels of C1-INH (80-85%) • 2) Normal Levels but dysfunctional • 3) Normal levels and function – only women? • X-linked dominant inheritance
Angioedema • Precipitants of HAE angioedema • Mental and physical stress • Trauma • Dental or surgical procedures • Infections • Menstruation • Pregnancy • Oral contraceptives containing estrogens
HAE- Treatment • Epinephrine, corticosteroids, and antihistamines are NOT effective in patients with HAE, AAE, and ACE inhibitor–induced angioedema. • These agents are recommended as second-line therapy. (in cases of angioedema due to allergic causes, these medications are first-line therapies.)
HAE - Treatment • 1st line treatment • Vapor-heated C1-INH concentrate • (500-2000U IV) • FFP ( may worsen attack?) • 2U IV • Other • tranexamic acid • epsilon-aminocaproic acid ( inhibit plasmin – plays role in initiating C’ cascade)
Angioedema - Classification • 1) Hereditary angioedema (HAE) • 2) Acquired angioedema (AAE) • 3) Angioedema associated with allergic reactions (which is often associated with urticaria) • 4) Angioedema secondary to medications • ACE / ARB • 5) Idiopathic angioedema
Angioedema- AAE • Rare • Type I – lymphomas / lymphoproliferativedz • Type II - autoantibodies ? cause
Angioedema - Classification • 1) Hereditary angioedema (HAE) • 2) Acquired angioedema (AAE) • 3) Angioedema associated with allergic reactions (which is often associated with urticaria) • 4) Angioedema secondary to medications • ACE / ARB • 5) Idiopathic angioedema
Angioedema – Allergy Induced • Allergy-induced angioedema • an IgE-mediated hypersensitivity reaction • Causes • Medications • Food • Environmental allergens (insect bites)
Anaphylaxis – Drug Therapy • Vasopressin ( Level C) • 4U bolus • 10U diluted in 10mls ( titrate to effect) • Surviving Sepsis Campaign guidelines • Recommend an AVP dosage of 0.03– 0.04 IU/min, a recent study suggested that 0.067 IU/min (4 IU/h)
Angioedema - Classification • 1) Hereditary angioedema (HAE) • 2) Acquired angioedema (AAE) • 3) Angioedema associated with allergic reactions (which is often associated with urticaria) • 4) Angioedema secondary to medications • ACE / ARB • 5) Idiopathic angioedema
Angioedema • 0.1-0.2% of patients treated with ACE inhibitors develop angioedema • Idiosyncratic Rxn • 14 fold increased risk in first month of treatment • Has occurred >1 yr after initiation
Angioedema- Emerg Facts • 94% of angioedemas in ED due to meds • Most of these due to ACE Inhibitors • As many as 22 % require intubation • 11% mortality • ARB’s also cause but incidence unknown (case reports) • Mainly losarten
Angioedema- Predictors • Airway Intervention (Zirkle et al 2000) • Increasing age • Symptoms ( eg. stridor, hoarseness, dyspnea) • Not correlated • Rapidity of onset of sx • Cause of angioedema • Gender • Previous history
Angioedema- Airway • Preferred techniques • Awake nasotracheal ( orotracheal ) • Cricothyrotomy • Tracheostomy