Immunopathology, Pathology, USMLE Step 1 - Full Vignette with Extended Explanations скачать в хорошем качестве

Immunopathology, Pathology, USMLE Step 1 - Full Vignette with Extended Explanations

A 33-year-old man with a history of hereditary angioedema presents to the emergency department with rapidly progressive hoarseness, throat tightness, and inspiratory stridor after a minor tongue bite. Despite initial home and prehospital interventions, his symptoms continue to worsen, with noted severe upper-airway swelling and respiratory distress. Which clinical signs and assessment findings should guide the next immediate step in management for this patient with impending airway compromise? VIDEO INFO Category: Immunopathology, Pathology, USMLE Step 1 Difficulty: Expert - Expert level - For those seeking deep understanding Question Type: Treatment Failure Case Type: Emergency - Emergency scenario requiring urgent decision-making Explore more ways to learn on this and other topics by going to https://endlessmedical.academy/auth?h... QUESTION A 33-year-old man with hereditary angioedema due to C1 inhibitor deficiency (type I) presents to the ED with rapidly progressive hoarseness, throat tightness, and inspiratory stridor developing over 60 minutes after a minor tongue bite while eating. He has had 6 lifetime laryngeal attacks and multiple abdominal attacks since adolescence.... OPTIONS A. Intravenous plasma-derived C1-esterase inhibitor 20 IU/kg immediately (75 kg 1,500 IU; three 500-IU vials) administered in the ED in parallel with preparation for an awake fiberoptic airway. B. Intravenous plasma-derived C1-esterase inhibitor 40 IU/kg as an empiric doubled dose now based on pharmacokinetic modeling for critical airway edema, despite guideline-standard dosing of 20 IU/kg for acute attacks. C. Subcutaneous icatibant 30 mg now with a plan to repeat a second 30 mg dose at 6 hours if inadequate response (maximum three doses/24 h), while continuing airway preparation and close monitoring for delayed laryngeal response. D. Intravenous recombinant C1-esterase inhibitor (conestat alfa) 50 IU/kg now with a plan to repeat at 60 minutes if inadequate, using an alternative C1-INH product rather than plasma-derived concentrate despite ready ED availability of pdC1-INH. CORRECT ANSWER A. Intravenous plasma-derived C1-esterase inhibitor 20 IU/kg immediately (75 kg 1,500 IU; three 500-IU vials) administered in the ED in parallel with preparation for an awake fiberoptic airway. EXPLANATION Intravenous plasma-derived C1-esterase inhibitor 20 IU/kg immediately (75 kg 1,500 IU; three 500-IU vials) administered in the ED in parallel with preparation for an awake fiberoptic airway. The essential teaching point is that laryngeal hereditary angioedema (HAE) is a bradykinin-mediated airway emergency that requires prompt on-demand therapy with a first-line agent that rapidly restores C1-INH activity. IV plasma-derived C1-INH at 20 IU/kg provides immediate substrate replacement, with the fastest onset to clinically meaningful airway improvement among guideline-preferred options in this context. Per the WAO/EAACI 2021 revision and the US HAEA 2020 guideline, pdC1-INH is the prioritized therapy for laryngeal attacks while the definitive airway is readied. Intravenous plasma-derived C1-esterase inhibitor 40 IU/kg as an empiric doubled dose now... is an off-label escalation not supported as routine initial therapy; standard dosing is 20 IU/kg, and additional dosing is considered only if the response is inadequate.... --------------------------------------------------- Our cases and questions come from the https://EndlessMedical.Academy quiz engine - multi-model platform. Each question and explanation is forged by consensus between multiple top AI models (GPT, Claude, Grok, etc.), with automated web searches for the latest research and verified references. Calculations (e.g. eGFR, dosages) are checked via code execution to eliminate errors, and all references are reviewed by several AIs to minimize hallucinations. Important note: This material is entirely AI-generated and has not been verified by human experts; despite stringent consensus checks, perfect accuracy cannot be guaranteed. Exercise caution - always corroborate the content with trusted references or qualified professionals, and never apply information from this book to patient care or clinical decisions without independent verification. Clinicians already rely on AI and online tools - myself included - so treat this book as an additional focused aid, not a replacement for proper medical education. Visit https://endlessmedical.academy for more AI-supported resources and cases. This material can not be treated as medical advice. May contain errors. ---------------------------------------------------