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Successful C1 inhibitor short-term prophylaxis during redo mitral valve replacement in a patient with hereditary angioedema.

Bernstein JA, Coleman S, Bonnin AJ - J Cardiothorac Surg (2010)

Bottom Line: Laryngeal swelling carries significant risk for asphyxiation.However, uncomplicated surgeries have been reported.Appropriate prophylaxis can reduce peri-operative morbidity in these patients, despite proteolytic cascade and complement activation during surgical trauma.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Division of Immunology/Allergy Section, University of Cincinnati, 231 Albert Sabin Way, Cincinnati, Ohio, USA. bernstja@ucmail.uc.edu

ABSTRACT
Hereditary angioedema is characterized by sudden episodes of nonpitting edema that cause discomfort and pain. Typically the extremities, genitalia, trunk, gastrointestinal tract, face, and larynx are affected by attacks of swelling. Laryngeal swelling carries significant risk for asphyxiation. The disease results from mutations in the C1 esterase inhibitor gene that cause C1 esterase inhibitor deficiency. Attacks of hereditary angioedema result from contact, complement, and fibrinolytic plasma cascade activation, where C1 esterase inhibitor irreversibly binds substrates. Patients with hereditary angioedema cannot replenish C1 esterase inhibitor levels on pace with its binding. When C1 esterase inhibitor is depleted in these patients, vasoactive plasma cascade products cause swelling attacks. Trauma is a known trigger for hereditary angioedema attacks, and patients have been denied surgical procedures because of this risk. However, uncomplicated surgeries have been reported. Appropriate prophylaxis can reduce peri-operative morbidity in these patients, despite proteolytic cascade and complement activation during surgical trauma. We report a case of successful short-term prophylaxis with C1 esterase inhibitor in a 51-year-old man with hereditary angioedema who underwent redo mitral valve reconstructive surgery.

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C1 INH action in plasma cascades. Depletion of C1 INH due to plasma cascade activation allows bradykinin overproduction in patients with hereditary angioedema. Bradykinin mediates acute swelling in these patients. Reproduced with permission from Weis[15].
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Figure 1: C1 INH action in plasma cascades. Depletion of C1 INH due to plasma cascade activation allows bradykinin overproduction in patients with hereditary angioedema. Bradykinin mediates acute swelling in these patients. Reproduced with permission from Weis[15].

Mentions: Histamine mediated allergic inflammation is not involved in HAE[7]. Instead, HAE attacks result from contact, complement, and fibrinolytic plasma cascade activation, where C1 INH is a suicide inhibitor[2]. People with HAE have defective C1 INH synthesis with typical C1 INH levels that are 5%-30% of normal.2 Bradykinin is generated in large quantities via the contact pathway once C1 INH is depleted (Figure 1)[2]. Excess bradykinin production leads to acute HAE attacks as a result of increased vasodilatation, vascular permeability, and contraction of nonvascular smooth muscle[3].


Successful C1 inhibitor short-term prophylaxis during redo mitral valve replacement in a patient with hereditary angioedema.

Bernstein JA, Coleman S, Bonnin AJ - J Cardiothorac Surg (2010)

C1 INH action in plasma cascades. Depletion of C1 INH due to plasma cascade activation allows bradykinin overproduction in patients with hereditary angioedema. Bradykinin mediates acute swelling in these patients. Reproduced with permission from Weis[15].
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2965712&req=5

Figure 1: C1 INH action in plasma cascades. Depletion of C1 INH due to plasma cascade activation allows bradykinin overproduction in patients with hereditary angioedema. Bradykinin mediates acute swelling in these patients. Reproduced with permission from Weis[15].
Mentions: Histamine mediated allergic inflammation is not involved in HAE[7]. Instead, HAE attacks result from contact, complement, and fibrinolytic plasma cascade activation, where C1 INH is a suicide inhibitor[2]. People with HAE have defective C1 INH synthesis with typical C1 INH levels that are 5%-30% of normal.2 Bradykinin is generated in large quantities via the contact pathway once C1 INH is depleted (Figure 1)[2]. Excess bradykinin production leads to acute HAE attacks as a result of increased vasodilatation, vascular permeability, and contraction of nonvascular smooth muscle[3].

Bottom Line: Laryngeal swelling carries significant risk for asphyxiation.However, uncomplicated surgeries have been reported.Appropriate prophylaxis can reduce peri-operative morbidity in these patients, despite proteolytic cascade and complement activation during surgical trauma.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Division of Immunology/Allergy Section, University of Cincinnati, 231 Albert Sabin Way, Cincinnati, Ohio, USA. bernstja@ucmail.uc.edu

ABSTRACT
Hereditary angioedema is characterized by sudden episodes of nonpitting edema that cause discomfort and pain. Typically the extremities, genitalia, trunk, gastrointestinal tract, face, and larynx are affected by attacks of swelling. Laryngeal swelling carries significant risk for asphyxiation. The disease results from mutations in the C1 esterase inhibitor gene that cause C1 esterase inhibitor deficiency. Attacks of hereditary angioedema result from contact, complement, and fibrinolytic plasma cascade activation, where C1 esterase inhibitor irreversibly binds substrates. Patients with hereditary angioedema cannot replenish C1 esterase inhibitor levels on pace with its binding. When C1 esterase inhibitor is depleted in these patients, vasoactive plasma cascade products cause swelling attacks. Trauma is a known trigger for hereditary angioedema attacks, and patients have been denied surgical procedures because of this risk. However, uncomplicated surgeries have been reported. Appropriate prophylaxis can reduce peri-operative morbidity in these patients, despite proteolytic cascade and complement activation during surgical trauma. We report a case of successful short-term prophylaxis with C1 esterase inhibitor in a 51-year-old man with hereditary angioedema who underwent redo mitral valve reconstructive surgery.

Show MeSH
Related in: MedlinePlus