Short communicationSuccessful perioperative management of three patients with hereditary angioedema without C1 esterase inhibitor therapy: A developing country perspective
Introduction
Hereditary angioedema (HAE) is a rare inherited disorder characterized by sudden and unpredictable appearance of swelling. Surgical procedures, even minor ones, are known to precipitate an attack in these patients. C1 esterase inhibitor (C1-INH) therapy may be effective for short term prophylaxis in such situations (Busse et al., 2017, Zanichelli et al., 2020). However, when this therapy is either not available or not affordable, one has to take recourse to alternative measures with fresh frozen plasma (FFP) and attenuated androgens. We report 3 such cases who were given short term prophylaxis with FFP and attenuated androgens, which successfully prevented procedure-related angioedema.
Section snippets
Case 1
A 6-year-old girl was diagnosed to have HAE at the age of 3. Her father had had similar complaints since early childhood. Laboratory investigations revealed low C4 levels (0.04 g/L; Normal 0.16–0.48 g/L), low C1-INH levels: 0.08 g/L (normal: 0.19–0.37) and low C1 inhibitor activity: 16.7% (normal: >68%). She was initiated on tranexamic acid (500 mg per day). However, because of inadequate response her therapy was switched to low dose stanozolol (0.5 mg per day). Her symptoms improved on this
Discussion
Episodes of angioedema in patients with HAE are unpredictable. However, an attack is often precipitated by surgical and dental procedures and may occasionally lead to life-threatening laryngeal edema (Hermans et al., 2012, Bork and Barnstedt, 2003). Up to 30% patients may develop an episode of angioedema after a surgical procedure in absence of prophylaxis (Aygören-Pürsün et al., 2013). Short term use of plasma derived C1-INH therapy has been recommended as the treatment of choice in these
CRediT authorship contribution statement
Ankur Kumar Jindal: Conceptualization, Methodology, Validation, Investigation, Data curation, Writing - original draft, Writing - review & editing, Visualization. Ankita Singh: . Gummadi Anjani: Conceptualization, Methodology, Data curation, Writing - original draft. Anit Kaur: Investigation, Data curation, Writing - original draft. Manojkumar Jaiswal: Data curation, Writing - original draft, Visualization. Seema Chopra: Data curation, Writing - original draft, Visualization. Uttam Saini: Data
Declaration of Competing Interest
The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
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