Elsevier

Immunobiology

Volume 225, Issue 6, November 2020, 152022
Immunobiology

Short communication
Successful perioperative management of three patients with hereditary angioedema without C1 esterase inhibitor therapy: A developing country perspective

https://doi.org/10.1016/j.imbio.2020.152022Get rights and content

Highlights

  • Surgical procedures, even minor ones, are known to precipitate an attack in HAE.

  • FFP and attenuated androgens may be used for short-term prophylaxis (STP).

  • We provide a risk assessment protocol to be considered while deciding STP.

Abstract

Background

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. However, there is limited experience with short term prophylaxis in countries where C1-INH therapy is not available.

Methods

To report our experience of using short term prophylaxis for a dental procedure, a Cesarean section and a major hip surgery in one patient each with HAE in resource constrained settings.

Results

All 3 patients were given FFP before and during the procedure. While the first (a 6-year-old girl) and third patient (a 60-year-old male) were already taking stanozolol and the dose was doubled 5 days before the surgery, the second patient (28-year-old woman) was not taking any prophylaxis and she was initiated on stanozolol on the day of Cesarean section. The first patient was also given additional FFP one day after the dental procedure. After the procedure, the dose of stanozolol was decreased to baseline in patient 1 and 3 while it was discontinued in patient 3.

All 3 patients tolerated the procedures well and had no related episodes of angioedema.

Conclusions

Dental and other major surgical procedures in patients with HAE are known to precipitate an episode of angioedema. In countries where C1-INH therapy is not available, attenuated androgens and FFP may be used to prevent these episodes.

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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