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

Authors
  • Jindal, Ankur Kumar1
  • Singh, Ankita2
  • Anjani, Gummadi2
  • Kaur, Anit2
  • Jaiswal, Manojkumar3
  • Chopra, Seema4
  • Saini, Uttam5
  • Mahajan, Shalvi6
  • Rawat, Amit2
  • Singh, Surjit2
  • Longhurst, Hilary7
  • 1 Allergy Immunology Unit Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. Electronic address: [email protected] , (India)
  • 2 Allergy Immunology Unit Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. , (India)
  • 3 Department of Oral Health Sciences, Postgraduate Institute of Medical Education and Research, Chandigarh, India. , (India)
  • 4 Department of Obstetrics and Gynaecology, Postgraduate Institute of Medical Education and Research, Chandigarh, India. , (India)
  • 5 Department of Orthopedics, Postgraduate Institute of Medical Education and Research, Chandigarh, India. , (India)
  • 6 Department of Anesthesia, Postgraduate Institute of Medical Education and Research, Chandigarh, India. , (India)
  • 7 Department of Clinical Immunology, University College Hospitals, London and Addenbrooke's Hospital, Cambridge, UK.
Type
Published Article
Journal
Immunobiology
Publication Date
Nov 01, 2020
Volume
225
Issue
6
Pages
152022–152022
Identifiers
DOI: 10.1016/j.imbio.2020.152022
PMID: 33197705
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

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. 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. 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. 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. Copyright © 2020 Elsevier GmbH. All rights reserved.

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