Short-term prophylaxis in patients with angioedema due to C1-inhibitor deficiency undergoing dental procedures: An observational study

Rawat

Kaur

et al. 2020

Preprint

Background: There is paucity of literature on long term follow-up of patients with Hereditary angioedema (HAE) from developing countries. Objective: This study was carried out to analyse the clinical manifestations, laboratory features and genetic profile of 32 patients (21 male and 11 female) from 23 families diagnosed with HAE between January 1996 and December 2019. Methods: Data were retrieved from medical records of the Paediatric Immunodeficiency Clinic, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Results: Median age at onset of symptoms was 6.25 years (range 1-25 years) and median age at diagnosis was 12 years (range 2-43 years). Serum complement C4 level was decreased in all patients.

Section: Discussionmentioning

confidence: 78%

Novel SERPING1 gene mutations and clinical experience of type 1 hereditary angioedema from North India

Rawat

Kaur

et al. 2020

Preprint

“…Use of attenuated androgens and FFP has also been suggested if C1-INH is not available. 56,57 We successfully used stanozolol and FFP for short-term prophylaxis in 2 patients in the present series.…”

Section: Discussionmentioning

confidence: 80%

“…Plasma‐derived C1‐INH therapy is recommended as short‐term prophylaxis during these procedures. Use of attenuated androgens and FFP has also been suggested if C1‐INH is not available 56,57 . We successfully used stanozolol and FFP for short‐term prophylaxis in 2 patients in the present series.…”

Section: Discussionmentioning

confidence: 84%

Novel SERPING1 gene mutations and clinical experience of type 1 hereditary angioedema from North India

Pediatric Allergy Immunology

Rawat

Kaur

et al. 2020

“…Several observational studies have shown that use of short-term prophylaxis during high-risk procedures is effective in preventing an attack of angioedema. [ 34 35 36 37 ]…”

Section: Management Of Haementioning

confidence: 99%

Hereditary Angioedema

Indian Dermatology Online Journal

Bishnoi

Dogra

2021

Hereditary angioedema (HAE) is an uncommon disorder with a global prevalence of approximately 1 in 10,000 to 1 in 50,000 population. This disease is grossly underrecognized in India because of lack of awareness and/or lack of diagnostic facilities. Clinical manifestations include swelling over face, eyes, lips, hands, feet, and genitals, abdominal pain, and life-threatening laryngeal edema. HAE should be suspected in all patients who present with angioedema without wheals and who do not respond to antihistamines and/or steroids. C1 levels, C1-INH levels, and C1-INH function should be checked in all patients suspected to have HAE. C1q levels should be assessed in patients with suspected autoimmune-mediated acquired angioedema. Management of HAE constitutes the treatment of acute attack and short-term and long-term prophylaxis. Because of lack of all first-line recommended medications, the management of HAE in India is a challenging task. Patients are managed using fresh frozen plasma (acute treatment), tranexamic acid, and attenuated androgens (prophylaxis). Even though attenuated androgens have been shown to be effective in the prevention of attacks of HAE, the side effect profile especially in children and in females is a serious concern. Hence, the treatment needs to be individualized considering the risk-benefit ratio of long-term prophylaxis. In this review, we provide an overview of diagnostic strategy for patients with HAE and the current treatment concepts with emphasis on currently available treatment options in resource-constrained settings.