Hereditary Angioedema Type I Triggered by Dental Treatment: A Case Report and Review of the Literature

Yashoda Jagatiya; Sangeetha Yogarajah; Luis Monteiro; Barbara Carey; Rui Albuquerque

doi:10.12968/DENU.2022.49.1.57

Hereditary Angioedema Type I Triggered by Dental Treatment: A Case Report and Review of the Literature

Yashoda Jagatiya, Sangeetha Yogarajah, Luis Monteiro, Barbara Carey, Rui Albuquerque^*

^*Corresponding author for this work

Centre for Oral, Clinical & Translational Sciences

Research output: Contribution to journal › Review article › peer-review

Abstract

Type 1 hereditary angioedema (HAE) is a rare genetic condition characterized by recurrent episodes of oedema caused by a deficiency of C1-esterase inhibitor (C1-INH). A 29-year-old male presented to the oral medicine department at Guy’s Hospital, London, with lip swelling following crown preparation and impressions. Haematological investigations showed reduced levels of complement C4 (0.02 g/L; reference range 0.1–0.4 g/L) and C1-INH function was <31% (reference range 85–99%). Immunology confirmed the diagnosis of type 1 HAE, with a de novo mutation. This case highlights how a detailed medical history and multidisciplinary teamwork ensure the correct diagnosis and management. CPD/Clinical Relevance: To demonstrate the various dental triggers, relevant signs and symptoms, and management options for patients diagnosed with hereditary angioedema to allow for effective decision-making in a primary dental setting.

Original language	English
Pages (from-to)	57-62
Number of pages	6
Journal	Dental Update
Volume	49
Issue number	1
DOIs	https://doi.org/10.12968/DENU.2022.49.1.57
Publication status	Published - 2 Jan 2022

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title = "Hereditary Angioedema Type I Triggered by Dental Treatment: A Case Report and Review of the Literature",

abstract = "Type 1 hereditary angioedema (HAE) is a rare genetic condition characterized by recurrent episodes of oedema caused by a deficiency of C1-esterase inhibitor (C1-INH). A 29-year-old male presented to the oral medicine department at Guy{\textquoteright}s Hospital, London, with lip swelling following crown preparation and impressions. Haematological investigations showed reduced levels of complement C4 (0.02 g/L; reference range 0.1–0.4 g/L) and C1-INH function was <31% (reference range 85–99%). Immunology confirmed the diagnosis of type 1 HAE, with a de novo mutation. This case highlights how a detailed medical history and multidisciplinary teamwork ensure the correct diagnosis and management. CPD/Clinical Relevance: To demonstrate the various dental triggers, relevant signs and symptoms, and management options for patients diagnosed with hereditary angioedema to allow for effective decision-making in a primary dental setting.",

author = "Yashoda Jagatiya and Sangeetha Yogarajah and Luis Monteiro and Barbara Carey and Rui Albuquerque",

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AU - Yogarajah, Sangeetha

AU - Monteiro, Luis

AU - Carey, Barbara

AU - Albuquerque, Rui

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AB - Type 1 hereditary angioedema (HAE) is a rare genetic condition characterized by recurrent episodes of oedema caused by a deficiency of C1-esterase inhibitor (C1-INH). A 29-year-old male presented to the oral medicine department at Guy’s Hospital, London, with lip swelling following crown preparation and impressions. Haematological investigations showed reduced levels of complement C4 (0.02 g/L; reference range 0.1–0.4 g/L) and C1-INH function was <31% (reference range 85–99%). Immunology confirmed the diagnosis of type 1 HAE, with a de novo mutation. This case highlights how a detailed medical history and multidisciplinary teamwork ensure the correct diagnosis and management. CPD/Clinical Relevance: To demonstrate the various dental triggers, relevant signs and symptoms, and management options for patients diagnosed with hereditary angioedema to allow for effective decision-making in a primary dental setting.

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Hereditary Angioedema Type I Triggered by Dental Treatment: A Case Report and Review of the Literature

Abstract

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