Hereditary Angioedema: Three Cases Report, Members of the Same Family

Abstract

BackgroundThis current clinical case report highlights three cases of Hereditary angioedema (HAE) patients who are all members of the same family (father and his two daughters). The father has C1–INH deficiency, while his daughters have low C1–INH levels: the first possesses only 10% function and the second has low C1–INH level with 0% function. Of note, the second daughter was discovered to have HAE at the age of 2, thus making her the youngest known HAE case report in the English literature.MethodsAssess the efficacy of administration of C1-INH before dental operation as regards the prevention of HAE episode, when total or partial C1-INH deficiency existResultsAcute angioedema leading to laryngeal oedema is a possibly fatal complication for HAE patients undergoing dental procedures. Use of both short-term and long-term HAE prophylaxis prior to dental operations might be life saving for those patients.ConclusionsPrevention and early recognition of potential laryngeal oedema that can occur as a complication of dental procedures may be lifesaving for HAE patients.

How we manage persons with hereditary angioedema

“…over time. Symptomatic onset may occur in children as young as 2 years, with mean age of onset in the range of 8-12 years (Roche et al, 2005;Bork et al, 2006a;Papamanthos et al, 2010). We recently reported that earlier age of symptom onset correlates with enhanced disease severity as well as increased delay to diagnosis (Christiansen et al, 2015a), both of which were true for our case.…”

Perioperative Management of Tooth Extractions for a Patient With Hereditary Angioedema

“…However, as described previously, tracheal intubation is a factor reportedly associated with an increased risk for a laryngeal attack of HAE. 4 Therefore, sedation was chosen to avoid the need for tracheal intubation. Sedation was chosen for another reason.…”

“…HAE attacks can be triggered by physical or psychological stress or can arise spontaneously without any apparent trigger. [2][3][4] Dental treatments and routine oral surgical procedures, such as tooth extraction, abound with factors that can trigger an attack of HAE. [2][3][4] Indeed, several cases of death resulting from HAE attacks have been reported after such procedures.…”

“…[2][3][4] Dental treatments and routine oral surgical procedures, such as tooth extraction, abound with factors that can trigger an attack of HAE. [2][3][4] Indeed, several cases of death resulting from HAE attacks have been reported after such procedures. 5 Therefore, patients with HAE are of special concern in dentistry and require precautionary preparations before treatment.…”

“…[1][2][3][4] Although almost any part of the body can be affected, HAE is of greatest concern and can be life-threatening when the upper airway is involved, particularly the larynx (laryngeal attack). HAE attacks can be triggered by physical or psychological stress or can arise spontaneously without any apparent trigger.…”