Hereditary Angioedema: Clinical Characteristics and Outcome of 36 Patients

Tanno, Luciana Kase; Pinto, L.; Motta, Antônio Abílio; Kalil, Jorge; Giavina‐Bianchi, Pedro

doi:10.1016/j.jaci.2006.12.445

Cited by 1 publication

(6 citation statements)

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“…The most commonly affected sites are the face, extremities, genitalia, oropharynx, larynx, and digestive system. However, rarer clinical manifestations, including intense headaches caused by brain edema, urinary retention, and acute pancreatitis, can also occur 12,35…”

Section: Diagnosismentioning

confidence: 99%

“…Attacks of laryngeal edema are particularly rare before three years of age and tend to occur later than other manifestations. During adolescence, there are substantial changes in disease activity, particularly in girls, for whom disease progression is worse because of their menstrual cycles and their use of contraceptives containing estrogen 6,11,12. …”

Section: Diagnosismentioning

confidence: 99%

“…However, rarer clinical manifestations, including intense headaches caused by brain edema, urinary retention, and acute pancreatitis, can also occur. 12 , 35 …”

Section: Diagnosismentioning

confidence: 99%

“…It has been reported that 5% of patients with HAE are asymptomatic, and 25% develop sporadic symptoms. 6 , 11 , 12 , A retrospective study analyzing 131,110 attacks in 221 patients with HAE reported that laryngeal edema occurred in less than 1% of the attacks, although over 50% of the patients had previously experienced that symptom at least once. 12 …”

Section: Diagnosismentioning

confidence: 99%

“…Left untreated, HAE attacks typically last 48-72 h. Although many attacks occur spontaneously, a number of triggering factors have been identified, including minor trauma, stress, infection, menstruation, pregnancy, alcohol consumption, a change in temperature, exercise, the use of angiotensin-converting enzyme (ACE) inhibitors, and the use of estrogen (including contraceptives and hormone replacement therapy). 6 , 11 , 12 ,…”

Section: Diagnosismentioning

confidence: 99%

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Brazilian guidelines for the diagnosis and treatment of hereditary angioedema

Giavina‐Bianchi

França

Grumach

et al. 2011

Clinics

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Hereditary angioedema is an autosomal dominant disease characterized by edema attacks with multiple organ involvement. It is caused by a quantitative or functional deficiency of the C1 inhibitor, which is a member of the serine protease inhibitor family. Hereditary angioedema is unknown to many health professionals and is therefore an underdiagnosed disease.The causes of death from hereditary angioedema include laryngeal edema with asphyxia. The estimated mortality rate in patients in whom the disease goes undetected and who are therefore incorrectly treated is 25-40%. In addition to edema of the glottis, hereditary angioedema often results in edema of the gastrointestinal tract, which can be incapacitating. Patients with hereditary angioedema may undergo unnecessary surgical interventions because the digestive tract can be the primary or only organ system involved, thus mimicking acute surgical abdomen. It is estimated that patients with hereditary angioedema experience some degree of disability 20-100 days per year.The Experts in Clinical Immunology and Allergy of the “Associação Brasileira de Alergia e Imunopatologia - ASBAI” developed these guidelines for the diagnosis, therapy, and management of hereditary angioedema.

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Section: Diagnosismentioning

confidence: 99%

Section: Diagnosismentioning

confidence: 99%

“…However, rarer clinical manifestations, including intense headaches caused by brain edema, urinary retention, and acute pancreatitis, can also occur. 12 , 35 …”

Section: Diagnosismentioning

confidence: 99%