Hereditary angioedema is an episodic swelling disorder with autosomal dominant inheritance. Attacks are characterized by brawny, self-limited, nonpruritic edema of the deep dermal layers of the skin that most often involve the hands and feet. They usually begin in childhood and become more severe after puberty. Patients also have episodic attacks of severe abdominal pain caused by edema of the ...

THREE TYPES OF HEREDITARY ANGIOEDEMA (HAE) HAVE BEEN DESCRIBED two are due to C1 inhibitor (C1-INH) deficiency (C1-INH-HAE types I and II) and one is characterized by normal C1-INH (nC1-INH-HAE). The management of pregnancy in patients with HAE is often a clinical challenge owing to potential worsening of the disease in relation to the physiological increase in estrogens and the limited treatme...

Until recently it was assumed that hereditary angioedema is a disease that results exclusively from a genetic deficiency of the C1 inhibitor. In 2000, families with hereditary angioedema, normal C1 inhibitor activity and protein in plasma were described. Since then numerous patients and families with that condition have been reported. Most of the patients by far were women. In many of the affec...

Icatibant (Firazyr(®)) is a novel subcutaneous treatment recently licensed in the European Union for acute hereditary angioedema. Hereditary angioedema, resulting from inherited partial C1 inhibitor deficiency, is a disabling condition characterized by intermittent episodes of bradykinin-mediated angioedema. Icatibant blocks bradykinin B2 receptors, attenutating the episode. Randomized double-b...

Accurate and timely diagnostics of acute abdominal pain, a common emergency, is crucial in decreasing unnecessary surgical interventions. We present the case of a patient, Xh. M. aged 21, transported to emergency after being wakened from sleep by severe, acute abdominal pain. The pain was non-radiating, colic, and associated with flatulence, nausea and vomiting. The family history was negative ...

BACKGROUND The treatment of hereditary angioedema (HAE) has undergone dramatic changes as newer medicines have become available in recent years. Optimal care of these patients requires a comprehensive management plan. Although several consensus papers have been published concerning the diagnosis and treatment of HAE, guidelines for a comprehensive management plan have not been developed. OBJE...

C1 esterase inhibitor (Berinert®) is generally used to treat severe attack of hereditary angioedema. We describe here the case of a patient who presented with a severe angioedema induced by angiotensin-converting enzyme inhibitors (ACEIs) endangering her life. It could be successfully treated with that medicine.

Emergency physicians (EP) frequently encounter angioedema involving the lips and tongue. However, angioedema from Angiotensin Converting Enzyme inhibitors or hereditary angioedema (HAE) can present with gastrointestinal symptoms due to bowel wall involvement. EPs should begin to consider this clinical entity as a potential cause for abdominal pain and associated gastrointestinal symptoms given ...

Introduction Hereditary angioedema (HAE) is a rare, genetic disease with deficiency or the occurrence of defective C1 esterase inhibitor. The result acutely occurring subcutaneous submucosal edema in various parts body, which can range from cosmetically disruptive to extremely painful life-threatening. In addition physical consequences, unpredictability many attacks and subsequent restrictions ...