Related Experiment Video
Updated: Apr 19, 2026
07:21
Subcutaneous Angiotensin II Infusion using Osmotic Pumps Induces Aortic Aneurysms in Mice
Published on: September 28, 2015
40.0K
Pathophysiology of Hereditary Angioedema
Sonia Caccia1, Chiara Suffritti2, Marco Cicardi3
1Department of Biotechnologies and Translational Medicine, University of Milan, Milan, Italy .
Pediatric Allergy, Immunology, and Pulmonology
|December 25, 2014
Summary
Hereditary angioedema (HAE) is caused by a genetic C1 inhibitor deficiency, leading to recurrent swelling attacks. This review details the contact system activation and bradykinin
Area of Science:
- Genetics
- Immunology
- Pathophysiology
Background:
- Hereditary angioedema (HAE) is an autosomal dominant disorder.
- It stems from a genetic deficiency of the C1 inhibitor.
- Over 200 mutations in the C1 inhibitor gene are linked to HAE.
Approach:
- This review synthesizes current knowledge on HAE.
- It examines the role of the contact system and bradykinin.
- Mechanisms of angioedema in C1 inhibitor deficiency are described.
Key Points:
- C1 inhibitor deficiency disrupts physiological control of the contact system.
- Bradykinin, derived from contact system activation, mediates angioedema.
- Recurrent angioedema attacks are a hallmark of HAE.
Conclusions:
- Understanding C1 inhibitor's role is crucial for HAE pathogenesis.
- The contact system's dysregulation explains angioedema development.
- This review provides insights into HAE mechanisms.
Related Concept Videos
01:18Pulmonary Edema II: Pathophysiology
2
Pulmonary edema is the accumulation of fluid in the interstitial and alveolar spaces of the lungs, impairing gas exchange and oxygen delivery. It may be cardiogenic or noncardiogenic, but both reduce oxygenation and lung compliance.Cardiogenic Pulmonary EdemaCardiogenic edema results from increased hydrostatic pressure in pulmonary capillaries, usually due to left ventricular dysfunction from myocardial infarction, heart failure, or valvular disease. Ineffective cardiac pumping causes blood to...
2
01:22Cerebral Edema ll: Pathophysiology
1
Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this...
1
01:29Hypertension II: Pathophysiology
1.7K
Hypertension is a chronic condition in which the blood's force against artery walls is excessively high, posing risks such as heart disease. The condition's underlying mechanisms involve complex interactions among the cardiovascular, kidney, and autonomic nervous systems.Renin-Angiotensin-Aldosterone System (RAAS): This system significantly influences blood pressure regulation. When blood pressure decreases, the kidneys secrete renin. This enzyme transforms angiotensinogen, a plasma protein,...
1.7K
01:21Cytotoxic Edema: Pathophysiology
1
Cytotoxic edema is a form of cerebral edema characterized by intracellular swelling of neurons, astrocytes, and other glial cells. It develops when the mechanisms responsible for maintaining ionic gradients across the cell membrane become impaired. Under normal physiological conditions, the sodium–potassium ATPase actively transports sodium ions out of the cell and potassium ions into the cell, preserving osmotic balance and enabling electrical signaling. This pump requires a continuous...
1
01:20Asthma: Pathogenesis and Management
1.7K
Asthma is a chronic pulmonary condition involving inflammation of the airways, hyper-reactivity, and reversible obstruction of the airways. This condition can significantly impact a person's quality of life, making breathing difficult and leading to distressing symptoms.
Asthma is classified as allergic and non-allergic. Allergens such as dust mites, pollen, and pet dander trigger allergic asthma, while factors like cold air, intense emotions, or exercise can induce non-allergic asthma.
Asthma is classified as allergic and non-allergic. Allergens such as dust mites, pollen, and pet dander trigger allergic asthma, while factors like cold air, intense emotions, or exercise can induce non-allergic asthma.
1.7K
01:19Cerebral Edema l: Introduction
1
Cerebral edema is a pathological increase in brain water content that disrupts intracranial pressure regulation and impairs neurological function. Because the cranial vault is rigid, even modest increases in tissue volume can compromise cerebral perfusion, distort neural structures, and initiate secondary injury. Cerebral edema develops through four principal mechanisms: vasogenic, cytotoxic, interstitial, and ionic.Vasogenic EdemaVasogenic edema arises from disruption of the blood–brain...
1