Related Experiment Video

Updated: May 23, 2025

Drug Treatment by Central Venous Catheter in a Mouse Model of Angiotensin II Induced Abdominal Aortic Aneurysm and Monitoring by 3D Ultrasound
10:09

Drug Treatment by Central Venous Catheter in a Mouse Model of Angiotensin II Induced Abdominal Aortic Aneurysm and Monitoring by 3D Ultrasound

Published on: August 4, 2022

3.1K

Hereditary Angioedema With Normal C1 Inhibitor: A Quarter Century of Forward Progress and Persisting Obstacles

Sandra C Christiansen1, Aleena Banerji2, Jonathan A Bernstein3

  • 1Division of Allergy, Department of Medicine, University of California San Diego, La Jolla, Calif.

The Journal of Allergy and Clinical Immunology. in Practice
|March 8, 2025
PubMed

Insights

Hereditary angioedema with normal C1 inhibitor (HAE-nl-C1INH) remains challenging to diagnose and treat. This review summarizes 25 years of progress, proposing a classification schema to guide physicians in managing patients with recurrent angioedema.

Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Hereditary angioedema with normal C1 inhibitor (HAE-nl-C1INH) was first described 25 years ago.
  • Despite progress, diagnosing, classifying, and treating HAE-nl-C1INH present significant clinical challenges.
  • Understanding the pathomechanisms of HAE-nl-C1INH is crucial for effective management.

Purpose of the Study:

  • To summarize current scientific consensus on HAE-nl-C1INH.
  • To provide insights for physicians caring for patients with HAE-nl-C1INH.
  • To propose a classification schema to aid in the evaluation of recurrent angioedema.

Main Methods:

  • Review of scientific literature over the past 25 years.
  • Synthesis of current data and expert opinion.
  • Development of a proposed classification schema for HAE-nl-C1INH.

Main Results:

  • Significant scientific developments have occurred in understanding HAE-nl-C1INH.
  • A proposed classification schema aims to facilitate clinical decision-making.
  • Accurate diagnosis and understanding of pathomechanisms are key to targeted treatment.

Conclusions:

  • The proposed classification schema can guide physicians in managing HAE-nl-C1INH.
  • Targeted treatment strategies can be designed based on accurate diagnosis and pathomechanism.
  • Further advances in understanding HAE-nl-C1INH are anticipated to improve patient outcomes.

Keywords:
3-OST-6AngioedemaAngiopoietin 1BradykininC1 inhibitorClassificationCoagulation factor XIIDiagnosisHereditary angioedemaHereditary angioedema with normal C1 inhibitorKininogenMast cellMyoferlinPlasminogen

More Related Videos

Vascular Balloon Injury and Intraluminal Administration in Rat Carotid Artery09:41

Vascular Balloon Injury and Intraluminal Administration in Rat Carotid Artery

Published on: December 23, 2014

16.3K
Subcutaneous Angiotensin II Infusion using Osmotic Pumps Induces Aortic Aneurysms in Mice07:21

Subcutaneous Angiotensin II Infusion using Osmotic Pumps Induces Aortic Aneurysms in Mice

Published on: September 28, 2015

37.1K

Related Concept Videos

Antihypertensive Drugs: Angiotensin-Converting Enzyme Inhibitors01:30

Antihypertensive Drugs: Angiotensin-Converting Enzyme Inhibitors

Angiotensin-converting enzyme (ACE), a vital component of the renin-angiotensin-aldosterone system, is abundant in lung endothelial cells. ACE converts the inactive decapeptide, angiotensin I, into the active octapeptide, angiotensin II. This potent vasoconstrictor narrows blood vessels, increasing resistance to blood flow and elevating blood pressure. Angiotensin II also stimulates aldosterone production, encouraging kidney cells to reabsorb more sodium and water from urine, thereby increasing...
440
Antihypertensive Drugs: Direct Renin Inhibitors01:25

Antihypertensive Drugs: Direct Renin Inhibitors

The renin-angiotensin-aldosterone system (RAAS) is an intricate physiological pathway involving numerous enzymes and hormones, including renin, angiotensin-converting enzyme (ACE), angiotensin I and II, and aldosterone. Imbalances within this system increase the production of angiotensin II and aldosterone. Increased angiotensin II levels promote vasoconstriction and blood pressure elevation. Concurrently, higher aldosterone levels stimulate sodium and water reabsorption in the kidneys,...
468
Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers01:26

Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers

Receptor tyrosine kinase inhibitors (TKIs) and calcium channel blockers (CCBs) are two critical categories of drugs employed in the treatment of pulmonary artery hypertension (PAH). PAH is a disease that causes high blood pressure in the pulmonary arteries, resulting in chest pain, fatigue, and shortness of breath.
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...
126
Heart Failure Drugs: Inhibitors of Renin-Angiotensin System01:26

Heart Failure Drugs: Inhibitors of Renin-Angiotensin System

The activation of the sympathetic nervous system and the renin-angiotensin-aldosterone system (RAAS) contributes to cardiac remodeling, and inhibiting the RAAS is a pharmacological target in heart failure management. As a result, neurohumoral modulation is a crucial treatment principle for managing heart failure. This approach involves using medications like ACE inhibitors (ACEIs), angiotensin receptor blockers (ARBs), β-blockers, mineralocorticoid receptor antagonists (MRAs), and neutral...
360
Angle Closure Glaucoma: Treatment01:28

Angle Closure Glaucoma: Treatment

Angle-closure glaucoma, or closed-angle glaucoma, is an eye condition where the iris bulges out and blocks the iridocorneal angle, resulting in a buildup of aqueous humor and increased intraocular pressure. Immediate medical attention is necessary due to the sudden onset of symptoms. The treatment for angle-closure glaucoma includes short-term and long-term approaches. Short-term treatment involves using eye drops like pilocarpine to lower intraocular pressure by increasing aqueous humor...
425
Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists01:18

Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists

Endothelins (ETs) are potent vasoactive peptides critical in the human body's various physiological and pathological processes. One of the most promising therapeutic strategies for treating pulmonary arterial hypertension (PAH) involves counteracting the effects of these endothelins using a class of drugs known as endothelin receptor antagonists.
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
122