Management of hereditary angioedema in pediatric patients

Henriette Farkas1, Lilian Varga, Gábor Széplaki

  • 13rd Department of Internal Medicine, Semmelweis University, Kútvölgyi út 4, H-1125, Budapest, Hungary. farkash@kut.sote.hu

Pediatrics
|August 29, 2007
PubMed

Insights

Hereditary angioneurotic edema, a rare genetic disorder, involves C1 inhibitor deficiency causing swelling. This review focuses on effective management strategies for pediatric hereditary angioneurotic edema, addressing a gap in current literature.

Area of Science:

  • Immunology
  • Genetics
  • Pediatrics

Background:

  • Hereditary angioneurotic edema (HAE) is a rare genetic disorder characterized by recurrent swelling episodes.
  • It results from a deficiency or dysfunction of the C1 inhibitor (C1-INH).
  • Conventional treatments like antihistamines and corticosteroids are often ineffective for HAE.

Purpose of the Study:

  • To review the experience and published data on managing pediatric HAE.
  • To provide a comprehensive approach to HAE in childhood.
  • To address the limited literature specifically on pediatric HAE management.

Main Methods:

  • Review of institutional experience with HAE.
  • Systematic review of published literature on pediatric HAE.
  • Analysis of diagnostic and therapeutic strategies for HAE in children.

Main Results:

  • HAE manifestations typically appear within the first two decades of life.
  • There is a significant delay between symptom onset and HAE diagnosis.
  • Current literature on pediatric HAE management is scarce.

Conclusions:

  • A specialized approach is needed for managing HAE in children.
  • Early diagnosis and appropriate treatment are crucial for improving outcomes in pediatric HAE.
  • Further research and guidelines for pediatric HAE are warranted.

Related Concept Videos

Angina IV: Management01:26

Angina IV: Management

IntroductionThe management of angina requires a comprehensive approach that includes pharmacological therapies, medical procedures, and lifestyle modifications.Pharmacological TherapiesAntiplatelet agents, such as aspirin, clopidogrel, prasugrel, and ticagrelor, play a pivotal role in preventing thrombus formation in patients with angina. These medications inhibit platelet aggregation and reduce the likelihood of myocardial infarction and other cardiovascular events.Anticoagulants, including...
Mitral Stenosis III: Medical Management01:26

Mitral Stenosis III: Medical Management

Mitral stenosis, a condition marked by the narrowing of the mitral valve, necessitates an integrated approach for effective management. This approach includes preventative measures, medical therapy, and surgical interventions to reduce symptoms and prevent complications.PreventionPrevention of mitral stenosis primarily focuses on reducing the incidence of bacterial infections, particularly streptococcal infections, which can lead to rheumatic fever and subsequent valvular damage. Timely...
Aortic Regurgitation III: Medical Management01:25

Aortic Regurgitation III: Medical Management

Aortic regurgitation (AR) is when the aortic valve does not close or seal properly, leading to backward blood circulation from the aorta into the left ventricle during diastole. Common causes of AR include rheumatic heart disease, congenital valve defects, and aortic root dilation. Managing AR requires a multifaceted approach to alleviate symptoms, preserve left ventricular function, and address the underlying cause of the regurgitation. Patients with symptomatic AR or significant left...
Pharmacokinetics in Pediatric Patients: Drug Excretion01:26

Pharmacokinetics in Pediatric Patients: Drug Excretion

In pediatric medicine, understanding the renal function and drug elimination nuances is crucial for administering safe and effective treatments. Newborns, in particular, display markedly slower renal functions than adults, profoundly affecting how drugs are cleared from their bodies. This slower drug clearance requires clinicians to extend the dosing intervals for many medications to prevent drug accumulation and toxicity while ensuring therapeutic efficacy.One key area where these adjustments...
Nephrotic Syndrome II : Assessment and Medical Management01:26

Nephrotic Syndrome II : Assessment and Medical Management

IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document any history...
Antiasthma Drugs: Mast Cell Stabilizers and Anti-IgE Drugs01:25

Antiasthma Drugs: Mast Cell Stabilizers and Anti-IgE Drugs

Asthma is a chronic respiratory condition for which new therapeutic avenues, including anti-inflammatory drugs like mast cell stabilizers and anti-IgE treatments, continue to be developed.
Mast cell stabilizers, such as cromolyn (also known as sodium cromoglycate) and nedocromil (Tilade), are effective drugs in asthma management. These stabilizers hinder histamine release by skillfully obstructing the activation of mast cells and other cellular entities. Notably, they navigate this task without...