Related Experiment Video

Updated: Jul 6, 2026

Determination of the Relative Cell Surface and Total Expression of Recombinant Ion Channels Using Flow Cytometry
11:32

Determination of the Relative Cell Surface and Total Expression of Recombinant Ion Channels Using Flow Cytometry

Published on: September 28, 2016

Ion channel diseases in children: manifestations and management

Peter J Schwartz1, Lia Crotti

  • 1Department of Cardiology and Molecular Cardiology Laboratory, IRCCS Fondazione Policlinico S. Matteo and University of Pavia, Pavia, Italy. pjqt@compuserve.com

Current Opinion in Cardiology
|April 3, 2008
PubMed

Insights

This review details the management of cardiac ion channel diseases, including long QT syndrome (LQTS). It covers current and emerging therapies, emphasizing gene-specific approaches and the challenges of implantable cardioverter defibrillators in children.

Area of Science:

  • Cardiology
  • Genetics
  • Pediatrics

Background:

  • The discovery of LQTS genes in 1995 increased awareness of cardiac ion channel diseases.
  • Diagnoses of these complex and potentially lethal conditions are becoming more frequent.
  • There is an increasing need for comprehensive management strategies.

Purpose of the Study:

  • To provide practical insights into the management of cardiac ion channel diseases.
  • To update clinicians on current therapeutic approaches and recent advances.
  • To address the specific challenges in pediatric patients.

Main Methods:

  • Review of current literature on cardiac ion channel disease management.
  • Focus on genetic bases, prevalence, and therapeutic strategies.
  • Consideration of traditional, novel, and gene-specific treatments.

Main Results:

  • Discussion of established and emerging therapies for LQTS and related disorders.
  • Exploration of gene-specific management advancements.
  • Analysis of implantable cardioverter defibrillator (ICD) use in children, including quality of life impacts and side effects.

Conclusions:

  • Empowering cardiologists to manage pediatric cardiac ion channel disease patients with increased confidence.
  • Facilitating better understanding of the unique medical issues associated with these disorders.
  • Guiding optimal treatment decisions for improved patient outcomes.
Abstract

Related Concept Videos

Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Barrett Esophagus-II: Clinical Manifestations and Management01:21

Barrett Esophagus-II: Clinical Manifestations and Management

Individuals with Barrett's esophagus are often asymptomatic, but they may experience symptoms commonly associated with GERD, such as heartburn and acid regurgitation. Additional symptoms can include difficulty swallowing, chest pain, unintentional weight loss, blood in the stool (which may appear black, tarry, or bloody), and episodes of vomiting.
To diagnose Barrett's esophagus, healthcare providers often recommend an endoscopy for those showing symptoms of acid reflux. The procedure entails...
Esophageal Strictures-II: Clinical Features and Management01:26

Esophageal Strictures-II: Clinical Features and Management

Patients with esophageal strictures often experience a range of symptoms. Initially, they may have difficulty swallowing solid foods, which can progress to include liquids. Additional symptoms may involve chest pain or discomfort, regurgitating food and fluids, heartburn, unintentional weight loss, coughing or choking during meals, and hoarseness.
Healthcare providers should gather a comprehensive medical history and conduct a physical examination for diagnosis. If esophageal stricture is...
Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption01:23

Pharmacokinetics in Pediatric Patients: Overview and Drug Absorption

Understanding the physiological differences in the pediatric population is crucial for effective pharmacotherapy. Neonates, infants, and children exhibit significant variations in gastric pH, gastric emptying time, intestinal transit time, and biliary function. These variations profoundly affect oral drug absorption, necessitating a nuanced approach to pediatric dosing.Neonates present with a unique physiological profile, having a gastric pH greater than 4 and faster and more irregular gastric...
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...
Pharmacokinetics in Pediatric Patients: Drug Distribution01:17

Pharmacokinetics in Pediatric Patients: Drug Distribution

Drug distribution in the pediatric population exhibits unique challenges and considerations due to the physiological differences between children, particularly neonates and infants, and adults. A crucial aspect of pediatric pharmacology is understanding how these differences impact the pharmacokinetics of various drugs, necessitating age-specific dosing strategies to ensure efficacy and safety.Neonates and infants have a higher total body water content, ~75%–90% of their body weight, compared...