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Related Concept Videos

Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
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Bradyarrhythmias are cardiac rhythm disorders characterized by a slower-than-normal heart rate, typically defined as fewer than 60 beats per minute. Some of which are discussed here:Sinus BradycardiaSinus bradycardia presents a heart rate lower than 60 beats per minute, with a regular rhythm originating from the SA node. The ECG typically shows normal P waves preceding each QRS complex, a normal PR interval (0.12 to 0.20 seconds), and a normal QRS duration (0.06 to 0.10 seconds).First-Degree AV...
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In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses a challenge in...
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Depolarizing blockers are administered through intravenous injection. Succinylcholine is the most common choice of depolarizing blockers in emergency clinical practices. Although they have a rapid onset, they readily diffuse away from the motor end plate into the extracellular fluid. They are metabolized by enzymes such as liver butyrylcholinesterase and plasma pseudocholinesterases. This produces a short duration of action, typically 5-10 minutes long, unlike nondepolarizing blockers, which...
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Overview
Disturbances in Heart Rhythm01:29

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Arrhythmia or dysrhythmia refers to an abnormal heart rhythm caused by a defect in the heart's conduction system. It can cause the heart to beat irregularly, too quickly, or too slowly, leading to symptoms like chest pain, shortness of breath, and fainting. Factors such as stress, caffeine, alcohol, nicotine, cocaine, certain drugs, congenital defects, diseases, and electrolyte abnormalities can trigger arrhythmias.
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Related Experiment Video

Updated: Jun 1, 2026

Benefits of Cardiac Resynchronization Therapy in an Asynchronous Heart Failure Model Induced by Left Bundle Branch Ablation and Rapid Pacing
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Rate-dependent left bundle-branch block in a child with propionic aciduria.

Kipp B Ardoin1, Douglas S Moodie, Christopher S Snyder

  • 1Section of Pediatric Cardiology, Department of Pediatrics, Ochsner Clinic Foundation, New Orleans, LA.

Ochsner Journal
|May 24, 2011
PubMed
Summary

A rare case of rate-dependent left bundle-branch block was observed in a pediatric patient with propionic acidemia and dilated cardiomyopathy. This electrocardiogram finding is typically postoperative but can occur in specific cardiac conditions.

Keywords:
Bundle-branch blockcardiac manifestations in propionic aciduriaexercise-induced (rate dependent) bundle-branch blockheart blocknormal heart conductionpropionic aciduria

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Area of Science:

  • Pediatric Cardiology
  • Cardiovascular Genetics
  • Metabolic Disorders

Background:

  • Left bundle-branch block (LBBB) on electrocardiogram is often a postoperative finding.
  • Rarely, LBBB can be associated with myocardial infarction, hypertrophic cardiomyopathy, or exercise.
  • Propionic acidemia is a rare metabolic disorder that can affect the heart.

Purpose of the Study:

  • To describe a unique case of rate-dependent LBBB in a pediatric patient with propionic acidemia and dilated cardiomyopathy.
  • To highlight the association between metabolic disorders and cardiac conduction abnormalities.

Main Methods:

  • Case report of a pediatric patient.
  • Electrocardiogram (ECG) analysis.
  • Review of relevant medical literature.

Main Results:

  • The patient presented with dilated cardiomyopathy and rate-dependent LBBB.
  • The LBBB pattern was linked to the underlying metabolic disorder and cardiac condition.

Conclusions:

  • Rate-dependent LBBB can occur in pediatric patients with propionic acidemia and dilated cardiomyopathy.
  • This case expands the known associations of LBBB beyond typical etiologies.