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Related Concept Videos

Breathing01:05

Breathing

The process of breathing, inhaling and exhaling, involves the coordinated movement of the chest wall, the lungs, and the muscles that move them. Two muscle groups with important roles in breathing are the diaphragm, located directly below the lungs, and the intercostal muscles, which lie between the ribs. When the diaphragm contracts, it moves downward, increasing the volume of the thoracic cavity and creating more room for the lungs to expand. When the intercostal muscles contract, the ribs...
Mechanism of Breathing I: Inspiration01:30

Mechanism of Breathing I: Inspiration

Introduction to Inspiration: The Respiratory System in Action
The respiratory system, an essential network for breathing, comprises the conducting and respiratory zones, each playing a crucial role in the overall process of respiration. Let us explore the detailed mechanism of inspiration, or inhalation, which is the first phase of the respiratory cycle.
Pathway of Air during Inspiration
During inspiration, air enters our body through the nose or mouth and moves through the conducting zone,...
Assessment of Ventilation II: Respiratory Depth and Rhythm01:29

Assessment of Ventilation II: Respiratory Depth and Rhythm

Respiratory Depth
Respiratory depth measures the volume of air inhaled or exhaled during a breath. It can vary from shallow to deep and typically remains consistent when a person is at rest or asleep. Occasionally, individuals will automatically inhale deeply, known as sighing, which inflates the lungs with more air than normal breathing.
To assess respiratory depth, observe the degree of chest excursion or movement:
Mechanism of Breathing II: Expiration01:23

Mechanism of Breathing II: Expiration

The Physiology of Expiration: A Seamless Respiratory Process
Expiration, or exhaling, is a complex physiological process that begins as the inspiratory muscles begin to relax. This relaxation triggers a series of events that epitomize the efficiency of the respiratory system.
Mechanism of Expiration:
Respiration01:24

Respiration

Overview of the Respiratory System and Energy Production
Energy production in the human body is primarily fueled by oxidation, a process where food molecules are burned by combining with oxygen to produce carbon dioxide and water. This vital metabolic process sustains life, and is supported intricately by the respiratory system.
Structure and Function of the Respiratory System:
The respiratory system is a complex network of structures that includes the nose, oropharynx, larynx, trachea,...
Pneumothorax-II01:27

Pneumothorax-II

Pneumothorax is a medical condition defined by the buildup of air in the pleural space between the lungs and the chest wall. This accumulation of air can lead to partial or complete lung collapse, resulting in a range of clinical manifestations. Understanding the clinical presentation and effective management strategies is crucial for healthcare professionals in providing timely and appropriate care to individuals with pneumothorax.
Clinical Manifestations:

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A Permanent Window for Investigating Cancer Metastasis to the Lung
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A Permanent Window for Investigating Cancer Metastasis to the Lung

Published on: July 1, 2021

A window to breathe again.

Andrés París-Muñoz1, Rosa M Alcobendas-Rueda2, Cristina Verdú-Sánchez3

  • 1CIBERER-ISCIII, IdiPAZ-CNIO Translational Research Unit in Pediatric Hemato-Oncology, La Paz University Hospital Research Institute, Spanish National Cancer Center, 28029 Madrid, Spain.

Med (New York, N.Y.)
|June 12, 2026
PubMed
Summary

A child with severe MDA5-positive dermatomyositis and lung failure survived using ECMO and CAR-T cell therapy. This case offers a potential model for personalized cellular therapy in critical pediatric autoimmune conditions.

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Published on: September 16, 2019

Area of Science:

  • Immunology
  • Pediatric Rheumatology
  • Cellular Therapy

Background:

  • Rapidly progressive MDA5-positive dermatomyositis can lead to life-threatening lung failure in children.
  • Extracorporeal membrane oxygenation (ECMO) provides critical life support for severe respiratory failure.
  • Chimeric antigen receptor (CAR)-T therapy is an emerging treatment for refractory autoimmune diseases.

Purpose of the Study:

  • To describe the successful management of a pediatric patient with fulminant MDA5-positive dermatomyositis and respiratory failure.
  • To report the outcomes of compassionate-use CD19 CAR-T therapy in this critical case.
  • To propose a framework for personalized cellular therapy in severe autoimmune conditions.

Main Methods:

  • Case report detailing a 10-year-old girl with MDA5-positive dermatomyositis.
  • Application of ECMO for management of fulminant lung failure.
  • Administration of CD19 CAR-T therapy on a compassionate-use basis.
  • Monitoring of immune reconstitution and clinical recovery post-therapy.

Main Results:

  • The patient survived fulminant lung failure through combined ECMO and CAR-T therapy.
  • Observed significant clinical recovery and immune reconstitution following treatment.
  • Successful discharge from hospital care was achieved.

Conclusions:

  • Compassionate-use CD19 CAR-T therapy, alongside supportive care like ECMO, can be effective in pediatric MDA5-positive dermatomyositis with severe lung failure.
  • This case highlights the potential of personalized cellular therapies for critical autoimmune diseases.
  • An interdisciplinary approach is crucial for managing complex pediatric autoimmune cases.