Pediatric Pulmonology 2023 year in review: Rare and diffuse lung disease

Daniel M Hinds1, Rebekah J Nevel2, Deborah R Liptzin3

  • 1Department of Pediatrics, University of Iowa School of Medicine, Iowa City, Iowa, USA.

PubMed

Insights

Advances in pediatric rare lung diseases like children's interstitial lung disease and primary ciliary dyskinesia are improving diagnosis and treatment. Multicenter studies and registries are key to understanding these challenging conditions.

Area of Science:

  • Pediatric Pulmonology
  • Rare Lung Diseases
  • Interstitial Lung Disease

Background:

  • Pediatric rare and diffuse lung diseases present diagnostic and therapeutic challenges.
  • Conditions include children's interstitial lung disease (chILD), noncystic fibrosis bronchiectasis, and primary ciliary dyskinesia (PCD).
  • Understanding pathophysiology and genotype/phenotype relationships is crucial for progress.

Purpose of the Study:

  • To review recent advancements in the diagnosis and treatment of pediatric rare and diffuse lung diseases.
  • To increase awareness and knowledge of chILD, noncystic fibrosis bronchiectasis, and PCD.
  • To inspire future research in the field.

Main Methods:

  • Review of papers published in Pediatric Pulmonology and other journals in 2023.
  • Analysis of research focusing on multicenter cooperation and patient registries.
  • Synthesis of findings on pathophysiology, genotype/phenotype correlations, and treatment outcomes.

Main Results:

  • Multicenter cooperation and patient registries are powerful tools for studying rare diseases.
  • Enhanced understanding of pathophysiology and genotype/phenotype relationships has been achieved.
  • Progress in treatment strategies for these pediatric lung conditions has been reported.

Conclusions:

  • Continued research is vital for advancing the care of children with rare lung diseases.
  • Collaboration and data sharing accelerate progress in understanding and treating these conditions.
  • This review aims to foster greater awareness and stimulate further investigation.

Related Concept Videos

Other Pulmonary Disorders01:17

Other Pulmonary Disorders

Respiratory disorders encompass a range of conditions with varying levels of severity. Asthma, marked by chronic airway inflammation and hypersensitivity, is one such condition. It can lead to airway obstruction due to factors like bronchial spasms, mucosal edema, increased mucus secretion, or epithelial damage. Asthma triggers are diverse, ranging from allergens to emotional upset, and treatment focuses on both immediate relief through bronchodilators and long-term inflammation suppression.
827
Common Respiratory Disorders01:31

Common Respiratory Disorders

Respiratory disorders, a prevalent health concern globally, are generally divided into two primary categories: upper and lower respiratory tract disorders. The categorization is based on the area of the respiratory system they affect.
Upper respiratory disorders impact the airways above the vocal cords, encompassing areas like the nose, sinuses, and throat. Various conditions fall under this category, including the common cold and allergic rhinitis. These disorders can stem from several causes,...
420
Pulmonary Hypertension: Classification and Pathogenesis01:30

Pulmonary Hypertension: Classification and Pathogenesis

Pulmonary hypertension (PH) is a severe health condition in which the mean pulmonary arterial pressure increases to 25 mmHg or more, even when the body is at rest. This high pressure in the blood vessels that transport blood from the heart to the lungs can cause various symptoms, including shortness of breath, can lead to right heart failure, and significantly affect the overall quality of life.
There are various classifications for PH, each relating to different underlying causes and also...
169
Pulmonary Cycle: Exhalation01:17

Pulmonary Cycle: Exhalation

In terms of human respiration, the act of expelling air, known as exhalation (or expiration), operates on the principle of pressure gradients. During expiration, the pressure within the lungs exceeds that of the surrounding atmosphere. Under normal conditions, quiet breathing involves passive exhalation and is free of muscular contractions. This is because the exhalation process is driven by the natural elastic recoil of the lungs and chest wall, both of which have an inherent tendency to...
1.5K
COPD: Pathogenesis and Clinical Features01:20

COPD: Pathogenesis and Clinical Features

Chronic obstructive pulmonary disease (COPD) is a group of lung conditions that progressively worsen over time, including chronic bronchitis and emphysema. This cluster of diseases collectively leads to a gradual and irreversible decline in lung function over time.
The primary cause for the onset of COPD is cigarette smoking and exposure to air pollution. These hazardous factors initiate a chain reaction within the lungs, resulting in chronic inflammation, damage to the airways, and a...
259
Chronic Obstructive Pulmonary Disease01:22

Chronic Obstructive Pulmonary Disease

COPD is defined as a heterogeneous lung condition marked by persistent respiratory symptoms such as dyspnea, cough, and sputum production, caused by abnormalities in the airways that cause airflow obstruction.
Smoking is a primary risk factor for COPD, with over 80% of patients having a history of it. Patients typically experience progressive dyspnea or labored breathing, frequent coughing, and recurrent pulmonary infections. Many eventually succumb to respiratory failure, characterized by...
1.2K