Related Experiment Video

Updated: Dec 14, 2025

Development of a Neonatal Piglet Acute Lung Injury Model Recreating the Early Environment of Preterm Infant Lungs
08:58

Development of a Neonatal Piglet Acute Lung Injury Model Recreating the Early Environment of Preterm Infant Lungs

Published on: October 31, 2025

411

Pulmonary alveolarproteinosis in children

Andrew Bush1,2, Rishi Pabary1,2

  • 1Imperial College, London, UK.

Breathe (Sheffield, England)
|July 21, 2020
PubMed

Insights

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by surfactant buildup. Diagnosis is key, as treatments vary widely between children and adults, with emerging therapies showing promise.

Area of Science:

  • Pulmonology
  • Genetics
  • Pediatric Medicine

Background:

  • Pulmonary alveolar proteinosis (PAP) is a rare lung condition characterized by alveolar filling with surfactant.
  • Distinct etiologies and clinical presentations exist between pediatric and adult PAP cases.
  • Genetic mutations and acquired conditions contribute to PAP's diverse origins.

Purpose of the Study:

  • To delineate the diagnostic approach for suspecting and confirming pediatric pulmonary alveolar proteinosis (PAP).
  • To emphasize PAP as an umbrella term, highlighting the necessity of identifying the underlying cause.
  • To compare and contrast the causes of PAP in children versus adults and review treatment strategies.

Main Methods:

  • Review of existing literature on pulmonary alveolar proteinosis.
  • Analysis of computed tomography (CT) findings characteristic of PAP.
  • Discussion of genetic testing and clinical evaluation for diagnosing PAP subtypes.

Main Results:

  • PAP presents with characteristic CT findings of ground-glass opacities and lobular outlining.
  • Genetic causes, including mutations in surfactant protein genes and GM-CSF receptor, are prominent in children.
  • Autoimmune PAP is more common in adults, while specific genetic defects are prevalent in pediatric cases.

Conclusions:

  • Accurate diagnosis of the underlying cause of PAP is crucial for effective treatment planning and genetic counseling.
  • Whole-lung lavage and granulocyte-macrophage colony-stimulating factor (GM-CSF) are established treatments, with stem cell transplantation as an emerging therapy for specific genetic forms.
  • Understanding the developmental differences in PAP etiology is essential for appropriate clinical management.

More Related Videos

Isolation of Pulmonary Artery Smooth Muscle Cells from Neonatal Mice08:02

Isolation of Pulmonary Artery Smooth Muscle Cells from Neonatal Mice

Published on: October 19, 2013

18.8K
Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets08:08

Increasing Pulmonary Artery Pulsatile Flow Improves Hypoxic Pulmonary Hypertension in Piglets

Published on: May 11, 2015

14.5K

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...
63.8K
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...
472
Chronic Obstructive Pulmonary Disease-II: Pathophysiology01:20

Chronic Obstructive Pulmonary Disease-II: Pathophysiology

Chronic Obstructive Pulmonary Disease (COPD) pathophysiology is intricate and multifaceted, involving a complex interplay of physiological processes. Understanding these mechanisms is crucial for effectively managing and treating COPD. Here is an in-depth look at the critical elements in the pathophysiology of COPD:
Chronic Inflammation
4.0K
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...
2.8K
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.
1.4K