Related Experiment Video

Updated: Jan 8, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
04:44

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease

Published on: June 16, 2020

20.7K

A case of pulmonary alveolar microlithiasis with Cor Pulmonale

Wen Chen1, Tao Gu

  • 1Department of Radiology, Beijing Hospital, the Fifth Clinical Medicine College of Peking University, Beijing, China.

Quantitative Imaging in Medicine and Surgery
|December 21, 2012
PubMed

Insights

Pulmonary alveolar microlithiasis (PAM), a rare lung disease, involves microlith deposits. This case highlights its association with Cor Pulmonale and diagnostic imaging findings.

Area of Science:

  • Pulmonology
  • Rare Diseases
  • Genetics

Background:

  • Pulmonary alveolar microlithiasis (PAM) is a rare autosomal recessive disorder.
  • Mutations in the SLC34A2 gene are associated with PAM.
  • PAM is characterized by microlith deposition in lung alveoli, often with subtle symptoms.

Observation:

  • A case of PAM presenting with Cor Pulmonale is described.
  • Echocardiography revealed pulmonary hypertension (82 mmHg).
  • Chest radiography showed diffuse, bilateral sandstorm-like micronodules, predominantly in lower lung fields.

Findings:

  • High-resolution CT (HRCT) scans demonstrated ground-grass opacities, thickened/calcified interlobular septa, and confluent calcified nodules.
  • Diagnosis was suspected based on imaging characteristics.
  • Transbronchial lung biopsy (TBLB) confirmed the presence of microliths, establishing the PAM diagnosis.

Implications:

  • This case underscores the importance of characteristic imaging findings in diagnosing PAM.
  • Understanding PAM's presentation aids in early detection and management of associated conditions like Cor Pulmonale.
  • Further research into SLC34A2 gene mutations can improve diagnostic accuracy and therapeutic strategies for PAM.
Keywords:
Cor PulmonaleHRCTPulmonary alveolar microlithiasis

More Related Videos

The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats07:29

The Left Pneumonectomy Combined with Monocrotaline or Sugen as a Model of Pulmonary Hypertension in Rats

Published on: March 8, 2019

11.2K
Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet09:22

Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet

Published on: November 4, 2015

12.5K

Related Concept Videos

Mitral Stenosis I: Introduction01:22

Mitral Stenosis I: Introduction

Mitral Valve Stenosis (MVS) is a heart condition where the mitral valve narrows, impeding blood circulation from the left atrium to the left ventricle. The etiology and pathophysiology of this condition are multifaceted, leading to a cascade of cardiovascular complications.Causes of Mitral Valve StenosisRheumatic Heart Disease: It is the main cause of mitral valve stenosis, particularly in developing nations. This condition arises from rheumatic fever, an inflammatory illness resulting from...
384
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
4.2K
Chronic Obstructive Pulmonary Disease-III: Symptoms and Complications.01:25

Chronic Obstructive Pulmonary Disease-III: Symptoms and Complications.

Understanding the variety of primary symptoms and systemic complications that characterize chronic obstructive pulmonary disease (COPD) is crucial for healthcare professionals.
Symptoms of COPD can be classified as primary or systemic. Primary symptoms relate to reduced airflow, while systemic or extrapulmonary symptoms relate to COPD's broader impact on the body.
Primary Symptoms of COPD:
3.5K
Pulmonary Tuberculosis II01:28

Pulmonary Tuberculosis II

Tuberculosis, or TB, is a bacterial infectious disease caused by Mycobacterium tuberculosis. While its primary impact is on the lungs, leading to pulmonary tuberculosis, it can also affect various other organs, a condition referred to as extrapulmonary tuberculosis.
Here is a detailed explanation of its pathophysiology:
Transmission: The process begins when a person inhales droplet nuclei containing M. tuberculosis. These are typically released into the air when an individual with pulmonary or...
1.3K
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...
536
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.
1.5K