Related Experiment Video

Updated: Jul 14, 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

Idiopathic pulmonary hemosiderosis: clinical profile and follow up of 26 children

S K Kabra1, Sumit Bhargava, Rakesh Lodha

  • 1Pediatric Pulmonology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi 110 029, India. skkabra@hotmail.com

Indian Pediatrics
|May 31, 2007
PubMed

Insights

Idiopathic pulmonary hemosiderosis (IPH) in children presents with cough and breathlessness. Treatment with prednisolone and hydroxychloroquine, followed by inhaled corticosteroids, may improve outcomes for pediatric IPH patients.

Area of Science:

  • Pediatric Pulmonology
  • Rare Diseases
  • Hematology

Background:

  • Idiopathic pulmonary hemosiderosis (IPH) is a rare condition causing lung bleeding in children.
  • Diagnosis relies on anemia, chest imaging, and identifying hemosiderin-laden macrophages.

Purpose of the Study:

  • To detail the clinical presentation and treatment outcomes of pediatric IPH patients.
  • To evaluate the effectiveness of a specific treatment regimen for IPH.

Main Methods:

  • Retrospective case series of 26 children diagnosed with IPH.
  • Reviewed clinical features, diagnostic methods (BAL, gastric aspirate), and treatment protocols.
  • Treatment included oral prednisolone, hydroxychloroquine (HCQ), and inhaled corticosteroids (ICS).

Main Results:

  • Common symptoms included cough (100%), breathlessness (85%), and fever (73%).
  • Hemosiderin-laden macrophages were found in 92% of BAL samples.
  • 17 patients showed no recurrence with initial prednisolone and HCQ; older age and hemoptysis correlated with poor response.

Conclusions:

  • Combined therapy with prednisolone and hydroxychloroquine, followed by ICS, shows promise for improving pediatric IPH survival.
  • Identifying factors associated with poor response can guide management strategies.
Abstract

Related Concept Videos

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...
Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies01:27

Chronic Obstructive Pulmonary Disease-IV: Assessement and Diagnostic Studies

Assessing and diagnosing Chronic Obstructive Pulmonary Disease (COPD) involves a detailed approach that includes a comprehensive review of medical history, physical examination, and a variety of diagnostic tests. This thorough evaluation is essential to ensure an accurate diagnosis and guide effective management strategies.
Medical History
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...
Chronic Obstructive Pulmonary Disease IV: Clinical Manifestations01:19

Chronic Obstructive Pulmonary Disease IV: Clinical Manifestations

Chronic Obstructive Pulmonary Disease, or COPD, is a long-term condition marked by persistent and only partially reversible airflow limitation. It involves two overlapping conditions—chronic bronchitis and emphysema—which often co-appear but differ in dominant symptoms and underlying mechanisms.Chronic Bronchitis FeaturesChronic bronchitis presents with a persistent productive cough and thick, sometimes purulent mucus due to airway inflammation, enlarged mucus glands, and goblet cell...
Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care01:29

Pulmonary Embolism II: Diagnostic Studies and Interprofessional Care

Diagnosing Pulmonary EmbolismDiagnosing pulmonary embolism (PE) involves clinical assessment and advanced imaging tests. The preferred diagnostic tool is the spiral (helical) CT scan or CT angiography (CTA), which uses intravenous contrast media to visualize the pulmonary vasculature and identify emboli.A ventilation-perfusion (V/Q) scan is an alternative for patients unable to receive contrast media. This scan includes both perfusion and ventilation scanning. Perfusion scanning involves...
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features01:24

Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features

Chronic bronchitis is a key phenotype of chronic obstructive pulmonary disease (COPD), characterized by airway-centered inflammation and mucus overproduction. It develops from long-term exposure to harmful particles or gases, most commonly cigarette smoke, which triggers a persistent inflammatory response.Cellular and Structural ChangesInflammation initially affects the large bronchi and later the smaller airways, with infiltration by immune cells, including neutrophils, macrophages, and...