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Interstitial lung disease in children--a report of four cases
M Z Norzila1, B H Azizi, C T Deng
1Institut Pediatrik, Hospital Kuala Lumpur.
Insights
Interstitial lung disease (ILD) in children is rare and often idiopathic. Management strategies varied, with some children improving while others unfortunately succumbed to the condition.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Interstitial Lung Disease
Background:
- Interstitial lung disease (ILD) is exceptionally uncommon in pediatric populations, with unknown etiologies in most cases.
- Limited understanding exists regarding the clinical progression and prognostic factors of childhood ILD.
Observation:
- This report details the management of four infants with ILD presenting with cough, respiratory distress, cyanosis, and failure to thrive.
- Three patients exhibited finger clubbing and right ventricular hypertrophy.
- Clinical interventions included oral steroids, with chloroquine and cyclophosphamide used for refractory cases.
Findings:
- Treatment responses were variable: one child became oxygen-independent, another required home oxygen, and two patients did not survive.
- Prognostic indicators such as sex, age of onset, radiographic findings, and histopathology warrant further investigation.
Implications:
- This case series highlights the challenges in managing pediatric ILD and underscores the need for further research into effective therapeutic approaches.
- Understanding prognostic factors is crucial for improving outcomes in children diagnosed with ILD.
Abstract:
Interstitial lung disease (ILD) is very rare in children. In the majority of cases the aetiology is unknown. Very little is known about the clinical course of this condition in children. Prognosis may be influenced by sex, age of onset of symptoms, radiographic features, presence of right ventricular hypertrophy and histopathology. We report our experience in managing four children with interstitial lung disease. All these children presented in early infancy with cough, respiratory distress, cyanosis and failure to thrive. Three of these children had finger clubbing and right ventricular hypertrophy. All patients received oral steroids. Chloroquine was added in two patients who showed no response. A trial of oral cyclophosphamide was started in one patient who failed with both drugs. One child is oxygen independent while another is on home oxygen therapy. The other two patients eventually died.