pediatric interstitial lung diseases
Thursday 31 July 2008
Interstitial lung disease is much rarer in children than in adults, and there are many more variants.
The literature, with occasional exceptions, consists of case reports and case series.
The chILD (Children’s Interstitial Lung Disease) network has published what is without doubt the most significant article in the field, in which a comprehensive classification, based on independent pathologic review of nearly 200 open-lung biopsies in children younger than 2 years, categorized 88% of 187 biopsies.
This classification consigns the practice of "lumping" of children into "fibrosing alveolitis of children" into the obscurity from which it previously emerged.
The categories that are proposed are as follows:
diffuse developmental disorders (the alveolar-capillary dysplasia–acinar dysplasia spectrum),
lung growth abnormalities (pulmonary hypoplasia from a variety of causes),
pulmonary interstitial glycogenosis,
surfactant protein (SP) dysfunction (SpB, SpC, and ABCA3 deficiency),
disorders of the normal host (mainly infectious or postinfectious, aspiration, or allergic alveolitis),
disorders resulting from systemic disease processes (very few cases, mainly pulmonary hemorrhagic syndromes),
disorders of the immunocompromised host (infectious and postinfectious predominant, and iatrogenic complications),
disorders masquerading as chILD (mainly pulmonary vascular and lymphatic disorders).
Although the final version of this classification may be modified, there is no doubt that this is a major and important review.
If an open-lung biopsy is to be performed for suspected chILD, a careful protocol should be used to ensure tissue is stored for all relevant tests, including electron microscopy.
Such children should have clinical data carefully and systematically recorded, and CT scans performed using standard protocols for evaluation by more than one radiologist.
Biopsies from patients with chILD should be seen by more than one pathologist who has extensive experience with such disorders.
These conditions are so rare that only by international collaboration, using standard protocols, are we likely to obtain large enough groups of patients for clinical trials, in particular of expensive, potentially toxic, anticytokine therapies.
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