Interstitial lung disease in children #MMPMID24752172
Kuo CS; Young LR
Curr Opin Pediatr 2014[Jun]; 26 (3): 320-7 PMID24752172show ga
Purpose of review: There has been tremendous progress in the approach to childhood interstitial lung diseases (ILD), with particular recognition that ILD in infants is often distinct from forms that occur in older children and adults. Diagnosis is challenging due to the rarity of ILD and the fact that presenting symptoms of ILD often overlap those of common respiratory disorders. This review summarizes newly published recommendations for diagnosis and management and highlights recent scientific advances in several specific forms of childhood ILD. Recent findings: Clinical practice guidelines emphasize the role for chest CT, genetic testing, and lung biopsy in the diagnostic evaluation of children with suspected ILD. Recent studies have better defined the characteristics and molecular understanding of several different forms of ILD, including Neuroendocrine cell Hyperplasia of Infancy (NEHI) and ILD due to mutations in genes affecting surfactant production and metabolism. Despite significant progress, definitive therapies are often lacking. Summary: Childhood ILD encompasses a collection of rare, diffuse lung diseases. Timely recognition of children with suspected ILD and initiation of appropriate diagnostic evaluations will facilitate medical management. Systematic approaches to clinical care and further study are needed to improve the outcomes of children with these rare disorders.
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Curr+Opin+Pediatr 2014 ; 26 (3): 320-7
