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Clin Med Insights Circ Respir Pulm Med. 2015 Oct 11;9:57-68. doi: 10.4137/CCRPM.S23282. eCollection 2015.

Interstitial Lung Disease in Childhood: Clinical and Genetic Aspects.

Clinical medicine insights. Circulatory, respiratory and pulmonary medicine

Hiroshi Kitazawa, Shigeo Kure

Affiliations

  1. Department of General Pediatrics, Division of Allergy, Miyagi Children's Hospital, Sendai, Japan.
  2. Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.

PMID: 26512209 PMCID: PMC4603523 DOI: 10.4137/CCRPM.S23282

Abstract

Interstitial lung disease (ILD) in childhood is a heterogeneous group of rare pulmonary conditions presenting chronic respiratory disorders. Many clinical features of ILD still remain unclear, making the treatment strategies mainly investigative. Guidelines may provide physicians with an overview on the diagnosis and therapeutic directions. However, the criteria used in different clinical studies for the classification and diagnosis of ILDs are not always the same, making the development of guidelines difficult. Advances in genetic testing have thrown light on some etiologies of ILD, which were formerly classified as ILDs of unknown origins. The need of genetic testing for unexplained ILD is growing, and new classification criteria based on the etiology should be adopted to better understand the disease. The purpose of this review is to give an overview of the clinical and genetic aspects of ILD in children.

Keywords: classification; diffuse lung disease; genetic testing; interstitial lung disease; surfactant protein

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