Abstract

Within the past few years, there is a rapid expansion in our understanding of childhood interstitial lung disease (chILD). chILD refers to a diverse group of rare chronic and complex respiratory disorders in children, mainly in infants under two years of age, which includes immunological and developmental abnormalities. These disorders involve the interstitium as well as the distal airspaces that result in restrictive lung physiology and significant impairment of gas exchange. chILD is clinically complex and associated with high morbidity and mortality. This review aimed to describe chILD classification, epidemiology, diagnostic approaches, morbidity, treatments, and the outcomes of chILD.

Highlights

  • Childhood interstitial lung disease refers to a diverse group of rare chronic and complex respiratory disorders in children, mainly in infants under two years of age, which includes immunological and developmental abnormalities

  • The previous reported prevalence is likely to be considerably underestimated, given the increased recognition of interstitial lung diseases in the pediatric population in recent years, due to (1) a recently developed classification system for classifying Childhood interstitial lung disease (chILD); (2) increased recognition, of the unique ILDs which occur in infants; and (3) increased use of thoracoscopic lung biopsy in pediatric patients for definitive diagnosis

  • There has been substantial confusion and difficulty associated with the description and classification of specific ILD in infants and young children with multiple terms used for similar abnormalities and sometimes the same term used for differing conditions; and

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Summary

Introduction

Childhood interstitial lung disease (chILD) refers to a diverse group of rare chronic and complex respiratory disorders in children, mainly in infants under two years of age, which includes immunological and developmental abnormalities. These disorders involve the interstitium as well as the distal airspaces that result in restrictive lung physiology and significant impairment of gas exchange. ChILD is clinically complex and associated with high morbidity and mortality.[1,2,3] The term “diffuse lung disease” has often been used for interstitial lung disease (ILD), because the interstitial compartment is not always involved. The term “chILD syndrome” has been approved by the chILD Research Network and the American Thoracic Society Committee on chILD in order to aid the diagnosis of the uncommon causes of diffuse lung disease phenotypes in children.[1,2]

Epidemiology of chILD
Pathophysiology of chILD
Classification of chILD
Diagnostic Approach to chILD
History and Physical Examination
Clinical Symptoms Are Predictive of Outcome in chILD
Physiological Testing
Laboratory Studies
Invasive Investigations
Morbidity Associated with chILD
Mortality Associated with chILD
General Measures
Pharmacologic Therapy
Other Specific Treatment Strategies
Findings
Monitoring of Response to Treatment

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