Abstract
The term children interstitial lung diseases (chILD) refers to a heterogeneous group of rare diseases that diffusely affect the lung. ChILD specific to children younger than 2 years of age include diffuse developmental disorders, growth abnormalities, specific conditions of undefined etiology (neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis) and surfactant protein disorders. Clinical manifestations are highly variable, ranging from the absence of relevant symptoms to a severe onset. Most commonly, chILD presents with nonspecific respiratory signs and symptoms, such as dyspnea, polypnea, dry cough, wheezing, recurrent respiratory infections and exercise intolerance. In the diagnostic approach to a child with suspected ILD, chest high resolution computed tomography and genetic tests play a central role. Then, if the diagnosis remains uncertain, laryngotracheal-bronchoscopy and lung biopsy are needed. Pharmacological treatment is mostly empiric and based on anti-inflammatory and immunomodulatory drugs including corticosteroids, hydroxychloroquine and azithromycin. Despite chILD overall rarity, pediatric pulmonologists must be familiar with these diseases in order to carry out a timely diagnosis and patient treatment.
Highlights
ChILD is a heterogeneous group of rare, chronic respiratory diseases, with a prevalence variably reported as a result of a diagnosis which is often challenging [1]
Clement et al [4] proposed the first children interstitial lung diseases (chILD) classification, based on etiology and physiopathology. This classification was extended by Deutsch et al in 2007 [5] on the base of lung biopsies collected in children less than 2 years of age
During the first two years of life [1,12], chILD clinical manifestations range from no symptoms to severe respiratory distress usually triggered by viral infections
Summary
ChILD (children interstitial lung disease, chILD) is a heterogeneous group of rare, chronic respiratory diseases, with a prevalence variably reported (from 0.13 to 16.2 per 100,000 children per year) as a result of a diagnosis which is often challenging [1]. Even if the term of Children interstitial lung disease, chILD, is conventionally used in the international scientific literature, the term Diffuse Lung Disease, DLD, would be more appropriate to describe these conditions, since many of them do not show exclusive involvement of the interstitium and alveoli, distal small airways and/or terminal bronchioles [2]. We use the term chILD meaning diffuse lung disease in children
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