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PDF Icon Bronchoscopy and BAL
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PDF Icon 6 Minute Walk Test
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PDF Icon Material asservation of a deceased patient

Proposed Best Practice Checklist: Management of Childrens’ Interstitial Lung Disease (chILD) in Europe

In 2012, the European Union FP-7 program supported a proposal to study rare lung diseases in children in Europe, entitled ‘Orphans Unite: chILD better together – European Management Platform for Childhood Interstitial Lung Diseases’. This is an area where there is no high quality evidence, and no randomised controlled trial of any treatment has been performed. This checklist set out the proposals of this group to try to harmonise practice across Europe, in order to facilitate the collection of uniform datasets in large groups of chILD patients. The checklist draws heavily on existing guidelines, in particular from the USA chILD Foundation.

Definition: What is chILD?

chILD is an umbrella term for a group of diffuse diseases, affecting to various extens the very distal airways and pulmonary parenchyma. These entities are also called diffuse parenchymal lung diseases (DPLD). A number of different classifications have been proposed. The best accepted classification covers only the 0-2 age group [1]. The same group’s classification of chILD age 2-16 is only published in abstract. The Deutsch classification included bronchopulmonary dysplasia as a cause of chILD. The chILD-EU group have specifically decided not to include uncomplicated BPD as chILD, because the diagnostic considerations and investigative pathways are so different. However, chILD can co-exist with other conditions, including prematurity, and so should be excluded in children with other respiratory conditions whose manifestations appear disproprtionately severe given the underlying cause.

chILD in elder children between 2 and 16 years has been reviewed in the European Multicenter study [2] and discussed again by Clement et al. [3], Deterding et al. [4], Dishop et al. [5] and Das et al. [6]. However, harmonisation is necessary to find out best practise management in diagnosis and therapy.

When to suspect chILD

chILD is rare (estimated prevalence <0.5/100,000 children) and comprises at least 200 different conditions. The presentation is non-specific, and the diagnosis will be missed if clinical suspicion is not maintained. The USA chILD foundation have suggested the term “chILD Syndrome”, and suggest further investigation is indicated if at least three of the following four criteria are present: (1) respiratory symptoms (cough, rapid and/or difficult breathing, or exercise intolerance), (2) respiratory signs (tachypnea, adventitious sounds, retractions, digital clubbing, failure to thrive, or respiratory failure), (3) hypoxemia, and (4) diffuse abnormalities on a chest radiograph or computed tomography (CT) scan, provided more common causes of diffuse lung disease have been excluded. Note that no specific duration of symptoms is specified; the pace of investigations will depend on clinical urgency. A term newborn, ventilator-dependent on high concentrations from birth will be investigated much more rapidly than an infant who is tachypnoeic, normoxaemic in room air and is thriving.