Interstitial lung diseases (ILD) or diffuse parenchymal lung diseases (DPLD) comprise a large number of disorders. Disease definition and classification allow advanced and personalized judgements on clinical disease, risks for genetic or environmental transmissions, and precision medicine treatments. Registers collect specific rare entities and use ontologies for a precise description of complex phenotypes. Here we present a brief history of ILD classification systems from adult and pediatric pneumology. We center on an etiologic classification, with four main categories: lung-only (native parenchymal) disorders, systemic disease-related disorders, exposure-related disorders, and vascular disorders. Splitting diseases into molecularly defined entities is key for precision medicine and the identification of novel entities. Lumping diseases targeted by similar diagnostic or therapeutic principles is key for clinical practice and register work, as our experience with the European children\'s ILD register (chILD-EU) demonstrates. The etiologic classification favored combines pediatric and adult lung diseases in a single system and considers genomics and other -omics as central steps towards the solution of \"idiopathic\" lung diseases. Future tasks focus on a systems\' medicine approach integrating all data and bringing precision medicine closer to the patients.

OBJECTIVE: To describe the clinical-radiological-pathological characteristics and treatment outcomes of children with suspected exogenous lipoid pneumonia (ELP).
METHODS: Systematic review. We searched electronic databases and reference lists published between 1967 and 2018, restricted to non-accidental cases.
RESULTS: Forty-four studies including 489 participants aged 1 day to 17 years from 13 countries were included. Cultural, medical, and behavioural rationale for oil-use was described. The clinical-radiological presentation varied widely. Diagnostic certainty was deemed highest if ELP was confirmed on bronchoalveolar lavage/frozen section lung biopsy with documented extracellular lipid on cytological staining and/or fat analysis. Non-tuberculous mycobacteria infection was identified in six studies: Mycobacterium fortuitum/chelonei, Mycobacterium smegmatis and Mycobacterium abscessus. Treatment comprised supportive therapy, corticosteroids, stopping oil, therapeutic lung-lavage and surgical resection. Outcomes were reported inconsistently.
CONCLUSIONS: Paediatric ELP resulting from cultural and medical practices continues to be described globally. Preventive interventions, standardized reporting, and treatment efficacy studies for cases not averted, are lacking. Protocol registration: PROSPERO CRD42017068313.

Patient education in children with rare chronic diseases like children\'s interstitial lung disease (chILD) remains a challenge.
To develop and evaluate a component-based educational program for individual counselling and to improve patients\' and caregivers\' self-efficacy and treatment satisfaction. Furthermore, to create chILD-specific educational material and assess physicians\' satisfaction with the intervention as well as patients\' health-related quality of life (HrQoL).
The study was conducted in two German centers for pediatric pulmonology, as a single-group intervention with pre-post-follow-up design.
Participants (N = 107, age: M = 7.67, SD = 5.90) showed significant improvement of self-efficacy (self-report: t = 2.89, p < 0.01; proxy-report: t = 3.03, p < 0.01), and satisfaction (patients: t = 3.56, p = 0.001; parents t = 6.38, p < 0.001) with the medical consultations. There were no pre-post differences in HrQoL. Participants were highly satisfied with the material and the physicians with the program.
The chILD education-program is a promising strategy to improve patients\' and their parents\' self-efficacy and treatment-satisfaction. Specific effects of the intervention need to be determined in a randomized controlled trial.
Healthcare providers managing pediatric patients with chILD, may choose to use a patient education-program specifically tailored to the needs of chILD patients and their families, such as the program described here, which is the first of its kind.

Exogenous lipoid pneumonia (ELP), an important cause of interstitial lung disease, often goes unrecognized. We conducted a retrospective study of children with histologically confirmed ELP at Red Cross Children\'s Hospital, South Africa. Twelve children of Zimbabwean heritage aged 2.1-10.8 months were identified between 2012 and 2017. Repeated oral administration of plant-based oil for cultural reasons was reported by 10 of 11 caregivers. Cough (12/12), tachypnoea (11/12), hypoxia (9/12), and diffuse alveolar infiltrates on chest radiography (12/12) were common at presentation. Chest computed tomography revealed ground-glass opacification with lower zone predominance (9/9) and interlobular septal thickening (8/9). Bronchoalveolar lavage specimens appeared cloudy/milky, with abundant lipid-laden macrophages and extracellular lipid on Oil-Red-O staining (12/12), with polymicrobial (6/12) and Mycobacterium abscessus (2/12) co-infection. Antibiotics, systemic corticosteroids, and therapeutic lavage were interventions in all eight and five patients, respectively. Clinicians should consider ELP in children with non-resolving pneumonia in settings with similar practices.