One-year outcomes in a multicentre cohort study of incident rare diffuse parenchymal lung disease in children (ChILD).

We performed a prospective, observational, cohort study of children newly diagnosed with children's interstitial lung disease (ChILD), with structured follow-up at 4, 8, 12 weeks and 6 and 12 months. 127 children, median age 0.9 (IQR 0.

Inter- and transgenerational epigenetic inheritance: evidence in asthma and COPD?

Evidence is now emerging that early life environment can have lifelong effects on metabolic, cardiovascular, and pulmonary function in offspring, a concept also known as fetal or developmental programming. In mammals, developmental programming is thought to occur mainly via epigenetic mechanisms, which include DNA methylation, histone modifications, and expression of non-coding RNAs. The effects of developmental programming can be induced by the intrauterine environment, leading to intergenerational epigenetic effects from one generation to the next.

Decreased cord blood IL-4, IL-13, and CCR4 and increased TGF-beta levels after fish oil supplementation of pregnant women.

Background: Altered intakes of n-3 and n-6 polyunsaturated fatty acids were suggested to modulate allergic disease, but intervention trials yielded inconclusive results. Because allergies are primed in early infancy and in utero, the fetus might be more accessible to nutritional intervention strategies.

Objective: We sought to investigate how supplementation of pregnant women with a fish oil (FO) preparation modulates allergy-related immune parameters in mothers and offspring.

Surfactant protein D regulates chemotaxis and degranulation of human eosinophils.

The collectin surfactant protein D (SP-D) is an important component of the pulmonary innate host defence. Up to now, little is known about the regulation of eosinophil function by SP-D. Various murine models of pulmonary hypersensitivity suggest that SP-D may be a potent anti-allergic protein.

Anti-inflammatory cytokines in cystic fibrosis lung disease.

Lung inflammation plays a pivotal role in the pathogenesis of airway disease in cystic fibrosis (CF). An imbalance between pro- and anti-inflammatory mediators has been observed and a deficiency in the anti-inflammatory response has been proposed, but this concept remains controversial. In the present study, the concentrations of two anti-inflammatory mediators, lipoxin A (LxA4) and Clara cell protein 10 (CC-10), were assessed in bronchoalveolar lavage fluid (BALF) of CF patients with a wide range of endobronchial inflammation and disease controls with neutrophilic inflammation unrelated to CF.

Oxidative damage to surfactant protein D in pulmonary diseases.

Surfactant protein D is an important innate host defence molecule that has been shown to interact with a variety of pathogens and to play a role in surfactant homeostasis. The aim of this study was to examine the influence of oxidation on surfactant protein D in different lung diseases. Bronchoalveolar lavage fluids (BALFs) from patients with different grade of protein oxidation were examined for changes in the primary chain and the quaternary structure of surfactant protein D.

Oxidative changes of bronchoalveolar proteins in cystic fibrosis.

Chronic bacterial infection and severe, polymorphonuclear neutrophil-dominated endobronchial inflammation are characteristic hallmarks of cystic fibrosis (CF) lung disease. The free radicals generated can be deleterious for structure and function of many proteins. The goal of this study was to investigate the degree of oxidation of pulmonary epithelial lining fluid proteins.

Anti-GM-CSF antibodies in paediatric pulmonary alveolar proteinosis.

Background: Auto-antibodies against granulocyte-macrophage colony stimulating factor (GM-CSF) may be central to the pathogenesis of adult sporadic pulmonary alveolar proteinosis (PAP). The role of anti-GM-CSF auto-antibodies in paediatric forms of PAP is as yet unclear.

Methods: Anti-GM-CSF auto-antibodies were determined with the help of an antigen capture assay using serum and/or bronchoalveolar lavage (BAL) fluid from 27 patients with PAP (nine adults, 15 children, three neonates) and from 185 children with different diseases as disease controls (various pulmonary conditions and patients with malignancies).

Proteolysis of surfactant protein D by cystic fibrosis relevant proteases.

Surfactant protein D (SP-D) is an important innate host defense molecule that has been shown to interact with cystic fibrosis (CF)-associated pathogens. Previous studies demonstrated that rat SP-D is highly resistant to degradation by a wide range of proteolytic enzymes. The aim of this study was to examine whether human SP-D can be degraded by CF relevant proteases ex vivo and in vitro.

Limited proteolysis of surfactant protein D causes a loss of its calcium-dependent lectin functions.

Surfactant protein D (SP-D) is a multimeric collagenous lectin that mediates the clearance of pathogens and modulates immune cell functions via its C-terminal carbohydrate recognition domain (CRD). We hypothesized that extracellular proteolysis of SP-D may result in a loss of its functional properties. Multimeric SP-D was partially digested by human leukocyte elastase (HLE) dose- and time-dependently.