Rationale: Acute lung dysfunction of noninfectious etiology, known as idiopathic pneumonia
syndrome (IPS), is a severe complication following hematopoietic stem cell transplantation …

Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by the accumulation of
alveolar surfactant and dysfunction of alveolar macrophages. PAP results in progressive …

Interstitial lung disease in children (chILD) is rare, and most centres will only see a few
cases/year. There are numerous possible underlying diagnoses, with specific and non …

Background Increasing the activity of defective cystic fibrosis transmembrane conductance
regulator (CFTR) protein is a potential treatment for cystic fibrosis. Methods We conducted a …

Abstract The Human Phenotype Ontology (HPO, https://hpo. jax. org) was launched in 2008
to provide a comprehensive logical standard to describe and computationally analyze …

Current viral vectors for gene therapy,, are associated with serious safety concerns,
including leukemogenesis, and nonviral vectors are limited by low gene transfer efficiency …

BACKGROUND: Asthma is characterized by a TH2 immune response. CD4+ CD25hi
regulatory T cells (Tregs) have been proposed to prevent allergic diseases through …

Abstract Interleukin-8 (IL-8) activates neutrophils via the chemokine receptors CXCR1 and
CXCR2. However, the airways of individuals with cystic fibrosis are frequently colonized by …

Rationale: Primary ciliary dyskinesia (PCD) is characterized by recurrent airway infections
and randomization of left–right body asymmetry. To date, autosomal recessive mutations …

Asthma is among the most frequent chronic diseases in childhood. Although numerous
environmental risk factors have already been identified, the basis for familial occurrence of …