▪ Abstract Surfactant protein C (SP-C) is a hydrophobic 35-amino acid peptide that co-
isolates with the phospholipid fraction of lung surfactant. SP-C represents a structurally and …

Genetic studies in familial lung fibrosis have demonstrated an association with surfactant
protein C genes: two mutations have been found resulting in protein misfolding and causing …

Rationale: ABCA3 is a member of the ATP-binding cassette family of proteins that mediate
the translocation of a wide variety of substrates, including lipids, across cellular membranes …

BRICHOS is a domain found in several proteins consisting of∼ 100 amino acids with
sequence and structural similarities. Mutations in BRICHOS domain have been associated …

We sought to determine the clinical course and histologic findings in lung biopsies from a
group of children who presented with signs and symptoms of interstitial lung disease (ILD) …

Background Desquamative interstitial pneumonia (DIP) and respiratory bronchiolitis-
associated interstitial lung disease (RB-ILD) are uncommon forms of interstitial lung disease …

Recent reports have linked mutations in the surfactant protein C gene (SFTPC) to familial
forms of pulmonary fibrosis, but it is uncertain whether deficiency of mature SP-C contributes …

OBJECTIVE: To determine the contribution of the surfactant protein C (SP-C) I73T mutation
to lung disease. STUDY DESIGN: Genomic DNA was obtained from 116 children with …

Interstitial lung disease in children represents a group of rare chronic respiratory disorders.
There is growing evidence that mutations in the surfactant protein C gene play a role in the …

This article uses OPTN/SRTR data to review trends in pediatric transplantation over the last
decade. In 2003, children younger than 18 made up 3% of the 82,885 candidates for organ …