ArticlePediatric research2016
Surfactant proteins in pediatric interstitial lung disease.
Article in Pediatric research, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 34 citations in OpenAlex.
- Biallelic LAMP3 variants in 5 families with interstitial lung disease: Evidence of a disease-gene association.Genetics in medicine : official journal of the American College of Medical Genetics · 2026Article
- Persistent tachypnoea of infancy and neuroendocrine cell hyperplasia of infancy: from systematic review to future directions.ERJ open research · 2025Article
- Infant Pulmonary Function Testing in Pediatric Diffuse Parenchymal Lung Disease.CHEST pulmonary · 2025Review
- The lost chILD: a case report of delayed diagnosis of surfactant protein C deficiency in a 15-year-old African male.Italian journal of pediatrics · 2025Article
- Structure and Function of Canine SP-C Mimic Proteins in Synthetic Surfactant Lipid Dispersions.Biomedicines · 2024Article
- Amyloid, Crohn's disease, and Alzheimer's disease - are they linked?Frontiers in cellular and infection microbiology · 2024Review
- Circulating Surfactant Protein D: A Biomarker for Acute Lung Injury?Biomedicines · 2023Review
- Diagnostic workup of childhood interstitial lung disease.European respiratory review : an official journal of the European Respiratory Society · 2023Review
- Genetics in Idiopathic Pulmonary Fibrosis: A Clinical Perspective.Diagnostics (Basel, Switzerland) · 2022Review
- Interstitial lung disease in infancy and early childhood: a clinicopathological primer.European respiratory review : an official journal of the European Respiratory Society · 2022Review
- Aerosol Delivery of Lung Surfactant and Nasal CPAP in the Treatment of Neonatal Respiratory Distress Syndrome.Frontiers in pediatrics · 2022Review
- The Potential of Lung Epithelium Specific Proteins as Biomarkers for COVID-19-Associated Lung Injury.Diagnostics (Basel, Switzerland) · 2021Review
- Article
- Genetic disorders of the surfactant system: focus on adult disease.European respiratory review : an official journal of the European Respiratory Society · 2021Article
- Surfactant replacement therapy: from biological basis to current clinical practice.Pediatric research · 2020Review
- Lipid-Protein and Protein-Protein Interactions in the Pulmonary Surfactant System and Their Role in Lung Homeostasis.International journal of molecular sciences · 2020Review
- Alveolar Dynamics and Beyond - The Importance of Surfactant Protein C and Cholesterol in Lung Homeostasis and Fibrosis.Frontiers in physiology · 2020Review
- Genetic causes of surfactant protein abnormalities.Current opinion in pediatrics · 2019Review
- Mutations in the thyroid transcription factor gene NKX2-1 result in decreased expression of SFTPB and SFTPC.Pediatric research · 2018Article
- RNA-based therapies for genodermatoses.Experimental dermatology · 2017Review
Corrections and comments
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Authors and funding
19 authors at 9 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundChildren's interstitial lung diseases (chILD) comprise a broad spectrum of diseases. Besides the genetically defined surfactant dysfunction disorders, most entities pathologically involve the alveolar surfactant region, possibly affecting the surfactant proteins SP-B and SP-C. Therefore, our objective was to determine the value of quantitation of SP-B and SP-C levels in bronchoalveolar lavage fluid (BALF) for the diagnosis of chILD.
methodsLevels of SP-B and SP-C in BALF from 302 children with chILD and in controls were quantified using western blotting. In a subset, single-nucleotide polymorphisms (SNPs) in the SFTPC promoter were genotyped by direct sequencing.
resultsWhile a lack of dimeric SP-B was found only in the sole subject with hereditary SP-B deficiency, low or absent SP-C was observed not only in surfactant dysfunction disorders but also in patients with other diffuse parenchymal lung diseases pathogenetically related to the alveolar surfactant region. Genetic analysis of the SFTPC promoter showed association of a single SNP with SP-C level.
conclusionSP-B levels may be used for screening for SP-B deficiency, while low SP-C levels may point out diseases caused by mutations in TTF1, SFTPC, ABCA3, and likely in other genes involved in surfactant metabolism that remain to be identified. We conclude that measurement of levels of SP-B and SP-C was useful for the differential diagnosis of chILD, and for the precise molecular diagnosis, sequencing of the genes is necessary.
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