Idiopathic pulmonary fibrosis beyond the lung: understanding disease mechanisms to improve diagnosis and management

F Luppi, M Kalluri, P Faverio, M Kreuter, G Ferrara - Respiratory Research, 2021 - Springer
Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive disorder with an estimated
median survival time of 3–5 years after diagnosis. This condition occurs primarily in elderly …

Pulmonary alveolar proteinosis in adults: pathophysiology and clinical approach

A Kumar, B Abdelmalak, Y Inoue… - The Lancet Respiratory …, 2018 - thelancet.com
Pulmonary alveolar proteinosis (PAP) is a diffuse lung disease that results from the
accumulation of lipoproteinaceous material in the alveoli and alveolar macrophages due to …

[HTML][HTML] Regeneration of thyroid function by transplantation of differentiated pluripotent stem cells

AA Kurmann, M Serra, F Hawkins, SA Rankin, M Mori… - Cell stem cell, 2015 - cell.com
Differentiation of functional thyroid epithelia from pluripotent stem cells (PSCs) holds the
potential for application in regenerative medicine. However, progress toward this goal is …

Diseases of pulmonary surfactant homeostasis

JA Whitsett, SE Wert, TE Weaver - Annual Review of Pathology …, 2015 - annualreviews.org
Advances in physiology and biochemistry have provided fundamental insights into the role
of pulmonary surfactant in the pathogenesis and treatment of preterm infants with respiratory …

Pulmonary alveolar proteinosis

BC Trapnell, K Nakata, F Bonella, I Campo… - Nature Reviews …, 2019 - nature.com
Pulmonary alveolar proteinosis (PAP) is a syndrome characterized by the accumulation of
alveolar surfactant and dysfunction of alveolar macrophages. PAP results in progressive …

Genetic disorders of the surfactant system: focus on adult disease

CHM van Moorsel, JJ van der Vis… - European Respiratory …, 2021 - Eur Respiratory Soc
Genes involved in the production of pulmonary surfactant are crucial for the development
and maintenance of healthy lungs. Germline mutations in surfactant-related genes cause a …

The role of genetic testing in pulmonary fibrosis: a perspective from the pulmonary fibrosis foundation genetic testing work group

CA Newton, JM Oldham, C Applegate, N Carmichael… - Chest, 2022 - Elsevier
Patients with familial pulmonary fibrosis represent a subset of patients with pulmonary
fibrosis in whom inherited gene variation predisposes them to disease development. In the …

Outcomes of lung transplantation for infants and children with genetic disorders of surfactant metabolism

WB Eldridge, Q Zhang, A Faro, SC Sweet… - The Journal of …, 2017 - Elsevier
Objective To compare outcomes of infants and children who underwent lung transplantation
for genetic disorders of surfactant metabolism (SFTPB, SFTPC, ABCA3, and NKX2-1) over 2 …

The world of rare interstitial lung diseases

K Buschulte, V Cottin, M Wijsenbeek… - European …, 2023 - Eur Respiratory Soc
The world of rare interstitial lung diseases (ILDs) is diverse and complex. Diagnosis and
therapy usually pose challenges. This review describes a selection of rare and ultrarare …

Genetics of bronchopulmonary dysplasia: An update

PM Lavoie, JH Rayment - Seminars in Perinatology, 2023 - Elsevier
Bronchopulmonary dysplasia (BPD) is a multi-factorial disease that results from a
combination of clinical factors, including lung immaturity, mechanical ventilation, oxidative …