Idiopathic pulmonary fibrosis beyond the lung: understanding disease mechanisms to improve diagnosis and management
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 …
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 …
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
Differentiation of functional thyroid epithelia from pluripotent stem cells (PSCs) holds the
potential for application in regenerative medicine. However, progress toward this goal is …
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 …
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 …
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 …
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 …
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 …
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 …
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 …
combination of clinical factors, including lung immaturity, mechanical ventilation, oxidative …