scholarly journals Congenital Pulmonary Malformation in Children

Scientifica ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-7 ◽  
Author(s):  
Montasser Nadeem ◽  
Basil Elnazir ◽  
Peter Greally
Keyword(s):  
Lung Development ◽  
Abnormal Development ◽  
Intrauterine Life ◽  
Lung Abnormalities ◽  
Congenital Pulmonary Malformation

Congenital Pulmonary Malformations (CPMs) are a group of rare lung abnormalities affecting the airways, parenchyma, and vasculature. They represent a spectrum of abnormal development rather than discrete pathological entities. They are caused by aberrant embryological lung development which occurs at different stages of intrauterine life.

FGFR-3 and FGFR-4 function cooperatively to direct alveogenesis in the murine lung

Development ◽  
1998 ◽  
Vol 125 (18) ◽  
pp. 3615-3623 ◽  
Author(s):  
M. Weinstein ◽  
X. Xu ◽  
K. Ohyama ◽  
C.X. Deng
Keyword(s):  
Lung Development ◽  
Receptor Gene ◽  
Branching Morphogenesis ◽  
Fgf Receptor ◽  
Murine Lung ◽  
Targeted Mutation ◽  
Lung Abnormalities ◽  
Postnatal Lung Development ◽  
Receptor Gene Expression ◽  
Multiple Stages

Mammalian lungs begin as an outpocket of the foregut, and depend on multiple stages of branching morphogenesis and alveogenesis to reach their final form. An examination of fgf receptor gene expression indicated that all four receptors (fgfr-1 to fgfr-4) are expressed in postnatal lungs at varying levels. We show that mice homozygous for a targeted mutation of fgfr-4 exhibited no overt abnormalities in the lungs or any other organ. However, mice doubly homozygous for disruptions of the fgfr-3 and fgfr-4 genes display novel phenotypes not present in either single mutant, which include pronounced dwarfism and lung abnormalities. Lungs of fgfr-3(−/−)fgfr-4(−/−)animals, which are normal at birth, are completely blocked in alveogenesis and do not form secondary septae to delimit alveoli. Consequently, air spaces in the lung are expanded and no alveoli can be seen. The mutant lungs failed to downregulate postnatal elastin deposition despite their normal levels of surfactant expression and cell proliferation. These data revealed a cooperative function of FGFR-3 and FGFR-4 to promote the formation of alveoli during postnatal lung development.

scholarly journals Mechanism of lung development in the aetiology of adult congenital pulmonary airway malformations

Thorax ◽  
2020 ◽  
Vol 75 (11) ◽  
pp. 1001-1003
Author(s):  
Bethany Taylor ◽  
Alexandra Rice ◽  
Andrew G Nicholson ◽  
Matthew Hind ◽  
Charlotte H Dean
Keyword(s):  
Lung Development ◽  
Cyst Formation ◽  
Immunohistochemical Markers ◽  
Thyroid Transcription Factor 1 ◽  
Thyroid Transcription Factor ◽  
Lung Abnormalities ◽  
Risk Of Malignancy ◽  
Adult Congenital ◽  
Pulmonary Airway ◽  
Transcription Factor 1

Congenital pulmonary airway malformations (CPAMs) are rare lung abnormalities that result in cyst formation and are associated with respiratory distress in infants and malignant potential in adults. The pathogenesis of CPAMs remains unknown but data suggest disruption of the normal proximo-distal programme of airway branching and differentiation. Here, we demonstrate that adult human CPAM are lined with epithelium that retains SOX-2 and thyroid transcription factor-1 immunohistochemical markers, characteristic of the developing lung. However, RALDH-1, another key marker, is absent. This suggests a more complex aetiology for CPAM than complete focal arrest of lung development and may provide insight to the associated risk of malignancy.

scholarly journals Developmental Pathways Underlying Lung Development and Congenital Lung Disorders

Cells ◽  
2021 ◽  
Vol 10 (11) ◽  
pp. 2987
Author(s):  
Inês Caldeira ◽  
Hugo Fernandes-Silva ◽  
Daniela Machado-Costa ◽  
Jorge Correia-Pinto ◽  
Rute Silva Moura
Keyword(s):  
Lung Development ◽  
Early Life ◽  
Growth And Development ◽  
Molecular Mechanisms ◽  
Developmental Process ◽  
Developmental Pathways ◽  
Lung Growth ◽  
Congenital Pulmonary Airway Malformation ◽  
Lung Abnormalities ◽  
Bronchogenic Cysts

Lung organogenesis is a highly coordinated process governed by a network of conserved signaling pathways that ultimately control patterning, growth, and differentiation. This rigorously regulated developmental process culminates with the formation of a fully functional organ. Conversely, failure to correctly regulate this intricate series of events results in severe abnormalities that may compromise postnatal survival or affect/disrupt lung function through early life and adulthood. Conditions like congenital pulmonary airway malformation, bronchopulmonary sequestration, bronchogenic cysts, and congenital diaphragmatic hernia display unique forms of lung abnormalities. The etiology of these disorders is not yet completely understood; however, specific developmental pathways have already been reported as deregulated. In this sense, this review focuses on the molecular mechanisms that contribute to normal/abnormal lung growth and development and their impact on postnatal survival.

Dexamethasone Effect on Embryonic Chick Respiratory Epithelium

1982 ◽  
Vol 40 ◽  
pp. 248-249
Author(s):  
M.R. Richter ◽  
R.V. Blystone
Keyword(s):  
Lung Development ◽  
Critical Role ◽  
Respiratory Epithelium ◽  
Chick Embryos ◽  
Embryonic Chick ◽  
White Leghorn ◽  
Lung Maturation ◽  
Synthetic Analogs ◽  
Lung Physiology ◽  
Avian Lung

Dexamethasone and other synthetic analogs of corticosteroids have been employed clinically as enhancers of lung development. The mechanism(s) by which this steroid induction of later lung maturation operates is not clear. This study reports the effect on lung epithelia of dexamethasone administered at different intervals during development. White Leghorn chick embryos were used so as to remove possible maternal and placental influences on the exogenously applied steroid. Avian lung architecture does vary from mammals; however, respiratory surfactant produced by the lung epithelia serves an equally critical role in avian lung physiology.

Normal and Abnormal Development of the Extremities and Trunk

1990 ◽  
Vol 17 (1) ◽  
pp. 13-21 ◽  
Author(s):  
David T. Netscher ◽  
Robert Peterson
Keyword(s):  
Abnormal Development

The role of WNT/b-catenin signaling in smooth muscle cells during lung development and repair

Pneumologie ◽  
2014 ◽  
Vol 68 (06) ◽  
Author(s):  
A Moiseenko ◽  
E El Agha ◽  
B MacKenzie ◽  
S De Langhe ◽  
S Bellusci
Keyword(s):  
Smooth Muscle ◽  
Smooth Muscle Cells ◽  
Lung Development ◽  
Muscle Cells

Disruption of mesenchymal glucocorticoid signaling attenuates embryonic lung development and results in post natal lethality in mice

2013 ◽  
pp. 1-1
Author(s):  
Rowan Hardy ◽  
Aiqing Li ◽  
Shihani Stoner ◽  
Jan Tuckermann ◽  
Markus Seibel ◽  
...  
Keyword(s):  
Lung Development ◽  
Embryonic Lung ◽  
Glucocorticoid Signaling
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