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Development of the Respiratory System (Including the Preterm Infant)

  • Stephen Joza
  • Martin PostEmail author
Chapter
  • 4.1k Downloads

Abstract

The developing mammalian lung is challenged by the requisite need for a gas-exchange surface area extensive enough to meet the needs of an organism’s oxygen consumption and CO2 removal. This is achieved first by the transformation of the primitive endoderm into the 105 conducting and 107 respiratory airways by iterative branching morphogenesis, followed by the extensive subdivision and successive maturation of the terminal airways into alveoli: the hundreds of millions of thin spherical cavities which facilitate gas exchange between the airways and the vascular system. Since the process of alveolar formation (alveolarization) occurs largely after birth, premature infants are at increased susceptibility to respiratory distress, often necessitating prolonged assisted ventilation. Despite major advances in the management of perinatal infant care, including improved mechanical ventilation modalities, prenatal steroid administration, and surfactant therapy, many such infants do not undergo normal alveolar development, resulting in the chronic lung disease, bronchopulmonary dysplasia (BPD). An appreciation of the complex cell and molecular interactions which govern normal lung morphogenesis is essential for understanding the aetiology of—and advancing treatments for—pulmonary diseases such as BPD.

Keywords

Idiopathic Pulmonary Fibrosis Congenital Diaphragmatic Hernia Lung Development Pulmonary Vasculature Congenital Diaphragmatic Hernia 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Abbreviations

ARDS

Acute respiratory distress syndrome

BASCs

Bronchoalveolar stem cells

BPD

Bronchopulmonary dysplasia

CDH

Congenital diaphragmatic hernia

DLCO

Pulmonary diffusing capacity for carbon monoxide

E

Embryonic day

ECM

Extracellular matrix

FGF

Fibroblast growth factor

Flk-1

Fetal liver kinase-1

Flt-1

Fms-like tyrosine kinase

Fox

Forkhead box

GC

Glucocorticoid

Hif

Hypoxia-inducible factor

Hox

Homeobox

IPF

Idiopathic pulmonary fibrosis

MMP

Matrix metalloproteinase

NF-κB

Nuclear factor kB

PDA

Patent ductus arteriosus

PDGF

Platelet-derived growth factor

pnd

Postnatal day

RA

Retinoic acid

RDS

Respiratory distress syndrome

SHH

Sonic hedgehog

SP-C

Surfactant protein C

TGF-β

Transforming growth factor beta

TIMP

Tissue inhibitor of metalloproteinases

TTF-1

Thyroid transcription factor 1

VA

Alveolar volume

VEGF-A

Vascular endothelial growth factor A

VEGFR

Vascular endothelial growth factor receptor

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Lung Biology Group, Program of Physiology and Experimental MedicineHospital for Sick Children Research InstituteTorontoCanada
  2. 2.Departments of Laboratory Medicine and PathologyUniversity of TorontoTorontoCanada
  3. 3.Departments of Laboratory Medicine and PediatricsUniversity of TorontoTorontoCanada

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