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INSIGHTS INTO THE PATHOGENESIS AND AETIOLOGY OF CONGENITAL DIAPHRAGMATIC HERNIA FROM RODENT MODELS

Published online by Cambridge University Press:  14 December 2005

JOHN J GREER
Affiliation:
Department of Physiology, Perinatal Research Centre, University of Alberta, Edmonton, AB, T6G 2S2, Canada
DOUGLAS W ALLAN
Affiliation:
Department of Neurology, The Children's Hospital, Harvard Medical School, Boston MA, United States of America
RANDAL P. BABIUK
Affiliation:
Department of Biomedical Sciences, College of Veterinary Medicine, Cornell University, Ithaca NY, United States of America
ROBIN CLUGSTON
Affiliation:
Department of Physiology, Perinatal Research Centre, University of Alberta, Edmonton, AB, T6G 2S2, Canada

Extract

Congenital diaphragmatic hernia (CDH) is a major life-threatening cause of respiratory failure in the newborn. Regions of the diaphragm failing to form during embryogenesis characterize the developmental anomaly. Normally, the diaphragm develops to form a continuous sheet that completely separates the thoracic and abdominal cavities prior to the major period of internal organ growth. However, in the instance of CDH, a significant proportion of the diaphragmatic musculature (typically the left dorsolateral region) is absent. Consequently, the developing viscera can invade the thoracic cavity, occupying space normally reserved for the growing lungs. As a result, newborns with CDH (∼1:2500 births) suffer from a combination of pulmonary hypoplasia, pulmonary hypertension and surfactant deficiency. Treatment strategies include nitric oxide administration, high frequency oscillatory ventilation, extracorporeal membrane oxygenation, exogenous surfactant administration and surgical repair of the diaphragm. Despite these advances in neonatal intensive care, mortality and morbidity remains high.

Type
Research Article
Copyright
© 2005 Cambridge University Press

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