Lung Development Research Project

Dr. Mala R. Chinoy established the Lung Development Research Program at Penn State Children's Hospital in 1995 in collaboration with Robert E. Cilley, M.D., a pediatric Surgeon.

The goal of the members of the lung development research is to address a common birth defect found in human newborns - pulmonary hypoplasia (PH) and congenital diaphragmatic hernia (CDH). Pulmonary hypoplasia occurs in 11 to 14 newborns in 10,000 live births, of which about 1 in 2,500 have associated congenital diaphragmatic hernia (CDH). Newborns with PH and CDH suffer from persistent pulmonary hypertension (PPH) and respiratory insufficiency or distress. Despite the availability of the surgical tools and other modern therapeutic treatments, this defect often results in death of these babies with abnormal lungs. The rate of mortality is about 50%.

The exact mechanisms or cause of these defects remain unknown. What is the trigger for disturbing the normal development of lungs in human babies before birth? Is the trigger hormonal or is it caused by some environmental toxins? Which genes may be involved in the formation of abnormal / insufficient lungs?

It is known that the herbicides and other teratogens, which are present in the environment, may cause certain defects in humans, especially when exposed during very early stages of pregnancy. We have, therefore, established a mouse model of pulmonary hypoplasia and congenital diaphragmatic hernia, which mimics the human condition, by using a herbicide – nitrofen.

Our hypothesis is that the human embryo is exposed to a teratogen or herbicide at a very critical time during pregnancy. This results in abnormal development (malformation) of lung and heart, result in respiratory distress, which affects the survival of the babies born with this defect. Respiratory failure as a manifestation of inadequate lung development is the most important reason for morbidity and mortality in the newborns today. We have been able to support our hypothesis through our studies and have published research articles providing some insight in understanding the molecular and genetic mechanisms of this human condition.

Our work is focused on identifying the genes responsible for this abnormal lung development and also on developing further understanding of the mechanisms involved in the defective embryonic development of heart, lung and blood vessel formation in lungs. More detailed work in this area will open avenues to help treat defects in unborn babies and in the long run prevent the fatal outcome of this defect in newborn babies. 

Dr. Chinoy is a well-established pulmonary scientist. She is invited for her research-related presentations at national and international conferences. Her recent lectures and seminars were in England, The Netherlands, India and Canada.

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