Rare Lung Diseases and the Effects of Perinatal Infection on Prematurity
The Rare Lung Disease Consortium, based at Cincinnati Children’s and funded by the NIH, links the basic science underlying the pathogenesis of several rare pulmonary diseases: pulmonary alveolar proteinosis (PAP), interstitial lung diseases (ILD) in children, lymphangioleiomyomatosis (LAM) and alpha-1 antitrypsin. Bringing together investigators and patients affected by rare lung diseases from the US, Japan and Europe, the group has made major advances in diagnosis and treatment.
Recently, researchers demonstrated that adult patients with PAP have high levels of autoimmune antibodies that cause the disease by blocking the protein GM-CSF and, therefore, macrophage function in the lung. Because early trials with GM-CSF inhalation have been effective, larger trials are under study.
Perinatal Infection and Prematurity
In an NIH-funded collaborative with Australian colleagues, our researchers have shown that prenatal exposure of the fetus to bacterial endotoxin or ureaplasma, both commonly associated with preterm labor and perinatal infection, creates a fetal inflammatory syndrome. In the setting of perinatal infection and lung injury, the gentler methods of resuscitation and ventilation in the premature newborn are effective and cause less injury to the lungs. Their research studies are leading to improvements in the use of prenatal steroids, timing of delivery, resuscitation, ventilation methods and the use of pulmonary surfactant, all of which contribute to better care for preterm infants.
