Abstract:
Although rare, diffuse lung disease is increasingly recognised as a cause of respiratory distress in newborns. However, many cases remain
undiagnosed as not all clinicians are aware that these diseases may present during the newborn period. An increased index of suspicion is
required in newborns not responding to conventional management, those with rapid deterioration of respiratory function and those with
a family history of lung disorders. A structured diagnostic approach including radiography, genetic testing and lung histology should be
followed, whereby a multidisciplinary team (neonatologist, pulmonologist, geneticist, radiologist and histopathologist) is involved. The
mainstay of management is mostly supportive, and may include ventilation, infection prevention and control and nutritional support.
Pharmacological therapy is based on anecdotal evidence. Although lung transplantation may be considered in some facilities, long-term
post-transplant survival is poor.
