Familial primary pulmonary hypoplasia

Primary pulmonary hypoplasia is a rare, isolated, genetic developmental defect during embryogenesis characterized by congential malformation of pulmonary parenchyma with absence of other anomalies. Neonatally patients present with decreased breath sounds, small lung volume and severe respiratory distress that is not responsive to aggressive treatment (including surfactant instillation/ mechanical respiratory support). It is usually not compatible with life.

Also known as: lung agenesis, primary pulmonary hypoplasia, pulmonary hypoplasia, primary

0 clinical trials for this condition and its sub-types, 0 tagged with Familial primary pulmonary hypoplasia itself.