Pulmonary hypoplasia | |
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Other names | Familial primary pulmonary hypoplasia |
This condition is inherited in an autosomal recessive manner | |
Specialty | Pulmonology |
Pulmonary hypoplasia is an incomplete development of the lungs, resulting in an abnormally low number or small size of bronchopulmonary segments or alveoli. A congenital malformation, most often occurs secondary to other fetal abnormalities that interfere with normal development of the lungs. Primary (idiopathic) pulmonary hypoplasia is rare and usually not associated with other maternal or fetal abnormalities.
Incidence of pulmonary hypoplasia ranges from 9–11 per 10,000 live births and 14 per 10,000 births.[1] Pulmonary hypoplasia is a relatively common cause of neonatal death.[2] It also is a common finding in stillbirths, although not regarded as a cause of these.