Congenital acinar dysplasia. Familial cause of a fatal respiratory failure in a neonate

Authors

• Kefah A. Al-Senan, Maternity and Children Hospital, Dammam, Kingdom of Saudi Arabia.
• Abdulhakiem K. Kattan, Section of Neonatology/Perinatology, Department of Pediatrics (MBC-58), King Faisal Specialist Hospital & Research Centre, PO Box 3354, Riyadh 11211, Kingdom of Saudi Arabia. Tel. +966 (1) 4427765. Fax. +966 (1) 4427784. E-mail: hakiem{at}kfshrc.edu.sa
• Fouad H. Al-Dayel, Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital & Research Centre, Riyadh, Kingdom of Saudi Arabia.

Abstract

Pulmonary hypoplasia is a rare cause of pulmonary insufficiency, and has a significant rate of morbidity and mortality among affected infants. In most cases, pulmonary hypoplasia is secondary to underlying abnormalities. These may include space occupying lesions, as in infants with congenital diaphragmatic hernia; malformation of chest wall resulting in a small thoracic cavity; severe and prolonged oligohydramnios; and neuromuscular disorders, which prevent normal fetal chest expansion. All lead to poor lung development. Primary pulmonary hypoplasia as a result of congenital acinar dysplasia is exceedingly rare and is diagnosed by exclusion of all known etiologies of secondary pulmonary hypoplasia.

Article Type

Case Report

First Page

88

Last Page

90

Recommended Citation

Al-Senan, Kefah A.; Kattan, Abdulhakiem K.; and Al-Dayel, Fouad H. (2003) "Congenital acinar dysplasia. Familial cause of a fatal respiratory failure in a neonate," Saudi Medical Journal: Vol. 24: Iss. 1, Article 21.
DOI: https://doi.org/10.15537/1658-3175.1944