PT  - JOURNAL ARTICLE
AU  - Kattan, Abdulhakeim K.
AU  - Bulagannawar, Prakash S.
AU  - Hussain, Iftikhar
TI  - Congenital alveolar proteinosis
DP  - 2004 Oct 01
TA  - Saudi Medical Journal
PG  - 1474--1477
VI  - 25
IP  - 10
4099  - http://smj.org.sa/content/25/10/1474.short
4100  - http://smj.org.sa/content/25/10/1474.full
SO  - Saudi Med J2004 Oct 01; 25
AB  - Pulmonary alveolar proteinosis is recently described as a rare cause of lung dysfunction and respiratory distress in term neonates. In several cases, a deficiency or insufficiency of surfactant protein B SP-B has been caused by a frame shift mutation in the gene encoding SP-B. Three siblings with congenital pulmonary alveolar proteinosis showed clinical and radiological evidence. Histopathological and immunohistochemical studies in the last sibling revealed deficiency of SP-B, one of the group of 3 specific lipoproteins that reduce the surface tension between air and liquid interface within pulmonary alveoli, suggesting a gene associated illness.