Korean J Perinatol.
2014 Mar;25(1):17-21. 10.14734/kjp.2014.25.1.17.
A Case of Pai Syndrome: First Reported Case in Korea
- Affiliations
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- 1Department of Pediatrics, Cheil General Hospital & Women's Health Care Center, Kwandong University College of Medicine, Seoul, Korea. ykleeped@hanmail.net
- KMID: 1917228
- DOI: http://doi.org/10.14734/kjp.2014.25.1.17
Abstract
- Pai syndrome is a rare disorder, first described in 1987. Diagnostic criteria are the presence of the nasal polyp and one of the following: midline cleft lip, congenital polyp of mid-anterior alveolar process, and pericallosal lipoma. Thirty-six cases of Pai syndrome have been described so far. We report 1 case of Pai syndrome accompanied by congenital nasal polyp and callosal lipoma with partial agenesis of corpus callosum, the first time in Korea.
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