| Literature DB >> 28745802 |
Tadashi Shiohama1, Katsunori Fujii1, Kenji Shimizu2, Hirofumi Ohashi2, Tomozumi Takatani1, Nobuhiko Okamoto3, Gen Nishimura4, Mitsuhiro Kato5, Naoki Shimojo1.
Abstract
Pallister-Killian syndrome (PKS) is rare genetic disorder caused by tetrasomy 12p mosaicism with supernumerary isochromosome 12p that manifests with intellectual disability, craniofacial dysmorphism, and epilepsy. Although PKS presents as a multisystem morphological defect, respiratory system involvement is rare, except for diaphragmatic hernia. We are the first to report a case of PKS with progressive subglottic stenosis. Subglottic stenosis is a potentially lethal condition due to severe respiratory obstruction and difficult intubation; therefore, further accumulation of cases is required to assess the causal link between PKS and subglottic stenosis.Entities:
Keywords: Pallister-Killian syndrome; buccal mucosal FISH; polymicrogyria; subglottic stenosis; tetrasomy 12p
Mesh:
Year: 2017 PMID: 28745802 DOI: 10.1111/cga.12240
Source DB: PubMed Journal: Congenit Anom (Kyoto) ISSN: 0914-3505 Impact factor: 1.409