Literature DB >> 28745802

Progressive subglottic stenosis in a child with Pallister-Killian syndrome.

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.
© 2017 Japanese Teratology Society.

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


  2 in total

1.  Clinical Variability of Pallister-Killian Syndrome in Two Egyptian Patients.

Authors:  Maha M Eid; Ola M Eid; Sawsan Abdel-Hadi; Nehal Hassib; Abdelrahman Madian; Hanan H Afifi; Ghada M H Abdel-Salam
Journal:  J Pediatr Genet       Date:  2019-11-21

Review 2.  Neuroimaging findings in Pallister-Killian syndrome.

Authors:  Emil Jernstedt Barkovich; Tarannum Musvee Lateef; Matthew T Whitehead
Journal:  Neuroradiol J       Date:  2017-12-20
  2 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.