Document Detail


The Pallister-Killian syndrome in a child with rare karyotype--a diagnostic problem.
MedLine Citation:
PMID:  18000682     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The Pallister-Killian syndrome is a clinically recognizable syndrome, usually due to a tissue-specific mosaicism for a 12p isochromosome [i(12p)]. We report a rare case of Pallister-Killian syndrome with 12p mosaicism, tetrasomy/trisomy/disomy in fibroblasts and trisomy/disomy in lymphocytes. Marker chromosomes were investigated with conventional cytogenetic techniques and fluorescent in situ hybridisation (FISH). The karyotype was established as: mos47,XX,+12p/47,XX,+i(12p)/46,XX. The cytogenetic result of the extra mosaic 12p presented in lymphocytes suggested the diagnosis of trisomy 12p, although, in combination with clinical manifestations, the Pallister-Killian syndrome was considered and confirmed by the cytogenetic analysis of fibroblasts.
Authors:
Robert Smigiel; Jacek Pilch; Izabela Makowska; Halina Busza; Ryszard Slezak; Maria M Sasiadek
Publication Detail:
Type:  Case Reports; Journal Article     Date:  2007-11-14
Journal Detail:
Title:  European journal of pediatrics     Volume:  167     ISSN:  1432-1076     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  2008 Sep 
Date Detail:
Created Date:  2008-07-28     Completed Date:  2009-01-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  1063-5     Citation Subset:  IM    
Affiliation:
Department of Genetics, Wroclaw Medical University, Marcinkowskiego 1, Wroclaw, 50-368, Poland. smigiel@gen.am.wroc.pl
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MeSH Terms
Descriptor/Qualifier:
Child, Preschool
Chromosome Aberrations*
Female
Humans
In Situ Hybridization, Fluorescence
Mosaicism
Pallister-Hall Syndrome / diagnosis,  genetics*,  physiopathology*

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