Document Detail


Thrombocytopenia in patients with 22q11.2 deletion syndrome and its association with glycoprotein Ib-beta.
MedLine Citation:
PMID:  12644781     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
PURPOSE: To elucidate whether thrombocytopenia in 22q11.2 deletion syndrome patients is associated with the hemizygosity of glycoprotein Ib-beta and to clarify the correlation of phenotype and genotype of this gene in 22q11.2 deletion syndrome patients with thrombocytopenia. METHODS: Platelet number, mean platelet volume, platelet agglutination, and the protein level of glycoprotein Ib-beta were measured in 22q11.2 deletion syndrome patients and controls. Phenotypes other than that of thrombocytopenia were also analyzed in these patients. RESULTS: The 22q11.2 deletion syndrome patients with thrombocytopenia had a larger mean platelet volume, lower agglutination to ristocetin, and lower protein level of glycoprotein Ib-beta than control patients. The 22q11.2 deletion syndrome patients with thrombocytopenia showed an increased risk of developing schizophrenia. CONCLUSIONS: Thrombocytopenia in 22q11.2 deletion syndrome patients is associated with decreased expression of glycoprotein Ib-beta because of the hemizygosity. 22q11.2 deletion syndrome patients with thrombocytopenia require total management, especially for schizophrenia.
Authors:
Taichi Kato; Kazuki Kosaka; Misa Kimura; Shin-Ichiro Imamura; Osamu Yamada; Kazumasa Iwai; Masahiko Ando; Kunitaka Joh-o; Kenji Kuroe; Akira Ohtake; Atsuyoshi Takao; Kazuo Momma; Rumiko Matsuoka
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Genetics in medicine : official journal of the American College of Medical Genetics     Volume:  5     ISSN:  1098-3600     ISO Abbreviation:  Genet. Med.     Publication Date:    2003 Mar-Apr
Date Detail:
Created Date:  2003-03-19     Completed Date:  2003-10-07     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9815831     Medline TA:  Genet Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  113-9     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Nagoya University Graduate School of Medicine, Nagoya, Japan.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Adult
Blotting, Western
Child
Child, Preschool
Chromosome Deletion*
Chromosomes, Human, Pair 22*
Female
Humans
In Situ Hybridization, Fluorescence
Male
Phenotype
Platelet Count
Platelet Glycoprotein GPIb-IX Complex / genetics*
Sequence Analysis, DNA
Thrombocytopenia / blood,  genetics*
Chemical
Reg. No./Substance:
0/Platelet Glycoprotein GPIb-IX Complex

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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