Document Detail


Blood chimerism in a girl with Down syndrome and possible freemartin effect leading to aplasia of the Müllerian derivatives.
MedLine Citation:
PMID:  20190264     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Cytogenetic and molecular genetic analysis in a case of sex-discordant dizygotic twins revealed blood chimerism in the girl (46,XY in blood and 47,XX, + 21 in fibroblasts) caused by feto-fetal transfusion from her healthy brother. The girl presented with Down syndrome, aplasia of the uterus and the Fallopian tubes and normal female external genitalia. We propose that the lack of Müllerian structures is caused by the effect of the Müllerian inhibiting substance transferred from the male to the female twin in early pregnancy. This disorder of sex development is known as freemartin phenomenon in female cattle from sex-discordant twin pairs.
Authors:
N Bogdanova; U Siebers; R Kelsch; A Markoff; A Röpke; R Exeler; J Tsokas; P Wieacker
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-02-26
Journal Detail:
Title:  Human reproduction (Oxford, England)     Volume:  25     ISSN:  1460-2350     ISO Abbreviation:  Hum. Reprod.     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-04-14     Completed Date:  2010-07-29     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8701199     Medline TA:  Hum Reprod     Country:  England    
Other Details:
Languages:  eng     Pagination:  1339-43     Citation Subset:  IM    
Affiliation:
Institute of Human Genetics, ZMBE Westfalian Wilhelms-University of Münster and University Clinics Münster, Münster, Germany.
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MeSH Terms
Descriptor/Qualifier:
Adult
Animals
Cattle
Child, Preschool
Chimera / genetics*
Chromosomes, Human, Y
Down Syndrome / blood,  genetics*,  immunology
Female
Fetofetal Transfusion / blood,  genetics
Freemartinism / genetics*
Haplotypes
Histocompatibility Testing
Humans
In Situ Hybridization, Fluorescence
Infant, Newborn
Karyotyping
Lymphocytes / ultrastructure
Male
Mullerian Ducts / abnormalities*
Pedigree
Pregnancy
Twins, Dizygotic

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


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