Document Detail


Galactose-1-phosphate uridyl transferase deficiency is not associated with Müllerian aplasia in Dutch patients.
MedLine Citation:
PMID:  19646668     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
STUDY OBJECTIVE: To study whether a deficiency in galactose-1-phosphate uridyl transferase (GALT) activity of mothers was an explanation for the occurrence of Müllerian aplasia of their daughters. DESIGN: A case control study. SETTING: The patients were selected from the outpatient clinic of the University Medical Center Nijmegen, and compared with the general population in The Netherlands. PARTICIPANTS: Patients (n=9) diagnosed with the syndrome of Müllerian aplasia and their mothers were included. INTERVENTIONS: A questionnaire for medical and family history was taken, and a venous blood sample and urine were collected. MAIN OUTCOME MEASURES: GALT activity (in blood), galactose and galactilol (in urine) were measured. Measured values were analyzed by Student's paired t-test. RESULTS: All patients and their mothers had normal GALT activities> or =20 micromol/h/g Hb. The mean value did not differ from the mean of the normal Dutch population, which was 31.6 (SD=5.0) mumol/h/g Hb. CONCLUSION: GALT deficiency is not an explanation for Müllerian aplasia, at least in the Dutch population.
Authors:
Roel Nijland; Francis E Hartog; Ron A Wevers; Ronald J A Wanders; Wim N P Willemsen
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric and adolescent gynecology     Volume:  22     ISSN:  1873-4332     ISO Abbreviation:  J Pediatr Adolesc Gynecol     Publication Date:  2009 Aug 
Date Detail:
Created Date:  2009-08-03     Completed Date:  2009-10-22     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9610774     Medline TA:  J Pediatr Adolesc Gynecol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  229-31     Citation Subset:  IM    
Affiliation:
Department of Obstetrics and Gynaecology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
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MeSH Terms
Descriptor/Qualifier:
Adult
Case-Control Studies
Female
Galactose / urine
Humans
Middle Aged
Mullerian Ducts / abnormalities*
Netherlands
Polymorphism, Single Nucleotide*
Pregnancy
Syndrome
UTP-Hexose-1-Phosphate Uridylyltransferase / genetics*
Young Adult
Chemical
Reg. No./Substance:
26566-61-0/Galactose; EC 2.7.7.10/UTP-Hexose-1-Phosphate Uridylyltransferase

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


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