Document Detail


Congenital myotonic dystrophy.
MedLine Citation:
PMID:  16260886     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We describe a case of severe congenital myotonic dystrophy (CDM). A 38-year-old primigravida, who was known to suffer from mild myotonic dystrophy (DM), conceived spontaneously and booked for confinement at 11 weeks in our unit. The couple had been fully counseled about the risks of transmission of this condition to their offspring before embarking on this pregnancy. Despite being fully aware of the risks, they declined prenatal diagnosis. The pregnancy was monitored by serial ultrasound scans. The diagnosis of CDM was suspected by ultrasound markers of borderline ventriculomegaly, polyhydramnios, and reduced fetal movements. The pregnancy ended prematurely at 33 weeks in an emergency caesarean section because of severe fetal compromise. The neonate died almost immediately after birth. The genetic analysis of cord blood confirmed severe DM. This case highlights the importance of ultrasound markers for the diagnosis of CDM in the absence of definitive prenatal diagnosis.
Authors:
K Upadhyay; A Thomson; M J M Luckas
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Fetal diagnosis and therapy     Volume:  20     ISSN:  1015-3837     ISO Abbreviation:  Fetal. Diagn. Ther.     Publication Date:    2005 Nov-Dec
Date Detail:
Created Date:  2005-11-01     Completed Date:  2006-01-19     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9107463     Medline TA:  Fetal Diagn Ther     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  512-4     Citation Subset:  IM    
Copyright Information:
Copyright (c) 2005 S. Karger AG, Basel.
Affiliation:
Department of Obstetrics and Gynaecology, Leighton Hospital, Mid Cheshire NHS Trust, Crewe, UK. upadhyayok@yahoo.com
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MeSH Terms
Descriptor/Qualifier:
Adult
Fatal Outcome
Female
Genetic Predisposition to Disease
Humans
Infant, Newborn
Male
Myotonic Dystrophy / diagnosis,  genetics,  ultrasonography*
Pregnancy
Pregnancy Complications / diagnosis,  genetics,  ultrasonography*
Treatment Refusal
Ultrasonography, Prenatal

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


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