Document Detail


Fryns anophthalmia-plus syndrome with hypoplastic adrenal glands.
MedLine Citation:
PMID:  18564500     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report a family with two consequent sibs with anophthalmia and cleft lip and palate. A 27 year old woman married to her first cousin was counseled for anophthalmia and cleft lip and palate detected during routine fetal ultrasonographic examination on the 23rd week of the pregnancy. Her obstetric history revealed a healthy girl aged 7 years and a boy with anophthalmia and cleft lip and palate who lived for 20 days in the neonatal intensive care unit. The current pregnancy was terminated after the diagnosis, and post mortem examination of the fetus revealed pre-maxilla agenesis, anophthalmia, cerebral ventricular dilatation, adrenal hypoplasia and single umbilical artery. Chromosome analysis resulted in normal karyotypes of the fetus and both parents. The inheritance pattern was regarded as autosomal recessive and the family was informed about the condition and risks during genetic counseling.
Authors:
O Ozalp; E E Ozcimen; Z Yilmaz; F Yanik; F I Sahin
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Genetic counseling (Geneva, Switzerland)     Volume:  19     ISSN:  1015-8146     ISO Abbreviation:  Genet. Couns.     Publication Date:  2008  
Date Detail:
Created Date:  2008-06-20     Completed Date:  2008-09-26     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9015261     Medline TA:  Genet Couns     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  43-6     Citation Subset:  IM    
Affiliation:
Baskent University Faculty of Medicine, Department of Medical Genetics, Ankara, Turkey.
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MeSH Terms
Descriptor/Qualifier:
Abortion, Induced
Adrenal Glands / abnormalities*
Adult
Anophthalmos / complications,  genetics*
Cleft Lip / complications,  diagnosis,  genetics
Cleft Palate / complications,  diagnosis,  genetics
Female
Fetal Diseases / diagnosis
Genetic Counseling
Humans
Pregnancy
Pregnancy Trimester, Second
Prenatal Diagnosis
Syndrome

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


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