Document Detail


The Cohen syndrome. Retinal lesions and granulocytopenia.
MedLine Citation:
PMID:  2348983     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A patient with the Cohen syndrome is described and the literature is reviewed. Electroretinography (ERG) showed that the patient had a tapeto-retinal degeneration. The authors believe that a retinitis pigmentosa-like phenotype is an obligatory sign in patients with the Cohen syndrome. Assessment of a tapeto-retinal dystrophy by electroretinography, however, is often not available for mentally retarded persons. The authors' patient also had granulocytopenia. This was previously described in Finnish patients with Cohen's syndrome. They speculate that this sign is more prevalent in the disorder than would appear from the literature. Alternatively, there may be a type I Cohen syndrome with granulocytopenia and a type II without.
Authors:
M Warburg; S A Pedersen; H Hørlyk
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Ophthalmic paediatrics and genetics     Volume:  11     ISSN:  0167-6784     ISO Abbreviation:  Ophthalmic Paediatr Genet     Publication Date:  1990 Mar 
Date Detail:
Created Date:  1990-07-11     Completed Date:  1990-07-11     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  8206832     Medline TA:  Ophthalmic Paediatr Genet     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  7-13     Citation Subset:  IM    
Affiliation:
Gentofte Hospital, Eye Clinic for Patients with Multiple Handicaps, Denmark.
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MeSH Terms
Descriptor/Qualifier:
Abnormalities, Multiple* / genetics
Adult
Agranulocytosis / complications*,  genetics
Electroretinography
Female
Fundus Oculi
Humans
Mental Retardation / complications,  genetics
Night Blindness / complications
Phenotype
Retina / pathology
Retinal Degeneration / complications*,  genetics,  pathology
Syndrome
Vision Disorders / complications,  genetics
Visual Acuity

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


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