Document Detail


Neurofibromatosis and fragile-X syndrome in the same patient.
MedLine Citation:
PMID:  3933345     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report on an 11 1/2-year-old boy with neurofibromatosis and the fragile-X syndrome. Clinical manifestation of neurofibromatosis include multiple cafe-au-lait spots, axillary freckles, congenital glaucoma, relative macrocephaly, radiologic findings of overtubulation of the long bones, and precocious puberty. The fragile-X syndrome manifests itself as mental retardation with behavior problems, macro-orchidism, and specific cytogenetic findings. The boy has normal serum hormone levels, but a greatly elevated FSH on a first morning void, which contains the nocturnally secreted gonadotropins. This seems to be the first reported occurrence of the fragile-X syndrome with another inherited disease.
Authors:
J A Mitchell; J Wray; K Michalski
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  American journal of medical genetics     Volume:  22     ISSN:  0148-7299     ISO Abbreviation:  Am. J. Med. Genet.     Publication Date:  1985 Nov 
Date Detail:
Created Date:  1985-12-13     Completed Date:  1985-12-13     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  7708900     Medline TA:  Am J Med Genet     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  571-5     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Child, Preschool
Fragile X Syndrome / complications*
Humans
Karyotyping
Male
Mental Retardation / complications
Neurofibromatosis 1 / complications*,  pathology
Puberty, Precocious / complications*
Sex Chromosome Aberrations / complications*
Spina Bifida Occulta / pathology
Testis / pathology
Personal Name Subject
Personal Name Subject:
WZE

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


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