Search on: BESSEL-HAGEN DISEASE 
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Descriptor English:   Exostoses, Multiple Hereditary 
Descriptor Spanish:   Exostosis Múltiple Hereditaria 
Descriptor Portuguese:   Exostose Múltipla Hereditária 
Synonyms English:   Bessel-Hagen Disease
Exostoses, Multiple, Type I
Exostosis, Familial
Exostosis, Hereditary Multiple
Exostosis, Multiple
Exostosis, Multiple Cartilaginous
Familial Exostoses
Hereditary Multiple Exostosis
Multiple Cartilaginous Exostoses
Multiple Hereditary Exostoses
Multiple Osteochondromas
Multiple Osteochondromatosis
Aclases, Diaphyseal
Aclasis, Diaphyseal
Cartilaginous Exostoses, Multiple
Cartilaginous Exostosis, Multiple
Chondrodysplasias, Hereditary Deforming
Deforming Chondrodysplasia, Hereditary
Deforming Chondrodysplasias, Hereditary
Diaphyseal Aclases
Familial Exostosis
Hereditary Deforming Chondrodysplasia
Hereditary Deforming Chondrodysplasias
Hereditary Exostoses, Multiple
Multiple Cartilaginous Exostosis
Multiple Exostoses
Multiple Exostoses, Hereditary
Multiple Exostosis
Multiple Exostosis, Hereditary
Multiple Osteochondroma
Osteochondroma, Multiple
Diaphyseal Aclasis
Chondrodysplasia, Hereditary Deforming
Exostoses, Hereditary Multiple
Exostoses, Multiple
Exostoses, Multiple Cartilaginous
Hereditary Multiple Exostoses
Osteochondromas, Multiple
Exostoses, Familial  
Tree Number:   C04.557.450.565.575.610.615.325
C04.700.330
C05.116.099.708.670.615.325
C05.116.540.310.500
C16.320.700.330
Definition English:   Hereditary disorder transmitted by an autosomal dominant gene and characterized by multiple exostoses (multiple osteochondromas) near the ends of long bones. The genetic abnormality results in a defect in the osteoclastic activity at the metaphyseal ends of the bone during the remodeling process in childhood or early adolescence. The metaphyses develop benign, bony outgrowths often capped by cartilage. A small number undergo neoplastic transformation. 
History Note English:   90; was EXOSTOSES, MULTIPLE 1968-89 
Allowable Qualifiers English:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications CN congenital
DI diagnosis DG diagnostic imaging
DH diet therapy DT drug therapy
EC economics EM embryology
EN enzymology EP epidemiology
EH ethnology ET etiology
GE genetics HI history
IM immunology ME metabolism
MI microbiology MO mortality
NU nursing PS parasitology
PA pathology PP physiopathology
PC prevention & control PX psychology
RT radiotherapy RH rehabilitation
SU surgery TH therapy
UR urine VE veterinary
VI virology  
Record Number:   28249 
Unique Identifier:   D005097 

Occurrence in VHL:
 

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