|
KMID : 0359819930220121309
|
|
Journal of Korean Neurosurgical Society
1993 Volume.22 No. 12 p.1309 ~ p.1318
|
|
|
Hereditary Aspect of Isolated Familial Acromegaly
|
|
이원창
신문수/이광수/정남/허승곤
|
|
|
Abstract
|
|
|
Acromegaly is classically considered a sporadic nonfamilial condition. The familial occurance of pituitary adenomas is encoutered as a manifestation of the Multiple Endocrine Neoplasia Type I (MEN 1). Moreover in MEN I which has an autosomal
dominant
inheritance, acfromegaly is seen in less than 10% of cases. Fmilial acromegly occuring on isolation has been reported only rarely. There have been only four reports of isolated familial acromegaly since firm biochemical diagnosis of GH excess
became
possible.
We experienced three members of isolated familial acromegaly over 2 generations, distinct from MEN I. In all of 3 patients, we had found no evidence of other endocrinopathies and chromosomal abnormalities.
We conclude that isolated familial acromegaly is regarded as a distinct clinical entity rather than as a part of MEN I. We think hereditary factor for this condition probably exist and so further study is needed.
|
|
|
KEYWORD
|
|
|
|
|
|
FullTexts / Linksout information
|
|
|
|
|
Listed journal information
|
|
 
|