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Journal of Korean Neurosurgical Society 1993;22(12): 1309-1318. |
| Hereditary Aspect of Isolated Familial Acromegaly. |
| W C Lee, M S Shin, K S Lee, N Jung, S K Huh |
| Department of Neurosurgery, Presbyterian Medical Center, Jeonju, Korea. |
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| ABSTRACT |
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Acromegaly is classically considered a sporadic nonfamilial condition. The familial occurance of pituitary adenomas is encountered as a manifestation of the Multiple Endocrine Neoplasia Type I(MEN 1). Moreover in MEN 1 which has an autosomal dominant inheritance, acromegaly is seen in less than 10% of cases. Familial acromegly occurring 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 1. 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. |
| Key Words:
Isolated familial acromegaly; Multiple endocrine Neoplasia Type I(MEN I); Autosomal dominant inheritance; Chromosomal abnormality |
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