Clinical and Genetic Studies of Tuberous Sclerosis.
- Author:
Johng Bong KAHNG
;
Young Ho WON
;
Young Pio KIM
- Publication Type:Original Article
- MeSH:
Crows;
Dermatology;
Female;
Humans;
Incidence;
Jeollanam-do;
Male;
Parents;
Sclerosis;
Sex Ratio;
Tuberous Sclerosis*;
Wills
- From:Korean Journal of Dermatology
1981;19(2):153-159
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The present clinical study was attempted to estimate the frequency of tuberous sclerosis using a paradigm. similar to the one employed by Crowe et al and to determine the genetic charaeteristics of the people of Chonnam Pravince regarding said disease. In addition other clinical manifestations were observed. Twenty-five subjects had visited the Department of Dermatology of Chonnam Medical University Hospital between 1970 and l980. Relatives of the subjects were examined whenever possible. The results of this study show that the incidence of this syndrome m Chonnam Province is probably in the region of l/80, 000~90,000. The sex ratio was nearly equal,12 males to 13 females. The mean age at first visit was about fifteen. OnIy 20 per cent of the cases have a familial history of the disease but the correct, incidence is probably higher since some patients provided incomplete family histories This clinical study suggests that tuberous sclerosis is an autosomal dominant inheritance disease with variable expressivity evidenced by the fact that although five of eleven of the groups offspring developed tuberosus sclerosis There were differences in the clinical features between the parent and the chiid.
