The Prevalence of Renal Malformation in Turner Syndrome in Korea

Rho, Kwang-Sik; Kim, Ji-Hong; Kim, Pyung-Kil; Chung, So-Jung; Kim, Duk-Hi

J Korean Soc Pediatr Nephrol > Volume 1(2); 1997 > Article

J Korean Soc Pediatr Nephrol 1997;1(2): 151-154.

소아 Turner증후군 환자에서 신기형의 동반율

노광식, 김지홍, 김병길, 정소정, 김덕희

¹연세대학교 의과대학 소아과학교실
²연세대학교 의과대학 소아과학교실
³연세대학교 의과대학 소아과학교실
⁴연세대학교 의과대학 소아과학교실
⁵연세대학교 의과대학 소아과학교실

The Prevalence of Renal Malformation in Turner Syndrome in Korea

Kwang-Sik Rho, Ji-Hong Kim, Pyung-Kil Kim, So-Jung Chung, Duk-Hi Kim

¹Department of Pediatrics, Yonsei University, Collage of Medicine
²Department of Pediatrics, Yonsei University, Collage of Medicine
³Department of Pediatrics, Yonsei University, Collage of Medicine
⁴Department of Pediatrics, Yonsei University, Collage of Medicine
⁵Department of Pediatrics, Yonsei University, Collage of Medicine

ABSTRACT

Purpose : The prevalence of renal malformation in Turner syndrome has been quoted as being greater than 50% in older series. Recently in 1988, Lippe's review gave a prevalence of 33%, significantly lower than all previous reports. In 1996, Flynn reported that renal malformation occurs in approximately 24% of all girls with Turner syndrome, and that it is seldom seen in girls with mosaic karyotype who form the predominant subgroup. The aim of this study was to evaluate prevalence of renal malformation by karyotype in Turner syndrome in Korea. Method : We evaluated 81 patients with Turner sundrome diagnosed in Yonsei University from Jan. 1987 to Dec. 1996. The patient entered in this study were those for whom both karyotype and ultrasound examination of the kidney were available. Result : 1) The karyotype showed: 45,X ; 29 cases (38%), mosaicism : 32 cases (40%), structural aberration ; 17 cases (22%). 2) Of the 29 cases of pure 45,X karyotype, 5(17%) had abnormal renal findings, while these were found in only 1 of the 30 mosaic cases(3.3%), and in 1 of the 17 structural aberration cases(6%). The malformation included 3 cases of horseshoe kidney, 2 cases of axial malrotation, hypoplastic kidney and simple cyst each one. There was no statistical significance between 3 groups (p=0.09).
Conclusion : We conclude that renal malformation occurs in 9.2% in this study, therefore Korean girls with Turner syndrome have lower rates of renal malformation.

Key words: Turner syndrome | Renal malformation | Karyotype