All issues > Volume 30(10); 1987

Original Article

J Korean Pediatr Soc. 1987;30(10):1143-1151. Published online October 31, 1987.

A Clinical Studies in Patients with Turner's Syndrome.

Chang Hyun Yang¹, Duk Hi Kim¹, Kir Young KIm¹, Young Ho Yang¹

¹Department of Pediatrics, College of Medicine, Yonsei University, Seoul, Korea
²Department of Obstetrics and Gynecolgy, College of Medicine, Yonsei University, Seoul, Korea

Abstract

This study is systematical cytogenetic and clinico-hormonal analysis of 42 cases who were diagnosed as Turner’s syndrome at the Yonsei Medical Center from Jan. 1971 to Dec. 1985. The careful history taking, physical examination, cytogenetic studies, radiologic and hormonal evalua- tion, and GnRH stimulation test were performed. The results were as follows. 1) The patients ranged in age from 14 months to 30 years at the time of diagnosis and median age was 18 years old. 2) Clinically, short stature and sexual infanitilism with primary amenorrhea were found in nearly most cases, except infants and early childhood; with hearing impariment in 5 cases, cardiovascular abonormlities in 5 cases, mental retardation in 4 cases, endocrine abnormalities in 3 cases, and hypoplasia of right kidney in 1 case. 3) The karyotypes showed; ① 45, XO in 19 cases (45.2%) ② 45,XO/46, XX in 13 cases (30.9%) ③ 45, XO/46, XY in 5 cases (11.9%) ④ 45, XO/47, XXX in 2 cases (4.8%) ⑤ 45, XO/46, XX/47, XXX in 1 case (2.4%) ⑥ 45, XO/44, X, -G in 1 case (2.4%) in 1 case (2.4%) ⑦ 46, X, i (Xq) in 1 case (2.4%) 4) The markedly exaggerated rise in both plasma LH and FSH concentration has been demonstrat- ed after pubertal age but plasma Estradiol concentration was normal in prepubertal and low in postubertal compared to normal. 5. 6 cases of Turner’s syndrome patients, who performed GnRH stimulation test, showed that the plasma LH and FSH concentration reached the maximal level at 30 min. after injection of GnRH.

Keywords :Turner’s syndrome