Abstract:

Objective To improve the recognition and treatment level of central precocious puberty in Turner syndrome (TS).Methods A case of central precocious puberty in 45,X TS was reported, and the related literatures were reviewed.Results ①A 7.5-year-old girl was referred with complaints of breast budding. Her height was 117.9 cm and weight was 32.5 kg, a physical examination revealed a Tanner stage Ⅱ for breast development and Tanner stage Ⅰ for pubic hair development, She showed no signs of Turner syndrome, such as webbed neck, cubitus valgus, high arched palate, and shield chest deformity. Lab examination: gonadotropin-releasing hormone stimulation test peak: luteinizing hormone was 11.9 U·L-1, follicule stimulating hormone was 34.2 U·L-1, estradiol was 39.3 ng·L-1, pelvis ultrasound showed enlarged ovaries, her bone age was 9.7 years. The patient was treated with gonadotropin releasing hormone analog for 2.7 years, her height was 131.4 cm and bone age was 12 years. Further, she has been given gonadotropin releasing hormone analog and recombinant human growth hormone for 2.3 years, her height was 148.4 cm and bone age was 13 years. After discontinuing drugs for 1.5 years, her height was 154.2 cm closed to genetic height. Lab examination showed luteinizing hormone was 11.9 U·L-1, follicule stimulating hormone was 50.5 U·L-1, estradiol was 38.9 ng·L-1, Chromosome analysis showed a karyotype of 45,X. ②A total of six patients associated with central precocious puberty in TS has been reported so far. Five of them were mosaic TS and other one was 46,X,del(X)(p11.2).Conclusion Central precocious puberty can occur in X-monosomy TS, the treatment of gonadotropin releasing hormone analog combined with recombinant human growth hormone can improve patient′s final height.