ESPE2021 ePoster Category 2 Sex differentiation, gonads and gynaecology or sex endocrinology (52 abstracts)
1Research Institute of Pediatric Endocrinology National Institute, Endocrinology Research Centre, Moscow, Russian Federation; 2Russian Medical Academy of Continuous Professional Education, Moscow, Russian Federation
Objective: assess advantages and disadvantages of the treatment of hypogonadotropic hypogonadism (HH) with rFSH and hCG.
Materials: We report the case of а 16-year old patient with complaints of no development of secondary sexual characteristics. Patient was 174 cm (SDS growth 0.25 SD, SDS growth velocity 1.62 SD, SDS BMI -0.4 SD, target height correction 1.01 SD) with Tanner G1 P1 (penis length was 5 cm). Laboratory studies included LH < 0.1 mIU/ml (0.8-7.6), FSH = 0/15 mIU/ml (0.7–11), testosterone 1.05 nmol/l (2.25-26.99), SHBG 115.5 (13.3-89.5), free testosterone index 0.9 % (15.5-102), inhibin B = 50.2 pg/ml (25-325). AMH=56.04 ng/ml (4,95-144,48). Bone age was 14 years (SDS = - 2.1 SD). GnRH test with triptorelin revealed LH peak of 1.81 ME/ml and LH/FSH ratio was 0.85.On hCG stimulation (2000 IU/m2) total testosterone was 3.3 nmol/l. Testicular volumes by ultrasound were 1.1 cm3 and 1.04 cm3. Olfactometry revealed mild hyposmia. MRI revealed no pathology. Karyotype was XY. Molecular genetic revealed no mutations. In 3 months of testosterone stimulation (3 injections of 100 mg once in 30 days) Tanner stage was P3G1, testicular volume raised to 1.12 cm3, testosterone was 0.87 nmol/l, inhibin B was 64.1 pg/ml, LH peak on triptorelin test 0.68, LH/FSH ratio was 0.3. A patient was diagnosed with HH.Therapy with rFSH 75 ME in a week and hCG 1000 ME in a week started.hCG titrated to 3000 ME/week. After 2 years patient was 188 cm(SDS growth = +2SD, target height correction + 1.43 SD), bone age was 15,5 years, Tanner stage progressed to P5G5, US-proven testicular volume was 7.1 and 11.3 in 16 months of therapy. After 7 months of therapy testosterone already was 20.3 mmol/l, SHBG lowered to 44.2 nmol/l. Estradiol wasn't increased. AMH firstly raised at 4th month (172/2 ng/ml) and then lowered to 10.56 at 16th month. Inhibin B raised to 153.1 pg/ml. In 4 months after withdrawal the therapy patient started to complain of libido loss, depression.LH <0.2 mME/l, FSH <0.2 mME/l, testosterone 0.55 nmol/l, SHBG 79.4 g/l, inhibin В 114.2 pg/ml. Replacement therapy with rFSH and LH continued.
Conclusion: therapy of rFSH amd hCG developed secondary sexual characteristics, raised testosterone with no estradiol increase. Testicular volume 8-10-fold increased, inhibin B increased threefold.AMH raised and then lowered. Therapy helped to induct puberty according to increased testosterone so better fertility can be expected.