ESPE Abstracts (2021) 94 P2-431

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.

Volume 94

59th Annual ESPE (ESPE 2021 Online)

Online,
22 Sep 2021 - 26 Sep 2021

European Society for Paediatric Endocrinology 

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