Kallmann Syndrome, Hypogonadotropic Hypogonadism
Conditions
Brief summary
Observe the therapeutic efficacy of human menopausal gonadotropin combining with human chorionic gonadotropin in adolescent boys with congenital hypogonadotropic hypogonadism.
Detailed description
Observe safety and efficacy of human menopausal gonadotropin and human chorionic gonadotropin treating congenital hypogonadotropic hypogonadism in teenagers; which as clinic recommendation, may provide clinical basis for establishing standard treatment guideline in the future. Establish technological process and follow-up precept for human menopausal gonadotropin and human chorionic gonadotropin injection treating congenital hypogonadotropic hypogonadism in teenagers. And find safety and effective dose for teenagers.
Interventions
Human Menopausal Gonadotropin injection treating congenital hypogonadotropic hypogonadism in teenagers
DRUGHuman Chorionic Gonadotropin
Human Chorionic Gonadotropin injection treating congenital hypogonadotropic hypogonadism in teenagers
Sponsors
Beijing Children's Hospital
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
MALE
Age
138 Months to 18 Years
Healthy volunteers
No
Inclusion criteria
Criteria A * Boy \>14yr without any sign of puberty, testis \<4ml * BA ≥12yr * Sex hormone (LH,FSH, T) are pre-pubertal level * No other hormones problems (other pituitary glands axis are normal except gonad axis) * No space occupying lesion, No tumor on MRI of pituitary and hypothalamus area * Kallmann's syndrome(KS) patients may companies with dysosmia or dysplasia of olfactory bulb or olfactory tract on MRI * Karyotype is 46，XY * Exclude chronic diseases, malnutrition Criteria B * For the boy \<14yr. who companies with micropenis or cryptorchid or hypospadias and they have anosmia or dysplasia of olfactory bulb/olfactory sulcus/olfactory structs on MRI include in. Criteria C * As the phenotype of hypogonadotropic hypogonadism are variant, some of them may have partial puberty. So, we enrolled them when they have testis volume \>4ml or the testosterone level \>200ng/L，companies anosmia or dysplasia of olfactory bulb /olfactory sulcus/ olfactory structs on MRI, and the puberty arrested in half a year. These patients can be diagnosed as Kallmann Syndrome.
Exclusion criteria
* Any ascertain reason contributes to the non puberty development (Chromosome abnormal, trauma, surgeries) or any ascertain disease such as Prader-Willi syndrome or hypergonadotropic hypogonadism * Systemic diseases (such as chronic kidney failure, Mediterranean anemia, poor controlled diabetes) * Protein-energy malnutrition * Eating disorder (such as anorexia nervosa, binge eating) * Any brain diseases history: tumors in brain or pituitary or after their surgeries
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| testicular volume | Change from Baseline testicular volume at 3 months after treatment |
Secondary
| Measure | Time frame |
|---|---|
| The levels of testosterone serum (It were measured with chemiluminescent immunoassay Elecsys) | Testosterone changes from 3 months onwards after treatment compared to pretreatment |
Countries
China
Contacts
Primary ContactYing Liu, master
Backup ContactChunxiu Gong, doctor
Outcome results
None listed