Gonadotropin deficiency secondary to hypogonadotropic hypogonadism or pituitary hormone deficiency Nutritional, Metabolic, Endocrine
Conditions
Interventions
The PinG study is an open-label randomised controlled study. The protocol is stratified according to disease severity, into those participants with partial congenital hypogonadotropic hypogonadism (CH
Sponsors
Queen Mary University of London
Eligibility
Sex/Gender
Male
Inclusion criteria
Inclusion criteria: 1. Age =12 and <35 years 2. Males 3. Participant or their parent/carer is willing and able to give informed consent for participation, with informed assent from the participant if <16 years 4. Able and willing to comply with all study requirements, including ability to participate in study for a minimum of 18 months 5. Willing to allow their General Practitioner (GP) to be notified of participation in the study 6. Diagnosis of gonadotropin deficiency (hypogonadotropic hypogonadism) by biochemical criteria (low or undetectable basal LH and FSH with low or undetectable testosterone) 7. Additional confirmatory features (at least one of): 7.1. Inhibin B <150 pg/ml 7.2. Red flags – small testes volumes, cryptorchidism, micropenis, synkinesis, anosmia 7.3. Clinical hypogonadotropic hypogonadism with additional pituitary hormone defects (GH, TSH, ACTH deficiency) 7.4. Peak LH on GnRH stimulation testing of <5 IU/l 7.5. Diagnosis of congenital hypogonadotropic hypogonadism confirmed by genetic testing 7.6. MRI pituitary/ olfactory bulbs demonstrating anatomical abnormalities consistent with hypogonadotropic hypogonadism 8. Patient requiring treatment to induce or complete the induction of puberty Additional inclusion criteria for partial GD: 1. Maximal testes volumes =4 ml Additional inclusion criteria for severe GD: 1. Maximal testes volumes <4 ml
Exclusion criteria
Exclusion criteria: 1. Age <12 or =35 years 2. Females 3. Functional hypogonadism due to low caloric intake, excessive exercise or chronic disease 4. Primary hypogonadism 5. Constitutional or self-limited delayed puberty 6. Active hypothalamic or pituitary tumour 7. Uncontrolled non-gonadal endocrinopathies (e.g. thyroid, adrenal or pituitary disorders) 8. Hypersensitivity to the active substance or excipients
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Capacity for spermatogenesis is measured by total sperm concentration =1 x 10e6/ml on semen analysis at 18 months of treatment | — |
Secondary
| Measure | Time frame |
|---|---|
| 1. Testicular development measured by testicular volume via orchidometer at 0, 6, 12, 18 (+/- 24) months of treatment 2. Testicular development measured by Inhibin B concentration in blood samples at 0, 6, 12, 18 (+/- 24) months of treatment 3. Sperm quality measured by maximal sperm concentration on semen analysis at 18 (+/- 24) months of treatment 4. Treatment length to achieve spermatogenesis measured by months to reach a total sperm concentration =1 x 10e6/ml on semen analysis at 18 + 24 months of treatment 5. Genetic contribution to response to treatment measured by whole genome sequencing at baseline with analysis of correlation of pathogenic variants identified with primary outcomes 6. Quality of life changes after treatment measured by questionnaire at baseline and end of treatment | — |
Countries
England, Scotland, United Kingdom
Contacts
Public ContactShared Team inbox
Outcome results
None listed