Delayed Puberty, Kallmann Syndrome, Hypogonadotropic Hypogonadism, GnRH Deficiency
Conditions
Keywords
delayed puberty, Kallmann Syndrome, Hypogonadotropic hypogonadism, GnRH Deficiency, kisspeptin, GnRH
Brief summary
The goal of this study is to test whether the hormone kisspeptin has the potential to prospectively diagnose adolescents with self-resolving or permanent delayed puberty. Some children with delayed puberty will eventually enter puberty on their own. However, some children with delayed puberty have a permanent condition and require medical treatment to undergo puberty. Right now, there is no reliable diagnostic tool to tell whether a child's delayed puberty will be self-resolving or permanent. The hormone kisspeptin has the potential to prospectively diagnose adolescents with self-resolving or permanent delayed puberty.
Detailed description
The investigators are seeking boys (ages 13.5-17 years) and girls (ages 12-17) years with a diagnosis of delayed puberty. Study participation involves 2 outpatient visits and two hospital admissions (one 11-hour, overnight admission and one 6-hour day admission) when subjects will receive two investigational drugs, the naturally occurring hormones kisspeptin and gonadotropin-releasing hormone (GnRH). Subjects will then be followed every 6 months until they reach 18 years of age to determine if their pubertal delay was self-resolved or permanent.
Interventions
0.24 nmol/kg
DRUGGnRH
75 ng/kg
Sponsors
Massachusetts General Hospital
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
OTHER
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
12 Years to 17 Years
Healthy volunteers
No
Inclusion criteria
Adolescent Boys * ages 13.5-17 years * testicular volume \<4 mL OR 4-8 ml with no change in the past 6 months by Prader orchidometer * first morning (before 9AM) LH \<2 mIU/mL and testosterone \<50 ng/dL Adolescent Girls * ages 12-17 years * Tanner stage I OR II breast development with no change in the past 6 months * first morning (before 9AM) LH \<2 mIU/mL and estradiol \<20 pg/ml All Subjects: * bone age less than chronological age * weight ≥ 28 kg * body mass index \>10th percentile and \<+3 SDS for bone age * blood pressure \>5th percentile and \<95th percentile for bone age and height * white blood cell counts, platelet counts, electrolytes, thyroid stimulating hormone (TSH), free thyroxine (T4) within reference range for age * erythrocyte sedimentation rate \<2X the upper limit of the reference range for age * hemoglobin within reference range for girls of the same chronological age * blood urea nitrogen (BUN), creatinine, prolactin not elevated * aspartate aminotransferase (AST) and alanine aminotransferase (ALT) no more than 2X the upper limit of the reference range * Insulin-like growth factor 1 (IGF-1) within reference range for bone age
Exclusion criteria
All Subjects: * history or presence of underlying condition that could cause delayed puberty (chronic illness, weight loss, abnormal cranial magnetic resonance imaging (MRI)) * history of an allergic drug reaction
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Average change in luteinizing hormone (LH) in response to kisspeptin | Within 30 minutes of administration |
Countries
United States
Outcome results
None listed