leuprolide and Dwarfism

Topics: 11-beta-Hydroxysteroid Dehydrogenases; Adrenal Hyperplasia, Congenital; Body Height; Bone Development; Child; Dwarfism; Female; Hirsutism; Hormones; Humans; Hydrocortisone; Leuprolide; Physical Examination; Prognosis; Puberty, Precocious; Triptorelin Pamoate; Ultrasonography

Topics: Algorithms; Child; Diagnosis, Differential; Dwarfism; Endocrine System Diseases; Genetic Testing; Growth; Growth Disorders; Growth Hormone; Hormones; Humans; Leuprolide; Reference Values; Testosterone; United States

Background The use of gonadotropin-releasing hormone agonists (GnRHa) for pubertal suppression has been associated with increased body mass index (BMI) in female subjects with central precocious puberty (CPP), although results have been so far conflicting. This study examined the effects of GnRHa therapy in both genders and in subjects treated for CPP, early puberty or short stature. Methods This was a longitudinal retrospective study of subjects followed at outpatient pediatric endocrinology clinics of an academic medical center from 2005 to 2014 receiving GnRHa therapy. Results At 12 months, subjects on depot GnRHa had a statistically significant increase in BMI standard deviation score (SDS) from baseline (0.13 ± 0.35, p < 0.02). Subjects with short stature (0.17 ± 0.34, p < 0.02) but not early or precocious puberty, and subjects with normal baseline BMI (0.18 ± 0.38, p < 0.02) had significant increases in BMI SDS; no significance was noted at 24 months. Male subjects did not have a significant increase in BMI SDS, whereas female subjects did (0.11 ± 0.36, p < 0.01). Conclusions Subjects with short stature, normal BMI at baseline and female sex had significant increases in BMI SDS at 12 months. This is the first study to show an increase in BMI SDS in children treated with GnRHa for short stature, and is one of the few studies to assess BMI changes in males.

Topics: Biomarkers; Body Composition; Body Mass Index; Body Weight; Child; Dwarfism; Female; Fertility Agents, Female; Follow-Up Studies; Gonadotropin-Releasing Hormone; Humans; Leuprolide; Longitudinal Studies; Male; Prognosis; Puberty, Precocious; Retrospective Studies; Sexual Maturation

We report on a 10-year-old Japanese girl with Robinow syndrome accompanied by partial growth hormone (GH) insufficiency. We started GH replacement therapy at the chronological age of 6.5 years. In this case, improvement in the growth velocity was remarkable, but bone maturation accelerated even more. In order to avoid further acceleration of bone maturation, we started to treat our patient at the chronological age of 9.8 years with GH combined with gonadal suppression therapy using a luteinizing hormone releasing hormone analogue. However, no improvement in height SDS for bone age was attained. Our observations suggest that the indication of GH therapy for patients with Robinow syndrome needs careful consideration.

Topics: Body Height; Bone Development; Child, Preschool; Dwarfism; Facial Bones; Female; Genitalia, Female; Human Growth Hormone; Humans; Leuprolide; Syndrome