Prolongation of growth by treatment of 11-hydroxylase deficiency with depot-leuprolide, growth hormone, and hydrocortisone.
A 4-10/12 year-old boy presented with tall stature and advanced secondary sexual characteristics. His bone age was 13 years giving him a height prediction of 147 cm. An initial 11-deoxycortisol level of 13,770 ng/dl and associated hypertension suggested the diagnosis of 11-hydroxylase deficiency, which was confirmed by dexamethasone suppression and genotyping. Treatment strategy was based on the premise that known hypothalamic priming resulting in early pubertal development could be averted by delaying puberty with leuprolide; also that effects of hydrocortisone and leuprolide on attenuating growth could be counteracted by growth hormone. The combined treatment resulted in a final height at age 12 years which was 25.4 cm greater than predicted, and bone density above average. We conclude that delaying puberty until an appropriate age, offsetting growth suppression, and improving bone mineralization can be effectively achieved using glucocorticoids, leuprolide and growth hormone in patients with 11-hydroxylase deficiency.
Chalmers
Laura J
LJ
Department of Pediatrics, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104 , USA.
Casas
Luis
L
New
Maria I
MI
Blackett
Piers R
PR
D000312:Adrenal Hyperplasia, Congenital / Q000188:drug therapy* / Q000201:enzymology / Q000235:genetics; D001827:Body Height / Q000187:drug effects / Q000502:physiology; D015519:Bone Density / Q000187:drug effects / Q000502:physiology; D002648:Child; D003692:Delayed-Action Preparations; D005838:Genotype; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use*; D006973:Hypertension / Q000188:drug therapy / Q000503:physiopathology; D016729:Leuprolide / Q000008:administration & dosage*; D008137:Longitudinal Studies; D008297:Male; D011629:Puberty, Precocious / Q000517:prevention & control; D013252:Steroid 11-beta-Hydroxylase / Q000235:genetics / Q000378:metabolism
D003692:Delayed-Action Preparations; D019382:Human Growth Hormone; D013252:Steroid 11-beta-Hydroxylase; D016729:Leuprolide; D006854:Hydrocortisone