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This version published online on November 2, 2004
Journal of Clinical Endocrinology & Metabolism, doi:10.1210/jc.2004-1438
A more recent version of this article appeared on January 1, 2005
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Submitted on July 21, 2004
Accepted on October 18, 2004

Adult height after ketoconazole treatment in patients with familial male-limited precocious puberty

Leandro Soriano-Guillén, Najiba Lahlou, Geneviève Chauvet, Marc Roger, Jean Louis Chaussain, and Jean Claude Carel*

Department of Pediatric Endocrinology and INSERM U561, Groupe hospitalier Cochin-Saint Vincent de Paul and Faculté Cochin - Université Paris V, Paris, France.; Laboratory of Hormonal Biochemistry, Groupe Hospitalier Cochin-Saint Vincent de Paul, 82 av Denfert Rochereau, 75014 Paris; IREM, Groupe Hospitalier Cochin-Saint Vincent de Paul, 82 av Denfert Rochereau, 75014 Paris; Department of Pediatrics, Centre Hospitalier Dr Schaffner, 99 Route de la Bassée, 62307 Lens

* To whom correspondence should be addressed.
Jean Claude Carel, E-mail: carel{at}paris5.inserm.fr

Familial male-limited precocious puberty is a rare cause of precocious puberty due to activating mutations of the LH receptor, leading to early onset virilization and short stature. Two therapeutic approaches have been proposed: the P450 cytochrome inhibitor ketoconazole or combined treatment with spironolactone and testolactone. Results on adult heights have not been reported so far with these two treatments and we present here results in 5 patients treated with ketoconazole at a median dose of 16.2 mg/kg/d for a median of 6.2 yr. Adult height was 173 [14] cm (median [IQR]), similar to target height (175 [9]) cm) and significantly higher than pretreatment predicted height (165 [12]) cm (P < 0.01)). During treatment, 39/58 (68%) testosterone measurements were <0.5 ng/ml (1.7 nmol/L), 9/58 (15%) between 0.5 and 1 ng/ml (3.5 nmol/L) and 10/58 (17%) above 1 ng/ml. We observed a physiological increase of GnRH stimulated LH levels after the age of 10 yr and none of the patients had early activation of the gonadotropic axis. Liver tolerance was excellent and only one patient had a transient and modest rise of serum transaminases. We conclude that ketoconazole is an efficient and well tolerated long-term treatment of familial male-limited precocious puberty that should be proposed as a first line therapy.
Key words: Testotoxicosis • adult height • ketoconazole • precocious puberty




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