HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Services Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Ladjouze, A. Articles by Léger, J. Search for Related Content PubMed PubMed Citation Articles by Ladjouze, A. Articles by Léger, J.

GH deficiency with central precocious puberty: a new rare disorder associated with a developmental defect of the hypothalamic–pituitary area

Pediatric Endocrinology Department, Reference Center for Rare Endocrine Growth Diseases, Assistance Publique-Hôpitaux de Paris, Robert Debré Hospital, Paris VII University, Paris, France, ¹ Pediatrics Department of Hautepierre Hospital, Strasbourg, France, ² Radiology Department, Robert Debré Hospital Paris, Paris, France, ³ Pediatrics Department of Troyes Hospital, Troyes, France, ⁴ Pediatrics Department of Lorient Hospital, Lorient, France and ⁵ Pediatric Endocrinology Department, Necker Enfants Malades Hospital, Paris, France

(Correspondence should be addressed to J Léger; Email: juliane.leger{at}rdb.aphp.fr)

Context: GH deficiency (GHD) associated with central precocious puberty (CPP) has been widely reported in cases of arachnoid cyst, septo-optic dysplasia, brain tumors, or after cerebral radiation therapy. However, idiopathic GHD associated with CPP has been reported in only one isolated case.

Objective: To evaluate the occurrence and clinical features of the association of nonacquired GHD and CPP.

Design and setting: This was a retrospective multicenter study.

Patients and methods: The study population was identified through a French nationwide multicenter network (about 3000 patients). We reviewed the medical records of all subjects diagnosed with nonacquired GHD and CPP, with or without developmental abnormalities of the hypothalamic–pituitary axis on cerebral magnetic resonance imaging (MRI), and without any known associated anomaly.

Results: We identified four patients with either isolated GHD (n = 1) or multiple anterior pituitary hormone deficiencies (n = 3). Clinical signs of CPP occurred at 6.4 ± 2.3 years in boys and 7.5 ± 0.5 years in girls, and GnRH analog therapy was started at 4.2 ± 1.6 years after the initiation of recombinant human GH treatment. Cerebral MRI demonstrated ectopic neurohypophysis associated with anterior pituitary hypoplasia in three out of the four patients. The morphology and position of the anterior pituitary and neurohypophysis were normal in one patient who displayed a persistence of the craniopharyngeal canal.

Conclusions: CPP is very rare in patients with nonacquired GHD and is mostly associated with developmental defects in the hypothalamic–pituitary area. Whether molecular mechanisms governing development and activation of the hypothalamic–pituitary axis share dependent factors remains to be explored.