Document Detail

Gonadotrophic status in adolescents with pituitary stalk interruption syndrome.
MedLine Citation:
PMID:  18088398     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Pituitary stalk interruption syndrome (PSIS) is a frequent cause of GH deficiency (GHD) and is commonly associated with other PH deficiencies (PHDs). Although previous reports have correlated multiple PHDs with severe anatomical lesions, the status of the gonadotrophic axis has not yet been thoroughly analysed. METHODS: We retrospectively reviewed the medical records of 27 patients (15 males, 12 females) with GHD and PSIS defined by MRI findings. The status of the gonadotrophic axis was evaluated in children who were at least 14.5 years (boys) or 13 years (girls). RESULTS: Out of 27 patients, five displayed spontaneous full pubertal development with normal hormonal values at the final evaluation, whereas 22 of 27 patients (81%) had complete (n = 18) or partial pubertal deficiency. Three girls had primary amenorrhoea with normal gonadotrophin values, raising the possibility of subtle disturbances of gonadotrophin pulsatility. Of the 21 patients with TSH or ACTH deficiency, 17 (81%) had complete gonadotrophin deficiency. Two of our six patients with apparently isolated GHD during childhood had gonadotrophin deficiency. Cryptorchidism was present at birth in six boys (40%). Of these six boys, one had normal pubertal development. Ten of 11 boys with micropenis at birth had gonadotrophin deficiency. CONCLUSIONS: Gonadotrophin deficiency is a common finding in adolescents with PSIS and is frequently associated with other PHDs. However its severity is variable, ranging from complete gonadotrophin deficiency to normogonadotrophic amenorrhoea. The occurrence of gonadotrophin deficiency in 33% of children with apparently isolated GHD and PSIS has important implications for the counselling and follow-up of these patients.
Diane Rottembourg; Agnès Linglart; Catherine Adamsbaum; Najiba Lahlou; Cécile Teinturier; Pierre Bougnères; Jean-Claude Carel
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Publication Detail:
Type:  Journal Article     Date:  2008-07-01
Journal Detail:
Title:  Clinical endocrinology     Volume:  69     ISSN:  1365-2265     ISO Abbreviation:  Clin. Endocrinol. (Oxf)     Publication Date:  2008 Jul 
Date Detail:
Created Date:  2008-07-07     Completed Date:  2009-07-23     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0346653     Medline TA:  Clin Endocrinol (Oxf)     Country:  England    
Other Details:
Languages:  eng     Pagination:  105-11     Citation Subset:  IM    
Pediatric Endocrinology, Children-Adult Endocrinology Department, Groupe hospitalier Cochin-Saint Vincent de Paul, Paris, France.
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MeSH Terms
Adolescent Development / physiology
Child, Preschool
Cryptorchidism / blood,  etiology,  physiopathology
Gonadotropins / blood*
Growth Disorders / blood,  etiology
Human Growth Hormone / deficiency*
Hypopituitarism / blood*,  complications,  pathology
Pituitary Gland / pathology
Puberty / blood,  physiology
Retrospective Studies
Reg. No./Substance:
0/Gonadotropins; 12629-01-5/Human Growth Hormone

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