title: Gonadotrophic status in adolescents with pituitary stalk interruption syndrome.
abstract: 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.
authors: Pediatric Endocrinology, Children-Adult Endocrinology Department, Groupe hospitalier Cochin-Saint Vincent de Paul, Paris, France.
authors: Diane
authors: D
authors: Rottembourg
authors: Agnès
authors: A
authors: Linglart
authors: Catherine
authors: C
authors: Adamsbaum
authors: Najiba
authors: N
authors: Lahlou
authors: Cécile
authors: C
authors: Teinturier
authors: Pierre
authors: P
authors: Bougnères
authors: Jean-Claude
authors: JC
authors: Carel
mesh_terms: D000293:Adolescent; D041923:Adolescent Development / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D003456:Cryptorchidism / Q000097:blood / Q000209:etiology / Q000503:physiopathology; D005260:Female; D006062:Gonadotropins / Q000097:blood*; D006130:Growth Disorders / Q000097:blood / Q000209:etiology; D019382:Human Growth Hormone / Q000172:deficiency*; D006801:Humans; D007018:Hypopituitarism / Q000097:blood* / Q000150:complications / Q000473:pathology; D007223:Infant; D008297:Male; D010902:Pituitary Gland / Q000473:pathology; D011627:Puberty / Q000097:blood / Q000502:physiology; D012189:Retrospective Studies; D013577:Syndrome
chemical_list: D006062:Gonadotropins; D019382:Human Growth Hormone