title: Comparisons in the epidemiology, diagnostic features and cure rate by transsphenoidal surgery between paediatric and adult-onset Cushing's disease.
abstract: OBJECTIVE
  There are few published comparisons between paediatric and adult-onset Cushing's disease (CD). We compare the epidemiology, diagnostic features and cure rate by transsphenoidal surgery (TSS) in these groups.
  
  
  METHODS
  Retrospective review of patient databases in a single university hospital centre.
  
  
  METHODS
  Totally, 41 paediatric (mean age 12.3 ± 3.5 years; range 5.7-17.8) and 183 adult (mean age 40 ± 13 years; range 18.0-95.0) patients with CD were investigated.
  
  
  RESULTS
  Paediatric CD was characterised by male (63%) and adult CD by a female predominance (79%, P<0.0001). There were small but significant differences in clinical presentation. Biochemical features of CD were comparable except the serum cortisol increase during a CRH test: mean change (105%, n=39) in paediatric and (54%, n=123) in adult subjects (P<0.0001). Macroadenomas were more common in adult (15%, 28/183) than in paediatric (2%, 1/41, P=0.04) CD. Corticotroph microadenomas were more easily visualised by pituitary magnetic resonance imaging (MRI) in adult (76%, 50/66) compared with paediatric (55%, 21/38, P=0.045) CD with poorer concordance of imaging with surgical findings in children (P=0.058). The incidence of ACTH lateralisation by bilateral simultaneous inferior petrosal sinus sampling was comparable in paediatric (76%, 25/33) and adult (79%, 46/58; P=0.95) patients with good surgical concordance in both (82% paediatric and 79% adult). Cure rates by TSS were comparable, with a paediatric cure rate of 69%.
  
  
  CONCLUSIONS
  Several features of paediatric CD are distinct: increased frequency of prepubertal CD in males, the different clinical presentation, the decreased presence of macroadenomas and the frequent absence of radiological evidence of an adenoma on MRI.
authors.affiliation: Department of Paediatric Endocrinology, Barts and The London School of Medicine and Dentistry, London EC1A 7BE, UK. h.l.storr@qmul.ac.uk
authors.forename: H L
authors.initials: HL
authors.lastname: Storr
authors.forename: K I
authors.initials: KI
authors.lastname: Alexandraki
authors.forename: L
authors.initials: L
authors.lastname: Martin
authors.forename: A M
authors.initials: AM
authors.lastname: Isidori
authors.forename: G A
authors.initials: GA
authors.lastname: Kaltsas
authors.forename: J P
authors.initials: JP
authors.lastname: Monson
authors.forename: G M
authors.initials: GM
authors.lastname: Besser
authors.forename: M
authors.initials: M
authors.lastname: Matson
authors.forename: J
authors.initials: J
authors.lastname: Evanson
authors.forename: F
authors.initials: F
authors.lastname: Afshar
authors.forename: I
authors.initials: I
authors.lastname: Sabin
authors.forename: M O
authors.initials: MO
authors.lastname: Savage
authors.forename: A B
authors.initials: AB
authors.lastname: Grossman
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D047748:Pituitary ACTH Hypersecretion / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D012189:Retrospective Studies; D013101:Sphenoid Sinus / Q000473:pathology / Q000601:surgery*; D016896:Treatment Outcome; D055815:Young Adult