Searchable abstracts of presentations at key conferences in endocrinology
Endocrine Abstracts (2008) 17 P23

BSPED2008 Poster Presentations (1) (56 abstracts)

Prepubertal Cushing's disease: diagnosis and therapeutic outcome

A Kumaran 1 , LF Chan 1 , L Martin 1 , F Afshar 2 , M Matson 3 , PN Plowman 4 , JP Monsoon 1 , GM Besser 1 , AB Grossman 1 , MO Savage 1 & HL Storr 1


1Department of Endocrinology, Barts and the Royal London School of Medicine and Dentistry, London EC1A 7BE, UK; 2Department of Neurosurgery, Barts and the Royal London School of Medicine and Dentistry, London EC1A 7BE, UK; 3Department of Radiology, Barts and the Royal London School of Medicine and Dentistry, London EC1A 7BE, UK; 4Department of Radiotherapy, Barts and the Royal London School of Medicine and Dentistry, London EC1A 7BE, UK.


Cushing’s disease (CD) is very rare in prepubertal children, and remains a challenge to diagnose and manage. We review the diagnostic features and therapeutic outcome of prepubertal (defined as testicular volume <4 ml (M) and breast stage <2 (F)) CD patients treated in a single centre. Fifteen prepubertal patients (median age 9.4 years; range 5.8–13.7) fulfilled standard diagnostic criteria for CD and there was male preponderance (12 M (80%), median age 9.1 years; 3F (20%), median age 10.6 years). Serum cortisol failed to suppress to <50 nmol/l during LDDST (0.5 mg, 6 h or 30 μg/kg per day, for 48 h) in all patients. Suppression of serum cortisol to <50% of baseline during a HDDST (2 mg, 6 h or 120 μg/kg per day for 48 h) occurred in 85% (11/13) of patients. A CRH test (1 μg/kg i.v.) induced a ≥20% increase of serum cortisol from baseline in all patients (mean change 222%). Pituitary MR imaging revealed a macroadenoma in one patient and corticotroph microadenomas in 60% (9/15) of patients. The concordance of pituitary imaging with findings at transsphenoidal surgery (TSS) in the patients with microadenomas was poor (29%, 4/14). In 9 patients, bilateral simultaneous inferior petrosal sinus sampling (BSIPSS) was performed and there was lateralisation of ACTH (an inter-petrosal sinus gradient ≥1.4 after 100 μg i.v. CRH) in 89% (8/9). Concordance of BSIPSS with the findings at TSS occurred in 78% (7/9) patients. Post TSS, the corticotroph adenoma was confirmed histologically in 43% (6/14). Bilateral adrenalectomy was performed in one patient because of extreme illness with respiratory failure. TSS alone achieved cure (serum cortisol <50 nmol/l) in 43% (6/14) of patients. Of these, 83% (5/6) underwent BSIPSS and there was 100% concordance of the site of ACTH secretion with the surgical findings. The remaining patients underwent post-TSS pituitary irradiation (45 Gy) and 89% (8/9) are cured. BSIPSS improved the cure rate by TSS alone to 56% (5/9). GH deficiency was common (77%, 10/13) especially after pituitary irradiation (89%, 8/9). Characteristic features of prepubertal CD include: an exuberant response of serum cortisol after iv CRH which is often diagnostic and frequent absence of the adenoma on MR imaging. Pre-operative BSIPSS was strongly predictive of the site of the adenoma and leads to a good prognosis for cure by TSS.

Volume 17

36th meeting of the British Society for Paediatric Endocrinology and Diabetes

British Society for Paediatric Endocrinology and Diabetes 

Browse other volumes

Article tools

My recent searches

No recent searches.