Endocrine Abstracts

Introduction: Paediatric Cushing’s disease is rare and up to 50% have incomplete cure.¹ Besides hypopituitarism growth failure is particularly common and maybe due to GH deficiency, target tissue resistance² or accelerated skeletal maturation ³. Our patient is an 18-year-old obese male who first presented at the age of 16 with delayed puberty and short stature.

Results: His 24 hr urinary cortisol was 1472 nmol/l, ACTH 56 nmol/l, low & high dose dexamethasone suppression test reduced the early morning cortisol from 1023 to 288 and from 575 to 25 respectively and the rest of his hormone profile was normal, His fasting blood sugar was 10.9, HbA1c 12% and bone age was 16 years. Visual fields were normal and MRI showed a right-sided pituitary macro adenoma and normal adrenals. Oral hypoglycemics were started and he underwent a trans-sphenoidal hypophysectomy with an uneventful recovery and pathology showed an ACTH – expressing pituitary adenoma. Pituitary testing showed (Table 1):

Conclusions: Paediatric Cushing’s can be missed for years and the overall prognosis is not as good as adult Cushing’s. Management is challenging where the feasibility and timing of treatment can be very difficult. Post operative dynamic pituitary testing can sometimes be essential to unmask underlying hormone deficiencies.

1. Leinung et al. 1995 JCEM; 80:2475–2479

2. Magiakou MA et al. 1994 NEJM; 331:629–636

3. Hayes AB et al. 1966 Pediatrics; 37:19–25