Investigation for Paediatric Cushing's Syndrome Using Twenty-Four-Hour Urinary Free Cortisol Determination

Horm Res Paediatr. 2016;86(1):21-6. doi: 10.1159/000446913. Epub 2016 Jun 10.

Abstract

Objective: Paediatric Cushing's syndrome (CS) remains a challenge to diagnose and exclude. We assessed the accuracy of 24-hour urinary free cortisol (UFC) determination in children referred for suspected CS.

Design: We conducted a retrospective study of paediatric patients referred to our centre with suspected CS between 1982 and 2014.

Patients: Of 66 subjects (mean age 12.9 years; range 4.4-16.9), there were 47 cases of CS (29 males), which included Cushing's disease (CD; 39 patients, 25 males), primary pigmented nodular adrenocortical disease (8 patients, 4 males) and 19 'controls' (6 males) in whom the diagnosis of CS was excluded.

Measurements: The subjects had between one and five 24-hour UFC collections analysed by radioimmunoassay, chemiluminescent immunoassay or liquid chromatography-mass spectrometry. The data were normalised, corrected for body surface area (m2) and assessed using receiver operating characteristic analysis and an independent two-tailed t test.

Results: The diagnostic accuracy of 24-hour UFC for CS was excellent (area under the curve 0.98, 95% CI 0.946-1.00, sensitivity 89%, specificity 100%).

Conclusions: Twenty-four-hour UFC is a reliable and practical investigation with high diagnostic accuracy for paediatric CS. However, further investigations may be required if the UFC is normal but there is a high diagnostic suspicion of CS.

Publication types

  • Clinical Trial

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Cushing Syndrome / diagnosis
  • Cushing Syndrome / urine*
  • Female
  • Humans
  • Hydrocortisone / urine*
  • Male
  • Retrospective Studies

Substances

  • Hydrocortisone