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Work-up and management of paediatric Cushing's syndrome

Savage, Martin O; Chan, Li F; Grossman, Ashley B; Storr, Helen L

Current Opinion in Endocrinology, Diabetes & Obesity: August 2008 - Volume 15 - Issue 4 - p 346–351
doi: 10.1097/MED.0b013e328305082f
Neuroendocrinology: Edited by William H. Ludlam

Purpose of review: Paediatric Cushing's syndrome presents a diagnostic and therapeutic challenge. Most paediatric endocrinologists have limited experience in managing children or adolescents with Cushing's syndrome and thus benefit from close consultation with adult colleagues. A protocol for investigation of the child with suspected Cushing's syndrome is presented followed by principles of management.

Recent findings: Cushing's syndrome is rare in childhood, but causes serious morbidity. Investigations have evolved and now include new genetic and imaging techniques as well as classical endocrine studies. In Cushing's disease trans-sphenoidal surgery has transformed management, although only a few surgeons have experience in children. Pituitary radiotherapy is effective second-line therapy.

Summary: Early diagnosis and treatment of Cushing's syndrome is vital for long-term outcome. The overall prognosis for Cushing's syndrome is good but challenges remain to ensure normal postcure growth and body composition.

Department of Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, London, UK

Correspondence to Dr Martin Savage, Department of Endocrinology, Barts and the London School of Medicine and Dentistry, John Vane Science Centre, Charterhouse Square, London, EC1M 6BQ, UK Tel: +44 20 7882 6233; fax: +44 20 7882 6234; e-mail: m.o.savage@qmul.ac.uk

© 2008 Lippincott Williams & Wilkins, Inc.