4.1 Article

Work-up and management of paediatric Cushing's syndrome

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LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MED.0b013e328305082f

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Cushing's disease; Cushing's syndrome; paediatrics; pituitary radiotherapy; pituitary surgery

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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.

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