Work-up and management of paediatric Cushing's syndrome.
OBJECTIVE
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.


RESULTS
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.


CONCLUSIONS
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.
Savage
Martin O
MO
Department of Endocrinology, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, London, UK. m.o.savage@qmul.ac.uk
Chan
Li F
LF
Grossman
Ashley B
AB
Storr
Helen L
HL
D000182:ACTH Syndrome, Ectopic / Q000473:pathology; D000306:Adrenal Cortex Neoplasms / Q000473:pathology; D000311:Adrenal Glands / Q000528:radiation effects; D001827:Body Height; D015992:Body Mass Index; D002648:Child; D002675:Child, Preschool; D003480:Cushing Syndrome / Q000145:classification / Q000175:diagnosis* / Q000628:therapy*; D005359:Fibrous Dysplasia, Polyostotic / Q000473:pathology; D006801:Humans; D017591:Petrosal Sinus Sampling; D047748:Pituitary ACTH Hypersecretion / Q000473:pathology; D010902:Pituitary Gland / Q000528:radiation effects; D011627:Puberty