文本框: CUSHING’S DISEASE IN CHILDREN - DIAGNOSTIC PROCEDURES AND OUTCOME OF TRANSNASAL OPERATION 
Willig RP1, Luedecke DK2 
University of Hamburg, Germany
1Children’s Hospital and 2Neurosurgery

Cushing’s disease is caused by elevated ACTH- and cortisol blood levels due to a pituitary adenoma. 55 children and adolescents were reevaluated after transsphenoidal adenomectomy to find out best diagnostic criteria and operation outcome.
Preoperative (preop) and postoperative (postop) hormone levels (ACTH, cortisol) were compared in urine and in blood prior to and after dexametasone- and CRF-testing, respectively. Cortisol and ACTH was determined during transnasal operation.
Cortisol (preop)-levels of 310 + 28 μg/l decreased (postop) to 146 + 28 μg/l and ACTH decreased from 109 + 14 to 38 + 12 ng/l. Dexametasone given preoperatively could neither sufficiently suppress cortisol nor ACTH, nor elevated basal free cortisol in the urine. CRF-stimulated high blood cortisol and ACTH levels before operation differed significantly from postoperative levels. ACTH and cortisol decreased significantly during the operation. 
Transsphenoidal microadenectomy detected ACTH producing adenomas in 52 out of 55 children including one macroadenoma. In 2 cases no adenoma was found, in 1 case there was a choristoma. One case relapsed after successful operation, in 2 cases pituitary insufficiency occured. Transient diabetes insipidus was observed 11 times, long term diabetes insipidus developed 15 times.
It is concluded that 24 h-urine cortisol and ACTH blood-determination during CRF-testing is best to diagnose ACTH producing pituitary adenomas.Transsphenoidal microadenectomy is safe and powerful to ope- 
rate on children with Cushing’s disease by experienced neurosurgeons.
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