The role of bilateral adrenalectomy in the treatment of XXXX .

@entity1 @entity4414 @entity28 @entity391 @entity1902 @entity4053 @entity134 @entity342 @entity221 @entity774
This report summarizes follow-up studies in 18 @entity1 who underwent bilateral adrenalectomy for @entity774 . Three of these @entity1 were young @entity1 with null/null mutations of @entity4414 , and the other 15 were adrenalectomized because of difficulties in their management on conventional therapy. The average duration of follow-up was 59 months and represents an aggregate of 90 postoperative years. The adrenals were removed laparoscopically in 13 @entity1 and by open flank incisions in five. Adrenal crises associated with severe illnesses occurred in five @entity1 at times when their glucocorticoid substitution was suboptimal. All were responsive to appropriate therapy. Two of these @entity1 were young @entity1 who had @entity4053 during @entity1902 or @entity221 associated with poor @entity391 . Significant elevations of adrenal @entity134 precursors, presumably from ectopic adrenal rests, were observed postoperatively in eight of the @entity1 . @entity1 and parents were nearly unanimous in their enthusiasm for adrenalectomy. In most, signs of @entity342 excess have decreased, and @entity28 has become less of a problem with lowering the dose of glucocorticoid. We conclude that adrenalectomy is a safe and efficacious method of managing @entity774 in selected @entity1 . Prophylactic adrenalectomy in young @entity1 with double null mutations remains experimental.
