Treatment of Primary Pigmented Nodular Adrenocortical Disease
Horm Metab Res 2022; 54: 721-730 DOI: 10.1055/a-1948-6990Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of
adrenocorticotropin hormone (ACTH)-independent Cushing’s syndrome (CS),
which mainly occurs in children and young adults. Treatment options with proven
clinical efficacy for PPNAD include adrenalectomy (bilateral or unilateral
adrenalectomy) and drug treatment to control hypercortisolemia. Previously, the
main treatment of PPNAD is bilateral adrenal resection and long-term hormone
replacement after surgery. In recent years, cases reports suggest that
unilateral or subtotal adrenal resection can also lead to long-term remission in
some patients without the need for long-term hormone replacement therapy.
Medications for hypercortisolemia, such as Ketoconazole, Metyrapone and Mitotane
et.al, have been reported as a preoperative transition for in some patients with
severe hypercortisolism. In addition, tryptophan hydroxylase inhibitor, COX2
inhibitor Celecoxib, somatostatin and other drugs targeting the possible
pathogenic mechanisms of the disease are under study, which are expected to be
applied to the clinical treatment of PPNAD in the future. In this review, we
summarize the recent progress on treatment of PPNAD, in which options of
surgical methods, research results of drugs acting on ...
Source: Hormone and Metabolic Research - Category: Endocrinology Authors: Liu, Xinming Zhang, Siwen Guo, Yunran Gang, Xiaokun Wang, Guixia Tags: Review Source Type: research
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