Affiliation:
1. Department of Endocrinology and Metabolism, The First Hospital of Jilin
University, Changchun, China
Abstract
AbstractPrimary 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 possible pathogenic
mechanisms, and the management during gestation are described in order to
provide new ideas for clinical treatment.
Funder
Key Laboratory of Science and Technology Department
Innovation Capability Project of Jilin Provincial Development and Reform Commission
Engineering Technology Research Center for Personalized Precision Diagnosis and Treatment, Science and Technology Department of Jilin Province
Subject
Biochemistry (medical),Clinical Biochemistry,Endocrinology,Biochemistry,General Medicine,Endocrinology, Diabetes and Metabolism
Reference89 articles.
1. Bilateral primary pigmented nodular adrenocortical disease. Rare cause of the
Cushing syndrome;B V Shenoy;Am J Surg Pathol,1984
2. Etiology and clinical profile of patients with Cushing’s syndrome: a
single center experience;A C Ammini;Indian J Endocrinol Metab,2014
3. Demographic characteristics, etiology, and comorbidities of patients with
Cushing’s syndrome: a 10-year retrospective study at a large general
hospital in China;J Zhou;Int J Endocrinol,2019
4. Adrenocortical tumors, primary pigmented adrenocortical disease
(PPNAD)/Carney complex, and other bilateral hyperplasias: the NIH
studies;C A Stratakis;Horm Metab Res,2007
5. The complex of myxomas, spotty pigmentation, and endocrine overactivity;J A Carney;Medicine,1985
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