| ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH) Associated With Insulinoma. | |
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MedLine Citation:
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PMID: 21324829 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Objective: To report a case of AIMAH associated with insulinomaMethods: We describe the clinical presentation of the case, discuss its management and review the current protocols for management of AIMAH and understanding of the aetiology of the disease.Results: A 64-year-old woman with multiple facial naevi experienced intermittent light headedness, tremor and confusion and was found to have a venous plasma glucose of 52 mg/dL (2.9 mmol/L). Hypoglycemia and hyperinsulinemia after 18 hours of fasting indicated the diagnosis of an insulinoma. Hepatic vein sampling for insulin after selective arterial calcium injection localized the insulinoma to the pancreatic head. The insulinoma was excised and has not recurred after more than three years. Bilateral adrenal masses were incidentally found on CT scan. The 24 hour urinary free cortisol value was elevated and serum ACTH suppressed. Overt signs of Cushing's syndrome were not considered to be present and subsequent urine cortisol measurements were within the normal range for several years. After 4 years, clinical and biochemical Cushing's syndrome was considered to have developed and bilateral adrenalectomy was performed. A histopathological diagnosis of AIMAH was made.Conclusion: This appears to be the first reported case of AIMAH associated with insulinoma. In the absence of other stigmata of multiple endocrine neoplasm type 1 (MEN-1) and in the presence of multiple naevi, it may represent a novel endocrinopathy syndrome. |
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Authors:
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Kevin T-K Lee; Richard D Arnott; Catriona A McLean; Jonathan W Serpell; Duncan J Topliss |
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Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-2-16 |
Journal Detail:
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Title: Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists Volume: - ISSN: 1934-2403 ISO Abbreviation: - Publication Date: 2011 Feb |
Date Detail:
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Created Date: 2011-2-17 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9607439 Medline TA: Endocr Pract Country: - |
Other Details:
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Languages: ENG Pagination: 1-16 Citation Subset: - |
Affiliation:
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Department of Endocrinology and Diabetes, The Alfred. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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