ACTH-Independent Macronodular Adrenocortical Hyperplasia (AIMAH): Report of Two Cases and the Analysis of Steroidogenic Activity in Adrenal Nodules.

Morioka, Motohiro; Ohashi, Yozo; Watanabe, Hironobu; Komatsu, Fumito; Jin, Tie-Xiong; Suyama, Bunzo; Tanaka, Hiroyoshi

doi:10.1507/endocrj.44.65

Cited by 30 publications

(26 citation statements)

References 14 publications

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“…We know steroidogenesis is inefficient in patients with AIMAH, since they have huge adrenal glands, with combined weights usually higher than 100 g, while their cortisol secretion is modest, so that they usually have mildly elevated urinary free cortisol and mild and long-standing symptoms. Morioka et al found the cortisol content in adrenal tissue from patients with AIMAH, measured by high performance liquid chromatography (HPLC), was definitely lower than that in cortisol-producing adenomas and even in normal adrenal glands (14). The activities of cytochrome P450c17, P450c21 and P450c11 were evaluated in one case, and all of them were reduced in the macronodules (14).…”

Section: Discussionmentioning

confidence: 98%

“…Morioka et al found the cortisol content in adrenal tissue from patients with AIMAH, measured by high performance liquid chromatography (HPLC), was definitely lower than that in cortisol-producing adenomas and even in normal adrenal glands (14). The activities of cytochrome P450c17, P450c21 and P450c11 were evaluated in one case, and all of them were reduced in the macronodules (14). Another possible explanation was given by in situ hybridization studies, which demonstrated that steroidogenic enzymes are differentially localized to the small and large cells that can be found in AIMAH adrenal tissue (15,16).…”

Section: Discussionmentioning

confidence: 99%

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Is unilateral adrenalectomy an alternative treatment for ACTH-independent macronodular adrenal hyperplasia?: Long-term follow-up of four cases

Lamas

Alfaro

Lucas

et al. 2002

European Journal of Endocrinology

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Objective: ACTH-independent macronodular adrenal hyperplasia is a rare cause of Cushing's syndrome. Bilateral adrenalectomy is considered the treatment of choice, but the patient is obliged to receive lifetime steroid replacement therapy and is susceptible to adrenal insufficiency crisis. New therapeutic alternatives are being proposed as new etiopathological features of the disease are known. Unilateral adrenalectomy of the largest gland can be a safe and effective alternative, but only short-term follow-up is reported in the literature. We present four consecutive patients with ACTH-independent macronodular hyperplasia and long-term remission of Cushing's syndrome after unilateral adrenalectomy. Subjects: Four consecutive patients (two males and two females, mean age 50.3 years) with Cushing's syndrome due to ACTH-independent macronodular adrenal hyperplasia underwent unilateral adrenalectomy of the largest gland. Results: The weight of the resected glands ranged from 26.8 to 210 g. Two patients suffered transient post-surgical adrenal insufficiency and had steroid replacement therapy for 60 and 14 months respectively. After a mean follow-up of 78.8 months (range 30-137 months) all the patients persist without any evidence of Cushing's syndrome. Urinary free cortisol and serum cortisol, after the adrenal insufficiency stage, have always stayed within their normal ranges, but cortisol circadian rhythm and suppressibility after dexamethasone have never normalized. No further enlargement of the contralateral gland has been documented 62 to 126 months after surgery in three of the four patients. Conclusions: Unilateral adrenalectomy can be an effective and safe alternative treatment for ACTH-independent macronodular adrenal hyperplasia, and can achieve long-term remission of Cushing's syndrome.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Is unilateral adrenalectomy an alternative treatment for ACTH-independent macronodular adrenal hyperplasia?: Long-term follow-up of four cases

Lamas

Alfaro

Lucas

et al. 2002

European Journal of Endocrinology

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“…Despite bilaterally enlarged glands, the degree of hypercortisolism is occasionally mild in subjects with AIMAH [8,9]. Subjects with mild hypercortisolism, such as preclinical Cushing's syndrome or adrenal incidentalomas, often display insulin resistance and glucose intolerance as their cardinal symptoms [10][11][12][13].…”

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confidence: 99%

Unilateral Adrenalectomy Improves Insulin Resistance and Diabetes Mellitus in a Patient with ACTH-Independent Macronodular Adrenal Hyperplasia

et al. 2003

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Abstract. ACTH-independent macronodular adrenal hyperplasia (AIMAH) is a rare cause of Cushing's syndrome. Bilateral adrenalectomy is considered to be a standard therapy for AIMAH, although lifetime replacement of glucocorticoids is necessary after the procedure. This paper describes a subject with AIMAH who underwent unilateral adrenalectomy of the predominantly enlarged gland and subsequently displayed an improvement in insulin resistance and diabetes mellitus, the cardinal symptoms before the operation, concomitant with alleviation of abnormal cortisol secretion. The patient was a 61-year-old man with a body mass index of 25.6 kg/m 2 . He was diagnosed as having diabetes mellitus, hypertension, and hyperlipidemia at 50 years of age. Eight years after diagnosis, bilateral enlargement of the adrenal glands was revealed by chance upon computed tomography of the abdomen. Typical manifestations of Cushing's syndrome were not demonstrated. Basal levels of serum and urinary cortisol had not increased, although the serum cortisol level displayed no circadian rhythm and no response to the administration of dexamethasone. Despite sulfonylurea treatment, the patient's HbA 1C level was as high as 7.6% (normal range 4.3-5.8%). Fasting insulin concentration was increased to 42.6 mU/ml, and the homeostasis model insulin resistance index (HOMA-R) was calculated to be 15.5 (with a normal range of less than 2.5), indicating severe insulin resistance. Unilateral adrenalectomy of the predominantly enlarged gland revealed that the resected gland consisted of multiple nodules of various sizes. Based on endocrinological, radiological, and pathological findings, a diagnosis of AIMAH was made. Ten months after the unilateral adrenalectomy, cortisol circadian rhythms were restored, and serum cortisol concentration was suppressed in response to the administration of low doses of dexamethasone, suggesting an improvement in the cortisol secretory pattern. Levels of HbA 1C , fasting insulin, and HOMA-R decreased to 5.7%, 12.7mU/ml, and 2.2, respectively. An improvement in hyperlipidemia was also observed. Insulin resistance and glucose intolerance are recognized as features of mild hypercortisolism. In the present case, unilateral adrenalectomy was effective in ameliorating insulin resistance and improving glycemic control. Unilateral adrenalectomy might be an alternative therapy for improvement of glucose and lipid metabolism in subjects with AIMAH.

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“…The microsomal and mitochondrial fractions were obtained by differential centrifugation [6]. The assay of P450 activities was performed according to the methods reported by Suzuki et al [3,4] and by ourselves [7]. In a reconstituted enzyme system including 100 ,umol/ l DOC as a substrate, 14 pumol/l bovine adrenodoxin, 0.3 pumol/l NADPH-adrenodoxin reductase as an electron transport system, 5 mmol/l isocitrate, 0.5 units/ml isocitrate dehydrogenase, and 5 mmol/l MgC12 as an NADPH-generating system were dissolved in 100 mmol/l potassium phosphate buffer.…”

Section: Evaluation Of Steroidogenic Activitiesmentioning

confidence: 99%

“…The reaction products were extracted with dichloromethane and analyzed by the same HPLC system as described above [5,7]. The statistical analysis was performed with Student's t-test.…”

Section: Evaluation Of Steroidogenic Activitiesmentioning

confidence: 99%

The Analysis of Steroidogenic Activity in Non-Hyperfunctioning Adrenocortical Adenoma.

et al. 1997

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Abstract. The steroidogenic activities in non-hyperfunctioning adrenal adenoma (NHFA) have not yet been fully examined. Steroid content both in adenoma and its non-tumorous adjacent adrenal tissue was measured by the HPLC system in 8 cases of NHFA and 2 cases of preclinical Cushing's syndrome (PCS). Activities of cytochrome P450c17ct, P450c21, P450c11 fl, P450c18 and aldosterone-synthesizing enzyme (P450aldo) were also determined by the enzyme reconstitution system. Steroid content in NHFA varied but no significant difference was seen between NHFA and normal control adrenals. Activities of all P450s except P450aldo were confirmed in NHFA, but they were almost equivalent to those in normal control adrenals. As no significant difference between NHFA and normal controls was observed in either steroid contents or P450s activities, the steroidogenic activity of NHFA is considered to be comparable to that of normal adrenals.

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ACTH-Independent Macronodular Adrenocortical Hyperplasia (AIMAH): Report of Two Cases and the Analysis of Steroidogenic Activity in Adrenal Nodules.

Cited by 30 publications

References 14 publications

Is unilateral adrenalectomy an alternative treatment for ACTH-independent macronodular adrenal hyperplasia?: Long-term follow-up of four cases

Is unilateral adrenalectomy an alternative treatment for ACTH-independent macronodular adrenal hyperplasia?: Long-term follow-up of four cases

Unilateral Adrenalectomy Improves Insulin Resistance and Diabetes Mellitus in a Patient with ACTH-Independent Macronodular Adrenal Hyperplasia

The Analysis of Steroidogenic Activity in Non-Hyperfunctioning Adrenocortical Adenoma.

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