Primary hyperparathyroidism associated with primary hyperaldosteronism

Ferriss, J. B.; Brown, J. J.; Cumming, A. M. M.; Fraser, Robert; Lever, A.F.; Peacock, Munro; Robertson, J. I. S.

doi:10.1530/acta.0.1030365

Cited by 24 publications

(15 citation statements)

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“…In our patients, the adrenal abnormalities were unilateral in 79%, which is in agreement with the 55%-83% reported in 2 other studies 85,235 but different from the results in another study of MEN1 patients 403 that reported that adrenal disease, when present, was bilateral in 60% of MEN1 cases. Although pheochromocytomas 59,71,214,235,503 , adrenal Cushing syndrome 59,235,403,454 , and primary hyperaldosteronism 25,143,178 have been reported in MEN1 patients, our study demonstrates these functional syndromes due to adrenal disease are uncommon, occurring in only 6% of the patients with adrenal disease (2/48 [4%] for adrenal Cushing; 1/48 [2%] for pheochromocytoma). In contrast to patients with sporadic ZES who develop Cushing syndrome, it is reported that in patients with MEN1/ZES 265 , the Cushing syndrome is almost entirely of pituitary origin, whereas in the sporadic ZES cases it is generally due to ectopic ACTH production by the gastrinoma 265 .…”

Section: Discussionmentioning

confidence: 55%

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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In patients with multiple endocrine neoplasia type 1 (MEN1), the most common functional pancreatic endocrine tumor (PET) syndrome is Zollinger-Ellison syndrome (ZES). ZES has been well studied in its sporadic form (that is, without MEN1); however, there are limited data on patients with MEN1 and ZES (MEN1/ZES), and the long-term natural history is largely unknown. To address this issue we report the results of a prospective long-term National Institutes of Health (NIH) study of 107 MEN1/ZES patients and compare our results with those of 1009 MEN1/ZES patients in 278 case reports and small series in the literature. Patients were clinically, radiologically, and biochemically evaluated yearly for all MEN1 manifestations (mean follow-up, 10 yr; range, 0.1-31 yr). Compared with patients from the literature, the NIH MEN1/ZES patients more frequently had pituitary (60%) and adrenal (45%) disease and carcinoid tumors (30%), but had equal frequency of hyperparathyroidism (94%), thyroid disease (6%), or lipomas (5%). Twenty-five percent of both the NIH and the literature patients lacked a family history of MEN1; ZES was the initial clinical manifestation of MEN1 in 40%. ZES onset preceded the diagnosis of hyperparathyroidism in 45%. However, ZES was rarely (8%) the only initial manifestation of MEN1 if careful testing was done. ZES occurred before age 40 years in 50%-60% of the current patients, in contrast to older studies. The diagnosis of ZES is delayed 3-5 years from its onset and is delayed as long as in sporadic ZES cases. Pituitary disease and carcinoid tumors (gastric > bronchial, thymic) are more frequent than generally reported, whereas a second functional PET is uncommon. In patients with MEN1/ZES without a family history of MEN1, the MEN1 manifestations are not as severe. This study shows that MEN1/ZES patients differ in many aspects from those commonly reported in older studies involving few MEN1/ZES patients. In this study we have identified a number of important clinical and laboratory features of MEN1/ZES that were not previously appreciated, which should contribute to earlier diagnosis and improve both short- and long-term management.

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Section: Discussionmentioning

confidence: 55%

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

et al. 2004

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“…These early compensatory responses, however, may not be adequate to preserve plasma ionized Ca 2ϩ concentrations during prolonged Aldost, as we noted herein, and this would represent a continuous stimulus to increased PTH secretion. As such, hyperparathyroidism has been observed in association with primary aldosteronism (14). Improvements in serum ionized Ca 2ϩ and circulating PTH levels occur after adrenal surgery in primary aldosteronism (28,29).…”

Section: Resultsmentioning

confidence: 99%

Loss of bone minerals and strength in rats with aldosteronism

Chhokar¹,

Sun²,

Bhattacharya³

et al. 2004

American Journal of Physiology-Heart and Circulatory Physiology

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gestive heart failure (CHF) is a clinical syndrome with origins rooted in a salt-avid state largely mediated by effector hormones of the circulating renin-angiotensin-aldosterone system. Other participating neurohormones include catecholamines, endothelin-1, and arginine vasopressin. CHF is accompanied by a systemic illness of uncertain causality. Features include the appearance of oxidative/nitrosative stress and a wasting of tissues including bone. Herein we hypothesized that inappropriate (relative to dietary Na ϩ ) elevations in plasma aldosterone (Aldo) contribute to an altered redox state, augmented excretion of divalent cations, and in turn, a loss of bone minerals and strength. In uninephrectomized rats that received chronic Aldo and 1% NaCl treatment for 4 -6 wk, we monitored plasma ␣1-antiproteinase activity, which is an inverse correlate of oxidative/nitrosative stress; plasma concentrations of ionized Mg 2ϩ and Ca 2ϩ ; urinary Mg 2ϩ and Ca 2ϩ excretion; and bone mineral composition and strength to flexure stress. Compared with controls, we found reductions in plasma ␣1-antiproteinase activity and ionized Mg 2ϩ and Ca 2ϩ together with persistently elevated urinary Mg 2ϩ and Ca 2ϩ excretion, a progressive loss of bone mineral density and content with reduced Mg 2ϩ and Ca 2ϩ concentrations, and a reduction in cortical bone strength. Thus the hypermagnesuria and hypercalciuria that accompany chronic Aldo-1% NaCl treatment contribute to the systemic appearance of oxidative/nitrosative stress and a wasting of bone minerals and strength. aldosterone; congestive heart failure; peripheral blood mononuclear cells; antiproteinase; parathyroid hormone CONGESTIVE HEART FAILURE (CHF), a clinical syndrome with characteristic signs and symptoms, arises from circulating renin-angiotensin-aldosterone system activation (38) and is accompanied by a systemic illness that includes oxidative/ nitrosative stress in the heart, systemic organs, circulating immune cells, and blood (10,20,34,37) and a catabolic state with wasting of lean tissue, fat, and bone (2, 3, 22, 32). Aldosteronism in rats, which is defined as inappropriate (relative to dietary Na ϩ ) and chronic elevations in plasma aldosterone (Aldo) comparable to those seen in CHF, ultimately leads to a proinflammatory coronary vascular phenotype with evidence of oxidative/nitrosative stress (e.g., 3-nitrotyrosine labeling) in invading inflammatory cells (36). These vascular lesions are preceded by an immunostimulatory state that involves activated peripheral blood mononuclear cells (PBMCs) and is induced by Ca 2ϩ loading and transduced by reactive oxygen species in these cells (1, 16). Herein we hypothesized that enhanced urinary excretion of Mg 2ϩ and Ca 2ϩ accompanies chronic Aldo with 1% NaCl treatment (Aldost) and leads to yet another component of this illness, namely, bone wasting. MATERIALS AND METHODSMale 8-wk-old Sprague-Dawley rats (Harlan, IN) were used in this study, which was approved by the institution's Animal Care and Use Committee. Unoperated and untr...

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“…However, in previous studies, pheochromocytoma as part of the MEN1 syndrome has been reported (ranging from 0 to 3%) (4,6). Three cases of aldosterone-secreting adrenal tumours as a possible part of the MEN1 syndrome have also been documented in the literature (16)(17)(18)(19). Therefore, the MEN1 syndrome may include the whole spectrum of adrenocortical and medullary pathology, but most of the adrenal lesions are non-functional.…”

Section: Discussionmentioning

confidence: 99%

Natural course of small adrenal lesions in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study

Schaefer

Shipotko

Meyer

et al. 2008

European Journal of Endocrinology

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Objective: Adrenal lesion is one of the features of multiple endocrine neoplasia type 1 (MEN1). This study aimed to assess prevalence, natural course and clinical relevance of small adrenal lesions without clinical symptoms, endocrine activity, or mechanical problems and thus without clear indication for surgical therapy by endoscopic ultrasound (EUS). Design and methods: Forty-nine patients with familial MEN1 were studied. Twenty-seven of these with adrenal lesions were detected by EUS and at least two performed EUS examinations were included into a subgroup where changes in adrenal morphology were studied by measuring changes in the largest diameter of the dominant adrenal tumour. Results: EUS detected adrenal lesions in 36 (73%) patients: 6 (12%) plump adrenals, 17 (35%) nodular hyperplasia, 12 (24%) adenomas and 1 (2%) cyst. Bilateral adrenal lesions were detected in 17 patients and unilateral in 19 patients. A change in the largest tumour diameter was found to be for nodular hyperplasia K0.02G1.41% per month (range K2.56 to 4.58%) and for adenomas K0.61G1.95% per month (range K6.25 to 1.15%). One patient had an adrenal cyst with significant growth. There was no evidence of carcinoma or metastatic disease during the study. Conclusions: The prevalence of adrenal lesions in MEN1 is higher than that reported earlier. Except one cystic lesion, no significant change in the tumour size was observed over a mean observation period of more than 2 years. In a typical situation, small adrenal lesions in MEN1 seem to be constant in their morphology.European Journal of Endocrinology 158 699-704

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Primary hyperparathyroidism associated with primary hyperaldosteronism

Cited by 24 publications

References 0 publications

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

Multiple Endocrine Neoplasia Type 1 and Zollinger-Ellison Syndrome

Loss of bone minerals and strength in rats with aldosteronism

Natural course of small adrenal lesions in multiple endocrine neoplasia type 1: an endoscopic ultrasound imaging study

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