Diagnosis and Treatment > Investigation > Adrenal venous sampling

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Takuya Higashitani Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

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Shigehiro Karashima Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

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Daisuke Aono Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

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Seigoh Konishi Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan
Department of Internal Medicine, Keiju Medical Center, Nanao, Ishikawa, Japan

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Mitsuhiro Kometani Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

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Rie Oka Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

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Masashi Demura Department of Hygiene, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

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Kenji Furukawa Health Care Center, Japan Advanced Institute of Science and Technology, Nomi, Ishikawa, Japan

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Yuto Yamazaki Department of Pathology, Tohoku University Hospital, Sendai, Miyagi, Japan

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Hironobu Sasano Department of Pathology, Tohoku University Hospital, Sendai, Miyagi, Japan

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Takashi Yoneda Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan
Department of Health Promotion and Medicine of the Future, Kanazawa University, Kanazawa, Ishikawa, Japan

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Yoshiyu Takeda Division of Endocrinology and Hypertension, Department of Cardiovascular and Internal Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Ishikawa, Japan

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Summary

Renovascular hypertension (RVHT) is an important and potentially treatable form of resistant hypertension. Hypercortisolemia could also cause hypertension and diabetes mellitus. We experienced a case wherein adrenalectomy markedly improved blood pressure and plasma glucose levels in a patient with RVHT and low-level autonomous cortisol secretion. A 62-year-old Japanese man had been treated for hypertension and diabetes mellitus for 10 years. He was hospitalized because of a disturbance in consciousness. His blood pressure (BP) was 236/118 mmHg, pulse rate was 132 beats/min, and plasma glucose level was 712 mg/dL. Abdominal CT scanning revealed the presence of bilateral adrenal masses and left atrophic kidney. Abdominal magnetic resonance angiography demonstrated marked stenosis of the left main renal artery. The patient was subsequently diagnosed with atherosclerotic RVHT with left renal artery stenosis. His left adrenal lobular mass was over 40 mm and it was clinically suspected the potential for cortisol overproduction. Therefore, laparoscopic left nephrectomy and adrenalectomy were simultaneously performed, resulting in improved BP and glucose levels. Pathological studies revealed the presence of multiple cortisol-producing adrenal nodules and aldosterone-producing cell clusters in the adjacent left adrenal cortex. In the present case, the activated renin-angiotensin-aldosterone system and cortisol overproduction resulted in severe hypertension, which was managed with simultaneous unilateral nephrectomy and adrenalectomy.

Learning points:

  • Concomitant activation of the renin-angiotensin-aldosterone system and cortisol overproduction may contribute to the development of severe hypertension and lead to lethal cardiovascular complications.

  • Treatment with simultaneous unilateral nephrectomy and adrenalectomy markedly improves BP and blood glucose levels.

  • CYP11B2 immunohistochemistry staining revealed the existence of aldosterone-producing cell clusters (APCCs) in the adjacent non-nodular adrenal gland, suggesting that APCCs may contribute to aldosterone overproduction in patients with RVHT.

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Yasufumi Seki Departments of Endocrinology and Hypertension, Tokyo, Japan

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Satoshi Morimoto Departments of Endocrinology and Hypertension, Tokyo, Japan

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Naohiro Yoshida Departments of Endocrinology and Hypertension, Tokyo, Japan

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Kanako Bokuda Departments of Endocrinology and Hypertension, Tokyo, Japan

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Nobukazu Sasaki Departments of Endocrinology and Hypertension, Tokyo, Japan

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Midori Yatabe Departments of Endocrinology and Hypertension, Tokyo, Japan

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Junichi Yatabe Departments of Endocrinology and Hypertension, Tokyo, Japan

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Daisuke Watanabe Departments of Endocrinology and Hypertension, Tokyo, Japan

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Satoru Morita Departments of Diagnostic Imaging and Nuclear Medicine, Tokyo, Japan

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Keisuke Hata Departments of Urology, Kidney Center, Tokyo, Japan

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Tomoko Yamamoto Departments of Surgical Pathology, Tokyo Women’s Medical University, Tokyo, Japan

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Yoji Nagashima Departments of Surgical Pathology, Tokyo Women’s Medical University, Tokyo, Japan

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Atsuhiro Ichihara Departments of Endocrinology and Hypertension, Tokyo, Japan

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Summary

Primary aldosteronism (PA) is more common than expected. Aberrant adrenal expression of luteinizing hormone (LH) receptor in patients with PA has been reported; however, its physiological role on the development of PA is still unknown. Herein, we report two unique cases of PA in patients with untreated Klinefelter’s syndrome, characterized as increased serum LH, suggesting a possible contribution of the syndrome to PA development. Case 1 was a 39-year-old man with obesity and hypertension since his 20s. His plasma aldosterone concentration (PAC) and renin activity (PRA) were 220 pg/mL and 0.4 ng/mL/h, respectively. He was diagnosed as having bilateral PA by confirmatory tests and adrenal venous sampling (AVS). Klinefelter’s syndrome was suspected as he showed gynecomastia and small testes, and it was confirmed on the basis of a low serum total testosterone level (57.3 ng/dL), high serum LH level (50.9 mIU/mL), and chromosome analysis. Case 2 was a 28-year-old man who had untreated Klinefelter’s syndrome diagnosed in his childhood and a 2-year history of hypertension and hypokalemia. PAC and PRA were 247 pg/mL and 0.3 ng/mL/h, respectively. He was diagnosed as having a 10 mm-sized aldosterone-producing adenoma (APA) by AVS. In the APA, immunohistochemical analysis showed co-expression of LH receptor and CYP11B2. Our cases of untreated Klinefelter’s syndrome complicated with PA suggest that increased serum LH levels and adipose tissues, caused by primary hypogonadism, could contribute to PA development. The possible complication of PA in hypertensive patients with Klinefelter’s syndrome should be carefully considered.

Learning points:

  • The pathogenesis of primary aldosteronism is still unclear.

  • Expression of luteinizing hormone receptor has been reported in aldosterone-producing adenoma.

  • Serum luteinizing hormone, which is increased in patients with Klinefelter’s syndrome, might contribute to the development of primary aldosteronism.

Open access
Katta Sai Department of Internal Medicine, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA

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Amos Lal Department of Internal Medicine, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA

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Jhansi Lakshmi Maradana Department of Internal Medicine, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA

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Pruthvi Raj Velamala Department of Internal Medicine, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA

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Trivedi Nitin Department of Endocrinology, Diabetes, and Metabolism, Saint Vincent Hospital at Worcester Medical Center, Worcester, Massachusetts, USA

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Summary

Mifepristone is a promising option for the management of hypercortisolism associated with hyperglycemia. However, its use may result in serious electrolyte imbalances, especially during dose escalation. In our patient with adrenocorticotropic hormone-independent macro-nodular adrenal hyperplasia, unilateral adrenalectomy resulted in biochemical and clinical improvement, but subclinical hypercortisolism persisted following adrenalectomy. She was started on mifepristone. Unfortunately, she missed her follow-up appointments following dosage escalation and required hospitalization at an intensive care level for severe refractory hypokalemia.

Learning points:

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G Leksic Division of Endocrinology and Diabetes, Department of Internal Medicine

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A M Alduk Department of Radiology, University Hospital Centre Zagreb, Zagreb, Croatia

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V Molnar Division of Endocrinology and Diabetes, Department of Internal Medicine

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A Haxhiu Division of Endocrinology and Diabetes, Department of Internal Medicine

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A Balasko Division of Endocrinology and Diabetes, Department of Internal Medicine

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N Knezevic Department of Urology, Department of Internal Medicine, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia

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T Dusek Division of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia

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D Kastelan Division of Endocrinology and Diabetes, Department of Internal Medicine, University Hospital Centre Zagreb, School of Medicine, University of Zagreb, Zagreb, Croatia

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Summary

Primary aldosteronism (PA) is characterised by aldosterone hypersecretion and represents a common cause of secondary hypertension. During diagnostic evaluation, it is essential to determine the aetiology of PA since the treatment of unilateral and bilateral disease differs significantly. Adrenal vein sampling (AVS) has been implemented as a gold standard test for the diagnosis of PA subtype. However, due to the AVS complexity, costs and limited availability, many patients with PA are being treated based on the computed tomography (CT) findings. In this article, we present two patients with discrepant CT and AVS results, demonstrating that AVS is the only reliable method for localising the source of aldosterone excess.

Learning points:

  • CT is an unreliable method for distinguishing aldosterone-producing adenoma (APA) from bilateral adrenal hyperplasia (BAH).

  • CT can be misleading in defining lateralisation of the aldosterone excess in case of unilateral disease (APA).

  • AVS is the gold standard test for defining the PA subtype.

Open access
M J Trott Radcliffe Department of Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK

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G Farah Radcliffe Department of Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK

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V J Stokes Radcliffe Department of Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK

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L M Wang Radcliffe Department of Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK

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A B Grossman Radcliffe Department of Medicine, Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK

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Summary

We present a case of a young female patient with a rare cause of relapsing and remitting Cushing’s syndrome due to ectopic ACTH secretion from a thymic neuroendocrine tumour. A 34-year-old female presented with a constellation of symptoms of Cushing’s syndrome, including facial swelling, muscle weakness and cognitive impairment. We use the terms ‘relapsing and remitting’ in this case report, given the unpredictable time course of symptoms, which led to a delay of 2 years before the correct diagnosis of hypercortisolaemia. Diagnostic workup confirmed ectopic ACTH secretion, and a thymic mass was seen on mediastinal imaging. The patient subsequently underwent thymectomy with complete resolution of her symptoms. Several case series have documented the association of Cushing’s syndrome with thymic neuroendocrine tumours (NETs), although to our knowledge there are a few published cases of patients with relapsing and remitting symptoms. This case is also notable for the absence of features of the MEN-1 syndrome, along with the female gender of our patient and her history of non-smoking.

Learning points

  • Ectopic corticotrophin (ACTH) secretion should always be considered in the diagnostic workup of young patients with Cushing’s syndrome

  • There is a small but growing body of literature describing the correlation between ectopic ACTH secretion and thymic neuroendocrine tumours (NETs)

  • The possibility of a MEN-1 syndrome should be considered in all patients with thymic NETs, and we note the observational association with male gender and cigarette smoking in this cohort

  • An exception to these associations is the finding of relatively high incidence of thymic NETs among female non-smoking MEN-1 patients in the Japanese compared with Western populations

  • The relapsing and remitting course of our patient’s symptoms is noteworthy, given the paucity of this finding among other published cases

Open access
Ya-Wun Guo Department of Medicine, Taipei City Hospital, Zhongxing Branch, Taipei, Taiwan
Department of Medicine, Division of Endocrinology and Metabolism, Taipei Veterans General Hospital, Taipei, Taiwan

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Chii-Min Hwu Department of Medicine, Division of Endocrinology and Metabolism, Taipei Veterans General Hospital, Taipei, Taiwan
Faculty of Medicine, National Yang-Ming University School of Medicine, Taipei, Taiwan

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Justin Ging-Shing Won Department of Medicine, Division of Endocrinology and Metabolism, Taipei Veterans General Hospital, Taipei, Taiwan
Faculty of Medicine, National Yang-Ming University School of Medicine, Taipei, Taiwan

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Chia-Huei Chu Faculty of Medicine, National Yang-Ming University School of Medicine, Taipei, Taiwan
Department of Otorhinolaryngology-Head and Neck Surgery, Division of Otology, Taipei Veterans General Hospital, Taipei, Taiwan

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Liang-Yu Lin Department of Medicine, Division of Endocrinology and Metabolism, Taipei Veterans General Hospital, Taipei, Taiwan
Faculty of Medicine, National Yang-Ming University School of Medicine, Taipei, Taiwan

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Summary

A functional lesion in corticotrophin (ACTH)-independent Cushing’s syndrome is difficult to distinguish from lesions of bilateral adrenal masses. Methods for distinguishing these lesions include adrenal venous sampling and 131I-6β-iodomethyl-19-norcholesterol (131I-NP-59) scintigraphy. We present a case of a 29-year-old Han Chinese female patient with a history of hypercholesterolaemia and polycystic ovary syndrome. She presented with a 6month history of an 8kg body weight gain and gradual rounding of the face. Serial examinations revealed loss of circadian rhythm of cortisol, elevated urinary free-cortisol level and undetectable ACTH level (<5pg/mL). No suppression was observed in both the low- and high-dose dexamethasone suppression tests. Adrenal computed tomography revealed bilateral adrenal masses. Adrenal venous sampling was performed, and the right-to-left lateralisation ratio was 14.29. The finding from adrenal scintigraphy with NP-59 was consistent with right adrenal adenoma. The patient underwent laparoscopic right adrenalectomy, and the pathology report showed adrenocortical adenoma. Her postoperative cortisol level was 3.2μg/dL, and her Cushingoid appearance improved. In sum, both adrenal venous sampling and 131I-NP-59 scintigraphy are good diagnostic methods for Cushing’s syndrome presenting with bilateral adrenal masses.

Learning points

  • The clinical presentation of Cushing’ syndrome includes symptoms and signs of fat redistribution and protein-wasting features.

  • The diagnosis of patients with ACTH-independent Cushing’s syndrome with bilateral adrenal masses is challenging for localisation of the lesion.

  • Both adrenal venous sampling and 131I-NP-59 scintigraphy are good methods to use in these patients with Cushing’s syndrome presenting with bilateral adrenal masses.

Open access
Rémi Goupil Endocrine Hypertension Research Centre, University of Queensland School of Medicine, Greenslopes and Princess Alexandra Hospitals, Ipswich Road, Woolloongabba, Brisbane, Queensland, 4102, Australia
Hôpital du Sacré-Coeur de Montréal, University of Montreal, Montreal, Quebec, H4J 1C5, Canada

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Martin Wolley Endocrine Hypertension Research Centre, University of Queensland School of Medicine, Greenslopes and Princess Alexandra Hospitals, Ipswich Road, Woolloongabba, Brisbane, Queensland, 4102, Australia

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Jacobus Ungerer Department of Chemical Pathology, Pathology Queensland, Brisbane, Queensland, 4001, Australia

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Brett McWhinney Department of Chemical Pathology, Pathology Queensland, Brisbane, Queensland, 4001, Australia

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Kuniaki Mukai Department of Biochemistry, Medical Education Center, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan

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Mitsuhide Naruse Department of Endocrinology, Metabolism and Hypertension, National Hospital Organization Kyoto Medical Center, Kyoto, Japan

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Richard D Gordon Endocrine Hypertension Research Centre, University of Queensland School of Medicine, Greenslopes and Princess Alexandra Hospitals, Ipswich Road, Woolloongabba, Brisbane, Queensland, 4102, Australia

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Michael Stowasser Endocrine Hypertension Research Centre, University of Queensland School of Medicine, Greenslopes and Princess Alexandra Hospitals, Ipswich Road, Woolloongabba, Brisbane, Queensland, 4102, Australia

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Summary

In patients with primary aldosteronism (PA) undergoing adrenal venous sampling (AVS), cortisol levels are measured to assess lateralization of aldosterone overproduction. Concomitant adrenal autonomous cortisol and aldosterone secretion therefore have the potential to confound AVS results. We describe a case where metanephrine was measured during AVS to successfully circumvent this problem. A 55-year-old hypertensive male had raised plasma aldosterone/renin ratios and PA confirmed by fludrocortisone suppression testing. Failure of plasma cortisol to suppress overnight following dexamethasone and persistently suppressed corticotrophin were consistent with adrenal hypercortisolism. On AVS, comparison of adrenal and peripheral A/F ratios (left 5.7 vs peripheral 1.0; right 1.7 vs peripheral 1.1) suggested bilateral aldosterone production, with the left gland dominant but without contralateral suppression. However, using aldosterone/metanephrine ratios (left 9.7 vs peripheral 2.4; right 1.3 vs peripheral 2.5), aldosterone production lateralized to the left with good contralateral suppression. The patient underwent left laparoscopic adrenalectomy with peri-operative glucocorticoid supplementation to prevent adrenal insufficiency. Pathological examination revealed adrenal cortical adenomas producing both cortisol and aldosterone within a background of aldosterone-producing cell clusters. Hypertension improved and cured of PA and hypercortisolism were confirmed by negative post-operative fludrocortisone suppression and overnight 1 mg dexamethasone suppression testing. Routine dexamethasone suppression testing in patients with PA permits detection of concurrent hypercortisolism which can confound AVS results and cause unilateral PA to be misdiagnosed as bilateral with patients thereby denied potentially curative surgical treatment. In such patients, measurement of plasma metanephrine during AVS may overcome this issue.

Learning points

  • Simultaneous autonomous overproduction of cortisol and aldosterone is increasingly recognised although still apparently uncommon.

  • Because cortisol levels are used during AVS to correct for differences in dilution of adrenal with non-adrenal venous blood when assessing for lateralisation, unilateral cortisol overproduction with contralateral suppression could confound the interpretation of AVS results

  • Measuring plasma metanephrine during AVS to calculate lateralisation ratios may circumvent this problem.

Open access
V Larouche Resident, Internal Medicine Residency Training Program, Department of Medicine, McGill University, Montreal, Quebec, Canada

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L Snell Division of General Internal Medicine, McGill University Health Centre, Montreal, Quebec, H4A 3J1, Canada

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D V Morris Division of Endocrinology, McGill University Health Centre, Montreal, Quebec, H4A 3J1, Canada

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Summary

Myxoedema madness was first described as a consequence of severe hypothyroidism in 1949. Most cases were secondary to long-standing untreated primary hypothyroidism. We present the first reported case of iatrogenic myxoedema madness following radioactive iodine ablation for Graves' disease, with a second concurrent diagnosis of primary hyperaldosteronism. A 29-year-old woman presented with severe hypothyroidism, a 1-week history of psychotic behaviour and paranoid delusions 3 months after treatment with radioactive iodine ablation for Graves' disease. Her psychiatric symptoms abated with levothyroxine replacement. She was concurrently found to be hypertensive and hypokalemic. Primary hyperaldosteronism from bilateral adrenal hyperplasia was diagnosed. This case report serves as a reminder that myxoedema madness can be a complication of acute hypothyroidism following radioactive iodine ablation of Graves' disease and that primary hyperaldosteronism may be associated with autoimmune hyperthyroidism.

Learning points

  • Psychosis (myxoedema madness) can present as a neuropsychiatric manifestation of acute hypothyroidism following radioactive iodine ablation of Graves' disease.

  • Primary hyperaldosteronism may be caused by idiopathic bilateral adrenal hyperplasia even in the presence of an adrenal adenoma seen on imaging.

  • Adrenal vein sampling is a useful tool for differentiating between a unilateral aldosterone-producing adenoma, which is managed surgically, and an idiopathic bilateral adrenal hyperplasia, which is managed medically.

  • The management of autoimmune hyperthyroidism, iatrogenic hypothyroidism and primary hyperaldosteronism from bilateral idiopathic adrenal hyperplasia in patients planning pregnancy includes delaying pregnancy 6 months following radioactive iodine treatment and until patient is euthyroid for 3 months, using amiloride as opposed to spironolactone, controlling blood pressure with agents safe in pregnancy such as nifedipine and avoiding β blockers.

  • Autoimmune hyperthyroidism and primary hyperaldosteronism rarely coexist; any underlying mechanism associating the two is still unclear.

Open access
Gautam Das Department of Diabetes and Endocrinology, Prince Charles Hospital, Cwm Taf University Health Board, Merthyr Tydfil, Mid Glamorgan, CF47 9DT, UK

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Peter N Taylor Department of Diabetes and Endocrinology, Prince Charles Hospital, Cwm Taf University Health Board, Merthyr Tydfil, Mid Glamorgan, CF47 9DT, UK

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Arshiya Tabasum Department of Diabetes and Endocrinology, Prince Charles Hospital, Cwm Taf University Health Board, Merthyr Tydfil, Mid Glamorgan, CF47 9DT, UK

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L N Rao Bondugulapati Department of Diabetes and Endocrinology, Maelor Hospital, Betsi Cadwaldr University Health Board, Wrexham, LL13 7TD, UK

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Danny Parker Department of Histopathology, University Hospital of Wales, Cardiff and Vale University Health Board, Cardiff, CF14 4XW, UK

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Piero Baglioni Department of Diabetes and Endocrinology, Prince Charles Hospital, Cwm Taf University Health Board, Merthyr Tydfil, Mid Glamorgan, CF47 9DT, UK

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Onyebuchi E Okosieme Department of Diabetes and Endocrinology, Prince Charles Hospital, Cwm Taf University Health Board, Merthyr Tydfil, Mid Glamorgan, CF47 9DT, UK

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David Scott Coombes Department of Endocrine Surgery, University Hospital of Wales, Cardiff and Vale University Health Board, Cardiff, CF14 4XW, UK

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Summary

Resistant hypertension is often difficult to treat and may be associated with underlying primary aldosteronism (PA). We describe the case of an elderly gentleman who presented with severe and resistant hypertension and was found to have a left adrenal incidentaloma during evaluation but had aldosterone excess secondary to unilateral adrenal hyperplasia (UAH) of the contralateral gland, which needed surgical intervention. A 65-year-old gentleman was evaluated for uncontrolled high blood pressure (BP) in spite of taking four antihypertensive medications. The high BP was confirmed on a 24-h ambulatory reading, and further biochemical evaluation showed an elevated serum aldosterone renin ratio (ARR) (1577 pmol/l per ng per ml per h). Radiological evaluation showed an adrenal nodule (15 mm) in the left adrenal gland but an adrenal vein sampling demonstrated a lateralization towards the opposite site favouring the right adrenal to be the source of excess aldosterone. A laparoscopic right adrenalectomy was performed and the histology of the gland confirmed nodular hyperplasia. Following surgery, the patient's BP improved remarkably although he remained on antihypertensives and under regular endocrine follow-up. PA remains the most common form of secondary and difficult-to-treat hypertension. Investigations may reveal incidental adrenal lesions, which may not be the actual source of excess aldosterone, but UAH may be a contributor and may coexist and amenable to surgical treatment. An adrenal vein sampling should be undertaken for correct lateralization of the source, otherwise a correctable diagnosis may be missed and the incorrect adrenal gland may be removed.

Learning points

  • Severe and resistant hypertension can often be associated with underlying PA.

  • ARR is an excellent screening tool in patients with suspected PA.

  • Lateralization with adrenal venous sampling is essential to isolate the source and differentiate between unilateral and bilateral causes of hyperaldosteronism.

  • Adrenal incidentalomas and UAH may coexist and the latter may often be the sole cause of excess aldosterone secretion.

  • Decisions about adrenalectomy should be made only after integrating and interpreting radiological and biochemical test findings properly.

Open access
S Hussain Department of Endocrinology, St Bartholomew's Hospital, London, UK

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E Panteliou Department of Endocrinology, St Bartholomew's Hospital, London, UK

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D M Berney Department of Pathology, St Bartholomew's Hospital, London, UK

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R Carpenter Department of Surgery, St Bartholomew's Hospital, London, UK

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M Matson Department of Radiology, St Bartholomew's Hospital, London, UK

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A Sahdev Department of Radiology, St Bartholomew's Hospital, London, UK

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M Bell Department of Endocrinology, Galway University Hospital, Galway, UK

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E O'Sullivan Department of Endocrinology, Galway University Hospital, Galway, UK

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W M Drake Department of Endocrinology, St Bartholomew's Hospital, London, UK

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Summary

We describe a young male patient with longstanding hypertension, who was diagnosed with primary hyperaldosteronism and treated by an attempted retroperitoneoscopic total unilateral adrenalectomy for a left-sided presumed aldosterone-secreting adenoma. Imaging had shown an unremarkable focal adrenal lesion with normal contralateral adrenal morphology, and histology of the resected specimen showed no adverse features. Post-operatively, his blood pressure and serum aldosterone levels fell to the normal range, but 9 months later, his hypertension recurred, primary aldosteronism was again confirmed and he was referred to our centre. Repeat imaging demonstrated an irregular left-sided adrenal lesion with normal contralateral gland appearances. Adrenal venous sampling was performed, which supported unilateral (left-sided) aldosterone hypersecretion. Redo surgery via a laparoscopically assisted transperitoneal approach was performed and multiple nodules were noted extending into the retroperitoneum. It was thought unlikely that complete resection had been achieved. His blood pressure returned to normal post-operatively, although hypokalaemia persisted. Histological examination, from this second operation, showed features of an adrenocortical carcinoma (ACC; including increased mitoses and invasion of fat) that was assessed as malignant using the scoring systems of Weiss and Aubert. Biochemical hyperaldosteronism persisted post-operatively, and detailed urine steroid profiling showed no evidence of adrenal steroid precursors or other mineralocorticoid production. He received flank radiotherapy to the left adrenal bed and continues to receive adjunctive mitotane therapy for a diagnosis of a pure aldosterone-secreting ACC.

Learning points

  • Pure aldosterone-secreting ACCs are exceptionally uncommon, but should be considered in the differential diagnosis of patients presenting with primary aldosteronism.

  • Aldosterone-producing ACCs may not necessarily show typical radiological features consistent with malignancy.

  • Patients who undergo surgical treatment for primary aldosteronism should have follow-up measurements of blood pressure to monitor for disease recurrence, even if post-operative normotension is thought to indicate a surgical ‘cure’.

  • Owing to the rarity of such conditions, a greater understanding of their natural history is likely to come from wider cooperation with, and contribution to, large multi-centre outcomes databases.

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