1/136. A case of aldosterone-producing adenoma with severe postoperative hyperkalemia.It is known that some patients with primary aldosteronism show postoperative hyperkalemia, which is due to inability of the adrenal gland to secrete sufficient amounts of aldosterone. However, hyperkalemia is generally neither severe nor prolonged, in which replacement therapy with mineralocorticoid is seldom necessary. We report a case of a 46-year-old woman with an aldosterone-producing adenoma associated with severe postoperative hyperkalemia. After unilateral adrenalectomy, the patient showed episodes of severe hyperkalemia for four months, which required not only cation-exchange resin, but also mineralocorticoid replacement. plasma aldosterone concentration (PAC) was low, although PAC was increased after rapid ACTH test. Histological examination indicated the presence of adrenocortical tumor and paradoxical hyperplasia of zona glomerulosa in the adjacent adrenal. immunohistochemistry demonstrated that the enzymes involved in aldosterone synthesis, such as cholesterol side chain cleavage (P-450scc), 3beta-hydroxysteroid dehydrogenase (3beta-HSD), and 21-hydroxylase (P-450c21), or the enzyme involved in glucocorticoid synthesis, 11beta-hydroxylase (P-450c11beta), were expressed in the tumor, but they were completely absent in zona glomerulosa of the adjacent adrenal. These findings were consistent with the patterns of primary aldosteronism. serum potassium level was gradually decreased with concomitant increase in PAC. These results suggest that severe postoperative hyperkalemia of the present case was attributable to severe suppression of aldosterone synthesis in the adjacent and contralateral adrenal, which resulted in slow recovery of aldosterone secretion. It is plausible that aldosterone synthesis of adjacent and contralateral adrenal glands is severely impaired in some cases with primary aldosteronism, as glucocorticoid synthesis in cushing syndrome.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/136. Hazards of laparoscopic adrenalectomy for Conn's adenoma. When enthusiasm turns to tragedy.A 74-year-old man with primary aldosteronism had a small tumor (27 x 23 mm) of his right adrenal gland successfully removed by a transperitoneal laparoscopy. Despite absence of malignancy in the resected tumor and complete relief of all symptoms in the immediate postoperative period, recurrence occurred 6 months later. The tumor behaved as a carcinoma spread in the peritoneal cavity, and the patient eventually died with peritoneal carcinomatosis. We suggest that the laparoscopic technique coupled with pneumoperitoneum may have favored this recurrence.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
3/136. A probable relationship between an endogenous digitalis-like substance and concentric cardiac hypertrophy in primary aldosteronism.A 44-year-old woman was admitted to our hospital due to severe hypertension. An electrocardiogram (ECG) and an echocardiogram showed severe left ventricular hypertrophy. Her plasma aldosterone level was elevated. magnetic resonance imaging revealed a small mass in the right adrenal gland. Before removal of the tumor, plasma endogenous digitalis-like substance (EDLS) levels were elevated. After removal of the tumor, EDLS levels quickly returned to the normal level. A series of echocardiograms and ECGs over a 6- year period after removal of the tumor showed marked regression of cardiac hypertrophy. These findings suggest that EDLS may be closely related to the development of concentric cardiac hypertrophy in primary aldosteronism.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
4/136. Primary aldosteronism caused by aldosterone-producing adenoma in pregnancy--complicated by EPH gestosis.pregnancy in conjunction with primary aldosteronism is an unusual occurrence. We report a 28-year-old woman who presented with mild hypertension and hypokalemia as manifestations of primary aldosteronism caused by an aldosterone-producing adenoma in the left adrenal gland during pregnancy. Although the diagnosis was straightforward, the patient refused to undergo the proposed operation during the second trimester of her pregnancy. She was not admitted to hospital until she developed EPH gestosis in the 27th week of gestation, which had an unfavourable outcome for the infant who died nine days after delivery. The patient underwent a laparoscopic adrenalectomy which resulted in normalization of blood pressure and blood potassium levels. In cases of aldosterone-producing adenoma, surgery in the second trimester is the most appropriate option to avoid a poor obstetric outcome.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
5/136. Primary aldosteronism due to unilateral adrenal hyperplasia: report of two cases and review of the literature.Unilateral adrenal hyperplasia (UAH) is a rare, surgically correctable subset of primary aldosteronism (PA), which shows similar endocrine features to aldosterone-producing adenoma (APA). We report here two Japanese patients with UAH. Case 1 was a 62-year-old man with a four-year history of hypertension. hypokalemia and suppressed plasma renin activity (PRA) with elevated plasma aldosterone concentration (PAC) were observed, while no adrenal nodules were identified by abdominal computed tomographic (CT) scan. Adrenal scintigraphy did not reveal definite localization. The selective adrenal-vein sampling for determinations of PAC showed an over-functioning left adrenal gland, and a left adrenalectomy was performed. Diffuse micronodular adrenocortical hyperplasia was observed. Case 2 was a 61-year-old man with a six-year history of hypertension. At the first visit to our hospital, hypokalemia and suppressed PRA with elevated PAC were observed. An abdominal CT scan showed a left adrenal mass 1.5 cm in diameter, while adrenal scintigraphy did not reveal definite laterality. A left adrenalectomy was performed, and three macronodules and diffuse micronodular adrenocortical hyperplasia were observed. hypokalemia, hypertension and endocrine data became normal, and both patients have been well with no signs of recurrence for eight years (case 1) and seven months (case 2) after surgery. Clinical characteristics and endocrine features of UAH are also reviewed.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
6/136. A case of unilateral adrenal hyperplasia: the diagnostic dilemma of hyperaldosteronism.OBJECTIVE: To report the successful laparoscopic surgical management of a case of right unilateral adrenal hyperplasia. methods: We present a case of unilateral adrenal hyperplasia and review the pertinent literature. The effectiveness of surgical treatment of unilateral adrenal hyperplasia is evaluated. RESULTS: A 50-year-old man had a 7-year history of hypertension, which had been treated with antihypertensive medications. On initial assessment, he was taking long-acting diltiazem, 240 mg twice a day, and his blood pressure was 150/84 mm Hg. He was noted to have recurrent hypokalemia, low plasma renin activity, increased plasma aldosterone concentration, and high urinary aldosterone levels. magnetic resonance imaging of the adrenal glands revealed a normal left adrenal gland and fullness of the right adrenal gland. Selective adrenal vein catheterization showed findings consistent with a right-sided adrenal gradient. Surgical removal of the right adrenal gland laparoscopically alleviated his symptoms and cured his hypertension and hypokalemia. Pathologic examination of the right adrenal gland revealed diffuse hyperplasia of the zona fasciculata and zona reticularis. plasma aldosterone, renin, and potassium levels returned to normal postoperatively, and his blood pressure declined to 120/70 mm Hg. At 1-year follow-up, the patient remained normotensive without the need for antihypertensive agents. CONCLUSIONS: This rare type of hyperaldosteronism, known as unilateral adrenal hyperplasia, is difficult to categorize not only because it fails to typify a conventional subtype but also because it is difficult to confirm the pathologic diagnosis. Recognition of the variety of provocative, biochemical, and imaging results that may be seen in this disorder is important in making the correct diagnosis. Determining unilaterality of disease by selective adrenal vein sampling can result in a cure of hypertension and hypokalemia by surgical resection.- - - - - - - - - - ranking = 2.5keywords = gland (Clic here for more details about this article) |
7/136. A chimeric CYP11B1/CYP11B2 gene in glucocorticoid-insuppressible familial hyperaldosteronism.Although a chimeric gene combining the 11beta-hydroxylase gene (CYP11B1) and the aldosterone synthase gene (CYP11B2) explains the pathophysiology of familial hyperaldosteronism (FH) type I, the contribution of this abnormality to FH type II has not been tested. We screened genomic dna from a Japanese family with FH type II for the CYP11B1/CYP11B2 gene. The index patient was a 27-year-old woman with hypertension. Hypokalaemia, elevated plasma aldosterone and suppressed plasma renin activity suggested primary aldosteronism. Though computed tomography failed to reveal an adrenal tumour, left adrenalectomy was indicated due to a high aldosterone concentration in left adrenal venous blood. The resected adrenal gland contained an adenoma. As her mother had also been diagnosed with primary aldosteronism due to an adenoma, we administered oral dexamethasone to our patient before the operation and observed the response of the blood pressure and plasma aldosterone concentration for 2 weeks. Both parameters remained elevated during the treatment period, confirming the diagnosis of FH type II. Total dna was isolated from blood cells of the index patient, her mother, and an unaffected brother. Samples were amplified by polymerase chain reaction using specific primers from CYP11B1 and CYP11B2. Unique dna fragments of 1.4 kb were obtained from the index patient and her mother, but not from the healthy subject. The CYP11B1/CYP11B2 chimeric gene was found in a Japanese family with FH type II.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
8/136. Combined primary aldosteronism and preclinical Cushing's syndrome: an unusual case presentation of adrenal adenoma.A 55-year-old woman was referred to our institution for evaluation of elevated plasma creatine phosphokinase, hypokalemia and hypertension. Her chief complaints were muscle weakness and polyuria. A left adrenal mass, 4 cm in diameter, was noted on computed tomography. Hormonal assessment demonstrated markedly elevated plasma aldosterone concentration, markedly low plasma renin activity, an abnormal diurnal variation in serum cortisol levels, suppressed baseline plasma adrenocorticotrophic hormone, and non-suppression of serum cortisol by dexamethasone suppression test. She showed no symptoms or signs suggestive of Cushing's syndrome. Adrenal scintigraphy with 131I-6-beta-iodomethyl-norcholesterol showed uptake on the left adrenal and inhibition of the contralateral adrenal gland. She was diagnosed with combined primary aldosteronism and preclinical Cushing's syndrome. Cases of combined primary aldosteronism and preclinical Cushing's syndrome are extremely rare. In patients with large aldosterone-producing adenoma, contralateral adrenal insufficiency should be anticipated after the removal of the tumor.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
9/136. diagnosis and management of primary aldosteronism in pregnancy: case report and review of the literature.Primary aldosteronism rarely complicates pregnancy. We present a woman with primary aldosteronism in pregnancy associated with severe preeclampsia. A 33-year-old Japanese woman with hypertension was referred to our hospital at 25 weeks of gestation. Her blood pressure was 180/100 mmHg, and laboratory tests identified a low serum potassium level and moderate proteinuria on urinalysis. The fetus was diagnosed with growth restriction. plasma renin activity (PRA) value was 2.2 ng/mL/h and plasma aldosterone concentration (PAC) was elevated (260 pg/mL). The patient was treated medically. At 27 weeks of gestation, we noted persistent late fetal heart rate decelerations associated with uterine contractions. Therefore, elective caesarean section was performed and she was delivered of a 698-g female. After delivery, PRA declined and PAC remained elevated. Abdominal computerized tomography scan and I131-iodochoresterol scan revealed a tumor in the left adrenal gland. Laparoscopic adrenalectomy was performed and confirmed the clinical diagnosis.- - - - - - - - - - ranking = 0.5keywords = gland (Clic here for more details about this article) |
10/136. Unique cases of unilateral hyperaldosteronemia due to multiple adrenocortical micronodules, which can only be detected by selective adrenal venous sampling.Primary aldosteronism is classified as aldosterone-producing adenoma (APA), idiopathic hyperaldosteronism (IHA), unilateral adrenal hyperplasia (UAH), primary adrenal hyperplasia (PAH), adrenal cancer, and glucocorticoid-remediable aldosteronism. We describe here 4 cases of primary aldosteronism due to unilateral hyperaldosteronemia, demonstrating unique histopathologic findings, such as unilateral multiple adrenocortical micronodules in the affected adrenals. Thirty-three patients with primary aldosteronism were consecutively admitted; 27 of them were treated by unilateral adrenalectomy. Four of them also had unilateral adrenal hypersecretion of aldosterone by selective adrenal venous sampling and adrenocortical multiple micronodules without an adenoma. These patients had hyporeninemic hyperaldosteronism with normokalemic hypertension. In these patients, furosemide plus upright test failed to increase plasma renin activity (PRA); the ratio of plasma aldosterone concentration (PAC) to PRA at 90 minutes after captopril administration was similar to that in patients with IHA and APA. Aldosterone concentrations were increased in each unilateral adrenal vein, and poorly encapsulated multiple adrenocortical micronodules from 2 to 3 mm in diameter were microscopically detected in the resected adrenal glands. Immunohistochemical analysis of steroidogenic enzymes, including cholesterol side chain cleavage, 3beta-hydroxysteroid dehydrogenase, 21-hydroxylase, 17alpha-hydroxylase, and 11beta-hydroxylase, indicated that the cortical cells within these micronodules were active in aldosterone production, while the non-nodular zona glomerulosa cells were inactive. We conclude that the clinical and pathologic characteristics of our 4 cases with unilateral multiple adrenocortical micronodules (UMN) are distinct from those of APA, IHA, UAH, and PAH. Furthermore, unilateral hyperaldosteronemia induced by UMN may be frequently misdiagnosed, because standard imaging tests, which cannot always detect tiny abnormalities of adrenals, showed "normal adrenal glands" in these patients. Thus, primary aldosteronism due to UMN should be carefully examined for differential diagnosis of each form of hyperaldosteronemia.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
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