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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary aldosteronism resulting from an adrenal adenoma is rare in children. An 8 1/2-year-old girl was found to have hypertension and spontaneous hypokalemia, both detected as incidental findings. Subsequent investigations revealed inappropriately elevated levels of plasma and urinary aldosterone with suppressed plasma renin activity. Adrenal vein blood sampling and venography suggested the presence of left adrenal adenoma which was confirmed at surgical exploration and pathologic examination. All the clinical and biochemical abnormalities were corrected by the adrenalectomy. The differential diagnoses of various types of hyperaldosteronism and/or hypokalemia in such a clinical setting are discussed.
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PMID:Childhood primary aldosteronism due to an adrenal adenoma: preoperative localization by adrenal vein catheterization. 736 May 50

The role of the autonomic nervous system in hypertension due to mineralocorticoid excess remains unclear. To address this issue, we performed power spectral analysis of blood pressure (BP) and RR interval oscillations in 20 patients with primary aldosteronism (PA), 54 patients with essential hypertension (EH) and 45 normotensive (NT) subjects. Blood pressure and the degree of organ damage were similar between PA and EH groups. Age did not differ between the three groups. The Mayer wave power spectrum (MWP) of BP (approx. 0.1 Hz), an index of sympathetic vasomotor tone, was smaller in patients with PA than in patients with EH either while subjects were supine (systolic/diastolic; 3.9 +/- 3.2 (SD)/1.5 +/- 1.3 vs. 5.5 +/- 4.2/2.1 +/- 1.6 mmHg2, P < 0.05 for both) or standing (7.6 +/- 6.6/3.0 +/- 3.0 vs. 17.7 +/- 23.7/7.2 +/- 8.3 mmHg2, P < 0.05 for both). Supine respiratory-related power spectrum (RRP) of the RR interval (approx. 0.25 Hz), an index of cardiac parasympathetic tone, was greater in patients with PA than in patients with EH (545 +/- 574 vs. 302 +/- 464 ms2, P < 0.01). The MWP of BP and the RRP of the RR interval were similar between patients with PA and NT subjects. Adrenalectomy reduced the 24-h mean BP (-18 mmHg for systolic BP, P < 0.001; -12 mmHg for diastolic BP, P < 0.01) and increased the 24-h mean heart rate (+8 bpm, P < 0.001). Furthermore, the diastolic MWP increased mildly (+32%, P < 0.05) and the RRP of the RR interval decreased dramatically (-75%, P < 0.01) following adrenalectomy. These results suggest that both vascular sympathetic and cardiac parasympathetic regulatory systems have minor roles in the maintenance of hypertension in patients with PA. The autonomic nervous system contributes more to the maintenance of BP following than prior to adrenalectomy. This information may be useful for the management of hypertension still persists after removal of adrenal adenoma.
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PMID:Normal sympathetic vasomotor and cardiac parasympathetic activities in patients with primary aldosteronism: assessment by spectral analysis. 761 99

A 46-year-old woman with rheumatoid arthritis had been on non-steroidal antiinflammatory agents for eighteen years until she developed cushingoid features and hypertension resistant to antihypertensive drugs. She had high plasma cortisol and 24 h urinary 17-hydroxycorticosteroids (17HCS) which were not suppressed by 8 mg dexamethasone per day for two days. The circadian rhythm of plasma cortisol was absent and plasma ACTH concentrations were suppressed before and after intravenous administration of CRH. Abdominal computed tomography demonstrated a tumor (3.0 x 3.0 x 2.3 cm) in the right adrenal gland and a 131I-6 beta-19-nor-methylcholesterol scan revealed marked uptake on the same side. The patient underwent a right adrenalectomy and the diagnosis of a cortisol secreting benign adenoma was histologically confirmed. Blood pressure declined and cushingoid features regressed, but three months after the operation and while the patient was on replacement, she complained of pain on motion, marked tenderness and swelling of fingers, wrists, elbows, knees and foot joints, and had very high rheumatoid factors. Treatment with immunosuppressive drugs and oral and intraarticular administration of glucocorticoids were necessary to relieve the clinical symptoms of rheumatoid arthritis. In summary, we report a patient with rheumatoid arthritis and Cushing's syndrome due to an adrenal adenoma, in whom rheumatoid arthritis was exacerbated after curing the Cushing's syndrome. This suggests that it is imperative to follow the development and/or course of autoimmune diseases after the treatment of Cushing's syndrome.
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PMID:Exacerbation of rheumatoid arthritis after removal of adrenal adenoma in Cushing's syndrome. 762 66

Cushing syndrome in pregnancy is rare. This is explained by the syndrome's association with amenorrhoea, oligomenorrhoea, infertility and abortions. Cushing syndrome commonly presents with hypertension, weight gain, diabetes, striae or truncal obesity, all of which can be consistent with pregnancy in women without Cushing syndrome. We describe a case of Cushing syndrome in pregnancy secondary to an adrenal cortical tumour which was discovered after an abnormal glucose tolerance test. The woman developed classical features of Cushing syndrome including gestational diabetes and hypertension and was managed successfully to term after a unilateral adrenalectomy at 23 weeks. The case is reported not only because of its rarity but also because the diagnosis was made after a routine screening test for gestational diabetes. Early diagnosis and treatment of adrenal adenoma causing Cushing syndrome in pregnancy reduces maternal and fetal morbidity and mortality.
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PMID:Cushing syndrome in pregnancy secondary to an adrenal cortical adenoma. 767 97

Conn's syndrome due to an adrenal adenoma is very rare in children. This paper reports a 14-year-old boy with primary hyperaldosteronism due to an adrenal adenoma. His biochemistry data were compatible with either bilateral adrenal hyperplasia or an adrenal adenoma. A dexamethasone test did not suppress aldosterone levels. Venous catheter sampling and 75Se-selenomethylcholesterol scanning suggested that the hyperaldosteronism originated at the right adrenal. Computed tomography showed an 8-mm low-density nodule in the right adrenal gland and magnetic resonance imaging confirmed the nodule which had high signal intensity on T2-weighted images consistent with a functioning adenoma. Surgery confirmed the right adrenal adenoma, and the patient was cured by right adrenalectomy. This case illustrates the difficulty of defining the aetiology of primary hyperaldosteronism and we review the biochemical and scanning techniques available to aid in diagnosis. Hypertension is unusual in children and endocrine causes are very rare, but Conn's syndrome should always be considered in the differential diagnosis.
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PMID:Primary hyperaldosteronism due to an adrenal adenoma in a 14-year-old boy. 772 19

An 8-year-old boy presented with precocious puberty and hypertension. He had hypokalemia, increased serum aldosterone and testosterone levels and low plasma renin activity. An adrenal adenoma was found using imaging methods and was removed. Postoperatively aldosterone, testosterone and blood pressure returned to normal. Renal ultrasonography findings were consistent with nephrocalcinosis, which might be explained by long lasting hypokalemic metabolic alkalosis and hypercalciuria. Precocious pseudopuberty progressed into true puberty due to the maturational effect of testosterone. Nephrocalcinosis was still present 8 years later and hypertension was recurring obviously as a consequence of increased peripheral resistance.
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PMID:Aldosterone and testosterone producing adrenal adenoma in childhood. 775 88

Primary hyperaldosteronism is rare in children and usually results from bilateral adrenocortical hyperplasia. Adrenal adenoma causing primary hyperaldosteronism (aldosteronoma) in children is even rarer, with only eight reported cases in children under 18 years of age. Aldosteronoma is a very rare, correctable cause of childhood hypertension. A review of all the reported cases, including that of the authors, is presented. Also discussed are the available biochemical and radiological investigations, the preoperative medical management, the surgical treatment, and the postoperative management.
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PMID:Aldosteronoma in childhood: a review of clinical features and management. 747 42

An 11-year-old girl was referred with hypertension, hypokalemia, low renin activity, metabolic alkalosis and hyperaldosteronism. Adrenal computed tomography revealed the presence of a large left adrenal adenoma of 4 x 4.5 cm in size. Following adrenalectomy, the child presented an unusual acute salt-loosing syndrome which necessitated administration of a large quantity of sodium chloride and corticoids. The cells from the tumor were plated. They rapidly proliferated to a monolayer. After incubation for 24 h, basal production of aldosterone (55.4 +/- 9.07 pmol/ml) was observed. This production was stimulated by the presence of ACTH 10(-8) M and KCl 55 mM; angiotensin II 10(-8) M failed to enhance aldosterone secretion. In the four experimental conditions, however (control, ACTH, angiotensin II and KCl), ANF 10(-8) M decreased aldosterone secretion. We conclude that hyperaldosterone secretion by an adrenal adenoma is not due to resistance to the inhibitory effect of ANF but rather to cell multiplication itself. In this patient, the post-surgical salt-loosing syndrome was attributed to the combined effect of the chronic state of high ANF, low potassium and low ACTH in the contralateral gland on the one hand and a certain degree of transitory renal unresponsiveness of the renal distal tubule to aldosterone on the other.
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PMID:An unusual case of an aldosterone-producing adenoma in a prepubertal girl with severe post-surgical adrenal suppression. 784 16

Ten cases of adrenal adenomas, one case with unilateral adrenal hyperplasia, and another case with apparent bilateral are reported, in whom an alternative pathway of aldosterone via 21-deoxyaldosterone is operative. They all manifested hypertension, low renin activity, low normal potassium values, as well as high urinary excretion rates of 21-deoxyaldosterone and its related metabolite Kelly's-M1 steroid. In all cases, urinary aldosterone metabolites (aldosterone-18-glucuronide and tetrahydroaldosterone) and aldosterone precursor 18-hydroxycorticosterone levels were normal. Hence, the adrenal lesions give rise to hyper-21-deoxyaldosteronism. 21-Deoxyaldosterone is a weak mineralocorticoid, and its elevated production in the presence of normal aldosterone can induce a pathological state of hypermineralocorticoidism. Adrenalectomy resulted in normalization of hypertension in six of eight and amelioration in two of eight cases. Six of seven adenoma cases examined as well as the case of unilateral adrenal hyperplasia were sensitive to ACTH. One of the seven adenomas and, as expected, the case with apparent bilateral hyperplasia were angiotensin responsive. Histologically and electron microscopically, the operated adenomas consisted predominantly of clear cells, characterized by mitochondria with tubulo-vesicular internal structure similar to those of the zona fasciculata (in contrast, our classical Conn's adenoma with normal 21-deoxyaldosterone excretion exhibited a more heterogenous histological appearance and were, in terms of ultrastructure, more similar to cells of the zona glomerulosa). Ultrastructurally and immunocytochemically, the clear cells of 21-deoxyaldosterone adenomas showed features of both the zona glomerulosa and the zona fasciculata and are, hence, considered to be hybrid cells. We conclude that the determination of 21-deoxyaldosterone and Kelly's-M1 should be considered in the diagnosis of mineralocorticoid-induced forms of hypertension, especially when an adrenal adenoma has been detected with an imaging procedure.
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PMID:A new subset of mineralocorticoid hypertension with excess of 21-deoxyaldosterone and Kelly's-M1 steroid: clinical and morphological findings. 788 25

21-Deoxyaldosterone has been postulated to be a precursor of aldosterone in an alternative biosynthesis pathway and Kelly's-M1 is considered to be its metabolite. In healthy volunteers, the excretion rate of 21-deoxyaldosterone and of Kelly's-M1 are significantly lower than the aldosterone metabolites, aldosterone-18-glucuronide and tetrahydro-aldosterone and than the aldosterone precursor 18-OH-corticosterone. Essential hypertension patients (with low and normal renin) excrete comparable values of 21-deoxyaldosterone and Kelly's-M1 as normotensives. In 66% of aldosterone-producing adenoma cases (APA) and in 60% of idiopathic hyperaldosteronism (IHA) patients, significantly raised values of 21-deoxyaldosterone and Kelly's-M1 were found. The patients with the high excretion rates of both steroids showed only moderately increased values of the aldosterone metabolites, aldosterone-18-glucuronide and tetrahydro-aldosterone, as well as of the aldosterone precursor 18-OH-corticosterone. In contrast, the latter mentioned steroids were excreted in higher amounts in those patients with normal excretion of 21-deoxyaldosterone and Kelly's-M1. Hence, it is suggested that aldosterone is produced alternatively either via 18-OH-corticosterone alone or additionally via 21-deoxyaldosterone. Furthermore, in three cases of "incidentally" discovered adrenal adenomas, 21-deoxyaldosterone and Kelly's-M1 were the only elevated steroids. After adrenalectomy, excretion of 21-deoxyaldosterone and of Kelly's-M1 and blood pressure returned to normal, which proves that these steroids play a role in blood pressure regulation. In essential hypertension, ACTH infusion induced a significant increase of 21-deoxyaldosterone and Kelly's-M1. However, the increase after angiotensin II was 3- to 6-fold higher than after ACTH. IHA patients proved to be more responsive to angiotensin II; and, in contrast, APA cases proved to be more sensitive to ACTH. The data suggest that beside the main route of aldosterone biosynthesis via 11-deoxycorticosterone, corticosterone and 18-OH-corticosterone an alternative pathway exists via 21-deoxyaldosterone in healthy and in hypertensive patients. There are similarities between the regulation of 21-deoxyaldosterone and the regulation of aldosterone. The determination of 21-deoxyaldosterone and its possible metabolite Kelly's-M1 might be appropriate in the diagnosis of mineralocorticoid-induced forms of hypertension, especially when an adrenal adenoma is discovered.
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PMID:Role of 21-deoxyaldosterone in human hypertension. 791 19


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