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

A patient with early infantile galactosialidosis presenting as congenital adrenal hyperplasia with clitoral hypertrophy and arterial hypertension is reported. Serum 17-alpha-OH-progesterone and plasma renin levels were elevated. Adrenal hyperplasia and thickening of the cardiac septum were detected by sonography; however, progressive hepatosplenomegaly, increasingly coarse features, and vacuolization of bone marrow and liver cells suggested a storage disorder. Combined deficiency of beta-galactosidase and sialidase enzyme activity in both lymphocytes and cultured fibroblasts was detected. This patient with early infantile galactosialidosis is the first reported who presented with congenital adrenal hyperplasia.
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PMID:Infantile galactosialidosis presenting with congenital adrenal hyperplasia and renal hypertension. 821 48

The occurrence of hypokalemia in association with high blood pressure is suggestive of primary hypermineralocorticism since in this case both abnormalities might result from a single mechanism. However, adrenal adenomas is well as the various forms of adrenal hyperplasia appear to be quite uncommon, whereas a number of other causes of potassium depletion are far more prevalent and may be associated with essential hypertension. The demonstration of the precise mechanism of both decreased serum potassium and increased blood pressure is a prerequisite for a successful treatment.
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PMID:[Arterial hypertension and hypokalemia]. 823 14

Primary hyperaldosteronism was detected in 1% of patients evaluated for secondary hypertension in a referral hospital in Southern India. The presence of hypokalemia with inappropriate kaliuresis (24 hr. urine K > 20mEq) was an important diagnostic clue. High resolution CT scans were found to be useful for localising the tumour. Preoperatively nifedipine and spironolactone were employed to correct hypertension and hypokalemia. The same drugs were also found to be excellent for long-term control of hypertension and hypokalemia in patients who had bilateral adrenal hyperplasia and aldosteronism.
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PMID:Primary hyperaldosteronism--diagnostic approach and management. 805 42

A 46-year-old man with known arterial hypertension for 10 years had, over the last two years, developed increasing obesity, particularly of the trunk, with other symptoms typical of Cushing's syndrome. Hormone analysis demonstrated hypercortisolism and decreased plasma ACTH concentration. The dexamethasone inhibition test failed to show any significant suppression of serum cortisol. Plasma ACTH was not increased in the corticotrophin-releasing hormone and the metyrapone tests. In the short ACTH test there was an excessive cortisol increase. Abdominal computed tomography revealed both adrenals to be enlarged (6 x 4 cm) and coarsely nodular. Adrenolytic treatment with ketoconazole (400 mg daily) caused symptoms of adrenal insufficiency, but a reduced dosage of 200 mg daily lowered the cortisol level to between 5 and 11 micrograms/dl and normalized the blood pressure and clinical signs of Cushing's syndrome disappeared. Subsequent bilateral adrenalectomy confirmed the diagnosis of massive macronodular adrenal hyperplasia. Substitution treatment with twice daily 25 mg cortisone acetate and 0.05 mg fludrocortisone was started postoperatively.
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PMID:[Bilateral massive macronodular adrenal gland hyperplasia. A rare cause of Cushing's syndrome]. 830 53

In its classical form, congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency is characterized by hypertension and abnormal sexual development. Suppression of ACTH secretion by means of administering glucocorticoids fulfills the therapeutic goal of reducing blood pressure and decreasing androgen production. The present report describes the case of a patient suffering from congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency who developed an acute adrenal crisis, unprovoked by stress, following interruption of glucocorticoid replacement therapy. It is suggested that patients on a suppressive dose of glucocorticoids for adrenal hyperplasia are at increased risk for acute adrenal insufficiency if therapy is interrupted, and that deoxycorticosterone (DOC) in the absence of a glucocorticoid is insufficient to prevent manifestations of adrenal crisis.
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PMID:Acute adrenal crisis complicating hypertensive congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency. 844 9

The diagnosis of primary aldosteronism (PA) is based on the finding of the combination of elevated urinary and/or plasma aldosterone and suppressed renin activity in patients with hypertension and hypokalemia. However, PA consists of a number of subsets, and diagnostic criteria for a correct identification of surgically remediable forms are of great interest. The methods and the results concerning our series of 113 patients with PA are presented in this review. Aldosterone producing adenoma (APA) and idiopathic hyperaldosteronism (IHA) were the most frequent forms, 51 and 44%, respectively. They had similar blood pressure levels, but hypokalemia was most frequently found in APA. Urinary and upright plasma aldosterone were similar, but supine plasma aldosterone was lower in IHA. Plasma aldosterone response to upright posture and angiotensin II infusion was absent in most cases of APA and present in IHA, but occasionally renin-responsive adenoma were found. Captopril failed to decrease plasma aldosterone in most patients with APA, and in a subgroup of patients with IHA. Patients with adenoma also had higher values of the aldosterone precursor 18-hydroxy-corticosterone, and of atrial natriuretic peptide, probably as a consequence of a greater degree of volume expansion. Among morphological studies, CT scan and adrenal radiocholesterol scintiscan provided similar results (85% accuracy): adrenal veins catheterization clarified almost all the remaining cases. Among the subsets of PA, 3 familiar cases of dexamethasone-suppressible hyperaldosteronism were recognized, with characteristically high levels of aldosterone, 18-hydroxy-corticosterone, 18-hydroxy-cortisol and 18-oxo-cortisol, due to the genetic abnormalities of the 11-18 hydroxylase system. Isolated cases of primary adrenal hyperplasia (with all functional tests resulting compatible with APA, but no tumour at surgery) and aldosterone producing carcinoma (1 case) have also been reported in the present study.
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PMID:Differential diagnosis in primary aldosteronism. 848 51

The combination of hypertension, hypokaliemia, and male pseudohermaphroditism or amenorrhea must prompt a search for a rare adrenal enzymatic defect, 17 alpha-hydroxylase/17,20-lyase deficiency. This is a report of the observation of a male patient in whom this rare deficit was diagnosed in adulthood on the basis of lifelong ambiguous external genitalia, hypogonadism, severe hypertension, bilateral adrenal hyperplasia, and biological markers evoking an excess of mineralocorticoids without hyperaldosteronism.
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PMID:Case report: 17 alpha-hydroxylase/17,20-lyase deficiency: a rare cause of endocrine hypertension. 878 79

Primary hyperaldosteronism (adrenal adenoma and idiopathic hyperplasia) is a disorder with hypertension, hypokalemia, elevated serum aldosterone and suppressed plasma renin activity. Hyperplasia is managed medically whereas adenomas are treated surgically. Selective adrenal venous catheterization and aldosterone measurement is a useful tool in making the distinction in 95% of cases. We report a case of bilateral idiopathic hyperplasia of the adrenal glands adequately treated with medications for 6 years followed by worsening. Selective catheterization was consistent with a right sided adenoma. Surgical removal of the right adrenal gland alleviated her symptoms. Pathological examination showed focal nodular hyperplasia. We propose that in the course of the disease the focal hyperplastic nodule became autonomous and behaved like an adenoma. Monitoring of patients with adrenal hyperplasia for recurrence of symptoms is prudent as surgery is beneficial in patients who develop an autonomous nodule.
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PMID:Idiopathic hyperplasia of the adrenal gland behaving like an aldosterone producing adenoma. 907 69

Primary aldosteronism is one of the differential diagnosis of secondary hypertension. This is usually caused by an aldosterone producing adenoma or bilateral adrenal hyperplasia which comprise about 65% and 30% of the cases, respectively. However, less than 1% of primary aldosteronism is caused by unilateral adrenal hyperplasia which is a relatively rare subset of primary aldosteronism. The clinical and biochemical manifestations of the disorder are indistinguishable from aldosterone-producing tumor, and a definitive diagnosis can only be made by pathological finding. A 33-year-old male Chinese patient presented with hypertension, hypokalemia, metabolic alkalosis, and the hypersecretion of aldosterone associated with suppressed plasma renin activity which is a typical hallmark of primary aldosteronism. Image studies including both magnetic resonance imaging (MRI) and 131I NIP-59 scan as well as postural test suggested an aldosterone-producing tumor of the right adrenal gland. Unilateral adrenectomy and pathological examination of the right adrenal gland eventually proved a case of unilateral adrenal hyperplasia. Blood pressure, plasma potassium, aldosterone and renin activity levels returned to normal two weeks after operation and had remained normal at up to one year of follow up. In addition, a saline loading test showed normal suppression of plasma aldosterone level one year after the operation, suggesting that the function of the left adrenal gland remains normal. The etiology of unilateral adrenal hyperplasia is unclear and the future recurrence of the disease is possible. Long-term follow-up is necessary to ensure the cure of this disorder.
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PMID:Primary aldosteronism due to unilateral adrenal hyperplasia: a case report. 917 1

Several important advances have been made in the pathogenesis of mineralocorticoid induced hypertension. A hybrid gene was found to be responsible for glucocorticoid remediable hypertension. This extra gene contains fragments of 11-beta-hydroxylase and aldosterone synthase. The hybrid gene is the result of an unequal crossing-over of the two genes located in close proximity on chromosome 8, and leads to the production of aldosterone and the hybrid steroids 18-hydroxycortisol and 18-oxocortisol. These hybrid steroids are also detected in patients with aldosterone producing adenoma but not in patients with hyperaldosteronism due to bilateral adrenal hyperplasia. In Apparent "Mineralocorticoid Excess", inherited as an autosomal recessive disorder, an increased ratio of urinary cortisol metabolite to cortisone is diagnostic. The syndrome is due to a deficiency of the renal enzyme 11-beta-hydroxysteroid dehydrogenase type II, which protects the mineralocorticoid receptor against cortisol that binds to the mineralocorticoid receptor like aldosterone. Liddle's syndrome is a rare entity and due to a constitute activation of an aldosterone dependent protein which triggers the amiloride sensitive sodium channel in the kidney. This results in hypokalemic hypertension with suppressed aldosterone and renin levels.
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PMID:[Mineralocorticoid-induced hypertension]. 924 33


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