1/9. Bartter's syndrome: clinical study of its treatment with salt loading and propranolol.The clinical course of a 9 year old black male with Bartter's syndrome who has been followed since age 1 1/2 years is presented. He had been unable to conserve sodium on a low salt diet and has failed to show improvement in serum electrolytes while on a sodium loading regimen. He is now refractory to spironolactone and KCI therapy, even in conjunction with supplemental sodium. On two separate trials his electrolytes have approached normal with the combination of propranolol and spironolactone along with KCI and NaCl supplementation. However, his response to this regimen has been a temporary one with relapse to the pretreatment chemical status despite increasing doses of propranolol and potassium chloride.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
2/9. Coexistence of three distinct adrenal tumors in the same adrenal gland in a patient with primary aldosteronism and preclinical Cushing's syndrome.A 62-year-old woman was admitted to our hospital because of hypokalemia. physical examination revealed no signs of excessive adrenocortical steroid production, as are found in Cushing's syndrome. Her plasma renin activity (PRA) was suppressed (0.10 ng/ml per h), and her serum aldosterone level was high (30.0 ng/dl). PRA was not increased after a renin-releasing test. Her plasma adrenocorticotropic hormone (ACTH) level was low (<5 pg/ml), but her serum cortisol level was normal (21.0 microg/dl). Administration of 8 mg dexamethasone did not suppress her plasma cortisol level. Finally, she was diagnosed with clinical primary aldosteronism associated with preclinical Cushing's syndrome. Magnetic resonance image revealed three sequential nodular masses (each 15 mm x 15 mm) in the right adrenal gland. A right adrenalectomy was performed by endoscopy. The three removed tumors appeared to have different characteristics. Microscopic examination revealed that the upper and lower tumors were adrenocortical adenomas, and the middle tumor was a black adenoma. Immunohistochemical staining for the enzymes involved in cortisol biosynthesis suggested that the upper tumor secreted aldosterone, whereas either or both of the two other tumors secreted cortisol. Surprisingly, at 33 years of age, she had been diagnosed with Cushing's syndrome, due to a cortisol-producing adrenocortical adenoma, and she had received a left adrenalectomy. Clinically and pathophysiologically, this was a very rare case.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
3/9. Primary hyperaldosteronism due to a functional black (pigmented) adenoma of the adrenal cortex.The clinical and pathologic findings are reported in a case of primary hyperaldosteronism due to a functional black (pigmented) adenoma of the adrenal cortex. Cortical atrophy of the involved gland was present, the mechanism of which was obscure.- - - - - - - - - - ranking = 5keywords = black (Clic here for more details about this article) |
4/9. cystinosis presenting with features suggesting bartter syndrome. Case report and literature review.Features suggestive of bartter syndrome (hypokalemia, hypochloremic metabolic alkalosis, and normal blood pressure despite hyperreninemia and hyperaldosteronism) were found in a 5-year-old black child with cystinosis and fanconi syndrome. review of his medical records revealed that these abnormalities had probably been present when he first became clinically ill at 2 years of age. sodium and potassium chloride supplementation lead to improved growth and strength, partial correction of his electrolyte abnormalities, and a decrease in markedly elevated plasma renin activity. literature review disclosed a similar presentation in four Caucasian children with cystinosis. Biochemical findings compatible with bartter syndrome can occur together with evidence of generalized proximal renal tubular dysfunction (fanconi syndrome) in nephropathic cystinosis.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
5/9. Primary tumorous aldosteronism due to a black adrenal adenoma: a light and electron microscopic study.A case of primary tumorous aldosteronism due to a black adrenal adenoma in a 37-year-old man is reported. light microscopy showed the tumour to consist predominantly of compact cells containing intracytoplasmic lipofuscin, accompanied by zona fasciculata and intermediate type cells. Electron microscopy identified compact and interface (inner zona fasciculata) type cells, and also demonstrated spironolactone bodies in a compact cell. The morphological appearance of the tumour and adjacent adrenal gland, combined with the clinical and biochemical findings, supports both its secretory function and its classification as a rare variant of the more common non-pigmented aldosteronoma.- - - - - - - - - - ranking = 5keywords = black (Clic here for more details about this article) |
6/9. Normotensive primary aldosteronism.A very unusual case of normotensive primary aldosteronism is described. A 25 year old woman first noticed numbness of both hands followed by a typical tetany attack. The blood pressure was within normal ranges. The results of other physical examinations were negative except for the findings of Trousseau's sign. Hypokalemi, increased potassium clearance, disturbed urine concentration and increased circulating plasma volume were noticeable. diagnosis was established by (1) increased levels of plasma aldosterone, (2) low plasma renin activity, (3) normal adrenocortical function and (4) typical aldosterone-producing adenoma. After removal of the adenoma, the abnormalities subsided. The lack of hypertension in primary aldosteronism is a rare condition. Several possible causes of normotension must be considered, such as the early phase of primary aldosteronism, essential hypotension associated with primary aldosteronism and lack of concomitant secretion of other mineralocorticoids from the adenoma, but these were all negligible. As blood pressure response to the administration of angiotensin ii was lower than is typical for primary aldosteronism, and the patient transiently suffered from frequent attacks of blackouts soon after the operation, the cause of normotension was thought to be due to the existence of a hypotensive mechanism which counteracts the increase in blood pressure in primary aldosteronism.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
7/9. hyperaldosteronism due to unsuspected adrenal carcinoma: discovery during investigation of hypertension in a young woman.During investigation for hypertension a 19-year-old black woman was found to have an unsuspected adrenal carcinoma. hyperaldosteronism was established as the cause of the hypertension by observing suppressed plasma renin activity and nonsuppressible plasma aldosterone concentration. The causal relationship was confirmed by a cure of the hypertension and a return in the responsiveness of the renin-angiotensin-aldosterone axis to normal after removal of the carcinoma. This report emphasizes the value of a comprehensive investigation of hypertension, especially in young patients.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
8/9. diagnosis and surgical treatment of primary aldosteronism in pregnancy: a case report.BACKGROUND: aldosterone-producing adrenal adenomas are rare, especially during pregnancy. We report a patient who presented in the early second trimester, was diagnosed with primary aldosteronism, and was treated successfully by adrenalectomy. CASE: A 17-year-old black nulliparous woman was found to have a blood pressure (BP) of 150/82 mmHg when she registered for prenatal care at 14 weeks' gestation. Initial laboratory assessment revealed a markedly diminished serum potassium level of 2.1 mmol/L. Further laboratory evaluation detected decreased random plasma renin activity and an elevated aldosterone level. magnetic resonance imaging revealed a 2-cm right adrenal lesion. She was diagnosed with an adrenal adenoma and successfully underwent an adrenalectomy at 17 weeks' gestation. Postoperatively, her BP and serum potassium level normalized. She spontaneously delivered a normal male infant at term. CONCLUSION: Although primary hyperaldosteronism is a rare clinical entity, it must be considered when hypertension and hypokalemia are present concurrently. Antepartum medical management can be difficult, often resulting in poor obstetric outcome. Surgery in the second trimester is an effective option.- - - - - - - - - - ranking = 1keywords = black (Clic here for more details about this article) |
9/9. Liddle's syndrome, an underrecognized entity: a report of four cases, including the first report in black individuals.Liddle's syndrome, a rare cause of hypokalemic hypertension, is characterized by a renal tubular sodium channel defect resulting in excessive sodium absorption and concomitant potassium wasting. In this disorder, although the clinical manifestations resemble primary aldosteronism, serum and urine aldosterone are suppressed. The syndrome is transmitted in an autosomal dominant pattern. It has been reported previously in white and oriental populations but not in the black individuals. We identified four patients (two of whom are black) in our nephrology clinic, with severe hypokalemic hypertension not correctly diagnosed for several years. All patients underwent an extensive work-up for secondary hypertension because of persistent severe hypertension (average blood pressure, 210/130 mm Hg) despite high-dose multi-drug therapy. Primary aldosteronism was excluded because of low serum aldosterone. Cushing's syndrome, pheochromocytoma, renal artery stenosis, and enzymatic deficiencies of cortisol synthesis (11 beta-hydroxylase, 17 alpha-hydroxylase, 5 beta-reductase, and 11 beta-hydroxysteroid dehydrogenase) were ruled out with extensive endocrine and radiologic studies. Once the diagnosis of Liddle's syndrome was suspected, all patients were treated with either triamterene or ameloride, with resolution of hypokalemia and correction of hypertension occurring within 5 to 7 days. Our findings suggest that Liddle's syndrome can occur in the black population. Although the actual incidence of this syndrome remains unknown, it may be significantly more common than we are led to believe since it is inherited in a Mendelian pattern. Whether there is a subset of low-renin, salt-sensitive black hypertensive patients who have the same or similar sodium channel defect remains to be elucidated.- - - - - - - - - - ranking = 8keywords = black (Clic here for more details about this article) |