1/3. Renal cysts and nephrocalcinosis in a patient with bartter syndrome type III.Chronic hypokalemia is known to induce renal cyst formation in some diseases including primary aldosteronism, distal renal tubular acidosis, Liddle disease and apparent mineralocorticoid excess syndrome. Although chronic hypokalemia is the main clinical feature of bartter syndrome, renal cyst formation in this disease has never been reported. We describe a patient with classic bartter syndrome who exhibited renal cysts and nephrocalcinosis. Direct sequencing analysis of the chloride channel CLC-Kb gene identified a heterozygous nonsense mutation (W610X) in exon 16 indicating a diagnosis of bartter syndrome type III. Although the precise mechanism underlying the development of renal cysts in our patient remains unclear, chronic hypokalemia and nephrocalcinosis may contribute to cyst development.- - - - - - - - - - ranking = 1keywords = aldosteronism (Clic here for more details about this article) |
2/3. A case of normoreninemic, normotensive primary aldosteronism associated with essential hypertension and nephrocalcinosis.A 36-year-old female case of normotensive normoreninemic primary aldosteronism with persistent hypokalemia and nephrocalcinosis is reported. She was referred to us for episodes of sudden muscle weakness during 8 years prior to admission. On the first day of admission, her blood pressure was 174/104 mmHg. On the second day of admission blood pressure normalized to 120/80 mmHg. Both of her parents were hypertensive. Arterial blood gas analysis showed metabolic alkalosis. Except an impaired urine concentration ability, renal functions were normal. Intravenous pyelogram showed numerous granular calcifications. Basal plasma renin activity was 1.0 approximately 1.5 ng/ml/hr and increased by sodium depletion. Plasma aldosterone concentration was 70 approximately 80 ng/dl and did not respond to various stimulations. blood pressure was dependent on sodium balance. It fell on salt restriction and rose on salt loading. blood pressure responses to vasoactive hormones were normal. Circulating plasma volume was within normal range. After removal of an adrenal adenoma, there was mild fall of blood pressure, serum potassium returned to normal level and plasma renin activity increased slightly. Histologically, there was renal tubular calcifications, and juxtaglomerular apparatus was normal. blood pressure was elevated to 160/100 mmHg when patient was followed at out-patient clinic after discharge. We concluded that she had essential hypertension associated with primary aldosteronism. Although sodium loss and an increase in urinary kallikrein were found, they did not seem to be the cause of normoreninemic normotensive state of this patient, and the pathogenesis remains to be elucidated.- - - - - - - - - - ranking = 6keywords = aldosteronism (Clic here for more details about this article) |
3/3. Aldosteronoma in childhood: a review of clinical features and management.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.- - - - - - - - - - ranking = 2keywords = aldosteronism (Clic here for more details about this article) |