1/10. ACE-inhibitors-induced metabolic acidosis in a child with nephrotic syndrome.In recent years there has been an increase in the use of ACE inhibitors in the paediatric population. We describe a case of hyperchloraemic metabolic acidosis with hypoaldosteronism in a 4-year-old boy with nephrotic syndrome who was receiving ACE inhibitors.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
2/10. Selective hypoaldosteronism in a patient with sjogren's syndrome: insensitivity to angiotensin ii.A 51-year-old Japanese woman with hypokalemia due to distal renal tubular acidosis associated with sjogren's syndrome exhibited a decreased plasma aldosterone level despite elevated plasma renin activity. Our studies revealed selective hypoaldosteronism with normal adrenoglucocorticoid function. In the presence of a low level of serum potassium (3.6 mEq/l), plasma levels of deoxycorticosterone and corticosterone were normal, while plasma aldosterone was very low. The levels of these three mineralocorticoids showed only minor changes during infusion of angiotensin ii. furosemide administration under almost the same level of serum potassium (3.7 mEq/l) resulted in only a slight increase of plasma aldosterone. Since hypokalemia might possibly suppress the synthesis of aldosterone in the zona glomerulosa, angiotensin ii was also infused under a normal level of potassium (4.3 mEq/l). However, angiotensin ii also failed to stimulate any secretion of aldosterone, despite a progressive rise in blood pressure and sufficient suppression of plasma renin activity. On the other hand, rapid ACTH administration in the presence of 4.4 mEq/l of serum potassium increased both plasma aldosterone and cortisol. These results suggest that adrenal insensitivity to angiotensin ii was the cause of the selective hypoaldosteronism in our patient, possibly due to a dysfunction of adrenal angiotensin ii receptors, a disorder of postreceptors or both.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
3/10. pseudohypoaldosteronism: case report and discussion of the syndrome.A 41-year-old man, complaining of leg cramps, was found to have persistent hyperkalemia. Except for mild hypertension, his physical examination and laboratory values to exclude connective tissue diseases and diabetes mellitus were normal. Renal function testing revealed a normal glomerular filtration rate and tubular capacity to acidify and dilute, as well as near-normal ability to concentrate his urine. Hormonal evaluation revealed a normal cortisol, as well as normal resting and stimulated renin and aldosterone levels. A selective defect in tubular potassium secretion was demonstrated. In the absence of aldosterone deficiency or renal dysfunction, it was assumed that the patient had primary renal resistance to aldosterone, known as pseudohypoaldosteronism. Treatment with hydrochlorothiazide controlled his hyperkalemia and hypertension. His case emphasizes the diagnostic and therapeutic factors that should be considered in evaluating and treating a non-hospitalized patient with sustained hyperkalemia.- - - - - - - - - - ranking = 0.8keywords = syndrome (Clic here for more details about this article) |
4/10. Adrenocortical insufficiency with normal serum cortisol levels and hyporeninaemia in a patient with acquired immunodeficiency syndrome (AIDS).The acquired immunodeficiency syndrome (AIDS) has been associated with abnormalities of adrenocortical function, and hypoaldosteronism due to hyporeninaemic hypoaldosteronism (HHA). We here report the case of a woman with AIDS associated with orthostatic hypotension, persistent hyponatraemia and hyperkalaemia, in whom basal serum cortisol levels were normal and serum renin activity was low. Subsequent post-mortem examination revealed almost complete adrenocortical destruction. A possible explanation of this apparently contradictory combination of findings is discussed, together with the therapeutic implications for similar cases.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
5/10. hyponatremia and hyperreninemic hypoaldosteronism in a critically ill patient: combination of insensitivity to angiotensin ii and tubular unresponsiveness to mineralocorticoid.A 62-year-old man with pneumonia and left flank pain had a clinical syndrome of hyponatremia, hypotension, dehydration, and high urinary sodium excretion in the presence of a normal glomerular filtration rate. The plasma level of antidiuretic hormone was relatively high despite decreased serum osmolality. Thyroid function and excretion of glucocorticoid and sex steroids were normal. The serum aldosterone level was very low despite elevated plasma renin activity. angiotensin ii failed to stimulate any secretion of aldosterone, despite the occurrence of a progressive rise in blood pressure. On the other hand, rapid ACTH administration increased both serum aldosterone and cortisol. The patient showed no effective response to increased salt intake, but large doses of mineralocorticoid resulted in a normal serum sodium level without dehydration. Subsequently, he suffered cardiac arrest secondary to ventricular tachycardia. Postmortem examination showed well differentiated adenocarcinoma in the left pleura and an intact, histologically normal adrenal zona glomerulosa and kidney. This is the first reported case of a critically ill patient with hyponatremia caused by hyperreninemic hypoaldosteronism possibly due to angiotensin ii insensitivity and tubular unresponsiveness to mineralocorticoid.- - - - - - - - - - ranking = 0.2keywords = syndrome (Clic here for more details about this article) |
6/10. New findings in apparent mineralocorticoid excess.We report two female siblings (ages 4 and 9 years) and one 8-year-old male with the syndrome of apparent mineralocorticoid excess (AME) presenting with low renin hypertension and hypoaldosteronism. The deficiency of 11 beta-hydroxysteroid dehydrogenase results in a defect of the peripheral metabolism of cortisol (F) to cortisone (E). As a result, the serum cortisol half-life (T1/2) is prolonged, ACTH is suppressed, and serum F is normal. The specific diagnosis of the disorder was made by the decreased ratio of the urinary metabolites of E (tetrahydrocortisone, THE) and F (tetrahydrocortisol, THF). Continuous i.v. hydrocortisone administration caused an increase in blood pressure and decrease in serum potassium demonstrating the abnormal mineralocorticoid activity of cortisol in these patients. Addition of spironolactone resulted in a decrease in blood pressure, rise in serum potassium and a gradual increase in plasma renin activity. These studies suggest that an abnormality in cortisol action or metabolism results in cortisol behaving as a potent mineralocorticoid and causing the syndrome of AME.- - - - - - - - - - ranking = 0.4keywords = syndrome (Clic here for more details about this article) |
7/10. Long-term follow-up of mitomycin C nephropathy.Two cases of mitomycin C nephropathy, which occurred after postoperative chemotherapy for advanced gastric cancer, were followed up for 6 (case 1) and 10 years (case 2). Hemolytic uremic syndrome developed 68 days (case 1) and 160 days (case 2) after the last administration of MMC with a total dose of 60 mg (case 1) and 40 mg (case 2). serum creatinine levels were normalized in case 1 and they remained at about 2 mg/dl in case 2. Hyporeninemic hypoaldosteronism was transiently seen in case 2. These data suggest that recovery from the acute phase of hemolytic uremic syndrome leads to a good long-term prognosis in MMC nephropathy.- - - - - - - - - - ranking = 0.4keywords = syndrome (Clic here for more details about this article) |
8/10. Reduced Na , K( )-ATPase activity in patients with pseudohypoaldosteronism.pseudohypoaldosteronism is a hereditary salt-wasting syndrome usually seen in infancy with weight loss, dehydration, and failure to thrive. The pathophysiologic origin of pseudohypoaldosteronism is unknown. The defect could be related to the unresponsiveness of target organs to mineralocorticoids resulting in hyponatremia, hyperkalemia, and markedly elevated plasma aldosterone and renin levels. Red blood cell Na , K( )-ATPase activity was measured in a pair of twins with pseudohypoaldosteronism, in an unrelated child with hypoaldosteronism, and in an age-matched group of 50 healthy infants and young children. The enzyme was assayed by a method that couples ATP hydrolysis with NADH oxidation. plasma renin and aldosterone levels were measured by RIA. Red blood cell Na , K( )-ATPase activity in the twins with pseudohypoaldosteronism was very low at the time of diagnosis (3 wk). In both twins a time-related gradual increase in enzyme activity was observed during the 1st mo of life, reaching control values between 6 and 8 mo of age. This increase was associated with both a reduction in salt requirement and clinical improvement. plasma renin activity and aldosterone levels were very high at the time of diagnosis. plasma renin activity reverted gradually to normal values, whereas aldosterone levels remained high throughout the follow-up period. The child with hypoaldosteronism had normal Na , K( )-ATPase activity at diagnosis and during follow-up.- - - - - - - - - - ranking = 0.2keywords = syndrome (Clic here for more details about this article) |
9/10. Hyporeninemic hypoaldosteronism associated with multiple myeloma: 11 years of follow-up.Hyporeninemic hypoaldosteronism is an important underlying condition, causing hyperkalemia with hyperchloremic metabolic acidosis, disproportionate to the degree of renal insufficiency present. The principal defect in this syndrome is a reduced level of plasma renin activity, which results in secondary hypoaldosteronism. diabetes mellitus is usually the primary underlying renal disease, though other causes of renal diseases associated with this syndrome have been described. This case report describes for the first time an elderly patient with multiple myeloma, in remission for more than 11 years, associated with the syndrome of hyporeninemic hypoaldosteronism at the time of diagnosis. The complete resolution of the syndrome after vigorous chemotherapy is an intriguing possibility.- - - - - - - - - - ranking = 0.8keywords = syndrome (Clic here for more details about this article) |
10/10. Liddle's syndrome: a 14-year follow-up of the youngest diagnosed case.The 14-year follow-up of a female patient with Liddle's syndrome (LS), a rare disease characterized by hypertension, hypokalemic alkalosis, and negligible aldosterone secretion due to renin suppression, is described. The disease was diagnosed at the age of 10 months (youngest identification). The patient was repeatedly investigated during follow-up for plasma renin activity (PRA), plasma aldosterone concentration (PA), serum sodium and potassium (K) concentration, blood pressure (BP), somatic anthropometry, and mental development. Noteworthy results included: persistent low circulating K, PRA, and PA and high BP, coinciding with unauthorized withdrawal of the triamterene therapy. These findings are in keeping with the hypothesis that LS results from a pathogenetic disorder which is not correctable with age. The triamterene therapy was effective in correcting the endocrine and metabolic disorders as well as arterial hypertension, but did not prevent a deficit in mental and physical development. However, the information derived from this study allows further clarification of the clinical picture of the disease.- - - - - - - - - - ranking = 1keywords = syndrome (Clic here for more details about this article) |
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