| Stone formation in renal allografts is rare. Although infection or renal tubular acidosis can predispose to calcium deposition in a renal allograft, hyperparathyroidism is usually an accompanying factor. parathyroidectomy is recommended as the treatment of choice when stone deposition or nephrocalcinosis occurs after transplantation. The reported case demonstrates that aggressive therapy is also necessary to eliminate calculi from the urinary system to avoid mechanical obstruction, continued infection or renal paraenchymal damage. ( info) |
132/158. Familial hypomagnesaemia--hypercalciuria leading to end-stage renal failure. Several disorders of hypomagnesaemia of hetary renal origin are now recognised. The cases of two sisters from a consanguineous marriage with the syndrome of renal magnesium wasting, hypercalciuria and nephrocalcinosis are presented. Pathological examination of the heterozygous parental kidneys revealed mild focal interstitial fibrosis. This condition is a previously unreported cause of end-stage renal failure in childhood, and this report suggests that transplantation from heterozygous parental donors can be successfully undertaken without recurrence of the syndrome. ( info) |
| 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. ( info) |
| We report a case of congenital magnesium-losing kidney with associated hypercalciuria and ocular derangements consisting of myopia, bilateral macular colobomata and bilateral papillary dysversion and hypoplasia. Our patient provides further evidence of the existence of an oculorenal syndrome associating abnormalities in the renal handling of magnesium and calcium, and myopia and chorioretinal disease. ( info) |
| nephrocalcinosis is uncommon in childhood, and almost always develops outside the newborn period. Over the last decade, nephrocalcinosis due to multifactorial pathogenesis has increased in very low birthweight infants. In this report a 23 day old full-term baby with nephrocalcinosis secondary to distal renal tubular acidosis is described. ( info) |
136/158. 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. ( info) |
| A 52-year-old woman was referred to our hospital because of 16-year history of polyuria and polydipsia. Hyposthenuria, hyperchloremic metabolic acidosis and the inabilities to acidify the urine after acid-loading test and to concentrate the urine in responses to water-deprivation and antidiuretic hormone administration allowed us to diagnose renal tubular acidosis and nephrogenic diabetes insipidus. Radiographic examinations revealed bilateral nephrocalcinosis. The patient was also found to have clinical and laboratory findings characteristic for Sjogren's syndrome. Thus the longstanding, poorly monitored distal renal tubular acidosis associated with sjogren's syndrome was considered to result in very rare renal complications-nephrocalcinosis and nephrogenic diabetes insipidus. In patients with renal tubular acidosis and/or nephrogenic diabetes insipidus of unknown etiology, therefore, sjogren's syndrome should be considered as one of primary disorders. ( info) |
138/158. Sodium thiosulphate in the treatment of renal tubular acidosis I with nephrocalcinosis. nephrocalcinosis is a common feature in renal tubular acidosis I (RTA-I) and contributes to renal insufficiency. We describe a patient, 37 years old, with RTA-I, mild renal failure and extended nephrocalcinosis. His disease was diagnosed in age 28 and patient is under treatment with sodium bicarbonate, potassium gluconate and sodium thiosulphate for 9 years. By this treatment nephrocalcinosis and renal function have not been worsened and patient is without clinical symptoms. ( info) |
| nephrocalcinosis and nephrolithiasis developed in five children after furosemide therapy for congestive heart failure. In four children renal calcifications were detected by ultrasonography and in one by autopsy. Discontinuation of the loop diuretic in three children resulted in resolution of the calcifications in two of the patients. Residual renal morbidity included reduced creatinine clearance, microscopic hematuria, and hypercalciuria. The phenomenon of renal calcifications associated with furosemide treatment is more frequent than previously recognized. ( info) |
| hypercalciuria and nephrocalcinosis are not uncommon in patients with Wilson's disease but have only once been reported as the presenting sign. We diagnosed Wilson's disease in a 17-year-old male patient 6 years after his first episode of gross hematuria and 2 years after detection of hypercalciuria and nephrocalcinosis. Therapy with penicillamine resulted only in a moderate reduction of urinary calcium excretion but oxalate excretion increased. ( info) |