1/39. hyperkalemia unresponsive to massive doses of aldosterone in a patient with renal tubular acidosis.In a 53-year-old male patient aldosterone-refractory hyperkalemia was associated with renal tubular acidosis (RTA) due to chronic interstitial nephritis accompanied by peritubular hyaline deposits in the distal nephron. The hyperkalemia was not caused by an adrenal disorder or acidosis and could not be abolished by diuretics, cortisone, longacting synthetic ACTH, excessive doses of DOCA and aldosterone. The results of our experimental studies carried out on the hyperkalemic RTA patient as well as on various control subjects and patients suggested the presence of a specific defect in renal K excretion associated with a decreased aldosterone responsiveness of the renal tubules presumably due to the peritubular pathology.- - - - - - - - - - ranking = 1keywords = nephritis, interstitial nephritis, interstitial (Clic here for more details about this article) |
2/39. Distal tubular dysfunction in lupus nephritis of childhood and adolescence.We describe a girl with lupus nephritis who presented with distal renal tubular acidosis and hyporeninemic hypoaldosteronism. While distal tubular dysfunction is well recognized in adult systemic lupus erythematosus (SLE), only a few pediatric patients have been reported. Evaluation of five pediatric patients with SLE revealed that distal tubular dysfunction in childhood and adolescence is rare.- - - - - - - - - - ranking = 3.185379168056keywords = nephritis (Clic here for more details about this article) |
3/39. Mesangial nephropathy in sjogren's syndrome.We describe a 36-year-old woman with Primary sjogren's syndrome (PSS). purpura, corneal perforation, metabolic acidosis, decreased glomerular filtration, hypokalemia, hyposthenuria, and polyuria were present. Chronic renal insufficiency and renal tubular acidosis type I were diagnosed. kidney biopsy revealed mesangial glomerulonephritis, interstitial nephritis, and tubular atrophy. Replacement treatment with saliva, tears, and potassium citrate was started. She was given prednisone and cyclophosphamide. This would be the first description of PSS, mesangial glomerulonephritis, and chronic renal insufficiency.- - - - - - - - - - ranking = 2.2741516672224keywords = nephritis, interstitial nephritis, interstitial (Clic here for more details about this article) |
4/39. Renal tubular acidosis and vasculitis associated with IgE deposits in the kidney and small vessels.We report a woman with a history of allergies, polyuria, polydipsia, proteinuria, renal loss of electrolytes, renal tubular acidosis, nephrocalcinosis, and palpable purpura. A proximal defect was excluded by a normal bicarbonate reabsorption curve, and a distal tubular defect was shown because urine pH did not decrease to less than 6.4 despite ammonium chloride-induced systemic acidosis. Moreover, furosemide failed to improve urinary acidification. urine-to-blood PCO(2) gradient was less than 14 mm Hg, although the urine bicarbonate level reached values as high as 89 mEq/L. Combining bicarbonate and neutral phosphate infusions increased the urine-to-blood PCO(2) gradient to only 20 mm Hg. These subnormal PCO(2) gradient values point to proton-pump dysfunction in the collecting tubule. Histological evidence of tubulointerstitial disease accompanied the tubular defects. The striking histological feature was the presence of immunoglobulin e (IgE) deposits in glomeruli, tubuli, and vessels. Concurrent with these findings, she had high serum IgE titers and CD23 levels. IgE antibodies from her serum were reactive against human renal tubuli, with binding to two regions that matched two different proteins present in cortex and medulla. One of these proteins corresponded to carbonic anhydrase ii (31 kd); the second, to an unidentified protein that seems attached to cell membranes. We suggest that these IgE antibodies could have had a pathogenic role in this patient's glomerular, tubular, and small-vessel disease.- - - - - - - - - - ranking = 2.06042301015keywords = tubulointerstitial, interstitial (Clic here for more details about this article) |
5/39. Systemic lupus erythematosus presenting initially as hydrogen ATPase pump defects of distal renal tubular acidosis.Tubulointerstitial involvement is well recognized in systemic lupus erythematosus. The tubular dysfunction is usually latent and usually presents after diagnosis of systemic lupus erythematosus. We report a case presenting that she is well previously and initially diagnosed as periodic paralysis of hypokalemia at emergency room and final diagnosis is systemic lupus erythematosus with H -ATPase pump defect of distal type renal tubular acidosis. kidney biopsy showed lupus nephritis classified as mesangial proliferative glomerulonephritis WHO class II B. Her renal tubular acidosis was subsided after steroid therapy was administered.- - - - - - - - - - ranking = 1.2844689223374keywords = nephritis, interstitial (Clic here for more details about this article) |
6/39. Systemic lupus erythematosus presenting initially as hydrogen ATPase pump defects of distal renal tubular acidosis.Tubulointerstitial involvement is well recognized in systemic lupus erythematosus. The tubular dysfunction is usually latent and usually presents after diagnosis of systemic lupus erythematosus. We report a case presenting that she is well previously and initially diagnosed as periodic paralysis of hypokalemia at emergency room and final diagnosis is systemic lupus erythematosus with H -ATPase pump defect of distal type renal tubular acidosis. kidney biopsy showed lupus nephritis classified as mesangial proliferative glomerulonephritis WHO class II B. Her renal tubular acidosis was subsided after steroid therapy was administered.- - - - - - - - - - ranking = 1.2844689223374keywords = nephritis, interstitial (Clic here for more details about this article) |
7/39. Unusual renal features of Lowe syndrome in a mildly affected boy.The oculocerebrorenal syndrome of Lowe (OCRL) is an X-linked disorder characterized by congenital cataracts, mental retardation, and renal tubular dysfunction. The gene responsible for OCRL was identified by positional cloning and encodes a lipid phosphatase, phosphatidylinositol 4,5, bisphosphate [PtdIns(4,5)P2]5-phosphatase, which localizes to the golgi apparatus and is suspected to play a role in Golgi vesicular transport [Suchy et al., 1995]. In addition to the ocular and renal manifestations, most boys with OCRL have cognitive problems and maladaptive behaviors including tantrums and stereotypies. We report a boy with a history of congenital cataracts and mild developmental delay who was also found to have hematuria with proteinuria but minimal signs of renal tubular dysfunction. Subsequent renal biopsy was compatible with a diagnosis of a noncomplement fixating chronic glomerulonephritis. Despite the atypical renal findings, skin fibroblast analysis for PtdIns (4,5)P2 5-phosphatase was performed, and enzyme activity was low, consistent with the diagnosis of OCRL. Western blot analysis from cell lysates showed the ocrl protein was decreased in size and amount. Our report shows atypical renal features of OCRL in a mildly affected boy. The possibility of OCRL should be considered in boys with cataracts and glomerular disease, even in the absence of renal tubular defects and frank mental retardation usually associated with the syndrome. Am. J. Med. Genet. 95:461-466, 2000. Published Wiley-Liss, Inc.- - - - - - - - - - ranking = 0.63707583361121keywords = nephritis (Clic here for more details about this article) |
8/39. Subacute cutaneous lupus erythematosus and life-threatening hypokalaemic tetraparesis: a rare complication.We describe a patient with lesions of subacute cutaneous lupus erythematosus associated with high titres of anti-Ro and anti-La antibodies who developed a rapidly progressive flaccid tetraparesis due to profound hypokalaemia. After investigation she was found to have distal renal tubular acidosis (dRTA) without pathological evidence of lupus nephritis. It is likely that her dRTA was a manifestation of associated sjogren's syndrome, which had been otherwise asymptomatic. This is the first report of such a complication in the dermatological literature.- - - - - - - - - - ranking = 0.63707583361121keywords = nephritis (Clic here for more details about this article) |
9/39. Flaccid paresis due to distal renal tubular acidosis preceding systemic lupus erythematosus.We report a 25-year-old woman presenting with a flaccid paresis due to severe hypokalaemia as a consequence of distal renal tubular acidosis (dRTA). Six years after presentation of dRTA, she developed overt symptoms of systemic lupus erythematosus (SLE). dRTA in SLE is often secondary to an interstitial nephritis. In contrast to other reports the dRTA did not resolve after treatment with prednisone in our patient. nephrocalcinosis might be one of the causal factors in the persistence of dRTA.- - - - - - - - - - ranking = 1keywords = nephritis, interstitial nephritis, interstitial (Clic here for more details about this article) |
10/39. Acute tubulointerstitial nephritis with uveitis syndrome presenting as multiple tubular dysfunction including Fanconi's syndrome.We describe an 11-year-old male patient with acute tubulointerstitial nephritis with uveitis (TINU) syndrome. He presented with easy fatigability, pallor, nocturia and weight loss. Laboratory examination disclosed anaemia, polyclonal hypergammaglobulinaemia, low molecular weight proteinuria, glycosuria, aminoaciduria, proximal and distal renal tubular acidosis, a urine concentration defect and decreased creatinine clearance. The multiple renal tubular dysfunction and slight glomerular dysfunction subsided spontaneously. Bilateral anterior uveitis was manifested 7 months after the onset of the disease. This is the first reported case of TINU syndrome with multiple proximal and distal tubular dysfunction including a complete type of Fanconi's syndrome.- - - - - - - - - - ranking = 20.446049584691keywords = tubulointerstitial, tubulointerstitial nephritis, nephritis, interstitial nephritis, interstitial (Clic here for more details about this article) |
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