1/37. Effect of camostat mesilate on urinary protein excretion in three patients with advanced diabetic nephropathy.Effective treatment has not yet been established for patients with persistent proteinuria and hypoproteinemia related to advanced diabetic nephropathy. We report three patients with diabetic nephropathy presented with the nephrotic syndrome who showed a marked decrease in proteinuria following the administration of camostat mesilate, a protease inhibitor. Each patient was resistant to treatment with an angiotensin-converting enzyme (ACE) inhibitor and a platelet-aggregation inhibitor. Camostat mesilate, 600 mg/day, orally, caused a marked decrease in urinary protein excretion after the 7th consecutive day of drug administration. There were no serious adverse effects. Its mechanism of action in this respect is not known. Camostat mesilate thus merits clinical trials in the treatment of nephrotic syndrome related to diabetic nephropathy.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
2/37. Renal complications in patients with diabetes mellitus associated with an A to G mutation of mitochondrial dna at the 3243 position of leucine tRNA.The substitution of guanine for adenine at position 3243 of the leucine tRNA gene of mitochondrial dna was originally described in association with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes). diabetes mellitus associated with the mutation (mitochondrial diabetes) is a different phenotype from MELAS. We identified 11 patients with the mutation among 385 Japanese diabetic patients: two had MELAS and nine had mitochondrial diabetes. We present data on a male patient with mitochondrial diabetes who developed the nephrotic syndrome at the age of 23. light microscopy revealed mesangial expansion, PAS-positive deposits and segmental sclerosis in the glomeruli. Scattered mesangial electron-dense deposits and thickening of the basement membrane were found on electron microscopy, suggesting that diabetic glomerulosclerosis accompanied by focal glomerulosclerosis (FGS). Mitochondrial diabetes may pre-dispose patients to renal complications, including forms of glomerulonephritis, such as FGS.- - - - - - - - - - ranking = 0.5keywords = nephrotic syndrome (Clic here for more details about this article) |
3/37. Diabetic nephropathy accompanied by iodine-induced non-autoimmune primary hypothyroidism: two case reports.We reported 2 diabetic patients with nephrotic syndrome due to advanced diabetic nephropathy complicated by non-autoimmune primary hypothyroidism. hypothyroidism developed along with the anasarcous status. Histological examinations of the thyroid gland revealed almost normal thyroid follicles without lymphocytic infiltration. The amounts of thyroid hormone lost into the extravascular space such as in urine and ascites were not sufficient to cause hypothyroidism alone. Serum total iodine levels measured during the hypothyroidal state in both cases were definitely elevated, and the perchlorate discharge test of both cases showed positive discharge (24 and 34%, respectively). The thyroid functions normalized after iodine restriction in the first case and initiating hemodialysis in the second case, in parallel with normalization of serum total iodine levels. These findings suggest that impaired renal handling of iodine resulting in elevation of serum iodine levels, rather than an autoimmune mechanism or extravascular hormone loss, played a principal role in the development of primary hypothyroidism found in these 2 patients, probably through a prolonged Wolff-Chaikoff effect.- - - - - - - - - - ranking = 0.5keywords = nephrotic syndrome (Clic here for more details about this article) |
4/37. Spontaneous remission of the nephrotic syndrome in diabetic nephropathy.A 28 year old woman, with diabetes since age 18, had the nephrotic syndrome, hypertension and renal insufficiency. The initial renal biopsy specimen revealed diffuse glomerulosclerosis with early nodular changes. After an initial decline in renal function, her creatinine clearance progressively improved and has remained normal. Within 2 years she had a spontaneous remission of the nephrotic syndrome despite the presence of more pronounced nodular glomerular lesions. Although the renal hemodynamic functions were normal, certain tubular functions were impaired. Since we found no etiology for the nephrotic syndrome other than diabetic glomerulopathy, the complete remission of the nephrotic syndrome and improvement in renal function were very unusual events.- - - - - - - - - - ranking = 3.5keywords = nephrotic syndrome (Clic here for more details about this article) |
5/37. A patient with apolipoprotein e2 variant (Q187E) without lipoprotein glomerulopathy.Four apolipoprotein (apo) E variants have been reported to be associated with lipoprotein glomerulopathy (LPG), which is characterized by type III hyperlipoproteinemia (type III HLP) and proteinuria and frequently leads to nephrotic syndrome. We report the histologic findings in the kidneys of a type III HLP patient with an apo E variant, apo E Toranomon, in which the glutamine at residue 187 in apo E is substituted by glutamic acid (Q187E). The patient also had type 2 diabetes mellitus. No evidence of lipoprotein thrombi suggestive of LPG was detected, however, and the histologic diagnosis was diabetic nephrosclerosis. This unique case illustrates that not all apo E variants result in LPG, and the location of mutations in the apo E protein is one of the important determinants for the development of LPG.- - - - - - - - - - ranking = 0.5keywords = nephrotic syndrome (Clic here for more details about this article) |
6/37. Reversible acute renal failure and nephrotic syndrome in a Type 1 diabetic patient.nephrotic syndrome is a condition commonly associated with end-stage renal disease secondary to diabetic nephropathy. It is usually associated with long-standing renal insufficiency, microalbuminuria, and overt proteinuria. We present a diabetic patient with acute oliguric renal failure and nephrotic syndrome. At presentation, he had a serum creatinine of 2.3 mg/dl, blood urea nitrogen (BUN) of 69 mg/dl, urinary protein excretion of 10.5 g/24 h, serum albumin of 1.3 g/dl, and a urine output < 400 cc/24 h. A renal biopsy was done and the renal pathology was compatible with early diabetic nephropathy. Despite intense diuretic therapy, the patient's renal condition did not improve, and peritoneal dialysis was started several months after diagnosis. After 8 months of dialysis therapy, the patient's renal parameters and urinary output spontaneously restored to normal limits (serum creatinine was 1.1 mg/dl, urinary albumin excretion was 411 mg/24 h, serum albumin was 4.3 g/dl, and normal urine output) and dialysis was discontinued. His renal function did not deteriorate after discontinuation of dialysis. We conclude that this patient's reversible acute renal failure and nephrotic syndrome were associated with minimal change disease and not due to diabetic nephropathy.- - - - - - - - - - ranking = 3keywords = nephrotic syndrome (Clic here for more details about this article) |
7/37. Management of steroid-sensitive nephrotic syndrome in children with type 1 diabetes.Four children with steroid-sensitive nephrotic syndrome (SSNS) coexisting with type 1 diabetes are presented. This number is higher than expected according to the estimated prevalence rates for each disease separately. In three, diabetes preceded nephrotic syndrome (NS), and in one it developed almost simultaneously. None of the patients had hypertension or retinopathy. Two had a renal biopsy: in one it was compatible with minimal change histology (MCH), and the other had MCH and early diabetic nephropathy changes. In addition to the two presented here, in 11 of 12 previously reported cases with biopsy proven SSNS coexisting with type 1 diabetes, the biopsy showed MCH. In none was treatment influenced by biopsy results. However, our experience suggests that daily steroid taper allows easier glycaemic control than alternate day steroids. We conclude that the indications for a renal biopsy in nephrotic children with and without insulin-dependent diabetes mellitus (IDDM) should be similar.- - - - - - - - - - ranking = 3keywords = nephrotic syndrome (Clic here for more details about this article) |
8/37. A case of idiopathic nodular glomerulosclerosis mimicking diabetic glomerulosclerosis (Kimmelstiel-Wilson type).A case of idiopathic nodular glomerulosclerosis mimicking diabetic Kimmelstiel-Wilson glomerulopathy is reported. The patient was a 45-year-old man suffering from nephrotic syndrome. light and electron microscopy revealed diffuse and nodular glomerulosclerosis indistinguishable from diabetic nodular glomerulosclerosis. diabetes mellitus, however, had been excluded both by extensive clinical and by laboratory investigation. The differential diagnosis also included amyloidotic and non-amyloidotic fibrillary glomerulopathy, light chain glomerulopathy, collagen type iii disease, immunotactoid glomerulopathy, and the sclerosing variant of membranoproliferative glomerulonephritis. immunohistochemistry and ultrastructural investigations, however, excluded these entities, and the diagnosis of idiopathic nodular glomerulosclerosis was made. This variant has to be included in the differential diagnosis of nodular glomerulopathy associated with nephrotic syndrome.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
9/37. Diffuse diabetic glomerulosclerosis in a patient with impaired glucose tolerance: report on a patient who later develops diabetes mellitus.Diabetic glomerulosclerosis might be seen in diabetics but its presence in patients with impaired glucose tolerance is quite rare. A 31-year-old woman who was admitted to our department was diagnosed with hypertension, nephrotic syndrome and impaired glucose tolerance. Her renal biopsy was compatible with diabetic glomerulosclerosis. She developed overt diabetes mellitus (DM) after one year of impaired glucose tolerance. hypertension might have accelerated the progression of diabetic nephropathy.- - - - - - - - - - ranking = 0.5keywords = nephrotic syndrome (Clic here for more details about this article) |
10/37. Successful treatment of necrotizing fasciitis associated with diabetic nephropathy.A 50-year-old woman with a 15-year history of type 2 diabetes mellitus was admitted to our hospital due to high fever and a skin lesion with severe pain, swelling and a sensation of heat in the right thigh. Laboratory examination showed elevated c-reactive protein (CRP), thrombocytopenia, nephrotic syndrome and renal dysfunction. Her blood glucose level had been well controlled. streptococcus agalactiae was detected in both the skin lesion and blood culture, and pathological examination revealed neutrophil infiltration in the fascia and muscle layer. The patient was diagnosed with necrotizing fasciitis, septic shock and disseminated intravascular coagulation. A combination therapy of antibiotics and surgical debridement resulted in the improvement of symptoms as supported by laboratory findings, and the skin lesion also showed improvement. Although group A streptococcus is well known to be implicated in the pathogenesis of necrotizing fasciitis, only S. agalactiae, belonging to group B streptococcus, was isolated from the tissue and blood cultures in this case. Although this organism is not virulent and rarely causes a necrotizing fasciitis, both the superficial fascial layer and underlying muscle were affected in this case. There have been only a few reports of necrotizing fasciitis due to S. agalactiae in patients with diabetes mellitus. Although the blood glucose level was well controlled in our patient, this disease might be caused by other factors, including diminished sense of touch and pain, abnormality of microcirculation and hypogammaglobulinemia due to nephrotic syndrome.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
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