Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011881 (diabetic nephropathy)
 10,836 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Camostat mesilate, a developed derivative of gabexate mesilate for oral use, was administered in a daily dose of 600 mg for 4 weeks to 17 patients with heavy proteinuria due to various nephropathies. Five patients had glomerulonephritis (3 patients with IgA nephropathy, one each with membranoproliferative GN and membranous nephropathy) and 3 had systemic vasculitis. These patients had been treated with glucocorticoid, cyclophosphamide, anticoagulants, and dipyridamole. Five patients had diabetic nephropathy and had been treated with conventional therapy including angiotensin converting enzyme inhibitors. Two cases with benign nephrosclerosis, one with Alport syndrome, and the rest with end-stage renal failure of undetermined cause were also included in this study. Urinary protein decreased promptly within 2 weeks (from 5.2 +/- 0.7 to 3.5 +/- 0.5, mean +/- SE, p less than 0.005), and serum total protein and albumin levels increased significantly. Serum creatinine levels did not change. Decreases in urinary protein excretion of more than 50% were observed in five out of eight patients with glomerulonephritis or systemic vasculitis, two out of five with diabetic nephropathy, and one with chronic renal failure. However, urinary protein excretion values remained at the same level in two patients with benign nephrosclerosis and a patient with Alport syndrome. We suggest that camostat mesilate caused a change in glomerular capillary permeability for macromolecules through its inhibitory effects on the kallikrein-kinin system, complement system, coagulation system, and platelet function, which contributed to the treatment of the various nephropathies.
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PMID:Effect of camostat mesilate on heavy proteinuria in various nephropathies. 279 62

Virtually all diseases affecting the native kidney recur in the kidney transplant with the exception of Alport syndrome, polycystic kidney disease, hypertension, chronic pyelonephritis, and chronic interstitial nephritis. Fortunately, in the majority of patients, recurrence of the original disease has minimal clinical impact, with only approximately 5% of all graft loss occurring as a result of recurrent disease. The primary renal diseases that commonly recur include membranoproliferative glomerulonephritis type II, IgA nephropathy, and focal and segmental glomerular sclerosis. The most common systemic disease that recurs is diabetic nephropathy. Living-related transplantation should be used with caution in patients with the hemolytic uremic syndrome, recurrent focal and segmental glomerular sclerosis, and membraneous glomerulonephritis. Fabry disease and primary hyperoxaluria type I are no longer absolute contraindications to kidney transplantation.
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PMID:Recurrent diseases in the kidney transplant. 802 19

Recent progress in molecular genetics has strongly influenced nephrology. Gene mutations have been identified which cause various monogenic hereditary renal diseases including Alport syndrome, autosomal dominant polycystic kidney disease, and tubular transporter disorders. The data obtained will be useful not only to develop new methods of diagnosis and treatment of such particular diseases but also to expand the knowledge of renal physiology and pathophysiology. In addition, genetic factors have been investigated which are involved in the development and progression of more common renal diseases such as IgA nephropathy and diabetic nephropathy. There have also been great advances in molecular studies of experimental renal diseases such as Heymann nephritis and in the use of transgenic and knockout mice. In this review we focused on the important achievements made recently in the field of molecular nephrology.
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PMID:Molecular genetics of renal diseases. 909 87

Gene therapy has the potential to provide a therapeutic strategy for numerous renal diseases such as diabetic nephropathy, chronic rejection, Alport syndrome, polycystic kidney disease, and inherited tubular disorders. In previous studies using cationic liposomes or adenoviral or retroviral vectors to deliver genes into the kidney, transgene expression has been transient and often associated with adverse host immune responses, particularly with the use of adenoviral vectors. The unique properties of recombinant adeno-associated viral (rAAV) vectors permit long-term stable transgene expression with a relatively low host immune response. The purpose of the present study was to evaluate gene expression in the rat kidney after intrarenal arterial infusion of a rAAV (serotype 2) vector encoding green fluorescence protein (GFP) induced by a cytomegalovirus-chicken beta-actin hybrid promoter. The left kidney of experimental animals was treated with either saline or transduced with rAAV2-GFP (0.125 ml/100 g body wt, 1 x 10(10)/ml infectious units) through the renal artery. A time-dependent expression of GFP was observed in all kidneys injected with rAAV2-GFP, with maximal expression observed at 6 wk posttransduction. The expression of GFP was restricted to cells in the S(3) segment of the proximal tubule and intercalated cells in the collecting duct, the latter identified by co-localization with H(+)-ATPase. No transduction was observed in the glomeruli or the intrarenal vasculature. These studies demonstrate successful transgene expression in tubular epithelial cells, specifically in the S(3) segment of the proximal tubule and intercalated cells, after intrarenal administration of a rAAV vector and provide the impetus for further studies to exploit its use as a tool for gene therapy in the kidney.
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PMID:Gene delivery in renal tubular epithelial cells using recombinant adeno-associated viral vectors. 1266 Mar 29

Nonimmune glomerulopathies are an area of significant research. This review discusses the development of focal segmental glomerulosclerosis, with particular attention to the role of the podocyte in the initiation of glomerulosclerosis and the contribution to glomerulosclerosis from capillary hypertension and soluble factors such as transforming growth factor beta, platelet-derived growth factor, vascular endothelial growth factor, and angiotensin. The effects of these factors on endothelial and mesangial cells are also discussed. In addition, we review our current understanding of the slit diaphragm (a specialized cell junction found in the kidney), slit diaphragm-associated proteins (including nephrin, podocin, alpha-actinin-4, CD2-associated protein, and transient receptor potential channel 6), and the role of these proteins in glomerular disease. We also discuss the most recent research on the pathogenesis of collapsing glomerulosclerosis, human immunodeficiency virus associated nephropathy, Denys-Drash, diabetic nephropathy, Alport syndrome, and other diseases related to the interaction between the podocyte and the glomerular basement membrane.
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PMID:Pathogenesis of nonimmune glomerulopathies. 1803 19

A 38-year-old man with mild sensorineural hearing loss, diabetes mellitus, proteinuria, and slight renal dysfunction was admitted to our hospital for a renal biopsy to determine the cause of kidney disease. His elder sister and mother also had sensorineural hearing loss and renal failure, suggesting the existence of a common genetic disease in this family. Although the clinical features of the patient were similar to features of Alport syndrome, renal biopsy revealed no sign of Alport syndrome. We next considered a possibility of a mitochondrial kidney disease described by Jansen in 1997. Indeed, genetic analysis of mitochondrial DNA clarified the existence of A3243G mutation in the patient and his sister. This syndrome should be recognized by nephrologists as a differential diagnosis of Alport syndrome, diabetic nephropathy, and primary glomerular diseases.
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PMID:A familial case of mitochondrial disease resembling Alport syndrome. 1818 Aug 72

Reversible posterior leukoencephalopathy syndrome (RPLS) is one of the important side effects of calcineurin inhibitors (CNIs). Magnetic resonance imaging (MRI) of the brain is useful for the diagnosis of RPLS, showing the edema primarily in the cortex and subcortical white matter of the posterior brain regions. Interruption of CNIs is essential for the treatment of patients with RPLS. Herein we have described 2 cases (1.7%) of RPLS induced by CNIs after kidney transplantation. The first case was a 56-year-old man with chronic renal failure due to diabetic nephropathy who received a living-related kidney transplantation in 2006. Initial immunosuppressive therapy consisted of cyclosporine, mycophenolate mofetil (MMF), prednisolone, and basiliximab. Four months after transplantation, he developed unconsciousness and paralysis. The second case was a 24-year-old woman with end-stage renal disease due to Alport syndrome who received an ABO-incompatible living-related kidney transplantation. Initial immunosuppressive therapy consisted of tacrolimus, MMF, prednisolone, and basiliximab. On postoperative day 3, she developed convulsions and unconsciousness. In both patients, RPLS was diagnosed with neurological symptoms and MRI findings at early stage of the disease, and they recovered rapidly from the disease by the interruption of CNIs. Our data demonstrated that early diagnosis and immediate interruption of CNIs were essential for the treatment of RPLS after kidney transplantation.
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PMID:Two cases of calcineurin inhibitor-associated reversible posterior leukoencephalopathy syndrome in renal transplant recipients. 1879 Feb 53

Certain renal diseases are characterized by alterations in the thickness of the glomerular basement membrane (GBM), as visualized by images of biopsy samples obtained by using a transmission electron microscope (TEM). Abnormal thinning, thickening, or variation in thickness can occur in familial hematuria, diabetes mellitus, and Alport syndrome, respectively. We propose image processing methods for the segmentation and measurement of the GBM. The methods include the split and merge algorithm, morphological image processing, skeletonization, and statistical analysis of the width of the GBM. The proposed methods were applied to 34 TEM images of six patients. The mean and standard deviation of normal GBM were estimated to be 368 +/- 177 nm; those of thin GBMs associated with familial hematuria were 216 +/- 95 nm; and those of thick GBM due to diabetic nephropathy were 1094 +/- 361 nm. Comparative analysis of the results of image processing with manual measurements by an experienced renal pathologist indicated low error in the range of 12 +/- 9 nm.
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PMID:Segmentation and analysis of the glomerular basement membrane using the split and merge method. 1916 53

Abnormal thinning, thickening, or variation in the thickness of the glomerular basement membrane (GBM) are caused by familial hematuria, diabetes mellitus, and Alport syndrome, respectively. We propose a semi-automated procedure for the segmentation and analysis of the thickness of the GBM in images of renal biopsy samples obtained by using a transmission electron microscope (TEM). The procedure includes the split-and-merge algorithm, morphological image processing, skeletonization, and statistical analysis of the width of the GBM. The procedure was tested with 34 TEM images of six patients. The mean and standard deviation of the GBM width for a patient with normal GBM were estimated to be 368 +/- 177 nm, those for a patient with thin GBM associated with familial hematuria were 216 +/- 95 nm, and those for a patient with thick GBM due to diabetic nephropathy were 1,094 +/- 361 nm. Comparative analysis of the results of image processing with manual measurements by an experienced renal pathologist indicated low error in the range of 12 +/- 9 nm.
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PMID:Analysis of the glomerular basement membrane in images of renal biopsies using the split-and-merge method: a pilot study. 1976 Feb 93

Chronic kidney disease is common with up to 5% of the adult population reported to have an estimated glomerular filtration rate of < 60 ml/min/1.73 m(2). A large number of pathogenic mutations have been identified that are responsible for 'single gene' renal disorders, such as autosomal dominant polycystic kidney disease and X-linked Alport syndrome. These single gene disorders account for < 15% of the burden of end-stage renal disease that requires dialysis or kidney transplantation. It has proved more difficult to identify the genetic susceptibility underlying common, complex, multifactorial kidney conditions, such as diabetic nephropathy and hypertensive nephrosclerosis. This review describes success to date and explores strategies currently employed in defining the genetic basis for a number of renal disorders. The complementary use of linkage studies, candidate gene and genome-wide association analyses are described and a collation of renal genetic resources highlighted.
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PMID:Unravelling the genetic basis of renal diseases; from single gene to multifactorial disorders. 1988 76


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