Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035078 (renal failure)
 31,970 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The nephropathy-XY gonadal dysgenesis syndrome in a 17-year-old phenotypical female with focal glomerulosclerosis was associated with renal failure in two sisters, one with crescentic glomerulonephritis at 27 months, and one with membranoproliferative glomerulonephritis at 10 years. Neither the propositus or the siblings had the distinctive mesangial sclerosis of nephropathy-XY dysgenesis, type 1 (Drash syndrome). The association of nephropathy-XY dysgenesis with familial nephritis of heterogeneous pathology suggests that nephropathy-XY dysgenesis, type 2, may relate to separate genetic loci for XY dysgenesis and glomerulopathy or reflect a loss of protection against familial renal disease when the Y chromosome is absent or defective.
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PMID:Nephropathy-gonadal dysgenesis, type 2: renal failure in three siblings with XY dysgenesis in one. 359 96

Of 240 adults with sickle cell anemia seen over 11 years, 12 had the nephrotic syndrome. In 9 (75%) the glomerular lesion, sickle glomerulopathy, consisted of mesangial expansion and basement membrane duplication. Six patients had type IV renal tubular acidosis. Four of the 9 Patients died within 24 months (17 +/- 5; mean +/- SD), while 5 survived 36 months or longer (80 +/- 49); no significant differences were seen between the former and the latter in age, admission serum creatinine and C3 levels, urinary protein excretion, or the frequency of renal tubular acidosis. Chronic azotemia developed in 3 and acute renal shutdown in another 2. Of 22 patients with sickle glomerulopathy (our 9 added to 13 from the literature) 11 died within 2 years. Ten of these (91%) had developed renal failure, compared to only 5 of the 11 (45%) who survived longer than 2 years (p less than 0.05). The 5-year mortality in the general population of sickle cell anemia is 3.75%, and 75% of patients aged 15 years or older survive 18 years or longer. The nephrotic syndrome, most often caused by sickle glomerulopathy, occurs in 4% of patients with sickle cell anemia, leading to renal failure in two-thirds and death in 2 years in half the patients. The development of chronic azotemia correlates strongly with early mortality. The prognosis is much worse than that in the general population of sickle cell anemia.
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PMID:Prognosis of the nephrotic syndrome in sickle glomerulopathy. A retrospective study. 360 31

Human hereditary nephritis refers to familial glomerular diseases which may progress to renal failure. Samoyed hereditary glomerulopathy has been shown previously to be a model for hereditary nephritis. Clinical and laboratory studies were performed to follow progression to renal failure in 44 dogs in a family with Samoyed hereditary glomerulopathy. Affected males appeared healthy for their first three months but then became progressively wasted. Proteinuria was detected between two to three months of age; after five months, urine protein electrophoresis showed pre-albumin, albumin and alpha and beta globulin peaks. From three months onward, a reduced glomerular filtration rate was detected. Serum albumin decreased while amylase, urea, creatinine and phosphate increased from four to five months of age. Death from renal failure occurred by 15 months. Carrier females also became thinner and developed proteinuria between two and three months of age, but neither renal failure nor death ensured. Hence, SHG progressed rapidly in affected males but not in carrier females.
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PMID:Samoyed hereditary glomerulopathy: serial, clinical and laboratory (urine, serum biochemistry and hematology) studies. 365 95

The kidney as a target organ for secondary microvascular complications of diabetes mellitus represents a major problem. The pathology of diabetic glomerulopathy is well known. The coexistence of immunocomplex-mediated glomerulonephritis and diabetes mellitus has rarely been reported. The presence of crescents in glomerular disease of diabetes mellitus has been usually ignored in the literature. The present study describes one patient with epithelial crescentic diabetic glomerulopathy with rapidly progressive renal failure.
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PMID:Epithelial crescent in diabetic glomeruli. A case report. 366 43

The pedigree of a line of Samoyed dogs with Samoyed hereditary glomerulopathy (SHG) was investigated to determine the mode of inheritance. Sixty percent of males were affected with severe renal disease that progressed to renal failure before 15 months of age. In contrast, female carriers showed less severe involvement and their disease did not progress to renal failure. This pattern is consistent with the inheritance of an X-linked dominant gene. A similar mode of inheritance has been postulated in some families with hereditary nephritis. Further similarities between SHG in dogs and hereditary nephritis in humans have been demonstrated previously in clinical and renal morphologic studies. The present study supports the view that this line of Samoyed dogs would be an appropriate model for studying the human disease.
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PMID:Mode of inheritance of Samoyed hereditary glomerulopathy: an animal model for hereditary nephritis in humans. 371 21

Outcome in 425 patients with persistent proteinuria has been assessed over a period of five to 20 years. Nephrotics and non-nephrotics are analysed separately. Clinical and laboratory findings do not correlate with renal histology. Seventy-eight patients had minimal histological lesions and four died (only one in renal failure). Fifty-one patients had endothelial cell proliferation, and 20-year survival was 70 per cent; renal failure occurred within five years in all 17 who progressed, and was independent of nephrotic status. In both membrano-proliferative disease (98 patients) and membranous glomerulopathy (59 patients) 20-year survival was 20 to 30 per cent. Epithelial cell proliferation (85 patients) had the worst prognosis, and survival after 12 years was negligible. In these last three groups decay in survival was almost linear against time and independent of nephrotic status. In focal proliferative disease 20-year survival in nephrotics (31 patients) was only 30 per cent, contrasting with non-nephrotics (23 patients) with 80 per cent survival. Of 105 patients presenting with proteinuria without symptoms, in 30 the proteinuria was postural and biopsies were not done. The other 75 showed a range of histological changes and prognosis similar to the group with symptoms.
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PMID:Proteinuric glomerular disease in adults: cumulative life tables over twenty years. 374 49

Samoyed hereditary glomerulopathy (SHG) in dogs resembles hereditary nephritis (HN) in man. Affected males and carrier females spontaneously develop proteinuria, but only males progress to renal failure. We examined the evolution of splitting of glomerular capillary basement membranes (GCBM) in affected male and carrier female dogs. At birth, examination by light microscopy (LM) in all dogs showed normal glomerular capillaries, but by electron microscopy (EM), many GCBM showed separation of endothelial from visceral epithelial cell basement membranes. By LM, glomerular capillaries of affected males appeared normal for up to 4 months, but then isolated and eventually all capillaries became thickened and split. However, by EM, a bilaminar appearance of the GCBM was seen within 1 month which evolved into multilaminar splitting that became extensive as renal failure ensued. Carrier females showed mild nonprogressive changes in GCBM. These results with SHG permitted speculation on the evolution of splitting of GCBM in human HN.
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PMID:Samoyed hereditary glomerulopathy (SHG). Evolution of splitting of glomerular capillary basement membranes. 379 18

During an evaluation for nephrotic syndrome, a 20-year-old woman was found by ultrasonographic examination to have large kidneys with multiple renal cysts suggestive of polycystic kidney disease. A subsequent renal biopsy revealed membranous glomerulopathy due to systemic lupus erythematosus, as well as the unexpected finding of glomerulocystic kidney disease (GCKD), an uncommon disorder previously reported to occur primarily in infants and children. No evidence of renal dysplasia was present and no cysts were found in any abdominal or pelvic organs. Other than one bifid renal pelvis, no significant congenital anomalies or structural chromosomal abnormalities were present. Ultrasonographic evaluation of the patient's family revealed similar-appearing cortical cysts in several members, all of whom had no clinical evidence of renal dysfunction. The pattern of involvement was compatible with autosomal dominant inheritance. Follow-up ultrasonograms of the patient and affected family members 1 year after the initial study showed enlargement of the cysts with development of additional cysts in two individuals and no change in the other family members. Although renal failure was present and progressed in our patient, renal function remained normal in all affected family members 1 year after detection of the renal cysts. This patient and her family provide additional insight into the inheritance and natural history of GCKD and demonstrate that this condition should be considered in the evaluation of multicystic renal disease in adults. In contrast to several previously reported cases, it appears that GCKD may be associated with normal renal function for many years.
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PMID:Familial adult glomerulocystic kidney disease. 382 63

Renal biopsy was performed in 20 graft recipients to characterise the histological features associated with poor renal function concomitant with cytomegalovirus infection (CMV). Eight patients presented with proteinuria, three had microscopic haematuria at onset, and five were hypertensive. Infection was accompanied by clinical symptoms (fever, leucopenia, mild hepatic damage, or pneumonitis) in 15 patients. In all cases, serum creatinine was greater than 2 mg/dl. All patients showed some glomerular alteration on biopsy, and vascular changes were the predominant feature in seven cases. IgM and complement (C3) were found in the glomeruli of five of six patients studied by immunofluorescence. Serum creatinine was below 2 mg/dl at ten to 26 months following the infectious episode in four patients and between 2-3 mg/dl in three patients. The remaining 13 developed irreversible rejection and end-stage renal failure. We conclude that CMV, the most commonly recognised viral infection following transplantation, can cause renal changes, both glomerular (CMV glomerulopathy) and vascular (transplant vasculopathy), which may induce poor graft function.
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PMID:Renal changes in cytomegalovirus infection. 630 1

The earliest manifestations of clinical diabetic nephropathy, including proteinuria, hypertension, and declining GFR, represent very advanced diabetic glomerulopathy with especially prominent mesangial expansion. Mesangial expansion, by restricting glomerular capillary filtration surface and lumenal volume, stimulates compensatory mechanisms analogous to those resulting from a marked reduction in nephron number. These compensatory mechanisms involve alterations in glomerular hemodynamics designed to maintain glomerular filtration but which ultimately injure the kidney. These hemodynamic perturbations are not specific to diabetes but represent a final common pathway toward endstage renal failure that also characterizes the remnant kidney. This thesis concludes that the onset of clinical diabetic nephropathy augurs inevitable decline in kidney function, and that only studies and interventions exercised before clinical nephropathy develops can influence understanding and outcome of diabetic nephropathy.
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PMID:Diabetic nephropathy. A perspective. 640 Jun 68


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