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)

Graft membranous nephropathy (MN) appears mainly de novo or, less frequently, develops in patients whose original disease was MN. The rarity of the latter occurrence contrasts with the frequency of MN as the original disease: the existence of renal recurrence may thus be questioned. We report a patient with terminal renal failure due to focal glomerulosclerosis; typical MN developed de novo in the first and recurred in the third graft. This observation establishes that recurrence of MN is a real phenomenon and demonstrates that the factor(s) determining recurrence may appear only after transplantation. Neither HBs nor antilymphocyte serum antigens were found along the basement membrane. The late onset of proteinuria after the third demonstrates that the delayed appearance of clinical signs of glomerular disease does not rule out the occurrence of MN.
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PMID:Recurrence of de novo graft membranous glomerulonephritis. 703 84

Anatomical and immunological studies were performed in two brothers with membranous glomerulonephritis. The older child presented with renal failure, Fanconi syndrome and anti-TBM antibody in his plasma. Renal biopsy revealed severe tubulo-interstitial disease with membranous glomerulonephritis. Because of rapidly progressive renal insufficiency the patient was started on hemodialysis. A cadaver renal allotransplantation was performed without success. Renal transplant biopsy showed severe lesions of vascular rejection without recurrence of the primary disease. The younger child was examined at six months for a nephrotic syndrome with mild renal insufficiency, and died at nine months. Anti TBM antibodies were not detected in his serum. The patient's mother presented with asymptomatic proteinuria, and anti TBM antibodies in her plasma. In the same family the uncle died at 3 months with a steroid resistant nephrotic syndrome. The relationships between the familial membranous glomerulonephritis and the tubulo interstitial disease are discussed.
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PMID:[Membranous glomerulonephritis in two brothers associated in one with tubulo-interstitial disease, Fanconi syndrome and anti-TBM antibodies (author's transl)]. 704 46

We report a 20-year-old patient with arteriohepatic dysplasia (Alagille's syndrome) who developed progressive renal failure associated with extensive renal lipidosis. A renal biopsy showed diffuse thickening of glomerular basement membrane mimicking idiopathic membranous glomerulonephritis on light microscopy. Electron microscopy, however, demonstrated numerous intramembranous and mesangial lipid deposits, quite similar to those described in familial lecithin cholesterol acyltransferase deficiency. We believe the renal lipidosis developed secondary to hyperlipidemia associated with longstanding intrahepatic cholestasis. This case illustrates that extensive lipid deposition in the glomerular basement membrane may occur in patients with arteriohepatic dysplasia, and it may lead to progressive renal failure.
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PMID:Renal lipidosis associated with arteriohepatic dysplasia (Alagille's syndrome). 715 49

Thirty-one patients who developed nephrotic syndrome when greater than 60 yr of age and who underwent renal biopsy are presented. In this group of the patients the most common histologic diagnosis was membranous nephropathy (52%); other diagnoses were amyloidosis (16%), end-stage renal disease (13%), chronic glomerulonephritis (9%), minimal change disease (6%), and lupus nephritis (3%). The prognosis in all these patients was poor; most of them died or were in terminal renal failure during the following-up period.
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PMID:Nephrotic syndrome in the elderly. 717 67

Two hundred and two children with primary nephrotic syndrome were studied. Clinical features at onset and evolution were studied and correlated with microscopic findings and response to treatment. Histologic type and steroid-response were correlated with urinary protein selectivity in a group of them. Mean follow-up (by the same group of nephrologists) was 6.6 years; 22% were followed for over 8 years. The presence of hematuria, hypertension and renal failure at onset and during the course of the disease were evaluated. 122 patients were steroid-responders, 64 of them had frequent relapses. After a mean follow-up of 6.3 years, 81% were on remission; 14% had transient proteinuria, and 5% developed persistent proteinuria with late resistance to steroid therapy. 80 children were steroid-resistant. Their mean follow-up period was 5.5 years (1.0-10.5 years). 65 of them were biopsied: 12 had minimal glomerular change; 27 focal and segmental sclerosis. 9 membrano-proliferative glomerulonephritis, 4 focal and global sclerosis, 4 membranous nephropathy, 4 diffuse mesangial proliferation, 2 diffuse mesangial sclerosis and 2 were not classifiable. Hematuria had a prognostic value only when it was permanent or macroscopic. Urinary protein selectivity was measured in 43 patients, and C3 in 81; hypocomplementemia was found only in the 9 children with membranoproliferative glomerulonephritis. 8 children had tubular dysfunction: 7 had focal and segmental sclerosis, and 1 diffuse mesangial sclerosis; glycosuria and aminoaciduria were present in all 8, but only 4 of them had deficient urinary acidification. Secondary infections appeared in 42 patients; 19 were steroid-resistant and 23 steroid-responders, 17 of them showed frequent-relapses, 3 had localized thromboembolism. 23 children went into chronic renal failure and 17 of them died, 13 in end-stage renal failure and 4 because of complications. Their first biopsies showed minimal change in 1, focal and segmental sclerosis in 8, membranoproliferative glomerulonephritis in 3, diffuse mesangial proliferation in 1, diffuse sclerosis in 2, and 2 were not classifiable.
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PMID:[Primary nephrotic syndrome. Follow-up of 202 pediatric patients]. 727 77

Thirty-five consecutive patients with renal biopsy appearances of idiopathic membranous nephropathy, who were initially studied between 1960 and 1969, were reevaluated in 1979 to determine (a) the long-term outcome of idiopathic membranous nephropathy, and (b) whether any clinical or biopsy features at onset would permit prediction of long-term outcome. After fifteen years, actuarial analysis showed that approximately half the patients would be in remission; the rest dead or on dialysis/transplantation programs. Entry into renal failure, in those who ran this course, started at three years after diagnosis and continued steadily for at least another ten years. Likewise, remission was seen as early as one year from onset, but patients were still going into remission after thirteen years of proteinuria. No feature in the glomeruli predicted outcome, but interstitial and vascular changes were seen more frequently in those whose disease persisted or worsened. No onset feature predicted a poor outcome reliably, but younger patients did better than older, and all three children with initial macroscopic hematuria went into remission.
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PMID:The long-term outcome of idiopathic membranous nephropathy. 727 92

Renal involvement in sarcoidosis has recently been emphasized with increasing reports of associated glomerulonephritis. We report in detail a further case of mesangiocapillary glomerulonephritis in association with sarcoidosis. In addition, a review of 75 cases of sarcoidosis at Royal Melbourne Hospital over a 10-year period revealed one case of membranous glomerulonephritis and one case of granulomatous interstitial nephritis. Seven patients in the series had nephrocalcinosis, and eight further patients had abnormalities of urinary sediment of clinical symptoms of renal involvement. These findings suggest that, though renal failure is rare, renal involvement in sarcoidosis is more frequent than has previously been reported.
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PMID:Renal disease and sarcoidosis. 727 72

Forty patients with systemic lupus erythematosus have been evaluated for clinicopathological evidence of renal involvement. Average age at the onset of the disease was 23 years; 90 percent of the patients were women. Indications for renal biopsy included clinical symptoms of nephropathy (34 patients) and signs of immunological activity (6 patients(. All biopsied specimens were examined under light microscope; 28 of them under immunofluorescence, and 18 under electron microscope. Histological classification was as follows: I) Normal kidney (2 patients); II) mesangial glomerulonephritis (6 patients); III) focal glomerulonephritis (10 patients); IV) diffuse proliferative glomerulonephritis (19 patients), and V) membranous glomerulonephritis (3 patients). Six patients had no clinical nephritis, two of them had normal kidneys and four had a mesangial glomerulonephritis. Patients in groups III, IV, and V showed clinical and biological evidence of renal involvement. Highest incidence of renal failure, nephrotic syndrome or high blood pressure was found among cases in group IV. Group IV accounted also for the highest mortality rate. Clinical course of 25 cases (1 of type II, 8 or type III, and 16 of type IV) on corticosteroid therapy for an average follow-up of 22.8 months has showed greater clinical improvement (proteinuria and renal function) of nephropathy type III than nephropathy type IV. Renal biopsy is a useful diagnostic and prognostic method in patients with systemic lupus erythematosus, even in those cases without clinical evidence of renal disease.
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PMID:[Lupus nephritis: clinicomorphological manifestations (author's transl)]. 739 5

Three adults with diabetes mellitus and nephrotic syndrome of recent onset underwent renal biopsies because of certain unusual clinical features. Despite heavy proteinuria, one patient had no evidence of diabetic retinopathy, one had preservation of normal renal function, while the third had sudden onset of renal failure. Microscopic examination of renal biopsy material disclosed pure membranous nephropathy in one, membranous nephropathy and nodular glomerulosclerosis in two. Because of significant differences in the natural history of these two glomerulopathies and the possible beneficial effects of steroid therapy in membranous nephropathy, we suggest that renal biopsies be performed in diabetic patients having persistent hematuria, sudden onset of renal failure, massive proteinuria without azotemia, retinopathy, or other evidence of microvascular disease, to uncover superimposed and treatable disorders that may influence the course of renal disease.
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PMID:Idiopathic membranous glomerulonephritis in diabetic patients: report of three cases and review of the literature. 739 88

Studies and textbooks from the 1970s and early 1980s list focal-segmental glomerulosclerosis (FSGS) as accounting for 10% to 15% of cases of idiopathic nephrotic syndrome in adults, although a recent review by D'Agati (Kidney Int 46:1223-1241, 1994) reported an approximately sevenfold increase in the incidence of FSGS from 1974 to 1993 in an active renal biopsy practice. To investigate possible changes in the incidence of FSGS in our renal biopsy practice, we reviewed reports from all nontransplant, adult (> or = 18 years) renal biopsies received in our laboratory from 1974 to 1993, which comprised 7,420 cases. All diagnoses of membranous nephropathy (MN), minimal change nephropathy (MCN), and FSGS made in each year were compiled; cases clearly or suspicious of being secondary to an underlying systemic disease, glomerulonephritis, or drug reaction were excluded. Relative frequencies of MN, MCN, and FSGS among these three diseases and among all biopsies were calculated for each year of the study. Regression analysis showed a significant (P < 0.001) increase in the odds of a diagnosis of FSGS over the study period: 7.6% per year among all biopsies and 6.8% per year among cases of MN, MCN, and FSGS only. Among all biopsies, the yearly incidence of FSGS increased from 4.0% +/- 0.6% (mean +/- SD) during the period between 1974 and 1979 to 12.2% +/- 2.0% during the period from 1987 to 1993. The odds of a diagnosis of MN (mean yearly incidence, 9.5% +/- 1.9%) did not vary significantly over the study period while the odds of a diagnosis of MCN (mean yearly incidence, 4.0% +/- 1.2%) declined at a rate of 2.2% per year (P < 0.03). Frequencies of diagnosis of MN, MCN, and FSGS by two pathologists were almost identical. Review of available slides from cases of FSGS revealed 21 (none before 1980) with characteristic histologic features of the collapsing glomerulopathy (CG) variant of FSGS. No more than four cases of CG were observed in any year of the study, and CG accounted for 4.7% of total FSGS cases for which diagnostic slides were available. Compared with 42 patients with non-CG FSGS, the CG cohort showed a greater percentage of black patients (86% v 38%), significantly higher mean levels of serum creatinine (3.8 +/- 2.7 mg/dL v 1.9 +/- 1.5 mg/dL) and urinary protein (14.3 +/- 9.6 g/24 hr v 7.7 +/- 5.8 g/24 hr) at the time of renal biopsy, and a greater likelihood of and more rapid progression to end-stage renal failure.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Increasing incidence of focal-segmental glomerulosclerosis among adult nephropathies: a 20-year renal biopsy study. 748 26


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