Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The term "renal osteodystrophy" is used to include skeletal disorders of patients with chronic renal failure: osteitis fibrosa, osteomalacia, osteosclerosis, osteoporosis and the frequently associated extraskeletal calcifications. It is the chronic glomerular disease with phosphate retention and resultant hyperphosphatemia on one hand and deficient 1,25 (OH)2 D3 and resultant hypocalcemia on the other to induce secondary hyperparathyroidism. The three most common causes of chronic renal failure in our patients are chronic glomerulonephritis, diabetic nephropathy, hypertensive nephropathy in decreasing frequency, polycystic renal disease occurs in five patients. Other miscellaneous causes include nephrotic syndrome, chronic pyelonephritis, systemic lupus erythematosus, periarteritis nodosa, interstitial nephritis and renal stones. The bone changes are similar in primary and secondary hyperparathyroidism and the incidence of brown tumor is about 3% in the former and 1.5 to 1.7% in the latter. We present one among the 94 dialyzed patients who has long-standing severe chronic renal failure from polycystic kidney disease and develops brown tumor in the mid ulna after 7 years on maintenance hemodialysis. The incidence of brown tumor in our series is about 1.1%. Because of increased longevity of the dialyzed patients, brown tumor from secondary hyperparathyroidism is now more commonly observed. Hyperphosphatemia with serum calcium-phosphate products exceeding plasma solubility of 60 to 75 mg/dl may induce soft tissue and vascular calcification. This explains the much higher incidence of soft tissue calcification in secondary than primary hyperparathyroidism; two of our patients with generalized Monckeberg's type arterial calcification and multiple periarticular calcifications in five patients have been observed.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Renal osteodystrophy. 164 77

The clinical features of eight patients, four females, aged 4 to 15 years under chronic hemodialysis for terminal renal failure (creatinine clearance 10 ml.min.1,73 m2 or less) are reported. Initial diseases were Alport syndrome, systemic lupus erythematosus, chronic glomerulonephritis (n = 2), bilateral polycystic kidney, prune belly syndrome and reflux nephropathy (n = 2). Distal vascular approach by means of arteriovenous fistulas was preferred for these patients and the kinetic urea model was used to evaluate the performance of the procedure. Patients required nine to twelve hours of hemodialysis per week for optimal results. Mean weight decreases of 1 to 3 kg and reductions in blood urea nitrogen and serum potassium of 40 mg.dl and 2,5 mEq.1, respectively, were observed. The main complications of hemodialysis were the disequilibrium syndrome, infections at the site of insertion of the arteriovenous fistulae and congestive heart failure. Three patients were submitted to renal transplantation with live donors homografts: one died and the other two remain alive but under chronic hemodialysis. Five children are attending school regularly, and two of them are waiting a kidney donor for transplantation. Despite encouraging results chronic hemodialysis in children constitutes only primary supportive therapy prior to renal transplantation.
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PMID:[Chronic hemodialysis in children]. 208 91

Factor D depleted serum did not support hemolysis of rabbit erythrocytes and solubilization of performed immune complexes. Fluid phase C3 cleavage increased in a dose dependent manner when D protein was added to normal or to factor D depleted serum. During short incubation factor D concentrations were correlated with the capacity of serum to solubilize immune complexes and to lyse rabbit erythrocytes. With prolonged incubation, the hemolytic activity decreased in a factor D dose dependent manner. This was probably due to fluid phase breakdown of C3 and factor B in the presence of high factor D concentrations. Hypocomplementemic sera from patients with active systemic lupus erythematosus (SLE) did not support solubilization of bovine serum albumin (BSA) anti-BSA complexes when factor D was added in excess. Patients with polycystic kidney disease with reduced renal function and high factor D concentrations showed increased concentrations of circulating C3d/dg fragments. The possibility was considered that high factor D concentrations in uremia might promote fluid phase C3 degradation and thereby limit the in vivo efficiency of alternative pathway activation on target surfaces.
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PMID:Influence of factor D concentrations on fluid phase C3 activation, lysis of rabbit erythrocytes and solubilization of immune complexes. 271 46

1. The influence that a patient's original disease had on the outcome of kidney transplantation was small when recent transplants (1983 to present) were examined. A difference of only 5% was seen in one-year graft survivals of first cadaver donors. The average one-year survival was 70%; patients with pylonephritis having the best (75%) and SLE patients the worst (65%) survival rates among the major disease categories. 2. There was little difference observed in patient survivals or functional graft survivals in first cadaver transplants among patients with different original diseases. This was also the case with cadaver regrafts; with the exception of polycystic kidney patients who had quite poor graft survival (41% at one year) with regrafts. Pretransplant blood transfusions resulted in increased cadaver graft survival; however the low number of nontransfused patients in many disease categories presented difficulties in examining this effect. 3. The use of cyclosporine resulted in increased cadaver graft survival in all disease categories. One-year cadaver graft survival of cyclosporine-treated patients averaged 75% compared to 63% with noncyclosporine-treated patients. A beneficial effect of cyclosporine use was not consistently seen in transplants involving living-related donors, although perhaps larger numbers of patients are required in order to confirm this observation. 4. Graft survival in diabetic patients was quite good in this analysis of recent transplants. First cadaver one-year graft survival in diabetics was only 4% below the overall average and 2% less with cyclosporine. Patients with both juvenile (75% one-year graft survival) and adult-onset (78% one-year graft survival) forms of the disease had good graft survival when cyclosporine was used for immunosuppression.
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PMID:Original disease of the recipient. 315 24

The number of hospitalized renal patients and the number of days they stayed in hospitals were studied in Finland 1969-1982. In 1969 3.3% and in 1982 1.7% of all bed-days available were occupied by renal patients. The number of hospitalized renal patients first decreased and after 1976 no notable changes were observed. A rising trend in the number of patients as well as in the corresponding bed-days was seen in diabetic nephropathy, lupus nephropathy, renal amyloidosis and polycystic kidney disease. A declining trend, in contrast, was observed in renal infections and nephritis. The proportion of patients over 70 years increased during the study period. An approximation of the outcome of renal diseases was made using the ratio of the number of deaths against the number of hospitalized renal patients. The outcome of patients under 50 years has improved. A slight improvement was observed in most renal diseases, partially due to the increased capacity of dialysis treatment and renal transplantations.
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PMID:Hospitalization for renal diseases in Finland 1969-1982. 318 39

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

Serum levels of complement factor D, a low molecular weight (LMW) protein, were high and inversely correlated with glomerular filtration rate (GFR) determined in 19 patients with lupus glomerulonephritis (LGN). Factor D was significantly closer correlated with S-creatinine than were two other LMW proteins, beta 2-microglobulin and gamma-trace in 22 LGN patients. Close correlations between each of the LMW proteins and S-creatinine were found in patients with a non-inflammatory disorder, polycystic kidney disease. Slightly increased beta 2-microglobulin concentrations were found in 15 of 20 systemic lupus erythematosus (SLE) patients without renal involvement, while factor D and gamma-trace showed normal values in most of these patients. The findings imply that serum concentrations of complement factor D in SLE are mainly determined by the GFR.
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PMID:Serum level of complement factor D in systemic lupus erythematosus--an indicator of glomerular filtration rate. 638 50

During the past decade, experimental and clinical evidence has indicated an important role for the renin-angiotensin system in the progressive destruction of nephrons in a wide variety of chronic renal diseases. Studies have indicated that in the subtotally nephrectomized rat model of progressive glomerulosclerosis, in experimental diabetes mellitus, in the chronic phase of puromycin aminonucleoside-induced nephrotic syndrome and in Heymann's nephritis, angiotensin-converting enzyme (ACE) inhibitors dramatically preserve both nephron structure and function. Clinical studies have similarly noted that chronic administration of ACE inhibitors inhibits progression of renal failure in type I diabetes and type II diabetes as well as primary glomerulopathies, sickle cell nephropathy, systemic lupus erythematosis, chronic pyelonephritis and adult polycystic kidney disease. Current evidence suggests that the beneficial effect of ACE inhibitors is primarily due to inhibition of angiotensin II production, and there is strong suggestive evidence for increases in local intrarenal activation of the renin-angiotensin system in these conditions. In obstructive uropathy, activation of the renin-angiotensin system has also been shown to be an important aspect of the early functional changes and may be of importance in the subsequent generation of interstitial fibrosis. In the obstructed kidney, renin and angiotensinogen production increase and type I angiotensin receptors decrease. Inhibitors of angiotensin II production and angiotensin II action partially reverse the vasoconstriction and the reduced renal blood flow, and abolish the changes in expression of AT1 MRNA induced by obstruction. Studies suggest that the angiotensin-mediated increases in tubulointerstitial fibrosis may be mediated by increased production of transforming growth factor-beta.
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PMID:Angiotensin II-mediated renal injury. 756 81

International and geographical differences in the survival rates of chronic dialysis patients can be explained by differences in primary renal disease, in the acceptance rate of elderly patients, and in predialysis comorbid conditions. Several studies have shown the effects of these factors on survival. However, in most studies, a large number of patients may leave for renal transplantation or transfer to other centers, so that precise analysis becomes impossible. Although the number of patients in our registry is not so large (n = 1,982), we have few such problems and were able to examine the effects of the above-mentioned factors on patient survival using the Cox proportional hazard model. Hazard ratios (HR) and 95% confidence intervals were 0.739 and 0.366-1.491 in patients with polycystic kidney disease (n = 38), 2.669 and 1.513-4.708 in patients with systemic lupus erythematosus (n = 39), 1.245 and 0.935-1.660 in patients with nephrosclerosis (n = 122), 1.815 and 1.447-2.229 in patients with diabetes mellitus (n = 374), and 1.595 and 1.201-2.117, respectively, in patients with other renal diseases (n = 146) when the HR in patients with chronic glomerulonephritis (n = 1,263) was taken as 1.00. HR and 95% confidence intervals were 1.222 and 1.016-1.470 in patients with one comorbid condition (n = 217) and 1.494 and 1.033-2.160, respectively, in patients with two comorbid conditions (n = 24) when the HR of patients with no predialysis comorbid conditions (n = 1,741) was taken as 1.00. Our data demonstrate the effects of renal diseases and number of predialysis comorbid conditions on the survival in chronic dialysis patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Effect of renal diseases and comorbid conditions on survival in chronic dialysis patients. 799 Oct 45

In a population of 716 patients with end-stage renal disease (ESRD), 46 patients (6.4%) were identified as having pancreatitis. Pancreatitis was significantly more common in those with alcohol abuse, systemic lupus erythematosus (SLE), and polycystic kidney disease. It was not significantly associated with hyperlipidemia, biliary tract disease, or hypercalcemia. Acute pancreatitis occurring before the patient developed ESRD was mainly alcohol-related and did not appear to be a significant risk factor for future episodes of pancreatitis during dialysis. Chronic calcific pancreatitis diagnosed before ESRD was almost invariably due to alcohol abuse, and tended to be a marker for recurrent acute exacerbation after development of ESRD, whether alcohol consumption continued or not. Pancreatitis occurring for the first time after ESRD in patients on dialysis was generally benign, and was usually accompanied by an uneventful recovery and few recurrent episodes. However, a significant elevation of the calcium x phosphate product was observed in these patients, occurring in about half the patients without any known precipitating factor. After kidney transplantation, the development of pancreatitis was associated with higher morbidity and mortality. Chronic calcific pancreatitis diagnosed after ESRD occurred only in patients with SLE; reported here for the first time, it may be a manifestation of long-standing disease, chronic steroid therapy, or both.
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PMID:Pancreatitis in patients with end-stage renal disease. 830 63


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