Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Twenty-seven patients with renal vein thrombosis were retrospectively studied to evaluate their long-term prognosis and relevant prognostic factors. Twenty-four patients presented with a nephrotic syndrome, and 15 had renal impairment (8 acute; 7 moderate). Ten patients had a previous history of proteinuria, and 14 of nephrotic syndrome. Renal biopsy performed in 20 patients, of whom 19 were nephrotic, showed membranous glomerulonephritis in 14, focal segmental glomerulosclerosis in three, minimal change glomerulonephritis in two, and periarteritis nodosa in one. Renal vein thrombosis was angiographically proven in all patients and was bilateral in 18, localised to the left renal vein in seven, and to the right in two. Thrombosis of the inferior vena cava was associated in seven patients. Ten patients were treated by anticoagulants alone, nine by surgical thrombectomy, seven by thrombolysis, and two did not receive any specific treatment. One patient underwent successively thrombectomy and then thrombolysis. Eleven patients died within the first 6 months, mainly from haemorrhagic complications (n = 5) or severe sepsis (n = 2). Survivors were followed up from 6 months to 19 years. Nephrotic syndrome improved or even disappeared in 12 patients, and renal function did not worsen throughout the follow-up in any patients. The main prognostic factors were initial renal function and type of nephropathy: patients with membranous glomerulonephritis had a significantly better renal function and a lower mortality rate than patients with other nephropathies. Initial renal insufficiency was significantly associated with a poor prognosis. There was no advantage, in terms of survival, kidney function and nephrotic syndrome, of either thrombectomy or thrombolysis over anticoagulants alone, despite two complete venous recanalisations after thrombolysis. Accordingly, patients with renal vein thrombosis from membranous glomerulonephritis should be treated by anticoagulants alone, since the long-term prognosis of this disease seems unaffected by intercurrent renal vein thrombosis. With respects to the risk-to-benefit ratio, thrombectomy should be avoided and thrombolysis considered only in patients with initial acute renal failure from acute renal vein thrombosis.
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PMID:The prognosis of renal vein thrombosis: a re-evaluation of 27 cases. 314 96

A circulating lupus anticoagulant factor was detected in a 38-year-old man with end-stage renal disease and a 'lupus-like' syndrome with a diffuse proliferative glomerulonephritis. When treated with steroids, the 'lupus' complications were controlled and the anticoagulant factor disappeared; however, renal function did not recover and the patient commenced regular haemodialysis. Four months later the patient received a cadaver kidney transplant. At transplantation and during follow-up there was neither clinical nor laboratory evidence of lupus activity, but 19 months after transplantation, when steroids were tapered to a low dose, the lupus anticoagulant factor was detected, and renal-vein thrombosis complicated by sepsis led to the patient's death. A membranous glomerulonephritis was found on autopsy. This is the first time in which a (probably 'de novo') membranous glomerulonephritis has been detected in the allograft of a patient with circulating lupus anticoagulant factor.
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PMID:Allograft membranous glomerulonephritis and renal-vein thrombosis in a patient with a lupus anticoagulant factor. 314 30

Eleven of 30 patients with MCTD, followed for a mean of 10 years, developed immune complex nephropathy (five membranous, two mesangial, one mixed, and one sclerosing) with NS in nine of 11. Another patient had membranous nephropathy at autopsy. Patients with renal disease tended to have more systemic manifestations than those without. NS was at times of abrupt onset, recurrent, and/or persistent. Anti-RNP and serum complement were not helpful in predicting nephritis. Seventy-two percent of nephropathy and 62% of NS episodes resolved or improved after corticosteroid therapy. Five patients became hypertensive, two developed chronic renal failure and required chronic dialysis, and one needed acute dialysis twice. One patient progressed to focal proliferative crescentic nephritis with necrotizing arteritis. Three patients with nephropathy died, two of pulmonary hypertension with acute cor pulmonale and one of overwhelming sepsis. Nephropathy is relatively common in MCTD, is associated with substantial morbidity, and with the risk of hypertension and chronic renal failure.
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PMID:Renal involvement in mixed connective tissue disease: a longitudinal clinicopathologic study. 356 25

Clinical and pathological findings and the effects of therapy were investigated in 90 cases of nephrotic syndrome (NS) in elderly patients aged over 60 years. Membranous nephropathy was the most frequent type of primary NS. Amyloidosis and malignancy were common causes of secondary NS. Damage to the interstitium in the kidney, such as focal mononuclear cell infiltration, fibrosis and thickening of the small arterial wall in membranous cases, was often observed. Stage I and II based on electron-microscopy, were mainly observed in the patients, with membranous nephropathy. Prednisolone and immunosuppressive agent were most effective in these patients with membranous nephropathy. Prednisolone alone was the most effective on minimal change NS in the elderly. In the course of therapy, side effects such as pneumonia, sepsis due to fungus infections, such as aspergillus and candida, and infection, such as cytomegalovirus and herpes zoster, were more frequently observed, especially in the cases of MPGN, DPGN with moderate to severe mesangial proliferation, with a decline in renal function (Ccr < 50 L/day) and secondary NS. In secondary NS, the prognosis of amyloidosis was very poor and the findings pointed to a relationship between malignancy and nephrotic syndrome.
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PMID:Nephrotic syndrome in the elderly--clinicopathological study. 810 8

Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS), but similar reports in children are lacking. We describe two children with MDS-associated steroid-responsive nephrotic syndrome (NS). Patient 1, who had MDS with myelofibrosis, presented with hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementemia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient hematological improvement occurred. Relapse subsequently occurred that manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease, but hematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, 2 years after the onset of the MDS. Patient 2, who had refractory anemia with clonal monosomy 19, presented with bowel disease, hepatosplenomegaly, anemia and non-organ-specific autoantibodies. Prednisone led to both clinical and hematological remission. The hematologic disease relapsed 12 months later, when nephrotic-range proteinuria, hematuria and mild azotemia were also found. Corticosteroid treatment led to long-lasting renal and hematologic remission, maintained by a small dosage of prednisone. In both patients, renal biopsy findings were consistent with those seen in idiopathic NS. A Medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis, and AL amyloidosis were found. We conclude: (1) that glomerular disease may be present and should be searched for in patients with MDS and (2) that MDS can be added to the list of rare conditions associated with corticosteroid-responsive NS in children.
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PMID:Glomerular involvement in myelodysplastic syndromes. 1179 99

The clinical significance of the glomerular tip lesion, characterized by podocyte prominence, capsular adhesion, and/or intracapillary foam cells at or adjacent to the urinary pole, remains unclear. It has been postulated that this lesion simply represents a response to heavy proteinuria, and cases of nephrotic syndrome with tip lesions, but no other histological abnormalities, may represent a form of minimal change nephropathy (MCN). However, others have reported that such lesions have a clinical course similar to that of primary focal segmental glomerulosclerosis (FSGS), and the tip lesion often is included among histological variants of FSGS. To determine whether tip lesions may be seen in MCN, we examined histological slides of kidneys from pre-1950 autopsies of patients with a diagnosis of lipoid nephrosis, a term that at that time comprised MCN and FSGS. Before the introduction of antibiotics and corticosteroid therapy, patients with nephrotic syndrome frequently died of sepsis. Eight such cases, with autopsies performed from 1924 to 1943, were identified in which no glomeruli had changes typical of classic FSGS or membranous nephropathy. More than 400 glomeruli were present in each case. Patient ages ranged from 3 to 45 years (six patients <11 years), all had marked edema (duration, 2 weeks to 21 months) and heavy proteinuria, and each died of sepsis and/or pneumonia (pneumococcal in six patients). Glomerular tip lesions were found in five of these eight cases (range, 3 to 26 lesions per case; 0.3% to 4.4% of total glomeruli present), with no predilection for the deep, middle, or superficial cortex. No tip lesions were seen in kidneys from autopsies of age-matched patients without a history of glomerular disease. These findings suggest that the glomerular tip lesion can occur in MCN and most likely represents a response to heavy proteinuria that is not disease specific.
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PMID:Glomerular tip lesion in minimal change nephropathy: a study of autopsies before 1950. 1204 27

From November 1998 to March 2000, two hundred patients over the age of 60 years (Elderly) with clinical renal disease were studied. 144 patients were between ages of 60-69 years, 46 between 70-79 years and 10 were above 80 years. The elderly patients (Male 165; Female 35) with renal disease constituted 11% (200/1816) of the total nephrology consultation during the study period. The clinical presentation included chronic renal failure (42.5%); acute renal failure (28%); nephrotic syndrome (14.5%); acute glomerulonephritis (7.5%); renal vascular disease (5%) and renal cystic disease (2.5%). Diabetic nephropathy, obstructive uropathy and hypertensive nephrosclerosis were the major causes of CRF, accounting for 80% of total CRF in the elderly. Chronic glomerulonephritis and chronic pyelonephritis (CPN) were less common and etiology of CRF was uncertain in 5.9% of cases. However, diabetic nephropathy was the commonest (49.4%) cause of chronic renal failure. We did not see a single case of ischemic nephropathy causing CRF in the present study. Prerenal ARF, obstructive uropathy and sepsis were contributing factors for ARF in 82% of the cases. Volume depletion due to gastrointestinal fluid loss and urinary tract obstruction on account of enlarged prostate were the leading causes of ARF in 20 (35.7%) and 8 (14.3%) cases respectively. Sepsis with or without multiorgan failure was the major (46.7%) cause of mortality in patients with ARF and overall mortality was 26.8%. The commonest (31%) cause of nephrotic syndrome was the idiopathic membranous nephropathy. Diabetic nephropathy related to type-2 diabetes mellitus was the second most common (24.1%) cause of nephrotic syndrome. Diffuse endocapillary proliferative GN of post infectious etiology was the commonest (73.3%) type of acute GN in our elderly patients. Renal cystic diseases were noted in 5 (ADPKD 3; Simple cyst-2) patients. Thus, overall spectrum of renal disease in our elderly patients is similar to that of developed nations except in two ways: (i) Endocapillary proliferative GN of post infectious origin was the commonest type of acute GN and (ii) Rarity or absence of ischemic nephropathy and atherosclerotic renal artery occlusive disease.
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PMID:Spectrum of renal diseases in the elderly: single center experience from a developing country. 1209 35

Hepatitis B virus (HBV)-associated glomerulonephritides have been increasingly reported, and the association between HBV and glomerulopathy is striking, especially in children. In this study, we investigated clinical and immunohistological features of HBV-associated glomerulonephritis in 14 children aged from 2.5 to 16 years (mean 10 years). The nephrotic syndrome was present in 9 (64%) and the nephritic syndrome in 8 children (57%). Five children had both nephrotic and nephritic syndrome together (35%). Renal insufficiency was determined in 4 of 14 patients (28%). Surface antigen (HBsAg) was present in all, with no history of clinically apparent hepatitis. Investigation of all renal tissue samples with light and immunofluorescence microscopy confirmed the diagnosis of membranous glomerulonephritis (MGN) in 6, membranoproliferative glomerulonephritis (MPGN) in 7, and IgA nephropathy (IgAN) in 1 child. Renal tissue samples were studied by the immunoperoxidase method for HBsAg in all cases; only in 4 children was HBsAg detected in the glomeruli. Examination of liver tissue samples was available in 4 cases, revealing chronic hepatitis in all, with additional development of cirrhosis in 1 and the presence of HBsAg in hepatocytes in all. Of the patients, 8 received corticosteroid treatment; 1 of them achieved a complete remission, while 4 had a partial remission with persistent proteinuria and hematuria. Four patients who received no treatment had a spontaneous remission within 5 months to 10 years following the onset of the renal disease. Two patients died of renal failure, while 1 died of intercurrent sepsis. The patient with IgAN received interferon-alpha 2a and lamuvidine, which resulted in a remission and a marked decrease in HBV DNA titer. The remaining 2 were lost to follow-up. Although MGN has been reported as the nephropathy most commonly associated with HBsAg antigenemia in adults, our study revealed that MPGN could occur in children as well as MGN, without any clinical or historical evidence of hepatitis. The present study provides further evidence for a causal relationship between HBV hepatitis and HBs antigenemia-related glomerulonephritides in the pediatric age group. It also indicates the prognosis (71%) of the associated nephropathies with or without treatment is quite favorable in childhood.
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PMID:Hepatitis-B virus associated nephropathies: a clinicopathological study in 14 children. 1248 86

Several reports have documented various forms of glomerular diseases in adults with myelodysplastic syndromes (MDS), but similar reports in children are lacking. We describe two children with MDS-associated with steroid-responsive nephrotic syndrome (NS). Patient 1, who had MDS with myelofibrosis, presented also hepatosplenomegaly, pancytopenia, chronic hepatitis, moderate proteinuria, hypocomplementamia and elevated ANA titer. During initial prednisone treatment proteinuria markedly diminished and partial but transient haematological improvement occurred. Relapse subsequently occurred that was manifested by overt NS and pancytopenia. High doses of prednisolone led to remission of the renal disease but haematological remission did not occur. Persisting pancytopenia and repeated infections terminated in sepsis, two years after the onset of MDS. Patient 2, who had refractory anaemia with clonal monosomy 19, manifested bowel disease, hepatosplenomegaly, anaemia and non-organic specific autoantibodies. Prednisone led to both clinical and haematological remission. Haematologic disease relapsed 12 months later, when nephrotic-range proteinuria, haematuria and mild azotaemia were also found. Corticosteroid treatment led to long-lasting renal and haematologic remission, maintained by a small dosage of prednisone. In both patients, renal biopsy findings were consistent with those seen in idiopathic NS. A Medline search disclosed 16 cases of glomerulopathy in the course of MDS in adult patients. Clinical features included NS, usually accompanied by renal insufficiency with either acute, chronic, or rapidly progressive glomerulonephritis. On biopsy, membranous nephropathy, crescentic or mesangial proliferative glomerulonephritis and AL amyloidosis, were found. We conclude: (1) that glomerular disease may be present and should be searched for in patients with MDS; (2) that MDS can be added to the list of rare conditions associated with corticosteroid-responsive NS in children.
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PMID:[Corticoid-sensitive nephrotic syndrome in children with myelodysplastic syndromes]. 1257 74

Rituximab is a human/murine chimeric monoclonal antibody primarily used for treating non-Hodgkin's B-cell lymphoma. Recently it has also been used in the treatment of several autoimmune diseases. A literature review was conducted to determine the efficacy of rituximab in the treatment of some of these autoimmune diseases. Multiple mechanisms proposed for the rituximab mediated B cell depletion are also discussed. The efficacy of rituximab is well-established and it is FDA approved for treatment of Rheumatoid arthritis. In this review, data on the use of rituximab is presented from 92 studies involving 1197 patients with the following diseases: systemic lupus erythematosus, idiopathic thrombocytopenic purpura, anti-neutrophil cytoplasmic antibody associated vasculitis, Grave's disease, autoimmune hemolytic anemia, pemphigus vulgaris, hemophilia A, cold agglutinin disease, Sjogren's syndrome, graft vs. host disease, thrombotic thrombocytopenic purpura, cryoglobulinemia, IgM mediated neuropathy, multiple sclerosis, neuromyelitis optica, idiopathic membranous nephropathy, dermatomyositis, and opsoclonus myoclonus. The efficacy varies among different autoimmune diseases. The cumulative data would suggest that in the vast majority of studies in this review, RTX has a beneficial role in their treatment. While rituximab is very effective in the depletion of B cells, current research suggests it may also influence other cells of the immune system by re-establishing immune homeostasis and tolerance. The safety profile of RTX reveals that most reactions are infusion related. In patients with autoimmune diseases the incidence of serious and severe side effects is low. Systemic infection still remains a major concern and may result in death.
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PMID:A review of the current use of rituximab in autoimmune diseases. 1900 Jul 86


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