Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

---

Query: UMLS:C0035078 (renal failure)
31,970 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fibrillary glomerulonephritis is an unusual kidney disease characterized by the deposition of immunoglobulins in a fibrillar pattern. Until recently it has been considered to involve the kidneys alone. We describe a patient who underwent renal transplantation and developed fibrillary glomerulonephritis with rapidly progressive renal failure and severe pulmonary hemorrhage two years and a half after transplantation. Nephropathy prior to transplantation was thought to be focal and segmental glomerulosclerosis. Diagnosis of fibrillary glomerulonephritis in renal allograft was confirmed by postmortem examination. 50% of the glomeruli with extracapillary crescents were observed on light microscopy. By immunofluorescence main deposition of IgA was detected in the glomerular capillar walls and the mesangium. Electron microscopy showed fibrillo-reticular deposits in the same place. Lung histology showed both old and recent areas of alveolar hemorrhage. Granular staining for IgA was observed in the alveolar walls by immunofluorescence. Ultrastructural analysis of the lung made evident fibrillo-reticulary deposits in the interstitium, similar than those observed in the glomeruli. The presence of these deposits in both renal and pulmonary tissues indicates the possibility of systemic involvement in fibrillary glomerulonephritis. In our case it could be related to the recurrence of this glomerulopathy in renal allograft.
...
PMID:Fibrillary glomerulonephritis and pulmonary hemorrhage in a patient with renal transplantation. 777 75

A 51-yr-old man presented with renal failure, proteinuria, hematuria, and hypertension. The serum contained two monoclonal protein spikes, an IgG4 lambda and free lambda light chains. Free gamma heavy chains were absent from serum. A bone marrow biopsy did not show evidence of a plasmalymphocytic dyscrasia. Renal biopsy showed a nodular glomerulopathy with linear deposits of gamma 4 heavy chains alone along glomerular, tubular, and vascular basement membranes and in mesangial regions. No light chains were detected in the kidney despite staining with antisera directed against both free and bound light chains and the F(ab')2 fragment of IgG. The term heavy-chain deposition disease is appropriate in view of the above features.
...
PMID:IgG heavy-chain deposition disease. 783 42

Idiopathic focal segmental glomerulosclerosis (FSGS) is an infrequent renal biopsy diagnosis in the elderly. In our single-centre referral registry there were only 17 cases seen in 822 biopsies performed in patients aged 60 or over giving an incidence of 2%. These seventeen patients ranged from age 61 to 78 at the time of biopsy and were followed a median period of 29.5 months. The incidence of nephrotic syndrome at baseline was similar to younger adults (70.5%), but both hypertension (71%) and renal insufficiency (53%) were higher. Fifty-three percent (9/17) of the patients were treated with either steroids or a combination of steroids and cytotoxic therapy. A complete remission in proteinuria was observed in 44% of the treated patients versus none in the untreated patients. No relapses were seen in those that had a complete remission. As well, none of the patients with a complete remission, versus 9/14 (63%) of the untreated or non-responsive patients progressed to renal failure during the observation period. One patient who was treated with cytotoxic therapy and steroids subsequently died of a pancreatic carcinoma. Idiopathic FSGS is an infrequent glomerulopathy in the elderly but it is important given its malignant natural history. Alternate day prednisone for up to 6 months may be a reasonable approach since a complete remission in proteinuria was seen in 44% of our treated patients and this response was closely linked to a good long-term prognosis. The risks of therapy however must be carefully weighed against the potential benefit in each case because of the advanced age of these patients.
...
PMID:Steroid therapy and prognosis of focal segmental glomerulosclerosis in the elderly. 792 61

In case of haemolytic uraemic syndrome, it is not always possible to identify on a pure clinical basis the different kidney lesions responsible for the syndrome. We report a series of six cases without thrombotic microangiopathy, which emphasizes the need to perform a kidney biopsy as early as possible, so as to confirm the actual usefulness of plasma exchanges (PE) commonly carried out in emergency in every case of adult haemolytic uraemic syndrome. PATIENTS AND METHODS--Files of patients who were treated for haemolytic uraemic syndrome over the past 14 years were reviewed. Patients in whom thrombotic microangiopathy had been excluded by renal histology data were studied. Every patient was promptly treated with hypotensive drugs, so as to obtain blood pressure levels not exceeding 160-90 mmHg. Dialysis was performed in two patients. Daily PE with fresh frozen plasma were carried out in three patients as early as the first 24 hours after admission, and discontinued immediately after thrombotic microangiopathy could be excluded. RESULTS--All the patients met the usual criteria for diagnosis of haemolytic uraemic syndrome. Elevated liver enzymes were also found in the four cases of preeclampsia, consisting with diagnosis of severe HELLP syndrome. One case was associated with oestrogen therapy. Glomerular lesions were seen in four patients: slight endotheliosis in three cases of preeclampsia; marked lesions of IgA mesangial deposits in the patient who had been treated by contraceptive pill. Three patients had acute tubular necrosis and three had intense lesions of nephrosclerosis. Complete remission was obtained in every case of preeclampsia. Renal failure persisted in two cases (IgA glomerulopathy and one case of nephrosclerosis). DISCUSSION--The histological heterogeneity of haemolytic uraemic syndrome has been already well demonstrated. Typical lesions of thrombotic microangiopathy are usually classified into predominant glomerular lesions, pure arteriolar and mixed lesions. In other cases, thrombotic microangiopathy is not found: kidney lesions may be glomerular (endotheliosis, various subtypes of glomerulonephritis), tubular (acute tubular necrosis) or vascular (nephroangiosclerosis). In every aetiological circumstance, several different lesions may be found together. The usefulness of PE has been proved in thrombotic thrombocytopenic purpura, has been suggested in haemolytic uraemic syndrome and to a lesser extent in persistently severe HELLP syndrome. Unfortunately, none of these reports gave any information about kidney lesions responsible of acute renal failure. CONCLUSION--The haemolytic uraemic syndrome is a syndrome: thrombotic microangiopathy has to be proven when treatment by PE is planned, except in some severe clinical circumstances.
...
PMID:A kidney biopsy is clearly mandatory to confirm the indication of plasma exchanges in adult haemolytic uraemic syndrome. 798 52

A 59-year-old man with end-stage renal failure from systemic vasculitis developed de novo immunotactoid glomerulopathy of the renal allograft, with clinical evidence of hematuria, proteinuria, and acute renal failure 6 weeks after cadaveric renal transplantation. The morphologic lesion of immunotactoid glomerulopathy and the clinical renal disease resolved during the following 2 weeks. The disease had not recurred in the subsequent 20 months of posttransplant follow-up. During the same period, the patient also developed systemic cytomegalovirus (CMV) infection with viremia, acute hepatitis, and bone marrow suppression. The clinical manifestations of CMV illness and the renal disease have subsided following the withdrawal of immunosuppressive agents and simultaneous treatment with ganciclovir. Although there is no direct proof that CMV infection was responsible for the development of immunotactoid glomerulopathy, the circumstantial evidence in this patient strongly suggests that these two disease were temporally linked. To our knowledge, the association between CMV infection and immunotactoid glomerulopathy has not been documented previously.
...
PMID:De novo immunotactoid glomerulopathy of the renal allograft: possible association with cytomegalovirus infection. 802 32

A 32-year-old Japanese male presented with abundant proteinuria, and renal biopsy revealed diffuse subendothelial and mesangial deposits in the glomeruli without hypercellularity or mesangial interposition. There was no clinical or laboratory evidence of systemic conditions such as collagen-vascular disease, paraproteinemia, or infection. Treatment with corticosteroid was ineffective and the patient eventually went into end-stage renal failure. To our knowledge, this is the first report of an idiopathic progressive glomerulopathy characterized by diffuse, global subendothelial and mesangial deposits, which suggests to us a novel insight into glomerular subendothelial/mesangial deposition.
...
PMID:Idiopathic progressive glomerulopathy with extensive subendothelial and mesangial immune deposit formation. 807 36

A distinct form of renal disease has been described in patients at various stages of HIV infection that is becoming increasingly important as a cause of morbidity and mortality. Black race and intravenous drug abuse appear to predispose one to its development. The HIV-associated nephropathy is characterized by nephrotic-range proteinuria, rapid progression to end-stage renal disease, a diffuse sclerosing glomerulopathy with significant tubulo interstitial disease seen on light microscopy, and tubuloreticular inclusions seen via electron microscopy. The entity can be separated from heroin-associated nephropathy. The pathogenesis is unclear. Possibilities include direct invasion of the virus, effects of other viruses, genetic factors, immune factors, and multiple growth factors. Not all patients with HIV infection and renal disease have HIV-associated nephropathy. Because of prognostic and therapeutic implications, it is crucial to differentiate these lesions. Some reports suggest a possible beneficial effect of zidovudine therapy, but more study is required. Patient survival is dependent on the stage of HIV infection. Dialysis therapy does not appear to substantially prolong life in most patients with AIDS and irreversible renal failure. Therefore, a number of ethical issues have arisen that deal with medical futility.
...
PMID:Human immunodeficiency virus-associated nephropathy: current concepts. 816 Jul 12

Renal insufficiency, which is present initially in almost half of patients with multiple myeloma, usually results from myeloma kidney or hypercalcemia. Neither the class of light chain nor the isoelectric point plays an important role in kidney failure. Acute renal failure must be treated with appropriate fluids and with electrolytes and hemodialysis if necessary. Plasma exchange may be helpful, but has not been proven as such. The presence of a nephrotic syndrome and a monoclonal kappa or lambda light chain in the urine almost always indicates primary amyloidosis (AL) or light-chain deposition disease. Amyloid fibrils must be distinguished from the fibrils of immunotactoid glomerulopathy.
...
PMID:Monoclonal proteins and renal disease. 819 2

Renal diseases characterized by Congo red-negative extracellular fibrillary deposits, either organized arrays of larger, microtubular fibrils (immunotactoid glomerulopathy [IT]) or smaller, randomly organized fibrils (fibrillary glomerulonephritis), have been recognized recently. The clinical significance, if any, of the distinction of these patterns has not been determined. On review of all renal biopsy specimens evaluated in a private referral renal pathology laboratory over the last 11 years, 26 cases with fibrillary glomerulonephritis pattern were identified and compared with our six most recent cases with the IT pattern. The fibrillary glomerulonephritis patients, 17 women and nine men, had an average age of 50 +/- 2 years and contributed 1% of the renal biopsy specimens examined. All patients had marked proteinuria and 16 had microscopic hematuria. Follow-up at 23 +/- 5 months in 25 of these patients revealed end-stage renal disease in 11 patients (44%) and one death due to renal failure. End-stage renal disease developed an average of 10 +/- 5 months after biopsy. One patient developed multiple myeloma. Twenty-four renal biopsy specimens showed proliferation, with crescents in seven. Immunofluorescence showed moderate to intense staining for immunoglobulin G and weaker staining for C3, in a predominantly mesangial pattern, with weaker glomerular basement membrane (GBM) staining, corresponding to electron microscopic deposit localization. In four cases, linear GBM staining by immunofluorescence corresponded to extensive subendothelial or transmembranous deposits. The average fibril diameter was 14.0 +/- 0.5 nm (range, 10.4 to 18.4 nm). Immunotactoid glomerulopathy patients (three women and three men) were significantly older, 62 +/- 2 years (P < 0.025). All had marked proteinuria, with microscopic hematuria in two patients. Associated hematopoietic diseases were present in four patients, with monoclonal proteins and/or abnormal plasma cell proliferation in three. One patient died of nonrenal causes. The remaining five patients have stable renal function at 20 +/- 5 months. Biopsy specimens showed proliferative (n = 3) or membranous-like (n = 3) patterns. Immunofluorescence showed immunoglobulin G and weaker C3 staining in a granular GBM pattern, with lesser mesangial staining. The microtubular fibril diameter was on average 43.2 +/- 10.3 nm (range, 16.8 to 90.0 nm). Thus, fibrillary glomerulonephritis and IT can be separated based on ultrastructurally distinct features. Patients with fibrillary glomerulonephritis are less likely than those with IT to have associated hematopoietic disease and also have poorer renal survival. We propose that classification based on these morphologic differences appears to have clinical significance.
...
PMID:Morphologic and clinical features of fibrillary glomerulonephritis versus immunotactoid glomerulopathy. 837 43

The association of renal Wegener's granulomatosis with other glomerular diseases is very rare. A case of anti-neutrophil cytoplasmic antibody-associated necrotizing glomerulonephritis superimposed on a membranous glomerulopathy in a patient with systemic Wegener's granulomatosis is reported. Renal failure was corrected by immunosuppressive therapy treatment, but a non-nephrotic-range proteinuria persisted for several months. The association of membranous glomerulopathy with anti-glomerular basement membrane disease and other autoimmune diseases is well described; however, anti-neutrophil cytoplasmic antibody-associated vasculitis superimposed on membranous glomerulopathy has not been reported previously.
...
PMID:Coexistence of anti-neutrophil cytoplasmic antibody-associated glomerulonephritis and membranous glomerulopathy. 843 94


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>

---