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

This article describes a case of Goodpasture's syndrome controlled by double filtration plasmapheresis (DFPP) combined with steroid and immunosuppressant therapy. A 48-year-old male, clerk, complaining of fever, dry cough and macroscopic hematuria, was admitted to our hospital. Microscopic hematuria was first pointed out at age 40 on an annual check up. His laboratory data on admission revealed severe anemia, azothemia, macroscopic hematuria and proteinuria. His chest radiograph and CT revealed diffuse nodular densities in bilateral lung fields. Specimens obtained by transbronchial lung biopsy and open renal biopsy revealed linear deposition of IgG by direct immunofluorescent antibody methods. Circulating antiglomerular basement membrane antibody level determined with radioimmunoassay was 1.8% on admission, but one week later it elevated to 5.6% with progression of dyspnea, hypoxemia, and renal failure. Steroid pulse therapy and a total of 6 double filtration plasmaphereses were performed in the first month. Subsequently hypoxemia and dyspnea disappeared, and the chest radiograph of the 40th hospital day showed no abnormal shadows. Two months later recurrence of pulmonary hemorrhage was noticed. Immunosuppressant administration (Cyclophosphamide 100 mg/day) and a total of 10 DFPP procedures were performed with success. By DFPP, circulating anti-GBM antibody fell rapidly to within normal ranges, and anti-GBM antibody level elevated in removed plasma. We think DFPP is effective to remove circulating anti-GBM antibody in Goodpasture's syndrome.
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PMID:[Double filtration plasmapheresis in case of Goodpasture's syndrome]. 221 5

The patient, a 28 year-old-man, was admitted to a hospital because of general fatigue and fever. He was pointed out renal dysfunction and was transferred to Nagasaki University Hospital. The laboratory data on admission showed moderate azotemia (BUN 43 mg/dl, Cr 5.4 mg/dl). A percutaneous renal biopsy on admission revealed a diffuse crescentic glomerulonephritis. A direct immunofluorescence of renal biopsy showed a linear pattern for IgG along the glomerular basement membrane. Radioimmunoassay of his serum for circulating anti-GBM antibody was strongly positive. Aggressive treatment with pulse therapy (methylprednisolone), plasmapheresis, and continuous heparin infusion was performed. He had markedly recovered from renal failure and escaped hemodialysis. The patient is making satisfactory process after 3 years.
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PMID:[Anti-glomerular basement membrane antibody-mediated glomerulonephritis remarkably improved by pulse therapy with methylprednisolone, plasmapheresis and continuous heparin infusion]. 262 42

In their review the authors discuss the history of Alport's syndrome, its nomenclature, incidence, genetics, clinical diagnosis. The authors emphasize electron microscopic findings and criteria of the diagnosis (haematuria or renal failure in the family, progressive nervous deafness, typical changes of the basal glomerular membrane (GBM) and ophtalmological findings of lenticonus or perimacular spots. Familial haematuria (FH) is according to the authors defined as haematuria in several members of the family. Based on data in the literature and the authors' experience, the authors discuss the differential diagnosis of FH where Alport's syndrome is relatively rare. A far more frequent unit is benign familial haematuria characterized morphologically as isolated thinning of the GBM. In some cases these patients are threatened by iatrogenic damage from unnecessary and invasive diagnostic method. The finding of thinned GBM and normal renal function in the parents and grandparents suggest a favourable prognosis also in child patients. Cases of familial glomerulonephritis or idiopathic syndrome with glomerulosclerosis or familial IgA nephropathies are relatively rare. Familial haematuria is are relatively rare. Familial haematuria is relatively frequent (according to the authors 20% of all obscure haematurias) and their diagnosis is based on systematic examination of the urine in other members of the patient's family who also suffer from haematuria.
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PMID:[Familial hematuria and Alport's syndrome]. 265 95

The diagnosis of recurrent renal disease after transplantation is dependent on an accurate and complete diagnosis of the initial cause of renal failure and a similar determination of the cause of graft failure. To be classified as recurrent, the disease in the renal graft must be identical to that seen in the native kidneys. Recurrence of disease accounts for less than 2% of all graft failures, but the overall incidence of recurrent disease is probably 5 to 10 times more common. The most frequent cause of recurrent disease is glomerulonephritis, which was first recognized to recur soon after renal transplantation was introduced. It was then recognized that a variety of metabolic disorders would recur, but it has taken 25 years of experience for a clear picture to emerge of recurrence in most conditions. No initial cause of renal failure poses a contraindication to at least one attempt at transplantation, although with Fabry's disease and oxalosis, a special assessment of the risks for the individual recipient is warranted. In some patients, experience has shown the need for a delay in the commitment to transplantation (eg, in those with anti-glomerular basement membrane [GBM] antibody glomerulonephritis or Henoch Schonlein purpura), the need for the choice of a particular immunosuppressive regimen (eg, in hemolytic uremic syndrome [HUS]), the need for avoidance of primary nonfunction (eg, in oxalosis), and the desirability of avoiding live kidney donation (eg, in heterozygote donors in Fabry's disease, high-risk recipients with focal glomerulosclerosis, and in recipients with HUS). Probably all types of glomerulonephritis recur, but with great variation in frequency and severity. In some forms of glomerulonephritis, recurrence may be frequent and definite on histopathological criteria but may only have a minor clinical expression (eg, dense deposit disease, anti-GBM antibody glomerulonephritis, IgA nephropathy), but in others, recurrence is less predictable yet it is clearly associated with premature graft failure (eg, focal glomerulosclerosis, membranous nephropathy). A common theme emerging is that where the initial glomerulonephritis is aggressive and causes kidney failure over a short time, recurrence is more likely, and when present, it will lead to graft failure with an increased frequency. Clinical manifestations, the frequency of recurrence, and the prognosis of the graft are now identified for most conditions. Unexpected observations have included the rarity of recurrent systemic lupus erythematosus (SLE), the immediate return of heavy proteinuria in focal glomerulosclerosis, and the predictable return of dense deposit disease.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Recurrence of disease following renal transplantation. 304 3

It has been proposed that ingestion of large amounts of dietary protein leads to sustained renal hyperperfusion and progressive glomerulosclerosis in rats. This hypothesis was tested in dogs, with 75% reduction in renal mass, maintained for 4 years on either 56, 27, or 19% dietary protein. Twelve of 21 dogs survived 4 years, and death due to renal failure was not correlated to diet. Dogs fed 56 and 27% protein had increased GFR and CPAH before and after reduction of renal mass compared to the 19% group. A pattern of deterioration of renal function, including proteinuria, was not found in any diet group. Nine of 11 dogs, fed 56, 27, or 19% protein had minimal glomerular lesions, including mesangial proliferation, GBM irregularities, adhesions, and sclerosis. Two other dogs, fed 56% protein, had more severe glomerular lesions. No significant ultrastructural differences were found in glomeruli among the three diet groups. These results do not support the hypothesis that high protein feeding had a significant adverse effect on either renal function of morphology in dogs with 75% nephrectomy.
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PMID:Long-term renal responses to high dietary protein in dogs with 75% nephrectomy. 370 9

A retrospective study was conducted to evaluate the efficacy of plasmapheresis in combination with different immunosuppressive drugs ("pulse" therapy, azathioprine or cyclophosphamide together with steroids) in nine patients presenting with rapidly progressive glomerulonephritis (RPGN) not mediated by antibody to glomerular basement membrane. Six of these patients had to be initially dialysed. All patients underwent renal biopsy, which revealed that seven patients had a minimum of 80% crescents and five had interstitial fibrosis. Recovery of renal function was observed in seven patients (78%). All patients without interstitial fibrosis were recompensated for at least 14 months after the acute onset of RPGN. Those who presented with interstitial fibrosis declined to endstage renal failure after 13 months requiring chronic hemodialysis treatment or cadaveric kidney transplantation. On the basis of these findings interstitial fibrosis seems to be a limiting factor for the prognosis of non-anti-GBM-antibody mediated RPGN.
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PMID:Treatment of non anti-GBM-antibody mediated, rapidly progressive glomerulonephritis by plasmapheresis and immunosuppression. 370 82

Twenty-eight patients aged from 18 mo. to 23 y. (at the onset of the diseases) affected by Alport's syndrome (AS), were studied during last 15 years. The mean time of observation was 10 +/- 0.7 years. Two cases progressed to renal failure at 15 and 21 years respectively, while 26 patients have normal renal function within the 2a decade of life. The diagnosis of hereditary nephritis (AS) was based on the following criteria: Existence of affected kindred. Perceptive deafness. Basal membrane ultrastructural abnormalities. A late exact diagnosis was made in some patients without ascertainable familiarity and without early ultrastructural glomerular study. Perceptive deafness occurred chiefly after 6-8 years of life, increasing the diagnostic difficulties. Isolated hematuria was present in 18 cases (64%) and associated proteinuria (or NS) in 10 (36%). Recurrent otitis media worsened the hearing loss in 5 cases (17.83%). An immuno-allergologic study was carried out because of the great frequency of allergic diseases, respiratory mainly. A significative decrease of plasmatic and secretory IgA was observed in those patients who underwent to recurrent otitis media. The features suggestive of AS in our patients, in addition to the familiarity, were gross haematuria in childhood and diffuse GBM splitting and splintering. Heavy proteinuria and nephrotic syndrome associated to early deafness and to male sex indicate a poor prognosis; but several females also can be affected by serious course of the disease.
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PMID:[Alport syndrome: 15-year follow-up in 28 cases]. 378 95

We report a case of de-novo anti-glomerular basement membrane antibody induced glomerulonephritis (anti-GBM GN) in a renal cadaveric transplant. The 52 year old male patient had developed end-stage renal failure secondary to malignant hypertension. His initial renal transplant lost function within two months because of severe allograft rejection without evidence of anti-glomerular basement membrane (anti-GBM) antibody formation. Eight months after his second cadaveric transplant he developed the nephrotic syndrome. This was followed by a rapid deterioration in graft function associated with the development of diffuse proliferative glomerulonephritis with 100% crescent formation. Linear staining of the GBM with IgG and C3 and the presence of circulating anti-GBM antibodies confirmed the anti-GBM antibody etiology of the glomerular lesion. Thus anti-GBM antibody induced glomerulonephritis can occur de-novo in a transplanted kidney despite routine immunosuppression. This represents either coincidental autoantibody production after transplantation or specific alloantibody production, stimulated by the introduction, by transplantation, of GBM neoantigens.
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PMID:De-novo anti-GBM-antibody-induced glomerulonephritis in a renal transplant. 388 31

This case report describes a patient who initially had a pleuritis and arthalgias. During the follow-up he developed first a membranous glomerulonephritis with nephrotic syndrome and subsequently a crescentic, rapidly progressive glomerulonephritis with glomerular basement membrane antibodies (anti-GBM). An analysis of the serum samples obtained during the follow-up revealed no infections at the onset of renal failure. However, anti-GBM could be demonstrated in the serum samples obtained 2 months before the deterioration of the renal function. The anti-GBM did not react with alveolar BM and the patient had no signs of pulmonary hemorrhage. The etiology and the sequence of the pathological events of rapidly progressive glomerulonephritis is discussed in the light of these observations.
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PMID:Transformation of membranous glomerulonephritis into crescentic glomerulonephritis with glomerular basement membrane antibodies. Serial determinations of anti-GBM before the transformation. 623 78

A review was made of the clinical histories, treatment, and renal histologic features of 12 patients with anti-glomerular basement membrane antibody-induced glomerulonephritis (anti-GBM disease). Two patients had milder histopathologic changes in their kidney biopsy specimens and achieved clinical remission. The other ten patients with severe lesions in their biopsy specimens progressed to renal failure. In addition, vascular lesions of thrombotic microangiopathy were present in six of 12 patients. Six patients had clinical laboratory evidence of microangiopathic hemolytic anemia. There are two important points to be noted from this study. First, the kidney biopsy specimen may be a useful indicator of disease severity and prognosis. Second, clinical laboratory or histologic evidence of thrombotic microangiopathy may occur in 75% of patients with anti-GBM disease.
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PMID:Thrombotic microangiopathy in anti-glomerular basement membrane glomerulonephritis. 633 10


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