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Recurrent membranous glomerulonephritis in two renal transplants 总被引:1,自引:0,他引:1
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Y O'Meara A Green M Carmody J Donohoe E Campbell O Browne J Walshe 《Nephrology, dialysis, transplantation》1989,4(8):730-734
A retrospective study of the clinical records and biopsy specimens of all transplants performed between 1974 and 1987 was carried out. Recurrent glomerulonephritis was diagnosed only in those patients who had precise histological classification of their original disease. Of a total of 737 transplants performed in 633 recipients. 603 were from cadaveric and 134 from living related donors. Of 295 patients who were clinically classed as having chronic glomerulonephritis, histological confirmation was available in 156 (54%) as follows: membranoproliferative glomerulonephritis 34%, diffuse proliferative glomerulonephritis 27%, crescentic glomerulonephritis 13% IgA neuropathy 10%, focal sclerosing glomerulonephritis 10%, and membranous nephropathy 7%. There were 24 cases of recurrence in 23 recipients. Of these, 16 occurred in living-related and 8 in cadaveric grafts. Membranoproliferative glomerulonephritis recurred in 14 (8 type I and 6 type II), focal sclerosing glomerulonephritis in 5, IgA neuropathy in 3, crescentic glomerulonephritis in 1 and membranous nephropathy in 1. Graft failure occurred in 13 patients (54%) and was directly attributable to recurrent disease in 12. Membranoproliferative glomerulonephritis caused 8 graft losses, focal sclerosing glomerulonephritis 2, IgA 1 and crescentic glomerulonephritis 1. Recurrence caused graft loss in 50% of cases in which it occurred. The overall incidence of recurrence was 18%. In contrast to other series, a significantly higher incidence of recurrence was seen in our living-related group. 相似文献
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Pérez-Sáez MJ Toledo K Navarro MD Lopez-Andreu M Redondo MD Ortega R Pérez-Seoane C Agüera ML Rodríguez-Benot A Aljama P 《Transplantation proceedings》2011,43(10):4005-4009
We present a case of a 45-year-old man who suffered from idiopatic membranoproliferative glomerulonephritis (MPGN) in the native kidney that relapsed after his first and second renal grafts. The patient was diagnosed in 1990 with lobular MPGN type I, receiving his first renal graft in 1996. In 2001, a biopsy showed recurrence of MPGN type I (rMPGN). He underwent a second renal graft in 2008. In January 2010, he experienced increased proteinuria and creatinine. Upon electron microscopy of a renal graft biopsy we diagnosed a new rMPGN. At the time of the biopsy, complement levels were normal, although C3 and C4 decreased further. We administered 12 plasmapheresis (PP) sessions and four doses of rituximab. Due to persistent renal impairment, we performed a new biopsy 3 months later, showing less severity of the acute lessions. He received a new cycle of treatment (PP + rituximab). One year later, his renal function was stable with a creatinine ranging between 2 and 2.5 mg/dL and a protein/creatinine ratio less than 1 mg/mg. We concluded that the treatment stopped the disease progression. 相似文献
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H E Leichter S C Jordan A H Cohen R B Ettenger I B Salusky R N Fine 《American journal of nephrology》1986,6(5):382-385
The previously reported detrimental effects of pregnancy on the course of membranoproliferative glomerulonephritis type II (MPGN type II) are limited and are usually considered to be mild. Based on these reports, a 19-year-old female with the diagnosis of MPGN type II who had stable renal function (creatinine 0.9 mg/dl) and a mild nephrotic syndrome with hypertension for 5 years of close follow-up was advised to complete her pregnancy. After a full-term pregnancy, complicated only by moderate nephrotic syndrome, a healthy female infant was born. Two weeks after delivery, the patient presented with acute renal failure and malignant hypertension, without evidence of hemolysis of hepatic failure. Immunologic parameters, including, C3, C4, antinuclear antibodies, circulating immune complexes as well as antibodies to glomerular basement membrane antigen and tubular basement membrane antigen were negative. Peritoneal dialysis was initiated and a renal biopsy was performed which showed MPGN type II with 50% crescents. Despite pulse therapy with methylprednisolone, renal function did not improve, resulting in the need for chronic dialysis. Although no specific nephritogenic mechanism was shown, the course of this patient should be considered when counseling female patients with MPGN type II, regarding the possibility of pregnancy exacerbating their disease, or resulting in rapidly progressive renal failure. 相似文献
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Animal models suggest a role for nephritic factor in the pathogenesis of glomerular disease, but evidence for a role in human disease is lacking. To assess its role, we applied a recently developed method that allows measurement of low levels of nephritic factor activity to stored serum specimens from three patients who had membranoproliferative glomerulonephritis (MPGN) type II. All three had had renal transplants, and one lost two of three transplants from recurrent disease. Evidence for a role for nephritic factor in human disease was a positive correlation between the level of nephritic factor activity and both the severity of recurrence and an increase in serum creatinine concentration. However, the hypocomplementemia was never severe; C3 levels of 49-76 mg/dl and nephritic factor levels of 89 U/ml were associated with severe recurrences. We have previously seen severe disease with mild hypocomplementemia. In contrast, patients with partial lipodystrophy often have severe hypocomplementemia and, presumably, high levels of nephritic factor yet have a mild glomerulonephritis. Disease severity and nephritic factor levels thus appear to be inversely related. The disease is progressive when only moderate amounts of nephritic factor have been circulating and C3 only mildly depressed. 相似文献
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Positive C1q staining associated with poor renal outcome in membranoproliferative glomerulonephritis
Takashi Takei Mitsuyo Itabashi Takahito Moriyama Ari Shimizu Yuki Tsuruta Ayami Ochi Kayu Nakayama Chihiro Iwasaki Keiko Uchida Kosaku Nitta 《Clinical and experimental nephrology》2013,17(1):92-98
Background
Pathogenesis and clinical prognosis of membranoproliferative glomerulonephritis (MPGN) has not yet been established.Methods
We conducted a retrospective study of 41 patients with MPGN (type I and III) and examined the renal survival. In addition, factors contributing to survival time were analyzed.Results
Fourteen patients (34 %) were classified into the renal death group. Patients with nephrotic syndrome and positive C1q staining of glomerular deposits showed a particularly poor prognosis. Significantly higher frequency of nephrotic syndrome and higher urinary protein excretion were observed in the renal death group (p = 0.0002, p = 0.0002) than in the renal survival group. The intensity of C1q staining was positively correlated with the severity of the proteinuria (p = 0.004). Factors that influenced the survival time were positive C1q staining of glomerular deposits (p = 0.003), presence of nephrotic syndrome (p = 0.004), serum albumin (p = 0.02), and proteinuria (p = 0.04).Conclusions
C1q staining in glomerular deposits and nephrotic syndrome were important factors influencing the prognosis and outcome in MPGN patients. C1q deposition may play a key role in the pathogenesis of MPGN, as evidenced by numerous observations, such as induction of proteinuria. 相似文献10.
García-de la Puente S Orozco-Loza IL Zaltzman-Girshevich S de Leon Bojorge B 《Pediatric nephrology (Berlin, Germany)》2008,23(6):929-935
The clinical outcome of patients with membranoproliferative glomerulonephritis (MPGN) varies, with some patients progressing
to end-stage renal disease. The aim of this retrospective study was to analyze the initial clinical signs and laboratory test
results associated with an MPGN prognosis. The study cohort consisted of 47 patients with idiopathic MPGN Type I treated at
the National Institute of Pediatrics, Mexico City, between 1971 and 2001. The median follow-up was 3 years. The three different
outcomes of interest were death, renal failure, and nephrotic syndrome. The patients’ ages ranged between 4 and 16 years.
All patients had different degrees of proteinuria, hyperlipidemia, and microscopic/macroscopic hematuria, and 85.1% of them
showed hypocomplementemia. Clinical outcomes varied, however, the most common was nephrotic syndrome, either alone or combined
with other syndromes, which accounted for 74.5% of all cases. Fifteen patients died. Treatment with methylprednisolone improved
the patient’s condition, while the use of chloroquine or cyclophosphamide worsened it. Twenty-two patients had some degree
of renal failure; glomerular filtration rate (GFR) levels and albumin values were negatively associated to renal failure,
while treatment with methylprednisolone decreased the probability of renal failure. Nephrotic syndrome persisted in 18 patients;
hemolytic complement and hemoglobin values were negatively associated with nephrotic syndrome, while macroscopic hematuria
was positively associated with it. Signs that suggested a poor prognosis during diagnosis were low GFR, low albumin, low hemolytic
complement, and macroscopic hematuria. Treatment with methylprednisolone seemed to improve prognosis, however, this needs
to be confirmed with randomized studies. 相似文献
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Iitaka K Moriya S Nakamura S Tomonaga K Sakai T 《Pediatric nephrology (Berlin, Germany)》2002,17(5):373-378
Seven patients with type III membranoproliferative glomerulonephritis (MPGN) were followed for 9–17 years. Their mean age
at presentation was 11.0 years. Their urinary abnormalities were detected by school urinary screening in five, and two patients
presented with nephrotic syndrome. With time, urinalysis became normal in five, proteinuria persisted in one and nephrotic
syndrome persisted in one. All had serum creatinine levels below 1 mg/dl at the last follow-up. All were initially hypocomplementemic.
Serum C3 levels became normal in five patients but decreased again in three without clinical changes. Mesangial proliferation
was initially slight or moderate in five but, except for one patient, histological changes persisted in follow-up biopsies
even though in two the urinalysis became normal. Electron micrographic studies using silver-methenamine staining revealed
subepithelial and subendothelial deposits associated with basement membrane disruption and layering of the lamina densa, an
abnormality typical of MPGN type III. These observations suggest long-term outcome of type III MPGN is good despite persisting
changes in type III lesions.
Received: 11 July 2001 / Revised: 11 October 2001 / Accepted: 12 October 2001 相似文献
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BACKGROUND: The role of terminal complement complexes (TCCs), which are the final products of complement activation, in the pathogenesis of human glomerulonephritis has not been completely elucidated. To clarify the clinical significance of TCCs in type I membranoproliferative glomerulonephritis (MPGN), we studied TCCs in plasma, renal tissue and urine in pediatric patients with this disease. PATIENTS AND METHODS: We measured the concentrations of TCC in plasma (n=25) and urine (n=13) using enzyme-linked immunosorbent assay. Frozen tissue from 18 renal biopsies were evaluated for the presence of TCC by direct immu-noperoxidase staining. RESULTS: At the early stage of the disease, TCC concentrations in plasma were elevated to above 0.5 arbitrary units (AU)/mL in 14 of 25 patients (high-TCC group), while the remaining 11 patients showed less than 0.5 AU/mL (low-TCC group). In the high-TCC group, TCCs were deposited more diffusely and intensely in the glomerulus, compared with those in the low-TCC group (p=0.034). Furthermore, urinary TCC concentrations in the high-TCC group were higher than those in the low-TCC group (p=0.0001). The high-TCC group showed not only a poorer response to steroid treatment, but also poorer prognosis than the low-TCC group. CONCLUSIONS: These results suggest that, in pediatric patients with type I MPGN, TCCs in circulation may play a particular role in TCC formation in the glomerulus and in urine. The TCC concentration in plasma could be used as a marker of responsiveness to steroid treatment and long-term prognosis. 相似文献
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Anita Leys Willem Proesmans Rita Van Damme-Lombaerts Boudewijn Van Damme 《Pediatric nephrology (Berlin, Germany)》1991,5(2):189-192
In three adolescents, suffering from membrano-proliferative glomerulonephritis type II, ophthalmoscopy and fluorescein angiography revealed retinal pigment epithelium lesions, referred to as basal laminar drusen. The patient with the longest renal history had the most pronounced fundus changes. These lesions, earlier described in adult patients, are believed to be specific for this particular form of chronic glomerulonephritis. 相似文献
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Kiyomasu T Shibata M Kurosu H Shiraishi K Hashimoto H Hayashidera T Akiyama Y Takeda N 《Nephron》2002,91(3):509-511
A nephrotic patient with membranoproliferative glomerulonephritis type II (MPGN II) was treated with cyclosporin A (CSA) and alternate-day low-dose prednisolone. This patient developed the nephrotic syndrome twice. The second episode of the nephrotic syndrome was steroid resistant, and therefore this patient was treated with a CSA regimen. During treatments with alternate-day low-dose prednisolone and CSA, this patient recovered from the nephrotic syndrome. We conclude that CSA therapy may be effective for patients with the steroid-resistant nephrotic syndrome caused by MPGN II. 相似文献
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Yanagihara T Hayakawa M Yoshida J Tsuchiya M Morita T Murakami M Fukunaga Y 《Pediatric nephrology (Berlin, Germany)》2005,20(5):585-590
In Japan, the school urinary screening system facilitates early detection and treatment of membranoproliferative glomerulonephritis (MPGN) in childhood. The present study investigated the long-term prognosis in 19 children with diffuse MPGN type I who received steroid therapy. Before signs of glomerulonephritis were confirmed, all patients displayed abnormal urinalysis results, predominantly through school urinary screening. Treatment comprised a regimen of alternate-day prednisolone after steroid pulse or cyclophosphamide therapy, and follow-up was continued for 10–24 years. Excluding 1 patient on short-term therapy, 18 patients received long-term alternate-day prednisolone therapy for 4–12 years. Treatment was discontinued when amelioration was confirmed on renal biopsy. As of the last observation, urinary abnormalities and hypocomplementemia had disappeared in 15 patients, while mild proteinuria without hypocomplementemia remained in 4 patients. No patients required hemodialysis. Moreover, no severe adverse effects attributable to treatment were identified other than mild short stature. Early detection and therapy using pulse methylprednisolone followed by alternate-day prednisolone was thus confirmed as safe and useful for treating diffuse MPGN type I. 相似文献
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Familial membranoproliferative glomerulonephritis 总被引:1,自引:0,他引:1
Bakkaloglu A.; Soylemezoglu O.; Tinaztepe K.; Saatci U.; Soylemezoglu F. 《Nephrology, dialysis, transplantation》1995,10(1):21-24
Four and two male sibs of two separate families who had biopsy-provenmembranoproliferative glomerulonephritis (MPGN) are presented.In the first family four sibs of the first-degree consanguineousmarriage showed the clinical picture of nephrotic syndrome withouthypocomplementaemia at initial laboratory findings. In the secondfamily two affected sibs showed nephrotic and nephritic syndromeson admission. Family investigations showed normal serum complement,immunoglobulins, T-cell subsets, urine analysis, and serum biochemistry.HLA typing in the two families revealed a common antigen HLAA2 in all affected sibs. Some other reports give suggestiveevidence of MPGN in siblings but this is the first report thatshowed the occurrence of MPGN in four sibs. Our data strengthenedthe concept that genetic factors are involved in the developmentof MPGN but additional immunogenetic studies will shed lighton the genetic aspects of the disease. 相似文献