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1.
In a retrospective analytical study involving 98 children with primary glomerulonephritis who were seen by us at our hospital during a 2-year period from 1984 through 1985 and who had renal biopsy performed previously, attempts were made to correlate pathological findings with both clinical findings and prognosis. The results are summarized as follows: 1) Of 87 patients with asymptomatic chronic glomerulonephritis, glomerular findings were those of minimal change lesion, mesangial proliferative nephritis, MPGN, membranous nephropathy and FGS or sclerosing nephritis in 29.9%, 51.7%, 13.8%, 1.1% and 3.5%, respectively. Among the other 11 patients in whom the diagnosis was made after manifesting the nephritic symptoms, minimal change was noted less frequently and MPGN was detected more frequently than in the aforementioned asymptomatic group. IgA nephropathy was estimated to account for 44.2% of cases of asymptomatic chronic nephritis. 2) Mild mesangial proliferation was observed relatively frequently and severe mesangial proliferation or MPGN rather infrequently in hematuria cases without proteinuria while in those with severe proteinuria minimal change lesion was uncommon and severe mesangial proliferative changes, MPGN or FGS were relatively frequent. 3) In 22 patients with IgA nephropathy and 11 with non-IgA nephritis the severity of glomerular changes was related to the intensity of proteinuria at the time of renal biopsy. 4) A 3 to 5 years' follow-up study of patients with mesangial proliferative nephritis inclusive of IgA nephropathy disclosed that 26-28% of patients became free from urinary abnormalities, 27-37% had persistent hematuria without proteinuria and 24-32% still had proteinuria of 2 plus or above. Patients with milder glomerular changes had a definitely better prognosis than those with severe glomerular lesions.  相似文献   

2.
The most recent studies, using the actuarial life-table technique, of the problem of long-term renal outcome and the factors that influence it in adult patients with one of the three most common types of chronic idiopathic immune complexes-mediated glomerulonephritis (IgA nephropathy [IgAN], membranous nephropathy [MN], and type I membranoproliferative glomerulonephritis [MPGN]) are reviewed. In the last decade, renal survival 10 years after onset has become similar to adult patients with idiopathic IgAN (80% to 87%) and idiopathic MN (75% to 83%), because of improvement of the renal survival of patients with MN. Renal survival at 10 years is worse for adult patients with idiopathic type I MPGN (60% to 64%). There is no substantial difference in the average renal survival times between different geographical regions, with the exception of a better prognosis for idiopathic MN in Japan. The presenting clinical factors that most strongly predict subsequent poor outcome are similar for the three types of glomerulonephritis and are rather nonspecific: (1) severe proteinuria, (2) impairment of renal function, and (3) arterial hypertension. As for the histological features, the most powerful predictor of subsequent progression in all three types of glomerulonephritis is tubulointerstitial damage, suggesting that a cell-mediated immune process believed to occur there may independently influence outcome in glomerular diseases.  相似文献   

3.
Summary: Several systemic viral infections have been associated with the development of glomerular lesions. of the viruses that cause liver disease hepatitis B was the first to be recognized. the recent availability of serologic tests for the hepatitis C virus (HCV) has uncovered an association between HCV infection and renal disease. the principal glomerular lesion that develops is that of a membranoproliferative glomerulonephritis (MPGN), usually in association with antigenaemia and circulating immune complexes that have the characteristics of mixed cryoglobulinaemia represented by polyclonal IgG and monoclonal IgM with rheumatoid factor activity. the presence of a very high percentage of anti-HCV seropositivity in cryoglobulinaemic forms of MPGN suggests that the virus plays an important role in the pathogenesis of the associated immune complex glomerulonephritis. Precipitates containing HCV-RNA and circulating anti-HCV IgG and IgM have been found in the majority of such cases. the course of the renal disease that develops is progressive. Treatment with interferon alpha appears to attenuate the progression of the renal lesions, and the response to treatment appears to be closely related to the clearance of hepatitis C viraemia. Renal lesions also occur in the absence of clinical evidence of liver disease or mixed cryoglobulinaemia. In addition to MPGN, membranous glomerulonephropathy, IgA nephropathy and focal segmental glomerulosclerosis have been reported in these cases of HCV infection. the prevalence of glomerular lesions in patients with HCV infection remains to be determined. the available serologic tests for HCV are still in evolution. In the meantime, all patients presenting with glomerular disease should be screened for HCV.  相似文献   

4.
A study of circulating immune complexes (CIC) was undertaken in 25 patients with primary IgA nephropathy and 13 patients with membranoproliferative glomerulonephritis (MPGN). Clinically, the 25 patients with IgA nephropathy were divided into two groups: the latent type, characterized by chance proteinuria and/or hematuria; and the acute onset type, revealing acute nephritic syndrome. Both the IgG class of CIC (IgG-IC) and the IgA class of CIC (IgA-IC) were measured by conglutinin binding enzyme immunoassay (C-assay). IgG-IC were found to be positive in 32% of the patients with IgA nephropathy, and in 77% of those with MPGN. IgA-IC were positive in 72% of the patients with IgA nephropathy, and in 54% of those with MPGN. Concerning the acute onset type of IgA nephropathy, IgG-IC and IgA-IC were found in 71% and 86% of the patients, respectively, which was more frequent than in the latent type group. Simultaneous presence of IgA-IC and glomerular IgA deposits detected by an immunofluorescence study was noted in 75% of the patients with IgA nephropathy. On the other hand, 78% of the patients with MPGN revealed IgG-IC and glomerular IgG deposits simultaneously. Thus, IgG-IC and IgA-IC appear to play important roles in the pathogenesis of MPGN and IgA nephropathy, respectively.  相似文献   

5.
We report two cases with congenital portosystemic shunt who developed renal lesions without liver cirrhosis. Clinically, both cases showed proteinuria and mild hematuria at 9 and 6 years of age, respectively. In one case, the renal lesion was associated with normal renal function, but nephrotic syndrome followed by chronic renal failure were noted in the other. Renal biopsies showed characteristic histological features of membranoproliferative glomerulonephritis (MPGN) with IgA deposits along the glomerular capillary wall. Our cases strongly suggest the association between congenital portosystemic shunt and renal region. The shunt ratio may be an important predisposing factor for this type of nephropathy since a high shunt ratio (> 90%) was noted in both cases.  相似文献   

6.
BACKGROUND: The Paulista Registry of Glomerulopathies was created in May 1999 and comprises several centres of S?o Paulo, the most populous Brazilian State, that concentrates people from all regions of the country who look for health care. METHODS: This report includes data from 2086 patients from Brazil submitted to renal biopsy due to the presumed diagnosis of glomerular diseases, registered prospectively since May 1999 until January 2005. Data were collected by the integrants of the 11 centres involved, utilizing a standardized questionnaire. RESULTS: The mean age of the patients was 34.5+/-14.6 years. Primary glomerular diseases were more frequent in males (55.1%) than in females; on the other hand, secondary glomerular diseases were more frequent in females (71.8%). The most common clinical presentation was nephrotic syndrome and the frequency of hypertension, at this time, was 55.5%. There was a predominance of indication of biopsies in the third, fourth and fifth decades of life. The most common primary glomerular diseases were focal and segmental glomerulosclerosis (29.7%), followed by membranous nephropathy (20.7%), IgA nephropathy (17.8%), minimal change disease (9.1%), membranoproliferative glomerulonephritis (7%), crescentic glomerulonephritis (4.1%), advanced chronic glomerulopathy (4%), non-IgA mesangial glomerulonephritis (3.8%), diffuse proliferative glomerulonephritis (2.5%), focal segmental proliferative glomerulonephritis (1%) and others (0.3%). The most frequent secondary glomerular disease was lupus nephritis, corresponding to 66.2% of the cases, followed by post-infectious glomerulonephritis (12.5%), diabetic nephropathy (6.2%), diseases associated to paraproteinaemia (4.9%), hereditary diseases (4.6%), vasculitis (3.2%), malignancies (0.9.%), secondary focal segmental glomerulosclerosis (0.6%) and others (0.9%). CONCLUSION: Focal segmental glomerulosclerosis was the most frequent primary glomerular disease, followed by membranous nephropathy and IgA nephropathy. Lupus nephritis predominated over all the other secondary glomerular diseases.  相似文献   

7.
We carried out a retrospective investigation in 220 patients to assess the influence of various parameters on the long-term course of membranoproliferative glomerulonephritis (MPGN) type I. 50 patients (23%) died during the follow-up period of 59 months on average, in another 57 (26%) end-stage renal failure developed. 54 patients (24%) suffered from chronic renal failure, stable renal function (creatinine below 1.3 mg/dl) was preserved in 59 patients (27%). 5 years after biopsy 49% of the patients had already died or needed regular dialysis treatment; after 10 years this proportion increased to 64%. Morphological findings: The outcome was--with the exception of focal crescent formations--not determined by the severity of glomerular changes; the survival rate, however, decreased significantly, if tubulointerstitial lesions were present as defined by acute renal failure, interstitial fibrosis or a combination of both. Clinical parameters: A progressive deterioration of renal function and an increasing number of renal deaths was noticed, when elevated serum creatinine levels at the time of biopsy and high blood pressure values during the follow-up period were observed. 26 patients died from hypertension, 18 of whom before reaching end-stage renal failure. Nephrotic syndrome and the degree of proteinuria as well as antiphlogistic and immunosuppressive treatment did not influence the prognosis of MPGN type I.  相似文献   

8.
The current recommendations of kidney biopsy in childhood idiopathic nephrotic syndrome (CINS) were put forward to minimize unnecessary kidney biopsies in underlying minimal change disease (MCD). However, there remains a diversity of opinion about the criteria for biopsying children with idiopathic nephrotic syndrome. This study was conducted to prospectively study their usefulness in avoiding biopsies in MCD and to evaluate further modifications for minimizing biopsies in CINS. Of 400 consecutive CINS patients, 222 patients were subjected to kidney biopsy according to the current recommendations. The histopathology spectrum of these selectively biopsied children revealed focal segmental glomerulosclerosis (FSGS) in 39%, MCD in 34.2%, membranoproliferative glomerulonephritis (MPGN) in 16.2%, mesangioproliferative glomerulonephritis (MesPGN) in 7.6%, membranous nephropathy (MN) in 1.8%, and diffuse mesangial sclerosis (DMS) in 0.9%. We observed that despite the current recommendations and efforts to minimize biopsy, 34% of children had MCD on histopathology. Two or more clinical (hematuria and hypertension) or biochemical (renal insufficiency) parameters were present in all children with MPGN. Low C3 was present only in children with MPGN. All the steroid responders were found to have MCD, FSGS, or MesPGN on biopsy. Cyclophosphamide response correlated better with steroid responsiveness (P=0.02) than with histo- pathology (P=0.80) in MCD, FSGS, and MesPGN. Based on these observations, we suggest some modifications in current recommendations for kidney biopsy to minimize biopsying children with MCD. These are (1) biopsies in children (age 1–16 years) should be restricted (a) to a subgroup with two or more clinical and biochemical parameters and (b) in steroid non-responders, (2) the decision to administer cyclophosphamide should be based on steroid response pattern without requiring a prior routine biopsy. Received: 16 July 1999 / Revised: 20 November 2001 / Accepted: 24 November 2001  相似文献   

9.
Renal survival rate of IgA nephropathy   总被引:2,自引:0,他引:2  
Y Chida  S Tomura  J Takeuchi 《Nephron》1985,40(2):189-194
In an attempt to identify prognostic indicators in IgA nephropathy, we evaluated the relationship between clinical and histological findings and changes in renal function in 81 patients with IgA nephropathy whose creatinine clearance was more than 80 ml/min at the time of renal biopsy. The incidence of patients whose creatinine clearance decreased to less than 60 ml/min during the follow-up period was calculated with the life table method to designate the renal survival rate. This rate was compared according to the clinical and histological findings at the time of renal biopsy. In conclusion, a statistically significant decrease in the renal survival rate was observed in patients with proteinuria of more than 1.0 g/day, hypertension, severe diffuse proliferative glomerulonephritis, diffuse proliferative glomerulonephritis with focal crescents and glomerular deposition of IgM and/or fibrinogen-related antigen.  相似文献   

10.
The prevalence of hypertension in 288 patients with primary chronic glomerulonephritis was compared with that observed in a control group of 3,477 subjects from the same geographic area. 23.3% of the patients and 12.8% of the general population were hypertensive (p less than 0.01). However, if only patients with normal renal function were considered, prevalence of hypertension (12.7%) was not higher than in the control group. Hypertension was more frequent in focal segmental sclerosis (30%) and in membranous glomerulonephritis (26%) than in IgA nephropathy (9%), membranoproliferative glomerulonephritis (11%) and IgM mesangial glomerulonephritis (12%). Five years after renal biopsy, 92% of normotensive and 47% of hypertensive patients remained with normal renal function (p less than 0.001). These findings suggest that the high prevalence of hypertension in chronic glomerulonephritis is related to the declining renal function. On the other hand, hypertension appears to represent a bad prognostic sign.  相似文献   

11.
Serum uric acid and renal prognosis in patients with IgA nephropathy   总被引:12,自引:0,他引:12  
Ohno I  Hosoya T  Gomi H  Ichida K  Okabe H  Hikita M 《Nephron》2001,87(4):333-339
BACKGROUND/AIMS: This study was designed to elucidate the clinical significance of serum uric acid (SUA) and the relationship between hyperuricemia and renal prognosis in IgA nephropathy. METHODS: The correlation between SUA and other clinical parameters were examined in 748 IgA nephropathy patients (432 males and 316 females). Among these patients, 226 (144 males and 82 females) who were followed for more than 5 years were examined for the relationship between hyperuricemia and renal prognosis. RESULTS: In IgA nephropathy, SUA correlated negatively with creatinine clearance (Ccr), and positively with urinary protein and tubulointerstitial damage. SUA was higher in patients with hypertension or diffuse proliferative glomerulonephritis. Hyperuricemia was a risk factor for renal prognosis, both in terms of serum creatinine (p = 0.0025) and Ccr (p = 0.0057). In 56 patients with normal Ccr at renal biopsy, the change of Ccr after more than 8 years was -22.3 +/- 20.8% in 13 patients with hyperuricemia, compared with +2.6 +/- 39.4% in 43 patients without hyperuricemia (p = 0.0238). Hyperuricemia was related independently to deterioration of Ccr (p = 0.0461). CONCLUSION: Hyperuricemia in IgA nephropathy is derived from both glomerular and tubulointerstitial damage, and correlated with hypertension. Hyperuricemia is a risk factor for renal prognosis in IgA nephropathy.  相似文献   

12.
The evolution of renal glomerular lesions was examined in biopsies taken from 33 patients with membranoproliferative glomerulonephritis (MPGN). 25 patients had a diffuse form of MPGN in the first biopsy (group A). Twenty-four of them still showed diffuse MPGN in subsequent biopsies, but one patient improved clinically and histologically 19 years after the initial biopsy. Out of 6 patients with focal MPGN in the first biopsy (group B), 4 developed diffuse MPGN, one remained with focal MPGN in the repeat biopsy, and another one was found in remission, as determined by both histological and clinical features. Group C represents two patients who had no histological findings of MPGN on initial biopsy but later showed evidence of a diffuse form of MPGN on subsequent biopsies. Thus, the focal form of MPGN may be found either in the development of diffuse MPGN or in its healing stage, and the prognosis will vary accordingly.  相似文献   

13.
One hundred and seventy-six patients with mesangial IgA nephropathy have been studied retrospectively. Mean follow up from apparent onset of the disease was 9.3 years and with follow up from the diagnostic renal biopsy of 4.6 years. Our aim was to evaluate the prognostic significance of sex, age and type of symptoms at onset. The degree of proteinuria, presence of hypertension or decreased renal function, histological lesions and IFL pattern at the time of the diagnostic renal biopsy were recorded. 17 of the patients developed End Stage Renal Failure (ESRF) during the study. According to the Logrank test (renal survival) and Cox stepwise proportional hazard model, severity of glomerular mesangial lesions and degree of proteinuria are the most important indicators of a poor prognosis. The significance of all other parameters disappear after correction for histological lesions and degree of proteinuria. Our conclusion is that a semiquantitative light microscopical examination is an excellent prognostic index in IgA nephropathy, as is a simple determination of protein excretion in the urine.  相似文献   

14.
目的探讨伴有高血压的IgA肾病的临床表现和病理特点的关系。方法回顾性分析我科经肾脏活体组织检查诊断为IgA肾病82例患者的一般情况、临床表现、病理资料。根据病程中血压状况分为高血压组(A组)33例;非高血压组(B组)49例。比较2组的临床和病理资料。结果A组伴有高血压的IgA肾病患者,占同期IgA肾病的40.24%。A组贫血、高尿酸血症、肾功能不全发生率高于B组(P〈0.05),24h尿蛋白定量大于B组(P〈0.05),而水肿、血尿的发生率无统计学差异。A组IgA肾病病理损害重度者的比例高于B组,病理类型以MsPGN为主,占60.61%。肾小球硬化和血管病变发生率较高、肾间质病变程度较重(P〈0.05),新月体形成率较低(P〈0.05)。结论伴有高血压的IgA肾病患者高尿酸血症、肾功能不全发生率高,24h尿蛋白量大,病理损害较重,预后较差。  相似文献   

15.
《Renal failure》2013,35(5):572-577
Background: A unique form of postinfectious glomerulonephritis (PIGN) with IgA-dominant deposition mimicking IgA nephropathy has been increasingly reported. Methods: We compared the clinical and histological features of 12 patients with postinfectious IgA-dominant glomerulonephritis to 134 patients with idiopathic IgA nephropathy. Results: In addition to hypocomplementemia and subepithelial hump-shaped deposits characteristic of PIGN, patients with postinfectious IgA-dominant glomerulonephritis had older age (62.3 ± 16.9 vs. 37.9 ± 16.3 years; p < 0.001) and more frequently presented with acute renal failure (83.3% vs. 10.4%; p < 0.001) than patients with idiopathic IgA nephropathy. Moreover, glomerular changes including endocapillary proliferation, neutrophil infiltration, and capillary loops deposits by immunofluorescence were more commonly present in postinfectious IgA-dominant glomerulonephritis group (p < 0.001). Conclusions: PIGN could be characterized by glomerular IgA-dominant deposition resembling idiopathic IgA nephropathy. It is essential to differentiate postinfectious IgA-dominant glomerulonephritis from idiopathic IgA nephropathy because of the different treatments and prognosis of the two diseases.  相似文献   

16.
Corpa MV  Soares V 《Renal failure》2002,24(3):347-352
Although systemic hypertension is very common in patients with glomerulonephritis there is a dispute if this alteration is consequence of the glomerulonephritis "per se" or is a consequence of the renal failure secondary to the glomerular lesion. With the aim to analyze the factors associated with systemic hypertension, 196 patients with different forms of nephritis were studied. The systemic arterial pressure was measured by standard sphygmomanometer, renal function was evaluated by the determination of the serum creatinine concentration or creatinine clearance. The diagnosis of the type of glomerulonephritis was made on the basis of an examination of kidney biopsy specimens. The prevalence of arterial hypertension among patients with glomerulonephritis was 62.7%. The hypertensive patients were older (hypertensive = 30.6 +/- 12.8; normotensive = 25.4 +/- 1.6 years; P = 0.03). The prevalence of arterial hypertension was lower in patients with minimal glomerular lesion (12.5%), though their ages were also lower (18.1 +/- 3.6 and 29.1 +/- 1.03 years; P = 0.03). Arterial hypertension did not correlate with the serum levels of creatinine and albumin; creatinine clearance and 24-h proteinuria. IN CONCLUSION: In the patients with glomerulonephritis, the presence of arterial hypertension was associated with a higher mean age whereas the intensity of proteinuria, the level of renal function or the type of glomerulonephritis was not different between the two groups.  相似文献   

17.
Recurrence of glomerulonephritis following renal transplantation is considered an important cause of allograft failure. The incidence of recurrence of glomerulonephritis varies widely depending on the definition of recurrence (pathologic recurrence or clinicopathologic recurrence) and the original glomerular disease. Moreover the impact of recurrence of glomerular disease on allograft outcome varies widely between different forms of glomerulonephritis. Whereas IgA nephritis recurs in up to one third of transplanted patients, this is not associated with adverse effects on graft survival. In contrast, recurrent focal segmental glomerulosclerosis and membranoproliferative glomerulopathy have an unfavorable prognosis. Overall, long-term graft survival in patients transplanted for glomerulonephritis is comparable to survival in patients with other causes of ESRD. In recent years, several mechanisms for recurrent disease after transplantation (e.g. PLA2R antibodies in membranous nephropathy and suPAR in FSGS) have been identified, and these findings have helped to elucidate the pathogenesis of glomerular diseases. Although renal transplantation is the treatment of choice for end-stage renal disease as a consequence of glomerulonephritis, further studies are required to develop optimal strategies to prevent, diagnose and treat recurrent glomerular diseases.  相似文献   

18.
Summary: Knowledge concerning childhood IgA nephropathy has expanded greatly in the last 10 years, and its importance as the major form of glomerulonephritis and major contributor to the end-stage renal disease is becoming apparent in children. IgA nephropathy is a slow progressive glomerular disease. Once renal function impairment develops, eventual progression to uraemia is inevitable, therefore a prophylactic approach to the progression of IgA nephropathy, based on an arbitrary period of therapy early in the course of disease in children with IgA nephropathy, warrants serious consideration and investigation. Our recent controlled trial in children with severe glomerular lesions based on renal biopsy demonstrates that 2 year prednisolone therapy combined with azathioprine, dipyridamole and heparin-warfarin benefits those with clinical evidence of disease of relatively short duration. the development of successful therapy during childhood will offer the potential to reduce the high morbidity and mortality associated with this disease in adults.  相似文献   

19.
BACKGROUND: The aim of our study was to examine patients with mesangioproliferative glomerulonephritis (MPGN), with or without glomerular IgA deposits, and to analyse the effect of different clinical and histopathological variables at the time of biopsy on progression to end-stage renal failure (ESRF) and death. METHODS: We retrospectively examined 273 patients who got this diagnosis in Norway from April 1988 to December 1990 after a renal biopsy. All patients were followed for a median duration of 34.8 months (0.8-68 months). RESULTS: The mean age at the time of biopsy was 40+/-17 years (range 1.1-79 years). Glomerular IgA deposits were present in 45% of the patients; IgA deposits did not affect prognosis. Three years after the time of biopsy, 7% had developed ESRF (chronic dialysis treatment or kidney transplantation) and 8% had died. By Kaplan-Meier analyses, the following clinical variables indicated progression to ESRF: increased serum creatinine, proteinuria > or =1 g/24 h, systolic blood pressure (BP) > or =160 mmHg, diastolic BP > or =90 mmHg, serum albumin <35 g/l, presence of urinary granular casts and age > or =60 years. Morphological variables indicating progression to ESRF were presence of focal mesangial sclerosis, focal glomerular crescents or necroses, benign nephrosclerosis and increased interstitial score. In the multivariable analysis, the following variables indicated progression to ESRF: increased serum creatinine (P<0.001), decreased serum albumin (P<0.05), increased diastolic BP (P<0.05), low age (P<0.05) and increased interstitial score (P<0.001). CONCLUSIONS: It is possible from clinical and histopathological variables to identify low-risk and high-risk patients at the time of biopsy. There is, however, considerable convergence. A major new observation is the finding of young age, decreased serum albumin and the presence of urinary granular casts as important clinical risk factors. Interstitial damage was the most important histopathological predictor of ESRF.  相似文献   

20.
Renal biopsies from 33 patients with membranoproliferative glomerulonephritis (MPGN) type I were reviewed to identify pathologic subtypes of this disease and assess their correlation to clinical features. The patients were divided into two groups: group A included 16 patients in chronic or end-stage renal failure and group B 17 patients with no evidence of renal insufficiency. At presentation, a nephrotic or nephritic syndrome and azotemia were equally common in both groups. The incidence of hypertension was significantly increased in group A (P less than 0.05), while recurrent gross hematuria was more common in group B. Nephrotic syndrome was more common during the course of illness in group A. Three subtypes of MPGN type I were recognized, based on whether duplication of glomerular capillary basement membranes was focal segmental (FS; 9 cases), diffuse global (DG; 18 cases), or mixed segmental and global (6 cases). Eight of nine patients showing FS MPGN type I were in group B (p less than 0.05). In contrast, 11 of 18 patients with DG MPGN type I and 4 of 6 with a segmental and global pattern were in group A (P = not significant). Therefore, FS MPGN is a good predictor of a favorable clinical outcome, whereas the other two subtypes are not. This was confirmed by a 100% actuarial kidney survival for the nine patients with FS MPGN and a 50% kidney survival of 7.5 years for patients with the other two subtypes.  相似文献   

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