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2.
Sci Rep ; 8(1): 5585, 2018 Apr 03.
Artigo em Inglês | MEDLINE | ID: mdl-29615640

RESUMO

This study investigated the clinicopathological characteristics of Henoch-Schönlein purpura nephritis (HSPN) in Chinese adult patients and analyzed the renal outcomes and prognostic risk factors for progression to end-stage renal disease (ESRD). Adult patients who had biopsy-proven HSPN were studied. Their clinicopathological data, renal prognoses and related risk factors were assessed. A total of 698 patients were studied, including 363 men (52.0%) and 335 women (48.0%). Most of the patients had hematuria (85.8%) and/or proteinuria (82.1%). During a median follow-up of 54.0 months, 32 patients (4.6%) progressed to ESRD. The 5- and 10-year cumulative renal survival rates from ESRD were 96.4% and 88.6%, respectively. Baseline urinary protein, renal insufficiency, glomerular sclerosis and tubular atrophy/interstitial fibrosis were independent predictors of renal outcomes. Both the time-average mean arterial pressure and proteinuria during follow-up also influenced the renal prognosis. The patients with a time-average proteinuria <0.4 g/day had the lowest rates of ESRD or a 50% decline in renal function. In conclusion, identifying of clinical and histological prognostic factors may permit the prediction of renal outcomes. The optimal goal of therapy for HSPN patients may be to lower proteinuria to <0.4 g/day and control hypertension to achieve an ideal renal outcome.

3.
Kidney Dis (Basel) ; 4(1): 10-19, 2018 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-29594138

RESUMO

Objectives: To evaluate the changing spectrum of kidney diseases over time in China using renal biopsy-proven cases. Methods: All patients over the age of 14 years who were diagnosed with a kidney disease by renal biopsy in the Renal Biopsy Registry of the National Clinical Research Center of Kidney Diseases in Jinling Hospital, Nanjing, from 2003 to 2014 were included. Results: In total, 40,759 cases of renal biopsy were analyzed. The mean age of the patients was 36.59 ± 14.12 years. 52.0$ of the patients were male. Primary glomerulonephritis (PGN), secondary glomerulonephritis, tubulointerstitial disease, and hereditary renal diseases accounted for 67.1, 26.4, 2.9, and 2.5$, respectively. IgA nephropathy (IgAN), membranous nephropathy (MN), minimal change disease, and focal segmental glomerulosclerosis were the leading PGN diagnoses. The frequency of MN increased significantly (p < 0.001) by doubling from 2003 to 2014. An analysis by age category indicated that the frequency of MN increased significantly over time (p < 0.001) in all age categories and increased by more than 2 times in the 14-24 age category. Lupus nephritis (LN) and Henoch-Schönlein purpura nephritis (HSPN) decreased significantly (p < 0.001), diabetic nephropathy (DN) increased nearly twice (p < 0.001), monoclonal immunoglobulin deposition disease (MIDD) tripled (p < 0.001), and hypertensive nephropathy (HT) (p < 0.001) and renal amyloidosis (AMY) (p < 0.05) showed an upward trend. An analysis by age category showed that hepatitis B-related nephritis has significantly decreased in the 14-24 age category (p < 0.001). Conclusion: PGN continued to be the predominant kidney disease in China with IgAN being the most common PGN. The frequency of MN increased significantly, with a maximum increase in young adults. LN and HSPN decreased significantly, DN and MIDD increased significantly, and HT and AMY also showed an increasing trend. The kidney disease trends presented in this study serve as a reference point for patient care, disease prevention, and public health interventions.

4.
Am J Kidney Dis ; 69(6): 788-795, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28215945

RESUMO

BACKGROUND: Observational studies suggest that patients with immunoglobulin A nephropathy (IgAN) with active proliferative lesions show a good response to immunosuppressive treatment. STUDY DESIGN: Multicenter, prospective, randomized, controlled trial. SETTING & PARTICIPANTS: 176 patients with IgAN with active proliferative lesions (cellular and fibrocellular crescents, endocapillary hypercellularity, or necrosis), proteinuria with protein excretion ≥ 1.0g/24h, and estimated glomerular filtration rate > 30mL/min/1.73m2. INTERVENTION: Mycophenolate mofetil (MMF) group: MMF, 1.5g/d, for 6 months and prednisone, 0.4 to 0.6mg/kg/d, for 2 months and then tapered by 20% per month for the next 4 months; prednisone group: prednisone, 0.8 to 1.0mg/kg/d, for 2 months and then tapered by 20% per month for the next 4 months. All patients were followed up for another 6 months. OUTCOMES: The primary end point was complete remission rate at 6 and 12 months. RESULTS: At baseline, median estimated glomerular filtration rates were 90.2 and 94.3mL/min/1.73m2 and mean proteinuria was protein excretion of 2.37 and 2.47g/24h in the MMF and prednisone groups, respectively. At 6 months, complete remission rates were 37% (32 of 86 patients) and 38% (33 of 88 patients); the between-group difference was not statistically significant (P=0.9). At 12 months, complete remission rates were 48% (35 of 73 patients) and 53% (38 of 72 patients) in the MMF and prednisone groups, respectively; the between-group difference was not statistically significant (P=0.6). Incidences of Cushing syndrome and newly diagnosed diabetes mellitus were lower in the MMF group than in the prednisone group. LIMITATIONS: Not all participants were treated with renin-angiotensin system blockers, relatively short follow-up. CONCLUSIONS: MMF plus prednisone versus full-dose prednisone did not differ in reducing proteinuria, but patients treated with the former had fewer adverse events in patients with IgAN with active proliferative lesions.


Assuntos
Glomerulonefrite por IGA/tratamento farmacológico , Glucocorticoides/administração & dosagem , Imunossupressores/uso terapêutico , Ácido Micofenólico/uso terapêutico , Prednisona/administração & dosagem , Adulto , Quimioterapia Combinada , Feminino , Taxa de Filtração Glomerular , Glomerulonefrite por IGA/patologia , Glomerulonefrite por IGA/urina , Glucocorticoides/uso terapêutico , Humanos , Masculino , Prednisona/uso terapêutico , Proteinúria/urina , Indução de Remissão , Resultado do Tratamento
5.
BMC Nephrol ; 18(1): 2, 2017 Jan 05.
Artigo em Inglês | MEDLINE | ID: mdl-28056860

RESUMO

BACKGROUND: The KDIGO Clinical Practice Guidelines for Glomerulonephritis recommended tacrolimus as an alternative regimen for the initial therapy for Idiopathic membranous nephropathy (IMN), however, large observational studies evaluating tacrolimus treatment in IMN remains rare. METHODS: A total of 408 consecutive IMN patients with nephrotic syndrome who were treated with tacrolimus in Jinling Hospital were included. The effectiveness and safety of tacrolimus treatment in IMN were analyzed in this study. RESULTS: The cumulative partial or complete remission after tacrolimus therapy were 50%, 63% and 67% at 6, 12 and 24 months, respectively, and the cumulative complete remission rates were 4%, 13% and 23%, respectively. Multivariate logistic analysis showed that higher tacrolimus exposure during induction treatment, female gender, higher eGFR and no history of previous immunosuppressive therapy were independently associated with higher probability of remission. A relapse occurred in 101 of the 271 (37.3%) patients with partial or complete remission, and 18 of the 95 (18.9%) patients with complete remission. Tapering duration of tacrolimus and complete remission versus partial remission status were independent factors associated with risk of relapse. A decline in eGFR was the most frequent adverse event during tacrolimus treatment. During tacrolimus treatment, a ≥40% decrease in eGFR was observed in 43 (10.5%) patients. CONCLUSIONS: Low dose tacrolimus is effective for IMN, with a total remission rate of 66% whereas with a rather high rate of relapse. However, the safety of tacrolimus treatment needs to be further validated in large randomized clinical trials.


Assuntos
Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/epidemiologia , Glomerulonefrite Membranosa/tratamento farmacológico , Glomerulonefrite Membranosa/epidemiologia , Tacrolimo/administração & dosagem , Adulto , China/epidemiologia , Estudos de Coortes , Relação Dose-Resposta a Droga , Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos/prevenção & controle , Feminino , Humanos , Imunossupressores/administração & dosagem , Estudos Longitudinais , Masculino , Prevalência , Recidiva , Fatores de Risco , Distribuição por Sexo , Resultado do Tratamento
6.
J Am Soc Nephrol ; 28(5): 1642-1650, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28028136

RESUMO

Idiopathic membranous nephropathy (MN) is associated with HLA; however, the HLA allele involved remains unknown. To identify the HLA risk alleles associated with phospholipase A2 receptor (PLA2R)-related MN in the Chinese population, we sequenced the entire MHC region in DNA samples from 99 patients with PLA2R-related MN, 50 patients with PLA2R-unrelated MN, and 100 healthy subjects. Two HLA risk alleles, HLA-DRB1*15:01 and HLA-DRB3*02:02, independently and strongly associated with an increased risk of PLA2R-related MN. After adjusting for HLA-DRB1*15:01 and HLA-DRB3*02:02, no other alleles showed significant association with PLA2R-related MN. A replication study in an independent cohort of 293 participants with PLA2R-related MN and 285 healthy controls validated these findings. In a joint analysis, a multivariate logistic regression model confirmed that HLA-DRB1*15:01 (odds ratio [OR], 24.9; 95% confidence interval [95% CI], 15.3 to 42.6; P=2.3×10-35) and HLA-DRB3*02:02 (OR, 17.7; 95% CI, 11.0 to 30.3; P=8.0×10-29) independently and strongly associated with PLA2R-related MN. As many as 98.7% of patients with PLA2R-related MN, compared with 43.9% of control subjects, carried at least one HLA risk allele. Subjects with either risk allele had higher odds of developing PLA2R-related MN than those without a risk allele (OR, 98.9; 95% CI, 44.4 to 281.7; P=2.5×10-23). These HLA risk alleles also associated with the age at disease onset in patients with PLA2R-related MN. In conclusion, our findings provide clear evidence that the HLA-DRB1*15:01 and HLA-DRB3*02:02 alleles independently and strongly associate with PLA2R-related MN in the Chinese population.


Assuntos
Glomerulonefrite Membranosa/genética , Cadeias HLA-DRB1/genética , Cadeias HLA-DRB3/genética , Receptores da Fosfolipase A2/fisiologia , Adulto , Alelos , Grupo com Ancestrais do Continente Asiático , Feminino , Glomerulonefrite Membranosa/imunologia , Cadeias HLA-DRB1/imunologia , Cadeias HLA-DRB3/imunologia , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem
7.
BMC Nephrol ; 17(1): 185, 2016 11 22.
Artigo em Inglês | MEDLINE | ID: mdl-27876003

RESUMO

BACKGROUND: The association between psoriasis and membranous nephropathy (MN) remains largely unclear. We examined the prevalence of serum PLA2R antibody and characterized the expression of PLA2R and THSD7A in glomeruli in patients with MN and psoriasis. METHODS: A total of 24 patients with MN without evidence of a secondary cause except psoriasis were enrolled. The clinical and pathological features were retrospectively analyzed. Serum anti-PLA2R antibody was measured using IFA Mosaic. Renal tissue samples stored in the laboratory bio-bank were used for PLA2R staining under immunofluorescence microscopy and THSD7A immunohistochemical analysis. RESULTS: Twenty-four patients (21 male and 3 female) with a mean age of 43.6 ± 15.7 years old were enrolled. Serum anti-PLA2R antibody was positive in 7 patients, which was significantly lower than the positivity observed in idiopathic MN (29.2% vs. 81.7%, P < 0.001). Glomerular PLA2R staining was positive in 7 patients with positive serum anti-PLA2R antibody. THSD7A staining was negative in all 24 patients. During the follow-up visits, 13 patients with negative serum PLA2R antibody achieved CR. In contrast, CR was only achieved in 1 patient with positive serum PLA2R antibody, PR was achieved in 2 patients. CONCLUSIONS: The prevalence of serum anti-PLA2R antibody and glomerular expression of PLA2R was significantly lower in patients with psoriasis and MN than in those with idiopathic MN, and THSD7A staining was negative, suggesting that MN is associated with psoriasis in the majority of patients. However, idiopathic MN might also accompany psoriasis in a minority of psoriatic patients with positive serum anti-PLA2R antibody.


Assuntos
Autoanticorpos/sangue , Glomerulonefrite Membranosa/metabolismo , Glomérulos Renais/metabolismo , Psoríase/sangue , Receptores da Fosfolipase A2/imunologia , Receptores da Fosfolipase A2/metabolismo , Adolescente , Adulto , Idoso , Feminino , Glomerulonefrite Membranosa/complicações , Glomerulonefrite Membranosa/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Proteinúria/etiologia , Psoríase/complicações , Psoríase/tratamento farmacológico , Estudos Retrospectivos , Trombospondinas/metabolismo , Resultado do Tratamento , Adulto Jovem
8.
Biomed Res Int ; 2016: 1634730, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27069916

RESUMO

OBJECTIVE: To investigate potential drugs for diabetic nephropathy (DN) using whole-genome expression profiles and the Connectivity Map (CMAP). METHODOLOGY: Eighteen Chinese Han DN patients and six normal controls were included in this study. Whole-genome expression profiles of microdissected glomeruli were measured using the Affymetrix human U133 plus 2.0 chip. Differentially expressed genes (DEGs) between late stage and early stage DN samples and the CMAP database were used to identify potential drugs for DN using bioinformatics methods. RESULTS: (1) A total of 1065 DEGs (FDR < 0.05 and fold change > 1.5) were found in late stage DN patients compared with early stage DN patients. (2) Piperlongumine, 15d-PGJ2 (15-delta prostaglandin J2), vorinostat, and trichostatin A were predicted to be the most promising potential drugs for DN, acting as NF-κB inhibitors, histone deacetylase inhibitors (HDACIs), PI3K pathway inhibitors, or PPARγ agonists, respectively. CONCLUSION: Using whole-genome expression profiles and the CMAP database, we rapidly predicted potential DN drugs, and therapeutic potential was confirmed by previously published studies. Animal experiments and clinical trials are needed to confirm both the safety and efficacy of these drugs in the treatment of DN.


Assuntos
Nefropatias Diabéticas/tratamento farmacológico , Descoberta de Drogas/métodos , Perfilação da Expressão Gênica/métodos , Glomérulos Renais/efeitos dos fármacos , Glomérulos Renais/metabolismo , Análise Serial de Tecidos/métodos , Adulto , Biópsia , Células Cultivadas , Análise por Conglomerados , Estudos Transversais , Nefropatias Diabéticas/patologia , Feminino , Histocitoquímica , Humanos , Glomérulos Renais/citologia , Masculino , Pessoa de Meia-Idade
9.
BMC Nephrol ; 17: 42, 2016 Apr 11.
Artigo em Inglês | MEDLINE | ID: mdl-27066888

RESUMO

BACKGROUND: This study aimed to investigate renal outcomes and their predictors in biopsy-proven hypertensive nephrosclerosis (HN) patients and to compare clinico-pathological characteristics and prognoses between benign nephrosclerosis (BN) and malignant nephrosclerosis (MN) patients. METHODS: Data for biopsy-proven HN patients were retrospectively analyzed. Renal survival rates and relationships between clinico-pathological characteristics and outcomes were assessed. RESULTS: A total of 194 patients were enrolled; the mean age at biopsy was 43.8 years, and male gender predominated (82.5 %). The median duration of hypertension was 5.0 years, and the mean systolic and diastolic blood pressures were 195 ± 37 and 126 ± 26 mmHg, respectively. The median serum creatinine (Scr) level, estimated glomerular filtration rate (eGFR), and proteinuria level were 1.61 mg/dl, 49.6 ml/min/1.73 m(2), and 0.80 g/24 h, respectively. BN and MN were found by renal biopsy in 55.2 % and 44.8 % of patients, respectively. At biopsy, MN patients were younger, and had higher median Scr and proteinuria levels, higher incidences of anemia, hypertensive heart disease and hypertensive retinopathy, and worse renal outcomes than BN patients. During a median follow-up period of 3.0 years, 36 patients (18.6 %) reached end-stage renal disease (ESRD), and the 5- and 10-year cumulative renal survival rates for HN patients were 84.5 % and 48.9 %, respectively. A decreased baseline eGFR, an increased baseline proteinuria level, anemia, increased percentage of global glomerulosclerosis and tubular atrophy and interstitial fibrosis (TAIF) were independent predictors of future ESRD. CONCLUSIONS: The clinico-pathological characteristics and prognoses were significantly different between the MN and BN patients. The renal outcomes of HN patients were independently associated with the baseline eGFR and proteinuria level, anemia, percentage of global glomerulosclerosis and TAIF.


Assuntos
Hipertensão/complicações , Falência Renal Crônica/patologia , Rim/patologia , Nefroesclerose/patologia , Adulto , Anemia/etiologia , Atrofia , Biópsia , Estudos de Coortes , Creatinina/sangue , Progressão da Doença , Feminino , Fibrose , Taxa de Filtração Glomerular , Cardiopatias/etiologia , Humanos , Retinopatia Hipertensiva/etiologia , Falência Renal Crônica/etiologia , Falência Renal Crônica/metabolismo , Túbulos Renais/patologia , Masculino , Pessoa de Meia-Idade , Nefroesclerose/etiologia , Nefroesclerose/metabolismo , Prognóstico , Proteinúria , Estudos Retrospectivos
10.
Clin Nephrol ; 85(5): 273-81, 2016 May.
Artigo em Inglês | MEDLINE | ID: mdl-26951969

RESUMO

OBJECTIVE: To compare the clinicopathological characteristics, treatment response, and prognosis between patients with IgAN nephropathy with minimal change disease (MCD-IgAN) and patients with minimal change disease (MCD). METHODS: 77 patients with biopsy-proven MCD-IgAN from the Jinling Hospital IgAN Registry and 77 patients with MCD followed up for ≥ 3 years were retrospectively reviewed. RESULTS: MCD-IgAN and MCD patients had similar clinical presentations, both were predominantly young males, the disease mainly manifested as nephrotic syndrome, and the patients rarely presented with microscopic hematuria. Compared with the MCD group, patients with MCD-IgAN had lower levels of baseline serum albumin (p < 0.01) and eGFR (p < 0.05), a higher level of urine n-acetylglucosaminidase (p < 0.01), higher proportion of mesangial hypercellularity (M1), and more severe acute tubulointerstitial lesions in renal pathology (p < 0.01, p < 0.01, respectively). After 8 weeks of corticosteroid therapy, no significant differences were observed in the rate of complete remission, partial remission, and no remission between MCDIgAN and MCD patients (88.3% vs. 90.9%, 10.4% vs. 5.2%, 1.3% vs. 3.9%, p > 0.05). The median time to achieve remission was 4 weeks (range 1 - 24 weeks) and 4 weeks (range 1 - 28 weeks), respectively. No significant difference existed in the efficacy of corticosteroid between the two groups. During 3.96 years (range 3.0 - 8.5 years) of follow-up, no patients in the two groups entered end-stage renal disease (ESRD), only 2 patients (2.6%) with MCD-IgAN had > 50% reduction of eGFR. CONCLUSIONS: MCD-IgAN may be controlled well achieving a comparable clinical outcome as MCD but more frequently necessitates additional immunosuppressive medication.


Assuntos
Corticosteroides/uso terapêutico , Glomerulonefrite por IGA/tratamento farmacológico , Glomerulonefrite por IGA/fisiopatologia , Nefrose Lipoide/tratamento farmacológico , Nefrose Lipoide/fisiopatologia , Acetilglucosaminidase/urina , Adolescente , Adulto , Feminino , Seguimentos , Taxa de Filtração Glomerular , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/patologia , Hematúria/etiologia , Humanos , Masculino , Nefrose Lipoide/complicações , Nefrose Lipoide/patologia , Síndrome Nefrótica/etiologia , Síndrome Nefrótica/patologia , Síndrome Nefrótica/fisiopatologia , Prognóstico , Indução de Remissão , Estudos Retrospectivos , Albumina Sérica/metabolismo , Fatores de Tempo , Adulto Jovem
11.
J Am Soc Nephrol ; 27(10): 3195-3203, 2016 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26989120

RESUMO

Serum phospholipase A2 receptor antibodies (SAbs) and glomerular phospholipase A2 receptor antigen (GAg) deposits have been observed in idiopathic membranous nephropathy (IMN). However, the clinical application of these two biomarkers, particularly GAg deposition, needs to be further evaluated. We measured SAb concentration by ELISA and GAg deposition by immunofluorescence in 572 patients with biopsy-proven IMN. Overall, 68.5% of patients (392 of 572) had detectable SAb (SAb+), and 98.7% of patients who were SAb+ (387 of 392) and 70.6% of patients who were SAb- (127 of 180) had GAg deposition (GAg+). Compared with patients who were SAb-/GAg+, patients who were SAb+/GAg+ exhibited higher levels of proteinuria (P<0.001) and a lower chance of proteinuria remission (P<0.001). In 52 patients who underwent repeat biopsies, patients who did not achieve remission had a higher SAb+ rate on the first biopsy than patients who went into remission (P=0.001). Furthermore, SAb+ levels persisted in patients who did not achieve remission but significantly decreased in patients who achieved remission by the second biopsy. Patients who did not achieve remission also had a higher GAg+ rate on the first biopsy than patients who achieved remission (P<0.01). Sustained GAg+ deposits correlated with disease relapse. In conclusion, combining the measurements of SAb levels and detection of GAg deposition may provide additional information regarding diagnoses, treatment response, and disease relapse in patients with IMN.


Assuntos
Autoanticorpos/imunologia , Glomerulonefrite Membranosa/imunologia , Glomérulos Renais/metabolismo , Receptores da Fosfolipase A2/imunologia , Receptores da Fosfolipase A2/metabolismo , Adulto , Autoanticorpos/sangue , Biópsia , Feminino , Glomerulonefrite Membranosa/sangue , Glomerulonefrite Membranosa/patologia , Humanos , Glomérulos Renais/patologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
12.
J Nephrol ; 29(4): 567-73, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-26537358

RESUMO

BACKGROUND: The clinicopathological characteristics, treatment response and long-term outcome of immunoglobulin (Ig)A nephropathy with minimal change disease (MCD-IgAN) are not well defined. METHODS: Patients with biopsy-proven MCD-IgAN from the Jinling Hospital IgA nephropathy Registry were systematically reviewed and compared with those with IgA nephropathy without minimal change disease (Non-MCD-IgAN). RESULTS: We compared data of 247 MCD-IgAN patients and 1,121 Non-MCD-IgAN patients. Compared to Non-MCD-IgAN, MCD-IgAN patients were younger,with male predominance, had higher levels of proteinuria, total cholesterol and estimated glomerular filtration rate (eGFR), lower incidence of hypertension and microhematuria, lower level of serum creatinine, and had less severe glomerular, tubulointerstitial and vascular lesions in renal pathology. In the Non-MCD-IgAN group, 157 patients (14.0 %) reached the renal endpoint and 103 patients (9.2 %) entered end-stage renal disease (ESRD). The 5-,10-, 15- and 20-year cumulative renal survival rates from ESRD, calculated by Kaplan-Meier method, were 95.0, 83.0, 72.9 and 65.4 %, respectively. In the MCD-IgAN group, no patients entered ESRD and only 4 (1.6 %) reached the renal endpoint. Patients with MCD-IgAN had a significantly better renal outcome than Non-MCD-IgAN (p < 0.01). At multivariate Cox analysis, proteinuria >1.0 g/day, hypertension, eGFR <60 ml/min/1.73 m(2), hypoproteinemia and hyperuricemia were independent risk factors of renal survival for Non-MCD-IgAN patients [hazard ratio (HR) 3.43, p < 0.001; HR 1.65, p < 0.05; HR 2.61, p < 0.001; HR 2.40, p < 0.001; HR 2.27, p < 0.001, respectively), but not for patients with MCD-IgAN. CONCLUSIONS: The long-term outcome of patients with MCD-IgAN is significantly better than that of patients with Non-MCD-IgAN.


Assuntos
Glomerulonefrite por IGA/complicações , Nefrose Lipoide/complicações , Adulto , Feminino , Seguimentos , Glomerulonefrite por IGA/tratamento farmacológico , Glomerulonefrite por IGA/patologia , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Prognóstico , Sistema de Registros , Fatores de Risco , Taxa de Sobrevida
13.
J Nephrol ; 28(4): 441-9, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25585823

RESUMO

BACKGROUND: Reversal of active glomerular lesions after immunosuppressive treatment in patients with IgA nephropathy (IgAN) and their association with prognosis have not been well established. METHODS: Sixty patients with IgAN who received repeat biopsies after immunosuppressive treatment were recruited. Reversal of renal pathological lesions was evaluated between the first and second biopsy. The end-point was defined as a 30% reduction in estimated glomerular filtration rate (eGFR) or end-stage renal disease after the second biopsy. RESULTS: Active glomerular lesions, i.e. endocapillary hypercellularity (E), crescents (C) and necrosis (N) were markedly decreased at the second biopsy after immunosuppressive therapy (36.7 vs. 8.3%, p < 0.001; 85.0 vs. 25.0%, p < 0.001; and 51.7 vs. 3.3%, p < 0.001). Patients with E, C or N at the first biopsy but reversed at the second biopsy showed significantly decreased median levels of proteinuria and hematuria. Such clinical changes were not observed in those with active lesions at both biopsies. After a median follow-up of 32 months, 25.0% of patients reached the end-point. Repeat biopsy confirmed that only tubular atrophy/interstitial fibrosis was associated with the renal outcome. CONCLUSIONS: Active glomerular lesions can be reversed by immunosuppressive treatment in patients with IgAN. The reversal is accompanied by improvement in proteinuria and hematuria. The reversal of these lesions during the disease process may explain the lack of significant correlation of these lesions with clinical outcomes in the present study as well as in previous evaluation studies of the Oxford classification of IgAN.


Assuntos
Glomerulonefrite por IGA/tratamento farmacológico , Imunossupressores/uso terapêutico , Falência Renal Crônica/prevenção & controle , Glomérulos Renais/efeitos dos fármacos , Adolescente , Adulto , Biópsia , Feminino , Taxa de Filtração Glomerular/efeitos dos fármacos , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/patologia , Glomerulonefrite por IGA/fisiopatologia , Humanos , Falência Renal Crônica/etiologia , Falência Renal Crônica/patologia , Falência Renal Crônica/fisiopatologia , Glomérulos Renais/patologia , Glomérulos Renais/fisiopatologia , Masculino , Indução de Remissão , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
14.
Nephrol Dial Transplant ; 30(2): 257-66, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25063425

RESUMO

BACKGROUND: The progression of diabetic nephropathy (DN) is frequently determined by clinical parameters; however, the predictive value of histologic lesions remains largely unknown. Our aim was to evaluate the relationship between histologic changes and renal outcome in patients with type 2 diabetes mellitus (T2DM). METHODS: A total of 396 patients with T2DM and biopsy-proven DN who received follow-up for at least 1 year were recruited. The severity of different histologic lesions was assessed using the pathologic classification established by the Renal Pathology Society. Renal outcomes were defined by progression to end-stage renal disease and doubling of serum creatinine. The influence of histologic findings on renal outcomes was assessed using univariate and multivariate Cox regression. RESULTS: A univariate Cox regression showed that the severity of glomerular and interstitial lesions had a significant impact on renal outcomes (P < 0.001). Scores of vascular lesions demonstrated no association with renal outcomes (P > 0.05). A multivariate COX analysis demonstrated that the glomerular classes and scores of interstitial fibrosis and tubular atrophy were significantly associated with renal outcomes when adjusting for baseline proteinuria, mean arterial pressure and estimated glomerular filtration rate (P < 0.05). The glomerular and interstitial lesions correlated significantly among each other. However, in several patients, the severity of interstitial lesions did not correlate with glomerular lesions. CONCLUSION: These findings indicated that the severity of glomerular and interstitial lesions were significantly associated with renal outcomes in patients with DN, whereas the vascular indexes did not have any impact on renal outcomes.


Assuntos
Diabetes Mellitus Tipo 2/patologia , Nefropatias Diabéticas/patologia , Glomérulos Renais/patologia , Doenças Vasculares/patologia , Biópsia , Creatinina/sangue , Diabetes Mellitus Tipo 2/sangue , Nefropatias Diabéticas/sangue , Progressão da Doença , Feminino , Humanos , Testes de Função Renal , Masculino , Pessoa de Meia-Idade , Prognóstico , Proteinúria/patologia , Fatores de Risco , Índice de Gravidade de Doença
15.
Am J Nephrol ; 40(1): 43-50, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24994520

RESUMO

BACKGROUND/AIMS: The long-term renal outcomes of patients with IgA nephropathy (IgAN) who present with recurrent macroscopic hematuria (RMH) have not been described in previous studies. METHODS: Patients with biopsy-proven primary IgAN in Jinling Hospital were divided into three groups according to different patterns of macroscopic hematuria (MH): RMH, isolated MH (IMH), and those without a history of MH (NMH). RESULTS: A total of 1,155 patients were enrolled in the study (158 in the RMH group, 256 in the IMH group, and 741 in the NMH group). At biopsy, patients with RMH were younger, had lower median proteinuria, a lower incidence of hypertension, and a higher estimated glomerular filtration rate than those in the NMH group. Pathologically, patients with RMH had a lower level of mesangial hypercellularity and segmental glomerulosclerosis as well as less tubular atrophy than those with NMH. The demographic and clinical features of patients with IMH fell between patients with RMH and those with NMH. During a median follow-up of 7.9 years, the 5-, 10- and 20-year cumulative renal survival after biopsy, as calculated by K-M methods, were 98, 91, and 91% in the RMH group, 95, 89, and 64% in the IMH group, and 95, 79, and 57% in the NMH group. The renal survival in patients with RMH was significantly better than patients with NMH or IMH. CONCLUSIONS: The long-term prognosis of patients who present with RMH is significantly better than patients with NMH or IMH.


Assuntos
Taxa de Filtração Glomerular , Glomerulonefrite por IGA/complicações , Hematúria/etiologia , Falência Renal Crônica/etiologia , Rim/patologia , Sistema de Registros , Adulto , Atrofia , Estudos de Casos e Controles , Progressão da Doença , Feminino , Glomerulonefrite por IGA/patologia , Glomerulonefrite por IGA/urina , Humanos , Hipertensão/complicações , Túbulos Renais/patologia , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Prognóstico , Proteinúria/etiologia , Recidiva , Adulto Jovem
16.
Nephrol Dial Transplant ; 29(12): 2251-9, 2014 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-25034755

RESUMO

BACKGROUND: Studies on biopsy-proven renal disease in the elderly (age ≥65 years) are extremely limited in China. The aim of this study was to examine the spectrum of renal diseases and their clinical presentations in elderly patients undergoing renal biopsy. METHODS: All native renal biopsies (n = 851) performed in patients aged ≥65 years from January 2003 to December 2012 were retrospectively analyzed. The results were compared with a control group of 28 574 patients aged 18-64 years undergoing renal biopsy over the same period. RESULTS: These 851 patients included 549 males and 302 females. Primary glomerular diseases (53.94%) occurred more frequently than secondary glomerular diseases (36.49%). The clinical manifestations were nephrotic syndrome (NS) in 29.49% of the patients, chronic renal failure in 24.68%, proteinuria and hematuria in 13.28%, proteinuria in 10.93%, acute kidney injury (AKI) in 10.81% and AKI and NS in 8.93%. Membranous nephropathy (MN) was the most frequent diagnosis (28.79%), followed by diabetic nephropathy (DN, 9.75%), IgA nephropathy (IgAN, 9.64%) and vasculitis (6.82%). When compared with the control group, the results showed that MN (P < 0.0001), DN (P < 0.0001), vasculitis (P < 0.0001) and amyloidosis (P < 0.0001) occurred more frequently and IgAN (P < 0.0001), lupus nephritis (P < 0.0001) and minimal change disease (P < 0.0001) occurred less frequently in the elderly. CONCLUSION: This study is the first and largest renal biopsy series to analyze patients aged ≥65 years in China, and the results obtained from this study may increase the knowledge of renal diseases in elderly patients.


Assuntos
Biópsia , Nefropatias/patologia , Glomérulos Renais/patologia , Adolescente , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , China/epidemiologia , Progressão da Doença , Feminino , Seguimentos , Humanos , Incidência , Nefropatias/epidemiologia , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
17.
BMC Nephrol ; 13: 158, 2012 Nov 27.
Artigo em Inglês | MEDLINE | ID: mdl-23181565

RESUMO

BACKGROUND: The Oxford classification of IgA nephropathy (IgAN) provides a useful tool for prediction of renal prognosis. However, the application of this classification in children with IgAN needs validation in different patient populations. METHODS: A total of 218 children with IgAN from 7 renal centers in China were enrolled. The inclusion criteria was similar to the original Oxford study. RESULTS: There were 98 patients (45%) with mesangial proliferation (M1), 51 patients (23%) with endocapillary proliferation (E1), 136 patients (62%) with segmental sclerosis/adhesion lesion (S1), 13 patients (6%) with moderate tubulointerstitial fibrosis (T1 26-50% of cortex scarred), and only 2 patients (1%) with severe tubulointerstitial fibrosis (T2, >50% of cortex scarred). During a median follow-up duration of 56 months, 24 children (12.4%) developed ESRD or 50% decline in renal function. In univariate COX analysis, we found that tubular atrophy/interstitial fibrosis (HR 4.3, 95%CI 1.8-10.5, P < 0.001) and segmental glomerulosclerosis (HR 9.2 1.2-68.6, P = 0.03) were significant predictors of renal outcome. However, mesangial hypercellularity, endocapillary proliferation, crescents, and necrosis were not associated with renal prognosis. In the multivariate COX regression model, none of these pathologic lesions were shown to be independent risk factors of unfavorable renal outcome except for tubular atrophy/interstitial fibrosis (HR 2.9, 95%CI 1.0-7.9 P = 0.04). CONCLUSIONS: We confirmed tubular atrophy/interstitial fibrosis was the only feature independently associated with renal outcomes in Chinese children with IgAN.


Assuntos
Glomerulonefrite por IGA/classificação , Glomerulonefrite por IGA/epidemiologia , Adolescente , Atrofia , Criança , Pré-Escolar , China/epidemiologia , Feminino , Fibrose , Seguimentos , Glomerulonefrite por IGA/diagnóstico , Glomerulosclerose Segmentar e Focal/diagnóstico , Glomerulosclerose Segmentar e Focal/epidemiologia , Humanos , Túbulos Renais/patologia , Masculino
18.
Am J Kidney Dis ; 60(5): 812-20, 2012 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-22819700

RESUMO

BACKGROUND: The Oxford classification of immunoglobulin A (IgA) nephropathy (IgAN) provides a histopathologic grading system that is associated with kidney disease outcomes independent of clinical features. We evaluated the Oxford IgAN classification in a large cohort of patients from China. STUDY DESIGN: Retrospective study. SETTING & PARTICIPANTS: 1,026 adults with IgAN from 18 referral centers in China. Inclusion criteria and statistical analysis were similar to the Oxford study. PREDICTORS: Histologic findings of mesangial hypercellularity score, endocapillary proliferation, segmental sclerosis or adhesion, crescents, necrosis, and tubular atrophy/interstitial fibrosis. Clinical features, blood pressure, estimated glomerular filtration rate (eGFR), proteinuria, and treatment modalities. OUTCOMES: Time to a 50% reduction in eGFR or end-stage renal disease (the combined event); the rate of eGFR decline (slope of eGFR); proteinuria during follow-up. RESULTS: Compared with the Oxford cohort, the Chinese cohort had a lower proportion of patients with mesangial hypercellularity (43%) and endocapillary proliferation (11%), higher proportion with segmental sclerosis or adhesion (83%) and necrosis (15%), and similar proportion with crescents (48%) and tubular atrophy/interstitial fibrosis (moderate, 24%; severe, 3.3%). During a median follow-up of 53 (25th-75th percentile, 36-67) months, 159 (15.5%) patients reached the combined event. Our study showed that patients with a mesangial hypercellularity score higher than 0.5 were associated with a 2.0-fold (95% CI, 1.5-2.8; P<0.001) higher risk of the combined event than patients with a score of 0.5 or lower. Patients with tubular atrophy/interstitial fibrosis of 25%-50% and >50% versus <25% were associated with a 3.7-fold (95% CI, 2.6-5.1; P<0.001) and 15.1-fold (95% CI, 9.5-24.2; P<0.001) higher risk of the combined event, respectively. Endocapillary proliferation, glomerular crescents, and necrosis were not significant. LIMITATIONS: Retrospective study; the therapeutic interventions were miscellaneous. CONCLUSIONS: We confirmed the associations of mesangial hypercellularity and tubular atrophy/interstitial fibrosis with kidney disease outcomes.


Assuntos
Glomerulonefrite por IGA/classificação , Adolescente , Adulto , Idoso , Grupo com Ancestrais do Continente Asiático , Criança , Pré-Escolar , Feminino , Glomerulonefrite por IGA/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
19.
Nephrol Dial Transplant ; 27(4): 1479-85, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-21965586

RESUMO

BACKGROUND: We sought to identify the long-term renal survival rate and related risk factors of progression to renal failure in Chinese adult patients with IgA nephropathy (IgAN) and to quantify the effects of proteinuria during the follow-up on outcome in patients with IgAN. METHODS: Patients with biopsy-proven primary IgAN in the Nanjing Glomerulonephritis Registry were studied. Renal survival and the relationships between clinical parameters and renal outcomes were assessed. RESULTS: One thousand one hundred and fifty-five patients were enrolled in this study. The 10-, 15- and 20-year cumulative renal survival rates, calculated by Kaplan-Meier method, were 83, 74 and 64%, respectively. At the time of biopsy, proteinuria>1.0 g/day [hazard ratio (HR) 3.2, P<0.001], estimated glomerular filtration rate (eGFR)<60 mL/min/1.73 m2 (HR 2.6, P<0.001), hypertension (HR 1.9, P<0.001), hypoproteinemia (HR 2.0, P<0.001) and hyperuricemia (HR 2.1, P<0.001) were the independent risk factors. Multivariate Cox analysis showed the time-average proteinuria (TA-P) during follow-up was the most important risk factor of renal failure. Patients with TA-P>1.0 g/day were associated with a 9.4-fold risk than patients with TA-P<1.0 g/day (P<0.001) and 46.5-fold risk than those with TA-P<0.5 g/day (P<0.001). Moreover, patients who achieved TA-P<0.5 g/day benefit much more than those with TA-P between 0.5 and 1.0 g/day (HR 13.1, P<0.001). CONCLUSIONS: Thirty-six percent of Chinese adult patients with IgAN progress to end stage renal disease within 20 years. Five clinical features-higher proteinuria, hypertension, impaired renal function, hypoproteinemia and hyperuricemia-are independent predictors of an unfavorable renal outcome. The basic goal of anti-proteinuric therapy for Chinese patients is to lower proteinuria<1.0 g/day and the optimal goal is to lower proteinuria to <0.5 g/day.


Assuntos
Glomerulonefrite por IGA/mortalidade , Hipertensão/mortalidade , Hiperuricemia/mortalidade , Hipoproteinemia/mortalidade , Falência Renal Crônica/mortalidade , Proteinúria/mortalidade , Adulto , China , Progressão da Doença , Feminino , Seguimentos , Taxa de Filtração Glomerular , Glomerulonefrite por IGA/complicações , Humanos , Hipertensão/etiologia , Hiperuricemia/etiologia , Hipoproteinemia/etiologia , Falência Renal Crônica/etiologia , Masculino , Prognóstico , Proteinúria/etiologia , Fatores de Risco , Taxa de Sobrevida , Fatores de Tempo
20.
Zhonghua Xin Xue Guan Bing Za Zhi ; 35(6): 548-51, 2007 Jun.
Artigo em Chinês | MEDLINE | ID: mdl-17711717

RESUMO

OBJECTIVE: To analyze the clinical features and outcome of patients with noncompaction of ventricular myocardium (NVM). METHODS: Clinical manifestations, electrocardiograms and echocardiographies data were analyzed in 18 patients with NVM. Mean follow-up period was (11 +/- 5) months. RESULTS: The patients aged from 1.5 to 71 years, 66.7% patients were males, familial history was observed in 2 cases, congestive heart failure was present in 14 cases, thromboembolic event occurred in 1 patient, arrhythmia induced syncopes were diagnosed in 2 patients and 1 patient was asymptomatic. Abnormal electrocardiograms were observed in all patients, including premature ventricular beats (7 cases), heart block (4 cases), and atrial fibrillations (4 cases). Echocardiographies showed that noncompaction of ventricular myocardium localized in the left ventricle in 17 patients, and right ventricle in 1 patient. The extension of noncompaction myocardium was predominantly at the apex (72%). N/C was 2.3 - 3.1. EF was less than 50% in 15 patients. Hypokinetic movements were observed in both noncompacted and compacted segments. During the follow-up, 1 patient with congestive heart failure received heart transplantation. ICD was implanted in one patient due to ventricular tachycardia. One patient suffered from sudden cardiac death. CONCLUSIONS: The most common clinical presentations of NVM are congestive heart failure, cardiac arrhythmias, and thromboembolism. Echocardiography is considered as the best tool for the diagnosis of NVM. ICD, heart transplantation and anticoagulation therapy could improve the prognosis of patients with NVM in selected cases.


Assuntos
Cardiomiopatias/diagnóstico , Miocárdio/patologia , Adolescente , Adulto , Idoso , Arritmias Cardíacas/diagnóstico , Cardiomiopatias/diagnóstico por imagem , Criança , Pré-Escolar , Ecocardiografia , Feminino , Insuficiência Cardíaca/diagnóstico , Ventrículos do Coração/anormalidades , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Adulto Jovem
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