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3.
Turk J Pediatr ; 66(1): 128-133, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38523389

RESUMO

BACKGROUND: Relapses or new-onset IgA nephropathy (IgAN) have been documented in patients after vaccination against SARS-CoV-2; however, only one adult patient has been reported in whom pre-existing IgAN worsened during coronavirus disease 2019 (COVID-19). CASE: We present the first pediatric case with biopsy-proven IgAN and genetically confirmed Alport syndrome, who developed end-stage kidney disease after an exacerbation of IgAN associated with COVID-19. The patient`s basal serum creatinine was 0.7-0.9 mg/dL before infection. He had not been vaccinated against COVID-19. He was admitted to the hospital with edema, hypertension, an elevated serum creatinine of 4.7 mg/ dL, and massive proteinuria. Three months before admission, he had been admitted to another hospital with COVID -19 and an elevated serum creatinine (1.9 mg/dL), but no biopsy had been performed at that time. The kidney biopsy revealed IgAN with 50% fibrocellular crescents with sclerosed glomeruli, tubular atrophy, and interstitial fibrosis. His serum creatinine did not decrease even after five administrations of pulse steroids, and hemodialysis was initiated. CONCLUSION: In conclusion, COVID -19 may pose a high risk for exacerbation of pre-existing glomerular disease. It is therefore necessary to closely monitor the kidney function of patients with underlying glomerulonephritis during and after COVID-19 and consider an early biopsy if serum creatinine does not return to baseline levels. In addition, this case report highlights the clinical importance of the co-occurence of IgAN and Alport syndrome.


Assuntos
COVID-19 , Glomerulonefrite por IGA , Glomerulonefrite , Nefrite Hereditária , Masculino , Adulto , Humanos , Criança , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico , Nefrite Hereditária/complicações , Creatinina , COVID-19/complicações , SARS-CoV-2 , Doença Aguda
4.
Int J Med Sci ; 21(4): 674-680, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38464822

RESUMO

Background: IgA nephropathy (IgAN) is a cause of chronic kidney disease (CKD). Tubular atrophy/interstitial fibrosis is associated with IgAN prognosis. However, simple tools for predicting pathological lesions of IgAN remain limited. Our objective was to develop a tool for evaluating tubular atrophy/interstitial fibrosis in patients with IgAN. Methods: In this cross-sectional study, 410 biopsy-verified IgAN patients were included. The factors associated with the incident interstitial fibrosis or tubular atrophy in IgAN were confirmed by using logistic regression analysis. A nomogram was developed using logistic regression coefficients to evaluate tubular atrophy or interstitial fibrosis. Receiver operating characteristic curves (ROC) and calibration curves were used to determine the discriminative ability and predictive accuracy of the nomogram. Results: In this study, the IgAN patients with tubular atrophy or interstitial fibrosis were older and had a higher percentage of males, hypertension and urinary protein excretion (UPE), with high levels of serum cystatin C, serum creatinine, high-sensitivity C-reactive protein and serum C4. The eGFRcr-cys equation calculated using serum creatinine, cystatin C and UPE were considered independent influencing factors of tubular atrophy or interstitial fibrosis in patients with IgAN. Furthermore, the nomogram demonstrated good discrimination (AUC: 0.87, 95% CI 0.81 to 0.93) and calibration in the validation cohort. Conclusion: The eGFRcr-cys and UPE are associated with tubular atrophy or interstitial fibrosis in patients with IgAN. Diagnostic nomogram can predict tubular atrophy or interstitial fibrosis in IgAN.


Assuntos
Glomerulonefrite por IGA , Masculino , Humanos , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/complicações , Cistatina C , Nomogramas , Creatinina , Estudos Transversais , Fibrose , Atrofia/complicações , Estudos Retrospectivos , Rim/patologia
5.
Ren Fail ; 46(1): 2323160, 2024 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-38466632

RESUMO

Anti-glomerular basement membrane (GBM) disease is a rare autoimmune condition characterized by the presence of positive anti-GBM autoantibodies, linear deposition of immunoglobulin G (IgG) along the GBM and severe kidney injury. In a limited number of cases, the association of anti-GBM disease with other glomerulonephritis has been reported. Herein, we present the case of a 66-year-old female patient with progressive worsen kidney function and decreased urine output. A renal biopsy revealed crescent glomerulonephritis with lineal IgG deposition along the GBM and mesangial IgA deposition, which supported the diagnosis of concurrent anti-GBM disease and IgA nephropathy (IgAN). In an extensive literature review, we identified a total of thirty-nine patients were reported anti-GBM disease combined with IgAN. The clinical characteristics of these patients demonstrate that the anti-GBM disease combined with IgAN tends to be milder with a more indolent course and a better prognosis than the classic anti-GBM disease, and its potential pathogenesis deserves to be further explored.


Assuntos
Doença Antimembrana Basal Glomerular , Glomerulonefrite por IGA , Glomerulonefrite , Feminino , Humanos , Idoso , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico , Doença Antimembrana Basal Glomerular/complicações , Doença Antimembrana Basal Glomerular/diagnóstico , Autoanticorpos , Imunoglobulina G
7.
Clin Lab ; 70(2)2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-38345967

RESUMO

BACKGROUND: Several studies indicated that chronic periodontitis (CP) and its subgingival bacteria correlated with IgA nephropathy (IgAN). Previous research has shown that prevalence of IgAN in chronic periodontitis patients is significantly higher than that in non CP patients in Xinjiang especially in ethnic Uyghur. The aim of this study is to investigate the distribution of plaque bacterial microbes in CP and IgAN patients and to find correlation between CP and IgAN. METHODS: All of the subgingival plaque samples including 7 healthy controls (N group), 8 CP patients, 14 IgAN patients, and 14 CP with IgAN patients were obtained from ethnic Uyghur people. To investigate the distribution of plaque microbe in Uyghur CP and IgAN patients, the 16s rRNA sequencing and comparative analysis of subgingival bacteria were performed. RESULTS: There were no statistically differences in the community richness estimator (Chao) and the diversity estimator (Shannon index) among four groups. The abundance of Burkholderiales (order), Ottowia (genus) in the plaque microbes were significantly higher in CP with IgAN patients than CP patients. The abundance of Eubacterium (genus) was significantly higher in CP with IgAN patients than IgAN patients. The abundance of Veillonella (genus) was significantly higher while Streptococcus (genus), Tannerella (genus) were significantly lower in CP patients than healthy volunteers. CONCLUSIONS: The composition and abundance of subgingival plaque microbes in Uyghur CP and IgAN patients were significantly different at several levels. Which suggested that abundance of subgingival bacteria is correlated to CP and IgAN.


Assuntos
População da Ásia Central , Periodontite Crônica , Gengiva , Glomerulonefrite por IGA , Humanos , Bactérias/genética , Bactérias/isolamento & purificação , Periodontite Crônica/complicações , Periodontite Crônica/microbiologia , Glomerulonefrite por IGA/complicações , RNA Ribossômico 16S/genética , Gengiva/microbiologia
8.
Clin Exp Med ; 24(1): 43, 2024 Feb 24.
Artigo em Inglês | MEDLINE | ID: mdl-38400937

RESUMO

The features of IgA nephropathy (IgAN) after SARS-CoV-2 infection have not been well characterized. In this study, we compared the clinical and pathological characteristics of patients with IgAN who had experienced SARS-CoV-2 infection to those who had not. We conducted a retrospective study that enrolled 38 patients with biopsy-proven IgAN following SARS-CoV-2 infection with 4 months (post-SARS-CoV-2 infection group) and 1154 patients with IgAN prior to the pandemic (pre-SARS-CoV-2 infection group). Among the SARS-CoV-2 group cases, 61% were females. The average duration from SARS-CoV-2 infection to renal biopsy was 78.6 days. Prior to SARS-CoV-2 infection, the patients had different presentations of nephropathy. One patient had isolated hematuria, two had isolated proteinuria, twenty presented with both hematuria and proteinuria, and one patient had elevated serum creatinine. Additionally, there were eight cases with uncertain nephropathy history, and six cases did not have a history of nephropathy. Following SARS-CoV-2 infection, five patients experienced gross hematuria, one case exhibited creatinine elevation, and five cases showed an increase in proteinuria. The group of patients infected with SARS-CoV-2 after the COVID-19 pandemic exhibited older age, higher hypertension ratio and lower eGFR values compared to the pre-SARS-CoV-2 infection group. As for pathological parameters, a higher proportion of patients in the post-SARS-CoV-2 infection group exhibited a higher percentage of sclerotic glomeruli and glomerular ischemic sclerosis. There were no significant differences observed between the two groups in terms of therapy involving steroids, immunosuppressants, or RAS inhibitors. IgA nephropathy patients who were infected with SARS-CoV-2 were generally older and experienced more severe kidney damage compared to those without SARS-CoV-2 infection.


Assuntos
COVID-19 , Glomerulonefrite por IGA , Feminino , Humanos , Masculino , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/patologia , Hematúria/etiologia , Hematúria/patologia , Estudos Retrospectivos , Pandemias , COVID-19/complicações , SARS-CoV-2 , Proteinúria
9.
Arch Esp Urol ; 77(1): 16-24, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38374008

RESUMO

AIM: The purpose of this study was to determine efficacy and safety of hydroxychloroquine (HCQ) for patients with IgA nephropathy (IgAN). METHODS: PubMed, Embase, Web of Science, Cochrane Central Register of Controlled Trials, Wanfang database, Chinese National Knowledge Infrastructure and VIP database up to February 2023 were searched for associated studies comparing HCQ with any other nonHCQ for treating IgAN. The effects of proteinuria, a 50% decrease in proteinuria, estimated glomerular filtration rate (eGFR) and adverse events in patients with IgAN were examined in a meta-analysis. Data were extracted and pooled using RevMan 5.3. RESULTS: Three randomized controlled trials (RCTs), two retrospective and two prospective studies (675 patients) that matched our inclusion criteria were identified. Compared with a control group, HCQ significantly reduced proteinuria (mean difference (MD): -0.26, 95% confidence interval (CI): -0.44 to -0.08, p < 0.01). Patients receiving HCQ plus renin-angiotensin system inhibitors (RASSi) had a better efficacy in proteinuria alleviation and a 50% decrease in proteinuria compared with control groups (MD: -0.38, 95% CI: -0.50 to -0.25, p < 0.001 and relative risk (RR) = 3.31, 95% CI: 1.73 to 6.36, p < 0.001). No appreciable variations were observed in eGFR between HCQ groups and control groups in treating patients with IgAN (MD: -2.00, 95% CI: -4.36 to 0.36, p = 0.10). Moreover, no serious adverse events were observed during HCQ treatment. CONCLUSIONS: Our results indicate HCQ is an efficient, secure treatment for IgAN.


Assuntos
Glomerulonefrite por IGA , Hidroxicloroquina , Humanos , Quimioterapia Combinada , Glomerulonefrite por IGA/tratamento farmacológico , Glomerulonefrite por IGA/complicações , Hidroxicloroquina/efeitos adversos , Hidroxicloroquina/uso terapêutico , Proteinúria/tratamento farmacológico , Proteinúria/complicações , Estudos Retrospectivos
10.
Med Ultrason ; 26(1): 15-20, 2024 Mar 27.
Artigo em Inglês | MEDLINE | ID: mdl-38244220

RESUMO

AIM: To investigate the application and the related influencing factors of ultrafast pulse wave velocity (ufPWV) in the evaluation of carotid artery elasticity in patients with Immunoglobulin A nephropathy (IgAN). MATERIAL AND METHODS: A total of 156 IgAN patients and 50 healthy individuals were selected as the control group. The ufPWV technique was employed to measure carotid arterial elasticity parameters, including carotid intima-media thickness (cIMT), pulse wave velocity at the beginning of systole (BS-PWV) and pulse wave velocity at the end of systole (ES-PWV). RESULTS: Across the three groups (control group, IgAN patients with normal renal function, and IgAN patients with renal dysfunction) there was an increasing trend observed in cIMT, BS-PWV, and ES-PWV. Additionally, ES-PWV exhibited higher sensitivity than BS-PWV. Correlation analysis revealed that BS-PWV and ES-PWV were positively correlated with age, body mass index (BMI), systolic blood pressure (SBP), high-sensitivity C-reactive protein (hs-CRP), creatinine (Cr), blood urea nitrogen (BUN), and uric acid (UA) and negatively correlated with estimated glomerular filtration rate (eGFR). CONCLUSION: The ufPWV technique allows for the rapid and direct measurement of arterial elasticity parameters in the neck and represents a novel approach for the early diagnosis and quantitative assessment of arterial stiffness risk in IgAN patients.


Assuntos
Doenças Cardiovasculares , Glomerulonefrite por IGA , Rigidez Vascular , Humanos , Análise de Onda de Pulso , Espessura Intima-Media Carotídea , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico por imagem , Doenças Cardiovasculares/diagnóstico por imagem , Artérias Carótidas/diagnóstico por imagem , Rigidez Vascular/fisiologia , Pressão Sanguínea
11.
Clin J Am Soc Nephrol ; 19(4): 438-451, 2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-38261310

RESUMO

BACKGROUND: Nephritis is a common manifestation of IgA vasculitis and is morphologically indistinguishable from IgA nephropathy. While MEST-C scores are predictive of kidney outcomes in IgA nephropathy, their value in IgA vasculitis nephritis has not been investigated in large multiethnic cohorts. METHODS: Biopsies from 262 children and 99 adults with IgA vasculitis nephritis ( N =361) from 23 centers in North America, Europe, and Asia were independently scored by three pathologists. MEST-C scores were assessed for correlation with eGFR/proteinuria at biopsy. Because most patients ( N =309, 86%) received immunosuppression, risk factors for outcomes were evaluated in this group using latent class mixed models to identify classes of eGFR trajectories over a median follow-up of 2.7 years (interquartile range, 1.2-5.1). Clinical and histologic parameters associated with each class were determined using logistic regression. RESULTS: M, E, T, and C scores were correlated with either eGFR or proteinuria at biopsy. Two classes were identified by latent class mixed model, one with initial improvement in eGFR followed by a late decline (class 1, N =91) and another with stable eGFR (class 2, N =218). Class 1 was associated with a higher risk of an established kidney outcome (time to ≥30% decline in eGFR or kidney failure; hazard ratio, 5.84; 95% confidence interval, 2.37 to 14.4). Among MEST-C scores, only E1 was associated with class 1 by multivariable analysis. Other factors associated with class 1 were age 18 years and younger, male sex, lower eGFR at biopsy, and extrarenal noncutaneous disease. Fibrous crescents without active changes were associated with class 2. CONCLUSIONS: Kidney outcome in patients with biopsied IgA vasculitis nephritis treated with immunosuppression was determined by clinical risk factors and endocapillary hypercellularity (E1) and fibrous crescents, which are features that are not part of the International Study of Diseases of Children classification.


Assuntos
Glomerulonefrite por IGA , Vasculite por IgA , Nefrite , Adulto , Criança , Humanos , Masculino , Adolescente , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/tratamento farmacológico , Glomerulonefrite por IGA/patologia , Vasculite por IgA/complicações , Vasculite por IgA/tratamento farmacológico , Vasculite por IgA/patologia , Taxa de Filtração Glomerular , Rim/patologia , Nefrite/complicações , Proteinúria/etiologia , Biópsia , Estudos Retrospectivos
12.
Nephrology (Carlton) ; 29(5): 268-277, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38186010

RESUMO

BACKGROUND: Corticosteroids remain contentious as a therapeutic option for IgA nephropathy. We conducted a retrospective cohort study to explore whether corticosteroid therapy is efficient and safe for IgAN patients with moderate proteinuria. METHODS: A total of 336 patients with renal biopsy-confirmed IgAN, estimated glomerular filtration (eGFR) over 15 mL/min/1.73 m2 and urine protein levels of 0.75-3.5 g/d were enrolled. According to the treatment protocol, we classified the enrolled patients into two groups: one receiving corticosteroids and the other receiving supportive care. Complete remission, partial remission, and no remission were applied to describe the efficacy assessments. The endpoint was defined as a 40% reduction in eGFR, the onset of ESRD, or renal disease-related death. RESULTS: Clinical and pathological progression risk factors were higher in corticosteroid-treated individuals. Logistic regression analysis revealed that the corticosteroid group was considerably related to a higher remission rate after adjustment for confounding factors. The occurrence of serious adverse events between the two groups was not found to be statistically significantly different. Then, we matched 95 couples of patients with similar baseline levels in both groups by propensity score matching. The results showed that corticosteroid-treated patients showed higher overall and complete remission rates than untreated patients. However, due to the relatively short follow-up period, no significant differences in the incidence of endpoint and survival analyses have been observed thus far. CONCLUSION: Corticosteroid therapy may benefit IgAN patients with moderate proteinuria via proteinuria reduction and renal function preservation.


Assuntos
Corticosteroides , Glomerulonefrite por IGA , Humanos , Corticosteroides/uso terapêutico , Taxa de Filtração Glomerular , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/tratamento farmacológico , Proteinúria/tratamento farmacológico , Proteinúria/etiologia , Estudos Retrospectivos
13.
J Korean Med Sci ; 39(3): e12, 2024 Jan 22.
Artigo em Inglês | MEDLINE | ID: mdl-38258359

RESUMO

BACKGROUND: The obesity epidemic is associated with the emergence of new kidney diseases including obesity-related glomerulopathy (ORG) and metabolic syndrome-associated disorders. However, the effects of obesity on prevalence and outcome of biopsy-proven kidney disease are not well known. METHODS: We analyzed 14,492 kidney biopsies in 18 hospitals from 1979 to 2018 in Korea. Obesity was defined as a body mass index value of ≥ 30 kg/m². RESULTS: The most common disease was IgA nephropathy (IgAN) in both obese and non-obese participants (33.7% vs. 38.9%). Obesity was associated with a higher risk of focal segmental glomerulosclerosis (FSGS) and hypertensive nephropathy (HT-N) (odds ratio [OR], 1.72, 95% confidence interval [CI], 1.37-2.17; OR, 1.96, 95% CI, 1.21-3.19) and a lower risk of IgAN (OR, 0.74, 95% CI, 0.62-0.88). During the median follow up of 93.1 ± 88.7 months, obesity increased the risk of end-stage kidney disease (ESKD) in patients with IgAN (relative risk [RR], 1.49, 95% CI, 1.01-2.20) and lupus nephritis (LN) (RR, 3.43, 95% CI, 1.36-8.67). Of 947 obese individuals, ORG was detected in 298 (31.5%), and 230 participants had other kidney diseases, most commonly, IgAN (40.9%) followed by diabetic nephropathy (15.2%). Participants with ORG, when combined with other renal diseases, showed higher risks for developing ESKD compared to those with ORG alone (RR, 2.48, 95% CI, 1.09-5.64). CONCLUSION: Obesity is associated with an increased risk of FSGS and HT-N, and also increase the ESKD risk in IgAN and LN patients. ORG in obese participants may have favorable renal outcomes if it occurs alone without any other renal disease.


Assuntos
Glomerulonefrite por IGA , Glomerulosclerose Segmentar e Focal , Hipertensão Renal , Nefrite , Humanos , Glomerulosclerose Segmentar e Focal/complicações , Glomerulosclerose Segmentar e Focal/epidemiologia , Rim , Obesidade/complicações , Biópsia , Estudos de Coortes , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico
15.
Med Sci Monit ; 30: e943912, 2024 Jan 28.
Artigo em Inglês | MEDLINE | ID: mdl-38281080

RESUMO

The clinical association of purpura, arthralgia, and arthritis was first described in 1837 in a publication by Johann Lukas Schönlein, a German physician. In 1874, Eduard Henoch, a student of Schönlein, reported cases of children with purpura, abdominal pain, bloody diarrhea, and joint pain. IgA vasculitis, or Henoch-Schönlein purpura, is a systemic hypersensitivity vasculitis caused by the deposition of immune complexes in small blood vessels, including the renal glomeruli and mesangium. In the skin, the presentation is with non-thrombocytopenic purpura or urticaria. Worldwide, IgA nephropathy is the most common cause of primary glomerulonephritis. Detection of IgA deposition in small blood vessels and the renal glomeruli is diagnostic in most cases. This article aims to review the history, current classification, epidemiology, presentation, and diagnosis of IgA vasculitis and nephropathy, disease associations or trigger factors, including infections, vaccines, and therapeutic agents, and highlights some future approaches to improve diagnosis and clinical management.


Assuntos
Glomerulonefrite por IGA , Hipersensibilidade , Vasculite por IgA , Vasculite , Criança , Humanos , Vasculite por IgA/diagnóstico , Vasculite por IgA/tratamento farmacológico , Imunoglobulina A/uso terapêutico , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/complicações , Vasculite/diagnóstico , Glomérulos Renais
16.
Medicine (Baltimore) ; 103(3): e36990, 2024 Jan 19.
Artigo em Inglês | MEDLINE | ID: mdl-38241588

RESUMO

BACKGROUND: Immunoglobulin A nephropathy (IgAN) is the most common type of primary glomerulonephritis, and recurrent IgAN is common after kidney transplantation (KT). Owing to the differences in various biopsy protocols and follow-ups in each study, the recurrence rate varies from 9.7% to 46%. Although the relapse rates are high, there is no definitive treatment for IgAN recurrence. METHODS: We present a case of successful management of proteinuria in recurrent IgAN after deceased donor KT. A 60-year-old man diagnosed with IgAN 20 years prior, who progressed to end-stage renal disease, underwent deceased donor KT 5 years prior and was admitted to our hospital with progressively increasing proteinuria. RESULTS: The pathological examination of the kidney biopsy specimen revealed recurrent IgAN. High-dose steroid treatment was initiated, and the patient was discharged while maintaining steroid treatment. However, outpatient follow-up showed that proteinuria did not decrease while steroids were maintained. Therefore, an angiotensin receptor blocker was administered after explaining its benefits to the patient. After the addition of angiotensin receptor blocker, proteinuria continued to decrease. CONCLUSION: This case report highlights the importance of using renin-angiotensin system inhibitors with supportive care in cases of suspected of recurrent IgAN after KT. It also emphasizes the need to prescribe renin-angiotensin system inhibitors when steroid therapy is unsuccessful in cases of recurrent IgAN after KT.


Assuntos
Glomerulonefrite por IGA , Transplante de Rim , Proteinúria , Humanos , Masculino , Pessoa de Meia-Idade , Antagonistas de Receptores de Angiotensina/uso terapêutico , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/tratamento farmacológico , Glomerulonefrite por IGA/cirurgia , Rim/patologia , Transplante de Rim/efeitos adversos , Proteinúria/tratamento farmacológico , Proteinúria/etiologia , Recidiva , Esteroides
17.
BMC Nephrol ; 25(1): 42, 2024 Jan 29.
Artigo em Inglês | MEDLINE | ID: mdl-38287343

RESUMO

BACKGROUND: Despite several clinical trials, the use of corticosteroid therapy for treating immunoglobulin A nephropathy (IgAN) remains controversial. We aimed to describe the use of corticosteroid therapy combined with supportive therapy in Norwegian patients with IgAN who had progressed to end-stage kidney disease. METHODS: We conducted a retrospective cohort study using data from the Norwegian Renal Registry. Overall, 143 patients with primary IgAN who progressed to end-stage kidney disease were divided into two groups: the corticosteroid group, who had been treated with corticosteroids and supportive therapy, and the non-corticosteroid group, which had underwent only supportive therapy. The kidney function, time to end-stage kidney disease, and adverse effects were described. The observation period lasted from the diagnostic kidney biopsy until the initiation of kidney replacement therapy. RESULTS: Of the 143 included patients, 103 underwent supportive therapy alone, and 40 were treated with corticosteroids. Most patients (94%) were treated with renin-angiotensin-system blockade, and all patients reached end-stage kidney disease after a median of 5 years (interquartile range; 2-9 years). Time from diagnosis until end-stage kidney disease was similar in the two study groups (p = 0.98). During 6 months of corticosteroid therapy, median eGFR declined from 21 (interquartile range; 13-46) mL/min/1.73 m2 to 20 (interquartile range; 12-40) mL/min/1.73 m2, and median proteinuria decreased from 5.5 g/24 h to 3.0 g/24 h. Most patients (87.5%) treated with corticosteroids reported adverse events. In our linear regression analysis investigating the time to ESKD, we found that age (ß = -0.079, p = 0.008) and proteinuria at diagnosis (ß = -0.50, p = 0.01) exhibited statistically significant associations with a delay in the progression to ESKD. CONCLUSIONS: In this cohort of Norwegian patients with IgAN, corticosteroid therapy did not affect the time from diagnosis until end-stage kidney disease among a cohort of patients who all reached end-stage kidney disease. The treatment was also associated with adverse effects.


Assuntos
Glomerulonefrite por IGA , Falência Renal Crônica , Humanos , Glomerulonefrite por IGA/tratamento farmacológico , Glomerulonefrite por IGA/epidemiologia , Glomerulonefrite por IGA/complicações , Estudos Retrospectivos , Corticosteroides/uso terapêutico , Falência Renal Crônica/complicações , Proteinúria/tratamento farmacológico , Progressão da Doença , Taxa de Filtração Glomerular
18.
CEN Case Rep ; 13(1): 14-18, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37088833

RESUMO

A Japanese boy developed nephrotic syndrome (NS) and had microscopic hematuria at 8 years old. Renal biopsy was performed. Light microscopy study revealed mesangial proliferation and all immunofluorescent stains (including IgA) were negative, so he was diagnosed with non-IgA diffuse mesangial proliferation (DMP). Complete remission was achieved at 13 days after the initiation of oral prednisolone, and hematuria also disappeared 3 days later, but the patient developed frequently relapsing nephrotic syndrome. Cyclosporine A (CyA) was introduced at 10 years old, and there were no relapses between then and when it was discontinued at 12 years old. A second renal biopsy revealed minimal change without CyA nephrotoxicity. However, there was repeated relapse of NS after discontinuation, so CyA was reintroduced 8 months later, and NS remained in remission thereafter. Microscopic hematuria appeared at 13 years old, however, with gross hematuria appearing at the time of infection. A third renal biopsy revealed mesangial proliferation with IgA-dominant deposition, so the patient was diagnosed with IgA nephropathy. Currently (14 years old), CyA treatment has been discontinued and the patient is undergoing lisinopril therapy for IgA nephropathy, but there are still relapses of NS. To the best of our knowledge, there have been no previous reports of a patient with non-IgA DMP at the onset of NS who had later development of IgA nephropathy. The patient showed non-IgA DMP at the onset, suggesting that NS with non-IgA DMP and IgA nephropathy has some common pathophysiology. Treatment for NS, such as PSL and/or CyA treatment, may suppress the clinical manifestation of late IgA nephropathy.


Assuntos
Glomerulonefrite por IGA , Síndrome Nefrótica , Masculino , Humanos , Criança , Adolescente , Síndrome Nefrótica/complicações , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/tratamento farmacológico , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/diagnóstico , Glomerulonefrite por IGA/tratamento farmacológico , Hematúria/diagnóstico , Hematúria/etiologia , Prednisolona/uso terapêutico , Ciclosporina/uso terapêutico , Doença Crônica , Recidiva , Imunoglobulina A
19.
Int Urol Nephrol ; 56(1): 345-353, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37378850

RESUMO

BACKGROUND: Immunoglobulin A Nephropathy (IgAN) is a heterogeneous disorder. Multiple ethnicities conducted studies to assess the effectiveness of the Oxford classification of IgAN in prognostication. However, there is no study on the Pakistani population. We aim to identify its prognostic effectivity in our patients. METHODS: We retrospectively reviewed the medical records of 93 biopsy-proven cases of primary IgAN. We collected the clinical and pathological data at baseline and on follow-ups. The median follow-up period was 12 months. We defined the renal outcome as a ≥ 50% decline in eGFR or end-stage renal disease (ESRD). RESULTS: Of 93 cases, 67.7% were males with a median age of 29. Glomerulosclerosis was the most prevalent lesion (71%). The median MEST-C was 3. On follow-up, median serum creatinine worsened from 1.92 to 2.2 mg/dL, and median proteinuria reduced from 2.3 g/g to 1.072 g/g. The reported renal outcome was 29%. T and C scores and MEST-C scores above 2 were significantly associated with pre-biopsy eGFR. On Kaplan-Meier analysis, the T and C scores' association was significant with the renal outcome (p-value 0.000 and 0.002). In univariate and multivariate analyses, the association of T-score (p-value 0.000, HR 4.691), total MEST-C score (p-value 0.019), and baseline serum creatinine (p-value 0.036, HR 1.188) were significant with the outcome. CONCLUSION: We validate the prognostic significance of the Oxford classification. T and C scores, baseline serum creatinine, and total MEST-C score significantly affect the renal outcome. Furthermore, we recommend the inclusion of the total MEST-C score in determining the IgAN prognosis.


Assuntos
Glomerulonefrite por IGA , Falência Renal Crônica , Feminino , Humanos , Masculino , Creatinina , Progressão da Doença , Taxa de Filtração Glomerular , Glomerulonefrite por IGA/complicações , Glomerulonefrite por IGA/patologia , Rim/patologia , Falência Renal Crônica/complicações , Prognóstico , Estudos Retrospectivos , Adulto
20.
Int Urol Nephrol ; 56(1): 275-282, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37336802

RESUMO

PURPOSE: The relationship between serum phosphorus and immunoglobulin A (IgA) nephropathy progression remains uncertain, especially normal-range serum phosphorus. Therefore, we herein examined the relationship between the normal-range serum phosphorus and the progression of IgA nephropathy. METHODS: One hundred sixty-two patients with primary IgA nephropathy were divided into three groups according to tertiles of baseline serum phosphorus (first tertile: 0.73-1.04 mmol/L; second tertile: 1.04-1.21 mmol/L; third tertile: 1.21-1.60 mmol/L). Estimated glomerular filtration rate (eGFR) was calculated using the chronic kidney disease epidemiology collaboration. The composite outcome was defined as a decrease of at least 50% in eGFR from baseline or end-stage kidney disease (ESKD). The association of serum phosphorus with IgA nephropathy progression was estimated using Cox proportional hazards models, adjusting for potential confounders. RESULTS: During a median 16 month follow-up period, 15 patients reached a composite outcome. In the crude Cox proportional hazard model, baseline serum phosphorus as a continuous variable was associated with increased risk for adverse renal outcomes [hazard ratio (HR) = 63.510, 95% confidence interval (CI) = 3.953-1020.284, P = 0.003], and the high tertile of serum phosphorus group had an increased risk of the composite outcome by using the low tertile group as the reference (HR = 11.895, 95% CI = 1.522-92.993, P = 0.018). After adjustment for traditional risk factors, the high tertile of serum phosphorus group was significantly related to IgA nephropathy progression compared with the low tertile group (HR = 9.424, 95% CI = 1.019-87.165, P = 0.048). CONCLUSIONS: Relatively higher serum phosphorus levels within the normal range were significantly associated with the progression of IgA nephropathy.


Assuntos
Glomerulonefrite por IGA , Falência Renal Crônica , Humanos , Glomerulonefrite por IGA/complicações , Estudos Retrospectivos , Progressão da Doença , Rim , Falência Renal Crônica/complicações , Taxa de Filtração Glomerular , Fósforo
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