scholarly journals Glomerulonephritis with Crescents in Children: Etiology and Predictors of Renal Outcome

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
K. Alsaad ◽  
N. Oudah ◽  
A. Al Ameer ◽  
K. Fakeeh ◽  
A. Al Jomaih ◽  
...  

Objective. To investigate the clinicopathological features and outcome of glomerulonephritis with crescents among Saudi children. Method. This is a retrospective study of cases of crescentic glomerulonephritis (CrGN) seen over a 9-year period. Histological features and renal function were recorded. Results. Thirty-seven cases were enrolled. The mean percent of glomeruli with crescents was 39% (±19). Lupus nephritis (LN) was the commonest etiology (54.1%). At presentation, the serum creatinine (SCr) was 218.2 (±174.3) umol/l, and 57.1% of the cases had nephrotic range proteinuria. By the end of the observation period, SCr dropped to 81.0 (±67.7) umol/l (P=0.001). Worsening renal function was associated with younger age (P=0.002), non-LN etiology (P=0.01), more crescents (P=0.019), and ATN (P=0.05). At the end of the followup, more patients in the LN group were dialysis-free (P=0.017) and had improved renal function (0.01) than in the non-LN group. Using multivariate analysis, the only independent factor found to predict need for dialysis or change in SCr level was percent of globally sclerosed glomeruli (P=0.034). Conclusion. LN is the main cause of CrGN in our cohort of children. The LN group had less globally sclerorsed glomeruli and better renal prognosis than the non-LN group.

2018 ◽  
Vol 48 (2) ◽  
pp. 137-146
Author(s):  
Dan Zhang ◽  
Jiong Zhang ◽  
Shaoshan Liang ◽  
Jinquan Wang ◽  
Zhihong Liu

Background: Fabry disease (FD) with life-threatening complications occurs as a result of organ damage in kidneys, heart, and brain. Only a few studies, especially from Asia, report their long-term outcome. Methods: In this monocentric study, patients with Fabry nephropathy confirmed by renal biopsy were clinically investigated in a comprehensive manner. The clinic-pathological features, progression, and risk factors for the outcome were analyzed. Results: Thirty-one patients were recruited, after median 62 months (range 8–156 months) follow-up, 23 of them had stable renal function while 8 underwent renal function deterioration. Frequent presenting symptoms included acroparesthesia (58.1%), edema (51.6%), hypo- or anhidrosis (38.7%), and angiokeratoma (32.3%). Left ventricular hypertrophy was present in 62.5% patients with renal function deterioration and 17.4% patients with stable renal function (p = 0.03). The renal cumulative survival rate of all patients was 64.5% in 10 years. Mainz Severity Score Index (MSSI) and segmental sclerosis are independent predictive factors for a more rapid progression of Fabry nephropathy. The receiver operating characteristic analysis demonstrated that the area under the curve for the prediction of renal function progression on the basis of MSSI and segmental sclerosis levels in patients with FD was 0.845 and 0.780, respectively. MSSI score ≥18 or segmental sclerosis ≥3.9% in patients with FD positively correlated with poor renal prognosis. Conclusions: FD’s clinical manifestations are heterogeneous and nonspecific. The ­10-year cumulative renal survival rate was low in Chinese patients. MSSI score and segmental sclerosis levels predict the renal prognosis of patients with FD sensitively.


2001 ◽  
Vol 12 (7) ◽  
pp. 1493-1500 ◽  
Author(s):  
MOTOSHI HATTORI ◽  
HIDEAKI KURAYAMA ◽  
YASUSHI KOITABASHI

Abstract. A retrospective investigation was conducted by members of the Japanese Society for Pediatric Nephrology from 1990 to 1997 to define the clinical features and outcome of antineutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis in children. Thirty-four ANCA-seropositive Japanese pediatric patients with biopsy-proven pauci-immune necrotizing crescentic glomerulonephritis were identified. Of these, 3 cases associated with Wegener's granulomatosis were excluded because of the small sample size. Among the 31 patients studied, 10 had a diagnosis of necrotizing crescentic glomerulonephritis alone and 21 had microscopic polyangiitis. Females predominated (87%), and the median age at onset was 12 yr. Twenty-six patients received treatment with cyclophosphamide and corticosteroids, and five patients received treatment with corticosteroids alone; 84% of patients achieved remission, and 39% of responders relapsed in a median of 24 mo. ANCA titers correlated with response to treatment and disease activity, with some exceptions. Patients were followed for a median of 42 mo (range, 3 to 96 mo). Nine of 31 patients (29.0%) progressed to end-stage renal disease, 6 (19.4%) had reduced renal function, and 15 (48.4%) had normal renal function at the last observation. One patient (3.2%) died from cytomegalovirus infection 3 mo after initiation of therapy. Life-table analysis showed 75% renal survival at 39 mo. Patients who subsequently developed end-stage renal disease (n= 9) had significantly higher average peak serum creatinine levels and more chronic pathologic lesions at diagnosis compared with patients with favorable renal outcome (n= 15). In conclusion, our clinical experience suggests that the clinical disease spectrum of ANCA-associated glomerulonephritis is similar in pediatric and adult patients, but there is a female predominance in children.


2012 ◽  
Vol 25 (1) ◽  
pp. 287-292 ◽  
Author(s):  
A. Gigante ◽  
C. Salviani ◽  
K. Giannakakis ◽  
E. Rosato ◽  
B. Barbano ◽  
...  

Renal-limited vasculitis is a pauci-immune crescentic glomerulonephritis with no signs of systemic involvement, representing one of the most common causes of rapidly progressive glomerulonephritis. The study aims to examine clinical and histological features in twenty-four patients with RLV diagnosed by the Nephrology Department of Sapienza University of Rome, Italy, evaluating the role of these parameters in predicting renal survival. Patients details, clinical and histological features and outcomes were recorded at the time of renal biopsy and over a mean follow-up period of 36±6 months. In our study, serum creatinine at presentation was significantly higher in patients who had a poor outcome than in those who survived with independent renal function (6.3±2.47 mg/dl vs 2.84±2.01 mg/dl, P= 0.002). The presence of C3c was found in the area of glomerular fibrinoid necrosis and in small arteries and arterioles with fibrinoid necrosis in 17 patients (P= 0.018). In conclusion, serum creatinine at presentation and focal C3c depositions in areas of glomerular and arteriolar fibrinoid necrosis were the best determinants of poor renal outcome, maybe underlining the pathogenic role of alternative pathway activation of complement system but also demonstrating the focal distribution of necrotizing lesions.


2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Yi-Chia Chan ◽  
Kai-Min Liu ◽  
Chao-Long Chen ◽  
Aldwin D. Ong ◽  
Chih-Che Lin ◽  
...  

Abstract Background and aims Glycogen storage disease type I (GSD-I) is an autosomal recessive disorder of carbohydrate metabolism, resulting in limited production of glucose and excessive glycogen storage in the liver and kidneys. These patients are characterized by life-threatening hypoglycemia, metabolic derangements, hepatomegaly, chronic kidney disease, and failure to thrive. Liver transplantation (LT) has been performed for poor metabolic control and delayed growth. However, renal outcome was diverse in pediatric GSD patients after LT. The aim of this study was to investigate the long-term outcome of renal function in pediatric GSD-I patients after living donor LT (LDLT), and to identify modifiable variables that potentially permits LT to confer native renal preservation. Methods The study included eight GSD-Ia and one GSD-Ib children with a median age of 9.0 (range 4.2–15.7) years at the time of LT. Using propensity score matching, 20 children with biliary atresia (BA) receiving LT were selected as the control group by matching for age, sex, pre-operative serum creatinine (SCr) and pediatric end-stage liver disease (PELD) score. Renal function was evaluated based on the SCr, estimated glomerular filtration rate (eGFR), microalbuminuria, and morphological changes in the kidneys. Comparability in long-term renal outcome in terms of anatomic and functional parameters will help to identify pre-LT factors of GSD-I that affect renal prognosis. Results The clinical and biochemical characteristics of the GSD and BA groups were similar, including immunosuppressive regimens and duration of follow-up (median 15 years) after LT. Overall, renal function, including eGFR and microalbuminuria was comparable in the GSD-I and BA groups (median eGFR: 111 vs. 123 ml/min/1.73m2, P = 0.268; median urine microalbuminuria to creatinine ratio: 16.0 vs. 7.2 mg/g, P = 0.099, respectively) after LT. However, in the subgroups of the GSD cohort, patients starting cornstarch therapy at an older age (≥ 6-year-old) before transplantation demonstrated a worse renal outcome in terms of eGFR change over years (P < 0.001). In addition, the enlarged kidney in GSD-I returned to within normal range after LT. Conclusions Post-LT renal function was well-preserved in most GSD-I patients. Early initiation of cornstarch therapy before preschool age, followed by LT, achieved a good renal prognosis.


2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Mineaki Kitamura ◽  
Yasushi Mochizuki ◽  
Tsuyoshi Matsuda ◽  
Yuta Mukae ◽  
Hiromi Nakanishi ◽  
...  

Abstract Background Higher serum uric acid (UA) levels are associated with poorer renal prognosis. In kidney transplantation, both donors and recipients are diagnosed as having chronic kidney diseases (CKD) based on renal function; however, their UA levels slightly vary. Elucidating the differences in UA would help improve kidney prognosis, especially for recipients. Therefore, we investigated UA levels in kidney transplant recipients by comparing them to those in their donors. Methods In this retrospective cross-sectional survey, background information and blood examination results were collected from the donors just before donation and after transplantation in the donors and recipients. Associations between UA and sex estimated glomerular filtration rate (eGFR), and body mass index (BMI) were evaluated. Data were assessed by the Wilcoxon rank-sum test for continuous variables and the chi-squared test for categorical variables; multiple linear regression analyses were performed to determine which factors were associated with renal function before and after transplantation. Results Participant characteristics were as follows. The mean donor age (n = 45, 16 men and 29 women) was 55 ± 11 years, and the mean recipient age (n = 45, 25 men and 20 women) was 46 ± 16 years. Sex-related differences (UA levels in men were predominant) existed in the UA of donors before (P < 0.001) and after donation (P < 0.001). Conversely, there were no significant sex-related differences in the UA of recipients (P = 0.51); the mean standardized eGFRs were similar in donors and recipients after transplantation. Multivariate linear regression analysis showed donor UA only correlated with donor sex before donation (P = 0.008). After donation, donor UA was associated with donor sex (P = 0.006), eGFR (P < 0.001), and BMI (P = 0.02). Notably, the UA of recipients after transplantation was only associated with eGFR (P = 0.003). Conclusions Sex has less impact on UA in recipients than in donors. UA has a greater impact on renal prognosis in women than men, even at the same UA level. Therefore, attention should be given to UA levels in female recipients. These findings can be useful for determining patient prognosis following kidney transplantation in both donors and recipients.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
HIROAKI TANAKA ◽  
Takayuki Fujii ◽  
JUNYA KOSHIZAKA ◽  
NOBUAKI YAMAUCHI ◽  
MAYU MORIMOTO ◽  
...  

Abstract Background and Aims In the treatment of idiopathic focal segmental glomerulosclerosis (FSGS) with nephrotic syndrome, the remission of proteinuria is considered to be an important goal. The partial remission of proteinuria improves renal survival, whereas it may progressively reduce the renal function. A study searched for a novel partial remission more accurately reflecting the long-term renal outcome. The goal of proteinuria reduction for improving the renal prognosis remains to be clarified. We examined factors associated with the long-term renal outcome of idiopathic FSGS. Method Of 148 patients with FSGS diagnosed based on kidney biopsy between 1981 and 2018, a retrospective cohort study was conducted involving 33 who had undergone immunosuppressive therapy for nephrotic syndrome, and had been followed-up for ≥1 year, excluding those with secondary FSGS. We examined the renal prognosis, regarding a 50% decrease in the estimated glomerular filtration rate (eGFR) as an outcome. We calculated the rate of decrease in the urinary protein level 4 and 8 months after the start of treatment, and estimated the rate of decrease associated with renal hypofunction using ROC analysis. Based on the results of ROC analysis, Cox’s proportional hazard analysis was performed using factors contributing to renal hypofunction as covariates. Results Concerning the background of the 33 patients, the mean follow-up period was 11.4 years, and there were 24 males. The mean age was 49.8 years, and the mean blood pressure was 100.5 mmHg. The mean urinary protein level, albumin (Alb) level, eGFR, and total cholesterol (TCho) level were 7.4 g/day, 2.1 g/dL, 44.3 mL/min/1.73 m2, and 369 mg/dL, respectively. Corticosteroid therapy was selected in 21 patients, whereas it was combined with steroid pulse therapy in 12. The daily dose of prednisolone was 37.3 mg. On ROC analysis, the rate of decrease in the urinary protein level after 4 months was 83.1% (AUC: 0.74, sensitivity: 0.80, specificity: 0.74), and that after 8 months was 85.7% (AUC: 0.78, sensitivity: 0.90, specificity: 0.65). Cox’s proportional hazard analysis, in which the data were adjusted with the sex, blood pressure, urinary protein level at the start of treatment, Alb level, eGFR, and treatment methods, showed that the rate of decrease in the urinary protein level after 4 months was significantly correlated with renal hypofunction: after 4 months: hazard ratio, 0.19 (95%CI: 0.04-0.77); p=0.0202; after 8 months: hazard ratio, 0.34 (95%CI: 0.05-1.37); p=0.1359. Conclusion In the treatment of idiopathic FSGS with nephrotic syndrome, the rate of decrease in the urinary protein level 4 months after the start of treatment was correlated with the long-term renal outcome.


1998 ◽  
Vol 9 (10) ◽  
pp. 1915-1923 ◽  
Author(s):  
C F Franssen ◽  
C A Stegeman ◽  
W W Oost-Kort ◽  
C G Kallenberg ◽  
P C Limburg ◽  
...  

Patients with anti-myeloperoxidase (MPO)-associated necrotizing crescentic glomerulonephritis (NCGN) may develop chronic renal failure (CRF) leading to end-stage renal disease despite an initially favorable response to treatment. The aim of this study was to determine the prognostic value of clinical, laboratory, and histopathologic features at the time of presentation and during follow-up for the development of CRF in 21 consecutive anti-MPO-positive patients with NCGN. Renal function did not recover in two of five patients who were dialysis-dependent at presentation. The remaining 19 patients all went into remission and were off dialysis at 3 mo after diagnosis. At long-term follow-up, nine of these patients had stable renal function and did not relapse (group A), five patients developed CRF without signs of relapse (group B), and five patients relapsed (group C). At diagnosis, serum creatinine, C-reactive protein, and anti-MPO levels did not differ between groups A, B, and C. Microscopic erythrocyturia resolved in all patients within 4 mo of treatment. BP at presentation and during follow-up did not differ between groups A, B, and C. Proteinuria at diagnosis and in the first 6 mo after diagnosis was higher in patients who developed CRF than in patients with a stable renal function. Anti-MPO levels at 3 mo had decreased compared with anti-MPO levels at diagnosis in groups A and C, whereas anti-MPO levels did not fall significantly in patients who developed CRF. The predictive value of a renal biopsy at diagnosis on long-term renal outcome was limited. In conclusion, a higher degree of proteinuria at diagnosis and during follow-up as well as persistently elevated anti-MPO levels after induction of remission are associated with the development of CRF and are predictive of poor renal outcome in anti-MPO-associated NCGN.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
N Tanaka ◽  
K Inoue ◽  
M Masuda ◽  
Y Furukawa ◽  
A Hirata ◽  
...  

Abstract Background Atrial fibrillation (AF) reduces the renal function. Renal dysfunction and AF often coexist. Catheter ablation (CA) of persistent AF can maintain a sinus rhythm and may improve the renal function. Purpose We sought to elucidate whether the estimated glomerular filtration rate (eGFR) in patients with persistent AF was increased after CA, especially with the presence of an AF recurrence. Methods We enrolled 487 persistent AF patients whose eGFR data were available both before and 1-year after the CA out of 512 patients in the EARNEST-PVI trial. Results The mean age was 65±9 year and 113 patients (24.8%) had long-standing persistent AF. We compared the eGFR at baseline with that 1-year after the CA. AF recurrences were recognized in 118 patients (25.8%). The eGFR was similar between the group without recurrence and that with recurrence at baseline (without AF recurrence vs. with AF recurrence; 63.8±14.3 vs. 62.7±13.6 mL/min/1.73m2, p=0.46). In patients without AF recurrence, the G1, G2, G3a, G3b, G4, and G5 were 13 (3.8%), 198 (58.4%), 98 (28.9%), 26 (7.7%), 3 (0.9%), and 1 (0.3%), respectively at baseline. In the patients with AF recurrence, the G1, G2, G3a, G3b, G4, and G5 were 3 (2.5%), 68 (57.8%), 38 (32.2%), 6 (5.1%), 3 (2.5%), and 0 (0%), respectively at baseline. The ΔeGFR was significantly higher in the patients without AF recurrence than in those with AF recurrence (without AF recurrence vs. with AF recurrence; 5.1 [−0.3, 10.8] vs. 3.0 [−3.0, 7.6], p=0.0033). In the patients without AF recurrence, a better eGFR class at 1-year after the CA than in those before the CA was recognized in 75 patients (22.1%), while it was recognized in 19 patients (16.1%) with AF recurrences. Conclusion Successful catheter ablation in patients with persistent AF led to a better renal outcome. FUNDunding Acknowledgement Type of funding sources: Private company. Main funding source(s): This study was funded by Medtronic, Johnson & Johnson, and Abbott.


2001 ◽  
Vol 40 (04) ◽  
pp. 107-110 ◽  
Author(s):  
B. Roßmüller ◽  
S. Alalp ◽  
S. Fischer ◽  
S. Dresel ◽  
K. Hahn ◽  
...  

SummaryFor assessment of differential renal function (PF) by means of static renal scintigraphy with Tc-99m-dimer-captosuccinic acid (DMSA) the calculation of the geometric mean of counts from the anterior and posterior view is recommended. Aim of this retrospective study was to find out, if the anterior view is necessary to receive an accurate differential renal function by calculating the geometric mean compared to calculating PF using the counts of the posterior view only. Methods: 164 DMSA-scans of 151 children (86 f, 65 m) aged 16 d to 16 a (4.7 ± 3.9 a) were reviewed. The scans were performed using a dual head gamma camera (Picker Prism 2000 XP, low energy ultra high resolution collimator, matrix 256 x 256,300 kcts/view, Zoom: 1.6-2.0). Background corrected values from both kidneys anterior and posterior were obtained. Using region of interest technique PF was calculated using the counts of the dorsal view and compared with the calculated geometric mean [SQR(Ctsdors x Ctsventr]. Results: The differential function of the right kidney was significantly less when compared to the calculation of the geometric mean (p<0.01). The mean difference between the PFgeom and the PFdors was 1.5 ± 1.4%. A difference > 5% (5.0-9.5%) was obtained in only 6/164 scans (3.7%). Three of 6 patients presented with an underestimated PFdors due to dystopic kidneys on the left side in 2 patients and on the right side in one patient. The other 3 patients with a difference >5% did not show any renal abnormality. Conclusion: The calculation of the PF from the posterior view only will give an underestimated value of the right kidney compared to the calculation of the geometric mean. This effect is not relevant for the calculation of the differntial renal function in orthotopic kidneys, so that in these cases the anterior view is not necesssary. However, geometric mean calculation to obtain reliable values for differential renal function should be applied in cases with an obvious anatomical abnormality.


1998 ◽  
Vol 37 (02) ◽  
pp. 130-133
Author(s):  
T. Kishimoto ◽  
Y. Iida ◽  
K. Yoshida ◽  
M. Miyakawa ◽  
H. Sugimori ◽  
...  

AbstractTo evaluate the risk factors for hypercholesterolemia, we examined 4,371 subjects (3,207 males and 1,164 females) who received medical checkups more than twice at an AMHTS in Tokyo during the period from 1976 through 1991; and whose serum total cholesterol was under 250 mg/dl. The mean follow-up duration was 6.6 years. A self-registering questionnaire was administered at the time of the health checkup. The endpoint of this study was the onset of hypercholesterolemia when the level of serum total cholesterol was 250 mg/dl and over. We compared two prognosis groups (normal and hypercholesterol) in terms of age, examination findings and lifestyle. After assessing each variable, we employed Cox's proportional hazards model analysis to determine the factors related to the occurrence of hypercholesterolemia. According to proportional hazards model analysis, total cholesterol, triglyceride and smoking at the beginning, and hypertension during the observation period were selected in males; and total cholesterol at the beginning and age were selected in females to determine the factors related to the occurrence of hypercholesterolemia.


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