Background Lupus nephritis is an uncommon severe glomerulonephritis which on kidney biopsy is classified into class I-V. The aim of this study was to describe differences in presentation, treatment and outcome between children with the different biopsy classes. Methods We compared, in a cohort of 428 children, clinical and laboratory data at onset, two-year and last follow-up (median of four years and four month) between children with the different biopsy classes. Results The number of children with the biopsy classes III, IV, V and mixed V and III or IV, were 132, 191, 43 and 62 respectively. At onset did children with a biopsy pattern of class III and IV show lower haemoglobin levels, 99 and 96 g/L, vs 104 and 112.5 g/L, respectively and lower complement C3 levels 0.37 and 0.35 g/L vs 0.44 and 0.59. Levels of double stranded DNA antibodies (dsDNA) and ESR showed no statistically significant differences between the groups. Reduced eGFR was found significantly more often in children with class IV compared to the other groups of children. Children with mixed class V and III or IV had the highest levels of proteinuria and lowest serum albumin levels. Treatment of the children in the different groups was mainly similar with the exceptions of less frequent use of initial methylprednisolone and cyclophosphamide in children with pure class V. At two years and last follow up no differences in neither inflammatory parameters nor complement and dsDNA levels were found. eGFR was also not significantly different at follow-up while between 9.6 and 31.9 percent of the children still showed impaired kidney function. Children with class V combined with III or IV did most often show continuing proteinuria. Discussion At onset children in the biopsy classes with proliferative glomerulonephritis showed more haematological signs of inflammation while those with class V, membranous nephritis, had higher levels of proteinuria. Children with mixed biopsy phenotypes showed a combination of these findings. Treatment and outcome were however surprisingly similar in children from all biopsy groups.

Lupus nephritis (LN) is a very severe manifestation of lupus. There is no consensus on which treatment goals should be achieved to protect kidney function in children with LN. We retrospectively analyzed trends of commonly used laboratory biomarkers of 428 patients (≤ 18 years old) with biopsy-proven LN class ≥ III. We compared data of patients who developed stable kidney remission from 6 to 24 months with those who did not. Twenty-five percent of patients maintained kidney stable remission while 75

Children with lupus have a higher chance of nephritis and worse kidney outcome than adult patients. We retrospectively analyzed clinical presentation, treatment and 24-month kidney outcome in a cohort of 382 patients (≤ 18 years old) with lupus nephritis (LN) class ≥ III diagnosed and treated in the last 10 years in 23 international centers. The mean age at onset was 11 years 9 months and 72.8

OBJECTIVE:We studied the rate of remission of LN in an international cohort of 248 children and adolescents with biopsy-proven LN. Five different definitions from scientific studies and the definitions recommended by the ACR and Kidney Disease: Improving Global Outcomes were used. METHODS:Anonymized clinical data in patients with biopsy-proven LN class ≥III (International Society of Nephrology/Royal Pathology Society) diagnosed and treated in the last 10 years in 23 international centres from 10 countries were collected. We compared the rate of patients in complete and partial remission applying the different definitions. RESULTS:The mean age at diagnosis was 11 years and 4 months, and 177 were females. The number of patients in complete and partial remission varied a great deal between the different definitions. At 24 months, between 50% and 78.8% of the patients were in full remission as defined by the different criteria. The number of patients in partial remission was low, between 2.3% and 25%. No difference in achieved remission was found between boys and girls or between children and adolescents (P > 0.05). Patients with East Asian ethnicity reached remission more often than other ethnicities (P = 0.03-0.0008). Patients treated in high-income countries showed a higher percentage of complete remission at 12 and 24 months (P = 0.002-0.000001). CONCLUSION:The rate of children and adolescents with LN achieving remission varied hugely with the definition used. Our results give important information for long-awaited treatment studies in children and young people.

Abstract Background and Aims Lupus Nephritis (LN) occurs in up to 80% of children with SLE and it affects the long term outcome and the overall survival. Achieving and maintaining renal remission is crucial. However definition of remission in children is not clearly defined. We compared the outcomes using different published definitions of complete and partial remission. Method 248 children with biopsy proven LN class III or higher (ISN/RPS) diagnosed and treated in 23 international centers in the last 10 years were included. Data regarding their renal outcome were collected for twenty-four months after the start of induction therapy. We applied seven definitions of remission to compare the number of children achieving partial and complete remission. Definitions applied have been adapted from the Bristol-Myers Squibb (BMS) trial, the American College of Rheumatology (ACR) recommendations, the Lupus Nephritis Assesment with Rituximab (LUNAR) trial, the Aspreva Lupus Management Study (ALMS) trial, the Abatacept and Cyclophosphamide Combination: Efficacy and Safety Study (ACCESS) trial, the Kidney Disease Improving Global outcomes (KDIGO) guidelines and the Two-Year, Randomized, Controlled Trial of Belimumab in Lupus Nephritis (BLISS-LN). We also focused on the BMS trial, the ACCESS trial and the KDIGO guidelines definitions to analyse the importance of gender, age, ethnicity and the economic income of the country (as defined by the World Bank) where patients had been treated. Results The mean age at diagnosis was 11 years and 4 month. 71.4% were females. They were mainly East-Asian (34.3%), South-Asian (24.6%) and Caucasian (18.6%). 42.7 % were from middle income countries and 57.3% high income countries. The kidney biopsies showed LN class III in 35.5%; class IV in 45.6% and class V in 18.9%. The different definitions varied significantly in terms of outcomes, with that of the ACCESS trial having the highest percentages of complete remission and the BMS trial the lowest (Figure 1). A relatively small percentage of children achieved partial remission during the follow-up for all the definitions (Figure 2). Focusing on the BMS, ACCESS and KDIGO definitions, we found no statistically significant differences of gender and age in the rate of children entering complete remission at 6, 12 and 24 months. East Asian children did however achieve remission more often than other ethnic groups (p < 0.05) (Figure 3). Children treated in high income countries showed a statistically significant higher percentage of complete remission at 12 and 24 months (p < 0.05) (Figure 4). Conclusion Rate of complete and partial remission varied considerably when using the different definitions. Ethnicity and income of the country where the patients were treated did influence outcome. The findings of our study can help in deciding how to define remission in urgently needed future treatment studies in children.

BackgroundSystemic lupus erythematosus (SLE) is a severe and life-long disease. Lupus Nephritis (LN) can occurs in up to 80% of children with SLE and it affects the long term renal outcome and the overall survival. Achieving and maintaining renal remission is essential. However definitions of complete and partial remission in children are not well defined.ObjectivesWe compared renal outcomes using different published definitions of complete and partial remission.Methods248 children with biopsy proven LN class III or higher (ISN/RPS) diagnosed and treated in 23 international centers in the last 10 years were included. Data regarding their renal outcome were collected for twenty-four months after the start of induction therapy. We applied seven definitions of remission to compare the number of children achieving partial and complete remission. Definitions applied have been adapted from the Bristol-Myers Squibb (BMS) trial, the American College of Rheumatology (ACR) recommendations, the Lupus Nephritis Assesment with Rituximab (LUNAR) trial, the Aspreva Lupus Management Study (ALMS) trial, the Abatacept and Cyclophosphamide Combination: Efficacy and Safety Study (ACCESS) trial, the Kidney Disease Improving Global outcomes (KDIGO) guidelines and the Two-Year, Randomized, Controlled Trial of Belimumab in Lupus Nephritis (BLISS-LN). We also focused on the BMS trial, the ACCESS trial and the KDIGO guidelines definitions to analyse the importance of gender, age, ethnicity and the economic income of the country (as defined by the World Bank) where patients had been treated.ResultsThe mean age at diagnosis was 11 years and 4 month. 71.4% were females. They were mainly East-Asian (34.3%), South-Asian (24.6%) and Caucasian (18.6%). 42.7% were from middle income countries and 57.3% high income countries. The kidney biopsies showed LN class III in 35.5%; class IV in 45.6% and class V in 18.9%. The different definitions varied substantially in terms of outcomes, with that of the ACCESS trial having the highest percentages of complete remission and the BMS trial the lowest. A relatively small percentage of children achieved partial remission during the follow-up for all the definitions. Focusing on the BMS, ACCESS and KDIGO definitions, we found no statistically significant differences of gender and age in the rate of children entering complete remission at 6, 12 and 24 months. East Asian children however achieved remission more often than other ethnic groups (p < 0.05). Children treated in high income countries showed a statistically significant higher percentage of complete remission at 12 and 24 months (p < 0.05).ConclusionsRate of complete and partial remission varied hugely applying the different definitions. Ethnicity and income of the country where the patients were treated influenced outcomes. Our results can help in deciding how to define remission in urgently needed future treatment studies in children and adolescents.

IntroductionSystemic lupus erythematosus is a chronic and severe disease characterized by flares and remissions where renal involvement is strongly related to the long term prognosis. Close monitoring of the children with lupus nephritis is fundamental for long term survival. The aim of this study was to describe trends of routine laboratory biomarkers after initial treatment in an international cohort of children with SLE nephritis.MethodsChildren (<17 years old) with ≥ class III biopsy (ISN/RPS) diagnosed and treated in the last 10 years in 23 international centers were included. Laboratory biomarkers at specific time points were retrospectively analyzed from diagnosis to 2 years follow-up and at last available follow-up and reported as medians (IQR).Results382 patients (278 girls), mean age 11 years, were included. 30% of patients were Asian, 23% Indian, 18.5% Caucasian, 7.8% Hispanic, 4% Subs African/African American, 2% Heterogeneous, 0.3% Semitic-Arabic. At diagnosis eGFR was 94 (64–119) ml/min/1.73 m2 and increased to 105 (83–128) ml/min/1.73 m2 within the first month. Initially Urine-Protein/Urine-Creatinine (UPr:UC) was in nephrotic range in 48% of cases, 2 (0.6–43) mg/mg, and improved during the first 6 months but was still raised in 24% of patients at 12 month-follow-up. Serum albumin was 31 (23–37) g/L and normalized within the first month in most children. Haemoglobin was initially 99 (84.2–115) g/L and improved in 2 months, however about 40% of patients had persistent low values below 120 g/L during follow-up. ESR was 49 (21–76) mm/hr and declined mainly during the first 3 months, but 49.7% of patients still had high ESR at 24 months. At diagnosis double stranded DNA was raised to 194.5 (29–401) IU/ml, rapidly decreased in the first three months and only normalized in half of the patients. C3 was 0.38 (0.8–0.6) g/L and C4 0.07 (0.04–0.15) g/L and improved within 2 months.ConclusionIn this cohort of children with lupus nephritis haemoglobin, serum albumin and complement showed the fastest improvements after start of treatment; often within two months. Other parameters like ESR, dsDNA and proteinuria took much longer to improve and in a substantial proportion of the children never reached normal values.

Hypokalemia and metabolic alkalosis can be present in different rare diseases, and the differential diagnosis of these forms is challenging. Apparent mineralcorticoid (AME) excess syndrome is one of these conditions. Characterized by increased blood pressure due to excessive sodium retention and plasma volume, it is caused by a mutation in the HSD11B2 gene encoding the oxydoreductase enzyme 11β-hydroxysteroide dehydrogenase type 2. We report the case of a child presenting with failure to thrive associated with early detection of hypokalemia, metabolic alkalosis, nephrocalcinosis and hypertension in which AME syndrome was detected. A novel mutation in the HSD11B2 gene was identified in this patient. In clinical pictures characterized by metabolic alkalosis and hypokalemia, the evaluation of renin, aldosterone and blood pressure is crucial for accurate diagnosis. AME syndrome is a rare disorder that can be an insidious but lethal disease, if untreated. With clinical signs appearing during the first days of life. Early diagnosis is imperative in order to enable prompt and adequate treatment to improve the outcome of these patients.

Rationale & Objective: The objective of this study is to evaluate the effect of body mass index (BMI) on estimated glomerular filtration rate (eGFR) levels in children with congenital solitary kidney (CSK). Moreover, we evaluated if other factors could influence this relationship. Study Design: Multicenter cross-sectional study. Setting & Participants: University hospital pediatrics departments. Subjects: Two hundred eighty-one patients with CSK. Predictors: Weight, height, BMI-SDS (standard deviation score), duration of overweight/obesity, pubertal stage, systolic (SBP) and diastolic (DBP) blood pressure, eGFR, and renal ultrasound were obtained at the last follow-up visit. The population was classified on the basis of nutritional status and divided in tertiles for duration of overweight/obesity. We compared eGFR levels among these categories. A simple regression was used to correlate eGFR with BMI-SDS. To evaluate if other factors could influence the relationship between eGFR and BMI-SDS, a general linear model was performed, including gender, birth weight,2.5 kg, age, BMI-SDS, SBP-SDS, DBP-SDS, RL-SDS (renal length), and presence of kidney injury at last follow-up as covariates. Results: The eGFR levels reduced gradually from underweight to obese patients (P = .047). The eGFR levels significantly increased across first and second tertiles of duration of overweight/obesity while they decreased across second and third tertiles of duration of overweight/obesity (P = .005). The eGFR and BMI-SDS at last follow-up were indirectly correlated (coefficient = -30, r(2) = 9.2%, P = .0004). A general linear model for eGFR variance (model R-2 = 26.37%; P = .02) confirmed an indirect and significant association of eGFR values with BMI-SDS as the only significant finding. Conclusions: In patients with CSK, the higher the BMI-SDS and the duration of overweight/obesity, the lower the eGFR levels. Primary prevention strategies to counteract overweight/obesity are mandatory in CSK patients. (C) 2019 by the National Kidney Foundation, Inc. All rights reserved.

PURPOSE:We assessed renal function outcome in children with congenital solitary kidney and evaluated prognostic risk factors.MATERIALS AND METHODS:We retrospectively studied the clinical charts of 210 children presenting with congenital solitary kidney at 2 pediatric nephrology and 5 pediatric units between January 2009 and October 2012. Children 0 to 18 years old with a congenital solitary kidney confirmed by scintigraphy were enrolled. Of the patients 146 were suitable for analysis. Median followup was 4.6 years. Primary outcome was decreased estimated glomerular filtration rate, and secondary outcome was occurrence of proteinuria and/or systemic hypertension. Primary outcome-free survival analysis was performed, including multiple regression analysis of significant risk factors.RESULTS:Decreased estimated glomerular filtration rate was present in 12% of children at a median age of 2.2 years. Primary outcome-free survival analysis revealed an estimated event-free survival of 82% (95% CI 74% to 91%) at 10 years. Estimated survival rate was significantly decreased in children with additional congenital anomalies of the kidney/urinary tract (54% vs 88% overall) or insufficient renal length vs expected for normal congenital solitary kidney. The latter was the strongest predictor of decreased estimated outcome-free survival (49% vs 89%, p <0.001). Occurrence of proteinuria and/or systemic hypertension was present in less than 5% of children.CONCLUSIONS:Some children with congenital solitary kidney show decreased glomerular filtration rate. Associated anomalies of the kidney/urinary tract and insufficient renal length appear to be significant risk factors. Adequate length of the congenital solitary kidney is a key parameter for maintenance of renal function and should be examined routinely during followup.

Dent disease is a rare X-linked tubulopathy with low molecular weight proteinuria, hypercalciuria, nephrolithiasis, nephrocalcinosis and progressive renal failure. We describe the case of a 9-year-old boy who presented with nephrotic-range albuminuria at the age of 3 years. In the absence of a clear diagnosis, a renal biopsy was performed at 4 years, which revealed minimal change disease. Due to the presence of low molecular weight proteinuria, even in the absence of hypercalciuria, a diagnosis of Dent disease was considered. While there were no mutations in the CLCN5 gene, the diagnosis was confirmed by the presence of a missense mutation (p.Arg318Cys) in the OCRL gene. Conclusion: Given the large phenotypic variability of the disease and based on our experience, we believe that children with low molecular weight proteinuria, even without hypercalciuria, should be investigated for Dent disease.

OBJECTIVE:To evaluate the yield, economic, and radiation costs of 5 diagnostic algorithms compared with a protocol where all tests are performed (ultrasonography scan, cystography, and late technetium(99)dimercaptosuccinic acid scan) in children after the first febrile urinary tract infections.METHODS:A total of 304 children, 2 to 36 months of age, who completed the diagnostic follow-up (ultrasonography, cystourethrography, and acute and late technetium(99)dimercaptosuccinic acid scans) of a randomized controlled trial (Italian Renal Infection Study 1) were eligible. The guidelines applied to this cohort in a retrospective simulation were: Melbourne Royal Children's Hospital, National Institute of Clinical Excellence (NICE), top down approach, American Academy of Pediatrics (AAP), and Italian Society of Pediatric Nephrology. Primary outcomes were the yield of abnormal tests for each diagnostic protocol; secondary outcomes were the economic and radiation costs.RESULTS:Vesicoureteral reflux (VUR) was identified in 66 (22%) children and a parenchymal scarring was identified in 45 (15%). For detection of VUR (47/66) and scarring (45/45), the top down approach showed the highest sensitivity (76% and 100%, respectively) but also the highest economic and radiation costs (€52 268. 624 mSv). NICE (19/66) and AAP (18/66) had the highest specificities for VUR (90%) and the Italian Society of Pediatric Nephrology had the highest specificity (20/45) for scars (86%). NICE would have been the least costly (€26 838) and AAP would have resulted in the least radiation exposure (42 mSv).CONCLUSIONS:There is no ideal diagnostic protocol following a first febrile urinary tract infection. An aggressive protocol has a high sensitivity for detecting VUR and scarring but carries high financial and radiation costs with questionable benefit.

Abstracts for IPNA Congress in Shanghai, China 2013s for IPNA Congress in Shanghai, China 2013 CAKUT: Ante/neonatal diagnosis Abstract# PSUN001 Congenital mesoblasticnephroma, Report of a case PSUN001 Congenital mesoblasticnephroma, Report of a case Richard Baquero Rodriguez, iliana Rubio Elorza, Luis Fernando Arias3, Margarita Tamayo4,Jose David Garcias 1 Pediatria y puericultura, Fellownefrologiapediatrica, Medellin, Colombia 2 Pediatria, Universidad de Antioquia, Medellin, Colombia 3 SeccionNefrologiaInfantil, NefrologaPediatra, Medellin, Colombia 4 Patologia, Hospital Universitario San Vicente Fundacion, Medellin, Colombia 5 Radiologia e imagenesdiagnosticas, Faculta de medicina, Medellin, Colombia Objective: Congenitalmesoblasticnephromais a congenital renal neoplasia. Diagnosed in the first six months of life. Its clinical behavior is benign and the treatment is surgical. StudyTo describe the case of a patientwith congenitalmesoblasticnephroma preterm and its diagnostic and therapeutic approach Methods: We report the case of a 30-week preterm patient with a history of perinatal asphyxia, requiring advanced neonatal resuscitation (perinatal trauma), initially with hematuria box that subsequently joined abdominal mass and hypertension, so suspected renal trauma (renal hematoma) performing imaging studies, information describing the review is based on your medical history, prior parental consent. Results: evidenced by renal ultrasonography enlarged right kidney with heterogeneous solid-looking lesion in the lower pole, with cystic areas suggesting dystrophic calcifications, suggestive of embryological renal tumor. It interconsultation child surgery, who performed right nephrectomy, Pathology confirmed the diagnosis of congenital mesoblasticnephroma Conclusions: the importance of history is key to the diagnosis, all preterm neonate with a history of asphyxia and perinatal trauma suspected who this hematuria, hypertension and abdominal mass should suspect this disease, early diagnosis is important to define management and prognosis thereof, adequate renal ultrasound is essential for diagnosis. Abstract# PSUN002 The cause and outcome of fetal hydronephrosis PSUN002 The cause and outcome of fetal hydronephrosis Behnaz Falakolaflaki, Mohammad Reza Jamshidi 1 Pediatrics,Zanjan University of Medical Sciences, Zanjan, Iran 2 Anesthesiology,Zanjan University of Medical Sciences, Zanjan, Iran Objective: Hydronephrosis is probably the most prevalent congenital abnormality detected prenatally by ultrasonography (1-5% of all pregnancies). The aim of this study was to determine the cause & postnatal outcome of fetal hydronephrosis. Method: In this retrospective cohort study, 250 term infants with prenatally diagnosed hydronephrosis and the diagnosis were confirmed postnatally, were enrolled. The degree of hydronephrosis was defined as mild (6 to <10 mm), moderate (10 to <15 mm) or severe (>15) using anteriorposterior diameter of the renal pelvis in the third trimester. Postnatal sonography was performed 3-7 days after birth. Voiding cystourethrogram was performed in 6-8 weeks time. In the absence of vesicoureteral reflux (VUR), DTPA scan was performed to exclude obstruction. Follow-up period was 48 months. The events of interest were course (resolution, surgical intervention, and no change), urinary tract infection (UTI), hypertension, chronic kidney disease (CKD), and death. Results: Of 250 cases with hydronephrosis, 135(54%) were mild, 84(33.6%) were moderate, and 31(12.4%) were severe. Hydronephrosis was caused by VUR in104(41.6%), ureteropelvic junction obstruction (UPJO) in 59(23.6%), ureterovesical junction obstruction in 27(10.8%), cystic diseases in 19(7.6%), posterior urethral valves in 2(0.8%), prunebelly syndrome in 1 (0.4%), and idiopathic in 38 (15.2%).During follow-up19 patients (7.6%) required surgical intervention while 177(70.8%) improved and 54 (21.6%)were with no change. UTI occurred in 93(37.2%) and 11(4.4%) patients developed CKD. The risk of CKD, UTI and surgery were greater in patients with severhydronephrosis (P< 0.05). Conclusion: In this study VUR and UPJO were the most common cause of hydronephrosis. There was a meaningful relationship between the degree of hydronephrosis and postnatal problems (UTI, CKD & surgical intervention). But any degree of fetal hydronephrosis is at risk of postnatal pathology. Abstract# PSUN003 Outcome of ureterocele in antenatally detected duplex kidneys PSUN003 Outcome of ureterocele in antenatally detected duplex kidneys Hannah L Bromley, Ashok D Ram, Robert Bryan Beattie, Rajesh G Krishnan 1 Children's Kidney Centre, University Hospital of Wales, Cardiff, United Kingdom 2 Department odPaediatric Surgery, University Hospital of Wales, Cardiff, United Kingdom 3 Department of Foetal Medicine, University Hospital of Wales, Cardiff, United Kingdom Objective: Ureteroceles are a common co-diagnosis in duplex kidneys. Management of such cases remains a challenge due to the significant comorbidity associated with ureteroceles. To assess the clinical outcome of ureterocele in antenatally diagnosed duplex kidneys to provide guidance on future management. Pediatr Nephrol (2013) 28:1533–1689 DOI 10.1007/s00467-013-2523-7