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Background: Patients diagnosed with congenital kidney malformations are at an increased risk of developing hypertension, proteinuria, and progressing to chronic kidney disease (CKD). The present study aimed to determine the frequency of masked hypertension and ambulatory arterial stiffness index (AASI) in patients with congenital kidney malformations., Methods: The study included 174 patients with congenital kidney malformations (48 patients with unilateral renal agenesis (URA), 40 patients with ectopic kidney (EK), 36 patients with horseshoe kidney (HK), 31 patients with multicystic dysplastic kidney (MCDK), 19 patients with unilateral renal hypoplasia (URH), and 45 healthy controls., Results: The mean age was 12.9 ± 2.9 years, and the male-to-female ratio was 1.5. No significant differences were observed between the congenital kidney malformations groups concerning age, sex, follow-up period, proteinuria, or estimated glomerular filtration rate (eGFR) (P > 0.05). Nevertheless, the prevalence of masked hypertension exhibited a statistically significant increase in the congenital kidney malformations groups (except the URH group) compared to the control group (P < 0.05). The AASI was significantly greater in the congenital kidney malformations groups than in the control group (P < 0.05). The nighttime diastolic blood pressure (DBP), mean arterial pressure (MAP), and DBP index were significantly different between the congenital kidney malformations groups (P < 0.05). However, there were no significant differences in nondipping pattern, proteinuria, or masked hypertension between the congenital kidney malformations groups., Conclusions: Patients with congenital kidney malformations should be periodically evaluated throughout life for BP. Based on the present findings, we strongly recommend ABPM for the diagnosis of masked hypertension and outcomes, including AASI score., Competing Interests: Declarations. Conflict of interest: The authors declare that they have no conflict of interest. Ethical approval: The study protocol was approved by the Ankara Etlik City Hospital Ethics Committee. Ankara Etlik City Hospital ethics commitee approved the study. Consent to participate: Not applicable. Consent for publication: Not applicable., (© 2024. The Author(s), under exclusive licence to Japanese Society of Nephrology.)

Background: Coronavirus disease 2019 (COVID-19) has been recognised as a risk factor for acute kidney injury (AKI). Our aim was to investigate the risk factors contributing to hospitalised and outpatient paediatric COVID-19-associated AKI., Methods: A retrospective observational study was conducted on patients aged 1 month to 18 years with diagnosed COVID-19-associated AKI applied to a tertiary paediatric referral hospital between March 1, 2020 and March 1, 2022., Results: A total of 6683 patients were evaluated and 486 patients were included in the study. Acute kidney injury was observed in 3.7% of outpatients and 23.9% of hospitalised patients. Multivariate logistic regression analysis showed that, on admission, a history of contact with a COVID-19 positive person (p < 0.001), age below 12 months (p = 0.004), presence of comorbidities (p < 0.001), abdominal pain (p = 0.008), anorexia (p = 0.003), dyspnoea (p = 0.005), higher lactate dehydrogenase values (p = 0.004), neutrophilia (p < 0.001), higher neutrophil-to-lymphocyte ratio (NLR) (p = 0.003), higher white blood cell counts (p = 0.006), elevated C-reactive protein (CRP) levels (p = 0.002), anaemia (p = 0.015), hypoalbuminaemia (p < 0.001), hyperglycaemia (p = 0.006), and presence of proteinuria (p = 0.003) were independent predictors of AKI. Higher rates of hospitalisation (p < 0.001) and admission to the paediatric intensive care unit (PICU) (p < 0.001), longer length of hospitalisation (p < 0.001), and greater need for mechanical ventilation (p < 0.001) were associated with AKI., Conclusions: This study reveals that not only hospitalised children, but also paediatric patients are at risk for AKI. The presence of comorbidities, abdominal pain, anorexia, dyspnoea, anaemia, inflammation, hypoalbuminaemia, proteinuria and history of contact with a COVID-19 positive person were the main risk factors for AKI. COVID-19-associated AKI was associated with worse outcomes., Competing Interests: Declarations. Conflict of interest: The authors report no conflicts of interest. Ethical approval: This study was conducted in accordance with the principles of the Declaration of Helsinki. Informed consent: Informed consent was waived due to the retrospective observational nature of the study., (© 2024. The Author(s).)

Objective: The importance of immature granulocytes (IGs) in most infectious and inflammatory diseases has been highlighted. This study aimed to determine the clinical usability and importance of changes in the peripheral complete blood count profile, including IG percentage (IG%) and IG count (IG#), during the relapse and remission phases in pediatric nephrotic syndrome (NS) patients., Methods: This retrospective observational study was performed at a tertiary care hospital between February 2020 and August 2022. Demographic characteristics and laboratory parameters were recorded. The IG count and IG% were measured using an automated hematological analyzer., Results: IG% and IG# were both higher during the relapse phase of NS than during the remission phase (0.29% ± 0.14%, versus 0.23% ± 0.14%, p  = 0.037 and 0.027 ± 0.015 × 10 3 /µL, versus 0.018 ± 0.014 × 10 3 /µL, p  = 0.005, respectively). The neutrophil to lymphocyte ratio (NLR), platelet (PLT), white blood cell (WBC), and neutrophil counts had a strong positive correlation with IG# ( r  = 0.397, p  < 0.001; r  = 0.352, p  < 0.001; r = 0.622, p  < 0.001; r  = 0.660, p  < 0.001, respectively). The NLR, PLT, WBC, and neutrophil counts had a strong positive correlation with IG% ( r  = 0.348, p  < 0.001; r  = 0.187, p  = 0.039; r  = 0.303, p  = 0.001; r = 0.426, p  < 0.001, respectively). Receiver operating characteristic curve analysis showed that IG# had the best AUC value of 0.69 (95% CI: 0.58-0.77; p  = 0.001) for the relapse phase of NS with a cutoff value of 0.025 × 10 3 /µL (sensitivity: 81.0%, specificity: 78.1%)., Conclusions: It is probable that a high level of immature granulocyte count has a positive correlation for NS relapse in pediatric patients. The IG % and IG# can be used together as biomarkers of inflammation in pediatric NS relapse.

Background: Crush syndrome (CS) is a systemic condition resulting from rhabdomyolysis caused by prolonged pressure on muscle tissue. It is estimated that CS will develop in approximately 2-5% of all injuries related to an earthquake, and acute kidney injury (AKI) will develop in approximately 1.5% of all injuries. The present study aimed to present the evaluation of pediatric patients with CS who developed AKI to determine the risk factors that can be determined beforehand for the need for dialysis and to present a new scoring developed for dialysis indication., Methods: Pediatric patients with CS and who underwent dialysis for AKI were included in the study. The study was conducted retrospectively and as single-center data. A renal scoring system that determines the indications for dialysis in patients with CS by parameters of eGFR, creatine phosphokinase (CPK), time under rubble, presence of amputation or fasciotomy, and urine volume was included., Results: The mean age was 11.53 ± 4.50 years, and 58.4% of the group were male. Among the 77 patients, 33 (42.8%) underwent kidney replacement therapy (KRT). Renal score, CPK level, and presence of myoglobinuria were determined to be the best markers for KRT indication in pediatric patients with CS (p < 0.01, p = 0.02, and p < 0.01, respectively)., Conclusions: We suggest that if a similar disaster occurs in the future, an easy and applicable renal scoring system can contribute to the prognosis by providing appropriate and early treatment for KRT. A higher resolution version of the Graphical abstract is available as Supplementary information., (© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Background: Acute post-streptococcal glomerulonephritis (APSGN) is an immune-mediated inflammatory respsonse in the kidneys caused by nephritogenic strains of group A β-hemolytic streptococcus (GAS). The present study aimed to present a large patient cohort of APSGN patients to determine the factors that can be used for predicting the prognosis and progression to rapidly progressive glomerulonephritis (RPGN)., Methods: The study included 153 children with APSGN that were seen between January 2010 and January 2022. Inclusion criteria were age 1-18 years and follow-up of ≥ 1 years. Patients with a diagnosis that could not be clearly proven clinically or via biopsy and with prior clinical or histological evidence of underlying kidney disease or chronic kidney disease (CKD) were excluded from the study., Results: Mean age was 7.36 ± 2.92 years, and 30.7% of the group was female. Among the 153 patients, 19 (12.4%) progressed to RPGN. The complement factor 3 and albumin levels were significantly low in the patients who had RPGN (P = 0.019). Inflammatory parameters, such as C-reactive protein (CRP), platelet-to-lymphocyte ratio, CRP/albumin ratio, and the erythrocyte sedimentation rate level at presentation were significantly higher in the patients with RPGN (P < 0.05). Additionally, there was a significant correlation between nephrotic range proteinuria and the course of RPGN (P = 0.024)., Conclusions: We suggest the possibility that RPGN can be predicted in APSGN with clinical and laboratory findings. A higher resolution version of the Graphical abstract is available as Supplementary information., (© 2023. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Aim Nephrotic syndrome is the most common childhood glomerular disorder, but data on the associated complications are limited and predisposing risk factors have not been fully defined. The aim of this study was to evaluate disease- and treatment-related acute and chronic complications in patients with childhood idiopathic nephrotic syndrome (INS), and to identify the risk factors involved in the development of complications. Methods This single-center study was performed at the pediatric nephrology department of a tertiary pediatric hospital in Turkey. The study included 411 patients with a diagnosis of childhood INS, 128 of whom had disease-related and treatment-related complications. Patients diagnosed and followed-up between January 2010 and January 2022 were evaluated retrospectively. Results Complications occurred in 31.1% of the 411 patients. Mean age at the time of diagnosis was 7.54 ± 4.37 years, and the male/female ratio was 0.9:1. Among the patients with complications, 96.9% were disease-related, and 50.8% were treatment-related complications. In older age, high proteinuria level, a low estimated glomerular filtration rate (eGFR) level at diagnosis, and female gender were significant risk factors for complication development (P = 0.000, P = 0.006, P = 0.04, and P = 0.07, respectively). Chronic kidney disease (CKD) developed in 7% of patients and 2.9% of patients had end-stage renal disease (ESRD). Additionally, three of 12 patients with progressive ESRD underwent transplantation. Also the incidence of ESRD was significantly higher in the patients with complications than in those without complications (P < 0.05). Conclusion The present findings suggest that careful monitoring of patients with childhood INS at risk for complications and implementation of personalized treatment programs can improve long-term outcomes, especially in patients that progress to ESRD and are followed-up with dialysis or transplantation as targeted therapy., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Karakaya et al.)

Background: The most well-known and common long-term complication in children with renal parenchymal scarring (RPS) is hypertension (HT). The present study aimed to evaluate the presence of HT in children with RPS based on ambulatory blood pressure monitoring (ABPM) and to compare the patients' blood pressure (BP) to that in healthy controls matched for age, gender, and BMI., Methods: The study included 55 patients aged < 18 years diagnosed with RPS who were followed up for ≥ 1 year and 48 healthy controls matched for age, gender, and BMI., Results: Mean age in the RPS group was 12.8 ± 3.3 years, and 49.1% of the group were female. Among the RPS patients, 28 were diagnosed with HT based on ABPM, of which 18 (32.7%) had an office BP < 90th percentile for age, gender, and height; seven had an office BP between the 90-95th percentiles for age, gender, and height; and three had an office BP > 95 percentile for age, gender, and height. The difference in detection of HT based on ABPM between the two groups was significant (P = 0.00)., Conclusions: Early diagnosis of HT via ABPM can help prevent development of kidney failure and cardiovascular disease in patients with RPS, significantly reducing the rates of morbidity and mortality. All children with RPS should be evaluated via ABPM, even if office BP measurements are normal. "A higher resolution version of the Graphical abstract is available as Supplementary information"., (© 2022. The Author(s), under exclusive licence to International Pediatric Nephrology Association.)

Introduction: A high vesicoureteral reflux (VUR) grade is among the specific risk factors for febrile urinary tract infection (febrile UTI) and renal scarring. The aim of this study was to examine the predictive value of some potential hematological parameters for high-grade VUR and renal scarring in children 2 to 24 months old with febrile UTI., Methods: We retrospectively examined the clinical features, laboratory tests, and imaging studies of 163 children 2 to 24 months old with a diagnosis of febrile UTI. The hematological parameters based on the neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and white blood cell count (WBC) were calculated using a receiver operating characteristic (ROC) analysis to select which one is suitable., Results: Of the 163 children with febrile UTI, 57 patients (35%) exhibited high-grade VUR. Regarding the predictive power for high-grade VUR, the median area under the curve (AUC) was 0.692 for NLR (sensitivity 61.4%, specificity 69.8%, P < 0.001) and 0.681 for PLR (sensitivity 63.2%, specificity 62.3%, P < 0.001). White blood cell count demonstrated the highest area under the ROC curve for diagnosis of high-grade VUR (0.884, 95% confidence interval 0.834-0.934) and an optimal cutoff value of 13.5 (sensitivity 80.7%, specificity 80.2%, P < 0.001). White blood cell count, with the highest AUC of 0.892 while the sensitivity and specificity were 83.3% and 82.8, was the preferred diagnostic index for renal scarring screening., Conclusions: White blood cell count, NLR, and PLR were useful biomarkers closely related to children with febrile UTI who are at risk for high-grade VUR can also act as a novel marker to accurate prediction of high-grade VUR and renal scarring. Also, NLR and PLR can serve as useful diagnostic biomarkers to distinguish high-grade VUR from low-grade VUR.

Introduction: The objective of this study was to determine the common indications of acute intermittent hemodialysis (IHD) treatment in childhood and to assess the characteristics of the procedure, complications, and prognosis., Methods: The study included 102 patients aged between 0 and 18 years, who had been acute dialysis indication and treated with IHD., Results: In terms of acute kidney injury (AKI) etiology, glomerular diseases were prominent in 56% of the patients, and 8.6% of surviving patients progressed to end-stage renal disease. Regarding the cause of AKI, having the glomerular disease was determined to be directly associated (p = 0.01) with the risk of progression to chronic kidney disease (CKD) regardless of age, gender, onset time of renal replacement therapy., Conclusion: Glomerular diseases were the most common in AKI etiology and were associated with the progression to CKD, independent of age, gender and the time of IHD onset in our study., (© 2022 International Society for Apheresis and Japanese Society for Apheresis.)

Background: Immunoglobulin A (IgA) vasculitis (also known as Henoch-Schonlein purpura) is the most common small vessel vasculitis of childhood. The long-term prognosis depends on renal involvement. The aim of this study was to evaluate the risk factors associated with renal involvement in children with IgA vasculitis and to investigate the best timing of urine examination to predict the presence of renal involvement at the sixth month after the diagnosis., Methods: In this prospective observational study, medical records, demographic data, clinical findings, laboratory tests, and urine microscopic examinations of pediatric patients diagnosed with IgA vasculitis were evaluated to identify potential risk factors associated with renal involvement., Results: A total of 178 patients with a median age of 6 years were involved in the study. Renal involvement was found in 24 (13.5%) patients. Most of the patients (85.7%), whose urine examination was found to be abnormal at the sixth month after the diagnosis, also had abnormalities in the first month urine examination. Factors significantly associated with renal involvement were as follows: older age, presence of hematuria and or proteinuria in the first month urine examination and patients who presented the disease in the spring season. Multivariate logistic regression analysis showed age ≥8 years ( p = 0.005), the season of onset ( p = 0.025), serum creatinine levels ( p = 0.016), and abnormal urine examinations at the first-month visits ( p = 0.005) significantly increased the risk of renal involvement., Conclusion: This study has demonstrated that the optimal date to predict the presence of hematuria and/or proteinuria in the sixth month is the urine examination performed in the first month of the disease. Therefore, we think that patients with IgA vasculitis who show a presence of hematuria and/or proteinuria in the first-month urine examination should be followed more closely.

Membranoproliferative glomerulonephritis and renal microangiopathies may manifest similar clinical presentations and histology. Many genetic mutations that cause these diseases have been reported. Studies on mutations in the gene encoding diacylglycerol kinase epsilon identified a novel pathophysiologic mechanism leading to atypical hemolytic uremic syndrome and/or membranoproliferative glomerulonephritis. Here, we present the different clinical presentations and treatments in 4 family members who carried the same homozygous diacylglycerol kinase epsilon mutation. The first patient (age 5 years, 3 months old at diagnosis) had nephrotic syndrome. The kidney biopsy was membranoproliferative glomerulonephritis; partial remission was achieved with cyclophosphamide, cyclosporine, and mycophenolate mofetil treatment. The second patient (age 5 years, 7 months at diagnosis) presented with overlapping atypical hemolytic uremic syndrome and membranoproliferative glomerulonephritis. Remission could not be achieved with cyclophosphamide, cyclosporine, and mycophenolate mofetil, and hemodialysis treatment was started. At 10 years from first admission, the patient had end-stage kidney disease, and kidney transplant was performed successfully. The third patient was admitted with the diagnosis of nephrotic syndrome at 13 months of age, kidney biopsy showed membranoproliferative glomerulonephritis, and spontaneous remission developed during followup. He presented with hemolytic uremic syndrome 15 months after the first admission, and dialysis was started. Remission was achieved with plasma infusion and eculizumab treatment. The fourth patient (a 7-month-old boy and brother of patient 3) had no clinical or laboratory findings. All patients had genetic analysis, and mutation in exon 2:c.473G>A(p. W158*) was detected. Our related patients with the same mutation showed different clinical and histological findings. However, we did not observe a clear genotype-phenotype correlation in patients with diacylglycerol kinase epsilon nephropathy, suggesting additional factors mediating phenotypic heterogeneity.

Henoch-Schönlein purpura (HSP) is the most common childhood systemic vasculitis. The present study aims to investigate the effectiveness of the immature granulocyte (IG) percentage as a new marker for predicting internal organ involvement in HSP. This study included 75 patients below 18 years old who were diagnosed with HSP. The mean age was 7.48±2.77 years. The male/female ratio was 1.14. The findings showed that 35 (46.7%) of the patients had an internal organ involvement. The mean IG percentage was 0.88±0.68 among the patient group with HSP internal organ involvement, while it was 0.31±0.15 in the group without internal organ involvement, and a significant difference was determined between the 2 groups (P=0.000). The findings showed that the patients with renal involvement had the highest mean IG percentage (IG; 1.00±0.21). When the cutoff value for the IG percentage was specified as 0.45 to predict internal organ involvement, the sensitivity was 77.1%, and the specificity was 85%. In this study, the findings showed that IG percentage increased among patients with internal organ involvement in HSP and that its sensitivity, specificity, and predictive values were higher in predicting internal organ involvement compared with other markers., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Children born with unilateral renal agenesis (URA) are thought to have a risk of developing hypertension, proteinuria, and progressive chronic kidney disease (CKD). The present study aimed to evaluate the long-term prognosis and clinical characteristics of children with URA., Methods: The study included 171 patients aged < 18 years diagnosed as URA who were followed-up for ≥ 1 year and 121 healthy controls matched for age, gender, and BMI., Results: Median age at diagnosis was 2 years (IQR: 1 month-16 years) and the incidence of URA in males (65.4%) was higher than in females. Among the patients, 21 (12.2%) had other urinary system anomalies. It was noted that 2.3% of the patients had proteinuria, 15.2% had hyperfiltration, and 2.9% had CKD. Hypertension based on ambulatory blood pressure monitoring (ABPM) was diagnosed in 18 (10.5%) of the patients, of whom 10 had masked hypertension. Diastolic blood pressure in the URA patients was significantly higher than in the healthy controls. The incidence of hypertension and CKD was significantly higher in the patients with other urinary system anomalies., Conclusions: Patients with a single functional kidney should be periodically evaluated throughout their lifetime for urine protein, blood pressure, and kidney functions. The most remarkable finding of this study is the importance of the use of ABPM for evaluating blood pressure in pediatric URA patients, especially for the detection of masked hypertension and the non-dipper phenomenon, which cannot be achieved with office blood pressure measurement., (© 2021. IPNA.)

Objective : Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent, self-limited attacks of fever with serositis. Acute recurrent arthritis is the most common form of musculoskeletal involvement in FMF; however, ≤5% of FMF patients can develop chronic arthritis, including sacroiliitis. It is difficult to determine if sacroiliitis is a musculoskeletal finding of FMF or if they are concomitant diseases-FMF and juvenile spondyloarthropathy (JSpA). The present study aimed to compare clinical and laboratory findings in FMF patients with concomitant sacroiliitis and JSpA patients with concomitant sacroiliitis. Materials and Methods : The medical files of patients diagnosed with FMF and JSpA with concomitant sacroiliitis were retrospectively evaluated. All patients had MRI findings consistent with sacroiliitis. Patient demographic data, clinical features, and laboratory findings were compared between the patients with FMF and concomitant sacroiliitis, and those with JSpA and concomitant sacroiliitis. Results : The study included 18 patients with FMF and sacroiliitis, and 38 patients with JSpA and sacroiliitis. The median (range) age at diagnosis of FMF accompanied by sacroiliitis and JSpA accompanied by sacroiliitis was 12.0 years (3.5-18 years) and 13 years (4-18 years), respectively. There weren't any significant differences in HLA-B27 positivity, family history of ankylosing spondylitis, presenting complaints, arthritis, enthesitis, or treatment between the 2 patient groups. Conclusion : The present findings show that pediatric patients with FMF and sacroiliitis, and those with JSpA and sacroiliitis have the same clinical and laboratory findings.

Acyclovir may cause acute kidney injury (AKI) due to the accumulation of relatively insoluble acyclovir crystals in renal tubules. The aim of this study was to evaluate risk factors associated with acyclovir-related AKI in children. Between January 2010 and December 2019, pediatric recipients of intravenous (IV) acyclovir were evaluated retrospectively. There were a total of 472 patients [249 (52.7%) boys] of which 32 (6.8%) had AKI [15 (46.8%) boys]. Patients with AKI had greater mean age, baseline creatinine level, and duration of treatment compared to patients without AKI (p<0.001). In the AKI group, concomitant nephrotoxic drug use was more frequent (p=0.032), and the percentage of patients treated with 1500 mg/m 2 /day dosage was higher (p<0.001). AKI was diagnosed at a mean of 4.3 ± 2.5 days after acyclovir initiation and creatinine levels returned to normal at a mean of 7.3 ± 3.6 days after AKI diagnosis. Only eight patients (25%) had vomiting which led to suspicion of AKI. Being older than 100.5 months (HR: 4.501, 95% CI: 1.802-11.241; p=0.001), use of 1500 mg/m 2 /day acyclovir (HR: 9.536, 95% CI: 2.157-42.158; p=0.003) and use of concomitant nephrotoxic drugs (HR: 5.043, 95% CI: 2.289-11.109; p<0.001) were the factors that independently increased the likelihood of nephrotoxicity.Conclusion: Most patients were asymptomatic when they were diagnosed with AKI. Clinicians should be aware of AKI risk in pediatric patients with risk factors (age >100.5 months, 1500 mg/m 2 /day dosage, concomitant use of nephrotoxic drugs). Acyclovir dosing should be evaluated in prospective, multicenter studies in order to identify the lowest possible therapeutic doses that do not increase AKI risk. What is Known: • Although acyclovir is mostly well tolerated, nephrotoxicity may be seen due to the accumulation of acyclovir crystals in renal tubules. • Older age, obesity, and concomitant use of other nephrotoxic drugs are reported to be risk factors for acyclovir-induced AKI in children. What is New: • In this study, pediatric patients with acyclovir-induced AKI were older, received treatment longer, received concomitant nephrotoxic drugs more commonly, and had higher acyclovir dosage and baseline creatinine levels compared to those without AKI. • Being older than 100.5 months of age, use of 1500 mg/m 2 /day dosage and use of nephrotoxic drugs concomitantly appear to be the prominent risk factors for AKI development in children treated with acyclovir., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Acute tubulointerstitial nephritis (ATIN) is a rare cause of acute kidney injury in children that can lead to chronic kidney disease. The aim of this study was to describe the presenting features, etiology, and clinical characteristics of childhood ATIN, and to evaluate treatment modalities and renal outcomes., Methods: The study included 38 patients who had been diagnosed with ATIN, were younger than 18 years old, and were admitted for at least 6 months of follow up., Results: The median age at diagnosis was 13.1 years (range 1.2-16.6 years). The female / male ratio was 1.37. The most common symptoms were abdominal pain (65.7%) and nausea / vomiting (55.2%). Twenty-three of the patients had a history of drug intake (60.5%), and the most active drugs in the etiology were non-steroidal anti-inflammatory drugs (56.5%). Eleven patients were given steroid therapy due to severe kidney involvement and rapid increases in serum creatinine values, and ten of those patients were drug-related ATIN cases. Other patients were given symptomatic treatment. Four patients needed several sessions of dialysis due to the severity of their acute renal failure. The follow-up creatinine and estimated glomerular filtration rate levels were not statistically different between the symptomatic and corticosteroid treated groups (P > 0.05)., Conclusions: Although our study was unable to show the beneficial effect of corticosteroid therapy on the extent of renal recovery, the use of steroids may be preferable in severe nephritis because of the rapid recovery of estimated glomerular filtration rate with corticosteroid therapy., (© 2020 Japan Pediatric Society.)

Congenital nephrotic syndrome (CNS) is a rare disorder characterized by massive proteinuria and marked edema manifesting in utero or during the first 3 months of life. CNS can be caused by congenital infections, allo-immune maternal disease or due to the genetic defects of podocyte proteins most commonly NPHS1. Here we present a case of Finnish-type congenital nephrotic syndrome along with feeding problems and abdominal distention which was diagnosed during follow-up as a gastric-duplication cyst with a novel mutation in the nephrin gene. CNS feeding problems are attributed mainly to primary disease but in literature there are case reports of patients with CNS and hypertrophic pyloric stenosis. NPHS1 is also expressed in the stomach tissue. Physicians should be aware of this rare extra-renal manifestation or coincidence of this rare disease.

Objective: To explore the frequency of MEFV gene mutations in children with Henoch-Schönlein purpura who had no prior familial Mediterranean fever diagnosis and to evaluate the association of MEFV mutations with the clinical and laboratory features of Henoch-Schönlein purpura., Methods: Data of 1120 patients diagnosed with Henoch-Schönlein purpura were reviewed retrospectively. The spectrum and degree of organ involvement and acute phase reactant levels were documented for each patient. Blood for MEFV gene mutation analysis was obtained either at the time of the Henoch-Schönlein purpura diagnosis or during follow-up visits. Pathological specimens of patients who underwent biopsy (renal/skin) were evaluated with special consideration for immunofluorescent examinations., Results: Two hundred and thirty-eight (21.3%) patients were found to have one of the MEFV mutations in which exon 10 mutations were the most common (16.7%). Abdominal pain, joint involvement, scrotal involvement, and relapse were more frequent, and acute-phase reactant levels were significantly high in patients with MEFV mutations. More severe characteristics were observed in the presence of homozygous exon 10 mutations. There was no significant association between exon 2 variants and clinical course of Henoch-Schönlein purpura. Patients carrying MEFV mutations did not have significantly higher levels of IgA deposits in the biopsy materials., Conclusion: Henoch-Schönlein purpura in patients with homozygous exon 10 MEFV mutations seems to be more severe than that in patients carrying other mutations. In patients with exon 10 MEFV mutations, Henoch-Schönlein purpura might be considered as an associated presentation of familial Mediterranean fever rather than a separate clinical entity. Key points • p.M694V mutation is more common in Henoch-Schönlein purpura than in the general population. • p.E148Q variants have no impact on clinical symptoms and laboratory findings in Henoch-Schönlein purpura patients. • The majority of Henoch-Schönlein purpura patients with familial Mediterranean fever have no IgA deposits. • Henoch-Schönlein purpura in familial Mediterranean fever patients may be considered as an integral clinical feature of familial Mediterranean fever.