Evaluation of immature granulocyte percentage and count in pediatric nephrotic syndrome.

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 × 103/µL, versus 0.018 ± 0.014 × 103/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 × 103/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.

Complications Related to Childhood Idiopathic Nephrotic Syndrome, Its Treatment and the Associated Risks in Patients.

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.

Predictors of rapidly progressive glomerulonephritis in acute poststreptococcal glomerulonephritis.

BACKGROUND: Acute post-streptococcal glomerulonephritis (APSGN) is an immune-mediated inflammatory respsonse in the kidneys caused by nephritogenic strains of group A ß-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.

The relevance of practical laboratory markers in predicting high-grade vesicoureteral reflux and renal scarring.

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.

Acute intermittent hemodialysis management in childhood: A single center experience.

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.

The importance of ambulatory blood pressure monitoring for diagnosing masked hypertension in patients with renal parenchymal scarring.

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".

Determining the effectiveness of the immature granulocyte percentage and systemic immune-inflammation index in predicting acute pyelonephritis.

AIM: The most serious form of urinary tract infection (UTI) is acute pyelonephritis (APN), which can result in bacteremia and renal scarring. This study aims to show the roles that the systemic immune-inflammation index (SII) and the immature granulocyte (IG) percentage play in predicting APN in pediatric patients by comparing them with traditional infection markers. By illustrating a significant relationship between APN, the IG percentage, and the SII, the study's contributions to the differential diagnosis of UTI can promote a rapid and appropriate treatment of APN. MATERIAL AND METHODS: The present study included 522 pediatric patients. The patients were divided into two groups: (I) an APN group (n = 236) and (II) a lower UTI group (n = 286). RESULTS: The mean age of the sample was 5.05 ± 4.7 years. According to the most appropriate cutoff values, the findings showed that among all the parameters, C-reactive protein (CRP) and the IG percentage had the highest sensitivities, specificities, and predictive values for predicting APN. CONCLUSION: This study emphasizes the support features of the IG percentage and the SII in the diagnosis of APN in pediatric patients, a topic that has recently attracted attention. The findings indicated that among all of the parameters behind CRP, the IG percentage and the SII had the highest sensitivities, specificities, and predictive values for forecasting APN. It is worth noting that these methods can be applied without additional costs or burdens to the patient.

Clinically Different Presentations of Family Members With the Same Homozygote Diacylglycerol Kinase Epsilon Mutation: Case Report.

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.

Clinical spectrum of immunoglobulin A vasculitis in children and determining the best timing of urine examination to predict renal involvement.

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.

An Assessment on the Effectiveness of the Immature Granulocyte Percentage in Predicting Internal Organ Involvement Among Children With Henoch-Schönlein Purpura.

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.

Comparison of pediatric patients with noninfectious idiopathic uveitis and noninfectious uveitis associated with an underlying systemic disease: from a referral center in Turkey.

Aim: The aim of this study was to determine the demographic, clinical, treatment, and outcome features of pediatric noninfectious uveitis patients at a Turkish tertiary center.Materials and methods: This retrospective cohort study included 101 pediatric patients with noninfectious uveitis. Location of uveitis, laterality, age at onset of uveitis, complications of uveitis, duration of follow-up, associated systemic diseases, laboratory findings, medications used, and status of uveitis at the time of data collection were obtained from the patients' files. The patients were divided into two groups according to etiology of uveitis, as follows; group 1: idiopathic noninfectious uveitis; group 2: noninfectious uveitis in association with an underlying systemic disease. Clinical findings were compared between the two groups.Results: Mean age at diagnosis of uveitis was 9.36 ± 4.24 years and mean duration of follow-up was 48.4 ± 9.2 months. Anterior uveitis was the most common form, accounting for 69.3% (n = 70) of the patients. There were 67 (66.3%) patients in group 1 and 34 (33.7%) patients in group 2. Mean age at diagnosis of uveitis was lower in group 2 (p < 0.05). Although gender, type of inflammation, laterality of eye involvement, and mean age at initiation of biological treatment did not differ between the groups; symptomatic uveitis was more common in group 1, whereas anterior uveitis was also more common in group 2 (p < 0.05). At the time of data collection 16 (15.8%) of the patients had active uveitis. Ocular complications were observed fewer in idiopathic noninfectious uveitis group than noninfectious uveitis associated with an underlying systemic disease (p < 0.05). Biological therapy was administered in 26.8% of the patients in group 1, versus 67.6% of those in group 2. Ocular complications were observed in 22.8% of the patients at presentation or during the follow-up.Conclusion: Herein a large retrospective cohort of noninfectious pediatric uveitis has been described. The findings indicate that noninfectious idiopathic uveitis is characterized by more symptomatic, later onset, and fewer complications than noninfectious uveitis associated with an underlying systemic disease.

Retrospective evaluation of children with unilateral renal agenesis.

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.

Similarities between pediatric FMF patients with sacroiliitis and pediatric juvenile spondyloarthropathy patients with sacroiliitis: a preliminary study.

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.

C3 glomerulopathy: experience of a pediatric nephrology center.

Background: C3 glomerulopathy (C3G) is an uncommon disease characterized by the deposition of complement factors in the glomeruli due to overactivation and dysregulation of the alternative pathway of complement.Objectives: This study aimed to describe the clinicopathological features, laboratory testing, clinical course, treatment, and outcomes of pediatric patients with C3G.Patients and Methods: We reviewed retrospectively the laboratory testing, kidney biopsy reports, and clinical features of 18 patients at our hospital from 2007 to 2019.Results: There were 18 cases, and the majority of the patients were girls (61.1%). The mean age at diagnosis was 11.3 ± 3.7 (5-17) years, and nephritic-nephrotic syndrome presentation in patients was more common (11 cases, 61.1%). Hematuria was found in 66.7% of the patients, of which the majority had microscopic hematuria (58.3%). Hypertension was observed in 10 (55.6%) patients. The mean glomerular filtration rate (eGFR) was 95.7 ± 47.3 mL/min/1.73 m2, and 24-h urinary protein excretion was 76.2 ± 48.6 mg/m2/h. Sixteen patients (88.9%) received renin-angiotensin-aldosterone system blockers (RASB), and two of them were taking RASB only. The majority of patients (83.3%) were treated with immunosuppressive therapy. Eculizumab was also given to one of them. At the last follow-up, two patients had levels of less than 60 mL/min/1.73 m2 for eGFR. Seven patients with immunosuppressive treatment achieved complete remission.Conclusion: C3G shows a variable clinical presentation and response to immunosuppressive therapy. In the present study, we observed that the most common presentation was nephritic and/or nephrotic syndrome and partially responded to treatment to RASB and immunosuppressants.

Gastric duplication cyst in an infant with Finnish-type congenital nephrotic syndrome: concurrence or coincidence?

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.

Clinical features in 305 patients with juvenile idiopathic arthritis: A single center Turkish Study.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatologic disease of childhood. The various subtypes of JIA differ in clinical features and treatments. The aim of this study was to analyze the frequency of JIA subtypes, patient demographic and clinical features, as well as the rates of macrophage activation syndrome, uveitis, and remission in Turkish JIA patients treated at a single center, and to compare the findings to those in the literature. METHODS: The files of all JIA patients treated at our pediatric rheumatology department between January 2017 and January 2019 were retrospectively reviewed. Patient demographic, clinical, and laboratory data were obtained from the patients' files and the hospital database. RESULTS: The study included 305 patients (180 females) with a mean age at onset of 7.83 ± 4.62 years. Among all the JIA subtypes, the most frequent was oligoarthritis (41.6%), followed by enthesitis-related arthritis (29.2%), rheumatoid factor (RF)-negative polyarthritis (13.4%), systemic arthritis (9.5%), RF-positive polyarthritis (2.6%), psoriatic arthritis (2.0%), and undifferentiated arthritis (1.6%). At the time of data collection, 278 patients (91.0%) were in remission, whereas 27 patients (9.0%) had active disease. Macrophage activation syndrome developed in 12 of the 29 (41.0%) systemic arthritis. Uveitis was noted in 32 (10.0%) patients. Biological agents were administered in 142 of the patients. CONCLUSIONS: The available data indicate that JIA as a whole is a heterogeneous disease with significant variability in course and long-term outcome. As such, each patient should be evaluated according to his / her disease subtype.

Clinical characteristics of childhood acute tubulointerstitial nephritis.

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.

Two pediatric cases of isotretinoin-induced sacroiliitis successfully treated with adalimumab.

Isotretinoin is widely used in severe acne. Isotretinoin has many side effects. Sacroiliitis is one of these side effects and has been rarely reported in the children. Herein, we present two children with isotretinoin-induced sacroiliitis resistant to anti-rheumatic drugs and successfully treated with adalimumab.

A case of Type 1 Dent disease presenting with isolated persistent proteinuria.

Dent disease is a rare X-linked recessive tubular disorder, characterized by the triad of low molecular-weight proteinuria, hypercalciuria, nephrocalcinosis and/or nephrolithiasis. It is caused by mutations in the CLCN5 gene or OCRL gene. Thirty to 80% of affected males develop end-stage kidney disease between the ages of 30 and 50 years. Some children were reported to present with isolated persistent proteinuria and a part of these patients were diagnosed as having focal segmental glomerulosclerosis with kidney biopsy. Although there is no specific treatment, treatment of proteinuria and hypercalciuria is thought to delay the progression of the disease. For this reason, awareness of the disease findings and early diagnosis are important. In this case report, we present a boy followed-up with isolated persistent proteinuria and then diagnosed as having Dent disease with mutation analysis that showed c.328_330delT (p.Phe110Trpfs27*) in the CLCN5 gene. The importance of researching low-molecular- weight proteinuria and considering Dent disease in the differential diagnosis of children presenting with isolated persistent proteinuria has been emphasized.