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Objectives: Postnatal evaluation of vesicoureteral reflux (VUR) remains controversial in patients with antenatal hydronephrosis (HN). The objective of this study was to identify the significance of mild postnatal HN as a marker of VUR and its severity. Materials and Methods: Sonographic findings of 248 children (351 kidneys) with persistent postnatal HN were evaluated for the incidence and severity of primary VUR. Results: The majority of patients had mild (67.8%) HN, followed by moderate (27.6%) and severe (4.6%) HN. VUR was identified in 14.7% of patients with mild HN, 18.5% of patients with moderate HN, and 18.7% of patients with severe HN. About 11.44% of patients with mild HN had low-grade VUR, followed by moderate (45.71 %) and severe grades (42.85%). Conclusion: A large number (89%) of patients with mild HN had moderate to severe VUR. In this cohort, renal sonography was not a reliable method for the prediction of VUR and its severity in patients with mild postnatal HN. However, it was reliable for high grades of VUR. These results are limited due to the study design and the inability to generalize these findings. These results should be replicated across other multiple clinics, thereby recruiting more variety of patients, to validate these study recommendations.

Introduction: Nephrolithiasis has been increasingly recognized in recent years. Urine metabolic abnormality is the main cause of renal stone in children. Therefore, identification and medical treatment of metabolic abnormalities have been suggested as an alternative approach to surgical treatments. Objectives: This study was performed to evaluate the therapeutic effect of urine alkalinization and metabolic management in children with renal stone. Patients and Methods: A total of 300 children (from 408 renal clinics) with nephrolithiasis were enrolled in this study. All of them were treated by supportive managements, including urine alkalinization and specific medical treatment of underlying metabolic abnormality. Improvement was defined as stone resolution, stone passage or decrease of stone dimension. Results: Mean age at diagnosis was 28.7 ± 2.6 months (1-150 months). About 78.8% of patients had metabolic abnormality, of which, hypercalciuria (51.7%) and hypocitraturia (33.4%) were the most common causes, respectively. Resolution of renal stone occurred in 89.7% of patients after one year follow up, more in children less than 5 years (P=0.003), and stones smaller than 5 mm (PConclusion: Pharmacologic treatment is recommended in young children with small nephrolithiasis. Pharmacologic treatment also suggested as a primary intervention in children with uncomplicated large renal stones, and prior to invasive surgical management.

Vesicoureteral reflux (VUR) is the most common congenital urinary tract abnormality in children. The objective of this study was to evaluate the diagnostic value of urine neutrophil gelatinase-associated lipocalin (NGAL) in children with primary vesicoureteral reflux (VUR). A total of 69 patients were evaluated in 2 groups with (32) and without (37) VUR. Patients with secondary VUR, infectious or inflammatory disorders, obstructive uropathies, and acute or chronic kidney disease were excluded. Urine NGAL level was measured by ELISA kit. Mean age of children with VUR was 36.84 ± 28.16, compared to those without VUR 32.32 ± 29.08, with no significant difference (p = 0.51). Mean urine NGAL (p = 0.012) and urine NGAL/Cr (p = 0.003) were higher in patients with VUR. In addition, urine NGAL/Cr increased significantly in patients with decreased parenchymal function, compared to those with normal DMSA scan. Using the cutoff value of 0.888, urine NGAL had 84% sensitivity and 81% specificity for diagnosis of VUR. Based on AUC (0.86), urine NGAL had acceptable diagnostic accuracy in children with VUR. The results of this study support the evidence that urine NGAL/Cr is a sensitive, specific and accurate biomarker for diagnosis of children with primary VUR.

Background: Hypertension (HTN) is a significant public health problem worldwide. Early diagnosis of HTN and its related risk factors has been considered as one of the main requirements of general healthcare in children. Objectives: This study aimed to examine the nomograms of normal systolic and diastolic blood pressure (BP) and the prevalence of asymptomatic HTN and pre-HTN in a population of school-aged children. Methods: Systolic and diastolic BP, height, and weight were measured in 5811 healthy school-aged children (2904 males and 2907 females) during healthcare visits. HTN was defined as systolic or diastolic BP ≥ 95th percentile for age, gender, and height of the screened population on ≥ 3 occasions. Pre-HTN was considered as systolic or diastolic BP between 90 - 95 percentile. Results: HTN and pre-HTN were detected in 8.4% and 7.8% of the children, respectively. The prevalence of HTN was 8.6% among the males and 8.2% among the females. Systolic HTN and pre-HTN were detected in 5.8 and 6.4% of the cases compared to diastolic HTN and pre-HTN observed in 5.9 and 6.1% of the participants, respectively. In addition, HTN was detected in 27.9% of children with obesity. Conclusions: According to the high incidence of asymptomatic HTN and pre-HTN in asymptomatic children, it is recommended to perform routine BP measurement during medical care visits of all healthy school children and to prevent and treat obesity in childhood.

PURPOSE: Early diagnosis and management of diabetic nephropathy (DN) might prevent or delay its progression to end-stage renal disease. The purpose of this study was to investigate whether changes in the duplex Doppler resistivity index (RI) are useful for the early identification of renal involvement in children and adolescents with insulin-dependent diabetes mellitus and associated conditions. MATERIALS AND METHODS: A total of 49 diabetic patients (two groups: 21 with DN and 28 without DN) were included in this study. DN was defined as 30–300 mg/l of albumin excretion in a random urine sample. The RI of the main renal arteries and their intrarenal branches (arcuate, interlobar) were evaluated with duplex Doppler ultrasound and correlated with age, renal length, duration of diabetes, and laboratory examinations. RESULTS: The mean age did not significantly differ between the two groups. The patients with DN had a significantly longer duration of type 1 diabetes (p = 0.02). The majority of patients (90.5%) had mild renal involvement with microalbuminuria and normal renal function. The mean RI was normal in both groups of patients, with no significant difference between the two groups. CONCLUSION: The RI did not increase in the early clinical stage of DN and was not a reliable marker for the screening of DN in patients with type 1 diabetes mellitus.

Pro-inflammatory cytokines have been suggested in the pathogenesis of idiopathic nephrotic syndrome (INS), with conflicting results. This study was performed to identify alteration of different serum interleukins (ILs) in children with INS, and their predictive value in response to steroid treatment. Three groups of children (27; steroid-sensitive INS, 21; steroid-resistant INS, and 19 healthy controls) with normal serum C3, negative serologic tests of hepatitis B virus (HBV), hepatitis C virus (HCV), human immune deficiency virus (HIV), and parasitic infections were included in this study. Serum concentrations of IL-1β, IL-2, IL-6, IL-8, IL-13, and IL-18 were measured, using quantitative colorimetric sandwich ELISA kits. Children with secondary nephrotic syndrome, inflammations, systemic disorders, and chronic kidney disease were excluded. The serum concentration of all ILs; except IL-13 and IL-18; was significantly higher in children with INS, compared with the healthy controls. Serum IL-2 had the highest sensitivity of (95.24%) in patients with INS. All of the serum ILs had acceptable accuracy in children with INS, compared with the control group. The serum concentration of IL-1β, IL-6, and IL-8 was significantly higher in children with steroid-sensitive nephrotic syndrome (SSNS), compared with steroid-resistant nephrotic syndrome (SRNS). All of these ILs had acceptable accuracy for the prediction of steroid response in patients with INS. Our findings suggested the pathogenic role of pro-inflammatory cytokines in children with INS, of which IL-1β, IL-6, and IL-8 were accurate biomarkers for the prediction of steroid response in these patients.

This is the presentation of a 19-month-old female admitted for recurrent lower urinary tract infection. Renal ultrasound and computed tomography showed a round bladder stone with no associated abnormality. Open cystolithotomy was performed, and a large bladder stone, measured 3.2 x 2 x 3.2 cm, and composed of magnesium ammonium phosphate (struvite) was removed. To the best of our research, this is the youngest patient, involved with a large struvite bladder stone.

Introduction: Idiopathic nephrotic syndrome has been considered a cell-mediated immunity disorder with T cell dysfunction. Objectives: The purpose of this study was to investigate immunologic dysfunction in children with steroid-sensitive nephrotic syndrome (SSNS) by evaluating serum interleukin-18 (IL-18). Patients and Methods: A total of 30 children with SSNS (n=20 males, n=10 females), aged 1–11 years were enrolled in this study. All of them were treated with steroids at the time of study. Serum IL-18 was compared during both active and remission phases of nephrotic syndrome. In addition, correlation between serum IL-18 with urine protein and serum biochemical variables was evaluated. Results: Mean serum IL-18 was higher in the active phase, and decreased significantly poststeroid treatment (4.87±1.43 ng/L versus 2.39± 0.94 ng/L; P=0.01). It had no correlation with other variables. Conclusion: According to increased serum IL-18 level, dysregulation of type-1 and type-2 mediated immune reactions is suggested in children with SSNS.

There is little information about the clinicopathological correlations of focal segmental glomerulosclerosis (FSGS) in children. Renal biopsies of 46 patients with primary FSGS were reviewed between 1994 and 2015. Clinical and laboratory findings were evaluated at the time of renal biopsy. Totally, not otherwise specified (NOS) was the most common pathologic variant in 38 patients. There was no correlation between age, gender, serum protein, urine protein, hematuria, and steroid response with five distinct pathologic variants and histopathologic lesions. Hgb level had a negative correlation to the glomerular hyalinosis, mesengial hypercellularity, mesengial deposition, and glomerular volume. Renal dysfunction and blood pressure had a significant positive association with the extent of tubular atrophy, interstitial fibrosis, synechiae in Bowman capsule, arteriolar hyalinosis, mononuclear infiltration, atherosclerosis, glomerular hyalinosis, mesengial hypercellularity, mesengial deposition, and glomerular sclerosis. In conclusion, serum creatinine and blood pressure were the significant predictors of histopathologic lesions in children with primary FSGS.

Alkaptonuria (ALK) is a rare genetic disorder, characterized by binding of ochronotic pigment to the connective tissues in different tissues. This is the first report of a child presented with primary distal renal tubular acidosis associated with ALK. Both disorders were managed by their specific medical treatments, with no further complication.

Summary Background Hematuria, either macroscopic or microscopic, is an incidental finding of multiple nephrologic or urologic disorders. Disturbances of urine inhibitors or promotors have been suggested as the potential causes of isolated idiopathic hematuria in children and its recurrence. Meanwhile, appropriate treatment of these risk factors might improve secondary asymptomatic or macroscopic hematuria. Objectives The aim of this study was to identify contribution of urinary biochemical abnormalities in children with isolated idiopathic hematuria. Methods About 522 children with isolated hematuria were evaluated in a prospective cross-sectional study. Data such as clinical manifestations, family history, laboratory examinations, structural anomalies, and urine biochemistry were obtained. Patients with nephrolithiasis, nephrocalcinosis, tubulointerstitial disorder, genitourinary abnormality, urinary tract infection, and glomerular disorder were excluded from the study. Variables such as calcium, citrate, oxalate, phosphate, uric acid, cystine, and magnesium were measured in 24-h urine collection. In addition, serum levels of electrolytes, urea, creatinine, parathyroid hormone, and bicarbonate were identified. Results Mean age at diagnosis was 5.9 years, and females outnumbered males (2/1). Of those, 88.5% had microscopic hematuria, and 12.6% experienced episodes of gross hematuria. Abdominal pain was the most common clinical manifestations. Urinary tract infection occurred in 30% of cases. Totally, 94% of patients had single or multiple metabolic abnormalities in 24-h urine excretion including hypocitraturia, 60.7%; hypomagnesuria, 58.2%; hyperuricosuria, 35.8%; hypercalciuria, 33.7%; hyperoxaluria, 33.7%; and cystinuria, 0.76%, respectively. About 8% of cases had mixed urine metabolic disturbances. Most patients had mild hematuria (red blood cell 30/hpf), with no statistical correlation to urine metabolic abnormalities. About 80% of patients had a history of nephrolithiasis in their relatives. Discussion Decreased urinary inhibitor concentration followed by increased stimulator concentration were the most common abnormalities in patients with idiopathic hematuria. Accordingly, measurement of urinary biochemical concentration is highly recommended in children with isolated hematuria. In addition, investigating the therapeutic effect of potassium citrate supplements is highly recommended in these patients to prevent future stone formation and treatment of hematuria. Variables No (%) Female (%) FH of NL (%) Gross hematuria (%) Hypocitraturia 60.7 70.9 (p 66.6 (p = 0.9) 80.1 (p = 0.9) 11 (p = 0.9) Hypomagnesuria 58.2 64.9 (p = 0.008) 71.1 (p = 0.005) 81.3 (p = 0.5) 10.5 (p = 0.98) Hyperuricosuria 35.8 39.6 (p = 0.12) 61.2 (p = 0.77) 84 (p = 0.11) 10.2 (p = 0.47) Hypercalciuria 33.7 28.5 (p = 0.2) 65.8 (p = 0.9) 83.6 (p = 0.26) 10.4 (p = 0.66) Hyperoxaluria 24.9 27.1 (p = 0.3) 70.8 (p = 0.2) 78.5 (p = 0.5) 11.5 (p = 0.68) FH, familial history; NL, nephrolithiasis.

Background and objectives Accurate diagnosis of acute pyelonephritis (APN) is clinically challenging in young children. The evaluation methods for APN and its major risk factor, vesicoureteral reflux (VUR), have been revised in recent years. The aim of this study was to evaluate the value of different radiologic investigations in children experiencing the first episode of APN. Materials and methods This study included 425 children of 2–12 months of age (17.5% male, 82.5% female) with APN. APN was defined as a positive urine culture associated with focal or diffuse decreased cortical uptake and preservation of renal contour indicated by DMSA renal scan. Results The mean age at diagnosis was 39.2 ± 1.56 months. The following signs were observed in the patients: fever ≥ 38.5 °C, 66.8%; positive CRP, 85.4%; ESR > 25, 78.6%; and leukocytosis > 13,000/mm 3 , 53.4%. Pyelocalyceal fullness and increased parenchymal echogenicity were the most common renal ultrasonography findings, and ultrasonography had 34% sensitivity and 53% specificity for these two findings. DMSA scan and ultrasound findings of APN were not significantly correlated ( P = .112). VUR was found in 42.2% of patients. DMSA scanning was not reliable for the diagnosis of VUR ( P = .956), with 42% sensitivity and specificity. VUR was identified in 42.7% of patients by ultrasound ( P = .001), and the diagnostic rate was related to the VUR severity. Ultrasound had 42.7% sensitivity and 27% specificity for the diagnosis of VUR. Conclusion Determination of inflammatory markers is recommenced for the evaluation of children with APN. In addition, normal ultrasound is a valuable imaging tool for excluding high grade VUR.

Objectives: Septicemia is an important cause of acute kidney injury (AKI) in neonates admitted to neonatal intensive care units. The aim of this study was to identify the predictive factors for AKI in neonates with culture-proven septicemia. Methods: Medical records of 138 newborns with septicemia were evaluated in 2 groups with (n, 65) and without (n, 73) AKI. Results: The mean age at diagnosis of AKI was 11.31 ± 9.92 days. AKI was more common in neonates with lower gestational age, birth weight, age, blood pressure, platelet count, and serum pH at admission. Moreover, AKI was associated with an increased incidence of intubation at birth, inotrope treatment, shock, disseminated intravascular coagulation (DIC), mechanical ventilation, and morality. Gestational age, initial serum pH, and DIC were the only independent predictors of AKI in the multivariate analysis. In addition, serum pH at admission was the most accurate laboratory marker for the prediction of AKI with 83% sensitivity and 60% specificity. Conclusions: Monitoring of renal function is highly recommended in low-birth-weight infants with severe septicemia.

Introduction: Different biomarkers have been investigated for prognosis of patients with nephrotic syndrome. Qualitative and quantitative changes have been reported in platelets in these patients. Objectives: The aim of this study was to identify platelet abnormalities and their prognostic value of steroid response in children with idiopathic nephrotic syndrome. Patients and Methods: Platelet counts and indices (mean platelet volume [MPV], platelet distribution width [PDW] and platelet larger cell ratio [PLCR]) were evaluated and compared in 122 children with active nephrotic syndrome (64%; steroid resistant and 36%; steroid sensitive). Results: Mean age at diagnosis was 55 months (6 to169 months), and males outnumbered females (1.7/1). Steroid resistant patients had significantly higher platelet counts and lower PLCR, compared to steroid sensitive group. Regarding area under the ROC curve, low MPV, high PDW and low PLCR showed relatively acceptable correlation with steroid resistance. Conclusion: Increased platelet counts in addition to low PLCR are the suggestive indicators of steroid resistance in children with idiopathic nephrotic syndrome.

Background: The accurate diagnosis and management of febrile urinary tract infection (UTI) is a clinical challenge in the absence of specific clinical and laboratory findings in infants and young children. Objectives: The aim of this study was to identify and compare the diagnostic and therapeutic implications of recently introduced cytokines for the diagnosis of acute pyelonephritis (APN). Methods: This multicenter prospective study was performed on 37 (female/male = 6.5:1) children with symptomatic culture-proven APN and 37 (female/male = 1.6/1) age-matched febrile children without UTIs as the control group. Urine samples were obtained before antibiotic treatment in both groups and 3 - 4 days after treatment in the UTI group, and evaluated for interleukin (IL)-1α, IL-1β, IL-2, IL-4, IL-6, IL-8, IL-10, tumor necrosis factor-α (TNFα), monocyte chemoattractant protein-1 (MCP-1), and vascular endothelial growth factor (VEGF) using an ELISA immunoassay kit. Results: Mean urinary IL-1α, IL-4, IL-6, and IL-8 concentrations significantly increased in the acute phase of APN compared to the control group, and decreased following antibiotic treatment. Conclusions: We recommend routine urinalysis and urine culture for the diagnosis of children with APN. Urinary IL-4 was a relatively good cytokine for the prediction and treatment-monitoring of children with acute febrile UTI.

Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by proximal muscle weakness, skin lesions, gastro intestinal, pulmonary, cardiac and small nerve damage. Renal involvement has been rarely reported in JDM. This is the report of a 7-year-old boy presented with nephrotic syndrome (NS) and subsequent renal failure. Clinical manifestations of JDM appeared gradually. Renal manifestations could be considered as a rare initial presentation of JDM.

Cyclosporine induced pain syndrome (CIPS) is a newly diagnosed complication of calcineurine inhibitors, mainly observed in solid organ and hematopoetic transplantations. The present case is a male child with steroid resistant nephrotic syndrome on low therapeutic level cyclosporine treatment. He presented with intractable and debilitating leg pain, with no reported history of previous injury or trauma. The pain was reluctant to antimicrobial and sedative treatment. MRI revealed bone marrow and soft tissue edema in the mid shaft of patient's right leg. Inspite of unusual manifestations, CIPS was suggested and cyclosporine discontinued. However, the pain did not improve and was resistant to calcium blocker. Subsequently, core decompression was performed as an unusual treatment of CIPS, revealing normal bone morphology. The pain improved rapidly and the patient was discharged a few days later.

The aim of this study was to determine the value of initial serum creatinine in prediction of End-stage renal disease (ESRD) in children with posterior urethral valves PUV. In this retrospective study, patients diagnosed with PUV admitted in Aliasghar Children’s Hospital in Tehran, Iran from 1991 through 2001 were studied. Based on the development of ESRD (need for dialysis) they were classified in two groups and their demographics, initial presentations, laboratory data, imaging findings and outcomes were reviewed and compared. Thirty six male patients with PUV, treated with valve ablation (13), vesicostomy (13), or high ureterostomy (10) were followed for developing ESRD. Sixteen of the 36 patients developed ESRD at their last follow-up. There was no significant difference in age at initial presentation, presence of urinary tract infections, incidence of vesicoureteral reflux, renal dysplasia or type of primary surgical intervention between the patients with or without ESRD. Patients who progressed to ESRD had serum creatinine concentration > 1.0 mg/dl at diagnosis as compared to those without ESRD (P = 0.001). Initial serum creatinine is a valuable factor for prediction of renal outcome in patients with PUV.

Hydatid disease of the kidney is rare, particularly in the isolated form, and tubulointerstitial nephritis is an extremely uncommon manifestation of renal hydatidosis. Here is the report of a patient with isolated renal hydatid disease who presented with acute renal failure secondary to tubulointerstitial nephritis. The patient responded to the combination of albendazole, corticosteroids and total nephrectomy.