Usefulness of Doppler ultrasound for the early diagnosis of diabetic nephropathy in type 1 diabetes mellitus.

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.

Utility of Urine N-acetyl-ß-D-glucosaminidase for Prediction of Renal Damage in Obese Children.

The incidence of obesity has increased globally in children and adults. In addition, renal dysfunction is an important complication of childhood obesity. This study was performed to identify the diagnostic value of N-acetyl-ß-D-glucosaminidase (NAG), a renal injury marker, for the early determination of renal damage in childhood obesity. Totally, 115 obese children and 115 healthy normal weight controls were enrolled in a cross-sectional case-control study. Urine NAG was measured in sample urine and normalized by urine creatinine (CR). In addition, correlation of urine NAG with other variables such as blood pressure (BP), blood glucose, and urine albumin was evaluated. Mean systolic BP (P <0.001), serum glucose (P = 0.047), urine albumin/Cr (P = 0.049), and urine NAG/Cr (P = 0.037) were significantly higher in obese children, compared with normal healthy controls. There was no correlation between urine NAG and urine albumin excretion. Urinary NAG/Cr was a simple and safe screening test for early determination of renal damage in children with obesity.

Utility of Urine Interleukines in Children with Vesicoureteral Reflux and Renal Parenchymal Damage.

PURPOSE: Vesicoureteral reflux (VUR) is the most common risk factor of urinary tract infection in children. Currently, diagnosis of VUR depends on invasive imaging studies, with a high radiologic burden. Therefore, different biomarkers have been introduced for the evaluation of these patients. The objective of this study was to identify alteration of urinary interleukins (ILs) excretion in children with primary VUR and renal parenchymal damage, for further clinical application. MATERIALS AND METHODS: Urinary concentrations of IL-1α, IL-1ß, IL-6, and IL-8 were evaluated in 34 children with VUR (cases) and 36 without VUR (control), during 2018-2019. Urinary concentrations of IL-1, IL-1, IL-6 and IL-8 were measured, using polyclonal antibody ELISA kit, and standardized to urine creatinine (Cr). Patients with infectious or inflammatory disorders, urolithiasis, immune deficiency, acute or chronic kidney disease, and secondary VUR were excluded from the study. RESULTS: Mean age of cases (36.00 ± 27.66) had no significant difference with the control (32.86±29.31) group (p=0.44). The majority of patients had moderate VUR (58.8%), followed by severe (35.3%) and mild (5.9%) grades. Urinary concentration of all ILs/Cr were significantly higher in patients with VUR, compared with those without VUR. There was no significant correlation between urine ILs/Cr with age, gender, serum electrolytes, urine specific gravity, renal ultrasound, laterality or severity of VUR, and DMSA renal scan. All urine ILs/Cr had acceptable sensitivity and accuracy for workup of children with primary VUR. CONCLUSION: Urine IL-1α, IL-1ß, IL-6 and IL-8/Cr were sensitive and accurate additionary screening biomarkers in children with primary VUR.

Predictive Value of Serum Interleukins in Children with Idiopathic Nephrotic Syndrome.

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-1ß, 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-1ß, 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-1ß, IL-6, and IL-8 were accurate biomarkers for the prediction of steroid response in these patients.

Validity of urine neutrophile gelatinase-associated lipocalin in children with primary vesicoureteral reflux.

BACKGROUND: 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). MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Alkaptonuria, a new association of distal renal tubular acidosis.

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.

Kidney Function in Patients With Different Variants of Beta-Thalassemia.

INTRODUCTION: Renal involvement is a rare complication of ß-thalassemia. Both tubular and glomerular dysfunction might occur in these patients. The aim of this study was to evaluate and compare kidney function in the major, intermedia, and minor variants of ß-thalassemia. MATERIALS AND METHODS: Renal tubular and glomerular function of 72 patients with ß-thalassemia (25 major, 23 intermedia, and 24 minor) were evaluated. Patients older than 40 years and those with chronic kidney disease, diabetes mellitus, congestive heart failure, associated infections, congenital anomalies of the kidney and urinary tract were excluded. Blood and urine samples were collected electrolytes and markers of kidney function. RESULTS: Mean age at the time of study was significantly higher in the minor group. The majority of patients with thalassemia major were males. Hematuria and pyuria occurred in 4% to 8% of the patients. Serum level of all variables were within normal limits, with no significant difference between the three groups. Glomerular filtration rate was nonsignificantly higher in the major and intermedia groups, compared to the minor variant. A significantly lower urine phosphorus and uric acid excretion was noted with the minor variant. Urine phosphorus and uric acid excretion increased more frequently in the major and intermedia groups. CONCLUSIONS: Tubular and glomerular functions appear to be well preserved in all variants of ß-thalassemia.

Validity of Neutrophil Gelatinase Associated Lipocaline as a Biomarker for Diagnosis of Children with Acute Pyelonephritis.

PURPOSE: Novel biomarkers have been investigated for various renal disorders, including urinary tract infection (UTI). The aim of this study was to assess whether urine neutrophil gelatinase associated lipocaline (NGAL), could represent a reliable biomarker for diagnosis and treatment of children with acute pyelonephritis (APN). MATERIALS AND METHODS: A total of 37 children (32 females, 5 males) with APN were included in this prospective study. Urine NGAL was measured before and 5-7 days after antibiotic treatment in the UTI group, using ELISA kit and compared with 26 (8 females, 18 males) control group children admitted for other bacterial infections. RESULTS: Mean age of the UTI group was 39 ± 28 months, compared to 43.6 ± 31.5 months for the control group with no statistically significant difference. Median urine NGAL level was significantly higher in patients with APN than the other subjects [0.48 (interquartile range (IQR): 0.15-0.72) vs. 0.065 (0.01-0.24), P = .001], and decreased significantly after antibiotic treatment (P = .002). Using a cutoff of 0.20 ng/mL, sensitivity and specificity of urine NGAL were 76% and 77% for prediction of APN, respectively. The area under the ROC curve (AUC) for urine NGAL was 0.75 (CI= 0.61-0.88), suggesting urine NGAL as a relatively good predictive biomarker of APN. CONCLUSION: Urine NGAL is a good biomarker for diagnosis and treatment monitoring of APN in children. .

Effect-Directed Analysis for the Antioxidant Compound in Salvia verticillata.

Salvia genus is one of the largest genera of the Lamiaceae family. Its species have been used for a wide variety of disorders in the local traditional medicine systems. Therefore, the genus has been the subject of several phytochemical and biological studies. The aim of the study was to identify the major antioxidant compound(s) from the methanol extract of Salvia verticillata using activity-guided fractionation. The crude extract showed strong antioxidant activities in DPPH and ß-carotene/linoleic acid tests. The ethyl acetate fraction also exhibited a potent free radical scavenging activity compared to the other fractions. Further fractionation and purification of the ethyl acetate fraction using chromatography methods yielded a compound with high antioxidant capacity. The isolated active compound was determined as chrysoeriol. It showed a dose-dependent free radical scavenging activity with an IC50 (DPPH scavenging) value of 93.32 (80.23 - 108.57) mM.

A Clinicopathologic Study of Children with Idiopathic Focal Segmental Glomerulosclerosis.

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.

Urine Neutrophil Gelatinase Associated Lipocalin to Creatinine Ratio: A Novel Index for Steroid Response in Idiopathic Nephrotic Syndrome.

OBJECTIVE: To find the value of urine neutrophil gelatinase associated lipocalin (NGAL) in differentiating steroid response in children with idiopathic nephrotic syndrome (INS). METHODS: A total of 52 children with INS (n = 27, steroid resistant; n = 25, steroid responsive) aged 1-16 y, along with 18 healthy control children were enrolled in this study. Urine NGAL as well as urine protein, and serum creatinine were analyzed during active phase of INS. RESULTS: Serum creatinine (P 0.032), and urine NGAL/Cr (P 0.001) were significantly higher in steroid resistant than steroid sensitive patients. The optimal cutoff value for urine NGAL/Cr with the highest sensitivity and specificity was 0.46 ng/mg and cut off value of 0.01 and 1.15 ng/mg had maximum sensitivity and specificity, respectively. CONCLUSIONS: Urine NGAL/Cr could be considered as a marker of steroid resistance in children with idiopathic nephrotic syndrome.

Recurrent Macroscopic Hematuria and Abdominal Pain: Questions and Answers.

A 6.5 yr old girl was admitted with a category of clinical signs and symptoms including recurrent gross hematuria, abdominal pain, and fever. After different examinations including genetic analysis, the disease was diagnosed as Familial Mediterranean fever (FMF). It is suggested to consider FMF as a rare cause of recurrent gross hematuria, which is responsive to colchicine treatment.

Early prediction of urinary tract infection in neonates with hyperbilirubinemia.

INTRODUCTION: Hyperbilirubinemia is a common manifestation of infectious disorders during the neonatal period. Urinary tract infection (UTI) is one of the serious bacterial infections with hyperbilirubinemia among newborn infants. OBJECTIVES: The aim of this study was to identify the early predictive risk factors of UTI in neonates with hyperbilirubinemia, to prevent its long-term complications. PATIENTS AND METHODS: A total of 95 neonatal hyperbilirubinemia were evaluated in 2 groups with (n = 40) and without UTI (n = 55). RESULTS: Mean age at diagnosis of UTI was 16.37 ± 8.86 days. Hyperbilirubinemia was detected in 70% of patients during the first week of life. There was a significant difference regarding the age at admission, duration of hyperbilirubinemia, serum bilirubin and creatinine, white blood cells (WBC) , and also Hgb levels between the 2 groups in univariate analysis. However, prolonged jaundice (OR = 10.3, P = 0.001) and serum bilirubin concentration (OR = 5.15, P = 0.001) were statistically associated with a positive urine culture in multivariate analysis. CONCLUSION: Screening of UTI is recommended in neonates with prolonged unexplained jaundice, leukocytosis, and increased serum creatinine.

Clinical Course and Effective Factors of Primary Vesicoureteral Reflux.

Vesicoureteral reflux (VUR) is one of the most important causes of urinary tract infection and renal failure in children. It is a potentially self-limited disease. The aim of this study was to evaluate the clinical course and significant factors in children with primary VUR. The medical charts of 125 infants and children (27.2 % males, 72.8% females) with all grades of primary VUR were retrospectively reviewed. Mean age at diagnosis was 22.3 ± 22.9 months. 52% of patients had bilateral VUR. Mild reflux (Grade I, II) was the most common initial grade. 53.6% of patients achieved spontaneous resolution. 30.1% of patients had decreased renal function on initial DMSA renal scan, significantly in males and severe VUR. Reflux nephropathy occurred in 17.6% of patients, especially in renal damage and male sex. No significant association was observed between recurrent urinary tract infection with the severity of VUR, and the presence of renal damage at admission. Age at diagnosis, gender, grade, laterality, the absence of recurrent urinary tract infection and renal damage had a significant correlation between spontaneous VUR resolution. Spontaneous resolution of primary VUR occurred significantly in female patients, age ≤ 30 months at diagnosis, mild-to-moderate VUR, unilateral reflux, the absence of recurrent urinary tract infection, and renal damage.

Trends in steroid response among children with idiopathic nephrotic syndrome.

INTRODUCTION: Idiopathic nephrotic syndrome (INS) is a common chronic illness in childhood and is initially treated with corticosteroids. Recent reports indicate that the incidence of steroid resistance and focal segmental glomerulosclerosis is on the rise. However, these reports involved different ethnic populations. The purpose of this study was to compare the characteristics of INS in Iranian children in different periods. MATERIALS AND METHODS: A retrospective chart review of children admitted with the diagnosis of new-onset INS was performed. Patients were divided into two groups based on date of presentation periods of 1991 to 2002 and 2005 to 2012. Steroid resistance was defined as persistent proteinuria (2+ and more) within 8 weeks of oral corticosteroid treatment. RESULTS: A total of 238 children included in this study (119 in each group). There was an insignificant decrease in the frequency of steroid resistance, along with an insignificant change in histopathology towards focal segmental glomerulosclerosis. CONCLUSIONS: These findings indicate that in contrast to other reports of INS from various ethnic compositions, a tendency to steroid resistance is still arguable in the population of Iranian children.

Changing trends in characteristics of infantile hydronephrosis.

BACKGROUND: Hydronephrosis (HN) or calycial dilatation is the most common prenatal urologic abnormality. The aim of this study was to identify the possible changes in demographic and clinical manifestations of infantile HN in previously and recently diagnosed patients. METHODS: 193 children with infantile HN admitted to Ali-asghar Children's Hospital in two different periods (group 1; 1997-2003, and group 2; 2005-2011) were evaluated in this cross-sectional study. Variables such as time at diagnosis, gender, clinical manifestations, etiology, laterality, grade, and associated anomalies were also evaluated. RESULTS: The mean age at diagnosis was 32.3± 42.6 (group 1) and 21.4± 36.4 (group 2), respectively. 69.8% of patients were males. 75% of prenatally diagnosed patients were asymptomatic. Urinary tract infection was the most common symptom, followed by pain, hematuria, and decreased renal function. Obstruction in ureteropelvic junction was the most common etiology, accounting for 39.6% of all patients. Neurogenic bladder, vesicoureteral reflux, nephrolithiasis, and ureterovesical junction obstruction were the other common etiologies. 55.5% of all patients had unilateral HN, which was more common on the left side. No significant difference documented between two groups of patients, except for mild HN (23.7% vs. 39%), which was more common in newly diagnosed patients (p= 0.001). CONCLUSION: There was no changing trend in demographic and clinical manifestations of infantile HN. However, the severity of infantile HN has been decreased significantly in recently diagnosed patients.

Epidermal nevus syndrome and dysplatic kidney disease.

Epidermal nevus syndrome is a rare congenital disorder, characterized by epidermal nevi and multiple organ involvement. Multicystic kidney disease has been very rarely reported in this syndrome. Here is the report of a boy presented with multiple epidermal nevi, cardiac anomaly, seizure attack, hemi hypertrophy, and multicystic dysplastic kidney complicated with Wilms' tumor. According to this association, it is suggested to search for dysplastic kidney disease in patients with neurocutaneous disorders.