Clinical Course of 250 Pediatric Cases of Vesicoureteral Reflux in Zahedan, Southeast of Iran

introduction: Although the incidence of primary reflux is 0.1-1.3% in the general population, it is much higher (29-50%) in children with urinary tract infection (UTI). This study aimed to evaluate the epidemiologic characteristics and outcome of vesicoureteral reflux (VUR) in Iranian children in Zahedan, southeast of Iran.Materials and Methods: In this historical cohort study, we reviewed all records of 250 patients diagnosed with VUR who were referred to a Pediatric Nephrology Clinic in Zahedan, Iran between April 2008 and September 2009 and were followed until June 2011. The diagnosis of VUR was made by convectional voiding cystourethgraphy (VCUG). Following the diagnosis, patients received prophylactic low-dose oral antibiotic and were followed by radionuclide cystography (RNC) for 18 months.Results: Of 250 patients with VUR, 153 (61.2%) were female with a mean age of 2.7 ±2.0 years at presentation. One hundred and thirty patients (52%) had unilateral VUR and 120 patients (48%) had bilateral VUR. In 54.4% of the patients, VUR was detected during the investigation for UTI and 13.2% had prenatal hydronephrosis. Voiding dysfunction was the most important condition with VUR (15.2%). Kidney ultrasound confirmed abnormal results in 73.2% of the patients.  Renal scaring secondary to VUR was detected in 85 (34%) patients at the time of VUR diagnosis.Conclusions: Our data showed that VUR was significantly higher in female children and the frequency of abnormal renal scan was significantly higher in children with high–grade VUR. Further studies may be needed to determine the risk factors and apply effective interventions to minimize the progression of renal damage.Keywords: Vesicoureteral reflux; Child; Zahedan

Giant Hydronephrosis Due to Congenital Uretropelvic Junction Obstruction, a Report of 2 cases

Ureteropelvic junction (UPJ) stenosis occurs in 13% of children. Antenatally, the diagnosis is possible by showing renal pelvis dilatation by obstetric ultrasonography. Giant hydronephrosis (GH) is a rare condition. There are different etiologies for GH which are mostly congenital and occur during infancy and childhood. GH was confirmed by complementary evaluations in a 2-month-old female infant with poor feeding, lethargy, and vomiting together with an antenatal sonography in favor of hydronephrosis, and a 5-year-old boy with an abdominal mass diagnosed by sonography following 2 weeks of progressive abdominal distention. Pyeloplasty was associated with an excellent result in both of them.Keywords: Hydronephrosis; Ureteral Obstruction; Congenital; Urologic Diseases

Clinical Signs and Causes of Chronic Kidney Disease in Pediatrics

Background: Chronic kidney disease (CKD) is a worldwide medical condition with increasing frequency that impairs the quality of life of children. This study aimed to assess the clinical signs and causes of CKD in children in Zahedan, Iran. Methods: This cross-sectional study was run on 489 children up to 20 years with stages of 3 to 5 CKDs, in Zahedan during 2010-2019. Informed written consent was obtained from the patients’ parents. Data analysis was performed by SPSS 20 considering 0.05 as significant level. Results: Amongst the clinical manifestations, edema (16.4%), (FTT) (13.5%), urinary symptoms (12.3%) and vomiting (11.5%) were more common and congenital structural anomalies (41.7%) were the most common cause of hospital admission. From among the studied children with CKD, 65.8% had stage 5, about 44.4% had conservative treatment and in total, 19.00% died. Most of the laboratory parameters were different in death and survival cases, for instance, Systolic and diastolic BP were higher in deaths when platelet blood was lower. Main causes of CKD (congenital structural anomalies, cystic/hereditary/congenital disease, glomerular diseases, Renal Tubular Disease, unknown origin and stone) had a significant association with gender (X2=13.42, p=0.02), treatments (X2=70.77, p<0.001), stage of CKD (X2=40.31, p<0.001) and survival (X2=11.59, p=0.041). Stages of CKD had a significant relation with treatment (X2=118.18, p<0.001), and survival (X2=26.5, p<0.001). Conclusion: The causes of CKD were significantly associated with treatments, stage of CKD, and survival. Stages of CKD had significant associations with treatment and survival. Therefore, more attention to children with these signs is essential for early diagnosis and proper treatment

Evaluation of Antibiotic Sensitivity of Urinary Tract Pathogens among Children in Zahedan, South East of Iran

Background Urinary tract infection (UTI), are the most frequent and serious infection in childhood around the world. The present study aimed to evaluate antibiotic sensitivity of urinary tract pathogens among children in Zahedan, South East Iran. Materials and Methods This cross-sectional study analyzed 396 patients aged 1-18 years with positive urine and suspected UTI. Gender and age of children, kind of isolated bacteria in urine culture, susceptibility and resistance of these bacteria to current antibiotics were studied. Bacterial growth for more than 105 CFU/ml was considered as positive. Data analyzed by SPSS version 21.0. Results The most common age of urinary tract infection were < 1 year and 1-7 years for boys and girls, respectively, and the variation of sex distribution was significant in different age groups (P = 0.003). The most prevalent cause of UTI was Escherichia coli(E.coli) (77%), in total ages and both gender, afterward was Enterobacter (8.1%) and Klebsiella (7.1%). E.coli sepsis was highly sensitive to nitrofurantoin (74.7%), ciprofloxacin (72.5%), and amikacin (64.6%), both highly resistant to trimethoprim and sulfametoxazole (74.8%), ampicillin (66.9%), and nalidixic acid(51.1%); and its resistance to ceftriaxone was increasing. Conclusion  In this study resulted that E. coli was the first responsible pathogen in proven culture of UTI in children, which was increased in resistance to popular antibiotics like Ampicillin, Sulfamethoxazole-trimethoprimand Nalidixic acid. In several countries, emphasize the need for local population specific surveillance for guiding empirical therapy for UTI in children

Prevalence of reflux nephropathy in Iranian children with solitary kidney: results of a multi-center study

Abstract Background: Given the importance of the function of the remnant kidney in children with unilateral renal agen‑ esis and the significance of timely diagnosis and treatment of reflux nephropathy to prevent further damage to the remaining kidney, we aimed to determine the prevalence of reflux nephropathy in this subgroup of pediatric patients. Methods: In general, 274 children referred to pediatric nephrologists in different parts of Iran were evaluated, of whom 199 had solitary kidney and were included in this cross‑sectional study. The reasons for referral included urinary tract infection (UTI), abnormal renal ultrasonography, being symptomatic, and incidental screening. Demographic characteristics, including age and gender were recorded. History of UTI and presence of vesicoureteral reflux (VUR) were evaluated. Results: Of the 274 children evaluated in this study with the mean age (SD) of 4.71 (4.24) years, 199 (72.6%) had solitary kidney. Among these, 118 (59.3%) were male and 81 (60.7%) were female, 21.1% had a history of UTI, and VUR was present in 23.1%. The most common cause of referral was abnormal renal ultrasonography (40.2%), followed by incidental screening (21.1%), being symptomatic (14.1%), and UTI (5.5%). In 116 children (58.3%), the right kidneys and in 83 (41.7%) the left kidneys were absent. Besides, 14.6% of the participants had consanguineous parents and 3% had a family history of solitary kidney. Upon DMSA scan, the single kidney was scarred in 13.1%, of which only 7.5% were associated with VUR. In addition, proteinuria and hematuria were observed in 6.5% and 1.5% of children, respectively. Conclusions: The prevalence of reflux nephropathy was 7.5% in children with solitary kidney with a male predomi‑ nance. Given the relatively high prevalence of reflux nephropathy in these children, screening for VUR in the remnant kidney appears to be essential in this population. Keywords: Vesicoureteral reflux, Nephropathy, Children, Solitary kidney, Renal agenesi

Mimicry and well known genetic friends: molecular diagnosis in an Iranian cohort of suspected Bartter syndrome and proposition of an algorithm for clinical differential diagnosis.

BACKGROUND: Bartter Syndrome is a rare, genetically heterogeneous, mainly autosomal recessively inherited condition characterized by hypochloremic hypokalemic metabolic alkalosis. Mutations in several genes encoding for ion channels localizing to the renal tubules including SLC12A1, KCNJ1, BSND, CLCNKA, CLCNKB, MAGED2 and CASR have been identified as underlying molecular cause. No genetically defined cases have been described in the Iranian population to date. Like for other rare genetic disorders, implementation of Next Generation Sequencing (NGS) technologies has greatly facilitated genetic diagnostics and counseling over the last years. In this study, we describe the clinical, biochemical and genetic characteristics of patients from 15 Iranian families with a clinical diagnosis of Bartter Syndrome. RESULTS: Age range of patients included in this study was 3 months to 6 years and all patients showed hypokalemic metabolic alkalosis. 3 patients additionally displayed hypercalciuria, with evidence of nephrocalcinosis in one case. Screening by Whole Exome Sequencing (WES) and long range PCR revealed that 12/17 patients (70%) had a deletion of the entire CLCNKB gene that was previously identified as the most common cause of Bartter Syndrome in other populations. 4/17 individuals (approximately 25% of cases) were found to suffer in fact from pseudo-Bartter syndrome resulting from congenital chloride diarrhea due to a novel homozygous mutation in the SLC26A3 gene, Pendred syndrome due to a known homozygous mutation in SLC26A4, Cystic Fibrosis (CF) due to a novel mutation in CFTR and apparent mineralocorticoid excess syndrome due to a novel homozygous loss of function mutation in HSD11B2 gene. 1 case (5%) remained unsolved. CONCLUSIONS: Our findings demonstrate deletion of CLCNKB is the most common cause of Bartter syndrome in Iranian patients and we show that age of onset of clinical symptoms as well as clinical features amongst those patients are variable. Further, using WES we were able to prove that nearly 1/4 patients in fact suffered from Pseudo-Bartter Syndrome, reversing the initial clinical diagnosis with important impact on the subsequent treatment and clinical follow up pathway. Finally, we propose an algorithm for clinical differential diagnosis of Bartter Syndrome

Evaluation of diagnostic value of procalcitonin in pediatric acute pyelonephritis

Background and Aim: Urinary tract infection (UTI) in children is among the prevalent infections of childhood, which occurs due to growth of bacteria in the urinary tract. The aim of this study was to evaluate the usefulness of procalcitonin (PCT) as a reliable marker for distinguishing urinary tract infection (UTI) with or without renal parenchyma (cystitis). Materials and Methods: Eighty children, who were suspicious of having UTI and had been referred to Ali Ibne Abitaleb hospital (in Zahedan) or pediatric clinics (June 2007- Oct 2009) were included in the study after their urine culture revealed their infection. Besides, their clinical and lab symptoms including erythrocyte sedimentation rate (ESR), C&ndash; reactive protein (CRP), serum WBC, and serum procalcitonin (PCT) were recorded. The patients were divided into two groups based on their lab clinical symptoms and radio-isotope scans, namely acute pyelonephritis and acute cystitis (lower UTI). Serum procalcitonin was measured in these cases in a semi-quantitative manner. Results: Fifty children with mean age of 4.89&plusmn;3.50 years were compared with 30 children with mean age of 5.20&plusmn;3.07 years. ESR, WBC, and PCT were significantly higher in patients with upper UTI (P<0.001 ), but CRP was not significantly different in the two groups. PCT, which was semi-quantitatively measured, when lower than 0.5 had a relationship with sensitivity, specificity, positive predictive value, and negative value of 72%, 83.3%, 87.8%, and 64.1% respectively. When PCT was more than 2, the relationship with the mentioned features was 50%, 96.6%, 96.2%, and 53.7%, respectively. The relationships in these two domains can both be assistant in differentiating pyelonephritis from cystitis. Conclusion: PCT was more sensitive and specific for the diagnosis of upper versus lower UTI compared with CRP, and it can be a better marker than CRP for early prediction of febrile pyelonephritis in children

Renal Tubular and Glomerular Function in Children with Iron Deficiency Anemia

Objective: Iron deficiency anemia (IDA) resulting from lack of sufficient iron for synthesis of hemoglobin is most common hematologic disease of infancy and childhood. There is little information about the renal function in iron deficiency anemia. The aim of the present study was to examine the renal function in children with iron deficiency anemia. Methods: This case-control study was performed in children with iron deficiency anemia (n=20) and healthy age matched subjects (n=20). Blood and urine samples were obtained for hematological and biochemical investigation. Statistical analysis was performed by unpaired sample t-test and Pearson's correlation coefficient. Findings: Fractional excretion of sodium (FENa+) was significantly higher in children with iron deficiency anemia than control subjects (P=0.006). There was no significant difference in the level of urine specific gravity, calcium/creatinine ratio, β2-microglobulin and creatinine clearance between case and control groups (P>0.05). There was no correlation between FENa and Hb (r=0.13, P=0.57), FENa+ and Fe+2 (r=0.079, P=0.72), FENa+ and TIBC (r=-0.083, P=0.71), FENa+ and ferritin (r=0.039, P=0.86) as well as Hb and β2-microglobulin (r=0.02, P=0.37) in IDA patients. Conclusion: The results showed that FENa+ was higher in children with iron deficiency anemia than in normal subjects. The findings revealed evidence of tubular damage in IDA; this needs confirmation by more investigation

Urinary Oxalate, Citrate and Uric Acid in Healthy Primary School Children in Zahedan

Background: Urolithiasis in children is associated with metabolic disorders.&nbsp; The most important metabolic disorders are hypercalciuria, hyperoxaluria, hypocitraturia, cysitinuria and hyperuricosuria. This study aimed to evaluate the level of urinary solutes in healthy primary school children in Zahedan, Iran. Methods: This study was carried out on primary school-aged children. A total of 1800 samples were randomly selected using multistage random sampling method. Morning urine samples of children were taken and sent to the laboratory. Urine creatinine was measured by colorimetric device without removing proteins with BT3000 and photometer analyzer based on Jaffe method. Oxalate, uric acid, and citrate were measured using Pars test kits with photometric method. Weight and height were measured using standard protocols. Body mass index (BMI) was calculated as the ratio of weight in kg to height in square of meter. The ratio of oxalate, citrate, and uric acid to creatinine were then measured based on age, sex, and BMI categories. Results: Of total, 1157 schoolchildren were participated in the study. The mean age of&nbsp; participants was 9 years (Range: 7-13 years) including 433 males (37.4%) and 724 females (62.6%).The mean level of Oxalate, citrate and uric acid to creatinine were estimated to be 0.02 &plusmn; 0.01, 2.98 &plusmn; 2.39 and 3.22 &plusmn;0.59, respectively. The prevalence of hpeyroxaluria, hypocitraturia and hyperuricosuria was 25.3%, 20.3% and 34.6%, respectively. There was significant relationship between age and ethnicity with urinary metabolic disorder. Conclusion: In this study, a high prevalence of urinary metabolic disorders were identified in school-aged children. The screening program of urinary solute for early detection of urinary metabolic disorder and kidney stones is recommended. &nbsp