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Holoprosencephaly (HPE) is a complex brain malformation resulting from incomplete cleavage of the prosencephalon, occurring between the 18th and 28th day of gestation. Endocrinologic dysfunctions such as diabetes insipidus (DI), hypothyroidism, and growth hormone deficiency are common in HPE and correlated with the degree of hypothalamic non-separation.

Introduction. Nephrotic syndrome (NS) is a common chronic kidney disorder during childhood. The most important characteristic of this disease is proteinuria. The Persian medicine (PM) has important dietary recommendations for strengthening the kidney function and treatment of this disease. The aim of this study was to investigate the effect of a diet including PM recommendations and general principles of Western medicine. Materials and Methods. Twenty children with nephrotic syndrome were randomly divided into intervention and control groups and monitored for one month. The control group received a diet based on the general principles of Western medicine. In the intervention group, in addition to the Western medicine diet, dietary recommendations of PM were also prescribed including the pomegranate (Cydonia oblonga mill.), quince (Cydonia oblonga mill.), and whole grains (wheat and barley). A 24-hour dietary questionnaire was applied and anthropometric and biochemical indices including spot urine protein (proteinuria), albumin (Alb), urea, creatinine (Cr), total cholesterol (TC), and triglyceride (TG) were measured before and after the study. Results. The amount of protein intake reduced significantly in the diet of both groups but the differences between the two groups were not significant. Proteinuria reduced significantly in both the Western and PM groups; however, proteinuria was significantly lower in the Persian medicine group compared to the control group. TC and Cr levels reduced significantly in the intervention group, although the changes were not significant compared to the control group. Conclusion. The results of this study showed that adding dietary recommendations of the Persian medicine to the general rules of the Western medicine diet reduced proteinuria and improved the combat against nephrotic syndrome.

Multiple inflammatory syndrome in children (MIS-C) is a multisystem inflammatory disease following COVID-19 in children. This disease occurs a few weeks after COVID-19. A child with this condition develops a cytokine release cascade that results in organ damage. The involved organs include the heart, lungs, brain, gastrointestinal tract, and central nervous system. Fever is present in all patients and Kawasaki-like symptoms are one of the common features in these children. In this article, we introduce a child with MIS-C who has skin, gastrointestinal, neurological, and renal symptoms and has been hospitalized in the PICU.

Background:Congenital hydronephrosis is one of the most common abnormalities of the upper urinary tract, which can be exacerbated by a variety of intrinsic or extrinsic triggers. The urinary tract system is one of the major organs complicated by COVID-19 infection. Case presentations:Here we report five patients with an established diagnosis of congenital hydronephrosis, who presented with acute abdominal pain and fever and an abrupt increase in the anteroposterior pelvic diameter (APD). Patients had a previous stable course and were under regular follow-up with serial ultra-sonographic studies. They underwent surgery or supportive treatment due to the later exacerbation of hydronephrosis. Based on the clinical and imaging findings, no plausible etiologies for these exacerbation episodes, including infection, nephrolithiasis or abdominal masses, could be postulated. The common aspect in all these patients was the evidence of a COVID-19 infection. Conclusions:Infection with COVID-19 in children with antenatal hydronephrosis may exacerbate the degree of hydronephrosis and renal APD in ultrasonography, which itself may be mediated by the increase in inflammatory mediators.

Background:Renal disorders are reported as both underlying conditions and complications in critical pediatric COVID-19 infection. Herein, we investigated the pattern of renal involvement, particularly acute kidney injury (AKI), among pediatric patients with COVID-19.MethodsWe prospectively enrolled hospitalized pediatric patients with the clinical diagnosis of COVID-19 infection. Demographic, clinical, and laboratory findings were gathered and analyzed, using a mixed method of qualitative and quantitative approaches and descriptive statistics.Results187 patients including 120 (64.2%) male and 67 (35.8%) female with COVID-19 infection at a median (interquartile range) of 60 (24-114) months were enrolled in this study.Most patients (n=108, 58.1%) were suffered from one or two underlying comorbidities, mainly malnutrition (77.4%), neurologic/learning disorders (21.4%) and malignancy (10.2%). Kidney was the third organ involved in 30 patients (16%) during hospital admission, after lung (53.5%) and gastrointestinal tract (39%). According to the KDIGO definition and classification, AKI was detected in 38.5% of patients (stage 1: 55.6%, stage 2: 36.1%, stage 3: 8.3%) at presentation or during follow up. Nine patients (4.8%) required hemodialysis and 16 (8.6%) patients were eventually died. There was no significant association between AKI and pediatric intensive care unit (PICU) admission (p=0.079), multisystem inflammatory syndrome in children (MIS-C) (p=0.051), comorbidities (p=0.067), and mortality rate (p=0.789).ConclusionsKidneys are one of the major organs affected by COVID-19 infection. Although kidney abnormalities resolve in the majority of pediatric COVID-19 infection, particular attention should be given to kidney involvement in COVID-19 patients, particularly children with history of malnutrition and renal disorders.

Hydronephrosis, a condition that is mostly congenital, is considered as the most common type of pediatric urinary tract disorder. The aim of this study was the evaluation of the prognosis and outcomes of hydronephrosis in cases of congenital hydronephrosis.In a cross-sectional study, run in a tertiary clinic of pediatric nephrology, from 2015 to 2020, patients with fetal hydronephrosis were selected. Ultrasonography, urinalysis and kidney function tests were ordered for all patients and in the presence of hydronephrosis, repeated ultrasonography, voiding cystourethrography and dimercaptosuccinic acid scan were performed. In cases with evidence of obstruction, a diethylenetriamine pentaacetic acid scan and relative surgical procedures were performed.Among 141 cases, mean age was 8 ± 1.4 years and 80.9% were male. Partial or complete obstruction in the right and left kidney was found in 16.3 and 24.8% of patients, respectively. The degree of hydronephrosis was mild in 46.1%, moderate in 39%, and severe in 9.2% of the patients. At the last follow-up period, hydronephrosis recovered in 46% of the patients, while 54% experienced persistence or exacerbation of the disease. Meanwhile, 7.1% of patients showed neurogenic bladder, 19.1% urinary tract infection and 22.7% urinary stones.Our study revealed that fetal hydronephrosis ends in complete recovery following birth in 46% of the cases. However, in cases experiencing persistent or exacerbating hydronephrosis, optimized treatment and/or surgical intervention are required. DOI: 10.52547/ijkd.6516.

Background Given the importance of the function of the remnant kidney in children with unilateral renal agenesis 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 predominance. 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.

Background: Information about renal involvement in pediatric patients with COVID-19 is limited, and there is not enough data about renal and urinary tract involvement in children infected with this novel virus. Objectives: This study aimed to determine the spectrum of kidney diseases in pediatric patients with COVID-19, admitted to a tertiary children’s hospital. Methods: This cross-sectional study was conducted on 71 pediatric patients with COVID-19 infection. Diagnosis of COVID-19 was established based on the guidelines by the Iranian Ministry of Health. The patients’ demographic characteristics, clinical symptoms, laboratory results, and renal ultrasonography findings were extracted from the hospital medical records. Results: On admission, 10% of patients had oliguria, 7.7% had edema, and 3% had hypertension. The first urinalysis indicated proteinuria, leukocyturia, and hematuria in 46%, 24%, and 23% of the patients, respectively. Overall, 40.7% of the patients showed some degree of renal involvement. During hospitalization, acute kidney injury (AKI) occurred in 34.5% of the patients. Based on the pediatric risk, injury, failure, loss of kidney function, and end-stage kidney disease (pRIFLE) classification, stage I (risk group) was found in 20% of patients, stage II (injury group) in 25% of patients, and stage III (failure group) in 55% of patients with AKI. The total mortality rate was estimated at 12.67%, and the incidence of in-hospital death was 30% in pediatric patients with severe COVID-19 infection associated with AKI. Conclusions: The prevalence of AKI was high in patients with COVID-19 infection hospitalized in our tertiary hospital. We also found that a decrease in renal function was associated with a higher risk of mortality. Overall, early detection of AKI and effective treatment may help reduce mortality in patients with COVID-19.

Introduction Hypercalcemia, defined as the total serum calcium concentration two standard deviations above the mean normal level, may be an emergency condition requiring proper initial diagnosis and immediate management. In association with nephrocalcinosis and hypercalciuria, differential diagnosis will be narrowed to limited diseases such as idiopathic hypercalciuria, hypervitaminosis A and D, and also renal tubular acidosis (RTA). Hypercalcemia is rarely presented in RTA with a few cases reported to date in the literature. Case presentation We reported a seven-month-old boy with complaints of refractory vomiting, restlessness, polyuria, polydipsia, and hypotonia. Laboratory tests revealed persistent hypercalcemia, hypokalemia, hypercalciuria, and metabolic acidosis with a normal anion gap. Bilateral nephrocalcinosis was detected in imaging studies. Based on these findings he was diagnosed with distal RTA and hypercalcemia and treated with polycitrate and Jaulie's solution. Conclusion Distal RTA should be considered as a differential diagnosis of hypercalcemia. Still, more studies are required for determining the pathway resulting in this rare association.

Food protein-induced enterocolitis syndrome (FPIES), is a non-IgE mediated food allergy presenting in infants younger than 12 months. Diagnostic delay may occur due to overlapping clinical symptoms with several conditions. Here, we present two cases of FPIES, mistakenly diagnosed and treated as Bartter syndrome. This study aims to emphasize the several features of this syndrome that may mimic other diagnoses and sometimes leading to near-death events due to delay in the diagnosis and improper treatment. The first patient was a 30-month-old boy with multiple episodes of profuse vomiting and diarrhea within 1 hour after breastfeeding, beginning from the first month of life progressing to hypokalemia and metabolic alkalosis at the age of 5 months leading to the diagnosis of Bartter syndrome. The second patient had a history of unremitting diarrhea which had been started soon after his first breastfeeding followed by biliary vomiting on the 7th day of life. He was treated in another hospital for neonatal sepsis, however, without an appropriate response to treatment. To conclude, despite the current belief on the rarity of FPIES, it is a more prevalent disease than expected with various non-specific manifestations imitating other conditions which may result in diagnostic delay and sometimes fatalities. To shed light on the importance of the physicians’ awareness of this syndrome, these two cases are presented here as examples of FPIES imitating other disorders.

Introduction: Urinary tract infections (UTIs) are among the most important diseases of children. UTI timely diagnosis and treatment is essential to prevent renal failure. Objectives: This study aims to investigate the association of sacral dimples with recurrent UTIs and urodynamic findings in a group of pediatric patients. Patients and Methods: This case-control study was conducted on children aged one month to fifteen years old with recurrent UTIs. The association of sacral dimples with recurrent UTIs and urodynamic findings were studied. Results: In total, 229 patients with recurrent UTIs were recruited. One hundred thirty-one patients were in the case group (with sacral dimple), and 98 patients were in the control group (without sacral dimple). One-hundred and nine children (82.2%) in the case group and 56 children (57.1%) in the control group had abnormal voiding cystourethrography (VCUG) (PP=0.568). Twenty-two patients (16.8%) in the case group and 13 patients (13.3%) in the control group had renal scarring (P=0.578). Urodynamic findings were abnormal in 117 children (89.3%) in the case group and 70 children (71.4%) in the control group (P=0.001, OR=3.34, 95% CI: 1.65-6.78). Conclusion: In this study, abnormal uroflowmetry and bladder compliance were significantly more common in children with recurrent UTIs with sacral dimple in comparison to recurrent UTIs in patients without sacral dimple. It is recommended that in cases of sacral dimples, patients should undergo a urodynamic study to detect bladder dysfunction.

Background: Imaging modalities such as voiding cystourethrography (VCUG), direct radionuclide cystography (DRNC), and renal cortical scintigraphy (RCS) with technetium-99m dimercaptosuccinic acid (Tc-99mDMSA), are helpful in detecting possible abnormalities of urinary tract such as vesicoureteral reflux (VUR). However, there are confounding data regarding the preference of these modalities. In the present study, we aimed to assess the role of Tc-99mDMSA scan in prediction of possible VUR and the subsequent need for VCUG or DRNC. Materials and Methods: Three hundred fifty seven children under 14 years old with first-time febrile urinary tract infection (UTI) who were admitted between April 2004 and February 2017 were enrolled. Data regarding to VCUG or DRNC in order to evaluate the presence of possible VUR were recorded. Finally, accuracy of Tc-99mDMSA scans in prediction of VUR were assessed. Results: Analyses showed a sensitivity and specificity of 95.65% and 14.72%, respectively for Tc-99mDMSA scan in detection of VUR. Patients with mild renal involvement in Tc-99mDMSA scans had a 3.5-fold greater risk of having VUR than those with a normal scans (odds ratio=3.5; 95% confidence interval [CI]: 1.4-8.58; p=0.007). In addition, the risk of VUR is up to 7.0-fold greater in children with moderate renal involvement (odds ratio=7.0; 95% CI: 2.6-13.2; p

Urinary tract infection (UTI) is common among pediatric population. Pyelonephritis, especially in young infants, is associated with a significant morbidity. Usually, clinical manifestations, laboratory findings, and imaging are used to differentiate between lower and upper UTI. Lack of specific clinical findings and commonly used nonspecific blood indices are important hamper differentiation between lower and upper UTI in early stages. Imaging techniques are neither cost benefit nor safe for detection of UTI. Recent efforts have focused on characterization of novel serum and urinary biomarkers for early detection of acute pyelonephritis in children. It seems that urinary NGAL, NAG, TNF-α and IL-8 may be used as novel markers for early diagnosis of acute pyelonephritis in children.

Background: TNF-? is one of the most potent cytokines in proinflammatory reactions. Objectives: This article contributes to evaluate the correlation between urinary TNF-? and inflammatory response markers in children with acute pyelonephritis. Patients and Methods: This cross sectional study, was performed on children with acute pyelonephritis. Fresh random urine samples were obtained before treatment of pyelonephritis. Urine samples were tested for TNF-? and creatinine. We also evaluated our patients with routine biochemical studies. Results: One hundred and twenty children with acute pyelonephritis were evaluated. Urinary TNF-?/Cr was 0.0051 0.00083 Pg/g creatinine. The authors found out a significant difference in urinary TNF-?/Cr between patients who have normal and abnormal levels of urine leukocytes, urine protein, urine culture, erythrocyte sedimentation rate and C- reactive protein. This study revealed a significant correlation between urinary TNF-alpha and urine WBC (r = 0.36, P value = 0.02), ESR (r = 0.75, P value = 0.03) and CRP (r = 0.58, P value = 0.02). Conclusions: We concluded that urinary TNF-?/Cr might be a good indicator for inflammatory response in children with acute pyelonephritis.

Background: Urinary tract infection (UTI) is common in children. UTI with or without vesico-ureteral reflux (VUR) may result in renal scarring. Severe renal scarring impairs renal function and may result in hypertension, renal insufficiency, and end stage renal disease requiring dialyses or transplantation. Beta 2 micro-globulin (2MG) is a low molecular weight protein freely filtered by the glomeruli and then actively reabsorbed normally up to 99.9% in the proximal tubules; its urinary excretion is an indication of proximal tubular cell dysfunction. Objectives: The current study aimed to determine whether urinary 2MG excretion would be elevated in patients with various grades of renal scar, and also its relationship with renal outcome in long term follow-up. Materials and Methods: Urinary 2MG and Creatinine (Cr) were measured in 83 spot urine samples of patients that 53 of them did DMSA renal scan both at the time of admission to confirm pyelonephritis, and 6 month later to detect scars. 2MG was measured by radioimmunoassay method using 2MG 96-test kit (RADIM Company; Germany), and the creatinine was measurd by spectrophotometry and was recorded as microgram per mg creatinine. Twenty children had various grades of renal scars.Results were compared with the ratios of 19 children with low uptake scanning, 14 children with normal scanning after recovery from pyelonephritis, and 30 normal children served as controls. EXCEL and SPSS softwares were employed to compare the mean urinary 2MG in groups by student t-test, ANOVA, and Unpaired t-test at P,0.05 significance level. Subsequently patients were followed up for 6 years.\\r\\n Results: The mean urinary 2MG/Cr ratio was significantly higher in the scarring group (5.23 10.6) than in the normal group (0.19 0.2), and in low uptake group (0.49 0.86) (P < 0.05). When mean 2MG/Cr ratios were compared for each grade of scarring; patients with sever scar (grade III) had higher values (14.69 15.82) than grades I (0.36 0.35) and II (3.37 5.20) (P < 0.05). Patients without renal scar had a 2MG/Cr ratio below 0.46 microgram/mg Cr. The mean 2MG was also higher in the refluxing group (3.45 7.97) than nonrefluxing group (0.23 0.24) ug/mgCr (P = 0.01). Three patients who had the highest 2MG/Cr ratio values (33.3, 27, and 26.6 microgram/mg Cr) had sever scar that rapidly progressed to ESRD. They were transplanted 2 years later; after transplantation they still had recurrent UTIs. 2nd patient underwent native nephrectomy for renal abscess.\\r\\n Conclusions: Results of the current study revealed that mean 2MG/Cr ratio was higher in patients with renal scar and poor outcome. Measurement of Urinary 2MG may be useful in the early detection of tubular damage in refluxing patients and patients with renal scars and has prognostic significance.

Background: Renal scintigraphy with technetium 99m labeled dimercaptosuccinic acid (99mTc-DMSA) is a traditional imaging technique commonly used to detect renal scar in patients with probable vesicoureteral reflux (VUR) and/or urinary tract infection (UTIs). We determined whether normal results of DMSA renal scan obviate the need for voiding cystourethrography (VCUG) in evaluating children with UTIs. Materials and Methods: We observed medical records from June 2006 to April 2007 retrospectively of 208 children admitted with community acquired UTIs to Mofid children hospital (Tehran, IR/Iran) a teaching hospital in Tehran in which their age was between 2-120 months. The association between DMSA renal scan results and VCUG findings performed 48 hours and 1 month after the diagnosis was evaluated. To examine the accuracy of abnormal DMSA results in predicting VUR, sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and negative and positive likelihood ratio (LRs) were calculated. Results: VUR was seen in 14.0% of renal units with normal results of DMSA and 17.3% of renal units with abnormal DMSA findings. High grade VUR (grade IIIV) was seen in 18 (7.1%) of the abnormal findings of DMSA group and in (2.8%) 1 of the normal DMSA results group (P = 0.56). In the group with previous UTI (n = 68), the sensitivity and NPV of abnormalities on DMSA renal scan for detecting the presence of VUR (grade IIIV) were 100%, and100%, respectively. In the group without evidence of previous UTI, the sensitivity and NPV of abnormalities on DMSA renal scan for detecting the presence of VUR (grade IIIV) were 93% and 97%, respectively. Totally the sensitivity and NPV of abnormalities on DMSA renal scan to detect the presence of VUR (grade IIIV) were 94% and 97%, respectively. Conclusions: As a screening test, DMSA renal scan is a high sensitive technique to assess VUR (grade IIIV) in children with UTI.

Background: The presence of vesicoureteral reflux (VUR) has been documented in 1.3% of the general population, 70% of infants with urinary tract infection (UTI), and 15–25% of children with UTI. Objectives: The main aims of this prospective cohort study were to compare the efficiency of different imaging techniques [renal ultrasonography, cortical scintigraphy with technetium-99m dimercaptosuccinic acid (99mTc DMSA)] in detecting VUR in patients with acute pyelonephritis. Patients and Methods: Between June 2003 and March 2010, we recruited a prospective cohort of patients aged 1 month to 14 years. Pediatric patients with documented urinary tract infections and imaging evidence of upper tract involvement were examined by DMSA scintigraphy, renal ultrasonography, and voiding cystourethrography (VCUG). The assessments included UTI profiling, kidney ultrasonography, and DMSA scans. Data were expressed as mean ± SD. Statistical tests were two-tailed and were considered significant when P ≤ 0.05. Results: A total of 1500 pediatric patients were eligible to receive treatment for pyelonephritis. DMSA scans were normal in 20.2% of the patients and abnormal in the remaining 79.8%, and the kidney ultrasonographies yielded normal results in 68.5% of the patients and abnormal results in the remaining 31.5%. There was a significant difference between the ultrasonography reports of patients with normal and abnormal DMSA scans. The VCUG results were normal in 74.1% of the patients and indicated VUR in the remaining 25.9% (VUR grade I in 10.7%, grade II in 7.3%, grade III in 4.7%, grade IV in 1.7%, and grade V in 1.5%). The refluxes were unilateral in 62.9% of the patients and bilateral in the remaining 37.1%. We found a significant correlation between the presence of VUR in VCUG and urological abnormality in ultrasonography (r = 0.14, P < 0.001). The incidence of VUR among patients showing severe abnormalities in DMSA scintigraphy, was significantly higher than that among patients with normal DMSA results or those showing mild to moderate changes in DMSA scintigraphy. Conclusions: We concluded that kidney ultrasonography and DMSA scans can be performed before VCUG in children with UTI. In addition, we recommend performing VCUG in cases of pyelonephritis only when the patients show abnormal kidney ultrasonography or DMSA scan results.

Studies of renal involvement in thalassemia syndromes have been varied and few. The most important cause of mortality and morbidity in these patients is organ failure due to iron deposition. We report here a cross-sectional study carried out between February 2005 and February 2006 on all beta-thalassemia major patients being treated in Mofid Children's hospital, Tehran. The aim of the study was to detect renal dysfunction in these patients. The patient cohort consisted of 103 patients with various disease severities. Fresh first morning urine samples were collected and analyzed for sodium (Na), potassium (K), calcium (Ca), creatinine (Cr), phosphate, uric acid (UA), N-acetyl beta-D-glucosaminidase (NAG) and amino acids. We also carried out a complete blood count evaluation and assayed fasting blood sugar and serum ferritin, sodium, potassium, creatinine, uric acid and amino acids in all patients. The mean age of our patient cohort was 12.5+/-5.53 years and 53.4% were female. Abnormal levels of urinary NAG were detected in 35.9% of patients (confidence interval 26-45%). Abnormal levels of fractional excretion (FE)-Na, FE-K and FE-UA and abnormal urine protein Pr/Cr and urine Ca/Cr ratios were present in 29.1, 7.8, 52.4, 0.3 and 22.3% of the patients, respectively. There was a significant relationship between urinary NAG and the age of the patient (R=0.35), duration of deferoxamine therapy (R= 0.31), duration of receiving blood transfusions (R=0.34) and level of fasting blood sugar (R=0.2). We concluded that renal disorders are not rare in patients with beta-thalassemia major and that they may increase in terms of frequency with age, increased duration of transfusion and deferoxamine usage and high levels of blood sugar.

Urinary tract infection (UTI) among children is sometimes associated with anorexia and sometimes failure to thrive. Appetite-regulating hormones may be a causative factor. Leptin regulates appetite, food intake, and body weight via hypothalamic melanocortin-4 receptor. Leptin is also a potential cytokine for inflammation. The aim of this study was to evaluate serum and urine leptin before and after treatment of children with UTI.In this before-after study, serum and urine leptin were measured in 40 patients with UTI at admission and 5 days after treatment. Pyelonephritis was suggested by signs and symptoms and confirmed with positive urine culture and dimercaptosuccinic acid renal scintigraphy. Other measurements included urinalysis, urine culture, urine creatinine level, complete blood count, erythrocyte sedimentation rate, C-reactive protein level and serum levels of urea, creatinine, glucose, cholesterol, and triglyceride.The mean serum leptin level was 6.85 ± 18.90 ng/mL before the treatment and 8.29 ± 18.30 ng/mL after the treatment, the difference of which was not significant (P = .64). There were significant correlations between serum leptin and age, weight, and C-reactive protein. Urine leptin levels were reduced significantly from 0.75 ± 0.82 ng/mL to 0.46 ± 0.27 ng/mL after the treatment (P = .03). A significant correlation was observed between urine leptin level with age and weight.Serum leptin level did not change significantly after treatment of UTI, but urine leptin significantly decreased. Serum leptin level was higher in patients with anorexia in comparison with children with normal appetite; however, the difference was not significant.

Tuberous sclerosis complex (TSC) is a multisystemic inherited autosomal dominant disease characterized by the development of hamartomas in the brain and kidneys. In about 2% of patients, polycystic kidney disease is present, which may result in different stages of renal insufficiency. Acute kidney failure has not been reported in infants with TSC. We report a female infant with TSC who was admitted to hospital with pyelonephritis, acute kidney injury, and polycystic kidney disease.

The widespread use of prenatal ultrasonography results in increased recognition of congenital hydronephrosis, a therapeutic and diagnostic challenge. This study was conducted to investigate the natural course of prenatal hydronephrosis and the accuracy of postnatal APD in determining the outcome.All newborns with prenatal hydronephrosis were followed up by ultrasonography after birth. Voiding cystoureterography, diethylene triaamine pentaacetic acid renal scintigraphy, and dimercaptosuccinic acid renal scintigraphy were done if indicated. The receiver operating characteristic curve was plotted to determine the best cutoff for the anterior-posterior pelvic diameter (APD) to distinguish surgical from spontaneously resolving group.Of 178 neonates, 42 (23%) required surgery. The area under the curve for APD to predict the need for surgery was 0.925 with an APD cutoff of 15 mm. The diagnostic value of APD for determining the need for surgery was determined by sensitivity and specificity of 95.2% and 73.5%, respectively.Postnatal APD on ultrasonography has a valuable diagnostic accuracy for requiring surgery and provides a useful guide for parental counseling.

The role of vitamin A in re-epithelialization of the damaged mucosal surfaces has been documented. The aim of this study was to evaluate the role of vitamin A in preventing renal scaring after acute pyelonephritis in children.This clinical trial study was conducted in children with acute pyelonephritis in Mofid Children Hospital (Tehran, Iran). Patients were randomly divided into two groups to receive ceftriaxone and vitamin A or ceftriaxone only. Dimercaptosuccinic acid (DMSA) renal scintigraphy was performed before the start of the treatment and 6 months later. Results were compared for renal scaring between the two groups.Seventy-six patients (11 boys and 65 girls) were enrolled. The mean age was 25 ± 24 months and 54 patients (71.1%) were under 2 years old. The average vitamin A level was 71 ± 24 microg/dL in the treatment group and it was 62 ± 18 µg/dL in the control group. Baseline DMSA scans were comparable between the two groups in terms of scarring (P = .53), but the second DMSA scans showed a significant change in progression of the renal injury and scaring in the control group compared to those treated with vitamin A as well as antibiotic (P.001).We found administration of the vitamin A was useful in decreasing the amount of the injury and scarring following the pyelonephritis. Based on our study, vitamin A can be used in conjunction with other treatments in the management of acute pyelonephritis in children.

In autosomal recessive distal renal tubular acidosis (DRTA), a substantial fraction of the patients have progressive bilateral sensorineural hearing loss. This coexistence is due to the mutations of a gene expressed both in the kidney and in the cochlea. The aim of this study was to assess the correlation between hearing loss and DRTA.In this study, 51 children diagnosed with renal tubular acidosis were evaluated. Diagnosis of DRTA was based on clinical manifestations and detection of normal anion gap metabolic acidosis, urine pH higher than 5.5, and positive urinary anion gap. Audiometry was performed in children with DRTA and sequencing of the ATP6V1B1 gene was done for those with sensorineural hearing loss.Twenty-seven patients (52.9%) had DRTA, of whom 51.9% were younger than 1 year old, 55.6% were boys, and 44.4% were girls. Eleven patients (40.7%) had bilateral sensorineural hearing loss, consisting of 5 of 15 boys (33.3%) and 6 of 12 girls (50.0%). There was no correlation between hearing loss and gender. Three patients with hearing loss had mutation in the ATP6V1B1 gene (11.1% of patients with DRTA and 27.3% of patients with DRTA and hearing loss).This study indicated that a significant percentage of the children with DRTA had sensorineural hearing loss and mutation in ATP6V1B1 gene. It is recommended to investigate hearing impairment in all children with DRTA.

Hypertension is one of the most common diseases in the world and a major risk factor for cardiovascular, renal, and neurologic diseases. It seems that hypertension and overweight in children are a growing epidemic. The aim of this study was to investigate the prevalence of hypertension in school-aged children in Tehran.In a cross-sectional study, blood pressure and anthropometric measurements were performed on school-aged children in Tehran from 2008 to 2009. Children aged 7 to 11 years from 5 public schools in Tehran were included. Blood pressure, weight, and height measurement were performed at the school. At each screening, 3 seated blood pressure, weight, and height measurements were made and at least after 3 minutes of rest and choosing proper cuff, blood pressure was measured by a pediatric nephrologist and a pediatric assistant.A total of 425 school-aged children were included. Twenty-four percent of the primary school children had hypertension and 12% were shown to be overweight. Hypertension was more common in students of the north of Tehran in comparison to other geographic parts of Tehran. There was a significant difference in the prevalence of hypertension between girl students of north of Tehran and girls of the other parts of Tehran.We concluded that hypertension is a common problem in school-aged children. Our study re-emphasized the need for prevention and control of high blood pressure in children to manage the global diseases burden due to hypertension.

INTRODUCTION. Urinary tract infection is the most common serious bacterial infection in children. The aim of this study was to compare the value of different laboratory and imaging techniques in detecting renal involvement in acute pyelonephritis. MATERIALS AND METHODS. In a cross-sectional study of patients 1 month to 14 years of age diagnosed with urinary tract infection were examined with systemic inflammatory markers, renal ultrasonography, voiding cystourethrography (VCUG), and technetium Tc 99m dimercaptosuccinic acid ((99m)Tc-DMSA) renal scintigraphy. A total of 1467 pediatric patients were eligible for treatment of pyelonephritis. Evaluations included a complete blood count, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), urinalysis, urine culture, and measurement of serum blood urea nitrogen and creatinine levels. RESULTS. The results of (99m)Tc-DMSA scans were normal in 20.2%, mild decreased cortical uptake in 45.0%, moderate decreased cortical uptake in 12.3%, severe decreased cortical uptake in 12.0%, and decreased cortical function plus irregularity or scar formation in 10.5%. Voiding cystourethrography showed vesicoureteral reflux in 25.9%. The sensitivity of (99m)Tc-DMSA for prediction of vesicoureteral reflux was 84.1% with a negative predictive value of 80.6%. Significant differences were found in the level of blood leukocyte count (P = .03), urine leukocyte count (P = .003), ESR (P = .008), and age (P = .04) between patients with normal and abnormal (99m)Tc-DMSA scan results. CONCLUSIONS. We found that in patient with clinical signs of pyelonephritis, (99m)Tc-DMSA renal scintigraphy can detect pyelonephritis more accurately than the other inflammatory and imaging tests.

Tumor necrosis factor-alpha (TNF-alpha) is an important mediator of the inflammatory response in serious bacterial infections. The aim of this study was to evaluate the potential of urinary TNF-alpha for diagnosis of acute pyelonephritis in children.This study was conducted from March 2006 to December 2007 on children with confirmed diagnosis of acute pyelonephritis. They all had positive renal scintigraphy scans for pyelonephritis and leukocyturia. The ratios of urinary TNF-alpha to urine creatinine level were determined and compared in patients before and after antibiotic therapy.Eighty-two children (13 boys and 69 girls) with acute pyelonephritis were evaluated. The mean pretreatment ratio of urinary TNF-alpha to urinary creatinine level was higher than that 3 days after starting on empirical treatment (P = .03). The sensitivity of this parameter was 91% for diagnosis of acute pyelonephritis when compared with demercaptosuccinic acid renal scintigraphy as gold standard.Based on our findings in children, the level of urinary TNF-alpha-creatinine ratio is acute increased in pyelonephritis and it decreases after appropriate therapy with a high sensitivity for early diagnosis of the disease. Further research is warranted for shedding light on the potential diagnostic role of urinary TNF-alpha in pyelonephritis in children.

The aim of this study was to assess urinary interleukin-8 (IL-8) levels in pyelonephritis and its relation with the clinical course of the infection and of inflammatory changes detected by renal scintigraphy.In this quasi-experimental before-after study, we evaluated 91 children aged 1 to 144 months (mean 34.4 +/- 35.2 months) with pyelonephritis. Inflammatory markers including erythrocyte sedimentation rate, C-reactive protein, leukocyte count, and urinary IL-8, together with the results of ultrasonography, voiding cystourethrography, and dimercaptosuccinic acid renal scintigraphy were evaluated in these children. The ratios of urinary IL-8 to creatinine (IL-8/C) before and after the treatment were compared with each other.Urinary IL-8/C levels were significantly higher after the empirical treatment in comparison with those before the treatment (0.19 +/- 0.21 versus 0.51 +/- 0.53, P.001). No correlation was found between the urinary IL-8 levels and leukocyturia, urine culture results, other inflammatory markers, or findings of imaging examinations.We found high urinary IL-8 levels in children with pyelonephritis. We also documented its increasing after the treatment. We conclude that evaluation of urinary IL-8 can be a noninvasive test for diagnosis of upper urinary tract infection and its response to treatment.

Prompt diagnosis and localization of pyelonephritis are of great importance in children. The urinary excretion of enzymes, and in particular N-acetyl-beta-D-glucosaminidase (NAG), is considered a simple noninvasive marker for detection of renal tubular dysfunction due to pyelonephritis. This study was performed to determine the diagnostic value of urinary NAG in acute pyelonephritis.In a quasi-experimental study conducted on 72 children with confirmed pyelonephritis, we measured urinary NAG, creatinine, and NAG-creatinine ratio before and after the treatment. Diagnostic values of these parameters were evaluated by considering the patients before and after the treatment as disease-positive and disease-negative groups, respectively.The patients were 18 boys (25.0%) and 54 girls (75.0%) with a mean age of 43.0 +/- 39.0 months. The mean levels of urinary NAG were 12.20 +/- 6.14 U/L and 5.46 +/- 7.98 U/L before and after the treatment, respectively (P.001). The sensitivity and specificity of urinary NAG-creatinine ratio for diagnosis of pyelonephritis were 73.6% and 77.3%, respectively, with a cutoff point of 10.16 U/g (area under the curve = 0.76, 95% confidence interval, 0.67 to 0.76). Significantly higher levels of urinary NAG were found in those who had a negative urine culture at diagnosis (8.8 +/- 10.4 U/L) compared to those with a positive urine culture (4.5 +/- 8.7 U/L).We concluded that urinary NAG is elevated in children with pyelonephritis and it can be considered as a further criterion in the diagnosis of upper urinary tract infection.