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Chronic kidney disease is a devastating disorder, which complicated the quality of life in affected patients. Determination the epidemiology of end stage renal disease (ESRD) seems necessary to decrease the occurrence of progressive renal damage in at risk patients. This study was performed to investigate the epidemiologic characteristics and treatment modalities of children with ESRD. A cross-sectional study was conducted on 115 children with ESRD admitted during 2020-2022 in a pediatric nephrology center in Southwest of Iran. All children were younger than 18 years and referred for renal replacement therapy (RRT). ESRD was defined as glomerular filtration rate less than 10-15 ml/min/1.73 m2 for at least 3 months. Information such as age of ESRD, gender, etiology of ESRD and type of RRT were obtained from their medical records. A total of 115 patients (53% male) were included. Mean age at the time of ESRD was 8.47 years. Males outnumbered females. The most common cause of ESRD was congenital abnormality of kidney & urinary tract (CAKUT) in 36.5% of patients, followed by hereditary disorders. The majority of patients were older than 5 years at the time of ESRD, with a significant correlation to the underlying disorder (P

Background: Vesicoureteral reflux (VUR) is the most common congenital urinary tract abnormality in children. Renal parenchymal damage is the most devastating complication of severe undiagnosed VUR. Different diagnostic biomarkers have been introduced as alternatives for radiologic evaluation in these patients. This review article aimed to increase the knowledge about the role of urine neutrophil gelatinase-associated lipocalin (uNGAL) in children affected by primary VUR and renal parenchymal damage. Methods: A systematic review of PubMed, Scopus, Web of Science, ProQuest, and Ovid was conducted in September 2022 to retrieve studies that investigated the correlation between uNGAL or uNGAL/Cr excretion and primary VUR in male/female patients younger than 18 years of age. Patients with secondary VUR, age older than 18 years, infectious or inflammatory disorders, obstructive uropathies, and acute or chronic kidney diseases were excluded. Two reviewers independently screened the titles and abstracts of the search results and then assessed the full texts selected from the pertinent studies. Results: Eighteen research articles with a total sample of 699 patients were found to measure uNGAL in VUR or renal scarring. UNGAL or uNGAL/Cr had increased excretion in the majority of children with primary VUR or RPD, with a positive or no correlation to the severity of VUR. Conclusions: Several studies addressed uNGAL and uNGAL/Cr as putative biomarkers for the prediction of VUR or reflux-associated RPD.

Introduction: Steroid dependent nephrotic syndrome (SDNS) is a difficult and troublesome presentation of idiopathic nephrotic syndrome (INS) in children, with complicated management and increased morbidity. Objectives: The aim of this study was to identify the predictive clinical and laboratory characteristics of patients with SDNS, for parents counseling, appropriate management and improving outcome. Patients and Methods: A total of 374 children with steroid sensitive INS were evaluated in two groups as steroid dependent (group 1=199) and non-steroid dependent (group 2= 175) INS. SDNS was defined as ≥2 relapses during steroid reducing treatment or 15 days after discontinuation of corticosteroids. Results: Mean age at presentation was significantly lower in children with SDNS than those without steroid dependency (P=0.022). Diagnostic age less than two years (P=0.016), total relapses (P

Background: Idiopathic nephrotic syndrome (INS) is one of the most common glomerular diseases in children with different pathological types and different responses to corticosteroids. A definitive diagnosis is essential for planning the treatment and determining the prognosis of these patients and currently, kidney biopsy is the only method for definitive diagnosis. However, this is an invasive procedure. In addition, in some cases, the biopsy is contraindicated or tissue obtained on biopsy is insufficient and may not represent the underlying disease. According to the recent hypothesis about the role of circulating permeability factors in the pathogenesis of INS, urine protein analysis as a noninvasive method to determine the specific biomarkers of the disease is of great interest to nephrologists and can be useful. Methods: In this case − control study, we analyzed urinary CD80 (uCD80) levels in 51 patients with INS using a special CD80 enzyme-linked immunosorbent assay kit and were compared between different groups of patients. Results: The highest urine CD80/creatinine ratio was found in patients with active minimal change disease and steroid-responsive nephrotic syndrome in the relapse stage of the disease. Conclusion: A significant level of uCD80 is correlated with better renal function and a more favorable response to steroids in patients with INS. Therefore, it can be concluded that a high level of uCD80 is correlated with a good prognosis in these patients.

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-β-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

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 (P

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; were 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.

Background: Preterm birth occurs in a large number of pregnancies, and its incidence has been reported to be on the rise. Acute kidney injury (AKI) is a common complication in the premature infants with respiratory distress syndrome (RDS). The present study aimed to determine the predictive factors, clinical courses, and outcomes of AKI in the neonates with the clinical and radiological manifestations of RDS. Methods: Medical records of 84 premature neonates with RDS were evaluated in two groups of case (with AKI) (n=34) and control (without AKI) (n=50). Diagnosis of AKI was based on the increased level of serum creatinine (>1.5 mg/dL) after the third day of birth or increasing serum creatinine level. In addition, blood pressure and laboratory findings, including complete blood count, serum electrolytes, and urine volume, were compared between the two groups. Results: Mean age of the infants with AKI was 5.41±3.29 days, and the majority of the patients had nonoliguric renal failure. Among the samples, 23.5% died, and 76.5% were discharged without renal impairment. Birth weight, systolic blood pressure, blood urea nitrogen, calcium, and pH on admission had significant correlations with the presence of AKI. Moreover, birth weight was observed to be a relatively accurate predictive factor for AKI (AUC=0.08; 95% CI=0.68-0.91), with 73.5% sensitivity and 80% specificity. Conclusion: According to the results, AKI was more common in the low-birth-weight infants with severe RDS compared to the other subjects

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.

Hypertension in newborn infants, particularly those requiring intensive care, is becoming increasingly recognized, with prevalence of 0.2-3%. Recent studies have established normative tables for blood pressure (BP) in both term and pre-term infants based on the gestational age, postnatal age, gender, weight and height, identifying the neonates at increased risk for early-onset cardiovascular disease. Common causes of neonatal hypertension include thromboembolic complications secondary to umbilical artery catheterization, congenital renal structural malformation, renovascular disease, aortic coarctation, as well as acute kidney injury and certain medications. A careful diagnostic evaluation should lead to identification of the underlying cause of hypertension in most infants. Treatment options should be tailored to the severity; and underlying cause of hypertension, including intravenous and/or oral therapy. This review summarizes recent work in these areas, focusing on optimal BP measurement, definition, evaluation and management of hypertension as well as advances in drug therapy of neonatal hypertension.

Hemolytic uremic syndrome (HUS) is a heterogeneous group of hemolytic disorders. Different terminologies have been described in HUS, which are as follows: (1) D+ HUS: Presentation with a preceding diarrhea; (2) typical HUS: D+ HUS with a single and self-limited episode; (3) atypical HUS (aHUS): Indicated those with complement dysregulation; (4) recurrent HUS: Recurrent episodes of thrombocytopenia and/or microangiopathic hemolytic anemia (MAHA) after improvement of hematologic abnormalities; and (5) familial HUS: Necessary to distinct synchronous outbreaks of D+ HUS in family members and asynchronous disease with an inherited risk factor. aHUS is one of the potential causes of end-stage renal disease (ESRD) in children. It has a high recurrence after renal transplantation in some genetic forms. Therefore, recognition of the responsible mechanism and proper prophylactic treatment are recommended to prevent or delay the occurrence of ESRD and prolong the length of survival of the transplanted kidney. A computerized search of MEDLINE and other databases was carried out to find the latest results in pathogenesis, treatment, and prevention of aHUS.

Urinary tract infection (UTI) is the most common serious bacterial infection in early life. Appropriate diagnosis and treatment prevent complications such as hypertension, proteinuria and end stage renal disease. A computerized search of MEDLINE, Embase and other databases was done to find the latest results about the treatment and prevention in pediatric UTI. Randomized control trials, systematic reviews and original articles were assessed. Search terms were "UTI, treatment, prophylaxis, prevention, and children". All children with complicated or simple UTI were included in our search study from neonatal period to late childhood and medical aspects of treatment were reviewed. Recently, treatment approaches have been changed by simplification of drug administration. Oral treatment is recommended especially in older infants and children instead of strict intravenous treatment and patient admission. In addition, prophylactic treatment becomes easier and limited to certain cases. In this article, we review the recent information and approaches in this setting.

Background and Aim: Intestinal obstruction is one of the important diseases in children that need to be diagnosed and treated appropriately to prevent complications. The present study was conducted to identify the most common causes of intestinal obstruction, age and sex prevalence, complications and associated anomalies in Iranian children. Materials and Methods: One hundred children aged under 12 years old with manifestation such as vomiting, abdominal pain, no fecal and gas passage and abdominal distention indicative of intestinal obstruction were studied. Different etiologies of obstruction were documented by of radiologic procedures or during surgery. Sixty and three patients were less than 1 month, 27 between 1-12 months and 10 had 1-12 years. Most of the patients (73) were male. Results: The most common causes were Hirschprung disease, invagination and imperforated anus with relatively equal prevalence. The most common age of hirschprung disease and imperforated anus was in neonatal period. Invagination was more prevalent during infancy. Intestinal perforation occured in 6 patients, mostly in small bowel atresia obstruction. Hirschprung disease and imperforated anus were most common causes of mortality. Associated anomalies were cardiovascular, urologic, genital and gastrointestinal abnormalities, especially in imperforated anus and hirschprung disease. Conclusion: The results of this study are largely confirming the findings of previous similar studies in other countries.

A 6.5 yr old girl was admitted with a category of clinical signs and symptoms including recurrent gross hematuria, ab-dominal 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 re-sponsive to colchicine treatment.

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.

Nephrolithiasis, obstructive renal failure, essential hypertension, and chronic tubulointerstitial nephritis have been considered as the renal complications of hyperuricemia. Massive proteinuria has been rarely reported as the primary manifestation of increased serum uric acid. This is the report of a child presented with proteinuira, hypertension, and glomerular scelrosis secondary to hypouricosuric hyperuricemia, who was treated by uric acid lowering management.

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.

Kidney disease is not a common complication in Down Syndrome (DS). A variety of renal and urologic problems have been described in these patients and some develop renal failure. Coincidence of polycystic kidney disease and DS is a rare entity. This is a report of a 3.5-month-old infant with DS, known to have early end-stage renal failure due to polycystic kidney disease.

Febrile convulsion (FC) is the most common seizure disorder in young children. Different predisposing factors have been suggested to enhance the susceptibility to febrile seizure and its recurrence. The main objective of this study was to identify the adverse effect of electrolytes disturbance in FC and its recur-rence. The medical records of 175 children with convulsive disorders were reviewed. Patients were divided into 3 groups. Group A (n=71) with simple febrile convulsion (FC) and group B (n=54) with recurrent FCs. Fifty children (group C) with non-FC served as control. Serum sodium and calcium concentrations were significantly lower in groups A and B compared to the control group. Serum sodium level was not significantly different between group A and B patients (134.4 vs. 134.7 mEq/l) but was significantly lower in group A than the control group (P= 0.014). Serum calcium concentration did not differ among the 3 groups. Minor abnormal levels of serum sodium concentration were detected in children with febrile convulsions. Thus, routine serum electrolytes screening are not recommended in febrile seizure.