Introduction: Vesicoureteral reflux (VUR) refers to the retrograde flow of the urine from the bladder to the ureter and kidney. In children with a febrile urinary tract infection (UTI), those with reflux are 3 times more likely to develop renal injury compared to those without reflux. Reflux nephropathy was once accounted for as much as 15-20% of end-stage renal disease in children and young adults. With greater attention to the management of UTIs and a better understanding of reflux, end-stage renal disease secondary to reflux nephropathy is uncommon. Reflux nephropathy remains one of the most common causes of hypertension in children. Reflux in the absence of infection or elevated bladder pressure does not cause renal injury. We sought to determine the association of infantile reflux nephropathy (IRN) with prenatal risk factors. 

Materials and Methods: In this study, 96 infants with reflux-related renal injury and 96 infants with VUR without reflux nephropathy were evaluated. Maternal information was assessed. Data was analyzed using SPSS version 18.

Results: The results of this study showed that age more than 35 years, pre-gestational hypertension, preeclampsia and eclampsia, preterm delivery, very low birth weight (VLBW), pre gestational diabetes mellitus, and maternal BMI<18.5kg/m2 (underweight) were prenatal risk factors for infantile reflux nephropathy.

Conclusions: The data suggests that prenatal factors may affect the risk of IRN. Adequate prenatal care and good maternal support can be effective in the prevention of reflux-related renal injury. 

Keywords: Vesico-Ureteral Reflux; Risk Factors; Prenatal; Infant.

Introduction: Tubulointerstitial disorders are characterized by diseases that affect the vascular and interstitial compartments of the kidney with relative sparing of the glomeruli. They might be either acute or chronic. Acute tubulointerstitial nephritis (TIN) is associated with acute renal failure due to either acute infection of the kidneys or reaction to medication. Chronicinterstitial nephritis is characterized by many syndromes of renal tubular dysfunction that may be primary or secondary due to renal tubular damage from a wide variety of causes. The aim of this study was to evaluate the pathologic characteristics of acute and chronic TIN and their probable causes.

Materials and Methods: All native kidney biopsy specimens with a diagnosis of tubulointerstitial nephritis admitted to Ali-Asghar Hospital, a pediatric referral center in Tehran, from 1983 to 2013 were retrospectively re-evaluated. The demographic data of the patients were collected and pathologic findings were reviewed.

Results: Forty-four patients, 18 males and 26 females with a mean age of 8.8 years (SD=4), were enrolled in this study. Thirty-seven (84%) patients had chronic and 7 (16%) had acute TIN. The disease was primary in 32 (72%) patients with a diagnosis of familial nephronophthisis and medullary cystic disease and 12 (28%) had other diseases. Kidney biopsy showed similar pathologic findings including periglomerular fibrosis (72%), different scores of interstitial fibrosis/tubular atrophy (91%), infiltration of inflammatory cells, and segmental and global glomerular sclerosis (89%).

Conclusions: Acute and chronic tubulointerstitial nephritis with different etiologies has similar pathologic findings. The patients mostly present in the late stages of the disease; therefore, determining the etiology is impossible. Many cases are congenital.

Introduction: Urinary Tract Infection (UTI) is one of the most common pediatric infections. UTI may create cystitis or pyelonephritis by involving bladder or renal parenchyma, respectively. Pyelonephritis, especially in pediatric patients, can lead to scar formation in kidneys and consequent complications such as hypertension, proteinuria, dysfunction and chronic renal insufficiency. The current study aimed to determine risk factors of acute rental cortical lesions in renal scintigraphy in children with UTI.

Materials and Methods: Fifty-three patients with significant renal cortical lesions and 53 cases without significant renal cortical lesions were compared based on the intensity of findings of DMSA scintigraphy within the first two weeks of diagnosis. Patients were divided into three groups of 1 month to 2 years, 2 to 4 years and 4 to 10 years.

Results: Of 106 patients, 11 males (20.8%) and 42 females (79.2%) had significant acute renal cortical lesions, whereas 15.1% of males and 84.9% of females had no significant acute renal cortical lesions. There was a significant difference in the degree of fever, the average interval between the onset of fever and treatment, mean level of CRP, leukocytosis and ESR in the two studied groups. The presence of Vesicoureteral Reflux (VUR), low initial hemoglobin and low initial BMI as random findings were associated with significant renal cortical lesions. Gender, age, grade of VUR and type of organism in urine culture had no significant association with significant renal cortical lesions.

Conclusions: In this study, delaying in treatment, high degree fever, leukocytosis, high initial ESR and CRP, existence of VUR and low initial BMI and hemoglobin levels were associated with an increase in the value of acute renal cortical lesions, so in these cases, DMSA scan is suggested.

Introduction: To investigate clinical presentation, metabolic risk factors and urinary tract abnormalities in paediatric urolithiasis.

 Materials and Methods: Between 2011 and 2012, 100 children (53 boys and 47 girls) were treated for urolithiasis. Clinical presentation, calculus localisation, urinary tract infection status, presence of anatomic abnormalities and urinary metabolic risk factors were retrospectively evaluated.

Results: The most common clinical features on admission were restlessness/irritability (62%), flank pain (33%) and gross hematuria (4%). Twenty-one percent of patients were detected incidentally during evaluation for other conditions. Urine random tests revealed metabolic abnormalities, including hypercalciuria (56%) and hypocitraturia (64%) in most cases. Anatomic malformation (32%) and urinary tract infections (UTI)(9%) were other presentations.

Conclusions: We concluded that most patients were symptomatic and hypocitraturia was the most common risk factor.

Introduction: Hyperhomocysteinemia is common in end stage renal diseases. We aimed to determine the prevalence of hyperhomocysteinemia in dialysis cases and define independent risk factors of the development of hyperhomocysteinemia.

Materials and Methods: The total plasma homocysteine values were measured in 46 dialysis patients including 20[43.4 %] girls and 26[56.6 %] boys aged 1.6-25 [19.9±6.5] years based on two different reference values for children [age dependent] and adults [cut off point of 15 µmol/L].

Results: Using the reference values for children, 26 cases [56.2 %] had hyperhomocysteinemia including 41.6% of CAPD and 2/3 of hemodialysis patients with no significant difference based on age, gender, duration and modality of dialysis, and dosage of folate supplement [p>0.05 for all]. Using a cut-off point of 15 µmol/L, hyperhomocysteinemia was reported in 30.4% of the patients including 11 hemodialysis and one CAPD [P=0.022], 10 out of 19 girls [52.6%%] and 4 out of 26 boys [15.4%] [p=0.063], but logistic regression analysis did not show any significant differences in the incidence rate of hyperhomocysteinemia according to the modality of dialysis and gender [P=0.998 and 0.137 respectively].

Conclusions: We found mild hyperhomocysteinemia as a common finding in dialysis patients; also, the prevalence of hyperhomocysteinemia was comparable in children and young adults. However, we noted that hemodialysis patients and females were more prone to more intense elevations of plasma homocysteine levels. We found that neither gender nor modality of dialysis played a role as risk factors for development of hyperhomocysteinemia in children and young adults.

Hypertension and hyponatremia together, is an uncommon entity in children. We here described a 10-year-old boy presented with hypertensive emergency and altered sensorium with hyponatremia. After initial stabilization USG (ultrasonography) Doppler showed shrunken right kidney with absence of flow in the right renal artery. Right Renal Resistive Index was 0.9. Therefore, patient underwent right total nephrectomy and blood pressure ultimately came under control.

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Acute poststreptococcal glomerulonephritis (APSGN) is one of the most common renal diseases resulting from a prior infection with group A β-hemolytic streptococcus. Manifestations of acute poststreptococcal glomerulonephritis ranges from subclinical infections to life threatening conditions. Typical clinical features of the disease include an acute onset with gross hematuria, edema, hypertension and moderate proteinuria (acute nephritic syndrome) 1 to 2 weeks after an antecedent streptococcal pharyngitis or 3 to 6 weeks after a streptococcal pyoderma. Patients with APSGN sometimes exhibit unusual clinical manifestations, which may lead to diagnostic delay or misdiagnosis of the disorder. Cardiogenic shock is uncommon but potentially fatal initial manifestations of ASPGN. There are very few reports of cardiogenic shock as the initial manifestation of APSGN. In patients presenting with cardiogenic shock, without a clear etiology, APSGN should be considered. We report a 07 year old boy presenting with cardiogenic shock as initial manifestations of APSGN.

Nephrolithiasis is quite common in children. It sometimes has a genetic basis and can lead to serious complications like urinary obstruction, multiple surgical interventions, or even renal insufficiency if left treated. Cystinic stones and cystinuria account for approximately 8% of the cases of nephrolithiasis in children. We studied seven pediatric patients, 1 to 3 years old (mean age: 20.5 months), with cystinic urinary stones receiving D-penicillamine plus other drugs to dissolve the stone. All of them tolerated the treatment very well and did not show any serious complication. All of our cases were managed with D-penicillamine that was initiated at a low dose and then increased progressively. We used low dose D-penicillamine, maximim15 mg/kg/day, which was beneficial without any specific side effects. D-penicillamine can be used safely in little children. Gradual induction and close observation with CBC, urine analysis, BUN, creatinine, and liver function tests may be required. D-penicillamine can prevent new stone formation and resolve the present cystinic calculi. Low dose D-penicillamine may be sufficient in treating cystinic calculi in children. We suggest more evaluations on the advantage of low dose D-penicillamine in cystinuria.

Keywords: D-Penicillamine; Cystinuria; Nephrolithiasis; Complications