N-acetyl-beta-D-glucosaminidase (NAG) is a lysosomal enzyme present in the proximal convoluted tubules of the kidneys that may be used as a marker of proximal tubular damage and nephrotoxicity. An abnormal urinary NAG excretion has been reported in different kinds of renal disorders such as acute kidney injury, urinary tract infection, vesicoureteral reflux; diabetes mellitus; nephrotic syndrome; glomerulonephritis; hypertension; perinatal asphyxia; heavy metals poisoning; drug nephrotoxicity, renal allograft rejection; and heart failure. This paper provides an overview of the diagnostic value of urinary NAG in the nephrourology field.

Keywords: N-acetyl-beta-D-glucosaminidase; Acute Kidney Injury; Kidney Diseases; Pyelonephritis; Hypertension; Vesico-Ureteral Reflux.

Introduction: Acute renal failure (ARF) is a clinical syndrome in which a sudden deterioration in renal function results in the inability of the kidneys to maintain fluid and electrolyte homeostasis. A classification system has been proposed to standardize the definition of acute kidney injury in adults. These criteria of risk, injury, failure, loss and end-stage renal disease were given the acronym of RIFLE. Our goal was to study the mean platelet volume (MPV) as a prognostic predictor of ARF in children. Mean platelet volume (MPV) is a machine-calculated measurement of the average size of platelets in blood and typically included in blood tests as part of CBC (Complete Blood Count). Since the average platelet size is larger when the body is producing increased numbers of platelets, MPV can be used to make inferences about platelet production in bone marrow or platelet destruction problems.

Material and Methods: The records of 200 patients with ARF were investigated prospectively. Complete blood count including MPV, erythrocyte sedimentation rate, serum C-reactive protein and electrolytes of patients were measured and compared.

Results: MPV values were low in loss (p=0.0012) and failure (p<0.005). The sensitivity and specificity of MPV for the diagnosis of loss and failure were higher than those of the other inflammation markers. MPV<8.2 fL was significantly associated with poor prognosis in renal functions.

Conclusions: MPV is a fast and reliable measurement with considerable predictive value for prediction of prognosis in acute renal failure.

Keywords: Acute Kidney Injury; Mean Platelet Volume; Blood Platelets; Acute Kidney Injury; Prognosis.

Introduction: The aim of the study was to find out the different grades of VUR and renal scar in children presented with single or recurrent episodes of UTI.

Materials and methods: Thirty six children from one month to 16 year with single or recurrent episodes of UTI were enrolled in this prospective cross sectional study which was done from July 2012 to June 2013. Grading of vesico-ureteric reflux, renal cortical scar and their co-relation outcome variables.

Results: Out of 36 patients, 33 (91.6%) were male and 3 (8.4%) were female. All the patients were divided into three groups based on their age (<12 month, 12-60 month and .60 month).VUR were detected in 37(51.39%) renal units among all the group of patients evaluated for UTI. Mild reflux (grade I and II) was found in 2 (2.78%), Moderate in 9 (12.5%) and severe in 26 (36.11%) cases. Out of 72 kidney units, 37 kidney units had different grades of cortical scar. Grade I & II renal scar was found in 12 (33.33%) patients in right kidney and 14 (38.88%) in left kidney. Higher grades of scar (grade III & IV) were found in right kidney and left kidney, 5 (13.88%) and 6 (16.66%) respectively. Positive co-relation was observed between grades of reflux and grades of renal scars (for right kidney r= -0.484, p<0.01; left kidney r= -0.424, p<0.01).

Introduction: Nephrotic syndrome is the most common chronic renal disease in children. Mostly, it is controlled by steroids. Many underlying pathologies exist in patients with steroid-resistant nephrotic syndrome (SRNS). Among them are ‘focal segmental glomerulosclerosis (FSGS) and ‘minimal change disease’ (MCD). Examining patients’ clinicopathologic characteristics can be helpful by giving an insight into the etiology of steroid resistance and determining patient prognosis.

Material and Methods: This cross-sectional study was performed in ‘Children’s Medical Center’ between 2001 and 2011. From 150 patients biopsied, seventy-one children with SRNS, aged 1-14 years, were included.

Results: Among 150 patients biopsied, 71 children (47.3%) had steroid-resistant nephrotic syndrome. Forty-four (62%) of these were boys. Upon pathologic investigation of SRNS cases, FSGS came in first, with the highest prevalence at a rate of 32.4%, and MGN came in last, at a rate of 5.6%. The mean age of disease onset was 4.7 years and the mean age of undergoing biopsy was six years.

Conclusions: In this study, the predominant pathologic pattern of steroid-resistant nephrotic syndrome was FSGS, a finding similar to that of most studies conducted in this field. MCD was observed in 21.1% of patients, which indicates the variety in reporting renal lesions, particularly, regarding the diagnoses of MCD, mesangio-proliferative glomerulonephritis and early stages of FSGS.

Keywords: Steroid-resistant; Nephrotic syndrome; Child.

Introduction: The kidney regulates sodium balance and is the principal site of sodium excretion. Sodium is unique among electrolytes because water balance, not sodium balance, usually determines its concentration. Although water balance is usually regulated by osmolality, volume depletion stimulates thirst, renal protection of water and ADH secretion. Volume reduction has priority over osmolality; volume depletion stimulates ADH secretion, even if a patient has hyponatremia. The aim of this study was to consider scar nephropathy in children with UTI and hyponatremia and compare it with children without hyponatremia.

Material and Methods: 200 children with pyelonephritis were included in this case–control study as case and control groups, respectively. Subjects were selected from children referred to the pediatric clinic of our hospital in Arak, Iran. Case group included children with hyponatremia and UTI (with VUR) and control group included children with UTI (With VUR) and normal serum sodium. Data was analyzed using SPSS ver.18

Results: Among 200 (100%) children in both groups, 5 children (5%) had normal sodium and reflux nephropathy and 23 children had hyponatremia and reflux nephropathy.

Conclusions: Hyponatremia in children with reflux nephropathy was significantly more common than children without reflux nephropathy. The observed correlation between reflux-related injury and hyponatremia necessitates evaluation of electrolytes in children with pyelonephritis.

Keywords: Pyelonephritis; Child; Hyponatremia; Vesico-Ureteral Reflux.

Streptococcal pharyngitis is common in the pediatric age group. Although its treatment is simply achieved by administration of a single dose of benzathine penicillin, oral penicillin for 10 days, or azithromycin for 5 days, it has serious complications such as rheumatic fever (RF) and chorea if left untreated. Treatment of pharyngitis does not prevent glomerulonephritis but prevents the spread of streptococci that can cause an epidemic in glomerulonephritis if they are of the nephritogenic strain. Post Streptococcal Glomerulonephritis (PSGN) is common in school age children and usually has a benign course as more than 95 percent of the cases recover the acute phase and less than 5 percent progress in their course to Rapidly Progressive Glomerulonephritis (RPGN) and ultimately about one percent develop End Stage Renal Failure (ESRF). Herein, we present a 12-year-old male with a history of untreated streptococcal pharyngitis who first came to our clinic with a history of arthralgia and arthritis, fatigue, hematuria, petechia, purpura, elevated levels of Blood Urea Nitrogen (BUN) and Creatinine (Cr), and low grade fever. Echocardiography revealed endocarditis which was treated. However, renal failure required renal replacement therapy (RRT), and massive proteinuria needed renal biopsy which revealed membrano proliferative glomerulonephritis (MPGN).

Keywords: Streptococcal Infections; Membranoproliferative; Glomerulonephritis; Pharyngitis; Renal failure.  

Williams-Beuren syndrome is a rare neurodevelopmental disease, in which mental retardation is common with this submicroscopic chromosomal deletion genetically disease.

We reported a boy with Williams-Beuren syndrome and highlighted the clinical characteristics and response to treatment with Ritaline of such an individual with ADHD and voiding dysfunction.

The patient was a six year-old boy with typical presentation of WBS and some psychiatric problems referred because of hyperactivity symptom and attention deficit.

Children with mental retardation and WBS have a comorbidity of psychiatric disorders and kidney anomalies.

Williams-Beuren syndrome can present with hyperactivity and Ritalin can improve some symptoms and voiding dysfunction.

Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) is a potentially life-threatening, complex, and multifaceted disease which may imitate other grave conditions. It presents with cutaneous drug eruptions, fever, hematologic abnormalities (an eosinophil count of 1500/mm3 or atypical lymphocytosis), and systemic involvement including hematologic, renal, pulmonary, hepatic, cardiac, gastrointestinal, neurologic, and endocrine abnormalities. Anticonvulsant therapies (mainly carbamazepine) are among the most important causative drugs.

Case report: Herein we present a10-year-old girl who developed skin rash, systemic symptoms, marked eosinophilia, and kidney involvement following anticonvulsive treatment with phenobarbital and sodium valproate. She experienced multiple hospitalizations due to an improper diagnosis and management.

Conclusion: Drug Induced Hypersensitivity Syndrome (DIHS) is a severe life-threatening disorder which mostly occurs due to aromatic anticonvulsive drugs. The disease may mimic other serious conditions and delay in the diagnosis and improper treatment may cause organ involvement and more severe outcomes.

Key words: Drug Hypersensitivity Syndrome; DRESS Syndrome; Drug Reaction with Eosinophilia and Systemic Symptoms; Drug Eruptions; Interstitial Nephritis.

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