Abbas Madani

Glomerular diseases in Iranian children: clinico-pathological correlations

Glomerular disease constitutes an important part of renal disease. The purpose of this study was to describe and correlate the types of glomerular diseases reported in native kidney biopsy specimens and their clinical presentations in Iranian children. The medical records and pathological diagnoses of patients with glomerular disease whose native kidney biopsy specimens were received by our hospital during a 10-year period were reviewed. In each patient, pathological diagnosis, clinical presentation, age at onset of disease, gender, and presence of nephrotic proteinuria, hematuria, and hypertension, were determined. Minimal-change disease was the most frequent glomerular disease and nephrotic syndrome was the most frequent clinical presentation encountered. For each disease, correlations between the clinical presentation and pathological diagnosis were made. This study describes various glomerular diseases encountered in Iranian children, with their respective clinical presentations.

Vesicoureteral Reflux and Primary Bladder Neck Dysfunction in Children: Urodynamic Evaluation and Randomized, Double-Blind, Clinical Trial on Effect of α-Blocker Therapy

PURPOSE Primary bladder neck dysfunction has been under diagnosed as a treatable cause of vesicoureteral reflux. We evaluated the effect of prazosin administration on vesicoureteral reflux resolution and urodynamic parameters in children with idiopathic primary reflux and primary bladder neck dysfunction. MATERIALS AND METHODS A total of 62 children (mean ± SD age 7.9 ± 2.4 years) with documented vesicoureteral reflux and urodynamics proved primary bladder neck dysfunction were randomized to receive either 0.025 mg/kg α-blocker (prazosin, 40 patients) or placebo (22) nightly for 1 week with a subsequent increase to 2 divided doses. Patients were followed for 12 months with clinical evaluation and uroflowmetry performed every 2 months, and each patient underwent complete urodynamic study at 6-month intervals. RESULTS In the placebo group no uroflowmetry or urodynamic parameter changed significantly at 1-year followup. A 60% decrease in reflux grade was observed in the treatment group compared to 17% in the placebo group. Mean maximal detrusor pressure, post-void residual and opening time were significantly decreased in both followup sessions in the prazosin group (p <0.05). Average flow rate improved from 4.30 to 12.80 ml per second at 6 months and to 13.10 ml per second at 12 months (both p <0.05). CONCLUSIONS Special attention should be given to secondary causes of vesicoureteral reflux (such as primary bladder neck dysfunction, an underdiagnosed entity in children), since conventional treatment will most likely fail if these conditions are not addressed promptly. In this study prazosin was effective therapy for children with vesicoureteral reflux and primary bladder neck dysfunction.

Comparison between fractional excretions of urea and sodium in children with acute kidney injury

Fractional excretion of sodium (FENa) has been said to be the most sensitive index for differentiating prerenal failure (PRF) from intrinsic renal failure (IRF). However, there are several instances of high FENa (>2%) in cases of PRF and low FENa (<1%) in IRF patients. In contrast, the fractional excretion of urea nitrogen (FEUN) is primarily dependent on passive forces, and many confounding variables that affect FENa have little effect on FEUN, if any. To compare FEUN with FENa, pediatric patients with acute kidney injury (AKI) were prospectively evaluated by history, physical examination, and obtaining appropriate laboratory data during a 1-year interval. Diagnosis of PRF or IRF was made in each patient, and renal failure indices were compared between two groups using chi-square and t test, as appropriate. Probability value (P value) <0.05 was considered significant. Receiver operating characteristic (ROC) plots for FEUN and FENa were drawn to compare the discriminative power of each index. Forty-three patients were enrolled in the study. There were 27 patients in the PRF and 16 in the IRF group. FENa was 2 ± 0.4 in PRF and 4.5 ± 1% in IRF patients (P < 0.05), and low FENa (<1%) was only seen in 44.4% of PRF patients, which was not statistically different from those with IRF (P > 0.05). FEUN was 23.6 ± 4.9% in PRF and 41.6 ± 4.8% in IRF patients (P < 0.05), and low FEUN (<35%) was seen in 77.8% of the PRF group (P < 0.05). Cutoff values of 30% and 1.6% were reached for FEUN and FENa, respectively. In conclusion, FEUN < 35% had higher sensitivity and specificity than FENa < 1% for differentiation of PRF from IRF.

NPHS2 gene mutation in an Iranian family with familial steroid-resistant nephrotic syndrome

1 Pediatrics Center of Excellence. Childrens Medical Center, Tehran University of Medical Sciences. Tehran (Iran) 2 Research Center for Immunodeficiencies. Childrens Medical Center, Tehran University of Medical Sciences. Tehran (Iran) 3 Department of Pediatrics. Faculty of Medicine, Hacettepe University. Ankara (Turkey) 4 Institute of Human Genetics, University Hospital of Magdeburg. Magdeburg (Germany) 5 Department of Immunology. Molecular Immunology Research Center, Research Center for Immunodeficiencies, Childrens Medical Center, Tehran University of Medical Sciences. Tehran (Iran)

An infant presenting with a non-functional kidney on dimercaptosuccinic acid scan: answer

Owing to complete deterioration of the right kidney’s function, seen on dimercaptosuccinic acid (DMSA) scan, presence of severe hydronephrosis, and a large, enhanced, well-defined, hyperechoic mass with an inhomogeneous density in the lower pole of the right kidney, extending into the pelvis and proximal ureter, shown on the third renal ultrasound and computed tomography (CT), the initial differential diagnoses included: xanthogranulomatous pyelonephritis [1–3], malakoplakia [4–6], inflammatory pseudotumour of the renal pelvis [7–9], and fungal infection [10, 11]. In this patient, the preoperative diagnosis was not identified. Therefore, with the patient under general anaesthesia, a percutaneous sonography-guided biopsy of the renal mass was performed, and findings of an examination of a frozen section were compatible with Wilm’s tumour. The patient underwent a right radical nephroureterectomy via a hemitransverse incision of the upper abdomen on 15 July 2006. The resected specimen showed a large, polypoid, light-yellow, glistening mass located in the pelvicalyceal system, extending into the proximal ureter without invading the surrounding tissues (Fig. 5). Histopathological examination showed features of Wilms’ tumour, exhibiting both epithelial and mesenchymal differentiation (Fig. 6). Thus, a final diagnosis of intrapelvic Wilms’ tumour, stage I, was considered. The immunohistochemical findings were positive for the Wilms’ tumour 1 gene (WT-1) in the tumour cells, but staining with myogenin showed negative reaction. On 17 July 2006, 3 days after her nephrectomy, she developed cough, dyspnoea, and fever. Lung auscultation revealed rhonchi. A chest X-ray did not show any evidence of pneumonia, but it disclosed hypoplasia of the third right rib (Fig. 7). On 22 August 2006, due to the chronic cough, a barium swallow was performed and displayed significant gastro-oesophageal reflux. The patient received combination chemotherapy with dactinomycin (AMD) and vincristine (VCR), in accordance with the National Wilms’ Tumor Study (NWTS) IV protocol of stage. An abdominal ultrasonogram at the last follow up (50 days after nephrectomy) showed no mass lesions in the ovaries. Pediatr Nephrol (2010) 25:257–260 DOI 10.1007/s00467-009-1210-1

Enalapril induced reversible acute renal failure detected by (99m)Tc-DMSA renal scan in a patient with bilateral renal artery stenosis: a case report.

The authors report and discuss a case of bilateral renal artery stenosis in a hypertensive 9 year-old girl that was first suspected with 99mTechnetium-dimercaptosuccinic acid renal scintigraphy. The scintigraphy showed signs of acute renal failure while the patient was on enalapril for controlling her hypertension. Bilateral renal artery stenosis was confirmed with computed tomography angiography. Hypertension resolved after bilateral renal artery angioplasty.

Clinical Course of Children and Adolescents with Primary Vesicoureteral Reflux: A retrospective study of 958 patients

Abstract Introduction. Vesicoureteral reflux (VUR) is the most common pediatric urologic abnormality and since it can predispose to urinary tract infection and resultant kidney scar it is an important issue in pediatric nephrourology. Methods. A retrospective chart review and follow-up of 958 patients with primary VUR was performed in the Children’s Medical Center, Tehran, Iran. Children with primary vesicoureteral reflux were included in the study and these parameters were studied: age, sex, clinical presentation, VUR grade, sonographic findings, DMSA changes, treatment modality (medical, surgical or endoscopic) and response to treatment, hypertension (presence/absence), urinary tract infection recurrence and development of new kidney scars in patients under medical treatment. Results. VUR was more prevalent in girls. Sonography was unable to detect VUR in many cases. Presence of renal scars was strongly associated with degree of reflux. Medical management was effective in a substantial percentage of patients and they experienced full resolution of reflux. This was especially true for lower degrees of VUR. 17.6% of patients developed new kidney scars on followup which was associated with higher degrees of VUR. Hypertension and breakthrough urinary tract infection was an uncommon finding in our patients. Conclusion. Medical management, which means using prophylactic antibiotics for prevention of urinary tract infection, is effective in many cases of VUR especially in cases with lower degrees of VUR. Surgical and endoscopic procedures must be reserved for patients with higher degrees of VUR unresponsive to conservative management or in whom new scars may develop.