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Author(s): Pinar Ozge Avar-Aydin [sup.1] , Zeynep Birsin Ozcakar [sup.1] , Nilgun Cakar [sup.1] , Suat Fitoz [sup.2] , Hatice Dilara Karakas [sup.1] , Fatos Yalcinkaya [sup.1] Author Affiliations: (1) [...], Introduction Familial Mediterranean fever (FMF) is the most common hereditary autoinflammatory disease with an increased risk for secondary amyloidosis. Since lifelong colchicine has been the treatment of choice that prevents renal amyloidosis, non-amyloid kidney diseases are more frequently considered in the differential diagnosis of proteinuria. Nutcracker syndrome (NCS) can be one of the confounding causes. This long-term retrospective study aimed to evaluate the causes of proteinuria in a pediatric cohort of patients with FMF and discuss changing trends in recent years. Methods Demographic, clinic, and laboratory data were extracted from electronic medical records of patients with FMF. All urine tests of the study population were reviewed. Patients were evaluated for persistent proteinuria and grouped according to the etiology of proteinuria. Results A total of 576 patients with FMF were identified with a mean follow-up of 6.3 years in the last 10 years; 8% had persistent proteinuria. The etiology was NCS in 67.5% of the patients with proteinuria, and renal amyloidosis was less commonly encountered (15%) without any new diagnosis for the last 8 years. Non-amyloid kidney diseases were also diagnosed in 17.5% of the patients. Patients with NCS had significantly lower BMI than other patients in the cohort and less subclinical inflammation, higher hemoglobin concentration, and milder levels of proteinuria with normal serum albumin and eGFR than other patients with proteinuria. Conclusion Nutcracker syndrome is the leading cause of proteinuria in children with FMF nowadays, and it should be kept in mind during the evaluation of proteinuria in these patients. Graphical Abstract A higher resolution version of the Graphical abstract is available as Supplementary information.

Introduction Congenital anomalies of the kidney and urinary tract (CAKUT) account for more than half of abdominal masses observed in neonates. The prevalence of CAKUT has been reported to range [...], Background Congenital anomalies of the kidney and urinary tract (CAKUT) commonly cause chronic kidney disease in children. While most CAKUT cases are sporadic, observed familial clustering suggests that the pathogenesis is influenced by genetic factors. Methods The purpose of the present study is to determine the frequency of the kidney and urinary tract anomalies in asymptomatic first-degree relatives of patients with CAKUT. A total of 218 index patients and their families followed at an academic hospital in Ankara, Turkey, were enrolled in the study. Results Family histories revealed at least one other member with a known kidney or urinary tract disease in 50% and CAKUT in 22.9% of the families. All asymptomatic first-degree relatives of 180 index patients were screened for kidney and urinary tract anomalies using ultrasound. New anomalies were diagnosed in 116 asymptomatic first-degree relatives (23%) in 87 families (48.3%). When family histories and ultrasound findings of 180 index patients were evaluated together, 129 first-degree relatives in 92 families (51.1%) had CAKUT. Conclusions This study suggests that genetic mechanisms might be very important in the pathogenesis of apparently sporadic CAKUT. Identification of the underlying gene mutations will provide further insights into the knowledge of the kidney and urinary tract development and pathogenesis of CAKUT. Keywords CAKUT * Familial clustering * First-degree relatives * Kidney and urinary tract anomalies * Renal ultrasound

Introduction Nutcracker syndrome (NS) refers to compression of the left renal vein (LRV) between the aorta and the superior mesenteric artery (SMA) which results in elevation of pressure in the [...], Nutcracker syndrome (NS) refers to compression of the left renal vein between the aorta and the superior mesenteric artery which results in left renal venous hypertension. The typical clinical presenting feature is hematuria. In this report we describe the case of patient with a single kidney who developed severe proteinuria due to NS. She was successfully treated with left renal vein transposition. This case clearly shows the relation between NS and severe proteinuria based on normal biopsy findings and the complete disappearance of proteinuria following surgery. Keywords Left renal vein transposition * Nutcracker syndrome * Proteinuria * Single kidney

Abstract Cystic nephroma (CN) is a rare, presumably benign, multilocular cystic renal tumor. Pulmonary sequestration (PS) also presents as cystic masses of non-functioning primitive lung tissue. We describe a 15-month-old [...]

Byline: Aydin Yagmurlu (1), Burhan Aksu (1), Meltem Bingol-Kologlu (1), Nurten Renda (2), Gulcin Altinok (3), Suat Fitoz (4), I. Haluk Gokcora (1), Huseyin Dindar (1) Keywords: Caustic ingestion; Esophageal stricture; Sphingolipids; Sphingosylphosphorylcholine; Wound healing Abstract: Using a new class of intracellular 2nd messengers to prevent stricture formation after caustic ingestion, sphingosylphosphorylcholine (SPC) has a wide spectrum of activity in cell growth regulation and signal transduction. Caustic esophageal burns were created with 15% NaOH in an experimental rat model. Control group animals (n=10) had esophageal burns with no treatment, whereas the SPC group (n=10) had esophageal burns gavaged with SPC for 7 days. Efficacy of treatment was assessed in 28 days by contrast esophagograms, histopathologic evaluation, and biochemically by tissue hydroxyproline (OHP) content. Contrast esophagograms demonstrated that SPC significantly prevented stricture formation. Obvious collagen deposition was present in submucosa, muscularis mucosa, and muscular layers in the control group compared with the SPC group. The damage to the esophageal wall on histopathologic examination was significantly lower in the SPC group (p&lt 0.05). Tissue OHP contents were significantly lower in the SPC-treated group (3.0+-0.1 ug/mg) compared with the control group (4.3+-0.2 ug/mg) (p&lt 0.05). We conclude that SPC improves healing following caustic esophageal burns. Furthermore, SPC is effective in preventing caustic esophageal strictures. These effects of SPC occur through its proliferative and specifically its remodeling effects on wound healing. Author Affiliation: (1) Department of Pediatric Surgery, Ankara University, School of Medicine, Gumus Cad. D-14.4 Konutkent--1 06530, Ankara, Turkey (2) Department of Biochemistry, Hacettepe University, Faculty of Medicine, Ankara, Turkey (3) Department of Pathology, Hacettepe University, Faculty of Medicine, Ankara, Turkey (4) Department of Radiology, Ankara University, School of Medicine, Ankara, Turkey Article History: Registration Date: 15/01/2004 Accepted Date: 17/12/2002 Online Date: 05/06/2004

Answers 1. The common characteristics, symptoms, and signs in both cases are: They were both born via cesarean section because of fetal distress, both had severe perinatal asphyxia, both needed [...]

Answers During any evaluation of patients with hypertension and hypokalemic metabolic alkalosis, hormones, including plasma renin activity (PRA), serum aldosterone, and serum cortisol concentration, should be checked [1]. The results [...]

Answers Our patient's main problem was the presence of medullary nephrocalcinosis (NC). Nephrocalcinosis is defined as calcification located in the renal parenchyma. It can be subdivided into two forms, medullary [...]

Answer Obstructive pathologies including posterior urethral valve (PUV) can be the initial diagnoses in patients with hydronephrosis and/or recurrent urinary tract infections (UTI). Other causes ofhydronephrosis such as ureteropelvic junction [...], Keywords Child * Neurogenic bladder * Ochoa syndrome

Case summaries Case 1 A female newborn weighing 2,820 g was born at 38 weeks' gestation via cesarean section because of fetal distress and breech presentation. The emergency cesarean section [...]

Case summary A 13-year-old boy was admitted to our hospital with recurrent headaches that had been localized to the occipital region for the past year. His medical history was unremarkable, [...]

Case summary A 17-year-old female patient was admitted to our hospital for further evaluation of bilateral nephrolithiasis, which was detected incidentally by plain radiography and abdominal ultrasonography during the evaluation [...]

Byline: Rahsan Vargun (1), Suat Fitoz (2), AydA+-n Yagmurlu (1,3) Author Affiliation: (1) Department of Pediatric Surgery, Ankara University, School of Medicine, Ankara, Turkey (2) Department of Radiology, Ankara University, School of Medicine, Ankara, Turkey (3) Ankara Universitesi Tip Fakultesi, Cocuk Cerrahisi Anabilim Dali, Dikimevi, 06100, Ankara, Turkey Article History: Registration Date: 28/11/2005 Accepted Date: 21/11/2005 Online Date: 09/12/2005

Abstract Abdominal pain during peritoneal dialysis can be due to an inflow pain or most frequently be associated with peritonitis. However, other unusual pathologies can also occur in patients with [...]

Case summary A 20-month-old boy was hospitalized at our institution with acute pyelonephritis. He had a history of two previous episodes of urinary tract infection (UTI) treated at an other [...]

Pulmonary venous abnormalities are generally diagnosed by echocardiography and often confirmed by cardiac catheterization. Although angiography has been the gold standard for evaluation it carries certain inherent risks, especially in small and sick infants. In this study we retrospectively assessed the utility of magnetic resonance angiography (MRA) and computed tomography angiography (CTA) in the evaluation of pulmonary venous abnormalities in pediatric patients. The results were compared with operative findings. We reviewed nine patients with total APVC and four with partial APVC. Twelve patients were infant (age range: 1 day to 8 months) and the other was 16 years old. MRA and/or CTA clearly visualized the anomalous drainage of pulmonary veins in all patients. Cardiac catheterization was performed five patients with one total anomalous pulmonary venous connection and four with partial anomalous pulmonary venous connection. Ten patients were operated on. Diagnoses of patients were confirmed by operative findings. In Conclusions, MR and CT angiography allow detailed and comprehensive evaluation of the APVC and are good diagnostic modalities for use in the preoperative assessment of the anomaly in pediatric patients.