: Pediatr Nephrol (2006) 21:1493–1635 # IPNA 2006 DOI 10.1007/s00467-006-0236-x ABSTRACTS UNCORRECTED PROOF 1494 Pediatr Nephrol (2006) 21:1493–1635 Pediatr Nephrol (2006) 21:1493–1635 1495 1496 Pediatr Nephrol (2006) 21:1493–1635 Pediatr Nephrol (2006) 21:1493–1635 1497 1498 Pediatr Nephrol (2006) 21:1493–1635 Pediatr Nephrol (2006) 21:1493–1635 1499 1500 Pediatr Nephrol (2006) 21:1493–1635 CONTENTS Oral Communication OC 11 Acute renal failure OC 1 Bladder disorders and voiding disturbances OC 12 - OC 13 Bone, growth and mineral metabolism OC 14 - OC 16 Chronic renal failure OC 5 - OC 6 Clinical nephrology OC 3 - OC 4 Glomerular disease OC 20 Hemodialysis OC 8 Hereditary renal disease OC 9 - OC 10 Hypertension OC 19 Peritoneal dialysis OC 17 - OC 18 Transplantation OC 7 Tubular and interstitial disorders OC 2 Urinary tract infection and VUR Oral Poster OP 31 - OP 33 Acute renal failure OP 11 Bladder disorders and voiding disturbances OP 55 Bone, growth and mineral metabolism OP 52 - OP 54 Chronic renal failure OP 12 - OP 17 Clinical nephrology OP 4 - OP 1O Experimental nephrology OP 41 - OP 49 Glomerular disease OP 38 - OP 40 Hereditary renal disease OP 51 Hypertension OP 1 - OP 3 Kidney development and cell biology OP 30 Obstructive urophathy OP 56 - OP 60 Peritoneal dialysis OP 21 - OP 29 Transplantation OP 34 - OP 35 Tubular and interstitial disorders OP 50 Urinary mineral abnormalities and urolithiasis OP 18 - OP 20 Urinary tract infection and VUR OP 36 - OP 37 Vasculitis and autoimmune related disease Pediatr Nephrol (2006) 21:1493–1635 1501 Poster Presentation PP 116 - PP 122 Acute renal failure PP 71 - PP 76 Bladder disorders and voiding disturbances PP 300 - PP 312 Bone, growth and mineral metabolism PP 313 - PP 342 Chronic renal failure PP 20 - PP 70 Clinical nephrology PP 10 Electrolyte and fluids PP 1 - PP 9 Experimental nephrology PP 343 - PP 393 Glomerular disease PP 102 - PP 115 Hemodialysis PP 159 - PP 196 Hereditary renal disease PP 277 - PP 299 Hypertension PP 11 - PP 16 Kidney development and cell biology PP 146 - PP 158 Obstructive urophathy PP 394 - PP 413 Peritoneal dialysis PP 17 - PP 19 Renal pysiology PP 197 - PP 246 Transplantation PP 123 - PP 145 Tubular and interstitial disorders PP 264 - PP 276 Urinary mineral abnormalities and urolithiasis PP 77 - PP 101 Urinary tract infection and VUR PP 247 - PP 263 Vasculitis and autoimmune related disease 1502 Pediatr Nephrol (2006) 21:1493–1635 COD. OC 1 COD. OC 2 ULTRASOUND BLADDER MEASUREMENTS CAN REPLACE Uneffectiveness of antibiotic prophylaxis in children with VUR. URODYNAMIC STUDY FOR DIAGNOSIS OF NON- Preliminary data from a controlled study. MONOSYMPTOMATIC NOCTURNAL ENURESIS Pennesi M, Peratoner L*, Giacomini A, Travan L, Bordugo A*, Minisini S Clinica L TAFURO, D DEL GAIZO, M VERNI`, L R IERVOLINO, R DEL GADO Pediatrica-IRCCS Burlo Garofolo-Trieste *Pediatria-Ospedale S.M. degli Angeli- DEPARTMENT OF PEDIATRICS, SECOND UNIVERSITY OF NAPLES , via L. De Pordenone Presenting Author: Pennesi Marco Crecchio , 80131 Napoli , Italy IRCCS Burlo Garofolo , via dell`Istria 65/1 , 34100 Trieste , Italy Purpose: we have compared urodynamic studies (UD) with ultrasound (US) measurements Background: Antibiotic prophylaxis in VUR affected children has never proved to be for diagnosis and follow-up of patients with Non-Monosymptomatic Nocturnal Enuresis effective in preventing recurrent urinary infections and secondary renal damage. (NMNE). Objective: To determine the efficacy of antibiotic prophylaxis in reducing UTI and Methods: 738 enuretics children with daytime voiding symptoms have been subordinated to secondary renal damage in VUR affected children. UD and to US bladder. The upper limits for US bladder wall thickness measurements are 3 Methods: 100 children diagnosed with grade 2 to 4 mono/bilateral VUR were enroled in the and 5 mm (full and empty bladder). 704 children (95,4%) have urodynamic signs of a study and have been randomized in two groups (prophylaxis versus non prophylaxis). overactive bladder and the US bladder wall measurements thickened. After a six-month At the entry of the study all children underwent to renal scan with dimercaptosuccinic acid antimuscarin treatment, we have repeated a new study with US and UD. Also after the first (DMSA) done at least 4 months after the UTI. cycle of therapy we have confirmed relation between urodynamic and ultrasound The children in the prophylaxis group were administered a two years antibiotic treatment. measurements. Basing on this evidence, we have modified diagnostic management of The DMSA and VCUG has been repeted after 2 years. patients with NMNE. In enuretics with daytime voiding symptoms and with significant US Results: 90 patients, (43 treated, 47 not treated) completed two years of follow up. measurement (bladder wall thickness >3mm and >5mm to full and empty bladder Total relapses has been 61 in 29 patients;36 in 16 children in the prophylaxis group vs 25 respectively) diagnostic management is concluded. This new protocol has been applied to among 13 children in the control group (0.3 relapses/year/patient). 296 patients with NMNE. Only 11 cases had a worsening in the DMSA scan: 2 border line at the entry became grade I After the first 6 months of therapy, a new US study has been performed in all patients. without UTI; 9 pathologic at the entry, 5 worsened without UTI and 4 with 1 or 2 UTI. Results: we found that 224 patients (75,7%) were full-responders with normal detrusor On the contrary in all the other cases with one or more UTI, we did not find any DMSA thickness; 54 patients (18,2%) were partial responders but without normalization of detrusor scan worsening. thickness; only 18 patients (6,1%) were classified as non-responders with a persistence of a Conclusions: thickened bladder wall. The UTI relapses have been more common n the children treated with antibiotic Conclusions:. According to our experience US, which is non-invasive, is especially useful prophylaxis. for diagnosis and follow-up of NMNE instead of UD, which is invasive and often traumatic There are no evidence of any relationship between the number of UTI relapses and the for children. progression of the renal damage. COD. OC 3 COD. OC 4 Glomerular sclerosis in kidneys with congenital nephrotic syndrome Nephrin is critical for the action of insulin on human glomerular (NPHS1) podocytes A-M Kuusniemi, A Kaukinen, A-T Lahdenkari, C Holmberg, R Karikoski, and H Jalanko RJM Coward , GI Welsh, A Koziell, J Tava ré, P W Mathieson, M A Saleem Hospital for Children and Adolescents and Biomedicum Helsinki, University of Helsinki, Bristol children`s hospital and Academic renal unit , Department of clinical sciences, North Finland Bristol, Southmead hospital, , BS10 5NB Bristol , UK , Biomedicum Helsinki, Haartmaninkatu 8, huone B526b , 00290 Helsinki , Finland The leading causes of albuminuria and end stage renal failure in the world are secondary to Congenital nephrotic syndrome of the Finnish type (NPHS1) is caused by mutations in the abnormalities in the production or cellular action of insulin including diabetes mellitus and NPHS1 gene encoding podocyte slit diaphragm protein nephrin. Children with NPHS1 have the hyperinsulinaemic metabolic syndrome. The human glomerular podocyte is a critical severe nephrotic syndrome from birth and are nephrectomized as infants. The removed cell for maintaining the filtration barrier of the kidney and preventing albuminuria. We have kidneys serve as a unique human material to analyze the development of renal failure in recently shown this cell to be insulin sensitive in respect to glucose uptake with kinetics kidneys with proteinuria. In this work, the histology and expression of glomerular cell similar to muscle cells1. We now show that the podocyte protein nephrin is essential for this markers and cytokines were studied in nephrectomized NPHS1 kidneys using light and process in podocytes. Unique conditionally immortalised podocytes from two different electron microscopy, immunohistochemistry, Western blotting, and cytokine array. The patients with nephrin mutations (natural human nephrin knockout models) are unresponsive results showed that mesangial sclerosis and obliteration of capillaries were evident in to insulin, knocking nephrin down with siRNA in wild type podocytes abrogates the insulin practically every glomerulus (1530 glomeruli studied). Severely damaged and well response and stable nephrin transfection of nephrin deficient podocytes rescues their insulin preserved nephrons were commonly found adjacent to each other. Several profibrotic response. Mechanistically the c terminus of nephrin is forming a protein-protein interaction mediators and enzymes were expressed in NPHS1 glomeruli. Few inflammatory cells with the vesicular SNARE protein VAMP2 (using yeast-2 hybrid studies and co- (NPHS1: 3.71.8 vs. controls: 1.10.2 cells/glomerular cross section (GCS); P<0.05) were immunoprecipitation) which functionally allows the GLUT1 and GLUT4 rich vesicles to detected in the glomeruli in spite of the increased expression of several chemokines. The fuse with the membrane of this cell. This work demonstrates a previously unsuspected role podocytes showed severe ultrastructural changes and hypertrophy but little hyperplasia of nephrin in vesicular docking and insulin responsiveness of podocytes. (MIB-1: 0.15/GCS in NPHS1 vs. 0.03/GCS in controls) or apoptosis (TUNEL: 0/GCS vs. 0.06/GCS). Moderate amounts (median 15.9 cells/ml vs. 0.7 cells/ml; P<0.05) of podocytes 1Coward et al. The Human Glomerular Podocyte Is a Novel Target for Insulin Action. were secreted into the urine of NPHS1 patients. No crescents, tip lesions or misdirected Diabetes 54:3095-3102. filtration into paraglomerular or paratubular space were detected. In conclusion, the results indicate that podocyte damage in NPHS1 kidneys lead to endocapillary lesions that progress to glomerular sclerosis. Glomerular tuft adhesions, crescent formation and misdirected filtration seem to play a minor role in this process.