17° RIUNIONE ANNUALE del REGISTRO DIALISI DEL LAZIO L’EMODIALISI nel LAZIO in ETA’ PEDIATRICA Stefano Picca Dipartimento di Nefrourologia, Unità di Dialisi Ospedale “Bambino Gesù” , Ist. di Ricerca Scientifica ROMA, Italia. Demographics of CKD due to CAKUT: Insights from the ERA/EDTA Registry •Elke Wühl, M.D. •Center for Pediatrics and Adolescent Medicine, University of Heidelberg, Germany • large longitudinal database of patients receiving RRT throughout Europe • complete population-based information from birth to adulthood available from 12 countries covering 150 million people Spectrum of Primary Renal Diagnoses ESCAPE and 4C-Study 4C ESCAPE Hereditary and other 17% CAKUT Hypo/Dysplasia 43% Obstructive Urop. 39% VUR 50% other 5% Glomerular disease 14% 69% Glomerulo- Polycystic pathies kidney 8% disease 6% CAKUT 63% Vasculitis Other 2% Unknown 6% Metabolic 5% Nephronophthisis 4% HUS 3% Interstitial nephropathy 2% Post-ischemic 1% Nefropatie di base all’ingresso (coorte 1995-2010) 45 43.2 Età media (DS) all’ingresso per nefropatia • CAKUT: 10,3 (4,9) • Glomerulopathies: 8,6 (6,2) • Hereditary: 11,0 (5,2) • Other: 7,7 (6,2) 40 35 30 24.0 25 19.2 20 15 13.6 10 5 0 Congenital Abnormalities Kidney Urinary Tract Glomerulopathies Hereditary Other Incidence rates of RRT in the ERA-EDTA Registry by age per million population 300 Diabetic or hypertensive GN GN 250 Cakut CKD 200 Hered.NP IRF 150 HUS unknown Metabolic Vasculitis 100 Misc unknown GN 50 PN_other + 85 -8 4 80 -7 9 75 -7 4 70 -6 9 64 -6 4 60 -5 9 54 -5 4 50 -4 9 45 -4 4 40 -3 9 35 -3 4 30 -2 9 25 -2 4 20 -1 9 15 -1 4 10 59 04 0 CAKUT CAKUT Diagnosis Codes ERA-EDTA Annual Report 2009 CODE • Pyelonephritis associated with neurogenic bladder 21 • Pyelonephritis due to congenital obstructive uropathy w/o VUR 22 • Pyelonephritis due to VUR without obstruction 24 • Renal hypoplasia (congenital) – unspecified 60 • Oligomeganephronic hypoplasia 61 • Congenital renal dysplasia w/o urinary tract malformation 62 • Syndrome of agenesis of abdominal muscles (prune belly) 66 Characteristics of RRT Patients in the ERA-EDTA Registry ALL CAKUT 947.5 44.9 212 930 4 765 Gender (% male) 61.5 61.5 CAKUT diagnoses (%) 2.2 100 Prevalence 2009 (cases pmp) Incidence (no of patients) 1.1.1999-31.12.2009 Neurogenic PN 5.6 Obstructive PN 22.9 Refluxive PN 41.9 Hypoplasia 17.3 Dysplasia 12.3 Caratteristiche deio pazienti pediatrici del RRDTL ALL CAKUT 167 42.9 12 576 54 Gender (% male) 62.6 64.8 CAKUT diagnoses (%) 1.5 100 Incidence 2010 (cases pmp) Incidence (no of patients) 1.1.1995-31.12.2010 Neurogenic PN 11.9 Refluxive PN 64.4 Hypoplasia 22.7 Prune Belly 1.0 Age Distribution at Start of RRT Percent of patients starting RRT at given age groups 16 Other diagnoses 12 CAKUT 10 8 6 4 2 10 9 -1 15 4 -1 20 9 -2 25 4 -2 30 9 -3 35 4 -3 40 9 -4 45 4 -4 50 9 -5 54 4 -5 60 9 -6 64 4 -6 70 9 -7 4 5- 4 0 0- Percent Patients 14 Age 17 16.1 16 15.4 15 14 altre nefropatie CAKUT 13.2 13 12 10.7 11 10.3 10.3 9.8 10 9.3 9 8.2 8 7.3 6.7 7 6.7 6.7 6.0 5.7 6 5 4.6 4 5.7 5.0 4.6 4.6 3.6 3.4 3.1 4.9 4.6 3.6 2.7 3 2.1 2 1.5 1.3 0.8 1 0.2 0.1 0.2 0.3 10-14 15-19 0.5 0 0-4 5-9 20-24 25-29 30-34 35-39 40-44 45-49 50-54 55-59 60-64 65-69 70-74 75-79 80-84 85+ 0.75 1.00 Kaplan-Meier survival estimates 0.50 63% 0.00 0.25 37% 0 12 24 36 48 60 72 analysis time cakut = 0 84 cakut = 1 96 108 120 0.40 0.50 0.60 0.70 0.80 0.90 1.00 Kaplan-Meier survival estimates 0 12 24 36 analysis time c h 48 g o 60 0.40 0.50 0.60 0.70 0.80 0.90 1.00 Kaplan-Meier survival estimates 0 12 24 36 analysis time 00_05 12_14 48 06_11 15_17 60 Anno di incidenza. RRDTL 1995-2010 15 14 13 13 12 12 11 10 10 10 10 10 9 9 9 8 8 8 7 6 5 5 5 5 4 4 4 4 3 2 1 0 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 Età all’ingresso, per sesso. RRDTL 1995-2010 16 15 femmine 14 maschi 5 13 12 3 11 10 9 8 6 5 7 2 4 3 4 6 11 5 10 3 1 4 2 1 1 4 1 5 3 6 1 5 5 4 2 3 3 1 3 3 5 4 3 2 2 1 0 0 1 2 3 4 5 6 7 8 9 ETA' 10 11 12 13 14 15 16 17 Metodica dialitica per anno. RRDTL, 1995-2010 emodialisi bicarbonato emodiafiltrazione dialisi peritoneale 100% 1 95% 90% 1 1 2 1 1 85% 3 4 1 80% 4 75% 2 2 5 2 1 70% 65% 60% 2 55% 5 50% 9 8 12 45% 40% 7 8 7 3 35% 8 6 30% 25% 2 5 5 2002 2003 2004 3 3 2 20% 15% 10% 5% 0% 1995 1996 1997 1998 1999 2000 2001 2005 2006 2007 2008 2009 2010 Età all’ingresso per metodica dialitica. RRDTL 1995-2010 Metodica Media DS Mediana RIQ HD 10,9 5,0 12 9-15 DP 4,7 5,0 4 0-8 Totale 9,5 5,6 11 5-14 Centro dialisi all’ingresso. RRDTL 1995-2010 Centro Dialisi all'ingresso Bambino Gesù Roma Diagest Roma San Benedetto Alatri Aurelia Hospital Roma Grassi Ostia Roma Life Hospital Roma Nomentana Hospital Mentana Nuova Villa Claudia Roma Policlinico Umberto I Roma San Giovanni Evangelista Tivoli Annunziatella Roma Bernardini Palestrina Città di Roma Civile Anagni Diaverum Fondi Nuova Itor Roma Ospedali Riuniti Anzio-Nettuno Parodi Delfino Colleferro Riabilitazione Assistenza Nepi San Giacomo Roma sconosciuto Totale N % 83 65,9 3 2,4 3 2,4 2 1,6 2 1,6 2 1,6 2 1,6 2 1,6 2 1,6 2 1,6 1 0,8 1 0,8 1 0,8 1 0,8 1 0,8 1 0,8 1 0,8 1 0,8 1 0,8 1 0,8 13 10,3 126 100,0 CENTR Paese di nascita. RRDTL 1995-2010 Paese di nascita Italia Romania Albania Bosnia-Erzegovina Bulgaria Etiopia Filippine Marocco Moldavia sconosciuto Totale N % 105 83,3 3,2 4 2,4 3 0,8 1 0,8 1 0,8 1 0,8 1 0,8 1 0,8 1 6,3 8 126 100,0 Paese di nascita per anno. RRDTL 1995-2010 ITALIA ESTERO 100% 95% 1 1 1 1 1 90% 2 1 85% 3 80% 4 2 2 75% 2 70% 65% 60% 55% 50% 10 5 45% 4 8 11 8 12 9 7 40% 7 3 35% 7 30% 6 3 3 25% 2 20% 15% 10% 5% 0% 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005 2006 2007 2008 2009 2010 Tipologia di accesso vascolare per anno. RRDTL, 2002-2010 Prima FAV all'arto superiore, distale FAV prossimale all'arto superiore CV Temporaneo Cv Permanente 100% 95% 1 90% 2 85% 2 1 1 80% 2 75% 1 4 70% 1 65% 2 60% 55% 50% 1 2 45% 40% 3 5 35% 30% 5 3 25% 3 1 3 20% 1 15% 10% 5% 0% 2002 2003 2004 2005 2006 2007 2008 2009 2010 Incidenza cumulativa di trapianto per nefropatia. RRDTL, 1995-2010 Mesi 12 24 36 48 60 Cakut Glomerulopathies Hereditary Other 0.28 0.29 0.08 0.08 0.52 0.45 0.39 0.17 0.74 0.66 0.39 0.41 0.81 0.83 0.39 0.41 0.88 0.87 0.51 0.41 0.00 0.25 0.50 0.75 1.00 Kaplan-Meier failure estimates 0 6 12 18 24 30 36 analysis time CAKUT Hereditary 42 48 Glomerulopathies Other 54 60 Incidenza cumulativa di trapianto per quartili di età all’ingresso. RRDTL, 1995-2010 1.00 Kaplan-Meier failure estimates 0-5 0.11 0.16 0.46 0.52 0.66 6-11 12-14 0.31 0.30 0.48 0.45 0.72 0.65 0.77 0.71 0.77 0.83 15-17 0.19 0.65 0.73 0.84 0.88 0.00 0.25 0.50 0.75 Mesi 12 24 36 48 60 0 6 12 18 24 30 36 analysis time 0-5 12-14 42 6-11 15-17 48 54 60 Dialysis Unit, “Bambino Gesù” Pediatric Hospital Roma, Italy. Doctor: S. Picca Headnurse: A. Tancredi Nurses: N. Avari V. Bandinu D. Ciullo E. Iacoella P. Lozzi F. Paolucci C. Pia Transplant Coordinators: S. Tesei I. Pannacci