Stefano PiccaDipartimento di Nefrourologia, Unit di DialisiOspedale Bambino Ges , Ist. di Ricerca ScientificaROMA, Italia.LEMODIALISI nel LAZIO in ETA PEDIATRICA 17 RIUNIONE ANNUALE del REGISTRO DIALISI DEL LAZIO

*

Demographics of CKD due to CAKUT:Insights from the ERA/EDTA Registry

Elke Whl, M.D.

Center for Pediatrics and Adolescent Medicine, University of Heidelberg, Germany

*

large longitudinal database of patients receiving RRT throughout Europecomplete population-based information from birth to adulthood available from 12 countries covering 150 million people

*

Spectrum of Primary Renal DiagnosesESCAPE and 4C-Study

*

Nefropatie di base allingresso (coorte 1995-2010)Et media (DS) allingresso per nefropatia CAKUT: 10,3 (4,9) Glomerulopathies: 8,6 (6,2) Hereditary: 11,0 (5,2) Other: 7,7 (6,2)

*

Incidence rates of RRT in the ERA-EDTA Registry

by age per million populationDiabetic or hypertensive GNunknownGNCAKUT

Diagramm3

0.93770140372.85621691930.57124338391.35805030880.31256713460.64669062320.22634171810.03233453120.97003593490.43112708220.0970035935

1.02750372181.93848640290.79446164050.32837747810.10592821870.26482054680.38134158750.0847425750.64616213430.73090470930.0741497531

2.01282889713.77151273151.13856988120.40663210040.12198963010.23381345770.31513987780.5184559280.80309839841.15890148620.1728186427

4.83730118614.69613480911.02580900641.20461975060.15057746880.39526585570.17881074421.00698682281.53400796372.64451679630.7340651605

8.91717779544.09242459210.65478793471.49050411460.11200319940.53416910470.2153907681.68004799043.63579616394.63520932751.3440383924

11.09599740473.52836752011.07766881271.06968608070.17562010280.35124020560.19158556671.83602834758.27809302786.01897988721.6125118531

13.11960670963.24313581791.71057998990.82045918070.17802416180.50311176170.13158307611.772501437514.33481511877.11322629272.0279274088

15.62442299492.81239613914.99200314690.77340893820.24217855640.41404720940.24217855641.984301720420.0383224919.25747062452.4764710447

18.920755992.575643101510.17174608970.90760756910.45789210690.44971546220.36794901452.305813824227.522586284512.32220366333.7530799479

22.6713382022.287212402417.34614902090.77695383130.56743819140.55870837310.55870837312.540377133940.296841409417.17155265435.3950277277

26.17023034272.115810933222.05145172610.73348112350.80870995670.45137299910.53600543643.084382160461.1704449823.05763736988.3409968789

31.79968770712.12414416922.39722601770.66639817071.25990904140.35402402820.84341018484.060863852697.502382347432.705572720311.214231716

35.37114675932.038649617820.7320300120.93294135051.77374034550.36856942241.12874385625.1714897085141.784049692947.69518682217.2306204988

41.94027572422.373977871218.86209740270.68754550372.46478576790.38917670020.98591430725.9803486263202.125405523767.275678906825.2835129559

43.94287808742.66229826419.6232321480.79270678653.61952910050.44870195461.21149527747.2988851282255.057147727999.357569480831.7082714586

41.76966081142.289262632115.93101618550.52540453854.14694296470.43158229951.08833797267.0366679264272.1595509472121.368448395434.2075883464

28.85607272021.806979505610.84187703360.25019716232.86336752430.33359621640.86179022574.8649448228213.0011841832113.72851011424.2691247444

10.50115013850.75008215273.637898440801.500164305500.18752053821.275139659792.972682833164.732089782111.4012487218

GN

Cakut

CKD

Hered.NP

IRF

HUS

Metabolic

Vasculitis

Misc

unknown

PN_other

All_g_abs

0-45-910-1415-1920-2425-2930-3435-3940-4445-4950-5454-5960-6464-6970-7475-7980-8485+Total

GN8797198514103513901695200023142597278330543071323329382226103828030550

Cakut26518337149947544241936031526222520417718317812265204765

CKD53751121097613522163912441987234521511800145413128493909715049

Hered.NP12631401281731341069911189786481535328901403

IRF2910121613222331566586121154190242221103401434

HUS60252342624465535564483432303023120702

Metabolic21363119252417314564578198768158315800

Vasculitis3851107195230229254282291328390449461488375175344350

Misc9061791634221037185225653366461665059364123101558117053145047662247999709

unknown40691142815387549191185150719672452314141415186664364684091172641222

PN_other9717781562022623174596188871077149619492120182387330412654

Total783602104819563170441458087534975412620157941968123809283963113826697144494985212638

FrequencyMissing=292

99999278006894403551983690171062575971160681131252703971291959461280047271222995531145499211063422059603867986822178770858076685952653292269359716312666374616762749318638259_RBREAK_391810233

All_g_abs

8726553126296021390409

971837531102536861697

19837111240122331517911417

51449910912816421910716328178

103547576173136225195422538156

13904421351342244242301037754202

16954192211062365172291852919262

20003606399931533125425651185317

2314315124411156554528233661507459

259726219878965646429146161967618

278322523457886485732865052452887

30542042151641213481390936431411077

307117718008115432984491231041411496

323318314545319030764611558151861949

293817813125324230814881705366432120

22261228492822123583751450464681823

1038653909103123117576624091873

2802097040053424791726304

GN

Cakut

CKD

Hered.NP

IRF

HUS

Metabolic

Vasculitis

Misc

unknown

PN_other

all_g_pmp

872655312629602139

97183753110253687

198371112401223315117

51449910912816421910778

103547576173136225195156

1390442135134224424230202

1695419221106236517229262

200036063999315331254317

23143151244111565545282459

2597262198789656464291618

2783225234578864857328887

305420421516412134813901077

307117718008115432984491496

323318314545319030764611949

293817813125324230814882120

22261228492822123583751823

10386539091031231175873

28020970400534304

GN

Cakut

CKD

Hered.NP

IRF

HUS

Metabolic

Vasculitis

PN_other

all_d_abs

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

GN

Cakut

CKD

Hered.NP

IRF

HUS

Metabolic

Vasculitis

PN_other

all_d_pmp

0-45-910-1415-1920-2425-2930-3435-3940-4445-4950-5454-5960-6464-6970-7475-7980-8485+Total

GN0.93770140371.02750372182.01282889714.83730118618.917177795411.095997404713.119606709615.624422994918.9207559922.67133820226.170230342731.799687707135.371146759341.940275724243.942878087441.769660811428.856072720210.5011501385

Cakut2.85621691931.93848640293.77151273154.69613480914.09242459213.52836752013.24313581792.81239613912.57564310152.28721240242.11581093322.1241441692.03864961782.37397787122.6622982642.28926263211.80697950560.7500821527

CKD0.57124338390.79446164051.13856988121.02580900640.65478793471.07766881271.71057998994.992003146910.171746089717.346149020922.051451726122.397226017720.73203001218.862097402719.62323214815.931016185510.84187703363.6378984408

Hered.NP1.35805030880.32837747810.40663210041.20461975061.49050411461.06968608070.82045918070.77340893820.90760756910.77695383130.73348112350.66639817070.93294135050.68754550370.79270678650.52540453850.25019716230

IRF0.31256713460.10592821870.12198963010.15057746880.11200319940.17562010280.17802416180.24217855640.45789210690.56743819140.80870995671.25990904141.77374034552.46478576793.61952910054.14694296472.86336752431.5001643055

HUS0.64669062320.26482054680.23381345770.39526585570.53416910470.35124020560.50311176170.41404720940.44971546220.55870837310.45137299910.35402402820.36856942240.38917670020.44870195460.43158229950.33359621640

Metabolic0.22634171810.38134158750.31513987780.17881074420.2153907680.19158556670.13158307610.24217855640.36794901450.55870837310.53600543640.84341018481.12874385620.98591430721.21149527741.08833797260.86179022570.1875205382

Vasculitis0.03233453120.0847425750.5184559281.00698682281.68004799041.83602834751.77250143751.98430172042.30581382422.54037713393.08438216044.06086385265.17148970855.98034862637.29888512827.03666792644.86494482281.2751396597

Misc0.97003593490.64616213430.80309839841.53400796373.63579616398.278093027814.334815118720.03832249127.522586284540.296841409461.1704449897.5023823474141.7840496929202.1254055237255.0571477279272.1595509472213.001184183292.9726828331

unknown0.43112708220.73090470931.15890148622.64451679634.63520932756.01897988727.11322629279.257470624512.322203663317.171552654323.057637369832.705572720347.69518682267.275678906899.3575694808121.3684483954113.72851011464.7320897821

PN_other0.09700359350.07414975310.17281864270.73406516051.34403839241.61251185312.02792740882.47647104473.75307994795.39502772778.340996878911.21423171617.230620498825.283512955931.708271458634.207588346424.269124744411.4012487218

Total8.43931263346.37687876810.653761031318.408095564227.311549383135.235778808944.954970955558.857201421979.7549930538110.1703073195148.5205239068204.9278499551274.227168086368.3687192897465.7227154138500.9544630198401.6776442525186.9579765724

all_d_pmp

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

0000000000

GN

Cakut

CKD

Hered.NP

IRF

HUS

Metabolic

Vasculitis

unknown

PN_other

cyst_a

0.93770140372.85621691930.57124338391.35805030880.31256713460.64669062320.22634171810.03233453120.97003593490.43112708220.0970035935

1.02750372181.93848640290.79446164050.32837747810.10592821870.26482054680.38134158750.0847425750.64616213430.73090470930.0741497531

2.01282889713.77151273151.13856988120.40663210040.12198963010.23381345770.31513987780.5184559280.80309839841.15890148620.1728186427

4.83730118614.69613480911.02580900641.20461975060.15057746880.39526585570.17881074421.00698682281.53400796372.64451679630.7340651605

8.91717779544.09242459210.65478793471.49050411460.11200319940.53416910470.2153907681.68004799043.63579616394.63520932751.3440383924

11.09599740473.52836752011.07766881271.06968608070.17562010280.35124020560.19158556671.83602834758.27809302786.01897988721.6125118531

13.11960670963.24313581791.71057998990.82045918070.17802416180.50311176170.13158307611.772501437514.33481511877.11322629272.0279274088

15.62442299492.81239613914.99200314690.77340893820.24217855640.41404720940.24217855641.984301720420.0383224919.25747062452.4764710447

18.920755992.575643101510.17174608970.90760756910.45789210690.44971546220.36794901452.305813824227.522586284512.32220366333.7530799479

22.6713382022.287212402417.34614902090.77695383130.56743819140.55870837310.55870837312.540377133940.296841409417.17155265435.3950277277

26.17023034272.115810933222.05145172610.73348112350.80870995670.45137299910.53600543643.084382160461.1704449823.05763736988.3409968789

31.79968770712.12414416922.39722601770.66639817071.25990904140.35402402820.84341018484.060863852697.502382347432.705572720311.214231716

35.37114675932.038649617820.7320300120.93294135051.77374034550.36856942241.12874385625.1714897085141.784049692947.69518682217.2306204988

41.94027572422.373977871218.86209740270.68754550372.46478576790.38917670020.98591430725.9803486263202.125405523767.275678906825.2835129559

43.94287808742.66229826419.6232321480.79270678653.61952910050.44870195461.21149527747.2988851282255.057147727999.357569480831.7082714586

41.76966081142.289262632115.93101618550.52540453854.14694296470.43158229951.08833797267.0366679264272.1595509472121.368448395434.2075883464

28.85607272021.806979505610.84187703360.25019716232.86336752430.33359621640.86179022574.8649448228213.0011841832113.72851011424.2691247444

10.50115013850.75008215273.637898440801.500164305500.18752053821.275139659792.972682833164.732089782111.4012487218

GN

Cakut

CKD

Hered.NP

IRF

HUS

Metabolic

Vasculitis

Misc

unknown

PN_other

cyst_pmp

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

GN

Cakut

CKD

Hered.NP

IRF

HUS

Metabolic

Vasculitis

PN_other

CAKUT_a

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

000000000

GN

Cakut

CKD

Hered.NP

IRF

HUS

Metabolic

Vasculitis

PN_other

CAKUT_pmp

PRD(PRD)agedm

0-45-910-1415-1920-2425-2930-3435-3940-4445-4950-5454-5960-6464-6970-7475-7980-8485+

-929192853568299132152206248255255250269226125312495

0Chronic renal failure; aetiology uncertain3150862284826728201053135517612204288638864936637462423966169538727

10Glomerulonephritis; histologically NOT examined491614128742855469679196810601252130014401379107855614712106

11Focal segmental glomerulosclerosis with nephrotic syndrome in children52526968443929464234253127312517111643

12IgA nephropathy (proven by immunofluorescence, not code 76 and not 85)10158024535846250755558755656642838825017860205256

13Dense deposit disease; membrano-proliferative GN; type II (proven by immunofluorescence and/or electron microscopy)05699301619182921182217161051251

14Membranous nephropathy1117253757749812413417520922823617469151665

15Membrano-proliferative GN; type I (proven by immunofluorescence and/or electron microscopy - not code 84 or 89)38103653649493108103125108131110106823621272

16Crescentic (extracapillary) glomerulonephritis (type I, II, III)38223752584750627699146170217237224111321651

17Focal segmental glomerulosclerosis with nephrotic syndrome in adults1210237810512813217818521319219017714111948241946

19Glomerulonephritis; histologically examined, not given above22114679193242289363434461516520526555467287115305156

20Pyelonephritis - cause not specified54650104140158193234314432490643807790575243675255

21Pyelonephritis associated with neurogenic bladder14183049504648393434372627181722482

22Pyelonephritis due to congenital obstructive uropathy with/without vesico-ureteric reflux8046981431341301189286544533344041383191252

23Pyelonephritis due to acquired obstructive uropathy2361529323850801061782764406687557734121754038

24Pyelonephritis due to vesico-ureteric reflux without obstruction15208718719918619915212913110285817369431661780

25Pyelonephritis due to urolithiasis0038816334583131194227293334415350159412340

29Pyelonephritis due to other cause2025151433296267838412014016012559211021

30Interstitial nephritis (not pyelonephritis) due to other cause, or unspecified (not mentioned above)31312183142447074106132162183214213170101271615

31Nephropathy (interstitial) due to analgesic drugs0001162011355910815822225624018867161388

32Nephropathy (interstitial) due to cis-platinum000033433713181020181000112

33Nephropathy (interstitial) due to cyclosporin A000214912184046638011273361710523

34Lead induced nephropathy (interstitial)00000102321101400015

39Drug induced nephropathy (interstitial) not mentioned above12491415173640629816216117814211752181128

40Cystic kidney disease - type unspecified5851091321458413314818719516015713076151401

41Polycystic kidneys; adult type (dominant)4517166416155211221814213319171569126311246912967812817

42Polycystic kidneys; infantile (recessive)37212019141510911122215147101120249

43Medullary cystic disease; including nephronophthisis54183622832243017112217975862409

49Cystic kidney disease - other specified type2031191153101720151317169102173

50Hereditary/Familial nephropathy - type unspecified9581725232719373428243128241050354

51Hereditary nephritis with nerve deafness (Alports Syndrome)041988118866458534232253418191440678

52Cystinosis22624643176755323111107

53Primary oxalosis189659031561731442185

54Fabrys disease0001144851113711101058

59Hereditary nephropathy - other specified type117221323302515222113181516710400371

60Renal hypoplasia (congenital) - type unspecified47386556323925443325273921293719121589

61Oligomeganephronic hypoplasia046103211000241310038

63Congenital renal dysplasia with or without urinary tract malformation11365917054323022281717810107442584

66Syndrome of agenesis of abdominal muscles (Prune Belly)96634301010013300040

70Renal vascular disease - type unspecified034762427578317330255285014412117215912634369504

71Renal vascular disease due to malignant hypertension020135180136197206252269217229275271217116442575

72Renal vascular disease due to hypertension113104094144228394589974145920382960372939572706109120418

73Renal vascular disease due to polyarteritis126391013143232356779112152117669759

74Wegeners granulomatosis10131827262546558411614821321324420089191537

75Cholesterolembolia00020237242128611091361578117630

76Glomerulonephritis related to liver cirrhosis00010022331391210681070

78Cryoglobulinaemic glomerulonephritis01011022467882885164

79Renal vascular disease - due to other cause (not given above and not code 84-88)52366614183374104159262421530469255502417

80Diabetes glomerulosclerosis or diabetic nephropathy - Type I302610956211441493171718351877209521652131181210803696718467

81Diabetes glomerulosclerosis or diabetic nephropathy - Type II2002284590254644148827083925495351323688154531224798

82Myelomatosis/light chain deposit disease00010521550116199281456580662572268943301

83Amyloid003931478090103205240361487572471355100213175

84Lupus erythematosus10205912715616416815913011110779644229721425

85Henoch-Schoenlein purpura06122727292111171318182322201493290

86Goodpastures Syndrome01334492919202830344668708157278604

87Systemic sclerosis (scleroderma)000059615193248505550301541339

88Haemolytic Uraemic Syndrome (including Moschcowitz Syndrome)60252342624465535564483432303023120702

89Multi-system disease - other (not mentioned above)2128211029325365908185139132121649944

90Tubular necrosis (irreversible) or cortical necrosis (different from 88)2910121613222331566586121154190242221103401434

91Tuberculosis0000127520183044636874482110411

92Gout010043161011184048373425132253

93Nephrocalcinosis and hypercalcaemic nephropathy1017714891929202243353928141297

94Balkan nephropathy00002123238109151342074

95Kidney tumour21626651217285399172221299334205101331620

96Traumatic or surgical loss of kidney4143965102023427380100105944013632

98Other identified renal disorders00000002010132330015

99Chronic renal failure; aetiology uncertain482940587499841321532042133214024825234832681363749

FR080000001017254666114204266280342374236842064

TotalTotal783602104819563170441458087534975412620157941968123809283963113826697144494985212638

FrequencyMissing=292

99999278006894403551983690171062575971160681131252703971291959461280047271222995531145499211063422059603867986822178770858076685952653292269359716312666374616762749318638259_RBREAK_391810233

0-45-910-1415-1920-2425-2930-3435-3940-4445-4950-5454-5960-6464-6970-7475-7980-8485+

-920.09700359350.20126361560.28464247030.49878786550.48247532030.65458401960.7662779141.03121191771.24285000451.79834257592.33209382862.65518021132.9370375853.24313916834.02336085964.24076520373.47496058771.1626273368

0Chronic renal failure; aetiology uncertain0.33412348870.52964109370.87425901592.14572893084.15273400715.36439586766.34694837878.226258706811.079353658815.373210078420.725543541230.05039250944.758149236964.032539738595.3342086212117.1276831917110.253549526363.5694624454

10Glomerulonephritis; histologically NOT examined0.04311270820.09533539690.16265284021.3269639442.47268601673.41660927284.28806024615.43729920226.46772601048.45046414319.967820396413.036414213914.973132786418.680481609320.625333179920.228074732615.45662469415.5131038227

11Focal segmental glomerulosclerosis with nephrotic syndrome in children0.56046520680.55082673740.70144037320.63995424250.37908775170.31132654590.22446524750.35936172890.34341908020.29681382320.23509010370.32278661390.31098045020.40214925690.37391829550.31899561270.30579653170.0375041076

12IgA nephropathy (proven by immunofluorescence, not code 76 and not 85)0.010778177100.15248703770.75288734412.11082952652.85781803663.57596359873.96079122924.53803784555.12440335955.22840390615.89345882194.92961602515.03335198923.7391829553.34007170911.66798108210.7500821527

13Dense deposit disease; membrano-proliferative GN; type II (proven by immunofluorescence and/or electron microscopy)00.05296410940.06099481510.08469982620.07754067650.23948195840.12384289520.14843201850.14717960580.25316473160.19747568710.18742448550.25339147790.22053346340.23930770910.1876444780.13899842350.0375041076

14Membranous nephropathy0.01077817710.01059282190.01016580250.06587764260.2153907680.2953610820.44119031410.57810365080.80131118711.08249747291.26008295581.82218249792.40721904032.95774292153.52978870953.26501391791.91817824440.5625616146

15Membrano-proliferative GN; type I (proven by immunofluorescence and/or electron microscopy - not code 84 or 89)0.03233453120.0847425750.10165802510.33879930490.45662842820.51089484450.72757700930.72653566930.88307763480.89917128791.17545051841.1245469131.50883107311.4269812341.58541357291.53868471991.00078864930.0750082153

16Crescentic (extracapillary) glomerulonephritis (type I, II, III)0.03233453120.0847425750.22364765520.34821039670.44801279750.46299845290.36378850460.39061057490.50695197550.6634661930.93095681061.52022082691.95802505672.81504479813.54474544144.20323630813.08576500191.2001314444

17Focal segmental glomerulosclerosis with nephrotic syndrome in adults0.01077817710.02118564370.10165802510.21645511140.67201919620.83818685430.99074316161.03121191771.45544276851.6150163912.00296768341.9991945122.18838094572.29614253112.10889918662.23296928871.33438486570.9000985833

19Glomerulonephritis; histologically examined, not given above0.23711989520.11652104060.46762691550.74347625231.6628167291.93182113092.23691229442.83583277363.54866382874.02444624994.85225974015.41448513686.05835988137.19976895366.984793765.38539651973.19696374071.1251232291

20Pyelonephritis - cause not specified0.05389088530.04237128750.06099481510.47055459010.89602559491.11758247241.222948591.5077568191.91333487542.74116295554.06235699185.10211099437.405941832110.468853235211.815818137910.78955748726.75532338252.5127752117

21Pyelonephritis associated with neurogenic bladder0.01077817710.04237128750.18298444520.28233275410.42216590530.39913659730.35604832370.37498615190.31888914590.29681382320.3197225410.38526144240.29946265570.35025903020.26922117280.31899561270.05559936940.0750082153

22Pyelonephritis due to congenital obstructive uropathy with/without vesico-ureteric reflux0.86225416430.48726980620.99624864611.34578612761.15449451651.0377551530.91334135210.71872345780.70319144990.47141018980.42316218660.34361155680.39160501130.51890226690.61322600460.71304901650.86179022570.3375369687

23Pyelonephritis due to acquired obstructive uropathy0.02155635410.03177846560.06099481510.1411663770.24985329090.25544742230.29412687610.39061057490.65413158130.92536074291.67384153832.87384211115.06782955858.665667857611.292332524214.504918152411.4534700976.5632188365

24Pyelonephritis due to vesico-ureteric reflux without obstruction0.16167265580.21185643750.88442481841.75987416691.71451051331.48478814191.5402960091.18745614761.05478717491.14360620120.95916762310.88506007040.93294135050.94699663711.03201449560.80687125560.44479495520.2250246458

25Pyelonephritis due to urolithiasis000.03049740750.07528873440.06892504580.12772371110.25542597130.35154951740.67866151561.14360620121.82429920462.36363101163.37471377424.33283392886.20704370536.56755673144.42014986751.5376684131

29Pyelonephritis due to other cause0.021556354100.0203316050.0470554590.12923446080.11175824720.25542597130.22655413340.50695197550.58489782810.78049914420.8746475991.38213533411.81615793422.39307709122.34555597551.64018139740.7875862604

30Interstitial nephritis (not pyelonephritis) due to other cause, or unspecified (not mentioned above)0.03233453120.13770668440.12198963010.16939965240.26708455230.33527474170.34056796180.54685480480.60507171270.92536074291.24127574751.68682036952.10775638452.7761271283.18578387773.18995612672.80776815491.0126109062

31Nephropathy (interstitial) due to analgesic drugs0000.00941109180.00861563070.04789639170.1548036190.08593432650.28618256680.51505928141.01558924791.64517048392.55695036813.32097450833.58961563683.52771618711.8625788750.6000657222

32Nephropathy (interstitial) due to cis-platinum00000.02584689220.02394819580.03096072380.02343663450.02452993430.06110872830.12224685390.18742448550.11517794450.25945113350.26922117280.18764447800

33Nephropathy (interstitial) due to cyclosporin A0000.01882218360.12061883010.07184458750.09288217140.1406198070.32706579070.40157164320.59242706130.83299771331.28999297850.94699663710.53844234550.31899561270.02779968470

34Lead induced nephropathy (interstitial)000000.007982731900.0156244230.02452993430.01745963670.00940360410.010412471400.01297255670.0598269273000

39Drug induced nephropathy (interstitial) not mentioned above0.01077817710.02118564370.040663210.08469982620.12061883010.11974097920.13158307610.28123961390.32706579070.54124873640.92155320651.68682036951.85436490662.30911508782.12385591852.19544039311.44558360450.6750739375

40Cystic kidney disease - type unspecified0.05389088530.0847425750.05082901260.0941109180.07754067650.10377551530.16254379990.35154951740.68683816041.16106583781.39173341381.9471321552.2459699182.07560906772.34820689582.43937821452.11277603730.5625616146

41Polycystic kidneys; adult type (dominant)0.04311270820.05296410940.01016580250.06587764260.13785009150.51089484451.24616913294.31234074669.174195428215.835890449920.057887646819.960707706118.071419493816.384339078116.811366565812.96623343258.22870667172.9253203957

42Polycystic kidneys; infantile (recessive)0.3987925510.22244925940.20331605020.17881074420.12061883010.11974097920.07740180950.07030990350.08994309240.104757820.20687929120.15618707130.16124912230.09080789670.14956731820.20640892580.05559936940

43Medullary cystic disease; including nephronophthisis0.05389088530.43430569680.84376160840.58348769170.24123766020.25544742230.18576434280.23436634490.1390029610.09602800160.20687929120.17701201410.10366015010.09080789670.07478365910.15011558240.16679810820.0750082153

49Cystic kidney disease - other specified type0.021556354100.03049740750.10352200980.07754067650.08781005140.03870090470.02343663450.08176644770.14840691160.1880720830.15618707130.14973132790.22053346340.23930770910.16888003020.2779968470.0750082153

50Hereditary/Familial nephropathy - type unspecified0.09700359350.05296410940.08132642010.15998856060.2153907680.18360283480.20898488560.14843201850.30253585640.29681382320.26330091610.2498993140.3570516280.36323158680.35896156370.1876444780.13899842350

51Hereditary nephritis with nerve deafness (Alports Syndrome)00.04237128750.19315024770.82817607861.0166444250.68651494730.49537158080.45310826690.43336217260.36665236980.30091533270.26031178540.39160501130.23350602010.28417790460.26270226930.11119873880

52Cystinosis0.02155635410.27541336870.24397926030.05646655080.03446252290.02394819580.00774018090.05468548050.04905986860.06110872830.04701802070.05206235710.03455338340.02594511330.04487019550.01876444780.02779968470.0375041076

53Primary oxalosis0.1940071870.09533539690.06099481510.0470554590.077540676500.02322054280.00781221150.04088322380.052378910.00940360410.07288729990.03455338340.01297255670.05982692730.07505779120.05559936940.0375041076

54Fabrys disease0000.00941109180.00861563070.03193092780.03096072380.0624976920.04088322380.09602800160.12224685390.07288729990.01151779450.01297255670.014956731800.02779968470

59Hereditary nephropathy - other specified type1.26104671530.23304208120.13215543260.21645511140.25846892160.19956829860.11610271420.17186865290.17170954010.11348763830.16926487470.15618707130.18428471120.09080789670.14956731820.075057791200

60Renal hypoplasia (congenital) - type unspecified0.50657432150.40252723120.66077716320.52702114090.2757001830.31132654590.19350452370.34373730590.26982927730.21824545820.2538973120.40608638530.24187368350.37620414350.55339907730.35652450830.33359621640.0375041076

61Oligomeganephronic hypoplasia00.04237128750.06099481510.0941109180.02584689220.01596546390.00774018090.00781221150000.02082494280.04607117780.01297255670.04487019550.018764447800

63Congenital renal dysplasia with or without urinary tract malformation1.21793400710.68853342180.92508802850.65877642610.46524405890.25544742230.23220542850.17186865290.22894605350.14840691160.15986127050.08329977130.11517794450.12972556670.10469712270.07505779120.11119873880.0750082153

66Syndrome of agenesis of abdominal muscles (Prune Belly)0.09700359350.06355693120.06099481510.02823327540.03446252290.023948195800.007812211500.0087298183000.01151779450.038917670.0448701955000

70Renal vascular disease - type unspecified00.03177846560.040663210.06587764260.05169378430.19158556670.20898488560.44529605540.67866151561.5102585712.83988845265.74768422219.790125283418.69345416631.663401263140.512442808535.11100177816.3517909299

71Renal vascular disease due to malignant hypertension00.021185643700.12234419340.43939716670.63861855571.05266460921.5390056651.68438882192.19991421912.52956951572.25950629752.63757492933.56745308514.05327432324.07188517343.22476342541.650180736

72Renal vascular disease due to hypertension0.01077817710.01059282190.03049740750.0941109180.34462522880.75037680291.11458605671.78118422143.22159803815.14186299619.159110439715.191795797223.473265091338.398767752455.773652957174.250919959975.225946802440.9169814324

73Renal vascular disease due to polyarteritis0.01077817710.02118564370.06099481510.02823327540.07754067650.07982731950.10062235230.1093709610.26165263250.27935418650.32912614520.69763558490.90990576161.45292634742.27342323672.19544039311.83477919030.3375369687

74Wegeners granulomatosis0.010778177100.13215543260.16939965240.23262202940.20755103060.19350452370.35936172890.44971546220.73330473971.09081808111.54104576972.45329021812.76315457143.64944256413.75288956082.47417193840.7125780451

75Cholesterolembolia0000.018822183600.01596546390.02322054280.05468548050.01635328950.03491927330.19747568710.29154919970.70258546151.41400867742.03411552752.94601830522.25177446080.6375698298

76Glomerulonephritis related to liver cirrhosis0000.0094110918000.01548036190.0156244230.02452993430.0261894550.12224685390.09371224280.13821353340.12972556670.08974039090.15011558240.02779968470

78Cryoglobulinaemic glomerulonephritis00.010592821900.00941109180.008615630700.01548036190.0156244230.03270657910.052378910.0658252290.08329977130.09214235560.02594511330.11965385460.15011558240.13899842350.0375041076

79Renal vascular disease - due to other cause (not given above and not code 84-88)0.05389088530.02118564370.03049740750.05646655080.05169378430.04789639170.10836253330.1406198070.26982927730.64600655640.97797483131.65558295533.01766214625.46144635947.92706786468.800526027.08891959891.8752053819

80Diabetes glomerulosclerosis or diabetic nephropathy - Type I0.032334531200.0203316050.05646655080.93910374854.48629535368.854767006411.663631765714.039299064316.019216634817.65056498521.814127618324.936024986627.644518270427.10159805820.265603628210.25808365492.5127752117

81Diabetes glomerulosclerosis or diabetic nephropathy - Type II0.0215563541000.01882218360.01723126140.06386185560.34830814270.70309903482.07686777075.622003004313.992562971628.196972596845.207343220564.253073201976.757947700769.203283500742.950512863911.7012815829

82Myelomatosis/light chain deposit disease0000.009411091800.03991365970.01548036190.11718317250.40883223831.01265892621.87131722542.92590446815.25211426977.52408287049.901356464910.73326414387.45031553.5253861179

83Amyloid000.03049740750.08469982620.26708455230.37518840150.6192144760.70309903480.8421944111.78961275762.25686499543.75890218155.60916589777.4203024177.04462068736.66137897042.77996847020.7875862604

84Lupus erythematosus0.010778177100.20331605020.55525441631.09418510151.24530618361.26938967571.31245153161.30008651791.13487638281.04380006041.11413444160.90990576160.83024362710.62818273640.54416898630.19459779290.0750082153

85Henoch-Schoenlein purpura00.06355693120.12198963010.25409947860.23262202940.23149922640.16254379990.08593432650.1390029610.11348763830.16926487470.18742448550.26490927240.28539624680.29913463640.26270226930.25019716230.1125123229

86Goodpastures Syndrome00.01059282190.03049740750.31997712130.42216590530.23149922640.1470634380.15624422990.22894605350.26189454990.3197225410.47897368520.78321002270.90807896711.21149527741.06957352480.75059148690.3000328611

87Systemic sclerosis (scleroderma)00000.04307815360.07184458750.04644108570.11718317250.15535625060.27935418650.45137299910.52062357080.63347869480.64862783370.44870195460.28146671710.11119873880.0375041076

88Haemolytic Uraemic Syndrome (including Moschcowitz Syndrome)0.64669062320.26482054680.23381345770.39526585570.53416910470.35124020560.50311176170.41404720940.44971546220.55870837310.45137299910.35402402820.36856942240.38917670020.44870195460.43158229950.33359621640

89Multi-system disease - other (not mentioned above)0.02155635410.01059282190.0203316050.07528873440.18092824510.07982731950.22446524750.24999076790.43336217260.56743819140.84632437330.84341018480.97901252831.80318537761.97428860032.27049818431.77917982090.3375369687

90Tubular necrosis (irreversible) or cortical necrosis (different from 88)0.31256713460.10592821870.12198963010.15057746880.11200319940.17562010280.17802416180.24217855640.45789210690.56743819140.80870995671.25990904141.77374034552.46478576793.61952910054.14694296472.86336752431.5001643055

91Tuberculosis00000.00861563070.01596546390.05418126660.03906105750.16353289530.15713672990.28210812440.45814874230.72562105040.88213385381.10679815470.90069349460.58379337870.3750410764

92Gout00.0105928219000.03446252290.02394819580.00774018090.0468732690.08176644770.09602800160.16926487470.41649885670.55285413370.47998459690.50852888190.46911119510.36139590110.0750082153

93Nephrocalcinosis and hypercalcaemic nephropathy0.010778177100.01016580250.06587764260.0603094150.11175824720.06192144760.07030990350.15535625060.25316473160.1880720830.22907437120.49526516140.45403948360.5833125410.52540453850.38919558580.0375041076

94Balkan nephropathy00000.01723126140.00798273190.01548036190.02343663450.01635328950.0261894550.07522883320.10412471420.10366015010.19458835010.19443751370.07505779120.05559936940

95Kidney tumour0.22634171810.06355693120.0203316050.05646655080.05169378430.03991365970.09288217140.13280759550.22894605350.46268037150.93095681061.79094508372.54543257373.87879444534.99554842793.84671179982.80776815491.237635552

96Traumatic or surgical loss of kidney0.04311270820.01059282190.040663210.02823327540.07754067650.04789639170.03870090470.0781221150.16353289530.20078582160.39495137420.76011041340.92142355611.29725566731.57045684111.76385809361.11198738810.4875533993

98Other identified renal disorders00000000.01562442300.008729818300.01041247140.03455338340.02594511330.04487019550.056293343400

99Chronic renal failure; aetiology uncertain0.51735249860.30719183430.40663210040.54584332450.63755667330.79029046260.65017519981.03121191771.25102664931.78088293922.00296768343.34240332484.63015336936.25277231647.82237074199.06322828937.45031555.1005586387

FR080000000.07982731950.13158307610.19530528740.37612565930.57616800981.07201087282.1241441693.0637333243.63231586855.11520228257.01790347866.56072558963.1503450415

TotalTotal8.43931263346.37687876810.653761031318.408095564227.311549383135.235778808944.954970955558.857201421979.7549930538110.1703073195148.5205239068204.9278499551274.227168086368.3687192897465.7227154138500.9544630198401.6776442525186.9579765724

Cystic KD

0-45-910-1415-1920-2425-2930-3435-3940-4445-4950-5454-5960-6464-6970-7475-7980-8485+

unknown5851091321458413314818719516015713076151401

dominant4517166416155211221814213319171569126311246912967812817

recessive37212019141510911122215147101120249

medullary54183622832243017112217975862409

other2031191153101720151317169102173

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

unknown

dominant

recessive

medullary

other

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

unknown

recessive

medullary

other

000

000

000

000

000

000

000

000

000

000

000

000

000

000

000

000

000

000

recessive

medullary

other

Cystic KD

0-45-910-1415-1920-2425-2930-3435-3940-4445-4950-5454-5960-6464-6970-7475-7980-8485+

unknown0.05389088530.0847425750.05082901260.0941109180.07754067650.10377551530.16254379990.35154951740.68683816041.16106583781.39173341381.9471321552.2459699182.07560906772.34820689582.43937821452.11277603730.5625616146

dominant0.04311270820.05296410940.01016580250.06587764260.13785009150.51089484451.24616913294.31234074669.174195428215.835890449920.057887646819.960707706118.071419493816.384339078116.811366565812.96623343258.22870667172.9253203957

recessive0.3987925510.22244925940.20331605020.17881074420.12061883010.11974097920.07740180950.07030990350.08994309240.104757820.20687929120.15618707130.16124912230.09080789670.14956731820.20640892580.05559936940

medullary0.05389088530.43430569680.84376160840.58348769170.24123766020.25544742230.18576434280.23436634490.1390029610.09602800160.20687929120.17701201410.10366015010.09080789670.07478365910.15011558240.16679810820.0750082153

other0.021556354100.03049740750.10352200980.07754067650.08781005140.03870090470.02343663450.08176644770.14840691160.1880720830.15618707130.14973132790.22053346340.23930770910.16888003020.2779968470.0750082153

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

00000

unknown

dominant

recessive

medullary

other

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

0000

unknown

recessive

medullary

other

000

000

000

000

000

000

000

000

000

000

000

000

000

000

000

000

000

000

recessive

medullary

other

0-45-910-1415-1920-2425-2930-3435-3940-4445-4950-5454-5960-6464-6970-7475-7980-8485+

21Pyelonephritis associated with neurogenic bladderPN_neurogenic14183049504648393434372627181722482

22Pyelonephritis due to congenital obstructive uropathy with/without vesico-ureteric refluxPN_obstructive8046981431341301189286544533344041383191252

24Pyelonephritis due to vesico-ureteric reflux without obstructionPN_refluxive15208718719918619915212913110285817369431661780

60Renal hypoplasia (congenital) - type unspecifiedHypodys_congenital47386556323925443325273921293719121589

61Oligomeganephronic hypoplasiaOligomeganephronia046103211000241310038

63Congenital renal dysplasia with or without urinary tract malformationDysplasia__CAKUT11365917054323022281717810107442584

66Syndrome of agenesis of abdominal muscles (Prune Belly)Prune_belly96634301010013300040

0000000

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PN_neurogenic

PN_obstructive

PN_refluxive

Hypodys_congenital

Oligomeganephronia

Dysplasia__CAKUT

Prune_belly

0-45-910-1415-1920-2425-2930-3435-3940-4445-4950-5454-5960-6464-6970-7475-7980-8485+

21PN_neurogenic0.01077817710.04237128750.18298444520.28233275410.42216590530.39913659730.35604832370.37498615190.31888914590.29681382320.3197225410.38526144240.29946265570.35025903020.26922117280.31899561270.05559936940.0750082153

22PN_obstructive0.86225416430.48726980620.99624864611.34578612761.15449451651.0377551530.91334135210.71872345780.70319144990.47141018980.42316218660.34361155680.39160501130.51890226690.61322600460.71304901650.86179022570.3375369687

24PN_refluxive0.16167265580.21185643750.88442481841.75987416691.71451051331.48478814191.5402960091.18745614761.05478717491.14360620120.95916762310.88506007040.93294135050.94699663711.03201449560.80687125560.44479495520.2250246458

60Hypodys_congenital0.50657432150.40252723120.66077716320.52702114090.2757001830.31132654590.19350452370.34373730590.26982927730.21824545820.2538973120.40608638530.24187368350.37620414350.55339907730.35652450830.33359621640.0375041076

61Oligomeganephronia00.04237128750.06099481510.0941109180.02584689220.01596546390.00774018090.00781221150000.02082494280.04607117780.01297255670.04487019550.018764447800

63Dysplasia__CAKUT1.21793400710.68853342180.92508802850.65877642610.46524405890.25544742230.23220542850.17186865290.22894605350.14840691160.15986127050.08329977130.11517794450.12972556670.10469712270.07505779120.11119873880.0750082153

66Prune_belly0.09700359350.06355693120.06099481510.02823327540.03446252290.023948195800.007812211500.0087298183000.01151779450.038917670.0448701955000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

0000000

PN_neurogenic

PN_obstructive

PN_refluxive

Hypodys_congenital

Oligomeganephronia

Dysplasia__CAKUT

Prune_belly

*

CAKUT Diagnosis CodesERA-EDTA Annual Report 2009Pyelonephritis associated with neurogenic bladderPyelonephritis due to congenital obstructive uropathy w/o VUR Pyelonephritis due to VUR without obstruction

Renal hypoplasia (congenital) unspecifiedOligomeganephronic hypoplasiaCongenital renal dysplasia w/o urinary tract malformationSyndrome of agenesis of abdominal muscles (prune belly)

CODE212224

60616266

*

Characteristics of RRT Patients in the ERA-EDTA Registry

ALLCAKUTPrevalence 2009 (cases pmp)947.544.9Incidence (no of patients) 1.1.1999-31.12.2009212 9304 765Gender (% male)61.561.5CAKUT diagnoses (%)2.2100Neurogenic PN5.6Obstructive PN22.9Refluxive PN41.9Hypoplasia17.3Dysplasia12.3

*

Caratteristiche deio pazienti pediatrici del RRDTL

ALLCAKUTIncidence 2010 (cases pmp)16742.9Incidence (no of patients) 1.1.1995-31.12.201012 57654Gender (% male)62.664.8CAKUT diagnoses (%)1.5100Neurogenic PN11.9Refluxive PN64.4Hypoplasia22.7Prune Belly1.0

*

Age Distribution at Start of RRTPercent of patients starting RRT at given age groups

CAKUT

Other diagnoses

*

*

63%37%

*

*

*

Anno di incidenza. RRDTL 1995-2010

*

Et allingresso, per sesso. RRDTL 1995-2010

*

Metodica dialitica per anno. RRDTL, 1995-2010

*

Et allingresso per metodica dialitica. RRDTL 1995-2010

MetodicaMediaDSMedianaRIQHD10,95,0129-15DP4,75,040-8Totale9,55,6115-14

*

Centro dialisi allingresso. RRDTL 1995-2010

*

Paese di nascita. RRDTL 1995-2010

*

Paese di nascita per anno. RRDTL 1995-2010

*

Tipologia di accesso vascolare per anno. RRDTL, 2002-2010

*

Incidenza cumulativa di trapianto per nefropatia. RRDTL, 1995-2010Mesi Cakut Glomerulopathies Hereditary Other 12 0.28 0.29 0.08 0.08 24 0.52 0.45 0.39 0.17 36 0.74 0.66 0.39 0.41 48 0.81 0.83 0.39 0.41 60 0.88 0.87 0.51 0.41

*

Incidenza cumulativa di trapianto per quartili di et allingresso. RRDTL, 1995-2010Mesi 0-5 6-11 12-14 15-17 12 0.11 0.31 0.30 0.19 24 0.16 0.48 0.45 0.65 36 0.46 0.72 0.65 0.73 48 0.52 0.77 0.71 0.84 60 0.66 0.77 0.83 0.88

*

Dialysis Unit, Bambino Ges Pediatric HospitalRoma, Italy.Doctor:S. Picca

Headnurse:Tancredi

Nurses:N. AvariV. BandinuD. CiulloE. IacoellaP. LozziF. PaolucciC. Pia

TransplantCoordinators:S. TeseiI. Pannacci

*