A. Zurowska has documented that she has no relevant financial relationships to disclose or conflict of interest to resolve.

Transcription

1 A. Zurowska has documented that she has no relevant financial relationships to disclose or conflict of interest to resolve.

2 PERİTONEAL DİALYSİS İN İNFANTS BELOW 1 YEAR OF AGE Aleksandra Żurowska Department Paediatrics, Nephrology & Hypertension Medical University of Gdansk Gdansk, Poland

3 Lecture overview ESPN Gen Med BRUSSELS 2015 Demography of infant dialysis Outcome Technical aspects of infant PD Major clinical issues Ethical issues 3

4 Demography

5 Choice of initial mode of RRT in infants and neonates ESPN Gen BRUSSELS Med All infants 0-1yrs PD HD 0 All neonates 0-1 month PD HD 857/927 ( 93%) children 0-1years age initiate RRT with peritoneal dialysis NAPRTCS Registry 2011 Annual Report 242/264 (93%) neonates (0-1 month) initiate RRT with peritoneal dialysis. ESPN/ERA-EDTA IPPN, ANZDATA, Japanese Registries Kidney Int 2014,86:

6 NAPRTCS Annual Report 2011 (data from 7039 children starting RRT) ESPN Brussels 2015 Gen Med 700 Age at index dialysis year age groups Peak number of patients in 1st year life among 7039 children initiating dialysis NAPRTCS 2011 Annual Report Increasing proportion of children 0-24months initiating dialysis NAPRTCS 2011 Annual Report 6

7 Neonatal peritoneal dialysis Proportion of neonates among infants initiating dialysis (33%) Week of age at dialysis initiation in neonates : 53% in 1st week of life infants 0-12 months neonates 0-1 month Coulthard et al.arch Dis Child 2002;87:511 BAPN: [ 29%] 31 neonates ( 1 month age ) / 106 infants week 1 week 2 week 3 week 4 Van Stralen et al. Kid Int 2014;86:168 ESPN/ ERA-EDTA Registry/ IPPN : Carey et al.. Pediatrics 2007;119:468 NAPRTCS : [35%] 193 neonates ( 1 month age ) /542 infants

8 Variability of infant RRT incidence rates influenced by non-medical factors ESPN Brussels 2015 Gen Med 5 infants in dialysis poulatio NAPRTCS ESPN ERA EDTA RRT incidence varied more between countries in patient ages 0 4 (IQR ) Countries with a higher RRT incidence were treating children at a younger age This effect was largely explained by country GDP per capita. National GDP had a stronger effect on the RRT incidence in the youngest patients (ages 0 4, 3.33 pmc/sd, P < ) Paediatric RRT incidence per country for the period Nicholas C. Chesnaye et al. Nephrol. Dial. Transplant. 2015;30: A trend in the association between neonatal mortality and RRT incidence in the youngest patient group was observed after adjustment for GDP per capita ( 1.84 pmc/sd, 95% CI 2.8 to 0.9, P = ) and public health expenditure ( 1.1 pmc/sd, 95% CI 2.4 to 0.25, P = 0.10) 8

9 Causes of CKD 5 in infants 9

10 Causes of CKD 5 in infants ESPN Brussels 2015 Gen Med Congenital and hereditary diseases 80-90% CAKUT- Congenital Anomalies Kidney & Urinary Tract: Renal hypo/dysplasia with or without VUR or obstruction Isolated or syndromic: VATER, CHARGE sequence, Prune Belly, Alagille, Jeune, Senior Loken, Caroli, Allagille, Pierre Robin, Potter, chromosomal abnormalities Congenital Nephrotic Syndrome- FCNS, DMS, Pierson, Denys Drash Hereditary nephropathies: ARPKD, infantile oxalosis Acquired causes 10-20% Cortical necrosis, HUS, Renal vascular thrombosis, ACE fetopathy 10

11 Outcome for children initiating dialysis in 1st year life

12 Survival of children starting RRT in infancy ESPN Brussels 2015 Gen Med Probabilities and HR of death at 4 yrs by age group 4 year survival %(95%CI) HR (95%CI) Overall Age group (yrs) ( ) 87.1 ( ) 95.3 ( ) 96.2 ( ) 96.3 ( ) 4.4 ( ) 1.4 ( ) 1.1 ( ) 1 ESPN/ERA-EDTA Registry N.Chesnaye et al. Pediatr Nephrol 2014;29:2403 Survival of neonates starting RRT in ESPN/ERA-EDTA Registry K.Van Stralen et al. Kid.Int.2014;86:168

13 Survival of children starting RRT in infancy ESPN Gen BRUSSELS Med 2015 NAPRTCS Annual Report % survival of children starting dialysis in first 24 months life ( 0-1 years) 2011 NAPRTCS Report Similar proportion of children 0-1 yrs changing dialysis mode due to excessive infection, access failure, fewer by choice than in older age groups 13

14 Causes of death 2014 INC Research, LLC 14

15 Cause of death by age groups 2011 NAPRTCS Annual Report ESPN Gen BRUSSELS Med 2015 Causes of death in children initiating dialysis 0-1 years of age Causes of death in children initiating dialysis 2-16 years of age Risk factors for mortality: comorbidities severe developmental delay, pulmonary hypoplasia, congenital heart disease 15

16 Technical issues

17 Catheters and their insertion ESPN Gen BRUSSELS Med 2015 Paediatric catheters are used for infants up to 10kg o Single cuff catheters for children < 3kg o Double cuff catheters in infants> 3kg (external cuff > 2cm from exit site) o Coiled catheters (shorter and less dislodgement complications) and straight catheters are used Catheter implantation o Dedicated surgeon! o Surgical procedure ( though laparascopic placement has been described in infants) o Partial omentectomy recommended o Paramedian or lateral entry into peritoneum with fixation of internal cuff in rectus muscle o Exit site lateral sometimes at umbilicus level outside diaper area, safe distance from ostomies on abdominal wall. Presternal exit site used by some centres o Immobilisation by dressing advocated to allow healing and prevent pulling and tearing o A delay of 2-3 weeks is beneficial o Elective herniotomy on contralateral site if evidence of hernia Zurowska A, Fischbach M, Watson A, Edefonti A, Stefanidis C for EPDWG Clinical practice recommendations for the care of infants with CKD5. Pediatr Nephrol 2013, 28:

18 Tubing and cyclers ESPN Gen BRUSSELS Med 2015 Paediatric tubing to allow low fill volumes Appropriate cyclers to allow low fill volumes and paediatric software automated PD can be performed in infants with fill volumes> 100ml fill volumes < 60ml associated with frequent alarms Infants < 2-3kg with fill volumes < 100ml need manual dialysis with commercially available 2 chamber sets with controlled temperature. inflow and outflow volumes can be measured with accuracy 1ml easily performed in hospital setting Zurowska A, Fischbach M, Watson A, Edefonti A, Stefanidis C for EPDWG Clinical practice recommendations for the care of infants with CKD5. Pediatr Nephrol 2013, 28:

19 Dialysis prescription ESPN Gen BRUSSELS Med 2015 Infants require: Lower fill volumes ( ml/m 2 ) last (day) fill volume ½ night fill volume IPP < 10cm H 2 O shorter dwell times ( 30-40min) More frequent exchanges (12-16) Longer duration dialysis (10 16hrs) Zurowska A, Fischbach M, Watson A, Edefonti A, Stefanidis C for EPDWG Clinical practice recommendations for the care of infants with CKD5. Pediatr Nephrol 2013, 28:

20 Dialysis fluids ESPN Gen BRUSSELS Med 2015 Infants should receive biocompatible fluids, to allow best possible long term preservation of peritoneal membrane function o Fluids with neutral ph preserve membrane function and children show lower IPP o Frequent cycles induce alkalosis and lower buffer content is then recommended o Lowest glucose possible though anuric infants will need higher glucose for adequate UF and low glucose fluid may lead to absorption of fluid in polyuric infants Calcium content ( 1.25 or 1.75mEq/l) individualized to achieve growth and Ca, P and PTH targets Icodextrin to be used with caution due to reports of hyponatremia, rebound hypoglycemia in daytime No evidence for beneficial use of aminoacid solutions 20

21 Major clinical issues

22 Comorbidities ESPN Gen BRUSSELS Med 2015 Comorbidities are frequently present in both isolated and syndomic forms of CAKUT: o Cardiac defects o Lung hypoplasia in oligohydramnios o Liver and gut disease o Central nervous system involvement o Ear defects o Ocular defects o Osseous defects o Reproductive system defects Many children will need a multiprofessional team approach ( nephrologist, urology surgeon, neonatologist, cardiologist, neurologist, orthopedist, ENT specialist) Many children will require surgical interventions during early childhood 22

23 Nutrition and growth 23

24 Nutrition KDOQI Guidelines Gastrostomy in infant with CKD Coleman JE, Watson AR, Rance CH, Moore E: Gastrostomy buttons for nutritional support on chronic dialysis. Nephrol Dial Transplant 13: , 1998 Rees L, Shaw V: Nutrition in children with CRF and on dialysis. Pediatr Nephrol 22: , 2007 Enteral feeding also enables adequate dietary intake and additionally the delivery of numerous oral medications prescribed, among them NaCl supplementation, irrespective of their volume and taste without the cooperation of the infant Rees L, Brandt M: Tube feeding in children with CKD: Technical and practical issues. Pediatr Nephrol 25: , 2010 KDOQI Work Group: KDOQI Clinical Practice Guideline for Nutrition in Children with Chronic Kidney Disease: 2008 Update. Am J Kidney Dis 53: S11 104, 2009 Zurowska A, Fischbach M, Watson A, Edefonti A, for EPDWG.Clinical practice guidelines for the care of infants with CKD5. Pediatr Nephrol 28: , 2013

25 L Rees, M Azocar, D Borzych, A Watson,A Buscher,A Edefonti, I Bilge,D Askenazi, GLeozappa, C Gonzales, K van Hoeck, D Secker, A Zurowska, KRonnholm, A Bouts,, HStewart,GAriceta, B Ranchin, B Warady, F Schaefer,for the IPPN registry Growth in Very Young Children Undergoing Chronic Peritoneal Dialysis J Am Soc Nephrol 22: , 2011 o Both nasogastric tube (NGT) and gastrostomy (GS) feeding improve nutritional status, but only GS feeding associated with stabilized linear growth o Minor regional variation in linear growth despite major differences in nutritional control in infants. o Multivariate Analysis of Factors Associated with Growth and Weight Gain: o the use of gastrostomy feeding, o biocompatible dialysis fluid, o growth hormone therapy o associated with improved linear growth.

26 Height z scoresand BMI up to Change in height Z score and weight 36 months age in neonates z-score in age groups initiating peritoneal dialysis NAPRTCS Annual report 2011

27 Ethical issues

28 Management dilemmas in witholding or withdrawal of RRT in neonates and infants. ESPN Gen BRUSSELS Med

29 Attitudes to management of infants with end stage renal disease infants. ESPN Gen BRUSSELS Med 2015 Among 270 survey responders from Canada, Germany, Japan, UK, USA, RRT is offered by 100% to some children 1-12 months age by 98% to some less than 1 month of age ( 93% in 1998) Among responding nephrologists 30% offer RRT to all neonates (41% in 1998) 50% to all infants 1-12 months 50% indicated that parents can never refuse RRT for infants 1-12 months 27% indicated that parents can never refuse RRT for neonates < 1 month The most influencing factor in rejecting RRT for infants was co-existing abnormalities Nurses were more likely to believe parents had the right to refuse RRT for infants 29

30 Management dilemmas in witholding or withdrawal of RRT in neonates and infants. ESPN Gen BRUSSELS Med 2015 The outcomes of infant RRT are seen as good enough for the treatment to be strongly recommended and even considered the standard of care. However the burdens of therapy are high enough, and the chances of a bad outcome high enough, that the treatment continues to be viewed as legally and ethically optional. Reaching the right decision in any particular case requires prudent and discerning judgement. J.D. Lantos, B.A. Warady. The evolving ethics of infant dialysis. Pediatr Nephrol 2013;28:

31 Saturday Gold Hall Plenary Symposium: Should we always dialyze and transplant mentally disabled patients? Panel: A. Edefonti (Milan, Italy), L. Willem (Leuven, Belgium), P. Cochat (Lyon, France), E. Verrina (Genova, Italy ), J. Groothoff (Amsterdam, The Netherlands), C.Van Geet (Leuven, Belgium), E. Levtchenko (Leuven, Belgium)

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34 Borzych D, Rees L, Ha IS, Chua A, Valles PG, Lipka M, Zambrano P, Ahlenstiel T, Bakkaloglu SA, Spizzirri AP, Lopez L, Ozaltin F, PrintzaN, Hari P, Klaus G, Bak M, Vogel A, AricetaG, Yap HK, Warady BA, Schaefer F; International Pediatric PD Network (IPPN). The bone and mineral disorder of children undergoing chronic peritoneal dialysis. Kidney Int Dec;78(12):

35 Borzych D, Rees L, Ha IS, Chua A, Valles PG, Lipka M, Zambrano P, Ahlenstiel T, Bakkaloglu SA, Spizzirri AP, Lopez L, Ozaltin F, PrintzaN, Hari P, Klaus G, Bak M, Vogel A, AricetaG, Yap HK, Warady BA, Schaefer F; International Pediatric PD Network (IPPN). The bone and mineral disorder of children undergoing chronic peritoneal dialysis. Kidney Int Dec;78(12):

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37 Kt/V in age groups

38 Recommended literature

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41 ESPN Gen BRUSSELS Med

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45 Survival of neonatal dialysis

46 Whereas both nasogastric tube (NGT) and gastrostomy (GS) feeding improve nutritional status, only GS feeding associates with stabilized linear growth in young infants undergoing CPD. The data points represent mean estimates at key time points of postnatal d... Lesley Rees et al. JASN 2011;22: by American Society of Nephrology

47 Minor regional variation in linear growth despite major differences in nutritional control in infants undergoing CPD. Course of BMI SDS and length SDS by region in 67 European, 27 North American, 33 Latin American, and 20 Turkish children. Lesley Rees et al. JASN 2011;22: by American Society of Nephrology

48 Fig.1 Length SDS and BMI SDS in infants Followed > 6 months in IPPN registry on a constant feeding modality. Fig 2. Length SDS in infants followed > 6 mo in IPPN registry, stratified by type of PD fluid.

49 Proportion of infants on PD and incidence of infant RRT ESPN Brussels 2015 Gen Med % infants in dialysis population NAPRTCS % ESPN ERA EDTA Age- incidence rates for ESRD in the young in Taiwan (n=8,104,970) from 1998 to

50 Peritonitis rates 50