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Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases

Background Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children. Methods We conducted a retrospective case note review across members of the E... Full description

Journal Title: Pediatric nephrology (Berlin West), 2018-10-29, Vol.34 (4), p.649-655
Main Author: Dufek, Stephanie
Other Authors: Ylinen, Elisa , Trautmann, Agnes , Alpay, Harika , Ariceta, Gema , Aufricht, Christoph , Bacchetta, Justine , Bakkaloglu, Sevcan , Bayazit, Aysun , Caliskan, Salim , do Sameiro Faria, Maria , Dursun, Ismail , Ekim, Mesiha , Jankauskiene, Augustina , Klaus, Günter , Paglialonga, Fabio , Pasini, Andrea , Printza, Nikoleta , Conti, Valerie Said , Schmitt, Claus Peter , Stefanidis, Constantinos , Verrina, Enrico , Vidal, Enrico , Webb, Hazel , Zampetoglou, Argyroula , Edefonti, Alberto , Holtta, Tuula , Shroff, Rukshana
Format: Electronic Article Electronic Article
Language: English
Subjects:
Age
Publisher: Berlin/Heidelberg: Springer Berlin Heidelberg
ID: ISSN: 0931-041X
Link: https://www.ncbi.nlm.nih.gov/pubmed/30374605
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title: Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases
format: Article
creator:
  • Dufek, Stephanie
  • Ylinen, Elisa
  • Trautmann, Agnes
  • Alpay, Harika
  • Ariceta, Gema
  • Aufricht, Christoph
  • Bacchetta, Justine
  • Bakkaloglu, Sevcan
  • Bayazit, Aysun
  • Caliskan, Salim
  • do Sameiro Faria, Maria
  • Dursun, Ismail
  • Ekim, Mesiha
  • Jankauskiene, Augustina
  • Klaus, Günter
  • Paglialonga, Fabio
  • Pasini, Andrea
  • Printza, Nikoleta
  • Conti, Valerie Said
  • Schmitt, Claus Peter
  • Stefanidis, Constantinos
  • Verrina, Enrico
  • Vidal, Enrico
  • Webb, Hazel
  • Zampetoglou, Argyroula
  • Edefonti, Alberto
  • Holtta, Tuula
  • Shroff, Rukshana
subjects:
  • 3123 Gynaecology
  • Age
  • Age Factors
  • Birth defects
  • Care and treatment
  • Central nervous system
  • Child, Preschool
  • Children
  • Complications
  • Congenital diseases
  • Congenital nephrotic syndrome
  • Disease Progression
  • Europe
  • Female
  • Health aspects
  • Hemodialysis
  • Humans
  • Infant
  • Infant dialysis
  • Infant, Newborn
  • Infants
  • Kidney diseases
  • Kidney Failure, Chronic - diagnosis
  • Kidney Failure, Chronic - etiology
  • Kidney Failure, Chronic - mortality
  • Kidney Failure, Chronic - therapy
  • Kidney transplantation
  • Kidney Transplantation - adverse effects
  • Kidney Transplantation - mortality
  • Male
  • Medicine
  • Medicine & Public Health
  • Nephrology
  • Nephrotic syndrome
  • Nephrotic Syndrome - congenital
  • Nephrotic Syndrome - diagnosis
  • Nephrotic Syndrome - mortality
  • Nephrotic Syndrome - therapy
  • Original Article
  • Outcome
  • paediatrics
  • Patient outcomes
  • Pediatrics
  • Peritoneal Dialysis
  • Peritoneum
  • Peritonitis
  • Renal Dialysis - adverse effects
  • Renal failure
  • Renal Insufficiency, Chronic - diagnosis
  • Renal Insufficiency, Chronic - etiology
  • Renal Insufficiency, Chronic - mortality
  • Renal Insufficiency, Chronic - therapy
  • Retrospective Studies
  • Risk Factors
  • Time Factors
  • Treatment Outcome
  • Urology
ispartof: Pediatric nephrology (Berlin, West), 2018-10-29, Vol.34 (4), p.649-655
description: Background Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children. Methods We conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS. Results Eighty children (50% male) with CNS were identified form 17 centers over a 6-year period. Chronic dialysis was started in 44 (55%) children at a median age of 8 (interquartile range 4–14) months. Of these, 17 (39%) were on dialysis by the age of 6 months, 30 (68%) by 1 year, and 40 (91%) by 2 years. Peritoneal dialysis (PD) was the modality of choice in 93%, but 34% switched to hemodialysis (HD), largely due to catheter malfunction ( n  = 5) or peritonitis ( n  = 4). The peritonitis rate was 0.77 per patient-year. Weight and height SDS remained static after 6 months on dialysis. In the overall cohort, at final follow-up, 29 children were transplanted, 18 were still on dialysis (15 PD, 3 HD), 19 were in pre-dialysis chronic kidney disease (CKD), and there were 14 deaths (8 on dialysis). Median time on chronic dialysis until transplantation was 9 (6–18) months, and the median age at transplantation was 22 (14–28) months. Conclusions Infants with CNS on dialysis have a comparable mortality, peritonitis rate, growth, and time to transplantation as infants with other primary renal diseases reported in international registry data.
language: eng
source:
identifier: ISSN: 0931-041X
fulltext: no_fulltext
issn:
  • 0931-041X
  • 1432-198X
url: Link


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titleInfants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases
creatorDufek, Stephanie ; Ylinen, Elisa ; Trautmann, Agnes ; Alpay, Harika ; Ariceta, Gema ; Aufricht, Christoph ; Bacchetta, Justine ; Bakkaloglu, Sevcan ; Bayazit, Aysun ; Caliskan, Salim ; do Sameiro Faria, Maria ; Dursun, Ismail ; Ekim, Mesiha ; Jankauskiene, Augustina ; Klaus, Günter ; Paglialonga, Fabio ; Pasini, Andrea ; Printza, Nikoleta ; Conti, Valerie Said ; Schmitt, Claus Peter ; Stefanidis, Constantinos ; Verrina, Enrico ; Vidal, Enrico ; Webb, Hazel ; Zampetoglou, Argyroula ; Edefonti, Alberto ; Holtta, Tuula ; Shroff, Rukshana
creatorcontribDufek, Stephanie ; Ylinen, Elisa ; Trautmann, Agnes ; Alpay, Harika ; Ariceta, Gema ; Aufricht, Christoph ; Bacchetta, Justine ; Bakkaloglu, Sevcan ; Bayazit, Aysun ; Caliskan, Salim ; do Sameiro Faria, Maria ; Dursun, Ismail ; Ekim, Mesiha ; Jankauskiene, Augustina ; Klaus, Günter ; Paglialonga, Fabio ; Pasini, Andrea ; Printza, Nikoleta ; Conti, Valerie Said ; Schmitt, Claus Peter ; Stefanidis, Constantinos ; Verrina, Enrico ; Vidal, Enrico ; Webb, Hazel ; Zampetoglou, Argyroula ; Edefonti, Alberto ; Holtta, Tuula ; Shroff, Rukshana ; ESPN Dialysis Working Group ; On behalf of the ESPN Dialysis Working Group
descriptionBackground Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children. Methods We conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS. Results Eighty children (50% male) with CNS were identified form 17 centers over a 6-year period. Chronic dialysis was started in 44 (55%) children at a median age of 8 (interquartile range 4–14) months. Of these, 17 (39%) were on dialysis by the age of 6 months, 30 (68%) by 1 year, and 40 (91%) by 2 years. Peritoneal dialysis (PD) was the modality of choice in 93%, but 34% switched to hemodialysis (HD), largely due to catheter malfunction ( n  = 5) or peritonitis ( n  = 4). The peritonitis rate was 0.77 per patient-year. Weight and height SDS remained static after 6 months on dialysis. In the overall cohort, at final follow-up, 29 children were transplanted, 18 were still on dialysis (15 PD, 3 HD), 19 were in pre-dialysis chronic kidney disease (CKD), and there were 14 deaths (8 on dialysis). Median time on chronic dialysis until transplantation was 9 (6–18) months, and the median age at transplantation was 22 (14–28) months. Conclusions Infants with CNS on dialysis have a comparable mortality, peritonitis rate, growth, and time to transplantation as infants with other primary renal diseases reported in international registry data.
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1EISSN: 1432-198X
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languageeng
publisherBerlin/Heidelberg: Springer Berlin Heidelberg
subject3123 Gynaecology ; Age ; Age Factors ; Birth defects ; Care and treatment ; Central nervous system ; Child, Preschool ; Children ; Complications ; Congenital diseases ; Congenital nephrotic syndrome ; Disease Progression ; Europe ; Female ; Health aspects ; Hemodialysis ; Humans ; Infant ; Infant dialysis ; Infant, Newborn ; Infants ; Kidney diseases ; Kidney Failure, Chronic - diagnosis ; Kidney Failure, Chronic - etiology ; Kidney Failure, Chronic - mortality ; Kidney Failure, Chronic - therapy ; Kidney transplantation ; Kidney Transplantation - adverse effects ; Kidney Transplantation - mortality ; Male ; Medicine ; Medicine & Public Health ; Nephrology ; Nephrotic syndrome ; Nephrotic Syndrome - congenital ; Nephrotic Syndrome - diagnosis ; Nephrotic Syndrome - mortality ; Nephrotic Syndrome - therapy ; Original Article ; Outcome ; paediatrics ; Patient outcomes ; Pediatrics ; Peritoneal Dialysis ; Peritoneum ; Peritonitis ; Renal Dialysis - adverse effects ; Renal failure ; Renal Insufficiency, Chronic - diagnosis ; Renal Insufficiency, Chronic - etiology ; Renal Insufficiency, Chronic - mortality ; Renal Insufficiency, Chronic - therapy ; Retrospective Studies ; Risk Factors ; Time Factors ; Treatment Outcome ; Urology
ispartofPediatric nephrology (Berlin, West), 2018-10-29, Vol.34 (4), p.649-655
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0Dufek, Stephanie
1Ylinen, Elisa
2Trautmann, Agnes
3Alpay, Harika
4Ariceta, Gema
5Aufricht, Christoph
6Bacchetta, Justine
7Bakkaloglu, Sevcan
8Bayazit, Aysun
9Caliskan, Salim
10do Sameiro Faria, Maria
11Dursun, Ismail
12Ekim, Mesiha
13Jankauskiene, Augustina
14Klaus, Günter
15Paglialonga, Fabio
16Pasini, Andrea
17Printza, Nikoleta
18Conti, Valerie Said
19Schmitt, Claus Peter
20Stefanidis, Constantinos
21Verrina, Enrico
22Vidal, Enrico
23Webb, Hazel
24Zampetoglou, Argyroula
25Edefonti, Alberto
26Holtta, Tuula
27Shroff, Rukshana
28ESPN Dialysis Working Group
29On behalf of the ESPN Dialysis Working Group
title
0Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases
1Pediatric nephrology (Berlin, West)
addtitle
0Pediatr Nephrol
1Pediatr Nephrol
descriptionBackground Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children. Methods We conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS. Results Eighty children (50% male) with CNS were identified form 17 centers over a 6-year period. Chronic dialysis was started in 44 (55%) children at a median age of 8 (interquartile range 4–14) months. Of these, 17 (39%) were on dialysis by the age of 6 months, 30 (68%) by 1 year, and 40 (91%) by 2 years. Peritoneal dialysis (PD) was the modality of choice in 93%, but 34% switched to hemodialysis (HD), largely due to catheter malfunction ( n  = 5) or peritonitis ( n  = 4). The peritonitis rate was 0.77 per patient-year. Weight and height SDS remained static after 6 months on dialysis. In the overall cohort, at final follow-up, 29 children were transplanted, 18 were still on dialysis (15 PD, 3 HD), 19 were in pre-dialysis chronic kidney disease (CKD), and there were 14 deaths (8 on dialysis). Median time on chronic dialysis until transplantation was 9 (6–18) months, and the median age at transplantation was 22 (14–28) months. Conclusions Infants with CNS on dialysis have a comparable mortality, peritonitis rate, growth, and time to transplantation as infants with other primary renal diseases reported in international registry data.
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03123 Gynaecology
1Age
2Age Factors
3Birth defects
4Care and treatment
5Central nervous system
6Child, Preschool
7Children
8Complications
9Congenital diseases
10Congenital nephrotic syndrome
11Disease Progression
12Europe
13Female
14Health aspects
15Hemodialysis
16Humans
17Infant
18Infant dialysis
19Infant, Newborn
20Infants
21Kidney diseases
22Kidney Failure, Chronic - diagnosis
23Kidney Failure, Chronic - etiology
24Kidney Failure, Chronic - mortality
25Kidney Failure, Chronic - therapy
26Kidney transplantation
27Kidney Transplantation - adverse effects
28Kidney Transplantation - mortality
29Male
30Medicine
31Medicine & Public Health
32Nephrology
33Nephrotic syndrome
34Nephrotic Syndrome - congenital
35Nephrotic Syndrome - diagnosis
36Nephrotic Syndrome - mortality
37Nephrotic Syndrome - therapy
38Original Article
39Outcome
40paediatrics
41Patient outcomes
42Pediatrics
43Peritoneal Dialysis
44Peritoneum
45Peritonitis
46Renal Dialysis - adverse effects
47Renal failure
48Renal Insufficiency, Chronic - diagnosis
49Renal Insufficiency, Chronic - etiology
50Renal Insufficiency, Chronic - mortality
51Renal Insufficiency, Chronic - therapy
52Retrospective Studies
53Risk Factors
54Time Factors
55Treatment Outcome
56Urology
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1Ylinen, Elisa
2Trautmann, Agnes
3Alpay, Harika
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6Bacchetta, Justine
7Bakkaloglu, Sevcan
8Bayazit, Aysun
9Caliskan, Salim
10do Sameiro Faria, Maria
11Dursun, Ismail
12Ekim, Mesiha
13Jankauskiene, Augustina
14Klaus, Günter
15Paglialonga, Fabio
16Pasini, Andrea
17Printza, Nikoleta
18Conti, Valerie Said
19Schmitt, Claus Peter
20Stefanidis, Constantinos
21Verrina, Enrico
22Vidal, Enrico
23Webb, Hazel
24Zampetoglou, Argyroula
25Edefonti, Alberto
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titleInfants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases
authorDufek, Stephanie ; Ylinen, Elisa ; Trautmann, Agnes ; Alpay, Harika ; Ariceta, Gema ; Aufricht, Christoph ; Bacchetta, Justine ; Bakkaloglu, Sevcan ; Bayazit, Aysun ; Caliskan, Salim ; do Sameiro Faria, Maria ; Dursun, Ismail ; Ekim, Mesiha ; Jankauskiene, Augustina ; Klaus, Günter ; Paglialonga, Fabio ; Pasini, Andrea ; Printza, Nikoleta ; Conti, Valerie Said ; Schmitt, Claus Peter ; Stefanidis, Constantinos ; Verrina, Enrico ; Vidal, Enrico ; Webb, Hazel ; Zampetoglou, Argyroula ; Edefonti, Alberto ; Holtta, Tuula ; Shroff, Rukshana
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03123 Gynaecology
1Age
2Age Factors
3Birth defects
4Care and treatment
5Central nervous system
6Child, Preschool
7Children
8Complications
9Congenital diseases
10Congenital nephrotic syndrome
11Disease Progression
12Europe
13Female
14Health aspects
15Hemodialysis
16Humans
17Infant
18Infant dialysis
19Infant, Newborn
20Infants
21Kidney diseases
22Kidney Failure, Chronic - diagnosis
23Kidney Failure, Chronic - etiology
24Kidney Failure, Chronic - mortality
25Kidney Failure, Chronic - therapy
26Kidney transplantation
27Kidney Transplantation - adverse effects
28Kidney Transplantation - mortality
29Male
30Medicine
31Medicine & Public Health
32Nephrology
33Nephrotic syndrome
34Nephrotic Syndrome - congenital
35Nephrotic Syndrome - diagnosis
36Nephrotic Syndrome - mortality
37Nephrotic Syndrome - therapy
38Original Article
39Outcome
40paediatrics
41Patient outcomes
42Pediatrics
43Peritoneal Dialysis
44Peritoneum
45Peritonitis
46Renal Dialysis - adverse effects
47Renal failure
48Renal Insufficiency, Chronic - diagnosis
49Renal Insufficiency, Chronic - etiology
50Renal Insufficiency, Chronic - mortality
51Renal Insufficiency, Chronic - therapy
52Retrospective Studies
53Risk Factors
54Time Factors
55Treatment Outcome
56Urology
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1Ylinen, Elisa
2Trautmann, Agnes
3Alpay, Harika
4Ariceta, Gema
5Aufricht, Christoph
6Bacchetta, Justine
7Bakkaloglu, Sevcan
8Bayazit, Aysun
9Caliskan, Salim
10do Sameiro Faria, Maria
11Dursun, Ismail
12Ekim, Mesiha
13Jankauskiene, Augustina
14Klaus, Günter
15Paglialonga, Fabio
16Pasini, Andrea
17Printza, Nikoleta
18Conti, Valerie Said
19Schmitt, Claus Peter
20Stefanidis, Constantinos
21Verrina, Enrico
22Vidal, Enrico
23Webb, Hazel
24Zampetoglou, Argyroula
25Edefonti, Alberto
26Holtta, Tuula
27Shroff, Rukshana
28ESPN Dialysis Working Group
29On behalf of the ESPN Dialysis Working Group
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0Dufek, Stephanie
1Ylinen, Elisa
2Trautmann, Agnes
3Alpay, Harika
4Ariceta, Gema
5Aufricht, Christoph
6Bacchetta, Justine
7Bakkaloglu, Sevcan
8Bayazit, Aysun
9Caliskan, Salim
10do Sameiro Faria, Maria
11Dursun, Ismail
12Ekim, Mesiha
13Jankauskiene, Augustina
14Klaus, Günter
15Paglialonga, Fabio
16Pasini, Andrea
17Printza, Nikoleta
18Conti, Valerie Said
19Schmitt, Claus Peter
20Stefanidis, Constantinos
21Verrina, Enrico
22Vidal, Enrico
23Webb, Hazel
24Zampetoglou, Argyroula
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abstractBackground Children with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children. Methods We conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS. Results Eighty children (50% male) with CNS were identified form 17 centers over a 6-year period. Chronic dialysis was started in 44 (55%) children at a median age of 8 (interquartile range 4–14) months. Of these, 17 (39%) were on dialysis by the age of 6 months, 30 (68%) by 1 year, and 40 (91%) by 2 years. Peritoneal dialysis (PD) was the modality of choice in 93%, but 34% switched to hemodialysis (HD), largely due to catheter malfunction ( n  = 5) or peritonitis ( n  = 4). The peritonitis rate was 0.77 per patient-year. Weight and height SDS remained static after 6 months on dialysis. In the overall cohort, at final follow-up, 29 children were transplanted, 18 were still on dialysis (15 PD, 3 HD), 19 were in pre-dialysis chronic kidney disease (CKD), and there were 14 deaths (8 on dialysis). Median time on chronic dialysis until transplantation was 9 (6–18) months, and the median age at transplantation was 22 (14–28) months. Conclusions Infants with CNS on dialysis have a comparable mortality, peritonitis rate, growth, and time to transplantation as infants with other primary renal diseases reported in international registry data.
copBerlin/Heidelberg
pubSpringer Berlin Heidelberg
pmid30374605
doi10.1007/s00467-018-4122-0
orcididhttps://orcid.org/0000-0002-6323-6673
oafree_for_read