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

被引：11

作者：

Dufek, Stephanie ^{[1
]}

Ylinen, Elisa ^{[2
,3
]}

Trautmann, Agnes ^{[4
]}

Alpay, Harika ^{[5
]}

Ariceta, Gema ^{[6
]}

Aufricht, Christoph ^{[7
]}

Bacchetta, Justine ^{[8
]}

Bakkaloglu, Sevcan ^{[9
]}

Bayazit, Aysun ^{[10
]}

Caliskan, Salim ^{[11
]}

Faria, Maria do Sameiro ^{[12
]}

Dursun, Ismail ^{[13
]}

Ekim, Mesiha ^{[14
]}

Jankauskiene, Augustina ^{[15
]}

Klaus, Guenter ^{[16
]}

Paglialonga, Fabio ^{[17
]}

Pasini, Andrea ^{[18
]}

Printza, Nikoleta ^{[19
]}

Conti, Valerie Said ^{[20
]}

Schmitt, Claus Peter ^{[4
]}

Stefanidis, Constantinos ^{[21
]}

Verrina, Enrico ^{[22
]}

Vidal, Enrico ^{[23
]}

Webb, Hazel ^{[1
]}

Zampetoglou, Argyroula ^{[21
]}

Edefonti, Alberto ^{[17
]}

Holtta, Tuula ^{[2
,3
]}

Shroff, Rukshana ^{[1
]}

机构：

[1] Great Ormond St Hosp Children NHS Fdn Trust, London WC1N 3JH, England

[2] Univ Helsinki, Helsinki, Finland

[3] Helsinki Univ Hosp, Helsinki, Finland

[4] Ctr Pediat & Adolescent Med, Heidelberg, Germany

[5] Marmara Univ, Sch Med, Istanbul, Turkey

[6] Univ Autonoma Barcelona, Hosp Univ Vall dHebron, Barcelona, Spain

[7] Med Univ Vienna, Vienna, Austria

[8] Hop Femme Mere Enfant, Lyon, France

[9] Gazi Univ Hosp, Ankara, Turkey

[10] Cukurova Univ, Adana, Turkey

[11] Cerrahpasa Med Fac, Istanbul, Turkey

[12] Ctr Materno Infantil Norte, Porto, Portugal

[13] Erciyes Univ, Kayseri, Turkey

[14] Ankara Univ Hosp, Ankara, Turkey

[15] Vilnius Univ, Vilnius, Lithuania

[16] KfH Pediat Kidney Ctr, Marburg, Germany

[17] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Milan, Italy

[18] Azienda Osped Univ St Orsola Malpighi, Bologna, Italy

[19] Aristotle Univ Thessaloniki, Pediat Dept 1, Thessaloniki, Greece

[20] Mater Dei Hosp Malta, Msida, Malta

[21] A&P Kyriakou Childrens Hosp, Athens, Greece

[22] IRCCS Giannina Gaslini, Genoa, Italy

[23] Univ Hosp Padova, Padua, Italy

来源：

PEDIATRIC NEPHROLOGY | 2019年 / 34卷 / 04期

关键词：

Congenital nephrotic syndrome; Infant dialysis; Complications; Outcome; CHRONIC DIALYSIS; PERITONEAL-DIALYSIS; CHILDREN; SURVIVAL; HEMODIALYSIS; EXPERIENCE; MANAGEMENT; MUTATIONS;

D O I：

10.1007/s00467-018-4122-0

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

BackgroundChildren 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.MethodsWe 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.ResultsEighty 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 6months, 30 (68%) by 1year, and 40 (91%) by 2years. 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 6months 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.ConclusionsInfants 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.

引用

页码：649 / 655

页数：7

共 25 条

[1] Treatment and outcome of congenital nephrotic syndrome [J].

Berody, Sandra ;

Heidet, Laurence ;

Gribouval, Olivier ;

Harambat, Jerome ;

Niaudet, Patrick ;

Baudouin, Veronique ;

Bacchetta, Justine ;

Boudaillez, Bernard ;

Dehennault, Maud ;

de Parscau, Loic ;

Dunand, Olivier ;

Flodrops, Hugues ;

Fila, Marc ;

Garnier, Arnaud ;

Louillet, Ferielle ;

Macher, Marie-Alice ;

May, Adrien ;

Merieau, Elodie ;

Monceaux, Francoise ;

Pietrement, Christine ;

Rousset-Rouviere, Caroline ;

Roussey, Gwenaelle ;

Taque, Sophie ;

Tenenbaum, Julie ;

Ulinski, Tim ;

Vieux, Rachel ;

Zaloszyc, Ariane ;

Moriniere, Vincent ;

Salomon, Remi ;

Boyer, Olivia .

NEPHROLOGY DIALYSIS TRANSPLANTATION, 2019, 34 (03) :458-467

[2] Management of children with congenital nephrotic syndrome: challenging treatment paradigms [J].

Dufek, Stephanie ;

Holtta, Tuula ;

Trautmann, Agnes ;

Ylinen, Elisa ;

Alpay, Harika ;

Ariceta, Gema ;

Aufricht, Christoph ;

Bacchetta, Justine ;

Bakkaloglu, Sevcan A. ;

Bayazit, Aysun ;

Cicek, Rumeysa Yasemin ;

Dursun, Ismail ;

Duzova, Ali ;

Ekim, Mesiha ;

Iancu, Daniela ;

Jankauskiene, Augustina ;

Klaus, Guenter ;

Paglialonga, Fabio ;

Pasini, Andrea ;

Printza, Nikoleta ;

Conti, Valerie Said ;

Faria, Maria do Sameiro ;

Schmitt, Claus Peter ;

Stefanidis, Constantinos J. ;

Verrina, Enrico ;

Vidal, Enrico ;

Vondrak, Karel ;

Webb, Hazel ;

Zampetoglou, Argyroula ;

Bockenhauer, Detlef ;

Edefonti, Alberto ;

Shroff, Rukshana .

NEPHROLOGY DIALYSIS TRANSPLANTATION, 2019, 34 (08) :1369-1377

[3] Twenty-five Years of Infant Dialysis: A Single Center Experience [J].

Hijazi, Rana ;

Abitbol, Carolyn L. ;

Chandar, Jayanthi ;

Seeherunvong, Wacharee ;

Freundlich, Michael ;

Zilleruelo, Gaston .

JOURNAL OF PEDIATRICS, 2009, 155 (01) :111-117

[4] Patient and transplant outcome in infants starting renal replacement therapy before 2 years of age [J].

Hogan, Julien ;

Bacchetta, Justine ;

Charbit, Marina ;

Roussey, Gwenaelle ;

Novo, Robert ;

Tsimaratos, Michel ;

Terzic, Joelle ;

Ulinski, Tim ;

Garnier, Arnaud ;

Merieau, Elodie ;

Harambat, Jerome ;

Vrillon, Isabelle ;

Dunand, Olivier ;

Morin, Denis ;

Berard, Etienne ;

Nobili, Francois ;

Couchoud, Cecile ;

Macher, Marie-Alice .

NEPHROLOGY DIALYSIS TRANSPLANTATION, 2018, 33 (08) :1459-1465

[5]

Holmberg C, 1996, KIDNEY INT, V49, pS51

[6] MANAGEMENT OF CONGENITAL NEPHROTIC SYNDROME OF THE FINNISH TYPE [J].

HOLMBERG, C ;

ANTIKAINEN, M ;

RONNHOLM, K ;

ALAHOUHALA, M ;

JALANKO, H .

PEDIATRIC NEPHROLOGY, 1995, 9 (01) :87-93

[7] Timing of renal replacement therapy does not influence survival and growth in children with congenital nephrotic syndrome caused by mutations in NPHS1: data from the ESPN/ERA-EDTA Registry [J].

Holtta, Tuula ;

Bonthuis, Marjolein ;

Van Stralen, Karlijn J. ;

Bjerre, Anna ;

Topaloglu, Rezan ;

Ozaltin, Fatih ;

Holmberg, Christer ;

Harambat, Jerome ;

Jager, Kitty J. ;

Schaefer, Franz ;

Groothoff, Jaap W. .

PEDIATRIC NEPHROLOGY, 2016, 31 (12) :2317-2325

[8] Congenital nephrotic syndrome [J].

Jalanko, Hannu .

PEDIATRIC NEPHROLOGY, 2009, 24 (11) :2121-2128

[9] Venous thromboembolism in pediatric nephrotic syndrome [J].

Kerlin, Bryce A. ;

Haworth, Kellie ;

Smoyer, William E. .

PEDIATRIC NEPHROLOGY, 2014, 29 (06) :989-997

[10]

Kim M S, 1998, Pediatr Transplant, V2, P305

← 1 2 3 →