Stephanie Dufek1, Elisa Ylinen2, Agnes Trautmann3, Harika Alpay4, Gema Ariceta5, Christoph Aufricht6, Justine Bacchetta7, Sevcan Bakkaloglu8, Aysun Bayazit9, Salim Caliskan10, Maria do Sameiro Faria11, Ismail Dursun12, Mesiha Ekim13, Augustina Jankauskiene14, Günter Klaus15, Fabio Paglialonga16, Andrea Pasini17, Nikoleta Printza18, Valerie Said Conti19, Claus Peter Schmitt3, Constantinos Stefanidis20, Enrico Verrina21, Enrico Vidal22, Hazel Webb1, Argyroula Zampetoglou20, Alberto Edefonti16, Tuula Holtta2, Rukshana Shroff23. 1. Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK. 2. University of Helsinki and Helsinki University Hospital, Helsinki, Finland. 3. Center for Pediatric and Adolescent Medicine, Heidelberg, Germany. 4. School of Medicine, Marmara University, Istanbul, Turkey. 5. Hospital Universitari Vall d'Hebron. Universitat Autonoma de Barcelona, Barcelona, Spain. 6. Medical University of Vienna, Vienna, Austria. 7. Hôpital Femme Mère Enfant, Lyon, France. 8. Gazi University Hospital, Ankara, Turkey. 9. Cukurova University, Adana, Turkey. 10. Cerrahpasa Medical Faculty, Istanbul, Turkey. 11. Centro Materno Infantil do Norte, Porto, Portugal. 12. Erciyes University, Kayseri, Turkey. 13. Ankara University Hospital, Ankara, Turkey. 14. Vilnius University, Vilnius, Lithuania. 15. KfH Pediatric Kidney Center, Marburg, Germany. 16. Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. 17. Azienda Ospedaliero-Universitaria Sant'Orsola-Malpighi, Bologna, Italy. 18. 1st Pediatric Department, Aristotle University, Thessaloniki, Greece. 19. Mater Dei Hospital Malta, Msida, Malta. 20. "A & P Kyriakou", Children's Hospital, Athens, Greece. 21. IRCCS Giannina Gaslini, Genoa, Italy. 22. University-Hospital of Padova, Padova, Italy. 23. Great Ormond Street Hospital for Children NHS Foundation Trust, London, WC1N 3JH, UK. Rukshana.Shroff@gosh.nhs.uk.
Abstract
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.
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.
Authors: Enrico Vidal; Karlijn J van Stralen; Nicholas C Chesnaye; Marjolein Bonthuis; Christer Holmberg; Aleksandra Zurowska; Antonella Trivelli; José Eduardo Esteves Da Silva; Maria Herthelius; Brigitte Adams; Anna Bjerre; Augustina Jankauskiene; Polina Miteva; Khadizha Emirova; Aysun K Bayazit; Christoph J Mache; Ana Sánchez-Moreno; Jérôme Harambat; Jaap W Groothoff; Kitty J Jager; Franz Schaefer; Enrico Verrina Journal: Am J Kidney Dis Date: 2016-12-10 Impact factor: 8.860
Authors: Alexandru R Constantinescu; Tej K Mattoo; William E Smoyer; Larry A Greenbaum; Jianli Niu; Noel Howard; Melissa Muff-Luett; Elizabeth B Benoit; Avram Traum; Ali A Annaim; Scott E Wenderfer; Emilee Plautz; Michelle N Rheault; Robert L Myette; Katherine E Twombley; Yu Kamigaki; Belkis Wandique-Rapalo; Mohammad Kallash; Tetyana L Vasylyeva Journal: Front Pediatr Date: 2022-09-14 Impact factor: 3.569