Risk Factors for Early Dialysis Dependency in Autosomal Recessive

Abstract

Objective To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis.

Study design A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life.

Results Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys.

Conclusions This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life. may be helpful in prenatal parental counseling in cases of suspected ARPKD.

C1 [Burgmaier, Kathrin; Weber, Lutz Thorsten; Doetsch, Joerg; Liebau, Max Christoph] Univ Hosp Cologne, Dept Pediat, Cologne, Germany.

[Kunzmann, Kevin] Heidelberg Univ, Inst Med Biometry & Informat, Heidelberg, Germany.

[Ariceta, Gema] Univ Hosp Vall dHebron, Dept Pediat Nephrol, Barcelona, Spain.

[Bergmann, Carsten] Bioscientia Ctr Human Genet, Ingelheim, Germany.

[Bergmann, Carsten] Univ Freiburg, Dept Med, Renal Div, Med Ctr, Freiburg, Germany.

[Buescher, Anja Katrin] Univ Hosp Essen, Dept Pediat 2, Essen, Germany.

[Burgmaier, Mathias] Rhein Westfal TH Aachen, Dept Internal Med 1, Univ Hosp, Aachen, Germany.

[Dursun, Ismail] Erciyes Univ, Fac Med, Dept Pediat Nephrol, Kayseri, Turkey.

[Duzova, Ali; Topaloglu, Rezan] Hacettepe Univ, Dept Pediat, Div Pediat Nephrol, Fac Med, Ankara, Turkey.

[Eid, Loai] Dubai Hosp, Dubai Kidney Ctr Excellence, Dept Pediat Nephrol, Dubai, U Arab Emirates.

[Erger, Florian] Univ Hosp Cologne, Inst Human Genet, Cologne, Germany.

[Erger, Florian; Liebau, Max Christoph] Univ Hosp Cologne, Ctr Mol Med, Cologne, Germany.

[Feldkoetter, Markus; Hoppe, Bernd] Univ Hosp Bonn, Dept Pediat, Div Pediat Nephrol, Bonn, Germany.

[Galiano, Matthias] Univ Erlangen Nurnberg FAU, Dept Pediat & Adolescent Med, Erlangen, Germany.

[Gessner, Michaela] Childrens Univ Hosp Tuebingen, Dept Gen Pediat & Hematol Oncol, Tubingen, Germany.

[Goebel, Heike] Univ Hosp Cologne, Inst Pathol, Cologne, Germany.

[Gokce, Ibrahim] Marmara Univ, Res & Training Hosp, Div Pediat Nephrol, Istanbul, Turkey.

[Haffner, Dieter; Pape, Lars] Hannover Med Sch, Dept Pediat Kidney Liver & Metab Dis, Hannover, Germany.

[Hooman, Nakysa] Iran Univ Med Sci, Ali Asghar Children Hosp, Dept Pediat Nephrol, Tehran, Iran.

[Jankauskiene, Augustina] Vilnius Univ, Inst Clin Med, Clin Children Dis, Vilnius, Lithuania.

[Klaus, Guenter] Univ Hosp Marburg, KfH Ctr Paediat Nephrol, Marburg, Germany.

[Koenig, Jens] Univ Hosp Muenster, Dept Gen Pediat, Munster, Germany.

[Litwin, Mieczyslaw; Wicher, Dorota] Childrens Mem Hlth Inst, Warsaw, Poland.

[Massella, Laura] Bambino Gesii Childrens Hosp, Nephrol & Dialysis Unit, IRCCS, Rome, Italy.

[Mekahli, Djalila] Univ Hosp Leuven, Dept Pediat Nephrol, Leuven, Belgium.

[Mekahli, Djalila] KU Leuven Univ Leuven, Dept Dev & Regenerat, Lab Pediat, PKD Res Grp, B-3000 Leuven, Belgium.

[Melek, Engin] Cukurova Univ, Dept Pediat Nephrol, Fac Med, Adana, Turkey.

[Mir, Sevgi] Ege Univ Med Fac, Dept Pediat Nephrol, Izmir, Turkey.

[Prikhodina, Larisa] Pirogov Russian Natl Res Med Univ, Res Clin Inst Pediat, Dept Inherited & Acquired Kidney Dis, Moscow, Russia.

[Ranchin, Bruno] Hosp Civils Lyon, Pediat Nephrol Unit, Hop Femme Mere Enfant, Lyon, France.

[Schild, Raphael] Univ Childrens Hosp, Univ Med Ctr Hamburg Eppendorf, Hamburg, Germany.

[Seeman, Tomas] Charles Univ Prague, Univ Hosp Motol, Fac Med 2, Dept Pediat, Prague, Czech Republic.

[Sever, Late] Istanbul Univ, Cerrahpasa Sch Med, Dept Pediat Nephrol, Istanbul, Turkey.

[Shroff, Rukshana] Great Ormond St Hosp Children NHS Fdn Trust, London, England.

[Soliman, Neveen A.] Cairo Univ, Ctr Pediat Nephrol & Transplantat, Kasr Al Ainy Sch Med, Dept Pediat, Cairo, Egypt.

[Stabouli, Stella] Aristotle Univ Thessaloniki, Hippokrat Hosp, Dept Pediat 1, Thessaloniki, Greece.

[Stanczyk, Malgorzata] Polish Mothers Mem Hosp Res Inst, Dept Pediat Immunol & Nephrol, Lodz, Poland.

[Tabel, Yilmaz] Inonu Univ, Fac Med, Dept Pediat Nephrol, Malatya, Turkey.

[Taranta-Janusz, Katarzyna] Med Univ Bialystok, Dept Paediat & Nephrol, Bialystok, Poland.

[Testa, Sara] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Pediat Nephrol Unit, Milan, Italy.

[Thumfart, Julia] Charite Univ Med Berlin, Dept Pediat Nephrol, Berlin, Germany.

[Thumfart, Julia] Free Univ Berlin, Berlin, Germany.

[Thumfart, Julia] Humboldt Univ, Berlin, Germany.

[Thumfart, Julia] Berlin Inst Hlth, Berlin, Germany.

[Wuehl, Elke; Schaefer, Franz] Heidelberg Univ, Ctr Pediat & Adolescent Med, Div Pediat Nephrol, Heidelberg, Germany.

[Wygoda, Simone] Hosp St Georg, Clin Children & Adolescents, Leipzig, Germany.

[Yilmaz, Alev] Istanbul Univ, Istanbul Fac Med, Pediat Nephrol Dept, Istanbul, Turkey.

[Zachwieja, Katarzyna] Jagiellonian Univ, Fac Med, Dept Pediat Nephrol & Hypertens, Med Coll, Krakow, Poland.

[Zagozdzon, Ilona] Med Univ Gdansk, Dept Nephrol & Hypertens Children & Adolescents, Gdansk, Poland.

[Zerres, Klaus] RWTH Univ Hosp Aachen, Inst Human Genet, Aachen, Germany.