Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)
Authors: Burgmaier Kathrin , Ariceta Gema , Bald Martin , Buescher Anja Katrin , Burgmaier Mathias , Erger Florian , Gessner Michaela , Gokce Ibrahim , König Jens , Kowalewska Claudia , Massella Laura , Mastrangelo Antonio , Mekahli Djalila , Pape Lars , Patzer Ludwig , Potemkina Alexandra , Schalk Gesa , Schild Raphael , Shroff Rukshana , Szczepanska Maria , Taranta-Janusz Katarzyna , Tkaczyk Marcin , Weber Lutz Thorsten , Wühl Elke , Wurm Donald , Wygoda Simone , Zagozdzon Ilona , Dötsch Jörg , Oh Jun , Schaefer Franz , Liebau Max Christoph ,   The ARegPKD consortium, Eid Loai Akram , Arbeiter Klaus , Ranguelov Nadejda , Collard Laure , De Mul Aurélie , Feldkoetter Markus , Seeman Tomas , Thumfart Julia , Grundmann Franziska , Galiano Matthias , Buchholz Björn , Buescher Rainer , Häffner Karsten , Gross Oliver , Bernhardt Wanja , Doyon Anke , Henn Michael , Halbritter Jan , Derichs Ute , Klaus Günter , Lange-Sperandio Bärbel , Uetz Barbara , Benz Marcus , Titieni Andrea , Staude Hagen , Leichter Heinz E. , Soliman Neveen A. , Lara Luis Enrique , de la Cerda Ojeda Francisco , Harambat Jerome , Ranchin Bruno , Fila Marc , Dossier Claire , Boyer Olivia , Marlais Matko , Stabouli Stella , Hooman Nakysa , Mencarelli Francesca , Morello William , Longo Germana , Emma Francesco , Ruzgiene Dovile , Wasilewska Anna , Balasz-Chmielewska Irena , Miklaszewska Monika , Stanczyk Malgorzata , Sikora Przemyslaw , Litwin Mieczyslaw , Morawiec-Knysak Aurelia , Teixeira Ana , Milosevski-Lomic Gordana , Prikhodina Larisa , Rus Rina , Jilani Houweyda , Melek Engin , Duzova Ali , Soylu Alper , Candan Cengiz , Sever Lale , Yilmaz Alev , Cicek Neslihan , Akinci Nurver , Mir Sevgi , Dursun Ismail , Tabel Yilmaz , Nalcacioglu Hulya ,   ESCAPE Study Group,   GPN Study Group
Published: September 2020

To test the association between bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD) and long-term clinical outcome and to identify risk factors for severe outcomes, a dataset comprising 504 patients from the international registry study ARegPKD was analyzed for characteristics and complications of patients with very early (≤ 3 months; VEBNE) and early (4–15 months; EBNE) bilateral nephrectomies. Patients with very early dialysis (VED, onset ≤ 3 months) without bilateral nephrectomies and patients with total kidney volumes (TKV) comparable to VEBNE infants served as additional control groups. We identified 19 children with VEBNE, 9 with EBNE, 12 with VED and 11 in the TKV control group. VEBNE patients suffered more frequently from severe neurological complications in comparison to all control patients. Very early bilateral nephrectomies and documentation of severe hypotensive episodes were independent risk factors for severe neurological complications. Bilateral nephrectomies within the first 3 months of life are associated with a risk of severe neurological complications later in life. Our data support a very cautious indication of very early bilateral nephrectomies in ARPKD, especially in patients with residual kidney function, and emphasize the importance of avoiding severe hypotensive episodes in this at-risk cohort.