Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)

Burgmaier K, Ariceta G, Bald M, Buescher AK, Burgmaier M, Erger F, Gessner M, Gokce I, König J, Kowalewska C, Massella L, Mastrangelo A, Mekahli D, Pape L, Patzer L, Potemkina A, Schalk G, Schild R, Shroff R, Szczepanska M, Taranta-Janusz K, Tkaczyk M, Weber LT, Wühl E, Wurm D, Wygoda S, Zagozdzon I, Dötsch J, Oh J, Schaefer F, Liebau MC, Eid LA, Arbeiter K, Ranguelov N, Collard L, De Mul A, Feldkoetter M, Seeman T, Thumfart J, Grundmann F, Galiano M, Buchholz B, Buescher R, Häffner K, Gross O, Bernhardt W, Doyon A, Henn M, Halbritter J, Derichs U, Klaus G, Lange-Sperandio B, Uetz B, Benz M, Titieni A, Staude H, Leichter HE, Soliman NA, Lara LE, de la Cerda Ojeda F, Harambat J, Ranchin B, Fila M, Dossier C, Boyer O, Marlais M, Stabouli S, Hooman N, Mencarelli F, Morello W, Longo G, Emma F, Ruzgiene D, Wasilewska A, Balasz-Chmielewska I, Miklaszewska M, Stanczyk M, Sikora P, Litwin M, Morawiec-Knysak A, Teixeira A, Milosevski-Lomic G, Prikhodina L, Rus R, Jilani H, Melek E, Duzova A, Soylu A, Candan C, Sever L, Yilmaz A, Cicek N, Akinci N, Mir S, Dursun I, Tabel Y, Nalcacioglu H (2020)

Publication Type: Journal article

Publication year: 2020

Journal

Book Volume: 10

Article Number: 16025

Journal Issue: 1

DOI: 10.1038/s41598-020-71956-1

Abstract

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.

Authors with CRIS profile

Involved external institutions

University College London (UCL) United Kingdom (GB) Universität zu Köln Germany (DE) Dubai Hospital United Arab Emirates (AE) Medical University Gdansk / Gdański Uniwersytet Medyczny Poland (PL) Instytut Centrum Zdrowia Matki Polki w Łodzi (ICZMP) / Polish Mother’s Memorial Hospital Research Institute Poland (PL) Medical University of Silesia in Katowice / Śląski Uniwersytet Medyczny w Katowicach (SUM) Poland (PL) Universitätsklinikum Heidelberg Germany (DE) Medizinische Universität Wien Austria (AT) Vall d'Hebron University Hospital / Hospital Universitari Vall d'Hebron Spain (ES) Universitätsklinikum Tübingen Germany (DE) Klinikum Stuttgart Germany (DE) Hôpital Mongi Slim Tunisia (TN) Universitätsklinikum Aachen (UKA) Germany (DE) Klinikum Saarbrücken Germany (DE) Universitätsklinikum Essen Germany (DE) Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico Italy (IT) University Children's Hospital / Univerzitetska dečja klinika u Beogradu (Tiršova) Serbia (RS) Istanbul University / İstanbul Üniversitesi Turkey (TR) Pirogov Russian National Research Medical University (RSMU / RNRMU) Russian Federation (RU) Universitätsklinikum Gießen und Marburg (UKGM) Germany (DE) Hospices Civils de Lyon (CHU) France (FR) Geneva University Hospitals / Hôpitaux universitaires de Genève (HUG) Switzerland (CH) Ali Asghar Children Hospital / (مرکز آموزشی درمانی حضرت علی اصغر (ص Iran, Islamic Republic of (IR) Ondokuz Mayıs University Turkey (TR) Universitätsklinikum Leipzig Germany (DE) Universitätsklinikum Münster Germany (DE) Universitätsklinikum Freiburg Germany (DE) Children's Memorial Health Institute / Instytut "Pomnik - Centrum Zdrowia Dziecka" Poland (PL) Şişli Etfal Eğitim ve Araştırma Hastanesi Turkey (TR) İnönü University Turkey (TR) Universitäts-Kinderspital Zürich Switzerland (CH) Klinikum Schwabing Germany (DE) Vilnius University / Vilniaus universitetas Lithuania (LT) Marmara University Turkey (TR) Universitätsklinikum Köln Germany (DE) Klinikum Dritter Orden Germany (DE) Klinikum St. Georg Germany (DE) Hacettepe University Turkey (TR) Medical University of Lublin / Uniwersytet Medyczny w Lublinie Poland (PL) Universitätsmedizin Rostock Germany (DE) Universitätsmedizin der Johannes Gutenberg-Universität Mainz Germany (DE) Universitätsklinikum Hamburg-Eppendorf (UKE) Germany (DE) Hôpital Universitaire Robert-Debré France (FR) Ege University / Ege Üniversitesi Turkey (TR) Centre Hospitalier Universitaire de Bordeaux / CHU Bordeaux France (FR) Ospedale Pediatrico Bambino Gesu Italy (IT) Charité - Universitätsmedizin Berlin Germany (DE) Ospedaliero Universitaria di Bologna Policlinico S.Orsola-Malpighi Italy (IT) Hôpital Necker-Enfants malades France (FR) Aristotle University of Thessaloniki Greece (GR) Azienda Ospedaliera di Padova Italy (IT) Istanbul Medeniyet University / İstanbul Medeniyet Üniversitesi Turkey (TR) Olgahospital / Olgäle Germany (DE) Cairo University Egypt (EG) Erciyes University / Erciyes Üniversitesi Turkey (TR) Ljubljana University Medical Centre (Ljubljana UMC) / Univerzitetni klinični center Ljubljana Slovenia (SI) Hospital de São João / Saint John Hospital Portugal (PT) Cliniques universitaires Saint-Luc (CHU St-Luc) Belgium (BE) Medical University of Bialystok Poland (PL) Universitätsklinikum Göttingen Germany (DE) Universitätsklinikum Bonn Germany (DE) Klinikum der Universität München Germany (DE) Motol University Hospital / Fakultní nemocnice v Motole Czech Republic (CZ) Jagiellonian University / Uniwersytet Jagielloński (UJ) Poland (PL) Dokuz Eylül Üniversitesi (DEU) Turkey (TR) Arnaud de Villeneuve France (FR) Klinikum der Medizinischen Hochschule Hannover Germany (DE) Krankenhaus St. Elisabeth und St. Barbara Germany (DE) Çukurova Üniversitesi Turkey (TR) Hospitales Universitarios Virgen Macarena y Virgen del Rocío Spain (ES) Katholieke Universiteit Leuven (KUL) / Catholic University of Leuven Belgium (BE)

How to cite

APA:

Burgmaier, K., Ariceta, G., Bald, M., Buescher, A.K., Burgmaier, M., Erger, F.,... Nalcacioglu, H. (2020). Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD). Scientific Reports, 10(1). https://dx.doi.org/10.1038/s41598-020-71956-1

MLA:

Burgmaier, Kathrin, et al. "Severe neurological outcomes after very early bilateral nephrectomies in patients with autosomal recessive polycystic kidney disease (ARPKD)." Scientific Reports 10.1 (2020).

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