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J Pediatr. 2018 Aug;199:22-28.e6. doi: 10.1016/j.jpeds.2018.03.052. Epub 2018 May 9.

Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease.

Author information

1
Department of Pediatrics, University Hospital of Cologne, Cologne, Germany.
2
Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany.
3
Department of Pediatric Nephrology, University Hospital Vall d'Hebron, Barcelona, Spain.
4
Bioscientia Center for Human Genetics, Ingelheim, Germany; Renal Division, Department of Medicine, University Freiburg Medical Center, Freiburg, Germany.
5
Department of Pediatrics II, University Hospital Essen, Essen, Germany.
6
Department of Internal Medicine I, University Hospital of the RWTH Aachen, Aachen, Germany.
7
Department of Pediatric Nephrology, Erciyes University, Faculty of Medicine, Kayseri, Turkey.
8
Department of Pediatrics, Division of Pediatric Nephrology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
9
Department of Pediatric Nephrology, Dubai Kidney Center Of Excellence, Dubai Hospital, Dubai, United Arab Emirates.
10
Institute of Human Genetics, University Hospital of Cologne, Cologne, Germany; Center for Molecular Medicine, University Hospital of Cologne, Cologne, Germany.
11
Department of Pediatrics, Division of Pediatric Nephrology, University Hospital Bonn, Bonn, Germany.
12
Department of Pediatrics and Adolescent Medicine, University of Erlangen-Nürnberg (FAU), Erlangen, Germany.
13
Department of General Pediatrics and Hematology/Oncology, Children's University Hospital Tuebingen, Germany.
14
Institute of Pathology, University Hospital of Cologne, Cologne, Germany.
15
Research and Training Hospital, Division of Pediatric Nephrology, Marmara University, Istanbul, Turkey.
16
Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany.
17
Department of Pediatric Nephrology, Ali-Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran.
18
Clinic of Children Diseases, Institute of Clinical Medicine, Vilnius University, Vilnius, Lithuania.
19
KfH Center of Paediatric Nephrology, University Hospital of Marburg, Marburg, Germany.
20
Department of General Pediatrics, University Hospital Muenster, Muenster, Germany.
21
The Children's Memorial Health Institute, Warsaw, Poland.
22
Nephrology and Dialysis Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
23
Department of Pediatric Nephrology, University Hospitals Leuven, Leuven, Belgium; KU Leuven - University of Leuven, Department of Development and Regeneration, Laboratory of Pediatrics, PKD research group, B-3000 Leuven, Belgium.
24
Department of Pediatric Nephrology, Cukurova University Faculty of Medicine, Adana, Turkey.
25
Department of Pediatric Nephrology, Ege University Medical Faculty, Izmir, Turkey.
26
Department of Inherited and Acquired Kidney Diseases, Research Clinical Institute for Pediatrics, Pirogov Russian National Research Medical University, Moscow, Russia.
27
Pediatric Nephrology Unit, Hôpital Femme Mere Enfant, Hospices Civils de Lyon, Lyon, France.
28
University Children's Hospital, University Medical Center Hamburg Eppendorf, Hamburg, Germany.
29
Department of Pediatrics, University Hospital Motol, 2nd Faculty of Medicine, Charles University Prague, Prague, Czech Republic.
30
Department of Pediatric Nephrology, Cerrahpaşa School of Medicine, Istanbul University, Istanbul, Turkey.
31
Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.
32
Department of Pediatrics, Center of Pediatric Nephrology and Transplantation, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt.
33
First Department of Pediatrics, Hippokration Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece.
34
Department of Pediatrics, Immunology and Nephrology, Polish Mother's Memorial Hospital Research Institute, Lodz, Poland.
35
Department of Pediatric Nephrology, Faculty of Medicine, İnönü University, Malatya, Turkey.
36
Department of Paediatrics and Nephrology, Medical University of Bialystok, Bialystok, Poland.
37
Pediatric Nephrology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.
38
Department of Pediatric Nephrology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health, Berlin, Germany.
39
Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, University of Heidelberg, Heidelberg, Germany.
40
Clinic for Children and Adolescents, Hospital St. Georg, Leipzig, Germany.
41
Pediatric Nephrology Department, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
42
Department of Pediatric Nephrology and Hypertension, Faculty of Medicine, Jagiellonian University Medical College, Krakow, Poland.
43
Department of Nephrology and Hypertension of Children and Adolescents, Medical University of Gdansk, Gdansk, Poland.
44
Institute of Human Genetics, RWTH University Hospital Aachen, Aachen, Germany.
45
Department of Pediatrics, University Hospital of Cologne, Cologne, Germany; Center for Molecular Medicine, University Hospital of Cologne, Cologne, Germany.
46
Department of Pediatrics, Université Catholique de Louvain Medical School, Saint-Luc Academic Hospital, Brussels, Belgium.
47
Clinique de l'Espérance, Centre de référence de Néphrologie Pédiatrique Sud, Montegnee, Belgium.
48
Pediatric Nephrology Unit, University Children's Hospital, Zurich, Switzerland.
49
Department of Pediatric Nephrology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin Institute of Health, Germany.
50
Institute of Human Genetics, University Hospital of Cologne, Cologne, Germany.
51
Department II of Internal Medicine, University Hospital of Cologne, Cologne, Germany.
52
Department of Nephrology and Hypertension, University of Erlangen-Nürnberg, Erlangen, Germany.
53
Department of General Pediatrics, Adolescent Medicine and Neonatology, Freiburg University Hospital, Freiburg, Germany.
54
Clinic for Nephrology and Rheumatology, University Medical Center Goettingen, Goettingen, Germany.
55
Children's Hospital St. Elisabeth and St. Barbara, Halle, Germany.
56
Nephrology Clinic Hannover, Hannover, Germany.
57
Pediatric Nephrology, Center for Paediatric and Adolescent Medicine, University Medical Clinic, Mainz, Germany.
58
Department of Pediatrics, University Hospital Marburg, Marburg, Germany.
59
Dr. von Haunersches Kinderspital, Ludwigs Maximilian University, Munich, Germany.
60
KfH Center of Pediatric Nephrology, Children's Hospital Munich Schwabing, Munich, Germany.
61
Klinik für Kinder- und Jugendmedizin, Klinikum Dritter Orden, München, Germany.
62
Department of Pediatrics, Klinikum Saarbrücken, Saarbrücken, Germany.
63
Olga Children's Hospital, Clinic Stuttgart, Stuttgart, Germany.
64
Children's University Hospital, Department of General Pediatrics and Hematology/Oncology, Tuebingen, Germany.
65
Department of Pediatric Nephrology, Medical University of Lublin, Lublin, Poland.
66
Children's Hospital, Poznan, Poland.
67
Pediatric Nephrology, Centro Hospitalar São João, Porto, Portugal.
68
Department of Nephrology, University Children's Hospital, Belgrade, Serbia.
69
Department of Pediatric Nephrology, Faculty of Medicine, Cukurova University, Adana, Turkey.
70
Department of Pediatric Nephrology, Dokuz Eylul University Medical Faculty, Izmir, Turkey.
71
Division of Pediatric Nephrology, Istanbul Medeniyet University, Göztepe Hospital, Istanbul, Turkey.
72
Pediatric Nephrology Department, Faculty of Medicine, Istanbul University, Istanbul, Turkey.
73
Division of Pediatric Nephrology, Sisli Etfal Training and Research Hospital, Istanbul, Turkey.
74
Department of Pediatric Nephrology, Ege University, Medical Faculty, Izmir, Turkey.

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.

KEYWORDS:

PKHD1; ciliopathy; oligohydramnios; renal replacement therapy

Comment in

PMID:
29753540
DOI:
10.1016/j.jpeds.2018.03.052
[Indexed for MEDLINE]

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