Klin Padiatr
DOI: 10.1055/a-2373-0785
Originalarbeit/Original Article

Distinguishing Features of Childhood Renal Dysplasia

1   Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey
,
Zeynep Birsin Özçakar
1   Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey
,
Nilgun Cakar
1   Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey
,
Burcu Biral Coşkun
1   Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey
,
Beyza Doğanay
2   Biostatistics, Ankara University School of Medicine, Ankara, Turkey
,
Fatos Yalcinkaya
1   Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey
› Author Affiliations

Abstract

Background Renal dysplasia (RD) is a major cause of chronic kidney disease (CKD) in childhood. Herein, we present a single-center experience about distinctive features of different subtypes of dysplasia.

Methods All children with RD admitted between January 2018 and June 2019 were included in this cross-sectional study. Patients were classified as having unilateral RD, bilateral RD, posterior urethral valves (PUV), and multicystic-dysplastic kidneys (MCDK).

Results A total of 198 patients (108 unilateral RD, 31 bilateral RD, 16 PUV, and 43 MCDK) with a follow-up period of 80 (1.5–240) months were included. The overall consanguinity rate was 22%, whereas it was 56% in bilateral RD patients. Two-thirds had additional urogenital anomalies. Hypertension was detected in 16% of the patients, and 20% had median proteinuria of 16 (5.2–173) mg/m2/h, which was significantly higher in the PUV and bilateral RD groups. The overall rate of CKD was 23%, which was more frequent in bilateral RD and PUV groups. The median age at end-stage renal disease was 91.5 (0.5–208) months, including 12 renal transplant patients.

Conclusions As different subtypes of RD have distinct clinical and prognostic features, the follow-up of RD patients should be individualized, and modifiable complications such as proteinuria and HT should be monitored closely.

Zusammenfassung

Hintergrund Die renale Dysplasie (RD) ist eine bedeutende Ursache für chronische Nierenerkrankungen (CKD) im Kindesalter. Hier präsentieren wir eine Einrichtungserfahrung aus einem einzelnen Zentrum zu den charakteristischen Merkmalen verschiedener Subtypen von Dysplasie.

Methoden Alle Kinder mit RD, die zwischen Januar 2018 und Juni 2019 aufgenommen wurden, wurden in diese Querschnittsstudie einbezogen. Die Patienten wurden als unilaterale RD, bilaterale RD, posteriore Harnröhrenklappen (PUV) und multizystisch-dysplastische Nieren (MCDK) klassifiziert.

Ergebnisse Insgesamt wurden 198 Patienten (108 unilaterale RD, 31 bilaterale RD, 16 PUV, 43 MCDK) mit einer Nachbeobachtungszeit von 80 (1,5–240) Monaten eingeschlossen. Die Gesamtkonsanguinitätsrate betrug 22%, während sie bei Patienten mit bilateraler RD 56% betrug. Zwei Drittel wiesen zusätzliche urogenitale Anomalien auf. Hypertonie wurde bei 16% der Patienten festgestellt und 20% wiesen eine mittlere Proteinurie von 16 (5,2–173) mg/m2/h auf, die in den PUV- und bilateralen RD-Gruppen signifikant höher war. Die Gesamt-CKD-Rate betrug 23%, was häufiger in den Gruppen mit bilateraler RD und PUV auftrat. Das mittlere Alter bei terminalem Nierenversagen betrug 91,5 (0,5–208) Monate, einschließlich 12 Patienten mit Nierentransplantation.

Schlussfolgerung Da verschiedene Subtypen von RD unterschiedliche klinische und prognostische Merkmale aufweisen, sollte das Follow-up von RD-Patienten individualisiert werden und modifizierbare Komplikationen wie Proteinurie und Hypertonie sollten genau überwacht werden.



Publication History

Article published online:
20 September 2024

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