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Kidney Week

Abstract: TH-PO866

Neurological Complications After Very Early Bilateral Nephrectomies in Patients with Autosomal Recessive Polycystic Kidney Disease (ARPKD)

Session Information

Category: Genetic Diseases of the Kidneys

  • 1001 Genetic Diseases of the Kidneys: Cystic

Authors

  • Burgmaier, Kathrin, University Hospital Cologne, Cologne, Germany
  • Büscher, Anja K., University of Duisburg-Essen, Pediatrics 2, Essen, Germany
  • Pape, Lars, Hannover Medical School, Hannover, Germany
  • Shroff, Rukshana, Department of Nephrology, London, United Kingdom
  • Szczepanska, Maria, SMDZ in Zabrze, SUM in Katowice, Zabrze, Poland
  • Wuehl, Elke, University of Heidelberg, Heidelberg, Germany
  • Schaefer, Franz S., University of Heidelberg, Heidelberg, Germany
  • Liebau, Max, University Hospital Of Cologne; Children's Hospital, Koeln, Germany
Background

Autosomal recessive polycystic kidney disease (ARPKD) is a pediatric disorder with pronounced phenotypic variability. Severely affected patients may undergo bilateral nephrectomies in the first months of life. The neurological outcome of these patients has not been studied in defined cohorts.

Methods

Within the international registry study ARegPKD 18 patients with very early (≤3 months of age at second nephrectomy, VEBNE) and 9 with early (3-15 months, EBNE) bilateral nephrectomies as well as 13 patients with very early dialysis onset (≤3 months, VED) not receiving bilateral nephrectomies were identified. Eleven patients with total kidney volumes (TKV) comparable to the TKV of VEBNE patients but without bilateral nephrectomies served as an additional control group. Descriptive statistics, multivariate and Kaplan-Meier analyses evaluated the neurological outcome and potential risk factors of the cohorts.

Results

Mean (SD) follow-up time ranged from 1.6(2.6) years for VED patients to 7.8(5.9) years for EBNE patients. VEBNE patients suffered more frequently from seizures (67%) and severe neurological complications (61%) in comparison to EBNE patients (seizures 11%, severe neurological complications 11%), VED patients (seizures 31%, severe neurological complications 15%) and patients of the TKV control group (seizures 27%, severe neurological complications 0%). In total, 5/51 (10%) patients suffered from severe hypotensive episodes. Multivariate Cox regression analysis revealed the report of a severe hypotensive episode as well as very early bilateral nephrectomies to be independent risk factors for severe neurological complications. Mortality, including mortality after palliative care, was highest among patients with very early onset of dialysis without bilateral nephrectomies.

Conclusion

Bilateral nephrectomies within the first three months of life may be associated with severe neurological sequelae. The indication for very early bilateral nephrectomies must be carefully considered.

Funding

  • Private Foundation Support