Kidney Week

Abstract: FR-PO308

Identifying ARPKD Patients at Risk for Dialysis in the First Year of Life – Data from the International Registry Study ARegPKD

Session Information

• Cystic Kidney Diseases - II
  November 03, 2017 | Location: Hall H, Morial Convention Center
  Abstract Time: 10:00 AM - 10:00 AM

Category: Genetic Diseases of the Kidney

• 801 Cystic Kidney Diseases

Authors

• Burgmaier, Kathrin, University Hospital Cologne, Cologne, Germany

Kathrin Burgmaier,

• Dursun, Ismail, Erciyes University, Kayseri, Turkey

Ismail Dursun,

• Kunzmann, Kevin, University of Heidelberg, Heidelberg, Germany

Kevin Kunzmann,

• Ranchin, Bruno, Université de Lyon, Lyon, France

Bruno Ranchin,

• Shroff, Rukshana, Department of Nephrology, London, United Kingdom

Rukshana Shroff,

• Wicher, Dorota, The Children's Memorial Health Institute, Department of Medical Genetics, Warsaw, Poland

Dorota Wicher,

• Wuehl, Elke, University of Heidelberg, Heidelberg, Germany

Elke Wuehl,

• Schaefer, Franz S., University of Heidelberg, Heidelberg, Germany

Franz S. Schaefer,

• Liebau, Max, University Hospital Cologne, Cologne, Germany

Max Liebau,

Group or Team Name

• For ARegPKD consortium

Background

Autosomal recessive polycystic kidney disease (ARPKD) is a severe disease of early childhood and an important reason for renal replacement therapy in the first year of life. Yet, ARPKD shows pronounced phenotypic variability, making pre- and perinatal counseling challenging. Risk markers for the need of dialysis in the first year have not been established for ARPKD patients.

Methods

We studied the clinical courses of 385 patients from 18 countries included in the international ARPKD registry study ARegPKD using the time-to-event endpoint `start of renal replacement therapy` and multivariate Cox regression.

Results

36 patients started dialysis in the first year of life (median age at start of dialysis 0.14 years (0.00-0.91 years)). 30 children started peritoneal dialysis (PD), no patient underwent transplantation. Four patients deceased postnatally due to respiratory failure without onset of dialysis. Oligo/anhydramnios, enlarged prenatal kidney volume, high standardized birth weight and low 10-min APGAR score were associated with an increased risk of dialysis need. Perinatal assisted breathing was associated with a markedly increased hazard ratio in the first 6 months of life but not thereafter.

Conclusion

The identification of indicators for the need of dialysis within the first year of life in ARPKD patients may allow predictive scoring to inform prenatal counseling.

Funding

• Private Foundation Support