ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: PO2018

Occurrence of Nephrogenic Systemic Fibrosis with Group II Gadolinium-Based Contrast Agent in a Pediatric Oncology Patient with AKI

Session Information

Category: Pediatric Nephrology

  • 1700 Pediatric Nephrology

Authors

  • Hanna, Christian, Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Scheuermann, Amanda, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Hopp, Amanda M., Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Young, Kara, Medical College of Wisconsin, Milwaukee, Wisconsin, United States
  • Dillon, John J., Mayo Clinic Minnesota, Rochester, Minnesota, United States
  • Van Why, Scott K., Medical College of Wisconsin, Milwaukee, Wisconsin, United States
Introduction

Nephrogenic Systemic Fibrosis (NSF) is a rare systemic disorder occurring in patients with chronic kidney disease (CKD) stage IV or V, end-stage renal disease (ESRD), or acute kidney injury (AKI). It is triggered by gadolinium-based contrast agents (GBCAs) and characterized by sclerodermic skin changes from fibrosis and internal organ damage. Almost all NSF cases are associated with group I GBCAs, and only extremely rare, unconfounded cases are reported with group II agent exposure including none in children. We present a case of a female child with acute myelogenous leukemia (AML) in remission and AKI on hemodialysis who presented with NSF six weeks following a magnetic resonance imaging (MRI) with group II GBCA.

Case Description

A 10-year-old female with intermediate risk AML in remission, complicated by prolonged neutropenia with colitis, invasive fungal sinus and pulmonary infection, and AKI with a renal biopsy-proven acute tubular necrosis on intermittent hemodialysis three times a week presented to the dermatology clinic for evaluation of progressive hardening of her skin. Dermatological examination revealed diffuse, indurated, and compressible plaques involving the lower back, buttocks, posterior thighs, and lateroposterior aspects of the arms. A skin biopsy showed findings consistent with NSF. Six weeks prior to her presentation, she underwent an MRI of the brain and orbits for exotropia evaluation and received intravenous gadobutrol injection, a group II GBCA. Treatment with photopheresis twice weekly over a 2-month period resulted in a gradual improvement of her condition.

Discussion

Our case of group II GBCAs induced NSF is exceptionally rare, with no unconfounded cases in pediatrics from group II GBCAs exposure reported to date. While NSF has been reported rarely in children who received group I GBCAs, the risk of NSF in children exposed to group II or even group III GBCAs is unknown. We strongly recommend that physicians continue kidney function screening prior to group II GBCAs administration in children and carefully evaluate the risk versus benefit of using or withholding group II GBCAs for clinically indicated MRIs in patients with CKD stage IV/V, ESRD, or AKI.