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Abstract: FR-PO325

An Atypical Case of Fungal Cyst Infection in ADPKD

Session Information

Category: Genetic Diseases of the Kidney

  • 801 Cystic Kidney Diseases

Authors

  • Onuchic, Laura, University of Sao Paulo, Sao Paulo, Brazil
  • Portela Neto, Antonio Abel, University of Sao Paulo, Sao Paulo, Brazil
  • Ferreira, Fernanda Trani, University of Sao Paulo, Sao Paulo, Brazil
  • Testagrossa, Leonardo A., University of Sao Paulo, Sao Paulo, Brazil
  • Watanabe, Elieser H., University of Sao Paulo, Sao Paulo, Brazil
  • Balbo, Bruno E., University of Sao Paulo, Sao Paulo, Brazil
  • Onuchic, Luiz F., University of Sao Paulo, Sao Paulo, Brazil
Background

Cyst infection is a significant cause of mortality in autosomal dominant polycystic kidney disease (ADPKD). It is typically associated with gram-negative bacteria and is most often related to the ascending urinary tract route. Fungal etiology is rare, with very few reports. Positron emission tomography/computed tomography (PET-CT) has become the most sensitive imaging technique to diagnose cyst infection.

Methods

A 34-year-old female with ADPKD was referred for persistent fever and malaise. She reported vaginal candidiasis 2 months prior to admission. Following right kidney obstruction secondary to ureteral calculus, she was submitted to double-J stent placement. After 2 days she developed fever and abdominal pain. Urine and blood cultures were negative; WBC count and C-reactive protein, however, were elevated. Initial CT scan was inconclusive for cyst infection. At this point she received broad-spectrum antibiotics, including ciprofloxacin. Despite treatment, the patient presented no clinical improvement and developed acute kidney injury, with a rise in serum creatinine from 0.38 to 2.3 mg/dL. She was then transferred to our referral center. In this scenario, PET-CT revealed high 18F-glucose uptake in multiple right kidney cysts. Ultrasound-guided percutaneous drainage of the dominant suspected cyst and culture of its material led to the diagnosis of Candida albicans infection. Histopathology analysis confirmed this finding, showing cystic and pericystic hypha and pseudohypha invasion. She was then started on fluconazole, but this therapy was not effective. Given the refractoriness to this treatment, she was submitted to right nephrectomy, which led to initiation of hemodialysis. Prolonged hospitalization led to urinary infection by carbapenemase-resistant Klebsiella pneumoniae followed by sepsis. In this setting, the patient was submitted to left nephectomy, which resulted in clinical improvement and hospital discharge.

Conclusion

We report a case of ascendant fungal cyst infection, a very unusual form of this complication in ADPKD. Antibiotic treatment algorithms targeting cyst infection do not contemplate antifungal therapy as an initial approach. This case underscores, however, the importance of considering such an etiology and the need of culturing cyst material in refractory cyst infections.

Funding

  • Government Support - Non-U.S.