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Abstract: SA-PO043

Diarrheal Dilemma: A Unique Cause of Atypical Hemolytic Uremic Syndrome

Session Information

Category: Acute Kidney Injury

  • 102 AKI: Clinical‚ Outcomes‚ and Trials

Authors

  • Bajaj, Arrsh, Mercy Health St Elizabeth Youngstown Hospital, Youngstown, Ohio, United States
  • Huang, Jin, Mercy Health St Elizabeth Youngstown Hospital, Youngstown, Ohio, United States
  • Negrete, Hilmer O., Mercy Health St Elizabeth Youngstown Hospital, Youngstown, Ohio, United States
Introduction

Thrombotic microangiopathies(TMA) comprise a variety of diseases ranging from ADAMTS13 deficiency, complement-mediated TMA, shiga-toxin mediated hemolytic uremic syndrome (STEC-HUS) and drug mediated TMA. Atypical HUS (aHUS) accounts for approximately 10% of HUS cases. Organisms implicated in aHUS include Streptococcus pneumoniae. Literature review demonstrates only ten adults with C. difficile associated aHUS (CD-aHUS). The case presented below would be the 11th known case.

Case Description

A 56-year-old female presented with abdominal pain, diarrhea and bright red blood per rectum. She recently had taken antibiotics for a dental procedure. Initial labs revealed a creatinine 0.9 mg/dL, WBC 30k, hemoglobin15.9 g/dL, platelets 200k. Imaging showed pancolitis. Further testing revealed negative stool cultures but positive EIA assay for CD. Her creatinine rapidly increased to 3.1. She also had new thrombocytopenia(18k), anemia(62g/dL) and urine sediment showed granular casts. Peripheral smear showed schistocytes. Lactate dehydrogenase (LDH) was 2131U/L, haptoglobin was <10 mg/dL. Complement and ADAMSTS13 levels were normal, anti-factor H antibodies were negative. Methylprednisolone 125mg daily for six days was started with daily PLEX (one volume exchange with fresh frozen plasma). Kidney biopsy showed diffuse global mesangiolysis, RBC fragmentation with scattered presence of luminal fibrin thrombi, all consistent with TMA and acute tubular injury. Her hospital course was complicated by right subcapsular kidney hematoma and COVID19 infection. She underwent eight PLEX sessions during her hospital stay. At the time of discharge, diarrhea had resolved, LDH and creatinine had reduced to 731 U/L and 1.7 mg/dL respectively. One month after discharge, the patient had completed 17 PLEX sessions and her serum creatinine had improved to 1.1 mg/dL. LDH was 297 U/L while haptoglobin continued to be <10 mg/dL, at that point was PLEX was discontinued.

Discussion

Atypical HUS is rare but has a high mortality rate, risk of progressing to end stage renal disease and relapse. About 40-60% of aHUS cases occur in individuals >18 years of age and has many triggers including CD. Literature review of CD-aHUS cases showed that out of ten patients, six underwent plasmapheresis with three patients using eculizumab. This case report highlights the efficacy of plasmapheresis as a treatment modality for CD-aHUS.