Severe AKI due to Cortical Necrosis in Postpartum Patient
- AKI: Biomarkers, Imaging, Interventions
November 04, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
- Choudhury, Mushfique, Dartmouth College Geisel School of Medicine, Hanover, New Hampshire, United States
- Trio, Pamela Summers, Dartmouth College Geisel School of Medicine, Hanover, New Hampshire, United States
- Block, Clay A., Dartmouth College Geisel School of Medicine, Hanover, New Hampshire, United States
Severe post-partum AKI is rare in the United States, but has a challenging differential diagnosis that often includes atypical hemolytic uremic syndrome (aHUS) , ATN and acute cortical necrosis (ACN). Establishing the diagnosis is critical to provide specific therapy .We present a case of aHUS in a post-partum patient in whom the diagnosis of aHUS was confounded by simultaneous influenza, peri-partum hemorrhage, and microangiopathic hemolysis, elevated liver enzymes, and HELLP syndrome, proteinuria, cardiomyopathy, and endometritis.
A 33-year-old woman was hospitalized for hypovolemic shock and coagulopathy following term, vaginal delivery complicated by severe hemorrhage..She received RBC transfusion besides vasopressor support for several hours. Oligo-anuric kidney failure ensued requiring dialysis support. Echo showed severe global left ventricular dysfunction. CT with contrast was consistent with cortical necrosis of kidney. Platelet count of 63,000/uL, high LDH, low haptoglobin, and presence of schistocytes were consistent with microangiopathy. C3 and C4 and ADAMTS13 activity were normal. Liver chemistries normalized soon . UPR was elevated at 2.1. Empirical therapy for aHUS with Eculizumab was initiated with rapid improvement in platelet count and urine output but dialysis dependence persisted. Kidney biopsy performed that demonstrated cortical necrosis and could not confirm or reject a diagnosis of aHUS. Functional complement assays were nondiagnostic. Genetic profile was negative for mutations .Because of her clinical response, Eculizumab was maintained for 6-months. Unfortunately, patient remained dialysis dependent.
Post-partum AKI can occur by a variety of mechanisms with variable prognosis and management. Eculizumab is specific treatment for aHUS but confers risk for infection and is expensive. Treatment of ATN or ACN is supportive, Alternative complement pathway functional and genetic profiles can facilitate diagnosis but results are delayed and may be non-informative as in this case. Kidney biopsy demonstrated cortical necrosis but patchy injury . ACN might have been demonstrable by CT or MRI by showing meduallary enhancement in the absence of cortical enhancement (“reverse rim sign”) but were not obtained. Although aHUS was suspected based on hematologic and biochemical data, ACN appears to have been the dominant process.