An Atypical Case of Typical Hemolytic Uremic Syndrome (HUS)
- AKI: Mechanisms - Case Reports
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 103 AKI: Mechanisms
- Massoud, Mark N., University of Utah Health, Salt Lake City, Utah, United States
- Sehmbey, Gurbir S., University of Utah Health, Salt Lake City, Utah, United States
- Abraham, Josephine, University of Utah Health, Salt Lake City, Utah, United States
Typical hemolytic uremic syndrome (HUS) is a rare cause of thrombotic microangiopathy in adults. Predominantly a pediatric disease, typical HUS is caused by Shiga toxin producing Escherichia Coli. We present the case of an adult with typical HUS.
A 60 years old woman with history of type 2 Diabetes Mellitus and no kidney disease was admitted to the ICU with a 5 day history of nausea, vomiting and bloody diarrhea. Her workup revealed colitis with sepsis, AKI, and thrombocytopenia. Platelet count was 61,000/mL and plummeted to 33,000/mL during the course. Serum creatinine on admission was 2.8 mg/dL and peaked to 6.0 mg/dL with progression to anuric renal failure over 3 days. Hemoglobin dropped from 16.9 g/dL on admission to a nadir of 6.6 mg/dL over 6 days requiring RBC transfusion. Features of non immune hemolytic anemia were detected including very low haptoglobin level (<1 mg/dL), elevated LDH level of 2301 IU/L and negative Coomb's test. Peripheral smear was notable for schistocytes. DIC panel with coagulation studies and fibrinogen levels were normal.
The patient received IVF resuscitation and was started on antibiotics with IV ceftriaxone and metronidazole. Shortly after admission she developed seizure and required intubation. She received hemodialysis for renal failure. The combination of MAHA, thrombocytopenia and AKI warranted consideration for TTP. ADAMTS 13 level was ordered and the patient was started empirically on plasma exchange pending ADAMTS13 level. Urine microscopy showed extensive granular casts. ADAMTS 13 came back normal (45%) and plasma exchange was discontinued. Shiga toxin-producing E Coli PCR was positive in the stools. Renal function improved and dialysis discontinued after 3 sessions. Thrombocytopenia completely resolved with improvement in anemia and a creatinine at discharge of 3.42 mg/dL.
Typical HUS caused by shiga-like toxin producing enteric organisms is most commonly encountered in children but can affect all ages and should be suspected when a patient presents with bloody diarrhea, MAHA, thrombocytopenia, AKI and neurological symptoms. The differential should always include TTP and empiric plasma exchange should be pursued while awaiting ADAMTS 13 levels as delaying treatment for TTP could be devastating. It is important to note that AKI, especially severe, is not a prominent feature of TTP and should warrant consideration of alternative diagnosis.