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논문분류	춘계학술대회 초록집
제목	Atypical Case of Thrombotic Microangiopathy in Pregnancy
저자	Tae Gyoung Kim
출판정보	2025; 2025(1):
키워드	acute renal failure, hemolytic uremic syndrome
초록	Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. It is classified into typical HUS, associated with Shiga toxin-producing Escherichia coli (STEC), and atypical HUS (aHUS), caused by complement dysregulation, often due to genetic mutations. While typical HUS is usually self-limiting, aHUS can progress to renal failure, requiring early diagnosis and targeted therapy due to its high risk of chronic kidney disease, cardiovascular complications, and mortality. We report a 38-year-old woman at 32 weeks of gestation admitted with eclampsia who underwent an emergency cesarean section. On admission, laboratory findings revealed serum creatinine 10.59 mg/dL, BUN 71.0 mg/dL, nephrotic-range proteinuria, hemoglobin 6.0 g/dL, platelet count 71,000/µL, schistocytes on peripheral smear, and D-dimer >20, consistent with TMA. Despite delivery, renal function did not recover, necessitating continuous renal replacement therapy (CRRT). After acidosis resolved, persistent azotemia required maintenance hemodialysis. A renal biopsy confirmed HUS, and STEC was detected, leading to denial of eculizumab since it is restricted to aHUS. However, TMA persisted without renal recovery, raising suspicion for aHUS. Echocardiography revealed reduced ejection fraction, regional wall motion abnormalities, and moderate mitral regurgitation, indicating cardiac involvement. Genetic testing identified a CFH mutation (NM_000186.4:c.3644G>A). The presence of STEC without gastrointestinal symptoms suggests she might have been an asymptomatic carrier. This case highlights the importance of timely genetic evaluation in persistent postpartum TMA to ensure appropriate management and avoid missing aHUS in complex presentations.
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