Hemolytic uremic syndrome
Hemolytic uremic syndrome is a thrombotic microangiopathy defined by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury, most often triggered in children by Shiga toxin-producing Escherichia coli O157:H7 after bloody diarrhea. Diagnosis rests on schistocytes with a negative Coombs test plus a normal ADAMTS13 level to exclude TTP, and typical STEC-HUS is managed supportively while antibiotics and antimotility agents are avoided, whereas complement-mediated atypical HUS requires eculizumab.
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What this article covers
- Overview
Definition: hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy in which platelet-rich microthrombi form in small vessels, producing the triad of MAHA, thrombocytopenia, and acute kidney injury (AKI).
- Epidemiology
Predominantly a pediatric disease: classic diarrhea-associated (D+) HUS chiefly affects children, following ingestion of undercooked meat or other food contaminated with E.
- Risk factors
Ingestion of undercooked meat or contaminated food or water: the principal exposure for Shiga toxin-producing E.
- Pathophysiology
Toxin-mediated endothelial injury (typical HUS): Shiga-like toxin from E.
- Clinical features
Preceding bloody diarrhea (typical HUS): a diarrheal prodrome from Shiga toxin-producing E.
- Differential diagnosis
Thrombotic thrombocytopenic purpura: more prominent fever and fluctuating neurologic signs than renal failure, and ADAMTS13 below 10% (thrombotic thrombocytopenic purpura).
- Diagnosis
Establish the TMA triad, then confirm the mechanism: the diagnosis rests on MAHA (schistocytes, elevated LDH, decreased haptoglobin, elevated indirect bilirubin and reticulocyte count, negative…
- Management
Start with supportive care for typical (STEC) HUS, the mainstay of treatment: IV fluids and electrolyte replacement to manage volume status and correct derangements from the preceding diarrhea, with…
- Complications
Acute kidney injury progressing to dialysis-dependent renal failure: the dominant acute complication, particularly in adults and severe pediatric cases.
- Prognosis
Typical (D+) HUS in children carries the best prognosis of the group, with most recovering kidney function (see Epidemiology for figures); adults with D+HUS fare worse.
- Prevention
Food safety to prevent STEC exposure: thoroughly cook ground meat (no pink color, killing E.
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Related articles
- Thrombotic thrombocytopenic purpura (TTP)
Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy caused by severe ADAMTS13 deficiency that lets ultralarge von Willebrand factor multimers drive platelet microthrombi; the key dyad of microangiopathic hemolytic anemia plus thrombocytopenia without another cause mandates the PLASMIC score, an ADAMTS13 activity and inhibitor level, and emergent plasma exchange started before results return.
- Disseminated intravascular coagulation
Disseminated intravascular coagulation is a secondary, systemic activation of coagulation and fibrinolysis, most often from sepsis, that simultaneously consumes platelets and clotting factors, causing microvascular thrombosis and bleeding. Diagnosis rests on a laboratory constellation, thrombocytopenia, prolonged PT and aPTT, low fibrinogen, and elevated D-dimer, in a predisposing clinical setting, and management centers on treating the underlying trigger while replacing blood components only for active or high-risk bleeding.
- Clostridioides difficile infection
Clostridioides difficile infection is toxin-mediated colitis that follows disruption of normal colonic flora, most often by antibiotics, and ranges from watery diarrhea to fulminant colitis with shock, ileus, or megacolon. Diagnosis rests on unformed stool testing (NAAT and/or toxin EIA) in a patient with three or more unformed stools in 24 hours, and treatment is chosen by IDSA/SHEA severity grade, oral vancomycin or fidaxomicin for 10 days, escalating to high-dose vancomycin plus IV metronidazole for fulminant disease.
- Escherichia coli infection
Escherichia coli is a gram-negative bacillus that causes most urinary tract infections and catheter-associated bloodstream infections, plus distinct diarrheagenic syndromes including travelers' diarrhea (ETEC) and hemorrhagic colitis with hemolytic uremic syndrome (EHEC/STEC). Diagnosis rests on culture with syndrome-specific colony-count thresholds, and management ranges from short-course oral antibiotics for cystitis to strictly supportive, antibiotic-avoiding care for EHEC.
- Acute kidney injury
Acute kidney injury is a sudden rise in serum creatinine or fall in urine output from prerenal, intrinsic, or postrenal causes, defined and staged by KDIGO criteria. Management targets the underlying cause, corrects volume and electrolyte derangements, and escalates to renal replacement therapy for refractory hyperkalemia, acidosis, volume overload, or uremia.
- HELLP syndrome
HELLP syndrome is a thrombotic microangiopathy of pregnancy, a severe-feature variant of preeclampsia defined by hemolysis, transaminases at least twice the upper limit of normal, and a platelet count under 100,000/uL. Delivery is the only curative treatment; management pairs blood pressure control and magnesium sulfate seizure prophylaxis with prompt, gestational-age-guided delivery.
- Preeclampsia
New-onset hypertension after 20 weeks of gestation with proteinuria or end-organ dysfunction, driven by abnormal placental spiral artery remodeling and widespread endothelial dysfunction. Eclampsia is preeclampsia complicated by seizures; magnesium sulfate treats and prevents seizures, and prompt delivery is the only definitive cure for both.
- Sepsis
Sepsis is life-threatening organ dysfunction caused by a dysregulated host response to infection, identified by an acute rise in SOFA score of 2 points or more in a patient with known or suspected infection. Survival depends on recognizing the pattern early and completing the hour-1 bundle: lactate, blood cultures before antibiotics, broad-spectrum antibiotics such as cefepime 2 g IV every 8 hours, and 30 mL/kg crystalloid for hypotension or a lactate of 4 mmol/L or higher.