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Chronic renal disorder
0%
0/21
Gastrointestinal arterio-venous malformation
Poor dietary nutrition intake
Inheritance of a beta-globin gene variant
5%
1/21
Toxin-mediated damage to endothelial cells and prothrombotic changes
90%
19/21
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This patient's clinical presentation is consistent with hemolytic uremic syndrome (HUS), which is primarily caused by Shiga-toxin producing E. coli O157:H7. Shiga-toxin binds to Gb3 receptors on endothelium, inhibiting protein synthesis, which leads to damage and activation of the prothrombotic pathway. HUS is characterized by a triad of microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and renal failure. The classic prodrome is fever, abdominal pain, and bloody diarrhea 1-3 days following ingestion of a contaminated meat. Widespread microthrombi in the vasculature leads to thrombocytopenia and hemolysis of RBCs. Microthombi in the renal parenchyma also causes acute renal failure. Razzaq reviews HUS and notes that the most common cause of this syndrome is infection with Shiga-toxin producing E. coli O157:H7. In children presenting with acute renal failure, HUS is the most common etiology and should be ruled out early during the presentation. The most important aspect of management is close monitoring and management of fluid and electrolyte status. RBC transfusion may be warranted to correct the anemia. Currently, the use of platelet transfusion, plasmapheresis, antibiotics, steroids, or anti-thrombotic agents remains controversial. Kavanagh et al. review atypical HUS, which is not caused by an infectious etiology. Rather, mutations in the genes for complement regulators leads to excessive activation of the complement system in the microvasculature. Much like typical HUS, this leads to widespread microthrombi with development of MAHA, thrombocytopenia, and renal failure. The prognosis for atypical HUS is much poorer, however, with most patients developing end-stage renal failure. Figure A is a blood smear showing schistocytes, which represent damaged fragments of red blood cells. Incorrect Answers: Answers 1-3: The patient's peripheral blood smear findings of schistocytes point to a process of hemolytic anemia, which is inconsistent with anemia of chronic disease, an A-V fistula, or iron-deficiency anemia. Answer 4: Abdominal pain and hemolytic anemia can be seen in sickle cell disease, but the peripheral smear would demonstrate sickled RBCs.
5.0
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