Yibian
 Shen Yaozi 
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diseaseHemolytic Uremic Syndrome in Children
aliasHemolytic Uremic Syndrome, HUS
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bubble_chart Overview

Hemolytic uremic syndrome (HUS) is a syndrome characterized by acute microvascular hemolytic anemia, thrombocytopenia, and acute renal failure, and is one of the main causes of acute renal failure in children, especially infants. Its etiology and pathogenesis have not yet been clarified, but it is often associated with infections, with a history of acute respiratory or gastrointestinal infections prior to the disease. There is a familial predisposition to the disease.

bubble_chart Clinical Manifestations

This condition can affect all age groups, especially infants and young children. It should be considered when hemolytic anemia, thrombocytopenia, and acute kidney injury occur after a prodromal stage dominated by gastrointestinal symptoms. The prodromal period lasts from 1 to 20 days (usually 6-7 days), mainly presenting with gastrointestinal symptoms such as vomiting, diarrhea (which may be bloody stool), and abdominal pain. A small number of children exhibit respiratory symptoms. A few days after the prodromal stage, acute onset occurs, primarily manifesting as hemolytic anemia and pallor, accompanied by symptoms of acute kidney failure, such as hypertension, oliguria, proteinuria, hematuria, azotemia, and metabolic acidosis. Bleeding mainly occurs in the gastrointestinal tract, with few cases of skin petechiae. Due to anemia, hypervolemia, and hypertension, children often experience heart failure. Additionally, neurological symptoms may include drowsiness and irritability, progressing to spasm and unconsciousness in severe cases. Some children may experience occasional relapses or recurrences during the convalescence stage.

bubble_chart Auxiliary Examination

  1. Peripheral blood examination: anemia, fragmented red blood cells on peripheral blood smear, increased reticulocytes, often elevated white blood cells, left shift of neutrophil nuclei, and thrombocytopenia.
  2. Urinalysis: hematuria, proteinuria, and cylindruria; hemoglobinuria may occur in severe cases of hemolysis.
  3. Blood generation and transformation examination: changes consistent with acute kidney failure.

bubble_chart Treatment Measures

  1. primarily deals with acute kidney failure. In recent years, there has been a tendency to initiate peritoneal dialysis or hemodialysis early, which is beneficial for improving prognosis. If the fluid overload is severe, arteriovenous hemofiltration can also be performed.
  2. Effectively control hypertension.
  3. Severe anemia, hemoglobin <50g/L時應及時輸血,以輸注新鮮紅細胞為宜,用量為2.5~5.Oml/kg。有條件時還可輸注血小板。
  4. In recent years, some have used prostaglandin E2 (PGE2) infusion, with a treatment course of 10 days. The use of high doses of vitamin E has also been reported to be effective.

bubble_chart Differentiation

  1. First, it should be differentiated from thrombotic thrombocytopenic purpura (TTP). TTP is similar to hemolytic uremic syndrome and easily confused, but TTP is more likely to occur in young and middle-aged individuals, with fever and central nervous system symptoms being more prominent, and skin bleeding also being more common.
  2. Hemolytic uremic syndrome can also be seen in systemic lupus erythematosus, polyarteritis nodosa, Sjögren's syndrome, rheumatoid arthritis, malignant hypertension, or rejection after kidney transplantation, so attention should be paid to the presence of primary diseases or predisposing factors.

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