A 6-year-old boy is brought in due to pallor and decreased energy. For the past 2 days, he has slept throughout the day. The patient developed fever, emesis, and diarrhea 10 days ago, but symptoms resolved after a few days without intervention. Temperature is 37.1 C (98.8 F), blood pressure is 120/70 mm Hg, and pulse is 145/min. The patient appears tired and has pale lips. The abdomen is soft and nontender. There are a few small bruises along the bilateral shins. Laboratory results are as follows:
一个 6 岁的男孩因面色苍白和精力下降被带来就诊。在过去的 2 天里，他整天都在睡觉。患者在 10 天前出现发热、呕吐和腹泻，但症状在几天后自行缓解，没有进行干预。体温为 37.1°C（98.8°F），血压为 120/70 mm Hg，脉搏为 145 次/分钟。患者看起来很疲惫，嘴唇苍白。腹部柔软且无压痛。双侧小腿有几处小淤伤。实验室结果如下：
| Complete blood count 全血细胞计数 | |
| Hemoglobin 血红蛋白 | 6.4 g/dL (64 g/L) |
| Platelets 血小板 | 30,000/mm3 (30 × 109/L) 30,000/mm3 (30 × 10^9/L) |
| Leukocytes 白细胞 | 15,200/mm3 (15.2 × 109/L) 15,200/mm3 (15.2 × 10^9/L) |
| Serum chemistry 血清化学 | |
| Creatinine 肌酐 | 2.4 mg/dL (212.2 μmol/L) |
| Liver function studies 肝功能研究 | |
| Total bilirubin 总胆红素 | 3.3 mg/dL (56.4 μmol/L) |
| Direct bilirubin 直接胆红素 | 0.1 mg/dL (1.71 μmol/L) |
| Coagulation studies 凝血研究 | |
| PT | 12 sec 12 秒 |
| Activated PTT 活化部分凝血酶时间 | 35 sec 35 秒 |
Urinalysis has 20-30 red blood cells/hpf. Which of the following is the most likely diagnosis?
尿液分析显示每高倍视野有 20-30 个红细胞。以下哪项最可能是诊断结果？
A. | Autoimmune hemolytic anemia 自身免疫性溶血性贫血 | |
B. | Disseminated intravascular coagulation 弥散性血管内凝血 | |
C. | Hemolytic uremic syndrome 溶血性尿毒症综合征 | |
D. | Immune thrombocytopenia 免疫性血小板减少症 | |
E. | Poststreptococcal glomerulonephritis 链球菌后肾小球肾炎 |
Hemolytic uremic syndrome | |
Pathogenesis 发病机制 |
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Clinical features 临床特征 |
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Laboratory findings 实验室检查结果 |
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Treatment 治疗 |
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BUN = blood urea nitrogen. | |
This patient's presentation is consistent with hemolytic uremic syndrome (HUS), which is characterized by the triad of hemolytic anemia, thrombocytopenia, and acute kidney injury. Most commonly seen in children, HUS usually occurs due to Shiga toxin–producing diarrheagenic pathogens such as Escherichia coli O157:H7 or, less commonly, Shigella.
该患者的表现与溶血性尿毒症综合征（HUS）一致，其特征为溶血性贫血、血小板减少和急性肾损伤的三联症。HUS 最常见于儿童，通常是由于产生志贺毒素的腹泻病原体引起，如大肠杆菌 O157:H7 或较少见的志贺氏菌。
Children with HUS often have fatigue, pallor, and bruising following resolution of a diarrheal (possibly bloody) illness. Laboratory findings include thrombocytopenia and anemia with evidence of microangiopathic hemolysis (eg, elevated indirect bilirubin, schistocytes). Acute kidney injury, due to microthrombotic renal vascular obstruction, is manifested by elevated creatinine and may present with hematuria, proteinuria, oliguria/anuria, and secondary hypertension.
患有溶血尿毒症综合症（HUS）的儿童通常在腹泻（可能伴有血性）疾病缓解后出现疲劳、面色苍白和淤血。实验室检查结果包括血小板减少和伴有微血管性溶血证据的贫血（例如，间接胆红素升高、裂形红细胞）。由于微血栓性肾血管阻塞引起的急性肾损伤表现为肌酐升高，可能伴有血尿、蛋白尿、少尿/无尿和继发性高血压。
Management of HUS is supportive and includes correction of fluid/electrolyte disturbances and treatment of hypertension. Profound anemia requires transfusion, and dialysis is indicated for severe kidney injury (eg, anuria). HUS improves in most patients within 2-3 weeks.
HUS 的管理是支持性的，包括纠正液体/电解质紊乱和治疗高血压。严重贫血需要输血，严重肾损伤（例如，无尿）时需要透析。大多数患者在 2-3 周内 HUS 会改善。
(Choice A) Autoimmune hemolytic anemia typically presents with isolated anemia due to autoantibodies causing red blood cell destruction. Acute kidney injury is not seen.
（选项 A）自身免疫性溶血性贫血通常表现为由于自身抗体导致红细胞破坏而引起的孤立性贫血。未见急性肾损伤。
(Choice B) Disseminated intravascular coagulation manifests in severely ill patients with anemia, thrombocytopenia, and abnormal coagulation studies. It is unlikely in this patient with normal PT and activated PTT.
（选择 B）弥散性血管内凝血在重病患者中表现为贫血、血小板减少和异常的凝血检查。在这位 PT 和活化 PTT 正常的患者中不太可能发生。
(Choice D) Immune thrombocytopenia presents with isolated thrombocytopenia, often after a viral infection. Anemia and acute kidney injury are not seen.
（选项 D）免疫性血小板减少症表现为孤立性血小板减少，通常在病毒感染后发生。未见贫血和急性肾损伤。
(Choice E) Poststreptococcal glomerulonephritis causes hematuria, hypertension, and acute kidney injury. Thrombocytopenia and anemia are uncommon, and preceding symptoms include group A Streptococcus skin or throat infection, not diarrhea.
（选项 E）链球菌后肾小球肾炎会导致血尿、高血压和急性肾损伤。血小板减少和贫血并不常见，前期症状包括 A 组链球菌皮肤或喉咙感染，而不是腹泻。
Educational objective: 教育目标：
Hemolytic uremic syndrome is characterized by the triad of hemolytic anemia, thrombocytopenia, and acute kidney injury. The presentation typically occurs after resolution of prodromal diarrhea caused by Escherichia coli O157:H7 or, less commonly, Shigella.
溶血性尿毒症综合症的特征是溶血性贫血、血小板减少和急性肾损伤的三联症。通常在由大肠杆菌 O157:H7 或较少见的志贺菌引起的前驱性腹泻缓解后出现。