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🧸 儿科学 · 第3课，共5课 · 7 min

法洛四联症发作、主动脉缩窄与草莓舌

Tet Spells, Coarctation and the Strawberry Tongue

💙 一名发绀的婴儿把膝盖拉向胸口，肤色随之改善。他不是在卖萌。他是在治疗自己的心脏缺陷，考试要你帮助他这样做。

💙 A blue infant pulls his knees to his chest and the color improves. He is not being cute. He is treating his own heart defect, and the exam wants you to help him do it.

💡 核心要点

法洛四联症发作：膝胸位、给氧、安抚、morphine。主动脉缩窄：手臂高于腿部。Kawasaki病：发热5天、草莓舌、手部脱皮；用IVIG和aspirin，密切观察冠状动脉。

Tet spell: knee-chest, oxygen, calm, morphine. Coarctation: arms higher than legs. Kawasaki: 5 days of fever, strawberry tongue, peeling hands; IVIG and aspirin, watch the coronaries.

🧠 为什么重要

法洛四联症（TETRALOGY OF FALLOT，包括肺动脉狭窄、室间隔缺损、主动脉骑跨和右心室肥厚）会引起发绀；在高发绀性发作（TET SPELL）期间，将婴儿置于膝胸位（KNEE-CHEST，年龄较大的儿童则蹲下），给予氧气，安抚孩子，并遵医嘱给予morphine以减轻发作。蹲下可增加体循环阻力，推动血液流经肺部。主动脉缩窄（COARCTATION）：手臂的血压和脉搏高于腿部，股动脉脉搏微弱或消失；要测量四肢血压。伴左向右分流的非发绀型（ACYANOTIC）缺陷（室间隔缺损、房间隔缺损、动脉导管未闭）会引起心力衰竭征象：喂养困难、吃奶时出汗、生长发育不良和呼吸急促；用柔软的奶嘴少量多次喂养，并遵医嘱给予digoxin和利尿剂。Kawasaki病（KAWASAKI DISEASE）：发热5天或以上，并伴有至少4项主要特征（共5项）：结膜发红但无分泌物、草莓舌（STRAWBERRY TONGUE）和红唇皲裂、皮疹、手足肿胀且后期脱皮（PEELING），以及颈部淋巴结肿大。特征较少的儿童（不完全性Kawasaki病）可能仍需要评估和治疗。治疗为IV免疫球蛋白和aspirin（这是aspirin为数不多的儿科用途之一），并用超声心动图监测冠状动脉瘤；IVIG后活疫苗推迟11个月接种。风湿热（RHEUMATIC FEVER）继发于未经治疗的A组链球菌感染：表现为关节疼痛、心脏炎、舞蹈症和皮疹；预防方法是完成治疗链球菌性咽喉炎的整个抗生素疗程。

TETRALOGY OF FALLOT (pulmonary stenosis, ventricular septal defect, overriding aorta, right ventricular hypertrophy) causes cyanosis; during a hypercyanotic TET SPELL, place the infant in the KNEE-CHEST position (older children squat), give oxygen, calm the child, and give morphine as ordered to reduce the spell. Squatting increases systemic resistance and pushes blood through the lungs. COARCTATION OF THE AORTA: blood pressure and pulses are HIGHER in the arms than the legs, with weak or absent femoral pulses; check four-extremity blood pressures. ACYANOTIC defects with left-to-right shunts (ventricular and atrial septal defects, patent ductus arteriosus) cause heart failure signs: poor feeding, sweating with feeds, failure to thrive and tachypnea; feed small amounts frequently with a soft nipple and give digoxin and diuretics as ordered. KAWASAKI DISEASE: fever for 5 or more days plus at least 4 of these 5 principal features: conjunctival redness without discharge, a STRAWBERRY TONGUE and cracked red lips, a rash, swollen hands and feet with later PEELING, and swollen cervical nodes. Children with fewer features (incomplete Kawasaki) may still need evaluation and treatment. Treatment is IV immune globulin and aspirin (one of the few pediatric uses of aspirin), with echocardiograms to watch for coronary artery aneurysms; live vaccines are delayed for 11 months after IVIG. RHEUMATIC FEVER follows untreated group A strep infections: joint pain, carditis, chorea and a rash; prevention is finishing the full course of antibiotics for strep throat.

🏥 真实场景

🫀 一名患法洛四联症的4岁儿童在抽血时哭闹，变得面色发暗、呼吸困难。他一下子蹲到了地板上。护士没有把他拉起来；她陪在他身边，给他吸氧，让蹲位发挥作用，同时呼叫给予morphine。几分钟内他的肤色恢复了。孩子的本能正是教科书上的干预措施。

🫀 A 4-year-old with tetralogy of Fallot becomes dusky and breathless while crying over a blood draw. He drops into a squat on the floor. The nurse does not pull him up; she stays with him, applies oxygen and lets the squat do its work while she calls for morphine. Within minutes his color returns. The child's instinct was the textbook intervention.

📌 要点总结

  • 法洛四联症发作：膝胸位或蹲位、给氧、安抚、morphineTet spells: knee-chest or squatting, oxygen, calm, morphine
  • 主动脉缩窄：上肢血压高于下肢，股动脉脉搏微弱Coarctation: upper extremity pressure higher than lower, weak femoral pulses
  • Kawasaki病：IVIG和aspirin；冠状动脉瘤是危险所在Kawasaki: IVIG and aspirin; coronary aneurysms are the danger

📖 本课术语

法洛四联症缺氧发作 (Tet spell): 法洛四联症中突然的发绀发作，通过膝胸位和吸氧缓解。

Kawasaki病 (Kawasaki disease): 一种儿童疾病，伴五天发热、草莓舌和手部脱皮，用IVIG和aspirin治疗以保护冠状动脉。

PROVe（法洛四联症） (PROVe (tetralogy of Fallot)): Pulmonary stenosis（肺动脉狭窄）、Right ventricular hypertrophy（右心室肥厚）、Overriding aorta（主动脉骑跨）、Ventricular septal defect（室间隔缺损）：法洛四联症的四个缺陷，是法洛四联症发作背后的发绀型心脏缺陷。

✅ 自我测试

一名患法洛四联症的婴儿急性发绀并烦躁不安。护士应首先采取哪项行动？An infant with tetralogy of Fallot becomes acutely cyanotic and irritable. Which action should the nurse take first?
  1. 将婴儿仰卧并伸直双腿Place the infant supine with legs extended
  2. 准备立即手术Prepare for immediate surgery
  3. 给一瓶配方奶安抚婴儿Offer a bottle of formula to calm the infant
  4. 将婴儿置于膝胸位Place the infant in a knee-chest position

答案: D · 将婴儿置于膝胸位

膝胸位可增加体循环血管阻力，使更多血液流向肺部，从而缓解发作；同时给予氧气。安抚婴儿有帮助，但在发绀发作期间喂奶会增加氧需求，对将血流重新导向肺部毫无作用。

The knee-chest position increases systemic vascular resistance, directing more blood to the lungs and relieving the spell; oxygen is given at the same time. Calming the infant helps, but offering a bottle during a cyanotic spell increases oxygen demand and does nothing to redirect blood flow to the lungs.

哪项评估发现提示婴儿患有主动脉缩窄？Which assessment finding suggests coarctation of the aorta in an infant?
  1. 股动脉脉搏洪大Bounding femoral pulses
  2. 股动脉脉搏微弱或消失Weak or absent femoral pulses
  3. 哭闹时改善的发绀Cyanosis that improves with crying
  4. 四肢血压相等Equal four-extremity blood pressures

答案: B · 股动脉脉搏微弱或消失

手臂血管远端的狭窄使上身血压升高、下身脉搏减弱，因此手臂血压高于腿部。洪大的脉搏出现在狭窄上方的手臂；股动脉脉搏位于狭窄下方，血流较少，因此微弱。

The narrowing distal to the arm vessels raises upper-body pressure and weakens lower-body pulses, so blood pressure is higher in the arms than the legs. Bounding pulses belong to the arms, above the narrowing; the femoral pulses lie below it and receive less flow, so they are weak.

一名儿童已发热6天，双侧结膜发红但无分泌物，红唇皲裂伴草莓舌，有皮疹且双手肿胀。护士预期哪种治疗？A child has had a fever for 6 days, bilateral conjunctival redness without discharge, red cracked lips with a strawberry tongue, a rash, and swollen hands. Which treatment does the nurse anticipate?
  1. 皮质类固醇和卧床休息Corticosteroids and bed rest
  2. 抗病毒药物Antiviral medication
  3. IV免疫球蛋白和aspirinIV immune globulin and aspirin
  4. 仅用抗生素Antibiotics only

答案: C · IV免疫球蛋白和aspirin

发热5天或以上并伴有至少四项主要特征（此处为结膜发红无分泌物、口腔改变、皮疹和双手肿胀）符合Kawasaki病的诊断标准；特征较少的儿童仍可能按不完全性Kawasaki病治疗。IVIG可降低冠状动脉瘤的风险，aspirin则因其抗炎和抗血小板作用而给予。仅用抗生素只能治疗猩红热等细菌性病因，但Kawasaki病不是感染，抗生素不能保护冠状动脉。

Fever for 5 or more days plus at least four principal features (here conjunctival redness without discharge, oral changes, rash and swollen hands) meets the criteria for Kawasaki disease; children with fewer features may still be treated for incomplete Kawasaki disease. IVIG lowers the risk of coronary artery aneurysms, and aspirin is given for its anti-inflammatory and antiplatelet effects. Antibiotics only would treat a bacterial cause such as scarlet fever, but Kawasaki disease is not an infection, and antibiotics do not protect the coronary arteries.

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