- Severe medium-sized vessel vasculitis
- The leading cause of acquired heart disease in children
- Late fall through early spring
- Complications : Coronary artery aneurysms, myocarditis, pericarditis,
pericardial effusion, valvular dysfunction, left ventricular dysfunction, and arrhythmias
Typical age < 5 y/o (1 - 8 years of age)
眼紅 嘴紅 脖子腫
紅斑 脫皮 燒五天
冠狀動脈瘤 + BCG Site
Coronary artery aneurysms or ectasia occurs in 15 - 25% of untreated children
在於沒有接受免疫球蛋白治療的情況下
大約 20-25%左右的病童可能會產生冠狀動脈瘤及冠狀動脈病變的併發症
如果有在發病 10 天內接受治療，產生併發症的機會約降低至 3-10%
- 眼睛：Nonpurulent bilateral conjunctivitis without exudate, iritis
- 嘴巴：Strawberry tongue, Mucositis
- 脖子：Lymph node enlargement
- 皮膚紅斑
- 指尖脫屑 Peeling of the skin in the hands, feet
and genital area may also occur in the later phases
- 發燒五天
- 發燒五天
The acute phase of disease results in myocarditis and pericarditis, which typically resolve without treatment.
Reduced left ventricular function and arrhythmias are more common in the acute phase of illness than in later phases.
Most coronary artery aneurysms develop during the third and fourth weeks of illness.
* Lab :
- Leukocytosis and left shift
- Elevated ESR, CRP
- Lymphocyte : typically drop, then rise during convalescence
- Reactive thrombocytosis :
Correlates with the risk of coronary artery changes
rise by the second week of illness and may reach 1,000,000/mm3
- Normocytic, normochromic anemia
- Pyuria
- Abnormal liver function :
a minority of children develop obstructive jaundice from hydrops of the gallbladder
- Arthrocentesis pleocytosis
- Hyponatremia
- CSF may display a mononuclear pleocytosis
* Treatment :
Kawasaki 最需注意 Coronary artery aneurysm
先測 Liver function & flu test
Aspirin 是肝代謝
如果 Elevated GOT/GPT, 仍用 aspirin 需擔心 Jaundice
如果不能使用 Aspirin, 可改用 Dipyridamole (不過是 off label use)
發病 7-10 天內給 IVIG : 2g/kg over 10-12 hrs
一般 48-72 小時內症狀會得到緩解
如果反應不佳再次給予 IVIG 2 g/kg
Administration of IV immunoglobulin (2g/kg over 12 hours)
results in rapid and dramatic symptomatic improvement in 90% of patients
and prevents aneurysm formation in 95%.
Second dose of IV immunoglobulin may be given (same dosage)
if the patient remains febrile 48 hours after the initial dose,
because refractory fever may be a risk factor for coronary artery abnormalities.
Combined Aspirin (30 - 50 mg/kg/d)
Meta-analyses suggest high-dose therapy (80 -120 mg/kg/d) confers no additional benefit
Following the acute phase, the aspirin dose is reduced to 3 - 5 mg/kg QD for 6 - 8 weeks.
主要的目的是取用高劑量阿斯匹靈抗發炎反應的作用
小朋友常常使用的依普芬 (常見的葡萄口味退燒止痛藥 ibuprofen)，
會拮抗阿斯匹靈抗血栓的作用，因此如果是因為有冠狀動脈瘤在使用阿斯匹靈的情況下，應該要盡量避免依普芬的使用
A single dose of corticosteroids administered before IV immunoglobulin does not improve coronary outcome.
輸注 IVIG 的同時給 高劑量 Aspirin 20-25 mg/kg Q6H (80-100mg/kg/day, 抗發炎作用)
直到退燒後轉為 低劑量 Aspirin 3-5 mg/kg/day (抗血小板作用, 避免血管阻塞)
持續治療 6-8週
直到冠狀動脈瘤, 冠狀動脈狹窄緩解
Cardio echo 需要門診追蹤10年, 好像可以申請重大傷病的樣子
由於 Kawasaki disease 治療必須使用 Aspirin
孩童建議接種
- Inactivated influenza vaccine
- 水痘疫苗 Varicella vaccine
以降低感染水痘或流行性感冒的風險
來避免產生 Reye's syndrome
使用 IVIG 後疫苗要隔11個月再打, 不然會被綜合就無效了
The use of infliximab, cyclosporine A, methotrexate, and cyclophosphamide
remains to be elucidated in future prospective trials.
* Atypical or incomplete kawasaki :
Only developed fever and cervical lymphadenopathy by the time of admission
(so-called KD with isolated cervical lymphadenopathy, KDiL)
除了發燒之外, 在其他五項典型臨床表現中, 僅符合二到三項
但有合併冠狀動脈病變
因不符合典型川崎氏症之診斷標準, 在診斷上較困難
必須用一些輔助性診斷指標
- Albumin ≤ 3 g/dL
- Elevated liver function
- Plt ≥ 450,000/mm3
- UA : WBC ≥ 10/HPF
- Anemia
- WBC ≥ 15,000/mm3
六項須符合至少三項且需做心臟超音波檢查才能診斷為川崎氏症
In one case series, these patients tended to be older and to have a more severe course,
with increased risk of coronary artery disease and lack of response to IVIG.
Patients < 1 year old and those > 9 years old tend to present with incomplete Kawasaki’s disease,
leading to delayed diagnosis and higher risk of coronary artery abnormalities
* A 錯誤。典型川崎氏症需發燒加上五個條件滿足四個以上, 題幹只滿足三個
* B 應是 incomplete kawasaki's disease，且輔助診斷標準中無 Cr
* C 應用 Aspirin 退燒
* D 在病毒感染的孩童上使用 Aspirin 可能造成 Reye's syndrome
感染流感或者水痘同時合併使用高劑量的阿斯匹靈，曾經有報告過會產生雷氏症候群(Reye’s syndrome)，
如果有發生類似事件，則應該跟醫師討論使用替代阿斯匹靈的藥物。
但是我們還是鼓勵施打流感疫苗，一方面死菌的流感疫苗和低劑量阿斯匹靈併用並不會出現雷氏症候群，
二來才能提高小朋友抵抗流感的能力，避免真正得到流行性感冒。
Reye’s syndrome則是一種病毒感染 (ex. 水痘 varicella, 流行性感冒 influenza)加上 Aspirin使用，
產生交互作用所誘發的急性腦部 (腦水腫、腦壓升高)、肝臟病變 (肝功能異常、肝腫大、脂肪沉積)，
致病的原因可能為病毒感染造成的免疫反應，
損害了線粒體功能和脂質代謝，Aspirin的使用會加劇此傷害，好發於 2個月至16歲的孩童。
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