1. Case discussion
R1 陳柏嵩

2. General data Sex： 女 Age： 1 year 3 month Chart Number： 173231xx
Date of Admission： 2015/12/08
Ethnicity： 台灣-福佬人

3. Chief complain
Persistent fever up to 40.2°C for 4 days with mucocutaneous change

4. Present illness
Fever since 4 days ago, intermittent, over 38°C; with nasal congestion and cough
Skin rash at trunk developed in 2 days, equivocal erythema of extremities
Conjunctivitis also noted
No vomiting, no diarrhea, no sputum…
Visit LMD, persist fever

6. Past history Birth History: G2P2, C/S, GA: 38 weeks, BBW: 2900g
Vaccination: As scheduled
Growth and Development:
2-year-5-month-old boy.
BH: 80 cm = 50-85th percentile
BW: 10.1 kg = 50-85th percentile
Past History:
1.Denied systemic or hereditary history, newborn screen normal
2.Hospitalization history: denied
Social History: Nothing particular, live in home with grandmother
Travel History: Denied
Contact history: brother had common cold last month
Current Medications: denied

7. Family history
No family history of hereditary disease

8. PE TPR:攝氏36.7度, P:156/min, R:32/min, BP:85/48mmHg
【Consciousness】: clear 【Sclera】: not icteric redness and fissure lip (+) strwberry tougue (+) conjuctivitis (+) 【Neck】: supple, LAP (-) 【Chest】: Inspection: symmetric expansion Palpation: no crepitus Percussion: resonance Auscultation: normal BS
【Heart】: Palpation: no heave, no thrill Percussion: no increase of dullness Auscultation: RHB, no murmur
【Abdomen】: Inspection: globular Palpation: soft, no organomegaly, pain (-), tenderness (- ), rebound (-) Percussion: tympanic, No shifting dullness Auscultation: BS: normoactive 【Limbs】: warm, no edema, cyanosis (-), lateral weakness (-), palmar erythema (-) 【Peripheral pulse】: symmetric and active 【Skin】:no edema,petechiae or ecchymosis maculopapular skin rash (+)
2019/2/25

9. Tentative diagnosis Fever with skin rash and conjunctivitis Adenovirus
Streptococcal pharyngitis
Epstein-Barr virus
Kawasaki disease

10. Lab data

11. Clinical course Cardiac echo Lab IVIG IVIG
Refractory Kawasaki disease?

12. Cardiac echo ASD type 2 1)Situs solitus, levocardia
2)No chamber enlargement
3)An interrupted IAS at secundum portion with size: 0.394cm; with L to R shunt
4)Good LV systolic function with LVEF: 61.8%
5)Normal coronary artery pattern, LCA size: 0.119cm; RCA size: 0.131cm
6)Mild tricuspid regurgitation, PG: 23mmHg
7)Trivial mitral regurgitation 8)Left arch, no CoA
Back

13. Follow up Lab
Back
2019/2/25

14. Clinical course-2 Lab Methylprednisolone IV Water’s view Kidney echo
Refractory Kawasaki disease?

15. Lab data
Back
2019/2/25

16. Water’s view
Back

17. Kidney echo
Right renal size: 6.03cm X 2.74cm Left renal size: 6.98cm X 2.82cm
left kidney is enlarged
Back

18. Refractory Kawasaki disease
Risk factors and current treatment

19. Resistant to initial IVIG
Fever beyond the 36-hour mark from completion of the 12-hour IVIG infusion
Patients who have persistent or recurrent fever more than 24 hours after completion of the initial treatment should also be assessed for intercurrent infection
2019/2/25

20. Incidence
10% to 15% patients with KD, initially treated with IVIG and aspirin
Fever persists or returns within 48 hours
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21. The study aimed to identify the risk factors of intravenous immunoglobulin (IVIG) unresponsiveness from the initial clinical parameters of the Taiwanese KD patients.

22. Abstract
There are two treatment strategies for patient with IVIG unresponsiveness
One is rescue therapy with a second course of IVIG, steroid, infliximab, or a combination of the above medication.
The other is add-on therapy, i.e., administration of IVIG and steroid to certain KD patients who are considered high risk for being IVIG nonresponders by using a scoring system, such as the Kobayashi scoring system
Regional or population differences in such risk scoring systems needs to be validated
For example, when applied the Kobayashi score to the KD patients in Taiwan, we found that the sensitivity and specificity to predict IVIG unresponsiveness was only 62% and 71%, respectively.
2019/2/25

24. Formosa score Cut-off points and score points for each variable:
Albumin < 3.5 g/dL: 1 point
Neutrophil percentage 60%: 2 points
Positive lymphadenopathy: 1 point.
The median score for the study population was 2
2019/2/25

25. 2019/2/25

27. Treatment for unresponsiveness
USA currently use IVIG and high-dose aspirin as initial therapy and second-line therapy for those Kawasaki disease patients who have persistent or recrudescent fever
Of other therapies in this review, infliximab and steroids have the most experience as alternative second and third-line therapies.
2019/2/25

28. The End