Case discussion R1 陳柏嵩

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

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

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

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

Family history No family history of hereditary disease

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

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

Lab data

Clinical course Cardiac echo Lab IVIG IVIG Refractory Kawasaki disease?

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

Follow up Lab Back 2019/2/25

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

Lab data Back 2019/2/25

Water’s view Back

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

Refractory Kawasaki disease Risk factors and current treatment

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

Incidence 10% to 15% patients with KD, initially treated with IVIG and aspirin Fever persists or returns within 48 hours 2019/2/25

The study aimed to identify the risk factors of intravenous immunoglobulin (IVIG) unresponsiveness from the initial clinical parameters of the Taiwanese KD patients.

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

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

2019/2/25

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

The End