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Abstrak :
Latar belakang: Penyakit Kawasaki adalah penyebab utama penyakit jantung didapat pada anak, yang merupakan suatu vaskulitis sistemik akut. Penyakit ini berhubungan dengan luaran aneurisme arteri koroner, yang dapat dicegah dengan pemberian imunoglobulin intravena (IGIV). Terapi baku emas pada penyakit Kawasaki adalah IGIV dosis tinggi (2 g/kgBB). Namun, IGIV dosis medium (1 g/kgBB) merupakan terapi berbiaya lebih rendah dan mungkin memiliki efikasi yang sama. Melalui penelitian ini, kami mengevaluasi keberhasilan terapi IGIV dosis 1 g/kgBB. Metode: Studi kohort retrospektif multisenter dari data rekam medis dengan total 507 pasien dengan penyakit Kawasaki komplit. Penelitian dilakukan di Rumah Sakit Umum Pusat Nasional Cipto Mangunkusumo dan Kawasaki Center, Indonesia dari Januari 2012 hingga Januari 2022. Pasien yang mendapatkan terapi IGIV dengan dosis 1 g/kgBB didefinisikan sebagai grup A, dan pasien yang mendapatkan terapi IGIV dengan dosis 2 g/kgBB didefinisikan sebagai grup B. Karakteristik dasar subjek dibandingkan antar-kelompok tersebut; demografi, hasil laboratorium, keterlibatan mukokutan, hari demam saat diberikan IGIV, durasi demam pasca-IGIV, lama rawat, dan temuan aneurisme arteri koroner dari ekokardiografi pada periode follow-up. Hasil: Sebanyak 24 pasien (grup A), mendapatkan IGIV dosis medium (1 g/kgBB). Sementara itu, sebanyak 483 pasien (grup B) mendapatkan IGIV dosis tinggi (2 g/kgBB). Distribusi usia dan jenis kelamin, nilai leukosit dan trombosit, hari demam saat diberikan IGIV, durasi demam pasca-IGIV, dan lama rawat tidak berbeda bermakna antar-kelompok (p >0,05). Semua pasien mengalami keterlibatan mukokutan. Berat badan menurut umur (WAZ) dan nilai CRP antar-kelompok bermakna secara statistik (p <0,05). Aneurisme arteri koroner tidak ditemukan pada pasien di grup A dan pada 9 pasien (1,9%) di grup B pada periode follow-up (p >0,05). Simpulan: Terapi dengan dosis inisial IGIV 1 g/kgBB untuk pasien dengan penyakit Kawasaki menunjukkan keberhasilan yang sama dengan IGIV dosis tinggi (2 g/kgBB). Hal ini dapat menjadi opsi terapi bagi negara berkembang. ......Background: Kawasaki disease (KD), the leading cause of acquired heart disease in children, is an acute childhood systemic vasculitis. It is associated with coronary artery aneurysms (CAA), that could be prevented by intravenous immunoglobulin (IVIG) administration. High-dose IVIG (2 g/kg) is usually given in the treatment of Kawasaki disease (KD). However, medium-dose IVIG (1 g/kg) is a low-cost treatment and may have the same efficacy. We aim to determine whether the treatment with IVIG at an initial dose of 1 g/kg is effective for preventing CAA. Methods: A multicenter retrospective cohort study was conducted. A total of 507 patients with complete KD who were treated with high-dose and medium-dose immunoglobulin at Cipto Mangunkusumo Hospital and Kawasaki Center, Indonesia from January 2012 to January 2022 were enrolled. Patients treated with a single infusion of medium-dose IVIG (1 g/kg) were defined as group A, and patients treated with high-dose IVIG (2 g/kg) were defined as group B. Patient characteristics were compared between the two groups; demographic features, laboratory findings, mucocutaneous involvement, day of fever, duration of fever after treatment, length of stay, and rates of CAA from echocardiography during the follow-up period. Results: Medium-dose IVIG was given in 24 patients (group A). High-dose IVIG was given in 483 patients (group B). Age and gender distributions, white blood cell and platelet counts, day of fever when IVIG was administered, duration of fever after IVIG treatment, and length of stay did not differ significantly between the two groups (p >0.05). All patients had mucocutaneous involvement. Median of WAZ was higher in group A (+0,35 vs -0,26; p <0.05). Median of concentrations of C-reactive protein was higher in group B (59,5 mg/L vs 81 mg/L; p <0.05). Coronary artery aneurysms were not found in group A and in 9 patients (1.9%) in group B during the follow-up period (p >0.05). Conclusion: Treatment of KD with IVIG at an initial dose of 1 g/kg could show the same effectiveness as the high-dose IVIG (2 g/kg) and might be an option for low- and middle-income country.

Abstrak :
ABSTRACT
Background: there is clearly growing population of young adults with potentially important coronary artery disease after Kawasaki disease (KD) during childhood, and cardiologist must be prepared to take care for them. As Kawasaki disease in adolescent and adult is rare and under-recognized, it is important to study data on patient presentations which may permit development of diagnostic criteria and treatment guidelines for this age group.This study aimed to compare the clinical profile of KD between adolescents (>10 years of age) and children ≤10 years. Methods: This is a cross sectional study. A total of 1150 KD cases (age 1-192 months) during the period of January 2003-December 2016 were analyzed. The clinical profile of subjects aged >10 years (adolescents) and ≤10 years (children) at acute phase of KD were compared. Results: we found 17 cases of KD in adolescents among 1150 total cases (1.5%). Incomplete KD was more often seen in adolescents compared to children ≤ 10 years of age (59% vs. 29%). Some clinical features were more frequently seen in children than in adolescents, e.g. conjunctivitis (85% in ≤ 10 years of age vs. 65% in > 10 years), mucosal changes (94% vs. 77%), rash (86% vs. 59%), and hand/foot changes (68% vs. 41%). While other clinical features were more often seen in adolescents, e.g., cervical lymphadenopathy (82% vs. 39%) and coronary dilatation (47% vs. 29%). Laboratory results (hemoglobin, leukocytes, erythrocyte sedimentation rate and C-reactive protein) did not differ much between the two groups. Conclusion: Kawasaki disease in adolescents has some different clinical profile from that of younger age. Majority of adolescent patients have incomplete presentation. Some clinical features such as conjunctivitis, mucosal changes, rash, and hand/foot changes are more often seen in children ≤ 10 years compared to in adolescents, while cervical lymphadenopathy and coronary dilatation are more frequently seen in adolescents. The ratio of male to female is much higher in adolescents.