Takeo Shin, Kanda Shoichiro, Takizawa Keiichi, Kajiho Yuko, Kinumaki Akiko
Pediatrics, The University of Tokyo, Tokyo, JPN.
Cureus. 2025 Mar 9;17(3):e80317. doi: 10.7759/cureus.80317. eCollection 2025 Mar.
Kawasaki disease (KD) primarily affects infants and young children, with cases in patients over 10 years of age being relatively rare. The standard treatment involves intravenous administration of high-dose intravenous immunoglobulin (IVIG) at 2 g/kg. In pediatric medicine, drug dosages are typically adjusted according to body size; however, in older children, body size-based dosing often results in excessively high doses, and adult dosages are frequently used as upper limits. Notably, no such upper limit exists for IVIG. Given that IVIG administration can cause severe cardiac and neurological complications due to hyperviscosity syndrome, careful dose management is essential. Here, we report the case of a 13-year-old KD patient weighing 53 kg who underwent repeated high-dose IVIG therapy. Serum IgG levels, total protein concentration, and hematocrit were monitored over time as an indicator of blood viscosity, allowing for dose adjustments. As a result, the patient successfully completed the treatment course without experiencing major adverse effects. Notably, the adjusted IVIG regimen effectively prevented the development of hyperviscosity syndrome. Furthermore, the patient did not develop coronary artery aneurysms, indicating that the treatment maintained both safety and therapeutic efficacy in managing Kawasaki disease. On the other hand, the increase in serum IgG levels following IVIG at a dose of 2 g/kg was greater in this patient compared to younger children receiving the same treatment. This case highlights the necessity of monitoring IgG levels to balance the efficacy and safety of IVIG therapy in older KD patients. However, there is no established threshold for IgG levels that would indicate a safe upper limit for IVIG administration, highlighting the need for further investigation.
川崎病(KD)主要影响婴幼儿,10岁以上患者的病例相对较少。标准治疗方法是静脉注射2 g/kg的大剂量静脉免疫球蛋白(IVIG)。在儿科学中,药物剂量通常根据体型进行调整;然而,对于年龄较大的儿童,基于体型的给药往往会导致剂量过高,因此常将成人剂量用作上限。值得注意的是,IVIG不存在这样的上限。鉴于IVIG给药可能因高粘滞综合征导致严重的心脏和神经并发症,谨慎的剂量管理至关重要。在此,我们报告一例体重53 kg的13岁KD患者接受重复大剂量IVIG治疗的病例。随着时间的推移,监测血清IgG水平、总蛋白浓度和血细胞比容作为血液粘度的指标,以便进行剂量调整。结果,患者成功完成治疗过程,未出现重大不良反应。值得注意的是,调整后的IVIG治疗方案有效预防了高粘滞综合征的发生。此外,患者未发生冠状动脉瘤,表明该治疗在川崎病的管理中保持了安全性和治疗效果。另一方面,与接受相同治疗的年幼儿童相比,该患者接受2 g/kg剂量IVIG后血清IgG水平的升高幅度更大。该病例强调了监测IgG水平以平衡IVIG治疗在老年KD患者中的疗效和安全性的必要性。然而,目前尚无确定的IgG水平阈值可表明IVIG给药的安全上限,这突出了进一步研究的必要性。