An Update on Reports of Atypical Presentations of Kawasaki Disease and the Recognition of IVIG Non-Responder Children

被引:6
作者
Conte, Cristiano [1 ]
Sogni, Francesco [1 ]
Rigante, Donato [2 ,3 ]
Esposito, Susanna [1 ]
机构
[1] Univ Parma, Univ Hosp, Dept Med & Surg, Pediat Clin, I-43126 Parma, Italy
[2] Fdn Policlin Univ A Gemelli IRCCS, Dept Life Sci & Publ Hlth, I-00168 Rome, Italy
[3] Univ Cattolica Sacro Cuore, I-00168 Rome, Italy
关键词
acetylsalicylic acid; coronary artery abnormality; coronary artery aneurysm; innovative biotechnologies; intravenous immunoglobulin; Kawasaki disease; personalized medicine; vascular complications; AUTOINFLAMMATORY SYNDROMES; CYTOMEGALOVIRUS-INFECTION; CORONARY ANEURYSMS; SHOCK SYNDROME; ACUTE-PHASE; INVOLVEMENT; MULTICENTER; THERAPY; HEART; HEMOPHAGOCYTOSIS;
D O I
10.3390/diagnostics13081441
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Kawasaki disease (KD) is an acute vasculitis with an intrinsic risk of severe involvement of coronary arteries. The worldwide spread of KD and the importance of early diagnosis for preventing cardiovascular complications have ascertained the need for updating guidelines for prompt disease recognition and treatment efficacy assessment. All KD patients who comply with the definition of classic or atypical disease should be treated with intravenous immunoglobulin (IVIG) soon after diagnosis. The objective of our narrative review was to analyze the medical literature about case reports with atypical KD in relation to diagnosis and potential identification of predictors of non-responsiveness to IVIG. Our analysis has shown that the seminal challenge in KD management is the timeliness of diagnosis, although both extreme variability and transience of clinical manifestations make this goal difficult. A non-negligible percentage of patients, especially in the first 6 months of life, might have atypical manifestations of KD, whose painstaking differential diagnosis may be tricky. Many attempts to develop universal scoring systems and detect children at higher risk of IVIG resistance have been rather unsuccessful. Additionally, KD may show different evolutions according to unraveled demographic, genetic, or epigenetic factors. Further research is needed to elucidate all open questions about KD and clarify the long-term outcome of its potential complications.
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页数:15
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