Understanding Kawasaki Disease on the Ground of Pediatric Growth and Lymphoid Tissue Maturation

Refer to the page 107-114 In Kawasaki disease (KD), this systemic vasculitis may be complicated by severe cardiac morbidity in young children or sudden cardiac death of which the initial pathological change starts within just a few febrile days. With regard to the etiology of KD, it is an enigma that many researchers have attempted to solve, but unfortunately, the cause of KD is unknown. There are no confirmative diagnostic laboratory tools for diagnosis of KD. Therefore, diagnosis of KD still depends on clinical criteria and cardiovascular imaging findings. Prolonged fever, among other clinical symptoms and signs, is one of the most important risk factors causing coronary complications in KD. In clinical practice, in patients with the incomplete form of KD, there is controversy between pediatric subspecialists, such as infection specialists, immunologists, and pediatric cardiologists. This controversy occurs during the narrow period of the therapeutic window regarding which immune modulation therapy could work and might reduce coronary complications. There has been an increasing incidence of KD in Korea and Japan, 1)2) and increasing trends of atypical or incomplete forms of KD complicated by severe coronary complications. Deciding on therapeutic options in patients with prolonged fever and partial features of KD is not easy. Therefore, performing randomized, controlled trials of therapeutic options in young patients with KD is difficult. Current consensus on the etiology of KD remains vague. Genetically susceptible individuals are thought to be triggered by a certain infectious agent, which develops clinical features of KD. KD then becomes complicated by coronary dilatation or aneurysms in severe cases. When diagnosing KD only with clinical criteria, several infectious diseases that commonly share clinical manifestations with KD are possible as a differential diagnosis. There is also a subgroup of patients with KD whose clinical course starts with marked enlargement of a cervical lymph node or neck mass. Typically, this group of patients is initially treated with several types of antibiotics. Finally, clinical features of full blown KD suddenly appear a few days later. Remission can be obtained by immune therapy along with suspending antibiotic therapy. Severe cases with prolonged fever and cervical lymphadenopathy may be complicated by coronary aneurysms. These findings have been reported by several studies. Jun et al. 6) presented the clinical characteristics of a subgroup of KD patients, in order to give information on the prevention of a delay in early diagnosis of KD and a coronary arterial lesion …