Clinical diagnosis
Case 188
【Progress】
Cardiac ultrasound five years before showed gigantic aneurysm of right coronary artery and left coronary artery (Fig. 2).
【Discussion】
Kawasaki disease is first documented by Dr. Kawasaki in 1967 (1). It has been more than 50 years since then. However, the etiology of this disease is still unclarified. Some infection might become trigger which make autoimmune system activate and damage mucocutaneous and vessel wall (2). It is believed to occur in children with genetic predisposition. It causes children less than 5 years several mucocutaneous-lymph-node lesions (1-5). Laboratory test does not supply the data specific for Kawasaki disease but only non-specific inflammatory data such as elevation of WBC and CRP. Diagnosis for Kawasaki disease depends on clinical symptoms. Namely, high fever, erythematous rash, conjunctivitis, red throat, strawberry tongue, cervical lymphadenopathy are known to be clue for Kawasaki disease. Juvenile arthritis is also other symptom for this disease. When antibiotics is not effective for these symptoms, Kawasaki disease should be though to be one of the differential diagnosis.
Pathologically, vasculitis with fibrinoid necrosis for Kawasaki disease causes these symptoms (6-8). Coronary aneurysm occurs with the incidence of up to 25 % when untreated (2, 3). Coronary aneurysm is categorized into saccular, fusiform, ectasia and giant. It causes thrombosis, distal embolization and aneurysm rupture. The reason why coronary aneurysm is selected rather than other medium-sized arteries is not clarified.
The prognosis for Kawasaki disease is dependent upon whether coronary aneurysm is formed or not. As the larger coronary aneurysm glows, the greater mortality rate elevates. The rupture and occlusion of coronary artery causes sudden death.
Aspirin and immunoglobulin are the initial treatment. When proper treatment is initiated within one week after the onset, the incidence of coronary aneurysm remarkably decrease and mortality rate reduces 1% to 0.1%. (2, 3)
In our case, she got Kawasaki disease in a six month-old whose clinical information were eye-bulbar conjunctiva and high fever and WBC 15630/mm3 and CRP 9.8 mg/dL on laboratory test. She passed time of approximately four weeks with antibiotics in a local hospital. When she came to our hospital, giant coronary aneurysm was already completed. Four years and six months later, chest radiograph showed giant aneurysm at both coronary arteries.
【Summary】
We present a five-year-old girl for cough and high fever of 37.9℃. Chest radiograph showed giant aneurysms corresponded to right and left coronary arteries. She was already diagnosed Kawasaki disease (muco-dermato-lymph node syndrome) at age of six-month-old. It is borne in mind that Kawasaki disease affect children aged less than 5 years and its clue symptoms are high fever, erythematous rash, conjunctivitis, red throat, strawberry tongue, cervical lymphadenopathy and juvenile arthritis. The pathological key finding is vasculitis with fibrinoid necrosis, leading to formation of coronary aneurysm. Aspirin and immunoglobulin are the initial and main treatment. The earlier diagnosis and the speedy treatment within one week after onset bring about interruption of the formation of coronary aneurysm and leading the marked decrease of mortality rate from 1% to 0.1%.
【References】
1.Kawasaki T. "[Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children]". Arerugi.1967; 16 : 178–222
2.Kawasaki T. "General review and problems in Kawasaki disease". Japanese Heart Journal. 1995; 36 (1): 1–12.
3.Fukushige J, et al. "Incidence and clinical features of incomplete Kawasaki disease". Acta Paediatrica. 1994; 83 (10): 1057–60.
4.Kim DS et al. "Kawasaki disease". Yonsei Medical Journal. 47 (6): 759–72.
5.Rowley AH, et al. "Kawasaki syndrome". Clinical Microbiology Reviews. 1998; 11 (3): 405–14.
6.Cassidy JT, et al.. Vasculitis. In: Cassidy JT, Petty RE, eds. Textbook of pediatric rheumatology. 3rd ed. Philadelphia, W.B: Saunders Company; 1995. p. 365-422
7.Smith LB, Newburger JW, Burns JC. "Kawasaki syndrome and the eye". The Pediatric Infectious Disease Journal. 1998; 8 (2): 116–8.
8.Scardina GA, Fucà G, Carini F, Valenza V, Spicola M, Procaccianti P, et al. "Oral necrotizing microvasculitis in a patient affected by Kawasaki disease". Medicina Oral, Patologia Oral y Cirugia Bucal. 2007; 12 (8): E560-4.
2020.4.15