Abstract
Background: Kawasaki Disease (KD) is a self-limiting and acute systemic vasculitis of childhood that leads to coronary artery abnormality in about 25% of untreated cases. KD is extremely rare in neonates. The purpose of this paper is to explore the clinical features and diagnosis and treatment of Neonatal Kawasaki Disease (NKD) for early identification. Case presentation: A boy was a 24-day-old full-term infant with no special birth history. He had fever, irritability and rash at the time of admission. After 4 days of treatment with empirical antibiotics, the rash and fever were still present, and the rash was progressively increased. It is considered that the infection was serious, and antibiotics were changed and given intravenous immunoglobulin. The temperature and rash were better than before. On day 5, he had cracking of lips, on day 9, he had lymph node enlargement in the neck, and molted fingers appeared on the 10th day. Thus the complete Kawasaki Disease (CKD) was diagnosed, and the patient was given intravenous immunoglobulin (IVIG) and aspirin. Within 48h after the start of this therapy, the fever declined, the chapped lips got better, the rash resolved, and distal extremity desquamation appeared. On day 5, 13, and 18, there was no abnormality in echocardiography and no coronary dilatation. On day 20, the chapped lips and the distal extremity desquamation got better. After discharge, the patient continued to take aspirin orally. The patient was followed up to 1 year old, with good prognosis and no carditis or coronary artery abnormalities. Our literature search revealed only a few reported cases of suspected NKD worldwide (18 cases, including 14 English literature and 4 Chinese literature) (Table 1), together with this case, 19 cases in total. Among these 19 cases, 12 were male and 7 were female, the age of onset was 1-26 days. Six patients had complete KD (31.5%), 13 patients were IKD. Clinical manifestations: there were 17 cases of fever (89.5%, 13cases of fever >5days), 13cases (68.4%) of early limb edema/swelling or peeling, 16 cases (84.2%)of skin rash, 14cases (73.7%)of oral mucosal changes (lips red/cleft or strawberry tongue), 10 cases (53%) of coronary artery changes, 7cases (36.8%) of conjunctival congestion, 3cases（15.8%）of cervical lymph node enlargement. Laboratory investigations: There were 5 cases (35.7%) with elevated ESR, 9 cases normal, 5cases unknown. CRP was normal in 6 cases (33%). Thrombocytopenia occurred in 11cases (64.7%). Among the 10 patients with coronary artery changes, 1 had an unknown prognosis due to loss of follow-up (patient 15). One patient died 2 days after discharge due to multiple organ failure (patient 17). 4 cases returned to normal (patient 3.6.7.14). The coronary artery changes in 4 cases still did not return to normal and were not followed up for a long time. Of the 19 NKD patients, 16 were sensitive to IVIG, 1did not use IVIG, and 1 was not sensitive to IVIG，and due to multiple organ failure, the parents gave up treatment and died two days after discharge.Conclusion: NKD is extremely rare, and its clinical manifestation is not typical and easy to be missed. If not treated early, it will potentially give rise to coronary artery aneurysms or expansion, ischemic heart disease, and sudden death. Early diagnosis and treatment are very important.
SHARE – workpackage 5: evidence-based recommendations for diagnosis and treatment of kawasaki disease and henoch schönlein purpura
