Objective: KAWASAKI DISEASE (KD) is an intense life-threatening vasculitis. The diagnosis of KAWASAKI DISEASE is made by clinical criteria. This DISEASE is a common rheumatologic DISEASE in Iran. The aim of this study was describe the demographics and clinical features of KD in Iranian patients. Material & Methods: A review was conducted for all cases of KD treated at Pediatric rheumatology department in Children's Medical Center between January 1994 and July 2004. The diagnostic criteria for typical KAWASAKI were based on the criteria of the Japan KAWASAKI DISEASE Research Committee. Atypical or incomplete KD has been described in which patients not strictly meeting the diagnostic criteria but have coronary artery changes. Color doppler echocardiograms were done at the time of diagnosis, 14 to 21 days, 60 days, and 1 year after treatment. Findings: One hundred fifty nine patients were identified. One hundred twenty five children (78.6%) fulfilled criteria for typical KD. Echocardiographic abnormalities were found in 30 cases (18.9%), including 9.6% with typical and 46.1% with atypical KAWASAKI. The incidence of atypical KAWASAKI in our study was about 22%. Coronary arteries aneurysms found in 66.7% and 33.3% was other abnormalities. Male to female ratio was more in patients with cardiac complication (2.3:1).Conclusion: KAWASAKI DISEASE should be considered in any infants or child (especially less than 2 years old) with a prolonged febrile illness. Demographic features of our patients were similar to reports from other country. The incidence of atypical KAWASAKI in our study was about 22%.

To review children with KAWASAKI DISEASE admitted in 17-Shahrivar hospital of Rasht from 1999 to 2007. We reviewed retrospectively 64 children with KAWASAKI DISEASE between 1999-2007 admitted in 17- Shahrivar Hospital of Rasht. Frequency distributions of variants including age, sex, season, clinical and laboratory manifestations, response to treatment and complications of the patients were abstracted using SPSS 14. Patients' age ranged from 2 month to 12 years (median: 41.5 months). The male / female ratio was 1:0.78. The most cases were admitted in the autumn and then spring. Fever in 100%, changes in lip and mouth in 92.1%, rash in 87.5%, Conjunctivitis in 82.8%, changes in extremities in 67.1%, and cervical lymphadenopathy in 59.3% were present. Coronary aneurysm was found in one case. The most common extracardiac complication in this study was vomiting (85.7%). With respect to KAWASAKI DISEASE differential diagnosis and its treatment, Better knowledge can help us to make diagnosis more accurately.

Background: Giant coronary artery aneurysms caused by KAWASAKI DISEASE are rare; however, they are one of the most serious complications and can be lethal.Case Presentation: We report a 3.5-month-old boy referred to us because of high fever for fifteen days, generalized maculopapular rash, irritability and cough. Transthoracic echocardiography showed dilatation of right coronary (RCA) and left main coronary (LCA) arteries. Serial echocardiography revealed rapidly progressive dilatation of coronary artery aneurysms of RCA and LCA. We performed invasive cardiac catheterization with selective coronary angiography when the boy was 16 months old. Selective right and left coronary arteriography showed a super giant fusiform aneurysm of RCA and a diffuse giant aneurysm of the proximal LCA. Regression of coronary artery aneurysms was not observed during 6 years of follow up.Conclusion: Pediatricians should be alert for possibility of incomplete KAWASAKI DISEASE in young infants with atypical presentation. They are at higher risk of coronary aneurysm formation. The diagnosis often was late with higher complication rate of coronary aneurysm.Echocardiography is an important tool for diagnosis of incomplete KAWASAKI DISEASE. Selective coronary angiography is the gold standard for diagnosis. and estimation of shape and size of aneurysms.

Background: KAWASAKI DISEASE is an acute multisystem vascular syndrome of unknown etiology that is the leading cause of acquired heart DISEASE in children of developed counties.Objectives: We aimed to evaluate the epidemiological characteristics and clinical manifestations of KAWASAKI DISEASE in children residing in the southwest of Iran.Patients and Methods: In this retrospective study, we reviewed the medical records of all children with KAWASAKI DISEASE who had been admitted to the main children’s hospital of Ahvaz, southwest Iran, from March 2000 to March 2010. Data regarding clinical and epidemiological characteristics, management, and the outcome of DISEASE for each patient were obtained. The patients were divided into cardiac and non-cardiac groups based on echocardiographic results.Results: In total, 104 patients with KAWASAKI DISEASE (66 boys and 38 girls) were enrolled in this study. The male to female ratio was 1.7: 1. The mean±SD age of the patients was 33.6±24.2 months. Most (87.2%) cases were from urban areas. The DISEASE occurred more frequently during winter and spring. Furthermore, 61.5% of the children had the criteria of classic KD, and 38.5% were labeled as incomplete KD. The mean±SD of the duration of hospital stay was 6.9±2.4 days. The mean time between illness and admission to the hospital was 6.47±2.6 days. The most common sign was fever, followed by conjunctivitis and oral changes. In total, 20% of the patients had cardiac abnormalities. There was no significant statistical difference between the cardiac and non-cardiac groups according to age, sex, clinical manifestations, laboratory findings, and cessation of fever. The duration of hospital stay and the time between onset of illness and diagnosis were longer in the cardiac group. All patients received intravenous immunoglobulin and aspirin. Only one patient continued to have cardiac abnormalities after 6 months of follow-up.Conclusions: KAWASAKI DISEASE is not rare in southwest of Iran. The age, gender distribution and clinical findings are similar to that of other reports. Patients with cardiac abnormalities had delayed treatment and prolonged hospital stays.

Background: KAWASAKI DISEASE is an acute vasculitis that occurs mainly in children. Cervical lymphadenopathy is one of the major presenting manifestations of KAWASAKI DISEASE. We report a case of KAWASAKI DISEASE with para aortic lymphadenopathy, as an unusual feature in this DISEASE. Case Presentation: This 2.5 year old girl presented with persistent high grade fever, erythematous rash, bilateral non purulent conjunctivitis, red lips, and edema of extremities. Laboratory results included an elevated erythrocyte sedimentation rate, leukocytosis, anemia, and positive C-reactive protein. On second day after admission she developed abdominal pain. Ultrasonography of abdomen revealed multiple lymph nodes around para aortic area, the largest measuring 12mm´6mm. Treatment consisted of aspirin and high dose intravenous g-globulin. Ultrasonography and CT scan of abdomen performed one week later showed disappearance of the lymph nodes.Conclusion: There are few previous reports of lymphadenopathy in unusual sites such as mediastinum in KAWASAKI DISEASE. Para aortic lymph nodes enlargement might be an associated finding with acute phase of KAWASAKI DISEASE. In these patients a close observation and ultrasonographic follow up will prevent unnecessary further investigation.