MUCOCUTANEOUS LYMPH NODE SYNDROME (MCLN) or Kawasaki disease (KD) is a notable disease of unknown etiology and although pediatricians are familiar with different aspects of it, they tend to make under and over diagnosis which can result in cardiovascular disorders, misapply of intravenous immunoglobulin (IVIG) and its consequences which can also cause a delay in diagnosis of mimicking diseases.

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: Kawasaki disease is an acute self-limited vasculitis of childhood characterized by fever, bilateral nonexudative conjunctivitis, erythema of lips and oral mucosa, changes in extremities, rash and cervical LYMPHadenopathy. Coronary artery aneurysm or ectasia develops in approximately 15% to 25% of untreated children and may lead to ischemic heart disease or sudden death.Objective: To evaluate the prevalence of clinical symptoms, laboratory findings and epidemiology of Kawasaki disease in children hospitalized at Quds children hospital, Qazvin, Iran.Methods: Between March 1994 and March 2005, a total of 29 consecutive Kawasaki patients were enrolled and studied retrospectively.Findings: Of 29 patients, 20 were males and 9 females. The male/female ratio was 1:0.45. The majority of patients (65.3%) were younger than 5 years old. Seasonal peak was found to be in winter (37.9%) and spring (34.5%). All patients had fever lasting from 8 to 21 days. The most common clinical features were oral mucosal changes (96.6%), changes in extremities (79.3%), conjunctivitis (75.8%), cervical LYMPHadenopathy (58.6%) and rash (58.6%). Echocardiography was abnormal in 14%.Conclusion: Kawasaki disease is present in our country and diagnostic work-up in cases with prolonged fever and unresponsive to antibiotics is strongly recommended, as the clinical manifestations of Kawasaki disease are in common with many other diseases.

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 (KD) is a frequent cause of acquired heart disease in infants and young children, which may be triggered by infectious agents, with the highest incidence in Asian countries. Several genotypes are identifi ed as susceptibility genes for KD, but none is known in the Iranian population. Objectives: Due to the racial diff erences in the frequency and signifi cance of the identifi ed genes, we aimed to investigate the 8p2223-rs2254546 genotypes in a sample of Iranian children and their association with aneurysm and resistance to treatment. Methods: In this prospective cross-sectional study, 100 children with KD as the sample group were compared with 100 matched unrelated healthy Iranian children with no history of KD or immune-related disease. The control subjects were ethnically recruited from the same hospital at the time of a routine physical examination. The 8p22-23-rs2254546 genotypes were analyzed using the polymerase chain reaction (PCR) and direct sequencing techniques, and the frequency of the three genotypes (GA, AA, and GG) was reported. Finally, the frequency of aneurysm and IVIG resistance was reported, and their associations with 8p22-23-rs2254546 genotypes were tested by SPSS software. Results: Of 100 patients with a mean age of 1. 9  1. 7 (0. 1-10. 2) years, 57% were boys and 43% were girls. Of them, 62% had GG, 30% GA, and 8% AA genotypes. Besides, 21% of the studied patients were resistant to IVIG, and 19% had aneurysm, but resistance to IVIG and aneurysm was not associated with GG and GA genotypes of 8p22-23-rs2254546 (P = 0. 29 and 0. 88, respectively). Conclusions: The majority of the children with KD were shown to have this genetic susceptibility, which shows the importance of the 8p22-23-rs2254546 genotype in Iranian children. However, this genotype was not associated with the risk of resistance to IVIG and aneurysm.

Kawasaki disease (KD) is an acute vasculitis SYNDROME of unknown etiology. It occurs in infants and young children, affecting mainly small and medium-sized arteries, particularly the coronary arteries. Generalized microvasculitis occurs in the first 10 days, and the inflammation persists in the walls of medium and small arteries, especially the coronary arteries, and changes to coronary artery aneurysms.We report the case of a 10-month-old girl referred to our center three months after the onset of disease due to the aneurysms of the coronary arteries. During the acute phase of her illness, she received 2 gr/kg intravenous gamma globulin; and after her referral to us, the patient was treated by antiaggregant doses of acetylsalicylic acid (ASA) (5 mg/kg) and Warfarin (1 mg/ daily). At three months’ follow-up, the aneurysms still persisted in the echocardiogram.

Background and purpose: Kawasaki disease is the causing agent of acute vasculitis in children with unknown etiology, and at present it is the leading cause of acquired heart diseases in the japenes and North American children. This study is designed to know about the epidemiological, clinical and treatmental features of this disease and its outcome in children living in sari.Materials and methods: This is a descriptive-review done on the files of the kawasaki patients admitted in the East region of Mazandaran hospitals. The criteria for selection of the patients were based on the American heart association diagnostic criteria. Data on individual features, clinical pictures, laboratory findings,treatment and outcome were recorded and analyzed statistically.Results: Of 29 admitted Kawasaki patients, 25 met the criteria for selection. Mean rate of annual incidence of the disease was 7.3 per hundred thousand in children under 5 years. Mean age for occurance was 38±22.3 months and age range was 3.5 to 80 months, in 40% of the cases it occurred in full season, ratio of boys to girls was 1.27/1, mean time between the onset of illness and diagnosis and treatment was 8.2±3.1 days and the least required clinical finding for diagnosis was LYMPHadenopathy of 73%. Five out of 25 patients had cardiovascular abnormalities (CVA) prior to the beginning of treatment (one with myocarditis and pericarditis, 4 with coronary artery abnormalities). Out of 25 patients, 22 gave response simultaneously to one dose of intravenous injection of immunoglubolin (IVIG) and high dose of aspirin (ASA). Recovery was observed in 2 patients with two doses and one with the third dose of IVIG in a 3 to 12 months period of follow up. All of the patients excluding one completely recovered from cardiovascular complications.Conclusion: In Mazandaran province, Kawasaki disease in one of the most common vasculitis conditions. The prevalence corresponds with the reports given in north America but more that the european countries and less then japan. Although 1/5 of the patients prior to the treatment suffered from cardiovascular abnormalities, most of them responded to the treatment with IVIG and ASA and recovered compaletely within 3 to 12 months follow up.

Kawasaki disease (KD) is a febrile vasculitis and is considered a leading cause of acquired coronary artery disease in children. A clinically critical complication is the coronary artery aneurysm, which may progress and lead to coronary stenosis or even obstruction. Herein, we describe a 14. 5-year-old boy with a history of KD at 6 months old, who developed multiple aneurysms along all the coronary branches. During the follow-up at the age of 14 years, the left coronary artery aneurysms regressed, while the aneurysm of the right coronary artery persisted and was complicated by obstruction at its proximal part, according to computed tomography angiography. However, the patient at the last follow-up was asymptomatic and well. The serious nature of KD coronary complications warrants follow-up visits. Since echocardiography alone may fail to reveal stenosis or obstruction, other adjunct follow-up imaging modalities such as conventional, computed tomography, and magnetic resonance angiography should be performed in patients with coronary aneurysms.

The charts of 27 patients with Kawasaki disease (KD) admitted to Nemazee Hospital in Shiraz from January 1991 to October 1998 were reviewed to identify the results of mean peroxides index (MPXI) values, a measure of neutrophil staining intensity, obtained by the Technicon H1 analyzer (Technicon Instruments Corp., Tarrytown, NY) within the first 10 days of the illness; 2 separate groups of patients were assessed as control subjects: 27 disease control (DC) children with fever plus one other KD criterion; and 27 laboratory control (Le) subjects with nonfebrile disorders interpreting also as a normal reference population. Compared with control groups, patients with KD had lower quantities of MPXI [(Meani±SD, -11.71±5.87 in KD group) vs. (1.53±4.30; p<0.001 in DC group, and 1.74±6.52, p<0.001 in LC group)]. Depending on the location of the cut-off point expressed on an interval scale, this test had the ability to be 100% specific (if MPXI<-6.0) and 100% sensitive (if MPXI>0).Considering the low prevalence of hereditary myeloperoxidase (MPO) deficiency (1 in 2000), measurement of MPXI, when performed as part of a complete count on an automated hematology instrument, could be counted as an important adjunct to clinical evaluation and also according to the low values of MPXI inpatients with KD, it can be included among the acquired causes of MPO deficiency.

Kawasaki disease (KD) is an inflammatory vasculitis. KD is classified into two groups based on clinical characteristics criteria, namely classic and incomplete. Cerebral vascular abnormality, especially arterial ischemic stroke (AIS) is very rare and unusual in KD. Here, we report a 4-year-old boy who was referred to our tertiary pediatric center with abrupt right hemiparesis and aphasia. At admission time, he had febrile illness and was toxic. On physical examination, we found unilateral left submandibular LYMPHadenopathy. On neurologic examination, we obtained right sided hemiparesis with hemiparetic gait and aphasia. His deep tendon reflexes (DTRs) of right extremities were exaggerated and his sensory system was intact. Based on these features, some differential diagnoses were suggested, such as acute encephalitis with focal signs, brain abscess, cerebral vasculitis, hemorrhagic insults, and ischemic stroke, etc. After a complete evaluation, especially brain MRI and MRA, our diagnosis was arterial ischemic stroke (AIS) following atypical KD. Based on these findings, we administered intravenous immunoglobulin (IVIG 2 gm/kg) and oral high dose aspirin (100 mg/kg/d). He responded to these anti-inflammatory treatments dramatically.