Lack of Association of Cervical Lymphadenopathy and Coronary Artery Complications in Kawasaki Disease

2006 ◽  
Vol 25 (6) ◽  
pp. 521-525 ◽  
Author(s):  
Rita Y. T. Sung ◽  
Yin-Ming Ng ◽  
Kai-Chow Choi ◽  
Geoffrey C. F. Mok ◽  
Y W. Cheng ◽  
...  
Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
Hyun Ok Jun ◽  
Eun Kyung Cho ◽  
Jeong Jin Yu ◽  
So Yeon Kang ◽  
Chang Deok Seo ◽  
...  

Introduction: Hemophagocytic lymphohistiocytosis(HLH) is a systemic inflammatory disorder characterized by uncontrolled histiocytic proliferation, hemophagocytosis and up-regulation of inflammatory cytokines. Thus, both HLH and Kawasaki disease(KD) are characterized by prolonged fever, and are diagnosed by a clinical and laboratory scoring system, concurrent manifestation of HLH and KD has been described in the literature. We describe two cases of children who diagnosed as KD initially, but after intravenous gamma globulin(IVIG) failed to produce clinical response, were found to have HLH. Case report: A 3-year-old boy who had previous KD history 5 months ago was admitted for 9day fever and skin rash. His symptoms were fulfilled KD criteria, and echocardiography showed dilated right coronary artery of 4.2mm. He was treated with 2 cycles of IVIG until fever subsided. However, 2 days later, he got fever again and cytopenia(Hb<9.0), hypertriglyceridemia, high level of ferritin was shown and had splenomegaly on physical examination. In the suspicion of HLH, bone marrow biopsy was done and revealed hemophagocytosis, consistent with HLH. A second case of 11-month-old boy admitted for 8-day fever with Kawasaki feature. Although, he showed incomplete feature(fever, skin rash, conjunctival injection, cervical lymphadenopathy), echocardiography showed dilated left main coronary artery(3.5mm) and treated with IVIG. However, 2days after IVIG administration, he was still pyrexial. The laboratory findings fulfilled 5 diagnostic criteria of HLH; bicytopenia(anemia, thrombocytopenia), hypofibrinogenemia, hyperferritinemia, hemophagocytosis in bone marrow, raised level of soluble IL-2 receptor. In both cases, the patients treated according to the HLH protocol 2004, and after that clinical symptoms and laboratory findings were improved. Several causes of febrile illness, EBV, CMV, rubella, parvo-viral infection, for example, were excluded. Comment: There is considerable overlap between the clinical syndromes of KD and HLH and early recognition and treatment of these two disease entity is imperative to avoid fatal outcomes in severe cases. Thus, these should both be considered and excluded in any child with unremitting fever and rash.


2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Tracey Dyer ◽  
Paul Dancey ◽  
John Martin ◽  
Suryakant Shah

Kawasaki disease (KD) is an acute systemic vasculitis of childhood. The diagnosis can be made in a patient who presents with a prolonged high fever and meeting at least four of five criteria including polymorphous rash, mucosal changes, extremity changes (including swelling and/or palmar and plantar erythema), bilateral nonsuppurative conjunctivitis, and unilateral cervical lymphadenopathy. Atypical KD refers to patients who have not met the full criteria and in whom atypical features may be present. We discuss a case of a 6-year-old male who presented to the Emergency Department with torticollis. A series of investigations for elevated inflammatory markers revealed dilated coronary artery aneurysms on echocardiogram, and thus he was diagnosed with atypical KD. His only other criteria were bilateral nonsuppurative conjunctivitis and a prior brief febrile illness. He was treated with high-dose intravenous immune globulin (IVIG) and low-dose aspirin. Low-molecular-weight heparin and atenolol were added due to the presence of giant aneurysms.


2020 ◽  
Vol 8 (B) ◽  
pp. 99-103
Author(s):  
Nguyen Huu Son ◽  
Tran Kiem Hao ◽  
Nguyen Thi Hoang Anh

INTRODUCTION: Kawasaki disease (KD) is an acute self-limited systemic vasculitis of unknown etiology which affects mainly children <5 years of age. If the disease is left untreated, it can lead to serious complications such as inflammation of the blood vessels. AIM: We aim to evaluate the clinical and laboratory findings and response to therapy of KD at Hue Central Hospital. METHODS: This is a retrospective study of patients with KD at Pediatric Center of Hue Central Hospital between January 2010 and December 2019. Clinical and laboratory examinations as well as the echocardiograms finding were analyzed. RESULTS: All patients were under 5 years old, in which boys were more than girls. Fever lasting over 5 days, changing in the mouth mucosa, and peripheral extremities were seen in all patients. About 73.2% had bilateral conjunctivitis and 78.0% had rash. About 42.3% of patients had cervical lymphadenopathy. Laboratory findings were noted with 84.5% of patients had hyperleukocytosis (>12,000/ mm3), 76.2% of patients had high serum C-reactive protein (CRP) levels (>100 mg/dl), 56% of patients had erythrocyte sediment rate >60 mm in the 1st h, and 34.5% of patients had thrombocytosis (platelet count >500,000/mm3) at the time of diagnosis. About 26.2% of patients had coronary artery lesions. Most patients (84.4%) had good outcome since the first dose of gamma-globulin and 13% of patients needed the second dose. There was a significant correlation between coronary artery abnormalities and no or late treatment of gamma-globulin. CONCLUSION: KD was very common in children under 5 years old with the high rate of coronary artery lesion. Treatment with gamma-globulin on or before 10 days of fever resulted in better coronary outcomes and decreased the total length of time of clinical symptoms.


2021 ◽  
Vol 2021 ◽  
pp. 1-5
Author(s):  
Chloe Kupelian ◽  
Bindu Sathi ◽  
Deepika Singh

Kawasaki disease is an acute multisystem vasculitis characterized by involvement of medium-sized vessels that mostly affects children under the age of 5 years. The presentation is typically preceded by five or more days of fever with additional clinical findings including rash, peripheral edema, mucositis, conjunctival changes, and unilateral cervical lymphadenopathy. The most feared complication of Kawasaki disease is development of coronary artery aneurysms. Common laboratory abnormalities include normocytic anemia, thrombocytosis, leukocytosis, and elevated inflammatory markers. Immune-mediated cytopenias such as autoimmune hemolytic anemia and thrombocytopenia are rarely seen at presentation in Kawasaki disease. We describe a unique case of a child presenting with autoimmune hemolytic anemia, who sequentially developed immune thrombocytopenia concerning for Evans’ syndrome and eventually diagnosed with Kawasaki Disease with coronary artery dilatation. Characteristic clinical findings including extremity edema, cracked lips, and rash developed later in the course. Our patient was treated with IVIG and steroids with significant clinical improvement and complete resolution of cytopenias and coronary aneurysms on long term follow up. Timely administration of IVIG prevents and minimizes the risk of long term cardiac consequences. Hence a high index of suspicion should be maintained for this relatively common pediatric illness, even in absence of more commonly seen laboratory findings.


2018 ◽  
Vol 43 (3) ◽  
pp. 143-148
Author(s):  
Nurun Nahar Fatema Begum ◽  
Abdul Ali Mia ◽  
Mamun Mostafi ◽  
Khaleda Akhter ◽  
Mahbuba Sultana ◽  
...  

Kawasaki disease (KD) is an acute self-limiting inflammatory disease associated with vasculities, affecting predominantly medium sized vessels and encountered these cases infrequently in clinical practice. The most concerning complication is coronary artery aneurysm (CAA) leading to myocardial infarction or sudden death. Fever is the essential feature. Kawasaki disease is invariably associated with an inflammatory process with elevated C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and white blood cell count. Early recognition and treatment with IVIG (Intravenous venous immunoglobulin), and Aspirin showed highest protection against development of coronary artery disease. Incidence of KD is more in East Asia or in Asian ancestry living in other countries. The reason for high incidence in developing country is still unknown. Recently a significant number of cases attended in day to day practice which led conducting this study to assess the pattern of presentation, management and outcome after treatment and to aware the health care providers about the nature of the disease. This observational study conducted during September 2014 to March 2016. All the cases reported to the department of paediatric echocardiography for coronary artery analysis and suspected as KD were included in the study. History, clinical examination and investigation reports were analyzed from records. Age, gender, clinical feature, management, follow-up, echocardiography findings and outcome were analyzed. Data were collected from records of Echocardiography Department and Paediatric Cardiology Department of Lab Aid Cardiac Hospital and Combined Military Hospital, Dhaka. Among 27 cases, 16 were male (49.25%) and 11 were female (40.75%). Age distribution showed 8 (29.63%) were under one year of age, 13(48.14%) were in 1-5 years age group, 6 (22.23%) were in more than 5 years age group. All 27 cases were presented with fever, strawberry tongue, conjunctivitis, skin rash with peeling. Raised ESR, CRP and neutrophilic leukocytosis were found in all 27 cases. Cervical lymphadenopathy was found in 15 (55.52%) cases. Coronary artery changes were observed in 24 cases (88.89%). IVIG was given in 25 (92.60%) cases and Aspirin was given in 27 cases. Complete cure was achieved in 25 (92.59%) cases and one case died from thrombo-embolic event of radial artery and amputation of hand. The outcome of KD depends on the severity of coronary artery involvement. Patients with aneurysm larger than 08 mm are at highest risk of myocardial infarction. Less than 08 mm aneurismal dilatation regress with time. In this series outcome was excellent due to timely diagnosis and proper intervention.


2017 ◽  
pp. 30-35
Author(s):  
Hung Viet Phan ◽  
Ngoc Minh Chau Nguyen

Objectives: To describe clinical and paraclinical features and treatment result of Kawasaki disease. Methods: The study is based on 34 children with Kawasaki disease treated at the Pediatric center of Hue Central Hospital from 1/2012-6/2013. Clinical examination, subclinical tests were done for each child at the time of diagnosis and every week after treatment. Results: 100% of children were under 5 years old, in which boys were more than girls, and 47.1% of patients lived in Hue. 100% of patients had fever over 5 days, bilateral conjunctivitis, changes in the mouth mucosa, changes in the peripheral extremities and rash. 41.2% of patients had cervical lymphadenopathy. Paraclinical manifestations were notably with 100% of patients had leukocytosis, 70.6% of patients had serum CRP levels > 30 mg/l, 55.9% of patients had ESR > 60 mm in first hour, 29.4% of patients had thrombocytosis over 500.000/mm3 at the time of positive diagnostic. 32.4% of patients had coronary artery lesions, 5.8% of patients had mitral regurgitation and pericardial effusion. 87.5% of patients had favorable progression after treatment with 1 single dose of gammaglobulin 2 g/kg. 12.5% of patients require treatment with second dose gammaglobulin. Conclusion: Kawasaki disease is very common in children <5 years old, the rate of coronary artery lesion is very high 32.4%. Favorable progression after treatment with high-dose gamaglobulin. Key words: Kawasaki disease, clinical manifestations; intravenous immunoglobulin treatment


Author(s):  
Zaib Bin Jawaid ◽  
Jin Ling Du ◽  
Sohail Iqbal ◽  
Lei Zhang

Abstract Background  Management of cardiovascular sequelae to Kawasaki disease (KD) is challenging to adult cardiologists. Vasculitis of medium-sized arteries especially coronary arteries often leads to focal intimal thickening and aneurysmal dilatation of one or more coronary arteries. It needs special attention to recognize coronary artery involvement because of potential long-term morbidity and mortality. We present a case of diagnostic dilemma in young adult Chinese male with KD. Case summary  This asymptomatic patient was found to have deep Q waves in anterior leads on screening electrocardiography and was thought to have myocarditis after depiction of wall motion abnormality on echocardiography, later to be confirmed to have left anterior descending artery (LAD) territory infarct on cardiac magnetic resonance imaging. Coronary computed tomography angiogram depicted proximal LAD aneurysm with calcified plaque/thrombus. Additionally, there was an 18 mm giant right coronary artery (RCA) aneurysm with braid-like appearance and soft plaque (mural thrombus). His previous medical history included fever and cervical lymphadenopathy. Because of the high risk he was commenced on long-term low-dose aspirin and β-adrenergic-blocking agent to reduce myocardial oxygen consumption; however, 3 years later, he presented to the emergency department with acute inferior myocardial infarction. He was noted to have total occlusion of the proximal RCA and was treated aggressively with thrombectomy and percutaneous balloon angioplasty followed by medical management with β-blockers, sacubitril/valsartan, clopidogrel, and rivaroxaban without subsequent adverse cardiovascular events. Discussion  Kawasaki disease is one of the main causes of coronary artery disease in young adults and can be easily overlooked.


2021 ◽  
Author(s):  
Jinling Hu ◽  
Weidong Ren

Abstract Objective:To identify factors predictive of coronary artery lesions (CALs) in children with Kawasaki disease (KD).Methods:The clinical data of 420 children with KD who were hospitalized between January 2018 and December 2020 were retrospectively evaluated after assignment to groups by the presence of coronary artery aneurysm (CAA), coronary artery dilation (CAD), or no CALs. The association between coronary artery damage and patient clinical and laboratory values was investigated by pairwise comparison of the three groups. Univariate and multivariate logistic regression identified independent risk factors. The predictive value of patient variables for development of CAA and CAD was estimated by receiver operating characteristic curve analysis.Results:CALs occurred in 17.6% (94/420) of children with KD. Duration of fever, cervical lymphadenopathy, intravenous immune globulin resistance, immunoglobulin (Ig)A, procalcitonin, hemoglobin (Hb), erythrocyte sedimentation rate (ESR), C-reactive protein, and others differed between patients with CALs and without CALs and were significantly associated with the development of CAA and CAD. The largest area under the curve was for combined CAA or CAD indicators, 0.851 for CAA (sensitivity of 68.09% and specificity of 60.62%) and 0.714 for CAD (sensitivity of 65.96% and specificity of 70.77%). Fever of >7 days before treatment was predictive of severe CAA or CAD.Conclusion:Fever duration, ESR, IgA, Hb, and cervical lymphadenopathy were independent risk factors of CAA; CRP, ESR, and cervical lymphadenopathy were independent risk factors of CAD, with combined factors having increased sensitivity and specificity. Early, active treatment is essential to reduce the occurrence of CALs.


2020 ◽  
Author(s):  
Yusuf Ziya Varlı ◽  
Kazim Oztarhan

Abstract Background: Kawasaki disease (KD) is the most common cause of coronary artery aneurysm (CAA) in children. This study aimed to determine the clinical characteristics, demographic features, frequency of coronary involvement, and resistance to intravenous immunoglobulin (IVIG) treatment in Turkey based on our data.Methods: Patients with KD were evaluated with demographic data, clinical, laboratory, and echocardiographic findings. Results: Between 2010–2019, a total of 259 patients (male/female: 1.67) were treated in our hospital, with 48 (%19) cases < 1 year of age. According to diagnostic criteria, 31% were diagnosed with typical KD and 69% with atypical (incomplete) KD. The frequency of clinical findings were as follows: changes in the lips and oral mucosa (79%); polymorphic rash (69%); conjunctivitis (65%); changes in the extremities (54%); and cervical lymphadenopathy (48%). There was no significant difference between typical and atypical KD in the frequency order. CAA development and IVIG resistance occurred in 11.6% and 12.3% of cases, respectively. IVIG resistance was more common in infants and hospitalization times were longer in this group. Coronary artery lesions existed in 45 patients; right coronary artery (RCA) alone (20%), left coronary artery (LCA) alone (44.5%), and RCA and LCA together were involved (35.5%). The left main coronary artery affected 20 patients, the left anterior descending artery (LAD) affected nine patients (45%), the left circumflex artery (LCx) affected two patients (10%), and the LAD and LCx together affected two patients (10%). None of the patients had myocardial infarctions or died during follow-up. Conclusion: KD is a systemic vasculitis common in pediatric infants in which coronary artery involvement affects prognosis. Due to IVIG resistance and increased coronary involvement accompanying this vasculitis, it is an important problem in countries where the disease is common. It is important to know the factors that increase the risk of coronary involvement and IVIG resistance development.


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