Abstract 132: Methylprednisolone Pulse Therapy for Kawasaki Disease with Symptomatic Myocarditis

Purpose: Kawasaki disease (KD) is an acute febrile illness of infants and young children that is characterized by a systemic vasculitis, especially involving the coronary arteries. Although, sometimes, subclinical myocarditis is combined in KD, symptomatic myocarditis is extremely uncommon. We report a 7 year old boy who developed hypotension and decreased left ventricular systolic function (EF 40%) in the acute phase of KD. Case: A 7 year old boy (height 115 cm, body weight 20 kg) was admitted because of 2 days of persistent fever and left cervical lymphadenopathy (white blood cell count 17,870 /mm 3 , C reactive protein 23.6 mg/dL). Conjunctiva injection and lip redness developed on the 4th day of illness, and hypotension and tachycardia (SBP 59/DBP 29 mmHg, HR 153/bpm) were combined. The echocardiography revealed a decreased ejection fraction (EF) (40%) without chamber dilatation and normal coronary artery size (LM 1.9mm, z score=-1.3, RCA 2.3mm, z score=0.4). The level of N terminal pro BNP was 28,000 pg/mL. With a diagnosis of KD with myocarditis, he was initially treated with inotropics and intravenous immunoglobulin (2 g/kg). Without clinical improvement in spite of initial treatment, A change of coronary arterial size (LM 2.9mm, z score=1.2, RCA 3.1mm, z score=2.3) was developed and decreased LV systolic function (EF 45%) and fever were persisted. Then, he was given 3 daily pulses of intravenous methylprednisolone followed by tapering doses of oral prednisolone. He showed prompt clinical recovery after pulse therapy of intravenous methylprednisolone (SBP 95/DBP 49 mmHg, HR 98/bpm). Although EF was improved (59%), coronary arterial dilatation was progressed (LM 3.4mm, z score=2.4 RCA 5.5mm, z score=7.9). Conclusions: The present case serves to highlight the fact that methylprednisolone should be considered as the priority in children with KD who have symptomatic myocarditis during the acute stage.

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Torticollis as Presentation for Atypical Kawasaki Disease Complicated by Giant Coronary Artery Aneurysms

Case Reports in Pediatrics ◽

10.1155/2018/4236264 ◽

2018 ◽

Vol 2018 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

Tracey Dyer ◽

Paul Dancey ◽

John Martin ◽

Suryakant Shah

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Systemic Vasculitis ◽

Cervical Lymphadenopathy ◽

Febrile Illness ◽

High Dose ◽

Coronary Artery Aneurysms ◽

Low Dose Aspirin ◽

Atypical Kawasaki Disease ◽

Mucosal Changes

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.

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Lymphadenitis as the Dominant Manifestation of Kawasaki Disease

PEDIATRICS ◽

10.1542/peds.93.3.525 ◽

1994 ◽

Vol 93 (3) ◽

pp. 525-528

Author(s):

Julie Kim Stamos ◽

Kathleen Corydon ◽

James Donaldson ◽

Stanford T. Shulman

Keyword(s):

Kawasaki Disease ◽

Cervical Lymphadenopathy ◽

Febrile Illness ◽

The United States ◽

Acute Febrile Illness ◽

Conjunctival Injection ◽

Coronary Abnormalities ◽

Acquired Heart Disease ◽

Mucosal Changes ◽

Infants And Young Children

Kawasaki disease (KD) is an acute febrile illness primarily affecting infants and young children. Its importance relates to the fact that 20% to 25% of untreated patients develop coronary abnormalities that can lead to myocardial infarction or even to death.1 KD is a leading cause of acquired heart disease in children in many regions, including the United States.2 Because there are no specific diagnostic tests for KD, the diagnosis is established by the presence of fever and four of five criteria without other explanation for the illness: (1) nonexudative conjunctival injection; (2) oral mucosal changes; (3) changes of the peripheral extremities; (4) rash, primarily truncal; and (5) cervical lymphadenopathy.

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Comprehensive pathogen detection in sera of Kawasaki disease patients by high-throughput sequencing: a retrospective exploratory study

BMC Pediatrics ◽

10.1186/s12887-020-02380-7 ◽

2020 ◽

Vol 20 (1) ◽

Author(s):

Yuka Torii ◽

Kazuhiro Horiba ◽

Satoshi Hayano ◽

Taichi Kato ◽

Takako Suzuki ◽

...

Keyword(s):

Kawasaki Disease ◽

High Throughput ◽

High Throughput Sequencing ◽

Sequence Data ◽

Early Stage ◽

Rna Virus ◽

Systemic Vasculitis ◽

Human Herpesvirus ◽

Acute Stage ◽

Serum Samples

Abstract Background Kawasaki disease (KD) is an idiopathic systemic vasculitis that predominantly damages coronary arteries in children. Various pathogens have been investigated as triggers for KD, but no definitive causative pathogen has been determined. As KD is diagnosed by symptoms, several days are needed for diagnosis. Therefore, at the time of diagnosis of KD, the pathogen of the trigger may already be diminished. The aim of this study was to explore comprehensive pathogens in the sera at the acute stage of KD using high-throughput sequencing (HTS). Methods Sera of 12 patients at an extremely early stage of KD and 12 controls were investigated. DNA and RNA sequences were read separately using HTS. Sequence data were imported into the home-brew meta-genomic analysis pipeline, PATHDET, to identify the pathogen sequences. Results No RNA virus reads were detected in any KD case except for that of equine infectious anemia, which is known as a contaminant of commercial reverse transcriptase. Concerning DNA viruses, human herpesvirus 6B (HHV-6B, two cases) and Anelloviridae (eight cases) were detected among KD cases as well as controls. Multiple bacterial reads were obtained from KD and controls. Bacteria of the genera Acinetobacter, Pseudomonas, Delfita, Roseomonas, and Rhodocyclaceae appeared to be more common in KD sera than in the controls. Conclusion No single pathogen was identified in serum samples of patients at the acute phase of KD. With multiple bacteria detected in the serum samples, it is difficult to exclude the possibility of contamination; however, it is possible that these bacteria might stimulate the immune system and induce KD.

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Abdominal lymphadenopathy in an adolescent with Kawasaki disease: a major sign?

International Journal of Adolescent Medicine and Health ◽

10.1515/ijamh-2016-0028 ◽

2016 ◽

Vol 29 (6) ◽

Author(s):

Danielle Bulkool ◽

Ana Vidal de Carvalho ◽

Aurea Grippa ◽

Marcia Fernandes ◽

Israel Figueiredo

Keyword(s):

Kawasaki Disease ◽

Systemic Vasculitis ◽

Relevant Literature ◽

Cervical Lymphadenopathy ◽

Diagnostic Challenge ◽

Clinical Criteria ◽

Pubmed Search ◽

Clinical Pictures ◽

Abdominal Lymphadenopathy

Abstract In adolescence, Kawasaki disease (KD) may present with varied clinical pictures, and the classic clinical criteria are often insufficient to establish the diagnosis. This report presents a clinical case of KD in an adolescent and a brief review of the relevant literature, based on a PubMed search using the terms “adolescence” and “KD”. A 13-year-old girl presented with fever, rash, hepatomegaly, and ultrasonographic evidence of abdominal lymphadenopathy. On week 2 after onset of symptoms, the patient developed desquamation of the extremities and thrombocytosis. Echocardiography was normal during the acute illness and remained unchanged at 2-year follow-up. Clinicians should be aware that, in adolescents, KD poses a diagnostic challenge and rarely corresponds to the classic presentation. On retrospective analysis, we believe that abdominal lymphadenopathy should have been recognized as a substitute manifestation of the classic symptom of cervical lymphadenopathy to establish the diagnosis of this fearsome systemic vasculitis syndrome.

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Methylprednisolone as an alternative therapy for Kawasaki disease: case series

Paediatrica Indonesiana ◽

10.14238/pi60.5.2020.283-6 ◽

2020 ◽

Vol 60 (5) ◽

pp. 283-6

Author(s):

Yudha Fadhol Arafah ◽

Sasmito Nugroho ◽

Noormanto Noormanto ◽

Nadya Arafuri ◽

Indah Kartika Murni

Keyword(s):

Kawasaki Disease ◽

Systemic Vasculitis ◽

Alternative Therapy ◽

Case Series ◽

Cervical Lymphadenopathy ◽

Conjunctival Hyperemia ◽

Disease Case ◽

The Usa ◽

Acquired Heart Disease ◽

The Uk

Kawasaki disease (KD), or mucocutaneous syndrome, is an acute, systemic vasculitis of small- and medium-sized arteries that predominantly affects patients younger than five years.1 KD is the leading cause of childhood acquired heart disease in the developed world.2 The incidence in those aged under 5 years varies widely throughout the world, accounting for 8.4 per 100,000 in the UK, 17.5 to 20.8 per 100,000 in the USA, and 239.6 per 100,000 in Japan.2 The diagnosis of classic KD is based on the simultaneous presence of high fever for 5 or more days with at least four of five other symptoms (bilateral conjunctival hyperemia, ulcerations of the lips and inflammation of the oral cavity, polymorphous rash, edema and desquamation of the extremities, and cervical lymphadenopathy), or fever associated with less than 4 of the diagnostic criteria and echocardiographic abnormalities of the coronary arteries.3

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Recurrent Kawasaki Disease: A Case Report of Three Separate Episodes at >4-Year Intervals

Children ◽

10.3390/children5110155 ◽

2018 ◽

Vol 5 (11) ◽

pp. 155 ◽

Cited By ~ 1

Author(s):

Nikita Goswami ◽

Katherine Marzan ◽

Elizabeth De Oliveira ◽

Sharon Wagner-Lees ◽

Jacqueline Szmuszkovicz

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Systemic Vasculitis ◽

High Dose ◽

Z Score ◽

Coronary Aneurysms ◽

Increased Risk ◽

The Right ◽

Work Up ◽

High Dose Aspirin

Kawasaki disease (KD) is a self-limited systemic vasculitis, most often occurring in children 1–5 years old. It has a 2% recurrence rate and is associated with coronary aneurysms (CA), which can develop within two weeks of onset. A 25% increased risk is noted in patients who are recalcitrant to treatment. We describe a patient with recurrence of KD three times, approximately four years apart. A 10-year-old female with two previous episodes of KD, at 11 months and five years of age), in which she met five out of five criteria for KD and had no coronary involvement, presented with 15 days of fever, conjunctivitis and mucocutaneous changes. Infectious work-up was negative, and she was diagnosed with incomplete KD meeting three out of five criteria. An echocardiogram (ECHO) on day 12 revealed dilation of the right coronary artery (RCA) and left coronary artery (LCA). Treatment with intravenous immunoglobulin (IVIG) and high-dose aspirin was started at an outside hospital. After transfer, serial ECHOs showed evolving coronary aneurysms, left anterior descending (LAD) z-score + 8.2 and RCA z-score + 4.0. She received 10 mg/kg infliximab (day 18) and began clopidogrel. A cardiac MRI (day 20) demonstrated progression of the LAD aneurysm, with a z-score + 13, and warfarin was started. To our knowledge, this is the first report of recurrent KD occurring three times at ~5 year intervals.

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Epidemiological Features and Clinical Study of Kawasaki Disease in Iran

ACTA MEDICA IRANICA ◽

10.18502/acta.v58i12.5154 ◽

2021 ◽

Author(s):

Parvin Akbariasbagh ◽

Saharnaz Talebiyan ◽

Yahya Aghighi ◽

Reza Raeeskarai ◽

Amirhosein Seyedhoseinpour ◽

...

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Clinical Features ◽

Systemic Vasculitis ◽

Febrile Illness ◽

Developed Countries ◽

Response To Treatment ◽

Unknown Etiology ◽

Laboratory Findings ◽

Female Ratio

Kawasaki disease (KD) is an acute febrile systemic vasculitis of unknown etiology and the major cause of pediatric acquired cardiac disease worldwide, particularly in developed countries. This study characterizes the epidemiologic and clinical features of KD in the Pediatric Rheumatology Department service in a general hospital. 120 patients with the diagnosis of KD between 1990 and 2009 were enrolled. We investigated the epidemiologic and clinical features of coronary artery involvement of the patients. Frequency of many parameters including age, sex, season, clinical and laboratory findings, response to treatment, and complications of the patients determined. During the 20-year study period, 120 patients <15 years of age were admitted for KD. Among them, 39.2% were at the extremes of the age spectrum, with 2.5% <6 months and 36.7% >5 years of age, male to female ratio of 1.3:1 and the classic KD to incomplete KD ratio of 3.1:1. KD recurred in 5% of all cases. It occurred most frequently in the winter and least frequently in the summer. The occurrence of coronary artery abnormalities (CAA) was 4.2%. Kawasaki disease should be considered in any pediatric patients with a prolonged refractory febrile illness in order to prevent CAA formation.

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A Retrospective Cohort Study of Kawasaki Disease in Hue Central Hospital for 10 Years (2010–2019)

Open Access Macedonian Journal of Medical Sciences ◽

10.3889/oamjms.2020.3858 ◽

2020 ◽

Vol 8 (B) ◽

pp. 99-103

Author(s):

Nguyen Huu Son ◽

Tran Kiem Hao ◽

Nguyen Thi Hoang Anh

Keyword(s):

Coronary Artery ◽

Kawasaki Disease ◽

Gamma Globulin ◽

Systemic Vasculitis ◽

Cervical Lymphadenopathy ◽

Response To Therapy ◽

High Rate ◽

Unknown Etiology ◽

Central Hospital ◽

Laboratory Findings

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.

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Eosinophil as the major factor of the nomogram model to differentiate Kawasaki disease from other febrile illnesses

10.21203/rs.2.19021/v1 ◽

2019 ◽

Cited By ~ 1

Author(s):

Xiao-Ping Liu ◽

Yi-Shuang Huang ◽

Ho-Chang Kuo ◽

Han-Bing Xia ◽

Yi Sun ◽

...

Keyword(s):

Logistic Regression ◽

Kawasaki Disease ◽

Systemic Vasculitis ◽

Laboratory Data ◽

Area Under The Curve ◽

Febrile Illness ◽

Validation Group ◽

Model Group ◽

Laboratory Findings ◽

Major Factors

Abstract Background: Kawasaki disease (KD) is a systemic vasculitis in children less than 5 years old. At present, no single laboratory data can distinguish KD from other febrile diseases. To establish a differential diagnosis laboratory data model between KD and other febrile diseases, which can avoid coronary artery complications.Methods: A total of 800 children (249 KD and 551 non-KD febrile illness) were enrolled. Laboratory findings were analyzed by of univariable，multivariable logistic regression and nomogram.Results: 562 children were randomly selected as the model group and 238 as the validation group. The predictive nomogram included eosinophil percentage（100 points）, C-reactive protein (93 points), alanine transaminase (84 points), albumin (79 points), and white blood cell（64 points）, which generated an area under the curve of 0.873 for model group, and 0.905 for validation. Eosinophil showed highest OR: 5.015 (95% CI: 3.068-8.197) during multiple logistic regression. The sensitivity and specificity were 84.1% and 86% in the validation group. The calibration curves of the validation group for the probability of KD showed nearly agreement to the actual probability.Conclusion: Eosinophil is the major factors in this nomogram model and had a high precision to predict KD. This is the first report from literature review showed the important role of eosinophil in KD.

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Abstract 210: Kawasaki Disease in Patients Older Than 10-years Old in a Children’s Hospital In Mexico City

Circulation ◽

10.1161/circ.131.suppl_2.210 ◽

2015 ◽

Vol 131 (suppl_2) ◽

Author(s):

ITZEL E RIOS-OLIVARES ◽

LUIS M GARRIDO-GARCIA

Keyword(s):

Kawasaki Disease ◽

Mexico City ◽

Systemic Vasculitis ◽

Clinical Manifestations ◽

Cervical Lymphadenopathy ◽

Skin Lesions ◽

Clinical Feature ◽

Unknown Etiology ◽

Cardiac Complications ◽

Increased Risk

Background: Kawasaki Disease (KD) is an acute febrile illness characterized by systemic vasculitis of unknown etiology. Cardiac sequelae, such as coronary artery aneurysms (CAA), are one of the most important aspects of this disease. Actually KD is most frequently presented in children younger than 5-years old. Objective: To describe the clinical and laboratory features, cardiac sequelae and outcome in children older than 10-years old with KD who were attended at the Instituto Nacional de Pediatría in Mexico City. Methods: An observational, descriptive, retrospective and transversal case study. We reviewed the medical records of patients older than 10-years diagnosed with KD from August 1995 to May 2014, and analyzed gender, age, clinical manifestations, hemoglobin, leucocyte count, platelet count, ESR, CRP, albumin, sodium, potassium, AST, ASL, time from the onset of the symptoms to diagnosis, treatment used, the development of CAA and outcome in the acute phase of the disease. Results: We studied 18 cases of KD in patients older than 10-years old, 72.2% (13 of 18) were male with a mean age of 154 months (range 120 to 200). The time from the onset of the fever to diagnosis was 10.6 ± 5.8 days, (range 3 to 21 days). Skin lesions were the most common manifestation of KD and cervical lymphadenopathy was the least common clinical feature. 2 patients presented with KD shock syndrome. Complete KD was diagnosed in only 50% (9 of 18) of our cases. 16 patients received IVGG, 2 patients required a second GGIV dose and 10 patients also received steroids. 6 of 18 patients (30%) developed CAA. There were no deaths in our group. Conclusions: KD in patients older than 10-years old represent a clinical challenge because in the majority of the cases they presented with an atypical clinical picture which contribute to a delayed diagnosis. Also there is an increased risk of developing cardiac complications and CAA in this group of patients.

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