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

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
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.

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

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
yeo hyang kim ◽  
Chae Ok Shin ◽  
Myung Chul Hyun ◽  
Dong Seok Lee
Keyword(s):  
Kawasaki Disease ◽  
Systolic Function ◽  
Systemic Vasculitis ◽  
Cervical Lymphadenopathy ◽  
Febrile Illness ◽  
Pulse Therapy ◽  
Left Ventricular ◽  
Acute Stage ◽  
Z Score ◽  
Intravenous Methylprednisolone

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.

Lymphadenitis as the Dominant Manifestation of Kawasaki Disease

PEDIATRICS ◽  
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.

Onset of Kawasaki disease immediately after birth

2018 ◽  
Vol 7 (2) ◽  
Author(s):  
Cristina Medeiros Ribeiro de Magalhães ◽  
Riccardo Pratesi ◽  
Lenora Gandolfi ◽  
Rosa Harume Uenishi ◽  
Claudia B. Pratesi
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
Rare Event ◽  
Cardiovascular Complications ◽  
Postnatal Period ◽  
Older Children ◽  
Coronary Artery Aneurysms ◽  
Common Causes ◽  
Therapeutic Measures

Abstract Kawasaki disease (KD) is one of the most common causes of childhood systemic vasculitis, with the potential to cause significant cardiovascular complications, especially if undiagnosed. An investigation of coronary artery abnormalities with the application of appropriate therapeutic measures can improve the prognosis in these infants, decreasing the risk of coronary artery aneurysms. Neonatal KD is a rare event, often characterized only by few of the clinical features observed in older children. In this case report, we describe a newborn male who presented an incomplete clinical presentation of KD, where symptoms of the disease erupted immediately after birth. The objective of this report is to draw the attention of pediatricians and neonatologists to the possible presence of KD in an asymptomatic newborn during the immediate postnatal period, even in the absence of the classic features of KD, such as fever.

scholarly journals Kawasaki Disease Presenting as Acute Acalculous Cholecystitis

2019 ◽  
Vol 3 (4) ◽  
pp. 383-386 ◽  
Author(s):  
Demis Lipe ◽  
Lindsey Bridges
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Acalculous Cholecystitis ◽  
Acute Acalculous Cholecystitis ◽  
High Dose ◽  
Coronary Artery Aneurysms ◽  
Abdominal Symptoms ◽  
Dose Aspirin ◽  
Serious Disease ◽  
High Dose Aspirin

Acute acalculous cholecystitis (AAC) is a rare, potentially serious disease that has been associated with Kawasaki disease (KD) in children. Studies suggest that patients presenting with severe abdominal symptoms secondary to KD have increased resistance to intravenous immunoglobulin (IVIG), and a higher rate of coronary artery aneurysms. We describe an eight-year-old boy who presented to the emergency department with severe abdominal pain and was diagnosed with AAC and KD. He was treated with IVIG and high-dose aspirin, achieving good response with complete symptom resolution. He had no coronary artery aneurysms or further complications and was discharged after three days.

Patient-Specific Hemodynamic Simulations in a Group of Patients With Coronary Artery Aneurysms Caused by Kawasaki Disease

2013 ◽  
Author(s):  
Dibyendu Sengupta ◽  
Jane C. Burns ◽  
Andrew Kahn ◽  
Alison L. Marsden
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Thrombus Formation ◽  
Anticoagulation Therapy ◽  
Febrile Illness ◽  
Maximum Diameter ◽  
Patient Specific ◽  
Coronary Artery Aneurysms ◽  
Patients At Risk ◽  
Life Threatening

Kawasaki disease (KD) is an acute febrile illness that can result in life threatening coronary artery aneurysms in up to 25% of untreated patients. These aneurysms put patients at risk for thrombus formation, myocardial infarction and sudden death. Currently, clinical decisions are made based on anatomy alone, with aneurysm diameter > 8mm as the arbitrary cutoff for anticoagulation therapy, despite a lack of evidence for this choice. We postulate that patient specific hemodynamics may be a better predictor for the risk of thrombosis than maximum diameter alone. To quantify hemodynamics, we performed computational fluid dynamics (CFD) simulations using patient specific models with custom coronary boundary conditions.

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

Children ◽  
2018 ◽  
Vol 5 (11) ◽  
pp. 155 ◽  
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.

Atypical Kawasaki Disease with Coronary Artery Aneurysms

1988 ◽  
Vol 5 (2) ◽  
pp. 137-144
Author(s):  
PAUL M. SEIB ◽  
DANIEL J. MURPHY ◽  
ACHI LUDOMIRSKY
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Coronary Artery Aneurysms ◽  
Atypical Kawasaki Disease

scholarly journals Epidemiological Features and Clinical Study of Kawasaki Disease in Iran

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.

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

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.

scholarly journals The Relationship Between TNF-Like Protein 1A and Coronary Artery Aneurysms in Children With Kawasaki Disease

2021 ◽  
Author(s):  
jing zhang ◽  
Haobo Weng ◽  
Qiongfei Pei ◽  
Penghui Yang ◽  
Wentao Fan ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Development Process ◽  
Systemic Vasculitis ◽  
Artery Aneurysm ◽  
Unknown Etiology ◽  
Coronary Artery Aneurysms ◽  
Tnf Superfamily ◽  
The Relationship ◽  
Necrosis Factor

Abstract Background: Kawasaki disease (KD) is an acute, systemic vasculitis of unknown etiology that occurs predominantly in infants and children, and the most crucial complication of KD is coronary artery aneurysm (CAA). Tumor necrosis factor (TNF)-like protein 1A (TL1A) is a member of the TNF superfamily, which possesses the ability of maintaining vascular homeostasis and regulating immune response. This study aims to examine the serum TL1A levels in KD patients, and to investigate the relationship between TL1A and CAAs in children with KD.Methods: Blood samples were recruited from 119 KD patients, 35 febrile controls (FCs) and 37 healthy controls (HCs). The KD group was further divided into KD with CAAs (KD-CAAs) and KD non-CAAs (KD-NCAAs) groups. Serum TL1A levels were measured using enzyme-linked immunosorbent assays, and clinical parameters were collected in KD patients. Results: Serum TL1A levels in the acute phase of KD patients were significantly higher than that in the FC and HC groups. In particular, serum TL1A were substantially increased in the KD-CAA group than that in the KD-NCAA group. Furthermore, TL1A levels were positively correlated with the duration of fever, time point of IVIG and WBC levels, but negatively correlated with levels of RBC, Hb and Albumin in the KD group. Conclusions: TL1A might be involved in the KD-associated vasculitis, and might be a factor in the development process of CAAs.

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