Multisystem inflammatory syndrome with features of Atypical Kawasaki disease during COVID-19 pandemic: Report of a case from India

Abstract There is a global concern of increasing number of children presenting with inflammatory syndrome with clinical features simulating Kawasaki disease, during ongoing COVID-19 pandemic. We report a very similar case of 5-year-old boy from a COVID-19 hotspot area in Kerala state of India who presented in late April 2020 with acute febrile illness with abdominal pain and loose stools followed by shock. On examination, child had bulbar conjunctivitis and extremity edema. Initial investigations showed high inflammatory parameters, elevated serum creatinine and liver enzymes. Echocardiography showed moderate LV dysfunction and normal coronaries. Cardiac enzymes were also elevated, suggesting myocarditis. He was treated with inotropic support, respiratory support with High Flow Nasal Cannula, IV Immunoglobulins, aspirin, steroids and diuretics. RT PCR for SARS-CoV-2 was negative twice. His clinical condition improved rapidly, was afebrile from day 2, inflammatory parameters decreased, left ventricular function improved and was discharged after 6 days of hospital stay.

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Affinché non MIS-Cappi il caso: la sindrome infiammatoria multisistemica SARS-CoV-2-correlata

Medico e Bambino pagine elettr ◽

10.53126/mebxxiv289 ◽

2021 ◽

Vol 24 (10) ◽

pp. 289-297

Author(s):

Salvatore Giordano ◽

Ilaria Pirrone ◽

Giulia Angela Restivo ◽

Filippo Collura ◽

Giovanni Francesco Saia ◽

...

Keyword(s):

Kawasaki Disease ◽

Rare Condition ◽

Good Prognosis ◽

Left Ventricular ◽

Case Definition ◽

Left Ventricular Ejection ◽

Ventricular Ejection Fraction ◽

Reactive Protein ◽

Atypical Kawasaki Disease ◽

Inflammatory Syndrome

Background - Covid-19 is less frequent and milder in children than in adults. However, cases of multisystem inflammatory syndrome temporally associated with SARS-CoV-2 (MIS-C) have been reported in children, whose phenotype resembles atypical Kawasaki disease. Objectives - The paper describes incidence as well as clinical, laboratoristic and radiological findings of MIS-C. Materials and methods - In the Paediatric Infectious Disease Centre of Palermo (Italy), a targeted surveillance for MIS-C from March 2020 to January 2021 was conducted. Case definition included WHO, UK and US criteria. Results - The paper reports the cases of 9 patients with MIS-C out of which 8 (88%) were hospitalized after September 2020. 44% were male and their median age was 6.5 years. Organ-system involvement included gastrointestinal (66%), cardiovascular (66%), mucocutaneous (88%), reticuloendothelial (77%) and respiratory (55%) systems. C-reactive protein (CRP), procalcitonin, D-dimer and pro-B-type natriuretic peptide levels (pro-BNP) were high in all patients. Chest radiography showed bilateral ground glass-opacities (55%) and pleural effusions (44%). Abdominal imaging findings included small-volume ascites (55%) and mesenteric lymphadenopathy (22%). Echocardiogram showed transient valves regurgitation (55%). In all the patients, the left ventricular ejection fraction was normal and coronary-artery aneurysms were not documented. They were treated with immunomodulating therapies. All patients neither received intensive care nor died. Conclusions - MIS-C represents a new systemic inflammatory syndrome with a phenotype resembling Kawasaki disease. MIS-C remains a rare condition, in which gastrointestinal and mucocutaneous involvement is predominant, nevertheless cardiovascular involvement must be investigated. MIS-C could be suspected even if SARS-CoV-2 exposure precedes the onset of the symptoms by more than 6 weeks. In most cases, a good prognosis might be expected.

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

Circulation ◽

10.1161/circ.131.suppl_2.132 ◽

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.

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474. Unique Treatment Challenges with Multisystem Inflammatory Syndrome in Children (MIS-C) compared to Kawasaki Disease Shock Syndrome

Open Forum Infectious Diseases ◽

10.1093/ofid/ofab466.673 ◽

2021 ◽

Vol 8 (Supplement_1) ◽

pp. S338-S339

Author(s):

Rachel Downey Quick ◽

Keren Hasbani ◽

Donald Murphey ◽

Mariosl Fernandez ◽

Kenneth Shaffer ◽

...

Keyword(s):

Kawasaki Disease ◽

Myocardial Dysfunction ◽

Left Ventricular ◽

Ivig Treatment ◽

Medical Treatments ◽

Cardiac Recovery ◽

Prompt Treatment ◽

Hospital Comparison ◽

Inflammatory Syndrome ◽

Ivig Administration

Abstract Background Kawasaki disease (KD) and Multisystem Inflammatory Syndrome in Children (MIS-C) associated with Coronavirus Disease 2019 present similarly with mucocutaneous symptoms and fever. Both syndromes can progress to shock. Successful treatments for MIS-C are largely based on proven KD management. As more patients with MIS-C are treated, protocols are adjusted. Infectious Diseases (ID) specialists are often early consultants in these cases. Understanding differences in how body systems are affected in MIS-C versus KD is essential for management. Figure 1. Cardiac changes among patients with Kawasaki Disease shock syndrome (KDSS) and Muti-system Inflammatory Syndrome (MIS-C) Methods This is a single hospital comparison of 25 cases of MIS-C with mucocutaneous presentation and symptoms of shock and 25 consecutive cases of KD Shock Syndrome (KDSS). Cases were compared for demographics, symptoms, cardiac abnormalities, medical treatments, and cardiac recovery. Results Patients with MIS-C develop symptoms of shock including sustained hypotension and tachycardia at 3 times the rate of patients with KD (45% vs 13%; p< 0.001). On echocardiogram, left ventricular myocardial dysfunction, assessed by ejection fraction, is more commonly noted in cases of MIS-C than KDSS (fig 1). About half of patients with MIS-C show left ventricular myocardial dysfunction initially with normalization by 6 months post-presentation in the majority (96%). Conclusion Cardiac changes and shock events related to KD and MIS-C are thought to be caused by differing inflammatory mediators. By comparing these two syndromes, we can determine ways to manage each optimally. MIS-C often results in left ventricular myocardial dysfunction, which is rarer in KD cases. Fluid resuscitation with multiple fluid boluses followed by inotropes to treat hypotension in cases of in MIS-C puts increased strain on the already weakened myocardium. Early intravenous immunoglobulin (IVIG) administration, even in the presence of mild hypotension, can simultaneously provide the patient with additional fluid and decrease the underlying inflammatory process. This prompt treatment might reduce the need for pressor support while protecting the myocardium from further damage. As early consultants in MIS-C, ID providers should be educated regarding the unique cardiac challenges of MIS-C and avoid delay in IVIG treatment and cardiologist and intensivist consultation. Disclosures All Authors: No reported disclosures

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KAWASAKI DISEASE AND MULTISYSTEM INFLAMMATORY SYNDROME IN CHILDREN WITH COVID-19 INFECTION

PEDIATRIA Journal named after G N SPERANSKY ◽

10.24110/0031-403x-2020-99-6-209-219 ◽

2020 ◽

Vol 99 (6) ◽

pp. 209-219

Author(s):

L.V. Bregel ◽

◽

M.M. Kostik ◽

L.Z. Fell ◽

O.S. Efremova ◽

...

Keyword(s):

Kawasaki Disease ◽

Hemophagocytic Syndrome ◽

Early Recognition ◽

Gastrointestinal Symptoms ◽

The United States ◽

Interleukin 10 ◽

Left Ventricular ◽

Urgent Care ◽

High Dose ◽

Inflammatory Syndrome

During the COVD-19 pandemic, some pediatric patients in many countries around the world experienced a syndrome resembling a severe Kawasaki disease (KD), often accompanied by shock. Due to the incomplete signs of the classic KD in the era before the present pandemic, in many publications from European countries and the United States, this condition was called «multisystem inflammatory syndrome in children – MIS-C» or «hyperinflammatory shock» or «Kawasaki-like syndrome». This syndrome with a new coronavirus infection is characterized by refractory fever, frequent gastrointestinal symptoms, heart damage (including coronary dilation in some patients, and acute left ventricular failure in the majority), increased ESR and CRP levels, neutrophilia, extremely high troponin levels, increased ferritin, AST, ALT, lactate dehydrogenase, creatine phosphate kinase, interleukin-6 and interleukin-10, coagulopathy with an increase in D-dimer and fibrinogen, thrombocytopenia, sometimes procalcitonin increase. The manifestations of a cytokine storm may meet the criteria for secondary hemophagocytic syndrome. The mechanism of myocardial damage remains unclear. Treatment with high-dose intravenous immunoglobulin is effective, and in the presence of signs of hemophagocytic syndrome, dexamethasone or methylprednisolone. Further research is needed to understand the pathogenesis, resemblance and differences of this syndrome with classic KD, understanding of heart injuiry and early recognition for the need of urgent care.

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Atypical Kawasaki Disease with Aortic Aneurysm

PEDIATRICS ◽

10.1542/peds.68.6.885 ◽

1981 ◽

Vol 68 (6) ◽

pp. 885-888 ◽

Cited By ~ 1

Author(s):

Charles E. Canter ◽

Richard J. Bower ◽

Arnold W. Strauss

Keyword(s):

Myocardial Infarction ◽

Kawasaki Disease ◽

Aortic Aneurysm ◽

Unusual Case ◽

Febrile Illness ◽

Signs And Symptoms ◽

The United States ◽

Abdominal Aortic ◽

Atypical Kawasaki Disease ◽

Arterial Aneurysms

Kawasaki disease (mucocutaneous lymph node syndrome), initially described in Japan, is now being seen with increasing frequency in the United States.1 The diagnosis is based on the typical constellation of signs and symptoms.1,2 Mortality is 1% to 2%, and significant morbidity results from aneurysmal formation in midsize arteries, especially the coronary arteries, which may result in rupture or myocardial infarction. We report an unusual case of Kawasaki disease. The initial febrile illness was an atypical presentation. A large abdominal aortic aneurysm developed, which subsequently was resected. Seven months after the febrile illness an asymptomatic myocardial infarction secondary to bilateral coronary arterial aneurysms was documented.

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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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Multisystem Inflammatory Syndrome with Features of Atypical Kawasaki Disease during the COVID-19 Pandemic

Case Reports in Pediatrics ◽

10.1155/2021/9950588 ◽

2021 ◽

Vol 2021 ◽

pp. 1-4

Author(s):

Reza Shahriarirad ◽

Anahita Sanaei Dashti ◽

Shabnam Hajiani Ghotbabadi

Keyword(s):

Kawasaki Disease ◽

Systemic Vasculitis ◽

Organ Damage ◽

Laboratory Evaluation ◽

Number Of Children ◽

Reactive Protein ◽

Public Crisis ◽

Exact Etiology ◽

Novel Coronavirus ◽

Inflammatory Syndrome

Background. Although Kawasaki disease (KD) is the most common self-limited systemic vasculitis in pediatrics, the exact etiology of the disease, its association with other diseases, and pathogens is still unknown. In order to achieve a better understanding and management of the disease, documentation and reporting of atypical cases is justified, particularly with the growing number of children with inflammatory syndrome with clinical features simulating KD during the COVID-19 pandemic. Here, we present a case of an atypical case of KD presenting as multisystem inflammatory syndrome (MIS) during the COVID-19 pandemic. Case Presentation. The patient is a 7-year-old girl who developed fever (39°C) and erythematous multiform rash on the abdomen and along with erythema and edema on the extremities. Laboratory evaluation revealed neutrophilia and lymphopenia along with elevated C-reactive protein, erythrocyte sedimentation rate, troponin, lactate dehydrogenase, ferritin, and D-dimer. Although the patient did not fulfill the KD criteria, based on approved guidelines and approaches regarding atypical KD and multisystem inflammatory syndrome in children (MIS-C) during the COVID-19 pandemic, intravenous immunoglobulin along with aspirin was administered for the patients. The patient’s symptoms resolved with an uneventful postdischarge course. Conclusion. Early diagnosis and treatment of patients meeting full or partial criteria for KD are critical to preventing end-organ damage and other long-term complications, especially during times of public crisis and global health emergencies, such as the novel coronavirus pandemic.

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The Trilogy of SARS-CoV-2 in Pediatrics (Part 2): Multisystem Inflammatory Syndrome in Children

The Journal of Pediatric Pharmacology and Therapeutics ◽

10.5863/1551-6776-26.4.318 ◽

2021 ◽

Vol 26 (4) ◽

pp. 318-338

Author(s):

Van L. Tran ◽

Sarah Parsons ◽

Andrew Nuibe

Keyword(s):

Kawasaki Disease ◽

Clinical Symptoms ◽

Gastrointestinal Symptoms ◽

Febrile Illness ◽

Current Treatment ◽

Published Data ◽

Treatment Considerations ◽

Cardiac Manifestations ◽

Effectiveness Studies ◽

Inflammatory Syndrome

Multisystem Inflammatory Syndrome in Children (MIS-C) was first recognized as a novel illness in 2020 with manifestations similar to other hyperinflammatory syndromes, such as Kawasaki disease or macrophage activation syndrome. Severity varies from a self-limited febrile illness to shock requiring inotropes and mechanical ventilation. Gastrointestinal symptoms and persistent fevers are the most common clinical symptoms, with the addition of cardiac manifestations inclusive of ventricular dysfunction and coronary artery aneurysms. With no controlled trials or comparative effectiveness studies evaluating treatment of MIS-C to date, current treatment with immunomodulatory agents has mainly been derived from previous experience treating Kawasaki disease. This article provides a comprehensive review summarizing published data for the evaluation and management of MIS-C, with a focus on pharmacotherapy treatment considerations.

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Multisystem Inflammatory Syndrome with Features of Atypical Kawasaki Disease during COVID-19 Pandemic

The Indian Journal of Pediatrics ◽

10.1007/s12098-020-03357-1 ◽

2020 ◽

Vol 87 (9) ◽

pp. 745-747 ◽

Cited By ~ 27

Author(s):

Abdul Rauf ◽

Ajay Vijayan ◽

Shaji Thomas John ◽

Raghuram Krishnan ◽

Abdul Latheef

Keyword(s):

Kawasaki Disease ◽

Atypical Kawasaki Disease ◽

Inflammatory Syndrome

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Multisystem inflammatory syndrome in Indian adolescents associated with SARS-CoV-2 infection: a case report

The Egyptian Journal of Internal Medicine ◽

10.1186/s43162-021-00085-6 ◽

2021 ◽

Vol 33 (1) ◽

Author(s):

Rahul D. Bhiwgade ◽

M. C. Nischitha ◽

Bhushan Shahare ◽

Shobhna Bitey

Keyword(s):

Kawasaki Disease ◽

Severe Disease ◽

Late Presentation ◽

Case Presentation ◽

Empiric Antibiotics ◽

Non Invasive ◽

Indian Adolescents ◽

Atypical Kawasaki Disease ◽

Polymerase Chain ◽

Inflammatory Syndrome

Abstract Background Adolescents with coronavirus disease 2019 (COVID-19) associated multisystem inflammatory syndrome (MIS) can present with shock and myocardial injury and mimic Kawasaki disease. Case presentation We describe 4 previously well adolescents (age 13–14 years), presenting with clinical features of MIS in children (MIS-C). All patients had nearly similar clinical presentation. Hematological investigations revealed elevated inflammatory markers, anemia, thrombocytopenia, and decreased neutrophil:lymphocyte ratio. All patients were negative on real-time polymerase chain reaction against severe acute respiratory syndrome coronavirus 2, but had elevated immunoglobulin G titers. Two patients had atypical Kawasaki disease. Three patients had severe disease with hypotensive shock and required intensive care with fluids and inotropes. Two patients required non-invasive respiratory support for dyspnea and one patient had biventricular dysfunction. All received empiric antibiotics, low-molecular weight heparin, steroids, and intravenous immunoglobulin. One patient succumbed, while others recovered well. Conclusions MIS-C may be a late presentation in adolescent with COVID-19. Individualized treatment with empiric antibiotics, immunomodulation, and thromboprophylaxis can result in significantly better outcome.

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