Management Of Kawasaki Disease Research Articles

Pitfalls in the diagnosis and management of Kawasaki disease: An update for the pediatric dermatologist

Kawasaki disease is easily diagnosed when it presents in its complete form, but because not all characteristic symptoms are always present at the same time, and the diagnosis of incomplete and atypical Kawasaki disease is often challenging, a delay in diagnosis or misdiagnosis often occurs. We present the diagnostic approach to Kawasaki disease with common pitfalls and explain how to avoid them. We also describe current practice and new trends in treatment.

Controversies in diagnosis and management of Kawasaki disease.

Kawasaki disease (KD) is a common medium vessel systemic vasculitis that usually occurs in small children. It has a predilection for the coronary arteries, but other medium sized arteries can also be involved. The etiology of this disorder remains a mystery. Though typical presentation of KD is quite characteristic, it may also present as incomplete or atypical disease in which case the diagnosis can be very challenging. As both incomplete and atypical forms of KD can be associated with serious coronary artery complications, the pediatrician can ill afford to miss these diagnoses. The American Heart Association has enunciated consensus guidelines to facilitate the clinical diagnosis and treatment of this condition. However, there are still several issues that remain controversial. Intravenous immunoglobulin remains the cornerstone of management but several other treatment modalities, especially glucocorticoids, are increasingly finding favour. We review here some of the contemporary issues, and the controversies thereon, pertaining to management of KD.

The emergence of Kawasaki disease in India and China.

Kawasaki disease (KD) is recognized as a leading cause of acquired heart disease in children in developed countries. Although global in distribution, Japan records the highest incidence of KD in the world. Epidemiological reports from the two most populous countries in the world, namely China and India, indicate that KD is now being increasingly recognized. Whether this increased reporting is due to increased ascertainment, or is due to a true increase in incidence, remains a matter of conjecture. The diagnosis and management of KD in developing countries is a challenging proposition. In this review we highlight some of the difficulties faced by physicians in managing children with KD in resource-constrained settings.

Atypical Kawasaki Disease in a 4 Years Old Child with Mumps

Kawasaki disease is an acute febrile condition seen in children. However, it is also well recognized that some patients do not fulfill the classic diagnostic criteria for the diagnosis of Kawasaki disease. The incomplete form of Kawasaki disease is termed as 'Incomplete KD' or 'Atypical KD'. This is a case of 4 years old child with fever and mumps. He had bilateral cervical adenitis. Patient failed to respond to IV antibiotics fulfilled the criteria of incomplete Kawasaki disease. The child was managed with high dose aspirin until the child was afebrile for 48 hours. Kawasaki disease is a common vasculitis in children. Atypical cases might be missed if there is concomitant viral illness. Hence the identification and management of Kawasaki disease is paramount to decrease the mortality related to the cardiac disease. Keywords: bilateral cervical adenitis; fever and mumps; failed to respond IV antibiotics; incomplete kawasaki disease.

Treatment and follow-up of Kawasaki disease

Kawasaki disease(KD)is an acute, self-limited and systemic vasculitis.The etiology and pathogenesis of KD still remain unclear.High-dose intravenous immunoglobulin(IVIG)combined with aspirin is the standard regimen of KD in acute stage, but the treatment of KD patients who were unresponsive to initial IVIG therapy still remains controversial.The main complication of KD is coronary artery lesions.Treatments of coronary artery lesions included antiplatelet, anticoagulant, thrombolytic therapy, percutaneous coronary interventions and coronary artery bypass grafting.In addition, follow-up and management of KD were also very important. Key words: Kawasaki disease; Intravenous immunoglobulin; Coronary artery lesions; Follow-up

CD8+ T Cells Contribute to the Development of Coronary Arteritis in the Lactobacillus casei Cell Wall Extract-Induced Murine Model of Kawasaki Disease.

Kawasaki disease (KD) is the leading cause of acquired heart disease among children in developed countries. Coronary lesions in KD in humans are characterized by an increased presence of infiltrating CD3+ T cells; however, the specific contributions of the different T cell subpopulations in coronary arteritis development remain unknown. Therefore, we sought to investigate the function of CD4+ and CD8+ T cells, Treg cells, and natural killer (NK) T cells in the pathogenesis of KD. We addressed the function of T cell subsets in KD development by using a well-established murine model of Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis. We determined which T cell subsets were required for development of KD vasculitis by using several knockout murine strains and depleting monoclonal antibodies. LCWE-injected mice developed coronary lesions characterized by the presence of inflammatory cell infiltrates. Frequently, this chronic inflammation resulted in complete occlusion of the coronary arteries due to luminal myofibroblast proliferation (LMP) as well as the development of coronary arteritis and aortitis. We found that CD8+ T cells, but not CD4+ T cells, NK T cells, or Treg cells, were required for development of KD vasculitis. The LCWE-induced murine model of KD vasculitis mimics many histologic features of the disease in humans, such as the presence of CD8+ T cells and LMP in coronary artery lesions as well as epicardial coronary arteritis. Moreover, CD8+ T cells functionally contribute to the development of KD vasculitis in this murine model. Therapeutic strategies targeting infiltrating CD8+ T cells might be useful in the management of KD in humans.

Clinical Aspects and Management of Kawasaki Disease: Experience at a Single Center Over a Seventeen-year Period

Clinical Aspects and Management of Kawasaki Disease: Experience at a Single Center Over a Seventeen-year Period

The Clinical Diagnosis and Management of Kawasaki Disease: a Review and Update.

Kawasaki disease is an acute, self-limited vasculitis of childhood and has become the leading cause of acquired pediatric heart disease in the USA. Prompt treatment is essential in reducing cardiac-related morbidity and mortality. The underlying etiology remains unknown. The disease itself may be the characteristic manifestation of a common pathway of immune-mediated vascular inflammation in susceptible hosts. The characteristic clinical features of fever for at least 5 days with bilateral nonpurulent conjunctivitis, rash, changes in lips and oral cavity, changes in peripheral extremities, and cervical lymphadenopathy remain the mainstay of diagnosis. Supplementary laboratory criteria can aid in the diagnosis, particularly in cases of incomplete clinical presentation. Diagnosis of Kawasaki disease can be challenging as the clinical presentation can be mistaken for a variety of other pediatric illnesses. Standard of care consists of intravenous immune globulin and aspirin. Corticosteroids, infliximab, and cyclosporine A have been used as adjunct therapy for Kawasaki disease refractory to initial treatment. There is ongoing research into the use of these agents in the initial therapy of Kawasaki disease.

Abstract O.33: Identification of Kawasaki Disease-specific Molecules in the Sera as Microbe-associated Molecular Patterns

Background: Kawasaki disease (KD) is a systemic vasculitis of unknown etiology. The innate immune system is involved in its pathophysiology at the acute phase. We have recently established a novel murine model of KD coronary arteritis by oral administration of a synthetic microbe-associated molecular pattern (MAMP). On the hypothesis that specific MAMPs exist in KD sera, we have searched them to identify KD-specific molecules and to assess the pathogenesis. Methods: The study subjects included 117 patients with KD (median age, 21 months; range 3-96 months; male/female, 65/52) and 101 controls with other febrile illnesses (DC: median age, 16 months; range 0-121 months; male/female, 61/40), and 5 normal controls (NC: median age, 6 months; range 3-39 months; male/female, 1/4). We performed liquid chromatography-mass spectrometry (LC-MS) analysis of fractionated serum and microbial samples by ethyl acetate. Results: KD serum samples elicited proinflammatory cytokine responses from human coronary artery endothelial cells (HCAECs). By LC-MS analysis of KD serum samples collected at 3 different periods, we detected a variety of KD-specific molecules in the lipophilic fractions that showed distinct m/z and MS/MS fragmentation patterns in each cluster. Serum KD-specific molecules showed m/z and MS/MS fragmentation patterns almost identical to those of MAMPs obtained from Bacillus cereus, Yersinia pseudotuberculosis and Staphylococcus aureus at the 1st study period, and from MAMPs from Bacillus cereus, Bacillus subtilis/Bacillus cereus/Yersinia pseudotuberculosis and Staphylococcus aureus at the 2nd and 3rd periods. These molecules decreased after intravenous immunoglobulin (IVIG) treatment. Conclusions: We herein conclude that serum KD-specific molecules possess molecular structures common to MAMPs from Bacillus cereus, Bacillus subtilis, Yersinia pseudotuberculosis and Staphylococcus aureus. Discovery of these KD-specific molecules offers novel insight into the diagnosis and management of KD as well as its pathogenesis.

G453(P) Management of kawasaki disease in a district general hospital. is there an optium aspirin dose?

Aims Kawasaki disease (KD) is the commonest cause of paediatric acquired heart disease in the UK. Early treatment significantly reduces coronary artery complications. This study aimed to identify cases of KD presenting to our district general hospital (DGH). We wished to see how well diagnosis and management adhered to local guidelines. Methods Retrospective review of case notes of patients admitted to our district general and diagnosed with KD between October 2011 and October 2014. Diagnosis and management was compared to local guideline. Results Eighteen KD patients of variable age (3 months to 9 years) and ethnicity were identified. Two thirds of cases had incomplete KD. All had a history of fever >5 days. Diagnostic features included conjunctivitis (16/18); mucosal changes (15/18); rash (11/18); cervical lymphadenopathy and peripheral extremity changes (8/18). Irritability was a common feature. BCG scar changes were also present (2/18). All cases received intravenous immunoglobulin (IVIG) at 2 g/kg and high dose aspirin at 30–100 mg/kg/day except one who received aspirin alone as afebrile at presentation. Five patients had suspicion of coronary artery dilatation/prominence on initial echocardiogram. Three subsequently developed coronary artery dilatation all of whom had received IVIG during first week of fever. Three cases were transferred to tertiary centre for second–line management encompassing repeat IVIG (3/18), infliximab (2/18) and corticosteroids (1/18). Conclusion Two thirds of KD cases were incomplete. Ninety–four percent had IVIG. Inflammatory markers, laboratory criteria and BCG scar change did not correspond with cardiovascular complications. Dose of aspirin given varied between 30–100 mg/kg/day depending on tertiary centre recommendation. All patients were managed appropriately. Those that failed to respond were referred to a tertiary centre. This study demonstrates most KD can be managed locally with adherence to clear guidelines and receiving tertiary advice when required. However clearer consensus on high–dose aspirin is needed.

Management of Kawasaki disease in resource-limited settings.

Management of Kawasaki disease in resource-limited settings.

Kawasaki disease-specific molecules in the sera are linked to microbe-associated molecular patterns in the biofilms.

BackgroundKawasaki disease (KD) is a systemic vasculitis of unknown etiology. The innate immune system is involved in its pathophysiology at the acute phase. We have recently established a novel murine model of KD coronary arteritis by oral administration of a synthetic microbe-associated molecular pattern (MAMP). On the hypothesis that specific MAMPs exist in KD sera, we have searched them to identify KD-specific molecules and to assess the pathogenesis.MethodsWe performed liquid chromatography-mass spectrometry (LC-MS) analysis of fractionated serum samples from 117 patients with KD and 106 controls. Microbiological and LC-MS evaluation of biofilm samples were also performed.ResultsKD samples elicited proinflammatory cytokine responses from human coronary artery endothelial cells (HCAECs). By LC-MS analysis of KD serum samples collected at 3 different periods, we detected a variety of KD-specific molecules in the lipophilic fractions that showed distinct m/z and MS/MS fragmentation patterns in each cluster. Serum KD-specific molecules showed m/z and MS/MS fragmentation patterns almost identical to those of MAMPs obtained from the biofilms formed in vitro (common MAMPs from Bacillus cereus, Yersinia pseudotuberculosis and Staphylococcus aureus) at the 1st study period, and from the biofilms formed in vivo (common MAMPs from Bacillus cereus, Bacillus subtilis/Bacillus cereus/Yersinia pseudotuberculosis and Staphylococcus aureus) at the 2nd and 3rd periods. The biofilm extracts from Bacillus cereus, Bacillus subtilis, Yersinia pseudotuberculosis and Staphylococcus aureus also induced proinflammatory cytokines by HCAECs. By the experiments with IgG affinity chromatography, some of these serum KD-specific molecules bound to IgG.ConclusionsWe herein conclude that serum KD-specific molecules were mostly derived from biofilms and possessed molecular structures common to MAMPs from Bacillus cereus, Bacillus subtilis, Yersinia pseudotuberculosis and Staphylococcus aureus. Discovery of these KD-specific molecules might offer novel insight into the diagnosis and management of KD as well as its pathogenesis.

Comparison of High-Dose versus Low-Dose Aspirin in the Management of Kawasaki Disease

To the Editor: Kawasaki disease (KD) is a vasculitis of an unknown origin, involving the small and medium size vessels [1, 2]. Standard treatment for acute phase of KD introduced by American Heart Association (AHA) and American Academy of Pediatrics (AAP) includes intravenous immunoglobulin (IVIG) therapy with a single dose of 2 g/kg plus oral administration of aspirin at high-dose of 80–100 mg/kg/d [3, 4]. During the acute phase of Kawasaki disease, Group 1 (control group), consisting of 27 children, received aspirin at a daily dose of 80–100 mg/kg/d for 14 d after the onset. Highdose aspirin was then changed to low-dose (3–5 mg/kg/d) for another 6–8 wk. Group 2 (study group) consisting of 42 children received aspirin at a daily dose of 3–5 mg/kg/d for 8–10 wk if no coronary artery aneurism was observed in echocardiography. All patients also received the standard IVIG therapy with a single dose of 2 g/kg. The mean duration of fever after the therapy was 41.96± 19.63 h in Group 1 and 46.00±50.49 h in Group 2 (P=0.694). Persistent fever was observed in 14.8 % of cases in Group 1 and 11.9 % of cases in Group 2 (P=0.729). Duration of the hospital stay was 6±1.3 d in Group 1 and 6.36±2.80 d in Group 2 (P=0.540). There was 1 (4 %) and 2 cases (5.3 %) of new coronary artery aneurism (CAA) formation in Group 1 and Group 2 respectively (P=1.000). The logic behind the use of aspirin regimen in KD is the probable benefits of its anti-inflammatory and anti-platelet effects at high and low dosages respectively [5]. According to the result of this study, it is most likely that the antiinflammatory dosage of aspirin has no advantage over the anti-platelet dosage regarding the duration of fever and days of hospital stay. This study was not powered enough to assess the differences between the two regimens regarding the CAA formation. Further studies with higher number of cases are needed to compare the effectiveness of high and low doses of aspirin in preventing CAA formation.

G448(P) A 5 year audit of typical and atypical Kawasaki Disease

Aims:Kawasaki disease is an acute systemic vasculitis with a rising incidence in the UK. Presentation and final diagnosis could be ‘typical’ or ‘atypical/incomplete’ and can be a diagnostic challenge for...

Kawasaki Disease: A Clinician’s Update

Aims. Kawasaki disease is an acute systemic vasculitis and is the most common cause of acquired heart disease in children in the developed world. This review aims to synthesise recent insights into the disease and provide an update for clinicians on diagnostic and treatment practices. Methods. We conducted a review of the literature exploring epidemiology, aetiology, diagnosis, and management of Kawasaki disease. We searched MEDLINE, Medline In-Process, Embase, Google Scholar, and reference lists of relevant articles. Conclusions. Kawasaki disease is a febrile vasculitis which progresses to coronary artery abnormalities in 25% of untreated patients. The disease is believed to result from a genetically susceptible individual's exposure to an environmental trigger. Incidence is rising worldwide, and varies widely across countries and within different ethnic groups. Diagnosis is based on the presence of fever in addition to four out of five other clinical criteria, but it is complicated by the quarter of the Kawasaki disease patients with “incomplete” presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%. Given its severe morbidity and potential mortality, Kawasaki disease should be considered as a potential diagnosis in cases of prolonged paediatric fever.

Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study)

Journal of Paediatrics and Child HealthVolume 49, Issue 1 p. 79-80 Heads UpFree Access Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study) First published: 16 January 2013 https://doi.org/10.1111/jpc.12048Citations: 1AboutSectionsPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL The use of steroid therapy in Kawasaki disease (KD) has a long and complicated history. A recent well-designed randomised controlled trial (RCT) provides strong evidence that the addition of a prolonged course of steroids to the standard treatment of intravenous immunoglobulin (IVIG) and aspirin significantly improves coronary artery (CA) outcomes in Japanese children at high risk of IVIG non-response.1 Of 2014 KD patients, only the 248 (12%) at high risk of IVIG failure were included, based on the authors' severity score. The incidence of CA abnormalities during the study period was 4/121 (3%) in the steroid treatment group and 28/121 (23%) in the standard treatment group (P < 0.0001); number needed to treat (NNT) to prevent CA abnormalities was five. At week 4, the incidence of CA abnormalities was 4/120 (3%) in the steroid treatment group and 15/120 (13%) in the standard treatment group (P = 0.014); NNT was 10. These are impressive results. Other findings also suggest that steroids may be of benefit in those at highest risk of IVIG non-response. A RCT of adjunctive primary steroids in an unselected KD population in the USA failed to show any benefit on CA outcomes (albeit with a shorter and different steroid regimen to the Japanese study).2 However, a post hoc subgroup analysis of those who subsequently required IVIG retreatment suggested a significant beneficial effect on CA outcomes.2 Caution is warranted in generalising the RAISE study findings as there are important differences between the study population and KD patients outside Japan.3 Crucially, Japanese risk-scoring systems, including that used in the RAISE study, have reasonable specificity but unacceptably low sensitivity in non-Japanese populations.4 There are other potentially important differences in the RAISE study: treatment was commenced at a median of day 4, which is earlier than is usual in Australia and other non-Japanese populations. Finally, the steroid regimen is partly intravenous and relatively prolonged, potentially increasing inpatient costs. The development of severity scores predictive of IVIG non-response in non-Japanese patients would allow further studies of adjunctive regimens, including steroids, in those with the most severe KD. References 1 Kobayashi T, Saji T, Otani T et al. Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial. Lancet; 379: 1613– 1620. 2 Newburger JW, Sleeper LA, McCrindle BW et al. Randomized trial of pulsed corticosteroid therapy for primary treatment of Kawasaki disease. N. Engl J. Med. 2007; 356: 663– 675. 3 Son MBF, Newburger JW. Management of Kawasaki disease: corticosteroids revisited. Lancet; 379: 1571– 1572. 4 Sleeper LA, Minich LL, McCrindle BM et al. Evaluation of Kawasaki disease risk-scoring systems for intravenous immunoglobulin resistance. J. Pediatr. 2011; 158: 831– 835. Reviewers: Katherine Chen, [email protected]; David Burgner, [email protected]; Nigel Curtis, [email protected] Citing Literature Volume49, Issue1January 2013Pages 79-80 ReferencesRelatedInformation

Pre-hospital recognition of Kawaski disease: a guide for paramedics

Journal of Paramedic PracticeVol. 4, No. 9 Career DevelopmentPre-hospital recognition of Kawaski disease: a guide for paramedicsAllan DaviesAllan DaviesSearch for more papers by this authorAllan DaviesPublished Online:16 Aug 2013https://doi.org/10.12968/jpar.2012.4.9.543AboutSectionsView articleView Full TextPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareShare onFacebookTwitterLinked InEmail View article References Brogan P, Bose A, Burgner D (2002) Kawasaki disease: an evidence based approach to diagnosis, treatment, and proposals for future research. BMJ 86: 286–90. adc.bmj.com/content/86/4/286 (accessed 30 August 2012) Google ScholarBUPA (2010) Kawasaki Disease. www.bupa.co.uk/individuals/health-information/directory/k/kawasaki-disease (last accessed 30 August 2012) Google ScholarBurns J, Glodé MP (2004) Kawasaki Syndrome Lancet 364(9433): 533–44 Crossref, Google ScholarCarlton-Conway, D, Ahluwalia R, Henry L et al. (2005) Behavior sequelae following acute Kawasaki disease. www.biomedcentral.com/1471-2431/5/14 (accesses 30 August 2012) Google ScholarGordon J, Kahn A, Burns J (2009) When Children With Kawasaki Disease Grow Up. J Am Coll Cardiol. 54(21): 1911–120 Crossref, Google ScholarGregory P, Ward A (2010) Sanders’ Paramedic Textbook. Elsevier-Mosby, London Google ScholarHiew T, Cheng H (1992) ECG Abnormalities in Kawasaki disease and their value in predicting coronory artery aneurysms Sing Med J 33: 262–7. smj.sma.org.sg/3303/3303a8.pdf (accessed 30 August 2012) Google ScholarJoint Royal Colleges Service Liaison Committee (2006). www.jrcalc.org.uk (accessed 30 August 2012) Google ScholarKing W, Schlieper A, Birdi N et al. (2000) The effect of Kawasaki disease on cognition and behavior. Arch Pediatr Adolesc Med 154(5): 463–8 Crossref, Google ScholarKawasaki Disease Foundation (2012) Kawasaki Disease. www.kfoundation.org (accessed 30 August 2012) Google ScholarLim TC, Yeo W, Loke K et al. (2009) Bilateral Facial Nerve Palsy in Kawasaki Disease. Ann Acad Med Singapore 38(8): 737–8. www.annals.edu.sg/pdf/38VolNo8Aug2009/V38N8p737.pdf (accessed 30 August 2012) Google ScholarMatsubara T, Mason W, Kashani I et al. (1996) Gastrointestinal haemorrhage complicating aspirin therapy in acute Kawasaki disease. 128(5pt1): 701–3 (accessed 30 August 2012) Google ScholarNagase H, Visse R Murphy G (2005) Structure and function of matrix metalloproteinases and TIMPs. Cardiovascular Res 69(3): 562–73 cardiovascres.oxfordjournals.org/content/69/3/562.full.pdf+html (accessed 30 August 2012) Google ScholarNational Institute for Clinical Excellence (2007) Feverish illness in children–Assessment and initial management in children younger than 5 years. www.nice.org.uk/nicemedia/live/11010/30523/30523.pdf (accessed 30 August 2012) Google ScholarNewburger J, Sleeper L, McCrindle B et al. (2007) Randomized Trial of Pulsed Corticosteroid Therapy for Primary Treatment of Kawasaki Disease. N Eng J Med. www.nejm.org/doi/full/10.1056/NEJMoa061235 (accessed 30 August 2012) Google ScholarRowley AH, Shulman ST (2010) Pathogenesis and management of Kawasaki disease. Expert Rev Anti Infect Ther 8(2): 197–203 Crossref, Google ScholarScheinfeld N (2012) Kawasaki Disease. emedicine.medscape.com/article/965367-overview (accessed 30 August 2012) Google ScholarSenzaki H (2006) The pathophysiology of coronary artery aneurysms in Kawasaki disease: role of matrix metalloproteinases’ BMJ. adc.bmj.com/content/91/10/847.short (accessed 30 August 2012) Google ScholarTaubert K, Shulman S (1999) Kawasaki Disease. Am Fam Physician 59(11): 3093–102 Google ScholarThatayatikom A, Jung L (2011) Pediatric Polyarteritis Nodosa. emedicine.medscape.com/article/1007428-overview#showall (accessed 30 August 2012) Google ScholarTizard E (2005) Complications of Kawasaki disease. Current Paediatrics 15: 62–8 Crossref, Google ScholarTortora G, Derrickson B (2009) Principles of anatomy and physiology (12th Edn) RR Donnelley Publishing, Chicago Google Scholar FiguresReferencesRelatedDetailsCited byA child with high fever: Kawasaki disease16 August 2013 | British Journal of Nursing, Vol. 22, No. 5 9 September 2012Volume 4Issue 9ISSN (print): 1759-1376ISSN (online): 2041-9457 Metrics History Published online 16 August 2013 Published in print 9 September 2012 Information© MA Healthcare LimitedPDF download

Epidemiology and Management of Kawasaki Disease

Kawasaki disease (KD) is an acute systemic vasculitis affecting young children and is rising in incidence worldwide. It is most common in children <5 years of age, males and those of Asian ethnicity. It is an important cause of acquired heart disease in children. Standard treatment with high-dose aspirin (acetylsalicylic acid; ASA) and intravenous immune globulin (IVIG) has been shown to decrease the rate of coronary artery aneurysm development. Anti-coagulation has an important place in the management of KD, although guidance based on evidence is lacking. Treatment of refractory KD is an area under intense study and may include IVIG, corticosteroids and/or tumour necrosis factor (TNF)-α inhibitors among immunosuppressive agents. Acute complications of KD include myocarditis/KD shock syndrome and macrophage activation syndrome, which necessitate appropriate awareness in order to initiate proper management.

Can Kawasaki Disease Be Managed?

Kawasaki Disease (KD) is the leading cause of acquired cardiovascular disease among children, but management of KD has received relatively little attention. In the US alone, about 5500 cases were estimated in 2009. KD is most common among Asian and Pacific Islander children but can affect all ethnicities and races. Timely and accurate diagnosis remains critical, but difficult: the etiology of KD is unknown, and no accurate diagnostic laboratory test has been developed. Continuing medical education can help physicians, clinicians, and nurse practitioners accurately diagnose and treat KD. A registry specific to KD or a surveillance system may be necessary to increase awareness among health care professionals and to decrease complications related to misdiagnosis.

Management of Kawasaki disease: corticosteroids revisited

Management of Kawasaki disease: corticosteroids revisited