9Atypical and incomplete Kawasaki disease
Section snippets
What is ‘typical’ Kawasaki disease?
The Japanese paediatrician Tomisaku Kawasaki has given his name to the febrile illness that he called ‘mucocutaneous lymph node syndrome’ in his seminal 1967 report of 50 patients. As with all ‘new’ diseases, Kawasaki disease (KD) appears to have affected children long before this early description. Dr. Kawasaki indeed was perceptive and astute enough to detect the common features among patients whose diagnoses were until then elusive. Descriptions of children with similar findings dot the
Shortcomings of traditional diagnostic criteria–definition of atypical and incomplete KD
A major impediment to diagnosing, treating and studying KD has been the absence of a ‘gold standard’ for defining the condition. Initially, Dr. Kawasaki proposed diagnostic criteria in response to a controversy about whether KD truly represented a condition discrete from Stevens–Johnson syndrome and erythema multiforme [4]. The subsequent discovery of cardiac involvement in KD largely resolved this controversy, but led to another in its place: Did children with coronary artery aneurysms (CAAs)
Organ system involvement not included in the classic criteria
Descriptions of gastrointestinal involvement are frequent, even at disease onset, and consist mainly of vomiting, diarrhoea and liver function abnormalities; hydrops of the gallbladder is also often found [12], [13], [14]. Unusual complications were described by Ravinder et al. in 2007 [15], with duodenal ulcer in one case and a retroperitoneal mass (lymphoid tissue at pathology report) in another child. Abdominal manifestations may sometimes be so acute and severe to mimic appendicitis or
Can diagnostic problems be solved with algorithms and/or protocols?
A new tool that would facilitate the diagnosis of KD, taking into account clinical and laboratory values, is certainly needed and would be most welcome [51]. The main problem is that as long as KD remains a clinical diagnosis, it will always be subject to bias, misjudgement and second-guessing. Conventional diagnostic criteria should be viewed as guidelines that are particularly useful in preventing overdiagnosis, but may result in failure to recognise incomplete forms of illness. The
Who should be treated?
Questions regarding therapy of KD overlap debates concerning classification and diagnosis, though they are not identical. The standards for diagnosing KD may be stricter or more lenient than the standards for using IVIG in a particular case. Nonetheless, given the expanding view of what KD and incomplete KD may be, approaches to treatment and follow-up are changing, too. Since 20–60% of children who develop CAAs never meet classic criteria for the diagnosis of KD, it is clear that the major
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Cited by (55)
A case of Kawasaki disease masked by pustular type psoriasiform eruptions
2018, JAAD Case ReportsCitation Excerpt :Further research is required to identify the exact pathophysiology that links KD and psoriasis. Given the increasing prevalence of incomplete KD, the current diagnostic criteria should be viewed as a guideline, and a new diagnostic tool that incorporates both clinical and laboratory values is required to improve the diagnosis of KD.10 A high index of suspicion is important because all children with untreated KD, regardless of their clinical presentation, are at high risk of cardiac complications.
Clinical Mimics: An Emergency Medicine-Focused Review of Streptococcal Pharyngitis Mimics
2018, Journal of Emergency MedicineCitation Excerpt :However, exudative pharyngitis is not commonly found in Kawasaki disease and suggests another condition (119–124). Fever may not respond to treatment, remaining >38.5°C despite antipyretics (123,124). Incomplete Kawasaki disease is defined by less than four signs, though fever is still typically present (115,123,124).
Clinical approach to fever of unknown origin in children
2017, Journal of Microbiology, Immunology and InfectionCitation Excerpt :Echocardiography is also very helpful when KD is suspected, even in young children with FUO who did not meet the typical criteria of KD.30,31 In five KD patients in this study, four were diagnosed with incomplete-type KD, based on echocardiographic finding and laboratory characteristics.32 Gallium scanning is capable of identifying inflammatory lesions and tumors throughout the entire body.
Kawasaki Disease With Coronary Artery Aneurysms: Psychosocial Impact on Parents and Children
2017, Journal of Pediatric Health CareCitation Excerpt :Coronary artery aneurysms (CAAs) can develop in 25% of untreated patients, with the prevalence reduced to approximately 4% if treated with intravenous immunoglobulin within 10 days of illness onset (McCrindle, 2009; Newburger et al., 2004). The diagnosis of KD is challenging and is dependent on clinical presentation and laboratory test results (Bandura, Pastorelli, Barbaranelli, & Caprara, 1999; Cimaz & Sundel, 2009). Caring for children with KD and CAA is complex and requires long-term follow up and management (Bandura et al., 1999; Dimitriades, Brown, & Gedalia, 2014; Manlhiot, Niedra, & McCrindle, 2013).
Has joint involvement lessened in Kawasaki disease?
2017, Reumatologia ClinicaCardiac manifestations in the acute phase of Kawasaki disease in a third level children’s hospital in Mexico City
2018, Archivos de Cardiologia de Mexico
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