Kawasaki Disease: Highlighting Importance of Laboratory Criteria and Minor Atypical Features of This Enigmatic Entity- A Case Series

@#Introduction: Kawasaki disease (KD) is an acute febrile illness of unknown etiology that primarily affects children younger than 5 years of age. The diagnosis is predominantly clinical and at times difficult, due to the absence of any confirmatory and specific diagnostic test. Early diagnosis of this disease is of paramount importance due to long term cardiovascular complications related to coronary artery aneurysm. Literature search has revealed many atypical presentations of Kawasaki disease not fulfilling the clinical diagnostic criteria. The reason for this could be the diversity in clinical manifestations involving gastrointestinal, endocrinal, musculoskeletal and nervous system. Case Series: Here we describe three cases with non-classic presentation of Kawasaki disease. These three cases presented with persistent fever unresponsive to antibiotics. Two cases (case one and three) later developed perianal rash and peeling that helped in early diagnosis. In the remaining case (case two) sequential appearance of features helped in the establishment of diagnosis. It was interesting to note that all the three cases were having identical laboratory parameters, highlighting the importance of laboratory investigations in case of atypical presentation. Conclusion: This case series culminates the importance of keeping the possibility of atypical Kawasaki disease (KD) as one of the differentials in patients with prolonged fever not responding to antibiotics, in the absence of classical diagnostic criteria.

Enfermedad de Kawasaki incompleta en un adolescente: informe de un caso / Incomplete Kawasaki disease in an adolescent: a case report

Se presenta el caso de un joven de doce años, con diagnóstico de enfermedad de Kawasaki (EK) incompleta, quien presentó, en el trascurso de la misma, un cuadro clínico compatible con hepatitis aguda. El diagnóstico definitivo se retrasó por la necesidad de descartar otros diagnósticos diferenciales relacionados con la hepatitis aguda. Aunque en la EK puede ocurrir una disfunción hepática, caracterizada por aumento de las transaminasas y bilirrubinas, el cuadro clínico de una hepatitis aguda es muy raro en el curso de esta enfermedad.

We report the case of a 12 year old boy with incomplete Kawasaki disease (KD). During the course of his illness he presented with symptoms of acute hepatitis, which delayed the final diagnosis. Although patients with KD may present with elevation of hepatic enzymes and bilirubin, associated acute hepatitis is of rare occurrence in this entity.

A Case of Atypical Kawasaki Disease with Severe Neutropenia

Leukocytosis and neutrophilia is common during the acute phase of Kawasaki disease whereas leukopenia is not common and severe neutropenia is rare. Severe neutropenia is defined as absolute neutrophil count less than 500/mm3. There are only few publicatons reporting of atypical Kawasaki disease with severe neutropenia. We report a case of atypical Kawasaki disease with severe neutropenia.

The Clinical Characteristics and Coronary Complication of Infantile Kawasaki Disease

PURPOSE: We evaluated Kawasaki disease(KD) in children in order to evaluate the clinical characteristics and coronary complication of infantile Kawasaki disease. METHODS: A total of 226 medical records of children with KD admitted to The Catholic University of Korea, St. Mary's Hospital, from 1994 to 2003 were retrospectively analyzed. RESULTS: The incidence of lymphadenopathy was statistically lower in infantile Kawasaki disease than in Kawasaki disease of children older than one year. The incidence of atypical Kawasaki disease in infant(32%) was greater than that of total patients(24%), but there was no statistically significant difference in two groups. The incidence of coronary complication in atypical Kawasaki disease in children younger than one year was 50%(6/12), which was greater than that of coronary complication in typical KD of children older than one year. CONCLUSION: Diagnostic difficulties in infantile Kawasaki disease are due to high incidence of atypical characteristics of Kawasaki disease. Yet, early treatment with IVGV and aspirin is necessary because of its high incidence of coronary complication.

A Case of Atypical Kawasaki Disease / 소아알레르기및호흡기학회지

Kawaski disease is the leading cause of acquired heart disease in children in many parts of the world. Atypical Kawasaki disease does not meet the diagnostic criteria of Kawasaki disease. Incomplete presentations make early diagnosis and timely treatment difficult. Delays in diagnosis and treatment are associated with an increased risk of coronary artery aneurysm. Thus, echocardiography should be considered in febrile infants of uncertain etiology. Clinical practitioners should have a high index of suspicion to diagnose and initiate prompt treatment to reduce the comorbidity of coronary arterial disease in patients with atypical Kawasaki disease. A 9-month-old girl had daily high spiking fever for 9 days and showed erythematous change on BCG vaccination site only. Echocardiography was performed and disclosed saccular aneurysm of left coronary artery and diffuse right coronary artery dilatation. She was treated with intravenous immunoglobulin and aspirin. After 6 months, a follow-up echocardiogram revealed decreased size of coronary arteries. In such cases, careful evaluation of clinical findings with erythematous change on BCG vaccination site are needed.

Clinical Features of Atypical Kawasaki Disease

PURPOSE: To identify clinical and laboratory features of atypical Kawasaki disease(KD), and to develop criteria for early diagnosis of atypical KD patients. METHODS: All patients with KD treated at our hospital from January 1998 to June 2000 were reviewed retrospectively. RESULTS: Among a total of 167 patients, 28(16.8%) were atypical KD of which seven(25%) were infants. Among the five cardinal symptoms, oral mucosal change(96.4%) occurred most frequently, followed by conjuntivitis(57.1%) and rash(46.4%). Most notable laboratory findings were anemia, and increased erythrocyte sedementation rate(ESR) or C-reactive protein(CRP). Coronary artery abnormalities developed in seven(25.8%) atypical KD patients, compared with 14.4% in typical KD patients. We considered oral mucosal change as major criterion, and conjunctivitis, rash, hematocrit 30 mm/hr or CRP >3.1 mg/dL as minor criteria. Proposed modification in diagnostic criteria for atypical KD include fever of >or=5 days;major criterion+>or=2 minor criteria, or fever of >or=5 days+4 minor criteria. CONCLUSION: The modified diagnostic criteria has yielded a sensitivity 89.3%. Our diagnostic criteria may be used for early diagnosis of atypical KD.

A Clinical Study of Atypical Kawasaki Disease: A Rate of Coronary Artery Involvement

PURPOSE: Without a specific diagnostic method, the clinical diagnostic criteria for atypical Kawasaki disease is known as the presence of coronary artery changes in a patient who did not meet the already estabilished clinical criteria. With this criteria, we are put into dilemma because atypical Kawasaki disease should exhibit coronary artery disease. So, we demonstrated the rate of coronary artery involvement in atypical Kawasaki disease by another diagnostic criteria. METHODS: This diagnostic criteria for atypical Kawasaki disease is "the clinical symptoms which fulfilled four out of six diagnostic criteria below of typical Kawasaki disease with or without the presence of coronary artery changes, and were inconsistent under a through clinical investigation with any other illness similar to Kawasaki disease." RESULTS: The incidence of coronary artery involvement in atypical Kawasaki disease during the acute phase (analysis of 30 cases who were admitted before the 15th day of illness), on the 30th day of illness (26 cases) and on the 60th day of illness(26 cases) were 20.0% (dilatation : 13.3%, aneurysm : 6.7%), 19.2% (dilatation : 19.2%, aneurysm : 0.0%), 7.7%(dilatation : 7.7%, aneurysm : 0.0%), respectively. CONCLUSOIN: Kawasaki disease may occur in atypical type, ranging over many severe involvements of vital organs including the coronary artery. Up to the present, there are no infallible diagnostic tests for Kawasaki disease and it is advisable to deemphasize the rigid adherence to the current already proposed diagnostic criteria for Kawasaki disease.

Clinical Study of Typical and Atypical Kawasaki Diseases

PURPOSE: Atypical patients of Kawasaki Disease (KD) are now discovered with increasing frequency. The goal of this study is to help diagnose and treat KD by comparing of clinical characteristics between typical and atypical KD. METHODS: We performed a retrospective analysis of 82 patients with typical or atypical KD from January 1993 to March 1997. RESULTS: Out of the 82 patients, 49 cases were diagnosed as having typical KD, while there were 33 cases of atypical KD. The mean age was 31.4 months and 25.5 months respectively. The rate of prevalence in the changes of extremities, polymorphous exanthema, and cervical lymphadenopathy was greater in typical KD than atypical KD. The number of patients with various combinations of the principal symptoms among the 33 atypical cases were 18. Mean duration of fever was 6.2 days in typical cases and 5.8 days in atypical cases. The most common clinical findings were cough and rhinorrhea in both groups and the most common disease was pneumonia CRP was significantly elevated in patients with typical KD than atypical cases. Coronary artery dilatations were confirmed by 2-D echo in 7 (19.4%) of 36 tested typical cases, while 2 (9.1%) of 22 tested atypical cases. Patients were treated with aspirin and high-doses of IV gamma-globulin. CONCLUSION: Atypical KD was characterized by the infrequent appearance of rash, cervical lymphadenopathy, changes of hands and feet. We thought that there wasn't a strong association between atypical KD and coronary involvement.

A Case of Right Coronary Arterial Occlusion with Normal Electrocardiogram in Atypical Kawasaki Diseases

Kawasaki disease is an acute febrile illness of unknown etiology that occurs predominantly in infants and early childhood. It had aroused intense interest because of the sequelae of coronary arteritis accompanied by coronary artery aneurysms and thrombotic occlusion, which may lead to ischemic heart disease or sudden death. Atypical Kawasaki disease is coined to describe patients who have coronary abnormalities, pathognomic findings of Kawasaki disease, but who do not exhibit at least four criteria of the disease. Giant coronary arterial aneurysm is a very rare complication after atypical Kawasaki disease, and in cases of coronary occlusion, the surface electrocardiograms usually show common ischemic changes. We report a case of right coronary arterial occlusion complicated after atypical Kawasaki disease. During 12 months, the serial electrocardiograms showed no evidence of ischemia, but coronary angiography disclosed giant aneurysms accompanied with occlusion and several collaterals in right side.