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Published on: September 14, 2010
[Kawasaki disease. Immunological evaluation of 26 cases]
S Krasovec1, L Bezrodnik, M I Gaillard
1Departamento de Inmunología, Hospital de Niños Ricardo Gutiérrez, Buenos Aires, Argentina. s.krasovec@yahoo.com.ar
Insights
Kawasaki disease (KD) immunologic studies revealed heterogeneous profiles in patients. No acute-stage laboratory findings reliably predicted cardiovascular involvement in this childhood vasculitis.
Area of Science:
- Immunology
- Pediatric Rheumatology
- Vasculitis Research
Context:
- Kawasaki disease (KD) is a critical childhood illness causing inflammation, particularly affecting the heart.
- Immune system activation, including lymphocytes and cytokines, is central to KD's development.
- Understanding KD's immunologic underpinnings is vital for predicting and managing its complications.
Purpose:
- To investigate the immunologic profile of patients with acute Kawasaki disease.
- To identify potential laboratory markers for predicting cardiovascular sequelae in KD.
- To analyze immune cell populations and cytokine levels during acute and convalescent stages.
Summary:
- Immunologic studies in 26 KD patients showed no consistent patterns in serum immunoglobulin or complement levels.
- Flow cytometry revealed variable percentages of immune cells (CD3, CD4, CD8, CD20, CD56, DR) and elevated CD25 in most patients.
- Intracellular cytokine analysis (TNF-alpha, IL1-beta, IL2, IFN-gamma) in peripheral mononuclear cells did not yield a characteristic profile, though two patients with coronary sequelae showed elevated monocyte TNF-alpha and IL1-beta during convalescence.
Impact:
- This study highlights the heterogeneous immune response in Kawasaki disease.
- No specific laboratory finding in the acute phase could predict cardiovascular involvement.
- Further research is needed to understand the immunopathogenesis and identify predictive markers for KD complications.
Abstract:
Kawasaki disease (KD) is an acute febrile vasculitis of childhood, characterized by multiple clinical and biochemical features of inflammation with special involvement of the heart. The activation of lymphocytes and monocytes/macrophages and their secreted soluble products, cytokines, play a central role in the pathogenesis of the disease. In this study we performed immunologic studies in 26 patients with KD. No constant pattern of serum levels of IgG, IgA, IgM, C3 and C4 fractions of complement measured by Nephelometry and neither autoantibodies, FAN and ANCA performed by indirect immunofluorescence were found in 22 patients in the acute stage. Variable percentages of CD3, CD4, CD8, CD20, CD56 and DR in peripheral mononuclear cells specifically stained and analysed by flow cytometry were seen among 25 patients in the acute stage. CD25 was elevated in 17/25 cases. Serum levels of TNF alpha performed by ELISA in 12 patients in acute stage were low. Intracellular cytokines such as TNF alpha, IL1 beta, IL2 and IFN gamma were measured in peripheral mononuclear cells of 15 patients in acute stage, in 5th and 30th days after gammaglobulin treatment, utilizing specific staining and analysis by flow cytometry showing no sole characteristic profile. In 2 patients there was an elevated percentage of TNF alpha and IL1 beta in monocytes during the convalescent stage; both had coronary sequelae. More research on this question is needed. In conclusion, immunologic studies showed an heterogeneous profile and no laboratory finding was registered in the acute stage that could be used as predictive factor of cardiovascular involvement.
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