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Late diagnosis of Kawasaki disease is associated with haptoglobin phenotype
1Pig Research Institute Taiwan, and Kaohsiung Medical University, Taiwan, Republic of China.
Insights
Haptoglobin (Hp) phenotype influences Kawasaki disease (KD) presentation. Hp 2-1 phenotype is linked to delayed KD diagnosis and increased coronary artery abnormality (CAA) risk, impacting patient outcomes.
Area of Science:
- Pediatrics
- Immunology
- Genetics
Background:
- Kawasaki disease (KD) is a critical pediatric illness causing inflammation and coronary artery abnormalities (CAA).
- Haptoglobin (Hp), an acute-phase protein, has known associations with coronary artery disease.
- Investigating the link between Hp phenotype and CAA in KD is crucial for understanding disease progression.
Purpose of the Study:
- To examine the association between haptoglobin (Hp) phenotype and the development of coronary artery abnormality (CAA) in patients with Kawasaki disease (KD).
Main Methods:
- Studied 47 KD patients before intravenous immunoglobulin (IVIG) and aspirin therapy.
- Measured serum protein levels and performed Hp phenotyping using Western immunoblotting.
- Assessed coronary artery abnormality (CAA) via echocardiography during acute and subacute KD stages.
Main Results:
- Patients with Hp 2-1 phenotype exhibited prolonged fever duration (8.8 days) compared to Hp 2-2 (6.4 days).
- No significant difference in serum Hp levels was observed between Hp phenotypes.
- Patients with Hp 2-1 showed a higher incidence of CAA (80%) and delayed/incomplete symptom presentation.
Conclusions:
- Haptoglobin (Hp) phenotype, specifically Hp 2-1, is associated with delayed diagnosis in Kawasaki disease (KD).
- The Hp 2-1 phenotype may indicate a higher risk for developing coronary artery abnormalities (CAA) due to atypical symptom presentation.
Background:
Kawasaki disease (KD) is an acute febrile illness characterized by multiple clinical and biochemical features of inflammation and the most common complications of coronary artery abnormality (CAA). Haptoglobin (Hp) is an acute-phase protein whose phenotype is known to be involved in coronary artery diseases. In this paper, we report the investigation of the association of Hp phenotype with the formation of CAA in KD.
Patients And Methods:
Forty-seven consecutive patients with clinically diagnosed KD were admitted. Sera were taken before therapy of intravenous immunoglobulins (IVIG) plus aspirin, and levels of serum proteins were measured by a rate immunonephelometer. The echocardiographic criteria for coronary artery abnormality were evaluated during acute or subacute stages. Hp phenotyping was performed by Western immunoblotting.
Results:
Duration of fever at diagnosis of KD was significantly different between patients with Hp 2-2 (6.4 +/- 1.2 days, n = 25) and with Hp1 allele (Hp 2-1 plus Hp 1-1; 8.8 +/- 3.5 days, n = 22). In contrast, serum levels of Hp between KD patients with Hp2-2 and with Hp1 allele (297 +/- 121 mg dL-1 vs. 330 +/- 101 mg dL-1, respectively) was not significantly different. On the other hand, no patients with Hp 2-2 (0/25) were recognized as having KD in subacute stage. However, 5 out of 20 patients with Hp 2-1 were recognized in subacute stage, and their incidence of CAA was 80.0% (4/5).
Conclusions:
Patients with Hp 2-1 have patterns of delayed or incomplete presentation of clinical symptoms. Therefore, the late diagnosis of KD is associated with haptoglobin phenotype.