Humans ; Mucocutaneous Lymph Node Syndrome ; etiology ; immunology ; metabolism ; physiopathology

Kawasaki disease (KD) is characterized with acute systemic vasculitis, occurs predominantly in children between 6 months to 5 years of age. Patients with this disease recover well and the disease is self-limited in most cases. Since it can lead to devastating cardiovascular complications, KD needs special attention. Recent reports show steady increases in the prevalence of KD in both Japan and Korea. However, specific pathogens have yet to be found. Recent advances in research on KD include searches for genetic susceptibility related to KD and research on immunopathogenesis based on innate and acquired immunity. Also, search for etiopathogenesis and treatment of KD has been actively sought after using animal models. In this paper, the recent progress of research on KD was discussed.
*Genetic Predisposition to Disease ; Heart Diseases/*complications ; Humans ; Mucocutaneous Lymph Node Syndrome/*diagnosis/etiology/physiopathology/therapy

OBJECTIVETo evaluate the late endothelial function in children with coronary aneurysm due to Kawasaki disease (KD).

METHODSThirty-one children with coronary aneurysms due to KD who had the disease course for more than 1 year and twenty-one age-matched healthy children were enrolled. Brachial artery endothelium-dependent and -independent flow-mediated dilation (FMD), carotid arterial stiffness index (SI) and intima-media thickness (IMT) were measured by high-frequency ultrasound.

RESULTSThere were 9 cases of medium and 22 cases of giant coronary aneurysms in the KD group. Twelve KD patients had evidence of myocardial ischemia. Compared to the normal controls, the endothelium-dependent FMD decreased (P<0.05), the carotid arterial SI increased (P<0.05), and the carotid arterial intima-media thickness increased significantly (P<0.05) in children with coronary aneurysms due to KD. The endothelium-dependent FMD decreased more significantly in 12 KD patients with myocardial ischemia than in those without any evidence of myocardial ischemia (P<0.05).

CONCLUSIONSLate endothelial dysfunction exists in children with coronary aneurysms due to KD, especially in those with myocardial ischemia.

Adolescent ; Child ; Coronary Aneurysm ; etiology ; physiopathology ; Endothelium, Vascular ; physiopathology ; Female ; Humans ; Male ; Mucocutaneous Lymph Node Syndrome ; complications ; physiopathology ; Vasodilation

OBJECTIVETo study serum levels of brain natriuretic peptide (BNP) in children with Kawasaki disease (KD) and the correlation between BNP levels and the heart function.

METHODSForty-three children with KD and thirty healthy children were enrolled. Serum levels of BNP were measured using ELISA. KD children received an echocardiographic examination, including measurements of left ventricular ejection fraction (LVEF), left ventricular shorten fraction (LVSF), cardiac index (CI) and left ventricular inflow velocity through the mitral annulus.

RESULTSMean serum level of BNP at the acute stage in children with KD was significantly higher than that at the recovery stage as well as the control group (p<0.01). The LVEF, LVSF and CI levels at the acute stage were significantly lower than those at the recovery stage in children with KD (p<0.05). The linear regression analysis showed that the BNP level was negatively correlated with the levels of LVEF, LVSF and CI (r=-0.63, -0.52, -0.53, p<0.05).

CONCLUSIONSThe serum BNP levels increase significantly in KD children at the acute stage, and are negatively correlated with the levels of LVEF, LVSF and CI. Measurement of serum BNP level is useful for the early diagnosis of KD.

Child ; Child, Preschool ; Female ; Heart ; physiopathology ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; blood ; diagnosis ; physiopathology ; Natriuretic Peptide, Brain ; blood

OBJECTIVE: To study the clinical features of children with recurrent Kawasaki disease (KD). METHODS: PubMed, Web of Science, Embase, CNKI, Wanfang Med Online, and Weipu Data were searched for case-control studies on the clinical features of initial and recurrent KD. The articles were screened according to the inclusion and exclusion criteria. RevMan 5.3 software was used to perform the Meta analysis. Effect models were selected based on the results of heterogeneity test, and then pooled RESULTS: A total of 9 case-control studies were included, with 12 059 children with KD in total, among whom 206 children had recurrent KD (127 boys/61.7%; 79 girls/38.3%). The results of the Meta analysis showed that compared with the initial KD onset, the children with recurrent KD had a shorter duration of fever ( CONCLUSIONS Current evidence shows that children with recurrent KD tend to have a shorter duration of fever and a lower incidence of swelling of the hands and feet. KD recurrence is more common in boys. Current evidence does not show an increased risk of developing coronary artery lesions in children with recurrent KD.
Child ; Chronic Disease ; Coronary Vessels/pathology* ; Edema/etiology* ; Female ; Fever/etiology* ; Humans ; Male ; Mucocutaneous Lymph Node Syndrome/physiopathology* ; Recurrence

OBJECTIVEKawasaki disease (KD) is a kind of febrile disorder without definite etiology. The pathologic change of KD is characterized by nonspecific vasculitis, which mainly involves the coronary artery. Some patients may have coronary angioma formation, and some of them will result in the coronary narrowing or embolism. Notwithstanding that KD has been one of the most common causes for acquired heart diseases in childhood in addition to the rheumatic fever, the pathogenesis of the vascular damage remains unknown. This study was conducted to explore the pathophysiological role of cell adhesion molecules (P-selectin and E-selectin) on the endothelial lesions in KD, and to look for the evidence of direct relationship between the plasma levels of soluble cell adhesion molecules (P- and E-selectin) and the incidence of the coronary artery lesion (CAL).

METHODSSoluble P-selectin (PS), E-selectin (ES), thromboxane-B(2)(TXB(2)), 6-keto-PGF(1)alpha (6-KPGF(1)alpha) were measured in 36 patients with KD, 20 patients with febrile disease and 30 healthy children by using double antibody sandwich enzyme linked immunosorbent assay (ELISA) and radioimmunoassay. Patients with KD were separated into acute phase group, subacute phase group, recovery phase group, coronary artery lesion group (CAL), non-coronary artery lesion group (NCAL), intravenous immunoglobulin (IVIG) effective group (body temperature back to normal after 48 hours of using IVIG), and IVIG ineffective group.

RESULTSPlasma PS and ES levels in the acute phase group [(211 +/- 28 and 186 +/- 14) ng/ml], subacute phase group [(238 +/- 27 and 151 +/- 13) ng/ml] and recovery phase group [(198 +/- 21 and 1008 +/- 9) ng/ml] were significantly higher than those in the healthy group [(102 +/- 36 and 72 +/- 10) ng/ml, P < 0.01]. The plasma PS levels remained higher after the treatment, but in IVIG effective group, the PS and ES levels declined significantly (P < 0.01) compared with those in acute phase group. Plasma PS and ES levels of CAL group [(281 +/- 78 and 210 +/- 52) ng/ml] were significantly higher than those of NCAL group [(217 +/- 15 and 108 +/- 10) ng/ml, P < 0.01]. In contrast to 1 week after the treatment, the PS and ES in IVIG effective group at the time point of 2 weeks after the treatment decreased more significantly (P < 0.01). While the PS and ES in IVIG ineffective group remained higher at the time point of 2 weeks after the treatment, which showed no significant difference compared with those 1 week after the treatment (P > 0.05). One week after the treatment, the PS levels of IVIG effective and ineffective groups did not descend, and there was no significant difference in PS between these two groups at this time point. Two weeks after the treatment, the PS and ES in IVIG ineffective group remained higher than those in IVIG effective group, and there was a significant difference between them. The peak level of PS appeared in the subacute phase. TXB(2) levels of KD in acute phase group increased markedly, which were significantly higher than those of healthy group [(345 +/- 127 and 190 +/- 69) ng/L, P < 0.01]. There was no significant difference between subacute phase group and healthy group. No significant difference was found between CAL group and NCAL group (P > 0.05). The levels of TXB(2) declined quickly after the treatment. The 6-KPGF(1)alpha level in KD of acute phase group, subacute phase group and recovery phase group [(7.1 +/- 2.8, 10.8 +/- 3.7 and 11.3 +/- 4.0) ng/L, respectively] was significantly lower than that of healthy group [(17.7 +/- 5.8) ng/L, P < 0.01], and the levels did not recover to normal even 2 weeks after the treatment. There was no significant difference 6-KPGF(1)alpha levels between CAL group and NCAL group (P > 0.05). In the febrile group, PS and ES levels showed no significant differences compared with healthy children (P > 0.05). ES level of KD patients was significantly correlated with CRP levels (r = 0.79 P < 0.01). In febrile group, there was no significant correlation between ES and CRP. There was a significant correlation between PS and PLT levels in KD patients (r = 0.75 P < 0.01), and no significant correlation between PS and PLT levels in febrile patients.

CONCLUSIONThe increase of plasma PS and ES levels in KD acute phase and subacute phase might play an important role in the pathophysiology of the endothelial damage. E- and P-selectin may potentially be a predictor of CAL in patients with KD.

Child ; Coronary Artery Disease ; physiopathology ; Coronary Vessels ; physiopathology ; E-Selectin ; blood ; Endothelium, Vascular ; physiopathology ; Humans ; Mucocutaneous Lymph Node Syndrome ; blood ; physiopathology ; P-Selectin ; blood

Acute Disease ; Case-Control Studies ; Child, Preschool ; Echocardiography, Doppler ; Female ; Heart ; physiopathology ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; diagnostic imaging ; physiopathology

OBJECTIVETo investigate the clinical features of children with incomplete Kawasaki disease (IKD), and to provide reference for the early diagnosis of IKD.

METHODSThe clinical data of 22 hospitalized children with IKD were analyzed retrospectively and compared with the data of 63 children with Kawasaki disease (KD) who were hospitalized during the same period of time. Another 20 children with pyrexia were enrolled as the control group.

RESULTSPyrexia was observed in all children. Compared with the KD group, the IKD group had significantly lower proportions of children with changes in the limbs, conjunctival hyperaemia, and cervical lymphadenectasis (P<0.05), a significantly higher serum level of glutamic-pyruvic transaminase (P<0.05), and significantly lower levels of plasma albumin, serum sodium, and interleukin-6 (P<0.05). There was no significant difference in the rate of γ-globulin application between the IKD and KD groups; however, the IKD group had a significantly higher incidence rate of coronary artery lesion than the KD group (P<0.05).

CONCLUSIONSThe symptoms and signs in children with IKD are untypical. The liver function test and serum hyponatremia and IL-6 measurements may be useful for the diagnosis of IKD.

Alanine Transaminase ; blood ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Liver ; physiopathology ; Male ; Mucocutaneous Lymph Node Syndrome ; diagnosis ; etiology ; physiopathology ; Retrospective Studies

OBJECTIVETo investigate renal artery injury caused by Kawasaki disease (KD).

METHODSForty-three children with KD were enrolled in the study. According to the blood pressure in the acute stage, these children were classified into normal blood pressure subgroup and increased blood pressure subgroup. Eighteen children with fever caused by acute upper respiratory tract infection were enrolled as the control group. The diameter of the origin of the main renal artery, hemodynamic parameters of the main renal artery and the renal interlobar artery, rennin activity, and levels of angiotensin II and aldosterone were compared between groups.

RESULTSDuring the acute stage of KD, both subgroups had a significantly smaller diameter of the origin of the main renal artery, a significantly higher resistance index (RI) of the main renal artery, and a significantly lower end-diastolic velocity (EDV) than the control group (P<0.05).The increased blood pressure subgroup had a significantly lower EDV of the interlobar artery than the normal blood pressure subgroup, a significantly higher RI than the normal blood pressure subgroup and the control group, as well as a significantly higher rennin activity and significantly higher levels of angiotensin II and aldosterone than the normal blood pressure subgroup (P<0.05). A significantly increased EDV and a significantly reduced RI of the renal interlobar artery were observed in the increased blood pressure subgroup in the subacute stage compared with the acute stage (P<0.05).

CONCLUSIONSKD may cause renal artery injury and early hemodynamic changes, resulting in a transient increase in blood pressure in some patients.

Blood Flow Velocity ; Blood Pressure ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome ; physiopathology ; Renal Artery ; physiopathology ; Renin-Angiotensin System ; physiology ; Vascular Resistance

We investigated the changes of CD5+ B cells in the peripheral blood of 20 Kawasaki disease (KD) patients. The percentage of CD5+ B cells in the total lymphocytes and in the total B cells significantly decreased during the acute phase of KD(p< 0.01), compared to that in the age-matched normal control subjects. After intravenous immunoglobulin(IVIG) treatment, the percentage of CD5+ B cells increased, but was still lower than that in the normal controls(p< 0.01). During the convalescent phase of the disease, the percentage of CD5+ B cells was restored to the normal levels. The levels of CD5+ B cell percentage in the total B cells of the patients with acute febrile disease showed similar levels to age-matched normal controls. The decreased CD5+ B cells in the patients with KD provides an additional abnormal immunological finding during the acute phase of the disease.
Acute Disease ; Antigens, CD5/*analysis ; B-Lymphocytes/*immunology ; Child ; Child Preschool ; Female ; Human ; Infant ; Male ; Mucocutaneous Lymph Node Syndrome/*immunology/physiopathology ; Reference Values