Little is known about platelet dynamics and the effect of antiplatelet therapy in Kawasaki disease (KD). This study sought to define platelet activation dynamics in KD patients by assaying platelet-derived microparticles (PDMPs). We measured plasma PDMPs levels in 46 patients with KD using an enzyme-linked immunosorbent assay (ELISA). Blood samples were collected before, at 2–5 days, and 9–15 days after intravenous immunoglobulin (IVIG) infusion, 2 months and 4–5 months after the onset of KD. We measured PDMP levels in 23 febrile and 10 afebrile control patients. In the acute phase of KD patients, PDMP levels increased significantly after IVIG treatment (12.04 ± 5.58 nmol before IVIG infusion vs. 19.81 ± 13.21 nmol at 2–5 days after IVIG infusion, P = 0.006). PDMP levels were negatively correlated with age and positively correlated with procalcitonin levels in the acute phase of KD. No significant difference was found in PDMP levels between KD patients with and without coronary artery lesion (CAL). Elevated PDMP levels after IVIG therapy significantly decreased below the pre-IVIG level in subacute phase (19.81 ± 13.21 nmol at 2–5 days after IVIG infusion vs. 8.33 ± 2.02 nmol at 9–15 days after IVIG infusion, P < 0.001), and PDMP levels stayed below the pre-IVIG level in the convalescent phase, during which antiplatelet therapy was given. However, PDMP levels rebounded after discontinuing aspirin in 17 patients. In conclusion, enhanced platelet activation was noted before treatment of KD and peaked immediately after IVIG treatment. Recurrent rising of PDMP levels was observed after discontinuing aspirin, although there were no significant differences between the PDMP levels at 2 months after the onset of KD and those at 4–5 months after the onset of the disease.
Aspirin ; Blood Platelets ; Coronary Vessels ; Enzyme-Linked Immunosorbent Assay ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Mucocutaneous Lymph Node Syndrome* ; Plasma ; Platelet Activation

PURPOSE: There is currently little evidence to support intravenous immune globulin (IVIG) therapy for pediatric myocarditis. The purpose of our retrospective study was to assess the effects of IVIG therapy in patients with presumed myocarditis on survival and recovery of ventricular function and to determine the factors associated with its poor outcome. METHODS: We reviewed all consecutive cases of patients with myocarditis with left ventricular dysfunction verified by echocardiogram who had visited 3 university hospitals between January 2000 and September 2009. These patients were divided into 2 groups. Group 1 consisted of 23 patients (69.6%) who received IVIG alone or IVIG in combination with steroids, and group 2 consisted of 10 patients (30.3%) who received neither IVIG nor other immunosuppressive agents. Clinical manifestations, laboratory results, echocardiographic findings, and outcomes were compared between these 2 groups. RESULTS: One year after the initial presentation, the difference in the probability of survival did not show statistical significance in IVIG-treated patients (P=0.607). Of the echocardiographic parameters on admission, a shortening fraction of less than 15% was associated with unremitting cardiac failure. Furthermore, anemic patients were more likely to have elevated N-terminal fragment levels of the B-type natriuretic peptide (NT-proBNP) in the progressed group (P=0.036). CONCLUSION: There was no difference between the IVIG-treated patients and the control patients in the degree of recovery of left ventricular function and survival. Prospective, randomized, clinical studies are needed to elucidate the effects of IVIG treatment during the acute stage of myocarditis on ultimate outcomes.
Child ; Heart Failure ; Hospitals, University ; Humans ; Immunoglobulins, Intravenous ; Immunosuppressive Agents ; Myocarditis ; Natriuretic Peptide, Brain ; Retrospective Studies ; Steroids ; Ventricular Dysfunction, Left ; Ventricular Function ; Ventricular Function, Left

PURPOSE: Although high morning blood pressure (BP) is known to be associated with the onset of cardiovascular events in adults, data on its effects in children with hypertension are limited. Our retrospective study aimed to define the clinical characteristics of children with morning hypertension (MH) and to determine its associated factors. METHODS: We reviewed 31 consecutive patients with hypertension, confirmed by the ambulatory blood pressure monitoring (ABPM). We divided these patients into 2 groups: the MH group (n=21, 67.7%), morning BP above the 95th percentile for age and height (2 hours on average after waking up) and the normal morning BP group (n=10, 32.3%). We compared the clinical manifestations, laboratory results, and echocardiographic findings including left ventricular hypertrophy (LVH) between the groups. RESULTS: The early/atrial (E/A) mitral flow velocity ratio in the MH group was significantly lower than that in the normal morning BP group. In addition, LV mass was higher in the MH group than in the normal morning BP group, although the difference was not statistically significant. The age at the time of hypertension diagnosis was significantly higher in the MH group than in the normal morning BP group (P=0.003). The incidence of hyperuricemia was significantly higher in the MH group than in the normal morning BP group. CONCLUSION: Older patients and those with hyperuricemia are at higher risk for MH. The rise in BP in the morning is an important factor influencing the development of abnormal relaxation, as assessed by echocardiography. Clinical trials with longer follow-up periods and larger sample sizes are needed to clarify the clinical significance of MH.
Adult ; Blood Pressure ; Blood Pressure Monitoring, Ambulatory* ; Child* ; Diagnosis ; Echocardiography ; Follow-Up Studies ; Humans ; Hypertension* ; Hypertrophy, Left Ventricular ; Hyperuricemia ; Incidence ; Relaxation ; Retrospective Studies ; Sample Size

No abstract available.
Heart Atria*

PURPOSE: Although a significant number of reports on new therapeutic options for refractory Kawasaki disease (KD) such as steroid, infliximab, or repeated intravenous immunoglobulin (IVIG) are available, their effectiveness in reducing the prevalence of coronary artery lesions (CAL) remains controversial. This study aimed to define the clinical characteristics of patients with refractory KD and to assess the effects of adjuvant therapy on patient outcomes. METHODS: We performed a retrospective study of 38 refractory KD patients from January 2012 to March 2015. We divided these patients into 2 groups: group 1 received more than 3 IVIG administration+ steroid therapy, (n=7, 18.4%), and group 2 patients were unresponsive to initial IVIG and required steroid therapy or second IVIG (n=31, 81.6%). We compared the clinical manifestations, laboratory results, and echocardiographic findings between the groups and examined the clinical utility of additional therapies in both groups. RESULTS: A significant difference was found in the total duration of fever between the groups (13.0±4.04 days in group 1 vs. 8.87±2.30 days in group 2; P=0.035). At the end of the follow-up, all cases in group 1 showed suppressed CAL. In group 2, coronary artery aneurysm occurred in 2 patients (6.4 %). All the patients treated with intravenous corticosteroids without additional IVIG developed CALs including coronary artery aneurysms. CONCLUSION: No statistical difference was found in the development of CAL between the groups. Prospective, randomized, clinical studies are needed to elucidate the effects of adjunctive therapy in refractory KD patients.
Adrenal Cortex Hormones ; Aneurysm ; Coronary Disease ; Coronary Vessels ; Echocardiography ; Fever ; Follow-Up Studies ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Infliximab ; Mucocutaneous Lymph Node Syndrome* ; Prevalence ; Prospective Studies ; Retrospective Studies

Body fat is an important source of adipokine, which is associated with energy balance and inflammatory and immune responses. However, the role of adipokines in coronary artery complications in Kawasaki disease (KD) has not yet been fully explained. We investigated whether serum adipokine level can be a useful marker for patients with KD who are at higher risk of developing coronary artery lesion (CAL). We measured adipokine levels and other inflammatory parameters in 40 patients with KD, 32 febrile controls, and 15 afebrile controls. Interleukin (IL)-6, tumor necrosis factor (TNF)-alpha and other laboratory parameters were also measured before and after intravenous immunoglobulin therapy, and in the convalescent phase. At admission, the serum resistin levels in KD children were significantly higher than those in controls (177.56 ng/mL in KD children, 76.48 ng/mL in febrile controls, and 17.95 ng/mL in afebrile controls). In patients with KD, resistin levels were significantly associated with decreased hemoglobin levels (P=0.049) and increased IL-6 levels (P=0.014). The serum IL-6 levels were significantly higher and body mass index was significantly lower in the group of KD with CALs than those without CALs (228.26 ng/mL vs. 39.18 ng/mL and 15.09 vs. 16.60, respectively). In conclusion, resistin is significantly elevated in KD patients, although it has no prognostic value of predicting coronary artery lesion in the acute stage.
Biological Markers/*blood ; Child ; Child, Preschool ; Coronary Vessels/pathology ; Echocardiography ; Female ; Hemoglobins/analysis ; Humans ; Immunoglobulins, Intravenous/therapeutic use ; Inflammation/blood/immunology ; Interleukin-6/*blood ; Male ; Mucocutaneous Lymph Node Syndrome/*blood/pathology ; Resistin/*blood ; Tumor Necrosis Factor-alpha/*blood

PURPOSE: To assess the incidence of coronary artery lesion(CAL) and the efficacy of intravenously administered immune globulin(IVGG) and aspirin therapy, identify risk factors for CAL, and analyze clinical characteristics in infants less than 12 months of age with Kawasaki disease. METHODS: Retrospective chart review of children less than 12 months of age with Kawasaki disease between 1994 and 1998, diagnosed at Chungnam National University Hospital. RESULTS: Of 202 patients with Kawasaki disease, 32(16 percent) were less than 1 year of age, including 7(3 percent) less than 6 months. Sex ratio of male to female was 2.5:1. Age at onset and Harada score were a predictor of the development of CAL:5(71 percent) of 7 children less than 6 months and 10(40 percent) of 25 children between 6 to 12 months of age acquired CAL (P<0.05), and 1(14 percent) of 7 children less than 6 months of age acquired giant CAL. No specific clinical or laboratory features predicted the development of CAL. Persistent(greater than 1 year) CAL were present in 2(7 percent) of 29 IVGG-treated children. The typical clinical features of Kawasaki disease was noted 24(75 percent) of 32 and the atypical one, 8(25 percent) of 32 children less than 12 months of age. CONCLUSION: Patients with Kawasaki disease of less than 12 months of age are at particularly increased risk of having CAL and difficulty in diagnosis due to atypical clinical features. So, it is suggested to intervene in the diagnostic criteria or risk factors for CAL, especially for patients with infant Kawasaki disease of less than 6 months of age.
Aspirin ; Child ; Chungcheongnam-do ; Coronary Vessels ; Diagnosis ; Female ; Humans ; Incidence ; Infant* ; Male ; Mucocutaneous Lymph Node Syndrome* ; Retrospective Studies ; Risk Factors ; Sex Ratio

PURPOSE: Microdeletion of chromosome 22q11.2 are associated with DiGeorge syndrome(DGS), velocardiofacial syndrome(VCFS) and conotruncal anornaly face syndrome(CTAFS). DGS was originally described as an irnmunodeficiency disorder secondary to impaired T cell production due to thymic aplasia or hypoplasia. But the frequency E: severity of immunodeficiency of other clinical syndromes associated with the chromosome 22qll deletion has not been investigated. This study was undertaken to investigate the frequency and severity of immunodeficiency, the relation- ship of the immunodeficiency to clinical phenotypes, and the change of immunologic status with age in CATCH 22 syndromes patients. METHODS: Sixteen patients with CATCH 22 syndrome with characteristic clinical phenotype and chromosome 22qll deletion were studied. Hurnoral and cellular irnmunities were examined by measuring serurn IgG, IgA, IgM level and by T cell subset through flow cytometry and lymphocyte proliferation test by common T cell mitogens respectively. RESULTS: 69Zo of patients with CATCH 22 syndrome were found to have evidence of immunocompromise. The severity of the immunodeficiency did not correlate with any particular phenotypic features nor was it restricted to patients who were categorized as having DiGeorge syndrome. The severity of immunodeficiency tended to be normalized with age. CONCLUSION: The presence of the immunocompromise is common and its severity cannot be predicted based on the clinical phenotype of CATCH 22 syndrome. Therefore, each child with CATCH 22 syndromes regardless of clinical phenotype should be extensively assessed for earlier detection of subclinical immunodeficiency.
Child ; DiGeorge Syndrome ; Flow Cytometry ; Humans ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Lymphocytes ; Mitogens ; Phenotype ; Ships ; T-Lymphocytes

Renal artery stenosis is a major cause of renovascular hypertension and the most common cause of treatable secondary hypertension. There are several methods to treat renal artery stenosis, including surgery, percutaneous transluminal renal angioplasty(PTRA), and renal artery stenting(RAS). But, renal artery embolization can be tried in atherosclerotic stenosis, multiple stenosis, microaneurysm, and stenosis difficult to try PTRA or RAS. We report a case of renovascular hypertension in a 14-year-old female who had multiple segmental renal artery stenosis. Hypertension was controlled by renal ablation therapy with renal artery embolization.
Adolescent ; Constriction, Pathologic ; Female ; Humans ; Hypertension ; Hypertension, Renovascular* ; Renal Artery Obstruction ; Renal Artery*

No abstract available.
Pregnancy*