• Clinical and Experimental Pediatrics
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• v.50 no.12
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• pp.1247-1251
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• 2007

Purpose : Pathologically, Kawasaki disease (KD) is associated with widespread vascular endothelial damage in the acute phase. The vasculitis induced endothelial injury leads to coagulation abnormalities. Abnormalities of endothelial function, platelet activation, and fibrinolysis are present during acute phase and long after the onset of KD. The aim of study is to evaluate the change of hemostatic markers in the clinical stages of KD and to assess the hemostatic markers to be a useful indicator of the development of coronary artery lesion (CAL). Methods : Seventy four KD patients diagnosed in Chungnam National University Hospital from November 2004 to June 2007. Eleven febrile control and eleven healthy children were selected for healthy control. All blood samples were collected before and after Intravenous gammaglobulin (IVGG), $2^{nd}$ week, and $4^{th}-8^{th}$ week of illness of KD. Results : Initial D-dimer level of Kawasaki disease showed meaningful difference compared to control group (P<0.05). D-dimer and fibrinogen degradation products (FDP) before IVGG increased compared with normal control group and decreased after IVGG administration. It is normalized until 2 weeks later, and continue to decreasing. D-dimer and FDP were significantly different according to the CAL before IVGG. Conclusion : The hemostatic markers may change to the clinical stage of KD, which may suggest the degree of endothelial injury. Increased some hemostatic markers may be the predictors for development of CAL.

• Clinical and Experimental Pediatrics
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• v.48 no.6
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• pp.640-648
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• 2005

Purpose : Kawasaki disease(KD) is known as an acute multi-systemic vasculitis with various immunologic abnormalities. Adhesion of leukocyte to endothelial cells is a key event in the sequence of inflammatory response. This study was performed to investigate the clinical significance of serum soluble intercellular adhesion molecule-1(sICAM-1) and soluble vascular cell adhesion molecule-1 (sVCAM-1) in acute and subacute stages of typical KD for diagnostic and prognostic value. Methods : A typical KD group was 32 patients who were hospitalized from Jan. 2002 to Jun. 2004 was enrolled. Control was 16 non-KD patients with febrile illness. sICAM-1 and sVCAM-1 were measured and compared by Echocardiographic and clinical findings and cardiac troponin T and I. Results : sICAM-1 and sVCAM-1 levels of acute KD were significantly elevated over control(P=0.019 vs. P=0.049, respectively) and sICAM-1 was significantly decreased in subacute stage(P=0.0015). sICAM-1 and sVCAM-1 had positive correlation with each other in both stages(P=0.0067, P=0.015, retrospectively). Neither sICAM-1 nor sVCAM-1 correctly reflected the coronary abnormalities and responsiveness to intravenous gammaglobulin(IVGG) in both stages. But sVCAM-1 was significantly increased in the carditis group in both stages(P=0.025, P=0.014, retrospectively) and had a positive correlation with troponin T(r=0.63, P=0.00063). Conclusion : The levels of sICAM-1 and sVCAM-1 were not very useful tools for detecting and predicting subsequent coronary abnormalities and responsiveness to IVGG in KD patients. However, sVCAM-1 appears to play a significant role in carditis of KD. Further studies are needed about various adhesion molecules and cytokines in the pathogenesis of KD.

• Clinical and Experimental Pediatrics
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• v.48 no.5
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• pp.539-544
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• 2005

Purpose : The aim of this study was to investigate the pathophysiologic role of serum E-selectin, vascular endothelial growth factor(VEGF)-induced cell adhesion mollecule in Kawasaki disease(KD) and to look for the evidence of direct relationship between the plasma levels of soluble E-selectin and the incidence of coronary artery lesion(CAL). Methods : Changes in plasma levels of sE-selectin(n=98) over time were measured by enzyme-linked immunosorbent assay(ELISA) in 23 patients with acute KD and 25 age-matched febrile children. Results : Compared with control values, the peak levels of plasma sE-selectin were significantly elevated($mean{\pm}S.E$. : $22.89{\pm}12.53ng/mL$ vs $10.65{\pm}3.42ng/mL$, P=0.01) in KD. 5 patients with CAL, plasma sE-selectin levels before treatment were higher than in 18 patients without CAL($mean{\pm}S.E$. : $39.43{\pm}15.08ng/mL$ and $19.00{\pm}8.32ng/mL$, respectively; P=0.01). Plasma sE-selectin declined rapidly in the majority of KD patients regardless of the presence of CAL. Plasma sE-selectin levels after treatment and convalesent period were similar in KD patients with and without CAL. The plasma levels sE-selectin were correlated with those of white blood cell count(r=0.299, P<0.05), CRP(r=0.430, P<0.05), serum albumin(r=-0.483, P<0.05), serum protein(r=-0.502, P<0.05) and hemoglobin(r=-0.372, P<0.05) not with those of ESR, platelet, or duration of fever. There were significant differences in the initial level of serum sE-selectin between KD with and without CAL($mean{\pm}S.E$. : $39.44{\pm}15.08ng/mL$ vs. $19.00{\pm}17.18ng/mL$) in multivariated linear tests. Conclusion : Plasma sE-selectin levels were significantly higher in KD than in other febrile illness. Higher plamsa levels of sE-selectin may have potential as a predictor of CAL in patients with KD.

• Clinical and Experimental Pediatrics
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• v.46 no.12
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• pp.1217-1223
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• 2003

Purpose : This study was conducted to assess clinical practices related to the evaluation and treatment of patients with acute gastrointestinal infection(AGI) in infants and children. This survey also evaluated the current opinion regarding the use of rotavirus vaccine. Methods : This survey was conducted using a self-administered questionnaire provided to 82 pediatricians in private clinics of Jeonbuk province. From April to June, 2002, 63 of 82 pediatricians(76.8 %) responded to the survey. Results : The annual proportion of patients with AGI was 13%. The highest proportion of patients with diarrhea were reported for the months of October through December. For the treatment of diarrhea, pediatricians preferred to prescribe lactobacillus products, oral rehydration solution, enzyme products, anti-emetics, anti-diarrheal formula, nothing by mouth(NPO) and bowel movement inhibitors in descending order of frequency. Most pediatricians(79.4%) prescribed antibiotics in less than 20% of patients with AGI. Amoxicillin was the most commonly prescribed antibiotics followed by trimethoprim/sulfamethoxazole(TMP/SMX) and amoxicillin/clavulanate. Clinical manifestations that pediatricians considered as important factors in prescribing antibiotics were bloody diarrhea, mucoid diarrhea, high fever, persisting disease and abdominal pain. Diagnostic procedures that pediatricians preferred for AGI patients were stool examination(rotavirus antigen, RBC, WBC), abdominal X-ray, and serum electrolytes. Pediatricians had a strong interest in rotavirus vaccine(71.4%). Conclusion : Acute gastrointestinal illness remains a common problem, especially during the winter months in Korea. AGI manifesting as watery diarrhea is appropriately treated with commonly available agents but pediatricians appear ready to consider additional approaches to decrease the sizable disease burden of AGI among children in Korea.

• Clinical and Experimental Pediatrics
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• v.52 no.2
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• pp.234-241
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• 2009

Purpose : We investigated the relationship between thyroid hormone and serum tumor necrosis factor (TNF-${\alpha}$), interleukin (IL-6) and N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP) in patients with Kawasaki disease (KD). Methods : Serum levels of thyroid hormone, TNF-${\alpha}$, IL-6, and NT-proBNP were measured in 52 KD patients in the acute and subacute phase and 10 patients with acute febrile illness (control group). TNF-${\alpha}$ and IL-6 were determined by sandwich enzyme-linked immunosorbent assay (ELISA). Echocardiography was performed to detect coronary artery lesions (CAL) in KD patients. Results : Low $T_3$ syndrome occurred in 63.5% of KD patients. $T_3$ in the acute phase of KD was lower than that in the control. In KD patients, $T_3$ was lowered in the acute phase and elevated in the subacute phase, whereas TNF-${\alpha}$, IL-6 and NT-proBNP were elevated in the acute phase and decreased in the subacute phase. NT-proBNP, and IL-6 were higher in patients with low $T_3$ than in those with normal $T_3$. In addition, $T_3$ inversely correlated with IL-6 and NT-proBNP. Of the 4 patients with CAL, 3 had very low $T_3$. Compared with intravenous immunoglobulin (IVIG)-responsive patients, IVIG-resistant patients had lower $T_3$ and higher IL-6 and NT-proBNP. Conclusion : $T_3$ decreases in the acute phase of KD and normalizes in the subacute phase without thyroid hormone replacement. Low $T_3$ may be partially induced by IL-6 rather than TNF-${\alpha}$, and is strongly associated with high NT-proBNP. $T_3$ in KD may be used for the differential diagnosis, monitoring the activity of the disease, and predicting the severity of inflammation.

• Clinical and Experimental Pediatrics
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• v.49 no.8
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• pp.857-863
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• 2006

Purpose : To evaluate the immunogenicity of hepatitis B vaccine among very low birth weight infants(VLBWI) who were vaccinated at 0, 1, 6 months of chronological age and to determine the factors associated with antibody formations. Methods : A total of 243 VLBWI admitted to Seoul and Gangneung Asan Medical Center neonatal intensive care units from 1997 to 2004 were included. Of 243, 13 infants were born to HBs Ag positive mother. All infants were given DNA recombinant vaccine at 0, 1, and 6 months of chronological age. Infants born to HBs Ag positive mothers received hepatitis B immunoglobulin at birth and a total of 4 doses of vaccinations. An antibody level over 10 mIU/mL, tested at 3-4 months after last vaccination, was regarded as a positive seroconversion. Results : The seroconversion rates were 84.4 percent and 84.5 percent for VLBWI and extremely low birth weight infants(ELBWI), respectively. Of 28 seronegative infants who were given revaccinations, 60.7 percent seroconverted, resulting in 95.3 percent, 97.5 percent seroconversion rates for VLBWI and ELBWI, respectively. 76.9 percent of infants born to HBsAg positive mothers seroconverted and none became hepatitis B carriers. Factors such as gestational age, sex, various neonatal illness, and kinds of vaccinations did not influence the formation of the hepatits B antibody, however, the higher the weight at time of first vacciation yielded better seroconversion rate. Conclusion : Revaccination of seronegative VLBWI after 3 doses of hepatitis B vaccinaton is very effective. Therefore, testing the immune status after the hepatitis B vaccination, a practice not routinely done, is highly recommended.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.474-480
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• 2008

Purpose : Stress hyperglycemia is common in critically ill adult patients. It is known as a predictor of increased mortality, and intensive insulin therapy has been shown to improve the prognosis in such patients. We have investigated the relationship between early stress hyperglycemia and clinical outcomes in preterm infants. Methods : In this study, 141 preterm infants with a gestational age of less than 30 weeks were enrolled. The hyperglycemic group was defined as that having maximum glucose of more than 150 mg/dL (n=61) during the first 48 h of life, and the non-hyperglycemic group was defined as that having maximum glucose of less than 150 mg/dL (n=80). Perinatal history, severity of illness using the Clinical Risk Index for Babies (CRIB) score, clinical outcomes, and mortality of the two groups were compared. Results : There was no significant difference in the gestational age between the two groups, but the birth weight (P<0.001) was significantly lower, and the CRIB score (P<0.001) was significantly higher in the hyperglycemic group. Disseminated intravascular coagulation (P<0.001) and clinically suspected sepsis (P=0.046) were more common in the hyperglycemic group. Mortality was markedly higher in the hyperglycemic group (11.3% vs. 41.0%, P<0.001). On performing a stepwise multiple logistic regression analysis, hyperglycemia (OR 3.787; 95% CI 1.324 to 10.829), the CRIB score (OR 1.252; 95% CI 1.047 to 1.496) and birth weight (OR 0.997; 95% CI 0.994 to 1.000) was independently associated with higher mortality. Conclusion : Stress hyperglycemia within the first 48 h of life is independently related to increased morbidity and mortality in preterm infants.

• Clinical and Experimental Pediatrics
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• v.51 no.9
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• pp.964-970
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• 2008

Purpose : Methylmalonic aciduria (MMA) and propionic aciduria (PA) are inborn errors in the catabolism of branched-chain amino acids. The study was undertaken to investigate the genotypes and clinical features of Korean patients with MMA and PA. Methods : This study examined 12 patients with MMA and eight with PA. We analyzed various clinical features, laboratory findings, treatments, and neuro-developmental outcomes. Diagnoses were based on the presence of characteristic compounds detected by amino acid analysis in serum and organic acid analysis in urine. Mutation analysis was performed in the genes of MUT, MMAA, MMAB, and MMACHC for MMA and PCCA and PCCB for PA. Results : Among the 20 patients, six patients were diagnosed before one month of age and nine patients were diagnosed after the newborn period. Five patients were diagnosed via a neonatal screening test. Patients with early-onset forms had more severe illness at presentation and generally poor outcomes. A favorable outcome was obtained in 55% patients; most of them were of a late-onset type or diagnosed by neonatal mass screening test without symptoms. Genotypes were confirmed in all patients with MMA. We detected 11 different mutations by MUT gene analysis in 10 patients, and three different mutations in MMACHC genes in two patients. PCCA and PCCB gene mutations were identified in 14 of the 16 alleles, in eight patients with PA. Conclusion : Organic aciduria is a fatal disease; however, better outcomes are expected whenever early diagnosis and prompt management are made possible. Mutation analysis is useful for confirming diagnoses and planning management strategies.

• Clinical and Experimental Pediatrics
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• v.51 no.5
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• pp.487-491
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• 2008

Purpose : Although Mycoplasma pneumoniae (M. pneumoniae) infection can cause wheezing in non-asthmatic children, the mechanisms of this symptom remain unclear. Vascular endothelial growth factor (VEGF) is a major mediator of angiogenesis and vascular permeability, and is also known to be elevated in cases of chronic pulmonary disease such as asthma. We hypothesized that VEGF may increase in children with acute M. pneumoniae pneumonia and wheezing. Methods : Nine patients with clinical and laboratory evidence of acute M. pneumoniae pneumonia were enlisted from children admitted to Korea University Hospital. They had had more than one episode of wheezing during the illness, which was confirmed by a physician; they comprised the wheezer group. The individuals with M. pneumoniae pneumonia without wheezing were 63 in number, and they comprised the non-wheezer group. Patients with a history of asthma or who had received asthma medications were excluded. Serum concentrations of VEGF, total IgE, eosinophil cationic protein (ECP), and peripheral blood eosinophil counts were measured. Results : The serum VEGF concentrations were higher in the wheezer group ($mean{\pm}SD$; $650.2{\pm}417.9pg/mL$) than in the non-wheezer group ($376.5{\pm}356.2pg/mL$, P=0.049). M. pneumoniae antibody (1:1,380 vs. 1:596, P=0.048) and serum total IgE (591.8 IU/mL vs. 162.2 IU/mL, P=0.032) were higher in the wheezer group than in the non-wheezer group. There were no differences between the two groups in terms of serum ECP concentration or blood eosinophil count. Conclusion : In the presence of wheezing, serum VEGF concentrations were higher in the children with M. pneumoniae pneumonia. This finding suggests that VEGF may associate with wheeze-related symptoms in children with acute M. pneumoniae pneumonia.

• Clinical and Experimental Pediatrics
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• v.48 no.12
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• pp.1370-1377
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• 2005

Purpose : Delineation of serum lipid and lipoprotein values in children after Kawasaki disease(KD) is important because of the predilection of this disease for the coronary arteries. Methods : The KD group was composed of 51 patients who were hospitalized from Jan. 2002 to Dec. 2003. Control was 25 patients with non-KD febrile illness. The levels of total lipid, phospholipid, triglyceride, HDL-cholesterol(HDL-C), LDL-cholesterol, total cholesterol, apolipoprotein A1(apo A1), apolipoprotein B, and Lipoprotein(a) were measured and compared with Echocardiographic findings. Measurements were obtained in four time periods: acute febrile, subacute, convalescent phase and 1 year after KD. Results : HDL-C($33.64{\pm}7.49mg/dL$ vs $50.43{\pm}14.41mg/dL$, P<0.01) and apo A1($99.75{\pm}6.39mg/dL$ vs $113.34{\pm}11.35mg/dL$, P<0.05) were decreased more in the acute febrile period of KD than in the control, but these changes were not correlated with cardiac complications. All lipid profiles were markedly elevated in the subacute stage and normalized in the convalescent stage; there were no changes until 1-year follow up. There were no significant differences in the changes of lipid profiles, including Lp(a) and coronary dilatation, in any time periods. Conclusion : KD is associated with important abnormalities in lipid metabolism, but these changes were transient and appear to be due to the disease itself. These data lead us to infer that KD dose not cause such permanent changes in lipid abnormalities as to be considered a risk factor for atherosclerosis, beyond that caused by the disease itself.