• Clinical and Experimental Pediatrics
• /
• v.53 no.2
• /
• pp.228-234
• /
• 2010

Purpose : Although intravenous immunoglobulin (IVIG) treatment is an effective first-line treatment for Kawasaki disease, 10-20% of the patients develop persistent fever or coronary artery complications. Medical records of Kawasaki disease patients were reviewed to assess the characteristic laboratory findings of IVIG nonresponsiveness. Methods : We reviewed the clinical records of 118 children with Kawasaki disease who were treated at the Chonnam National University Hospital from March 2003 to February 2008. The laboratory findings of the IVIG-responder group (n=110) and the IVIG-nonresponder group (n=8) were compared at admission day and at 48 hours and 14 days after IVIG administration. Results : At admission, the level of creatine kinase (CK) was lower (P =0.03) and that of total protein was higher (P <0.01) in the nonresponders than in the responders. At 48 hours after IVIG administration, the white blood cell (WBC) count (P =0.04) and neutrophil% (P <0.01) was higher in the nonresponders than in the responders. The neutrophil% (P <0.01) and CK (P =0.01) level at admission was lower than that at 48 hours after IVIG administration in the responders; this decrease was not as apparent in the nonresponders. Conclusion : IVIG nonresponders have lower CK and higher total protein levels at admission and higher WBC count and neutrophil% at 48 hours after IVIG administration. The decrease in the neutrophil% and CK level between at admission and at 48 hours after IVIG administration is remarkably higher in responders than in nonresponders.

• Annals of Clinical Neurophysiology
• /
• v.8 no.1
• /
• pp.6-15
• /
• 2006

Intravenous immunoglobulin (IVIg) is the treatment of choice for many autoimmune neuropathic disorders such as Guillain-Barre syndrome (GBS), chronic inflammatory Demyelinating neuropathy (CIDP), and multifocal motor neuropathy (MMN). IVIg is preferred because the adverse reactions are milder and fewer than the other immune-modulating methods such as steroid, other immunosuppressant such as azathioprine, and plasmapheresis. IVIg also has been used in other autoimmune neuromuscular disorders (inflammatory myopathy, myasthenia gravis, and Lambert-Eaton myasthenic syndrome) and has been known as safe and efficient agent in these disorders. Since IVIg would get more indications and be used more commonly, clinicians need to know the detailed mechanism of action, side effects, and practical points of IVIg.

• Clinical and Experimental Pediatrics
• /
• v.49 no.7
• /
• pp.784-789
• /
• 2006

Purpose : $Henoch-Sch{\ddot{o}}nlein$ purpura(HSP) is a systemic vasculitis involving small vessels of skin, gastrointestinal(GI) tract and kidney. Digestive involvement of HSP can be serious with massive GI bleeding, perforation, and intussusception. However, some patients do not respond to conventional corticosteroid therapy. In this study, we investigated the efficacy of intravenous immunoglobulin (IVIG) for serious digestive manifestations not responding to steroid. Methods : From April 1999 to January 2005, 22 children diagnosed as HSP with severe GI symptoms were included. Initially, all patients were treated with intravenous methylprednisolone. IVIG 2 g/kg of body weight was infused in children refractory to steroid therapy. Clinical data were reviewed retrospectively. Results : Among 22 children, 12 children underwent IVIG therapy. The mean duration of corticosteroid therapy was $5.61{\pm}4.9$ days before IVIG therapy, and 11 of 12 patients experienced disappearance of GI manifestations after the initiation of IVIG infusion. In one patient, IVIG was ineffective in relieving abdominal pain, but melena subsided. Comparison of the duration of hospitalization between IVIG group and corticosteroid group revealed no significant difference($12.8{\pm}7.6$ days vs. $13.2{\pm}7.8$ days, P=0.777). But, the total duration of abdominal pain decreased in IVIG group although the difference between two groups was not significant($8.8{\pm}8.1$ days vs. $14.8{\pm}16.9$ days, P=0.306). Among 10 children treated with steroid only, 2 children were operated for bowel perforation and intussusception. In contrast, there was no perforation in 12 children who underwent IVIG therapy. Conclusion : IVIG could be the alternative therapy to corticosteroids in children with severe digestive manifestations of HSP.

• Clinical and Experimental Pediatrics
• /
• v.59 no.12
• /
• pp.477-482
• /
• 2016

Purpose: We conducted a study to determine which factors may be useful as predictive markers in identifying Kawasaki disease (KD) patients with a high risk of resistance to intravenous immunoglobulin (IVIG) and developing coronary artery lesions (CAL). Methods: We enrolled 287 patients in acute phase of KD at a single center. The demographic, clinical and laboratory data were collected retrospectively. Results: There were 34 patients in the IVIG resistant group. The IVIG resistant group had significantly higher serum N-terminal-pro-brain natriuretic protein (NT-proBNP) levels (P<0.01) and polymorphonuclear neutrophil (PMN) percentage (P<0.01) in comparison to the IVIG responders. The results yielded sensitivity (78.8%, 60.6%), specificity (58.2%, 90%) and cutoff value (628.6 pg/mL, 80.3%) of NT-proBNP and PMN respectively, in predicting IVIG resistance. Despite IVIG administration, 13 of the 287 patients developed CAL. The patients in the CAL group had higher NT-proBNP levels (P<0.01) and higher PMN percentage (P<0.01). In these patients, the results yielded sensitivity (73.3%, 56.7%), specificity (67.9%, 88.9%) and cutoff value (853.4 pg/mL, 80.3%) of NT-proBNP and PMN respectively, for predicting CAL. The area under the curve (AUC) for predicting resistance to IVIG was NT-proBNP 0.712, PMN 0.802. The AUC for predicting CAL was NT-proBNP 0.739, and PMN 0.773. Conclusion: Serum NT-proBNP levels and PMN percentage were significantly elevated in patients with KD with IVIG resistance and CAL. Thus, they may be useful predicting markers for IVIG resistance and development of CAL in KD patients.

• Clinical and Experimental Pediatrics
• /
• v.49 no.12
• /
• pp.1348-1353
• /
• 2006

Purpose : The reticuloendothelial system is composed of sinusoidal capillaries, through which even large protein molecules are freely movable between plasma and interstitial space, including the lymphatic system. Therefore, high-dose intravenous immunoglobulin (IVIG) would cause a redistribution of proteins between two compartments. To investigate this hypothesis, we measured plasma protein and lipid levels in patients with Kawasaki disease before and after high-dose IVIG treatment. Methods : Thirty four children with Kawasaki disease who had complete responses to high-dose IVIG treatment (1 g/kg/day for two consecutive days), were analyzed. Before and after the administration of IVIG, serum analyses were performed for such parameters as total protein, albumin, ${\gamma}$-globulins (IgG, IgM, IgA), ${\alpha}1-$, ${\alpha}2-$, and ${\beta}-$ globulin fractions, and lipid profiles (total cholesterol, HDL-cholesterol, LDL-cholesterol and triglyceride). Results : The levels of ${\gamma}$-globulins including IgG, IgM, IgA were significantly increased, and IgG was increased by $1,779{\pm}304mg/dL$ after two-dose of IVIG infusion. The levels of albumin, ${\alpha}1-$, ${\alpha}2-$, and ${\beta}$ globulin fractions were significantly decreased by 18 percent, 24 percent, 19 percent and 12 percent, respectively. HDL-cholesterol level was significantly decreased by 20 percent, while LDL-cholesterol and triglyceride levels were significantly increased by 21 percent and 50 percent, respectively. The total cholesterol level was not changed. Conclusion : High-dose IVIG treatment decreased the levels of a variety of proteins except immunoglobulins, and the increase of IgG after IVIG treatment was lower than expected. Our results suggest that a part of infused IVIG and plasma proteins, including etiologic proteins for Kawasaki disease, may be distributed to the extravascular compartments. The rapid improvement of symptoms induced by IVIG in Kawasaki disease might be explained by this mode of action of IVIG.

• Clinical and Experimental Pediatrics
• /
• v.50 no.10
• /
• pp.982-986
• /
• 2007

Purpose : Intravenous immunoglobulin (IVIG) is effective for the treatment of idiopathic thrombocytopenic purpura (ITP) in children. Recently, several reports have been published that show its impact on the absolute neutrophil count. The present study was performed to confirm these findings. Methods : Data on 26 ITP patients were analyzed. Patients with febrile illness or increased C-reactive protein levels at presentation, which would influence the neutrophil counts, were excluded to determine the sole impact of IVIG. In addition, patients who received steroid treatment were also excluded. Results : Sixteen boys and ten girls were analyzed. For patients who received an IVIG dose of 0.4 g/kg/day (n=17), the absolute neutrophil count (ANC) measured next day was significantly decreased. For patients who received an IVIG dose of 1 g/kg/day (n=9), the ANC measured the next day was also significantly decreased. However, the decrease was more profound in the high-dose group compared to the low-dose group. Among six cases with profoundly decreased ANC greater than $1,000/mm^3$, four patients (67%) received IVIG at a dose of 1 g/kg/day. All four cases with increased ANC were treated with IVIG dose of 0.4 g/kg/day, and three cases (75%) among them had a febrile reaction during IVIG administration. None of the cases with decreased ANC had a febrile reaction. No cases had infectious complications reported. Conclusion : IVIG treatment for ITP patients appears to suppress the ANC. This decrease of ANC was more pronounced when a higher dose of IVIG was used. Some cases with increased ANC counts after IVIG use were found only in low-dose IVIG group, and was associated with febrile reactions during IVIG use.

• Korean Journal of Clinical Pharmacy
• /
• v.27 no.3
• /
• pp.127-135
• /
• 2017

Background: Kawasaki disease (KD) is an acute febrile, systemic vasculitis as a leading cause of acquired heart disease in children. Intravenous immunoglobulin G (IVIG) and aspirin are the standard initial therapy in the treatment of acute KD. The purpose of this study was to investigate drug utilization in children with KD, and to compare "IVIG + high-dose aspirin" and "IVIG + moderate-dose aspirin" in preventing cardiac complications. Methods: We analyzed pediatric patient sample data compiled by the Health Insurance Review & Assessment Service from 2010 to 2015. We identified patients with KD using the KCD-6 code of M30.3. We excluded patients in chronic phase or ${\geq}1$0 years. We also excluded patients who were diagnosed KD in November or December. Drug utilization pattern were assessed in acute KD patients and 30-day and 60-day cardiac complications were investigated between "IVIG + high-dose aspirin" group and "IVIG + moderate-dose aspirin" group. Results: In acute phase, IVIG was administered to 95.8% patients, and 57.1% patients were prescribed moderate-dose aspirin and 25% patients were with high-dose aspirin. Steroid use was rapidly increased from 4.0% in 2010 to 11.3% in 2015. Both 30-day and 60-day cardiac complications occurred less in "IVIG + high-dose aspirin" group compared to "IVIG + moderate-dose aspirin" group, but not statistically significant (0.9% vs 1.8%, p=0.252 for 30-day complication rate; 1.5% vs 2.7%. p=0.073 for 60-day complication rate). Conclusion: We were not able to demonstrate which aspirin therapy is superior for preventing cardiac complications in acute KD patients and further research is warranted.

• Clinical and Experimental Pediatrics
• /
• v.59 no.4
• /
• pp.178-182
• /
• 2016

Purpose: Medium-dose (1 g/kg) intravenous immunoglobulin (IVIG) is effective in the majority of patients with Kawasaki disease (KD) but some patients who do not respond to medium-dose IVIG are at high risk for the development of coronary artery lesions (CALs). The purpose of this study was to identify the clinical predictors associated with unresponsiveness to medium-dose IVIG and the development of CALs. Methods: A retrospective study was performed in 91 children with KD who were treated with mediumdose IVIG at our institution from January 2004 to December 2013. We classified the patients into responders (group 1; n=68) and nonresponders (group 2; n=23). We compared demographic, laboratory, and echocardiographic data between the 2 groups. Results: Multivariate logistic regression analysis identified 6 variables as predictors for resistance to medium-dose IVIG. We generated a predictive scoring system assigning 1 point each for percentage of neutrophils ${\geq}65%$, C-reactive protein ${\geq}100mg/L$, aspartate aminotransferase ${\geq}100IU/L$, and alanine aminotransferase ${\geq}100IU/L$, as well as 2 points for less than 5 days of illness, and serum sodium level ${\leq}136mmol/L$. Using a cutoff point of ${\geq}4$ with this scoring system, we could predict nonresponsiveness to medium-dose IVIG with 74% sensitivity and 71% specificity. Conclusion: If a patient has a low-risk score in this system, medium-dose IVIG can be recommended as the initial treatment. Through this process, we can minimize the adverse effects of high-dose IVIG and incidence of CALs.

• Annals of Clinical Neurophysiology
• /
• v.8 no.1
• /
• pp.48-52
• /
• 2006

Background: Intravenous immunoglobulin (IVIg) has been administered for various immune-mediated neurological diseases such as autoimmune neuropathy, inflammatory myopathies, and other autoimmune neuromuscular disorders. The purpose of this study is to investigate side effects and complications of IVIg therapy in neuromuscular disorders. Methods: We enrolled 29 patients (age 8~63 years) with IVIg therapy for various neurological diseases including Guillain-Barre syndrome, myasthenia gravis, dermatomyositis, polymyositis, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. IVIg therapy was used at a dose of 0.4 g/kg body weight/day for 5 consecutive days. Results: 10 patients (34%) had adverse events. There are adverse events in 16 courses (11%) among total 145 courses. The majority of patients presented with mild side effects, mostly asymptomatic laboratory changes. Rash or mild headache occurred in 3 patients. One patient showed a serious side effect of deep vein thrombosis. Conclusions: IVIg therapy is safe for a variety of immune-mediated neurological diseases in our study.

• Clinical and Experimental Pediatrics
• /
• v.52 no.10
• /
• pp.1161-1166
• /
• 2009

Purpose : Kawasaki disease—the most common cause of acquired heart disease in children—incidence is increasing yearly. Therefore, we evaluated the predictive indicators of coronary complications of Kawasaki disease based on clinical and laboratory data. Methods : Between January 2005 and March 2008, of the 201 children with Kawasaki disease treated at the Gil Hospital of Gachon University of Medicine and Science, 51 had coronary artery lesions (Group II) and 150 had no lesions (Group I). The reasons for coronary artery lesions were deduced from the clinical and laboratory data. Results : Analysis of the 2 groups revealed that fever duration and days of fever after and before initial intravenous gammaglobulin (IVIG) treatment were significantly longer in Group 2 than in Group I. IVIG infusions were statistically higher in Group II than in Group I. As per the laboratory data, C-reactive protein (CRP) value was significantly higher in Group II. Collectively, >10 days of fever duration, >48 h of fever duration after, and >10 days of fever before IVIG treatment increased the risk of coronary artery lesions 6-, 5-, and 3.5-fold, respectively. Furthermore, additional IVIG courses and higher CRP level increased the risk of coronary artery lesions 4-fold and 2-3-fold, respectively. Conclusion : The following 3 factors were responsible for increased risk of coronary artery lesions in children with Kawasaki disease: fever duration and days of fever after and before IVIG treatment. To identifythe predictive indicators of coronary complications, it is necessary to further elucidate the relationship between well-known forecasting factors.