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Review Article
Immunology
Immunopathogenesis of COVID-19 and early immunomodulators
Kyung-Yil Lee, Jung-Woo Rhim, Jin-Han Kang
Clin Exp Pediatr. 2020;63(7):239-250.   Published online June 18, 2020
The novel coronavirus disease 2019 (COVID-19) is spreading globally. Although its etiologic agent is discovered as severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), there are many unsolved issues in COVID-19 and other infectious diseases. The causes of different clinical phenotypes and incubation periods among individuals, species specificity, and cytokine storm with lymphopenia as well as the mechanism of damage to organ...
Predictors and management of intravenous immunoglobulin-resistant Kawasaki disease
Min Seob Song
Clin Exp Pediatr. 2019;62(4):119-123.   Published online March 15, 2019
Kawasaki disease (KD) is a systemic vasculitis that mainly affects younger children. Intravenous immunoglobulin (IVIG) resistant cases are at increasing risk for coronary artery complications. The strategy on prediction of potential nonresponders and treatment of IVIG-resistant patients is now controversial. In this review the definition and predictors of IVIG-resistant KD and current evidence to guide management are discussed.
Original Article
Cardiology
Prediction of nonresponsiveness to medium-dose intravenous immunoglobulin (1 g/kg) treatment: an effective and safe schedule of acute treatment for Kawasaki disease
Kyung Pil Moon, Beom Joon Kim, Kyu Jin Lee, Jin Hee Oh, Ji Whan Han, Kyung Yil Lee, Soon Ju Lee
Clin Exp Pediatr. 2016;59(4):178-182.   Published online April 30, 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...

Meta-analysis of factors predicting resistance to intravenous immunoglobulin treatment in patients with Kawasaki disease
Jin-Young Baek, Min Seob Song
Clin Exp Pediatr. 2016;59(2):80-90.   Published online February 29, 2016
Purpose

Studies have been conducted to identify predictive factors of resistance to intravenous immunoglobulin (IVIG) for Kawasaki disease (KD). However, the results are conflicting. This study aimed to identify laboratory factors predictive of resistance to high-dose IVIG for KD by performing meta-analysis of available studies using statistical techniques.

Methods

All relevant scientific publications from 2006 to 2014 were identified through PubMed searches. For...

Predictors of nonresponse to intravenous immunoglobulin therapy in Kawasaki disease
Hyo Min Park, Dong Won Lee, Myung Chul Hyun, Sang Bum Lee
Clin Exp Pediatr. 2013;56(2):75-79.   Published online February 25, 2013
Purpose

It has been reported that 10% to 20% of children with Kawasaki disease (KD) will not respond to intravenous immunoglobulin (IVIG) treatment. In this study, we aimed to identify useful predictors of therapeutic failure in children with KD.

Methods

We examined 309 children diagnosed with KD at the Kyungpook National University Hospital and the Inje University Busan Paik Hospital between January 2005...

The characteristic laboratory findings of non-responsiveness to intravenous immunoglobulin in children with Kawasaki disease
Han Gil Cho, Young Kuk Cho, Jae Sook Ma
Clin Exp Pediatr. 2010;53(2):228-234.   Published online February 15, 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...
Case Report
Change of neutrophil count after treatment of intravenous immunoglobulin in children with idiopathic thrombocytopenic purpura
Jun Young Park, Ji Ae Park, Seong Shik Park, Young Tak Lim
Clin Exp Pediatr. 2008;51(2):204-208.   Published online February 15, 2008
Purpose : The aim of this study was to investigate the incidence and course of neutropenia following intravenous immunoglobulin (IVIG) therapy in children with idiopathic thrombocytopenic purpura (ITP). Methods : From January 2001 to June 2006, fifty-four patients with ITP were enrolled in this study. Forty-two of 54 patients were treated with IVIG, while the other 12 were treated with...
Original Article
Change of absolute neutrophil count after intravenous immunoglobulin administration for the children with idiopathic thrombocytopenic purpura
Hyun Jung Shin, In Kug Bang, Byung Kyu Choe, Jin-Bok Hwang, Jun Sik Kim, Heung Sik Kim
Clin Exp Pediatr. 2007;50(10):982-986.   Published online October 15, 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...
The effects of high-dose intravenous immunoglobulin on plasma protein and lipid levels in the patients with Kawasaki disease
Keun Young Lee, Dong-Un Kim, Hyun Seung Lee, Pil Sang Jang, Young-Hoon Kim, Jin Tack Kim, Hyun Hee Kim, Kyung-Yil Lee, Joon-Sung Lee
Clin Exp Pediatr. 2006;49(12):1348-1353.   Published online December 15, 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...
Case Report
Steroid and enalapril therapy - possible cause of toxic epidermal necrolysis
Dong Wook Kim, Da Eun Jung, Ja Wook Koo
Clin Exp Pediatr. 2006;49(3):332-336.   Published online March 15, 2006
Toxic epidermal necrolysis (TEN) is a rare, acute and life-threatening cutaneous drug reaction. TEN is characterized by the sudden onset of extensive necrosis in the epidermis and frequent mucous membrane involvement. The pathogenesis has not yet been elucidated. In addition, no particular treatment for TEN has been established. We report a case of TEN in a 14-year-old-boy, which might have...
Original Article
The Effectiveness of Intravenous Immunoglobulin for Clinically Suspected Neonatal Sepsis
Hyun Jung Na, Ji Young Kim, Gyeong Hoon Lee, Jun Hwa Lee, Eun Jin Choi, Jin Kyung Kim, Hai Lee Chung, Woo Taek Kim
Clin Exp Pediatr. 2005;48(11):1187-1192.   Published online November 15, 2005
Purpose : The purpose of this study is to determine the effectiveness of intravenous immunoglobuin (IVIG) administration in fullterm neonates having clinically suspected neonatal sepsis. Methods : Forty full-term neonates admitted to the neonatal intensive care unit with clinically suspected neonatal sepsis, who had at least two positive diagnostic criteria were enrolled. Twenty neonates were enrolled into the IVIG arm and...
Inhibition of Vascular Endothelial Growth Factor-induced Endothelial Cell Differentiation by Intravenous Immunoglobulin and Methylprednisolone
Hyoun Ah Choi, Kyung Hwa Ha, Jong Seo Yoon, Yoon Lee, Joon Sung Lee, Ji Whan Han
Clin Exp Pediatr. 2005;48(8):886-893.   Published online August 15, 2005
Purpose : Kawasaki disease is the most common cause of systemic vasculitis in children less than 5 years of age. Recent immunohistochemistry findings suggest that many vascular growth factors play a role in the formation of the coronary artery lesions. Active remodeling of the coronary artery lesions in Kawasaki disease continues in the form of intimal proliferation and neoangiogenesis for...
Statistical Analysis of 1,000 Cases of Kawasaki Disease Patients Diagnosed at a Single Institute
Dae Hwan Hwang, Kyoung Mi Sin, Kyong Min Choi, Jae Young Choi, Jun Hee Sul, Dong Soo Kim
Clin Exp Pediatr. 2005;48(4):416-424.   Published online April 15, 2005
Purpose : To find the risk factors associated with coronory artery lesions, non-responsiveness to intravenous immunoglobulin(IVIG) treatment, and recurrences in Kawasaki disease patients. Methods : We retrospectively analyzed 1,000 Kawasaki disease patients who were admitted to Yonsei University Medical Center from September 1990 to December 2003. We compared between responder and non-responder groups to IVIG treatment as well as between relapsed...
The Effects of Intravenous Immunoglobulin(IVIG) and Methylprednisolone on the mRNAs Expressions of VEGF, VCAM-1 and IL-1β of Human Umbilical Vein Endothelial Cells(HUVEC) Stimulated by IL-1β
Soh Yeon Kim, Sun Jeong Lim, Ji Whan Han, Kyung Yil Lee, Joon Sung Lee
Clin Exp Pediatr. 2004;47(12):1325-1333.   Published online December 15, 2004
Purpose : Kawasaki disease(KD) manifests a systemic vasculitis of unknown etiology in young children. Vascular endothelial growth factor(VEGF), vascular cell adhesion molecule-1(VCAM-1) and interleukin-1 beta(IL-1β) may play important roles in the pathogenesis of KD. Intravenous immunoglobulin(IVIG) and methylprednisolone(MP) are therapeutically effective for KD, however, the precise mechanisms of the two drugs are still unknown. We investigated the therapeutic efficacy of...
Aseptic Meningitis Secondary to High-Dose Intravenous Immunoglobulin Therapy in Kawasaki Disease
Ae Ra Cho, Jee Yoon Park, Kyung Hyo Kim, Young Mi Hong
Clin Exp Pediatr. 2004;47(5):561-566.   Published online May 15, 2004
Purpose : Kawasaki disease is a multisystemic inflammatory vasculitis and associated with neurologic features such as aseptic meningitis. High-dose intravenous immunoglobulin(IVIG) is used to treat Kawasaki disease and generally considered to be safe, but rare cases of aseptic meningitis with unknown etiology have been reported. The aseptic meningitis associated with Kawasaki disease was compared with meningitis as the adverse reaction...
Prediction of Intravenous Immunoglobulin Non-responders in Patients with Kawasaki Disease
Gi Bum Lee, Ji-Won Lee, Kyung-Yil Lee
Clin Exp Pediatr. 2004;47(1):90-94.   Published online January 15, 2004
Purpose : We evaluated the effects of intravenous immunoglobulin(IVIG) on the levels of laboratory indices examined serially according to the responsiveness to IVIG therapy in children with Kawasaki disease(KD). Methods : Children with KD(n=63) who had been treated with IVIG at a dosage of 2.0 g/kg were classified into two groups : the IVIG-resistant(consistent fever over 48 hours after initiation...
Case Report
Arthritis in the Subacute Stage of Kawasaki Disease after Responding to Intravenous Immunoglobulin Treatment
Kyung-Yil Lee, Jin-Hee Oh, Dea-Kyun Koh
Clin Exp Pediatr. 2003;46(11):1124-1127.   Published online November 15, 2003
We evaluated the clinical and laboratory characteristics of five children with Kawasaki disease who had showed arthritis after responding to intravenous immunoglobulin(IVIG) treatment. Age distribution was between 13 months and six years of age(mean 3.2?.6 years). There were two males and three females. Arthritis occurred when acute symptoms were subsiding, with the average onset on day 5.8?.8 after final IVIG...
A Case of Henoch-Schonlein Purpura Nephritis Complicating Encephalopathy Accompanied by Hypertension and Cerebral Vasculitis
Hee Ra Choi, Eo Jin Kim, Myoung Bum Choi, Jae Young Lim, Chan Hoo Park, Hyang Ok Woo, Hee Sang Youn
Clin Exp Pediatr. 2003;46(10):1040-1043.   Published online October 15, 2003
Henoch-Shonlein purpura(HSP) is a systemic small-vessel vasculitis that primarily affects the skin, gastrointestinal tract, joints, and kidneys. The nervous system may be involved, less commonly than other organs. When the central nervous system(CNS) was involved, headache, changes in mental status, seizures, and focal neurologic deficits have been reported. Hypertension, uremic encephalopathy, metabolic abnomalities, electrolyte abnormalities, or cerebral vasculitis were suggested...
Original Article
Safety and Efficacy of Early Treatment with Intravenous Immunoglobulin in Patients with Kawasaki Disease
Hyun Jin Kim, Hae Won Yom, Hae Soon Kim, Sejung Sohn
Clin Exp Pediatr. 2003;46(10):1019-1023.   Published online October 15, 2003
Purpose : To determine the differences in clinical characteristics, blood chemistry and coronary artery complications between patients with Kawasaki disease who received intravenous immunoglobulin (IVIG) within the fourth day of illness and after the fifth day of illness. Methods : A retrospective chart review was conducted of all children with Kawasaki disease who were admitted to Ewha Mokdong Hospital between January...
Alteration of Biochemical Profiles after High-Dose Intravenous Immunoglobulin Administration in Kawasaki Disease
Ji-Won Lee, Kyung-Yil Lee
Clin Exp Pediatr. 2003;46(8):817-820.   Published online August 15, 2003
Purpose : Intravenous immunoglobulin(IVIG) has been used as an immunomodulatory treatment for several immune-mediated diseases. The early effect of high-dose IVIG on biochemical profiles including lipids and proteins was evaluated in patients with Kawasaki disease(KD). Methods : Twelve children with KD(nine boys) were treated with IVIG of 2 g/kg over 12 hours. Serial sera were collected from the patients four times...
Laboratory Values in Patients with Kawasaki Disease after Intravenous Immunoglobulin : Comparison of Patients with Coronary Artery Lesions to those without Coronary Artery Lesions
Min-Young Park, Kyung-Yil Lee, Ji-Whan Han, Hyung-Shin Lee, Ja-Hyun Hong, Kyung-Tai Whang
Clin Exp Pediatr. 2003;46(2):162-166.   Published online February 15, 2003
Purpose : We evaluated the effects of intravenous immunoglobulin(IVIG) on level of laboratory parameters examined serially according to the existence of coronary artery lesions in children with Kawasaki disease. Methods : Children with Kawasaki disease(n=63), treated with IVIG at a dose of 2.0 g/kg, were classified as a group with coronary artery lesions(CALs+ group, n=9) or a group without coronary...
Changes in Mean Platelet Volume and Platelet Distribution Width after Therapy in Childhood Idiopathic Thrombocytopenic Purpura
Jong Tai Kim, Kyung Won Lee, Soon Nam Kim, Moon Kyu Kim
Clin Exp Pediatr. 2002;45(4):505-511.   Published online April 15, 2002
Purpose : Mean platelet volume(MPV) and platelet distribution width(PDW) are useful parameters in evaluating disorders of platelets. In cases with idiopathic thrombocytopenic purpura(ITP), they change as platelet count increases. In this study, we compared the values of MPV and PDW in ITP patients at diagnosis with those of normal children. We also studied whether the early changes in MPV may...
Case Report
A Case of the Use of Intravenous Immunoglobulin of Treatment for HyperIgE Syndrome
Yoon Ji Choi, Dong IL Lee, Hong Bae Kim
Clin Exp Pediatr. 2001;44(11):1301-1304.   Published online November 15, 2001
Hyper-IgE syndrome is a rare congenital immune deficiency disease characterized by severe eczema, recurrent infection of the sinopulmonary tract, cold subcutaneous abscess and high serum IgE levels. It is an autosomal dominant disease with incomplete penetrance. We examined the case of a 12 month old infant with hyperIgE syndrome since neonate. The diagnosis of hyper IgE syndrome was made because...
A Case of Aseptic Meningitis Following Administration of Intravenous Immunoglobulin in A Patient with Idiopathic Thrombocytopenic Purpura
Su Jin Kim, Ji Eun Kim, Hei Won Hwang, Ji Sun Mok, Dong Seok Lee, Doo Kwun Kim, Sung Min Choi, Woo Taek Kim
Clin Exp Pediatr. 2001;44(7):823-826.   Published online July 15, 2001
Intravenous immunoglobulin(IVIG) has been widely used to treat idiopathic thrombocytopenic purpura in childhood. Aseptic meningitis has been reported as a rare complication of IVIG therapy. This report is on an 11 year-old boy with ITP who suffered from aseptic meningitis following the administration of IVIG. He was given 1 g/kg of IVIG for 2 days, and on the fourth day...
A Case of Acute Renal Failure Following Intravenous Immunoglobulin Therapy in a Child
Sung-Wook Park, Kyung-Yil Lee, Kyung-Yil Lee, Kye-Nam Yoon, Sang-Won Cha, Dong-Jun Lee, Ji-Whan Han, Kyung-Tai Whang
Clin Exp Pediatr. 2000;43(5):720-724.   Published online May 15, 2000
Intravenous immunoglobulin(IVIG) therapy is associated with a number of complications, including rare acute renal failure(ARF). Although the cause of IVIG-associated ARF is unknown, it may be related to the stabilizing agent used in IVIG preparations. ARF following IVIG infusion has not been previously described in children. We report a 4-year-old girl with Kawasaki disease who suffered from ARF following IVIG...
Original Article
Immunologic Alterations of Peripheral Mononuclear Cells by Intravenous Immunoglobulin in the Acute Stage of Kawasaki disease
Ji-Whan Han, Kyung-Yil Lee, Kyong-Su Lee
Clin Exp Pediatr. 1998;41(6):813-824.   Published online June 15, 1998
Purpose : High dose intravenous immunoglobulin(IVIG) therapy is effective in reducing the incidence of coronary artery aneurysm in Kawasaki disease(KD) patients, however, the precise mechanisms by which IVIG reverses the immune activation is unknown. Methods : The sera and peripheral mononuclear cells(PBMCs) were obtained from 10 KD patients in the acute stage(24 hrs before) and 24 hrs after one dose of IVIG(2g/kg) treatment. We measured...
The Study of Transplacental Transfer of Immunoglobulin G in Premature Infants
Sung Soo Moon, Yoon Ki Kang, Soo Chul Cho, Jung Soo Kim
Clin Exp Pediatr. 1998;41(6):747-753.   Published online June 15, 1998
Purpose : Premature infants have low serum immunoglobulin G(IgG) levels because IgG is transplacentally acquired, primarily after 32-34 weeks of gestational age. We studied the transplacental transfer of serum IgG in preterm infants. Methods : The IgG levels in the sera were measured by radial immunodiffusion method(Behring nephelometer, Germany). Results : There was a significant difference between IgG concentration and gestational age; the IgG concentration increased...
Clinical Trial of 2g/kg/day of Intravenous Immunoglobulin Single Dose as Compared with 400mg/kg/day of Intravenous Immunoglobulin for 5days in Kawasaki Disease
Seok Hee Oh, Heung Tag Kim, Hae Jin Chech, Kyuchul Choeh
Clin Exp Pediatr. 1997;40(11):1582-1587.   Published online November 15, 1997
Purpose : Since the effect of intravenous immunoglobulin (IVIG) therapy in Kawasaki disease was reported in 1984, the combined therapy of IVIG & oral aspirin has been popular. In early period, the protocol of 400mg/kg/day for 3-5 days of IVIG had been used, but rescently, the protocol of 2g/kg/day single dose has been preferred. So authers performed a clinical study to compare th efficacies...
Immunologic Abnormalitiesin Kawasaki Diseaseand the Effect ofIntravenousImmunoglobulin
Joon Sung Lee, Bin Cho, Sung Hoon Cho
Clin Exp Pediatr. 1996;39(5):691-702.   Published online May 15, 1996
Purpose : It has been suggested that immunologic abnormalities play a role in the pathogenesis of vascular injury in Kawasaki disease and the immunologic abnormalities could be regulated by high-dose intravenous immunoglobulin(IVIG). We investigated the peripheral blood lymphocyte subsets, the serum levels of interleukin-6(IL-6) and tumor necrosis factor-α(TNF-α) and the effect of high-dose IVIG(400 mg/kg/day for 5 days) on these factors during acute stage of...
Effect of Intravenous Immunoglobulin(IVIG) on Renal Function in Very Low Birth Weight Infants
Hwa Young Jang, Haeng Mi Kim
Clin Exp Pediatr. 1996;39(4):475-481.   Published online April 15, 1996
Purpose : This study was conducted to evaluate the effect of IVIG infusion on renal function in VLBW infants. IVIG has been proved quite safe in neonates given prophylactic and therapeutic doses. But nephrotoxicity is not recognized as adverse in IVIG therapy, only several previous adult cases have been noted. Methods : For a total of ten VLBW infants who had...
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