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Case Report
A Case of Systemic Castleman's Disease in a Child and Successful Treatment with Oral Prednisolone
So Eun Koo, Mee Jeong Lee, Jeong Eun Kim, Joo Ryung Huh, Thad Ghim
Clin Exp Pediatr. 2005;48(4):443-447.   Published online April 15, 2005
Castleman's disease is a rare non-neoplastic lymphoproliferative disorder of unknown etiology. It is divided into three histologic subtypes; hyaline-vascular(HV), plasma cell(PC) type and mixed type (HV-PC). It has two clinical expressions. The localized form, which presents as a slow growing mass, has a relatively benign clinical course. The multicentric form is multilocated and holds significant morbidity. The mainstay of treatment...
Original Article
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...
Case Report
A Case of Mycoplasma pneumoniae Pneumonia Complicated with Guillain-Barr Syndrome and Encephalitis
Soon Bum Lee, Hee Jung, Yong Seok Lee, Bum Sun Kwon, Jeesuk Yu
Clin Exp Pediatr. 2004;47(12):1338-1348.   Published online December 15, 2004
The most common pathogen of respiratory tract infection among school-age children and adolescents is Mycoplasma pneumoniae, which causes clinical manifestations of pneumonia, acute asthmatic attack, pharygitis, and tonsilitis. It can also cause extrapulmonary infections that involves skin, the nervous system, the digestive system, the cardiovascular system, and the hematopoietic system. It is reported that the central nervous system symptoms may...
Original Article
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...
Echocardiographic Evaluation of Left Ventricular Function in Kawasaki Disease : Effect of Immune Globulin
Mi-Jin Jung
Clin Exp Pediatr. 2004;47(6):665-671.   Published online June 15, 2004
Purpose : The purpose of this investigation was to identify the impaired myocardial function and improvement with immune globulin in Kawasaki disease. Methods : The myocardial performance and diastolic function were evaluated in 52 patients. M-mode and Doppler echocardiography were performed before(pre) and after immune globulin treatment : two to nine days(pre), five to 15 days(period one), 14 to 35...
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...
Manifestation of Coronary Artery Lesions after Immunoglobulin Re-treatment in Initial Immunoglobulin-resistant Kawasaki Disease
Hyo Jung Suk, In Sung Kim, Jo Won Jung
Clin Exp Pediatr. 2004;47(4):424-429.   Published online April 15, 2004
Purpose : To evaluate the predictable factors for why initial intravenous immune globulin(IVIG) therapy failed and the outcome of coronary lesions after additional IVIG retreated in initial IVIG- resistant Kawasaki disease(KD). Methods : Retrospective studies were performed on 284 cases of KD treated with one episode of high-dose IVIG and 63 cases with additional IVIG retreatment at this hospital from January...
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...
Urinary Excretion of Various Urinary Proteins in Children with Vesicoureteral Reflux
Da Eun Jung, Ja Wook Koo
Clin Exp Pediatr. 2003;46(10):977-982.   Published online October 15, 2003
Purpose : This study aimed to examine the excretion of various urinary proteins in children with a history of urinary tract infection(UTI), with or without vesicoureteral reflux(VUR) or reflux nephropathy, and to identify means of predicting the severity of VUR or the presence of reflux nephropathy as indicated by these markers, and to know how these markers are changed after...
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...
Comparison of Effectiveness between Two Different Protocols of Treatment of IV γ-globulin in Idiopathic Thrombocytopenic Purpura
Jae Ho Jang, Moonsouk Lee, Mira Park, Kyuchul Choeh
Clin Exp Pediatr. 2003;46(4):358-362.   Published online April 15, 2003
Purpose : Several methods of IV γ-globulin(IVG) infusion are effective in the treatment of autoimmune disease, including idiopathic thrombocytopenic purpura(ITP). But it is not known which method is more effective in the treatment of ITP. The effectiveness of these two methods of IVG infusion was studied in terms of platelet recovery rate, side effects and recurrence rate. Methods : Forty seven...
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...
Clinical Characteristics in Patients with Kawasaki Disease Who Received Intravenous Gamma-globulin Retreatment
Deok-Soo Kim, Yoon-Soo Hahn, Heon-Seok Han
Clin Exp Pediatr. 2002;45(11):1411-1416.   Published online November 15, 2002
Purpose : Although the use of intravenous gamma-globulin(IVGG) in Kawasaki disease(KD) is effective in reducing clinical symptoms and coronary artery complications, 20-30% of patients have persistent or recrudescent fever and ongoing clinical symptoms. In these patients, the additional infusion of IVGG is considered. The authors studied the characteristics of patients who received IVGG retreatment, and compared them with the patients...
High-dose Intravenous Immune Globulin Retreatment in Kawasaki Disease
So Yun Shim, Mi Young Heo, Hae Soon Kim, Sejung Sonh
Clin Exp Pediatr. 2002;45(10):1273-1277.   Published online October 15, 2002
Purpose : To determine clinical features, laboratory findings and cardiac abnormalities of highdose immune globulin(IVIG) retreatment in patients with Kawasaki disease, and to report effectiveness of retreatment. Methods : Retrospective study of 174 children diagnosed with Kawasaki disease at Ewha Mokdong hospital from March, 1999 to July, 2001. Results : Twenty(11.5%) of 174 patients were retreated with high-dose IVIG. After this, only two patients(1.1%) did not...
Case Report
A Case of Thyroxine Binding Globulin Deficiency with Hypothyroidism
Dong-Chul Lee, Sun-Hee Lee, Jae-Hong Yu
Clin Exp Pediatr. 2002;45(6):796-799.   Published online June 15, 2002
A child diagnosed with congenital hypothyroidism after newborn screening and follow up thyroid function test at 1 month of life in another general hospital demonstrated euthyroid state with thyroxine(T4) supplementation until the age of 22 months of life, when he was transferred to our hospital, where he was diagnosed as thyroxine binding globulin(TBG) deficiency with low T4 and TBG. Withdrawal...
Original Article
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...
Characterization of Mutations in Bruton's Tyrosine Kinase(Btk) Gene from Unrelated 3 X-linked Agammaglobulinemia(XLA) Families in Korea
Chang-Hwa Song, Eun-Kyeong Jo, Jeong-Kyu Park, Jung-Soo Kim, Soo-Jong Hong, Jae-Ho Lee
Clin Exp Pediatr. 2002;45(3):302-310.   Published online March 15, 2002
Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells(PBMC) from three XLA families in Korea. Methods : Heparinized venous blood samples were collected from four XLA patients...
Characterization of Bruton's Tyrosine Kinase Genetic Mutations in One Korean X-linked Agammaglobulinemia Family
Eun-Kyeong Jo, Chang-Hwa Song, Jeong-Kyu Park, Young-Jong Baek, Hye-Young Rhu, Jae-Ho Lee, Tai-Ju Hwang, Hoon Kook
Clin Exp Pediatr. 2002;45(2):183-191.   Published online February 15, 2002
Purpose : X-linked agammaglobulinemia(XLA) is an immunodeficiency caused by abnormalities in Bruton's tyrosine kinase(Btk), and is characterized by a deficiency of peripheral blood B cells. We studied the cytoplasmic expression of Btk protein and analyzed the Btk gene in peripheral blood mononuclear cells from two siblings and one cousin with XLA, as well as additional family members. Methods : Btk...
Protein and Genetic Analysis of Bruton's Tyrosine Kinase(Btk) in Three Korean X-linked Agammaglobulinemia(XLA) Families
Eun-Kyeong Jo, Chang-Hwa Song, Jeong-Kyu Park, Oh Kyung Lee, Dong-Soo Kim
Clin Exp Pediatr. 2002;45(1):44-54.   Published online January 15, 2002
Purpose : Mutations in the Bruton' s tyrosine kinase(Btk) gene are responsible for X-linked agammaglobulinemia( XLA), an immunodeficiency caused by a block in B cell differentiation. In this report we characterize the protein expression and genetic mutations of Btk in four Korean patients with three unrelated XLA families. Methods : The resulting Btk proteins were characterized by a flow cytometry and the mutations were analyzed...
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...
Identification of a Novel Mutation of Bruton`s Tyrosine Kinase(BTK) Gene in a X-linked Agammaglobulinemia(XLA) Family
Young-Jong Baek, Jae-Ho Lee, Jung-Soo Kim, Chang-Hwa Song, Jeong-Kyu Park, Hwa-Jung Kim, Eun-Kyeong Jo
Clin Exp Pediatr. 2000;43(12):1599-1607.   Published online December 15, 2000
X-linked agammaglobulinemia(XLA) is a heritable humoral immunodeficiency disease characterized by inefficient expansion of pre-B cells into later B cell stages or incomplete differentiation of B cell precursors to pre-B cells. The gene mutated in XLA was identified as a cytoplasmic tyrosine kinase, named Bruton`s tyrosine kinase(BTK). In this report we investigated the characteristics of immune cells, the patterns of intracellular...
Original Article
Intravenous Gamma-globulin Retreatment in Kawasaki Disease
Young Ah Lee, Hyun Kee Chung
Clin Exp Pediatr. 2000;43(11):1488-1494.   Published online November 15, 2000
Purpose : This study was designed to determine the outcome and safety of intravenous gamma- globulin(IVGG) retreatment in Kawasaki disease. Methods : A clinical observation of the therapeutic effects, laboratory findings and echocardiograms was carried out on 72 patients with Kawasaki disease in Kosin University Hospital from 1991 to 1999. 27 patients were treated with 1g/kg/day IVGG for 2 days,...
Selective Gamma Globulin Treatment in Kawasaki Disease : The Comparison between Single 1g/kg and 2g/kg
Su Mi Ihn, Ren Zhe Ann, Hong Ryang Kil, Young Hun Chung
Clin Exp Pediatr. 2000;43(7):920-925.   Published online July 15, 2000
Purpose : Treatment of Kawasaki disease with intravenous gamma globulin(IVGG), together with aspirin, has been demonstrated to be safe and effective in preventing coronary artery lesion and systemic inflammation, but optimal IVGG dosage and administration method are still controversial. We compared the therapeutic efficacy and clinical response of single IVGG 1g/kg to that of IVGG 2g/kg for comparable risk group of Kawasaki disease. Methods :...
Case Report
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
Outcome of Cardiac Complication in Patients of Kawasaki Disease Treated with High-dose Gammaglobulin
Hyang-Sook Kim, Jong-Tae Kim, Ji-Yon Lee, Jo-Won Jung, Ki-Soo Pai
Clin Exp Pediatr. 2000;43(5):658-666.   Published online May 15, 2000
Purpose : We performed the study to evaluate the risk factors associated with cardiac complication and the outcome of coronary lesions after high-dose(2g/kg, 1dose) gammaglobulin(IVIG) treatment in patients with Kawasaki disease(KD). Methods : Retrospective studies were performed on 338 cases of KD treated with high-dose IVIG at this hospital from May 1994 to March 1999. Results : Among 338 patients,...
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...
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