• Clinical and Experimental Pediatrics
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• 제59권7호
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• pp.298-302
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• 2016

Purpose: Recent reports showed that plasma N-terminal pro-brain natriuretic peptide (NT-proBNP) could be a useful biomarker of intravenous immunoglobulin (IVIG) unresponsiveness and coronary artery lesion (CAL) development in Kawasaki disease (KD). The levels of these peptides are critically influenced by age; hence, the normal range and upper limits for infants and children are different. We performed an age-adjusted analysis of plasma NT-proBNP level to validate its clinical use in the diagnosis of KD. Methods: The data of 131 patients with KD were retrospectively analyzed. The patients were divided into 2 groups-group I (high NT-proBNP group) and group II (normal NT-proBNP group)-comprising patients with NT-proBNP concentrations higher and lower than the 95th percentile of the reference value, respectively. We compared the laboratory data, responsiveness to IVIG, and the risk of CAL in both groups. Results: Group I showed significantly higher white blood cell count, absolute neutrophil count, C-reactive protein level, aspartate aminotransferase level, and troponin-I level than group II (P<0.05). The risk of CAL was also significantly higher in group I (odds ratio, 5.78; P=0.012). IVIG unresponsiveness in group I was three times that in group II (odds ratio, 3.35; P= 0.005). Conclusion: Age-adjusted analysis of plasma NT-proBNP level could be helpful in predicting IVIG unresponsiveness and risk of CAL development in patients with KD.

• Childhood Kidney Diseases
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• 제9권2호
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• pp.275-281
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• 2005

저자들은 생체신장이식을 받은 후 발열과 빈혈을 주소로 내원한 2명의 환아에서 혈청 PCR을 통해 PV B19 감염을 진단한 후 면역글로불린 정맥투여로 싱공적으로 치료하고 재발 없이 관찰중인 2례를 경험하였기에 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제59권8호
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• pp.335-340
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• 2016

Purpose: This study aimed to evaluate the clinical course of childhood immune thrombocytopenia (ITP) and to assess the risk factors for developing chronic ITP Methods: The records of 64 children diagnosed with ITP from November 2005 and December 2014 at single center were retrospectively analyzed. Results: The median age at diagnosis and the median platelet count were 1 year (range, 1 month to 15 years) and $9{\times}10^9/L$ (range, $0-84{\times}10^9/L$), respectively. No patient experienced severe bleeding. Nineteen children (29.7%) spontaneously recovered their platelet count to ${\geq}100{\times}10^9/L$ at a median of 10 days. In total 45 patients (70.3%) received intravenous immunoglobulin (IVIG) as first-line therapy, and showed platelet recovery at 1 week. The final diagnosis of 55 (85.9%) and 9 patients (14.1%) was acute and chronic ITP, respectively. Older age, absence of prior infection and insidious onset of symptoms were significantly associated with the development of chronic ITP. Among the patients who received IVIG, those with platelet count <$45{\times}10^9/L$ at 1 month after IVIG showed a significantly higher incidence of chronic ITP compared to those with platelet count ${\geq}45{\times}10^9/L$ (88.8% vs. 44.4%, P<0.01). Conclusion: In most patients, ITP runs a benign course and approximately 86% of them recover within 1 year of their initial diagnosis. The potential impact of the risk factors of chronic ITP on clinical practice needs to be explored and further studies are warranted to determine whether IVIG influences the course of ITP.

• Clinical and Experimental Pediatrics
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• 제60권7호
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• pp.208-215
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• 2017

Purpose: Activation of Toll-like receptor 2 (TLR2) present on circulating monocytes in patients with Kawasaki disease (KD) can lead to the production of proinflammatory cytokines and interleukin-10 (IL-10). We aimed to determine the association of the frequency of circulating TLR2+/ CD14+ monocytes (FTLR2%) with the outcomes of KD, as well as to compare FTLR2% to the usefulness of sIL-10. Methods: The FTLR2% in patients with KD was measured by flow cytometry. Serum levels of IL-10 (sIL-10) were determined in 31 patients with KD before the initial treatment with intravenous immunoglobulin (IVIG) and in 21 febrile controls by using enzyme-linked immunosorbent assay. Patients were classified as having coronary artery lesions (CALs) based on the maximal internal diameters of the proximal right coronary artery and proximal left anterior descending coronary artery one month after the initial diagnosis. Results: We found that FTLR2% greater than 92.62% predicted CALs with 80% sensitivity and 68.4% specificity, whereas FTLR2% more than 94.61% predicted IVIG resistance with 66.7% sensitivity and 71.4% specificity. Moreover, sIL-10 more than 15.52 pg/mL predicted CALs and IVIG resistance with 40% and 66.7% sensitivity, respectively, and 73.7% and 76.2% specificity, respectively. Conclusion: We showed that measuring FTLR2% before the initial treatment could be useful in predicting CAL development with better sensitivity than sIL-10 and with results comparable to sIL-10 results for the prediction of IVIG resistance in patients with KD. However, further studies are necessary to validate FTLR2% as a marker of prognosis and severity of KD.

• 약학회지
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• 제45권6호
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• pp.656-663
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• 2001

The quality of an intravenous immunoglobulin preparation (IVIG) is reflected by the degree of nonspecific activation of complements, the so-called anticomplementary activity (ACA). ACA of aggregates in IVIG was investigated using method by the European Pharmacopoeia and Clq-coated microtiter enzyme-linked immunosorbent assay (ELISA). Both the EP method and the ELISA method showed a dose response curve with the amount of complements bound increasing with the percentage content of aggregates in immunoglobulin standard. The correlation between the two tests was good (r=0.96, r=0.99). However, the correlation was not found when the ACA (EP method) of IVIG product was compared with its aggregate percentage. These results emphasize that the method of aggregate formation affects ACA and that estimation of the percentage distribution of aggregates by HPLC may not reflect ACA. In analysing WIG product for Clq binding activity test with the ELISA, the result by using Protein A-HRP correlated with aggregate percentage (r=0.84). But the correlation decreased (r=0.48) when the result used Protein A-AP(having poorer sensitivity than HRP) was compared with aggregate percentage. As a result, some variation between the two methods, due to differences in assay principles, is to be expected. However, ELISA technique has the advantage in that it is easier to perform, more precise and less subject to reagent variability, and is the more suitable screening method than HPLC analysis.

• Clinical and Experimental Reproductive Medicine
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• 제32권2호
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• pp.165-170
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• 2005

Objective: The aim of present study was to evaluate the effectiveness of low-dose intravenous immunoglobulin (IVIg) therapy in women with recurrent spontaneous abortions (RSA) and elevated pre-conceptional peripheral blood CD56+Natural Killer (NK) cell percentage. Study Design: Retrospective case control study. Materials and Methods: Thirty three women with RSA and elevated pre-conceptional peripheral blood CD56+NK cell percentage who had received low-dose IVIg therapy (400 mg/kg per day, every 4 week, until 20 gestational weeks) were included in this study. Controls were nine women with RSA and elevated pre-conceptional peripheral blood CD56+ Natural Killer (NK) cell percentage who had not received IVIg therapy were included in this study. Medical records of study and control groups were retrospectively analyzed and we compared the successful pregnancy outcomes between two groups. Successful pregnancy outcome was defined as pregnancy ongoing beyond 25 gestational weeks. Results: Age, number of previous abortions, pre-conceptional CD56+NK cell percentage and type of RSA were not statistically different between two groups. Otherwise, twenty-five women who received IVIg therapy (25/33, 75.8%) but, only three women who had not received (3/9, 33.3%) had a successful pregnancy outcome and the rate difference between two groups was statistically significant. Conclusion: Based on our study, low-dose IVIg therapy have a effective role in treatment of RSA patients with elevated pre-conceptional peripheral blood CD56+ Natural Killer (NK) cell percentage, but more larger scaled prospective study is needed for available of conclusive evidence.

• Clinical and Experimental Pediatrics
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• 제46권10호
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• pp.1019-1023
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• 2003

목 적 : 가와사끼병 환아에서 면역글로불린을 조기 투여하였을 때 치료 실패의 빈도와 관상 동맥의 합병증이 증가하는 지를 알아보고자 본 연구를 시행하였다. 방 법 : 2001년 1월부터 2002년 6월 사이에 이화의대 목동병원 소아과에서 가와사끼병으로 치료받은 환아 87명의 입원 및 외래 기록을 후향적으로 조사하였다. 면역글로불린을 발열 4일 이전에 투여받은 환아를 조기투여군(34명), 발열 5일 이후에 투여 받은 환아를 대조군(53명)으로 하였다. 두 군간의 임상양상, 혈액 검사, 발열기간, 치료 및 관상 동맥 합병증을 비교 조사하였다. 결 과 : 1) 두 군간의 인구학적 특징의 차이는 없었다(P>0.05). 2) 총 발열기간은 조기 투여군과 대조군이 각각 $4.8{\pm}2.5$일과 $7.4{\pm}3.0$일로 조기 투여군에서 유의하게 짧았다(P<0.05). 면역글로불린 투여 후 발열 기간과 입원기간은 두 군에서 유의한 차이를 보이지 않았다(P>0.05). 3) 혈색소, 백혈구수, 혈소판수, 혈침속도, CRP, AST, ALT, 알부민은 두 군간에 유의한 차이가 없었다(P>0.05). 4) 면역글로불린 재투여 빈도는 조기 투여군과 대조군에서 각각 11.8%와 5.7%로 유의한 차이를 보이지 않았다(P>0.05). 5) 관상 동맥 합병증은 조기 투여군에서 11.7%와 대조군에서 18.9%로 유의한 차이가 없었다(P>0.05). 6) 가와사끼병의 재발은 각각 3%와 2%로 두 군간에 유의한 차이가 없었다(P>0.05). 결 론 : 가와사끼병에서 면역글로불린 조기투여는 총 발열기간을 감소시키고, 면역글로불린 치료 실패율과 관상 동맥류 발생을 증가시키지 않았다.

• 나누는사람들
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• 통권30호
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• pp.4-7
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• 2005

• 한국임상수의학회지
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• 제30권1호
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• pp.61-65
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• 2013

An American Cocker Spaniel (3-year-old, intact female, 6.0 kg) was referred to the Veterinary Medical Teaching Hospital of Chungnam National University for evaluation of pustules and crusts in the periocular region, dorsal and ventral region of the trunk, and digits. Complete blood count (CBC) revealed leukocytosis with mature neutrophilia, and a serum biochemistry profile revealed hypoalbuminemia. Tape strip tests identified numerous neutrophils and acatholytic cells. Histopathology identified intraepithelial pustules with neutrophils and acantholytic keratinocytes. Definitive diagnosis of pemphigus foliaceus (PF) was made by direct immunofluorescence (DIF) test with goat anti-canine IgG antibody. The human intravenous immunoglobulin (IVIG) was administered at a rate of 15 ml/h over 6 hours for 4 days. After that, the dog was maintained on prednisolone (2.2 mg/kg, PO, SID) and azathioprine (2.0 m/kg, PO, SID). An infusion of IVIG (0.5 g/kg) was repeated 3 days after 4 weeks. After 10 weeks, the dog showed the remarkable regression of lesions.

• Clinical and Experimental Pediatrics
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• 제58권3호
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• pp.84-88
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• 2015

Kawasaki disease (KD) is an acute systemic vasculitis that predominantly affects children, and can result in coronary artery lesions (CAL). A patient with KD who is resistant to treatment with intravenous immunoglobulin (IVIG) has a higher risk of developing CAL. Incomplete KD has increased in prevalence in recent years, and is another risk factor for the development of CAL. Although the pathogenesis of KD remains unclear, there has been increasing evidence for the role of genetic susceptibility to the disease since it was discovered in 1967. We retrospectively reviewed previous genetic research for known susceptibility genes in the pathogenesis of KD, IVIG resistance, and the development of CAL. This review revealed numerous potential susceptibility genes including genetic polymorphisms of ITPKC, CASP3, the transforming growth factor-${\beta}$ signaling pathway, B lymphoid tyrosine kinase, FCGR2A, KCNN2, and other genes, an imbalance of Th17/Treg, and a range of suggested future treatment options. The results of genetic research may improve our understanding of the pathogenesis of KD, and aid in the discovery of new treatment modalities for high-risk patients with KD.