scholarly journals Intravenous Immunoglobulin Treatment in Kawasaki Disease Decreases the Incidence of Myopia

2021 ◽  
Vol 10 (7) ◽  
pp. 1381
Author(s):  
Hun-Ju Yu ◽  
Meng-Ni Chuang ◽  
Chiao-Lun Chu ◽  
Pei-Lin Wu ◽  
Shu-Chen Ho ◽  
...  

Kawasaki disease (KD) is a systemic vasculitis that primarily affects children under the age of 5 years old. The most significant complication is coronary artery lesions, but several ocular manifestations have also been reported. Recently, one study revealed an increasing incidence of myopia among KD patients. Therefore, the aim of this study was to assess the difference in myopic incidence between Kawasaki disease (KD) patients treated with aspirin and intravenous immunoglobulin (IVIG). Materials and methods: We carried out a nationwide retrospective cohort study by analyzing the data of KD patients (ICD-9-CM code 4461) from Taiwan’s National Health Insurance Research Database (NHIRD) during the period of 1996–2013. Results: A total of 14,102 diagnosed KD were found in Taiwan during the study period. After excluded missing data, treatment strategy and age distribution, a total of 1446 KD patients were enrolled for analysis including 53 of which received aspirin (without IVIG) and 1393 of which were treated with IVIG. Patients who had myopia, astigmatism, glaucoma, cataract, etc. prior to their KD diagnosis were excluded. The age range was 0 to 6 years old. According to the cumulative curves, our results demonstrated that the myopic incidence in the IVIG group was significantly lower than the aspirin group (hazard ratio: 0.59, 95% confidence intervals: 0.36~0.96, p = 0.02). Treatment with IVIG for KD patients may have benefit for myopia control. Conclusion: Compared to aspirin, IVIG may decrease the myopic risk in KD patients. However, it needs further investigation including clinical vision survey of myopia due to the limitations of this population-based study.

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Kazuyuki Ikeda ◽  
Tomonaga Ameku ◽  
Yui Nomiya ◽  
Masahiro Nakamura ◽  
Satoshi Matsui ◽  
...  

Introduction: Kawasaki disease (KD) is a systemic vasculitis of unknown origin. Although the treatment of intravenous immunoglobulin (IVIG) significantly resolves inflammation, 10-20% of KD patients have persistent or recurrent fever after the administration of IVIG, and IVIG-resistant patients have a particularly high risk of developing coronary artery abnormalities. Hypothesis: The mechanisms of IVIG-resistant KD have been analyzed using the patients’ leukocyte samples. However, vascular endothelial cells (ECs), closely related to the vasculitis of KD, have not been examined in the previous reports. We propose a hypothesis that ECs are mainly involved in the etiology of IVIG-resistance. Methods: The purpose of this study is to establish new in vitro disease models of vasculitis using induced pluripotent stem cell (iPSC) technology, and clarify the mechanisms of IVIG-resistance in KD. Dermal fibroblasts or T cells from 2 IVIG-resistant and 2 IVIG-responsive KD patients were reprogrammed by episomal vectors encoding Oct3/4, Sox2, Klf4, L-Myc, LIN28, and p53 shRNA. The iPSC lines were then differentiated into ECs by using a previously-reported differentiation method, and the EC samples were subjected to the microarray analyses. Results: The KD patient-derived iPSCs could be differentiated into ECs. The gene expression profiles were compared between iPS-derived ECs (iPS-ECs) generated from IVIG-resistant and IVIG-responsive KD patients. We found that 107 genes were at least two fold up-regulated and 101 genes were at least two fold down-regulated in iPS-ECs from IVIG-resistant KD patients compared with those from IVIG-responsive patients. The Principle Component Analysis (PCA) was performed, but the gene expression levels showed no significant differences between the groups. The Gene Set Enrichment Analysis (GSEA) revealed that the gene sets related to IL-6, NRAS (a member of the RAS oncogene family) and breast cancer were up-regulated in iPS-ECs from IVIG-resistant KD patients. Conclusions: Taking into account that the concentration of IL-6 has been reported to be elevated in acute phase of IVIG-resistant KD, our results suggest that the up-regulation of IL-6 related genes in ECs might be involved in the pathogenesis of IVIG-resistant KD.


Author(s):  
Karoline Rossi ◽  
Danilo José Silva Moreira ◽  
Juliana Brito da Fonseca ◽  
Suzana dos Santos Vasconcelos ◽  
Vinicius Faustino Lima de Oliveira ◽  
...  

Kawasaki disease (KD) or Mucocutaneous Lymph node Syndrome is a systemic vasculitis, which mainly affects children under five years of age with Asian descent, but can also reach other age groups, as well as any other breed. The clinical picture of KD has three stages: acute febrile stage, in which conjunctival congestion, oral mucositis, erythema, flaking, polymorphic rash and laterocervical lymphadenopathy, appear as main symptoms; the subacute stage, which occurs at the end of fever, and leads to the appearance of skin flaking in the limbs, arthritis, arthralgia and thrombocytosis and finally the stage of convalescence that arises when symptoms are almost dissipating and continues until their normalization. The most used treatment occurs from the administration of intravenous immunoglobulin, which for better prognosis of the pathology should be initiated early.


2018 ◽  
Vol 24 (9_suppl) ◽  
pp. 163S-170S
Author(s):  
Wen-Ya Lin ◽  
Jiaan-Der Wang ◽  
Yu-Tse Tsan ◽  
Wei-Cheng Chan ◽  
Kwok-Man Tong ◽  
...  

Recurrent hemarthrosis in patients with hemophilia (PWH) results in chronic arthropathy requiring total joint replacement (TJR). This study aimed to compare the difference in TJR rate between patients with hemophilia A (HA) and hemophilia B (HB). A final total of 935 PWH (782 HA and 153 HB) without inhibitors were collected from the Taiwan’s National Health Insurance Research Database between 1997 and 2013. Demographics, clinical characteristics, and TJR rate were compared between the 2 groups. The annual use of clotting factor concentrate was not different between HA and HB groups ( P = .116). The rate of comorbidities except for 29 PWH having HIV who were all in the HA group was also not different between the 2 groups. A total of 99 (10.6%) PWH had undergone 142 TJR procedures during the study period. All of them had received on-demand therapy. No difference was found in the cumulative incidence of TJR between HA and HB ( P = .787). After adjusting for various confounders including age, pyogenic arthritis, and HIV infection, no increased risk of TJR was found in patients with HA versus Patients with HB (hazard ratio: 0.92, 95% confidence interval 0.54-1.58). This finding suggests that the rate of TJR between patients with HA and HB is not significantly different.


2021 ◽  
Vol 9 ◽  
Author(s):  
Hsiao-Wen Chu ◽  
Chien-Heng Lin ◽  
Ming-Chih Lin ◽  
Ya-Chi Hsu

Introduction: Genetic susceptibility and immune dysregulation play important roles in the pathogenesis of Kawasaki disease (KD). However, it is still unclear whether KD causes immune disorder later in life or whether inherited susceptibility to immune disorders causes KD. The aim of this study was to elucidate whether inherited immune disease properties from mothers increase the risk of KD from a population-based perspective.Method: Taiwan's National Health Insurance Research Database was the main data source in this study. Parents and children were linked using the Taiwan Maternal and Child Health Database. Patients diagnosed with KD and younger than 18 years from 2004 to 2015 were enrolled as the study population. The control group was randomly selected from individuals without the diagnosis of KD matched by age, index year, sex, and urbanization level at a ratio of 1 to 10. The prevalence of maternal autoimmune and allergic diseases was compared between groups.Results: In total, 7,178 children were found to have been diagnosed with Kawasaki disease. Then 71,780 children matched by index year, gender, and urbanization were randomly selected to serve as the control group. Children born from mothers with asthma and allergic rhinitis had a higher risk of developing KD. Children of mothers with an autoimmune disorder had a significantly increased tendency to develop KD. Maternal numbers of autoimmune disorders showed a dose-dependent relationship with KD incidence.Conclusion: This is the first population-based study to investigate maternal immune diseases and the risk of KD in their children. Children of mothers with immune disorders tend to have a higher risk of KD.


PeerJ ◽  
2018 ◽  
Vol 6 ◽  
pp. e4539 ◽  
Author(s):  
Wei-Te Lei ◽  
Chien-Yu Lin ◽  
Yu-Hsuan Kao ◽  
Cheng-Hung Lee ◽  
Chao-Hsu Lin ◽  
...  

Background Kawasaki disease (KD) is an immune-mediated systemic vasculitis, and infection plays an important role in the pathophysiology of KD. The susceptibility to infectious disease in patients with KD remains largely unclear. This study aimed to investigate the risk of respiratory tract infection (RTI)-related hospitalizations in children with KD. Methods Data from the Taiwanese National Health Insurance Research Database was analyzed. We excluded patients with history of congenital abnormality, allergic diseases, or hospitalization history. Children with KD were selected as KD group and age- and sex-matched non-KD patients were selected as control group with 1:4 ratio. Both cohorts were tracked for one year to investigate the incidences of RTI-related hospitalizations. Cox regression hazard model was used to adjust for confounding factors and calculate the adjusted hazard ratio (aHR). Results Between January 1996 and December 2012, 4,973 patients with KD were identified as the KD group and 19,683 patients were enrolled as the control group. An obviously reduced risk of RTI-related hospitalizations was observed in KD patients (aHR: 0.75, 95% CI [0.66–0.85]). The decreased risk persisted through the first six-months follow-up period with a peak protection in 3–6 months (aHR: 0.49, 95% CI [0.37–0.64]). Conclusions KD patients had approximately half reduction of risk for RTI-related hospitalizations. The protective effects persisted for at least six months. Further studies are warranted to elucidate the entire mechanism and investigate the influences of intravenous immunoglobulin.


2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Mei-Chen Ou-Yang ◽  
Ho-Chang Kuo ◽  
I-Chun Lin ◽  
Jiunn-Ming Sheen ◽  
Fu-Chen Huang ◽  
...  

Kawasaki disease (KD) is an acute febrile vasculitic syndrome of early childhood often complicated by coronary artery lesion that drastically reduces the quality of life. The study aimed to identify a reliable marker for predicting nonresponsiveness to the first course of intravenous immunoglobulin (IVIG) in KD patients. A total of 63 patients with KD were enrolled in the study (IVIG response, 58; IVIG resistance, 5). Plasma samples were collected before and after IVIG infusion for measurement of biomarkers. Patients’ clinical characteristics and laboratory data were also analyzed. A receiver operating characteristic curve was generated to identify a cut-off value for predicting IVIG resistance. Among the biomarkers, the difference in plasma clusterin concentrations before and after IVIG infusion (CLUSTER 12) was significantly related to IVIG resistance (P=0.040; 95% confidence interval (CI): −25.8% to −6.0%). Using a CLUSTER 12 cut-off value of <8.52 mg/L, the odds ratio for IVIG resistance was 11.467 (95% CI: 1.186 to 110.853). Patients with plasma CLUSTER 12 concentrations >8.52 mg/L had a much higher risk of IVIG resistance than those with CLUSTER 12 concentrations <8.52 mg/L. Plasma clusterin concentration shows promise as a candidate biomarker for predicting IVIG resistance in patients with KD.


2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Wei-Pin Chang ◽  
Shyh-Jong Wu ◽  
Wei-Chiao Chang ◽  
Ho-Chang Kuo

Background.It is unclear if the prevalence of Kawasaki disease (KD) correlates with the degree of urbanization. We hypothesized that the prevalence of KD is more pronounced in urban versus rural environments.Methods.The National Health Insurance (NHI) program was implemented in Taiwan in 1995 and covers most of the population (>99%). We used the NHI database to investigate the epidemiological features of KD. A total of 115 diagnosed patients with KD from 1997 to 2010 were included, together with 1,150 matched controls without KD. Chi-square analyses were performed to investigate the difference between modern city and rural environments.Results.Of the 1265 sampled subjects (claims data from 1,000,000 random subjects), the mean age of the KD study group and control group was 2.08 ± 1.66 and 2.08 ± 1.64 years, respectively. After matching for age, sex, and same index date, no statistically significant differences in urbanization level and geographical location of the patients' residence were observed.Conclusion.Urbanization did not appear to be an important effect modifier of Kawasaki disease in Taiwan.


2021 ◽  
Vol 12 ◽  
Author(s):  
Dong Liu ◽  
Meixuan Song ◽  
Fengchuan Jing ◽  
Bin Liu ◽  
Qijian Yi

Kawasaki disease (KD) is a systemic vasculitis that predominantly damages medium- and small-sized vessels, and mainly causes coronary artery lesions (CALs). The diagnostic criterion of KD mainly depends on clinical features, so children could be easily misdiagnosed and could suffer from CALs. Through analysis, a total of 14 immune-related DEGs were obtained, of which IL1B, ADM, PDGFC, and TGFA were identified as diagnostic markers of KD. Compared with the non-KD group, KD patients contained a higher proportion of naive B cells, activated memory CD4 T cells, gamma delta T cells, and neutrophils, while the proportions of memory B cells, CD8 T cells, activated memory CD4 T cells, and activated NK cells were relatively lower. In conclusion, immune-related genes can be used as diagnostic markers of KD, and the difference in immune cells between KD and non-KD might provide new insight into understanding the pathogenesis of KD.


2021 ◽  
Author(s):  
Yusuf Ziya Varlı ◽  
Ahmet Okay Caglayan ◽  
Kaya Bilguvar ◽  
Murat Gunel ◽  
Kazim Oztarhan

Abstract Objective Kawasaki disease (KD) is an acute febrile systemic vasculitis and the most common cause of coronary artery aneurysm (CAA) in children. Intravenous immunoglobulin (IVIG) therapy is used to prevent fever and systemic inflammation. However, IVIG resistance is the most important risk factor of morbidity and mortality. It has been identified several single nucleotide polymorphisms (SNPs) related to IVIG resistance and this research aims to analyze these polymorphisms in our study population. Methods Patients diagnosed with KD (n:259) were analyzed retrospectively. Blood samples were taken from a randomized subgroup (n:97). Previously reported IVIG resistance related exonic SNPs at five different gene loci (IL16, TNFSF14, NFATC2, DERL3, SAMD9L) were evaluated by whole exome sequencing (WES). Results Between 2010–2019, 259 patients (male/female: 1,67) with KD were submitted to our clinic. CAA and IVIG resistance rates were 11.6% and 21.6%, respectively. The risk of developing CAA was significantly increased in patients with IVIG resistance (p < 0.001). As a result, IVIG resistance frequency increased in the presence of three SNPs. These are "rs11556218"(p.Asn1147Lys), "rs344560"(p.Lys214Glu), "rs12479626"(p.His446Arg), and are located in IL16, TNFSF14, NFATC2 genes, respectively. Conclusions Until now, KD-related genetic data mostly obtained from studies involving large cohorts from Northeast Asian countries. In the analysis of this largest Turkish cohort in the literature, we found that, similar with previous studies, the IL16 gene may be plays important role in the IVIG resistance mechanism.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Weiming Cheng ◽  
Shu-Yi Lin ◽  
Yu-Hua Fan ◽  
Sheng-Wen Chen

AbstractSurgery is traditionally a male-dominated field, and gender differences exist despite the growing numbers of female surgeons. A handful of studies have evaluated the condition in Asian societies. We aimed to examine the difference between female and male surgeons in urology, general surgery, and gynecology by analyzing a nationwide, population-based database. We identified surgeons with a clinical experience of six to thirteen years between 1995 to 2013 from the National Health Insurance Research Database. We collected patient numbers and revenue per month in outpatient and inpatient care, as well as monthly numbers of surgeries conducted by female and male surgeons in urology, general surgery, and gynecology, for analysis. Original student’s t-test and wilcoxon rank sum test was used to compare the differences between female and male surgeons, and p values less than 0.05 were considered statistically significant. Female urologists and general surgeons had a significantly higher ratio of female patients in Taiwan. Female urologists had patient numbers, revenues, and numbers of surgeries comparable to male urologists. In contrast, female general surgeons had significantly less involvement in outpatient and inpatient care and had low monthly revenues. Female general surgeons contradictorily performed more oncological surgeries per month than males. However, the difference in numbers of oncological surgeries was not significant after excluding breast cancer surgeries. Female gynecologists had a similar amount of outpatients and outpatient revenue but significantly less inpatient care and numbers of surgeries per month. A gender-based gap exists among surgeons in Taiwan. The gap between females and males appeared narrower in urology than in general surgery and gynecology. Management of diseases related to female sex organs, including breast, were more common among female surgeons. Efforts should be made to decrease gender stereotypes, to ensure that patients receive the best care regardless of the sex of the surgeons.


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