scholarly journals High-density genotyping of immune loci in Kawasaki disease and IVIG treatment response in European-American case–parent trio study

2014 ◽  
Vol 15 (8) ◽  
pp. 534-542 ◽  
Author(s):  
A Shendre ◽  
H W Wiener ◽  
D Zhi ◽  
A I Vazquez ◽  
M A Portman ◽  
...  
2013 ◽  
Vol 11 (1) ◽  
Author(s):  
Michael A Portman ◽  
Howard W Wiener ◽  
Miriam Silva ◽  
Aditi Shendre ◽  
Sadeep Shrestha

Children ◽  
2021 ◽  
Vol 8 (5) ◽  
pp. 317
Author(s):  
Ling-Sai Chang ◽  
Ken-Pen Weng ◽  
Jia-Huei Yan ◽  
Wan-Shan Lo ◽  
Mindy Ming-Huey Guo ◽  
...  

(1) Background: Desquamation is a common characteristic of Kawasaki disease (KD). In this study, we analyzed patients’ varying desquamation levels in their hands or feet, in correlation with clinical presentation, to assess the relationship. (2) Methods: We retrospectively reviewed children with KD. We analyzed their age, laboratory data before intravenous immunoglobulin (IVIG) treatment and coronary artery abnormalities (CAA) based on the desquamation level of their hands and feet. We classified the desquamation level from 0 to 3 and defined high-grade desquamation as grade 2 and 3. (3) Results: We enrolled a total 112 patients in the study. We found the hands’ high-grade desquamation was positively associated with age and segmented neutrophil percentage (p = 0.047 and 0.029, respectively) but negatively associated with lymphocyte and monocyte percentage (p = 0.03 and 0.006, respectively). Meanwhile, the feet’s high-grade desquamation was positively associated with total white blood cell counts (p = 0.033). Furthermore, we found that high-grade hand desquamation had less probability of CAA formation compared with that of a low grade (7.1% vs. 40.8%, p = 0.016). (4) Conclusions: This report is the first to demonstrate that the desquamation level of hands or feet in KD is associated with different coronary artery abnormalities and laboratory findings.


2018 ◽  
Vol 104 (7) ◽  
pp. 640-646 ◽  
Author(s):  
Robert M R Tulloh ◽  
Richard Mayon-White ◽  
Anthony Harnden ◽  
Athimalaipet V Ramanan ◽  
E Jane Tizard ◽  
...  

ObjectiveKawasaki disease (KD) is an increasingly common vasculitis with risk of coronary artery aneurysms (CAAs). The last UK survey was in 1990, whereas current epidemiology, treatment patterns and complication rates are unknown. The aim of this study was to address this knowledge gap.MethodsA British Paediatric Surveillance Unit survey in the UK and Ireland from 1 January 2013 to 28 February 2015 ascertained demographics, ethnicity, seasonal incidence, treatment and complication rates.Results553 cases were notified: 389 had complete KD, 46 had atypical KD and 116 had incomplete KD; 2 were diagnosed at postmortem with an incidence of 4.55/100 000 children under 5 years, with a male to female ratio of 1.5:1 and a median age of 2.7 years (2.5 months–15 years). Presentation was highest in January and in rural areas. Most were white (64%), and Chinese and Japanese Asians were over-represented as were black African or African mixed-race children. 94% received intravenous immunoglobulin (IVIG). The overall CAA rate was 19%, and all-cardiac complications affected 28%. Those with CAA received IVIG later than in those without (median 10 days vs 7 days). Those under 1 year had fewer symptoms, but the highest CAA rate (39%). Overall 8 of 512 cases (1.6%) had giant CAA, and 4 of 86 cases (5%) under 1 year of age developed giant CAA. Mortality from KD was 0.36%.ConclusionsThe UK and Ireland incidence of KD has increased and is more frequently seen in winter and rural areas. Delayed IVIG treatment is associated with CAA, suggesting earlier and adjunctive primary treatment might reduce complications to prevent CAA, particularly in the very young.


Rheumatology ◽  
2018 ◽  
Vol 58 (5) ◽  
pp. 770-775 ◽  
Author(s):  
Jong Gyun Ahn ◽  
Yoonsun Bae ◽  
Dongjik Shin ◽  
Jiho Nam ◽  
Kyu Yeun Kim ◽  
...  

Abstract Objectives Kawasaki disease (KD) is an acute systemic vasculitis of unknown aetiology that affects infants and young children. Recent reports of elevated serum high mobility group box 1 (HMGB1) level during the acute phase of KD and its relationship to poor response to IVIG treatment suggest a possible association of HMGB1 polymorphisms with KD. We investigated the association between the polymorphisms of the HMGB1 gene, KD susceptibility, coronary artery lesions, and KD response to IVIG treatment. Methods Whole genome sequencing of the HMGB1 gene was performed to identify causative variants. Two tagging single nucleotide polymorphisms of the HMGB1 gene were selected using linkage disequilibrium analysis. The tagging single nucleotide polymorphisms were genotyped using the TaqMan Allelic Discrimination assay in a total of 468 subjects (265 KD patients and 203 controls). Results The HMGB1 single nucleotide polymorphisms were not associated with KD susceptibility. However, in KD patients, there was a significant association of rs1412125 with coronary artery lesions formation in the recessive model (GG vs AA + GA: odds ratio = 4.98, 95% CI = 1.69–14.66, P = 0.005). In addition, rs1412125 was associated with IVIG resistance in the recessive (GG vs AA + GA: odds ratio = 4.11, 95% CI = 1.38–12.23, P = 0.017) and allelic models (G vs A: odds ratio = 1.80, 95% CI = 1.06–3.06, P = 0.027). Conclusion The rs1412125 in HMGB1 might be a risk factor for the development of coronary artery lesions and IVIG resistance in KD patients.


2020 ◽  
Author(s):  
I Hsin Tai ◽  
Pei-Lin Wu ◽  
Mindy Ming-Huey Guo ◽  
Jessica Lee ◽  
Chi-Hsiang Chu ◽  
...  

Abstract Background: Kawasaki Disease (KD) is considered a major acquired heart disease in children under the age of 5. Coronary artery lesions (CAL) can occur in serious cases despite extreme therapy efforts. Previous studies have reported low serum albumin level was associated with disease outcome, but no further investigation was addressed yet. Method: This retrospective (case-control) study randomly included children with KD who were admitted and underwent laboratory tests before undergoing IVIG treatment in this institution, the largest tertiary medical center in southern Taiwan from 2012-2016. PNI, an albumin-based formula product, was evaluated as a predictor of CAL the first time. The progression of CAL was monitored using serial echocardiography for six months. We performed multivariable logistic regression analysis on the laboratory test and PNI with the disease outcome of the KD patients. Result: Of the 284 children, 158 had CAL, including transient dilatation, while the other 126 did not develop CAL during the 6-month follow-up period. A multivariate logistic regression model revealed that PNI and platelet count are significant predictors of CAL with a 95% confidence interval estimator of 2.532 (1.394-4.599) and1.004 (1.002-1.006), respectively. Using PNI to predict CAL presence gave an area under the receiver-operating-characteristics (ROC) curve of 0.596, and the PNI cut-off point is taken as 55.24, with a sensitivity of 0.509 and specificity of 0.678. Conclusion: This is the first study to demonstrate that PNI, an albumin-based formula product, is a useful index with clearly cut-off value for predicting CAL formation prior to initial IVIG therapy and thus warn clinicians to adopt aggressive therapeutic and coronary arteries imaging surveillance strategies before CAL can develop.


Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
lijian xie ◽  
Cuizhen Zhou ◽  
Renjian Wang ◽  
Tingting Xiao ◽  
Jie Shen ◽  
...  

Introduction: The incidence of Kawasaki disease (KD) in China is increasing for years. The current coronary artery lesion (CAL) incidence is 5-10% in KD with intravenous immunoglobulin (IVIG) treatment. And the 10-20% KD patients still exhibit IVIG resistance. However, little clinical evidence on the occurrence of either CAL or IVIG resistance for big KD sample study in China during the past decade. Objective: In order to find clinical risk factors of CAL and IVIG resistance of KD in China. Methods: We retrospectively analyzed the clinical manifestations, laboratory results, treatment and complications of cardiac vascular of 602 KD cases from 2007 to 2012 admitted at Shanghai Children’s Hospital. The SAS 9.2 edition was used for statistical analysis. The mean ± standard deviation or the median were used for measurements. Case numbers and percentages were used for the count number. The t-test and the Mann-Whitney test were both used for mean comparisons. Single factor and multi-factor logistic regression analyses were used to analyze the risk factors. Results: 1. The KD gender male to female ratio was 1.85: 1. The KD median age was 2.0 years old (one month to 11.7 years old). 20.1% cases (121 of 602) exhibited CAL. There was no difference of CAL incidence between the gender (p=0.09). 2. The incidence of bright red cracked lips (p=0.001), peeling of the skin of the toes (p=0.021) and perianal skin peeling (p=0.031) are less in group with CAL. 3. Among the 602 cases, there were 525 cases that were sensitive to IVIG therapy. 100 of those cases had CAL with an incidence of 19.1%. Among the 26 IVIG resistance cases, there were 9 cases with CAL with an incidence of 34.6%, which was higher than the IVIG sensitive group (p=0.05). 4. ESR (p=0.014), CRP (p=0.017), PLT (p=0.003) and Hb (p=0.032) were much higher in the IVIG resistance group than the IVIG sensitive group, even though the IVIG resistance group started the IVIG treatment earlier (p=0.003). 5. Logistic regression analysis was conducted to show that GPT≥80IU/L was the independent risk factor of IVIG resistance, risk ratio was 2.945 (p=0.012) . Conclusion: This research suggests that risk factors of clinical evidence for IVIG resistance and CAL in KD.


2013 ◽  
Vol 23 (9) ◽  
pp. 455-462 ◽  
Author(s):  
Robert Makowsky ◽  
Howard W. Wiener ◽  
Travis S. Ptacek ◽  
Miriam Silva ◽  
Aditi Shendre ◽  
...  

2020 ◽  
Vol 8 ◽  
Author(s):  
Fan Yan ◽  
Huayong Zhang ◽  
Ruihua Xiong ◽  
Xingfeng Cheng ◽  
Yang Chen ◽  
...  

Background: In the latest 2017 American Heart Association guidelines for Kawasaki disease (KD), there are no recommendations regarding the early administration of intravenous immunoglobulin (IVIG). Therefore, the purpose of this systematic review and meta-analysis was to investigate the effects of early IVIG therapy on KD.Methods: We searched databases including the PubMed, Medline, the Cochrane Library, and the Clinicaltrials.gov website until July 2019.Results: Fourteen studies involving a total of 70,396 patients were included. Early treatment with IVIG can lead to an increased risk of IVIG unresponsiveness [OR 2.24; 95% CI (1.76, 2.84); P = 0.000]. In contrast to the studies performed in Japan [OR 1.27; 95% CI (0.98, 1.64); P = 0.074] that found no significant difference in coronary artery lesions (CAL) development, studies conducted in China [OR 0.73; 95% CI (0.66, 0.80); P = 0.000] and the United States [OR 0.50; 95% CI (0.38, 0.66); P = 0.000] showed a reduced risk in the occurrence of CAL with early IVIG treatment.Conclusions: At present, the evidence does not support the treatment with IVIG in the early stage of the onset of KD. But, early IVIG treatment could be a protective factor against the development of CAL, which needs to be further clarified.


2016 ◽  
Vol 9 (S1) ◽  
Author(s):  
Sung-Chou Li ◽  
Wen-Ching Chan ◽  
Ying-Hsien Huang ◽  
Mindy Ming-Huey Guo ◽  
Hong-Ren Yu ◽  
...  

Sign in / Sign up

Export Citation Format

Share Document