scholarly journals Spontaneous Duodenal Perforation as a Complication of Kawasaki Disease

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Kambiz Masoumi ◽  
Arash Forouzan ◽  
Hossein Saidi ◽  
Hazhir Javaherizadeh ◽  
Ali Khavanin ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Intestinal Tract ◽  
Systemic Vasculitis ◽  
Cardiac Complications ◽  
Duodenal Perforation ◽  
Gi Bleeding ◽  
Visceral Organs

Kawasaki disease is generally known as a systemic vasculitis that often concerns doctors due to its serious cardiac complications; however, other visceral organs may get involved as well. Surgical manifestations of the intestinal tract in Kawasaki disease are rare. In this report, we describe the case of a 2.5-year-old boy with typical Kawasaki disease who presented with GI bleeding and surgical abdomen. The diagnosis of duodenal perforation was confirmed.

scholarly journals Long-term evaluation of endothelial function in Kawasaki disease patients

2012 ◽  
Vol 23 (4) ◽  
pp. 517-522 ◽  
Author(s):  
Fátima F. Pinto ◽  
Sérgio Laranjo ◽  
Filipa Paramés ◽  
Isabel Freitas ◽  
Miguel Mota-Carmo
Keyword(s):  
Risk Factors ◽  
Endothelial Dysfunction ◽  
Kawasaki Disease ◽  
Augmentation Index ◽  
Systemic Vasculitis ◽  
Cardiac Complications ◽  
Control Group ◽  
Reactive Hyperaemia ◽  
Coronary Lesions

AbstractBackgroundKawasaki disease is an acute systemic vasculitis. Cardiac complications are frequent and include endothelial dysfunction in patients with coronary anomalies. So far, the presence of endothelial dysfunction in patients with no coronary lesions has not been demonstrated. Peripheral arterial tonometry (Endo-PAT) measures the microvascular function in response to local ischaemia and has been validated in adult population, but its use in children is scarce.AimTo evaluate endothelial dysfunction in children as a long-term complication after Kawasaki disease using Endo-PAT.MethodsWe evaluated two groups of subjects: (1) Kawasaki disease patients over 11 years of age, diagnosed for >5 years, with no coronary lesions, or any other risk factors for cardiovascular disease; (2) control group of individuals without cardiovascular risk factors. Patients and controls were clinically accessed. Endo-PAT was performed to determine reactive hyperaemia index and augmentation index.ResultsA total of 35 individuals (21 males, age 21 ± 6 years) were evaluated (group 1: 19; controls: 16). Kawasaki disease patients presented significant lower reactive hyperaemia index (1.68 ± 0.49 versus 2.31 ± 0.53; p = 0.001). Augmentation index was similar in both groups (−10 ± 7 versus −11 ± 5; p > 0.005). Most patients with Kawasaki disease disclosed endothelial dysfunction (68%) compared with only 12% in controls.ConclusionsEndo-PAT is feasible and reproducible in the child population. Endothelial dysfunction is a frequent long-term complication in patients after Kawasaki disease with normal appearing coronary arteries. However, these results need validation in a larger population.

Abstract 210: Kawasaki Disease in Patients Older Than 10-years Old in a Children’s Hospital In Mexico City

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
ITZEL E RIOS-OLIVARES ◽  
LUIS M GARRIDO-GARCIA
Keyword(s):  
Kawasaki Disease ◽  
Mexico City ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Cervical Lymphadenopathy ◽  
Skin Lesions ◽  
Clinical Feature ◽  
Unknown Etiology ◽  
Cardiac Complications ◽  
Increased Risk

Background: Kawasaki Disease (KD) is an acute febrile illness characterized by systemic vasculitis of unknown etiology. Cardiac sequelae, such as coronary artery aneurysms (CAA), are one of the most important aspects of this disease. Actually KD is most frequently presented in children younger than 5-years old. Objective: To describe the clinical and laboratory features, cardiac sequelae and outcome in children older than 10-years old with KD who were attended at the Instituto Nacional de Pediatría in Mexico City. Methods: An observational, descriptive, retrospective and transversal case study. We reviewed the medical records of patients older than 10-years diagnosed with KD from August 1995 to May 2014, and analyzed gender, age, clinical manifestations, hemoglobin, leucocyte count, platelet count, ESR, CRP, albumin, sodium, potassium, AST, ASL, time from the onset of the symptoms to diagnosis, treatment used, the development of CAA and outcome in the acute phase of the disease. Results: We studied 18 cases of KD in patients older than 10-years old, 72.2% (13 of 18) were male with a mean age of 154 months (range 120 to 200). The time from the onset of the fever to diagnosis was 10.6 ± 5.8 days, (range 3 to 21 days). Skin lesions were the most common manifestation of KD and cervical lymphadenopathy was the least common clinical feature. 2 patients presented with KD shock syndrome. Complete KD was diagnosed in only 50% (9 of 18) of our cases. 16 patients received IVGG, 2 patients required a second GGIV dose and 10 patients also received steroids. 6 of 18 patients (30%) developed CAA. There were no deaths in our group. Conclusions: KD in patients older than 10-years old represent a clinical challenge because in the majority of the cases they presented with an atypical clinical picture which contribute to a delayed diagnosis. Also there is an increased risk of developing cardiac complications and CAA in this group of patients.

Controversies in the medical management of Kawasaki disease

1991 ◽  
Vol 1 (3) ◽  
pp. 234-239 ◽  
Author(s):  
Elfriede Pahl ◽  
William H. Neches ◽  
José A. Ettedgui
Keyword(s):  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
Cardiovascular Complications ◽  
Cardiac Complications ◽  
Optimal Dosage ◽  
Duration Of Treatment ◽  
Clinical Criteria ◽  
Intravenous Gammaglobulin ◽  
Arterial Aneurysms

SummarySummary Mucocutaneous lymph node syndrome, now called Kawasaki disease, affects children of all ages and races. The cause of this systemic vasculitis is unknown, thus the diagnosis is based solely on clinical criteria. Coronary arteritis and the formation of coronary arterial aneurysms are the most serious early cardiac complications, while long term sequels, such as coronary stenosis and myocardial infarction, may occur. Current medical therapy is aimed solely at preventing or decreasing the occurrence of these cardiovascular complications in patients with this disease. Past treatment included antibiotics, steroids, and nonsteroidal anti-inflammatory agents. Aspirin remains the most widely used drug, although the use of intravenous gammaglobulin has also become common. When used in combination, these two agents may reduce the incidence of coronary arterial aneurysms. The optimal dosage and duration of treatment has not yet been determined.

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 ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Systemic Vasculitis ◽  
Age Distribution ◽  
Population Based ◽  
Research Database ◽  
Ocular Manifestations ◽  
Missing Data Treatment ◽  
The Difference ◽  
Age Range

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.

scholarly journals The “Intermediate” CD14 + CD16 + monocyte subpopulation plays a role in IVIG responsiveness of children with Kawasaki disease

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Yi Seul Kim ◽  
Hyun Jin Yang ◽  
Seung-Jung Kee ◽  
Insu Choi ◽  
Kisoo Ha ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Laboratory Tests ◽  
Febrile Illness ◽  
Cardiac Complications ◽  
Study Cohort ◽  
Ivig Infusion ◽  
Functional Studies ◽  
Ivig Resistance ◽  
Resistant Group ◽  
Intermediate Monocytes

Abstract Background Kawasaki disease (KD) is an acute, self-limited febrile illness of unknown cause. Intravenous immunoglobulin (IVIG)-resistance are related to greater risk for permanent cardiac complications. We aimed to determine the correlation between monocytes and the phenotype of KD in relation to IVIG responsiveness in children. Materials and methods The study cohort included 62 patients who were diagnosed with KD, 20 non febrile healthy controls (NFC), and 15 other febrile controls (OFC). In all enrolled patients, blood was taken at least 4 times and laboratory tests were performed. In addition, subtypes of monocytes were characterized via flow cytometry. Results The numbers of intermediate monocytes were significantly lower in IVIG-resistant group compared to IVIG-responsive group before IVIG infusion (p < 0.0001). After infusion, intermediate monocytes decreased in the responsive group, while a trend of increase was observed in the resistant group. Only intermediate monocytes were significant in logistic regression with adjusted OR of 0.001 and p value of 0.03. Conclusions CD14 + CD16 + intermediate monocyte may play an important role in IVIG responsiveness among KD children. Low starting levels of intermediate monocytes, followed by a dramatic increase post-IVIG infusion during acute phase of KD are associated with IVIG-resistance. Functional studies on intermediate monocyte may help to reveal the pathophysiology.

scholarly journals Exposures associated with the onset of Kawasaki disease in infancy from the Japan Environment and Children’s Study

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Sayaka Fukuda ◽  
Shiro Tanaka ◽  
Chihiro Kawakami ◽  
Tohru Kobayashi ◽  
Shuichi Ito ◽  
...  
Keyword(s):  
Risk Factors ◽  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
First Trimester ◽  
Study Data ◽  
Causal Effects ◽  
Birth Cohort Study ◽  
Multivariate Logistic Regression ◽  
Maternal Thyroid ◽  
Infants And Young Children

AbstractKawasaki disease (KD) is an acute systemic vasculitis that mainly affects infants and young children. The etiology of KD has been discussed for several decades; however, no reproducible risk factors have yet been proven. We used the Japan Environment and Children’s Study data to explore the association between the causal effects of exposure during the fetal and neonatal periods and KD onset. The Japan Environment and Children’s Study, a nationwide birth cohort study, has followed approximately 100,000 children since 2011. We obtained data on exposures and outcomes from the first trimester to 12 months after birth. Finally, we included 90,486 children who were followed for 12 months. Among them, 343 children developed KD. Multivariate logistic regression revealed that insufficient intake of folic acid during pregnancy (odds ratio [OR], 1.37; 95% CI 1.08–1.74), maternal thyroid disease during pregnancy (OR, 2.03; 95% CI 1.04–3.94), and presence of siblings (OR, 1.33; 95% CI 1.06–1.67) were associated with KD onset in infancy. In this study, we identified three exposures as risk factors for KD. Further well-designed studies are needed to confirm a causal relationship between these exposures and KD onset.

Abstract 132: Methylprednisolone Pulse Therapy for Kawasaki Disease with Symptomatic Myocarditis

Circulation ◽  
2015 ◽  
Vol 131 (suppl_2) ◽  
Author(s):  
yeo hyang kim ◽  
Chae Ok Shin ◽  
Myung Chul Hyun ◽  
Dong Seok Lee
Keyword(s):  
Kawasaki Disease ◽  
Systolic Function ◽  
Systemic Vasculitis ◽  
Cervical Lymphadenopathy ◽  
Febrile Illness ◽  
Pulse Therapy ◽  
Left Ventricular ◽  
Acute Stage ◽  
Z Score ◽  
Intravenous Methylprednisolone

Purpose: Kawasaki disease (KD) is an acute febrile illness of infants and young children that is characterized by a systemic vasculitis, especially involving the coronary arteries. Although, sometimes, subclinical myocarditis is combined in KD, symptomatic myocarditis is extremely uncommon. We report a 7 year old boy who developed hypotension and decreased left ventricular systolic function (EF 40%) in the acute phase of KD. Case: A 7 year old boy (height 115 cm, body weight 20 kg) was admitted because of 2 days of persistent fever and left cervical lymphadenopathy (white blood cell count 17,870 /mm 3 , C reactive protein 23.6 mg/dL). Conjunctiva injection and lip redness developed on the 4th day of illness, and hypotension and tachycardia (SBP 59/DBP 29 mmHg, HR 153/bpm) were combined. The echocardiography revealed a decreased ejection fraction (EF) (40%) without chamber dilatation and normal coronary artery size (LM 1.9mm, z score=-1.3, RCA 2.3mm, z score=0.4). The level of N terminal pro BNP was 28,000 pg/mL. With a diagnosis of KD with myocarditis, he was initially treated with inotropics and intravenous immunoglobulin (2 g/kg). Without clinical improvement in spite of initial treatment, A change of coronary arterial size (LM 2.9mm, z score=1.2, RCA 3.1mm, z score=2.3) was developed and decreased LV systolic function (EF 45%) and fever were persisted. Then, he was given 3 daily pulses of intravenous methylprednisolone followed by tapering doses of oral prednisolone. He showed prompt clinical recovery after pulse therapy of intravenous methylprednisolone (SBP 95/DBP 49 mmHg, HR 98/bpm). Although EF was improved (59%), coronary arterial dilatation was progressed (LM 3.4mm, z score=2.4 RCA 5.5mm, z score=7.9). Conclusions: The present case serves to highlight the fact that methylprednisolone should be considered as the priority in children with KD who have symptomatic myocarditis during the acute stage.

Association Between the lncRNA TDRG1 rs8506 C>T Polymorphism and Susceptibility To Kawasaki Disease in Chinese Children

2021 ◽  
Author(s):  
Jinqing Li ◽  
Kai Guo ◽  
Hongyan Yu ◽  
Yufen Xu ◽  
Chuanzi Yang ◽  
...  
Keyword(s):  
Coronary Artery ◽  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
Recessive Model ◽  
Chinese Children ◽  
Future Research ◽  
Human Testis ◽  
Non Coding Rna ◽  
Vegf Pathway ◽  
Endothelial Growth Factor

Abstract Background Kawasaki disease (KD) is an acute systemic vasculitis with unknown pathogen, and the formation of coronary artery lesions/aneurysm (CAL/CAA) is the most common sequela. Environmental and genetic factors have been suggested to be involved in the pathogenesis of KD. Human testis development related 1(TDRG1) is a newly identified long non-coding RNA (lncRNA) which stimulates the vascular endothelial growth factor (VEGF) pathway. The aim of this study was to investigate the association between genetic polymorphism of TDRG1 and the risk of KD. Methods A total of two cohorts from Guangzhou (988 KD patients and 1054 controls) and Beijing (564 KD patients and 1221 controls) were enrolled in the present study. Rs8506 of TDRG1 was chosen for TaqMan genotyping assay. Results Logistic regression suggested that lncRNA TDRG1 rs8506 C > T polymorphism was not associated with the risk of KD in both Guangzhou and Beijing cohort (dominant model and recessive model: P > 0.05). Moreover, we also did not observe a significant association between the lncRNA TDRG1 rs8506 C > T polymorphism and the risk of KD patients with different ages, gender or coronary artery outcomes in both Guangzhou and Beijing cohort. Conclusions The present study revealed that the lncRNA TDRG1 rs8506 C > T polymorphism might not be associated with susceptibility to KD in Chinese children. Future research with a larger sample size should be performed to confirm these results.

Systemic Vasculitis Syndromes

2017 ◽  
Author(s):  
Alexandra Villa-Forte ◽  
Brian F Mandell
Keyword(s):  
Kawasaki Disease ◽  
Blood Vessels ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Plain Radiograph ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Saddle Nose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Saddle Nose Deformity

Vasculitis is defined by histologic evidence of inflammation that involves the blood vessels. The diagnosis of a specific primary vasculitic disorder depends on the pattern of organ involvement, the histopathology, the size of affected blood vessels, and the exclusion of diseases that can cause “secondary” vasculitis. This review presents an approach to the patient suspected of having vasculitis, and goes on to discuss small vessel vasculitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosa, Kawasaki disease, large vessel arteritis, and Behçet disease. Figures show classification of the systemic vasculitis syndromes, the relationships among the causes of small vessel (“hypersensitivity”) vasculitis, palpable purpura of the distal extremities, saddle nose deformity, the nodular infiltrates of the lung in granulomatosis with polyangiitis shown on plain radiograph as well as computed tomography, necrotizing scleritis, livedo reticularis, and angiograms of a patient with Takayasu arteritis. Tables list selected laboratory tests for patients with multisystem disease and possible vasculitis, practical comments on immunosuppressive therapies for vasculitis, features of vasculitis, diagnostic criteria for Kawasaki disease, and giant cell arteritis. This review contains 8 highly rendered figures, 5 tables, and 59 references.

Abstract 16564: Analysis of the Mechanisms of Intravenous Immunoglobulin-Resistant Kawasaki Disease Using iPS Cell Technology

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Kazuyuki Ikeda ◽  
Tomonaga Ameku ◽  
Yui Nomiya ◽  
Masahiro Nakamura ◽  
Satoshi Matsui ◽  
...  
Keyword(s):  
Gene Expression ◽  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Expression Profiles ◽  
Systemic Vasculitis ◽  
Dermal Fibroblasts ◽  
Gene Set Enrichment Analysis ◽  
Recurrent Fever ◽  
Ips Cell ◽  
Ivig Resistance

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.

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