scholarly journals Peripheral Gangerene, an Unusual Presentation of Infantile Kawasaki: A Case Report and Literature Review

2021 ◽  
Vol 2021 ◽  
pp. 1-7
Author(s):  
Fatemeh Tahghighi ◽  
Maryam Bakhtiari Koohsorkhi ◽  
Vahid Ziaee
Keyword(s):  
Kawasaki Disease ◽  
Systemic Vasculitis ◽  
Systolic Dysfunction ◽  
Unknown Origin ◽  
Mitral Insufficiency ◽  
Prolonged Fever ◽  
Young Infants ◽  
Peripheral Gangrene ◽  
Coronary Abnormalities ◽  
Coronary Ectasia

Introduction. Diagnosing infantile Kawasaki disease with atypical symptoms is difficult, and it also has higher risk of coronary abnormalities which is one of the most common complications of KD. Other complications such as pericardial effusion, mitral insufficiency, congestive heart failure, myocardial systolic dysfunction, and systemic vasculitis were also reported. Peripheral gangrene and necrosis are among the rare complications of this systemic vasculitis. Case Presentation. We report an 8-month-old girl with prolonged fever, generalized petechial rash, cracked erythematous lips, edema, and coronary ectasia who received two doses of IVIG in another center, but short after her discharge, she started to develop a necrotic plaque on her knee. She was admitted in our hospital, and the repeat echocardiography showed sustained coronary ectasia. She received 3 doses of methylprednisolone pulse therapy and was discharged with aspirin and prednisolone. In the follow-up visits, the coronary ectasia was resolved and the necrotic ulcer was healing with a scar. Conclusions. The diagnosis of Kawasaki disease and echocardiographic evaluation of the coronary arteries should be considered in young infants with prolonged fever of unknown origin. Peripheral gangrene is a rare but important complication of infantile Kawasaki disease, although the exact mechanism in not fully understood.

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 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.

scholarly journals Old and New Challenges in Uveitis Associated with Behçet’s Disease

2021 ◽  
Vol 10 (11) ◽  
pp. 2318
Author(s):  
Julie Gueudry ◽  
Mathilde Leclercq ◽  
David Saadoun ◽  
Bahram Bodaghi
Keyword(s):  
Behçet’S Disease ◽  
Structural Damage ◽  
Behcet’S Disease ◽  
Behçet's Disease ◽  
Systemic Vasculitis ◽  
Retinal Vasculitis ◽  
Unknown Origin ◽  
Current Data ◽  
Ocular Involvement ◽  
Behcet's Disease

Behçet’s disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. However, the diagnosis of BD is still a major challenge. In the absence of a reliable biological marker, diagnosis is based on clinical diagnostic criteria and may be delayed after the appearance of the onset sign. However, therapeutic management of BD needs to be introduced early in order to control inflammation, to preserve visual function and to limit irreversible structural damage. The aim of this review is to provide current data on how innovations in clinical evaluation, investigations and treatments were able to improve the prognosis of uveitis associated with BD.

Haemolytic uraemic syndrome with peripheral gangrene and Kawasaki disease in a 15-month-old child

2016 ◽  
Vol 37 (1) ◽  
pp. 70-73 ◽  
Author(s):  
Sriram Krishnamurthy ◽  
Anbazhagan Jagadeesh ◽  
Venkatesh Chandrasekaran ◽  
Subramanian Mahadevan ◽  
Nachiappa Ganesh Rajesh
Keyword(s):  
Kawasaki Disease ◽  
Haemolytic Uraemic Syndrome ◽  
Peripheral Gangrene

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.

Lymphadenitis as the Dominant Manifestation of Kawasaki Disease

PEDIATRICS ◽  
1994 ◽  
Vol 93 (3) ◽  
pp. 525-528
Author(s):  
Julie Kim Stamos ◽  
Kathleen Corydon ◽  
James Donaldson ◽  
Stanford T. Shulman
Keyword(s):  
Kawasaki Disease ◽  
Cervical Lymphadenopathy ◽  
Febrile Illness ◽  
The United States ◽  
Acute Febrile Illness ◽  
Conjunctival Injection ◽  
Coronary Abnormalities ◽  
Acquired Heart Disease ◽  
Mucosal Changes ◽  
Infants And Young Children

Kawasaki disease (KD) is an acute febrile illness primarily affecting infants and young children. Its importance relates to the fact that 20% to 25% of untreated patients develop coronary abnormalities that can lead to myocardial infarction or even to death.1 KD is a leading cause of acquired heart disease in children in many regions, including the United States.2 Because there are no specific diagnostic tests for KD, the diagnosis is established by the presence of fever and four of five criteria without other explanation for the illness: (1) nonexudative conjunctival injection; (2) oral mucosal changes; (3) changes of the peripheral extremities; (4) rash, primarily truncal; and (5) cervical lymphadenopathy.

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.

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