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1.
Pediatr Rheumatol Online J ; 22(1): 43, 2024 Apr 14.
Article En | MEDLINE | ID: mdl-38616268

BACKGROUND: Kawasaki disease has been described across the globe, although publications from Africa are limited. To our knowledge, there are no publications on Kawasaki disease from Kenya, which triggered this report. METHODS: A retrospective cross-sectional study was undertaken to identify in-patients with a discharge diagnosis of Kawasaki disease, over 2 different 5-year periods, at two pediatric hospitals in Nairobi, Kenya. We reviewed the medical records of all patients and report their clinical findings, diagnostic workup and treatment. In addition, we undertook a detailed review of the literature. RESULTS: Twenty-three patients with Kawasaki disease were identified, of those 12 (52.2%) had incomplete disease. The mean age was 2.3 years (SD+/-2.2) (range 0.3-10.3) with a male to female ratio of 1:1. The mean duration of fever at diagnosis was 8.3 days (SD+/-4.7) (range 2-20). Oral changes were the most common clinical feature and conjunctivitis the least common. Thrombocytosis at diagnosis was seen in 52% (12/23). Twenty-one patients (91.3%) were treated with intravenous immunoglobulin and all except 1 received aspirin. Baseline echocardiograms were performed in 95.7% (22/23) and found to be abnormal in 3 (13.6%). Follow-up data was limited. Our literature review identified 79 publications with documented cases of Kawasaki disease in children from 22 countries across the African continent with a total of 1115 patients including those from this report. Only 153 reported cases, or 13.7%, are from sub-Saharan Africa. CONCLUSIONS: This is the first publication on Kawasaki disease from Kenya and one of the largest reports from sub-Saharan Africa. It is the first to have a complete review of the number of published cases from the African continent. Challenges in the diagnosis and management of Kawasaki disease in many African countries include disease awareness, infectious confounders, access and cost of intravenous immunoglobulin, access to pediatric echocardiography and follow-up. Increasing awareness and health care resources are important for improving outcomes of Kawasaki disease in Africa.


Mucocutaneous Lymph Node Syndrome , Child , Child, Preschool , Female , Humans , Male , Cross-Sectional Studies , Immunoglobulins, Intravenous/therapeutic use , Kenya/epidemiology , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/epidemiology , Mucocutaneous Lymph Node Syndrome/therapy , Retrospective Studies , Infant
2.
Sci Rep ; 14(1): 7261, 2024 03 27.
Article En | MEDLINE | ID: mdl-38538656

Although intravenous immunoglobulin (IVIG)-resistant Kawasaki disease (KD) presents with persistent inflammatory stimulation of the blood vessels and an increased risk of coronary artery dilatation. However, the pathogenesis of this disease is unclear, with no established biomarkers to predict its occurrence. This study intends to explore the utility of S100A12/TLR2-related signaling molecules and clinical indicators in the predictive modeling of IVIG-resistant KD. The subjects were classified according to IVIG treatment response: 206 patients in an IVIG-sensitive KD group and 49 in an IVIG-resistant KD group. Real-time PCR was used to measure the expression of S100A12, TLR2, MYD88, and NF-κB in peripheral blood mononuclear cells of patients, while collecting demographic characteristics, clinical manifestations, and laboratory test results of KD children. Multi-factor binary logistic regression analysis identified procalcitonin (PCT) level (≥ 0.845 ng/mL), Na level (≤ 136.55 mmol/L), and the relative expression level of S100A12 (≥ 10.224) as independent risk factors for IVIG-resistant KD and developed a new scoring model with good predictive ability to predict the occurrence of IVIG-resistant KD.


Immunoglobulins, Intravenous , Mucocutaneous Lymph Node Syndrome , Child , Humans , Infant , Immunoglobulins, Intravenous/therapeutic use , Mucocutaneous Lymph Node Syndrome/therapy , S100A12 Protein , Toll-Like Receptor 2/genetics , Toll-Like Receptor 2/metabolism , Leukocytes, Mononuclear/metabolism , Retrospective Studies
3.
Ital J Pediatr ; 50(1): 22, 2024 Feb 03.
Article En | MEDLINE | ID: mdl-38310292

BACKGROUND: Kawasaki disease shock syndrome (KDSS), though rare, has increased risk for cardiovascular complications. Early diagnosis is crucial to improve the prognosis of KDSS patients. Our study aimed to identify risk factors and construct a predictive model for KDSS. METHODS: This case-control study was conducted from June, 2015 to July, 2023 in two children's hospitals in China. Children initially diagnosed with KDSS and children with Kawasaki disease (KD) without shock were matched at a ratio of 1:4 by using the propensity score method. Laboratory results obtained prior to shock syndrome and treatment with intravenous immunoglobulin were recorded to predict the onset of KDSS. Univariable logistic regression and forward stepwise logistic regression were used to select significant and independent risk factors associated with KDSS. RESULTS: After matching by age and gender, 73 KDSS and 292 KD patients without shock formed the development dataset; 40 KDSS and 160 KD patients without shock formed the validation dataset. Interleukin-10 (IL-10) > reference value, platelet counts (PLT) < 260 × 109/L, C-reactive protein (CRP) > 80 mg/ml, procalcitonin (PCT) > 1ng/ml, and albumin (Alb) < 35 g/L were independent risk factors for KDSS. The nomogram model including the above five indicators had area under the curves (AUCs) of 0.91(95% CI: 0.87-0.94) and 0.90 (95% CI: 0.71-0.86) in the development and validation datasets, with a specificity and sensitivity of 80% and 86%, 66% and 77%, respectively. Calibration curves showed good predictive accuracy of the nomogram. Decision curve analyses revealed the predictive model has application value. CONCLUSIONS: This study identified IL-10, PLT, CRP, PCT and Alb as risk factors for KDSS. The nomogram model can effectively predict the occurrence of KDSS in Chinese children. It will facilitate pediatricians in early diagnosis, which is essential to the prevention of cardiovascular complications.


Mucocutaneous Lymph Node Syndrome , Shock , Child , Humans , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/therapy , Interleukin-10 , Case-Control Studies , Immunoglobulins, Intravenous , Risk Factors , Retrospective Studies
4.
J Nippon Med Sch ; 91(2): 218-226, 2024 May 21.
Article En | MEDLINE | ID: mdl-38233123

BACKGROUND: Adipose tissue-derived mesenchymal stem cells (ADSCs) are used for the treatment of various diseases because of their rapid proliferation and high anti-inflammatory and tissue repair properties. Kawasaki disease is a systemic vasculitis with coronary arteritis and aneurysms occurring in pediatric patients. In this study, we examined serologically and pathologically whether the administration of human ADSCs (hADSCs) to a mouse model of Kawasaki disease could suppress vasculitis. METHODS: Candida albicans water-soluble fractions were intraperitoneally injected into DBA/2 mice for 5 consecutive days to generate a mouse model of Kawasaki disease. The model mice were intravenously administered hADSCs or phosphate-buffered saline (PBS). Serum samples collected on days 15 and 29 were used to compare cytokine levels. Mouse hearts dissected on day 29 were subjected to hematoxylin and eosin and immunohistological staining using Galectin-1 (Gal-1), a protein involved in cardiovascular homeostasis, and CD44, a cell-surface marker of hADSCs. RESULTS: Comparison of inflammation-related cytokines showed a significant decrease in IL-1α expression at day 15 (P<0.05) and IL-6 expression at day 29 (P<0.01) in the hADSCs-treated group compared to the PBS group. Evaluation by hematoxylin and eosin staining showed decreased inflammatory cell infiltration and a tendency towards increased Gal-1 expression in the hADSCs group. CD44 expression was not observed in both the groups. The survival curve showed that the hADSCs group had a significantly longer survival time (P<0.05). CONCLUSIONS: The present experimental results indicate that hADSCs have an early anti-inflammatory effect, and that Gal-1 may be involved in preventing inflammation and reducing tissue damage.


Adipose Tissue , Disease Models, Animal , Mucocutaneous Lymph Node Syndrome , Animals , Mucocutaneous Lymph Node Syndrome/therapy , Humans , Adipose Tissue/cytology , Mice, Inbred DBA , Interleukin-6/metabolism , Mesenchymal Stem Cell Transplantation/methods , Interleukin-1alpha/metabolism , Hyaluronan Receptors/metabolism , Mesenchymal Stem Cells , Coronary Vessels/pathology , Male , Mice
5.
Can J Cardiol ; 40(1): 58-72, 2024 01.
Article En | MEDLINE | ID: mdl-37290536

BACKGROUND: Patients with multisystem inflammatory syndrome in children (MIS-C) and Kawasaki disease (KD) have overlapping clinical features. We compared demographics, clinical presentation, management, and outcomes of patients according to evidence of previous SARS-CoV-2 infection. METHODS: The International Kawasaki Disease Registry (IKDR) enrolled KD and MIS-C patients from sites in North, Central, and South America, Europe, Asia, and the Middle East. Evidence of previous infection was defined as: Positive (household contact or positive polymerase chain reaction [PCR]/serology), Possible (suggestive clinical features of MIS-C and/or KD with negative PCR or serology but not both), Negative (negative PCR and serology and no known exposure), and Unknown (incomplete testing and no known exposure). RESULTS: Of 2345 enrolled patients SARS-CoV-2 status was Positive for 1541 (66%) patients, Possible for 89 (4%), Negative for 404 (17%) and Unknown for 311 (13%). Clinical outcomes varied significantly among the groups, with more patients in the Positive/Possible groups presenting with shock, having admission to intensive care, receiving inotropic support, and having longer hospital stays. Regarding cardiac abnormalities, patients in the Positive/Possible groups had a higher prevalence of left ventricular dysfunction, and patients in the Negative and Unknown groups had more severe coronary artery abnormalities. CONCLUSIONS: There appears to be a spectrum of clinical features from MIS-C to KD with a great deal of heterogeneity, and one primary differentiating factor is evidence for previous acute SARS-CoV-2 infection/exposure. SARS-CoV-2 Positive/Possible patients had more severe presentations and required more intensive management, with a greater likelihood of ventricular dysfunction but less severe coronary artery adverse outcomes, in keeping with MIS-C.


COVID-19 , Mucocutaneous Lymph Node Syndrome , Systemic Inflammatory Response Syndrome , Child , Humans , COVID-19/epidemiology , SARS-CoV-2 , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/epidemiology , Mucocutaneous Lymph Node Syndrome/therapy , Registries
7.
Cardiol Young ; 34(4): 793-802, 2024 Apr.
Article En | MEDLINE | ID: mdl-37830370

BACKGROUND: Children who develop coronary artery aneurysms after Kawasaki disease are at risk for cardiovascular morbidity, requiring health care transition and lifelong follow-up with an adult specialist. Follow-up losses after health care transition have been reported but without outcome and patient experience evaluation. OBJECTIVE: The Theoretical Domains Framework underpinned our aim to explore the required self-care behaviours and experiences of young adults' post-health care transition. METHODS: A qualitative description approach was used for virtual, 1:1 interviews with 11 participants, recruited after health care transition from a regional cardiac centre in Ontario. Directed content analysis was employed. RESULTS: Health, psychosocial, and lifestyle challenges were compounded by a sense of loss. Six themes emerged within the Theoretical Domains Framework categories. Participants offered novel health care transition programme recommendations. CONCLUSIONS: The realities of health care transition involve multiple, overlapping stressors for young adults with Kawasaki disease and coronary artery aneurysms. Our findings will inform a renewed health care transition programme and will include outcome evaluation.


Coronary Aneurysm , Mucocutaneous Lymph Node Syndrome , Transition to Adult Care , Child , Young Adult , Humans , Mucocutaneous Lymph Node Syndrome/therapy , Patient Transfer , Coronary Aneurysm/etiology , Coronary Aneurysm/therapy , Ontario
8.
Zhongguo Dang Dai Er Ke Za Zhi ; 25(12): 1198-1210, 2023 Dec 15.
Article Zh | MEDLINE | ID: mdl-38112136

Kawasaki disease (KD) is an acute self-limiting vasculitis, and it is the most common cause of acquired heart disease in children under 5 years old. One of the improvement goals in pediatric quality control work for the year 2023, as announced by the National Health Commission, is to reduce the incidence of cardiac events and KD-related mortality in children with KD. In order to standardize the diagnosis, treatment, and long-term management practices of KD in China, and effectively prevent and reduce the incidence of coronary artery lesions and long-term adverse effects, the guideline working group followed the principles and methods outlined by the World Health Organization and referenced existing evidence and experiences to develop the "Evidence-based guidelines for the diagnosis and treatment of Kawasaki disease in children in China (2023)". The guidelines address the clinical questions regarding the classification and definition of KD, diagnosis of different types of KD, treatment during the acute phase of KD, application of echocardiography in identifying complications of KD, and management of KD combined with macrophage activation syndrome. Based on the best evidence and expert consensus, 20 recommendations were formulated, aiming to provide guidance and decision-making basis for healthcare professionals in the diagnosis and treatment of KD in children.


Heart Diseases , Mucocutaneous Lymph Node Syndrome , Vasculitis , Child , Humans , Child, Preschool , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/therapy , Mucocutaneous Lymph Node Syndrome/complications , Vasculitis/complications , Vasculitis/drug therapy , Heart , China , Immunoglobulins, Intravenous/therapeutic use
9.
Int J Mol Sci ; 24(18)2023 Sep 11.
Article En | MEDLINE | ID: mdl-37762250

Kawasaki disease (KD) is an acute inflammatory disorder that primarily affects children and can lead to coronary artery lesions (CAL) if not diagnosed and treated promptly. The original clinical criteria for diagnosing KD were reported by Dr. Tomisaku Kawasaki in 1967 and have been used for decades. However, research since then has highlighted the limitations of relying solely on these criteria, as they might lead to underdiagnosis or delayed diagnosis, potentially increasing the risk of coronary artery complications. This review appears to discuss several important aspects related to KD diagnosis and management. The current diagnostic methods for KD might need updates, especially considering cases that do not fit the typical clinical criteria. Recognizing diagnostic pitfalls and distinguishing KD from other conditions that might have similar clinical presentations is essential. The differences and similarities between KD and Multisystem Inflammatory Syndrome in Children (MIS-C), another inflammatory condition that has been associated with COVID-19, were also reviewed. The review explores the potential role of eosinophil count, new biomarkers, microRNA panels, and scoring systems in aiding the diagnosis of KD. Overall, the review article provides a comprehensive overview of the evolving landscape of KD diagnosis and management, incorporating new diagnostic methods, biomarkers, and treatment approaches to improve patient outcomes and reduce the risk of complications.


COVID-19 , MicroRNAs , Mucocutaneous Lymph Node Syndrome , Child , Humans , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/therapy , COVID-19/diagnosis , Coronary Vessels , COVID-19 Testing
11.
Zhongguo Dang Dai Er Ke Za Zhi ; 25(6): 566-571, 2023 Jun 15.
Article Zh | MEDLINE | ID: mdl-37382124

OBJECTIVES: To study the role of plasma exchange combined with continuous blood purification in the treatment of refractory Kawasaki disease shock syndrome (KDSS). METHODS: A total of 35 children with KDSS who were hospitalized in the Department of Pediatric Intensive Care Unit, Hunan Children's Hospital, from January 2019 to August 2022 were included as subjects. According to whether plasma exchange combined with continuous veno-venous hemofiltration dialysis was performed, they were divided into a purification group with 12 patients and a conventional group with 23 patients. The two groups were compared in terms of clinical data, laboratory markers, and prognosis. RESULTS: Compared with the conventional group, the purification group had significantly shorter time to recovery from shock and length of hospital stay in the pediatric intensive care unit, as well as a significantly lower number of organs involved during the course of the disease (P<0.05). After treatment, the purification group had significant reductions in the levels of interleukin-6, tumor necrosis factor-α, heparin-binding protein, and brain natriuretic peptide (P<0.05), while the conventional group had significant increases in these indices after treatment (P<0.05). After treatment, the children in the purification group tended to have reductions in stroke volume variation, thoracic fluid content, and systemic vascular resistance and an increase in cardiac output over the time of treatment. CONCLUSIONS: Plasma exchange combined with continuous veno-venous hemofiltration dialysis for the treatment of KDSS can alleviate inflammation, maintain fluid balance inside and outside blood vessels, and shorten the course of disease, the duration of shock and the length of hospital stay in the pediatric intensive care unit.


Continuous Renal Replacement Therapy , Mucocutaneous Lymph Node Syndrome , Shock , Humans , Child , Plasma Exchange , Mucocutaneous Lymph Node Syndrome/therapy , Renal Dialysis , Plasmapheresis
12.
Rev Med Interne ; 44(7): 354-380, 2023 Jul.
Article En | MEDLINE | ID: mdl-37349225

Kawasaki disease (KD) is an acute vasculitis with a particular tropism for the coronary arteries. KD mainly affects male children between 6 months and 5 years of age. The diagnosis is clinical, based on the international American Heart Association criteria. It should be systematically considered in children with a fever, either of 5 days or more, or of 3 days if all other criteria are present. It is important to note that most children present with marked irritability and may have digestive signs. Although the biological inflammatory response is not specific, it is of great value for the diagnosis. Because of the difficulty of recognising incomplete or atypical forms of KD, and the need for urgent treatment, the child should be referred to a paediatric hospital as soon as the diagnosis is suspected. In the event of signs of heart failure (pallor, tachycardia, polypnea, sweating, hepatomegaly, unstable blood pressure), medical transfer to an intensive care unit (ICU) is essential. The standard treatment is an infusion of IVIG combined with aspirin (before 10 days of fever, and for a minimum of 6 weeks), which reduces the risk of coronary aneurysms. In case of coronary involvement, antiplatelet therapy can be maintained for life. In case of a giant aneurysm, anticoagulant treatment is added to the antiplatelet agent. The prognosis of KD is generally good and most children recover without sequelae. The prognosis in children with initial coronary involvement depends on the progression of the cardiac anomalies, which are monitored during careful specialised cardiological follow-up.


Coronary Aneurysm , Mucocutaneous Lymph Node Syndrome , Vasculitis , Child , Humans , Male , Infant , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/therapy , Mucocutaneous Lymph Node Syndrome/complications , Aspirin/therapeutic use , Fever/etiology , Vasculitis/complications , Coronary Aneurysm/diagnosis , Coronary Aneurysm/etiology , Coronary Aneurysm/therapy , Immunoglobulins, Intravenous/therapeutic use
13.
Pediatr Cardiol ; 44(6): 1277-1284, 2023 Aug.
Article En | MEDLINE | ID: mdl-37126143

Kawasaki Disease (KD) is still the most common acquired heart disease in children below the age of five years; it has been well described in the developed world; however, data from the Arab world are limited to case reports or single-center case series. In an effort of optimizing KD research in the Arab world, a group of physicians and researchers established the KD Arab Initiative (Kawarabi) in 2021, and published the first survey, which showed disparities in the availability of intravenous immunoglobulin (IVIG); this had prompted Kawarabi to assess the access to care and therapy of KD patients in Arab countries. A 32 structured questions survey was conducted in thirteen Arab countries and addressed KD patients' access to healthcare in urban and rural settings. The survey results showed that access to care was uniform across large, mid-size cities and rural areas in 7/13 (54%) countries, while in 6/13 (46%) countries, it was in favor of large and mid-size cities over rural areas. The quality of medical services received by children with KD in large cities was rated as excellent in 6/13 or good in 7/13 countries compared to fair in 4/13 or poor in 4/13 countries in rural areas. Availability of IVIG was limited (23%) in mid-size cities and almost impossible (23%) in rural areas. The KD patients in mid-size cities and rural areas have limited access to standard healthcare in the Arab world. This survey laid the foundation for future Kawarabi endeavors to improve the care of children with KD.


Heart Diseases , Mucocutaneous Lymph Node Syndrome , Child , Humans , Infant , Child, Preschool , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/epidemiology , Mucocutaneous Lymph Node Syndrome/therapy , Immunoglobulins, Intravenous/therapeutic use , Arabs , Health Services Accessibility
14.
Front Cell Infect Microbiol ; 13: 1101428, 2023.
Article En | MEDLINE | ID: mdl-37234775

Background: Kawasaki disease (KD) is a vascular inflammatory disease with unknown pathogenesis. There are few studies on KD combined with sepsis worldwide. Purpose: To provide valuable data regarding clinical characteristics and outcomes related to pediatric patients with KD combined with sepsis in pediatric intensive care unit (PICU). Methods: We retrospectively analyzed the clinical data of 44 pediatric patients admitted in PICU at Hunan Children's Hospital with KD combined with sepsis between January 2018 and July 2021. Results: Of the 44 pediatric patients (mean age, 28.18 ± 24.28 months), 29 were males and 15 were female. We further divided the 44 patients into two groups: KD combined with severe sepsis (n=19) and KD combined with non-severe sepsis (n=25). There were no significant between-group differences in leukocyte, C-reactive protein, and erythrocyte sedimentation rate. Interleukin-6, interleukin-2, interleukin-4 and procalcitonin in KD with severe sepsis group were significantly higher than those in KD with non-severe sepsis group. And the percentage of suppressor T lymphocyte and natural killer cell in severe sepsis group were significantly higher than those in non-severe group, while the CD4+/CD8+ T lymphocyte ratio was significantly lower in KD with severe sepsis group than in KD with non-severe sepsis group. All 44 children survived and were successfully treated after intravenous immune globulin (IVIG) combined with antibiotics. Conclusion: Children who develop with KD combined with sepsis have different degrees of inflammatory response and cellular immunosuppression, and the degree of inflammatory response and cellular immunosuppression is significantly correlated with the severity of the disease.


Mucocutaneous Lymph Node Syndrome , Sepsis , Male , Humans , Child , Female , Infant , Child, Preschool , Mucocutaneous Lymph Node Syndrome/therapy , Mucocutaneous Lymph Node Syndrome/drug therapy , Retrospective Studies , Sepsis/complications , Sepsis/drug therapy , Immunoglobulins, Intravenous/therapeutic use , Intensive Care Units, Pediatric
15.
Curr Allergy Asthma Rep ; 23(6): 341-350, 2023 06.
Article En | MEDLINE | ID: mdl-37171672

PURPOSE OF REVIEW: Since it first appeared, multisystem inflammatory syndrome in children (MIS-C) associated with coronavirus disease 2019 (COVID-19) has been compared to Kawasaki disease (KD). Although there were early parallels between MIS-C and KD, key differences emerged over time. Here, we aim to compare the pathogenesis, clinical presentation, treatment, and outcomes of MIS-C and KD. RECENT FINDINGS: In this article, we review and compare MIS-C and KD, highlighting differentiating features. We discuss the epidemiological and immunological factors along with clinical and laboratory features which discern MIS-C from KD. We also compare treatment and our understanding of long-term outcomes. Though parallels exist between MIS-C and KD, distinguishing the two is important for clinical management of patients, counseling about natural history, and determining long-term monitoring. While both MIS-C and KD are characterized by profound inflammation and inflammatory vasculopathy, further study is needed to determine whether they are distinct immunopathogenic disorders.


COVID-19 , Mucocutaneous Lymph Node Syndrome , Humans , Child , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/therapy , Systemic Inflammatory Response Syndrome/diagnosis , Systemic Inflammatory Response Syndrome/therapy , Inflammation
16.
F1000Res ; 12: 235, 2023.
Article En | MEDLINE | ID: mdl-37065507

Kawasaki disease is an acute, febrile disease that is not typically fatal if treated and affects infants and children more commonly. More than 80% of the afflicted patients are under the age of four. This disease most commonly affects coronary arteries. In a minority of cases, Aneurysms can burst or produce thrombosis, and they can cause infarction. The distinctive redness in the palms and soles of the feet might result from a delayed-type hypersensitivity reaction to a cross-reactive or recently discovered antigen (s). Autoantibodies against epithelial cells and smooth muscle cells are produced as a result of subsequent macromolecule synthesis and polyclonal white blood cell activation, which intensifies the redness. Kawasaki disease's clinical manifestations range from oral skin disease to the blistering of the mucosa, symptoms involving the hands and the feet, skin disease of the palms and soles, a desquamative rash, and cervical lymphatic tissue enlargement (so it is also referred to as tissue layer lymphatic tissue syndrome). Most untreated patients develop some vessel sequelae, from well-organized coronary inflammation to severe arterial blood vessel dilatation to giant artery aneurysms with rupture or occlusion, infarction, and thrombosis. With human gamma globulin administration, reasonable standards of medical care, and the use of analgesics, the speed of symptomatic progression and inflammatory artery changes are reduced. In this review, we have covered the immunology of Kawasaki disease, its biomarkers, and the neurological manifestations of this multisystem illness. We have also included a discussion on its pathogenesis, diagnosis, and treatment.


Mucocutaneous Lymph Node Syndrome , Infant , Child , Humans , Mucocutaneous Lymph Node Syndrome/therapy , Mucocutaneous Lymph Node Syndrome/drug therapy , Aspirin/therapeutic use , Biomarkers , Coronary Vessels , Acute Disease , Immunity
17.
Zhongguo Dang Dai Er Ke Za Zhi ; 25(3): 238-243, 2023 Mar 15.
Article Zh | MEDLINE | ID: mdl-36946156

Kawasaki disease (KD) is a febrile disease mainly observed in children aged <5 years, with medium- and small-vessel vasculitis as the main lesion. Although KD has been reported for more than 50 years and great progress has been made in the etiology and pathology of KD in recent years, there is still a lack of specific indicators for the early diagnosis of KD, especially with more difficulties in the diagnosis of incomplete Kawasaki disease (IKD). At present, there are no clear diagnostic criteria for IKD, which leads to the failure of the timely identification and standardized treatment of IKD in clinical practice and even induce the development of coronary artery lesion. This article reviews the concept, epidemiological features, diagnosis, treatment, and follow-up management of IKD, in order to deepen the understanding of IKD among clinical workers and help to improve the clinical diagnosis and treatment of KD in China.


Mucocutaneous Lymph Node Syndrome , Child , Humans , Infant , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/therapy , Coronary Vessels , China
18.
Rev Prat ; 73(2): 179-186, 2023 Feb.
Article Fr | MEDLINE | ID: mdl-36916262

DIAGNOSIS AND MANAGEMENT OF KAWASAKI DISEASE. Kawasaki disease (KD) is a multi-systemic vasculitis predominantly affecting children under 5 years of age. KD's incidence is 30 times higher in Asia than in France. Elective lesional tropism for coronary arteries makes it the leading cause of acquired heart disease in children in developed countries. Diagnosis is based on the presence of fever of 5 days or more and the presence of at least for clinical criteria of cutaneous-mucosal inflammation. KD is believed to be the result of an aberrant inflammatory response to an infectious trigger in a genetically predisposed individual. Timely initiation of treatment with intravenous immunoglobulin has reduced the incidence of coronary artery aneurysms from 25% to 5%. Severe KD requires rapid treatment intensification. Coronary artery lesions are the main determinant of the prognosis. Acute coronary syndrome in adults can be a result of long-term sequelae of the disease. Different imaging techniques are required to adequately follow these patients according to the American Heart Association recommendations.


MALADIE DE KAWASAKI INFANTILE: DIAGNOSTIC ET PRISE EN CHARGE. La maladie de Kawasaki est une vascularite multisystémique qui touche principalement les enfants de moins de 5 ans. Son incidence est 30 fois plus élevée en Asie qu'en France. Son tropisme lésionnel électif pour les artères coronaires en fait la première cause de cardiopathie acquise chez l'enfant dans les pays développés. Le diagnostic est suspecté devant une fièvre de cinq jours ou plus associée à au moins quatre critères cliniques d'inflammation cutanéo-muqueuse. Elle est probablement liée à des agents infectieux entraînant une réponse inflammatoire inappropriée chez un sujet prédisposé. La précocité de la prise en charge par immunoglobulines intraveineuses diminue l'incidence des atteintes coronaires de 25 à 5 %. Les formes sévères nécessitent une intensification thérapeutique d'emblée. Son pronostic est corrélé à la survenue de lésions coronariennes et à leur évolution. Les complications coronariennes peuvent se traduire, à l'âge adulte, par un syndrome coronaire aigu secondaire à des séquelles de la maladie. Différentes techniques d'imagerie permettent de suivre les patients, selon les recommandations de l'American Heart Association.


Heart Diseases , Mucocutaneous Lymph Node Syndrome , Child , Adult , Humans , Child, Preschool , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/therapy , Mucocutaneous Lymph Node Syndrome/complications , Fever , Immunoglobulins, Intravenous/therapeutic use , Prognosis
20.
J Pediatr ; 253: 266-269, 2023 02.
Article En | MEDLINE | ID: mdl-36208665

OBJECTIVE: The objective of this study was to investigate the differences in the clinical characteristics of Kawasaki disease between older and younger children. STUDY DESIGN: This retrospective study examined 405 children with Kawasaki disease admitted to Showa University Northern Yokohama Hospital between 2015 and 2019. RESULTS: Eligible patients were classified into the older (≥3.0 years of age, n = 169) and younger (<3.0 years of age, n = 236) groups. Skin rash was found in significantly fewer cases (112 [66.3%] vs 229 [97.0%], P < .001 in the younger group). Cervical lymphadenopathy was more common in older children (153 [90.5%] vs 165 [69.9%], P < .001) and in incomplete Kawasaki disease (3 or 4 findings) (34 [20.1%] vs 25 [10.6%], P = .0078). The diagnosis was more delayed in older children (median: 5.0 days vs 4.0 days, P = .003) than the younger group. Additionally, fever nonresponsive to a single intravenous immunoglobulin was more common, and the duration of fever was significantly longer in the older group (48 [28.4%] vs 47 [19.9%], P = .0479). CONCLUSIONS: Kawasaki disease should be suspected in children aged >3.0 years with cervical lymphadenopathy and fever, despite the absence of skin rash. Additionally, incomplete Kawasaki disease, fever unresolved by a single intravenous immunoglobulin infusion, and the tendency to delay treatment initiation are more common in children aged >3.0 years.


Exanthema , Lymphadenopathy , Mucocutaneous Lymph Node Syndrome , Humans , Child , Infant , Mucocutaneous Lymph Node Syndrome/diagnosis , Mucocutaneous Lymph Node Syndrome/epidemiology , Mucocutaneous Lymph Node Syndrome/therapy , Retrospective Studies , Immunoglobulins, Intravenous/therapeutic use , Fever/epidemiology , Fever/etiology , Fever/drug therapy , Exanthema/epidemiology , Exanthema/etiology , Lymphadenopathy/epidemiology , Lymphadenopathy/etiology
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