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Sample records for acute kawasaki disease

  1. Kawasaki disease

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    ... lymph node syndrome; Infantile polyarteritis Images Kawasaki's disease - edema of the hand Kawasaki's disease, peeling of the fingertips References Dominguez SR, Anderson MS. Advances in the treatment of Kawasaki disease. Curr Opin Pediatr . 2013;25( ...

  2. Genetics Home Reference: Kawasaki disease

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    ... Email Facebook Twitter Home Health Conditions Kawasaki disease Kawasaki disease Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Kawasaki disease is a sudden and time-limited (acute) ...

  3. Kawasaki Disease

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    Kawasaki disease is a rare childhood disease. It makes the walls of the blood vessels in the ... veins, and capillaries. No one knows what causes Kawasaki disease. Symptoms include High fever that lasts longer ...

  4. Kawasaki Disease

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    ... Life7. Questions8. Resources What is Kawasaki disease? Kawasaki disease is a rare illness. It can occur in infants and children younger ... may have diarrhea, vomiting, and stomach pain. Kawasaki disease might make your child irritable. The illness can last for a few weeks. What causes ...

  5. Hemophagocytosis in the Acute Phase of Fatal Kawasaki Disease in a 4 Month-Old Girl.

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    Doğan, Vehbi; Karaaslan, Erhan; Özer, Samet; Gümüşer, Rüveyda; Yılmaz, Resul

    2016-07-01

    Kawasaki disease is a systemic vasculitis predominately affecting coronary arteries. Hemophagocytic lymphohistiocytosis can complicate the course of Kawasaki disease. Rare cases of secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of Kawasaki disease have been reported. We report here a 4 month-old girl with diffuse coronary ectasia and secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of incomplete Kawasaki disease. Due to the large overlap in clinical symptoms, the presence of atypical findings for Kawasaki disease should suggest the possible diagnosis of hemophagocytic lymphohistiocytosis in these patients.

  6. History of Kawasaki disease.

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    Kawasaki, Tomisaku; Naoe, Shiro

    2014-04-01

    We describe a short history of Kawasaki disease. In 1967, we published a paper entitled 'Infantile acute febrile mucocutaneous lymph node syndrome with specific desquamation of the fingers and toes. Clinical observation of 50 cases'; this was the first report on what is now called Kawasaki disease. Since then, many reports on cardiology, treatment, epidemiology, pathology and etiology of Kawasaki disease have been published. Furthermore, a recent Chapel Hill Consensus Statement on Kawasaki disease in the classification of vasculitis is given, along with a figure on the relationship and classification of childhood vasculitis by autopsy material.

  7. [Kawasaki's disease].

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    Cortes, J; Martínez, B; Montini, C; Barraza, P; Reyes, A

    1989-08-01

    We described a case of Kawasaki's disease in a chilean girl, one year and 5 months old of age, who presented the oral characteristics, cutaneous and systemic manifestation of the condition, that is not very common for the dentist but that it is necessary to know due to the heart complications and the mortality associated with the disease, and it is necessary that the dentist recognize early this condition.

  8. What Is Kawasaki Disease?

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    ANSWERS by heart Cardiovascular Conditions What is Kawasaki Disease? Kawasaki disease is a children’s illness. It’s also known as Kawasaki syndrome or mucocutaneous lymph node syndrome. About 75 percent of people ...

  9. ATYPICAL KAWASAKI DISEASE.

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    Ristovski, Ljiljana; Milankov, Olgica; Vislavski, Melanija; Savić, Radojica; Bjelica, Milena

    2016-01-01

    Kawasaki disease is an acute vasculitis which occurs primarily in children under the age of 5. The etiology of the disease is still unknown. Diagnostic criteria for Kawasaki disease are fever and at least four of the five additional clinical signs. Incomplete Kawasaki disease should be taken into consideration in case of all children with unexplained fever for more than 5 days, associated with 2 or 3 of the main clinical findings of Kawasaki disease. The diagnosis of incomplete Kawasaki disease is based on echocardiographic findings indicating the involvement of the coronary arteries. Cardiac complications, mostly coronary artery aneurysm, can occur in 20% to 25% of untreated patients and in 4% of treated patients. CASE REPORT. In this report we present a case of atypical Kawasaki disease in a 3.5-month-old infant. As soon as the diagnosis was made, the patient received high doses of intravenous immunoglobulin, with the initial introduction of ibuprofen, then aspirin with a good clinical response. Due to the presence of aneurysm of coronary arteries, further therapy involved aspirin and clopidogrel over the following 3 months, and then only aspirin for 2 years. There was a gradual regression of the changes in the coronary blood vessels to the normalization of the echocardiographic findings after 2 years. Kawasaki disease is the second most common vasculitis of childhood, so it should be included in the differential diagnosis for any child with a prolonged unexplained fever. Atypical Kawasaki disease should be taken into consideration in cases when not all clinical criteria are present but coronary abnormalities are documented.

  10. Revisiting the role of steroids and aspirin in the management of acute Kawasaki disease.

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    Dhanrajani, Anita; Yeung, Rae S M

    2017-09-01

    Kawasaki disease is an acute multisystem childhood vasculitis with a predilection for the coronary arteries. The role of corticosteroids and acetyl salicylic acid (ASA) in the treatment of acute Kawasaki disease are matters of ongoing debate and changing attitudes from one extreme to the other. Recent work has provided new evidence to guide our thinking about these two therapeutic agents, which will be the focus of this review. Corticosteroids are effective and well tolerated in Kawasaki disease, both as initial adjunctive treatment in those at high-risk for poor outcome, and as rescue therapy after failed intravenous immunoglobulin (IVIG).Higher doses of ASA (> 30 mg/kg/day) in the acute phase of Kawasaki disease, have no clear benefit over antiplatelet doses in improving coronary outcome. Corticosteroids should be used in patients at high-risk for poor coronary outcome, and in patients who fail IVIG. The absence of widely applicable and validated risk-scoring systems in Kawasaki disease outside of Japan remains a limiting factor to identify high-risk children. Current evidence does not demonstrate any advantage of high-dose over low-dose ASA in the acute phase of Kawasaki disease, in preventing coronary artery aneurysms.

  11. Kawasaki Disease (For Parents)

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    ... Teaching Kids to Be Smart About Social Media Kawasaki Disease KidsHealth > For Parents > Kawasaki Disease Print A A A What's in this ... Complications Diagnosis Treatment en español La enfermedad de Kawasaki Kawasaki disease is an illness that involves the ...

  12. Evidence of Acute Mycoplasma Infection in a Patient with Incomplete and Atypical Kawasaki Disease: A Case Report

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    M. Ebrahim

    2011-01-01

    Full Text Available The etiology of Kawasaki disease remains unknown despite extensive studies. Some researchers suggest that it is caused by an infectious agent. This is a case report where a patient with incomplete Kawasaki disease was found to have evidence compatible with acute Mycoplasma pneumoniae infection. This is one of the several case reports linking Mycoplasma pneumoniae to Kawasaki disease as a possible trigger. This is perhaps due to a superantigen or is mediated by some other mechanism. Accurate and timely testing for Mycoplasma infections is difficult and has its limitations. Despite this, Mycoplasma pneumoniae should be considered in the differential and workup for Kawasaki disease.

  13. How Is Kawasaki Disease Diagnosed?

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    ... page from the NHLBI on Twitter. How Is Kawasaki Disease Diagnosed? Kawasaki disease is diagnosed based on your child's signs ... are the first to suspect a child has Kawasaki disease. Pediatricians are doctors who specialize in treating ...

  14. Kawasaki disease and acute haemolytic anaemia after two IVIG infusions

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    Albena Telcharova-Mihaylovska

    2016-05-01

    Full Text Available Kawasaki disease (KD is one of the most common vasculitis disorders of childhood, affecting predominantly medium-sized arteries, particularly the coronary arteries. For treatment, high-dose intravenous immunoglobulin (IVIG is indicated. IVIG infusions are usually safe and well tolerated even though serious complications can be observed. We present a brief overview of KD and report a two-year-old girl with KD and two IVIG infusions (Gammagard® because of persistent fever after the completion of the first IVIG. Haemolytic anaemia developed after IVIG retreatment. The direct antiglobulin test after haemolysis was positive. The etiology of the haemolysis was related to the presence of transient, passively acquired antibodies that cause a direct antibody-mediated attack. There are few reports of haemolytic anaemia after IVIG infusions. The haemolysis in KD is dose-dependent and occurs more frequently after the second IVIG dose. Non-0 blood group patients are at greater risk. Another factor increasing the risk of haemolysis is also the presence of anaemia due to inflammation in KD.

  15. Primary percutaneous coronary intervention for acute myocardial infarction in a pediatric patient with giant coronary aneurysm due to Kawasaki disease.

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    Mongiovì, Maurizio; Alaimo, Annalisa; Vernuccio, Federica; Pieri, Daniele

    2014-01-01

    We report a case of acute myocardial infarction in an 8-year-old boy with a history of Kawasaki disease and giant coronary aneurysms in the right and left coronary arteries. We performed coronary angiography and percutaneous coronary intervention 4 hours after the onset of symptoms. This case suggests that primary percutaneous coronary intervention might be safe and effective in the long-term treatment of acute myocardial infarction due to coronary sequelae of Kawasaki. © 2013 Wiley Periodicals, Inc.

  16. Acute myocardial infarction in a 35-year-old man with coronary artery aneurysm most probably caused by Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    Saeed Alipour Parsa; Isa Khaheshi; Koosha Paydary; Habib Haybar

    2014-01-01

    We present a 35-year-old man with history of Kawasaki disease who referred with myocardial infarction, and angiography, revealing aneurysm of left main and left anterior descending coronary arteries. The patient underwent percutaneous coronary intervention and thrombectomy and was discharged after 6 d. Coronary artery sequels of Kawasaki disease should be considered as one of the underlying causes of acute myocardial infarction in young adults.

  17. Th17- and Treg-related cytokine and mRNA expression are associated with acute and resolving Kawasaki disease.

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    Guo, M M-H; Tseng, W-N; Ko, C-H; Pan, H-M; Hsieh, K-S; Kuo, H-C

    2015-03-01

    Kawasaki disease is a vasculitis most commonly afflicting children Kawasaki disease. Blood samples were obtained from 186 children with Kawasaki disease at 24 h before IVIG therapy, followed by 3 days and 21 days after IVIG therapy. Thirty children with an acute febrile infectious disease and 30 healthy children were obtained as control. Plasma levels of Th17- and Treg-related cytokines including IL-6, IL-17A, IL-10, TGF-β, and mRNA expression levels of RORγt and Foxp3 were tested. Patients with Kawasaki disease had higher levels of plasma IL-17A (25.35 ± 3.21 vs 7.78 ± 1.78 pg/ml, P Kawasaki disease was associated with higher IL-17A and IL-6, a cytokine profile similar to other autoimmune diseases. IVIG therapy resulted in increased expression of Treg-related FoxP3. IVIG resistance was associated with higher levels of IL-10 and IL-17A. Our findings provide further evidence that Kawasaki disease is an autoimmune-like disease. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  18. What Are the Signs and Symptoms of Kawasaki Disease?

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    ... Twitter. What Are the Signs and Symptoms of Kawasaki Disease? Major Signs and Symptoms One of the main symptoms during the early part of Kawasaki disease, called the acute phase, is fever. The ...

  19. Percutaneous coronary intervention for acute myocardial infarction in a pediatric patient with coronary aneurysm and stenosis due to Kawasaki disease.

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    Drossner, David M; Chappell, Clay; Rab, Tanveer; Kim, Dennis

    2012-06-01

    We report the case of an acutely ill 3-year-old female, with a previous medical history of Kawasaki disease, who presented to care with an acute myocardial infarction. We describe the coordinated therapies employed by pediatric and adult cardiologists aimed to establish coronary revascularization.

  20. Oxidative stress and Kawasaki disease: how is oxidative stress involved from the acute stage to the chronic stage?

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    Yahata, Tomoyo; Hamaoka, Kenji

    2017-01-01

    Inflammation and oxidative stress are closely related. Further, oxidative stress plays an important role in the pathology of inflammation-based Kawasaki disease. An excessive in vivo production of reactive oxygen species increases oxidative stress in the body, which triggers an endless vicious spiral of inflammation reactions and reactive oxygen metabolites. This presumably forms diffuse vasculitis in the acute phase. Acute inflammation and oxidative stress can be rapidly controlled by treatments; however, they may remain for a long time. This has recently been identified as a problem in the chronic phase of Kawasaki disease. Generally, the presence of vascular inflammation and oxidative stress impairs blood vessels, leading to the onset of atherosclerosis, which is a widely recognized risk. The current discussion focuses on whether the same is valid for blood vessels in the chronic phase of Kawasaki disease.

  1. N-terminal pro-brain natriuretic peptide can be an adjunctive diagnostic marker of hyper-acute phase of Kawasaki disease.

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    Kwon, Hyuksool; Lee, Jin Hee; Jung, Jae Yun; Kwak, Young Ho; Kim, Do Kyun; Jung, Jin Hee; Chang, Ikwan; Kim, Kyuseok

    2016-12-01

    The purpose of this study was to determine whether the serum N-terminal pro-brain natriuretic peptide (NT-proBNP) level could be a useful marker for Kawasaki disease in the pediatric emergency department (PED) and in the presence of fever duration of 4 days or less (hyper-acute phase of Kawasaki disease). Medical records of patients who were 1 month to 15 years old of age and presented at the PED with suspected Kawasaki disease from January 1, 2010, to December 31, 2014, were collected retrospectively. Two hundred thirty-nine patients with a history of fever for 4 days or less were diagnosed with Kawasaki disease, as well as 111 patients with other febrile diseases, and were enrolled. The NT-proBNP level was significantly higher in patients with Kawasaki disease (Kawasaki disease vs. other febrile disease group, 444.8 (189.7-951.5) vs. 153.4 (68.9-287.6) pg/mL; p Kawasaki disease was 0.763 (95 % CI 0.712-0.814). NT-proBNP might be an adjunctive laboratory marker for hyper-acute phase of Kawasaki disease in the PED. What is Known: • N-terminal pro-brain natriuretic peptide level has been reported as a useful marker for diagnosis in patients with the acute phase of Kawasaki disease. • But, in the cases of less than 5 days of fever, the appropriate level of NT-proBNP for differentiating Kawasaki disease in PED has not been yet evaluated. What is New: • NT-proBNP might be an adjunctive laboratory marker for hyper-acute phase of Kawasaki disease.

  2. [Clinical utility of ulinastatin, urinary protease inhibitor in acute Kawasaki disease].

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    Saji, Tsutomu

    2008-02-01

    Ulinastatin, a trypsin inhibitor, is useful as a first-line or a second-line treatment regimen including alternative therapy for IVIG-resistant or IVIG nonresponder Kawasaki disease (KD) patients. Mechanisms involving protections against tissue organs and endthelial cell and anti-inflammatory effects by ulinastatin, are dependent on the inhibition of PMN-derived elastase, tumor necrosis factor alpha (TNFalpha), and other proinflammatory cytokines/interleukins(IL-1, IL-6, IL-8). Ulinastatin also suppresses the activation of PMN cells, macrophages, and platelets. Although almost no statistical data related to the definitive effect in acute stage of KD, ulinastatin have shown possible effects, but not always, in a part of KD patients. The indications of clinical use include shock and pancreatitis. Off-label uses of ulinastatin have been reported in hematological, hepatic, renal, OB/Gy diseases and cardiovascular diseases including vasculitis syndromes. The efficacy of ulinastatin in aKD remained to be investigated.

  3. Serum adipokine profiles in Kawasaki disease.

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    Kemmotsu, Yasushi; Saji, Tsutomu; Kusunoki, Natsuko; Tanaka, Nahoko; Nishimura, Chiaki; Ishiguro, Akira; Kawai, Shinichi

    2012-02-01

    Adipokines are cytokines derived from adipose tissue. Recently it has been established that adipokines are closely linked to the pathophysiology of not only metabolic diseases, such as diabetes mellitus, obesity, and atherosclerosis, but also to inflammation and immune diseases. In this study we measured serum levels of adipokines in patients with acute Kawasaki disease to investigate the role of adipokines in the pathophysiology of Kawasaki disease. Serum resistin, high-molecular-weight (HMW) adiponectin, leptin, and visfatin levels were measured by enzyme-linked immunosorbent assay in a total of 117 subjects: 56 patients with acute Kawasaki disease, 30 healthy children, and 31 patients with acute infectious diseases. Serum resistin levels in patients with Kawasaki disease were significantly higher than those of healthy children and patients with acute infectious diseases. In contrast, mean serum HMW adiponectin, leptin, and visfatin levels in patients with Kawasaki disease exhibited no statistically significant differences compared with those in healthy children and patients with infectious diseases. Serum resistin levels decreased significantly after administration of intravenous immune globulin. Serum resistin levels on admission were significantly higher in nonresponders compared with responders to intravenous immune globulin therapy. A multivariate model revealed that C-reactive protein was a factor that was significantly related to elevated serum resistin level in patients with Kawasaki disease. In patients with Kawasaki disease, serum resistin levels were elevated, but decreased to nearly normal after intravenous administration of immune globulin. In contrast, serum HMW adiponectin, leptin, and visfatin levels showed no statistically significant changes. These findings suggest that resistin plays an important role, while other adipokines do not play a major role, in the pathogenesis of Kawasaki disease.

  4. Kawasaki disease shock syndrome: a rare and severe complication of Kawasaki disease.

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    Çakan, Mustafa; Gemici, Hakan; Aktay-Ayaz, Nuray; Keskindemirci, Gonca; Bornaun, Helen; İkizoğlu, Tarkan; Çeliker, Alpay

    2016-01-01

    Kawasaki disease is an acute systemic vasculitis that occurs most commonly in young children. It affects medium-sized muscular arteries and the coronary arteries are the predominant site of involvement. Morbidity and mortality is generally due to coronary artery aneurysms that develop during the chronic phase. Although it is well known that Kawasaki disease can cause myocarditis, tachycardia and heart failure during acute stage, Kawasaki disease shock syndrome has been recently described. It is characterized by hypotension, signs and symptoms of poor perfusion and a shock-like state. Herein we describe two cases of Kawasaki disease shock syndrome that were treated in the pediatric intensive care unit and followed a course without morbidity or mortality.

  5. Diagnosis and management of kawasaki disease.

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    Saguil, Aaron; Fargo, Matthew; Grogan, Scott

    2015-03-15

    Kawasaki disease is an acute, systemic vasculitis that predominantly affects patients younger than five years. It represents the most prominent cause of acquired coronary artery disease in childhood. In the United States, 19 per 100,000 children younger than five years are hospitalized with Kawasaki disease annually. According to U.S. and Japanese guidelines, Kawasaki disease is a clinical diagnosis. Classic (typical) Kawasaki disease is diagnosed based on the presence of a fever lasting five or more days, accompanied by four out of five findings: bilateral conjunctival injection, oral changes such as cracked and erythematous lips and strawberry tongue, cervical lymphadenopathy, extremity changes such as erythema or palm and sole desquamation, and polymorphous rash. Incomplete (atypical) Kawasaki disease occurs in persons with fever lasting five or more days and with two or three of these findings. Transthoracic echocardiography is the diagnostic imaging modality of choice to screen for coronary aneurysms, although other techniques are being evaluated for diagnosis and management. Treatment for acute disease is intravenous immunoglobulin and aspirin. If there is no response to treatment, patients are given a second dose of intravenous immunoglobulin with or without corticosteroids or other adjunctive treatments. The presence and severity of coronary aneurysms and obstruction at diagnosis determine treatment options and the need, periodicity, and intensity of long-term cardiovascular monitoring for potential atherosclerosis.

  6. Transmission of acute infectious illness among cases of Kawasaki disease and their household members.

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    Tsai, Hsing-Chen; Chang, Luan-Yin; Lu, Chun-Yi; Shao, Pei-Lan; Fan, Tsui-Yen; Cheng, Ai-Ling; Hu, Jen-Jan; Yeh, Shu-Jen; Chang, Chien-Chih; Huang, Li-Min

    2015-01-01

    Kawasaki disease (KD) is a disease of unknown cause and the causative agent is most likely to be infectious in nature. To investigate the household transmission pattern of infectious illness and etiology, we thus initiated a prospective case and household study. We enrolled KD cases and their household members from February 2004 to September 2008. The KD cases and their household members accepted questionnaire-based interviews of the contact history, signs of infection, and symptoms to check whether clusters of infectious illness occurred. A total of 142 KD cases and 561 household members were enrolled. Among the 142 KD cases, 136 cases (96%) were typical KD, and six (4%) were atypical KD. Of the 561 household members, 17% were siblings, 46% were parents, 18% were grandparents, and the others were cousins or babysitters. Prior to the onset of their KD illness, 66% (94/142) KD cases had contact with ill household members. On the same day of the onset of KD cases' illness, 4% (6/142) KD cases had household members with illness. After KD cases' disease onset, 70% (100/142) KD cases had at least one other family member with illness. Overall, 61% (343/561) of all the household members had acute infectious illness during KD cases' acute stage, and 92% (130/142) of the families had clusters of infectious illness. A total of 66% KD cases had positive contact with ill household members prior to their disease onset and 92% of families had clusters of infectious illness, so KD is strongly associated with infections. Copyright © 2014. Published by Elsevier B.V.

  7. Persistent high fever for more than 10 days during acute phase is a risk factor for endothelial dysfunction in children with a history of Kawasaki disease.

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    Mori, Yasuhiko; Katayama, Hiroshi; Kishi, Kanta; Ozaki, Noriyasu; Shimizu, Tatsuo; Tamai, Hiroshi

    2016-07-01

    Endothelial dysfunction has previously been reported in children with a history of Kawasaki disease, but the determinants of endothelial function in Kawasaki disease patients are still unknown. In this study, we investigated endothelial function in Kawasaki disease patients and attempted to identify risk factors for persistent endothelial dysfunction. Using high-resolution ultrasound, we measured the percent flow-mediated dilatation, an arterial response to reactive hyperemia, to evaluate endothelial function in 67 patients with a history of Kawasaki disease and 28 age- and sex-matched control subjects. We divided the Kawasaki disease patients into a group with impaired endothelial function (the percent flow-mediated dilatation below -2 standard deviations of the control group) and a group with normal endothelial function (the percent flow-mediated dilatation more than -2 standard deviations of control). Logistic multiple regression analysis was performed to identify independent predictors of impaired endothelial function. In Kawasaki disease patients, the percent flow-mediated dilatation was significantly lower than in the control subjects (9.8±3.6%, compared with 13.1±3.4%, pKawasaki disease patients (3 patients with coronary artery lesions and 10 patients without coronary artery lesions), the percent flow-mediated dilatation was below -2 standard deviations of control. Logistic multiple regression analysis showed that a febrile period of longer than 10 days during the acute phase was the significant risk factor for endothelial dysfunction (odds ratio: 8.562; 95% confidence interval: 1.366-53.68). Presence of coronary artery lesions was not a determinant of endothelial dysfunction. Systemic endothelial dysfunction exists in children with a history of Kawasaki disease, and a febrile period of longer than 10 days during the acute phase is an independent predictor of endothelial dysfunction irrespective of coronary artery involvement. Copyright © 2015 Japanese

  8. Vaccines and Kawasaki disease.

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    Esposito, Susanna; Bianchini, Sonia; Dellepiane, Rosa Maria; Principi, Nicola

    2016-01-01

    The distinctive immune system characteristics of children with Kawasaki disease (KD) could suggest that they respond in a particular way to all antigenic stimulations, including those due to vaccines. Moreover, treatment of KD is mainly based on immunomodulatory therapy. These factors suggest that vaccines and KD may interact in several ways. These interactions could be of clinical relevance because KD is a disease of younger children who receive most of the vaccines recommended for infectious disease prevention. This paper shows that available evidence does not support an association between KD development and vaccine administration. Moreover, it highlights that administration of routine vaccines is mandatory even in children with KD and all efforts must be made to ensure the highest degree of protection against vaccine-preventable diseases for these patients. However, studies are needed to clarify currently unsolved issues, especially issues related to immunologic interference induced by intravenous immunoglobulin and biological drugs.

  9. Kawasaki Disease: A Clinician’s Update

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    Nathan Jamieson

    2013-01-01

    Full Text Available Aims. Kawasaki disease is an acute systemic vasculitis and is the most common cause of acquired heart disease in children in the developed world. This review aims to synthesise recent insights into the disease and provide an update for clinicians on diagnostic and treatment practices. Methods. We conducted a review of the literature exploring epidemiology, aetiology, diagnosis, and management of Kawasaki disease. We searched MEDLINE, Medline In-Process, Embase, Google Scholar, and reference lists of relevant articles. Conclusions. Kawasaki disease is a febrile vasculitis which progresses to coronary artery abnormalities in 25% of untreated patients. The disease is believed to result from a genetically susceptible individual’s exposure to an environmental trigger. Incidence is rising worldwide, and varies widely across countries and within different ethnic groups. Diagnosis is based on the presence of fever in addition to four out of five other clinical criteria, but it is complicated by the quarter of the Kawasaki disease patients with “incomplete” presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%. Given its severe morbidity and potential mortality, Kawasaki disease should be considered as a potential diagnosis in cases of prolonged paediatric fever.

  10. Kawasaki disease: etiopathogenesis and novel treatment strategies.

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    Agarwal, Shreya; Agrawal, Devendra K

    2017-03-01

    Kawasaki disease is an acute febrile systemic vasculitis that predominantly occurs in children below five years of age. Its etiopathogenesis is still not clear, but it is thought to be a complex interplay of genetic factors, infections and immunity. Areas covered: This review article discusses in detail Kawasaki disease, with particular emphasis on the recent updates on its pathogenesis and upcoming alternate treatment options. Though self-limiting in many cases, it can lead to severe complications like coronary artery aneurysms and thrombo-embolic occlusions, and hence requires early diagnosis and urgent attention to avoid them. Intravenous immunoglobulin (IVIG) with or without aspirin has remained the sole treatment option for these cases, but 10-15% cases develop resistance to this treatment. Expert commentary: There is a need to develop additional treatment strategies for children with Kawasaki disease. Targeting different steps of pathogenesis could provide us with alternate therapeutic options.

  11. Kawasaki Disease: Complications, Treatment and Prevention

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    ... Peripheral Artery Disease Venous Thromboembolism Aortic Aneurysm More Kawasaki Disease: Complications, Treatment and Prevention Updated:May 8, ... possibility of heart and coronary artery involvement makes Kawasaki disease unpredictable, but these problems usually are not ...

  12. Erythema at BCG Inoculation Site in Kawasaki Disease Patients.

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    Rezai, Mohammad Sadegh; Shahmohammadi, Soheila

    2014-08-01

    Kawasaki disease is an acute, self-limiting childhood systemic vasculitis with unknown etiology. Because there is no diagnostic test for Kawasaki disease, the diagnosis is based on clinical criteria. An important clinical sign that is not included in the classical clinical criteria for Kawasaki disease is a reaction at the Bacille Calmette-Guérin inoculation site that are present in about 30-50% of Kawasaki disease patients. of this review was to highlight the usefulness of the inflammation at the Bacille Calmette-Guérin inoculation site for early diagnosis of Kawasaki disease, we conducted a literature review on Medline in PubMed area, Google scholar, Magiran and Scientific Information Database using the search terms "Kawasaki disease, Erythema, BCG, inoculation site, children, cardiac complications, coronary artery lesion, aneurysm, incomplete Kawasaki in 2013. A total of 15 articles had been found. Erythema at the Bacille Calmette-Guérin inoculation site was found in 49.87% of Kawasaki disease patients. Coronary artery abnormalities were found in 10.3% of cases. According to this review, BCGitis is more prevalent than cervical lymphadenopathy and rash and it can be a useful criterion in the diagnosis of incomplete Kawasaki disease in cases not fulfills the classic criteria of at least four of the five findings.

  13. Genetic polymorphisms in Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    Ho-chang KUO; Wei-chiao CHANG

    2011-01-01

    Kawasaki disease (KD) is an acute febrile systemic vasculitis,and the cause of KD is not well understood.It is likely due to multiple interactions between genes and environmental factors.The development of genetic association and genome-wide association studies (GWAS) has opened an avenue to better understanding the molecular mechanisms underlying KD.A novel ITPKC signaling pathway was recently found to be responsible for the susceptibility to KD.Furthermore,the GWAS demonstrated the functionally related susceptibility loci for KD in the Caucasian population.In the last decade,the identification of several genomic regions linked to the pathogenesis of KD has made a major breakthrough in understanding the genetics of KD.This review will focus on genetic polymorphisms associated with KD and describe some of the possible clinical implications and molecular mechanisms that can be used to explain how genetic variants regulate the pathogenesis in KD.

  14. Update on etio and immunopathogenesis of Kawasaki disease.

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    Takahashi, Kei; Oharaseki, Toshiaki; Yokouchi, Yuki

    2014-01-01

    This review first discusses the pathogenesis of Kawasaki disease based on the results of recently performed studies aimed at identifying Kawasaki disease-susceptibility genes and the results of analyses of the immune system. Following that, we discuss the findings generated using a murine Kawasaki disease arteritis model and speculate regarding the mechanism of Kawasaki disease onset based on immune function aberrations seen in that model. Recent advances in gene analysis studies of Kawasaki disease are contributing not only to prediction of disease susceptibility but also to improving our understanding of the pathogenesis of Kawasaki disease and development of new improved therapies. In addition, Th17/Treg imbalance is observed in patients with acute-phase Kawasaki disease. Th17/Treg imbalance may be an important factor causing disturbed immunological function. IL-17 induced by Th17 cells have proinflammatory properties and act on inflammatory cells, thereby inducing expression of cytokines and chemokines and resulting in tissue inflammation. Kawasaki disease vasculitis may be triggered by aberrant activation of inflammatory cytokines mediated by IL-17 that is produced by Th17 cells that have been activated by some infectious agent(s).

  15. Diagnosis of incomplete Kawasaki disease

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    Jeong Jin Yu

    2012-03-01

    Full Text Available Several authors suggested that the clinical characteristics of incomplete presentation of Kawasaki disease are similar to those of complete presentation and that the 2 forms of presentation are not separate entities. Based on this suggestion, a diagnosis of incomplete Kawasaki disease in analogy to the findings of complete presentation is reasonable. Currently, the diagnosis of incomplete Kawasaki disease might be made in cases with fewer classical diagnostic criteria and with several compatible clinical, laboratory or echocardiographic findings on the exclusion of other febrile illness. Definition of incomplete presentation in which coronary artery abnormalities are included as a necessary condition, is restrictive and specific. The validity of the diagnostic criteria of incomplete presentation by the American Heart Association should be thoroughly tested in the immediate future.

  16. [Use of the urinary trypsin inhibitor ulinastatin for acute Kawasaki disease].

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    Kawamura, Yoichi; Kanai, Takashi; Takeshita, Seiichiro; Nonoyama, Shigeaki

    2014-09-01

    Intravenous immunoglobulin (IVIG) therapy is widely recognized as standard treatment for Kawasaki disease(KD). However, about 20 % of KD patients are resistant to IVIG and are considered to be a high risk group for coronary artery lesions (CAL). Ulinastatin(UTI) is one of the neutrophil elastase inhibitors used for patients with pancreatitis or circulatory shock, and several studies have shown its efficacy for KD. Recently, we demonstrated that initial UTI treatment combined with IVIG decreased the number of patients requiring addi- tional rescue treatment and the occurrence of CAL. In this study, no severe adverse events occurred. Further research and a prospective trial are needed to prove the clinical efficacy and demonstrate the limits of UTI in patients with KD.

  17. [Aftermaths of lesions of coronary arteries in Kawasaki disease].

    Science.gov (United States)

    Vostokova, A A; Grunina, E A; Klemenov, A V

    2016-01-01

    Kawasaki disease, also known as cutaneous-mucous-glandular mucocutaneous glandular syndrome, is acute systemic vasculitis of small-to-medium calibre arteries, frequently involving coronary arteries, affect almost exceptionally children, with reports concerning cases of Kawasaki syndrome in 20-to-30-year-old adults being extremely rare. The most serious manifestation of Kawasaki disease is coronaritis and formation of coronary artery aneurysms. The dynamics of the formed coronary aneurysms and, consequently, the fate of patients may be different. Thrombosis of an aneurysm in the early period of the disease and stenosing of the affected coronary artery later on present possible complications of Kawasaki disease and potential causes of myocardial infection in young adults. Increased risk of coronary artery thromboses in Kawasaki disease is conditioned by a decrease in velocity of blood flow and its turbulent pattern in the aneurysms, endothelial dysfunction due to currently existing or endured coronaritis and thrombocytosis typical of this pathology. Predisposing factors of coronary artery stenosing are unfavourable haemodynamic conditions appearing at the sites of the "entry" and "exit" of the aneurysm. Described herein are two case reports of myocardial infarction, one of which being a complication of an acute case of Kawasaki disease in a 29-year-old patient, with the second one being a consequence of coronary artery stenosing in a 25-year-old patient who had endured Kawasaki disease in his childhood.

  18. Doença de Kawasaki Kawasaki disease

    Directory of Open Access Journals (Sweden)

    Patrícia Aparecida de Castro

    2009-08-01

    Full Text Available A doença de Kawasaki é vasculite sistêmica e aguda de etiologia desconhecida. Constitui a principal causa de doença cardíaca adquirida em crianças nos EUA. Ocorre mais frequentemente em meninos, 80% dos casos em crianças com menos de cinco anos, sendo rara após os oito anos. Pode atingir crianças de todas as raças, tendo maior incidência entre os descendentes asiáticos. Caracteriza-se por febre, conjuntivite bilateral não exsudativa, eritema e edema de língua, lábios e mucosa oral, alterações de extremidades, linfonodomegalia cervical, exantema polimórfico. Aneurismas e estenoses de artérias coronárias são comuns em percentual que varia de 20 a 25% dos pacientes não tratados, podendo posteriormente levar a infarto agudo do miocárdio e morte súbita. O tratamento com imunoglobulina intravenosa é efetivo e deve ser iniciado precocemente a fim de evitar sequelas cardíacas. O desenvolvimento de testes diagnósticos, terapêuticas mais específicas e a prevenção dessa doença potencialmente fatal em crianças dependem dos contínuos avanços na determinação de sua etiopatogenia.Kawasaki disease is a systemic acute vasculitis of unknown etiology. It is the leading cause of acquired heart disease in children in the USA. It occurs more frequently in boys and eighty percent of the cases occur in children under five years of age. The disease rarely occurs after eight years and it can affect children of all races, with higher incidence among Asian descendants. Kawasaki disease is characterized by fever, bilateral non-exudative conjunctivitis, redness and swelling of the tongue, lips and oral mucosa, abnormalities in the extremities, cervical lymph node, and polymorphic exanthema. Aneurysms and stenoses of coronary arteries occur in pproximately 20 to 25% of untreated patients and subsequently can lead to acute myocardial infarction and sudden death. Treatment with intravenous immunoglobulin is effective and should be initiated

  19. T-Helper Cytokine Profiles in Patients with Kawasaki Disease.

    Science.gov (United States)

    Lee, Sang Bum; Kim, Young Hyun; Hyun, Myung Chul; Kim, Yeo Hyang; Kim, Hee Sun; Lee, Young Hwan

    2015-11-01

    Kawasaki disease is an acute systemic vasculitis of which pathogenesis suspected is caused by immune dysregulation. The goal of this study is to evaluate the activation pattern of T helper cell type 1 (Th1) and T helper cell type 2 (Th2) in patients with Kawasaki disease. Prospective study of 60 patients (male 36, female 24) with diagnosis of Kawasaki disease were enrolled. One hundred and eighty blood samples from these patients were collected according to the different clinical stages {before initial intravenous immunoglobulin (IVIG), 5 days after initial IVIG, 2 months after initial IVIG}. The plasma level of Th1 cytokines; interferon-gamma (IFN-γ) & interleukin (IL)-2 and Th2 cytokines; IL-4 & IL-10 were measured by enzyme-liked immunosorbent assay. In all patients, the plasma level of Th1 cytokines (IFN-γ, IL-2) and Th2 cytokines (IL-4 and IL-10) were markedly elevated during the acute stage of Kawasaki disease. Since then, the plasma level of all these cytokines decreased significantly along with the process of clinical stages. Regardless of the existence of coronary artery lesion or no response to initial IVIG treatment, there were no significant differences between them. These data suggest that both Th1 and Th2 cells may be activated simultaneously during the acute stage of Kawasaki disease. Further studies are therefore required to establish the difference of activation pattern of T helper cells between Kawasaki disease and other inflammatory diseases.

  20. [Cardiac and extracardiac complications in Kawasaki disease].

    Science.gov (United States)

    Kamada, Masahiro

    2014-09-01

    In terms of frequency and prognosis, discussions of the complications of Kawasaki disease have focused on coronary artery aneurysms and stenosis. However, as revealed by autopsy findings, medium and small muscular arteries in various organs could be injured in patients with Kawasaki disease, leading to the development of a variety of complications. Accurate diagnosis of Kawasaki disease can be especially difficult when incomplete Kawasaki disease is accompanied by rare complications involving the central nervous system, digestive system, and other systems in the body, which leads to delay in treatment and subsequent development of coronary artery aneurysms and delayed improvement of the existing complications. Doctors who treat Kawasaki disease should be familiar with the rare but important complications of Kawasaki disease.

  1. Cardiac magnetic resonance feature tracking in Kawasaki disease convalescence.

    Science.gov (United States)

    Bratis, Konstantinos; Hachmann, Pauline; Child, Nicholas; Krasemann, Thomas; Hussain, Tarique; Mavrogeni, Sophie; Botnar, Rene; Razavi, Reza; Greil, Gerald

    2017-01-01

    The objective of this study was to determine whether left ventricular (LV) myocardial deformation indices can detect subclinical abnormalities in Kawasaki disease convalescence. We hypothesized that subclinical myocardial abnormalities due to inflammation represent an early manifestation of the disease that persists in convalescence. Myocardial inflammation has been described as a global finding in the acute phase of Kawasaki disease. Despite normal systolic function by routine functional measurements, reduced longitudinal strain and strain rate have been detected by echocardiography in the acute phase. Peak systolic LV myocardial longitudinal, radial, and circumferential strain and strain rate were examined in 29 Kawasaki disease convalescent patients (15 males; mean [standard deviation] age: 11 [6.6] years; median interval from disease onset: 5.8 [5.4] years) and 10 healthy volunteers (5 males; mean age: 14 [3.8] years) with the use of cardiac magnetic resonance (CMR) feature tracking. Routine indices of LV systolic function were normal in both groups. Comparisons were made between normal controls and (i) the entire Kawasaki disease group, (ii) Kawasaki disease subgroup divided by coronary artery involvement. Average longitudinal and circumferential strain at all levels was lower in patients compared to normal controls. In subgroup analysis, both Kawasaki disease patients with and without a history of coronary involvement had similar longitudinal and circumferential strain at all levels and lower when compared to controls. There were lower circumferential and longitudinal values in Kawasaki disease patients with persisting coronary artery lesions when compared to those with regressed ones. In this CMR study in Kawasaki disease convalescent patients with preserved routine functional indices, we detected lower circumferential and longitudinal strain values compared to normal controls, irrespective of the coronary artery status.

  2. Examination of the findings on {sup 201}Tl, {sup 123}I-BMIPP dual cardiac SPECT in acute phase of Kawasaki disease

    Energy Technology Data Exchange (ETDEWEB)

    Koga, Yutaka [Matsushita-Kotobuki Electronics Industries Ltd., Ohzu, Ehime (Japan); Ishizuka, Takehiro; Otabe, Hiroyuki; Kori, Takeo; Ota, Toshiko; Suzuki, Kazushige

    1999-02-01

    In 42 cases with acute phase of Kawasaki disease (3-27 days since pathopoiesis), {sup 201}Tl, {sup 123}I-BMIPP dual cardiac SPECT was performed and the reduction of the isotope-uptake was macroscopically assessed. On cardiac SPECT, 28 of 42 cases (67%), and 19 of 30 cases (63%) who were normal on electrocardiogram, echocardiogram and blood CK levels showed abnormal images. In a part of cases showing abnormal cardiac SPECT, coronary arteriography was performed, and constrictive lesions with more than 25% were not observed. In acute phase of Kawasaki disease, the risk of cardiac complication was thought to be low. But these results suggest that there are cardiac complications detectable by {sup 201}Tl, {sup 123}I-BMIPP dual cardiac SPECT. This cardiac lesions were thought to be intramyocardinal microangiitis or abnormal coronary micro circulation cased by myocarditis associating microangiitis. (K.H.)

  3. Examination of the findings on [sup 201]Tl, [sup 123]I-BMIPP dual cardiac SPECT in acute phase of Kawasaki disease

    Energy Technology Data Exchange (ETDEWEB)

    Koga, Yutaka (Matsushita-Kotobuki Electronics Industries Ltd., Ohzu, Ehime (Japan)); Ishizuka, Takehiro; Otabe, Hiroyuki; Kori, Takeo; Ota, Toshiko; Suzuki, Kazushige

    1999-02-01

    In 42 cases with acute phase of Kawasaki disease (3-27 days since pathopoiesis), [sup 201]Tl, [sup 123]I-BMIPP dual cardiac SPECT was performed and the reduction of the isotope-uptake was macroscopically assessed. On cardiac SPECT, 28 of 42 cases (67%), and 19 of 30 cases (63%) who were normal on electrocardiogram, echocardiogram and blood CK levels showed abnormal images. In a part of cases showing abnormal cardiac SPECT, coronary arteriography was performed, and constrictive lesions with more than 25% were not observed. In acute phase of Kawasaki disease, the risk of cardiac complication was thought to be low. But these results suggest that there are cardiac complications detectable by [sup 201]Tl, [sup 123]I-BMIPP dual cardiac SPECT. This cardiac lesions were thought to be intramyocardinal microangiitis or abnormal coronary micro circulation cased by myocarditis associating microangiitis. (K.H.)

  4. Erythema Marginatum as an Unusual Presentation for Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    I. Sedighi

    2008-10-01

    Full Text Available Introduction: Kawasaki disease is an acute, systemic vacuity that predominantly manifests in infants and young children. Severe systemic inflammation and vacuities can cause cardiovascular involvement, particularly coronary artery injury that includes dilatation or aneurysm. The final goal of treatment for acute Kawasaki disease، therefore، is to diminish systemic inflammation and vacuities as soon as possible, thereby, preventing progression to coronary artery injury and thrombosis.Case Report: In this report we present a case of Kawasaki disease in a 7-year-old boy with prolonged fever and cervical adenopathy presented with erythema marginatum as a first skin presentation. We treated the patient with intravenous gamma-globulin (IVIG by six day after admission. He developed cardiac and pulmonary involvement two days later.Conclusion: Any skin rash is described in Kawasaki disease except for bullous and vesicular lesions. The common skin manifestation of Kawasaki disease are erythematous and maculopapular rash, although it may appear urticarial, scarlatiniform, erythema multiforme-like, or as erythroderma. Erythema marginatum had a limited differential diagnosis in pediatric patients. The major cause of erythema marginatum is rheumatic fever but there is no report of erythema marginatum in face and cervical region and Kawasaki disease should be considered in differential diagnosis of erythema marginatum in face and neck.

  5. Kawasaki disease: A brief history.

    Science.gov (United States)

    Burns, J C; Kushner, H I; Bastian, J F; Shike, H; Shimizu, C; Matsubara, T; Turner, C L

    2000-08-01

    Tomisaku Kawasaki published the first English-language report of 50 patients with Kawasaki disease (KD) in 1974. Since that time, KD has become the leading cause of acquired heart disease among children in North America and Japan. Although an infectious agent is suspected, the cause remains unknown. However, significant progress has been made toward understanding the natural history of the disease and therapeutic interventions have been developed that halt the immune-mediated destruction of the arterial wall. We present a brief history of KD, review progress in research on the disease, and suggest avenues for future study. Kawasaki saw his first case of KD in January 1961 and published his first report in Japanese in 1967. Whether cases existed in Japan before that time is currently under study. The most significant controversy in the 1960s in Japan was whether the rash and fever sign/symptom complex described by Kawasaki was connected to subsequent cardiac complications in a number of cases. Pathologist Noboru Tanaka and pediatrician Takajiro Yamamoto disputed the early assertion of Kawasaki that KD was a self-limited illness with no sequelae. This controversy was resolved in 1970 when the first Japanese nationwide survey of KD documented 10 autopsy cases of sudden cardiac death after KD. By the time of the first English-language publication by Kawasaki in 1974, the link between KD and coronary artery vasculitis was well-established. KD was independently recognized as a new and distinct condition in the early 1970s by pediatricians Marian Melish and Raquel Hicks at the University of Hawaii. In 1973, at the same Hawaiian hospital, pathologist Eunice Larson, in consultation with Benjamin Landing at Los Angeles Children's Hospital, retrospectively diagnosed a 1971 autopsy case as KD. The similarity between KD and infantile periarteritis nodosa (IPN) was apparent to these pathologists, as it had been to Tanaka earlier. What remains unknown is the reason for the

  6. Atypical Kawasaki disease and gastrointestinal manifestations

    OpenAIRE

    Singh, Ravinder; Ward, Colin; Walton, Mark; Persad, Rabin

    2007-01-01

    Kawasaki disease is a syndrome that usually occurs in infants and children. It is characterized by an exanthem, enanthem, fever, lymphadenopathy, and polyarteritis of variable severity. The present report describes cases in which an initial presentation of Kawasaki disease included abdominal and gastrointestinal symptomatology.

  7. Purified protein derivative anergy in Kawasaki disease.

    Science.gov (United States)

    Kollmann, T R; Klein, E J; Stefanelli, C B; Marcuse, E K

    2001-01-01

    It was previously reported from Italy that all patients with Kawasaki disease had a positive tuberculin intradermal test. In this study from Seattle, WA, nine patients with Kawasaki disease showed no reaction to intradermal tuberculin. The difference in results might be caused by the different tuberculin products.

  8. Atypical Kawasaki disease and gastrointestinal manifestations

    Science.gov (United States)

    Singh, Ravinder; Ward, Colin; Walton, Mark; Persad, Rabin

    2007-01-01

    Kawasaki disease is a syndrome that usually occurs in infants and children. It is characterized by an exanthem, enanthem, fever, lymphadenopathy, and polyarteritis of variable severity. The present report describes cases in which an initial presentation of Kawasaki disease included abdominal and gastrointestinal symptomatology. PMID:19030366

  9. Quantitative evaluation of myocardial fibrosis by cardiac integrated backscatter analysis in Kawasaki disease.

    Science.gov (United States)

    Xie, Lijian; Wang, Renjian; Huang, Min; Zhang, Yongwei; Shen, Jie; Xiao, Tingting

    2016-01-12

    Kawasaki disease is an acute, systemic vasculitis that affects the coronary arteries. However, the relationship between myocardial fibrosis and Kawasaki disease has been completely unknown until now. We aimed to provide quantitative information about myocardial fibrosis using cardiac integrated backscatter in Han race Kawasaki disease patients. Ninety Kawasaki disease patients and 90 healthy control subjects were recruited. Based on Kawasaki disease status, the patients were categorized into 3 groups: acute, subacute, and convalescence phase. Based on coronary artery status, the Kawasaki disease patients were categorized into 3 groups: without coronary artery lesions, with coronary artery dilation, and with coronary artery aneurysms. All subjects underwent two-dimensional and Doppler examinations to measure clinical echocardiographic parameters. Myocardial fibrosis was detected with calibrated integrated backscatter imaging. Left ventricle systolic functions were normal in both the Kawasaki disease and control participants. The myocardial calibrated integrated backscatter values of the left ventricles of the acute (p Kawasaki disease patients were significantly greater than those of the healthy controls. The left ventricle myocardial calibrated integrated backscatter values were significantly smaller in the Kawasaki disease patients without coronary artery lesions than in the Kawasaki disease patients with coronary artery dilations and coronary artery aneurysms in different phases. The left ventricle myocardial calibrated integrated backscatter results were positively correlated with coronary artery status in the acute (r = 0.331, p Kawasaki disease. Our findings may suggest that myocardial fibrosis occurs during early episodes of Kawasaki disease given uncertainties that exist regarding correlations of calibrated integrated backscatter and myocardial fibrosis.

  10. Kawasaki Disease in India, Lessons Learnt Over the Last 20 Years.

    Science.gov (United States)

    Singh, Surjit; Kawasaki, Tomisaku

    2016-02-01

    Over the last 20 years, Kawasaki disease is being increasingly recognized in India and it may soon replace acute rheumatic fever to become the commonest cause of acquired heart disease amongst children. However, the vast majority of children with Kawasaki disease in India are still not being diagnosed. Diagnosis of Kawasaki disease is based on a constellation of clinical findings which have a typical temporal sequence. All pediatricians must we familiar with the nuances involved in arriving at a diagnosis of Kawasaki disease. With early diagnosis and prompt treatment, the risk of coronary artery abnormalities can be significantly reduced.

  11. Sensorineural hearing loss in patients with Kawasaki disease.

    Science.gov (United States)

    Park, Sun Young; Kim, Young Hyun; Kim, Yeo Hyang; Hyun, Myung Chul; Lee, Young Hwan

    2015-11-01

    Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease. Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated. Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss. The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases.

  12. The effect of TNFalpha blockade in complicated, refractory Kawasaki disease

    DEFF Research Database (Denmark)

    Stenbøg, Elisabeth; Nielsen, Bent Windelborg; Hørlyck, Arne

    2006-01-01

    In Kawasaki disease (KD), a systemic vasculitis of childhood, serum levels of proinflammatory cytokines such as tumour necrosis factor alpha (TNFalpha) are elevated during the acute phase of the disease. Although the majority of children recover completely from a single dose of intravenous...

  13. Sensorineural Hearing Loss Associated with Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    Hitoshi Tanimoto

    2014-04-01

    Full Text Available Kawasaki disease (KD is an acute systemic vasculitis of children characterized by fever, rash, conjunctival hyperemia, oropharyngeal erythema, edema and erythema of the hands and feet, and cervical adenopathy [1]. A variety of other features are also characteristics of this syndrome, including coronary artery aneurysms, urethritis, anterior uveitis, mild hepatobiliary dysfunction, and gallbladder hydrops. Coronary artery abnormalities, including aneurysms and ectasia, occur in approximately 20% of untreated patients [2]. Neurologic involvement is rare. Cranial nerve palsy, especially involving the seventh nerve, has been reported [3], as well as hemiparesis caused by cerebral thrombosis and infarction, and convulsions. Although about 30% of patients with acute KD in the United States have been reported to suffer mild sensorineural hearing loss (SNHL [4], only a few such cases have been reported in Japan. On the other hand, in both countries, a few cases of severe or profound SNHL in children who were in the acute phase of KD have been documented [5].

  14. Segmented Coronary Artery Aneurysms and Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    Hamid Reza Ghaemi

    2011-05-01

    Full Text Available Kawasaki disease (KD is an acute vasculitis syndrome of unknown etiology. It occurs in infants and young children,affecting mainly small and medium-sized arteries, particularly the coronary arteries. Generalized microvasculitis occurs in the first 10 days, and the inflammation persists in the walls of medium and small arteries, especially the coronary arteries, and changes to coronary artery aneurysms.We report the case of a 10-month-old girl referred to our center three months after the onset of disease due to the aneurysmsof the coronary arteries. During the acute phase of her illness, she received 2 gr/kg intravenous gamma globulin; and afterher referral to us, the patient was treated by antiaggregant doses of acetylsalicylic acid (ASA (5 mg/kg and Warfarin (1 mg/daily. At three months’ follow-up, the aneurysms still persisted in the echocardiogram.

  15. [Kawasaki disease: interdisciplinary and intersocieties consensus (clinical guidelines). Brief version].

    Science.gov (United States)

    2016-08-01

    Kawasaki disease is an acute self-limiting systemic vasculitis. It is the most common cause of acquired heart disease, with the risk of developing coronary artery aneurysms, myocardial infarction and sudden death. Diagnosis is based on the presence of fever in addition to other clinical criteria. The quarter of the Kawasaki disease patients have "incomplete" presentation. Treatment with intravenous immunoglobulin within ten days of fever onset improves clinical outcomes and reduces the incidence of coronary artery dilation to less than 5%. Non-responders to standard therapy have shown a successful response with the use of corticosteroids and/or biological agents. The long-term management must be delineated according to the degree of coronary involvement in a multidisciplinary manner. To facilitate the pediatrician's diagnosis, treatment and monitoring of Kawasaki disease, a group of experts from the Argentine Society of Pediatrics and the Argentine Society of Cardiology carried out a consensus to develop practical clinical guidelines.

  16. Marked eosinophilia in a patient with history of severe atypical Kawasaki disease.

    Science.gov (United States)

    O'Byrne, Michael L; Cohen, Meryl S

    2013-01-01

    An infant with recent atypical, treatment-refractory Kawasaki disease presented with marked eosinophilia. Workup failed to identify an etiology. The eosinophilia spontaneously resolved. Eosinophilia has been observed in the acute phase of Kawasaki disease, but has not been reported following recovery. © 2012 Wiley Periodicals, Inc.

  17. Kawasaki disease and immunisation: A systematic review.

    Science.gov (United States)

    Phuong, Linny Kimly; Bonetto, Caterina; Buttery, Jim; Pernus, Yolanda Brauchli; Chandler, Rebecca; Felicetti, Patrizia; Goldenthal, Karen L; Kucuku, Merita; Monaco, Giuseppe; Pahud, Barbara; Shulman, Stanford T; Top, Karina A; Trotta, Francesco; Ulloa-Gutierrez, Rolando; Varricchio, Frederick; de Ferranti, Sarah; Newburger, Jane W; Dahdah, Nagib; Singh, Surjit; Bonhoeffer, Jan; Burgner, David

    2017-03-27

    Kawasaki disease is a complex and potentially serious condition. It has been observed in temporal relation to immunisation. We conducted a systematic literature review using various reference sources to review the available evidence published in the literature. We identified twenty seven publications reporting a temporal association between immunisation and Kawasaki disease. We present a systematic review of data drawn from randomised controlled trials, observational studies, case series and reports, and reviews. Overall there was a lack of standardised case definitions, making data interpretation and comparability challenging. Although a temporal relationship between immunisation and Kawasaki disease is suggested, evidence for an increased risk or a causal association is lacking. Implementation of a standardised Kawasaki disease case definition would increase confidence in the findings and add value to future studies of pre- or post-licensure vaccine safety studies. Copyright © 2016. Published by Elsevier Ltd.

  18. Kawasaki disease: review of 21 cases

    Directory of Open Access Journals (Sweden)

    Cem Arat

    2016-03-01

    Conclusion: Kawasaki disease has difficulty in diagnosis because of having broad spectrum of presenting symptoms. Early diagnosis and treatment is very important in preventing coronary artery abnormalities. [Cukurova Med J 2016; 41(1.000: 97-104

  19. Kawasaki disease - an Indian perspective.

    Science.gov (United States)

    Singh, Surjit; Kawasaki, Tomisaku

    2009-07-01

    Kawasaki disease (KD) was first reported from Japan in 1967 by a young pediatrician, Tomisaku Kawasaki, while working at the Red Cross Hospital in Tokyo. Soon therafter, Marian Melish independently reported children with a similar clinical profile from Hawaii in the United States. KD has now been reported from all parts of the world, including several centers in India. Based on the epidemiology and clinical features, an infectious etiology has been suspected for long but no definitive causative agent has been implicated so far. Like many other vasculitides, the diagnosis of this condition is based on the recognition of a temporal sequence of clinical features, none of which is pathognomonic in isolation. KD is believed to be the commonest vasculitic disorder of children. Incidence rates as high as 60-150 per 100,000 children below 5 years of age have been reported from several countries. In India (as also perhaps in many other developing countries), however, majority of children with KD continue to remain undiagnosed probably because of the lack of awareness amongst pediatricians. The clinical features of KD can be confused with other common conditions like scarlet fever and the Stevens Johnson syndrome, if the clinician is not careful. Development of coronary artery abnormalities (CAA) is the hallmark of KD and accounts for most of the morbidity and mortality associated with the disease. Prompt recognition of the disease and early initiation of treatment with intravenous immunoglobulin (IVIG) results in significant reduction in the occurrence of CAA. It is, therefore, imperative for the pediatrician to diagnose and treat KD expeditiously. KD should be considered in the differential diagnosis of all febrile illnesses in young children where the fever persists for more than 5-7 days.

  20. Angioscopic observation of coronary lesions in Kawasaki disease

    Science.gov (United States)

    Ishikawa, Hiromi

    1993-05-01

    Kawasaki disease is an acute systematic angiitis, specifically affecting the coronary arteries in young children. Coronary aneurysms develop in 10 to 15% of patients with Kawasaki disease. The coronary aneurysm frequently changes into coronary stenosis and obstruction. Hitherto, we could observe and access the severity of these coronary lesions with the use of two dimensional echocardiography and selective coronary angiography. Angiography is the most sensitive means available for diagnosing significant coronary lesions and their severity. However, it is insensitive for detecting intraluminal minute and pathologic changes such as intimal thickening, regeneration of endothelium and thrombus. Recent advance in fiberscopie technology, enabled us to observe the inside of coronary artery percutaneously. We tried to use this angioscopic technique for the observation of coronary lesions in eight patients with Kawasaki disease at chronic stage.

  1. Vascular function long term after Kawasaki disease: another piece of the puzzle?

    Science.gov (United States)

    Pinto, Fátima F; Gomes, Inês; Loureiro, Petra; Laranjo, Sérgio; Timóteo, Ana T; Carmo, Miguel M

    2017-04-01

    Kawasaki disease is an acute systemic vasculitis. Cardiac complications are frequent and include endothelial dysfunction in patients with coronary anomalies. Thus far, endothelial dysfunction in patients with no coronary lesions is poorly understood. Our aim was to access the vascular function in adolescents and young adults long term after Kawasaki disease, but without coronary aneurysms or any other cardiac risk factors. We carried out a single-centre prospective study in a Portuguese population. We evaluated two groups of subjects: (1) Kawasaki disease patients over 11 years of age, diagnosed >5 years ago, 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 assessed. Endo-PAT and carotid intima-media thickness assessment were performed to determine vascular function. In total, 43 Kawasaki disease patients were assessed and compared with 43 controls. Kawasaki disease patients presented a decreased reactive hyperaemia index compared with controls (1.59±0.45 versus 1.98±0.41; pKawasaki disease group. There were no statistically significant changes with regard to laboratory data. Children with Kawasaki disease may have long-term sequelae, even when there is no discernible coronary artery involvement in the acute stage of the disease. Further research is needed to assess whether known strategies to improve endothelial function would bring potential benefits to Kawasaki disease patients.

  2. Giant coronary artery aneurysms complicating Kawasaki disease in Mexican children.

    Science.gov (United States)

    Garrido-García, Luis M; Morán-Villaseñor, Edna; Yamazaki-Nakashimada, Marco A; Cravioto, Patricia; Galván, Fernando

    2017-08-29

    One of the most important complications of Kawasaki disease is the development of giant coronary aneurysms. Risk factors for their development are still not clear. A retrospective analysis was conducted at the National Institute of Paediatrics in Mexico City, Mexico. It included all patients with a diagnosis of acute Kawasaki disease between August, 1995 and August, 2015. Clinical and laboratory findings, as well as echocardiographic measurements, were recorded. Patients with giant coronary aneurysms (z-score⩾10) were compared with the rest of the patients. A value of pKawasaki disease. Of them, 34 developed giant coronary aneurysms during the acute stage of the disease. In the multivariate analysis, patients younger than 1 year, those with a higher duration of illness at the time of diagnosis, and those who received additional intravenous immunoglobulin showed a significantly higher frequency of giant coronary aneurysms. One of the main factors associated with the development of giant coronary aneurysms was the delay in the diagnosis of Kawasaki disease. This finding highlights the importance of maintaining a high suspicion of the disease, which would enable an early diagnosis and prompt treatment and decrease the risk for developing giant coronary aneurysms.

  3. Management of Kawasaki disease in adults.

    Science.gov (United States)

    Denby, Kara J; Clark, Daniel E; Markham, Larry W

    2017-07-27

    Kawasaki disease is the most common childhood vasculitis in the USA and the most common cause of acquired cardiac disease in children in developed countries. Since the vast majority of Kawasaki disease initially presents at Kawasaki disease, making it increasingly relevant for adult cardiologists as this population transitions into adulthood. As the 2017 American Heart Association (AHA) and 2014 Japanese Circulation Society (JCS) guidelines emphasise, Kawasaki disease requires rigorous follow-up with cardiac stress testing and non-invasive imaging to detect progressive stenosis, thrombosis and luminal occlusion that may lead to myocardial ischaemia and infarction. Due to differences in disease mechanisms, coronary disease due to Kawasaki disease should be managed with different pharmacological and non-pharmacological treatment algorithms than atherosclerotic coronary disease. This review addresses gaps in the current knowledge of the disease and its optimal treatment, differences in the AHA and JCS guidelines, targets for future research and obstacles to transition of care from adolescence into adulthood. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  4. Sensorineural hearing loss in Kawasaki disease.

    Science.gov (United States)

    Aggarwal, Varun; Etinger, Veronica; Orjuela, Andres F

    2016-01-01

    Kawasaki disease is a common nonspecific vasculitis seen in childhood. The most significant long-term sequela is coronary artery aneurysm. However, the spectrum of complications involves not only the heart, but also other organs such as the eyes, skin, kidneys, gallbladder, liver, and central nervous system. Sensorineural hearing loss (SNHL) is a relatively unrecognized complication of the disease. Although most of the complications (except coronary artery aneurysm) are self-limiting, SNHL can be persistent. It is, especially important in infants and young children who might not be able to report the hearing deficits and are most likely to have cognitive and speech delays if this hearing loss is not addressed in a timely manner. We report a child with Kawasaki disease who had SNHL during the 2(nd) week of the illness. The aim of this article is to briefly review the pathophysiology behind this hearing loss and strongly emphasize the importance of universal hearing evaluation in all children diagnosed with Kawasaki disease. This screening in children with Kawasaki disease may provide some timely intervention if needed. Since most Kawasaki disease patients will be seen by cardiologists, we hope to create more awareness about this complication to the cardiology community as well.

  5. Sensorineural hearing loss in Kawasaki disease

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    Varun Aggarwal

    2016-01-01

    Full Text Available Kawasaki disease is a common nonspecific vasculitis seen in childhood. The most significant long-term sequela is coronary artery aneurysm. However, the spectrum of complications involves not only the heart, but also other organs such as the eyes, skin, kidneys, gallbladder, liver, and central nervous system. Sensorineural hearing loss (SNHL is a relatively unrecognized complication of the disease. Although most of the complications (except coronary artery aneurysm are self-limiting, SNHL can be persistent. It is, especially important in infants and young children who might not be able to report the hearing deficits and are most likely to have cognitive and speech delays if this hearing loss is not addressed in a timely manner. We report a child with Kawasaki disease who had SNHL during the 2 nd week of the illness. The aim of this article is to briefly review the pathophysiology behind this hearing loss and strongly emphasize the importance of universal hearing evaluation in all children diagnosed with Kawasaki disease. This screening in children with Kawasaki disease may provide some timely intervention if needed. Since most Kawasaki disease patients will be seen by cardiologists, we hope to create more awareness about this complication to the cardiology community as well.

  6. Spontaneous closure of patent ductus arteriosus after an episode of Kawasaki disease: a case report

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    Lin Ming-Chih

    2012-01-01

    Full Text Available Abstract Introduction Kawasaki disease is regarded as systemic vasculitis. Many experts believe that not only coronary arteries but also other small arteries are involved during the period of systemic inflammation. However, the evidence to support this point view is limited. Case presentation We report the case of a one-year-four-month-old Taiwanese girl whose patent ductus arteriosus was incidentally found during an episode of Kawasaki disease. The ductus closed spontaneously after the acute phase of Kawasaki disease. Conclusions In this patient, the patent ductus arteriosus may have closed spontaneously after Kawasaki disease due to its involvement in the generalized vasculitis that this disease incurs. This would support the theory that the vasculitis of Kawasaki disease is limited not only to coronary arteries but also to all medium- sized arteries.

  7. Comparison of sup 99 Tc sup m -HMPAO-labelled white blood cells and sup 67 Ga citrate scans to detect myocarditis in the acute phase of Kawasaki disease

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    Kao, C.H.; Hsieh, K.S.; Wang, Y.L.; Chen, C.W.; Liao, S.Q.; Wang, S.J.; Yeh, S.H. (Taichung Veterans General Hospital, Taiwan (China))

    1991-11-01

    Myocardial imaging with {sup 99}Tc{sup m}-HMPAO-labelled white blood cells (WBC) and {sup 67}Ga citrate was used to detect myocarditis in the acute phase of Kawasaki disease among 22 infants and children; 18 cases of myocarditis were detected by {sup 99}Tc{sup m}-HMPAO-labelled WBC heart scans, but only one case was detected by {sup 67}Ga citrate heart scans. In conclusion, {sup 99}Tc{sup m}-HMPAO-labelled WBC scanning provides a more sensitive method than {sup 67}Ga citrate scanning in the detection of myocarditis in Kawasaki disease. (author).

  8. Kawasaki Disease and Peripheral Gangrene in Infancy.

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    Malekzadeh, Iran; Ziaee, Vahid; Sadrosadat, Taravat; Moardinejad, Mohammad-Hassan; Sayadpour-Zanjani, Keyhan

    2015-12-01

    Early diagnosis and treatment of Kawasaki disease as the most common cause of acquired heart disease in childhood, may significantly improve the prognosis. Diagnosing infantile Kawasaki (younger than a year) is difficult because of obscure symptoms; at the same time they are at the higher risk of coronary abnormalities. We report three infants with prolonged (more than 5 days) fever and peripheral gangrene without any other clinical manifestations of Kawasaki disease. Kawasaki was diagnosed due to dilation of coronary artery and other aortic branches, thrombocytosis, and rising of ESR and CRP. All patients were treated with high dose aspirin, IVIG and pulse therapy with methylprednisolone. Additionally, cytotoxic drugs or infliximab were used for two of them because of severe aneurysms in the aortic branches. All 3 patients received aspirin with anti-platelet aggregation dose and 2 patients heparin as an anti-coagulant agent for longtime. After adequate treatment, peripheral gangrene, arterial dilations and aneurysms improved, but during 12 months follow-up coronary aneurysms did not improve completely. Peripheral gangrene must be regarded as an important sign of infantile Kawasaki disease early treatment of which can prevent severe permanent coronary involvements and sequels.

  9. Coronary artery bypass grafting for Kawasaki disease

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    GUO Hong-wei; CHANG Qian; XU Jian-ping; SONG Yun-hu; SUN Han-song; HU Sheng-shou

    2010-01-01

    Background Kawasaki disease (KD) is the leading cause of pediatric ischemic heart disease. The incidence of serious coronary sequelae is low and about 2%-3% of patients with KD, but once myocardial infarction occurs in children, the mortality is quite high and 22% at the first infarction.This study aimed to evaluate the efficacy of coronary artery bypass grafting (CABG) in patients with KD.Methods Eight patients with a history of KD underwent CABG between October 1997 and July 2005. The number of bypass grafts placed was 2 to 4 per patient (mean 2.5±0.8). Various bypass grafts were used in patients, i.e. the left internal mammary artery (LIMA) in 3 patients, bilateral internal mammary artery (IMA) in 2 patients, LIMA plus gastroepiploic artery (GEA) in 1 patient and total saphenous vein grafts (SVGs) in 2 patients. The combined procedures included ventricular aneurysmectomy in 1 patient, mitral valve plasty in 1 and right coronary aneurysmectomy in 1. One patient was not able to wean from cardiopulmonary bypass (CPB), after being supported with intra-aortic balloon pump (IABP), the patient was weaned from CPB successfully.Results One patient died of low cardiac output syndrome and acute renal failure 19 days after operation. Other patients recovered and were discharged uneventfully. During the follow-up that ranged from 3 to 57 months (mean 27 months),clincal angina disappeared or improved. Cardiac function was in Class Ⅰ-Ⅱ (NYHA).Conclusion CABG is a safe and effective procedure for Kawasaki coronary artery disease. However long-term results need to be followed up.

  10. Possible involvement of infection with human coronavirus 229E, but not NL63, in Kawasaki disease.

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    Shirato, Kazuya; Imada, Yoshio; Kawase, Miyuki; Nakagaki, Keiko; Matsuyama, Shutoku; Taguchi, Fumihiro

    2014-12-01

    Although human coronavirus (HCoV)-NL63 was once considered a possible causative agent of Kawasaki disease based on RT-PCR analyses, subsequent studies could not confirm the result. In this study, this possibility was explored using serological tests. To evaluate the role of HCoV infection in patients with Kawasaki disease, immunofluorescence assays and virus neutralizing tests were performed. Paired serum samples were obtained from patients with Kawasaki disease who had not been treated with γ-globulin. HCoV-NL63 and two antigenically different isolates of HCoV-229E (ATCC-VR740 and a new isolate, Sendai-H) were examined as controls. Immunofluorescence assays detected no difference in HCoV-NL63 antibody positivity between the patients with Kawasaki disease and controls, whereas the rate of HCoV-229E antibody positivity was higher in the patients with Kawasaki disease than that in controls. The neutralizing tests revealed no difference in seropositivity between the acute and recovery phases of patients with Kawasaki disease for the two HCoV-229Es. However, the Kawasaki disease specimens obtained from patients in recovery phase displayed significantly higher positivity for Sendai-H, but not for ATCC-VR740, as compared to the controls. The serological test supported no involvement of HCoV-NL63 but suggested the possible involvement of HCoV-229E in the development of Kawasaki disease. © 2014 Wiley Periodicals, Inc.

  11. Influenza infection and Kawasaki disease

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    Xijing Huang

    2015-06-01

    Full Text Available INTRODUCTION: The objective of this study was to investigate the possible link between influenza (Flu infection and Kawasaki disease (KD. METHODS: We examined the medical records of 1,053 KD cases and 4,669 influenza infection cases hospitalized at our institute from January 1, 2011 to December 31, 2013. Cases of KD with concomitant influenza infection formed the KD + Flu group. Each KD + Flu case was matched with 2 KD cases and 2 influenza infection cases, and these cases were assigned to the KD group and Flu group, respectively. The differences in the principal clinical manifestations, course of disease, incomplete KD rate, intravenous immunoglobulin (IVIG resistance rate, and echocardiographic detection results between the KD + Flu group and KD group were compared. The fever durations and laboratory test results of these three groups were compared. RESULTS: 1 The seasonal variations of the KD + Flu group, KD group and Flu group were similar. 2 The morbidity rate of incomplete KD was higher in the KD + Flu group compared with the KD group. 3 Patients in the KD + Flu group exhibited a longer time to KD diagnosis compared with patients in the KD group. 4 The KD + Flu group exhibited the longest fever duration among the three groups. 5 The CRP and ESR values in the KD + Flu group were higher those in the Flu or KD groups. CONCLUSIONS: Concomitant influenza infection affects the clinical manifestations of KD and can impact the laboratory test results and the diagnosis and treatment of the disease. However, it remains unclear whether influenza contributes to KD etiology.

  12. Comorbidity of Kawasaki disease and group A streptococcal pleural effusion in a healthy child: a case report

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    Alhammadi AH

    2013-07-01

    Full Text Available Ahmed H Alhammadi, Mohamed A HendausGeneral Pediatrics Section, Department of Pediatrics, Hamad Medical Corporation, Doha, QatarBackground: Kawasaki disease is an acute self-limiting vasculitis that affects children. The most dreaded complication of Kawasaki disease reported in the literature over the years is coronary artery disease, which is considered as the main cause of acquired heart disease. However, pulmonary associations with Kawasaki disease have been overlooked. We present a rare, if not unique, case of Kawasaki disease associated with group A streptococcus pleural effusion in the English language literature. A search of the PubMed database was carried out, using a combination of the terms “Kawasaki disease”, “pneumonia”, and “group A streptococcus”. The majority of studies conducted in children with Kawasaki disease have concentrated on the coronary artery implications. Kawasaki disease is considered a self-limiting illness, but can have detrimental consequences if not diagnosed early. When there is a prolonged inflammatory reaction, with no infectious agent identified or remittent fever unresponsive to antibiotics, Kawasaki disease should be taken into consideration. Elevated Vβ2+ T cells compared with healthy controls suggest possible involvement of a superantigen in the etiology of Kawasaki disease, so it is wise that the health care provider concentrates not only on the cardiac consequences, but also on pulmonary associations.Keywords: Kawasaki disease, pneumonia, group A streptococcus

  13. How Is Kawasaki Disease Treated?

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    ... 11 months before having the measles and chicken pox vaccines. Immune globulin can prevent those vaccines from ... the acute phase of the disease. In a small number of children, the coronary arteries don't ...

  14. Has joint involvement lessened in Kawasaki disease?

    Science.gov (United States)

    Álvarez, Eva Pilar; Rey, Francis; Peña, Sara Carolina; Rubio, Aranzazu; Calvo, Cristina; Collado, Paz

    Kawasaki disease (KD) is an acute systemic vasculitis affecting medium-sized arteries, particularly the coronary arteries. Classic diagnosis is based in prolonged fever and different clinical features, including acute arthritis. Our objective is to determine the prevalence of arthritis at the moment of the diagnosis, the response to intravenous immunoglobulin infusion and the relation with cardiac findings. Retrospective study through review of medical records of 42 patients with KD from 1988 to 2013. Demographic, clinical, laboratory variables and treatment were reviewed. Male sex was predominant (57%). Fever (100%), exanthema (92,9%), conjunctivitis (78,6%), oropharingeal changes (76,2%), cervical lymphadenopathy (71,4%), edema (52,4%) and peripheral desquamation (46,3%) were reported. Eight patients presented ecocardiography alterations (ectasia and aneurism). Acute articular involvement was reported in 7 (16%) patients, including oligoarticular (57%), monoarticular (29%) and polyarticular (14%) patterns. All patients had elevation of acute phase reactants with neutrophilia (57%) and hypoalbuminemia (71,5%), but showed a good therapeutic response to intravenous immunoglobulin, without sequelaes. Sixteen patients had incomplete KD nine males, with 100% of fever exanthema (75%), conjunctivitis (56%) and 50% of cervical lymphadenopathy. Whereas oropharingeal changes and edemas was described in 44% and 25% of them. Four patients with incomplete KD had coronary artery abnormalities. Acute arthritis was an uncommon finding (16%) and resulted in no sequelae. Maybe the treatment with intravenous immunoglobulin and aspirin prevents the development of articular abnormalities and then leading to a decrease in its follow-up requierement by reumathologist. The cardiovascular sequelae, mainly incomplete KD, remains determining its prognosis. The presence of articular involvement seems not to have influence over cardiac involvement. Copyright © 2016 Elsevier España, S

  15. Prediction of responsiveness or non-responsiveness to treatment of acute Kawasaki disease using 1 gram per kilogram of immunoglobulin--an effective and cost-saving schedule of therapy.

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    Ichihashi, Ko; Shiraishi, Hirohiko; Momoi, Mariko

    2009-06-01

    Standard treatment of acute Kawasaki disease involves giving 2 grams per kilogram of immunoglobulin intravenously along with aspirin. More than half of the patients with acute Kawasaki disease, nonetheless, can be cured by giving only 1 gram per kilogram of immunoglobulin, thus reducing this aspect of the cost of treatment by half. Our purpose was to predict those patients with acute Kawasaki disease who would respond to treatment with 1 gram per kilogram of immunoglobulin given intravenously on the basis of their clinical profiles and laboratory findings prior to the initial treatment. We performed a retrospective review of the clinical records of consecutive patients with acute Kawasaki disease treated in our hospital with intravenous immunoglobulin from January, 2001, to December, 2005.During this period, we treated in this fashion 98 patients with acute Kawasaki disease. 65% of these needing immunoglobulin therapy were cured by giving 1 gram per kilogram. The neutrophil count and the percentage of white blood cells representing neutrophils, along with aspirate aminotransferase, alanine aminotransferase, bilirubin and C reactive protein, were all significantly lower, and sodium was significantly higher, in those responding to 1 gram per kilogram of immunoglobulin when compared to those who did not respond. The days of illness at the first intravenous treatment was later in those responding than in those failing to respond. We generated a score for prediction, assigning a point for each of C reactive protein equal to or greater than 10 mg/dl, sodium equal to or lower than 133 meq/l, alanine aminotransferase equal to or greater than 110 IU/l, and 2 points for the percentage of white blood cells representing neutrophils equal to or greater than 70%. Using a cut-off point of a score less than 2, we were able to identify those responding with 60% sensitivity, and 91% specificity.Thus, we are now able to predict those patients with acute Kawasaki disease who will

  16. Kawasaki disease with facial nerve paralysis.

    Science.gov (United States)

    Larralde, Margarita; Santos-Muñoz, Andrea; Rutiman, Ricardo

    2003-01-01

    Kawasaki disease (KD) is a multisystem disorder with varying clinical expression. We describe an instance of facial nerve paralysis in a patient with KD. A 5-month-old boy developed fever, irritability, and diarrhea, treated 8 days later with cefaclor and ibuprofen. Three days later a confluent, erythematous and papular rash appeared, his lips were reddened and swollen, and his white blood count and platelet count were 20,900/mm(3) and 558,000/mm(3), respectively. He was admitted to the hospital with a diagnosis of KD, and an echocardiogram showed a right coronary aneurysm. The patient then developed an acute, right-sided, facial nerve peripheral paralysis that resolved over the next 6 weeks. He was treated with intravenous immune globulin (IVIG) 2 g/kg and aspirin 100 mg/kg/day with improvement of signs and symptoms. This report documents facial nerve paralysis as an uncommon complication of KD and points out that it may be a marker of increased risk of cardiovascular disease in this disorder.

  17. Missed Kawasaki disease in childhood presenting as myocardial infarction in adults

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    Bhagwat, Ajit; Mukhedkar, Sachin; Ekbote, Shriganesh; Gordon, John B.

    2015-01-01

    Kawasaki disease (KD) is an acute, self-limited vasculitis that occurs in young children and was first described by Japanese pediatrician Tomisaku Kawasaki in 1967. Although originally thought to be a rare condition, KD has become the most common cause of acquired heart disease in the pediatric population in developed countries. The majority of patients with KD appear to have a benign prognosis, but a subset of patients with coronary artery aneurysms are at risk for ischemic events and requir...

  18. Comorbidity of Kawasaki disease and group a streptococcal pleural effusion in a healthy child: a case report.

    Science.gov (United States)

    Alhammadi, Ahmed H; Hendaus, Mohamed A

    2013-01-01

    Kawasaki disease is an acute self-limiting vasculitis that affects children. The most dreaded complication of Kawasaki disease reported in the literature over the years is coronary artery disease, which is considered as the main cause of acquired heart disease. However, pulmonary associations with Kawasaki disease have been overlooked. We present a rare, if not unique, case of Kawasaki disease associated with group A streptococcus pleural effusion in the English language literature. A search of the PubMed database was carried out, using a combination of the terms "Kawasaki disease", "pneumonia", and "group A streptococcus". The majority of studies conducted in children with Kawasaki disease have concentrated on the coronary artery implications. Kawasaki disease is considered a self-limiting illness, but can have detrimental consequences if not diagnosed early. When there is a prolonged inflammatory reaction, with no infectious agent identified or remittent fever unresponsive to antibiotics, Kawasaki disease should be taken into consideration. Elevated Vβ2+ T cells compared with healthy controls suggest possible involvement of a superantigen in the etiology of Kawasaki disease, so it is wise that the health care provider concentrates not only on the cardiac consequences, but also on pulmonary associations.

  19. Oral necrotizing microvasculitis in a patient affected by Kawasaki disease.

    Science.gov (United States)

    Scardina, Giuseppe Alessandro; Fucà, Gerlandina; Carini, Francesco; Valenza, Vincenzo; Spicola, Michele; Procaccianti, Paolo; Messina, Pietro; Maresi, Emiliano

    2007-12-01

    Kawasaki disease (KD) was first described in 1967 by Kawasaki, who defined it as "mucocutaneous lymph node syndrome". KD is an acute systemic vasculitis, which mainly involves medium calibre arteries; its origin is unknown, and it is observed in children under the age of 5, especially in their third year. The principal presentations of KD include fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. Within KD, oral mucositis - represented by diffuse mucous membrane erythema, lip and tongue reddening and lingual papillae hypertrophy with subsequent development of strawberry tongue - can occur both in the acute stage of the disease (0-9 days), and in the convalescence stage (>25 days) as a consequence of the pharmacological treatment. KD vascular lesions are defined as systemic vasculitis instead of systemic arteritis. This study analyzed the anatomical-pathological substrata of oral mucositis in a baby affected by Kawasaki disease and suddenly deceased for cardiac tamponade caused by coronary aneurysm rupture (sudden cardiac death of a mechanical type).

  20. An Adult Case of Kawasaki Disease in a Pregnant Japanese Woman: A Case Report

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    Kyoko Kanno

    2011-04-01

    Full Text Available Kawasaki disease is an acute febrile disease predominantly seen in young children. We report a case of Kawasaki disease in a 32-year-old pregnant woman. She developed a generalized erythematous skin rash accompanied by high fever. Bilateral conjunctival congestion, tender cervical lymphadenopathy, an edematous lower lip and peripheral edema followed by desquamation were observed. She was successfully treated with aspirin and intravenous gammaglobulin (1 g/kg/day. Her course was not complicated by coronary artery aneurysm and she delivered a healthy baby. To the best of our knowledge, this is the first case of Kawasaki disease in a pregnant woman. We suggest that Kawasaki disease should be included in the differential diagnosis of a generalized, erythematous skin rash accompanied by high fever in adults.

  1. Extremely refractory Kawasaki disease with disseminated intravascular coagulation.

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    Koh, Young Kwon; Lee, Jae Hee; Park, Yeong Bong

    2017-07-01

    Disseminated intravascular coagulation is a rare complication of Kawasaki disease and appears in Kawasaki disease patients. We report a case of refractory Kawasaki disease complicated with disseminated intravascular coagulation and giant coronary aneurysm. A 5-month-old boy presented with Kawasaki disease with coagulopathy. Although the coagulopathy improved after fresh-frozen plasma and antithrombin-III administration, the fever persisted despite two rounds of intravenous immunoglobulin, along with intravenous methylprednisolone pulse therapy and infliximab administration. Despite all efforts to treatment, the patient had giant coronary aneurysms and died suddenly.

  2. Risk of coronary artery involvement in Kawasaki disease.

    Science.gov (United States)

    Soriano-Ramos, María; Martínez-Del Val, Elena; Negreira Cepeda, Sagrario; González-Tomé, María I; Cedena Romero, Pilar; Fernández-Cooke, Elisa; Albert de la Torre, Leticia; Blázquez-Gamero, Daniel

    2016-04-01

    Kawasaki disease refers to systemic vasculitis with risk of coronary artery disease. Our objective is to identify risk factors associated with coronary artery disease in patients with complete and incomplete Kawasaki disease. Descriptive, retrospective study conducted in patients diagnosed with Kawasaki disease in a tertiary-care hospital between 2008 and 2014. The American Heart Association diagnostic criteria were used to define complete and incomplete Kawasaki disease. Thirty-one children were diagnosed with Kawasaki disease; 24 met the criteria for the complete form, and 7, for the incomplete form of this condition. Five had coronary artery disease. One of them had incomplete Kawasaki disease (1/7= 14.3%), and the remaining four had the complete form (4/24= 16.7%). No significant differences were found between both groups (p= 1.0). Patients with coronary artery involvement had a higher C-reactive protein level (median: 16.2 mg/dL versus 8.4 mg/dL, p= 0.047) and lower albuminemia (median: 3.2 mg/dL versus 3.99 mg/dL, p= 0.002). The risk of coronary artery involvement in incomplete Kawasaki disease is similar to that in complete Kawasaki disease; therefore, in patients with the incomplete form, immunoglobulin therapy should not be delayed. In our population, C-reactive protein and albumin levels were related to a higher risk of coronary artery involvement. Sociedad Argentina de Pediatría.

  3. The serum concentration of soluble interleukin-2 receptor in patients with Kawasaki disease.

    Science.gov (United States)

    Teraura, Hiroyuki; Kotani, Kazuhiko; Minami, Takaomi; Takeshima, Taro; Shimooki, Osamu; Kajii, Eiji

    2017-03-01

    Kawasaki disease is a febrile disease of childhood that is associated with increased inflammatory cytokines and immunoregulatory abnormalities. While the serum concentrations of soluble IL-2 receptor can change under such pathologies, the relevance of the soluble IL-2 receptor concentration in patients with Kawasaki disease has not been specified. We aimed to summarize the existing studies that reported the soluble IL-2 receptor concentrations in patients with Kawasaki disease. Original articles that were published up to July 2016 were collected using a PubMed/Medline-based search engine. A total of nine articles that reported the serum soluble IL-2 receptor concentrations in acute-phase Kawasaki disease were eligible. All of the articles described a high soluble IL-2 receptor concentration in patients with Kawasaki disease relative to the level of controls or the reference range. Two of five articles on patients with coronary artery aneurysms described a significantly higher soluble IL-2 receptor concentration in patients with coronary artery aneurysms than patients without. Two articles on patients with intravenous immunoglobulin therapy described a significant decrease of the soluble IL-2 receptor concentration after the therapy. Accordingly, the serum soluble IL-2 receptor can be a potent marker of disease activity and therapeutic effects in patients with Kawasaki disease; further studies are thus warranted for its use in the clinical setting.

  4. Renal histology of mucocutaneous lymph node syndrome (Kawasaki disease).

    Science.gov (United States)

    Salcedo, J R; Greenberg, L; Kapur, S

    1988-01-01

    Renal involvement is well described in patients with mucocutaneous lymph node syndrome (MCLNS), or Kawasaki disease and is manifested by mild azotemia, hematuria, pyuria or cylinduria, and more often, proteinuria. Renal morphology during the acute stages of the illness has never been reported. In this paper we describe the renal histopathologic changes in a child with MCLNS. The glomerular histopathologic findings suggest immune complex damage to the kidney as a possible mechanism of nephrotoxicity in MCLNS. Presence of kidney lesions, which speak in favor of the injurious role of immune complexes in MLCNS may be relevant to the understanding of the pathogenesis of the vascular lesions that are characteristic of this disease.

  5. Differential expression of miR-145 in children with Kawasaki disease.

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    Chisato Shimizu

    Full Text Available BACKGROUND: Kawasaki disease is an acute, self-limited vasculitis of childhood that can result in structural damage to the coronary arteries. Previous studies have implicated the TGF-β pathway in disease pathogenesis and generation of myofibroblasts in the arterial wall. microRNAs are small non-coding RNAs that modulate gene expression at the post-transcriptional level and can be transported between cells in extracellular vesicles. To understand the role that microRNAs play in modifying gene expression in Kawasaki disease, we studied microRNAs from whole blood during the acute and convalescent stages of the illness. METHODOLOGY/PRINCIPAL FINDINGS: RNA isolated from the matched whole blood of 12 patients with acute and convalescent Kawasaki disease were analyzed by sequencing of small RNA. This analysis revealed six microRNAs (miRs-143, -199b-5p, -618, -223, -145 and -145* (complementary strand whose levels were significantly elevated during the acute phase of Kawasaki disease. The result was validated using targeted qRT-PCR using an independent cohort (n = 16. miR-145, which plays a critical role in the differentiation of neutrophils and vascular smooth muscle cells, was expressed at high levels in blood samples from acute Kawasaki disease but not adenovirus-infected control patients (p = 0.005. miR-145 was also detected in small extracellular vesicles isolated from acute Kawasaki disease plasma samples. Pathway analysis of the predicted targets of the 6 differentially expressed microRNAs identified the TGF-β pathway as the top pathway regulated by microRNAs in Kawasaki disease. CONCLUSION: Sequencing of small RNA species allowed discovery of microRNAs that may participate in Kawasaki disease pathogenesis. miR-145 may participate, along with other differentially expressed microRNAs, in regulating expression of genes in the TGF-β pathway during the acute illness. If the predicted target genes are confirmed, our findings suggest a model of

  6. Absence of association of FCGR2A gene polymorphism rs1801274 with Kawasaki disease in Greek patients.

    Science.gov (United States)

    Chatzikyriakidou, Anthoula; Aidinidou, Louiza; Giannopoulos, Andreas; Papadopoulou-Legbelou, Kyriaki; Kalinderi, Kallirhoe; Fidani, Liana

    2015-04-01

    Kawasaki disease is an acute, febrile syndrome in infancy, characterised by vasculitis of medium-sized arteries, and affects predominantly young children. Family-based studies on Kawasaki disease supports the contribution of genetic factors in disorder manifestation. In a recent genome-wide association study, the polymorphism rs1801274 of FCGR2A [Fc fragment of immunoglobulin G, low-affinity IIa, receptor] gene has been implicated in disease pathogenesis. The aim of the present study was to explore the association of this variant, for the first time, in a group of Kawasaki-diseased patients of Greek origin. A total of 47 Kawasaki-diseased children and 50 control subjects were enrolled in the study. Polymerase chain reaction-restriction fragment length polymorphism assay was performed in rs1801274 genotyping. No association was observed between this polymorphism genotypes' or alleles' distribution between Kawasaki-diseased patients and controls. Furthermore, no association was revealed between this polymorphism and cardiovascular complications in Kawasaki-diseased patients. In the literature, the reported data over this polymorphism association with Kawasaki disease in Caucasian patients are contradictory. In addition, the disease shows low prevalence in the Caucasian populations. Therefore, the independent genetic association studies on rs1801274 with Kawasaki disease in various Caucasian groups increase the amount of genetic data, which could be used in a future meta-analysis, increasing the statistical power of the resultant conclusions.

  7. Unusual imaging presentation of infantile atypical Kawasaki disease.

    Science.gov (United States)

    Kumar, Nishith; Mittal, Mahesh Kumar; Sinha, Mukul; Gupta, Arpita; Thukral, Brij Bhushan

    2016-01-01

    Kawasaki disease is a systemic medium vessel vasculitis of unknown etiology affecting children under 5 years of age. There are no specific diagnostic tests, and thus, the diagnosis of the disease is primarily made on the basis of clinical criteria. Unusual presentations of Kawasaki disease have been variably reported from different parts of the world. However, presentation of the disease in the form of peripheral thromboembolism and florid non-coronary aneurysms has rarely been described This report describes the imaging findings in infantile atypical Kawasaki disease with aneurysms of multiple medium-sized arteries, including coronary arteries, emphasizing the detection of clinically silent aneurysms in the disease.

  8. Unusual imaging presentation of infantile atypical Kawasaki disease

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    Nishith Kumar

    2016-01-01

    Full Text Available Kawasaki disease is a systemic medium vessel vasculitis of unknown etiology affecting children under 5 years of age. There are no specific diagnostic tests, and thus, the diagnosis of the disease is primarily made on the basis of clinical criteria. Unusual presentations of Kawasaki disease have been variably reported from different parts of the world. However, presentation of the disease in the form of peripheral thromboembolism and florid non-coronary aneurysms has rarely been described This report describes the imaging findings in infantile atypical Kawasaki disease with aneurysms of multiple medium-sized arteries, including coronary arteries, emphasizing the detection of clinically silent aneurysms in the disease.

  9. Postnatal Risk of Acquiring Kawasaki Disease: A Nationwide Birth Cohort Database Study.

    Science.gov (United States)

    Wu, Mei-Hwan; Lin, Min-Tai; Chen, Hui-Chi; Kao, Feng-Yu; Huang, San-Kuei

    2017-01-01

    To investigate the postnatal risk of Kawasaki disease and coronary complications from a nationwide birth cohort in Taiwan, a country with the third-highest incidence of Kawasaki disease worldwide. We enrolled children born between 2000 and 2009 with complete postnatal medical care records for 2000-2014 in the Taiwan national database. Out of a total of 2 150 590 live births, we identified 6690 (62.6% boys) patients with Kawasaki disease. The onset was mostly (93.9%) within the first 5 years of life (median, 16 months; 38% during infancy), but was rare within the first 3 months of life. The overall cumulative incidence of Kawasaki disease by age 5 years was 2.78‰ (3.33‰ for boys and 2.17‰ for girls; P Kawasaki disease recurred more often in younger patients (cumulative incidence, 2.3% in infants vs 1.7% in children aged 1-4 years). Coronary complications occurred in 16.2% of the patients, including 4 cases of acute myocardial infarction (3 occuring during the acute stage and 1 occurring 5 years later). The probability of a major cardiac event (infarction, undergoing percutaneous coronary intervention or coronary artery bypass grafting, or death) by adolescence was 1.9%. The postnatal risk of Kawasaki disease was 3‰-4‰ and increased with every birth year. Patients with Kawasaki disease are at substantial risk for a major cardiac events during childhood. Copyright © 2016 Elsevier Inc. All rights reserved.

  10. Cardiac complications in children with Kawasaki disease in our own experience.

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    Gowin, Ewelina; Małecka, Ilona; Stryczyńska-Kazubska, Joanna; Michalak, Michał; Wysocki, Jacek; Górzna-Kamińska, Hanna

    2016-01-01

    Kawasaki disease is the most common cause of acquired heart disease in children in developed countries. The incidence of Kawasaki disease varies from 180 in Japan through 20 in United States to 5-8 in the European countries per 100,000 children younger than 5 years of age. To evaluate cardiac complications in children hospitalised with Kawasaki disease. Retrospective analysis of the medical records of patients hospitalised with Kawasaki disease in the Specialist Mother and Child Healthcare Facility in Poznan (Poland) in 2008-2014. The diagnosis was based on the American Heart Association criteria. Study group included 30 patients (25 boys and 5 girls). The mean age was 49 months; 21 (70%) children were younger than 5 years of age. All patients had oral mucosal lesions, while an elevated leukocyte count was observed in a minority of patients. Cardiac involvement was detected in 18 (60%) patients, aneurysms in 4 (13.3%) patients, coronary artery dilatation in 6 patients, pericarditis in 6 patients, mitral regurgitation in 3 patients, and aortic regurgitation in 2 patients. In 5 children, more than 1 cardiac abnormality was detected. During 12 months of follow-up, coronary artery dilatation resolved in 5 children, and 1 patient developed aneurysm. Our findings suggest that Kawasaki disease should be considered in the differential diagnosis of children with prolonged fever. During the acute stage of the disease, children with Kawasaki disease require regular cardiac evaluation, and long-term care is needed when cardiovascular complications occur. A central case reporting system to monitor all cases of Kawasaki disease in the Polish paediatric population should be introduced.

  11. Cardiac magnetic resonance feature tracking in Kawasaki disease convalescence

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    Konstantinos Bratis

    2017-01-01

    Conclusion: In this CMR study in Kawasaki disease convalescent patients with preserved routine functional indices, we detected lower circumferential and longitudinal strain values compared to normal controls, irrespective of the coronary artery status.

  12. [Predicting value of thrombospondin-2 for coronary artery dilatation in patients with Kawasaki disease].

    Science.gov (United States)

    Song, R X; Li, X H; Zhang, T; Cui, X D; Fu, J

    2016-04-01

    To investigate the predictive value of thrombospondin-2 in children with coronary artery dilatation secondary to Kawasaki disease. This study was a retrospective controlled study, the patients were divided into Kawasaki disease group and control group.Patients first time hospitalized in Children's Hospital Affiliated to Capital Institute of Pediatrics during the acute phase of Kawasaki disease were enrolled in Kawasaki disease group from May 2013 to August 2014.Sixty-four Kawasaki disease patients were enrolled into the Kawasaki disease group based on inclusion and exclusion criteria, including 39 males and 25 females, with an average age of 2.3 years.According to the results of echocardiography of children with Kawasaki disease, patients were divided into coronary artery dilatation group and non-coronary artery dilatation group, there were 33 cases in the coronary dilatation (CAD) group, 26 males and 7 females, the average age was 2.0 years, and 31 cases in the non-coronary dilatation (NCAD) group, 13 males and 18 females, the average age was 2.6 years.The control group consisted of children with fever but excluding vascular inflammatory diseases and healthy children, 32 cases in fever group, 19 males and 13 females, mean age was 4.5 years; in which healthy group had 32 cases, 18 males and 14 females and the average age was 3.3 years. Enzyme linked immunosorbent assay (ELISA) method was used to measure the levels of thrombospondin-1 (TSP-1) and TSP-2 in the plasma of each group, and the related laboratory parameters, and general information was analyzed.Multiple sets of normal distribution of data were compared by analysis of unifactorial analysis of variance and the two groups were compared by two independent samples t-test.Analysis of covariance was used to remove the effect of age.The χ(2) test was used to analyze categeorical data and receiver operating characteristic (ROC) curve for evaluating the predictive value of TSP-2. Compared with the febrile group

  13. A male infant had subdural effusion and paroxysmal supraventricular tachycardia during the febrile episode of Kawasaki disease: a case report and literature review.

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    Chou, Chia-Pei; Lin, I-Chun; Kuo, Kuang-Che

    2016-05-28

    Kawasaki disease is an acute, febrile, self-limiting, inflammatory systemic vasculitis seen in early childhood, most commonly in those below 5 years of age. In Kawasaki disease, the coronary arteries are most commonly affected, which may lead to asymptomatic coronary artery ectasia or formation of an aneurysm. Paroxysmal supraventricular tachycardia(PSVT) is a severe and rare cardiovascular complication of Kawasaki disease. A case of Kawasaki disease presenting with unusual findings, including subdural effusion and PSVT is reported. This is a 4-month-10-day-old boy presents with anterior fontanelle bulging and moderate bilateral subdural effusion at the acute stage of Kawasaki disease and PSVT at the subacute stage of Kawasaki disease. The subdural effusion was resolution after intravenous immunoglobulin(IVIG) administration. And the PSVT was subsided after administered 3 doses of adenosine, 1 dose of amiodarone loading and Propranolol twice per day use. At 1-year follow-up has made a complete recovery with no arrhythmia episodes, developmental effects or abnormal neurologic findings. Subdural effusion in the acute stage of Kawasaki disease may be an inflammatory response. It may resolves spontaneously after anti-inflammatory treatment such as IVIG infusion. PSVT is a severe cardiovascular complication of Kawasaki disease. In those who taking aspirin, we need to carefully observe the heart rhythm and PSVT side effects, especially in the first month.

  14. Kawasaki disease in Sicily: clinical description and markers of disease severity

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    Maggio, M.; Corsello, G; Prinzi, E.; Cimaz, R

    2016-01-01

    Background Kawasaki disease (KD) is an acute systemic vasculitis of small and middle size arteries; 15-25 % of untreated patients and 5 % of patients treated with intravenous immunoglobulin (IVIG) develop coronary artery lesions (CAL). Many studies tried to find the most effective treatment in the management of resistant KD and to select the risk factors for CAL. Our data are assessed on children from west Sicily, characterized by a genetic heterogeneity. Methods We studied the clinical data ...

  15. Epidemiologic pictures of Kawasaki disease in Shanghai from 1998 through 2002.

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    Huang, Guo-Ying; Ma, Xiao-Jing; Huang, Min; Chen, Shu-Bao; Huang, Mei-Rong; Gui, Yong-Hao; Ning, Shou-Bao; Zhang, Tuo-Hong; Du, Zhong-Dong; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2006-01-01

    Epidemiologic features of Kawasaki disease in China is still not clear. A questionnaire form and diagnostic guidelines for Kawasaki disease were sent to hospitals in Shanghai, which provided with pediatric medical care. All patients with Kawasaki disease diagnosed during January 1998 through December 2002 were recruited in this study. A total of 768 patients with Kawasaki disease were reported. The incidence rates of Kawasaki disease for each year were 16.79 (1998), 25.65 (1999), 28.16 (2000), 28.05 (2001), and 36.76 (2002) per 100,000 children under 5 years of age. The male/female ratio was 1.83:1. The age at onset ranged from 1 month to 18.8 years (median: 1.8 years). The disease occurred more frequently in spring and summer. Fever was the most common clinical symptom, followed by oral changes, extremities desquamate, rash, conjunctive congestion, lymphadenopathy, extremities swelling, and crissum desquamate. Cardiac abnormalities were found in 24.3% of patients. The most common cardiac abnormality was coronary artery lesions including dilatation (68%) and aneurysm (10%). The case-fatality rate at acute stage of the disease was 0.26%. A second onset of the disease occurred in 1.82% of patients. The incidence rate of Kawasaki disease in Shanghai is lower than that reported in Japan, but higher than those in western countries. The increasing trend in incidence, sex distribution and cardiac abnormalities are similar to those in previous reports. The seasonal distribution is similar to the report from Beijing and different from other reports.

  16. Common variants in CASP3 confer susceptibility to Kawasaki disease.

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    Onouchi, Yoshihiro; Ozaki, Kouichi; Buns, Jane C; Shimizu, Chisato; Hamada, Hiromichi; Honda, Takafumi; Terai, Masaru; Honda, Akihito; Takeuchi, Takashi; Shibuta, Shoichi; Suenaga, Tomohiro; Suzuki, Hiroyuki; Higashi, Kouji; Yasukawa, Kumi; Suzuki, Yoichi; Sasago, Kumiko; Kemmotsu, Yasushi; Takatsuki, Shinichi; Saji, Tsutomu; Yoshikawa, Tetsushi; Nagai, Toshiro; Hamamoto, Kunihiro; Kishi, Fumio; Ouchi, Kazunobu; Sato, Yoshitake; Newburger, Jane W; Baker, Annette L; Shulman, Stanford T; Rowley, Anne H; Yashiro, Mayumi; Nakamura, Yoshikazu; Wakui, Keiko; Fukushima, Yoshimitsu; Fujino, Akihiro; Tsunoda, Tatsuhiko; Kawasaki, Tomisaku; Hata, Akira; Nakamura, Yusuke; Tanaka, Toshihiro

    2010-07-15

    Kawasaki disease (KD; OMIM 611775) is an acute vasculitis syndrome which predominantly affects small- and medium-sized arteries of infants and children. Epidemiological data suggest that host genetics underlie the disease pathogenesis. Here we report that multiple variants in the caspase-3 gene (CASP3) that are in linkage disequilibrium confer susceptibility to KD in both Japanese and US subjects of European ancestry. We found that a G to A substitution of one commonly associated SNP located in the 5' untranslated region of CASP3 (rs72689236; P = 4.2 x 10(-8) in the Japanese and P = 3.7 x 10(-3) in the European Americans) abolished binding of nuclear factor of activated T cells to the DNA sequence surrounding the SNP. Our findings suggest that altered CASP3 expression in immune effecter cells influences susceptibility to KD.

  17. Juvenile polyarteritis nodosa associated with toxoplasmosis presenting as Kawasaki disease.

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    Başaran, Özge; Çakar, Nilgün; Gür, Gökçe; Kocabaş, Abdullah; Gülhan, Belgin; Çaycı, Fatma Şemsa; Çelikel, Banu Acar

    2014-04-01

    Polyarteritis nodosa (PAN) is a vasculitis characterized by inflammatory necrosis of medium-sized arteries. Juvenile PAN and Kawasaki disease (KD) both cause vasculitis of the medium-sized arteries, and share common features. They have overlapping clinical features. Treatment should be managed according to the severity of symptoms and persistence of clinical manifestations. Herein is described the case of a 14-year-old boy first diagnosed with KD, who then fulfilled the criteria for juvenile PAN due to the development of severe myalgia, persistent fever, polyneuropathy and coronary arterial dilatation. He also had acute toxoplasmosis at the onset of vasculitis symptoms. The final diagnosis was of juvenile PAN associated with toxoplasmosis infection. Toxoplasma infection can be considered as an etiological agent for PAN and other vasculitis syndromes. Awareness of toxoplasmosis-related PAN facilitates early diagnosis, and instigation of appropriate treatment.

  18. [Brain hemorrhage in a patient with Kawasaki disease].

    Science.gov (United States)

    Yamazaki-Nakashimada, Marco Antonio; Rivas-Larrauri, Francisco; Alcántara-Salinas, Adriana; Hernández-Bautista, Victor; Rodríguez-Lozano, Ana Luisa

    2013-01-01

    Kawasaki disease is an acute, self-limiting vasculitis of unknown origin, characterized by fever, palms and soles edema, cervical lymphadenopathy, strawberry tongue, and non-exudative conjunctivitis. It is a multisystemic vasculitis that affects predominantly infants and young children. The most feared complication is the development of coronary aneurysms that occurs up to 25% of untreated patients; however there are reports of extra coronary involvement. Herein we present the case of a 2 year-old girl who had a severe symptomatology and persistent fever despite intravenous gammaglobulin. Two years later she presented right hemiparesia and headache, with data from CAT and MRI suggestive of brain mass and deviation of the midline, secondary to left frontoparietal haemorrhage that was treated with a craniotomy. She was discharged on prednisone, ASA and rehabilitation.

  19. A case of atypical Kawasaki disease with giant coronary artery aneurysm containing thrombus.

    Science.gov (United States)

    Micallef, Eynaud S; Attard, Montalto S; Grech, V

    2016-01-01

    Kawasaki disease (KD) is an acute febrile, systemic vasculitic syndrome of unknown etiology, occurring primarily in children younger than 5 years of age. Administration of IVIG within the first 10 days after onset of fever in combination with high dose aspirin reduces the risk of coronary artery damage in KD. Though rare, giant aneurysms of the coronary arteries may develop in untreated cases and prove extremely challenging to manage. A 9-month-old Caucasian boy presented to our paediatric emergency department with a 4-week history of intermittent pyrexia and irritability. Typical mucocutaneous signs of Kawasaki Disease were absent upon presentation. A trans-thoracic echocardiogram identified a giant aneurysm of the left anterior descending artery with thrombus formation in-situ and the child was managed with intravenous immunoglobulin, steroids, high dose aspirin therapy and later warfarinisation. Cardiovascular sequelae of Kawasaki disease include giant coronary artery aneurysms with thrombosis. Enlargement of a coronary aneurysm after the acute phase of Kawasaki disease is uncommon and the outcome of interventional approaches poorly studied.

  20. Pathogenetic determinants in Kawasaki disease: the haematological point of view.

    Science.gov (United States)

    Del Principe, Domenico; Pietraforte, Donatella; Gambardella, Lucrezia; Marchesi, Alessandra; Tarissi de Jacobis, Isabella; Villani, Alberto; Malorni, Walter; Straface, Elisabetta

    2017-04-01

    Kawasaki disease is a multisystemic vasculitis that can result in coronary artery lesions. It predominantly affects young children and is characterized by prolonged fever, diffuse mucosal inflammation, indurative oedema of the hands and feet, a polymorphous skin rash and non-suppurative lymphadenopathy. Coronary artery involvement is the most important complication of Kawasaki disease and may cause significant coronary stenosis resulting in ischemic heart disease. The introduction of intravenous immunoglobulin decreases the incidence of coronary artery lesions to less than 5%. The etiopathogenesis of this disease remains unclear. Several lines of evidence suggest that an interplay between a microbial infection and a genetic predisposition could take place in the development of the disease. In this review, we summarize the state of the art of pathogenetic mechanisms of Kawasaki disease underscoring the relevance of haematological features as a novel field of investigation.

  1. IVIG reduced vascular oxidative stress in patients with Kawasaki disease.

    Science.gov (United States)

    Takatsuki, Shinichi; Ito, Yuka; Takeuchi, Daiji; Hoshida, Hiroshi; Nakayama, Tomotaka; Matsuura, Hiroyuki; Saji, Tsutomu

    2009-07-01

    Oxidative stress (OS) contributes to the acute phase of Kawasaki disease (KD) in a manner that is as yet unknown. In the present study OS in the acute phase of KD was investigated by measuring urinary 8-iso-prostaglandin F2alpha (8-iso-PG) and evaluating its correlation to the efficacy of intravenous immunoglobulin (IVIG) administration. The 62 patients with acute phase of KD were enrolled, as well as 20 healthy children (HC) and 20 with acute febrile illness (FI). Urinary samples were obtained before and after administration of IVIG. The HC and FI groups also had inflammatory markers evaluated at the same time. The 8-iso-PG was significantly elevated in the 62 KD patients (719 +/-335 pg/mg Cr) without IVIG administration compared with those with FI (583 +/-213 pg/mg Cr) as well as HC (443 +/-288 pg/mg Cr) (PIVIG: 16 received 2 g/kg for 1 day; 17 received 1 g/kg for 1 day; 7 received 400 mg . kg(-1) . day(-1) for 5 days. All regimens of IVIG reduced the 8-iso-PG level at 7 days after initiation. OS provokes vasculitis in KD, the activation of which was reduced by IVIG. The urinary level of 8-iso-PG is a useful marker of the effectiveness of IVIG in the acute phase of KD.

  2. Cardiovascular imaging in children and adults following Kawasaki disease.

    Science.gov (United States)

    Dietz, S M; Tacke, C E; Kuipers, I M; Wiegman, A; de Winter, R J; Burns, J C; Gordon, J B; Groenink, M; Kuijpers, T W

    2015-12-01

    Kawasaki disease (KD) is a paediatric vasculitis with coronary artery aneurysms (CAA) as its main complication. Two guidelines exist regarding the follow-up of patients after KD, by the American Heart Association and the Japanese Circulation Society. After the acute phase, CAA-negative patients are checked for cardiovascular risk assessment or with ECG and echocardiography until 5 years after the disease. In CAA-positive patients, monitoring includes myocardial perfusion imaging, conventional angiography and CT-angiography. However, the invasive nature and high radiation exposure do not reflect technical advances in cardiovascular imaging. Newer techniques, such as cardiac MRI, are mentioned but not directly implemented in the follow-up. Cardiac MRI can be performed to identify CAA, but also evaluate functional abnormalities, ischemia and previous myocardial infarction including adenosine stress-testing. Low-dose CT angiography can be implemented at a young age when MRI without anaesthesia is not feasible. CT calcium scoring with a very low radiation dose can be useful in risk stratification years after the disease. By incorporating newer imaging techniques, detection of CAA will be improved while reducing radiation burden and potential complications of invasive imaging modalities. Based on the current knowledge, a possible pathway to follow-up patients after KD is introduced. Key Points • Kawasaki disease is a paediatric vasculitis with coronary aneurysms as major complication. • Current guidelines include invasive, high-radiation modalities not reflecting new technical advances. • Cardiac MRI can provide information on coronary anatomy as well as cardiac function. • (Low-dose) CT-angiography and CT calcium score can also provide important information. • Current guidelines for follow-up of patients with KD need to be revised.

  3. Can Coronary Artery Involvement in Kawasaki Disease be Predicted?

    Science.gov (United States)

    Ghelani, Sunil J; Kwatra, Neha S; Spurney, Christopher F

    2013-03-26

    Coronary artery involvement is seen in approximately 15-20% of children with Kawasaki disease. There is conflicting literature regarding the clinical and laboratory findings associated with coronary artery involvement. In this retrospective study, we attempt identification of predictive factors for coronary artery involvement at our institute and review the existing literature. A review of 203 patients (65% males) with Kawasaki disease was performed, of whom 33 (16.3%) had coronary artery involvement. High erythrocyte sedimentation rate, high platelet count, low hematocrit, low albumin levels, and refractory Kawasaki disease showed significant association with coronary artery involvement. High erythrocyte sedimentation rate and refractory Kawasaki disease were found to be independent predictors of coronary artery involvement. Review of literature suggested a wide range of coronary involvement (60%), and highly conflicting clinical and laboratory associations. It remains difficult to accurately determine risk of coronary artery involvement, although some laboratory markers may provide information that is helpful for parental counseling and clinical follow up. Future identification of novel biomarkers and host predispositions may further our understanding of coronary artery risks and help personalize therapy for Kawasaki disease.

  4. The Clinical Diagnosis and Management of Kawasaki Disease: a Review and Update.

    Science.gov (United States)

    Zhu, Frank H; Ang, Jocelyn Y

    2016-09-01

    Kawasaki disease is an acute, self-limited vasculitis of childhood and has become the leading cause of acquired pediatric heart disease in the USA. Prompt treatment is essential in reducing cardiac-related morbidity and mortality. The underlying etiology remains unknown. The disease itself may be the characteristic manifestation of a common pathway of immune-mediated vascular inflammation in susceptible hosts. The characteristic clinical features of fever for at least 5 days with bilateral nonpurulent conjunctivitis, rash, changes in lips and oral cavity, changes in peripheral extremities, and cervical lymphadenopathy remain the mainstay of diagnosis. Supplementary laboratory criteria can aid in the diagnosis, particularly in cases of incomplete clinical presentation. Diagnosis of Kawasaki disease can be challenging as the clinical presentation can be mistaken for a variety of other pediatric illnesses. Standard of care consists of intravenous immune globulin and aspirin. Corticosteroids, infliximab, and cyclosporine A have been used as adjunct therapy for Kawasaki disease refractory to initial treatment. There is ongoing research into the use of these agents in the initial therapy of Kawasaki disease.

  5. Predicting factors for refractory kawasaki disease.

    Science.gov (United States)

    Do, Young-Sun; Kim, Ki-Won; Chun, Jin-Kyong; Cha, Byung Ho; Namgoong, Mee Kyung; Lee, Hae Yong

    2010-05-01

    About 10-15% of Kawasaki disease (KD) is refractory to intravenous immunoglobulin (IVIG) therapy. This study was designed to investigate the predicting factors for refractory KD. We reviewed retrospectively the clinical records of 77 patients with typical KD admitted at Wonju Christian Hospital from January, 2005, to December, 2008. The variance of laboratory and demographic parameters between the IVIG-responsive group and IVIG-resistant group were analyzed. Thirteen patients with urinary tract infections were randomly collected as a febrile control group. Among 77 patients diagnosed with complete KD, 13 patients (16.9%) were IVIG-resistant. The febrile period and hospital days were significantly longer in the IVIG-resistant group than IVIG-responsive group (pIVIG-resistant group (p=0.025). The Kobayashi score could differentiate these two groups (p=0.015). Fewer lymphocytes was observed during the subacute phase in the IVIG-resistant group (p=0.032). Coronary arterial dilatations (CADs) were observed in 10.9% (7/64) of IVIG-responders and 38.5% (5/13) of IVIG-resistant patients (p=0.038). The percentage of neutrophils and lymphocytes in patients with KD, in addition to known risk factors for refractory KD, may help predict IVIG-resistance in patients with KD.

  6. Procalcitonin Levels in Patients with Complete and Incomplete Kawasaki Disease

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    Hwa Jin Cho

    2013-01-01

    Full Text Available Incomplete Kawasaki disease (iKD is considered to be a less complete form of Kawasaki disease (cKD, and several differences in the laboratory presentations of iKD and cKD have been noted. We investigated serum procalcitonin levels in patients with iKD, cKD, and other febrile diseases (a control group. Seventy-seven patients with cKD, 24 with iKD, and 41 controls admitted to our hospital from November 2009 to November 2011 were enrolled in the present study. We obtained four measurements of serum procalcitonin levels and those of other inflammatory markers from each patient. Samples were taken for analysis on the day of diagnosis (thus before treatment commenced; D0 and 2 (D2, 14 (D14, and 56 days (D56 after intravenous immunoglobulin infusion. We obtained control group data at D0. The mean D0 serum procalcitonin levels of cKD patients (0.71±1.36 ng/mL and controls (0.67±1.06 ng/mL were significantly higher than those of iKD patients (0.26±0.26 ng/mL (P=0.014 and P=0.041, resp.. No significant difference in mean procalcitonin level was evident among groups at any subsequent time. In conclusion, the serum procalcitonin level of patients with acute-stage cKD was significantly higher than that of iKD patients.

  7. Doença de Kawasaki: experiência clínica em hospital universitário Kawasaki disease: clinical experience in a university hospital

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    Angela Esposito Ferronato

    2010-06-01

    Full Text Available OBJETIVO: A doença de Kawasaki é uma vasculite sistêmica aguda de etiologia desconhecida. Seu diagnóstico baseia-se em critérios clínicos. O objetivo deste estudo foi descrever os casos de pacientes com doença de Kawasaki internados no Hospital Universitário da Universidade de São Paulo entre janeiro/2000 e junho/2008. MÉTODOS: Dentre todos os pacientes internados na Enfermaria de Pediatria no período acima, foram selecionados aqueles cujo CID de alta foi doença de Kawasaki. Realizou-se estudo descritivo por meio da análise dos prontuários dessas crianças. RESULTADOS: Foram encontrados 18 casos. A média de internações foi de 2,1 casos/ano. A idade variou de três meses a nove anos. A proporção meninos:meninas foi 1:1,25. Receberam outros diagnósticos prévios 17 pacientes, sendo escarlatina em 2/3 dos casos. O tempo de febre antes do diagnóstico variou de cinco a 11 dias. Nove crianças apresentaram quatro sinais sugestivos de doença de Kawasaki; oito apresentaram cinco sinais e uma apresentou dois sinais, o que foi considerado doença de Kawasaki incompleta. Receberam gamaglobulina 15 crianças (entre o sexto e o décimo dias de evolução e 11 (73% ficaram afebris após infusão da medicação. Os demais tiveram febre até 24 horas após a administração. Todos os pacientes realizaram ecocardiograma e três apresentaram aneurisma leve da coronária. CONCLUSÕES: A doença de Kawasaki é habitualmente confundida com outras doenças, o que causa retardo no tratamento e aumento no risco de complicações cardíacas.OBJECTIVE: Kawasaki disease is an acute systemic vasculitis of unknown etiology. Its diagnosis is based on clinical criteria. This study aimed to describe Kawasaki disease cases treated at the University Hospital of Universidade de São Paulo, from January/2000 to June/2008. METHODS: Among all patients admitted to the pediatric ward during this period, patients whose discharge ICD was Kawasaki disease were

  8. Recent Advances in Kawasaki Disease - Proceedings of the 3rd Kawasaki Disease Summit, Chandigarh, 2014.

    Science.gov (United States)

    Singh, Surjit; Sharma, Dhrubajyoti; Bhattad, Sagar; Phillip, Saji

    2016-01-01

    Kawasaki disease (KD) is the most common cause of acquired heart disease in children in Japan, North America and Europe. It is now being increasingly recognized from the developing countries as well. If not diagnosed and treated in time, KD can result in coronary artery abnormalities in approximately 15-25% cases. The long-term consequences of these abnormalities may manifest in adults as myocardial ischemia and congestive heart failure. Intravenous immunoglobulin (IVIg) remains the drug of choice for treatment of KD, but several new agents like infliximab, cyclosporine, glucocorticoids and statins are now being increasingly used in these patients. While echocardiography has been the preferred imaging modality hitherto, CT coronary angiography has emerged as an exciting new supplementary option and provides an entirely new dimension to this disease. The incidence of KD has shown a progressive increase in several countries and it is likely that this disease would impact public health programmes in the near future even in the developing countries.

  9. Coronary CT angiography and MR angiography of Kawasaki disease

    Energy Technology Data Exchange (ETDEWEB)

    Goo, Hyun Woo [University of Ulsan College of Medicine, Department of Radiology, Asan Medical Center, 388-1 Poongnap-2dong, Songpa-gu, Seoul (Korea); Park, In-Sook; Ko, Jae Kon; Kim, Young Hwee [University of Ulsan College of Medicine, Department of Paediatric Cardiology, Asan Medical Center, Seoul (Korea)

    2006-07-15

    Although the incidence of coronary artery aneurysms has diminished in patients with Kawasaki disease, coronary artery involvement is still regarded as a major complication of the disease, significantly affecting morbidity and mortality. Recent technical advances in coronary CT angiography (CTA) and MR angiography (MRA) have led to the possibility of using these two imaging methods as minimally invasive alternatives to the more invasive diagnostic catheter angiography in evaluating coronary artery abnormalities, such as aneurysm, stenosis, and occlusion. In this article, we describe imaging techniques and findings of coronary CTA and MRA in Kawasaki disease. (orig.)

  10. Four cases with Kawasaki disease and viral infection: aetiology or association.

    Science.gov (United States)

    Giray, Tuba; Biçer, Suat; Küçük, Öznur; Çöl, Defne; Yalvaç, Zerrin; Gürol, Yeşim; Yilmaz, Gülden; Saç, Ahmet; Mogol, Yigit

    2016-12-01

    The aetiology of Kawasaki disease has not yet been precisely determined. It has been associated with a variety of bacterial and viral agents. Some viruses including human adenovirus, coronavirus, and parainfluenza virus type 3 have been isolated from patients with Kawasaki disease. Clinical presentation of patients with human coronavirus and adenovirus infections mimics Kawasaki disease. In addition, these viruses may also be detected in Kawasaki disease as a coinfection. In this report, we present four Kawasaki disease patients infected with adenovirus, coronavirus OC43/HKU1 and parainfluenza virus type 3.

  11. Differential protein analysis of serum exosomes post-intravenous immunoglobulin therapy in patients with Kawasaki disease.

    Science.gov (United States)

    Zhang, Li; Song, Qi-Fang; Jin, Jing-Jie; Huang, Ping; Wang, Zhou-Ping; Xie, Xiao-Fei; Gu, Xiao-Qiong; Gao, Xue-Juan; Jia, Hong-Ling

    2017-08-14

    Kawasaki disease, which is characterised by systemic vasculitides accompanied by acute fever, is regularly treated by intravenous immunoglobulin to avoid lesion formation in the coronary artery; however, the mechanism of intravenous immunoglobulin therapy is unclear. Hence, we aimed to analyse the global expression profile of serum exosomal proteins before and after administering intravenous immunoglobulin. Two-dimensional electrophoresis coupled with mass spectrometry analysis was used to identify the differentially expressed proteome of serum exosomes in patients with Kawasaki disease before and after intravenous immunoglobulin therapy. Our analysis revealed 69 differential protein spots in the Kawasaki disease group with changes larger than 1.5-fold and 59 differential ones in patients after intravenous immunoglobulin therapy compared with the control group. Gene ontology analysis revealed that the acute-phase response disappeared, the functions of the complement system and innate immune response were enhanced, and the antibacterial humoral response pathway of corticosteroids and cardioprotection emerged after administration of intravenous immunoglobulin. Further, we showed that complement C3 and apolipoprotein A-IV levels increased before and decreased after intravenous immunoglobulin therapy and that the insulin-like growth factor-binding protein complex acid labile subunit displayed reverse alteration before and after intravenous immunoglobulin therapy. These observations might be potential indicators of intravenous immunoglobulin function. Our results show the differential proteomic profile of serum exosomes of patients with Kawasaki disease before and after intravenous immunoglobulin therapy, such as complement C3, apolipoprotein A-IV, and insulin-like growth factor-binding protein complex acid labile subunit. These results may be useful in the identification of markers for monitoring intravenous immunoglobulin therapy in patients with Kawasaki disease.

  12. CT Analysis of Retropharyngeal Abnormality in Kawasaki Disease

    Energy Technology Data Exchange (ETDEWEB)

    Roh, Kyung Min; Lee, Sun Wha; Yoo, Heong Hyun [School of Medicine, Ewha Womans University, Seoul (Korea, Republic of)

    2011-11-15

    To retrospectively compare the imaging characteristics of retropharyngeal density and associated findings for Kawasaki disease with those for non-Kawasaki disease, and identify the distinguishing features which aid the CT diagnosis of Kawasaki disease with retropharyngeal low density. Among the enhanced neck CT performed in children less than 8-years old with clinical presentation of fever and cervical lymphadenopathy over a 6-year period, only cases with retropharyngeal low density (RLD) were included in this study. The 56 cases of RLD were divided into two groups; group A included cases diagnosed as Kawasaki disease (n = 34) and group B included cases diagnosed as non-Kawasaki disease (n = 22). We evaluated the CT features including the thickness of RLD and its extent into the deep neck spaces, as well as soft tissue change in the adjacent structure. We also scored the extent of RLD into the deep neck spaces and the soft tissue changes in the adjacent structure. The thickness of RLD was greater in group A than in group B (group A, 6.0 {+-} 2.1; group B, 4.6 {+-} 1.5, p = 0.01). The score of the RLD extent into the deep neck spaces was significantly greater in group A than in group B (group A, 2.3 {+-} 1.3; group B, 0.8 {+-} 1.0, p < 0.01). Also, the score of the adjacent soft tissue changes was greater in group A than in group B (group A, 2.0 {+-} 1.1; group B, 1.0 {+-} 1.0, p < 0.01). If children present with fever and cervical lymphadenopathy that display retropharyngeal low density with extension into more deep neck spaces as well as changes in more adjacent soft tissue, the possibility of Kawasaki disease should be considered.

  13. Seasonality of Kawasaki disease: a global perspective.

    Directory of Open Access Journals (Sweden)

    Jane C Burns

    Full Text Available BACKGROUND: Understanding global seasonal patterns of Kawasaki disease (KD may provide insight into the etiology of this vasculitis that is now the most common cause of acquired heart disease in children in developed countries worldwide. METHODS: Data from 1970-2012 from 25 countries distributed over the globe were analyzed for seasonality. The number of KD cases from each location was normalized to minimize the influence of greater numbers from certain locations. The presence of seasonal variation of KD at the individual locations was evaluated using three different tests: time series modeling, spectral analysis, and a Monte Carlo technique. RESULTS: A defined seasonal structure emerged demonstrating broad coherence in fluctuations in KD cases across the Northern Hemisphere extra-tropical latitudes. In the extra-tropical latitudes of the Northern Hemisphere, KD case numbers were highest in January through March and approximately 40% higher than in the months of lowest case numbers from August through October. Datasets were much sparser in the tropics and the Southern Hemisphere extra-tropics and statistical significance of the seasonality tests was weak, but suggested a maximum in May through June, with approximately 30% higher number of cases than in the least active months of February, March and October. The seasonal pattern in the Northern Hemisphere extra-tropics was consistent across the first and second halves of the sample period. CONCLUSION: Using the first global KD time series, analysis of sites located in the Northern Hemisphere extra-tropics revealed statistically significant and consistent seasonal fluctuations in KD case numbers with high numbers in winter and low numbers in late summer and fall. Neither the tropics nor the Southern Hemisphere extra-tropics registered a statistically significant aggregate seasonal cycle. These data suggest a seasonal exposure to a KD agent that operates over large geographic regions and is

  14. Seasonality of Kawasaki disease: a global perspective.

    Science.gov (United States)

    Burns, Jane C; Herzog, Lauren; Fabri, Olivia; Tremoulet, Adriana H; Rodó, Xavier; Uehara, Ritei; Burgner, David; Bainto, Emelia; Pierce, David; Tyree, Mary; Cayan, Daniel

    2013-01-01

    Understanding global seasonal patterns of Kawasaki disease (KD) may provide insight into the etiology of this vasculitis that is now the most common cause of acquired heart disease in children in developed countries worldwide. Data from 1970-2012 from 25 countries distributed over the globe were analyzed for seasonality. The number of KD cases from each location was normalized to minimize the influence of greater numbers from certain locations. The presence of seasonal variation of KD at the individual locations was evaluated using three different tests: time series modeling, spectral analysis, and a Monte Carlo technique. A defined seasonal structure emerged demonstrating broad coherence in fluctuations in KD cases across the Northern Hemisphere extra-tropical latitudes. In the extra-tropical latitudes of the Northern Hemisphere, KD case numbers were highest in January through March and approximately 40% higher than in the months of lowest case numbers from August through October. Datasets were much sparser in the tropics and the Southern Hemisphere extra-tropics and statistical significance of the seasonality tests was weak, but suggested a maximum in May through June, with approximately 30% higher number of cases than in the least active months of February, March and October. The seasonal pattern in the Northern Hemisphere extra-tropics was consistent across the first and second halves of the sample period. Using the first global KD time series, analysis of sites located in the Northern Hemisphere extra-tropics revealed statistically significant and consistent seasonal fluctuations in KD case numbers with high numbers in winter and low numbers in late summer and fall. Neither the tropics nor the Southern Hemisphere extra-tropics registered a statistically significant aggregate seasonal cycle. These data suggest a seasonal exposure to a KD agent that operates over large geographic regions and is concentrated during winter months in the Northern Hemisphere extra-tropics.

  15. Sterile pyuria as the initial finding in a case with Kawasaki disease

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    Bahar Büyükkaragöz

    2015-09-01

    Full Text Available Kawasaki disease is a multisystemic vasculitis of pediatric age group. Early diagnosis and treatment is crucial as coronary artery aneurysms may develop in 20-25% of untreated cases in the 2nd-3rd weeks of the disease. The most common urinary finding in the active period is sterile pyuria; which is believed to be a sign of a more severe systemic inflammation. In this study, a 3.5 year old boy who admitted with high fever was presented. As he had pyuria and elevated acute phase reactants, antibiotic treatment was started with a pre-diagnosis of acute pyelonephritis. Later, with the persistence of fever, detection of a negative urine culture result and emergence of dermal, mucosal and conjunctival changes, incomplete Kawasaki disease (KD was diagnosed. In conclusion, it should be remembered that prolonged fever with sterile pyuria can be the initial findings in KD; before the emergence of typical lesions.

  16. Characteristics and Fate of Systemic Artery Aneurysm after Kawasaki Disease.

    Science.gov (United States)

    Hoshino, Shinsuke; Tsuda, Etsuko; Yamada, Osamu

    2015-07-01

    To determine the long-term outcome of systemic artery aneurysms (SAAs) after Kawasaki disease (KD). We investigated the characteristics and the fate of SAAs in 20 patients using medical records and angiograms. The age of onset of KD ranged from 1 month to 20 months. The interval from the onset of KD to the latest angiogram ranged from 16 months to 24 years. The regression rate of peripheral artery aneurysm and the frequency of stenotic lesions were analyzed by the Kaplan-Meier method in 11 patients who had undergone initial angiography within 4 months. The mean duration of fever was 24 ± 12 days. All 20 patients had at least 1 symmetric pair of aneurysms in bilateral peripheral arteries, and 16 patients had multiple SAAs. The distributions of SAAs was as follows: brachial artery, 30; common iliac artery, 20; internal iliac artery, 21; abdominal aortic aneurysm, 7; and others, 29. The frequencies of regression of SAA and of the occurrence of stenotic lesions at 20 years after the onset of KD were 51% and 25%, respectively (n = 42). The diameter of all SAAs in the acute phase leading to stenotic lesions in the late period was >10 mm. SAAs occurred symmetrically and were multiple in younger infants and those with severe acute vasculitis. The fate of SAAs resembles that of coronary artery aneurysms, and depends on the diameter during the acute phase. Larger SAAs can lead to stenotic lesions in the late period. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. Comportamiento clínico-epidemiológico de la enfermedad de Kawasaki Clinical-epidemiological behavior of Kawasaky' disease

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    Milagros Morales Leiva

    2011-06-01

    Full Text Available INTRODUCCIÓN. La enfermedad de Kawasaki es una vasculitis sistémica, aguda, febril, de evolución autolimitada, con alto riesgo de secuelas en niños menores de 5 años, si no se diagnostica y trata en su estadio inicial. Fue objetivo de este estudio describir el comportamiento clínico-epidemiológico de la enfermedad de Kawasaki en la infancia. MÉTODOS. Se realizó un estudio descriptivo, de aspectos clínicos y epidemiológicos, de los pacientes con diagnóstico de enfermedad de Kawasaki atendidos en el Hospital Pediátrico «William Soler» entre enero del 2002 y diciembre del 2009. Las variables estudiadas fueron edad, sexo, manifestaciones clínicas, fecha de inicio de los síntomas y de diagnóstico, exámenes complementarios y tratamiento, y respuesta al tratamiento. RESULTADOS. El 100 % de los pacientes eran menores de 8 años. El 57,1 % fueron del sexo masculino. La enfermedad se observó en mayor frecuencia en la época de sequía. En el 100 % de los pacientes se documentó fiebre de más de 5 días de evolución, inyección conjuntival, lesiones orofaríngeas y eritema palmar y plantar; en el 85,7 % de los pacientes se halló exantema polimorfo y en el 57,1 %, adenopatía única cervical. La eritrosedimentación se observó elevada en el 100 % de los casos y en el 85,7 % se determinó anemia. Tres pacientes (42,8 % presentaron alteraciones coronarias. Todos los pacientes fueron tratados con inmunoglobulina endovenosa (Intacglobin y aspirina; en 3 de los casos fue necesario administrar una segunda dosis de inmunoglobulina endovenosa.INTRODUCTION. The Kawasaki's disease is a systemic, acute, febrile of auto-course vasculitis with a high risk of sequelae in children under 5 years old if it is not diagnosed and treated in its early stage. The aim of present study was to describe the clinical-epidemiological behavior of the Kawasaki's disease in childhood. METHODS. A descriptive study was conducted of the clinical and epidemiological

  18. [Giant coronary aneurysms in infants with Kawasaki disease].

    Science.gov (United States)

    Sánchez Andrés, Antonio; Salvador Mercader, Inmaculada; Seller Moya, Julia; Carrasco Moreno, José Ignacio

    2017-08-01

    Kawasaki disease (KD) is an acute vasculitis of unknown origin and predominant in males. The long-term effects of the disease depend on whether there are coronary lesions, particularly aneurysms. The prognosis of patients with giant aneurysms is very poor due to their natural progression to coronary thrombosis or severe obstructive lesions. A series of 8 cases is presented where the epidemiology and diagnostic methods are described. The treatment of the acute and long-term cardiovascular sequelae is also reviewed. A descriptive analysis was conducted on patients admitted to the Paediatric Cardiology Unit of La Fe University Hospital (Valencia) with KD and a coronary lesion. More than one artery was involved in all patients. Although early diagnosis was established in only two cases, none of the patients had severe impairment of ventricular function during the acute phase. Treatment included intravenous gammaglobulin and acetylsalicylic acid at anti-inflammatory doses during the acute phase. A combination of dual antiplatelet therapy and corticosteroids was given in cases of coronary thrombosis. The silent aneurysms continue to persist. KD is the most common cause of acquired heart disease in children. The delay in diagnosis is associated with a greater likelihood of coronary lesions that could increase the risk of cardiovascular events in adulthood. Thus, this subgroup requires close clinical monitoring for a better control of cardiovascular risk factors over time. Copyright © 2016 Asociación Española de Pediatría. Publicado por Elsevier España, S.L.U. All rights reserved.

  19. Resistant Kawasaki disease, case report and literatures review

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    M.J. Saffar

    2006-01-01

    Full Text Available A child with aggressive and resistant Kawasaki disease with coronary aneurysm is described. Despite two doses of immune globulin and high dose of aspirin the patient was treated with the third dose of immune globulin. The patient apparently responded to normalization of symptoms and signs, with no more progression of coronary artery abnormalities.

  20. Evaluation of echogenicity of the heart in Kawasaki disease.

    Science.gov (United States)

    Nagata, Hazumu; Yamamura, Kenichiro; Uike, Kiyoshi; Nakashima, Yasutaka; Hirata, Yuichiro; Morihana, Eiji; Mizuno, Yumi; Ishikawa, Shiro; Hara, Toshiro

    2014-08-01

    Pathologic studies of the heart in patients with Kawasaki disease (KD) revealed vasculitis, valvulitis, myocarditis, and pericarditis. However, there have been no studies on the quantitative determination of multi-site echogenicity of the heart in KD patients. It is also undetermined whether the degree of echogenicity of each site of the heart in patients with KD might be related to the response to intravenous immunoglobulin (IVIG) treatment. In 81 KD patients and 30 control subjects, we prospectively analyzed echogenicity of the heart. Echogenicity was measured in four sites: coronary artery wall (CAW), mitral valve (MV), papillary muscle (PM), and ascending aortic wall (AAo wall) by the calibrated integrated backscatters (cIBs). The cIB values of all measurement sites at acute phase in KD patients were significantly higher than those in control subjects (KD patients vs control subjects; CAW, 19.8 ± 6.2 dB vs 14.5 ± 2.0 dB, p IVIG nonresponders were significantly higher than those in responders. Conclusion: Echogenicity of the heart in KD patients at the acute phase increased not only in the coronary artery wall but also in other parts of the heart. Echogenicity of CAW might be helpful in determining the unresponsiveness of IVIG treatment.

  1. Recognising Kawasaki disease in UK primary care: a descriptive study using the Clinical Practice Research Datalink.

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    Moore, Abigail; Harnden, Anthony; Mayon-White, Richard

    2014-08-01

    Kawasaki disease is a rare childhood illness that can present non-specifically, making it a diagnostic challenge. The clinical presentation of Kawasaki disease has not been previously described in primary care. To describe how children with an eventual diagnosis of Kawasaki disease initially present to primary care in the UK. The Clinical Practice Research Datalink was used to find cases coded as Kawasaki disease. Hospital Episode Statistics, hospital admissions, and hospital outpatient attendances were used to identify the children with a convincing diagnosis of Kawasaki disease. Questionnaires and a request for copies of relevant hospital summaries, discharge letters, and reports were sent to GPs of the 104 children with a diagnosis of Kawasaki disease between 2007 and 2011. Most children presented with few clinical features typical of Kawasaki disease. Of those with just one feature, a fever or a polymorphous rash were the most common. By the time that most children were admitted to hospital they had a more recognisable syndrome, with three or more clinical features diagnostic of Kawasaki disease. Most GPs did not consider Kawasaki disease among their differential diagnoses, but some GPs did suspect that the child's illness was unusual. The study highlighted the difficulty of early diagnosis, with most children having a non-specific presentation to primary care. GPs are encouraged to implement good safety netting, and to keep Kawasaki disease in mind when children present with fever and rashes. © British Journal of General Practice 2014.

  2. Development of Kawasaki disease in a patient with PFAPA.

    Science.gov (United States)

    Ninomiya, Takahito; Takada, Hidetoshi; Nagatomo, Yusaku; Nanishi, Etsuro; Nagata, Hazumu; Yamamura, Kenichiro; Doi, Takehiko; Ikeda, Kazuyuki; Hara, Toshiro

    2013-12-01

    Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA) is one of the autoinflammatory diseases of unknown etiology characterized by regularly recurrent fever episodes with attacks lasting 3-6 days every 3-8 weeks associated with at least one of the three cardinal clinical signs: aphthous stomatitis, pharyngitis, and cervical adenitis. Kawasaki disease (KD) is an acute, self-limited systemic vasculitis that occurs predominantly in infants and young children. In most KD patients, i.v. immunoglobulin leads to a rapid amelioration of clinical symptoms and significantly decreases the risk of coronary artery aneurysms. Although the etiology of KD is still unknown, it was reported that innate immunity was activated in the patients. Described herein is a patient with PFAPA who developed KD. This is the first report of KD development in a PFAPA patient. The association between KD and PFAPA may represent a genetic predisposition to dysregulated innate immune response. © 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.

  3. Pediatric Kawasaki Disease and Adult Human Immunodeficiency Virus Kawasaki-Like Syndrome Are Likely the Same Malady.

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    Johnson, Raymond M; Bergmann, Kelly R; Manaloor, John J; Yu, Xiaoqing; Slaven, James E; Kharbanda, Anupam B

    2016-09-01

    Background.  Pediatric Kawasaki disease (KD) and human immunodeficiency virus (HIV)(+) adult Kawasaki-like syndrome (KLS) are dramatic vasculitides with similar physical findings. Both syndromes include unusual arterial histopathology with immunoglobulin (Ig)A(+) plasma cells, and both impressively respond to pooled Ig therapy. Their distinctive presentations, histopathology, and therapeutic response suggest a common etiology. Because blood is in immediate contact with inflamed arteries, we investigated whether KD and KLS share an inflammatory signature in serum. Methods.  A custom multiplex enzyme-linked immunosorbent assay (ELISA) defined the serum cytokine milieu in 2 adults with KLS during acute and convalescent phases, with asymptomatic HIV(+) subjects not taking antiretroviral therapy serving as controls. We then prospectively collected serum and plasma samples from children hospitalized with KD, unrelated febrile illnesses, and noninfectious conditions, analyzing them with a custom multiplex ELISA based on the KLS data. Results.  Patients with KLS and KD subjects shared an inflammatory signature including acute-phase reactants reflecting tumor necrosis factor (TNF)-α biologic activity (soluble TNF receptor I/II) and endothelial/smooth muscle chemokines Ccl1 (Th2), Ccl2 (vascular inflammation), and Cxcl11 (plasma cell recruitment). Ccl1 was specifically elevated in KD versus febrile controls, suggesting a unique relationship between Ccl1 and KD/KLS pathogenesis. Conclusions.  This study defines a KD/KLS inflammatory signature mirroring a dysfunctional response likely to a common etiologic agent. The KD/KLS inflammatory signature based on elevated acute-phase reactants and specific endothelial/smooth muscle chemokines was able to identify KD subjects versus febrile controls, and it may serve as a practicable diagnostic test for KD.

  4. Qing Re Liang Xue Decoction Alleviates Hypercoagulability in Kawasaki Disease

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    Jiao-yang Chen

    2015-01-01

    Full Text Available Objective. Kawasaki disease (KD is a multisystemic autoimmune vasculitis. Intravenous immunoglobulin (IVIG is the first-line treatment for KD. It is unclear whether traditional Chinese medicine (TCM has an effect on KD. We aimed to observe the clinical efficacy of TCM on acute KD via serum interleukin-33 (IL-33 and tumor necrosis factor alpha (TNF-α measurements. Methods. Thirty-one KD patients were treated with Qing Re Liang Xue decoction and Western medicine (integrative medicine treatment group, while 28 KD patients were treated with Western medicine only (Western medicine treatment group. Thirty patients were included in a febrile group and 28 healthy children were included in the control group. Clinical characteristics and laboratory findings were gathered and compared. Serum IL-33 and TNF-α levels were measured by multiplex Luminex assay. Results. The platelet count in the integrative medicine treatment group was significantly lower than that in the Western medicine treatment group. The integrative medicine group had a shorter fever duration and lower IL-33 and TNF-α levels than those in the Western medicine group, but there were no significant differences between the two KD groups after treatment. Conclusion. Qing Re Liang Xue decoction improved the hypercoagulable state of KD patients. Potential myocardial protective effects require further research.

  5. Epidemiology of Kawasaki disease in Italy: surveillance from national hospitalization records.

    Science.gov (United States)

    Cimaz, Rolando; Fanti, Eleonora; Mauro, Angela; Voller, Fabio; Rusconi, Franca

    2017-08-01

    Kawasaki disease is a systemic vasculitis with an acute and self-limited course. The incidence of the disease differs widely among ethnic groups and is higher in the Asian populations. In Italy, no recent data are available. We studied the epidemiology of Kawasaki disease in the years 2008-2013 in children 0-14 years old in Italy using hospital ICD-9 discharge codes with a thorough data cleaning for duplicates in order to select the first hospital admission for the disease. The disease peaked in the first 2 years of life, with 85.5% of cases under 5 years. Male/female ratio was 1.4:1. The incidence rate was 5.7 per 100,000 children 0-14 years old and 14.7 for children younger than 5 years. The incidence rose slightly during the study period and had a seasonal distribution, with higher incidence in spring. A coronary artery aneurysm was recorded in 2.2% of the patients younger than 5 years of age. This is the first epidemiologic study on Kawasaki disease incidence in the country of Italy. Figures are in line but slightly higher than those reported for other European countries. What is known: • Kawasaki disease is more common in Asian populations. • European incidence data are scarce. What is new: • Epidemiological data in Italy show similar incidence, albeit slightly higher, than in other European countries. • Incidence data slightly rose in the recent past.

  6. Review of Kawasaki Patients in Yazd

    OpenAIRE

    2009-01-01

    Introduction: Kawasaki syndrome is an acute febrile vasculitis with multiorgan involvment in children. The objective of this study was assessment of demographic, clinical and laboratory data of Kawasaki patients in Yazd. Methods: In a descriptive-analytic study, the hospital records of patients treated for Kawasaki disease in all hospitals with pediatric wards between March 1996 and March 2006 at Yazd were reviewed and demographic, clinical and laboratory data recorded in a questionnaire and ...

  7. Preventing Long-Term Cardiac Damage in Pediatric Patients With Kawasaki Disease.

    Science.gov (United States)

    Williams, Kelly

    Kawasaki disease is currently the leading cause of long-term cardiac damage in pediatric patients in the United States. Kawasaki disease is diagnosed based on symptomatology and by ruling out other etiology. There is a significant need for an improved, standardized treatment protocol for patients diagnosed with Kawasaki disease and a more rapid initiation of treatment for these patients. Decreasing the cardiac damage caused by Kawasaki disease with timely diagnosis and treatment needs be a principal goal. Copyright © 2016 National Association of Pediatric Nurse Practitioners. Published by Elsevier Inc. All rights reserved.

  8. Natriuretic Peptides in Kawasaki Disease: the Myocardial Perspective

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    Nagib Dahdah

    2013-01-01

    Full Text Available Making a diagnosis of Kawasaki disease with certainty may be challenging, especially since the recognition of cases with incomplete diagnostic criteria and its consequences. In order to build the diagnostic case in daily practice, clinicians rely on clinical criteria established over four decades ago, aided by non specific laboratory tests, and above all inspired by experience. We have recently studied the diagnostic value of N-terminal pro B-type natriuretic peptide to improve the diagnostic certainty of cases with complete or incomplete clinical criteria. Our working hypothesis was based on the fact that myocarditis is present in nearly all Kawasaki disease patients supported by histology data. In this paper, we review these facts and the myocardial perspective from the diagnostic and the mechanistic standpoints.

  9. Clinical aspects of 100 patients with Kawasaki disease.

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    Tizard, E J; Suzuki, A; Levin, M; Dillon, M J

    1991-02-01

    We report 101 episodes of Kawasaki disease in 100 patients seen over a 12 year period. A total of 35 patients had cardiac involvement ranging from pericardial effusion to coronary artery aneurysms with ischaemic complications, which resulted in death in one patient. Laboratory investigations showed leucocytosis, thrombocytosis, and a raised erythrocyte sedimentation rate to be common features and the first two variables were significantly associated with cardiac involvement. Treatment regimens changed over the study period. Aspirin was used in most patients often in conjunction with dipyridamole and from 1986 intravenous immunoglobulin was given routinely to those patients seen early in the illness. Additional therapeutic measures in individual patients included prostacyclin, heparin, streptokinase, and plasma exchange/exchange transfusion. Attention is drawn to the uncertainity of the long term cardiovascular consequences in the light of adults reported with premature atherosclerotic lesions of similar appearance to those seen in Kawasaki disease.

  10. Establishment of Kawasaki disease database based on metadata standard.

    Science.gov (United States)

    Park, Yu Rang; Kim, Jae-Jung; Yoon, Young Jo; Yoon, Young-Kwang; Koo, Ha Yeong; Hong, Young Mi; Jang, Gi Young; Shin, Soo-Yong; Lee, Jong-Keuk

    2016-07-01

    Kawasaki disease (KD) is a rare disease that occurs predominantly in infants and young children. To identify KD susceptibility genes and to develop a diagnostic test, a specific therapy, or prevention method, collecting KD patients' clinical and genomic data is one of the major issues. For this purpose, Kawasaki Disease Database (KDD) was developed based on the efforts of Korean Kawasaki Disease Genetics Consortium (KKDGC). KDD is a collection of 1292 clinical data and genomic samples of 1283 patients from 13 KKDGC-participating hospitals. Each sample contains the relevant clinical data, genomic DNA and plasma samples isolated from patients' blood, omics data and KD-associated genotype data. Clinical data was collected and saved using the common data elements based on the ISO/IEC 11179 metadata standard. Two genome-wide association study data of total 482 samples and whole exome sequencing data of 12 samples were also collected. In addition, KDD includes the rare cases of KD (16 cases with family history, 46 cases with recurrence, 119 cases with intravenous immunoglobulin non-responsiveness, and 52 cases with coronary artery aneurysm). As the first public database for KD, KDD can significantly facilitate KD studies. All data in KDD can be searchable and downloadable. KDD was implemented in PHP, MySQL and Apache, with all major browsers supported.Database URL: http://www.kawasakidisease.kr.

  11. Blood N-terminal Pro-brain Natriuretic Peptide and Interleukin-17 for Distinguishing Incomplete Kawasaki Disease from Infectious Diseases.

    Science.gov (United States)

    Wu, Ling; Chen, Yuanling; Zhong, Shiling; Li, Yunyan; Dai, Xiahua; Di, Yazhen

    2015-06-01

    To explore the diagnostic value of blood N-terminal pro-brain natriuretic peptide (NT-proBNP) and interleukin-17(IL-17) for incomplete Kawasaki disease. Patients with Kawasaki disease, Incomplete Kawasaki disease and unclear infectious fever were included in this retrospective study. Their clinical features, and laboratory test results of blood NT-proBNP and IL-17 were collected and compared. 766 patients with complete clinical information were recruited, consisting of 291 cases of Kawasaki disease, 74 cases of incomplete Kawasaki disease, and 401 cases of unclear infectious diseases. When the consistency with indicator 2 and 3 in Kawasaki disease diagnosis criteria was assessed with blood IL-17 ?11.55 pg/mL and blood NT-proBNP ? 225.5 pg/dL as the criteria, the sensitivity and specificity for distinguishing incomplete Kawasaki disease and infectious diseases reached 86.5% and 94.8%, respectively. When we chose the consistency with indicator 1 and 2 in Kawasaki disease diagnosis criteria, the appearance of decrustation and/or the BCG erythema, blood IL-17 ?11.55 pg/mL and blood NT-Pro BNP ?225.5 pg/dL as the criteria, the sensitivity and specificity for distinguishing incomplete Kawasaki disease and infectious diseases was 43.2% and 100%, respectively. Blood NT-proBNP and IL-17 are useful laboratory indicators for distinguishing incomplete Kawasaki disease and infectious diseases at the early stage.

  12. Efficacy of magnetic resonance coronary angiography for evaluating coronary arterial lesions due to Kawasaki disease

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    Inaba, Rikako; Suzuki, Atsuko; Sato, Katsuhiko; Ono, Masae; Hosina, Kiyoshi; Furuyama, Tamio; Takemura, Atsusi; Korenaga, Tateo [Tokyo Teishin Hospital (Japan)

    2002-11-01

    To reduce the number of invasive X-ray coronary angiography (XCA) in patients with coronary arterial lesions (CAL) due to Kawasaki disease, we evaluated the efficacy of noninvasive magnetic resonance coronary angiography (MRCA). We preformed MRCA on 30 patients with Kawasaki disease (age 4 months to 37 years; medium 9 years) by SIEMENS MAGNETOM Symphony 1.5 T. MRCA with free respiration using navigator echo 3D fast low angle shot (3D-FLASH) was performed on 10 young children. Evaluation of MRCA was based on the findings of 2D echo cardiogram (30 patients) and XCA (14 patients). All 17 aneurysms (100%) were detected by MRCA; four out of five dilatations were detected by MRCA (80%), but a slight dilatation was not detected (i.e., appeared to be a normal coronary artery). We observed seven stenoses (100%) that were detected on XCA and one new stenosis on MRCA; both of two aortocoronary bypass graphs (one in each of two patients; 100%) were observed to be patent. These results demonstrated that MRCA was highly beneficial, first for detecting CAL after the acute phase even in young infants, second for reducing the number of XCA examination times for following up CAL, and third for screening of CAL in adults with a history of Kawasaki disease. (author)

  13. Kawasaki disease: current aspects on aetiopathogenesis and therapeutic management.

    Science.gov (United States)

    Alexoudi, Iliana; Kanakis, Meletios; Kapsimali, Violetta; Vaiopoulos, George

    2011-07-01

    Kawasaki disease (KD) is a vasculitis that affects mainly children of 6 months to 4 years old. It is important to be early recognised so as to limit the inflammatory cascade that may lead to aneurysmatic dilatations of coronary arteries. The causative agent of KD has not been still indentified and the aetiopathogenetic theories are based on epidemiologic, laboratory and histological data. The management of the disease is divided according to the clinical stage and patients' follow up should be continued for years after the disease onset. The exact period is determined by the risk level of the KD.

  14. Incomplete Kawasaki disease associated with complicated Streptococcus pyogenes pneumonia: A case report.

    Science.gov (United States)

    Leahy, Timothy Ronan; Cohen, Eyal; Allen, Upton D

    2012-01-01

    A three-year-old boy presented with community-acquired pneumonia complicated by empyema. Streptococcus pyogenes (group A streptococcus) was identified on culture of the pleural fluid. The patient improved with antibiotic therapy and drainage of the empyema. During his convalescence, the patient developed persistent fever, lethargy and anorexia. His inflammatory markers were elevated, and repeat cultures were negative. Although the patient had none of the classical mucocutaneous features of Kawasaki disease, an echocardiogram was performed, which revealed coronary artery dilation. The patient was diagnosed with incomplete Kawasaki disease and treated with intravenous immunoglobulin and high-dose acetylsalicylic acid. The fever subsided within 48 h. To the authors' knowledge, the present report is the first report of Kawasaki disease associated with complicated S pyogenes pneumonia. It emphasizes the importance of considering incomplete Kawasaki disease among children with persistent fever, the role of echocardiography in diagnosis, and the potential link between Kawasaki disease and superantigen-producing organisms such as S pyogenes.

  15. Intravenous immunoglobulin treatment responsiveness depends on the degree of CD8+ T cell activation in Kawasaki disease.

    Science.gov (United States)

    Ye, Qing; Gong, Fang-Qi; Shang, Shi-Qiang; Hu, Jian

    2016-10-01

    Kawasaki disease (KD) has become the most common cause of acquired heart disease in children and is also a risk factor for ischemic heart disease in adults. However, Kawasaki disease lacks specific laboratory diagnostic indices. Thus, this study analyzed the T cell activation profiles of Kawasaki disease and assessed their value in the diagnosis of Kawasaki disease and the prediction of intravenous immunoglobulin (IVIG) sensitivity. We analyzed human leukocyte antigen-DR (HLA-DR), CD69 and CD25 expression on peripheral blood CD4+ and CD8+ T cells during the acute phase of KD. We compared the percentages of HLA-DR+/CD69+/CD25+ T cells in the CD4+ and CD8+ T cell populations of IVIG-effective and IVIG-resistant groups. Receiver operating characteristic curves were used to assess the diagnostic value of the above parameters. The median percentage of CD8+HLA-DR+ T cells and the median ratio of CD8+HLA-DR+ T cells/CD8+CD25+ T cells were significantly elevated in the patient group compared with those in the control group during the acute phase of KD. Regarding the diagnosis of Kawasaki disease, the area under the ROC curve was 0.939 for the percentage of CD8+HLA-DR+ T cells. There was a significant difference in the ratio of CD8+HLA-DR+ T cells/CD8+CD69+ T cells between IVIG-resistant patients and IVIG-sensitive patients. Regarding IVIG sensitivity, the area under the ROC curve was 0.795 for it. Excessive CD8+ T cell activation, as well as an imbalance between CD8+ T cell activation and inhibition, underlies the pathogenesis of Kawasaki disease. The percentage of CD8+ HLA-DR+ T cells may be used as an index to diagnose Kawasaki disease. IVIG inhibits CD8+ T cell activation, but excessive CD8+ T cell activation may cause IVIG resistance. The ratio of CD8+HLA-DR+ T cells/CD8+CD69+ T cells may be used as a predictor of IVIG sensitivity. Copyright © 2016. Published by Elsevier Inc.

  16. Childhood vaccines and Kawasaki disease, Vaccine Safety Datalink, 1996-2006.

    Science.gov (United States)

    Abrams, Joseph Y; Weintraub, Eric S; Baggs, James M; McCarthy, Natalie L; Schonberger, Lawrence B; Lee, Grace M; Klein, Nicola P; Belongia, Edward A; Jackson, Michael L; Naleway, Allison L; Nordin, James D; Hambidge, Simon J; Belay, Ermias D

    2015-01-03

    Kawasaki disease is a childhood vascular disorder of unknown etiology. Concerns have been raised about vaccinations being a potential risk factor for Kawasaki disease. Data from the Vaccine Safety Datalink were collected on children aged 0-6 years at seven managed care organizations across the United States. Defining exposure as one of several time periods up to 42 days after vaccination, we conducted Poisson regressions controlling for age, sex, season, and managed care organization to determine if rates of physician-diagnosed and verified Kawasaki disease were elevated following vaccination compared to rates during all unexposed periods. We also performed case-crossover analyses to control for unmeasured confounding. A total of 1,721,186 children aged 0-6 years from seven managed care organizations were followed for a combined 4,417,766 person-years. The rate of verified Kawasaki disease was significantly lower during the 1-42 days after vaccination (rate ratio=0.50, 95% CL=0.27-0.92) and 8-42 days after vaccination (rate ratio=0.45, 95% CL=0.22-0.90) compared to rates during unexposed periods. Breaking down the analysis by vaccination category did not identify a subset of vaccines which was solely responsible for this association. The case-crossover analyses revealed that children with Kawasaki disease had lower rates of vaccination in the 42 days prior to symptom onset for both physician-diagnosed Kawasaki disease (rate ratio=0.79, 95% CL=0.64-0.97) and verified Kawasaki disease (rate ratio=0.38, 95% CL=0.20-0.75). Childhood vaccinations' studied did not increase the risk of Kawasaki disease; conversely, vaccination was associated with a transient decrease in Kawasaki disease incidence. Verifying and understanding this potential protective effect could yield clues to the underlying etiology of Kawasaki disease. Copyright © 2014. Published by Elsevier Ltd.

  17. Augmented TLR2 Expression on Monocytes in both Human Kawasaki Disease and a Mouse Model of Coronary Arteritis

    OpenAIRE

    I-Chun Lin; Ho-Chang Kuo; Ying-Jui Lin; Feng-Shen Wang; Lin Wang; Shun-Chen Huang; Shao-Ju Chien; Chien-Fu Huang; Chih-Lu Wang; Hong-Ren Yu; Rong-Fu Chen; Yang, Kuender D.

    2012-01-01

    BACKGROUND: Kawasaki disease (KD) of unknown immunopathogenesis is an acute febrile systemic vasculitis and the leading cause of acquired heart diseases in childhood. To search for a better strategy for the prevention and treatment of KD, this study compared and validated human KD immunopathogenesis in a mouse model of Lactobacillus casei cell wall extract (LCWE)-induced coronary arteritis. METHODS: Recruited subjects fulfilled the criteria of KD and were admitted for intravenous gamma globul...

  18. 我国川崎病的流行病学特征%Epidemiological features of Kawasaki disease in China

    Institute of Scientific and Technical Information of China (English)

    潘晶莹

    2013-01-01

    川崎病是一种以全身血管炎为主要病变的急性发热出疹性小儿疾病.川崎病致冠状动脉病变已成为小儿最常见的后天性心脏病.近年来国内外围绕川崎病的流行病学调查已大量展开,通过总结近20年来我国川崎病的流行病学特征,包括不同地区在发病率、年龄、性别、民族、家族史、季节性、复发率、心脏损害、病死率方面的特征,以期为我国川崎病的临床诊疗提供依据.%Kawasaki disease,also known as mucocutaneous lymph node syndrome,is an acute,febrile,multisystem disease in children.The coronary artery lesions caused by Kawasaki disease has become the most common acquired heart disease in children.In recent years,epidemiological investigation on Kawasaki disease has been launched in the world.It can provide the basis for clinical diagnosis and treatment of Kawasaki disease through summarizing the epidemiological characteristics of Kawasaki disease in the different regions in China in recent 20 years,including the characteristics of incidence,age,gender,nationality,family history,seasonal onset,relapse rate,mortality and heart damage.

  19. Multimodality Cardiac Imaging in a Patient with Kawasaki Disease and Giant Aneurysms

    Science.gov (United States)

    Srinivasan, Ranjini; Weller, Rachel; Einstein, Andrew J.

    2016-01-01

    Kawasaki disease is a well-known cause of acquired cardiac disease in the pediatric and adult population, most prevalent in Japan but also seen commonly in the United States. In the era of intravenous immunoglobulin (IVIG) treatment, the morbidity associated with this disease has decreased, but it remains a serious illness. Here we present the case of an adolescent, initially diagnosed with Kawasaki disease as an infant, that progressed to giant aneurysm formation and calcification of the coronary arteries. We review his case and the literature, focusing on the integral role of multimodality imaging in managing Kawasaki disease. PMID:27872783

  20. Prevalence and predictors of coronary artery disease in adults with Kawasaki disease.

    Science.gov (United States)

    Garg, Jalaj; Krishnamoorthy, Parasuram; Palaniswamy, Chandrasekar; Paudel, Rajiv; Chatterjee, Saurav; Ahmad, Hasan; Snyder, Christopher

    2015-08-01

    Accelerated coronary atherosclerosis in patients with Kawasaki disease, in conjunction with coronary artery aneurysm and stenosis that characterise this disease, are potential risk factors for developing coronary artery disease in young adults. We aimed to determine the prevalence and predictors of coronary artery disease in adult patients with Kawasaki disease. All patients aged 18-55 years of age diagnosed with Kawasaki disease were sampled from Nationwide Inpatient Sample database using International Classification of Diseases 9th revision (ICD 9 code 446.1) from 2009 to 2010. Demographics, prevalence of coronary artery disease, and other traditional risk factors in adult patients with Kawasaki disease were analysed using ICD 9 codes. The prevalence of Kawasaki disease among adults was 0.0005% (n=215) of all in-hospital admissions in United States. The mean age was 27.3 years with women (27.6 years) older than men (27.1 years). Traditional risk factors were hypertension (21%), hyperlipidaemia (15.6%), diabetes (11.5%), tobacco use (8.8%), and obesity (8.8%), with no significant difference between men and women. Coronary artery disease (32.4%), however, was more prevalent in men (44.7%) than in women (12.1%; p=0.03). In multivariate regression analysis, after adjusting for demographics and traditional risk factors, hypertension (OR=13.2, p=0.03) was an independent risk factor of coronary artery disease. There was increased preponderance of coronary artery disease in men with Kawasaki disease. On multivariate analysis, hypertension was found to be the only independent predictor of coronary artery disease in this population after adjusting for other risk factors.

  1. [Occurrence of Tsutsugamushi disease infection by Orientia tsutsugamushi, Kawasaki serotype, in Yamagata Prefecture, Japan].

    Science.gov (United States)

    Otani, Katsumi; Kaneko, Akiko; Aoki, Toshiya; Murata, Toshio

    2009-09-01

    Of 95 Tsutsugamushi disease case occurring in Yamagata prefecture from 1999 to 2006, four-all women-involved the O. tsutsugamushi Kawasaki serotype. The three major symptoms were fever, exanthema, and eschar present from mid-October to early November. Serodiagnosis by indirect immunofluoresence assay showed elevated IgG and IgM antibody titers against the Kawasaki serotype antigen, with IgM higher than IgG. Nested PCR detected 56-kDa DNA in three of the cases. DNA was amplified in Kawasaki-specific PCR. Two cases for which sequencing was done using nested PCR-amplified DNA showed an identity of 99.8% for the Kawasaki strain (Accession number: M63383). These results confirmed the occurrence of Tsutsugamushi disease infection involving Kawasaki serotype in Yamagata prefecture.

  2. Comparative analysis of incomplete Kawasaki disease and typical Kawasaki disease%不完全川崎病与典型性川崎病对比分析

    Institute of Scientific and Technical Information of China (English)

    郑礼林; 吕勇; 潘家华

    2014-01-01

    目的 探讨不完全川崎病与典型性川崎病的异同以便早诊断、早治疗.方法 回顾分析60例确诊为川崎病患儿的临床和实验室资料.结果 不完全川崎病临床诊断指标中的症状出现率较典型性川崎病低(x2 =16.46、10.10、11.71、34.43,均P<0.01),两者实验室检查指标WBC、Pt、ESR及CRP之间差异无统计学意义(x2 =0.04、0.12、0.04、0.26,均P>0.05),两者冠状动脉病变发生率差异有统计学意义(x2=31.43,P<0.05).结论 川崎病的早期诊断要结合临床特点、实验室检查和超声心动图检查.%Objective To investigate the similarities and differences of incomplete Kawasaki disease and typical Kawasaki disease,in order to provide basis for early diagnosis and treatment.Methods The clinical and laboratory data of 60 children with Kawasaki disease were retrospectively analyzed.Results The incidence rate of symptoms in clinical diagnostic criteria was lower in incomplete Kawasaki disease than that in typical Kawasaki disease(x2 =16.46,10.10,11.71,34.43,all P < 0.01).No statistical differences of leukocytes,platelet,erythrocyte sedim-entation rate and C-reactive protein were found in the incomplete Kawasaki disease and typical Kawasaki disease(x2 =0.04,0.12,0.04,0.26,all P > 0.05).The incidence rate of coronary artery lesions had significant difference between incomplete Kawasaki disease and typical Kawasaki disease (x2 =31.43,P < 0.01).Conclusion The early diagnosis of Kawasaki disease could be confirmed by representative clinical characteristics,laboratory examination and ultrasound cardiogram.

  3. Relationship between eosinophilia & coronary artery lesions in Kawasaki disease: a survey in Imam Khomeini Hospital

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    Shariat M.

    2008-03-01

    Full Text Available Background: Kawasaki disease (KD is an acute vasculitis in children. Eosinophilia, a reflection of the host's immune response that can cause tissue damage, has been associated with KD, with eosinophils preferentially accumulating in the microvasculature. In early-stage Kawasaki disease (KD, lesions (perivasculitis and vasculitis first form in the microvessels, which can then extend to the larger vessels and result in coronary artery aneurysms, possibly leading to myocardial infarction even in young children. Overall, the prevalence of coronary artery aneurysms in children with Kawasaki disease is about 10-18%, which is much higher among those not treated early in the course of the illness.  We performed this study to gain a better understanding of the initial pathogenesis of KD and to assess the relationship between eosinophilia and coronary artery disease.Methods:  The data from forty-eight patients at Vali-asr Hospital of the Tehran University of Medical Sciences (1996-2006 were included in this cross-sectional descriptive analysis. The presence and degree of coronary artery disease was assessed by echocardiography. Data was analyzed via Fisher's exact test and student's t-test using SPSS software, v. 11.5.Results: Eosinophilia was seen in 10 cases (22% and cardiac lesions were observed in 19 cases (41%. The frequency of microvessel lesions was significantly lower in patients with eosinophilia (10% with eosinophilia versus 50% without eosinophilia, p<0.03. The frequency of microvessel lesions was lower in males than in females (35 vs. 44%, respectively, although this was not significant. We found no correlation between the frequency of microvessel lesions and age.Conclusions: In spite of the controversies regarding eosinophilia and microvessel lesions, in this study the number of circulating eosinophils was associated with fewer cardiac lesions. Comparative studies are needed to determine the exact relationship.

  4. Platelet Endothelial Cell Adhesion Molecule-1 Gene Polymorphisms are Associated with Coronary Artery Lesions in the Chronic Stage of Kawasaki Disease.

    Science.gov (United States)

    Lu, Wen-Hsien; Huang, Sin-Jhih; Yuh, Yeong-Seng; Hsieh, Kai-Sheng; Tang, Chia-Wan; Liou, Huei-Han; Ger, Luo-Ping

    2017-05-01

    Kawasaki disease is the most common cause of pediatric acquired heart disease. The role of platelet endothelial cell adhesion molecule-1 in the inflammatory process has been documented. To date, no report has investigated the relationship between coronary artery lesions of Kawasaki disease and platelet endothelial cell adhesion molecule-1 polymorphisms. A total of 114 Kawasaki disease children with coronary artery lesions and 185 Kawasaki disease children without coronary artery lesions were recruited in this study. The TaqMan assay was conducted to identify the genotype in this case-control study. In three single nucleotide polymorphisms (Leu125Val, Ser563Asn, and Arg670Gly) of platelet endothelial cell adhesion molecule-1, we found that the Leu-Ser-Arg haplotype was associated with a significantly increased risk for coronary artery lesions in the chronic stage (odds ratio 3.05, 95% confidence interval 1.06-8.80, p = 0.039), but not for coronary artery lesions in the acute stage. Analysis based on the diplotypes of platelet endothelial cell adhesion molecule-1 also showed that Kawasaki disease with one or two alleles of Leu-Ser-Arg had a significantly increased risk of chronic coronary artery lesions (odds ratio 3.38, 95% confidence interval 1.11-10.28, p = 0.032) and had increased platelet counts after Kawasaki disease was diagnosed, as compared to those with other diplotypes. The haplotype of platelet endothelial cell adhesion molecule-1 Leu-Ser-Arg might be associated with the increased platelet counts and the following risk of chronic coronary artery lesions in a dominant manner in Kawasaki disease.

  5. 川崎病的研究进展%Research Progress of Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    刘丽莎

    2012-01-01

    Kawasaki disease is an acute fehrile rash disease of systemic vascllitis with predilection in infants and yuong children under 5 years of age,the etiology of which is still unkown.It is associated with the development of coronary artery abonormalities such as coronary artery dilation and coronary aneurysm, wasaki disease is rising and has received extensive attention from scholars from various countries.Here is to make a review on the current situation of research of Kawasaki disease.%川崎病是一种以全身血管炎为主要病变的急性发热性出疹性疾病,好发于5岁以下婴幼儿,但其病因和发病机制目前尚未完全清楚,其严重的并发症是心血管系统损害,并可形成冠状动脉扩张和冠状动脉瘤,已经成为小儿获得性心脏病的主要病因.近年来该病的发病率呈上升趋势,得到了各国学者的广泛关注和重视,现就目前有关川崎病的研究进展予以综述.

  6. Echocardiography in Diagnosis and Prevention of the Coronary Artery Lesions in Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    Pan Baoquan; Xu Zongyi; Zheng Manlei; Feng Zhekang

    2000-01-01

    @@Kawasaki disease (muco-cutaneous lymph node syndrome, MCLS) was first reported by Kawasaki in 1967. It was characteried by nonvascular polymorphous rash, fever, ulcer in oral mucosa, edema of hands of feet, cervix lymphadenopathy and desquamations in peripheral extremities. Because of unknown pathogens, no lab examination was available as a single easily recognized diagnostic marker; the diagnosis is based on clinical features. The main complication of Kawasaki disease is coronary artery (CA) lesion; the prognosis is determined by the degree of pathological change of the coronary artery[1.2].

  7. Kawasaki Disease Presenting As Oculoglandular Syndrome: Accidental Concurrence or Something Beyond That?

    Directory of Open Access Journals (Sweden)

    Sedighi

    2016-07-01

    Full Text Available Introduction Kawasaki disease (KD is an acute febrile illness of childhood that can lead to significant coronary artery abnormalities, particularly in untreated patients. Diagnosis of KD is made in the presence of its standard criteria, including bilateral conjunctivitis. Some patients do not fulfill the diagnostic criteria and are known as atypical KD. Case Presentation In this report, we describe a 12-year-old boy presenting with prolonged fever, unilateral conjunctivitis, ipsilateral preauricular lymphadenopathy, skin rashes, and finger scaling. The initial evaluation for KD was negative, so he received gentamycin as a case of oculoglandular syndrome. The fever subsided, but he developed bilateral conjunctivitis later in course of the disease. A second echocardiograph revealed coronary artery dilation. He immediately received intravenous immunoglobulin (IVIG and aspirin and was discharged from hospital with a recommendation of close follow-up. Conclusions To our knowledge, KD presenting with unilateral conjunctivitis and oculoglandular syndrome is not reported to date. G iven that delay in diagnosis and treatment of KD can cause serious cardiac complications, the diagnosis of Kawasaki disease should be considered in such cases.

  8. Septated pericarditis associated with Kawasaki disease: a brief case report.

    Science.gov (United States)

    Sonçaği, Arzu; Devrim, Ilker; Karagöz, Tevfik; Dilber, Embiya; Celiker, Alpay; Ozen, Seza; Seçmeer, Gülten

    2007-01-01

    Kawasaki disease (KD) is primarily the systemic vasculitis of childhood that affects mainly the medium-sized arteries, such as the coronary arteries. KD is the leading cause of acquired heart disease, whereas the incidence of rheumatic fever has declined. The most serious complication is coronary artery involvement. Among the children with KD who developed cardiac complications, pericarditis is a rare complication, with an incidence of 0.07%. We report our experience in a 5.5-year-old child with KD complicated with aneurysm of the left anterior descendant coronary artery and septated pericardial effusion, which has not been reported in the literature. The pericardial effusion disappeared very dramatically with intravenous immunoglobulin (IVIG) therapy. We would like to point out that septated pericardial effusion in cases of KD do not need any further therapy other than IVIG and high-dose acetylsalicylic acid.

  9. ITPKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms.

    Science.gov (United States)

    Onouchi, Yoshihiro; Gunji, Tomohiko; Burns, Jane C; Shimizu, Chisato; Newburger, Jane W; Yashiro, Mayumi; Nakamura, Yoshikazu; Yanagawa, Hiroshi; Wakui, Keiko; Fukushima, Yoshimitsu; Kishi, Fumio; Hamamoto, Kunihiro; Terai, Masaru; Sato, Yoshitake; Ouchi, Kazunobu; Saji, Tsutomu; Nariai, Akiyoshi; Kaburagi, Yoichi; Yoshikawa, Tetsushi; Suzuki, Kyoko; Tanaka, Takeo; Nagai, Toshiro; Cho, Hideo; Fujino, Akihiro; Sekine, Akihiro; Nakamichi, Reiichiro; Tsunoda, Tatsuhiko; Kawasaki, Tomisaku; Nakamura, Yusuke; Hata, Akira

    2008-01-01

    Kawasaki disease is a pediatric systemic vasculitis of unknown etiology for which a genetic influence is suspected. We identified a functional SNP (itpkc_3) in the inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene on chromosome 19q13.2 that is significantly associated with Kawasaki disease susceptibility and also with an increased risk of coronary artery lesions in both Japanese and US children. Transfection experiments showed that the C allele of itpkc_3 reduces splicing efficiency of the ITPKC mRNA. ITPKC acts as a negative regulator of T-cell activation through the Ca2+/NFAT signaling pathway, and the C allele may contribute to immune hyper-reactivity in Kawasaki disease. This finding provides new insights into the mechanisms of immune activation in Kawasaki disease and emphasizes the importance of activated T cells in the pathogenesis of this vasculitis.

  10. Platelet immune complex interaction in pathogenesis of Kawasaki disease and childhood polyarteritis.

    OpenAIRE

    Levin, M; Holland, P C; Nokes, T J; Novelli, V; Mola, M; Levinsky, R J; Dillon, M J; Barratt, T M; Marshall, W C

    1985-01-01

    The role of platelets in the pathogenesis of vasculitis and the formation of coronary artery aneurysms was studied in 19 children with Kawasaki disease and five with polyarteritis. All patients with Kawasaki disease developed thrombocytosis in the third week of illness. The peak platelet count was significantly correlated (p less than 0.005) with the subsequent development of coronary artery aneurysms. The rise in platelet count was associated with the appearance in the circulation of a facto...

  11. Autoimmunity in X-linked agammaglobulinemia: Kawasaki disease and review of the literature.

    Science.gov (United States)

    Behniafard, Nasrin; Aghamohammadi, Asghar; Abolhassani, Hassan; Pourjabbar, Sarvenaz; Sabouni, Farah; Rezaei, Nima

    2012-02-01

    Although autoimmunity phenotype is surprisingly common in patients with different types of primary antibody deficiency, it is much less frequent in X-linked agammaglobulinemia (XLA). Herein, we report on a 15-month-old boy with XLA who also suffered from Kawasaki disease. The current case presentation is the first report of an association between Kawasaki disease and XLA. XLA could be considered as a special opportunity to understand autoimmunity in the near absence of immunoglobulins.

  12. Role of Antioxidants in Horse Serum-mediated Vasculitis in Swine: Potential Relevance to Early Treatment in Mitigation of Coronary Arteritis in Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    Saji Philip

    2017-08-01

    Conclusion: Serum sickness is a prototype of immune complex vasculitis, and the severity can be ameliorated with antioxidants. A trial of therapeutic dosages of vitamins A, E, and C in acute phase of Kawasaki disease, may be effective in mitigation of coronary artery lesion in addition to intravenous immunoglobulin and aspirin.

  13. Markers of a recent bocavirus infection in children with Kawasaki disease: "a year prospective study".

    Science.gov (United States)

    Bajolle, F; Meritet, J-F; Rozenberg, F; Chalumeau, M; Bonnet, D; Gendrel, D; Lebon, P

    2014-12-01

    Retrospective studies and case-reports have suggested the possible role of various viruses in the pathogenesis of the Kawasaki disease. To determine prospectively the incidence of Kawasaki diseases associated with a recent bocavirus infection in the course of a year. Thirty-two children with Kawasaki disease were enrolled in a 13 months prospective study to assess the frequency of human bocavirus type 1 infections. Seasonal shedding of virus, markers of recent infection such as viraemia, viral load, and serum interferon alpha were analyzed. Three of 32 (9%) children had HBoV-DNA in the serum suggesting a recent infection. HBoV-DNA was detected in naso-pharyngeal aspiration of 7/32 (21.8%) children with Kawasaki Disease and six of them (18%) had an increased viral load. No common respiratory viruses were isolated from the 32 patients with the exception of one adenovirus. The seven bocaviruses were identified during the winter-spring season. In addition, 4 of 7 of Kawasaki disease patients shedding bocavirus had detectable interferon alpha in the blood, indicating a possible active or recent viral infection. This study shows that a recent bocavirus infection is concomitant with the onset of some cases of Kawasaki disease. Bocavirus may be a cofactor in the pathogenesis of this disease as previously reported for other infectious agents. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  14. Kawasaki disease incidence in children and adolescents: an observational study in primary care.

    Science.gov (United States)

    Hall, Gillian C; Tulloh, Louise E; Tulloh, Robert M R

    2016-04-01

    Kawasaki disease is reported to be increasing in incidence and is the commonest childhood cause of acquired heart disease in the Western world. To determine the current UK incidence of Kawasaki disease across childhood and adolescence; and investigate trends over time and season. An observational, descriptive study in the UK. The Health Improvement Network (THIN) database of primary healthcare records was searched for codes or text indicating Kawasaki disease. Identified records were compared with a study case definition and a date of onset was assigned to cases. The incidence, age/sex distribution, and trend in seasonal and temporal distribution were estimated (2008-2012). A total of 110 episodes of Kawasaki disease in 109 children were identified from 3.9 million person-years observation. The incidence of Kawasaki disease was 2.8 per 100 000 person-years (95% confidence interval [CI] = 2.3 to 3.4) when aged Kawasaki disease remains low and has stabilised in the UK, GPs should recognise that the condition occurs throughout childhood and across the seasons. © British Journal of General Practice 2016.

  15. Sonographic Gallbladder Abnormality Is Associated with Intravenous Immunoglobulin Resistance in Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    Chih-Jen Chen

    2012-01-01

    Full Text Available Objective. Kawasaki disease (KD is an acute systematic vasculitis in children which causes coronary arterial lesions and hydrops of gallbladder. Our objective is to correlate the clinical significance and influence on disease outcome of patients with gallbladder abnormalities in Kawasaki dissease. Methods. Children who met KD diagnosis criteria and were admitted for IVIG treatment were retrospectively enrolled for analysis. Patients with abdominal sonography were divided into 2 groups based on the absence (Group A, N=61 or presence (Group B, N=16 of gallbladder abnormalities (GBA, defined as hydrops or acalculous cholecystitis. Between the two groups, clinical features, demographic data (including admission days, coronary artery lesions, IVIG resistance, and laboratory data before/after IVIG treatment were collected for analysis. Results. The presence of sonographic gallbladder abnormalities is correlated with higher levels of serum CRP, GPT, and neutrophils. It also points to an increased number of IVIG resistance rates in group B. There was no significant statistical difference among clinical features, age, gender, admission days, or coronary artery lesions between the two groups. Conclusion. Sonographic gallbladder abnormalities are associated with higher CRP, GPT, neutrophil and IVIG resistance in KD. It can be used as a predictor of IVIG resistance in patients with KD.

  16. Role of the Egami Score in Predicting Intravenous Immunoglobulin Resistance in Kawasaki Disease Among Different Ethnicities.

    Science.gov (United States)

    Loomba, Rohit S; Raskin, Alexander; Gudausky, Todd M; Kirkpatrick, Edward

    Early treatment with intravenous immunoglobulin (IVIG) is necessary to help reduce the risk of coronary artery abnormalities, such as coronary artery aneurysms and to help alleviate symptoms, in Kawasaki disease. Some patients, however, do not respond to an initial dose of IVIG and require additional doses. Prediction of these IVIG nonresponders may be of assistance in altering initial therapy to make it more effective. The Egami score has been validated in the Japanese population to predict IVIG nonresponders but has shown to be ineffective in US populations. This study evaluates the Egami score in a Midwest US population, subdividing patients by race and the diagnosis of typical or atypical type of Kawasaki disease. Patients were included in the study if they met criteria for Kawasaki disease and received IVIG in the inpatient setting. A total of 182 patients were studied, and in all studied groups, the Egami score had poor sensitivity at predicting IVIG nonresponders. Sensitivity of the score differed between races and differed between typical and atypical Kawasaki disease. The Egami score, as well as other systems, have been validated to predict IVIG nonresponders. These, however, lack sensitivity in the US population. Other scores developed in the United States have also lacked sensitivity, likely due to the absence of race or Kawasaki disease classification as variables. The development of a sensitive scoring system to predict IVIG nonresponders in US populations will require the incorporation of race and Kawasaki disease classification, factors that seem to alter IVIG response.

  17. Expression of CD11b as an adhesion molecule on neutrophils in children with Kawasaki disease.

    Directory of Open Access Journals (Sweden)

    Behzad Heidari

    2014-08-01

    Full Text Available Inflammation of blood vessels is a characteristic feature of Kawasaki disease. Neutrophils play a key role in the inflammatory responses where movement of neutrophils toward the site of inflammation depends on CD11b/CD18 expression as adhesion molecules on these cells. The purpose of this study was to investigate CD11b/CD18 expression in patients with Kawasaki disease upon diagnosis and after treatment.The study included 20 children with Kawasaki disease aged from 3 months to 8 years. Mean fluorescence intensity of CD11b levels on diagnosis and at 1-2 and 6 weeks after intravenous immunoglobulin (IVIG therapy was measured in these patients. Level of CD11b was measured in age-matched healthy children and febrile children (each 21 as negative and positive controls, respectively.Mean fluorescence intensity of CD11b in Kawasaki patients was lower than that of the control groups before and after 1-2 weeks of IVIG therapy. There were no significant differences in CD11b in Kawasaki patients either with aneurysm or without aneurysm. The CD11b levels at the diagnosis time and after treatment with IVIG in our patients with Kawasaki were lower than the control groups.

  18. Association between Kawasaki Disease and Autism: A Population-Based Study in Taiwan

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    Ho-Chang Kuo

    2014-04-01

    Full Text Available Objective: The association between Kawasaki disease and autism has rarely been studied in Asian populations. By using a nationwide Taiwanese population-based claims database, we tested the hypothesis that Kawasaki disease may increase the risk of autism in Taiwan. Materials and Methods: Our study cohort consisted of patients who had received the diagnosis of Kawasaki disease (ICD-9-CM: 446.1 between 1997 and 2005 (N = 563. For a comparison cohort, five age- and gender-matched control patients for every patient in the study cohort were selected using random sampling (N = 2,815. All subjects were tracked for 5 years from the date of cohort entry to identify whether they had developed autism (ICD-9-CM code 299.0 or not. Cox proportional hazard regressions were then performed to evaluate 5-year autism-free survival rates. Results: The main finding of this study was that patients with Kawasaki disease seem to not be at increased risk of developing autism. Of the total patients, four patients developed autism during the 5-year follow-up period, among whom two were Kawasaki disease patients and two were in the comparison cohort. Further, the adjusted hazard ratios (AHR (AHR: 4.81; 95% confidence interval: 0.68–34.35; P = 0.117 did not show any statistical significance between the Kawasaki disease group and the control group during the 5-year follow-up. Conclusion: Our study indicated that patients with Kawasaki disease are not at increased risk of autism.

  19. Downregulation of Th17 cells and the related cytokines with treatment in Kawasaki disease.

    Science.gov (United States)

    Rasouli, Manoochehr; Heidari, Behzad; Kalani, Mehdi

    2014-11-01

    Given the inflammatory nature of Kawasaki disease (KD) and the pro-inflammatory properties of Th17, this study aimed to determine the frequency of Th17 cells and the levels of corresponding cytokines in acute phase of KD and to evaluate their alterations one and eight weeks after treatment. Th17 and the related cytokine levels were measured in 21 KD patients and 42 positive and negative controls, using flow cytometry and ELISA, respectively. Th17, IL-17, IL-22 and IL-23 were significantly higher (P0.05) with the positive controls. Furthermore, Th17, IL-17, IL-22 and IL-23 were significantly higher in patients before treatment than those one and eight weeks after. Considering the downregulation of Th17 and its related cytokines with aspirin and intravenous immunoglobulin therapy implies the probable role of Th17 in KD pathogenesis.

  20. 川崎病的治疗进展%Advances in the treatment of Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    王晓华(综述); 赵建美(审校)

    2014-01-01

    川崎病(Kawasaki disease,KD)是一种好发于5岁以下儿童的急性全身血管炎性疾病。高达25%的未治疗患儿会并发KD最严重的并发症---冠状动脉损害(coronary artery lesions,CAL)。 KD发病率有逐年增加的趋势,已成为小儿后天获得性心脏病最主要的病因。目前KD已有标准的治疗方案,即大剂量静脉注射丙种球蛋白(intravenous im-munoglobulin,IVIG)联合阿司匹林,然而关于治疗时机和剂量尚有争议。 IVIG无反应性KD和CAL仍是治疗的难点。本综述就近年来有关KD治疗尤其对IVIG无反应性KD和CAL的治疗方法进行总结。%Kawasaki disease(KD) is acute systemic vasculitis that occurs mainly in children under 5 years of age. Up to 25%of untreated KD patients develop coronary artery lesions(CAL), which is the most serious complication. The annual inci-dence of KD is increasing and KD has become the most common cause of acquired heart disease in children. Standard treat-ment of the acute KD phase is established and consists of high-dose intravenous immunoglobulin(IVIG) and Aspirin, however, timing of treatment and dosage of drugs are controversial. The optimal treatment for IVIG-nonresponsive KD and CAL still re-main difficult. This review covers the current evidence for treatment of KD, especially for IVIG-nonresponsive KD and CAL.

  1. Use of Corticosteroid in Children with Unresponsiveness to Intravenous Immunoglobulin in Kawasaki Disease

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    Abdolkarim Hamedi

    2017-08-01

    Full Text Available Background Kawasaki Disease (KD is a vasculitis with multi-organ involvementof unknown etiology; it is the most common cause of pediatric-heart diseases in developed countries. Treatment with Intravenous Immunoglobulin (IVIG prevents coronary artery lesions; although there are some IVIG-resistant cases, combination therapy with corticosteroids and IVIG is one of the recommendations for treatment of these cases. The aim of this study was to compare these three options for treatment of Kawasaki Disease and to evaluate their ability to deal with coronary artery complication of Kawasaki Disease. Materials and Methods A prospective cross- sectional study of hospitalized cases of Kawasaki Disease, conducted in pediatric department of Imam Reza hospital, Mashhad-Iran, during 2013 to 2015 (18 months. Based on demographic and clinical data of these patients, children with high risk of unresponsiveness to IVIG therapy (based on Harada score, were determined and treated with IVIG and corticosteroids- combination initially. Follow-up patients for heart complications were 6 weeks. Results Twenty five patients (89.2% out of total 28 hospitalized patients in this period of time who fulfilled diagnostic criteria were considered as complete Kawasaki Disease. Coronary Artery Lesions (CALs were shown in 4 patients during the follow-up period, with high risk in patients with incomplete presentation (33.3% versus 12%, P

  2. Clinical and laboratory characteristics of children with Kawasaki disease

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    Fatih Akın

    2015-03-01

    Full Text Available Objective: In this study, we aimed to evaluate clinical and laboratory characteristics of patients with Kawasaki disease (KD in Konya region of Turkey. Methods: The hospital records of patients who were hospitalized with the diagnosis of KD in the Pediatrics Clinics of Konya Training and Research Hospital between May 2010 and June 2012 were reviewed retrospectively. Results: Seven cases were found to have the diagnosis of KD, two of whom were incomplete KD. Oropharynx changes were the most common (100% feature in our patients. Five (71% patients had bulbar conjunctivitis. Three (43% patients had erythema at the site of BCG inoculation. Adenopathy was present in all of our patients with the classical form. A desquamation was observed in one case at the seventh day of fever. No cardiac manifestation was seen. Elevated erythrocyte sedimentation rate and thrombocytosis were present in all patients. All of the patients were received intravenous immunoglobulin in the first ten days of the fever. Conclusion: KD should be considered as a possible diagnosis in any child presenting with prolonged fever. BCG reaction can be attributed as a diagnostic criterion for incomplete form of the disease especially in countries where BCG vaccination is routinely performed. Early treatment is essential to prevent cardiovascular complications.

  3. Identification of candidate diagnostic serum biomarkers for Kawasaki disease using proteomic analysis

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    Kimura, Yayoi; Yanagimachi, Masakatsu; Ino, Yoko; Aketagawa, Mao; Matsuo, Michie; Okayama, Akiko; Shimizu, Hiroyuki; Oba, Kunihiro; Morioka, Ichiro; Imagawa, Tomoyuki; Kaneko, Tetsuji; Yokota, Shumpei; Hirano, Hisashi; Mori, Masaaki

    2017-01-01

    Kawasaki disease (KD) is a systemic vasculitis and childhood febrile disease that can lead to cardiovascular complications. The diagnosis of KD depends on its clinical features, and thus it is sometimes difficult to make a definitive diagnosis. In order to identify diagnostic serum biomarkers for KD, we explored serum KD-related proteins, which differentially expressed during the acute and recovery phases of two patients by mass spectrometry (MS). We identified a total of 1,879 proteins by MS-based proteomic analysis. The levels of three of these proteins, namely lipopolysaccharide-binding protein (LBP), leucine-rich alpha-2-glycoprotein (LRG1), and angiotensinogen (AGT), were higher in acute phase patients. In contrast, the level of retinol-binding protein 4 (RBP4) was decreased. To confirm the usefulness of these proteins as biomarkers, we analyzed a total of 270 samples, including those collected from 55 patients with acute phase KD, by using western blot analysis and microarray enzyme-linked immunosorbent assays (ELISAs). Over the course of this experiment, we determined that the expression level of these proteins changes specifically in the acute phase of KD, rather than the recovery phase of KD or other febrile illness. Thus, LRG1 could be used as biomarkers to facilitate KD diagnosis based on clinical features. PMID:28262744

  4. [Three Cases of Moyamoya Disease with a History of Kawasaki Disease].

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    Kawasaki, Toshinari; Arakawa, Yoshiki; Sugino, Toshiya; Mitsuhara, Takafumi; Funaki, Takeshi; Kikuchi, Takayuki; Koyanagi, Masaomi; Yoshida, Kazumichi; Kunieda, Takeharu; Takahashi, Jun C; Takagi, Yasushi; Miyamoto, Susumu

    2015-11-01

    Here, we report three cases of moyamoya disease with a history of Kawasaki disease. A 33-year-old man was found to have stenotic lesions of the internal carotid arteries(ICAs)on both sides at a nearby hospital where he visited complaining of headache and lisping. He had received immunoglobulin therapy for Kawasaki disease at the ages of 1, 2, and 6 years. MRI showed only a chronic ischemic lesion in the white matter. Angiography showed occlusion at the terminal portion of the ICAs on both sides. He was diagnosed with moyamoya disease, but as he had no symptoms and preserved cerebral blood flow (CBF), he was kept under observation. An 8-year-old boy was diagnosed with moyamoya disease and underwent right encephaloduroarteriosynangiosis at a nearby hospital. He had received immunoglobulin therapy for Kawasaki disease at the age of 1 year. His ischemic symptoms worsened. Although MRI detected no apparent ischemic lesion, angiography revealed severe stenosis at the terminal portions of the ICAs on both sides, and 123I-IMP SPECT showed CBF impairment. Bilateral direct bypass was performed. His father was subsequently also diagnosed with moyamoya disease. A 4-year-old girl with epilepsy was diagnosed with moyamoya disease at a nearby hospital. She had been treated with aspirin for Kawasaki disease at the age of 1 year. MRI detected no remarkable ischemic lesions, but angiography revealed mild stenosis at the terminal portions of the ICAs on both sides. Five months later, her ischemic symptoms were worsening with progressing stenotic lesions, and she underwent bilateral direct bypass.

  5. National survey of pediatric hospitalizations due to Kawasaki disease and coronary artery aneurysms in the USA.

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    Okubo, Yusuke; Nochioka, Kotaro; Sakakibara, Hiroshi; Testa, Marcia; Sundel, Robert P

    2017-02-01

    Several studies revealed the epidemiology of Kawasaki disease-related hospitalizations among children in the USA and other countries. However, disparities of developing coronary artery aneurysms by race/ethnicity, patient socioeconomic status, and geographic locations remain unknown in the USA. Hospital discharge record data of patients with Kawasaki disease aged 19 years or younger were obtained from the 2003, 2006, 2009, and 2012 Kid's Inpatient Database. The data were weighted to estimate the annual hospitalization rates with respect to age, gender, and race/ethnicity in the USA. Multivariable logistic regression was conducted to ascertain the factors associated with the development of coronary artery aneurysms. Total annual hospitalization rates of Kawasaki disease showed a decreasing trend, ranging from 6.54 per 100,000 children in 2003 to 6.11 per 100,000 children in 2012 (p aneurysms among patients with Kawasaki disease ranged from 2.25 to 3.20%. Factor associated with the development of coronary artery aneurysms was hospitals in West (OR 2.15, 95% CI 1.42-3.26). Race/ethnicity, health insurance status, and household income were not associated with the development of coronary artery aneurysms. Total hospitalization rates of Kawasaki disease showed a decreasing trend. Children admitted to hospitals in West region were more likely to develop coronary artery aneurysms.

  6. Hemodynamic simulations in coronary aneurysms of children with Kawasaki disease

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    Sengupta, Dibyendu; Burns, Jane; Marsden, Alison

    2009-11-01

    Kawasaki disease (KD) is a serious pediatric illness affecting the cardiovascular system. One of the most serious complications of KD, occurring in about 25% of untreated cases, is the formation of large aneurysms in the coronary arteries, which put patients at risk for myocardial infarction. In this project we performed patient specific computational simulations of blood flow in aneurysmal left and right coronary arteries of a KD patient to gain an understanding about their hemodynamics. Models were constructed from CT data using custom software. Typical pulsatile flow waveforms were applied at the model inlets, while resistance and RCR lumped models were applied and compared at the outlets. Simulated pressure waveforms compared well with typical physiologic data. High wall shear stress values are found in the narrow region at the base of the aneurysm and low shear values occur in regions of recirculation. A Lagrangian approach has been adopted to perform particle tracking and compute particle residence time in the recirculation. Our long-term goal will be to develop links between hemodynamics and the risk for thrombus formation in order to assist in clinical decision-making.

  7. Variations in ORAI1 Gene Associated with Kawasaki Disease.

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    Onouchi, Yoshihiro; Fukazawa, Ryuji; Yamamura, Kenichiro; Suzuki, Hiroyuki; Kakimoto, Nobuyuki; Suenaga, Tomohiro; Takeuchi, Takashi; Hamada, Hiromichi; Honda, Takafumi; Yasukawa, Kumi; Terai, Masaru; Ebata, Ryota; Higashi, Kouji; Saji, Tsutomu; Kemmotsu, Yasushi; Takatsuki, Shinichi; Ouchi, Kazunobu; Kishi, Fumio; Yoshikawa, Tetsushi; Nagai, Toshiro; Hamamoto, Kunihiro; Sato, Yoshitake; Honda, Akihito; Kobayashi, Hironobu; Sato, Junichi; Shibuta, Shoichi; Miyawaki, Masakazu; Oishi, Ko; Yamaga, Hironobu; Aoyagi, Noriyuki; Yoshiyama, Megumi; Miyashita, Ritsuko; Murata, Yuji; Fujino, Akihiro; Ozaki, Kouichi; Kawasaki, Tomisaku; Abe, Jun; Seki, Mitsuru; Kobayashi, Tohru; Arakawa, Hirokazu; Ogawa, Shunichi; Hara, Toshiro; Hata, Akira; Tanaka, Toshihiro

    2016-01-01

    Kawasaki disease (KD; MIM#61175) is a systemic vasculitis syndrome with unknown etiology which predominantly affects infants and children. Recent findings of susceptibility genes for KD suggest possible involvement of the Ca(2+)/NFAT pathway in the pathogenesis of KD. ORAI1 is a Ca(2+) release activated Ca(2+) (CRAC) channel mediating store-operated Ca(2+) entry (SOCE) on the plasma membrane. The gene for ORAI1 is located in chromosome 12q24 where a positive linkage signal was observed in our previous affected sib-pair study of KD. A common non-synonymous single nucleotide polymorphism located within exon 2 of ORAI1 (rs3741596) was significantly associated with KD (P = 0.028 in the discovery sample set (729 KD cases and 1,315 controls), P = 0.0056 in the replication sample set (1,813 KD cases vs. 1,097 controls) and P = 0.00041 in a meta-analysis by the Mantel-Haenszel method). Interestingly, frequency of the risk allele of rs3741596 is more than 20 times higher in Japanese compared to Europeans. We also found a rare 6 base-pair in-frame insertion variant associated with KD (rs141919534; 2,544 KD cases vs. 2,414 controls, P = 0.012). These data indicate that ORAI1 gene variations are associated with KD and may suggest the potential importance of the Ca(2+)/NFAT pathway in the pathogenesis of this disorder.

  8. Genetic variants of CD209 associated with Kawasaki disease susceptibility.

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    Ho-Chang Kuo

    Full Text Available BACKGROUND: Kawasaki disease (KD is a systemic vasculitis with unknown etiology mainly affecting children in Asian countries. Dendritic cell-specific intercellular adhesion molecule-3 grabbing non-integrin (DC-SIGN, CD209 in humans was showed to trigger an anti-inflammatory cascade and associated with KD susceptibility. This study was conducted to investigate the association between genetic polymorphisms of CD209 and the risk KD. METHODS: A total of 948 subjects (381 KD and 567 controls were recruited. Nine tagging SNPs (rs8112310, rs4804800, rs11465421, rs1544766, rs4804801, rs2287886, rs735239, rs735240, rs4804804 were selected for TaqMan allelic discrimination assay. Clinical phenotypes, coronary artery lesions (CAL and intravenous immunoglobulin (IVIG treatment outcomes were collected for analysis. RESULTS: Significant associations were found between CD209 polymorphisms (rs4804800, rs2287886, rs735240 and the risk of KD. Haplotype analysis for CD209 polymorphisms showed that A/A/G haplotype (P = 0.0002, OR = 1.61 and G/A/G haplotype (P = 0.0365, OR = 1.52 had higher risk of KD as compared with G/G/A haplotype in rs2287886/rs735239/rs735240 pairwise allele analysis. There were no significant association in KD with regards to CAL formation and IVIG treatment responses. CONCLUSION: CD209 polymorphisms were responsible for the susceptibility of KD, but not CAL formation and IVIG treatment responsiveness.

  9. Variations in ORAI1 Gene Associated with Kawasaki Disease.

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    Yoshihiro Onouchi

    Full Text Available Kawasaki disease (KD; MIM#61175 is a systemic vasculitis syndrome with unknown etiology which predominantly affects infants and children. Recent findings of susceptibility genes for KD suggest possible involvement of the Ca(2+/NFAT pathway in the pathogenesis of KD. ORAI1 is a Ca(2+ release activated Ca(2+ (CRAC channel mediating store-operated Ca(2+ entry (SOCE on the plasma membrane. The gene for ORAI1 is located in chromosome 12q24 where a positive linkage signal was observed in our previous affected sib-pair study of KD. A common non-synonymous single nucleotide polymorphism located within exon 2 of ORAI1 (rs3741596 was significantly associated with KD (P = 0.028 in the discovery sample set (729 KD cases and 1,315 controls, P = 0.0056 in the replication sample set (1,813 KD cases vs. 1,097 controls and P = 0.00041 in a meta-analysis by the Mantel-Haenszel method. Interestingly, frequency of the risk allele of rs3741596 is more than 20 times higher in Japanese compared to Europeans. We also found a rare 6 base-pair in-frame insertion variant associated with KD (rs141919534; 2,544 KD cases vs. 2,414 controls, P = 0.012. These data indicate that ORAI1 gene variations are associated with KD and may suggest the potential importance of the Ca(2+/NFAT pathway in the pathogenesis of this disorder.

  10. Variations in ORAI1 Gene Associated with Kawasaki Disease

    Science.gov (United States)

    Suzuki, Hiroyuki; Kakimoto, Nobuyuki; Suenaga, Tomohiro; Takeuchi, Takashi; Hamada, Hiromichi; Honda, Takafumi; Yasukawa, Kumi; Terai, Masaru; Ebata, Ryota; Higashi, Kouji; Saji, Tsutomu; Kemmotsu, Yasushi; Takatsuki, Shinichi; Ouchi, Kazunobu; Kishi, Fumio; Yoshikawa, Tetsushi; Nagai, Toshiro; Hamamoto, Kunihiro; Sato, Yoshitake; Honda, Akihito; Kobayashi, Hironobu; Sato, Junichi; Shibuta, Shoichi; Miyawaki, Masakazu; Oishi, Ko; Yamaga, Hironobu; Aoyagi, Noriyuki; Yoshiyama, Megumi; Miyashita, Ritsuko; Murata, Yuji; Fujino, Akihiro; Ozaki, Kouichi; Kawasaki, Tomisaku; Abe, Jun; Seki, Mitsuru; Kobayashi, Tohru; Arakawa, Hirokazu; Ogawa, Shunichi; Hara, Toshiro; Hata, Akira; Tanaka, Toshihiro

    2016-01-01

    Kawasaki disease (KD; MIM#61175) is a systemic vasculitis syndrome with unknown etiology which predominantly affects infants and children. Recent findings of susceptibility genes for KD suggest possible involvement of the Ca2+/NFAT pathway in the pathogenesis of KD. ORAI1 is a Ca2+ release activated Ca2+ (CRAC) channel mediating store-operated Ca2+ entry (SOCE) on the plasma membrane. The gene for ORAI1 is located in chromosome 12q24 where a positive linkage signal was observed in our previous affected sib-pair study of KD. A common non-synonymous single nucleotide polymorphism located within exon 2 of ORAI1 (rs3741596) was significantly associated with KD (P = 0.028 in the discovery sample set (729 KD cases and 1,315 controls), P = 0.0056 in the replication sample set (1,813 KD cases vs. 1,097 controls) and P = 0.00041 in a meta-analysis by the Mantel-Haenszel method). Interestingly, frequency of the risk allele of rs3741596 is more than 20 times higher in Japanese compared to Europeans. We also found a rare 6 base-pair in-frame insertion variant associated with KD (rs141919534; 2,544 KD cases vs. 2,414 controls, P = 0.012). These data indicate that ORAI1 gene variations are associated with KD and may suggest the potential importance of the Ca2+/NFAT pathway in the pathogenesis of this disorder. PMID:26789410

  11. The expression of p53 gene in peripheral blood lymphocyte of acute Kawasaki disease%p53基因与川崎病患者淋巴细胞凋亡关系探讨

    Institute of Scientific and Technical Information of China (English)

    易岂建; 杨锡强; 李成荣; 张远维; 王莉佳

    2001-01-01

    目的:进一步探讨川崎病(KD)急性期患者外周血淋巴细胞凋亡延迟的机理。方法:采用斑点杂交(Dot-blot)检测淋巴细胞p53基因mRNA表达水平;流式细胞仪(FCM)检测p53蛋白质表达阳性细胞百分率。结果:KD患者外周血淋巴细胞p53基因mRNA和p53蛋白质表达水平降低,与正常儿童比较差异显著(P<0.005);当给予静脉注射免疫球蛋白(IVIG)治疗后或加入抗IL-6单抗培养时,外周血淋巴细胞p53基因mRNA和P53蛋白质表达水平提高。结论:KD急性期患者外周血淋巴细胞p53基因表达水平降低,其原因可能与本病患者异常升高的IL-6有关。p53基因具有促进细胞凋亡的作用,KD患者外周血淋巴细胞凋亡延迟可能与高浓度IL-6抑制p53基因的表达有关。%Objective: To further explore the mechanism of inhibited apoptosis of peripheral blood mononuclear cell (PBMC) in acute Kawasaki disease(KD). Methods: The expression level of p53 gene mRNA was determined by dot-blot; p53 protein positive cell percentage was detected by flow cytometry (FCM). Results:The expression of p53 gene mRNA and p53 protein in acute KD patients were decreased(P<0.001), but increased after treating with intravenous immunoglobulin(IVIG) in vivo or adding anti-IL-6 monoantibody(mAb)into PBMC culture in vitro. Conclusion. The decreased expression of p53 gene mRNA and p53 protein may be associated with the high concentration of IL-6 in KD patients, p53 gene expression could induce lymphocyte apoptosis. Thus, the expression of p53 gene inhibited by the increased IL-6 production might be related to delaying or depressing apoptosis of PBMC in KD.

  12. A systematic review of validated methods for identifying Kawasaki disease using administrative or claims data.

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    Williams, Candice L; Sathe, Nila A; Krishnaswami, Shanthi; McPheeters, Melissa L

    2013-12-30

    To identify and assess algorithms used to identify Kawasaki syndrome/Kawasaki disease in administrative and claims databases. We searched the MEDLINE database from 1991 to September 2012 using controlled vocabulary and key terms related to Kawasaki disease. We also searched the reference lists of included studies. Two investigators independently assessed the full text of studies against pre-determined inclusion criteria. Two reviewers independently extracted data regarding participant and algorithm characteristics. Our searches identified 177 citations of which 22 met our inclusion criteria. All studies used algorithms including International Classification of Diseases, Ninth Revision (ICD-9) code 446.1 either alone, or with evidence of intravenous immunoglobulin (IVIG) administration, or with ICD-10 code M30.3. Six studies confirmed diagnoses by medical chart review. Three of these six studies reported validation statistics, with positive predictive values of 74%, 84%, and 86%, respectively. All studies that reported algorithms used either the ICD-9 code 446.1 either alone, with evidence of IVIG administration or with ICD-10 code M30.3. The ICD-9 code 446.1 alone produced positive predictive values of 74%, 84%, and 86% in separate studies in Georgia and California. The sensitivity of these codes to detect Kawasaki disease is unknown, as no sampling of medical records for missed true cases of Kawasaki disease was done. Further research would be helpful to determine whether the relatively high positive predictive values found in southern California are seen elsewhere and also to evaluate the performance of other codes to identify cases of Kawasaki disease and the sensitivity of the narrow algorithms that have been used to date. Copyright © 2013 Elsevier Ltd. All rights reserved.

  13. Evaluation of coronary arterial lesions due to Kawasaki disease using optical coherence tomography.

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    Kakimoto, Nobuyuki; Suzuki, Hiroyuki; Kubo, Takashi; Suenaga, Tomohiro; Takeuchi, Takashi; Shibuta, Shoichi; Ino, Yasushi; Akasaka, Takashi; Yoshikawa, Norishige

    2014-08-01

    Optical coherence tomography (OCT) is a high-resolution intracoronary arterial imaging modality. We describe 2 patients who were admitted to undergo coronary angiography and OCT for follow-up of Kawasaki disease with coronary artery aneurysms. OCT clearly demonstrated thrombus, stenosis, fibrotic intimal thickening with lamellar calcification, and partial disappearance of the tunica media at the aneurysm site. In addition, focal calcification, intimal thickening, and medial irregularity were observed even in regions of coronary arterial walls that appeared to be normal using coronary angiography. OCT is useful for evaluating coronary arterial sequelae of Kawasaki disease. Copyright © 2014 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.

  14. Five years of Kawasaki disease in the Netherlands a national surveillance study

    NARCIS (Netherlands)

    Tacke, C.E.; Breunis, W.B.; Pereira, R.R.; Breur, J.M.; Kuipers, I.M.; Kuijpers, T.W.

    2014-01-01

    Background: The aim of this study was to evaluate the incidence, disease presentation, treatment and cardiac outcome of Kawasaki disease (KD) in The Netherlands. Methods: The national Dutch Pediatric Surveillance Unit was used to prospectively register new KD cases from 2008 through 2012.

  15. Kawasaki disease mimicking a parapharyngeal abscess: a case report.

    Science.gov (United States)

    Cai, Qianyun; Luo, Rong; Gan, Jing; Zhang, Li; Qu, Yi; Mu, Dezhi

    2015-05-01

    Parapharyngeal abscess (PPA)-like lesion is a very rare manifestation of Kawasaki disease (KD). Here we report a Chinese case of KD initially mimicking PPA, which is the first one reported in Asia.A 3-year-old male patient presented with fever, drooling, and bilateral painful cervical lymphadenopathy for 3 days. Chest X-ray and echocardiogram were normal. With substantial elevation of white blood count and C-reactive protein, purulent cervical lymphadenitis was considered. Symptoms did not improve after treatment with vancomycin, and the patient further developed trismus and restricted neck movement. Neck CT revealed a 2 × 1.5 cm hypodense lesion in the right parapharyngeal space with peripheral enhancement. PPA was suspected and on the 3rd day following admission, the patient received surgical incision and drainage. One milliliter of serous fluid was drained without bacterial growth on cultures. Fever persisted after surgery. As the clinical course proceeded, additional major signs of KD gradually evolved, and on the 6th day following admission the patient completely fulfilled the diagnostic criteria for KD. Rapid clinical improvement was observed following treatment with high-dose immunoglobulin and aspirin. Due to the parapharyngeal operation, the patient was fed milk through a nasogastric tube for 15 days. His neck incision became infected but healed gradually following dressing change and antibiotic treatment. Currently he remains asymptomatic during regular follow-up and repeated echocardiograms are normal.Both pediatricians and otolaryngologists can learn from this case that KD may initially manifest as PPA. Careful observation for major signs of KD during the clinical course can help to achieve a prompt and correct diagnosis. Thus, unnecessary surgery and cardiac complications of KD may be avoided.

  16. Predictors of nonresponse to intravenous immunoglobulin therapy in Kawasaki disease

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    Hyo Min Park

    2013-02-01

    Full Text Available &lt;b&gt;Purpose:&lt;/b&gt; It has been reported that 10% to 20% of children with Kawasaki disease (KD will not respond to intravenous immunoglobulin (IVIG treatment. In this study, we aimed to identify useful predictors of therapeutic failure in children with KD. &lt;b&gt;Methods:&lt;/b&gt; We examined 309 children diagnosed with KD at the Kyungpook National University Hospital and the Inje University Busan Paik Hospital between January 2005 and June 2011. We retrospectively reviewed their medical records and analyzed multiple parameters in responders and nonresponders to IVIG. &lt;b&gt;Results:&lt;/b&gt; Among the 309 children, 30 (9.7% did not respond to IVIG. They had significantly higher proportion of neutrophils, and higher levels of aspartate aminotransferase, alanine aminotransferase (ALT, total bilirubin, and N-terminal fragment of B-type natriuretic peptide than did responders. IVIGnonresponders had a significantly longer duration of hospitalization, and more frequently experienced coronary artery lesion, and sterile pyuria. No differences in the duration of fever at initial treatment or, clinical features were noted. &lt;b&gt;Conclusion:&lt;/b&gt; Two independent predictors (ALT?#248;4 IU/L, total bilirubin?#240;.9 mg/dL for nonresponse were confirmed through multivariate logistic regression analysis. Thus elevated ALT and total bilirubin levels might be useful in predicting nonresponse to IVIG therapy in children with KD.

  17. Diagnostic value of dual-source CT in Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    CHAO Bao-ting; WANG Xi-ming; WU Le-bin; CHEN Jie; CHENG Zhao-ping; WU Da-wei; DUAN Yan-hua

    2010-01-01

    Background Doppler color echocardiography is a common method for detecting coronary artery lesions in patients with Kawasaki disease(KD).However,the diagnostic accuracy for the whole coronary artery lesions is limited.The purpose of this study was to compare the diagnostic value of dual-source computed tomography (DSCT) and Doppler color echocardiography for the assessment of coronary artery lesions caused by KD.Methods Sixteen patients,12 with typical KD and 4 with atypical KD,underwent DSCT and Doppler color echocardiography.The position and internal diameter of each coronary artery lesion was measured.Correlation analysis was used to compare the diagnostic value of the two imaging modalities.Results ln the typical KD group, seven patients did not have any coronary artery Iesion as confirmed by both DSCT scans and Doppler color echocardiography;in four patients proximal coronary artery injuries were identified by both modalities;in one patient an aneurysm in the middle and distal segments of the coronary artery was detected by DSCT but was negative in Doppler color echocardiography.In the atypical KD group,three cases showed the same results with both modalities,while one case with coronary artery stenosis in the middle segment was identified by DSCT but not detected by Doppler color echocardiography.There was a good correlation between the two imaging modalities(Kappa value,0.768(≥0.75)).Conclusion DSCT coronary artery angiography is an accurate,non-invasive,and valuable technique for detecting and following up coronary artery lesions in patients with KD.

  18. Sudden death as a late sequel of Kawasaki disease: postmortem CT demonstration of coronary artery aneurysm.

    Science.gov (United States)

    Okura, Naoki; Okuda, Takahisa; Shiotani, Seiji; Kohno, Mototsugu; Hayakawa, Hideyuki; Suzuki, Atsuko; Kawasaki, Tomisaku

    2013-02-10

    Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that primarily affects the coronary artery (CA) and presents during childhood. The characteristic coronary arterial lesion of KD is an aneurysm. Ischemic heart disease derived from a CA aneurysm is experienced approximately two decades after the onset of acute KD. In recent years, the primary issue of concern has been asymptomatic adults with a CA aneurysm caused by undiagnosed KD. We present a case of sudden death as a late KD sequel in a young adult. A postmortem CT scan revealed a coarse calcification of a left anterior descending CA aneurysm, which was confirmed at the time of autopsy. A postmortem CT scan is useful in cases of sudden death where the detection of a calcified CA aneurysm would suggest to the forensic pathologist that the deceased suffered from a late sequel of KD. The use of screening postmortem CT scans for young people may detect cases of unsuspected CA aneurysms, raising the possibility of untreated KD. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

  19. INTESTINAL PSEUDO-OBSTRUCTION AND TRANSIENT CARDIOVASCULAR ABNORMALITIES IN KAWASAKI DISEASE

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    Maria L Avila-Aguero

    2005-08-01

    Full Text Available SUMMARYWe describe a 2 year-old boy with severe vasculitis who presented with a typical Kawasaki disease complicated with an intestinal pseudo-obstruction, gallbladder hydrops, myocarditis and transient coronary abnormalities despite early administration of intravenous immunoglobulin treatment.RESUMENDescribimos el caso de un niño de 2 años con vasculitis grave que presentó un cuadro típico de enfermedad de Kawasaki complicada con una pseudo-obtrucción intestinal, hidrops vesicular, miocarditis y anormalidades coronarias transitorias, a pesar de la administración temprana de tratamiento con inmunoglulina intravenosa

  20. Usefulness of real-time 3-dimensional echocardiography to identify and quantify left ventricular dyssynchrony in patients with Kawasaki disease.

    Science.gov (United States)

    Yu, Yi; Sun, Kun; Xue, Haihong; Chen, Sun; Yang, Jianping

    2013-06-01

    The role of left ventricular (LV) dyssynchrony in Kawasaki disease is unknown. This study sought to establish values for real-time 3-dimensional (3D) echocardiographically derived LV dyssynchrony parameters and identify and quantify LV dyssynchrony in patients with Kawasaki disease. Forty patients hospitalized for Kawasaki disease were analyzed retrospectively, and 40 sex- and age-matched healthy control volunteers were also enrolled. The systolic dyssynchrony index (percentage of the cardiac cycle) from 16 and 12 LV segments on real-time 3D echocardiography was analyzed to calculate LV dyssynchrony (defined as the standard deviation of the time to reach the minimum systolic volume for 16 LV segments) according to a 17-segment model. We analyzed the 3D LV ejection fraction (LVEF), end-diastolic volume, and end-systolic volume in the patients with Kawasaki disease compared to the controls. The 16-segment systolic dyssynchrony index ± SD was significantly higher in the patients with Kawasaki disease: 2.73% ± 0.96% compared to 2.01% ± 0.85% in the controls (P Kawasaki disease was 2.65% ± 0.93% compared to 1.98% ± 0.81% in the controls (PKawasaki disease and an LVEF of less than 50% had a significantly higher systolic dyssynchrony index compared to patients with an LVEF of 50% or greater (2.89% ± 0.79% versus 2.26% ± 0.73%; P Kawasaki disease, and global systolic function was impaired. The LVEF measured by a biplane method was sufficiently related to the LVEF measured by echocardiography. Real-time 3D echocardiography is a noninvasive and feasible method for identifying and evaluating LV dyssynchrony in children with Kawasaki disease. Left ventricular dyssynchrony is significantly impaired and related to LV systolic function in patients with Kawasaki disease.

  1. 英夫利昔单抗治疗川崎病的应用进展%Progress in the application of infliximab in Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    刘亚萍

    2015-01-01

    川崎病是一种以全身性血管炎为主要病变的急性、自限性疾病.冠状动脉病变是川崎病最常见、最严重的并发症.目前静脉注射丙种球蛋白(intravenous immunoglobulin gamma,IVIG)联合大剂量阿司匹林口服的标准治疗方案显著降低了川崎病冠状动脉病变的发病率,然而近年来报道的IVIG无反应型以及难治性川崎病病例不断增多,增加了临床治疗的难度.现有研究进展表明英夫利昔单抗为治疗川崎病,尤其是IVIG无反应型以及难治性川崎病提供了新方法.该文对英夫利昔单抗治疗川崎病的应用进展进行综述.%Kawasaki disease is an acute and self-limited systemic vasculitis.Coronary artery lesions are the most common complications.The principal treatment with intravenous immunoglobulin and high doses of oral aspirin has significantly decreased the morbidity of coronary artery lesions.However, increased cases of IVIG non-responsiveness and refractory Kawasaki disease in recent years make the treatment more difficult.The existing progress indicates that infliximab provides a new therapeutic option for Kawasaki disease, especially for IVIG non-responsiveness and refractory Kawasaki disease.This paper reviews recent studies associated with application of infliximab in Kawasaki disease.

  2. 成人川崎病1例报告%One case of adult Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    周雅娟; 孙璐; 于宪一; 严志涵; 黄芬

    2012-01-01

    川崎病多发于儿童,目前病因未明,易发生冠状动脉改变,临床表现多样化,目前无特异性实验室诊断指标,易被误诊.成人川崎病更少见,极易漏诊及误诊.本文对1例成人川崎病的诊断和治疗进行回顾性分析,以提高临床医生对川崎病的认识,早期诊治,改善预后.%Kawasaki disease is far more frequent in children than in adults. The pathogenesis of Kawasaki disease is unknown, but it involves changes to the coronary artery and other diverse clinical manifestations. There are currently no specific laboratory diagnostic indexes, and especially since the disease is rare in adults, so it is extremely easy to misdiagnose or to overlook entirely. Our retrospective analysis of an diagnosis of and treatment for Kawasaki disease in an adult provides a guide to clinical doctors in terms of understanding Kawasaki disease, early diagnosis of it, and improved prognosis.

  3. Usefulness of Neutrophil to Lymphocyte Ratio in Prediction of Coronary Artery Lesions in Patients with Kawasaki Disease.

    Science.gov (United States)

    Demir, Fikri; Karadeniz, Cem; Özdemir, Rahmi; Yozgat, Yılmaz; Çelegen, Kübra; Karaaslan, Utku; Demirol, Mustafa; Meşe, Timur; Ünal, Nurettin

    2015-10-01

    Kawasaki disease is an inflammatory condition. Neutrophil to lymphocyte ratio is a marker reflecting inflammation. The aim of the study is to evaluate usefulness of neutrophil to lymphocyte ratio in diagnosis of Kawasaki disease and in prediction of coronary artery lesions. Retrospective cross-sectional study. Seventy-five children with Kawasaki disease and 66 controls were retrospectively enrolled. Their leukocyte, neutrophil, and lymphocyte counts were recorded. Abnormally distributed data were shown as median (interquartile range). Cases having coronary artery diameter two standard deviation above mean were diagnosed to have coronary artery lesions. Median age of Kawasaki disease patients was 34 months. Twentyfive of those (33.33%) had incomplete Kawasaki disease and twenty-six (34.66%) had coronary artery lesions. Leukocyte [12.61 (6.09)×10(3)/µL vs. 8.48 (5.58)×10(3)/µL], neutrophil [6.73 (4.10) ×10(3)/µL vs. 4.62 (5.47)×10(3)/µL], and lymphocyte [4.04 (2.91)×10(3)/µL vs. 3.02 (2.57) ×10(3)/µL] counts were significantly higher in Kawasaki disease patients compared to controls (all p values Kawasaki disease and incomplete Kawasaki disease cases did not differ, while comparison of patients with and without coronary artery lesions revealed significantly higher neutrophil to lymphocyte ratio values in former group [2.02 (1.63) vs. 1.50 (1.28), p=0.01]. The cut-off neutrophil to lymphocyte ratio value for prediction of coronary artery lesions was determined as 1.32. Neutrophil to lymphocyte ratio values in Kawasaki patients with coronary lesions were significantly higher than the ones without and values greater than 1.32 were useful in prediction of coronary lesions.

  4. Genome-wide linkage and association mapping identify susceptibility alleles in ABCC4 for Kawasaki disease

    NARCIS (Netherlands)

    Khor, Chiea Chuen; Davila, Sonia; Shimizu, Chisato; Sheng, Stephanie; Matsubara, Tomoyo; Suzuki, Yasuo; Newburger, Jane W.; Baker, Annette; Burgner, David; Breunis, Willemijn; Kuijpers, Taco; Wright, Victoria J.; Levin, Michael; Hibberd, Martin L.; Burns, Jane C.

    Background Kawasaki disease (KD) is a self limited vasculitis in which host genetics plays a prominent role. To further the understanding of the role of host genetics in KD, a three-stage genetic study was conducted that began with a family linkage study and ultimately involved more than 3000

  5. Genome-wide linkage and association mapping identify susceptibility alleles in ABCC4 for Kawasaki disease

    NARCIS (Netherlands)

    Khor, C.C.; Davila, S.; Shimizu, C.; Sheng, S.; Matsubara, T.; Suzuki, Y.; Newburger, J.W.; Baker, A.; Burgner, D.; Breunis, W.; Kuijpers, T.; Wright, V.J.; Levin, M.; Hibberd, M.L.; Burns, J.C.

    2011-01-01

    Kawasaki disease (KD) is a self limited vasculitis in which host genetics plays a prominent role. To further the understanding of the role of host genetics in KD, a three-stage genetic study was conducted that began with a family linkage study and ultimately involved more than 3000 individuals to

  6. Genome-wide linkage and association mapping identify susceptibility alleles in ABCC4 for Kawasaki disease

    NARCIS (Netherlands)

    Khor, C.C.; Davila, S.; Shimizu, C.; Sheng, S.; Matsubara, T.; Suzuki, Y.; Newburger, J.W.; Baker, A.; Burgner, D.; Breunis, W.; Kuijpers, T.; Wright, V.J.; Levin, M.; Hibberd, M.L.; Burns, J.C.

    2011-01-01

    Kawasaki disease (KD) is a self limited vasculitis in which host genetics plays a prominent role. To further the understanding of the role of host genetics in KD, a three-stage genetic study was conducted that began with a family linkage study and ultimately involved more than 3000 individuals to id

  7. Genome-wide linkage and association mapping identify susceptibility alleles in ABCC4 for Kawasaki disease

    NARCIS (Netherlands)

    Khor, Chiea Chuen; Davila, Sonia; Shimizu, Chisato; Sheng, Stephanie; Matsubara, Tomoyo; Suzuki, Yasuo; Newburger, Jane W.; Baker, Annette; Burgner, David; Breunis, Willemijn; Kuijpers, Taco; Wright, Victoria J.; Levin, Michael; Hibberd, Martin L.; Burns, Jane C.

    2011-01-01

    Background Kawasaki disease (KD) is a self limited vasculitis in which host genetics plays a prominent role. To further the understanding of the role of host genetics in KD, a three-stage genetic study was conducted that began with a family linkage study and ultimately involved more than 3000 indivi

  8. A genome-wide association study identifies novel and functionally related susceptibility loci for Kawasaki Disease

    NARCIS (Netherlands)

    Burgner, D.; Davila, S.; Breunis, W.B.; Ng, S.B.; Li, Y.; Bonnard, C.; Ling, L.; Wright, V.J.; Thalamuthu, A.; Odam, M.; Shimizu, C.; Burns, J.C.; Levin, M.; Kuijpers, T.W.; Hibberd, M.L.

    2009-01-01

    Kawasaki disease (KD) is a pediatric vasculitis that damages the coronary arteries in 25% of untreated and approximately 5% of treated children. Epidemiologic data suggest that KD is triggered by unidentified infection(s) in genetically susceptible children. To investigate genetic determinants of KD

  9. Cardiac lesions and initial laboratory data in Kawasaki disease: a nationwide survey in Japan.

    Science.gov (United States)

    Kuwabara, Masanari; Yashiro, Mayumi; Kotani, Kazuhiko; Tsuboi, Satoshi; Ae, Ryusuke; Nakamura, Yosikazu; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2015-01-01

    Cardiac lesions, such as coronary dilatation, aneurysms, narrowing, myocardial infarction, and valvular lesions, sometimes occur in Kawasaki disease, but most studies have only evaluated cardiac lesions in the later phase of the disease. This study was undertaken to clarify the related factors between cardiac lesions and laboratory data in the initial phase of Kawasaki disease. We conducted a cross-sectional study using data for 26 691 patients from the 22nd nationwide survey of Kawasaki disease in Japan, the observation period of which was from January 2011 through December 2012. We excluded patients with recurrent Kawasaki disease and who were more than seven days from the start of symptoms at admission. We analyzed 23 155 cases (13 353 boys; mean age: 923 ± 734 days) with available laboratory data for white blood cell count, platelet count, serum albumin, and C-reactive protein (CRP). Cardiac lesions were detected in 984 cases (656 boys and 328 girls); lesions were classified as coronary dilatation (764 cases), coronary aneurysm (40), giant coronary aneurysm (6), coronary narrowing (3), and valvular lesions (204). The significant related factors of initial coronary dilatation were male sex (odds ratio [OR] 1.73), older age (OR per 100 days increase 1.03), higher platelet count (OR per 10 000 cells/µL increase 1.006), lower albumin (OR per 1 g/dL increase 0.66), and higher CRP (OR per 1 mg/dL increase 1.02). The factors related to coronary aneurysm were higher platelet count (OR 1.01) and lower albumin (OR 0.34). No factors were significantly related to giant coronary aneurysm. The related factors of valvular lesions were age (OR 0.98), and higher CRP (OR 1.05). Clinicians should consider male sex, older age, higher platelet count, lower albumin levels, and higher CRP levels when assessing risk of cardiac lesions in the initial phase of Kawasaki disease.

  10. Incomplete Kawasaki disease with recurrent skin peeling: a case report with the review of literature.

    Directory of Open Access Journals (Sweden)

    Parmar R

    2003-01-01

    Full Text Available Kawasaki disease (KD is an acute systemic vasculitis of unknown aetiology that has largely replaced rheumatic heart disease as a cause of acquired heart disease in children of many developed countries. We report a case of incomplete KD in a five-year-old girl. The diagnosis of incomplete KD was made after exclusion of conditions with similar presentation. She was treated with intravenous immunoglobulin following which she made an uneventful recovery but demonstrated thrombocytosis in the second week of convalescence. During the six-month follow up period, she had two episodes of recurrent skin peeling a phenomenon, which is recently reported with KD but not with atypical or incomplete KD. It is important for the treating physicians to become aware of the incomplete KD as prompt diagnosis and early treatment of these patients with intravenous immunoglobulin is vital for the prevention of lethal coronary complications. Physicians need to have a "high index of suspicion" for KD and even, higher for IKD.

  11. An apparent case of undiagnosed donor Kawasaki disease manifesting as coronary artery aneurysm in a pediatric heart transplant recipient.

    Science.gov (United States)

    Friedland-Little, Joshua; Aiyagari, Ranjit; Gajarski, Robert J; Schumacher, Kurt R

    2013-02-01

    We present a case of coronary ectasia and LAD coronary artery aneurysm with angiographic characteristics of Kawasaki disease in a three-yr-old girl two-yr status post-orthotopic heart transplant. Coronary anomalies were noted during initial screening coronary angiography two yr after transplant. Subsequent review of the donor echocardiogram revealed that the LMCA had been mildly dilated prior to transplant. In the absence of any symptoms consistent with Kawasaki disease in the transplant recipient, this appears to be a case of Kawasaki disease in the organ donor manifesting with coronary anomalies in the transplant recipient. The patient has done well clinically, and repeat coronary angiography has revealed partial regression of coronary anomalies. Given multiple reports in the literature of persistent abnormalities of coronary artery morphology and function after Kawasaki disease, close monitoring is warranted, with consideration of potential coronary protective medical therapies. © 2012 John Wiley & Sons A/S.

  12. Comparation of clinical and paraclinical findings among patient with Kawasaki disease in Bandar abbas Koodakan Hospital in 2011-14

    Science.gov (United States)

    Borjali, Davood

    Title: Comparation of clinical and paraclinical findings among patient with Kawasaki disease in Bandar abbas Koodakan Hospital in 2011-14 Kawasaki disease(KD) is a kind of vasculitis diagnosed by clinical manifestation and it caused acquired heart disease in children because of coronary arteries involvement. Method: patient divided to three group of American Japanese and incomplete and also study in two group according to fever days and then clinical features and laboratory data were checked. Result: A total of 150 patients were enrolled during the study period. number of patients with incomplete Kawasaki disease was 128 american group was 28 and Japanese was 4 patients, the most prevalent symptom was scaling of extremities(61 bladder most seen in group with fever more than five days. Keyword: Kawasaki , epidemiology , criteria

  13. Left ventricular non-compaction revealed by aortic regurgitation due to Kawasaki disease in a boy with LDB3 mutation.

    Science.gov (United States)

    Hachiya, Akira; Motoki, Noriko; Akazawa, Yohei; Matsuzaki, Satoshi; Hirono, Keiichi; Hata, Yukiko; Nishida, Naoki; Ichida, Fukiko; Koike, Kenichi

    2016-08-01

    Kawasaki disease (KD) is an acute febrile illness of childhood characterized by systemic vasculitis, especially coronary arteritis. Aortic valve regurgitation (AVR) is a relatively common complication. There have been no reports to date of heart failure and left ventricular non-compaction (LVNC) after acute KD, although the precise etiology of this condition remains unclear. A 6-month-old boy with KD was admitted to hospital. Despite high-dose i.v. gammaglobulin for dilation of the coronary artery, moderate AVR appeared, and thereafter he developed heart failure. A rough, dense LV myocardium indicated LVNC. On genetic testing a heterogenous 163G > A substitution changing a valine to isoleucine in LIM domain binding protein 3 (LDB3) was identified. Additional cardiac stress, such as that caused by AVR and/or KD might have triggered cardiac failure in the form of LVNC due to LDB3 mutation.

  14. Kawasaki disease in children and its nursing%小儿川崎病及护理

    Institute of Scientific and Technical Information of China (English)

    胡敬华

    2014-01-01

    目的:川崎病又称为皮肤黏膜淋巴结综合征,是一种以变态反应性全身小血管炎为主要病理改变的结缔组织病,主要表现为急性发热、皮疹、皮肤黏膜病损和淋巴结肿大。我国近年来发病率明显增多,常导致严重的冠状动脉病变和心脏损害。因此,了解病情、加强治疗护理措施,对缩短病程、减少并发症、加快疾病康复,有着十分重要的意义。%Kawasaki disease is also been called skin mucous membrane lymph node syndrome.It is a kind of allergic systemic vasculitis as the main pathological changes of connective tissue disease.Its main performances are acute fever,rash,skin and mucous membrane lesions and lymph nodes.In recent years,the incidence of this disease has increased significantly in our country, often leading to coronary artery disease and serious heart damage.Therefore,understanding of the disease,strengthen the treatment and care measures have a very important significance in shortening the course of disease,decreasing the complications and accelerating recovery from disease.

  15. Kawasaki disease in Sicily: clinical description and markers of disease severity.

    Science.gov (United States)

    Maggio, Maria Cristina; Corsello, Giovanni; Prinzi, Eugenia; Cimaz, Rolando

    2016-11-02

    Kawasaki disease (KD) is an acute systemic vasculitis of small and middle size arteries; 15-25 % of untreated patients and 5 % of patients treated with intravenous immunoglobulin (IVIG) develop coronary artery lesions (CAL). Many studies tried to find the most effective treatment in the management of resistant KD and to select the risk factors for CAL. Our data are assessed on children from west Sicily, characterized by a genetic heterogeneity. We studied the clinical data of 70 KD Sicilian children (36 males: 51 %; 34 females: 49 %), analysed retrospectively, including: demographic and laboratory parameters; echocardiographic findings at diagnosis, at 2, 6 and 8 weeks, and at 1 year after the onset of the illness. Forty-seven had Typical KD, three Atypical KD and twenty Incomplete KD. Age at the disease onset ranged from 0.1 to 8.9 years. IVIG were administered 5 ± 2 days after the fever started. Defervescence occurred 39 ± 26 hours after the first IVIG infusion. Fifty-six patients (80 %) received 1 dose of IVIG (responders); 14 patients (20 %) had a resistant KD, with persistent fever after the first IVIG dose (non responders). Ten (14 %) non responders responded to the second dose, 4 (5 %) responded to three doses; one needed treatment with high doses of steroids and Infliximab. Cardiac involvement was documented in twenty-two cases (eighteen with transient dilatation/ectasia, fifteen with aneurysms). Pericardial effusion, documented in eleven, was associated with coronaritis and aneurysms, and was present earlier than coronary involvement in seven. Hypoalbuminemia, D-dimer pre-IVIG, gamma-GT pre-IVIG showed a statistically significant direct correlation with IVIG doses, highlighting the role of these parameters as predictor markers of refractory disease. The persistence of elevated CRP, AST, ALT levels, a persistent hyponatremia and hypoalbuminemia after IVIG therapy, also had a statistical significant correlation with IVIG doses. Non responders

  16. Serum and urinary interleukin-6 (IL-6) levels as predicting factors of Kawasaki disease activity.

    Science.gov (United States)

    Chow, Y M; Lin, C Y; Hwang, B

    1993-01-01

    From January 1988 to Autumn 1991, 60 patients suffering from Kawasaki disease (KD) were recruited in this study. Their ages ranged from 4 months to 5 years. Diagnosis was based on the criteria revised in 1984 by the KD Research Committee in Japan. Of these, 12 cases developed coronary aneurysms. First, blood samples from 60 KD patients were taken on admission before aspirin and/or intravenous immunoglobulin (IVIG) treatment. Convalescent blood samples were taken 3 months after onset of disease. The control group included (1) 10 cases of viral infection with skin rash and fever (aged 5 months to 5 years) and (2) 10 age and sex matched normal children admitted for elective pediatric surgery such as inguinal hernia. Second, urinary samples were collected from 32 cases during the acute phase of KD. Of these, 10 cases had pyuria and/or proteinuria. The results showed that the serum IL-6 levels from KD patients during the first week of acute phase were significantly increased while undetectable in the convalescent sera and controls. There was also a statistical difference between the with and without coronary aneurysm groups during the first week (336.8 +/- 95.1 vs 125.5 +/- 56.5 pg/ml, P Urinary IL-6 levels were significantly elevated in KD patients with pyuria and/or proteinuria (156.6 +/- 77.7 pg/mg Cr) and undetectable in the group without pyuria and proteinuria and controls during the first week. There was no difference between with and without coronary aneurysm. These results suggest that serum IL-6 level is a useful factor for predicting formation of coronary aneurysm even within one week after onset of disease.(ABSTRACT TRUNCATED AT 250 WORDS)

  17. [Kawasaki disease is more prevalent in rural areas of Catalonia (Spain)].

    Science.gov (United States)

    Sánchez-Manubens, Judith; Antón, Jordi; Bou, Rosa; Iglesias, Estibaliz; Calzada-Hernandez, Joan; Rodó, Xavier; Morguí, Josep-Antón

    2017-02-23

    Kawasaki disease (KD) is an acute self-limited systemic vasculitis relatively common in childhood. The etiology of KD is still unknown, although clinical, laboratory and epidemiological features suggest an infectious origin or trigger. Differences on incidence between countries have been related to specific genetic factors, ethnicity, country of birth and some other sociocultural and environmental factors. We present a population-based study on incidence of KD in Catalonia (Spain), focusing on differences between patients in rural and non-rural areas of the region. Observational population-based study including all Pediatric Units in Catalan hospitals, between 2004 and 2014. A 12-month (March 2013-March 2014) prospective collection of new cases of KD was carried out to determine the incidence of KD. The rest of the data was retrieved retrospectively. Data from 399 patients over the 10-year study period was analyzed. Among the total KD patients, 353 (88.5%) lived in non-rural areas and 46 (11.5%) in rural areas. It was found that there is a significant difference (P<.001) between the percentage of rural population observed in patients with KD (11.5%), and the expected 5% of the Catalan population. This is the first population-based study showing significant differences on KD incidence rates between rural and non-rural areas. Copyright © 2017. Publicado por Elsevier España, S.L.U.

  18. Clinical Manifestations Vary with Different Age Spectrums in Infants with Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    Hao-Chuan Liu

    2012-01-01

    Full Text Available Background. Kawasaki disease (KD is an acute systemic vasculitis with unknown etiology. The diagnosis of KD depends on clinical manifestations. The prevalence of coronary artery abnormality (CAA is 11.0% and results in cardiac sequelae, such as myocardial infarction or coronary aneurysm, which are the most serious complications in KD. Methods. We divided KD's children into different age groups: ≤6 months old, 7 months to 1 year old, and >1 year old, respectively. Different parameters were compared in each group. Results. Infants ≤6 months old are less likely to fulfill KD's major diagnostic criteria within 10 days, are prone to develop incomplete KD with the lowest cholesterol level, and have the greatest chance to have CAA and the laboratory features associated with CAA, such as the longest time needed to confirm CA diagnosis, lower hemoglobin level, lower albumin level, and higher platelet count. Infants <1 year old develop higher percentage of leukocytosis and sterile pyuria. But this group has fewer patients with neck lymphadenopathy.

  19. CFD-based Thrombotic Risk Assessment in Kawasaki Disease Patients with Coronary Artery Aneurysms

    Science.gov (United States)

    Sengupta, Dibyendu; Kung, Ethan; Kahn, Andrew; Burns, Jane; Marsden, Alison

    2012-11-01

    Coronary aneurysms occur in 25% of untreated Kawasaki Disease (KD) patients and put patients at increased risk for myocardial infarction and sudden death. Clinical guidelines recommend using aneurysm diameter >8 mm as the arbitrary criterion for treating with anti-coagulation therapy. This study uses patient-specific modeling to non-invasively determine hemodynamic parameters and quantify thrombotic risk. Anatomic models were constructed from CT angiographic image data from 5 KD aneurysm patients and one normal control. CFD simulations were performed to obtain hemodynamic data including WSS and particle residence times (PRT). Thrombosis was clinically observed in 4/9 aneurysmal coronaries. Thrombosed vessels required twice as many cardiac cycles (mean 8.2 vs. 4.2) for particles to exit, and had lower mean WSS (1.3 compared to 2.8 dynes/cm2) compared to vessels with non-thrombosed aneurysms of similar max diameter. 1 KD patient in the cohort with acute thrombosis had diameter < 8 mm. Regions of low WSS and high PRT predicted by simulations correlated with regions of subsequent thrombus formation. Thrombotic risk stratification for KD aneurysms may be improved by incorporating both hemodynamic and geometric quantities. Current clinical guidelines to assess patient risk based only on aneurysm diameter may be misleading. Further prospective study is warranted to evaluate the utility of patient-specific modeling in risk stratifying KD patients with coronary aneurysms. NIH R21.

  20. Urinary Lactate Dehydrogenase Activity and Its Isozyme Patterns in Kawasaki Disease.

    Science.gov (United States)

    Kawamura, Yoichi; Takeshita, Seiichiro; Kanai, Takashi; Takizawa, Mari; Yoshida, Yusuke; Tsujita, Yuki; Nonoyama, Shigeaki

    2017-01-01

    Abnormal urinary findings, such as sterile pyuria, proteinuria, and microscopic hematuria, are often seen in the acute phase of Kawasaki disease (KD). We investigated the potential significance of urinary lactate dehydrogenase (U-LDH) activity and its isozyme patterns in KD. Total U-LDH activity and its isozymes (U-LDH1-5) levels were compared among 120 patients with KD, 18 patients with viral infection (VI), and 43 patients with upper urinary tract infection (UTI) and additionally compared between intravenous immunoglobulin (IVIG) responders (n = 89) and nonresponders (n = 31) with KD. Total U-LDH activity was higher in KD (35.4 ± 4.8 IU/L, P IVIG nonresponders of KD had significantly higher levels of total U-LDH activity (45.1 ± 4.7 IU/L, P IVIG responders (32.0 ± 2.8 IU/L). KD patients have increased levels of total U-LDH activity, especially U-LDH-1 and U-LDH2, indicating a unique pattern of U-LDH isozymes different from that in UTI patients.

  1. Multiple giant succular and fusiform right and left coronary artery aneurysms after early and adequate treatment of atypical kawasaki disease with unusual presentation.

    Directory of Open Access Journals (Sweden)

    Mostafa Behjati-Ardakani

    2014-06-01

    Full Text Available The major complication of Kawasaki disease is coronary artery dilatation and aneurysm. It occurs in approximately 15-25% of untreated children with Kawasaki Disease. Early diagnosis and treatment with Intravenous immune globulin (IVIG and aspirin (ASA can reduce the incidence of coronary artery abnormality to 2%-5%. We report one case of Atypical Kawasaki Disease with Multiple giant coronary artery aneurysms despite early adequate treatment with IVIG and ASA.

  2. A Novel Truncated Form of Serum Amyloid A in Kawasaki Disease.

    Directory of Open Access Journals (Sweden)

    John C Whitin

    Full Text Available Kawasaki disease (KD is an acute vasculitis in children that can cause coronary artery abnormalities. Its diagnosis is challenging, and many cytokines, chemokines, acute phase reactants, and growth factors have failed evaluation as specific biomarkers to distinguish KD from other febrile illnesses. We performed protein profiling, comparing plasma from children with KD with febrile control (FC subjects to determine if there were specific proteins or peptides that could distinguish the two clinical states.Plasma from three independent cohorts from the blood of 68 KD and 61 FC subjects was fractionated by anion exchange chromatography, followed by surface-enhanced laser desorption ionization (SELDI mass spectrometry of the fractions. The mass spectra of KD and FC plasma samples were analyzed for peaks that were statistically significantly different.A mass spectrometry peak with a mass of 7,860 Da had high intensity in acute KD subjects compared to subacute KD (p = 0.0003 and FC (p = 7.9 x 10-10 subjects. We identified this peak as a novel truncated form of serum amyloid A with N-terminal at Lys-34 of the circulating form and validated its identity using a hybrid mass spectrum immunoassay technique. The truncated form of serum amyloid A was present in plasma of KD subjects when blood was collected in tubes containing protease inhibitors. This peak disappeared when the patients were examined after their symptoms resolved. Intensities of this peptide did not correlate with KD-associated laboratory values or with other mass spectrum peaks from the plasma of these KD subjects.Using SELDI mass spectrometry, we have discovered a novel truncated form of serum amyloid A that is elevated in the plasma of KD when compared with FC subjects. Future studies will evaluate its relevance as a diagnostic biomarker and its potential role in the pathophysiology of KD.

  3. Genome-wide association study identifies FCGR2A as a susceptibility locus for Kawasaki disease.

    Science.gov (United States)

    Khor, Chiea Chuen; Davila, Sonia; Breunis, Willemijn B; Lee, Yi-Ching; Shimizu, Chisato; Wright, Victoria J; Yeung, Rae S M; Tan, Dennis E K; Sim, Kar Seng; Wang, Jie Jin; Wong, Tien Yin; Pang, Junxiong; Mitchell, Paul; Cimaz, Rolando; Dahdah, Nagib; Cheung, Yiu-Fai; Huang, Guo-Ying; Yang, Wanling; Park, In-Sook; Lee, Jong-Keuk; Wu, Jer-Yuarn; Levin, Michael; Burns, Jane C; Burgner, David; Kuijpers, Taco W; Hibberd, Martin L

    2011-11-13

    Kawasaki disease is a systemic vasculitis of unknown etiology, with clinical observations suggesting a substantial genetic contribution to disease susceptibility. We conducted a genome-wide association study and replication analysis in 2,173 individuals with Kawasaki disease and 9,383 controls from five independent sample collections. Two loci exceeded the formal threshold for genome-wide significance. The first locus is a functional polymorphism in the IgG receptor gene FCGR2A (encoding an H131R substitution) (rs1801274; P = 7.35 × 10(-11), odds ratio (OR) = 1.32), with the A allele (coding for histadine) conferring elevated disease risk. The second locus is at 19q13, (P = 2.51 × 10(-9), OR = 1.42 for the rs2233152 SNP near MIA and RAB4B; P = 1.68 × 10(-12), OR = 1.52 for rs28493229 in ITPKC), which confirms previous findings(1). The involvement of the FCGR2A locus may have implications for understanding immune activation in Kawasaki disease pathogenesis and the mechanism of response to intravenous immunoglobulin, the only proven therapy for this disease.

  4. [Association of new functional SNP rs72689236 of CASP3 with Kawasaki disease: a meta-analysis].

    Science.gov (United States)

    Peng, Qian; Chen, Chang-Hui; Wu, Qing; Yang, Yuan

    2013-06-01

    To investigate the association of rs72689236, a new functional single nucleotide polymorphism (SNP) of the gene encoding caspase-3 (CASP3), with the occurrence and development of Kawasaki disease by a meta analysis. A literature search was performed using databases at home and abroad according to inclusion and exclusion criteria, to acquire studies on the relationship between rs72689236 and Kawasaki disease published up to November 2012, including case-control studies and transmission disequilibrium tests. An integrated meta analysis was performed using RevMan 5.1 software after the studies were screened and evaluated. Six studies were extracted for systematic review of the association between rs72689236 and Kawasaki disease. The frequency of allele A of the SNP was significantly higher in patients with Kawasaki disease than in the controls (OR=1.34, 95%CI=1.24-1.46, PKawasaki disease in children with allele A (AA+AG) increased by approximately 44% compared with children with GG (OR=1.44, 95%CI=1.27-1.65, PKawasaki disease patients with coronary artery lesions than in those without coronary artery lesions (OR=1.51, 95%CI=1.10-2.07, P= 0.01); the risk for coronary artery lesions in Kawasaki disease patients with allele A (AA+AG) increased by approximately 59% compared with Kawasaki disease patients with GG (OR=1.59, 95%CI= 1.00-2.53, P=0.05]. No association between this SNP and the therapeutic effect of intravenous immunoglobulin (IVIG) was found in patients with Kawasaki disease. The allele A of functional SNP rs72689236 of CASP3 increases the risk for Kawasaki disease, and it may be used as the genetic marker for susceptibility to coronary artery lesions as a complication of Kawasaki disease. Currently, there is still no sufficient evidence that this SNP has an impact on the therapeutic effect of IVIG in patients with Kawasaki disease, and more studies are needed to investigate the feasibility of its application in individualized treatment.

  5. Kawasaki Disease in Latin American Children: Past, Current, and Future Challenges.

    Science.gov (United States)

    Ulloa-Gutierrez, Rolando; Salgado, Andrea P; Tremoulet, Adriana H

    2014-12-01

    Kawasaki disease (KD) is the leading cause of acquired cardiac disease in children in developed countries and Asia. However, there is a paucity of data available from Latin America. In response to the gap in knowledge about KD in Latin America, a group of pediatric infectious disease researchers from the Kawasaki Disease Research Center at the University of California San Diego and the Sociedad Latinoamericana de Infectología Pediátrica joined efforts during the last decade to address this problem. The Red de Enfermedad de Kawasaki en América Latina (Latin American Kawasaki Disease Network) was launched in 2013 to study the epidemiology of KD among children from the major pediatric tertiary referral hospitals in Latin America. This multinational multicenter network is primarily composed of pediatric infectious diseases, cardiology, rheumatology, and immunology subspecialists and pediatricians from 20 countries, and it is one of the world's largest networks to study the general epidemiology of KD. The first 2 prospective and retrospective multinational multicenter studies looking at the epidemiology of KD in the region were initiated in 2014. Future plans for the network include establishing collaborative research alliances and projects with other centers around the world. To date [ 1], there have been no published studies describing the overall incidence and prevalence of KD in Latin American children. The most important and recent epidemiological study addressing this issue, related to Chile, was published in 2012 [ 2]. Of these, the most recent relevant study addressed the seasonality of KD in different parts of the globe, including some Latin American and Caribbean countries [ 4]. In this document, we briefly summarize relevant available information from Latin America. Although there have been other publications from individual countries that are outside the scope of this communication, the majority of these reports are single case reports, or case series

  6. Sets of serum exosomal microRNAs as candidate diagnostic biomarkers for Kawasaki disease

    Science.gov (United States)

    Jia, Hong-Ling; Liu, Chao-Wu; Zhang, Li; Xu, Wei-Jun; Gao, Xue-Juan; Bai, Jun; Xu, Yu-Fen; Xu, Ming-Guo; Zhang, Gong

    2017-01-01

    Although Kawasaki disease is the main cause of acquired heart disease in children, no diagnostic biomarkers are available. We aimed to identify candidate biomarkers for diagnosing Kawasaki disease using serum exosomal microRNAs (miRNAs). Using frozen serum samples from a biobank, high-throughput microarray technologies, two-stage real-time quantitative PCR, and a self-referencing strategy for data normalization, we narrowed down the list of biomarker candidates to a set of 4 miRNAs. We further validated the diagnostic capabilities of the identified miRNAs (namely, CT(miR-1246)-CT(miR-4436b-5p) and CT(miR-197-3p)-CT(miR-671-5p)) in 79 samples from two hospitals. We found that this 4-miRNA set could distinguish KD patients from other febrile patients as well as from healthy individuals in a single pass, with a minimal rate of false positives and negatives. We thus propose, for the first time, that serum exosomal miRNAs represent candidate diagnostic biomarkers for Kawasaki disease. Additionally, we describe an effective strategy of screening for biomarkers of complex diseases even when little mechanistic knowledge is available. PMID:28317854

  7. Comparative effectiveness of intravenous immunoglobulin from different manufacturing processes on Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    Ming-Chih Lin[

    2014-01-01

    Background: The comparative effectiveness of intravenous immunoglobulin (IVIG) for Kawasaki disease was regarded as inconclusive in the international guidelines. However, several new evidences have been published in recent years. Data sources: A literature search of PubMed was conducted using key words of "Kawasaki disease or mucocutaneous lymph node syndrome" and "immunoglobulin" in combination. Only original articles published after 2004 were selected. A total of 813 papers were found in PubMed. These papers were screened manually by their titles and abstracts. Results: Patients treated with IVIG prepared by betapropiolactonation might have worse outcome (a higher non-responsive rate in one report and a higher rate of coronary aneurysm in two reports). Storage of IVIG in acidic solution might be correlated with a higher rate of coronary aneurysm (two reports). Conclusions: Different processes of preparation and conditions of preservation of IVIG may have profound effects on its clinical effectiveness. Randomized controlled studies are needed to further elucidate this issue.

  8. Kawasaki disease: clustering in infants and pre-school children in Kuwait.

    Science.gov (United States)

    Jawad, N H; Shaltout, A; al-Momem, J; Nahar, A

    1997-03-01

    We report five children who presented within a 2-month period and who all fulfilled at least four of the five criteria essential for the diagnosis of Kawasaki disease. They were three girls and two boys aged between 5 months and 3 years. Two of them had atypical presentations; one mimicked infectious mononucleosis and the other had severe abdominal pain and was later found to have hydrops of the gall bladder. Although treatment was started within the 1st 10 days of the illness, echocardiographic changes were found in three cases: one had myocarditis and the other two showed in the coronary arteries. The occurrence of five cases in such as short period of time is similar to the clusterings of Kawasaki disease reported in Japan and the USA, and strongly suggests the presence of a causative infectious agent.

  9. Comparative effectiveness of intravenous immunoglobulin from different manufacturing processes on Kawasaki disease.

    Science.gov (United States)

    Lin, Ming-Chih

    2014-05-01

    The comparative effectiveness of intravenous immunoglobulin (IVIG) for Kawasaki disease was regarded as inconclusive in the international guidelines. However, several new evidences have been published in recent years. A literature search of PubMed was conducted using key words of "Kawasaki disease or mucocutaneous lymph node syndrome" and "immunoglobulin" in combination. Only original articles published after 2004 were selected. A total of 813 papers were found in PubMed. These papers were screened manually by their titles and abstracts. Patients treated with IVIG prepared by betapropiolactonation might have worse outcome (a higher non-responsive rate in one report and a higher rate of coronary aneurysm in two reports). Storage of IVIG in acidic solution might be correlated with a higher rate of coronary aneurysm (two reports). Different processes of preparation and conditions of preservation of IVIG may have profound effects on its clinical effectiveness. Randomized controlled studies are needed to further elucidate this issue.

  10. 川崎病患儿的护理%Nursing care of children with kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    张琴

    2014-01-01

    目的:探讨川崎病患儿静脉滴注大剂量丙种球蛋白、口服阿司匹林、双嘧达莫等治疗的护理体会。方法:观察138例川崎病患儿临床表现,遵医嘱正确给药,观察给药后的疗效,并采取相应的护理措施。结果:由于药物使用及时,观察和护理得当,138例川崎病患儿全部治愈出院。结论:仔细评估、基础护理、药物护理、心理护理的措施对减轻川崎病患儿痛苦、治疗和预后有重要意义。%Objective:Investigate children with kawasaki disease high-dose intravenous drip gamma globulin, oral aspirin and dipyridamole nursing experience of treatment. Methods:Observe 138 cases of children with kawasaki disease clinical manifestations, prescribed dosing correctly, to observe the curative effect after the treatment, and take the corresponding nursing measures.Result:Due to drug use, timely observation and care properly, al 106 cases of children with kawasaki disease cure the hospital.Conclusion:Careful evaluation, basic nursing, medication nursing, psychological nursing measures to relief the children with kawasaki disease, treatment and prognosis.

  11. Infliximab as the First Retreatment in Patients with Kawasaki Disease Resistant to Initial Intravenous Immunoglobulin.

    Science.gov (United States)

    Youn, Youngmin; Kim, Jisoo; Hong, Young Mi; Sohn, Sejung

    2016-04-01

    Forty-three patients with Kawasaki disease who were resistant to initial intravenous immunoglobulin (IVIG) were randomized to receive either a second dose of IVIG (n = 32) or an infliximab (n = 11). With IVIG retreatment 21 patients (65.6%) responded, and with infliximab 10 patients (90.9%) responded. The infliximab group had shorter duration of fever and fewer days of hospitalization. Coronary artery outcomes and adverse events were similar.

  12. Kawasaki disease and the emerging coronary artery disease epidemic in India: is there a correlation?

    Science.gov (United States)

    Singh, Surjit; Aulakh, Roosy; Kawasaki, Tomisaku

    2014-04-01

    Although Kawasaki disease (KD) is now being increasingly reported from India, the vast majority of children with KD are still not being diagnosed and treated. A recent study from Chandigarh has shown that the incidence of KD is at least 4.54/100,000 children below 15 y of age. Extrapolations of this figure suggest that a minimum of 17,417 new cases of KD would be occurring every year in our country. A significant proportion of these children may develop coronary artery abnormalities. These children would then be at risk of developing myocardial ischemia as young adults. It is authors' contention that (undiagnosed) KD in childhood may be contributing to the growing pool of coronary artery disease (CAD) in India. Similarly, a missed diagnosis of KD in childhood should be considered as a possibility while evaluating adults with CAD, especially when there are no overt risk factors and no family history of the disease.

  13. Involvement of innate and adaptive immunity in a murine model of coronary arteritis mimicking Kawasaki disease.

    Science.gov (United States)

    Schulte, Danica J; Yilmaz, Atilla; Shimada, Kenichi; Fishbein, Michael C; Lowe, Emily L; Chen, Shuang; Wong, Michelle; Doherty, Terence M; Lehman, Thomas; Crother, Timothy R; Sorrentino, Rosalinda; Arditi, Moshe

    2009-10-15

    Kawasaki disease (KD) is the most common cause of acquired cardiac disease and acute vasculitis in children in the developed world. Injection of a cell wall extract isolated from Lactobacillus casei (LCCWE) into mice causes a focal coronary arteritis that histopathologically mimics the coronary lesions observed in KD patients. In this study we used this model to investigate the participation of T cells, B cells, and dendritic cells (DC) in the development of coronary arteritis. RAG1(-/-), B cell(null), and wild-type (WT) mice were injected with a single dose of LCCWE (500 microg/mouse i.p.). None of the RAG1(-/-) mice developed coronary arteritis, whereas 70% of WT and 100% of B cell(null) mice developed coronary lesions, indicating that T cells were required for lesion formation. When splenocytes isolated from LCCWE-treated mice were restimulated with LCCWE, we observed significant IFN-gamma secretion in WT but not in RAG1(-/-) mice. Immunohistochemical staining showed F4/80(+) macrophages, activated MIDC-8(+) myeloid DCs (mDC), plasmacytoid DCs, and colocalization of CD3(+) T cells with mDCs in coronary artery lesions, suggesting an Ag-driven process. T cells but not B cells are required for LCCWE-induced coronary arteritis. Similar to human lesions, the coronary lesions contain macrophages, activated mDCs, and plaslmacytoid DCs all in close proximity to T cells, further strengthening the relevance of this mouse model to the immunopathology of coronary disease in KD. These studies are consistent with the interpretation that macrophages and DCs may collaborate with T cells in the pathological mechanisms of coronary arteritis.

  14. IL-1 Inhibition May Have an Important Role in Treating Refractory Kawasaki Disease

    Science.gov (United States)

    Dusser, Perrine; Koné-Paut, Isabelle

    2017-01-01

    Kawasaki disease (KD) is an acute inflammatory vasculitis occurring in young children before 5 years and representing at this age, the main cause of acquired heart disease. A single infusion of 2 g/kg of intravenous immunoglobulins along with aspirin has reduced the frequency of coronary artery aneurysms from 25 to 5%. However, 10–20% of patients do not respond to standard treatment and have an increased risk of cardiac complications and death. The development of more potent therapeutic approaches of KD is an urgent need. Phenotypical and immunological similarities between KD and systemic juvenile idiopathic arthritis led to the hypothesis that KD could be considered as an autoinflammatory disease. New insights regarding KD’s pathogenesis have merged from the combination of genetic and transcriptomic data revealing the key role of interleukin-1 (IL-1) signaling in the pathogenesis of the vasculitis. Once activated, IL-1α and IL-1β trigger a local proinflammatory environment-inducing vasodilatation and attracting monocytes and neutrophils to sites causing tissue damage and stress. Both IL-1α and IL-1β have been shown to induce myocarditis and aneurysm formation in Lactobacillus casei cell-wall extract mouse model of KD; both being successfully improved with IL-1 blockade treatment such as anakinra. Treatment failure in patients with the high-risk inositol-triphosphate 3-kinase C genotype was associated with highest basal and stimulated intracellular calcium levels, increased cellular production of IL-1β, and IL-18, and higher circulating levels of both cytokines. Three clinical trials of IL-1 blockade enrolling KD patients are currently being conducted in Western Europe and in USA, they could change KD outcome. PMID:28400731

  15. Periodic fever in MVK deficiency: a patient initially diagnosed with incomplete Kawasaki disease.

    Science.gov (United States)

    Thors, Valtyr S; Vastert, Sebastiaan J; Wulffraat, Nico; van Royen, Annet; Frenkel, Joost; de Sain-van der Velden, Monique; de Koning, Tom J

    2014-02-01

    Mevalonate kinase deficiency (MKD) is a rare autosomal recessive disorder causing 1 of 2 phenotypes, hyperimmunoglobulin D syndrome and mevalonic aciduria, presenting with recurrent fever episodes, often starting in infancy, and sometimes evoked by stress or vaccinations. This autoinflammatory disease is caused by mutations encoding the mevalonate kinase (MVK) gene and is classified in the group of periodic fever syndromes. There is often a considerable delay in the diagnosis among pediatric patients with recurrent episodes of fever. We present a case of an 8-week-old girl with fever of unknown origin and a marked systemic inflammatory response. After excluding infections, a tentative diagnosis of incomplete Kawasaki syndrome was made, based on the finding of dilated coronary arteries on cardiac ultrasound and fever, and she was treated accordingly. However, the episodes of fever recurred, and alternative diagnoses were considered, which eventually led to the finding of increased excretion of mevalonic acid in urine. The diagnosis of MKD was confirmed by mutation analysis of the MVK gene. This case shows that the initial presentation of MKD can be indistinguishable from incomplete Kawasaki syndrome. When fever recurs in Kawasaki syndrome, other (auto-)inflammatory diseases must be ruled out to avoid inappropriate diagnostic procedures, ineffective interventions, and treatment delay.

  16. 川崎病合并低钠血症38例临床分析%Clinical Analysis of 38 Cases of Kawasaki Disease With Low Sodium Blood

    Institute of Scientific and Technical Information of China (English)

    李美花

    2016-01-01

    Objective To observe and analyze the clinical features of Kawasaki's disease combined with hyponatremia. Methods The clinical manifestations of 38 cases of Kawasaki disease complicated with hyponatremia were collected, and the results of statistical laboratory examination were collected. Results Kawasaki's disease complicated with hyponatremia in acute phase of white blood cell count, neutrophil percentage, CRP, AST, ALT increased significantly, serum albumin and serum sodium decreased signiifcantly, most associated with organ damage. Conclusion Kawasaki disease complicated with hyponatremia in children with acute inlfammation and multiple organ damage.%目的:观察并分析川崎病合并低钠血症的临床特点。方法收集38例川崎病合并低钠血症患儿观察临床表现,统计实验室检查结果。结果川崎病合并低钠血症患儿急性期血白细胞总数、中性粒细胞百分比、CRP、AST、ALT明显升高,血白蛋白及血钠离子明显降低,多数合并有脏器损伤。结论川崎病合并低钠血症患儿急性期炎症反应重,损伤多个脏器。

  17. Detection Rate and Clinical Impact of Respiratory Viruses in Children with Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    Ja Hye Kim

    2012-12-01

    Full Text Available &lt;B&gt;Purpose:&lt;/B&gt; The purpose of this prospective case-control study was to survey the detection rate of respiratory viruses in children with Kawasaki disease (KD by using multiplex reverse transcriptasepolymerase chain reaction (RT-PCR, and to investigate the clinical implications of the prevalence of respiratory viruses during the acute phase of KD. &lt;B&gt;Methods:&lt;/B&gt; RT-PCR assays were carried out to screen for the presence of respiratory syncytial virus A and B, adenovirus, rhinovirus, parainfluenza viruses 1 to 4, influenza virus A and B, metapneumovirus, bocavirus, coronavirus OC43/229E and NL63, and enterovirus in nasopharyngeal secretions of 55 KD patients and 78 control subjects. &lt;B&gt;Results:&lt;/B&gt; Virus detection rates in KD patients and control subjects were 32.7% and 30.8%, respectively (P=0.811. However, there was no significant association between the presence of any of the 15 viruses and the incidence of KD. Comparisons between the 18 patients with positive RT-PCR results and the other 37 KD patients revealed no significant differences in terms of clinical findings (including the prevalence of incomplete presentation of the disease and coronary artery diameter. &lt;B&gt;Conclusion:&lt;/B&gt; A positive RT-PCR for currently epidemic respiratory viruses should not be used as an evidence against the diagnosis of KD. These viruses were not associated with the incomplete presentation of KD and coronary artery dilatation.

  18. Urinary Lactate Dehydrogenase Activity and Its Isozyme Patterns in Kawasaki Disease

    Science.gov (United States)

    Kawamura, Yoichi; Kanai, Takashi; Takizawa, Mari; Yoshida, Yusuke; Tsujita, Yuki; Nonoyama, Shigeaki

    2017-01-01

    Abnormal urinary findings, such as sterile pyuria, proteinuria, and microscopic hematuria, are often seen in the acute phase of Kawasaki disease (KD). We investigated the potential significance of urinary lactate dehydrogenase (U-LDH) activity and its isozyme patterns in KD. Total U-LDH activity and its isozymes (U-LDH1-5) levels were compared among 120 patients with KD, 18 patients with viral infection (VI), and 43 patients with upper urinary tract infection (UTI) and additionally compared between intravenous immunoglobulin (IVIG) responders (n = 89) and nonresponders (n = 31) with KD. Total U-LDH activity was higher in KD (35.4 ± 4.8 IU/L, P < 0.05) and UTI patients (66.0 ± 8.0 IU/L, P < 0.01) than in VI patients (17.0 ± 6.2 IU/L). In the isozyme pattern analysis, KD patients had high levels of U-LDH1 and U-LDH2, while UTI patients had high levels of U-LDH3, U-LDH4, and U-LDH5. Furthermore, IVIG nonresponders of KD had significantly higher levels of total U-LDH activity (45.1 ± 4.7 IU/L, P < 0.05), especially U-LDH1 and U-LDH2 (P < 0.05), than IVIG responders (32.0 ± 2.8 IU/L). KD patients have increased levels of total U-LDH activity, especially U-LDH-1 and U-LDH2, indicating a unique pattern of U-LDH isozymes different from that in UTI patients. PMID:28348604

  19. Relationship between serum sodium level and coronary artery abnormality in Kawasaki disease

    Science.gov (United States)

    Park, Sora; Kim, Ji Hong

    2017-01-01

    Purpose Kawasaki disease (KD) is an immune-related multisystemic vasculitis that occurs in children, especially ensuing from a coronary artery abnormality. Sodium level is known to be related to vascular injury, which could affect the progress of KD. The purpose of this study was to determine the serum sodium levels that could predict the occurrence of cardiac and coronary artery events in KD. Methods We conducted a retrospective review of medical records for 104 patients with KD from January 2015 to December 2015. Patients with serum Na levels of <135 mEq/L at the time of initial diagnosis were assigned to the hyponatremia group. Laboratory findings and echocardiographic data were analyzed for various aspects. Results Among the 104 patients with KD, 91 were included in the study, of whom 48 (52.7%) had hyponatremia. The degree of fever, white blood cell count, percentage of neutrophils, percentage of lymphocytes, total bilirubin level, brain natriuretic peptide level, erythrocyte sedimentation rate, and C-reactive protein level were higher in the patients with hyponatremia. They also demonstrated a trend of larger coronary artery diameters based on Z scores. Conclusion The severity of vascular inflammation in acute KD with hyponatremia might worsen the prognosis of coronary vasculature. Although no statistically significant correlation was found between the initial serum sodium levels and coronary arteriopathy in the patients with KD in this study, a long-term follow-up study with a larger number of enrolled patients should be designed in the future to elucidate the relationship between serum sodium level and coronary arteriopathy in patients with KD. PMID:28289432

  20. Urinary Lactate Dehydrogenase Activity and Its Isozyme Patterns in Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    Yoichi Kawamura

    2017-01-01

    Full Text Available Abnormal urinary findings, such as sterile pyuria, proteinuria, and microscopic hematuria, are often seen in the acute phase of Kawasaki disease (KD. We investigated the potential significance of urinary lactate dehydrogenase (U-LDH activity and its isozyme patterns in KD. Total U-LDH activity and its isozymes (U-LDH1-5 levels were compared among 120 patients with KD, 18 patients with viral infection (VI, and 43 patients with upper urinary tract infection (UTI and additionally compared between intravenous immunoglobulin (IVIG responders (n=89 and nonresponders (n=31 with KD. Total U-LDH activity was higher in KD (35.4±4.8 IU/L, P<0.05 and UTI patients (66.0±8.0 IU/L, P<0.01 than in VI patients (17.0±6.2 IU/L. In the isozyme pattern analysis, KD patients had high levels of U-LDH1 and U-LDH2, while UTI patients had high levels of U-LDH3, U-LDH4, and U-LDH5. Furthermore, IVIG nonresponders of KD had significantly higher levels of total U-LDH activity (45.1±4.7 IU/L, P<0.05, especially U-LDH1 and U-LDH2 (P<0.05, than IVIG responders (32.0±2.8 IU/L. KD patients have increased levels of total U-LDH activity, especially U-LDH-1 and U-LDH2, indicating a unique pattern of U-LDH isozymes different from that in UTI patients.

  1. Hepcidin-Induced Iron Deficiency Is Related to Transient Anemia and Hypoferremia in Kawasaki Disease Patients

    Science.gov (United States)

    Huang, Ying-Hsien; Kuo, Ho-Chang; Huang, Fu-Chen; Yu, Hong-Ren; Hsieh, Kai-Sheng; Yang, Ya-Ling; Sheen, Jiunn-Ming; Li, Sung-Chou; Kuo, Hsing-Chun

    2016-01-01

    Kawasaki disease (KD) is a type of systemic vasculitis that primarily affects children under the age of five years old. For sufferers of KD, intravenous immunoglobulin (IVIG) has been found to successfully diminish the occurrence of coronary artery lesions. Anemia is commonly found in KD patients, and we have shown that in appropriately elevated hepcidin levels are related to decreased hemoglobin levels in these patients. In this study, we investigated the time period of anemia and iron metabolism during different stages of KD. A total of 100 patients with KD and 20 control subjects were enrolled in this study for red blood cell and hemoglobin analysis. Furthermore, plasma, urine hepcidin, and plasma IL-6 levels were evaluated using enzyme-linked immunosorbent assay in 20 KD patients and controls. Changes in hemoglobin, plasma iron levels, and total iron binding capacity (TIBC) were also measured in patients with KD. Hemoglobin, iron levels, and TIBC were lower (p < 0.001, p = 0.009, and p < 0.001, respectively) while plasma IL-6 and hepcidin levels (both p < 0.001) were higher in patients with KD than in the controls prior to IVIG administration. Moreover, plasma hepcidin levels were positively and significantly correlated with urine hepcidin levels (p < 0.001) prior to IVIG administration. After IVIG treatment, plasma hepcidin and hemoglobin levels significantly decreased (both p < 0.001). Of particular note was a subsequent gradual increase in hemoglobin levels during the three weeks after IVIG treatment; nevertheless, the hemoglobin levels stayed lower in KD patients than in the controls (p = 0.045). These findings provide a longitudinal study of hemoglobin changes and among the first evidence that hepcidin induces transient anemia and hypoferremia during KD’s acute inflammatory phase. PMID:27187366

  2. Clinical analysis on 179 children with Kawasaki disease%179例川崎病临床分析

    Institute of Scientific and Technical Information of China (English)

    李艳春; 鲁继荣; 王冰; 李善玉

    2012-01-01

    Objective; To compare the clinical characteristics of typical Kawasaki disease and incomplete Kawasaki disease, improve the levels of diagnosis and treatment for Kawasaki disease. Methods; The clinical data of 179 children with Kawasaki disease from Jilin province from January 2005 to January 2009 were analyzed retrospectively. The clinical characteristics of typical Kawasaki disease and incomplete Kawasaki disease, the curative effects of intravenous gamma globulin (single - dose) and intravenous gamma globulin ( non - single -dose), the curative effects of gamma globulin for typical Kawasaki disease and incomplete Kawasaki disease were compared, respectively. Results; Among 179 children with Kawasaki disease, 111 children were found with typical Kawasaki disease and 68 children were found with incomplete Kawasaki disease, the incidence of incomplete Kawasaki disease showed an increasing trend year by year; except perianal desquamation, the incidences of other clinical manifestations in typical Kawasaki disease group were higher than those in incomplete Kawasaki disease group ( P < 0. 05); except coronary artery dilatation, there was no significant difference in the incidences of other complications between the two groups. In typical Kawasaki disease group, the count of white blood cells increased significantly (the average count; 13. 8 X 109/L) , the incidence of anemia increased significantly (51.4% ) , compared with incomplete Kawasaki disease group, there was significant difference (P <0. 01) ; in the two groups, more than 80% of the children were sensitive to gamma globulin therapy, the curative effect of intravenous gamma globulin (single - dose) was better than that of intravenous gamma globulin ( non - single - dose) (P < 0.05) . Conclusion; The incidence of incomplete Kawasaki disease increases significantly, compared with typical Kawasaki disease, incomplete Kawasaki disease has its own characteristics, early diagnosis and treatment are recommended.%目

  3. Ruptured pediatric posterior cerebral artery aneurysm 9 years after the onset of Kawasaki disease: a case report.

    Science.gov (United States)

    Tanaka, Satoshi; Sagiuchi, Takao; Kobayashi, Ikuo

    2007-06-01

    A 12-year-old boy who had a history of Kawasaki disease 9 years ago experienced a subarachnoid hemorrhage by ruptured right posterior cerebral artery aneurysm. On day 1 operation, as the aneurysm was very fragile and bled easily, two intraoperative ruptures, including a very premature rupture, were encountered. As a result, a left hemiparesis especially severe in the left hand was caused by the right anterior thalamic infarction due to the occlusion of a thalamo-perforating artery arising near the neck of the aneurysm. The histopathological examination of the intraoperative excised aneurysmal dome disclosed the thickening of the endothelial inner due to the endothelial hypertrophy and the invasion of inflammatory cells. This finding of the aneurysm was partially mimicking the finding of the coronary artery of the patients with Kawasaki disease. The combination of cerebral aneurysm and Kawasaki disease has never been reported until now, and the etiology of the aneurysm of this patient is unclear.

  4. Changes in plasma hydrogen sulfide and nitric oxide levels and their clinical significance in children with Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    LI Xiao-hui; ZHANG Chao-ying; WU Jian-xin; ZHANG Ting

    2011-01-01

    Background Cardiac involvement is the most common complication of Kawasaki disease (KD); however,the underlying mechanisms are not understood.The present study was designed to investigate changes in plasma hydrogen sulfide (H2S) and nitric oxide (NO) levels in the acute and recovery stages of KD children and to examine their clinical significance.Methods Thirty-five KD patients and 32 healthy children were enrolled in the study.KD patients were divided into two subgroups:a non-cardiac involvement group and a cardiac involvement group.Plasma H2S levels were measured using the sulfur-sensitive electrode method and plasma NO levels and NO synthase activity were determined using the nitrate reductase method both before and after intravenous immune globulin (IVIG) therapy.Results Plasma H2S levels significantly decreased in KD patients during the acute phase of the disease and NO levels were significantly increased,compared with the control group (P <0.01).After treatment with IVIG,both plasma H2S and NO levels significantly increased (P <0.01).The plasma levels of H2S were significantly lower in the cardiac involvement group compared with the non-cardiac involvement group (P<0.05).Conclusion H2S and NO may play a role in the pathophysiological process of inflammation during the acute phase of KD.Endogenous H2S may exert protective effects with respect to cardiac complications in KD.

  5. Ulinastatin, a urinary trypsin inhibitor, for the initial treatment of patients with Kawasaki disease: a retrospective study.

    Science.gov (United States)

    Kanai, Takashi; Ishiwata, Takahiro; Kobayashi, Tohru; Sato, Hiroki; Takizawa, Mari; Kawamura, Yoichi; Tsujimoto, Hiroshi; Nakatani, Keigo; Ishibashi, Naoko; Nishiyama, Mitsunori; Hatai, Yoshiho; Asano, Yuh; Kobayashi, Tomio; Takeshita, Seiichiro; Nonoyama, Shigeaki

    2011-12-20

    Markedly activated neutrophils or higher plasma levels of neutrophil elastase are involved in the poor response to intravenous immunoglobulin (IVIG) and the formation of coronary artery lesions (CAL) in patients with acute Kawasaki disease. We hypothesized that ulinastatin (UTI), by both direct and indirect suppression of neutrophils, would reduce the occurrence of CAL. We retrospectively analyzed the clinical records of patients with Kawasaki disease between 1998 and 2009. Three hundred sixty-nine patients were treated with a combination of UTI, aspirin, and IVIG as an initial treatment (UTI group), and 1178 were treated with a conventional initial treatment, and IVIG with aspirin (control group). The baseline characteristics did not demonstrate notable differences between the two groups. The occurrence of CAL was significantly lower in the UTI group than in the control group (3% versus 7%; crude odds ratio [OR], 0.46; 95% confidence interval [CI], 0.25-0.86; P=0.01). The OR adjusted for sex, Gunma score (the predictive score for IVIG unresponsiveness), and dosage of initial IVIG (1 or 2 g/kg) was 0.32 (95% CI, 0.17-0.60; P<0.001). In addition, most CAL occurred in patients requiring additional rescue treatment and the proportion of those patients was significantly lower in the UTI group than in the control group (13% versus 22%; crude OR, 0.52; 95% CI, 0.38-0.73; P<0.001). The adjusted OR was 0.30 (95% CI, 0.20-0.44; P<0.001). UTI was associated with fewer patients requiring additional rescue treatment and reduction of CAL in this retrospective study.

  6. Meta-analysis of the relationship between single nucleotide polymorphism rs72689236 of caspase-3 and Kawasaki disease.

    Science.gov (United States)

    Xing, Yanlin; Wang, Hong; Liu, Xiaomei; Yu, Xianyi; Chen, Rui; Wang, Ce; Yu, Xuexin; Sun, Le

    2014-10-01

    Kawasaki disease is a pediatric systemic vasculitis of unknown etiology, for which a genetic influence is suspected. But whether single nucleotide polymorphism (SNP) of caspase-3 rs72689236 is associated with Kawasaki disease is controversial. The aim of our study is to assess the association between the SNP of caspase-3 and risk for Kawasaki disease. We searched PubMed, MEDLINE, EMBASE, Springer, Elsevier Science Direct, Cochrane Library Google scholar, CNKI (China National Knowledge Infrastructure, in Chinese) and Wanfang database (in Chinese) to identify studies investigating the association between rs72689236 polymorphism and Kawasaki disease occurrence. There were five eligible studies, which included 4,241 (case group 1,560; control group 2,681) participants in this meta-analysis. Pooled odds ratios (ORs) and 95 % confidence intervals (95 % CIs) were calculated in a fixed-effects model (the Mantel-Haenszel method) or a random-effects model (the DerSimonian and Laird method) when appropriate. Significant associations were found under the overall ORs for A-allele comparison (A vs. G, pooled OR 1.33, 95 % CI 1.21-1.46), AA versus GG comparison (pooled OR 1.64, 95 % CI 1.35-2.00), GA versus GG comparison (pooled OR 1.42, 95 % CI 1.24-1.63), recessive model (AA vs. GG + GA, pooled OR 1.37, 95 % CI 1.15-1.64) and dominant model (AA + GA vs. GG, pooled OR 1.47, 95 % CI 1.29-1.67). This meta-analysis suggested that SNP rs72689236 of caspase-3 might be associated with susceptibility of Kawasaki disease and the allele A might increase the risk of Kawasaki disease in Asian samples such as Japanese and Chinese. In addition, individual studies with large sample size are needed to further evaluate the associations in various ethnic populations.

  7. Comparison of early and late intravenous gamma globulin treatment of Kawasaki disease on fever and cardiovascular complications.

    Science.gov (United States)

    Mohammadzadeh, Iraj; Noei, Somayyeh; Babazadeh, Kazem; Zamani, Hassan; Barari-Savadkoohi, Rahim; Alizadeh-Navaei, Reza

    2016-01-01

    Cardiac involvement was the major leading cause of death in patients with Kawasaki and IVIG administration reduces cardiac complications. The objective of this study was to determine the frequency of cardiovascular complications and duration of fever with regard to the time of intravenous immunoglobulin (IVIG) administration of patients with Kawasaki disease. This follow-up study was done on all patients with Kawasaki disease who were hospitalized at Amirkola Children's Hospital between 2006 and 2011. Diagnosis of Kawasaki was clinical and included fever more than 5 days with 4 of 5 signs containing mucosal changes, scaling and skin rash, bilateral nonexudative conjunctivitis, cervical lymph adenopathy and edema in lower extremities. After diagnosis of Kawasaki, all patients received standard treatment (intravenous immunoglobulins and aspirin) and undergoing cardiac echocardiography in 2 weeks, 2 months and 6 months. Information including age, sex, sign of diseases, laboratory findings, and cardiac complications in echocardiography were recorded. This study was performed on 100 patients (61 boys and 39 girls) with Kawasaki disease. The mean age of children was 2.8±2.6 years. Cardiac complication rate was 47% at the onset of the disease and had reached to 7% at the end of the sixth month (P=0.000). Distribution of cardiovascular complications in the second week, the second month and the sixth month after treatment was not significantly different according to the start of time of treatment (p>0.05). Duration of fever in patients who received treatment before 10(th) day (1.5±1.3) did not have significant difference (P=0.78) with patients who received after 10(th) day (1.6±0.9). Result shows that most of patients (99%) responded to the treatment with IVIG and ASA and cardiovascular complication ratio decreased. There was not significant relationship between duration of fever and time of IVIG treatment initiation.

  8. [Identification of mutations associated with coronary artery lesion susceptibility in Kawasaki disease by targeted enrichment of genomic region sequencing technique].

    Science.gov (United States)

    Zhu, D Y; Song, S R; Xie, L J; Qiu, F; Yang, J; Xiao, T T; Huang, M

    2017-07-02

    Objective: To screen and identify the mutations in Kawasaki disease by targeted enrichment of genomic region sequencing technique and investigate susceptibility genes associated with coronary artery lesion. Method: This was a case-control study.A total of 114 patients diagnosed as Kawasaki disease treated in Shanghai Children's Hospital between December 2015 and November 2016 were studied and another 45 healthy children who were physically examined in outpatient department were enrolled as control group. Patients were divided into two groups based on the results of echocardiogram. Peripheral venous blood was obtained from patients and controls. Genomic DNA was extracted. SeqCap EZ Choice libraries were prepared by targeted enrichment of genomic region technology. Then the libraries were sequenced to identify susceptibility genes associated with coronary artery lesion in patients diagnosed as Kawasaki disease.Susceptible genes were identified by Burden test, Pearson chi-square test or Fisher's exact probability test. Result: There was statistically significant difference in TNFRSF11B(rs2073618)G>C(p.N3K)mutation and GG/GC/CC genotype between Kawasaki disease group and control group(χ(2)=15.52, P=0.00). There was statistically significant difference in TNFRSF13B(rs34562254)C>T(p.P251L)mutation(χ(2)=10.40, P=0.01)and LEFTY1(rs360057)T>G(p.D322A)mutation(χ(2)=8.505, P=0.01)between patients with coronary artery lesions and those without. Conclusion: Targeted enrichment of genomic region sequencing technology can be used to do primary screening for the susceptible genes associated with coronary artery lesions in Chinese Kawasaki patients and may provide theoretical basis for larger sample investigation of risk prediction score standard in Kawasaki disease.

  9. Coronary artery abnormalities in Kawasaki disease - Comparison between CT and MR coronary angiography

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jong Woo; Goo, Hyun Woo [Dept. of Radiology and Research Inst. of Radiology, Univ. of Ulsan Coll. of Medicine, Asan Medical Center, Seoul (Korea, Republic of)], e-mail: hwoogoo@amc.seoul.kr

    2013-03-15

    Background: Although CT coronary angiography (CTCA) and MR coronary angiography (MRCA) are increasingly used in patients with Kawasaki disease, comparison of coronary artery assessability and diagnostic performance between the two imaging modalities has been rarely performed. Purpose: To investigate which imaging modality, CTCA or MRCA, is better for evaluating coronary artery abnormalities in patients with Kawasaki disease. Material and Methods: Between 2003 and 2011, 56 patients (38 boys/men; age range, 1-24 years) with Kawasaki disease underwent CTCA or MRCA (group A). Of these, 17 underwent both CTCA and MRCA (group B). Visibility of 11 coronary arterial segments in each patient was graded on a four-point scale. Coronary artery aneurysm, stenosis, and occlusion were evaluated by CTCA and MRCA, based on a reference standard obtained from cardiac catheterization, echocardiography, follow-up CTCA and MRCA, and clinical history. Coronary artery assessability and diagnostic performance were compared between CTCA and MRCA. Results: In per-segment analysis, more segments were assessable on CTCA than on MRCA in both groups. In per-patient analysis of group B, no significant difference in the assessability was found between CTCA (95.0%, 128.3/135 segments) and MRCA (92.4%, 124.8/135 segments) (P > 0.05). Sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of CTCA vs. MRCA were 93.1% vs. 77.9% (P < 0.001), 99.2% vs. 99.7% (P = 0.65), 96.8% vs. 98.7% (P = 0.65), 98.2% vs. 94.1% (P < 0.001), and 98.0% vs. 94.9% (P = 0.008), respectively, in group A, and 91.8% vs. 70.4% (P < 0.001), 99.5% vs. 99.5% (P = 1.000), 98.5% vs. 98.0% (P = 1.000), 97.2% vs. 91.1% (P = 0.006), and 97.6% vs. 92.3% (P = 0.004), respectively, in group B. Conclusion: Although CTCA and MRCA show comparable assessability in per-patient analysis, CTCA shows higher diagnostic performance than MRCA for evaluating coronary artery abnormalities in patients with Kawasaki

  10. Diagnosis of Kawasaki Disease%川崎病的诊断

    Institute of Scientific and Technical Information of China (English)

    李万镇

    2006-01-01

    川崎病(Kawasaki Disease,KD),又名皮肤粘膜淋巴结综合征(MCLS),1967年日本川崎氏首先报告,其病因、发病机理不明,是全身血管炎为主要病变的急性热性发疹性疾病,多侵犯冠状动脉,部分患儿形成冠状动脉瘤,其中少部分患儿冠状动脉可发生狭窄或血栓,甚至导致心肌梗死.

  11. Progress in Research of Incomplete Kawasaki Disease%不完全川崎病的研究进展

    Institute of Scientific and Technical Information of China (English)

    郭鹏飞; 易岂建

    2009-01-01

    @@ 川崎病(kawasaki disease, KD)是一种以全身弥漫性血管炎为主要病变的急性发热出疹性疾病,80%发生于5岁以下的儿童[1].1967 年日本 Kawasaki Tomisaku 博士首先描述本病.该病目前已取代风湿热成为小儿后天性心脏病的主要原因[2].

  12. 不典型川崎病18例临床诊治分析%Clinical Diagnosis and Treatment of 18 Cases of Atypical Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    覃艳

    2015-01-01

    目的:了解不典型川崎病的诊疗方法,提高基层医院医师对该病的诊治水平,降低川崎病冠状动脉损害发病率。方法结合相关文献,对18例川崎病患儿进行回顾分析。结果18例川崎病均符合不典型川崎病的诊断标准。结论提高对不典型川崎病的诊断水平,早期诊断、中期治疗,可有效降低川崎病并发症尤其是冠状动脉损害的发生。%Objective To understand methods for diagnosis and treatment of atypical Kawasaki disease, in order to improve the level of diagnosis and treatment of primary hospital doctors and reduce the incidence of coronary artery in Kawasaki disease.Methods With related literature, retrospective analysis of 18 patients Kawasaki disease in children.Results18 cases of Kawasaki disease were in line with the diagnostic criteria of atypical Kawasaki disease. ConclusionTo improve the diagnostic level of atypical Kawasaki disease and early diagnosis, treatment can effectively reduce the incidence of Kawasaki disease, especialy coronary artery lesions.

  13. Population-Based Study of the Association between Urbanization and Kawasaki Disease in Taiwan

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    Wei-Pin Chang

    2013-01-01

    Full Text Available 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.

  14. FCGR2A Promoter Methylation and Risks for Intravenous Immunoglobulin Treatment Responses in Kawasaki Disease

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    Ho-Chang Kuo

    2015-01-01

    Full Text Available Kawasaki disease (KD is characterized by pediatric systemic vasculitis of an unknown cause. The low affinity immunoglobulin gamma Fc region receptor II-a (FCGR2A gene was reported to be involved in the susceptibility of KD. DNA methylation is one of the epigenetic mechanisms that control gene expression; thus, we hypothesized that methylation status of CpG islands in FCGR2A promoter associates with the susceptibility and therapeutic outcomes of Kawasaki disease. In this study, 36 KD patients and 24 healthy subjects from out-patient clinic were recruited. Eleven potential methylation sites within the targeted promoter region of FCGR2A were selected for investigation. We marked the eleven methylation sites from A to K. Our results indicated that methylation at the CpG sites G, H, and J associated with the risk of KD. CpG sites B, C, E, F, H, J, and K were found to associate with the outcomes of IVIG treatment. In addition, CpG sites G, J, and K were predicted as transcription factors binding sites for NF-kB, Myc-Max, and SP2, respectively. Our study reported a significant association among the promoter methylation of FCGR2A, susceptibility of KD, and the therapeutic outcomes of IVIG treatment. The methylation levels of CpG sites of FCGR2A gene promoter should be an important marker for optimizing IVIG therapy.

  15. Progress of Diagnosis and Treatment of Kawasaki Disease%川崎病诊治进展

    Institute of Scientific and Technical Information of China (English)

    张晓梅; 孙景辉

    2011-01-01

    The etiology and pathogenesis of Kawasaki disease remains unknown. The etiology may be correlated to infection,genetic susceptibility and super- immune response, pathogenesis relates to immune response ,cytokines and inflammatory mediators, vascular endothelial dysfunction,platelet activation,susceptibility genes polymorphism and so on. Treatment with intravenous immune globulin and aspirin remains the primary management strategy and steroid therapy remains controversial. In severe circumstances,coronary artery surgery or inter ventional means in children with Kawasaki disease has been required,albeit infrequently.%川崎病的病因及发病机制至今未明.其病因可能与感染、遗传易感及超免疫反应有关,发病机制与免疫反应、细胞因子及炎性介质、血管内皮功能紊乱、血小板活化、易感基因多态性等有关.联合应用丙种球蛋白和阿司匹林是川崎病的首选治疗方案.对于糖皮质激素的使用临床尚有争议,严重冠状动脉病变可采用外科及介入治疗.

  16. Novel predictors of intravenous immunoglobulin resistance in Chinese children with Kawasaki disease.

    Science.gov (United States)

    Fu, Pei-pei; Du, Zhong-dong; Pan, Yue-song

    2013-08-01

    The purpose of this study was to develop a predictive scoring system to identify intravenous immunoglobulin resistance in children with Kawasaki disease, to implement additional therapies early in the course of their illness and prevent coronary artery lesions. We performed a retrospective review of children with Kawasaki disease treated within 10 days of fever onset. To identify independent predictors of intravenous immunoglobulin resistance, multivariable logistic regression models were constructed using variables selected by univariable analysis. The independent predictors were combined into a new scoring system and compared with 2 existing systems. The discriminatory capacity of the scoring system was assessed using the area under the receiver operating characteristic curves. By logistic regression analysis, polymorphous exanthema, changes around the anus, days of illness at initial treatment, percentage of neutrophils, C-reactive protein levels, albumin levels, and total bilirubin proved to be independent predictors of intravenous immunoglobulin resistance. The new scoring system gave an area under the receiver operating characteristic curve of 0.672. In this scoring system, 2 risk strata were identified: low risk, with scores of 0-3, and high risk, with scores of ≥4. The sensitivity was 54.1% and the specificity was 71.2%. The new scoring system had a higher specificity and sensitivity for Chinese children, compared with the Kobayashi scoring system and the Egami scoring system, but, unfortunately, the new scoring system was not good enough to be widely used because of its low sensitivity.

  17. Progress of diagnosis and treatment on incomplete Kawasaki disease%不完全川崎病诊治进展

    Institute of Scientific and Technical Information of China (English)

    李永柏

    2010-01-01

    @@ 川崎病(Kawasaki disease,KD)因日本医生川崎富作1967年首次报道而得名,学者几十年观察、研究,现公认KD系小儿多发的急性、自限性、自身免疫性血管炎综合征.

  18. Regression and Complications of z-score-Based Giant Aneurysms in a Dutch Cohort of Kawasaki Disease Patients

    NARCIS (Netherlands)

    Dietz, S.M.; I.M. Kuipers (Irene); Koole, J.C.D.; J.M.P.J. Breur (Johannes M.P.J.); Fejzic, Z.; Frerich, S.; M. Dalinghaus (Michiel); A.A. Roest; Hutten, B.A.; T.W. Kuijpers (Taco W.)

    2017-01-01

    textabstractKawasaki disease (KD) is a pediatric vasculitis. Its main complication is the development of coronary artery aneurysms (CAA), with giant CAA at the end of the spectrum. We evaluated regression and event-free rates in a non-Asian cohort of patients with giant CAA using the current z-score

  19. Assessment of coronary artery lesions in children with Kawasaki disease: evaluation of MSCT in comparison with 2-D echocardiography

    Energy Technology Data Exchange (ETDEWEB)

    Xing, Yanlin; Wang, Hong; Yu, Xianyi; Chen, Rui [Shengjing Hospital of China Medical University, Department of Pediatrics, Shenyang (China); Hou, Yang [Shengjing Hospital of China Medical University, Department of Radiology, Shenyang (China)

    2009-11-15

    Transthoracic two-dimensional echocardiography is an effective method for detecting coronary arterial injury in Kawasaki disease. However, its accuracy in the diagnosis of coronary arterial lesions is limited. To investigate the value of multislice spiral CT for coronary angiography for observing the coronary arterial injury caused by infantile Kawasaki disease. Coronary angiography, using a 64-slice spiral CT scanner, and 2-D echocardiography were performed in 48 children with Kawasaki disease in whom the position, internal diameter, and length of each coronary artery were measured. MSCT showed coronary artery injury in 15 of the 48 children. Among these 15 children, 20 coronary artery branches showed complications, including the left coronary artery branches in 15 (31.2%) and the right coronary artery branches in 5 (10.4%). Complications in the left coronary artery branches included dilation in 12 (25.0%) and stenosis, calcification and the combination of the two in one each, and the right coronary artery branches showed dilation; two branches also showed beaded changes. MSCT also showed dilation in the left anterior descending arteries in two children. These children showed no abnormality on 2-D echocardiography. MSCT is a valuable examination method for detecting coronary artery injury in Kawasaki disease. (orig.)

  20. Hospitalisation with infection, asthma and allergy in Kawasaki disease patients and their families: genealogical analysis using linked population data.

    Directory of Open Access Journals (Sweden)

    Rebecca J Webster

    Full Text Available BACKGROUND: Kawasaki disease results from an abnormal immunological response to one or more infectious triggers. We hypothesised that heritable differences in immune responses in Kawasaki disease-affected children and their families would result in different epidemiological patterns of other immune-related conditions. We investigated whether hospitalisation for infection and asthma/allergy were different in Kawasaki disease-affected children and their relatives. METHODS/MAJOR FINDINGS: We used Western Australian population-linked health data from live births (1970-2006 to compare patterns of hospital admissions in Kawasaki disease cases, age- and sex-matched controls, and their relatives. There were 295 Kawasaki disease cases and 598 age- and sex-matched controls, with 1,636 and 3,780 relatives, respectively. Compared to controls, cases were more likely to have been admitted at least once with an infection (cases, 150 admissions (50.8% vs controls, 210 admissions (35.1%; odds ratio (OR = 1.9, 95% confidence interval (CI 1.4-2.6, P = 7.2×10⁻⁶, and with asthma/allergy (cases, 49 admissions (16.6% vs controls, 42 admissions (7.0%; OR = 2.6, 95% CI 1.7-4.2, P = 1.3×10⁻⁵. Cases also had more admissions per person with infection (cases, median 2 admissions, 95% CI 1-5, vs controls, median 1 admission, 95% CI 1-4, P = 1.09×10⁻⁵. The risk of admission with infection was higher in the first degree relatives of Kawasaki disease cases compared to those of controls, but the differences were not significant. CONCLUSION: Differences in the immune phenotype of children who develop Kawasaki disease may influence the severity of other immune-related conditions, with some similar patterns observed in relatives. These data suggest the influence of shared heritable factors in these families.

  1. Clinical significance of matrix metalloproteinase 9 and tissue inhibitor of metalloproteinase 1 and 2 in Kawasaki disease

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    Ki Wook Yun

    2010-04-01

    Full Text Available Purpose : Kawasaki disease (KD is a systemic vasculitis, a leading cause of pediatric acquired heart disease. Histopathological findings of coronary artery lesion (CAL in KD indicate destruction of the coronary artery wall with diffuse vasculitis. Matrix metalloproteinases (MMPs and their endogenous tissue inhibitors (TIMPs might play central roles in this process. Special attention to MMP-9 has recently been emerging. This study was performed to investigate the clinical significance of MMP-9 and its inhibitors, TIMP-1 and TIMP-2, in KD. Methods : We compared 47 KD patients with 14 febrile controls. Serum MMP-9 and TIMP-1, TIMP-2 were measured by ELISA and compared according to clinical stages and coronary involvement. Results : In acute stage, MMP-9 and TIMP-1 were significantly higher, whereas TIMP-2 was lower, in KD than those in febrile controls (P &lt;0.05. The elevated MMP-9 levels in acute phase significantly decreased during the subacute and convalescent phases (P &lt;0.05. During acute phase, the MMP-9, TIMP-1, and MMP-9/TIMP-2 levels in the CAL group were lower than those in the non-CAL group, but they increased significantly in the subacute phase (P &lt;0.05. MMP-9 has a positive correlation with TIMP-1 in the acute and subacute phases, and negative correlation with TIMP-2 in the subacute and convalescent phases (P &lt;0.05. Conclusion : These results suggest that MMP-9, TIMP-1, and the imbalance in MMP-9 and TIMP-2 might play important roles on the pathophysiology of KD and especially on the development of CAL. However, further larger studies are needed.

  2. Assessment of the ability of myocardial contrast echocardiography with harmonic power Doppler imaging to identify perfusion abnormalities in patients with Kawasaki disease at rest and during dipyridamole stress.

    Science.gov (United States)

    Ishii, M; Himeno, W; Sawa, M; Iemura, M; Furui, J; Muta, H; Sugahara, Y; Egami, K; Akagi, T; Ishibashi, M; Kato, H

    2002-01-01

    The aim of our study was to assess the ability of myocardial contrast echocardiography (MCE) with harmonic power Doppler imaging (HPDI) to identify perfusion abnormalities in patients with Kawasaki disease at rest and during pharmacological stress imaging with dipyridamole. Results were compared with those of 99mTc-tetrofosmin single-photon emission computed tomography (SPECT) imaging as the clinical reference standard. MCE with HPDI was performed on 20 patients with a history of Kawasaki disease. Images were obtained at baseline and during dipyridamole infusion (0.56 mg x kg(-1)) in the apical two- and four-chamber views. Myocardial opacification suitable for the analysis was obtained in all patients. Nine patients with stenotic lesions had a reversible defect after dipyridamole infusion detected by both MCE with HPDI and SPECT, and 3 patients with a history of myocardial infarction had a partially or completely irreversible defect detected by both methods. Three patients with coronary aneurysm without stenotic lesion, 4 patients with regressed coronary aneurysm, and 2 patients with normal coronary artery in acute phase also had normal perfusion at rest and after pharmacological stress by both methods. A 96% concordance (kappa = 0.87) was obtained when comparing the respective segmental perfusion scores using the two methods at baseline, and an 86% concordance (kappa = 0.81) was obtained at postdipyridamole infusion. After combining baseline and postdipyridamole images, each segment was labeled as having normal perfusion, irreversible defects, or reversible defects. Using these classifications, concordance for the two methods was 92% (kappa = 0.87). MCE with HPDI is a safe and feasible method by which to detect asymptomatic ischemia due to severe stenotic lesion, and it may be an important addition to the modalities used to identify patients at risk for myocardial infarction as a complication of Kawasaki disease.

  3. The experience with 113 patients with Kawasaki disease in Fars Province, Iran.

    Science.gov (United States)

    Asadi-Pooya, Ali Akbar; Borzoee, Mohammad; Amoozgar, Hamid

    2006-01-01

    This study was conducted to determine the epidemiology, clinical manifestations, cardiac involvements and laboratory findings of Kawasaki disease (KD) in Fars Province, Iran from January 1991 to December 2002. One hundred and thirteen patients with KD were confirmed. This syndrome occurred mostly in males (ratio=2.1:1). The most commonly affected age group was one to five years old. There was no secondary case in families, none of the patients were related and recurrence of disease was seen in only one patient. Prolonged fever and thrombocytosis were significant risk factors for developing coronary artery disease (p4 mm) and three patients had aneurysm of coronary artery in their first echocardiography. It has been suggested that a high index of clinical suspicion of KD could improve diagnosis and implementation of preventive treatment.

  4. Role of Antioxidants in Horse Serum-mediated Vasculitis in Swine: Potential Relevance to Early Treatment in Mitigation of Coronary Arteritis in Kawasaki Disease.

    Science.gov (United States)

    Philip, Saji; Lee, Wen-Chuan; Cherian, Kotturathu Mammen; Wu, Mei-Hwan; Lue, Hung-Chi

    2017-08-01

    Horse serum-induced immune complex coronary vasculitis in swine is the first experimental model to mimic most of the pictures of Kawasaki disease. Immune complex mechanism has been implicated as one of the possible mechanisms in the pathogenesis of vasculitis in Kawasaki disease. Antioxidants have a significant role in the reduction of cardiovascular diseases in both human and animal studies. We tried giving vitamins A, E, and C to treat immune complex vasculitis, in the hope of mitigating coronary vasculitis in Kawasaki disease. Our study group consisted of 30 pure bred male piglets of 2-3 months of age, and they were divided into test and control groups. The test (AEC) group (n = 20) received two doses of horse serum, 10 mL (0.65 g protein)/kg body weight at 5-day intervals, and oral vitamins A, E, and C once daily for 14 days. The control group (n = 10) was further divided into the saline group (n = 3) receiving two doses of normal saline and the horse serum group (n = 7) receiving two doses of horse serum at 5-day intervals. Piglets were observed for the rashes and coronary artery dimensions. Both the AEC and the control horse serum group developed rashes after horse serum infusions, but the AEC group developed significantly fewer rashes, and no rashes were seen in the saline group. The control horse serum group (mean ± standard deviation = 2.13 ± 0.72) showed significant coronary artery dilatation, whereas there was no significant dilatation in the AEC group (mean ± standard deviation = 0.81 ± 0.58) or the control saline group (p = 0.002). Serum sickness is a prototype of immune complex vasculitis, and the severity can be ameliorated with antioxidants. A trial of therapeutic dosages of vitamins A, E, and C in acute phase of Kawasaki disease, may be effective in mitigation of coronary artery lesion in addition to intravenous immunoglobulin and aspirin. Copyright © 2017. Published by Elsevier B.V.

  5. Kawasaki disease and ENSO-driven wind circulation

    Science.gov (United States)

    Ballester, Joan; Burns, Jane C.; Cayan, Dan; Nakamura, Yosikazu; Uehara, Ritei; Rodó, Xavier

    2013-05-01

    disease (KD) is the most common cause of acquired heart disease in children worldwide. Recently, a climatological study suggested that KD may be triggered by a windborne agent traveling across the north Pacific through the westerly wind flow prevailing at midlatitudes. Here we use KD records to describe the association between enhanced disease activity on opposite sides of the basin and different phases of the El Niño-Southern Oscillation (ENSO) phenomenon, via the linkage to these tropospheric winds. Results show that years with higher-than-normal KD cases in Japan preferentially occur during either El Niño Modoki or La Niña conditions, while in San Diego during the mature phase of El Niño or La Niña events. Given that ENSO offers a degree of predictability at lead times of 6 months, these modulations suggest that seasonal predictions of KD could be used to alert clinicians to periods of increased disease activity.

  6. Cardiac involvement in Kawasaki disease in Pakistani children

    Directory of Open Access Journals (Sweden)

    Saleem Akhtar

    2012-01-01

    Conclusions: A higher incidence of coronary artery involvement was found in our study. Presentation after 10 days of illness increases the risk of coronary artery involvement. High index of suspicion among the general pediatricians about the disease can possibly be helpful for early referral and treatment.

  7. Hemodynamic simulations in coronary aneurysms of a patient with Kawasaki Disease

    Science.gov (United States)

    Sengupta, Dibyendu; Marsden, Alison; Burns, Jane

    2010-11-01

    Kawasaki Disease is the leading cause of acquired pediatric heart disease, and can cause large coronary artery aneurysms in untreated cases. A simulation case study has been performed for a 10-year-old male patient with coronary aneurysms. Specialized coronary boundary conditions along with a lumped parameter heart model mimic the interactions between the ventricles and the coronary arteries, achieving physiologic pressure and flow waveforms. Results show persistent low shear stress in the aneurismal regions, and abnormally high shear at the aneurysm neck. Correlation functions have been derived to compare wall shear stress and wall shear stress gradients with recirculation time with the idea of localizing zones of calcification and thrombosis. Results are compared with those of an artificially created normal coronary geometry for the same patient. The long-term goal of this work is to develop links between hemodynamics and thrombotic risk to assist in clinical decision-making.

  8. Increased Risk of Atopic Dermatitis in Preschool Children with Kawasaki Disease: A Population-Based Study in Taiwan

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    Peng Yeong Woon

    2013-01-01

    Full Text Available Kawasaki disease (KD is an acute febrile systemic vasculitis and has been reported to be associated with allergic disease. The risk of atopic dermatitis (AD in preschool children with KD has not been investigated. The study was to determine the longitudinal risk of the development of AD in preschool children with KD. A nationwide 5-year population-based study was performed using data from the National Health Insurance Database in Taiwan between 1999 and 2003. The risk factors for AD were compared between the 2 study groups during the follow-up period using the Cox proportional hazards model. In addition, plasma interleukin (IL-5 levels were analyzed in normal subjects and KD patients. Among the 1440 subjects included, 21.6% developed AD during the 5-year follow-up period, of which 30.3% and 18.7% belonged to the study cohort and the comparison group, respectively. Children with KD were 1.25 times more likely to have AD than those in controls (P=0.04. Levels of IL-5 and IgE were significantly higher in KD patients. Children with KD had a higher risk of developing AD during the 5-year follow-up period than the control group. Increased IL-5 and IgE levels may be key factors contributing to the risk of AD.

  9. Urine proteomics for discovery of improved diagnostic markers of Kawasaki disease

    Science.gov (United States)

    Kentsis, Alex; Shulman, Andrew; Ahmed, Saima; Brennan, Eileen; Monuteaux, Michael C; Lee, Young-Ho; Lipsett, Susan; Paulo, Joao A; Dedeoglu, Fatma; Fuhlbrigge, Robert; Bachur, Richard; Bradwin, Gary; Arditi, Moshe; Sundel, Robert P; Newburger, Jane W; Steen, Hanno; Kim, Susan

    2013-01-01

    Kawasaki disease (KD) is a systemic vasculitis of unknown etiology. Absence of definitive diagnostic markers limits the accuracy of clinical evaluations of suspected KD with significant increases in morbidity. In turn, incomplete understanding of its molecular pathogenesis hinders the identification of rational targets needed to improve therapy. We used high-accuracy mass spectrometry proteomics to analyse over 2000 unique proteins in clinical urine specimens of patients with KD. We discovered that urine proteomes of patients with KD, but not those with mimicking conditions, were enriched for markers of cellular injury such as filamin and talin, immune regulators such as complement regulator CSMD3, immune pattern recognition receptor muclin, and immune cytokine protease meprin A. Significant elevations of filamin C and meprin A were detected in both the serum and urine in two independent cohorts of patients with KD, comprised of a total of 236 patients. Meprin A and filamin C exhibited superior diagnostic performance as compared to currently used markers of disease in a blinded case-control study of 107 patients with suspected KD, with receiver operating characteristic areas under the curve of 0.98 (95% confidence intervals [CI] of 0.97–1 and 0.95–1, respectively). Notably, meprin A was enriched in the coronary artery lesions of a mouse model of KD. In all, urine proteome profiles revealed novel candidate molecular markers of KD, including filamin C and meprin A that exhibit excellent diagnostic performance. These disease markers may improve the diagnostic accuracy of clinical evaluations of children with suspected KD, lead to the identification of novel therapeutic targets, and allow the development of a biological classification of Kawasaki disease. PMID:23281308

  10. Bull's eye dermatoscopy pattern at bacillus Calmette-Guérin inoculation site correlates with systemic involvements in patients with Kawasaki disease.

    Science.gov (United States)

    Tseng, Han-Chi; Ho, Ji-Chen; Guo, Mindy Ming-Huey; Lo, Mao-Hung; Hsieh, Kai-Sheng; Tsai, Wen-Chien; Kuo, Ho-Chang; Lee, Chih-Hung

    2016-09-01

    For the past decades, although the rash at the bacillus Calmette-Guérin (BCG) inoculation site has been recognized as a diagnostic clue in Kawasaki disease, the present study is the first known one attempting to characterize BCG inoculation by dermatoscopy in Kawasaki disease and correlate the grade of BCG reaction with systemic involvement. Thirty-four patients diagnosed with Kawasaki disease by pediatric specialists were enrolled. We performed detailed history taking, laboratory examination, physical examination and dermatoscopy examinations. Based on the BCG reaction pattern by dermatoscopy, we were able to characterize three patterns: (A) Bull's eye pattern in 18 patients; (B) faint homogenous erythema in nine; and (C) central white patch in seven. Patients from group A exhibited the highest elevation of blood aspartate aminotransferase levels (P Kawasaki disease. © 2016 Japanese Dermatological Association.

  11. Evaluation of myocardial deformation in patients with Kawasaki disease using speckle-tracking echocardiography during mid-term follow-up.

    Science.gov (United States)

    Dedeoglu, Reyhan; Barut, Kenan; Oztunc, Funda; Atik, Sezen; Adrovic, Amra; Sahin, Sezgin; Cengiz, Dicle; Kasapcopur, Ozgur

    2017-04-05

    Speckle-tracking echocardiography is a recently developed technique for the evaluation of myocardial deformation or strain. Our objective was to examine strain through a mid-term follow-up of patients with Kawasaki disease.

  12. Mortality among persons with a history of Kawasaki disease in Japan: can paediatricians safely discontinue follow-up of children with a history of the disease but without cardiac sequelae?

    Science.gov (United States)

    Nakamura, Yosikazu; Aso, Eiko; Yashiro, Mayumi; Uehara, Ritei; Watanabe, Makoto; Tajimi, Morihiro; Oki, Izumi; Ojima, Toshiyuki; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2005-04-01

    To clarify the question of whether patients with Kawasaki disease suffer a higher mortality rate after the incidence of the disease in comparison with age-matched healthy individuals. Between July 1982 and December 1992, 52 collaborating hospitals collected data on all patients having a new, definite diagnosis of Kawasaki disease. Patients were followed up until 31 December 2001 or their death. The expected number of deaths was calculated from Japanese vital statistics data and compared with the observed number. Of 6576 patients enrolled, 29 (20 males and 9 females) died. The standardized mortality ratio (SMR: the observed number of deaths divided by the expected number of deaths based on the vital statistics in Japan) was 1.15 (95% CI: 0.77-1.66). In spite of the high SMRs during the acute phase, the mortality rate was not high after the acute phase for the entire group of patients. Although the SMR after the acute phase was 0.75 for those without cardiac sequelae, six males (but none of the females) with cardiac sequelae died during this period; and the SMR for the male group with cardiac sequelae was 1.95 (95% CI: 0.71-4.25). The mortality from congenital anomalies of the circulatory system was elevated, but no increase in cancer deaths was observed. Although it was not statistically significant, the mortality rate among males with cardiac sequelae due to Kawasaki disease appeared to be higher than in the general population. On the other hand, the mortality rates for females with the sequelae and both males and females without sequelae were not elevated.

  13. The incidence of Kawasaki disease after vaccination within the UK pre-school National Immunisation Programme: an observational THIN database study.

    Science.gov (United States)

    Hall, Gillian C; Tulloh, Robert Mr; Tulloh, Louise E

    2016-11-01

    To provide expected incidence rates of Kawasaki disease after vaccination in routine clinical practice and as recommended within a pre-school National Immunisation Programme (NIP). A post-immunisation risk period when Kawasaki disease onset might be associated with vaccination was defined as 28 days. Immunisation records for children under 6 years were identified from The Health Improvement Network (THIN) database of electronic UK primary health care records (2008-2012) and linked to previously validated cases of Kawasaki disease with an assigned date of onset. Kawasaki disease incidence in the risk period after a complete NIP recommended set of vaccinations was estimated for five vaccination stages individually and in total. A total of 642 170 complete pre-school immunisation stages from 275 986 children were included. Six cases of Kawasaki disease had onset in the risk period after any NIP stage providing an incidence of 12.8 per 100 000 person years (95%CI 5.7, 28.4). The incidence after any single immunisation stage ranged from 0 to 27.4 (95%CI 8.8, 84.8) per 100 000 person years. There were few cases of Kawasaki disease in the risk period after any NIP vaccination combination. The incidence rates will aid in the interpretation of clinical trials and post-marketing surveillance of new vaccines. Copyright © 2016 John Wiley & Sons, Ltd. Copyright © 2016 John Wiley & Sons, Ltd.

  14. Noninvasive quantification of coronary endothelial function by SPECT imaging in children with a history of Kawasaki disease

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    Cicala, Silvana; Paladini, Rodolfo; Leva, Francesco de [Santobono-Pausilipon Children Medical Hospital, Division of Cardiology, Department of Paediatrics, Naples (Italy); Pellegrino, Teresa; Caprio, Maria Grazia [Institute of Diagnostic and Nuclear Development, SDN Foundation, Naples (Italy); Storto, Giovanni [IRCCS, CROB, Rionero in Vulture (Italy); Mainolfi, Ciro; Cuocolo, Alberto [Federico II University, Department of Biomorphological and Functional Sciences, Naples (Italy); National Council of Research, Institute of Biostructures and Bioimages, Naples (Italy)

    2010-12-15

    The feasibility of coronary function estimation by single photon emission computed tomography (SPECT) has been recently demonstrated. The aim of this study was to apply SPECT imaging in patients with previous Kawasaki disease (KD) to assess the coronary functional status at long-term follow-up of the acute phase of the disease. Sixteen children with a history of KD underwent {sup 99m}Tc-sestamibi imaging at rest and during the cold pressor test (CPT). Myocardial blood flow (MBF) was estimated by measuring first transit counts in the pulmonary artery and myocardial counts from SPECT images. Coronary endothelial function was expressed as the ratio of the CPT to rest MBF. Six KD patients without coronary artery lesions served as controls and ten with coronary artery aneurysms during the acute phase of the disease were separated into two groups: group 1 (n = 4) with regressed and group 2 (n = 6) with persistent aneurysm at follow-up. The estimated coronary endothelial function was higher in controls compared to patients with coronary artery aneurysms (2.5 {+-} 0.3 vs 1.7 {+-} 0.7, p < 0.05). A significant difference in coronary endothelial function among groups was found (F = 5.21, p < 0.02). Coronary endothelial function was higher in patients of group 1 than in those of group 2 (1.9 {+-} 0.6 vs 1.4 {+-} 0.7, p < 0.02). SPECT may be applied as a noninvasive method for assessing coronary vascular function in children with a history of KD, demonstrating an impaired response to the CPT, an endothelial-dependent vasodilator stimulus. These findings reinforce the concept that coronary endothelial dysfunction may represent a long-term sequela of KD. (orig.)

  15. Enfermedad de Kawasaki

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    Carlos Coronel Carvajal

    2003-09-01

    Full Text Available Se realizó una revisión actualizada de la enfermedad de Kawasaki, con el objetivo de familiarizar a pediatras y médicos de la familia con los elementos clínicos de la enfermedad, para que de esta manera pueda ser diagnosticada y manejada oportunamente. La enfermedad de Kawasaki fue descrita por Tomisaki Kawasaki en 1967, y se manifiesta como un síndrome febril en la niñez temprana. La enfermedad se diagnostica con mayor frecuencia en los menores de 5 años, a través de una serie de criterios clínicos establecidos. La forma típica de la enfermedad tiene 3 fases de duración variable. La afectación cardíaca es la manifestación más importante de la enfermedad, del 10 al 40 % de los niños muestran signos de vasculitis coronaria, manifestada por aneurismas de las arterias coronarias.An updated review of Kawasaki disease was carried out to make pediatricians and family physicians familiar with the clinical elements of the disease so that it can be properly diagnosed and managed. Kawasaki disease was described by Tomisaki Kawasaki in 1967 and manifests as a febrile syndrome at early childhood. The disease is very often diagnosed in under five years-old children on the basis of a series of set clinical criteria. The typical form of the disease had 3 phases of variable duration. The cardiac effect is the most important manifestation of the disease since 10 to 40 % of children show signs of coronary vasculitis in the form of coronary arterial aneurysms.

  16. Kawasaki Disease

    Science.gov (United States)

    ... Genitals and Urinary Tract Glands & Growth Head Neck & Nervous System Heart Infections Learning Disabilities Obesity Orthopedic Prevention Sexually Transmitted Skin Tobacco Treatments Injuries & ...

  17. Research Advances in the Treatment of Kawasaki Disease%川崎病治疗的研究进展

    Institute of Scientific and Technical Information of China (English)

    韩冬

    2011-01-01

    川崎病病因及发病机制尚不明确,易累及冠状动脉而造成冠状动脉损伤,甚至冠状动脉瘤,严重影响患儿预后.冠状动脉损伤是目前我国乃至全世界发达国家常见的后天性心脏病之一.近年来,川崎病及其所致的冠状动脉损伤的治疗已有相对统一的标准,目前糖皮质激素、乌司他丁、英夫利昔单抗等在临床得到有限使用,但对于丙种球蛋白无反应性川崎病及冠状动脉病变的治疗等仍存在不同观点.%The etiology and pathogenesis of Kawasaki disease remains unclear, which frequently involves coronary artery and results in coronary artery injuries and aneurysm, seriously affecting the prognosis of pediatric patients. Coronary artery injury is one of the most common acquired heart diseases in China and even in the developed countries around the world. In recent years, a uniform standard has been established for the treatment of Kawasaki disease and coronary artery injury secondary to Kawasaki disease. In addition,the use of glucocorticoids, urinary trypsin inhibitor and infliximab are restricted in clinical practice. However, some different views of the treatment of no-response to gamma globulin in Kawasaki disease and coronary artery disease still exists.

  18. Revisiting the role of environmental and climate factors on the epidemiology of Kawasaki disease.

    Science.gov (United States)

    Rodó, Xavier; Ballester, Joan; Curcoll, Roger; Boyard-Micheau, Joseph; Borràs, Sílvia; Morguí, Josep-Anton

    2016-10-01

    Can environmental factors, such as air-transported preformed toxins, be of key relevance to the health outcomes of poorly understood human ailments (e.g., rheumatic diseases such as vasculitides, some inflammatory diseases, or even severe childhood acquired heart diseases)? Can the physical, chemical, or biological features of air masses be linked to the emergence of diseases such as Kawasaki disease (KD), Henoch-Schönlein purpura, Takayasu's aortitis, and ANCA-associated vasculitis? These diseases surprisingly share some common epidemiological features. For example, they tend to appear as clusters of cases grouped geographically and temporarily progress in nonrandom sequences that repeat every year in a similar way. They also show concurrent trend changes within regions in countries and among different world regions. In this paper, we revisit transdisciplinary research on the role of environmental and climate factors in the epidemiology of KD as a paradigmatic example of this group of diseases. Early-warning systems based on environmental alerts, if successful, could be implemented as a way to better inform patients who are predisposed to, or at risk for, developing KD. Further research on the etiology of KD could facilitate the development of vaccines and specific medical therapies.

  19. Augmented TLR2 expression on monocytes in both human Kawasaki disease and a mouse model of coronary arteritis.

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    I-Chun Lin

    Full Text Available BACKGROUND: Kawasaki disease (KD of unknown immunopathogenesis is an acute febrile systemic vasculitis and the leading cause of acquired heart diseases in childhood. To search for a better strategy for the prevention and treatment of KD, this study compared and validated human KD immunopathogenesis in a mouse model of Lactobacillus casei cell wall extract (LCWE-induced coronary arteritis. METHODS: Recruited subjects fulfilled the criteria of KD and were admitted for intravenous gamma globulin (IVIG treatment at the Kaohsiung Chang Gung Memorial Hospital from 2001 to 2009. Blood samples from KD patients were collected before and after IVIG treatment, and cardiovascular abnormalities were examined by transthoracic echocardiography. Wild-type male BALB/c mice (4-week-old were intraperitoneally injected with LCWE (1 mg/mL to induce coronary arteritis. The induced immune response in mice was examined on days 1, 3, 7, and 14 post injections, and histopathology studies were performed on days 7 and 14. RESULTS: Both human KD patients and LCWE-treated mice developed coronary arteritis, myocarditis, valvulitis, and pericarditis, as well as elevated plasma levels of interleukin (IL-2, IL-6, IL-10, monocyte chemoattractant protein (MCP-1, and tumor necrosis factor (TNF-α in acute phase. Most of these proinflammatory cytokines declined to normal levels in mice, whereas normal levels were achieved in patients only after IVIG treatment, with a few exceptions. Toll-like receptor (TLR-2, but not TLR4 surface enhancement on circulating CD14+ monocytes, was augmented in KD patients before IVIG treatment and in LCWE-treated mice, which declined in patients after IVIG treatment. CONCLUSION: This result suggests that that not only TLR2 augmentation on CD14+ monocytes might be an inflammatory marker for both human KD patients and LCWE-induced CAL mouse model but also this model is feasible for studying therapeutic strategies of coronary arteritis in human KD by

  20. Augmented TLR2 expression on monocytes in both human Kawasaki disease and a mouse model of coronary arteritis.

    Science.gov (United States)

    Lin, I-Chun; Kuo, Ho-Chang; Lin, Ying-Jui; Wang, Feng-Shen; Wang, Lin; Huang, Shun-Chen; Chien, Shao-Ju; Huang, Chien-Fu; Wang, Chih-Lu; Yu, Hong-Ren; Chen, Rong-Fu; Yang, Kuender D

    2012-01-01

    Kawasaki disease (KD) of unknown immunopathogenesis is an acute febrile systemic vasculitis and the leading cause of acquired heart diseases in childhood. To search for a better strategy for the prevention and treatment of KD, this study compared and validated human KD immunopathogenesis in a mouse model of Lactobacillus casei cell wall extract (LCWE)-induced coronary arteritis. Recruited subjects fulfilled the criteria of KD and were admitted for intravenous gamma globulin (IVIG) treatment at the Kaohsiung Chang Gung Memorial Hospital from 2001 to 2009. Blood samples from KD patients were collected before and after IVIG treatment, and cardiovascular abnormalities were examined by transthoracic echocardiography. Wild-type male BALB/c mice (4-week-old) were intraperitoneally injected with LCWE (1 mg/mL) to induce coronary arteritis. The induced immune response in mice was examined on days 1, 3, 7, and 14 post injections, and histopathology studies were performed on days 7 and 14. Both human KD patients and LCWE-treated mice developed coronary arteritis, myocarditis, valvulitis, and pericarditis, as well as elevated plasma levels of interleukin (IL)-2, IL-6, IL-10, monocyte chemoattractant protein (MCP)-1, and tumor necrosis factor (TNF)-α in acute phase. Most of these proinflammatory cytokines declined to normal levels in mice, whereas normal levels were achieved in patients only after IVIG treatment, with a few exceptions. Toll-like receptor (TLR)-2, but not TLR4 surface enhancement on circulating CD14+ monocytes, was augmented in KD patients before IVIG treatment and in LCWE-treated mice, which declined in patients after IVIG treatment. This result suggests that that not only TLR2 augmentation on CD14+ monocytes might be an inflammatory marker for both human KD patients and LCWE-induced CAL mouse model but also this model is feasible for studying therapeutic strategies of coronary arteritis in human KD by modulating TLR2-mediated immune activation on CD14

  1. Predictive value of red blood cell distribution width for coronary artery lesions in patients with Kawasaki disease.

    Science.gov (United States)

    Xu, Haiyan; Fu, Songling; Wang, Wei; Zhang, Qing; Hu, Jian; Gao, Lichao; Zhu, Weihua; Gong, Fangqi

    2016-08-01

    Recent studies have shown that elevated red blood cell distribution width is associated with poor outcome in cardiovascular diseases. In order to assess the predictive value of red blood cell distribution width, before treatment with intravenous immunoglobulins, for coronary artery lesions in patient with Kawasaki disease, we compared 83 patients with coronary artery lesions and 339 patients without coronary artery lesions before treatment with intravenous immunoglobulin. Clinical, echocardiographic, and biochemical values were evaluated along with red blood cell distribution width. A total of 422 consecutive patients with Kawasaki disease were enrolled into our study. According to receiver operating characteristic curve analysis, the optimal red blood cell distribution width cut-off value for predicting coronary artery lesions was 14.55% (area under the curve was 0.721; p=0.000); eighty-three patients (19.7%) had coronary artery lesions, and 70% of the patients with coronary artery lesions had red blood cell distribution width level >14.55%. Logistic regression analysis revealed that fever duration >14 days (odds ratio was 3.42, 95% confidence interval was 1.27-9.22; p=0.015), intravenous immunoglobulin resistance (odds ratio was 2.33, 95% confidence interval was 1.02-5.29; p=0.04), and red blood cell distribution width >14.55% (odds ratio was 3.49, 95% confidence interval was 2.01-6.05; p=0.000) were independent predictors of coronary artery lesions in patients with Kawasaki disease. In Conclusion, red blood cell distribution width may be helpful for predicting coronary artery lesions in patients with Kawasaki disease.

  2. Crystalline-Like Keratopathy after Intravenous Immunoglobulin Therapy with Incomplete Kawasaki Disease: Case Report and Literature Review

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    Elif Erdem

    2013-01-01

    Full Text Available A 7-year-old girl had presented with high body temperature and joint pain which continued for 3 days. Because of the prolonged history of unexplained fever, rash, bilateral nonpurulent conjunctival injection, oropharyngeal erythema, strawberry tongue, and extreme of age, incomplete Kawasaki disease was considered and started on an intravenous immunoglobulin infusion. Six days after this treatment, patient was referred to eye clinic with decreased vision and photophobia. Visual acuity was reduced to 20/40 in both eyes. Slit-lamp examination revealed bilateral diffuse corneal punctate epitheliopathy and anterior stromal haze. Corneal epitheliopathy seemed like crystal deposits. One day after presentation, mild anterior uveitis was added to clinical picture. All ocular findings disappeared in one week with topical steroid and unpreserved artificial tear drops. We present a case who was diagnosed as incomplete Kawasaki disease along with bilateral diffuse crystalline-like keratopathy. We supposed that unusual ocular presentation may be associated with intravenous immunoglobulin treatment.

  3. Coronary artery thrombus resulting in sudden cardiac death in an infant with Kawasaki disease and giant coronary artery aneurysms

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    Umakumaran Ponniah

    2013-01-01

    Full Text Available We report a case of a six-month-old Hispanic male infant who had Kawasaki disease and coronary artery aneurysms on echocardiography. He died suddenly five months later in spite of aggressive medical therapy. Autopsy showed extensive coronary artery thrombosis. Giant coronary artery aneurysms need diligent follow up as they pose significant risks including risk of thrombus, myocardial infarction and sudden death.

  4. The changes of negative-regulatory factors A20, IRF-4 and TRAF4 of toll-like receptor signal pathways in immunological pathogenesis of Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    GUO BING WANG; CHENG RONG LI; JUN YANG; YING ZHU

    2007-01-01

    To investigate the role of negative-regulatory factors A20, IRF-4 and TRAF4 of the toll-like receptor (TLR) signal pathways in immunological pathogenesis of Kawasaki disease (KD), 48 children with Kawasaki disease, 16 children with infectious disease (ID) and 16 age-matched healthy children were studied. Reverse-transcription PCR (RT-PCR) and real-time PCR were used to evaluate the expression levels of negative-regulatory and effective factors in toll-like receptor 4 (TLR4) signal pathways and proinflammatory factors in peripheral blood monocyte/maerophage (MC). In this study, expression levels of TLR4, MD-2, MyD88, IRAK-4, TRAF6, TAK1, TAB1 and TAB2 Mrna in KD group were detected to be elevated significantly during acute phase of KD. Transcription levels of negative-regulatory factors A20, IRF-4 and TRAF4 Mrna in KD or ID patients increased remarkably. However, expressions of IRF-4 and TRAF4 in KD patients were detected to be lower than that in ID patients, except that transcription levels of A20 were found to be higher than that in ID patients. Simultaneously, expressions of proinflammatory cytokines such as L-1β, IL-6 and TNF-α in KD patients were significantly elevated compared with those in ID patients. Furthermore, it was found that stimulation of lipopelysaccharide (LPS)remarkably up-regulated the expressions of negative-regulatory factors A20, IRF-4 and TRAF4 in KD patients or healthy volunteers. The Mrna levels of all the three factors in KD patients were found to be lower than that in the latter. In addition, transcription levels of IRF-4 and TRAF4 in KD patients with coronary artery lesion (KD-CAL+ ) were detected to be lower than those in KD patients without coronary artery lesion (KD-CAL-) during acute phase, while that of A20 in KD-CAL+ group were lower than that in the latter. And the levels of expressions of proinflammatory eytokines in KD-CAL+ group were found to be higher than those in KD-CAL- group ( P<0.01 ). These findings suggest that

  5. Advances in epidemiology, etiology and pathogenesis of Kawasaki disease%川崎病流行病学、病因和发病机制的新进展

    Institute of Scientific and Technical Information of China (English)

    杨波; 朱义杰(综述); 罗军; 张召艳(审校)

    2014-01-01

    川崎病(KD)是一种以急性全身血管炎为主要病理变化的疾病,好发于6个月至4岁的婴幼儿。KD主要累及冠状动脉,导致动脉瘤和血栓形成,引起严重的并发症甚至猝死。目前KD的病因和发病机制尚不完全清楚。文章就KD的流行病学、病因、发病机制及易感因素等新进展作一综述。%Kawasaki disease (KD) is an acute systemic vasculitis that primarily affects young children between 6 months and 4 years old. Coronary arteritis is an important clinical feature of KD because it is associated with aneurysms and thromboembolic events that can lead to severe complications, even sudden death. To date, the etiology and pathogenesis of Kawasaki disease has not been understood completely. In this paper, we will review the recent advances in epidemiology, etiology, pathogenesis and genetic susceptibility of Kawasa-ki disease.

  6. Clinical analysis of 25 cases with atypical Kawasaki disease%不典型川崎病25例临床分析

    Institute of Scientific and Technical Information of China (English)

    华旭丹

    2011-01-01

    目的 探讨临床不典型川崎病的诊断方法,提高对不典型川崎病的诊断水平,降低儿童后天性心脏病的发病率.方法 回顾性分析25例不典型川崎病的临床表现、实验室检查和预后情况等,并结合相关文献,探讨不典型川崎病的早期诊断和治疗方法.结果 25例川崎病患儿均符合不典型川崎病的诊断标准.结论 结合多方面临床资料对不典型川崎病诊断具有重要的早期诊断价值.%Objective To study the clinical diagnosis of 25 cases with atypical Kawasaki diseases and improve the diagnosis of atypical Kawasaki disease to reduce the morbidity of acquired heart disease in child.Methods Combined with the literature, retrospective analysis of 25 cases of atypical Kawasaki disease in clinical manifestation, laboratory examination and prognosis were performed to explore the early diagnosis and treatment to atypical Kawasaki disease between October 2002 and October 2010. Results Twenty-five cases of Kawasaki disease met the diagnostic criteria of atypical Kawasaki disease. Conclusion It is critical to perform a comprehensive analysis on all the clinical data obtained in determining atypical Kawasaki disease in early diagnosis.

  7. Tropospheric winds from northeastern China carry the etiologic agent of Kawasaki disease from its source to Japan.

    Science.gov (United States)

    Rodó, Xavier; Curcoll, Roger; Robinson, Marguerite; Ballester, Joan; Burns, Jane C; Cayan, Daniel R; Lipkin, W Ian; Williams, Brent L; Couto-Rodriguez, Mara; Nakamura, Yosikazu; Uehara, Ritei; Tanimoto, Hiroshi; Morguí, Josep-Anton

    2014-06-03

    Evidence indicates that the densely cultivated region of northeastern China acts as a source for the wind-borne agent of Kawasaki disease (KD). KD is an acute, coronary artery vasculitis of young children, and still a medical mystery after more than 40 y. We used residence times from simulations with the flexible particle dispersion model to pinpoint the source region for KD. Simulations were generated from locations spanning Japan from days with either high or low KD incidence. The postepidemic interval (1987-2010) and the extreme epidemics (1979, 1982, and 1986) pointed to the same source region. Results suggest a very short incubation period (<24 h) from exposure, thus making an infectious agent unlikely. Sampling campaigns over Japan during the KD season detected major differences in the microbiota of the tropospheric aerosols compared with ground aerosols, with the unexpected finding of the Candida species as the dominant fungus from aloft samples (54% of all fungal strains). These results, consistent with the Candida animal model for KD, provide support for the concept and feasibility of a windborne pathogen. A fungal toxin could be pursued as a possible etiologic agent of KD, consistent with an agricultural source, a short incubation time and synchronized outbreaks. Our study suggests that the causative agent of KD is a preformed toxin or environmental agent rather than an organism requiring replication. We propose a new paradigm whereby an idiosyncratic immune response, influenced by host genetics triggered by an environmental exposure carried on winds, results in the clinical syndrome known as acute KD.

  8. Noninvasive Evaluation of Myocardial Systolic Dysfunction in the Early Stage of Kawasaki Disease: A Speckle-Tracking Echocardiography Study.

    Science.gov (United States)

    Hematian, Mohammad-Nasir; Torabi, Shirin; MalaKan-Rad, Elaheh; Sayadpour-Zanjani, Keyhan; Ziaee, Vahid; Lotfi-Tolkaldany, Masoumeh

    2015-06-01

    Evaluation of myocardial function by speckle-tracking echocardiography is a new method for the early diagnosis of systolic dysfunction. We aimed to determine myocardial speckle-tracking echocardiography indices in Kawasaki Disease (KD) patients and compare them with the same indices in control subjects. Thirty-two patients (65.5% males) with KD and 19 control subjects with normal echocardiography participated in this study. After their demographic characteristics and clinical findings were recorded, all the participants underwent transthoracic echocardiography. Strain (S), Strain Rate (SR), Time to Peak Strain (TPS), and Strain Rate (TPSR), longitudinal velocity and view point velocity images in the two, three, and four-chamber views were semi-automatically obtained via speckle-tracking echocardiography. Among the patients, Twenty-four cases (75%) were younger than 4 years. Mean global S and SR was significantly reduced in the KD patients compared to controls (17.03 ± 1.28 vs. 20.22 ± 2.14% and 1.66 ± 0.16 vs. 1.97 ± 0.25 1/second, respectively), while there were no significant differences regarding mean TPS, TPSR, longitudinal velocity and view point velocity. Using repeated measure of analysis of variances, we observed that S and SR decreased from base to apical level in both groups. The change in the pattern of age adjusted mean S and SR across levels was significantly different between the groups (P < 0.001 for both parameters). We showed changes in S and SR assessed in KD patients versus control subjects in the acute phase of KD. However, we suggest that further studies be undertaken to compare S and SR in the acute phase and thereafter in KD patients.

  9. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association.

    Science.gov (United States)

    McCrindle, Brian W; Rowley, Anne H; Newburger, Jane W; Burns, Jane C; Bolger, Anne F; Gewitz, Michael; Baker, Annette L; Jackson, Mary Anne; Takahashi, Masato; Shah, Pinak B; Kobayashi, Tohru; Wu, Mei-Hwan; Saji, Tsutomu T; Pahl, Elfriede

    2017-04-25

    Kawasaki disease is an acute vasculitis of childhood that leads to coronary artery aneurysms in ≈25% of untreated cases. It has been reported worldwide and is the leading cause of acquired heart disease in children in developed countries. To revise the previous American Heart Association guidelines, a multidisciplinary writing group of experts was convened to review and appraise available evidence and practice-based opinion, as well as to provide updated recommendations for diagnosis, treatment of the acute illness, and long-term management. Although the cause remains unknown, discussion sections highlight new insights into the epidemiology, genetics, pathogenesis, pathology, natural history, and long-term outcomes. Prompt diagnosis is essential, and an updated algorithm defines supplemental information to be used to assist the diagnosis when classic clinical criteria are incomplete. Although intravenous immune globulin is the mainstay of initial treatment, the role for additional primary therapy in selected patients is discussed. Approximately 10% to 20% of patients do not respond to initial intravenous immune globulin, and recommendations for additional therapies are provided. Careful initial management of evolving coronary artery abnormalities is essential, necessitating an increased frequency of assessments and escalation of thromboprophylaxis. Risk stratification for long-term management is based primarily on maximal coronary artery luminal dimensions, normalized as Z scores, and is calibrated to both past and current involvement. Patients with aneurysms require life-long and uninterrupted cardiology follow-up. These recommendations provide updated and best evidence-based guidance to healthcare providers who diagnose and manage Kawasaki disease, but clinical decision making should be individualized to specific patient circumstances. © 2017 American Heart Association, Inc.

  10. 川崎病合并冠状动脉损害的遗传学基础%The genetic basis of coronary artery lesions in Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    时艳艳

    2010-01-01

    Kawasaki disease (KD) is an acute systemic vasculitis and its main life-threatening complications are coronary artery lesions(CAL), including coronary artery stenosis, thrombosis, aneurysm rupture, or sudden death. KD has now surpassed acute rheumatic fever as the leading cause of acquired heart disease in children. Therefore, it is important to identify as early as possible KD patients who are at risk for the development of CAL and intervent them timely. Genetic factors are thought to have important influences on the development and progress of Kawasaki disease and its CAL. In previous reports, several genetic polymorphisms, such as VEGF, MMPs and CD14 gene, were associated with the development of CAL. This review will introduce the study of gene polymorphisms in susceptibility to CAL in children with KD recently.%川崎病(KD)主要危及生命的并发症是冠状动脉损害(CAL),引起冠状动脉狭窄、血栓形成、动脉瘤破裂等,甚至猝死.KD现已成为儿童后天性心脏病的主要病因.因此,早期发现哪些KD患儿是发生CAL的易患人群,并予及时干预十分重要.遗传因素在KD及其CAL的发生发展中起重要作用,目前已发现多个基因的多态性与CAL相关.该文主要就近年来国内外关于KD合并CAL基因多态性的研究作一综述.

  11. Identification of 4-Trimethylaminobutyraldehyde Dehydrogenase (TMABA-DH as a Candidate Serum Autoantibody Target for Kawasaki Disease.

    Directory of Open Access Journals (Sweden)

    Atsuko Matsunaga

    Full Text Available Kawasaki disease (KD, an acute vasculitis that preferentially affects coronary arteries, is still the leading cause of acquired heart disease in children. Although the involvement of immune system malfunction in the onset of KD is suggested, its etiology still remains to be clarified. We investigated autoantibodies in KD patients, which are frequently found in sera from patients with autoimmune diseases, vasculitides and arteritides. We performed two-dimensional western blotting and LC-MS/MS to analyze the antigens of autoantibodies, detected two protein spots with 4 out of 24 sera from KD patients but not with 6 control sera, and identified the antigens as 4-trimethylaminobutyraldehyde dehydrogenase (TMABA-DH. A slot blot analysis with TMABA-DH as an antigen also revealed higher reactivities of patients' sera than control sera (positive rates: 18/43 vs 3/41. Using an enzyme-linked immunosorbent assay (ELISA, we found that the reactivity of anti-TMABA-DH antibodies in sera from KD patients was significantly higher than that in sera from age-matched controls. The optimal cut-off value of 0.043 had a sensitivity of 83.7% and a specificity of 80.0% in detecting KD patients (positive rates: 37/43 for KD patients, 9/41 for controls. Immunohistochemistry performed on thin sections of rat heart revealed that TMABA-DH colocalized with myosin light chains in cardiac myocytes. Patient sera with high reactivity gave similar immunostaining pattern. These results suggest that the detection of anti-TMABA-DH autoantibody could be a potential strategy for a diagnosis of KD.

  12. 川崎病临床诊治的新观念%New concepts for diagnosis and treatment of Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    谢利剑; 沈捷

    2014-01-01

    Kawasaki disease has been the major etiology of aquired heart problems in children. Mecha-nisms of Kawasaki disease are still unclear in the past years. Some Kawasaki disease patients were not responsive to intravenous immunoglobulin therapy. And corticosteroid therapy remains controversial in the treatment of Ka-wasaki disease. A few patients with Kawasaki disease presented severe systemic symptoms,and even died for it. It is difficult to evaluate the severity of coronary artery lesions and prognosis of Kawasaki disease. In this article, we reviewed some new concepts on diagnosis and treatments on Kawasaki disease.%川崎病导致的心血管并发症目前已经成为儿童获得性心脏病的主要病因。尽管川崎病的诊治研究已经历了数十年,其具体病因仍不完全明确。部分川崎病患者对丙种球蛋白治疗没有反应,激素的应用目前仍然存在争议。临床中一些患者可出现严重的全身症状,甚至死亡。冠状动脉病变严重程度与预后判断并非易事。该文对近年来川崎病诊治中提出的新观点重点阐述。

  13. Emergency coronary artery bypass grafting for cardiogenic shock due to left main coronary artery obstruction caused by Kawasaki disease in a 4-year-old boy.

    Science.gov (United States)

    Tamaki, Wataru; Tsuda, Etsuko; Nakajima, Hiroyuki; Kobayashi, Junjiro; Shiono, Junko

    2014-04-01

    We describe the case of a 4-year-old boy whose clinical course after Kawasaki disease resulted in coronary artery bypass grafting (CABG) due to acute myocardial infarction (AMI) causing cardiogenic shock. He had developed an ischemic cardiomyopathy due to severe localized stenosis of the left main coronary artery (LCA) and went into cardiogenic shock due to AMI on the day before a scheduled operation. He underwent successful emergency CABG within 4 h of MI. Postoperatively his neurological status was intact. This is the first report of a successful emergency CABG in a small child with cardiogenic shock due to LCA occlusion. CABG should be undertaken in small patients when appropriate indications exist, if bodyweight is >10 kg.

  14. To Investigate the Diagnosis and Treatment of Kawasaki Disease%川崎病的诊治探讨

    Institute of Scientific and Technical Information of China (English)

    郭振

    2015-01-01

    Objective A retrospective analysis of 25 cases of the treatment process Kawasaki disease, treatment of disease Kawasaki general pathological features and clinical effective. Methods 25 cases of Kawasaki disease patients as the research object in our hospital from 2008 January to 2011 October, analysis the clinical features of all patients, and the clinical outcomes of all patients were analyzed. Results Children with Kawasaki disease often showed dilatation of coronary artery wall thickening or rough, liver function damage and myocardial enzyme WBC, blood rising phenomenon. Conclusion Clinical medicine that EB virus infection and mycoplasma infection and Kawasaki disease had a certain relationship, and the experiment shows better clinical effect of single dose of a large number of gamma globulin for this disease, and can be used in clinical practice.%目的:回顾性分析25例川崎病的诊疗过程,研究川崎病的一般病理特点以及临床上有效的治疗方式。方法选取我院2008年1月~2011年10月收治的25例川崎病患者作为研究对象,分析所有患者的临床表现特点,并对所有患者的临床治疗效果进行总结分析。结果川崎病患儿常表现有冠状动脉扩张或管壁增厚毛糙、血 WBC、肝功能损害及心肌酶升高等现象。结论临床医学上认为 EB 病毒感染以及支原体感染都和川崎病具有一定的联系,并且实验中显示单剂大量丙种球蛋白对该疾病的临床疗效较好。

  15. Classification of coronary artery tissues using optical coherence tomography imaging in Kawasaki disease

    Science.gov (United States)

    Abdolmanafi, Atefeh; Prasad, Arpan Suravi; Duong, Luc; Dahdah, Nagib

    2016-03-01

    Intravascular imaging modalities, such as Optical Coherence Tomography (OCT) allow nowadays improving diagnosis, treatment, follow-up, and even prevention of coronary artery disease in the adult. OCT has been recently used in children following Kawasaki disease (KD), the most prevalent acquired coronary artery disease during childhood with devastating complications. The assessment of coronary artery layers with OCT and early detection of coronary sequelae secondary to KD is a promising tool for preventing myocardial infarction in this population. More importantly, OCT is promising for tissue quantification of the inner vessel wall, including neo intima luminal myofibroblast proliferation, calcification, and fibrous scar deposits. The goal of this study is to classify the coronary artery layers of OCT imaging obtained from a series of KD patients. Our approach is focused on developing a robust Random Forest classifier built on the idea of randomly selecting a subset of features at each node and based on second- and higher-order statistical texture analysis which estimates the gray-level spatial distribution of images by specifying the local features of each pixel and extracting the statistics from their distribution. The average classification accuracy for intima and media are 76.36% and 73.72% respectively. Random forest classifier with texture analysis promises for classification of coronary artery tissue.

  16. A simple method for assessment of human anti-Neu5Gc antibodies applied to Kawasaki disease.

    Directory of Open Access Journals (Sweden)

    Vered Padler-Karavani

    Full Text Available N-glycolylneuraminic acid (Neu5Gc is an immunogenic sugar of dietary origin that metabolically incorporates into diverse native glycoconjugates in humans. Anti-Neu5Gc antibodies are detected in all human sera, though with variable levels and epitope-recognition profiles. These antibodies likely play a role in several inflammation-mediated pathologies including cardiovascular diseases and cancer. In cancer, they have dualistic and opposing roles, either stimulating or repressing disease, as a function of their dose, and some of these antibodies serve as carcinoma biomarkers. Thus, anti-Neu5Gc antibodies may signify risk of inflammation-mediated diseases, and changes in their levels could potentially be used to monitor disease progression and/or response to therapy. Currently, it is difficult to determine levels of anti-Neu5Gc antibodies in individual human samples because these antibodies recognize multiple Neu5Gc-epitopes. Here we describe a simple and specific method for detection and overall estimation of human anti-Neu5Gc antibodies. We exploit the difference between two mouse models that differ only by Neu5Gc-presence (wild-type or Neu5Gc-absence (Cmah(-/- knockout. We characterize mouse serum from both strains by HPLC, lectin and mass-spectrometry analysis and show the target Neu5Gc-epitopes. We then use Cmah(-/- knockout sera to inhibit all non-Neu5Gc-reactivity followed by binding to wild-type sera to detect overall anti-Neu5Gc response in a single assay. We applied this methodology to characterize and quantify anti-Neu5Gc IgG and IgA in sera of patients with Kawasaki disease (KD at various stages compared to controls. KD is an acute childhood febrile disease characterized by inflammation of coronary arteries that untreated may lead to coronary artery aneurysms with risk of thrombosis and myocardial infarction. This estimated response is comparable to the average of detailed anti-Neu5Gc IgG profile analyzed by a sialoglycan microarray

  17. Kawasakis syndrom hos voksne

    DEFF Research Database (Denmark)

    Roed, Casper; Skinhøj, Peter

    2009-01-01

    Kawasaki syndrome (KS), which is febrile systemic vasculitis complicated by coronary aneurysms, was initially described in children, but an increasing number of case-reports address adult-KS. The disease seems to be infectious of origin, but the causative agent has not been established. The debut...

  18. Identification of novel susceptibility Loci for kawasaki disease in a Han chinese population by a genome-wide association study.

    Directory of Open Access Journals (Sweden)

    Fuu-Jen Tsai

    Full Text Available Kawasaki disease (KD is an acute systemic vasculitis syndrome that primarily affects infants and young children. Its etiology is unknown; however, epidemiological findings suggest that genetic predisposition underlies disease susceptibility. Taiwan has the third-highest incidence of KD in the world, after Japan and Korea. To investigate novel mechanisms that might predispose individuals to KD, we conducted a genome-wide association study (GWAS in 250 KD patients and 446 controls in a Han Chinese population residing in Taiwan, and further validated our findings in an independent Han Chinese cohort of 208 cases and 366 controls. The most strongly associated single-nucleotide polymorphisms (SNPs detected in the joint analysis corresponded to three novel loci. Among these KD-associated SNPs three were close to the COPB2 (coatomer protein complex beta-2 subunit gene: rs1873668 (p = 9.52×10⁻⁵, rs4243399 (p = 9.93×10⁻⁵, and rs16849083 (p = 9.93×10⁻⁵. We also identified a SNP in the intronic region of the ERAP1 (endoplasmic reticulum amino peptidase 1 gene (rs149481, p(best = 4.61×10⁻⁵. Six SNPs (rs17113284, rs8005468, rs10129255, rs2007467, rs10150241, and rs12590667 clustered in an area containing immunoglobulin heavy chain variable regions genes, with p(best-values between 2.08×10⁻⁵ and 8.93×10⁻⁶, were also identified. This is the first KD GWAS performed in a Han Chinese population. The novel KD candidates we identified have been implicated in T cell receptor signaling, regulation of proinflammatory cytokines, as well as antibody-mediated immune responses. These findings may lead to a better understanding of the underlying molecular pathogenesis of KD.

  19. IL-1β is Crucial for Induction of Coronary Artery Inflammation in a Mouse Model of Kawasaki Disease

    Science.gov (United States)

    Lee, Young Ho; Schulte, Danica J.; Shimada, Kenichi; Chen, Shuang; Crother, Timothy R.; Chiba, Norika; Fishbein, Michael C.; Lehman, Thomas J.A.; Arditi, Moshe

    2012-01-01

    Background Kawasaki disease (KD) is the most common cause of acute vasculitis and acquired cardiac disease in US children. Untreated, children may develop coronary artery aneurysms, myocardial infarction and sudden death as a result of the illness. Up to a third of KD patients fail to respond to intravenous gammaglobulin (IVIG), the standard therapy, and alternative treatments are being investigated. Genetic studies have indicated a possible role for IL-1β in KD. We therefore explored the role of IL-1β in a murine model of KD. Methods and Results Using an established mouse model of KD that involves injection of Lactobacillus casei cell wall extract (LCWE), we investigated the role of IL- 1β and caspase-1 (activated by the inflammasome and required for IL-1β maturation) in coronary arteritis, and evaluated the efficacy of IL-1 receptor antagonist (IL-1Ra) as a potential treatment. LCWE-induced IL-1β maturation and secretion was dependent on the NLRP3 inflammasome in macrophages. Both caspase1-deficient and IL-1R-deficient mice were protected from LCWE-induced coronary lesions. Injection of recombinant IL-1β to caspase-1-deficient mice restored the ability of LCWE to cause coronary lesions in response to LCWE. Furthermore, daily injections of the IL-1Ra prevented LCWE-mediated coronary lesions, up to three days after LCWE injection. Conclusions Our results strongly suggest that caspase-1 and IL-1β play critical roles in the development of coronary lesions in this KD mouse model, blocked by IL-1Ra. Therefore, anti-IL-1β treatment strategies may constitute an effective, more targeted treatment of KD to prevent coronary lesions. PMID:22361326

  20. Association of the Resistin Gene Promoter Region Polymorphism with Kawasaki Disease in Chinese Children

    Directory of Open Access Journals (Sweden)

    Ruixi Liu

    2012-01-01

    Full Text Available Objectives. The −420C>G polymorphism located in the resistin gene (RETN promoter has recently been suggested to play a potential role in proinflammatory conditions and cardiovascular disease. This study investigated the association of the RETN promoter polymorphism with Kawasaki disease (KD and its clinical parameters in Chinese children. Methods. We compared patients with complete KD to incomplete KD children. Genotyping of the RETN promoter polymorphism was performed using MassARRAY system, and serum resistin levels were estimated using the sandwich enzyme immunoassay method. Results. There was no significant difference in RETN (−420C>G genotypes between KD and control groups. However, the frequency of the G allele was higher in iKD patients than in cKD children due to a significantly increased frequency of the GG genotypes. Serum levels of resistin were significantly higher in KD patients than in controls regardless of the presence of coronary artery lesions (CALs. Conclusion. The present findings suggest that while resistin may play a role in the pathogenesis of KD, there is no apparent association between CAL and the RETN (−420C>G gene polymorphism in KD children. However, the diagnosis of iKD is challenging but can be supported by the presence of the G allele and the GG genotypes.

  1. Adolescent Kawasaki disease: usefulness of 64-slice CT coronary angiography for follow-up investigation

    Energy Technology Data Exchange (ETDEWEB)

    Carbone, Iacopo; Cannata, David; Algeri, Emanuela; Galea, Nicola; Napoli, Alessandro; Catalano, Carlo; Passariello, Roberto; Francone, Marco [Sapienza University of Rome, Department of Radiological, Onchological and Anatomopathological Sciences, Policlinico Umberto I, Rome (Italy); De Zorzi, Andrea [Bambino Gesu Hospital, Cardiology Division, Rome (Italy); Bosco, Giovanna; D' Agostino, Rita [Sapienza University of Rome, Unit of Paediatric Cardiology, Policlinico Umberto I, Rome (Italy); Menezes, Leon [University College of London, Institute of Nuclear Medicine, London (United Kingdom)

    2011-09-15

    Kawasaki disease (KD) is a systemic vasculitis that mainly affects coronary arteries in children, and requires regular follow-up from the time of diagnosis. To evaluate the feasibility of 64-slice CT angiography (CTA) for follow-up of patients with KD using previously performed invasive catheter coronary angiography (CCA) as reference standard. The study group comprised 12 patients (age 17.6 {+-} 2.9 years, mean{+-}SD) with a diagnosis of KD and a previously performed CCA (interval, 32.6 {+-} 13.5 months) who underwent 64-slice cardiac CTA. The quality of the images for establishing the presence of coronary abnormalities was determined by two observers. The CTA findings were compared with those from the prior CCA. Adequate image quality was obtained in all patients. Mean effective dose for CTA was 6.56 {+-} 0.95 mSv. CTA allowed accurate identification, characterization and measurement of all coronary aneurysms (n = 32), stenoses (n = 3) and occlusions (n = 9) previously demonstrated by CCA. One patient with disease progression went on to have percutaneous coronary intervention. Coronary lesions were reliably evaluated by 64-slice CTA in the follow-up of compliant patients with KD, reducing the need for repeated diagnostic invasive CCA. Hence, in an adequately selected patient population, the role of CCA could be limited almost only to therapeutic procedures. (orig.)

  2. Decreased nitric oxide production after intravenous immunoglobulin treatment in patients with Kawasaki disease.

    Science.gov (United States)

    Wang, Chih-Lu; Wu, Yu-Tsun; Lee, Chia-Jung; Liu, Hsiu-Chin; Huang, Li-Tung; Yang, Kuender D

    2002-10-01

    To assess nitric oxide (NO) production and different displays of NO synthase (NOS) isoforms in patients with Kawasaki disease (KD) before and after intravenous immunoglobulin (IVIG) treatment. Blood and urine samples were collected from patients with KD before IVIG treatment and 3 days afterward. Age-matched febrile patients with various diseases served as control patients. Plasma NOx (NO3- + NO2-) levels were measured as NO production. Real-time reverse transcriptase-polymerase chain reaction and immunohistochemical staining were used to detect differential NOS isoform expression in mononuclear leukocytes (MNCs). Patients with KD (n = 40) had higher levels of NOx than the non-KD febrile control patients. The elevated NOx levels in patients with KD were significantly associated with the occurrence of coronary artery dilation (>3 mm). These elevated NOx levels significantly decreased after IVIG treatment (157.8 +/- 15.9 micromol/L vs 57.0 +/- 4.27 micromol/L, P urinary NOx levels was not changed after IVIG treatment. Inducible but not constitutive NOS mRNA and protein in MNCs were prominently expressed but significantly decreased after IVIG treatment. IVIG may suppress iNOS expression of MNCs in patients with KD, thus decreasing NO-mediated inflammatory responses and coronary artery dilation.

  3. 小儿川崎病22例临床分析%Clinical Analysis of 22 Cases of Children with Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    汪萍; 雷勇

    2015-01-01

    目的总结小儿川崎病的临床特点。方法回顾性分析我院儿科2010年1月~2014年12月住院确诊的22例川崎病患儿的临床资料。结果22例川崎病患儿,完全型川崎病20例,不完全型川崎病2例,IVIG无反应型川崎病1例,发生冠状动脉损害6例。主要临床表现发生率依次为发热、结膜充血、口唇及口腔黏膜改变、颈部淋巴结肿大等,疾病早期ESR和CRP明显增高,50%以上的患儿病程中合并肝功能异常、心电图异常和贫血,早期规范合理静脉使用IVIG可有效减少和预防冠状动脉损害。结论小儿川崎病临床表现多样,易合并冠状动脉损害,临床上应加强对不完全型川崎病和IVIG无反应型川崎病的早期诊断。%Objective To investigate the clinical features of Kawasaki disease in children.Methods Retrospective analysis of pediatric between January 2010 and December 2010 in the hospital diagnosis of the clinical data of 22 cases of children with kawasaki disease.Results In 22 cases of Kawasaki disease,20 cases were complete Kawasaki disease,2 cases were incomplete Kawasaki disease,1 case was intravenous immunoglobulin unresponsive Kawasaki Disease,and 6 cases were identified with coronary artery damage.The main clinical manifestations were fever,conjunctival congestion,lips and oral mucosa changes,cervical lymphadenopathy.In the early stage,ESR and CRP increased significantly,50%of the children have hepatic dysfunction,abnormal electrocardiogram and anemia.Early and timely standardized vein using IVIG can ef ectively reduce and prevent the coronary artery damage.Conclusion Kawasaki disease in children has many dif erent and diverse manifestations,and is easily complicated with coronary artery damages.Much more at ention should be paid to diagnose the incomplete Kawasaki disease and Intravenous immunoglobulin unresponsive Kawasaki Disease.

  4. Evidence of correlation between TGFBR2 gene expression mediated by NF-kB signaling pathways and Kawasaki disease in children.

    Science.gov (United States)

    Gao, Qinling; Yuan, Shuhua; Yuan, Dawei

    2017-09-15

    We explored the correlation between the TGFBR2 gene that is mediated by NF-kb signaling pathways and the pathogenesis of Kawasaki disease in children. In this study, 43 children with Kawasaki disease from April 2014 to January 2016 at our hospital were selected as the observation group, and 42 healthy children were selected as the control group. The mRNA expression levels of NF-kb gene and TGFBR2 gene in different groups were detected using fluorescence quantitative PCR. The protein expression levels of the NF-kb and TGFBR2 were detected using enzyme-linked immunosorbent assay (ELISA) in different groups. The expression levels of NF-kb and TGFBR2 in the observation group and the control group were detected using immunohistochemistry. Compared to the control group, the mRNA expression levels of NF-kb and TGFBR2 were 12.3 times and 27.5 times as high as those in the control group respectively and there were significant differences between the two groups (pKawasaki disease were significantly higher than those in healthy subjects (pKawasaki disease than that in healthy children (4.5%); there was significant difference between the two groups (pKawasaki disease in children through NF-kB signaling pathways.

  5. Assessment Of Coronary Artery Aneurysms Using Transluminal Attenuation Gradient And Computational Modeling In Kawasaki Disease Patients

    Science.gov (United States)

    Grande Gutierrez, Noelia; Kahn, Andrew; Shirinsky, Olga; Gagarina, Nina; Lyskina, Galina; Fukazawa, Ryuji; Owaga, Shunichi; Burns, Jane; Marsden, Alison

    2015-11-01

    Kawasaki Disease (KD) can result in coronary artery aneurysms (CAA) in up to 25% of patients, putting them at risk of thrombus formation, myocardial infarction and sudden death. Clinical guidelines recommend CAA diameter >8 mm as the arbitrary criterion for initiating systemic anticoagulation. KD patient specific modeling and flow simulations suggest that hemodynamic data can predict regions at increased risk of thrombosis. Transluminal Attenuation Gradient (TAG) is determined from the change in radiological attenuation per vessel length and has been proposed as a non-invasive method for characterizing coronary stenosis from CT Angiography. We hypothesized that CAA abnormal flow could be quantified using TAG. We computed hemodynamics for patient specific coronary models using a stabilized finite element method, coupled numerically to a lumped parameter network to model the heart and vascular boundary conditions. TAG was quantified in the major coronary arteries. We compared TAG for aneurysmal and normal arteries and we analyzed TAG correlation with hemodynamic and geometrical parameters. Our results suggest that TAG may provide hemodynamic data not available from anatomy alone. TAG represents a possible extension to standard CTA that could help to better evaluate the risk of thrombus formation in KD.

  6. Transluminal Attenuation Gradient for Thrombotic Risk Assessment in Kawasaki Disease Patients with Coronary Artery Aneurysms

    Science.gov (United States)

    Grande Gutierrez, Noelia; Kahn, Andrew; Burns, Jane; Marsden, Alison

    2014-11-01

    Kawasaki Disease (KD) can result in coronary aneurysms in up to 25% of patients if not treated early putting patients at risk of thrombus formation, myocardial infarction and sudden death. Clinical guidelines for administering anti-coagulation therapy currently rely on anatomy alone. Previous studies including patient specific modeling and computer simulations in KD patients have suggested that hemodynamic data can predict regions susceptible to thrombus formation. In particular, high Particle Residence Time gradient (PRTg) regions have shown to correlate with regions of thrombus formation. Transluminal Attenuation Gradient (TAG) is determined from the change in radiological attenuation per vessel length. TAG has been used for characterizing coronary artery stenoses, however this approach has not yet been used in aneurysmal vessels. The aim of this study is to analyze the correlation between TAG and PRTg in KD patients with aneurysms and evaluate the use of TAG as an index to quantify thrombotic risk. Patient specific anatomic models for fluids simulations were constructed from CT angiographic image data from 3 KD aneurysm patients and one normal control. TAG values for the aneurysm patients were markedly lower than for the non-aneurysmal patient (mean -18.38 vs. -2). In addition, TAG values were compared to PRTg obtained for each patient. Thrombotic risk stratification for KD aneurysms may be improved by incorporating TAG and should be evaluated in future prospective studies.

  7. Coronary magnetic resonance angiography and vessel wall imaging in children with Kawasaki disease

    Energy Technology Data Exchange (ETDEWEB)

    Greil, Gerald F.; Hofbeck, Michael; Sieverding, Ludger [University of Tuebingen, Department of Pediatric Cardiology, Children' s Hospital, Tuebingen (Germany); Seeger, Achim; Miller, Stephan; Claussen, Claus D. [University of Tuebingen, Department of Diagnostic Radiology, Tuebingen (Germany); Botnar, Rene M. [Technical University Munich, Department of Nuclear Medicine, Cardiovascular Division, Munich (Germany)

    2007-07-15

    In patients with Kawasaki disease (KD) serial evaluation of the distribution and size of coronary artery aneurysms (CAA) is necessary for risk stratification and therapeutic management. To apply whole-heart coronary MR angiography (CMRA) and black-blood coronary vessel wall imaging in children with KD. Six children (mean age 4.6 years, range 2.5-7.8 years) with KD underwent CMRA using a free-breathing, T2-prepared, three-dimensional steady-state free-precession (3D-SSFP), whole-heart approach with navigator gating and tracking. Vessel walls were imaged with an ECG-triggered and navigator-gated double inversion recovery (DIR) black-blood segmented turbo spin-echo sequence. There was complete agreement between CMRA and conventional angiography (n=6) in the detection of CAA (n=15). Excellent agreement was found between the two techniques in determining the maximal diameter (mean difference 0.2{+-}0.7 mm), length (mean difference 0.1{+-}0.8 mm) and distance from the ostium (mean difference -0.8{+-}2.1 mm) of the CAAs. In all subjects with a CAA, abnormally thickened vessel walls were found (2.5{+-}0.5 mm). CMRA accurately defines CAA in free-breathing sedated children with KD using the whole-heart approach and detects abnormally thickened vessel walls. This technique may reduce the need for serial X-ray coronary angiography, and improve risk stratification and monitoring of therapy. (orig.)

  8. Dobutamine stress radionuclide ventriculography reveals silent myocardial dysfunction in Kawasaki disease

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    Hamamichi, Yuji; Ichida, Fukiko; Tsubata, Shinichi [Toyama Medical and Pharmaceutical Univ., Sugitani (Japan). Faculty of Medicine] (and others)

    2002-01-01

    Dobutamine (DOB) stress radionuclide ventriculography (RVG) is proposed for evaluating left ventricular performance in patients with Kawasaki disease (KD). Dobutamine stress RVG, up to 15{mu}g{center_dot}kg{sup -1}{center_dot}min{sup -1}, was performed in 40 patients with a history of KD, some of whom had a perfusion defect (PD group) on dipyridamole stress thallium-201 myocardial imaging, some of whom had no perfusion defects (NPD group), and some of whom had no coronary artery lesions (C group). No significant differences in either systolic or diastolic indices of the left ventricle at rest were observed between the 3 groups. Although hemodynamic responses were similar in all patients after DOB stress, early diastolic index of the first third filling fraction decreased only in the PD group and was significantly lower in this group compared with the C group (p<0.01). The asynchrony index increased significantly in those patients with coronary stenosis after DOB stress (p<0.05). No serious side-effects were observed during the study. Even late after onset, patients with myocardial ischemia as a result of KD still had impaired early diastolic filling and asynchronous relaxation of the left ventricle. As an alternative to exercise testing, DOB stress RVG is a safe and promising means for serially evaluating left ventricular performance in patients with KD. (author)

  9. Endothelial progenitor cell transplantation ameliorates elastin breakdown in a Kawasaki disease mouse model

    Institute of Scientific and Technical Information of China (English)

    CHEN Zhi; DU Zhong-dong; LIU Jun-feng; LU Dun-xiang; LI Li; GUAN Yun-qian; WAN Sui-gui

    2012-01-01

    Background Coronary artery damage from Kawasaki disease (KD) is closely linked to the dysfunction of endothelial progenitor cells (EPCs).The aim of the present study was to evaluate the therapeutic effect of EPCs transplantation in KD model.Methods Lactobacillus casei cell wall extract (LCWE)-induced KD model in C57BL/6 mice was established.The model mice were injected intravenously with bone marrow-derived in vitro expanded EPCs.Histological evaluation,number of circulating EPCs and the function of bone marrow EPCs were examined at day 56.Results Inflammation was found around the coronary artery of the model mice after 14 days,Elastin breakdown was observed after 56 days.CM-Dil labeled EPCs incorporated into vessel repairing foci was found.At day 56,the number of peripheral EPCs in the KD model group was lower than in EPCs transplanted and control group.The functional index of bone marrow EPCs from the KD model group decreased in proliferation,adhesion and migration.Increased number of circulating EPCs and improved function were observed on the EPCs transplanted group compared with model group.Conclusion Exogenously administered EPCs,which represent a novel strategy could prevent the dysfunction of EPCs,accelerate the repair of coronary artery endothelium lesion and decrease the occurrence of aneurysm.

  10. ITPKC single nucleotide polymorphism associated with the Kawasaki disease in a Taiwanese population.

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    Ho-Chang Kuo

    Full Text Available Kawasaki disease (KD is characterized by systemic vasculitis with unknown etiology. Previous studies from Japan indicated that a gene polymorphism of ITPKC (rs28493229 is responsible for susceptibility to KD. We collected DNA samples from 1,531 Taiwanese subjects (341 KD patients and 1,190 controls for genotyping ITPKC. In this study, no significant association was noted for the ITPKC polymorphism (rs28493229 between the controls and KD patients, although the CC genotype was overrepresented. We further combined our data with previously published case/control KD studies in the Taiwanese population and performed a meta-analysis. A significant association between rs28493229 and KD was found (Odds Ratio:1.36, 95% Confidence Interval 1.12-1.66. Importantly, a significant association was obtained between rs28493229 and KD patients with aneurysm formation (P = 0.001, under the recessive model. Taken together, our results indicated that C-allele of ITPKC SNP rs28493229 is associated with the susceptibility and aneurysm formation in KD patients in a Taiwanese population.

  11. IL-31 associated with coronary artery lesion formation in Kawasaki disease.

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    Wan-Ning Tseng

    Full Text Available Kawasaki disease (KD is known to be associated with T help (Th 2 reaction and subsequently allergic diseases. Interleukin-31 (IL-31 has also been reported to be involved in Th2 mediated diseases such as allergic diseases. However, the role of IL-31 in KD has not been previously reported. The aim of this study is to investigate whether IL-31 is associated with KD and its clinical outcome.A total of 78 KD patients who met the criteria of KD were enrolled in this study as well as 20 age-matched controls. Plasma samples were conducted to measure IL-31 before intravenous immunoglobulin (IVIG treatment (KD1, within 3 days after IVIG treatment (KD2 and at least 3 weeks after IVIG treatment (KD3 by utilizing enzyme-linked immunosorbent assay (ELISA.Our findings showed that IL-31 expression was higher in KD patients after IVIG treatment significantly (KD2>KD1: 1265.0±199.3 vs. 840.2±152.5 pg/ml, p<0.0001. Further analysis revealed that IL-31 level was significantly higher in KD patients with coronary artery lesion (CAL (656.6±139.5 vs. 1373.0±422.0 pg/ml, p = 0.04 before IVIG treatment (KD1. There were no significant differences between the IVIG resistance and IVIG responsiveness groups.IL-31 was increased after IVIG treatment in patients with KD and was significantly associated with CAL formation. The results from this study may help to identify a novel risk factor for predicting KD and CAL formation.

  12. 川崎病发病机制研究进展%New Progress of the Research on Pathogenesis of Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    袁静泊; 黄先玫

    2012-01-01

    川崎病的发病机制一直没有完全清楚.但最近由于分子生物学的进步和人类基因组项目的 完成,使得川崎病的发病机制有了重大的突破.本文就川崎病发病机制的研究进展做一综述.%The pathogenesis of Kawasaki disease has not been understood clearly. But recently, with the progress of molecular biology and completion of the human genome project, there is a major breakthrough in the understanding of pathogenesis of Kawasaki disease. In this paper, we will summarize the progress of the research on the pathogenesis of Kawasaki disease.

  13. Perifosine inhibits lipopolysaccharide (LPS)-induced tumor necrosis factor (TNF)-α production via regulation multiple signaling pathways: new implication for Kawasaki disease (KD) treatment.

    Science.gov (United States)

    Shen, Jie; Liang, Li; Wang, Chunlin

    2013-07-26

    Kawasaki disease (KD) is a multisystem vasculitis of unknown etiology, with coronary artery aneurysms occurring in majority of untreated cases. Tumor necrosis factor (TNF)-α is the pleiotropic inflammatory cytokine elevated during the acute phase of KD, which induces damage to vascular endothelial cells to cause systemic vasculitis. We here investigated the potential role of perifosine, a novel Akt inhibitor, on TNFα expression in LPS-stimulated macrophages and in ex-vivo cultured peripheral blood mononuclear cells (PBMCs) of acute KD patients. Here, we found that perifosine inhibited LPS-induced TNFα expression and production in mouse macrophages (RAW 264.7 cells and bone marrow-derived macrophages (BMDMs)). Meanwhile, perifosine administration down-regulated TNFα production in PBMCs isolated from acute KD patients. For the mechanism study, we found that perifosine significantly inhibited Akt and ERK/mitogen-activated protein kinases (MAPK) signaling, while activating AMP-activated protein kinase (AMPK) signaling in both patients' PBMCs and LPS-stimulated macrophages. Interestingly, although perifosine is generally known as an Akt inhibitor, our data suggested that ERK inhibition and AMPK activation, but not Akt inactivation were possibly involved in perifosine-mediated inhibition against TNFα production in monocytes. In conclusion, our data suggested that perifosine significantly inhibited TNFα production via regulation multiple signaling pathways. The results of this study should have significant translational relevance in managing this devastating disease.

  14. 超声斑点追踪技术评价川崎病急性期患者左心室局部收缩功能%Evaluation of left ventricnlar systolic function in children at acute stage of Kawasaki disease using speckie-iracing imaging

    Institute of Scientific and Technical Information of China (English)

    杨霞; 黎春雷; 伍玉晗; 宋家琳; 童春; 徐鑫; 邓又斌

    2009-01-01

    目的 应用超声斑点追踪技术(speckle-tracking imaging,STI)研究川崎病急性期患者左室收缩功能.方法 记录27例川崎病急性期患者(8例冠状动脉扩张)与19例正常儿童的左室心尖位长轴观、二腔观及四腔观,左室二尖瓣环水平、乳头肌水平、心尖水平短轴观的二维灰阶图像,分别测量左室收缩期峰值纵向应变(longitudinal strain,SI)、径向应变(radial strain,SR)与圆周应变(circumferentialstrain,SC).结果 ①川崎病急性期患儿左室大部分节段收缩期峰值SL与SR均低于正常组(P0.05).③川崎病急性期患儿中8例冠状动脉扩张者(含7支冠状动脉瘤样扩张)左室收缩期峰值SL、SR均低于19例冠状动脉未扩张者,但大部分节段差异无统计学意义(P>0.05).结论 急性期川崎病患儿无论有无冠状动脉扩张左室长轴和短轴收缩功能均降低.%Objective To access the left ventricular sysytolic function in children at acute stage of Kawasaki disease using speckle-tracing imaging. Methods Two-dimensional echocardiograghic images of 27 patients and 19 normals were collected in apical long-axis view, two-champer view, four-champer view, short-axis views at the levels of mitral annulus, papillary muscle and apex. The systolic peak values of longitudinal strain, radial strain and circumferential strain were measured by speckle-tracking imaging. Results Compared with controls the values of longitudinal strain and radial strain are lower in most segments in patients(P 0.05). Longitudinal strain and radial strain measured were lower in 8 patients with coronary ectasia(including 7 cass with coronary artery aneurysms in vessels) than other 19 patients,but the two groups have no significant differences(P>0.05). Conclusions Long-axis and short-axis function of the heart decreased regardless of coronary artery ectasia.

  15. Medication Nursing for Children with Kawasaki Disease%川崎病患儿的用药护理

    Institute of Scientific and Technical Information of China (English)

    马新华

    2013-01-01

    Objective To summarize the medication nursing methods of Kawasaki disease. Methods Data of 57 children with Kawasaki disease in our hospital were analyzed and summarized from September 2009 to June 2012. Results The main drug used in the clinical were aspirin, human immunoglobulin, cephalo-sporins and glucocorticoid. Rashes and gastrointestinal tract dysfunction were the main side effects. Of the 57 children with Kawasaki disease, 6 were cured, 50 were improved, and only 1 patient discharged from hospital without cure. Conclusion Being an important process, professional medication nursing were indispensable for preventing the recurrence of the disease and promoting rehabilitation of the children.%目的 对川崎病患儿用药护理工作进行总结.方法 对2009年9月至2012年6月收治的57例川崎病患儿的病例资料进行分析,总结用药护理方法.结果 川崎病治疗的主要用药有阿司匹林、人免疫球蛋白、头孢类抗生素和糖皮质激素,不良反应主要有皮疹及胃肠道功能紊乱.57例患儿中,治愈6例,好转50例,未愈要求离院1例.结论 专业的用药护理对促进川崎病患儿恢复健康及防止病情复发至关重要,也是确保护理安全的重要环节.

  16. Review of Kawasaki Patients in Yazd

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    P Roozbeh

    2009-01-01

    Full Text Available Introduction: Kawasaki syndrome is an acute febrile vasculitis with multiorgan involvment in children. The objective of this study was assessment of demographic, clinical and laboratory data of Kawasaki patients in Yazd. Methods: In a descriptive-analytic study, the hospital records of patients treated for Kawasaki disease in all hospitals with pediatric wards between March 1996 and March 2006 at Yazd were reviewed and demographic, clinical and laboratory data recorded in a questionnaire and analyzed by SPSS, version 13 . Chi-square test was used to assess statistical differences. Results: 60.4% of patients were male and male /female ratio was 1.52. Seven percent of them were less than 5 years old. Duration of fever before treatment was in range of 1-20 days with mean of 9.1±5.07 days. Fever less than 5 days, 6-10 days, 11-15 days and more than 15 days was seen in 45.8%, 25 %, 22.9% and 6.2%, respectively. The most common symptoms were non purulent conjunctivitis (83.3%, strawberry tongue (79% ,dry-cracked lips(77%, skin rash(66.6% and cervical lymphadenopathy(47.9%. Leukocytosis, anemia and thrombocytosis were seen in 64%, 80% and 45%, respectively. Cardiac involvement was seen in 39.5% of the patients. Conclusion: The Kawasaki disease is predominantly seen in children less than 5 years old and most common symptoms include non purulent conjunctivitis ,strawberry tongue, dry-cracked lips, skin rash and cervical lymphadenopathy .

  17. Analysis of the risk factors for coronary artery lesion(CAL)secondary to Kawasaki disease children%川崎病并发冠状动脉病变高危因素分析

    Institute of Scientific and Technical Information of China (English)

    郑礼林; 潘家华

    2012-01-01

    Objective To analyze the risk factors for CAL secondary to Kawasaki disease children.Methods The clinical data of 60 patients with Kawasaki disease were retrospectively analyzed.Results There were 27 CAL secondary to Kawasaki disease in 60 Kawasaki disease patients.9 patients were age ≤ 1 year.16 CAL secondary to Kawasaki disease were male.11 CAL secondary to Kawasaki disease were female.6 CAL secondary to Kawasaki disease were WBC>20 × 109/L、21 CAL secondary to Kawasaki disease were WBC ≤20 × 109/L;17 CAL secondary to Kawasaki disease were PLT>300 x 109/L、10 CAL secondary to Kawasaki disease were PLT≤300 x 109/L;9 CAL secondary to Kawasaki disease were ESR>100mm/1h、15 CAL secondary to Kawasaki disease were ESR ≤ 100mm/1 h;5 CAL secondary to Kawasaki disease were fever duration>10d using IVIG、22 CAL secondary to Kawasaki disease were fever duration ≤ 10d using IVIG.There were no significant differences(P>0.05),9 CAL secondary to Kawasaki disease were CRP>100mg/L、18 CAL secondary to Kawasaki disease were CRP≤ 100mg/L,showed significant difference(P<0.01).Conclusion Kawasaki disease patients had CAL secondary to Kawasaki disease has no difference between male and female.The high risk factors for CAL secondary to Kawasaki disease are age ≤ 1 year,CRP>100mg/L.%目的 分析川崎病并发冠状动脉病变的高危因素.方法 回顾性分析60例川崎病患儿的临床资料.结果 60例川崎病患儿中27例并发冠状动脉病变,其中1岁以内9例并发冠状动脉病变;男16例、女11例并发冠状动脉病变,WBC>20×109/L 6例、WBC≤20×109/L 21例并发冠状动脉病变;PLT>300×109/L 17例、PLT≤300×109/L 10例并发冠状动脉病变;ESR>100 mm/1 h 9例、ESR≤100 mm/1 h 15例并发冠状动脉病变;发热天数>10 d使用IVIG 5例、发热天数≤10 d使用IVIG 22例并发冠状动脉病变;CRP>100 mg/L 9例、CRP≤100 mg/L 18例并发冠状动脉病变.结论 川崎病并发冠状动脉

  18. Significance of serum 25-hydroxyvitamin D3 and interleukin-6 levels in immunoglobulin treatment of Kawasaki disease in children.

    Science.gov (United States)

    An, Xinjiang; Fu, Mingyu; Tian, Jing; Xue, Ying; Xu, Hui

    2016-09-01

    The aim of the study was to investigate the significance of the level of serum 25-hydroxyvitamin D3 [25-(OH)D3] and interleukin (IL)-6 in serum prior to and after immunoglobulin treatment in children suffering from Kawasaki disease in order to provide a reference for the successful treatment of Kawasaki disease in children. From February, 2013 to February, 2015, 45 patients with Kawasaki disease were enrolled in the observation group. The normal control group comprised 43 healthy volunteers and the feverish control group 46 patients with respiratory infection and fever. Venous blood was collected from each case before and after immunoglobulin treatment and the level of 25-(OH)D3 and IL-6 in the serum were measured using fluorescent quantitative PCR, enzyme-linked immunosorbent assay and western blotting. Before treatment, the level of 25-(OH)D3 in the feverish control group was significantly lower than that of the normal control group, while the level of 25-(OH)D3 in the observation group was significantly higher than that of the normal control group. The level of 25-(OH)D3 in the feverish control group was lower than the IL-6 level in the normal children, but the difference was not statistically significant (P>0.05). The level 25-(OH)D3 in the observation group was significantly higher than the IL-6 level in the normal control group. The serum content of 25-(OH)D3 was significantly higher after the treatment compared to before treatment levels and after treatment IL-6 level was only slightly lower. It was observed that the 25-(OH)D3 level in the observation group was significantly increased after immunoglobulin treatment and this was positively correlated with the effects of the treatment. The IL-6 level had no significant changes after treatment and had little correlation with the treatment effect. The results suggested that 25-(OH)D3 may be involved in the occurrence of Kawasaki disease in children and in the aggravation of the disease to some extent.

  19. Transforming growth factor beta receptor II polymorphisms are associated with Kawasaki disease

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    Yu Mi Choi

    2012-01-01

    Full Text Available Purpose : Transforming growth factor beta receptor 2 (TGFBR2 is a tumor suppressor gene that plays a role in the differentiation of striated cells and remodeling of coronary arteries. Single nucleotide polymorphisms (SNPs of this gene are associated with Marfan syndrome and sudden death in patients with coronary artery disease. Cardiovascular remodeling and T cell activation of TGFBR2 gene suggest that the TGFBR2 gene SNPs are related to the pathogenesis of Kawasaki disease (KD and coronary artery lesion (CAL. Methods : The subjects were 105 patients with KD and 500 healthy adults as controls. Mean age of KD group was 32 months age and 26.6% of those had CAL. We selected TGFBR2 gene SNPs from serum and performed direct sequencing. Results : The sequences of the eleven SNPs in the TGFBR2 gene were compared between the KD group and controls. Three SNPs (rs1495592, rs6550004, rs795430 were associated with development of KD (P=0.019, P=0.026, P=0.016, respectively. One SNP (rs1495592 was associated with CAL in KD group (P=0.022. Conclusion : Eleven SNPs in TGFBR2 gene were identified at that time the genome wide association. But, with the change of the data base, only six SNPs remained associated with the TGFBR2 gene. One of the six SNPs (rs6550004 was associated with development of KD. One SNP associated with CAL (rs1495592 was disassociated from the TGFBR2 gene. The other five SNPs were not functionally identified, but these SNPs are notable because the data base is changing. Further studies involving larger group of patients with KD are needed.

  20. NT-proBNP as a useful tool in diagnosing incomplete Kawasaki disease

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    Dong Won Lee

    2010-04-01

    Full Text Available Purpose : To determine the efficacy of the N-terminal fragment of B-type natriuretic peptide (NT-proBNP as a useful diagnostic method in children with incomplete Kawasaki disease (KD. Methods : Ninety-six patients who were diagnosed as having KD between January 2008 and June 2009 were enrolled in the study. American Heart Association recommendations for diagnosis were used, and patients were divided into the complete KD and incomplete KD groups. Blood tests including NT-proBNP were performed on admission day. Nineteen patients who had other febrile diseases other than KD were enrolled as control. Results : Thirty-three patients (34% had incomplete KD. Change in the lips and oral cavity and conjunctivitis were the most common clinical features, but their frequency was lower than complete KD (76% vs 98%, 76% vs 90%. Patients with incomplete KD exhibited significantly higher NT-proBNP level than that of control (1,407.7¡?#?633;.5 pg/mL vs 126.2¡?#?35.;5 pg/mL, P &lt;0.001. An NT-proBNP cutoff value of 158 pg/mL provided a sensitivity of 81% and a specificity of 74% for diagnosis of incomplete KD. Conclusion : NT-proBNP assay can be clinically useful for the diagnosis of incomplete KD, if the patient has persistent fever, change in the lips and oral cavity, and conjunctivitis, and if the patient with those symptoms is suspected to have incomplete KD.

  1. Endothelial progenitor cell down-regulation in a mouse model of Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    LIU Jun-feng; DU Zhong-dong; CHEN Zhi; LU Dun-xiang; LI Li; GUAN Yun-qian; WAN Sui-gui

    2012-01-01

    Background Cardiovascular complications of Kawasaki disease (KD) are a common cause of heart disease in pediatric populations.Previous studies have suggested a role for endothelial progenitor cells (EPCs) in coronary artery lesions associated with KD.However,long-term observations of EPCs during the natural progression of this disorder are lacking.Using an experimental model of KD,we aimed to determine whether the coronary artery lesions are associated with down-regulation of EPCs.Methods To induce KD,C57BL/6 mice were administered an intraperitoneal injection of Lactobacillus casei cell wall extract (LCWE; phosphate buffered saline used as control vehicle).Study groups included:group A (14 days following LCWE injection),group B (56 days following LCWE injection) and group C (controls).Numbers of circulating EPCs (positively staining for both CD34 and FIk-1 while staining negative for CD45) were evaluated using flow cytometry.Bone marrow mononuclear cells were cultured in vitro to expand EPCs for functional analysis.In vitro EPC proliferation,adhesion and migration were assessed.Results The model was shown to exhibit similar coronary artery lesions to KD patients with coronary aneurysms.Numbers of circulating EPCs decreased significantly in the KD models (groups A and B) compared to controls ((0.017±0.008)% VS.(0.028±0.007)%,P<0.05 and (0.016±0.007)% vs.(0.028±0.007)%,P <0.05).Proliferative,adhesive and migratory properties of EPCs were markedly impaired in groups A and B.Conclusion Coronary artery lesions in KD occur as a consequence of impaired vascular injury repair,resulting from excess consumption of EPCs together with a functional impairment of bone marrow EPCs and their precursors.

  2. A child with Epstein-Barr Virus-associated hemophagocytic lymphohistiocytosis complicated by coronary artery lesion mimicking Kawasaki disease.

    Science.gov (United States)

    Kato, Shogo; Yoshimura, Ken; Tanabe, Yuko; Kimata, Takahisa; Noda, Yukihiro; Kawasaki, Hirohide; Kaneko, Kazunari

    2013-10-01

    There is considerable overlap between hemophagocytic lymphohistiocytosis (HLH) and Kawasaki disease (KD) in terms of aberrant immune response though the etiology of KD remains unknown. We present a case fulfilling the criteria of both HLH and KD complicated by coronary artery dilatation: HLH was confirmed to be triggered by Epstein-Barr virus. This case alarms us the possibility that even patients with HLH may be complicated by coronary artery lesion, which is one of the hallmarks of KD. We would like to draw attention that if features of KD become apparent in patients with HLH, echocardiographic examinations should be performed not to miss coronary artery lesion.

  3. Progress in pathogenesis of Kawasaki disease%川崎病发病机制研究进展

    Institute of Scientific and Technical Information of China (English)

    唐诗

    2009-01-01

    @@ 川崎病(kawasaki disease,KD)又称皮肤黏膜淋巴结综合征(Inucocutaneous lymph node syndrome,MCLS),是一种以全身血管炎为主要病变的急性发热出疹性疾病.主要影响婴幼儿,世界各国均有发生,以亚裔发病率最高.该病已取代风湿热成为我国小儿后天性心脏病主要原因之一.

  4. [Streptococcal toxic shock syndrome or Kawasaki disease? Two case studies of children with group A streptococcal pneumonia empyema].

    Science.gov (United States)

    Bosland, A; Arlaud, K; Rousset-Rouvière, C; Fouilloux, V; Paut, O; Dubus, J-C; Bosdure, E

    2011-12-01

    We report 2 cases of children with group A streptococcus pyogenes pleuropneumonia, in one child associated with Kawasaki disease and in the other with streptococcal toxic shock syndrome. These 2 features, with theoretically well-defined clinical and biological criteria, are difficult to differentiate in clinical practice, however, likely due to their pathophysiological links. In case of clinical doubt, an echocardiography needs to be performed to search for coronary involvement and treatment including intravenous immunoglobulins, and an antibiotic with an anti-toxin effect such as clindamycin has to be started early.

  5. Use of Lagrangian transport models and Sterilized High Volume Sampling to pinpoint the source region of Kawasaki disease and determine the etiologic agent

    Science.gov (United States)

    Curcoll Masanes, Roger; Rodó, Xavier; Anton, Jordi; Ballester, Joan; Jornet, Albert; Nofuentes, Manel; Sanchez-Manubens, Judith; Morguí, Josep-Anton

    2015-04-01

    Kawasaki disease (KD) is an acute, coronary artery vasculitis of young children, and still a medical mystery after more than 40 years. A former study [Rodó et al. 2011] demonstrated that certain patterns of winds in the troposphere above the earth's surface flowing from Asia were associated with the times of the annual peak in KD cases and with days having anomalously high numbers of KD patients. In a later study [Rodó et al. 2014], we used residence times from an Air Transport Model to pinpoint the source region for KD. Simulations were generated from locations spanning Japan from days with either high or low KD incidence. In order to cope with stationarity of synoptic situations, only trajectories for the winter months, when there is the maximum in KD cases, were considered. Trajectories traced back in time 10 days for each dataset and location were generated using the flexible particle Lagrangian dispersion model (FLEXPART Version 8.23 [Stohl et al. 2005]) run in backward mode. The particles modeled were air tracers, with 10,000 particles used on each model run. The model output used was residence time, with an output grid of 0.5° latitude × longitude and a time resolution of 3 h. The data input used for the FLEXPART model was gridded atmospheric wind velocity from the European Center for Medium-Range Weather Forecasts Re-Analysis (ERA-Interim at 1°). Aggregates of winter period back-trajectories were calculated for three different regions of Japan. A common source of wind air masses was located for periods with High Kawasaki disease. Knowing the trajectories of winds from the air transport models, a sampling methodology was developed in order to capture the possible etiological agent or other tracers that could have been released together. This methodology is based on the sterilized filtering of high volumes of the transported air at medium tropospheric levels by aircraft sampling and a later analyze these filters with adequate techniques. High purity

  6. Inflammation @ the heart of Kawasaki disease: Immunogenetic studies on etiology, pathophysiology and treatment effect

    NARCIS (Netherlands)

    Breunis, W.B.

    2010-01-01

    Bij de ziekte van Kawasaki raken de bloedvaten in het lichaam ontstoken. Om onbekende redenen is er een voorkeur voor de kransslagaderen. In Nederland is dit een zeldzame aandoening, maar in andere landen (bijvoorbeeld Japan) komt de ziekte veel vaker voor. Willemijn Breunis onderzocht welke genen

  7. Kawasaki disease: Studies on etiology, treatment and long-term follow-up

    NARCIS (Netherlands)

    Tacke, C.E.A.

    2014-01-01

    De ziekte van Kawasaki is een pediatrische vasculitis waarbij als complicatie coronaire arteriële aneurysmata kunnen ontstaan. Carline Tacke beschrijft in de 5 delen van haar proefschrift verschillende studies met betrekking tot de etiologie, behandeling en lange termijn uitkomsten van dit zeldzame

  8. Kawasaki disease: Studies on etiology, treatment and long-term follow-up

    NARCIS (Netherlands)

    Tacke, C.E.A.

    2014-01-01

    De ziekte van Kawasaki is een pediatrische vasculitis waarbij als complicatie coronaire arteriële aneurysmata kunnen ontstaan. Carline Tacke beschrijft in de 5 delen van haar proefschrift verschillende studies met betrekking tot de etiologie, behandeling en lange termijn uitkomsten van dit zeldzame

  9. Inflammation @ the heart of Kawasaki disease: Immunogenetic studies on etiology, pathophysiology and treatment effect

    NARCIS (Netherlands)

    Breunis, W.B.

    2010-01-01

    Bij de ziekte van Kawasaki raken de bloedvaten in het lichaam ontstoken. Om onbekende redenen is er een voorkeur voor de kransslagaderen. In Nederland is dit een zeldzame aandoening, maar in andere landen (bijvoorbeeld Japan) komt de ziekte veel vaker voor. Willemijn Breunis onderzocht welke genen m

  10. Kawasaki disease in Mongolia: results from 2 nationwide retrospective surveys, 1996-2008.

    Science.gov (United States)

    Davaalkham, Dambadarjaa; Nakamura, Yosikazu; Baigalmaa, Davaakhuu; Davaa, Gombojav; Chimedsuren, Ochir; Sumberzul, Nyamjav; Lkhagvasuren, Tserenkhuu; Uehara, Ritei; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2011-01-01

    Kawasaki disease (KD) has been reported in many countries. However, the incidence of KD in Mongolia is not known. This is the first report of incident cases of KD in Mongolia, which were identified using data from 2 nationwide surveys. Two nationwide retrospective surveys were conducted: medical histories were collected from patients aged 0 to 16 years who were hospitalized countrywide between 1996 and 2008. Hospital records for these patients were also reviewed. Nationwide training seminars on KD were conducted before each survey. For the nationwide surveys, the participation rates among all hospitals with pediatric wards were 97% and 94%. Inpatient medical histories from 1996 through 2008 were reviewed, and, among children younger than 16 years, 9 patients with KD were investigated. The age of KD patients ranged from 1.4 to 14 years; 7 of 9 patients were male. Six (67%) patients fulfilled all 6 clinical diagnostic criteria; the other 3 (33%) were defined as having KD based on the presence of 5 such criteria. Fever persisting 5 or more days, bilateral conjunctival congestion, and changes of the lips and oral cavity were the most common symptoms, and cervical lymphadenopathy was the least common symptom. Cardiac sequelae developed in 5 of the patients, 4 of whom were older than 10 years. The results of these nationwide surveys reveal that KD cases do exist in Mongolia. However, knowledge of KD among Mongolian pediatricians is likely to be poor. Thus, there is a need to augment their understanding to improve management of KD patients. Further studies are crucial to clarify the epidemiologic characteristics of KD in Mongolia.

  11. Kawasaki Disease in Mongolia: Results From 2 Nationwide Retrospective Surveys, 1996–2008

    Science.gov (United States)

    Davaalkham, Dambadarjaa; Nakamura, Yosikazu; Baigalmaa, Davaakhuu; Davaa, Gombojav; Chimedsuren, Ochir; Sumberzul, Nyamjav; Lkhagvasuren, Tserenkhuu; Uehara, Ritei; Yanagawa, Hiroshi; Kawasaki, Tomisaku

    2011-01-01

    Background Kawasaki disease (KD) has been reported in many countries. However, the incidence of KD in Mongolia is not known. This is the first report of incident cases of KD in Mongolia, which were identified using data from 2 nationwide surveys. Methods Two nationwide retrospective surveys were conducted: medical histories were collected from patients aged 0 to 16 years who were hospitalized countrywide between 1996 and 2008. Hospital records for these patients were also reviewed. Nationwide training seminars on KD were conducted before each survey. Results For the nationwide surveys, the participation rates among all hospitals with pediatric wards were 97% and 94%. Inpatient medical histories from 1996 through 2008 were reviewed, and, among children younger than 16 years, 9 patients with KD were investigated. The age of KD patients ranged from 1.4 to 14 years; 7 of 9 patients were male. Six (67%) patients fulfilled all 6 clinical diagnostic criteria; the other 3 (33%) were defined as having KD based on the presence of 5 such criteria. Fever persisting 5 or more days, bilateral conjunctival congestion, and changes of the lips and oral cavity were the most common symptoms, and cervical lymphadenopathy was the least common symptom. Cardiac sequelae developed in 5 of the patients, 4 of whom were older than 10 years. Conclusions The results of these nationwide surveys reveal that KD cases do exist in Mongolia. However, knowledge of KD among Mongolian pediatricians is likely to be poor. Thus, there is a need to augment their understanding to improve management of KD patients. Further studies are crucial to clarify the epidemiologic characteristics of KD in Mongolia. PMID:21691035

  12. Gene-Gene Associations with the Susceptibility of Kawasaki Disease and Coronary Artery Lesions.

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    Ho-Chang Kuo

    Full Text Available Kawasaki disease (KD is a systemic vasculitis primarily affecting children < 5 years old. Genes significantly associated with KD mostly involve cardiovascular, immune, and inflammatory responses. Recent studies have observed stronger associations for KD risk with multiple genes compared to individual genes. Therefore, we investigated whether gene combinations influenced KD susceptibility or coronary artery lesion (CAL formation. We examined 384 single-nucleotide polymorphisms (SNPs for 159 immune-related candidate genes in DNA samples from KD patients with CAL (n = 73, KD patients without CAL (n = 153, and cohort controls (n = 575. Individual SNPs were first assessed by univariate analysis (UVA and multivariate analysis (MVA. We used multifactor dimensionality reduction (MDR to examine individual SNPs in one-, two-, and three-locus best fit models. UVA identified 53 individual SNPs that were significantly associated with KD risk or CAL formation (p < 0.10, while 35 individual SNPs were significantly associated using MVA (p ≤ 0.05. Significant associations in MDR analysis were only observed for the two-locus models after permutation testing (p ≤ 0.05. In logistic regression, combined possession of PDE2A (rs341058 and CYFIP2 (rs767007 significantly increased KD susceptibility (OR = 3.54; p = 4.14 x 10(-7, while combinations of LOC100133214 (rs2517892 and IL2RA (rs3118470 significantly increased the risk of CAL in KD patients (OR = 5.35; p = 7.46 x 10(-5. Our results suggest varying gene-gene associations respectively predispose individuals to KD risk or its complications of CAL.

  13. Verification of risk scores to predict i.v. immunoglobulin resistance in incomplete Kawasaki disease.

    Science.gov (United States)

    Kanamitsu, Kiichiro; Kakimoto, Hisako; Shimada, Akira; Nakata, Yusei; Ochi, Hiroaki; Watanabe, Hirokazu; Iwasaki, Yuka; Tokorodani, Chiho; Kanazawa, Akane; Maruyama, Hidehiko; Miyazawa, Mari; Nishiuchi, Ritsuo; Kikkawa, Kiyoshi

    2016-02-01

    A recent study indicated the efficacy of the addition of prednisolone to i.v. immunoglobulin (IVIG) as initial treatment in patients with higher risk of IVIG resistance. Several different risk scores for predicting IVIG resistance have been proposed, mainly based on typical Kawasaki disease (KD) patients. We investigated the utility of the risk scores to predict IVIG resistance in incomplete KD. Clinical records of incomplete KD patients who received a single dose of IVIG between 2005 and 2012 at Kochi Health Sciences Center were retrospectively reviewed. Patients were classified into an IVIG-responsive group and an IVIG-resistant group. The Kobayashi, Egami, and Sano risk scores were calculated for each patient and the proportion of high-risk patients was compared between the two groups for each risk score. For 51 incomplete KD patients, Kobayashi (66.7% vs 47.6%, P = 0.253), Egami (55.6% vs 38.1%, P = 0.274), and Sano (57.1% vs 10.8%, P = 0.068) risk scores identified a higher proportion of high-risk patients in the IVIG-resistant group compared with the IVIG-responsive group, but significant difference was not observed. Sano risk score had the highest OR (6.19; 95%CI: 1.00-38.26). The proportion of patients identified as being at high risk for IVIG resistance using the Kobayashi, Egami, and Sano risk scores, respectively, was not significantly different between the IVIG-responsive group and the IVIG-resistant group for incomplete KD. Among the three risk scores, the Sano risk score has the best ability to predict IVIG resistance in incomplete KD. © 2015 Japan Pediatric Society.

  14. Corticosteroid Therapy Might be Associated with the Development of Coronary Aneurysm in Children with Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    Chun-Na Zhao; Zhong-Dong Du; Ling-Ling Gao

    2016-01-01

    Background:Coronary artery lesions (CALs) are known to be the main complication in children with Kawasaki disease (KD).Instead of intravenous immunoglobulin (IVIG),corticosteroid therapy has been accepted to be used for children with KD who are unresponsive to IVIG.This study aimed to evaluate risk factors for CALs in children with KD.Methods:We retrospectively reviewed the clinical records of 2331 children with KD from January 2005 to December 2014.To identify the independent risk factors for CALs,multivariable logistic regression models were constructed using significant variables identified from univariate logistic regression analysis.Results:The incidence of CALs was 36.0% (840 of 2331),including 625 (26.8%) coronary artery dilations and 215 (9.2%) coronary artery aneurysms (CAAs).Multivariable logistic regression analysis identified that male,incomplete KD,longer fever duration,and C-reactive protein (CRP) > 100 mg/L were independent risk factors for coronary artery dilatations.On the other hand,male,incomplete KD,longer fever duration,prolonged days of illness at the initial treatment,corticosteroid therapy,sodium ≤133 mmol/L,and albumin <35 g/L were the independent risk factors for CAAs.In addition,corticosteroid therapy,prolonged days of illness at the initial treatment,and albumin <35 g/L were the independent risk factors for giant CAAs.Conclusions:CALs might be associated with male sex,incomplete KD,longer fever duration,prolonged days of illness at the initial treatment,albumin <35 g/L,sodium ≤ 133 mmol/L,CRP > 100 mg/L,and corticosteroid therapy.Corticosteroid therapy was an independent risk factor for CAAs and giant CAAs.Thus,corticosteroids should be used with caution in the treatment of KD with the risk for CALs.

  15. Epidemiologic features of Kawasaki disease in Shanghai from 2003 through 2007

    Institute of Scientific and Technical Information of China (English)

    MA Xiao-jing; YU Cen-yan; HUANG Min; CHEN Shu-bao; HUANG Mei-rong; HUANG Guo-ying

    2010-01-01

    Background The epidemiologic pictures of Kawasaki disease (KD) in Shanghai from 1998 through 2002 were reported,while the current status of KD in the following five years remains unknown.Methods A questionnaire form and diagnostic guidelines for KD were sent to 50 hospitals providing pediatric medical care in Shanghai, China. All patients with KD diagnosed during January 2003 through December 2007 were recruited.Results In total, 1187 cases of KD were enrolled. The incidence of KD was 36.78 to 53.28 (mean 46.32±6.51) per 100 000children under the age of 5 years between 2003 and 2007, which was higher than the year from 1998 to 2002 of (27.32±7.11) per 100 000, (t=4.406, P=0.002). Ages at onset ranged from 12 days to 13.6 years (median 1.8 years). It occurred more frequently in summer and spring. Coronary arterial lesions (CAL), defined as ectasia or aneurysm,accounted for 19.8% (232 cases). Flattened or inverted T wave was the most frequent finding (194 cases, 20.5%) by electrocardiogram. Intravenous gamma-globulin was administrated to 1028 cases (86.6%). The occurrence of CAL seemed less frequent in the patients received gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice.Conclusions The incidence of KD was increasing in Shanghai. Treatment with intravenous gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice resulted in less coronary sequelae.

  16. Kawasaki disease-specific molecules in the sera are linked to microbe-associated molecular patterns in the biofilms.

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    Takeshi Kusuda

    Full Text Available Kawasaki disease (KD is a systemic vasculitis of unknown etiology. The innate immune system is involved in its pathophysiology at the acute phase. We have recently established a novel murine model of KD coronary arteritis by oral administration of a synthetic microbe-associated molecular pattern (MAMP. On the hypothesis that specific MAMPs exist in KD sera, we have searched them to identify KD-specific molecules and to assess the pathogenesis.We performed liquid chromatography-mass spectrometry (LC-MS analysis of fractionated serum samples from 117 patients with KD and 106 controls. Microbiological and LC-MS evaluation of biofilm samples were also performed.KD samples elicited proinflammatory cytokine responses from human coronary artery endothelial cells (HCAECs. By LC-MS analysis of KD serum samples collected at 3 different periods, we detected a variety of KD-specific molecules in the lipophilic fractions that showed distinct m/z and MS/MS fragmentation patterns in each cluster. Serum KD-specific molecules showed m/z and MS/MS fragmentation patterns almost identical to those of MAMPs obtained from the biofilms formed in vitro (common MAMPs from Bacillus cereus, Yersinia pseudotuberculosis and Staphylococcus aureus at the 1st study period, and from the biofilms formed in vivo (common MAMPs from Bacillus cereus, Bacillus subtilis/Bacillus cereus/Yersinia pseudotuberculosis and Staphylococcus aureus at the 2nd and 3rd periods. The biofilm extracts from Bacillus cereus, Bacillus subtilis, Yersinia pseudotuberculosis and Staphylococcus aureus also induced proinflammatory cytokines by HCAECs. By the experiments with IgG affinity chromatography, some of these serum KD-specific molecules bound to IgG.We herein conclude that serum KD-specific molecules were mostly derived from biofilms and possessed molecular structures common to MAMPs from Bacillus cereus, Bacillus subtilis, Yersinia pseudotuberculosis and Staphylococcus aureus. Discovery of these KD

  17. [Phosphatase and tensin homolog deleted on chromosome ten/phosphatidyl Inositol 3-kinase/vascular endothelial growth factor signaling pathway changes in the rabbit Kawasaki disease model].

    Science.gov (United States)

    Niu, L; Fu, M Y; Tian, J; He, X H; Zhang, H N; Wang, Q W; Wang, Y; Li, C L; Wang, Z Z; An, X J

    2016-03-01

    To observe the changes of phosphatase and tensin homolog deleted on chromosome ten(PTEN)/ phosphatidyl Inositol 3-kinase(PI3K)/ vascular endothelial growth factor(VEGF)signaling pathway in a rabbit Kawasaki disease model. Model of Kawasaki disease was established in weanling Japanese big-eared rabbits with 10% bovine serum venous injection (2.5 ml/kg, 2 times, and 2 week's interval) through the ear. Twenty four rabbits were divided into 4 groups: control group (without injection of 10% bovine serum albumin, six rabbits); 1 day group (sacrificed a the second day after the establishment of Kawasaki disease models, six rabbits); 7 day group (sacrificed at the seventh day after establishment of Kawasaki disease model, six rabbits); 30 day group (sacrificed at the thirtieth day after establishment of Kawasaki disease model, six rabbits). Pathological analysis was performed on coronary artery tissue samples. The express of PTEN and PI3K were detected by immunohistochemistry. The levels of VEGF and CK were also examined with ELISA and white blood cells were counted. (1) Coronary artery of model groups was thinner, distorted and had enlarged lumen. (2) PTEN expression in 1 d group, 7 d group and 30 d group were 58.5 ± 12.9, 73.2±9.9 and 109.6 ± 24.4, respectively, significantly higher than in the control group (25.5 ± 6.9, P0.05) and significantly lower than 1 d and 7 d group (both P0.05). (6)White blood cell count were significantly higher in 1 d group, 7 d group and 30 d group than in control group (all PKawasaki disease model and the signaling pathway might be involved in this model.

  18. 68例不典型川崎病临床特征分析%68 cases of atypical kawasaki disease clinical characteristics analysis

    Institute of Scientific and Technical Information of China (English)

    陈颖; 石明芳

    2016-01-01

    Objective To explore the clinical features of atypical kawasaki disease and early diagnosis.Methods In our hospital in August 2006 to August 2006 were analyzed in 68 cases of atypical kawasaki disease and the clinical data of 65 patients with typical kawasaki disease were analyzed, and summarize the early diagnosis of atypical kawasaki disease and effective method of treatment.Results After treatment, 68 patients were clinical cure, the average length of hospital stay (13.6 ±3.3) days.Conclusions Early diagnosis and timely treatment is the key to the treatment of atypical kawasaki disease.%目的:探讨不典型川崎病的临床特征与早期诊断。方法:对我院2006年8月~2014年8月收治的68例不典型川崎病患者及65例典型川崎病患者的临床资料进行分析,总结不典型川崎病的早期诊断及治疗的有效方法。结果:经过治疗,68例患者均实现临床治愈,平均住院时间(13.6±3.3)天。结论:早期诊断,及时治疗是治疗不典型川崎病的关键。

  19. [Clinical features and course of Kawasaki disease in central Tunisia: a study about 14 cases collected over a period of three years (2000-2002)].

    Science.gov (United States)

    Chemli, Jalel; Kchaou, Habib; Amri, Fethi; Belkadhi, Adel; Essoussi, Ahmed Sahloul; Gueddiche, Neji; Harbi, Abdelaziz

    2005-08-01

    To analyze the clinical features and course of Kawasaki disease in central Tunisia. We studied retrospectively 14 cases of children with Kawasaki disease collected in tunisian center during three years (2000-2002). The study is about 11 boys and 3 girls (sex - ratio: 3.6/1) aged from 6 months to 8 years (mean age : 4 years). Twelve patients had at least 5 diagnostic criteria of the illness, the two others had an incomplete form. We noted cardiac complications in seven patients treated belatedly, beyond 10 days of progression, because of atypical clinical presentations. All patients had all a middle caliber coronary aneurysm that was complicated by a thrombus in three cases, associated with pericarditis and minimal mitral insufficiency in a case and with a cardiac rhythm disturbance (block of branch) in another case. Besides the cardiac complications, several other visceral manifestation could be noted: joint symptoms in five cases, GI tract symptomes in three cases, neuro-meningeal in two cases and urinary trad symptomes in two other cases. Specific treatment (aspirin with antiinflammatory dose and intravenous immune globulin (IVIG)) has been instituted in all patients. The course was favorable for 12 patients with fast regression of clinical manifestation and progressive normalisation of biologic values. Two patients did not respond to the initial IVIG treatment, and had to recense received an additional course of IGIV but without clinical nor biological improvement. These two patients were treated with corticosteroids. Cardiac lesions disappeared completely in all patients even for those with thrombosis and in patients with IVIG-resistant Kawasaki disease. Only one patient had kept neurologic sequellae: aphasia, bevavioral problemes and partial epilepsy. Kawasaki disease is not rare in our region. Incomplete or atypical presentations are frequent and are a source of diagnostic delay. Coronary aneurysm due to the delay of treatment often regresses even in patients

  20. Kawasaki Shock Syndrome in a 12-Year-Old Girl Mimicking Septic Shock

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    Vindika Prasad Sinhabahu

    2016-01-01

    Full Text Available Kawasaki disease is diagnosed when fever lasts for more than 5 days with the presence of four out of five of the following clinical features: bilateral conjunctival congestion, changes in the lips and oral cavity, polymorphous exanthem, changes in peripheral extremities, and acute nonpurulent cervical lymphadenopathy (Nakamura et al., 2012. The average age of onset is 2 years and 90% of patients are below 5 years of age. Boys are more affected than girls (Cox and Sallis, 2009. This case report describes an adolescent female who was initially managed as having septic shock and subsequently found to have Kawasaki shock syndrome.

  1. Early combined treatment with steroid and immunoglobulin is effective for serious Kawasaki disease complicated by myocarditis and encephalopathy.

    Science.gov (United States)

    Suga, Kenichi; Inoue, Miki; Ono, Akemi; Terada, Tomomasa; Kawahito, Masami; Mori, Kazuhiro

    2016-01-01

    Severe-type Kawasaki disease (KD) complicated by serious myocarditis and encephalopathy can be successfully treated without abnormality of the coronary arteries by steroid pulse treatment and intravenous immunoglobulin (IVIg). A 4-year-old Japanese girl was diagnosed with KD due to a 6-day history of fever, rash, flushed lips, conjunctival hyperemia, palmar edema, and cervical lymphadenopathy. The day after initiation of IVIg and aspirin, cardiac gallop rhythm was identified. Cardiac ultrasonography revealed severe left ventricular dysfunction. Disturbance of consciousness, hallucinations, and slurred speech were also observed. Magnetic resonance imaging showed no abnormalities, but electroencephalography revealed high-voltage slow waves. Despite this serious disease, cardiac function and neurological abnormalities showed complete recovery without dilatation of the coronary arteries by steroid pulse treatment and additional IVIg. Follow-up at 15 months revealed no abnormality of the coronary arteries. In conclusion, we suggest that early combined treatment with steroid and IVIg is effective for serious KD complicated by myocarditis and encephalopathy.

  2. Increased frequency of immunoglobulin (Ig)A-secreting cells following Toll-like receptor (TLR)-9 engagement in patients with Kawasaki disease.

    Science.gov (United States)

    Giordani, L; Quaranta, M G; Marchesi, A; Straface, E; Pietraforte, D; Villani, A; Malorni, W; Del Principe, D; Viora, M

    2011-03-01

    Kawasaki disease (KD) is an acute vasculitis affecting mainly infants and children. Human B cells express Toll-like receptor (TLR)-9, whose natural ligands are unmethylated cytosine-guanine dinucleotide (CpG) motifs characteristic of bacterial DNA. The aim of this study was to clarify the pathogenesis of KD analysing the activation status of peripheral blood mononuclear cells (PBMC), focusing on B lymphocyte activation and functions. Ten patients and 10 age-matched healthy donors were recruited from the Bambino Gesù Hospital of Rome, Italy and enrolled into this study. We determined phenotype profile and immunoglobulin (Ig) production of PBMC from KD patients and age-matched controls. We found that the frequency of CD19(+) B lymphocytes and CD19(+) /CD86(+) activated B lymphocytes from KD patients during the acute phase before therapy was increased significantly. Moreover, B lymphocytes of acute-phase KD patients were more prone to CpG oligodeoxynucleotide (ODN) activation compared with the age-matched controls, as assessed by a significant increase of the number of IgA-secreting cells (SC). In the same patients we found a marked increase of IgM, IgG, interleukin (IL)-6 and tumour necrosis factor (TNF)-α production compared with the control group. In addition, in two convalescent KD patients, conventional treatment with intravenous immunoglobulin (IVIG) restored the normal frequency of CD19(+) B cells, the number of IgA-, IgM- and IgG-SC and the production of IL-6 and TNF-α. Our findings indicate that the percentages of peripheral B lymphocytes of acute-phase KD patients are increased and are prone to bacterial activation in terms of increased numbers of IgA-SC and increased production of IL-6 and TNF-α inflammatory cytokines. Thus, our data support the hypothesis of an infectious triggering in KD.

  3. 川崎病患儿血清白介素-17的变化%The changes of serum IL-17 in children with Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    李蕴言; 吴菱; 马田瑞; 陈圆玲; 狄亚珍

    2013-01-01

    Objectives To explore the change of interleukin-17 (IL-17) in Kawasaki disease (KD). Methods Fourty KD pediatric patients, among them 12 patients with echocardiographic abnormalities in acute phase, 25 age-matched non-KD patients were enrolled. The level of serum IL-17 was measured by enzyme linked immunosorbent assay in acute and convalescent phase of KD patients and non-KD patients. At the same time, C-reactive protein (CRP), globulin, albumin were detected. Results In acute phase of KD patients, the level of serum IL-17 were signiifcantly higher than that in convalescent phase of KD patients and non-KD patients (P0.05). In acute phase of KD patients with echocardiography abnormalities, the level of serum IL-17 was signiifcantly higher than that with non-echocardiography abnormalities (P0.05);急性期超声心动图异常KD患儿的血清IL-17水平高于超声心动图正常的患儿,差异有统计学意义(P<0.05)。KD患儿急性期血清IL-17与CRP、球蛋白水平呈正相关(r=0.750、0.750,P均<0.05),而与白蛋白水平呈负相关(r=-0.779, P<0.05)。结论 IL-17参与KD的免疫损伤过程,血清IL-17水平是KD活动的指标之一,IL-17水平与心血管损害相关。

  4. 可诱导共刺激分子在川崎病患儿外周血T淋巴细胞的表达%Inducible co-stimulator expression in peripheral T cells from children with Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    马颐姣; 李永柏; 杨军; 王国兵

    2011-01-01

    Objective To investigate the role of signal transduction of inducible co-stimulator (ICOS) in immunological pathogenesis of Kawasaki disease. Methods Fotal 48 patients with Kawasaki disease were enrolled in Kawasaki disease group, including 16 cases with coronary artery lesion (CAL) and 32 cases without coronary artery lesion (non-CAL). Thirty patients treated with intravenous immunoglobulin (IVIG) were enrolled in IVIG-treatment group, including 23 cases sensitive to IVIG and 7 cases non-sensitive to IVIG. Forty-five age-matched children (healthy or acute bronchitis patients) were included in control group. Real-time PCR were used to evaluate the levels of ICOSmRNA expression in peripheral blood mononuclear cells. Results Compared with control group, the mRNA levels of ICOS in Kawasaki disease group were sgnificantly up-regulated (17.97 ±7.22 vs 8.01 ± 5.15, P < 0.01). Compared with nonCAL in Kawasaki disease group, the mRNA levels of ICOS in CAI group were up-regulated significantly (29.09 ±10.55 vs 11.68 ± 5.11, P < 0.01) .Comparcd with Kawasaki disease group, the mRNA levels of ICOS in IVIG-treatment group were down-regulated (13.03 ± 5.15 vs 17.97 ± 7.22, P < 0.05). Compared with IVIG sensitivity group in IVIGtreatment group, the mRNA levels of ICOS in non-sensitivity group were up-regulated significantly (21.57 ± 6.22 vs 9.85 ±5.89, P < 0.05) . Conclusions Expressions of ICOS was up-regulated during acute phase of Kawasaki disease, which suggested that ICOS might be one of the significant factors of immune aberrance in Kawasaki disease with coronary artery lesion. As a key treatment method, IVIG might be related to down-regulated expression of ICOS in Kawasaki disease.%目的 探讨可诱导共刺激分子(ICOS)在川崎病(KD)及KD伴有冠状动脉损害,IVIG非敏感型KD发病机制中的作用.方法 应用荧光定量PCR检测48例KD组和30例KD IVIG治疗组ICOSmRNA表达的变化.同时以25例同龄健康儿童和20例急性支气

  5. Hemolytic anemia following intravenous immunoglobulin therapy in patients treated for Kawasaki disease: a report of 4 cases

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    Berard Roberta

    2012-04-01

    Full Text Available Abstract Background Hemolytic anemia is a rare but reported side effect of intravenous immunoglobulin (IVIG therapy. The risk of significant hemolysis appears greater in those patients who receive high dose IVIG. The etiology is multifactorial but may relate to the quantity of blood group antibodies administered via the IVIG product. Findings We describe 4 patients with significant hemolytic anemia following treatment with IVIG for Kawasaki disease (KD. Direct antibody mediated attack as one of the mechanisms for hemolysis, in this population, is supported by the demonstration of specific blood group antibodies in addition to a positive direct antiglobulin test in our patients. Conclusions Clinicians should be aware of this complication and hemoglobin should be closely monitored following high dose IVIG therapy.

  6. Mycoplasma Infection as a cause of Persistent Fever after Intravenous Immunoglobulin Treatment of Patients with Kawasaki Disease: Frequency and Clinical Impact

    Science.gov (United States)

    Yoon, Kyung Lim; Cha, Sung-Ho; Moon, Sung Kyoung; Jung, Hae Woon

    2017-01-01

    Background Mycoplasma is a common cause of respiratory infections and may require differential diagnosis from Kawasaki disease (KD). In this study, we investigated the frequency and clinical manifestations of mycoplasma infection in patients with KD. Materials and Methods Medical records of 375 in-patients admitted for treatment during the acute stage of KD, were collected, and reviewed retrospectively. Of these patients, 152 (40.5%) were also tested for recent mycoplasma infection. Patients with positive results (anti-mycoplasma IgM Ab >1:640 or cold agglutinin >1:64) were designated as the case group (n = 37, 24.3%) whereas those with negative results were designated as the control group (n = 115, 75.7%). Clinical findings of the two groups were compared. Results Patients in the case group were older than those in the control group (mean age, 48.2 ± 32.1 months, vs. 31.7 ± 21.7 months; P = 0.001). There were significant differences between the case and control groups in the changes in the extremities (78.3% vs. 57.4%, respectively; P = 0.031), and in fever duration (6.5 ± 2.5 days vs. 5.4 ± 1.5 days; P = 0.047). Of the 37 patients with positive mycoplasma testing, 7 (18.9%) had persistent fever even after the symptoms and signs of systemic inflammation (acute phase of KD) had been resolved. These patients were positive for mycoplasma infection during further evaluation of persistent fever, and all of them responded to macrolide antibiotics. Conclusions We found that mycoplasma infection is somewhat related to KD. When fever persists after resolution of the acute stage of KD, mycoplasma infection may be considered as a possible cause of fever in preschool-aged children.

  7. Changes of serum cystatin C in children with Kawasaki disease and its role in the pathogenesis of coronary artery lesions%川崎病患儿血清胱抑素C的变化及其与冠状动脉病变的关系

    Institute of Scientific and Technical Information of China (English)

    姜毅; 李温慈; 徐海滨; 邵山鹰

    2011-01-01

    目的:观察胱抑素C在川崎病患儿血清中的变化及与川崎病患儿冠状动脉病变的关系.方法:选取温州市儿童医院42例川崎病和21例发热1周以内的呼吸道感染(对照组)患儿,川崎病患儿在急性期、亚急性期、恢复期接受血清胱抑素C检测,并通过心脏彩色多谱勒超声检查明确患儿冠状动脉病变情况.对照组入院后检测血清胱抑素C水平.结果:川崎病患儿在急性期和亚急性期血清胱抑素C水平均较对照组明显下降(P<0.05),而且冠状动脉病变组较无冠状动脉病变组下降更为显著,恢复期血清胱抑素C水平与对照组比较差异无统计学意义(P>0.05).结论:血清胱抑素C水平在川崎病急性期,尤其在冠状动脉病变时明显下降,亚急性期和恢复期持续不升可能与川崎病发生冠状动脉损伤程度有关,故监测胱抑素C水平可作为预测患儿冠状动脉病变的重要参考指标.%Objective; To investigate the changes of serum cystatin C in children with Kawasaki disease and its role in the pathogenesis of coronary artery lesions. Methods: Fourty two children with Kawasaki disease and 21 febrile controls were studied. The duration of Kawasaki disease was divided into three phases, including the acute phase, the subacute phase and the convalescent phase. Serum cystatin C was detected by immunoturbidimetry in three phases, respectively. The coronary artery lesions were checked by doppler echocardiograph. Results; The levels of serum cystatin C in Kawasaki disease patients decreased significantly in acute phase and subacute phase (P < 0.05), and the levels of serum cystatin C in the group with coronary artery lesions decreased more significantly than the group without coronary artery lesions. The levels of serum cystatin C in Kawasaki disease patients in conval escent phase were not significantly lower than those of the control group. Conclusion: The levels of serum cystatin C dropped obviously in

  8. Clinical analysis of misdiagnosis of 17 cases of kawasaki disease%川崎病17例临床误诊分析

    Institute of Scientific and Technical Information of China (English)

    杨志雄

    2015-01-01

    川崎病是临床较为少见的一种疾病,一些非典型病例常容易发生漏诊和误诊,给患者的治疗造成很大的困扰。本文简要介绍川崎病17例临床误诊分析,为临床提供探讨。%Kawasaki disease is a disease that is rare in clinic.Some atypical cases are easily missed diagnosis and misdiagnosis. It causes great distress for the treatment of patients.In this paper,the author briefly introduces the clinical analysis of misdiagnosis of 17 cases of kawasaki disease,to provide study for clinical.

  9. Estudo prospectivo das complicações da Doença de Kawasaki: análise de 115 casos Prospective study of Kawasaki Disease complications: review of 115 cases

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    Natália Ribeiro de M. Alves

    2011-06-01

    após a fase aguda da doença, eventualmente resultando em sequelas permanentes. Quanto mais precoce forem o diagnóstico e a intervenção terapêutica com a administração de IgG IV, menor será a ocorrência de complicações. Presença de trombocitose, anemia e de atividade inflamatória elevada e por tempo prolongado são fatores de risco para o aparecimento de complicações.OBJECTIVE: To draw attention to complications that might arise in any Kawasaki disease (KD stage, risk factors contributing to the onset of complications and possible transient or permanent disease sequelae. METHODS: Prospective study (clinical cohort conducted between April 2002 and April 2009 of 115 patients with KD admitted to the Pediatric Rheumatology Clinic of the General Hospital of the Federal District, Brazil. All patients were sequentially assessed with clinical and laboratory examinations, Doppler echocardiography, imitanciometry, auditory evoked potentials, psychological evaluation, ophthalmologic examination and, in one patient with chorea, cerebral magnetic resonance angiography. In all patients, a questionnaire assessing the possible presence of cognitive, emotional, behavioral and social disorders was applied. RESULTS: Twenty-five patients (21.7% had coronary aneurisms. Thirty eight patients (33% had a sensorineural auditory loss during the acute and subacute phases of the disease and 13 patients (11.3% maintained the auditory loss six months after the first assessment. Other complications observed were as follows: facial palsy in one patient (0.9%, ataxia in acute and subacute phases in 11 (9.5%; 15 patients had ophthalmologic complications (13.2%, with uveitis in 13, papilledema in one patient, and conjunctival hemorrhage in another patient. One patient experienced chorea (0.9%, with a magnetic resonance angiography showing changes consistent with cerebral ischemia. In one patient, a thoracic aorta aneurism was found (0.9% and another patient had a necrotizing vasculitis

  10. 凉山彝族地区不完全川崎病临床调查分析%Clinical investigation of incomplete Kawasaki disease in Yi nationality of Liangshan

    Institute of Scientific and Technical Information of China (English)

    石艳; 戴莉; 杨尧; 杨玉湘; 张海; 周静; 黄婷婷

    2011-01-01

    Objective To investigate the incidence, age structure, clinical features, complications and treatment conditions of incomplete Kawasaki disease in Liangshan Yi area in the past 9 years.Methods In this study, retrospective analysis and follow-up study were conducted on 83 confirmed cases of incomplete Kawasaki disease from hospitals in 17 counties of the prefecture, and the comparison was made with the complete Kawasaki disease.Results There were 83 confirmed cases of incomplete Kawasaki disease out of 307 cases of Kawasaki disease, with the proportion of incomplete Kawasaki disease.There were no significant differences in gender and age between incomplete Kawasaki disease and complete Kawasaki disease (P>0.05).The most common clinical manifestation was fever, followed by the changes of oral mucosa, and lymphadenectasis was very rare.There were significant differences in the symptoms, except fever, between incomplete Kawasaki disease and complete Kawasaki disease (P<0.05 ).Coronary artery lesions occurred in 37 cases,accounting for 44.58 percent of incomplete Kawasaki disease cases, indicating no significant difference from those of complete Kawasaki disease ( P>0.05 ).However, there was significant difference in incidence of moderate to severe coronary artery lesions between the complete and incomplete Kawasaki disease groups ( P<0.01 ).In the second week of the course of incomplete Kawasaki disease, platelets count significantly increased, while the white blood cells count decreased considerably, which were consistent with the symptoms of complete Kawasaki disease.Serum CRP concentration in incomplete Kawasaki disease group was 37.86±48.89mg/L, which was significantly lower than that in the complete Kawasaki disease group (P<0.05).R-Globulin sensitivity of incomplete Kawasaki disease was high, which was significantly different from that of complete Kawasaki disease(P<0.01).Conclusion The incidence rate of incomplete Kawasaki disease in Liangshan Yi

  11. To Investigate the Correlation between the Typical Clinical Manifestations of Children with Kawasaki Disease%小儿川崎病的典型临床表现相关性探讨

    Institute of Scientific and Technical Information of China (English)

    彭琪

    2014-01-01

    目的:探讨小儿川崎病的临床表现,为治疗此病提供参考。方法我院于2010年3月至2013年3月收治了74例小儿川崎病患者,收集这些患者的临床资料,分析他们的临床表现。结果比较完全性川崎病组和不完全性川崎病组的体征和症状,完全性川崎病组和不完全性川崎病组的患儿在发热、球结膜充血、口唇改变这3个症状上,并没有完全的差异和区别;但是在皮疹、肛周红肿、颈部淋巴异常、手脚蜕皮这几个症状上,完全性川崎病组的发生率明显高于不完全性川崎病组。结论由于完全性川崎病和不完全性川崎病的临床表现有很多不同的地方,所以在检查的时候,必须将实验室检查的八项指标全部纳入进去,这样才能防止出现误诊和漏诊的现象。%ObjectiveTo investigate the clinical manifestations of Kawasaki disease in children, to give reference for the treatment of this disease. MethodsIn our hospital from March 2010 to March 2013 treated 74 cases of children with Kawasaki disease patients, collected the clinical data of these patients, analyze their clinical manifestations.ResultsCompared with complete Kawasaki disease group and incomplete Kawasaki disease group of signs and symptoms, complete Kawasaki disease group and incomplete Kawasaki disease group in children with fever, conjunctival congestion, lips change these 3 symptoms, and no difference and distinction; but in a rash, perianal irritation, abnormal cervical lymph, and hands molt these symptoms, the incidence of Kawasaki disease group was signiifcantly higher than that of incomplete Kawasaki disease group.ConclusionThe clinical manifestations of Kawasaki disease and incomplete Kawasaki disease in many different places, so when the examination, must be eight indicators of laboratory examination into account, so as to prevent misdiagnosis phenomenon.

  12. Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomised, open-label, blinded-endpoints trial.

    Science.gov (United States)

    Kobayashi, Tohru; Saji, Tsutomu; Otani, Tetsuya; Takeuchi, Kazuo; Nakamura, Tetsuya; Arakawa, Hirokazu; Kato, Taichi; Hara, Toshiro; Hamaoka, Kenji; Ogawa, Shunichi; Miura, Masaru; Nomura, Yuichi; Fuse, Shigeto; Ichida, Fukiko; Seki, Mitsuru; Fukazawa, Ryuji; Ogawa, Chitose; Furuno, Kenji; Tokunaga, Hirohide; Takatsuki, Shinichi; Hara, Shinya; Morikawa, Akihiro

    2012-04-28

    Evidence indicates that corticosteroid therapy might be beneficial for the primary treatment of severe Kawasaki disease. We assessed whether addition of prednisolone to intravenous immunoglobulin with aspirin would reduce the incidence of coronary artery abnormalities in patients with severe Kawasaki disease. We did a multicentre, prospective, randomised, open-label, blinded-endpoints trial at 74 hospitals in Japan between Sept 29, 2008, and Dec 2, 2010. Patients with severe Kawasaki disease were randomly assigned by a minimisation method to receive either intravenous immunoglobulin (2 g/kg for 24 h and aspirin 30 mg/kg per day) or intravenous immunoglobulin plus prednisolone (the same intravenous immunoglobulin regimen as the intravenous immunoglobulin group plus prednisolone 2 mg/kg per day given over 15 days after concentrations of C-reactive protein normalised). Patients and treating physicians were unmasked to group allocation. The primary endpoint was incidence of coronary artery abnormalities during the study period. Analysis was by intention to treat. This trial is registered with the University Hospital Medical Information Network clinical trials registry, number UMIN000000940. We randomly assigned 125 patients to the intravenous immunoglobulin plus prednisolone group and 123 to the intravenous immunoglobulin group. Incidence of coronary artery abnormalities was significantly lower in the intravenous immunoglobulin plus prednisolone group than in the intravenous immunoglobulin group during the study period (four patients [3%] vs 28 patients [23%]; risk difference 0·20, 95% CI 0·12-0·28, pKawasaki disease in Japan. Further study of intensified primary treatment for this disease in a mixed ethnic population is warranted. Japanese Ministry of Health, Labour and Welfare. Copyright © 2012 Elsevier Ltd. All rights reserved.

  13. 小儿川崎病误诊35例临床分析%Clinical Analysis of 35 Cases Misdiagnosed Children with Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    柯长春

    2014-01-01

    Objective: To investigate the Kawasaki disease (KD) and the clinical symptoms and signs, laboratory examination, diagnosis and misdiagnosis. Methods: a retrospective analysis was made on 35 cases in our hospital Kawasaki disease data. Results: the early misdiagnosis rate was 29.7% (35 / 118), misdiagnosis and the shortest time of 3 days, the longest 20 days. Conclusion: to deepen the understanding of Kawasaki disease, improve related laboratory tests, can ef ectively reduce the misdiagnosis rate and rate of missed diagnosis.%目的:探讨川崎病(KD)的临床症状及体征、实验室检查、诊断及误诊原因。方法对我院35例误诊的川崎病患儿资料进行了回顾性分析。结果早期误诊率29.7%(35/118),误诊时间最短3d,最长20d。结论加深对川崎病的理解,及时完善相关实验室检查,可有效降低该病的误诊率和漏诊率。

  14. Assessment of coronary artery aneurysms in paediatric patients with Kawasaki disease by multidetector row CT angiography: feasibility and comparison with 2D echocardiography

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    Chu, W.C.W.; Lam, W.W.M. [Department of Diagnostic Radiology and Organ Imaging, Chinese Univ. of Hong Kong, Shatin (China); Mok, G.C.F.; Yam, M.; Sung, R.Y.T. [Dept. of Pediatrics, Chinese Univ. of Hong Kong, Shatin (China)

    2006-11-15

    Transthoracic ECHO is the locally accepted method for coronary surveillance of patients with Kawasaki disease but it may have limited visualization in the older child. To assess the feasibility of multidetector CT (MDCT) angiography in the follow-up of coronary artery aneurysms in children with previous Kawasaki disease. Six children (5 boys, 1 girl; mean age 11.5 years) with known Kawasaki disease and coronary artery involvement underwent CT coronary angiography using 16-detector MDCT. The visualized lengths and diameter of all coronary segments were measured. The number, size and location of coronary artery aneurysms were recorded and compared with recent ECHO. Twelve coronary artery aneurysms (seven saccular, five fusiform) were identified by MDCT angiography. One saccular aneurysm at the junction of the distal right coronary artery and posterior descending artery was not detected by ECHO while the remaining six in proximal segments were detected by both modalities. Two of five fusiform aneurysms were not detected by ECHO due to their small sizes. Excellent agreement was found between CT and ECHO for maximal diameter and length of the visualized aneurysms. MDCT angiography accurately defines coronary artery aneurysms. It is more sensitive for detecting aneurysms at distal coronary segments and fusiform aneurysms of small size.

  15. 不完全川崎病38例临床分析%Clinical analysis of 38 cases Incomplete Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    周宇

    2013-01-01

    Objective: to analyze the clinical features of incomplete Kawasaki disease. Methods: retrospective analysis of our hospital in 2009 January ~2013 year in April admitted 38 cases of incomplete Kawasaki disease in clinical manifestation, ultrasound heartbeat graph, electrocardiogram, chest X-ray, laboratory examination results. Results: incomplete Kawasaki disease clinical manifestations were fever, finger and toe end peeling, chapped lips redness, rash, bulbar conjunctiva hyperemia, hand and foot swel ing, cervical lymph nodes, perianal skin erythema, BCG vaccination. In addition, there are 3 cases of hand-foot-mouth disease complicated with incomplete Kawasaki disease. Conclusion: the clinical manifestation in children with Kawasaki disease, after exclusion of other viral disease, should be done as soon as possible laboratory examination and ultrasound heartbeat graph examination related, in order to get early diagnosis, early treatment, as far as possible to reduce the coronary artery damage.%  目的分析不完全川崎病的临床特点。方法回顾性分析我院2009年1月~2013年4月收治的不完全川崎病38例的临床表现、超声心动图、心电图、X线胸片、实验室检查结果。结果不完全川崎病的临床表现依次为发热,指趾端脱皮,口唇潮红干裂,皮疹,眼球结膜充血,手足硬肿,颈淋巴结肿大,肛周脱皮,卡介苗接种处红斑。此外有3例手足口病合并不完全川崎病。结论对具有川崎病临床表现的患儿,在排除其他出疹性疾病后,应尽早做相关的实验室检查和超声心动图检查,以得到早期诊断,早期治疗,尽可能减少患儿冠状动脉的损害。

  16. Serum ferritin levels as a useful diagnostic marker for the distinction of systemic juvenile idiopathic arthritis and Kawasaki disease.

    Science.gov (United States)

    Mizuta, Mao; Shimizu, Masaki; Inoue, Natsumi; Kasai, Kazuko; Nakagishi, Yasuo; Takahara, Tadamori; Hamahira, Kiyoshi; Yachie, Akihiro

    2016-11-01

    The clinical features and laboratory parameters of patients with Kawasaki disease (KD) and systemic juvenile idiopathic arthritis (s-JIA) tend to overlap. Furthermore, there have been no definitive biomarkers for these diseases, making clinical diagnosis difficult. The purpose of this study was to investigate the diagnostic value of serum ferritin levels for differentiating KD from s-JIA and predicting the disease severity of KD. We analyzed 228 patients with KD and 81 patients with s-JIA. Serum ferritin levels were compared between patients with s-JIA and KD. Furthermore, serum ferritin levels in patients with KD were compared with respect to clinical features such as responsiveness to intravenous immunoglobulin (IVIG) therapy. Serum ferritin levels in KD patients with no response to IVIG therapy were significantly higher than those in KD patients with a good response to IVIG therapy. Serum ferritin levels in patients with KD needing plasma exchange (PE) were significantly higher than those in patients not needing PE. However, serum ferritin levels overlapped between severe KD patients with nonresponsiveness to IVIG therapy or needing PE and other patients with mild KD. Furthermore, patients with s-JIA showed a distinct elevation of serum ferritin levels compared with KD patients. The cutoff value of serum ferritin levels for differentiating KD from s-JIA was 369.6 ng/ml. Serum ferritin levels were significantly elevated in s-JIA patients compared with KD patients. Measurement of serum ferritin levels can be useful for differentiating s-JIA from KD.

  17. CD40 Gene Polymorphisms Associated with Susceptibility and Coronary Artery Lesions of Kawasaki Disease in the Taiwanese Population

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    Ho-Chang Kuo

    2012-01-01

    Full Text Available Background. Kawasaki disease (KD is characterized by systemic vasculitis of unknown etiology. Our previous studies showed expression of CD40 ligand on CD4+ T cells correlated to the coronary artery lesion (CAL and disease progress in KD. Other studies from Japan suggested the role of CD40L in the pathogenesis of CAL, and this might help explain the excessive number of males affected with KD but cannot be reproduced by Taiwanese population. This study was conducted to investigate the CD40 polymorphism in KD and CAL formation. Methods. A total of 950 subjects (381 KD patients and 569 controls were investigated to identify 2 tagging single-nucleotide polymorphisms (tSNPs of CD40 (rs4810485 and rs1535045 by using the TaqMan allelic discrimination assay. Results. A significant association was noted with regards to CD40 tSNPs (rs1535045 between controls and KD patients (P=0.0405, dominant model. In KD patients, polymorphisms of CD40 (rs4810485 showed significant association with CAL formation (P=0.0436, recessive model. Haplotype analysis did not yield more significant results between polymorphisms of CD40 and susceptibility/disease activity of KD. Conclusions. This study showed for the first time that polymorphisms of CD40 are associated with susceptibility to KD and CAL formation, in the Taiwanese population.

  18. IL-1 signaling is critically required in stromal cells in Kawasaki Disease Vasculitis Mouse Model. Role of both IL-1α and IL-1β

    Science.gov (United States)

    Lee, Youngho; Wakita, Daiko; Dagvadorj, Jargalsaikhan; Shimada, Kenichi; Chen, Shuang; Huang, Ganghua; Lehman, Thomas J.A.; Fishbein, Michael C.; Hoffman, Hal M.; Crother, Timothy R.; Arditi, Moshe

    2015-01-01

    Objective Kawasaki disease (KD) is the most common cause of acute vasculitis and acquired cardiac disease among US children. We have previously shown that both TLR2/MyD88 and IL-1β signaling are required for the Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis mouse model. The objectives of this study were to investigate the cellular origins of IL-1 production, the role of CD11c+ Dendritic Cells (DCs) and macrophages and the relative contribution of hematopoietic and stromal cells for IL-1 responsive cells, as well the MyD88 signaling in LCWE-induced KD mouse model of vasculitis. Approach and Results Using mouse knockout models as well as antibody depletion, we found that both IL-1α and IL-1β were required for LCWE-induced KD. Both DCs and macrophages were necessary and we found that MyD88 signaling was required in both hematopoietic and stromal cells. However, IL-1 response and signaling was critically required in non-endothelial stromal cells, but not hematopoietic cells. Conclusions Our results suggest that IL-1α and IL-1β as well as CD11c+ DCs and macrophages are essential for the development of KD vasculitis and coronary arteritis in this mouse model. Bone marrow chimera experiments suggest that MyD88 signaling is important in both hematopoietic and stromal cells, while IL-1 signaling and response is required only in stromal cells, but not in endothelial cells. Determining the role IL-1α and IL-1β and of specific cell types in the KD vasculitis mouse model may have important implications for the design of more targeted therapies and understanding of the molecular mechanisms of KD immunopathologies. PMID:26515418

  19. KEGG DISEASE / Acute encephalitis [KEGG DISEASE

    Lifescience Database Archive (English)

    Full Text Available DISEASE: H01417 Entry H01417Disease Name Acute encephalitis Description Acute encep...ns Infections caused by dsDNA viruses H01417Acute encephalitis Human diseases in ICD-10 classification [BR:b...of the central nervous system G04Encephalitis, myelitis and encephalomyelitis H01417Acute encephalitis Patho...elines for management. Journal Eur J Neurol 12:331-43 (2005) KEGG DISEASE / Acute encephalitis ...

  20. Clinical Analysis of Early Diagnosis of Atypical Kawasaki Disease%早期诊断不典型川崎病的临床分析

    Institute of Scientific and Technical Information of China (English)

    张利

    2013-01-01

    Objective:To research the early clinical diagnosis method of atypical kawasaki disease. Methods:The clinical datas of 22 children with atypical kawasaki disease were retrospectively analyzed, and compared with the diagnosis standard of kawasaki disease. The pathogenic factors, clinical symptoms, pathological examination and symptomatic treatment were summed up. Results:All sick children had fever symptom, and had 2~3 items of typical kawasaki disease diagnosis standard. At the same time, 14 cases had 3 items, which accounted for 63.6%;8 cases had 2 items, which accounted for 36.4%;Among them, 20 patients (90.9%) had skin rash symptoms, which accounted the top three. 16 cases had fingers peeling, accounted for 72.7%, and 15 cases (68.2%) had myrica rubra tongue, lips red, dry and cracked;The top three of pathologic examination in children:22 cases coronary artery expansion, accounting 100%, 21 cases (95.5%) showed high white blood cell count, and high CRP in 18 cases, accounting for 81.8%. Among them, the earliest was characterized by high WBC count and increased ESR. Conclusions:Atypical kawasaki disease due to the onset of symptoms is not obvious, easy to be missed diagnosis or misdiagnosis. Atypical kawasaki disease shall comply with 2~3 items of clinical diagnosis standards of kawasaki disease, and can be confirmed by combining the pathological mri and echocardiography comprehensive diagnosis.%目的:探讨不典型川崎病的早期临床诊断方法。方法:回顾性分析22例不典型川崎病患儿的临床资料,与川崎病的诊断标准进行比较,总结其发病情况、临床症状、病理检查和对症治疗特点。结果:患儿均有发热症状,具备典型川崎病诊断标准的2~3项,同时具备3项的有14例,占63.6%,具备2项的有8例,占36.4%;其中,前三位症状为皮疹20例,占90.9%,指端脱皮16例,占72.7%,杨梅舌、口唇发红、干燥并皲裂15例,占68.2%;患儿病理检查异常前三位

  1. Lack of Association between CLEC5A Gene Single-Nucleotide Polymorphisms and Kawasaki Disease in Taiwanese Children

    Directory of Open Access Journals (Sweden)

    Ya-Ling Yang

    2012-01-01

    Full Text Available Background. Kawasaki disease is characterized by systemic vasculitis of unknown etiology. Previous genetic studies have identified certain candidate genes associated with susceptibility to KD and coronary artery lesions. Host innate immune response factors are involved in modulating the disease outcome. The aim of this study was to investigate CLEC5A (C-type lectin domain family 5 genetic polymorphisms with regards to the susceptibility and outcome of KD. Methods. A total of 1045 subjects (381 KD patients and 664 controls were enrolled to identify 4 tagging single-nucleotide polymorphisms (tSNPs of CLEC5A (rs1285968, rs11770855, rs1285935, rs1285933 by using the TaqMan Allelic Discrimination Assay. The Hardy-Weinberg equilibrium was assessed in cases and controls, and genetic effects were evaluated by the chi-square test. Results. No significant associations were noted between the genotypes and allele frequency of the 4 CLEC5A tSNPs between controls and patients. In the patients, polymorphisms of CLEC5A showed no significant association with coronary artery lesion formation and intravenous immunoglobulin treatment response. Conclusions. This study showed for the first time that polymorphisms of CLEC5A are not associated with susceptibility to KD, coronary artery lesion formation, and intravenous immunoglobulin treatment response in a Taiwanese population.

  2. On What Day of Illness Does the Dilatation of Coronary Arteries in Patients With Kawasaki Disease Begin?

    Science.gov (United States)

    Fuse, Shigeto; Mori, Toshihiko; Kuroiwa, Yuki; Hirakawa, Satoshi

    2017-08-25

    In the present study we used echocardiography to investigate coronary artery diameter at the time of diagnosis of Kawasaki disease (KD), before the start of treatment.Methods and Results:Diameters of the right, left main, left anterior descending, and left circumflex coronary arteries were determined in 410 patients before KD treatment commenced. The maximum Z-score was considered to be the pretreatment, maximum coronary artery Z-score (preZmax). The cumulative probability of coronary arterial dilatation was analyzed using the Kaplan-Meier method. In the present study, 31 patients (7.6%) had a preZmax ≥3.0, 56 (13.7%) had a preZmax ≥2.5, and 96 (23.4%) had a preZmax ≥2.0. The cumulative probability of a preZmax ≥2.0 was >20% on Day 5 of illness, 40% on Day 7, and 70% on Day 10. The positive predictive value (PPV) of a preZmax of 2.0 was approximately 0.9 on Day 5 of illness. The present study demonstrates that the coronary arteries may dilate before Day 5 of illness, and that the rate of dilatation increases gradually until Day 10. Because preZmax 2.0 has high PPV after Day 5 of illness, it is a useful marker of coronary artery dilatation in the early phase of KD.

  3. The roles of Ca2+/NFAT signaling genes in Kawasaki disease: single- and multiple-risk genetic variants.

    Science.gov (United States)

    Wang, Wei; Lou, Jiao; Zhong, Rong; Qi, Yan-qi; Shen, Na; Lu, Xu-zai; Wang, Yu-jia; Zhang, Qing; Zou, Li; Duan, Jia-yu; Ke, Jun-tao; Miao, Xiao-ping; Gong, Fang-qi

    2014-06-06

    Ca(2+)/nuclear factor of activated T-cells (Ca(2+)/NFAT) signaling pathway may play a crucial role in Kawasaki disease (KD). We investigated 16 genetic variants, selected by bioinformatics analyses or previous studies, in 7 key genes involved in this pathway in a Chinese population. We observed a significantly or marginally increased KD risk associated with rs2720378 GC + CC genotypes (OR = 1.39, 95% CI = 1.07-1.80, P = 0.014) or rs2069762 AC + CC genotypes (OR = 1.28, 95% CI = 0.98-1.67, P = 0.066), compared with their wild type counterparts. In classification and regression tree analysis, individuals carrying the combined genotypes of rs2720378 GC or CC genotype, rs2069762 CA or CC genotype and rs1561876 AA genotype exhibited the highest KD risk (OR = 2.12, 95% CI = 1.46-3.07, P < 0.001), compared with the lowest risk carriers of rs2720378 GG genotype. Moreover, a significant dose effect was observed among these three variants (Ptrend < 0.001). In conclusion, this study implicates that single- and multiple-risk genetic variants in this pathway might contribute to KD susceptibility. Further studies on more comprehensive single nucleotide polymorphisms, different ethnicities and larger sample sizes are warranted, and the exact biological mechanisms need to be further clarified.

  4. Single-nucleotide polymorphism rs7251246 in ITPKC is associated with susceptibility and coronary artery lesions in Kawasaki disease.

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    Ho-Chang Kuo

    Full Text Available Kawasaki disease (KD is a multi-systemic vasculitis that preferentially affects children. A single nucleotide polymorphism (SNP in inositol 1,4,5-trisphosphate 3-kinase C (ITPKC has been identified to be an important polymorphism in the risk of KD. This study was conducted to comprehensively investigate the associations between all tagging SNPs of ITPKC in the risk of KD in a Taiwanese population. A total of 950 subjects (381 KD patients and 569 controls were recruited. Seven tagging SNPs (rs11673492, rs7257602, rs7251246, rs890934, rs10420685, rs2607420, rs2290692 were selected for TaqMan allelic discrimination assay. Clinical data of coronary artery lesions (CAL and aneurysms were collected for analysis. A significant association was found between rs7251246 in ITPKC and CAL formation. Haplotype analysis for ITPKC polymorphisms also confirmed this association in the patients with CAL and aneurysm formation. This is the first study to identify that SNP rs7251246 in ITPKC is associated with the severity of KD.

  5. Disruption of Endothelial Cell Homeostasis Plays a Key Role in the Early Pathogenesis of Coronary Artery Abnormalities in Kawasaki Disease

    Science.gov (United States)

    Ueno, Kentaro; Ninomiya, Yumiko; Hazeki, Daisuke; Masuda, Kiminori; Nomura, Yuichi; Kawano, Yoshifumi

    2017-01-01

    Disruption of endothelial cell homeostasis may be associated with the pathogenesis of coronary artery abnormalities (CAA) in Kawasaki disease (KD). We sought to clarify the poorly understood pathogenic role of endothelial cell survival and death in KD vasculitis. Human umbilical vein endothelial cells (HUVECs) stimulated with sera from KD patients, compared with sera from patients with bacterial infections, exhibited significant increases in cytotoxicity, high mobility group box protein 1 (HMGB-1), and caspase-3/7 and a decrease in phosphorylated Akt/Akt (pAkt/Akt) ratios. HUVECs stimulated with sera from KD patients treated with immunoglobulin (IG) showed significantly decreased cytotoxicity, HMGB-1, and caspase-3/7 levels and increased pAkt/Akt ratios, as compared with results for untreated HUVECs (P < 0.001, P = 0.008, P = 0.040, and P < 0.001, respectively). In HUVECs stimulated with sera from KD patients, the increased cytotoxicity levels and the suppression of increased pAkt/Akt ratios after subsequent IG treatment were closely related to the development of CAA (P = 0.002 and P = 0.035). Our data reveal that shifting the balance toward cell death rather than survival appears to perturb endothelial cell homeostasis and is closely related to the development of CAA. The cytoprotective effects of IG treatment appear to ameliorate endothelial cell homeostasis. PMID:28255175

  6. Marked Acceleration of Atherosclerosis following Lactobacillus casei induced Coronary Arteritis in a Mouse Model of Kawasaki Disease

    Science.gov (United States)

    Chen, Shuang; Lee, Young Ho; Crother, Timothy R.; Fishbein, Michael; Zhang, Wenxuan; Yilmaz, Atilla; Shimada, Kenichi; Schulte, Danica J; Lehman, Thomas J.A.; Shah, Prediman K.; Arditi, Moshe

    2012-01-01

    Objective To investigate if Lactobacillus casei cell wall extract (LCWE)-induced Kawasaki Disease (KD) accelerates atherosclerosis in hypercholesterolemic mice. Method and Resuslts Apoe−/− or Ldlr−/− mice were injected with LCWE (KD mice) or PBS, fed high fat diet for 8 weeks, and atherosclerotic lesions in aortic sinuses (AS), arch (AC) and whole aorta were assessed. KD mice had larger, more complex aortic lesions with abundant collagen, and both extracellular and intracellular lipid and foam cells, compared to lesions in control mice despite similar cholesterol levels. Both Apoe−/− KD and Ldlr−/− KD mice showed dramatic acceleration in atherosclerosis vs. controls, with increases in en face aortic atherosclerosis and plaque size in both the AS and AC plaques. Accelerated atherosclerosis was associated with increased circulating IL-12p40, IFN-γ, TNF-α, and increased macrophage, DC, and T cell recruitment in lesions. Furthermore, daily injections of the IL-1Ra, which inhibits LCWE induced KD vasculitis, prevented the acceleration of atherosclerosis. Conclusions Our results suggest an important pathophysiologic link between coronary arteritis/vasculitis in the KD mouse model and subsequent atherosclerotic acceleration, supporting the concept that a similar relation may also be present in KD patients. These results also suggest that KD in childhood may predispose to accelerated and early atherosclerosis as adults. PMID:22628430

  7. The baby Kawasaki disease 27 example nursing intervention%婴儿川崎病的27例护理干预

    Institute of Scientific and Technical Information of China (English)

    李智军

    2014-01-01

    目的:探讨婴儿川崎病的护理干预措施和效果。方法选择婴儿川崎病的患者27例进行分析,随机分为对照组14例患者实施常规的护理措施,观察组13例患者实施综合护理措施,比较两组患者满意度以及疾病知晓率。结果观察组患者满意度以及疾病知晓率明显优于对照组,经比较有显著差异有统计学意义(P <0.05)。结论婴儿川崎病实施综合护理措施能够提高医治效果,促进患儿的健康康复。%Objective to investigate the nursing baby Kawasaki disease.Method the choice of baby Kawasaki disease in 27 patients were analyzed,divided into the control group 14 cases the implementation of routine nursing measures, divided into the observation group of 13 cases of comprehensive nursing measures, compared two groups of patients satisfaction and awareness rate, disease. Results the rate of observation group was better than control group awareness of patient satisfaction and disease, the difference has statistical significance (P < 0.05). Conclusion the baby Kawasaki disease comprehensive nursing measures can enhance the treatment effect, promote the rehabilitation of children with health.

  8. KEGG DISEASE / Acute alcohol sensitivity [KEGG DISEASE

    Lifescience Database Archive (English)

    Full Text Available DISEASE: H01071 Entry H01071Disease Name Acute alcohol sensitivity Description Alde...bolism Congenital disorders of carbohydrate metabolism H01071Acute alcohol sensit...eases. Journal Cardiovasc Res 88:51-7 (2010) KEGG DISEASE / Acute alcohol sensitivity ...

  9. Whole genome sequencing of an African American family highlights toll like receptor 6 variants in Kawasaki disease susceptibility

    Science.gov (United States)

    Veeraraghavan, Narayanan; Levy, Eric; Ribeiro dos Santos, Andre M.; Yang, Hai; Hibberd, Martin L.; Tremoulet, Adriana H.; Harismendy, Olivier; Ohno-Machado, Lucila; Burns, Jane C.

    2017-01-01

    Kawasaki disease (KD) is the most common acquired pediatric heart disease. We analyzed Whole Genome Sequences (WGS) from a 6-member African American family in which KD affected two of four children. We sought rare, potentially causative genotypes by sequentially applying the following WGS filters: sequence quality scores, inheritance model (recessive homozygous and compound heterozygous), predicted deleteriousness, allele frequency, genes in KD-associated pathways or with significant associations in published KD genome-wide association studies (GWAS), and with differential expression in KD blood transcriptomes. Biologically plausible genotypes were identified in twelve variants in six genes in the two affected children. The affected siblings were compound heterozygous for the rare variants p.Leu194Pro and p.Arg247Lys in Toll-like receptor 6 (TLR6), which affect TLR6 signaling. The affected children were also homozygous for three common, linked (r2 = 1) intronic single nucleotide variants (SNVs) in TLR6 (rs56245262, rs56083757 and rs7669329), that have previously shown association with KD in cohorts of European descent. Using transcriptome data from pre-treatment whole blood of KD subjects (n = 146), expression quantitative trait loci (eQTL) analyses were performed. Subjects homozygous for the intronic risk allele (A allele of TLR6 rs56245262) had differential expression of Interleukin-6 (IL-6) as a function of genotype (p = 0.0007) and a higher erythrocyte sedimentation rate at diagnosis. TLR6 plays an important role in pathogen-associated molecular pattern recognition, and sequence variations may affect binding affinities that in turn influence KD susceptibility. This integrative genomic approach illustrates how the analysis of WGS in multiplex families with a complex genetic disease allows examination of both the common disease–common variant and common disease–rare variant hypotheses. PMID:28151979

  10. 远程护理干预在川崎病患儿康复中的应用%Remote nursing intervention for pediatric Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    张玲芝

    2016-01-01

    Objective To investigate the effect of remote nursing intervention for children with Kawasaki disease.Methods 70 children with Kawasaki disease treated at our hospital from January,2015 to June,2016 were selected as study objects and were randomly divided into a control group and an observation group according to hospitalization order,35 for each group.The control group received routine discharge health education;in addition,the observation group were intervened with remote nursing care through phone,email,and QQ group.The nursing effect and the health knowledge about kawasaki disease commanded by their parents were compared between these two groups.Results The rates of regular medication and subsequent visits were 97.1% and 91.4% in the observation group and were 65.7% and 57.1% in the control group (P<0.05).The readmission rate and the incidence of complications were 5.7% and 2.9% in the observation group and were 22.9% and 20.0% in the control group (P<0.05).The health knowledge about kawasaki disease commanded by their parents was better in the observation group than in the control group (P<0.05).Conclusions Remote nursing intervention could improve the nursing effect on Kawasaki disease and the health knowledge about kawasaki disease commanded by their parents,promote the children's rehabilitation,and reduce the readmission rate and the incidence of complications.%目的 探讨远程护理干预在川崎病出院患儿中的应用效果.方法 选择2015年1月至2016年4月在本院治疗的川崎病患儿70例作为研究对象,按就诊顺序随机分为对照组和观察组,各45例.对照组进行常规出院健康宣教,观察组在对照组基础上通过电话、邮件和QQ群相结合的方式对患儿实施远程护理干预,比较干预后两组患儿的护理效果和家长对疾病健康知识的掌握情况.结果 观察组患儿的规律服药率和按时复诊率分别为97.1%和91.4%,显著优于对照组的65.7

  11. Long-Term Prognosis for Patients with Kawasaki Disease Complicated by Large Coronary Aneurysm (diameter ≥6 mm)

    Science.gov (United States)

    Bang, Ji Seok; Kwon, Bo Sang; Song, Mi Kyung; An, Hyo Soon; Song, Young Whan; Bae, Eun Jung; Noh, Chung Il

    2017-01-01

    Background and Objectives Some patients with Kawasaki disease (KD) develop large coronary aneurysms and subsequent coronary stenosis or obstruction, leading to ischemic heart disease. This study examined the long-term outcomes of patients with KD complicated by large coronary aneurysms. Subjects and Methods The medical records of 71 patients (53 men and 18 women) diagnosed with large coronary aneurysms (diameter ≥6 mm) between December 1986 and December 2013 were retrospectively reviewed from our institutional database. Results The mean age at onset was 4.6±3.3 years, and the mean follow-up duration was 12.5±6.9 years. Maximum coronary artery internal diameter ranged from 6.1 to 25 mm. Giant coronary aneurysms occurred in 48 patients and coronary aneurysms 6-8 mm in diameter developed in 23 patients. Coronary stenosis and/or complete occlusion occurred in 30 patients (42.3%). Catheter and/or surgical interventions (mean: 1.5 interventions, range: 1-5 interventions) were performed in 20 patients (28.2%), 9 months to 18 years after KD onset, resulting in 33.7% cumulative coronary intervention rates at 20 years after onset. There were no differences in cumulative coronary intervention rates between two coronary aneurysm groups (6-8 mm vs. ≥8 mm). Myocardial infarction occurred in 7 patients with a giant aneurysm and there was one death. Conclusions Long-term survival of patients with KD complicated by large coronary aneurysm was good even though 28.2% of patients underwent multiple catheter or surgical interventions. Careful follow-up is also necessary in KD patients with coronary aneurysms 6-8 mm in diameter, such as those with giant aneurysms. PMID:28765744

  12. Association between GRIN3A gene polymorphism in Kawasaki disease and coronary artery aneurysms in Taiwanese children.

    Directory of Open Access Journals (Sweden)

    Ying-Ju Lin

    Full Text Available Kawasaki disease (KD is pediatric systemic vasculitis with the classic complication of coronary artery aneurysm (CAA. It is the leading cause of acquired cardiovascular diseases in children. Some severe cases present with multi-organ involvement or neurological dysfunction. To identify the role of the glutamate receptor, ionotropic, N-methyl-d-aspartate 3A (GRIN3A in KD, we investigated genetic variations in GRIN3A in a Taiwanese cohort of 262 KD patients (76 with and 186 without CAA complications. We used univariate and multivariate regression analyses to identify the associations between clinical characteristics and GRIN3A genetic variations in KD. According to univariate regression analysis, CAA formation in KD was significantly associated with fever duration (p < 0.0001, first Intravenous immunoglobulin (IVIG used (days after day one of fever (p < 0.0001, and the GRIN3A (rs7849782 genetic variant (p < 0.001. KD patients with GG+GC genotype showed a lower rate of developing CAA (GG+GC genotype: odds ratio = 0.26; 95% CI = 0.14-0.46. Significant associations were identified between KD with CAA complication and the GRIN3A (rs7849782 genetic variant by using multivariate regression analysis. Specifically, significant correlations were observed between KD with CAA complications and the presence of GG+GC genotypes for the GRIN3A rs7849782 single-nucleotide polymorphism (full model: odds ratio = 0.25; 95% CI = 0.14-0.46. Our results suggest that a polymorphism of the GRIN3A gene may play a role in KD pathogenesis.

  13. Reevaluation of the Efficacy of Intravenous Gammaglobulin in the Prevention and Treatment of Coronary Artery Lesion in Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    QIN Lijun; SAUMU Tobbi Mweri; WANG Hongwei; SHI Hong; HU Xiufen; CHENG Peixuan

    2005-01-01

    Summary: In order to objectively evaluate the efficacy of intravenous gammaglobulin (IVIG) in the prevention and treatment of coronary artery lesion (CAL) in Kawasaki disease (KD) and the related factors influencing the IVIG efficacy, 314 children with KD were reviewed retrospectively and comparatively and were divided into IVIG plus aspirin group and ASA group. The occurrence and restoration of CAL in these two groups as well as many laboratory and clinical indexes including average hospital stay (days), total fever duration, defervescence time, platelet count, erythrocyte sedimentation rate, C reactive protein etc. were observed. The incidence of CAL was 39.5 % in the children with KD. In the IVIG+ASA group, the incidence of CAL was 34.3 % and 56.0 % in ASA group respectively (P10 days (P<0.05). About 13.4 % of the CAL treated with IVIG was not recovered at the 12 th month of the course, mostly in the groups in which only ASA was administered and IVIG treatment was started 10 days later. The hospital stay (days), defervescence time, total fever duration, platelet count, erythrocyte sedimentation rate and C reactive protein were significantly reduced in IVIG+ASA group as compared with those in the ASA group (P<0.05). IVIG treatment can remarkably shorten the course of patients with KD and decrease the incidence of CAL, but the efficacy of IVIG in the prevention and treatment of KD disease is not as expected by people, therefore, reevaluation of the practical efficacy of IVIG is required.

  14. Clarithromycin Plus Intravenous Immunoglobulin Therapy Can Reduce the Relapse Rate of Kawasaki Disease: A Phase 2, Open-Label, Randomized Control Study.

    Science.gov (United States)

    Nanishi, Etsuro; Nishio, Hisanori; Takada, Hidetoshi; Yamamura, Kenichiro; Fukazawa, Mitsuharu; Furuno, Kenji; Mizuno, Yumi; Saigo, Kenjiro; Kadoya, Ryo; Ohbuchi, Noriko; Onoe, Yasuhiro; Yamashita, Hironori; Nakayama, Hideki; Hara, Takuya; Ohno, Takuro; Takahashi, Yasuhiko; Hatae, Ken; Harada, Tatsuo; Shimose, Takayuki; Kishimoto, Junji; Ohga, Shouichi; Hara, Toshiro

    2017-07-06

    We previously reported that biofilms and innate immunity contribute to the pathogenesis of Kawasaki disease. Therefore, we aimed to assess the efficacy of clarithromycin, an antibiofilm agent, in patients with Kawasaki disease. We conducted an open-label, multicenter, randomized, phase 2 trial at 8 hospitals in Japan. Eligible patients included children aged between 4 months and 5 years who were enrolled between days 4 and 8 of illness. Participants were randomly allocated to receive either intravenous immunoglobulin (IVIG) or IVIG plus clarithromycin. The primary end point was the duration of fever after the initiation of IVIG treatment. Eighty-one eligible patients were randomized. The duration of the fever did not differ between the 2 groups (mean±SD, 34.3±32.4 and 31.1±31.1 hours in the IVIG plus clarithromycin group and the IVIG group, respectively [P=0.66]). The relapse rate of patients in the IVIG plus clarithromycin group was significantly lower than that in the IVIG group (12.5% versus 30.8%, P=0.046). No serious adverse events occurred during the study period. In a post hoc analysis, the patients in the IVIG plus clarithromycin group required significantly shorter mean lengths of hospital stays than those in the IVIG group (8.9 days versus 10.3 days, P=0.049). Although IVIG plus clarithromycin therapy failed to shorten the duration of fever, it reduced the relapse rate and shortened the duration of hospitalization in patients with Kawasaki disease. URL: http://www.umin.ac.jp/ctr/index.htm. Unique identifier: UMIN000015437. © 2017 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley.

  15. Analysis of the Clinical Nursing Experience of Children Kawasaki disease%浅谈小儿川崎病的临床护理体会

    Institute of Scientific and Technical Information of China (English)

    李利阳

    2015-01-01

    Kawasaki disease (KD), also known as Mucocutaneous lymph node syndrome, mostly in infants and young children, coronary artery lesion which it caused may lead to Ischemic heart disease in children, severe cases of coronary artery rupture, infarction and sudden death. This paper through to the clinical nursing of 45 cases of children , the clinical nursing care of children with Kawasaki disease system research, improved the clinical effect of treatment and care of children, reduce the harm to the children with the disease.%川崎病(KD)又称皮肤黏膜淋巴结综合征,多见于婴幼儿,它引起的冠状动脉损害可导致儿童缺血性心脏病,严重者冠状动脉破裂、梗塞而猝死。本文通过对45例患儿的治疗护理,对小儿川崎病的临床护理进行系统的研究,提高了对患儿的临床治疗和护理效果,减少该病对患儿的伤害。

  16. Granulocyte colony-stimulating factor ameliorates coronary artery elastin breakdown in a mouse model of Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    Liu Junfeng; Chen Zhi; Du Zhongdong; Lu Dunxiang

    2014-01-01

    Background Coronary artery damage from Kawasaki disease (KD) is closely linked to the dysfunction of the endothelial progenitor cells (EPCs).The aim of the present study was to evaluate the modulatory effect of granulocyte colony stimulating factor (G-CSF) on EPCs and elastin breakdown of coronary arteries in a KD mouse model.Methods A Lactobacillus casei cell wall extract (LCWE)-induced KD model was established in C57BL/6 mice that were subsequently administrated with recombinant human G-CSF (rhG-CSF).Nω-nitro-L-arginine methyl ester (L-NAME) was administrated for the negative intervention.Evaluations included coronary artery lesions,EPC number and functions,and the plasma concentration of nitric oxide (NO).Results Elastin breakdown was found in the coronary arteries of model mice 56 days after injection of LCWE.The number of circulating EPCs,plasma concentration of NO,and functions of bone marrow EPCs,including proliferation,adhesion,and migration abilities,were all lower in the KD model group compared with those in the control group.After administration of rhG-CSF,the number of circulating EPCs and plasma concentration of NO were increased significantly compared with those in the KD model group.There were also increases in the functional indexes of EPCs.Furthermore,rhG-CSF administration improved the elastin breakdown effectively.However,these protective effects of rhG-CSF on coronary arteries were attenuated by L-NAME.Conclusion The present study indicated that the administration of G-CSF prevents elastin breakdown of the coronary arteries by enhancing the number and functions of EPCs via the NO system,and then accelerates the repair of coronary artery lesions in the KD.

  17. High sensitivity C-reactive protein and endothelial function in Chilean patients with history of Kawasaki disease.

    Science.gov (United States)

    Borzutzky, Arturo; Gutiérrez, Miguel; Talesnik, Eduardo; Godoy, Iván; Kraus, Jonathan; Hoyos, Rodrigo; Arnaiz, Pilar; Acevedo, Mónica

    2008-07-01

    Kawasaki disease (KD) produces endothelial inflammation, which may lead to dilatation and aneurysms of coronary and peripheral arteries. Previous studies have suggested that these patients can present endothelial dysfunction that can predispose to coronary vascular events late after KD. The purpose of this study was to determine the cardiovascular risk profile and endothelial function of Chilean children with history of KD. In a prospective case-control study, 11 patients with history of KD (age 10.6 +/- 2.0 years, interval from initial episode 8.1 +/- 3.6 years) and 11 healthy, age-, gender-, and BMI z score-matched controls were evaluated with blood pressure (BP), a fasting lipid profile, high sensitivity C-reactive protein (hsCRP), and flow-mediated dilatation of the brachial artery (FMD). One KD patient (9.1%) had persistent coronary aneurysms. There was a significant difference of mean and log-transformed concentrations of hsCRP between case and control groups (2.3 +/- 3.0 vs 0.5 +/- 0.3 mg/l, P = 0.045). None of the patients with elevated hsCRP had persistent coronary arterial lesions. No difference was found in systolic BP z score between the case and control groups. Diastolic BP z score was significantly higher in cases than controls (P = 0.039). There were no significant differences of FMD between cases and controls. Mean fasting total cholesterol, high-density and low-density lipoprotein, and triglycerides in cases were normal, with no significant difference vs controls. This study shows that Chilean children with history of KD have increased levels of hsCRP, possibly reflecting persistent low-grade inflammation. The prognostic value of hsCRP in KD patients deserves further investigation.

  18. Effect of pravastatin on endothelial dysfunction in children with medium to giant coronary aneurysms due to Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    Chao Duan; Zhong-Dong Du; Yu Wang; Li-Qun Jia

    2014-01-01

    Background: Ongoing low-grade inflammation and endothelial dysfunction persist in children with coronary lesions diagnosed with Kawasaki disease (KD). Statins, frequently used in the management of high cholesterol, have also shown to improve surrogate markers of infl ammation and endothelial dysfunction. This study was undertaken to investigate the effi cacy and safety of pravastatin in children with coronary artery aneurysms due to KD. Methods: The study enrolled 14 healthy children and 13 male children, aged 2-10 years, with medium-to-giant coronary aneurysms for at least 12 months after the onset of KD. Pravastatin was given orally to the KD group at a dose of 5 mg/day for children under 5 and 10 mg/day for children older than 5 years. To determine the effects of pravastatin on endothelial function, high-frequency ultrasound was performed before the start of the study and 6 months after pravastatin therapy. The parameters measured were brachial artery flow-mediated dilation (FMD), non-flow mediated dilation (NMD), and carotid artery stiffness index (SI). High sensitive C-reactive protein (hs-CRP) levels, the circulating endothelial progenitor cells (EPCs) number, and serum lipid profiles were also determined at baseline and after 6 months of pravastatin treatment. Results: Before treatment, the KD group had significantly decreased FMD (P0.05). No signifi cant complications were noted with paravastatin therapy. Conclusions: Pravastatin improves endothelial function and reduces low-grade chronic infl ammation in patients with coronary aneurysms due to KD. Children with coronary aneurysms due to KD may benefit from statin therapy.

  19. 影像学技术在川崎病动物实验中的应用%Imaging technology used in the animal models of Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    苏新星; 范晓晨

    2013-01-01

    川崎病是儿科常见的自身免疫性疾病,可引起全身性血管炎,并好发于冠状动脉,从而造成严重的并发症冠状动脉损害,是构成儿童后天性心脏病的主要病因之一.其病因至今尚不明确,临床研究其发病机制也存在诸多限制,所以,川崎病动物实验的开展对于疾病的诊治具有重要的意义,现就相关影像学技术在动物实验中所起的作用作一简单综述.%Kawasaki disease ( KD ) is a multisystem vasculitis and the leading cause of acquired heart disease in children of the world, but its etiology and pathophysiological mechanism is unknown. Many animal models have been studied in order to investigate further its mechanism and the potential factors of coronary artery lesions. This article summarizes some imaging technology used in research into the animal models of Kawasaki disease.

  20. Incidence Rate and Epidemiological and Clinical Aspects of Kawasaki Disease in Children of Maghrebi Origin in the Province of Quebec, Canada, Compared to the Country of Origin

    Directory of Open Access Journals (Sweden)

    Arbia Abir Gorrab MD

    2016-02-01

    Full Text Available The incidence of Kawasaki disease in Maghreb countries is apparently low, unlike those living in the province of Quebec, Canada. This retrospective study compared Maghrebi children living in Quebec to the countries of origin, Morocco, Algeria, and Tunisia. The annualized incidence rate in Quebec (18.49/year/100 000 children under 5 years of age was 4 to 12 times higher than in Tunisia, Morocco, and Algeria (0.95, 4.52, and 3.15, respectively. The prevalence of incomplete diagnostic criteria was higher in Quebec at 39%, Morocco 43%, and Tunisia 39% compared to Algeria at 8%, with minimal delayed diagnosis (7% only in Quebec compared to 30%, 35%, and 62%, respectively (P < .001. The rate of coronary aneurysms was comparable however (11% in Quebec vs 4%, 10%, and 25%, in Tunisia, Morocco, and Algeria, respectively; P = .31. The higher incidence of Kawasaki disease in the Maghreb community in Quebec versus the countries of origin seems due to underdiagnosis, which represents a public health concern in those countries.

  1. 不完全型川崎病的临床特点分析%Analysis of clinical characteristics in patients with incomplete Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    李丹

    2014-01-01

    Objective:To explore clinical characteristics of patients with incomplete Kawasaki disease (KD). Methods:The medical records of 110 KD patients were reviewed retrospectively. Results:110 KD patients, 32 ( 29%) had incomplete KD. Com-pared with KD, the patients with incomplete KD were much smaller, the diagnosis was made later, the main clinical characteristics were less, and coronary artery lesions were more common (P<0. 05). Conclusions:Incomplete KD has the same clinical characteris-tics with classic KD, but is more difficult to diagnosis resulting in delayed treatment and a greater risk of coronary lesions.%目的:探讨不完全型川崎病( Kawasaki disease, KD)的临床特点。方法:回顾性分析110例诊断为KD患者的临床资料。结果:不完全型KD患者32例,占29%。与KD相比较,不完全型KD患者年龄更小,诊断延迟,主要临床表现较少,冠脉扩张发生率高( P<0.05)。结论:不完全型KD和典型KD有相似的临床特征,易贻误治疗,加大了冠脉损伤的风险。

  2. 不完全性川崎病的临床特征、早期诊断及治疗%Clinical Features,Early Diagnosis and Treatment of Incomplete Kawasaki Disease in Children

    Institute of Scientific and Technical Information of China (English)

    余莉; 王一斌; 乔莉娜; 华益民; 朱琦; 石晓青; 周开宇; 刘瀚旻

    2015-01-01

    Objective To explore the clinical features, early diagnosis and treatment of incomplete Kawasaki disease ( iKD) in children in order to early recognition and diagnosis of iKDs. Methods Retrospectively study on children with KD( Ka-wasaki disease)including 174 cases of iKD patients and 317 cases of cKD(complete Kawasaki disease)patients admitted in the West China Second University hospital from May,2011 to April,2014. The sex,age,clinical features,laboratory tests in acute phase,IVIG( intravenous immunoglobulin) treatment and CALs( coronary artery lesions) were analysed between the iKDsand cKDs, respectively. Results ①There were no significant differences in sex and age between iKDs and cKDs(P>0. 05),but the inci-dences of exanthema,bilateral bulbar conjunctival injection,oral and pharyngeal hyperemia,strawberry tongue,erythema of palms and soles,cervical lymphadenopathy in iKDs were lower than in cKDs(P 0. 05).③The time of diagnosis in iKDswere later than in cKDs. There were no significant difference in the incidence of CALs,response of IVIG and duration of fever betweeniKDs and cKDs(P>0. 05). Conclusion The incidences of typical clinical symptoms of KD in iKDswere lower than in cKDs. These la-boratory tests including WBC,Hct,ESR,AST,ALT,PA,HDL,sterile pyuria are help for diagnosis of iKDs. The CALs caused by iKDs were decreased by using of IVIG within 10 days after initial fever.%目的:探讨不完全性川崎病的临床特征、诊断及治疗,为临床医生识别和早期诊断提供依据。方法回顾性分析2011年4月至2014年3月在我医院住院治疗的川崎病( Kawasaki disease,KD)患者临床资料,对174例不完全性川崎病( incomplete Kawasaki disease,iKD)及317例完全性川崎病( complete Kawasaki disease,cKD)的性别、发病年龄、临床表现、急性期实验室检查、IVIG( intravenous immunoglobulin)治疗及冠状动脉损伤( coronary artery lesions,CALs)进行比较。结果①iKD与cKD患者性别及年

  3. 丙种球蛋白无反应型川崎病小儿临床特征分析%Clinical features of intravenous immunoglobulin-resistant Kawasaki disease in children

    Institute of Scientific and Technical Information of China (English)

    何兵; 杨锦艳; 李小欧; 金红芳

    2013-01-01

    Objective The present study was designed to analyse the clinical features of intravenous immunoglobulin-resistant Kawasaki disease in children. Method A retrospective analysis of 102 hospitalized children with Kawasaki disease was conducted. Result Compared with those of intravenous immunoglobulin responders of Kawasaki disease cases, children with intravenous immunoglobulin-resistant Kawasaki disease had a prolonged duration of fever, and increased morbidity of coronary artery lesions and ST-T abnormalities in ECG. ST-T abnormalities in ECG before treatment and prolonged duration of fever are risks for intravenous immunoglobulin-resistant Kawasaki disease. Conclusions Children with intravenous immunoglobulin-resistant Kawasaki disease had a longer duration of fever and an increased rate of abnormal ST-T changes in ECG. Children having a longer duration of fever and abnormal ST-T have an increased possibility to have intravenous immunoglobulin-resistant Kawasaki disease.%目的 分析静脉注射丙种球蛋白(IVIG)无反应型川崎病患儿的临床特点及危险因素,并探讨其治疗及预后.方法 对住院治疗的102例川崎病患儿病例进行回顾性分析.结果 与反应组相比,无反应组患儿总发热天数明显延长,冠状动脉病变、心电图ST-T段改变的发生率明显升高.治疗前ST-T段改变和发热天数较长是发生IVIG无反应的危险因素.结论 IVIG无反应型川崎病较IVIG反应型川崎病发热时间延长,更易发生冠状动脉病变及心电图ST-T段改变.治疗前ST-T段改变和发热天数较长的患儿发生IVIG无反应的可能性更高.

  4. The long-term prognosis and follow-up of Kawasaki disease with coronary artery lesions%川崎病合并冠状动脉损害的远期转归与随访

    Institute of Scientific and Technical Information of China (English)

    施婷婷

    2010-01-01

    Kawasaki disease (KD) is an acute, self-limited vasculitis of children and the principal cause of acquired heart disease in children in developed countries. The recent follow-up studies of KD revealed that cardiac sequelae had been observed for many years in a minority of patients with KD. Cardiac sequelae especially the coronary artery aneurysm is the leading cause of ischemic heart disease or even the sudden death.The long-term management of KD was developed in many countries in order to prevent the cardiac events.This paper reviews the long-term prognosis and follow-up of KD.%近年对川崎病的追踪随访发现,大部分患儿预后良好,但仍有少数患儿长期遗留心血管后遗症,可发展为缺血性心脏病,一旦发生心肌梗死,病死率高.因此,美国、日本及我国等均制订了相应的川崎病患儿长期随访方案,应用各种随访手段以观察其冠状动脉损害及转归,预防川崎病引起的心血管意外发生.

  5. Treatment response in Kawasaki disease is associated with sialylation levels of endogenous but not therapeutic intravenous immunoglobulin G.

    Directory of Open Access Journals (Sweden)

    Shohei Ogata

    Full Text Available OBJECTIVES: Although intravenous immunoglobulin (IVIG is highly effective in Kawasaki disease (KD, mechanisms are not understood and 10-20% of patients are treatment-resistant, manifesting a higher rate of coronary artery aneurysms. Murine models suggest that α2-6-linked sialic acid (α2-6Sia content of IVIG is critical for suppressing inflammation. However, pro-inflammatory states also up-regulate endogenous levels of β-galactoside:α2-6 sialyltransferase-I (ST6Gal-I, the enzyme that catalyzes addition of α2-6Sias to N-glycans. We asked whether IVIG failures correlated with levels of α2-6Sia on infused IVIG or on the patient's own endogenous IgG. METHODS: We quantified levels of α2-6Sia in infused IVIG and endogenous IgG from 10 IVIG-responsive and 10 resistant KD subjects using multiple approaches. Transcript levels of ST6GAL1, in patient whole blood and B cell lines were evaluated by RT-PCR. Plasma soluble (sST6Gal-I levels were measured by ELISA. RESULTS: There was no consistent difference in median sialylation levels of infused IVIG between groups. However, α2-6Sia levels in endogenous IgG, ST6GAL1 transcript levels, and ST6Gal-I protein in serum from IVIG-resistant KD subjects were lower than in responsive subjects at both pre-treatment and one-year time points (p <0.001, respectively. CONCLUSIONS: Our data indicate sialylation levels of therapeutic IVIG are unrelated to treatment response in KD. Rather, lower sialylation of endogenous IgG and lower blood levels of ST6GALI mRNA and ST6Gal-I enzyme predict therapy resistance. These differences were stable over time, suggesting a genetic basis. Because IVIG-resistance increases risk of coronary artery aneurysms, our findings have important implications for the identification and treatment of such individuals.

  6. The Validity of a Scoring System in Predicting Intravenous Immunoglobulin Treatment Failure in Children With Kawasaki Disease

    Directory of Open Access Journals (Sweden)

    Nateghian

    2015-12-01

    Full Text Available Background Between 10 and 20% of children with Kawasaki disease (KD will not respond to intravenous immunoglobulin (IVIG treatment, and are prone to coronary abnormalities. A variety of predicting scoring systems, including the Kobayashi system, have been proposed, but have not yet been evaluated using Iranian patients. Objectives To evaluate the Kobayashi scoring system with regard to predicting response to IVIG treatment in Iranian children. Patients and Methods All patients who received a final diagnosis of KD at Aliasghar children’s hospital between 1982 and 2013, and who met the inclusion criteria, were enrolled in this retrospective cohort study. We excluded patients with missing data, abnormal echocardiographic finding on admission, late admission, atypical or afebrile cases, and those who had received an insufficient amount of IVIG. We compared demographic and echocardiographic data before IVIG, and within 7 days of treatment, as well as C reactive protein (CRP, sodium, aspartate aminotransferase, platelet levels, neutrophil percentage, age of patients, and duration of fever before IVIG administration, in treatment responders and non-responders. Results Of the 141 cases, 97 patients met the criteria and were enrolled. Of these, 19 (19.6% did not respond to IVIG. A total of 61.8% of patients were male, and the mean patient age was 36.9 months (SD = 32.1 months. Echocardiographic evaluation revealed early coronary involvement in 15.3% of patients, and coronary abnormalities were diagnosed in 10% of patients within the first 10 days of presentation and concurrent with their IVIG treatment. A between-groups comparison of quantitative CRP, absolute neutrophil count, and platelet count showed that platelet count alone was significantly higher in nonresponders (P = 0.04. With regard to items of Kobayashi scoring system, data were present for just 41 cases, but a significant difference between the two groups was shown, with the treatment

  7. Incidence Rate and Epidemiological and Clinical Aspects of Kawasaki Disease in Children of Maghrebi Origin in the Province of Quebec, Canada, Compared to the Country of Origin.

    Science.gov (United States)

    Gorrab, Arbia Abir; Fournier, Anne; Bouaziz, Asma Abed; Spigelblatt, Linda; Scuccimarri, Rosie; Mrabet, Ali; Dahdah, Nagib

    2016-01-01

    The incidence of Kawasaki disease in Maghreb countries is apparently low, unlike those living in the province of Quebec, Canada. This retrospective study compared Maghrebi children living in Quebec to the countries of origin, Morocco, Algeria, and Tunisia. The annualized incidence rate in Quebec (18.49/year/100 000 children under 5 years of age) was 4 to 12 times higher than in Tunisia, Morocco, and Algeria (0.95, 4.52, and 3.15, respectively). The prevalence of incomplete diagnostic criteria was higher in Quebec at 39%, Morocco 43%, and Tunisia 39% compared to Algeria at 8%, with minimal delayed diagnosis (7%) only in Quebec compared to 30%, 35%, and 62%, respectively (P disease in the Maghreb community in Quebec versus the countries of origin seems due to underdiagnosis, which represents a public health concern in those countries.

  8. Usefulness of 64-slice MDCT for follow-up of young children with coronary artery aneurysm due to Kawasaki disease: Initial experience

    Energy Technology Data Exchange (ETDEWEB)

    Peng Yun [Imaging Center, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China)], E-mail: ppengyun@yahoo.com; Zeng Jinjin [Imaging Center, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China); Du Zhongdong [Pediatric Cardiovascular Department, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China); Sun Guoqiang [Imaging Center, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China); Guo Huling [Pediatric Cardiovascular Department, Beijing Children' s Hospital Affiliated to Capital Medical University, 56, Nanlishi Road, Xicheng District, Beijing 100045 (China)

    2009-03-15

    To evaluate the initial application and value of 64-slice multidetector computed tomography as an alternative diagnostic modality in the follow-up of young children with coronary artery aneurysm due to Kawasaki disease. Twelve boys (mean age 5.1 years, range 1.8-7.8 years) for follow-up (time range from 1.1 to 5.1 years) of known Kawasaki disease and coronary artery aneurysm underwent 64-slice MDCT ECG-gated coronary angiography. All data were acquired without breath holding. Two pediatric radiologists independently assessed image quality and the diameter of all coronary segments were measured for each patient. The number, position, shape and size of each coronary artery aneurysm were observed and compared with those of ECHO performed previously. A total of 118/156 segments permitted visualization with diagnostic image quality, the CT measurements showed good inter-observer and intra-observer reliability, coefficients were 0.93 and 0.88, respectively. A total of 30 coronary artery aneurysms were identified with measured mean of 7.5 {+-} 3.8 mm in diameter, and of 12.4 {+-} 9.1 mm in longitudinal lengths.10 tumors were small, 8 tumors were medium and 12 tumors were giant aneurysm. The affected segments included LM7/12(58.3%), 9/12(75%) of LAD1, 4/12(33.3%) of LAD2, 2/12(16.7%) of LCX1; 6/12(50%) of RCA1, 9/12(75%) of RCA2 and 4/12(33.3%) of RCA3, including affected two segments in 9 tumors and three segments in 1 tumor. Calcifications were found in 5 aneurysms and 3/5 with thrombosis; six stenotic segments were found. ECHO failed to detect 8 tumors with 2/8 in LAD, 1/8 in LCX and 5/8 in RCA, and those included 4 small aneurysms. The use of 64-slice MDCT angiography proved valuable for monitoring young children with Kawasaki disease. However, further study is necessary to specify the sensitivity and specificity of MDCT in the follow-up.

  9. 川崎病再发三次一例报道并文献复习%Kawasaki Disease Recrudesced With Three Times:One Case Report and Literature Review

    Institute of Scientific and Technical Information of China (English)

    史春云; 王建国; 夏明倩; 王冬萌

    2016-01-01

    Objective To investigate the etiology and clinical characteristics of recurrent Kawasaki disease. Methods One child whose Kawasaki disease recrudesced with three times in Children's Hospital of Baoding from March 2011 to August 2014 was analyzed retrospectively,the clinical characteristics and laboratory results were analyzed. Results The Kawasaki disease of this child recrudesced with three times within 2. 5 years. When Kawasaki disease recrudesced,the onset of disease was pyogenic tonsillitis,and causes of infection existed obviously,and WBC,PLT,CRP and ESR of recurrent Kawasaki disease were obviously higher than those of primary disease respectively, but the incidence of liver damage had not changed obviously. When Kawasaki disease recrudesced for the second time and third time,causes of infection were not found,but auxiliary examination results showed obvious disorders in the humoral and cellular immunity. Conclusion The children with primary Kawasaki disease should be followed up,especially when fever occurs,attention should be paid to the typical clinical manifestation of Kawasaki disease and coronary artery lesion,thus recurrent Kawasaki disease can be diagnosed and treated early.%目的:探讨川崎病再发病例的病因及临床特征。方法回顾性分析保定市儿童医院2011年3月—2014年8月川崎病再发3次患儿1例,分析其临床特点及相关实验室检查。结果本例患儿在2年半的时间内川崎病再发3次,川崎病再发时以化脓性扁桃体炎起病,有感染诱因,白细胞计数、血小板计数、C 反应蛋白、红细胞沉降率均较川崎病初发时明显升高,肝功能受损无明显改变。第2次和第3次川崎病再发时均未找到明确感染因素,实验室检查示该患儿体液免疫功能及细胞免疫功能紊乱。结论临床工作中应对川崎病初发患儿加强随访,特别是出现发热等表现时,应注意观察川崎病特征性临床表现及冠状动脉病

  10. 川崎病患儿心型脂肪酸结合蛋白和脑利钠肽的变化及与心功能的关系%Changes of serum heart type fatty acid binding protein and brain natriuretic peptide and their clinical significance in children with Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    王金菊; 陈宗波; 王文棣

    2015-01-01

    Objective To explore the serum levels changes of heart-type fatty acid-binding protein and brain natriuretic peptide in children with Kawasaki disease,as well as their links with the heart function. Methods A total of 53 children with Kawasaki disease admitted to our hospital from February 2008 to March 2012 were selected and at the same time 50 healthy controls were randomly selected. The serum levels for the children in acute phase,recovery period of Kawasaki disease and control group children were determined by en-zyme-linked immnoabsorbent assay. The internal diameter of coronary artery,LVEF,LVSF,CI and E/A of chil-dren were measured by two-dimensional echocardiography. Results The heart-type fatty acid-binding protein and brain natriuretic peptide concentrations of children with Kawasaki disease in acute stage[(18. 23 ± 13. 81) ng/ml,(517. 2 ±213. 2) pg/ml] were higher than those in recovery stage[(6. 29 ± 1. 26) ng/ml,(92. 1 ± 46. 0) pg/ml](P<0. 05). They were also higher for children in acute stage than those of control group[(6. 26 ± 1. 60) ng/ml,(37. 6 ± 7. 6) pg/ml]. The LVEF,LVSF,CI of Kawasaki disease for children in acute stage were lower than those in recovery phase[(60. 3 ± 3. 6)% vs. (64. 8 ± 4. 3)%,(30. 6 ± 2. 5)% vs. (34. 9 ± 1. 9)%,(3. 1 ± 0. 3)% vs. (3. 5 ± 0. 3)%]. Linear correlation analysis showed the heart-type fatty acid-binding protein and brain natriuretic peptide′concentrations in Kawasaki disease were negatively correlated with LVEF (P<0. 05). The heart-type fatty acid-binding protein′positive rates were also higher than brain natriuretic peptide for the children in acute and recovery phase. Conclusion The heart-type fatty acid-binding protein and brain na-triuretic peptide could be used as the biochemical markers for myocardial damage of children with Kawasaki dis-ease. The heart-type fatty acid-binding protein was more significant than brain natriuretic peptide. The heart-type fatty acid-binding protein and brain

  11. ADVANCES IN RESEARCH OF ANIMAL MODEL AND PATHOGENESIS OF KAWASAKI DISEASE%川崎病的动物模型及其发病机制研究进展

    Institute of Scientific and Technical Information of China (English)

    刘潇婷; 朝鲁门

    2016-01-01

    Kawasaki disease has gradually replaced rheumatic fever became one of the major cause of acquired heart disease in children, but its etiology and pathogenesis has been unclear. However, animal model of Kawasaki disease has provided an important foundation of etiology and pathogenesis re-search. Since the Kawasaki disease discovered,Many scholars use kinds of inducers,such as Candida, Lactobacillus casei,bovine serum and horse serum injected to animals such as mice, rabbit dog and swine to build animal model of Kawasaki disease,and use these model for further study on etiology and pathogenesis of Kawasaki disease. This article summarize the existing animal model of Kawasaki disease and its associated evaluation and application.%川崎病已逐渐代替风湿热成为儿童后天性心脏病的主要原因之一,但目前该病的发病诱因以及发病机制尚未明确。建立川崎病的动物模型可为找到川崎病的发病诱因及研究其发病机制提供重要实验基础。自川崎病发现以来,很多学者利用各种诱导剂,如白色念珠菌,干酪乳杆菌,牛血清,马血清等在动物中来诱导产生川崎病动物模型,其中小鼠为研究最多应用最广的动物模型,用这些模型进一步研究川崎病的发病诱因及其发病机制,并取得一定进展。本文将对现有川崎病动物模型及其相关的评价与应用作一综述。

  12. Research of echocardiography on coronary artery lesion of Kawasaki disease%川崎病冠状动脉损害的超声研究现状

    Institute of Scientific and Technical Information of China (English)

    王嘉俊

    2012-01-01

    Coronary artery lesions caused by Kawasaki disease have main effect to its prognosis.The lesions can be showed as arteriitis,arteriectasis,aneurysm and arterial stenosis stenosis.Ultrasonic cardiography is the first choice to detect the lesions,especially in dilatation and aneurysm formation of the coronary artery.It can definitely show the diameter and the degree of dilatation of the coronary artery.With the development of ultrasonic equipments as well as the applications of new technique,ultrasonic cardiography is playing an important role in Kawasaki disease of early diagnosis,therapeutic effect,prognosis estimation and Iongterm follow -up.%川崎病冠状动脉损害是影响患儿预后的主要因素.冠状动脉损害可表现为动脉炎、动脉扩张、动脉瘤和动脉狭窄等.超声心动图检查是检测川崎病并发冠状动脉损害的首选方法,尤其对冠状动脉扩张和冠状动脉瘤形成的诊断具有特异性,能清晰地显示冠状动脉内径及扩张程度.随着超声设备的升级及相关新技术的应用,超声心动图对川崎病的早期诊断、疗效观察、评估预后和长期随访发挥着愈来愈重要的作用.

  13. 不完全川崎病的早期诊断和临床特征%Early Diagnosis and Clinical Features of Incomplete Kawasaki Disease in Children

    Institute of Scientific and Technical Information of China (English)

    罗泽民; 樊映红; 刘德松

    2011-01-01

    Objective To explore the early diagnosis and clinical features of incomplete Kawasaki disease (IKD) in children,in order to promote early diagnosis,reduce or avoid coronary artery lesions (CAL) caused by delayed diagnosis of Kawasaki disease(KD). Methods The clinical features of 42 children with IKD and 147 children with KD collected from Jan. 2000 to Jan. 2010 were retrospectively analyzed.The data between 2 groups in age, gender,clinic features [ such as fever, iymphadenectasis, finger or toe tip desquamation, conjunctival hyperemia, hardness hydropsia, rash, oral mucosa congestion, crissum desquamation, erythema in Bacillus Calmette- Guerin (BCG) inoculation place ], laboratory examinations [ including white blood cell, hemoglobin, platelet, erythrocyte sedimentation rate, C reactive protein( CRP), albumin,alanine aminotransferase and serum sodium] ,heart color Doppler examine and electrocardiogram were analyzed by SPSS 17.0 software. Results There was no difference in gender between IKD group and KD group, but IKD group had younger age of onset and longer persistence time of fever. IKD group had lower incidence of extremity hardness hydropsia, oral mucosa congestion, conjunctival hyperemia and rash in the acute stage. Erythema in BCG inoculation appeared often in KD group. IKD group had higher white blood count and CRP. There was not statistical difference in abnormal electrocardiogram between 2 groups, but IKD group had higher CAL incidence rate. Conclusions IKD children have higher incidence of CAL. Lower onset age, longer persistence of fever, erythema in BCG inoculation place, and obvious WBC and CRP rise are helpful for diagnosis of IKD. As early as possible complete laboratory examination is very important to reduce delayed diagnosis and avoid CAL.%目的 探讨不完全川崎病(IKD)的早期诊断和临床特征,以提高川崎病(KD)的早期诊断率,减少KD延迟诊断及冠状动脉病变(CAL)的发生.方法 对2000年1月-2010年1月收治的42

  14. Analysis on the children with Kawasaki disease combined with severe infection%川崎病合并重症感染患儿的预后分析

    Institute of Scientific and Technical Information of China (English)

    聂抒; 韩燕燕; 韩立志

    2012-01-01

    Objective: To explore the relationship between severe infection and prognosis of Kawasaki disease. Methods; A total of 271 children with Kawasaki disease who were diagnosed and treated in pediatric department of the hospital and got fever within seven days from May 2005 to May 2010 were selected, and all the children conformed to the diagnostic criteria revised on the Third International Kawasaki Disease Symposium. All the children were divided into severe infection group (73 children) and non - severe infection group ( 198 children) according to the score of children with critical illness ( preliminary draft) . Forty - four children with severe pneumonia ( including 17 children with serum Mycoplasma pneumoniae IgM titer^l: 160) , 20 children with viral encephalitis, and 9 children with sepsis were included into severe infection group. Results; There was significant difference in the number of children under one year old between severe infection group and non - severe infection group (P 0. 05) . The numbers of children with platelet 60 mm/h, and C - reactive protein >45 mg/L in severe infection group were significantly higher than those in non -severe infection group (P 0. 05 ) . After treating the children with gamma globulin for 36 hours, the number of children with pyretolysis in severe infection group was less than that in non - severe infection group (x2 = 13. 155, P 0. 05) ; the number of children with coronary arteriectasis within six months in severe infection group was significantly higher than that in non - severe infection group (x2 = 4. 595 , P < 0. 05) . Conclusion; The children with severe infection have high probability of coronary artery lesions, which indicates that severe infection can be used as an early indicator of poor prognosis of Kawasaki disease. The reaction of children with Kawasaki disease combined with severe infection to gamma globulin therapy is poor, and the children are prone to combine with coronary arteriectasis. Thus, the

  15. Clinical Analysis Of Early Diagnosis and Treetment Of 33 Children With incomplete Kawasaki Disease%小儿不完全性川崎病33例早期诊治分析

    Institute of Scientific and Technical Information of China (English)

    王士杰; 李晓春; 程贤高

    2014-01-01

    目的:总结小儿不完全性川崎病临床特点,提高本病早期诊断和治疗水平。方法:回顾分析33例不完全性川崎病患儿的临床和实验室检查资料。结果:患儿均有持续高烧症状,患儿的临床症状均不足4项诊断标准要求,实验室检测值和心脏彩超检查结果均有不同程度的异常改变,丙种球蛋白加阿司匹林治疗有显著疗效。结论:小儿不典型性川崎病特征性临床表现较少,对有持续高热,川崎病典型性临床表现不显著的患儿,应结合实验室检测指标及心脏彩超结果尽早确诊并行免疫球蛋白治疗,以改善患儿预后。%Objective:To analyze the clinical data of incomplete, Kawasaki Disease in children.Methods:The clinical and laboratory data of 33 children with incomplete Kawasaki Disease were retrospectively analyzed. Results:All children had persistent high fever, clinical symptoms of diagnostic standards in children were less than four, The data of echocardiography and laboratory test were abnormal changes in varying degrees, Intravenous immunoglobulin and Aspirin are effective for treatment of incomplete Kawasaki. Conclusion:Children with incomplete Kawasaki dis-ease characteristic clinical manifestation is less, have a fever,the typical clinical features of Kawasaki disease was not significant in children,should be combined with laboratory test index and cardiac ultrasound results,diagnosed early parallel intravenous immunoglobulin treatment to improve prognosis of incomplete Kawasaki Disease.

  16. Significance of platelet parameters and C-reactive protein in Kawasaki disease children%血小板参数和C反应蛋白在川崎病患儿中的变化及意义

    Institute of Scientific and Technical Information of China (English)

    王新宝; 崔红

    2015-01-01

    ABSTRACT:Objective To investigate the clinical significance of platelet parameters and the variation of C reactive protein on the first week onset of Kawasaki disease(before therapy)and two to three weeks (after therapy). Methods 42 cases of children with Kawasaki disease and 30 cases of the healthy children examined at the same period (control group)were enrolled in the study.Blood platelet (PLT),mean platelet volume (MPV),platelet hematocrit (PCT),and platelet distribution width (PDW )and C-reactive protein were detected in these patients.The difference of platelet parameters and C-reactive protein were compared between Kawasaki disease group and control group.These parameters were also compared between coronary artery disease and without coronary artery disease patients.Results At the first week onset of Kawasaki disease,the parameters of PLT,MPV and PDW in Kawasaki disease group were significantly higher than control group.After the administration of treatment,MPV and PDW were lower than those before the treatment.Before treatment no significant differences of platelet parameters and C-reactive protein were found in Kawasaki disease children with absence of coronary artery dissease. However, after administration of treatment,MPV,PCT,PDW and C-reactive protein were lower in the group without coronary atery lesions than those with coronary artery disease in Kawasaki disease children.Conclusion Platelet parameters and C-reactive protein exerted great significance in the pathogenesis of Kawasaki disease in children which might play an important roles in assessing the development and selecting clinical therapy.%目的:探讨川崎病患儿发病第1周(治疗前)、第2~3周(治疗后)血小板参数和 C 反应蛋白的变化及临床意义。方法对42例川崎病患儿(观察组)及30例同期体检健康儿童(对照组)取空腹静脉血测定血小板计数(PLT)、血小板平均体积(MPV)、血小板压积(PCT)、和血小板分布宽度(PDW)及 C 反

  17. Research Progress in Diagnosis and Treatment of Coronary Artery Lesions in Kawasaki Disease%川崎病并发冠状动脉损害的诊断及治疗进展

    Institute of Scientific and Technical Information of China (English)

    吴丹

    2011-01-01

    川崎病是一种主要发生于儿童的非特异性全身性血管炎性综合征,迄今病因未明,病变可累及全身中、小血管,特别是冠状动脉,可发生冠状动脉狭窄、血栓形成,导致缺血性心脏病、心肌梗死及猝死,威胁患儿长期生活质量及寿命,在一些发达国家,该病已超过风湿热成为儿童后天性心脏病的首位病因.在此就川崎病冠状动脉损害的诊断及治疗的最新进展予以综述.%Kawasaki disease is nonspecific systemic vasculitic syndrome that mainly afflicts children.The etiology of Kawasaki remains unknown.The disease involves systemic medium and small blood vessels.It is noted that coronary artery lesions are subject to coronary artery stenosis and thrombosis,further leading to ischemic heart disease, myocardial infarction, and sudden death.Kawasaki disease has been threatening the quality of life and life expectancy of children, whereas in some developed countries, it has been the leading cause of pediatric acquired heart disease, surpassing rheumatic fever.This article reviews the recent progress in the diagnosis and treatment of coronary artery lesion in Kawasaki disease.

  18. Inkomplet Kawasakis syndrom med koronaraneurisme hos et spædbarn

    DEFF Research Database (Denmark)

    Vinding, Rebecca Kofod; Debes, Nanette Mol; Stantchev, Hristo

    2014-01-01

    We report a four-month-old boy with incomplete Kawasaki syndrome. He developed a coronary artery aneurysm. Incomplete Kawasaki syndrome is rare in Denmark, and only reported in five patient cases in the past 25 years. It is difficult to diagnose Kawasaki disease; early diagnosis and treatment...... reduces the risk for cardiac sequel. In lack of diagnostic test, we recommend that the algorithm from the American Heart Association to diagnose incomplete Kawasaki is implemented in Denmark. The algorithm is based on classic clinical features, echocardiography and laboratory findings....

  19. Inkomplet Kawasakis syndrom med koronaraneurisme hos et spædbarn

    DEFF Research Database (Denmark)

    Vinding, Rebecca Kofod; Debes, Nanette Mol; Stantchev, Hristo

    2014-01-01

    We report a four-month-old boy with incomplete Kawasaki syndrome. He developed a coronary artery aneurysm. Incomplete Kawasaki syndrome is rare in Denmark, and only reported in five patient cases in the past 25 years. It is difficult to diagnose Kawasaki disease; early diagnosis and treatment...... reduces the risk for cardiac sequel. In lack of diagnostic test, we recommend that the algorithm from the American Heart Association to diagnose incomplete Kawasaki is implemented in Denmark. The algorithm is based on classic clinical features, echocardiography and laboratory findings....

  20. 6个月以下婴儿川崎病33例临床分析%A clinical analysis of 33 cases Kawasaki disease of infants aged under 6 months

    Institute of Scientific and Technical Information of China (English)

    邓琼仙; 吕洁; 赵文利

    2012-01-01

    Objective For early diagnosis of Kawasaki disease and to decrease the misdiagnosis rate, this article analyze clinical features of Kawasaki disease in the infants. Methods The clinical data of 33 infants aged 1 to 6 months suffering from Kawasaki disease were retrospectively analyzed. Results In this study, the number of boys was more than that of girls. The incidence of the no-typical Kawasaki disease was higher, that of the typical one and the misdiagnosis rate was higher. Conclusion Early diagnosis and timely treatment are difficult in younger infants younger than 6 months with KD, echocardiogram becomes an important inplement for diagnosis.%目的 分析6个月龄以下小婴儿川崎病的临床发病特点,利于早期诊断.方法 收集2006年6月~2011年6月我院收治的6个月龄以下川崎病患儿33例临床资料进行回顾性分析.结果 6个月龄以下小婴儿川崎病以男孩多发,不典型病例多见,误诊率较高.结论 婴儿川崎病临床表现无特异性,冠状动脉异常可作为小婴儿非典型川崎病唯一确诊的金指标.

  1. 血管内皮祖细胞与川崎病冠状动脉并发症的血管新生%Endothelial progenitor cells and the neovascularization in patients with coronary aneurysms due to Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    段超; 杜忠东

    2011-01-01

    @@ 川崎病(Kawasaki disease ,KD)是一种主要发生在5岁以下婴幼儿的急性发热出疹性疾病,冠状动脉(简称冠脉)损害是其最严重的并发症,严重时可引起心肌梗死、猝死等,严重威胁儿童的身心健康.

  2. 基于BP神经网络建立的川崎病早期诊断模型%BP Neural Network Model for Early Diagnosis of Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    黄江; 陈剑锋

    2011-01-01

    In order to diagnose Kawasaki Disease during early phase, clinical symptoms (temperature, rash, conjunctival injec-tion, erythema of thelips, and oral mucosal changes) and laboratory data (white blood cell, neutrophil, platelet, c -reactive protein, and erythrocyte sedimentation rate) of 156 children with Kawasaki disease or infectious diseases were used to develop a BP neural net-work model. 90 random cases were trained using MATLAB software for setting up the BP neural network model. The other 66 cases were analyzed to predict diagnosis of Kawasaki disease using this model. Results showed that the predict accuracy in patients with Ka-wasaki disease and children with infectious diseases were 97. 4% and 92. 9% , respectively. Our result indicates that the BP neural network model is likely to provide an accurate test for early diagnosis of Kawasaki disease.%为早期诊断川崎痛,应用BP神经网络原理建立川崎病的诊断模型.以156例川崎病与非川崎病患者的体温、皮疹、口腔黏膜改变、实验室检查结果等9项指标等作为BP神经网络的输入参数,在MATLAB7程序中对其中随机抽取的90例学习样本进行训练并建模.以剩余的66例作为测试样本进行预测,结果表明该模型对川崎病和非川崎病的预测准确率分别为97.4%、92.9%,提示此模型可有效地判别出川崎病与非川崎病,可用于川崎病的早期辅助诊断.

  3. Significance of platelet activation state and platelet parameters in patients with kawasaki disease%川崎病患者血小板活化状态及血小板参数的观察及临床意义

    Institute of Scientific and Technical Information of China (English)

    田建良; 沈瑛

    2012-01-01

    Objective ;To explore the clinical significance of changes of platelet glycoprotein CD61 ,CD62P and platelet parameter PLT, MPV and PDW in children with Kawasaki disease. Methods:The expression of platelet surface activity glycoprotein CD61 and CD62P in 50 patients with Kawasaki disease were measured by flow cytome-try. The platelet parameters were measured in all patients using automatic blood analyzer, then the results were compared with the control group. Results: The levels of platelet CD61, CD62P, MPV and PDW in patients with Kawasaki disease were obviously higher than" those in normal control group (F <0. 01) ; The levels of platelet CD61 and CD62P in patients with Kawasaki disease combined with coronary artery disease were higher than those in patients without coronary artery disease(P <0. 01 or P <0. 05) ; The platelet CD61 ,CD62P had significantly positive correlation with MPV, PDW in patients with kawasaki disease (allP<0.01), CD61 also had significantly positive correlation with CD62P (P <0.01). Conclusion: Platelet activation participated in the pathological process of kawasaki disease, and CD61 and CD62P have a certain relationship with kawasaki disease combined with coronary artery disease.%目的:探讨川崎病患儿血小板膜糖蛋白CD61、CD62P以及血小板参数PLT、MPV、PDW的变化及意义.方法:采用流式细胞术测定50例川崎病患者血小板活表面活性标记糖蛋白CD61、CD62P的表达,同时应用全自动血球分析仪对血小板的参数进行测量,并与对照组比较.结果:川崎病患儿血小板CD61、CD62P、PLT和MPV水平明显高于正常对照组(P<0.01);川崎病合并冠状动脉病变组患者与非冠状动脉病变组患者相比,血小板CD61、CD62P水平增高(P<0.01或P<0.05);血小板CD61、CD62P与MPV、PLT之间呈显著正相关关系(均P<0.01);CD61、CD62P表达之间显著正相关(P<0.01).结论:血小板活化参与了川崎病的病理过程,而且CD61和CD62P与川崎

  4. 120例川崎病合并脏器损害回顾性分析%Retrospective analysis of 120 cases with Kawasaki disease complicated organ damage

    Institute of Scientific and Technical Information of China (English)

    葛婷; 都鹏飞

    2012-01-01

    Objective: To retrospectively analyze clinical types and risk factors for children with Kawasaki disease (KD) complicated organ damage. Methods: Clinical data of 120 KD children admitted from Jan 2005 to Oct 2010 were retrospectively summarized and analyzed. Analyses were performed on types of organ damage complicated in KD, relationship among age, fever process, blood biochemical indexes, treatment duration and organ damage. Results: (1) Organ damages of KD included: cardiovascular system damage (n = 82, 68. 3%), respiratory system damage (n= 55, 45.83%), liver damage (n = 77, 64.2%), gastrointestinal abnormalities (n = 18, 15.0%), urinary system abnormalities (n = 15, 12. 50%), blood system abnormalities mainly manifesting platelet increase (n = 133, 94. 17%); (2) Incidence rates of multi - organ dysfunction of KD children with age one year old (85. 0% vs. 73. 1%), fever process <10d (87. 8% vs. 64. 8%) and PLT <300 × 109/L (79. 0% vs. 50.0%) respectively, P<0. 05 all. Conclusion; Kawasaki disease can lead to multi - organ dysfunction, especially in children with Kawasaki disease with age

  5. 黏附分子与川崎病发病机制关系的研究进展%Advances in research of the relationship between adhesion molecules and pathogenesis of Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    朝鲁门; 陈柏谕

    2014-01-01

    Adhesion molecules (AM) are a class of molecules that can mediate cell-to-cell and cell-to-matrix interaction. They participate in the cellular recognition, signal transduction, cell proliferation and differentiation, cell stretching and movement through ligand-to-receptor interaction. AM are the molecular basis of immune response, inflammation, blood coagulation, tumor metastasis, wound healing and a series of physiological and pathological processes. Kawasaki disease (KD) is an acute systemic small vasculitis syndrome, mainly affecting coronary artery. KD is the main cause for acquired heart disease in children. To study the relationship between AM and pathogenesis of KD is important in the understanding of KD pathogenesis, prevention and treatment of coronary artery lesions complicated with KD. This review focused on the relationship between AM and pathogenesis of KD.%黏附分子(AM)是介导细胞与细胞间或细胞与基质间相互作用的一类分子总称,通过配体-受体相结合的形式参与细胞间识别、信号转导、细胞增殖与分化、细胞伸展与运动,是免疫应答、炎症发生、凝血、肿瘤转移、创伤愈合等一系列生理病理过程的分子基础。川崎病(KD)是一种急性全身中、小血管炎性综合征,主要累及冠状动脉,是儿童后天获得性心脏病的主要原因之一。研究AM与KD发病机制的关系对了解KD发病机制、预防及治疗KD心血管系统并发症有积极作用。

  6. Enfermedad de Kawasaki

    Directory of Open Access Journals (Sweden)

    Sandra Matiz Mejía

    2017-05-01

    Full Text Available La enfermedad de Kawasaki es un estado febril agudo, cuya incidencia es de 175 por cada 100.000 niños menores de 5 años. Es una vasculitis con predilección por los vasos de pequeño y mediano calibre, especialmente por las arterias coronarias; sin tratamiento pueden desarrollarse aneurismas coronarios que conllevan riesgo de infarto agudo de miocardio y muerte súbita. Se describe el caso de un niño de 4 años a quien se diagnosticó enfermedad de Kawasaki y aneurismas fusiformes moderados en las arterias coronarias derecha e izquierda. En control a los dos meses desarrolló nuevo cuadro febril con hipercolesterolemia, trombocitosis y alteración de las pruebas hepáticas. En ecocardiograma de control se evidenció aneurisma gigante en la coronaria izquierda y mediante angiografía coronaria se detectaron dos aneurismas en la coronaria derecha. En vista de que se consideró un caso de evolución atípica, se realizó revisión de la literatura mundial en enfermedad de Kawasaki y se recomendó diagnóstico y tratamiento precoz para disminuir la morbilidad con compromiso cardiovascular severo y/o la mortalidad.

  7. Successful Thrombolytic Therapy in a Child with Kawasaki Disease and Acute Myocardial Infarction

    Institute of Scientific and Technical Information of China (English)

    潘宝全; 黄国峰; 吴玉清; 张旭明

    2002-01-01

    @@ CLINICAL MATERIALS The patient was 7 year -old boy, body weight 20kg. He was admitted on May l0th , 2002 because offever, rashes, enlarged lymphnodes, conjunctivae in-jection, strawberry tongue, edema and erythema of thehands and feet.

  8. Research progress of non-cardiovascular complications in Kawasaki disease%川崎病非心血管系统并发症研究进展

    Institute of Scientific and Technical Information of China (English)

    翁海美; 项如莲

    2009-01-01

    @@ 川崎病(Kawasaki disease,KD)目前己取代风湿热成为儿童后天性心脏病最常见的病因.KD的病理改变为全身性血管炎,累及多脏器,包括心脏、脑、肝脏、胃肠道、肺、肾脏等.其中心血管病变最常见也最严重,未经治疗的冠状动脉损害(coronary artery lesions,CAL)占20%~25%左右,部分可形成冠状动脉瘤(coronary artery aneurysm,CAA),后期可发生冠状动脉狭窄或血栓形成,甚至导致心肌梗死,目前已引起临床广泛重视.但心血管外其他系统损害却未得到足够重视,特别在不完全KD,其往往以心血管外表现为首发症状,极易造成临床的误诊、漏诊,延误治疗,严重影响患儿预后.本文就KD非心血管系统并发症作一综述.

  9. Acute graft versus host disease

    Directory of Open Access Journals (Sweden)

    Vogelsang Georgia B

    2007-09-01

    Full Text Available Abstract Acute graft-versus-host disease (GVHD occurs after allogeneic hematopoietic stem cell transplant and is a reaction of donor immune cells against host tissues. Activated donor T cells damage host epithelial cells after an inflammatory cascade that begins with the preparative regimen. About 35%–50% of hematopoietic stem cell transplant (HSCT recipients will develop acute GVHD. The exact risk is dependent on the stem cell source, age of the patient, conditioning, and GVHD prophylaxis used. Given the number of transplants performed, we can expect about 5500 patients/year to develop acute GVHD. Patients can have involvement of three organs: skin (rash/dermatitis, liver (hepatitis/jaundice, and gastrointestinal tract (abdominal pain/diarrhea. One or more organs may be involved. GVHD is a clinical diagnosis that may be supported with appropriate biopsies. The reason to pursue a tissue biopsy is to help differentiate from other diagnoses which may mimic GVHD, such as viral infection (hepatitis, colitis or drug reaction (causing skin rash. Acute GVHD is staged and graded (grade 0-IV by the number and extent of organ involvement. Patients with grade III/IV acute GVHD tend to have a poor outcome. Generally the patient is treated by optimizing their immunosuppression and adding methylprednisolone. About 50% of patients will have a solid response to methylprednisolone. If patients progress after 3 days or are not improved after 7 days, they will get salvage (second-line immunosuppressive therapy for which there is currently no standard-of-care. Well-organized clinical trials are imperative to better define second-line therapies for this disease. Additional management issues are attention to wound infections in skin GVHD and fluid/nutrition management in gastrointestinal GVHD. About 50% of patients with acute GVHD will eventually have manifestations of chronic GVHD.

  10. 超敏C反应蛋白在小儿川崎病冠状动脉损害中的意义%The Relationship between High Sensitivity C Reactive Protein and Coronary Aretery Lesions in Children’s Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    杨洪

    2013-01-01

      目的 研究超敏C反应蛋白与冠状动脉损害的关系,探讨预测冠状动脉损害的指标。方法 对74例川崎病患儿在治疗前和治愈后行血清HsCRP的检测,并通过心脏彩超了解有无冠状动脉的损害以及损害情况。结果 川崎病患儿在急性期发病时血清HsCRP水平明显升高,治愈后上述指标基本恢复正常。有冠状动脉损害的川崎病患儿血清HsCRP水平明显高于无冠状动脉损害的。结论 川崎病患儿的血清HsCRP和冠状动脉损害呈正相关,是预测冠状动脉损害的重要参考指标。%Objective To research the relationship between high sensitivity C reactive protein and coronary aretery lesions. Method 74 cases with Kawasaki disease before treatment and cure after serum HsCRP, and by echocardiography and understand whether coronary artery damage and damage. Results The level of serum HsCRP was significantly increased in acute onset of children with Kawasaki disease, these indexes returned to normal after cure. Coronary artery lesions in patients with Kawasaki disease serum HsCRP levels were significantly higher than those without coronary artery lesion. Conclusion The serum HsCRP in children with Kawasaki disease and coronary artery damage was positively correlated with coronary lesions, is an important reference index.

  11. Peripheral Endothelial (DysFunction, Arterial Stiffness and Carotid Intima-Media Thickness in Patients after Kawasaki Disease: A Systematic Review and Meta-Analyses.

    Directory of Open Access Journals (Sweden)

    Sanne M Dietz

    Full Text Available Kawasaki disease (KD is a systemic pediatric vasculitis. Its main complication is the development of coronary arterial aneurysms (CAA, causing an increased risk for ischemia and myocardial infarction. It is unclear whether KD patients, apart from the presence of CAA, have an increased cardiovascular disease (CVD risk due to the previous systemic vasculitis. The aim of this study was to systematically review and meta-analyse the literature regarding surrogate markers for CVD risk in KD patients.Medline and Embase were searched for articles comparing endothelial dysfunction (flow-mediated dilation, nitroglycerin-mediated dilation and peripheral arterial tonometry, vascular stiffness (stiffness index, pulse wave velocity and carotid intima-media thickness (cIMT between patients and controls. Two investigators assessed the articles for eligibility and evaluated quality.Thirty studies were included. For all outcomes, moderate to high heterogeneity between studies was found. Most studies reported a decreased flow-mediated dilation in the whole KD- and CAA-positive group compared to controls, while data on CAA-negative patients were conflicting. The stiffness index was increased in the majority of studies evaluating the whole KD- and CAA-positive group, but not in most studies on CAA-negative patients. Mean cIMT was neither significantly increased in the whole KD-group nor in the CAA-positive group nor in most studies studying CAA-negative patients. Studies measuring maximum cIMT were conflicting.Literature suggests that surrogate markers for CVD risk in KD patients are increased in CAA-positive but not in CAA-negative patients. This may indicate that CAA-positive patients should be monitored for CVD in later life. The results of this review have to be interpreted with care due to substantial heterogeneity between studies and methodological limitations, as well as the lack of long-term follow-up studies.

  12. Activated myeloid dendritic cells accumulate and co-localize with CD3+ T cells in coronary artery lesions in patients with Kawasaki disease.

    Science.gov (United States)

    Yilmaz, Atilla; Rowley, Anne; Schulte, Danica J; Doherty, Terence M; Schröder, Nicolas W J; Fishbein, Michael C; Kalelkar, Mitra; Cicha, Iwona; Schubert, Katja; Daniel, Werner G; Garlichs, Christoph D; Arditi, Moshe

    2007-08-01

    Emerging evidence implicating the participation of dendritic cells (DCs) and T cells in various vascular inflammatory diseases such as giant cell arteritis, Takayasu's arteritis, and atherosclerosis led us to hypothesize that they might also participate in the pathogenesis of coronary arteritis in Kawasaki disease (KD). Coronary artery specimens from 4 patients with KD and 6 control patients were obtained. Immunohistochemical and computer-assisted histomorphometric analyses were performed to detect all myeloid DCs (S-100(+), fascin(+)), all plasmacytoid DCs (CD123(+)) as well as specific DC subsets (mature myeloid DCs [CD83(+)], myeloid [BDCA-1(+)] and plasmacytoid DC precursors [BDCA-2(+)]), T cells (CD3(+)), and all antigen-presenting cells (HLA-DR(+)). Co-localization of DCs with T cells was assessed using double immunostaining. Significantly more myeloid DCs at a precursor, immature or mature stage were found in coronary lesions of KD patients than in controls. Myeloid DC precursors were distributed equally in the intima and adventitia. Mature myeloid DCs were particularly abundant in the adventitia. There was a significant correlation between mature DCs and HLA-DR expression. Double immunostaining demonstrated frequent contacts between myeloid DCs and T cells in the outer media and adventitia. Plasmacytoid DC precursors were rarely found in the adventitia. In conclusion, coronary artery lesions of KD patients contain increased numbers of mature myeloid DCs with high HLA-DR expression and frequent T cell contacts detected immunohistochemically. This suggests that mature arterial myeloid DCs might be activating T cells in situ and may be a significant factor in the pathogenesis of coronary arteritis in KD.

  13. Aggressive and acute periodontal diseases.

    Science.gov (United States)

    Albandar, Jasim M

    2014-06-01

    Inflammatory periodontal diseases are highly prevalent, although most of these diseases develop and progress slowly, often unnoticed by the affected individual. However, a subgroup of these diseases include aggressive and acute forms that have a relatively low prevalence but show a rapid-course, high rate of progression leading to severe destruction of the periodontal tissues, or cause systemic symptoms that often require urgent attention from healthcare providers. Aggressive periodontitis is an early-onset, destructive disease that shows a high rate of periodontal progression and distinctive clinical features. A contemporary case definition of this disease is presented. Population studies show that the disease is more prevalent in certain geographic regions and ethnic groups. Aggressive periodontitis is an infectious disease, and recent data show that in affected subjects the subgingival microbiota is composed of a mixed microbial infection, with a wide heterogeneity in the types and proportions of microorganisms recovered. Furthermore, there are significant differences in the microbiota of the disease among different geographic regions and ethnicities. There is also evidence that the Aggregatibacter actinomycetemycomitans-JP2 clone may play an important role in the development of the disease in certain populations. The host response plays an important role in the susceptibility to aggressive periodontitis, where the immune response may be complex and involve multiple mechanisms. Also, genetic factors seem to play an important role in the pathogenesis of this disease, but the mechanisms of increased susceptibility are complex and not yet fully understood. The available data suggest that aggressive periodontitis is caused by mutations either in a few major genes or in multiple small-effect genes, and there is also evidence of gene-gene and gene-environment interaction effects. Diagnostic methods for this disease, based on a specific microbiologic, immunologic or

  14. Early Clinical Analysis of Atypical Kawasaki Disease%不典型川崎病的早期临床分析

    Institute of Scientific and Technical Information of China (English)

    夏玉平

    2016-01-01

    Objective To get acquaintance with the early clinical manifestations of atypical Kawasaki Disease,improve the diagnosis rate of it at early phase,and reduce the incidence of acquired heart disease in children. Methods Using retro-spective analysis on the 79 cases of Kawasaki Disease in our hospital during January 2008 and July 2014,find out valuable la-boratory examinations and common clinical features outside of the traditional diagnositic criterion. Using two kinds of dignosit-ic criteria named A,B ( A is the traditional dignositic criterion,B is the referential dignositic criterion) to analysis. Results Of the 79 cases,the abnormality rates of laboratory examinations are erythrocyte sedimentation rate increasing(91. 14%),C react protein increasing ( 88. 61%) , platelet count increasing ( 87. 34%) , total number of white blood cells increasing (86. 08%);Among the common clinical features outside of the traditional diagnosis standard,the incidence of crissum in-flamed skin peeling is 53. 17%,BCG scar swelling rate is 49. 37%. Criteria B added the increasing erythrocyte sedimentation rate,elevated c-reactive protein, platelet count, total number of white blood cells, crissum inflamed skin peeling, BCG scar swelling to the dignositic criterion, and the dignositic rate at 6 days is 77. 22% compare to 56. 96% by Criteria A ( P <0. 05). Conclusion Combining with the increasing erythrocyte sedimentation rate,elevated c-reactive protein,platelet count, total number of white blood cells,crissum inflamed skin peeling,BCG scar redness,we can improve the early diagnostic rate of atypical Kawasaki Disease.%目的:了解不典型川崎病( KD)的早期临床表现,提高不典型川崎病的早期诊断水平,降低儿童后天性心脏病的发病率。方法将本院2008年1月至2014年7月收治的79例川崎病进行回顾性临床分析,分别用A、B两种诊断标准(A为传统诊断标准、B为参考诊断标准)对其进行分析。结果实验室检查指

  15. Association of CCR2-CCR5 haplotypes and CCL3L1 copy number with Kawasaki Disease, coronary artery lesions, and IVIG responses in Japanese children.

    Directory of Open Access Journals (Sweden)

    Manju Mamtani

    Full Text Available BACKGROUND: The etiology of Kawasaki Disease (KD is enigmatic, although an infectious cause is suspected. Polymorphisms in CC chemokine receptor 5 (CCR5 and/or its potent ligand CCL3L1 influence KD susceptibility in US, European and Korean populations. However, the influence of these variations on KD susceptibility, coronary artery lesions (CAL and response to intravenous immunoglobulin (IVIG in Japanese children, who have the highest incidence of KD, is unknown. METHODOLOGY/PRINCIPAL FINDINGS: We used unconditional logistic regression analyses to determine the associations of the copy number of the CCL3L1 gene-containing duplication and CCR2-CCR5 haplotypes in 133 Japanese KD cases [33 with CAL and 25 with resistance to IVIG] and 312 Japanese controls without a history of KD. We observed that the deviation from the population average of four CCL3L1 copies (i.e., four copies was associated with an increased risk of KD and IVIG resistance (adjusted odds ratio (OR=2.25, p=0.004 and OR=6.26, p=0.089, respectively. Heterozygosity for the CCR5 HHF*2 haplotype was associated with a reduced risk of both IVIG resistance (OR=0.21, p=0.026 and CAL development (OR=0.44, p=0.071. CONCLUSIONS/SIGNIFICANCE: The CCL3L1-CCR5 axis may play an important role in KD pathogenesis. In addition to clinical and laboratory parameters, genetic markers may also predict risk of CAL and resistance to IVIG.

  16. Multicenter, Single-Arm, Phase IV Study of Combined Aspirin and High-Dose “IVIG-SN” Therapy for Pediatric Patients with Kawasaki Disease

    Science.gov (United States)

    Yoon, Kyung Lim; Lee, Hae Yong; Yu, Jeong Jin; Lee, Jae Young; Han, Mi Young; Kim, Ki Yong

    2017-01-01

    Background and Objectives Intravenous immunoglobulin-SN (IVIG-SN) is a new human immunoglobulin product. Its safety is ensured by pathogen-elimination steps comprising solvent/detergent treatment and a nanofiltration process. This multicenter clinical study was designed to evaluate the efficacy and safety of combined aspirin and high-dose IVIG-SN therapy in pediatric patients with Kawasaki disease (KD). Subjects and Methods We evaluated coronary artery lesions (CALs) at 2 and 7 weeks after administering IVIG-SN; total fever duration; and variations in erythrocyte sedimentation rate, N-terminal pro B-type natriuretic peptide or B-type natriuretic peptide, and creatine kinase-myocardial band level before and after treatment with IVIG-SN (2 g/kg). Adverse events were monitored. Results Forty-five patients were enrolled, three of whom were excluded according to the exclusion criteria; the other 42 completed the study. The male:female ratio was 0.91:1, and the mean age was 29.11±17.23 months. The mean fever duration before IVIG-SN treatment was 6.45±1.30 days. Although most patients had complete KD (40 patients, 90.91%), four had atypical KD (9.09%). After IVIG-SN treatment, one patient (2.38%) had CALs, which was significantly lower than the incidence reported previously (15%) (p=0.022), but not significantly different from recent data (5%). There were no serious adverse events, though 28 patients (63.64%) had mild adverse events. Three adverse drug reactions occurred in 2 patients (eczema, anemia, and increased eosinophil count), all of which were transient. Conclusion IVIG-SN treatment in patients with KD was safe and effective.

  17. Study on the relationship between mean platelet volume and platelet distribution width with coronary artery lesion in children with Kawasaki disease.

    Science.gov (United States)

    Liu, Ruixi; Gao, Fang; Huo, Junming; Yi, Qijian

    2012-01-01

    Mean platelet volume (MPV) and platelet distribution width (PDW) are correlated with platelet function and may be a more sensitive index than platelet number as a marker of clinical interest in various disorders. Therefore, this study was designed to answer the following questions: do MPV and PDW levels change in Kawasaki disease (KD), is there any relation between CAL in children with MPV and PDW and whether MPV and PDW might support a diagnosis of incomplete KD. A total of 309 KD patients and 160 sex-age matched healthy subjects were enrolled into the study. For all subjects following tests were performed: MPV, PDW, platelet count, white blood cells counts (WBC), C reactive protein (CRP) and erythrocyte sedimentation rate (ESR). Patients with CALs were assigned to three groups depending on the extent of CALs which were visualized by echocardiography: dilatation and/or ectasia, aneurysm and giant aneurysms. We compared patients with fever and four or five of the principal criteria (complete KD, cKD) to the other patients (iKD). Compared with healthy controls a significant decrease in MPV and PDW (p platelet count, CRP and ESR (p all children with KD. There were no statistically differences in MPV and PDW between KD with CALs and KD without CALs (p > 0.05). However, MPV and PDW were significantly lower in patients with iKD than in group with cKD (p = 0.003, p = 0.014, respectively). It was first shown that patients with KD have lower MPV and PDW than control subjects. The diagnosis of iKD is challenging but can be supported by the presence of lower MPV and PDW.

  18. 川崎病合并幼年特发性关节炎一例%Kawasaki disease combined with systemic juvenile idiopathic arthritis:a case report

    Institute of Scientific and Technical Information of China (English)

    汤昔康; 陈泽楷; 覃丽君

    2014-01-01

    In clinics,pediatric fever is commonly encountered characterized with a variety of causes, complex condition and rapid changes. This article reported the diagnosis and treatment of a child with typical kawasaki disease (KD)combined with systemic juvenile idiopathic arthritis (SJIA). After the failed therapy of administration of IVIG,the child then developed arthritis. The diagnosis was corrected to KD complicated with SJIA. The symptoms were alleviated after effective treatment. Albeit KD and SJIA share similar manifestations in clinical and laboratory tests,much attention should be paid to distinguish the differences and avoid treatment delay.%临床工作中,儿科发热患儿多见,病因多样,部分患儿病情复杂且变化快。该文报道了1例川崎病合并幼年特发性关节炎患儿的诊治过程。患儿经丙种球蛋白治疗无反应后,出现关节炎症状,修正诊断为川崎病合并全身型幼年特发性关节炎,调整治疗方案后得到缓解。该例提示,川崎病及全身型幼年特发性关节炎虽有许多类似的临床表现及实验室数据,但应根据其不同点进一步鉴别诊断,以防延误治疗。

  19. KEGG DISEASE / Acute myeloid leukemia (AML) [KEGG DISEASE

    Lifescience Database Archive (English)

    Full Text Available DISEASE: H00003 Entry H00003Disease Name Acute myeloid leukemia (AML) Description Acute.... Category Cancer Brite Human diseases [BR:br08402] Cancers Cancers of haematopoietic and lymphoid tissues H00003Acute...atopoietic and related tissue C92Myeloid leukaemia H00003Acute myeloid leukemia (AML) Cancer-accociated carb...ohydrates [br08441.html] H00003 Pathway hsa05221Acute myeloid leukemiahsa05202Transcriptional misregulation ... or t(16; 16)(p13, q22), (CBF-beta/MYH11) ICD-O: 9866/3, Tumor type: Acute promyelocytic leukaemia (AML with

  20. Acute Chagas Disease in a Returning Traveler

    Science.gov (United States)

    Carter, Yvonne L.; Juliano, Jonathan J.; Montgomery, Susan P.; Qvarnstrom, Yvonne

    2012-01-01

    Acute Chagas disease is rarely recognized, and the risk for acquiring the disease is undefined in travelers to Central America. We describe a case of acute Chagas disease in a traveler to Costa Rica and highlight the need for increased awareness of this infection in travelers to Chagas-endemic areas. PMID:23091192

  1. Enfermedad de Kawasaki

    Directory of Open Access Journals (Sweden)

    Viviana Molina Alpízar

    2015-03-01

    Full Text Available La enfermedad de Kawasaki es una vasculitis aguda sistémica, afectando predominantemente arterias de mediano calibre, incluyendo las arterias coronarias, la mayoría de los casos ocurren en niños menores de 5 años, con una leve predominancia en el sexo masculino, muestra un pico estacional. Es la principal causa de cardiopatía adquirida en los países desarrollados. La etiología es desconocida y la enfermedad tiene 4 fases: febril aguda, subaguda, convalecencia y crónica. El diagnóstico de la enfermedad es clínico. El tratamiento con inmunoglobulina intravenosa y aspirina dentro de los 10 días posteriores al inicio de la enfermedad disminuye la incidencia de aneurismas coronarios a menos de 5%.

  2. Focus on acute diarrhoeal disease

    Institute of Scientific and Technical Information of China (English)

    Fabio Baldi; Maria Antonia Bianco; Gerardo Nardone; Alberto Pilotto; Emanuela Zamparo

    2009-01-01

    Diarrhoea is an alteration of normal bowel movement characterized by an increase in the water content,volume, or frequency of stools. Diarrhoea needs to be classified according to the trends over time (acute or chronic) and to the characteristics of the stools (watery, fatty, inflammatory). Secretory diarrhoeas,mostly acute and of viral aetiology in more than 70% of cases, are by far the most important subtype of diarrhoeas in terms of frequency, incidence and mortality (over 2.5 million deaths/year in developing countries). Natural and synthetic opiates such as morphine, codeine, and loperamide which react with endogenous opiates (enkephalins, beta-endorphins,dynorphins) mainly act on intestinal motility and slow down transit. An antidiarrhoeal drug developed in recent years, racecadotril, acts as an enkephalinase inhibitor.Clinical studies have shown that it is just as effective as loperamide in resolving acute diarrhoea but with greater reduction in pain and abdominal distension.Some studies have explored the prevalence of diarrhoea in old age. An epidemiological study carried out in Italy by 133 General Practitioners on 5515 elderly outpatients reported a prevalence of diarrhoea, defined according to the Rome criteria, of 9.1%. Infectious diseases (19%) and drug use (16%) were the most commoncauses of diarrhoea in old age. Regardless of the cause,the treatment of elderly patients with diarrhoea must include rehydration and nutritional support. Every year,more than 50 million tourists travel from industrialized countries to places where hygiene levels are poor. At least 75% of those travelling for short periods mention health problems, and in particular traveller's diarrhoea.

  3. Treatment of Kawasaki Disease with Glucocorticoid and Following-up Strategy%川崎病糖皮质激素疗法及随访策略

    Institute of Scientific and Technical Information of China (English)

    王宏伟; 程佩萱

    2007-01-01

    川崎病(Kawasaki disease,KD)能否应用糖皮质激素(GCS)治疗及针对川崎病的冠状动脉(简称冠脉)损伤问题如何进行科学的随访,是临床儿科医生特别关注的问题。

  4. Is acute recurrent pancreatitis a chronic disease?

    OpenAIRE

    Mariani, Alberto; Testoni, Pier Alberto

    2008-01-01

    Whether acute recurrent pancreatitis is a chronic disease is still debated and a consensus is not still reached as demonstrated by differences in the classification of acute recurrent pancreatitis. There is major evidence for considering alcoholic pancreatitis as a chronic disease ab initio while chronic pancreatitis lesions detectable in biliary acute recurrent pancreatitis (ARP) seem a casual association. Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation, hereditary a...

  5. Effect of nursing intervention on clinical therapeutic effect of Kawasaki disease%护理干预对川崎病治疗效果的临床影响

    Institute of Scientific and Technical Information of China (English)

    刘雪冰

    2016-01-01

    Objective To explore impact of nursing intervention on clinical therapeutic effect of Kawasaki disease.Methods Eighty patients with Kawasaki disease, hospitalized in our hospital during May 2014 and May 2016, were divided into experimental group and control group, they were given nursing intervention and routine nursing care respectively, and nursing effect was comparatively analyzed. Results The cognition on the disease in families and the satisfaction level on quality of care service of experimental group was significantly higher compared with the control group (P<0.05). The complication rate of test group (2.50%) was significantly lower than that (27.50%) in control group (P<0.05).Conclusions The clinical impact of nursing intervention on the treatment of Kawasaki disease was significant, it increases the awareness rate of the disease and the rate of satisfaction and prevents complications.%目的:探究护理干预对川崎病治疗效果的临床影响。方法随机选取2014年5月至2016年5月本院收治的80例川崎病患儿,将其平均分为试验组、对照组两组,分别给予护理干预、常规护理措施,对比分析护理效果情况。结果试验组患儿家属对疾病认知情况以及对护理服务质量满意程度与对照组患儿的相比明显较高(P<0.05)。试验组患儿的并发症发生率(2.50%)与对照组患儿(27.50%)的相比明显较低( P<0.05)。结论护理干预对川崎病治疗效果的临床影响显著,提高对疾病认知率及护理满意度,有效预防并发症,意义重大。

  6. 川崎病患儿血清白细胞介素-6、-10、-1水平变化及意义%Changes of serum IL-6, IL-10 and IL-1 in patients with Kawasaki disease and its clinical significance

    Institute of Scientific and Technical Information of China (English)

    吕慧; 贠国俊

    2012-01-01

    Objective To observe the serum levels of IL-6,IL-10 and IL-1 in children with Kawasaki disease and its clinical significance.Methods 38 childrey with of Kawasaki disease were selected as observction group,then selected 38 cases of normal children as control group.Serum IL-6,IL-10 and IL-1 levels were observed two groups.Results Observation group IL-6,IL-10 and IL-1 levels were higher(t =10.3877,15.1010,15.1243,all P<0.05),acute phase of observation group IL-6,IL-10,IL-1,etc.levels were significantly higher than the sub-acute phase patients ( t =9.7594,11.6486,11.6622,all P < 0.05 ) and control group ( t =11.7032,11.7718,11.8267,all P <0.05 ).48h apoptosis rate in the control group( 2.8 ± 0.8 )% was significantly lower than in children with Kawasaki disease(38.3 ±7.9)% (t =38.59,P <0.01 ).Platelets increased[ (464.0 ± 110.2) × 109/L] of the IL-1 level in children with Kawasaki disease(663 ±94)ng/L was significantly higher than those without elevated platelet [ (307.0 ±104.9) × 109 /L ] of children( 492 ± 92 ) ng/L ( t =13.1044,P < 0.05 ).Conclusion Thedetection of serum IL-6,IL-10 and IL-1 levels may aid clinical diagnosis of the condition of children with Kawasaki disease,complications and treatment,have important clinical significance.%目的 观察川崎病患儿血清白细胞介素-6(IL-6)、白细胞介素-10(IL-10)、白细胞介素-1(IL-1)水平变化及其临床意义.方法 选取38例川崎病患儿(观察组)和38例健康儿童(对照组)血清IL-6、IL-10和IL-1水平,观察两组差异及对治疗效果的影响.结果 观察组IL-6、IL-10和IL-1水平均高于对照组(t=10.3877、15.1010、15.1243,均P<0.05),观察组急性期IL-6、IL-10、IL-1等水平明显高于亚急性期患儿(t=9.7594、11.6486、11.6622,均P<0.05)及对照组(t=11.7032、11.7718、11.8267,均P<0.05).对照组48 h内细胞凋亡率为(2.8±0.8)%,明显低于川崎病患儿的(38.3±7.9)%(t=38.59,P<0.01).血小板升高[(464.0±110.2)×109/L

  7. Acute exacerbations of fibrotic interstitial lung disease.

    Science.gov (United States)

    Churg, Andrew; Wright, Joanne L; Tazelaar, Henry D

    2011-03-01

    An acute exacerbation is the development of acute lung injury, usually resulting in acute respiratory distress syndrome, in a patient with a pre-existing fibrosing interstitial pneumonia. By definition, acute exacerbations are not caused by infection, heart failure, aspiration or drug reaction. Most patients with acute exacerbations have underlying usual interstitial pneumonia, either idiopathic or in association with a connective tissue disease, but the same process has been reported in patients with fibrotic non-specific interstitial pneumonia, fibrotic hypersensitivity pneumonitis, desquamative interstitial pneumonia and asbestosis. Occasionally an acute exacerbation is the initial manifestation of underlying interstitial lung disease. On biopsy, acute exacerbations appear as diffuse alveolar damage or bronchiolitis obliterans organizing pneumonia (BOOP) superimposed upon the fibrosing interstitial pneumonia. Biopsies may be extremely confusing, because the acute injury pattern can completely obscure the underlying disease; a useful clue is that diffuse alveolar damage and organizing pneumonia should not be associated with old dense fibrosis and peripheral honeycomb change. Consultation with radiology can also be extremely helpful, because the fibrosing disease may be evident on old or concurrent computed tomography scans. The aetiology of acute exacerbations is unknown, and the prognosis is poor; however, some patients survive with high-dose steroid therapy.

  8. Acute Ischemic Stroke and Acute on Chronic Kidney Disease

    Directory of Open Access Journals (Sweden)

    Raja Ahsan Aftab

    2016-06-01

    Full Text Available Ischemic stroke is due to either local thrombus formation or emboli that occlude a cerebral artery, together with chronic kidney disease represent major mortality and morbidity. Here wer present a case of 53 years old Malay man, admitted to a hospital in Malaysia complaining of sudden onset of weakness on right sided upper and lower limb associated with slurred speech. Patient was also suffering from uncontrolled hypertension, hyperlipidemia, chronic kidney disease stage 4, and diabetes mellitus(un controlled. He was diagnosed with acute ischemic stroke with cranial nerve 7 palsy (with right hemiparesis, acute on chronic kidney disease precipitated by dehydration and ACE inhibitor, and hyperkalemia. Patients with ischemic disease and chronic kidney disaese require constant monitering and carefull selected pharmacotherapy. Patient was placed under observation and was prescribed multiple pharamacotherpay to stabalise detoriating condition. Keywords: ischemic disease; chronic kidney disease; uncontrolled hypertension. | PubMed

  9. Is acute recurrent pancreatitis a chronic disease?

    Institute of Scientific and Technical Information of China (English)

    Alberto Mariani; Pier Alberto Testoni

    2008-01-01

    Whether acute recurrent pancreaUtis is a chronic disease is still debated and a consensus is not still reached as demonstrated by differences in the classification of acute recurrent pancreatitis.There is major evidence for considering alcoholic pancreatitis as a chronic disease ab initio while chronic pancreatitis lesions detectable in biliary acute recurrent pancreatitis (ARP) seem a casual association.Cystic fibrosis transmembrane con ductance regulator (CFTR) gene mutation,hereditary and obstructive pancreatitis seem an acute disease that progress to chronic pancreatitis,likely as a consequence of the activation and proliferation of pancreatic stellate cells that produce and activate collagen and therefore fibrosis.From the diagnostic point of view,in patients with acute recurrent pancreatitis Endoscopic ultrasound (EUS) seems the more reliable technique for an accurate evaluation and follow-up of some ductal and parenchymal abnormalities suspected for early chronic pancreatitis.

  10. Application Value of Clinical Nursing Path in Children With Kawasaki Disease During Treatment%临床护理路径在川崎病患儿治疗中的应用价值探讨

    Institute of Scientific and Technical Information of China (English)

    张秀玲

    2015-01-01

    目的 探究在对川崎病患儿施以治疗的过程中,辅以临床护理路径后,对治疗效果的影响.方法 随机抽取我院收治患有川崎病的患儿40例作为本次研究的对象,根据其入院时间的先后平均分为观察组与对照组.两组患儿在入院后,接受院方提供的针对性治疗以及常规的护理服务,观察组患儿在对照组治疗的基础上加行临床护理路径的护理模式,对比两组患儿的住院时间以及住院费用.结果 观察组患儿的住院时间以及住院费用均少于对照组(P<0.05).结论 在对患有川崎病的患儿施以治疗过程中,辅以临床护理路径的护理服务后,可以有效的提高患儿的治疗效果,同时减少住院时间以及住院费用,减少了患儿家庭的经济压力.%Objective To explore the applied to children in Kawasaki disease treatment process, combined with clinical nursing pathway, on the treatment effect. Methods 40 cases of children with Kawasaki disease were randomly selected in our hospital as the research object, according to the admission time were divided into the observation group and the control group. Two groups of patients after admission, targeted therapy and conventional care the hospital provides the patients in the observation group, while in the control group on the basis of the nursing mode of clinical nursing path, the cost of hospitalization were compared between the two groups as well as in hospital. Results The hospitalization time of observation group patients and hospital costs were lower than the control group (P < 0.05). Conclusion Children with Kawasaki during the treatment, nursing and clinical nursing path, can effectively improve the treatment of children, at the same time, hospitalization time, hospitalization costs, reduce the family economic pressure.

  11. 追踪延伸护理服务在川崎病患儿中的应用%Application of tracking extension nursing service for pediatric kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    刘红艳

    2013-01-01

    目的:探讨追踪延伸护理服务在川崎病患儿中的应用效果.方法:选择2010年8月~2012年9月在我院儿科收治的123例川崎病患儿,根据有无开展追踪延伸护理服务分为对照组56例和观察组67例,对照组患儿给予川崎病常规护理,观察组患儿给予追踪延伸护理服务.比较两组患儿家属健康知识掌握程度、基础护理质量合格率和护理服务满意度.结果:观察组患儿家属健康知识掌握程度优于对照组(P<0.05),观察组患儿基础护理合格率和患儿家属对护理服务的满意度高于对照组(P<0.05).结论:追踪延伸护理服务能够减少川崎病患儿住院时间和住院费用,提高患儿家属对疾病的认识程度及对护理服务的满意度.%Objective:To investigate the tracking extension of nursing service mode in patients with Kawasaki disease effect and application of.Methods:From August 2010 to September 2012 in our department treated 123 cases of children with Kawasaki disease as the research object,according to whether to carry out tracking extension of nursing service were divided into control group and observation group,control group were given routine nursing only Kawasaki disease,whereas in observation group was given the tracking extension of nursing service mode under the guidance of nursing interventions on the children nursing intervention,compared two groups of children with anxiety and depression,the quality of basic nursing qualification rate and patients satisfaction with nursing service.Results:The children of observation group family health knowledge is better than that of control group (P < 0.05),to observe the qualified group of children with basic nursing and the rate of children with family satisfaction to nursing service was higher than that of the control group (P < 0.05).Conclusion:Track extending nursing of children with Kawasaki disease can reduce hospitalization and hospitalization time,improve the degree of

  12. 36例不完全川崎病临床分析%Clinical analysis of 36 cases of incomplete Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    邹长新

    2008-01-01

    川崎病(Kawasaki disease,KD)的诊断主要依赖于临床表现而缺乏特异性的实验室资料,对典型KD诊断不难,而不完全KD的早期诊断比较困难,容易误诊。现将我院1997年10月至2007年10月诊治的81例KD进行回顾性分析,旨在提高对不完全KD的认识。

  13. Acute Respiratory Disease Associated with Mannheimia ...

    African Journals Online (AJOL)

    Acute Respiratory Disease Associated with Mannheimia Haemolytica ... to the Veterinary Teaching Hospital (VTH), University of Agriculture, Abeokuta, Nigeria. Mannheimia spp was isolated from the nasal swab and lymph node and lung ...

  14. Current research status of catheter interventional treatment for coronary arterial lesions caused by Kawasaki disease%川崎病冠状动脉病变介入疗法的研究现状

    Institute of Scientific and Technical Information of China (English)

    黄平; 胡大一; 金小燕; 金丽玲

    2002-01-01

    @@ 川崎病(Kawasaki disease, KD)是一种病因不明,以全身中、小动脉炎性病变为主要病理改变的急性发热性疾病,常见于5岁以下的小儿,以冠状动脉(简称冠脉)病变为其最严重的并发症.KD急性期冠脉瘤(coronary artery aneurysm, CAA)的发生率约为10%~20%.随访研究表明,约50%的CAA于病后1~2年内自行消退,80%的轻、中度CAA于病后5年内消退.CAA直径>5 mm者可能进展为狭窄,且瘤体越长可能性越大.

  15. Thrombolysis in Acute Cerebrovascular Disease

    Institute of Scientific and Technical Information of China (English)

    刘泽霖

    2003-01-01

    @@ Large-scale trials have shown that thrombolytic therapy reduces mortality and preserves left ventricular function in patients with acute myocardial infarction (AMI). That's a rationale for the use of thrombolytic agents in the management of ischemic stroke.

  16. Consideraciones orales del síndrome de Kawasaki: Descripción de un caso clínico Oral considerations of Kawasaki syndrome: A case report

    Directory of Open Access Journals (Sweden)

    M.C. Figueiredo

    2010-08-01

    Full Text Available El objetivo del presente estudio es presentar un caso clínico y revisar la literatura existente sobre el Síndrome de Kawasaki (SK tan frecuente en pacientes con necesidades especiales. Las características clínicas de esta patología se observan ya en niños muy jóvenes, en la gran mayoría de los casos menores de 5 años de edad. Esta es una patología que puede llevar al óbito, pues revela un compromiso cardíaco importante. Hay señales y síntomas en la cavidad oral en la fase aguda de la enfermedad, como la lengua de "frambuesa", eritema con fisura labial y eritema en la mucosa oral, siendo importante que el dentista tenga el conocimiento de esta patología y encamine el paciente para tratamiento médico con urgencia. Se observó en este caso una característica peculiar, que es la retención prolongada de la dentición temporaria. Una vez diagnosticado y tratado, el conocimiento del Síndrome de Kawasaki permite al dentista planear con seguridad un tratamiento dentario a largo plazo. Con eso puede evitarse que los dientes permanentes erupcionen ectópicamente, así como también mantener una profilaxis bucal de rutina, preservando la salud oral de los pacientes portadores de este síndrome.The aim of this study is to review what literature presents about Kawasaki Disease (KD, common in patients with special needs. Clinical evidence of this pathology may be observed in young children, who are usually less than five years old. This pathology may lead patients to death since it involves serious heart conditions. There are signs and symptoms in the oral cavity during the acute phase, for example "strawberry" tongue, red lesions with lips fissure and red lesions of oral mucous. Therefore it is important for the dentist to know about this pathology in order to send the patient to urgent medical treatment. A peculiar characteristic was observed in this case, long term retention of temporary dentition. Once the disease has been diagnosed and

  17. Diarrheal Diseases - Acute and Chronic

    Science.gov (United States)

    ... Patient Education & Resource Center GI Health and Disease Recursos en Español What is a Gastroenterologist Video and ... Education & Resource Center Home GI Health and Disease Recursos en Español What is a Gastroenterologist? Podcasts and ...

  18. Study on Cognitive Model of Kawasaki Disease with Coronary Artery Lesions by Bayes Method%基于贝叶斯公式的川崎病患儿并发冠状动脉损伤的认知模型研究

    Institute of Scientific and Technical Information of China (English)

    周雪晴; 宋萍; 黄仕鑫; 罗亚玲

    2016-01-01

    Objective This study aims to establish the Bayes Model of Kawasaki diseases with coronary artery lesions (CAL)in combination by Bayes method,investigate the Cognitive Model of Kawasaki diseases with CAL. Research was performed according to the clinical biomarkers of Kawasaki diseases. Methods We obtained clinical records of 784 patients with Kawasaki disease and hospitalized in Children′s Hospital Affiliated to Chongqing Medical University from September 2014 to September 2015. These records were retrospectively analyzed,univariate analysis on influencing factor for Kawasaki disease with CAL was carried out,and the statistical significance factors were included in the Bayes Model. Results The univariate analysis results indicated that the gender,age,duration of the fever,PLT,Hb,CRP,and Alb were risk factors for Kawasaki disease with CAL( P < 0. 05). Bayes Model showed sensitivity was 58. 1% ,specificity was 74. 4% ,and coincidence rate was 71. 6% . Conclusion The major risk factors of Kawasaki disease with CAL were gender,age,duration of the fever,PLT,Hb,CRP, and Alb. The Bayes Model improved the sensitivity for predicting the Kawasaki disease children with CAL.%目的:根据川崎病临床生物指标,结合贝叶斯方法,建立川崎病并发冠状动脉损伤(CAL)的贝叶斯模型,对川崎病并发 CAL 的认知模型进行初步探讨。方法回顾性分析2014年9月—2015年9月重庆医科大学附属儿童医院784例住院川崎病患儿的临床资料,对影响川崎病患儿并发 CAL 的因素进行单因素分析,将有统计学意义的指标纳入贝叶斯模型。结果784例患儿中356例(45.4%)并发 CAL,单因素分析显示,性别、发病年龄、热程、血小板计数(PLT)、血红蛋白( Hb)、C 反应蛋白( CRP)、清蛋白( Alb)是川崎病患儿并发 CAL 的影响因素(P <0.05)。贝叶斯模型的灵敏度为58.1%,特异度为74.4%,符合率为71.6%。结论川崎病并发 CAL

  19. 冠状动脉旁路移植术治疗6例儿童川崎病并发冠状动脉病变%Coronary artery bypass grafting for coronary disease due to Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    赵强; 朱丹; 王哲; 陈安清; 刘俊

    2010-01-01

    Objective To determine the efficacy of coronary artery bypass grafting (CABG) in children with coronary obstructive disease subsequent to Kawasaki disease. Methods Between Feb 2005 and Sep 2009, 6 children with ischemic heart disease due to Kawasaki disease comprised the study group. The age of patients at operation was ranged from 6 to 12 years with a mean of ( 8.0 ± 2.3 ) years. The preoperative EF was ranged from 0.33 to 0.71 with a mean of 0.57 ± 0.15. There was one case with moderate mitral valve regurgitation. Results All patients survived the procedures. The mean number of bypass graft was 2.0 ±0.6,with 4 internal thoracic arteries and 7 radial arteries. Mitral valve repair was employed in 1 case. The cardisc function ( NYHA and EF) was significantly improved postoperatively. Postoperative CTA showed no graft disease in all cases. Conclusion CABG using the arterial grafts can provide attractive mid-term results in patients with obstructive coronary arteries associated with Kawasaki disease.%目的 总结冠状动脉旁路移植治疗儿童川崎病并发冠状动脉病变的近、中期疗效.方法 2005年2月至2009年9月,6例川崎病并发冠状动脉病变病儿接受冠状动脉旁路移植,其中男5例,女1例;年龄6~12岁.确诊川崎病0.5~5.0年.冠状动脉左主干闭塞1例,左、右冠状动脉瘤样病变5例.心功能(NYHA)分级Ⅱ级1例,Ⅲ级5例.术前心脏超声示左室舒张末内径(LVDD)39~54 mm;左室收缩未内径(LVSD)23~45 mm;左室射血分数(LVEF)0.33~0.71;二尖瓣中度反流1例.均在体外循环下手术,移植血管均用动脉,平均旁路移植血管(2.0±0.6)根.其中左乳内动脉4根,桡动脉7根.同期冠状动脉成形术4例,二尖瓣成形术1例.结果 无手术死亡,体外循环平均(95.6±31.0)min;主动脉阻断平均(57.8±33.9)min.术后LVDD 32~56 mm,LVSD 21~39 mm,LVEF 0.45~0.71.冠状动脉CT示移植血管均通畅.均获随访,无远期死亡,病儿生长

  20. 261例川崎病合并感染的临床分析%Clinical Analysis of Kawasaki Disease Complicating With Infection: 261 Cases

    Institute of Scientific and Technical Information of China (English)

    王小川; 蔡雯雯; 贾苍松

    2012-01-01

    目的 探讨川崎病(KD)合并感染患儿的临床表现、诊断、治疗方案及其预后.方法 选择2003年1月至2009年1月在本院确诊的261例KD患儿就诊、治疗和随访的临床病历资料为研究对象.按照KD是否合并感染,将其分为感染组(n=65,24.9%)与非感染组(n=196,75.1%);按照KD是否典型,将其分为典型KD组(n=184,70.5%)与不完全性KD组(n=77,29.5%);再按照是否合并冠状动脉损害(Cals),将其分为合并Cals组(n=24)及未合并Cals组(n=237).采取回顾性分析方法,分别分析不同年龄KD患儿合并感染的临床表现,比较典型KD组与不完全性KD组及合并Cals组与未合并Cals组的感染临床特征及预后(本研究遵循的程序符合本院人体试验委员会制定的伦理学标准,得到该委员会批准).感染组与非感染组,典型KD组与不完全性KD组及合并Cals组与未合并Cals组KD患儿的发病年龄中位数、性别及合并其他疾病情况等比较,差异无统计学意义(P>0.05).结果 261例KD患儿中,不同年龄患儿感染率比较,差异均有统计学意义(P0.05).合并Cals组与未合并Cals组患儿感染率比较,差异亦无统计学意义(P>0.05).结论 KD发病时可并存感染,但感染并非与KD预后相关.临床治疗KD时,应注意并发感染情况,同时应全面合理地检查,并合理选用抗菌药物治疗.%Objective To analyze clinical characteristics, diagnosis, treatment methods and prognosis of children with Kawasaki disease (KD) combined with infection. Methods Clinical data, follow-up data and treatment data for 261 KD children who recurited into the Department of Pediatrics, West China Second University Hospital from January 2003 to January 2009 were retrospectively analyzed. According to whether amalgamative infection, they were divided into infection group (n=65,24. 9%) and non-infection group (n=196,75. 1%). According to KD types, they were further divided into typical KD group (n=184, 70.5%) and incomplete KD

  1. 川崎病并发无菌性脑膜炎31例临床治疗分析%Kawasaki disease complicated with aseptic meningitis clinical treatment of 31 cases were analyzed

    Institute of Scientific and Technical Information of China (English)

    罗云娇; 杜曾庆; 杨小涛

    2014-01-01

    objective the article is to review the 112 cases of Kawasaki disease (Kd) treaded in our department from January to december 2013 with the emphasis of their characteristics,diagnosis,treatment of patients with aseptic meningitis in 31 cases which were retrospectively analyzed. Methods according to Kawasaki disease and aseptic meningitis treatment, the patients were treated with aspirin 50mg/d/ kg,divided into 2 - 3 oral,and given mannitol intracranial pressure lowering treatment. all patients were given intravenous immunoglobulin therapy. Before discharging,cases in the group were given two dimensional echocardiogram Beckoning graph (2de) examination. results the clinical symptoms of all patients were disappeared,their condition improved and discharged. normal cardiac 2de examination of 5 cases of abnormal coronary artery:26 (83.87%) cases had different degrees of coronary artery lesions. conclusion the present (Kd) has replaced the wind damp heat and has become the most important cause of acquired heart disease for children. in recent years Kd with aseptic meningitis cases is increasing,Kd concurrent aseptic meningitis is Kawasaki disease in severe cases,in this group of cases were complicated with multiple organ injury. clinical attention should be paid. Pediatricians should raise awareness of Kd, especially Kd with aseptic meningitis cases. early diagnosis,early treatment is the key to reduce coronary artery disease complicated by Kd.%目的:对我科2013年1月至12月收治的112例川崎病(Kd)中并发无菌性脑膜炎31例病例的临床特征、诊断、治疗进行回顾性分析。方法本组病例入院后按川崎病并无菌性脑膜炎治疗,本组病例均给予阿司匹林50mg/d/㎏,分2~3次口服,并给予甘露醇脱水降颅压等治疗。全部病例均给予静脉丙种球蛋白治疗。出院前本组病例均做了心脏二维超声心动图(2de)检查。结果全部病例出院时临床症状消失

  2. 运用Delphi法确立川崎病中医基本证候分类的研究%Using Delphi Method to Establish TCM syndrome classification of Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    王文革; 申广生; 汪受传

    2011-01-01

    Objective: To screen out the TCM syndrome classification indices of Kawasaki disease by Delphi method.Methods:Two rounds of questionnaire survey were carried out by 40 experts according to Delphi method. The data was analyzed with SPSS software for positive degree, concentricity and coordination. Results:The recovery rate of the first round questionnaire was 90.0% and that of the second round was 92.5%. The variance coefficient of syndrome of disease involving weifen and qifen, syndrome of flaring heat in qifen and yingfen, syndrome of deficiency of both qi and yin were smaller than 0.20. The variance coefficient of syndrome of lingering heat due to deficiency of yin was 0.576 of the second round questionnaire. Conclusions:Syndrome of disease involving weifen and qifen, syndrome of flaring heat in qifen and yingfen, syndrome of deficiency of both qi and yin could be taken as the TCM syndrome classification indices of Kawasaki disease.%目的:运用德尔菲法(Delphi法)筛选川崎病中医基本证候分类的指标.方法:通过Delphi法对全国40位专家进行两轮问卷调查,利用SPSS软件,对问卷反馈的数据进行积极度、集中程度、协调程度的统计与评价.结果:第1轮问卷回收率90.0%,第2轮问卷回收率92.5%.第2轮问卷邪在卫气证、气营两燔证、气阴两伤证3项指标的变异系数(CV)均在0.20以内,热恋阴伤证的CV值为0.576.结论:邪在卫气证、气营两燔证、气阴两伤证可作为川崎病的基本证候分类.

  3. Analysis of risk factors for children with incomplete Kawasaki disease%小儿不完全川崎病的危险因素分析

    Institute of Scientific and Technical Information of China (English)

    江月明; 林玉婵; 涂燕青; 黄韬

    2012-01-01

    Objective To study the risk factors for children with incomplete Kawasaki disease(IKD) to decrease the development of coronary artery lesions (CAL).Methods All children diagnosed as IKD from Jan.2005 to Apr.2011 in our department were reviewed retrospectively for their clinical data,laboratory values and treatment measures.Results Eight of the children (8/36,22.2% ) were positive for CAL.The count of white blood cell( WBC),count of platelet(PLT),hematocrit and C reactive protein(CRP) level were ( 18.36 ± 4.63) × 109/L,(450.30 ± 155.40) × 109/L,( 25.63 ± 3.53 ),( 18.30 ± 3.80) mg/L and ( 13.48 ±3.27) × 109/L,(350.60 ± 56.80) × 109/L,( 33.78 ± 2.24 ),(9.70 ± 2.50) mg/L in the CAL group and non-CAL group respectively.And there were significant differences on the four indexes between CAL group and non-CAL group ( t =2.58,2.65,2.73,2.48,respectively,P < 0.05 ).Pyretolysis time of children first undergoing globulin treatment was (2.5 ± 1.5 ) d and ( 1.5 ± 1.0 ) d in children under 1-year old and those above l-year old respectively,and the difference was significant ( t =2.35,P < 0.05).Conclusion CAL should be cautiously prevented in IKD infants under l-year old with a fever lasting for over 5 days,decreased hematocrit and elevated WBC count,PLT count,Hct and CRP.Infant IKD patients are not so sensitive to intravenous gamma globulin and tend to occur CAL.They should be given an early diagnosis and timely treatment.%目的 探讨小儿不完全川崎病(IKD)的危险因素,以减少冠状动脉损害(CAL).方法 按照IKD诊断标准,对2005年1月至2011年4月在我院儿科住院的36例IKD患儿临床资料、实验室资料及治疗进行分析.结果 36例IKD患儿中发生CAL 8例(22.2%).合并CAL组与无CAL组白细胞计数分别为(18.36±4.63)、(13.48±3.27)×109/L,血小板计数分别为(450.30±155.40)、(350.60±56.80)×109/L,红细胞比容分别为(0.25±0.03)、(0.33±0.02),C反应蛋白分别为(18.30±3.80)、(9.70±2.50) mg/L,

  4. An Acute Hemorrhagic Infectious Disease:Ebola Virus Disease

    Institute of Scientific and Technical Information of China (English)

    JIAO Lei; XU An-hua; FENG Chao; QIU Qian-qian; TANG Qi-ling; LIU Xiao-huan

    2014-01-01

    Ebola virus disease (EVD) is an acute hemorrhagic infectious disease caused by ebola virus, with high infectivity and fatality rate. At present, it mainly occurs in areas of Central Africa and West Africa and no effective vaccine and antiviral drugs are available for the clinical treatment.

  5. 临床护理路径在川崎病患儿治疗中的应用效果观察%Application effect observation of clinical nursing path for children with Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    陈燕丹; 谢泽漫; 黄楚芝

    2015-01-01

    目的:探究临床护理路径在川崎病患儿治疗中的应用效果。方法41例川崎病患儿按照入院顺序分为观察组21例和对照组20例,观察组患儿实施临床护理路径,对照组患儿采取常规护理措施,比较两组患儿临床效果。结果观察组患儿平均住院天数和平均住院费用分别为(6.5±1.5)d、(2696.3±64.9)元,与对照组的(10.0±1.5)d、(3859.6±50.3)元相比占显著优势,差异具有统计学意义(P<0.01);观察组患者对护理工作的总满意度为90.48%,与对照组的65.00%相比明显升高,差异具有统计学意义(P<0.05)。结论临床护理路径应用在川崎病患儿的治疗中具有重要的临床意义,可以有效缩短住院时间,减少住院费用,提高护理质量,建议广泛使用。%Objective To explore the application effect of clinical nursing path for children with Kawasaki disease. Methods A total of 41 children with Kawasaki disease were s divided into observation group with 21 cases and control group with 20 cases, according to their admission order. The observation group received clinical nursing path, and the control group received conventional nursing measures. The clinical effects were compared between the two groups. Results The average hospital stays and average hospitalization cost of the observation group were (6.5±1.5) d and (2696.3±64.9) yuan, which were better than (10.0±1.5) d and (3859.6±50.3) yuan of the control group, and the difference had statistical significance (P<0.01). The total satisfaction of nursing was 90.48% in the observation group, which was obviously higher than 65.00% in the control group, and the difference was statistically significant (P<0.05). Conclusion The application of clinical nursing path in the treatment of children with Kawasaki disease has important clinical significance, and this method can effectively reduce hospital stays and hospitalization cost, and improves the nursing quality as well

  6. Seasonal trend of acute pelvic inflammatory disease.

    Science.gov (United States)

    Xholli, Anjeza; Cannoletta, Marianna; Cagnacci, Angelo

    2014-05-01

    Many infections follow a seasonal trend. Aim of our study was to check whether acute pelvic inflammatory disease (PID) follows a seasonal progress. In a retrospective study on 12,152 hospital records, 158 cases of acute pelvic inflammatory disease were identified. Periodogram analysis was applied to the date of pelvic inflammatory disease admission and to related environmental factors, such as temperature and photoperiod. Pelvic inflammatory disease follows a seasonal rhythm with mean to peak variation of 23 % and maximal values in September (±37.2 days). The rhythm, more evident in married women, is related to the rhythm of temperature advanced by 2 months and of photoperiod advanced by 3 months. Cases of pelvic inflammatory disease are more frequent than expected in unmarried (36 vs. 17.3/34,626, p = 0.015), particularly divorced women 30-40 years of age. Our study evidences a seasonal trend and confirms unmarried, particularly divorced status, as important risk factor for acute pelvic inflammatory disease.

  7. Analysis of 11 cases of mycoplasma pneumoniae infection combined with Kawasaki disease%川崎病合并肺炎支原体肺炎11例分析

    Institute of Scientific and Technical Information of China (English)

    朱巧; 过建春

    2013-01-01

    Objective To study the clinical characteristics of pediatric Kawasaki disease complicating mycoplasma pneumoniae pneumonia.Methods Retrospective analysis was conducted on 11 children who had been diagnosed with Kawasaki disease with Mycoplasma pneumoniae pneumonia.Results The 11 cases presented with varying degrees of fever,conjunctival congestion,skin rashes,lymphadenectasis,distal extremities lesions,heart and lung lesions.8 of them were standartly treated with azithromycin,while 3 of them were treatad with azithromycin and erythromycin.2 patients with pleural effusion complicated by lobar pneumonia consolidation were treated with gamma globulin combined aspirin.All of the 11 patients were healed.Conclusion Infections are common at the diagonosis of KD.Reasonable examination and antibiotics is useful to cure KD with MPP.%目的 分析川崎病(KD)合并肺炎支原体肺炎(MPP)临床特点,以提高对KD合并MPP的认识和诊治水平.方法 对临床诊断为川崎病的11例患儿的临床资料进行回顾性分析.结果 11例患儿出现不同程度的发热、眼结膜充血、皮疹、淋巴结肿大、四肢末梢病变、心脏及肺部病变等情况;8例患儿采用阿奇霉素规范治疗,3例患儿采用阿奇霉素并红霉素治疗,其中2例大叶性肺炎实变合并胸腔积液患儿应用丙种球蛋白联合阿司匹林治疗,均痊愈.结论 肺炎支原体肺炎导致的免疫紊乱可能与川崎病的发生有关,抗感染治疗可改善预后.

  8. 干酪乳杆菌细胞壁萃取物诱导小鼠川崎病模型研究进展%Progress of lactobacillus casei cell wall extract-induced mouse model of Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    高立超(综述); 龚方戚(审校)

    2014-01-01

    川崎病在发达国家已经取代风湿热成为儿童最常见的后天性心脏病,但其病因仍然不明。川崎病动物模型可以帮助我们更好地研究川崎病和制定新的有效的治疗方案。在已有的动物模型中,单次腹腔注射干酪乳杆菌细胞壁萃取物诱导小鼠冠状动脉炎模型较为成熟。该文就干酪乳杆菌细胞壁萃取物诱导的小鼠川崎病模型的建立及其相关研究进展作一综述。%Kawasaki disease has replaced rheumatic fever as the most common acquired heart disease in childhood,but its etiology remains unknown. The development and analyses of animal models will help us under-stand KD and find new and effective therapeutic strategies for it. Among these existing animal models, mouse modelof coronary artery inflammation induced by a single intraperitoneal injection of lactobacillus casei cell wall extract is a relatively mature model. In this review,we present an overview of how this model established and some progress in research of the mouse model.

  9. 进一步提高川崎病的临床诊疗水平%Further improve the diagnostic and therapeutic level for Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    杜军保

    2006-01-01

    川崎病(Kawasaki disease,KD)是一种急性、自限性的全身血管炎,1967年由日本Tomisaku Kawasaki首次报道。该病主要累及婴儿和年幼的儿童。据统计,1991-1992年全日本5岁以下儿童发病率为90/10万,1997-1998年为112/10万。但是至今为止,我国KD的发病率还不清楚,上海和北京地区的发病率分别为16.8-36.8/10万和26-31/10万。今后在我国还应进一步开展全国范围大样本、多中心、前瞻性的研究,以便寻找出该病在我国的流行病学特点。

  10. Treatment of Acute Pelvic Inflammatory Disease

    Directory of Open Access Journals (Sweden)

    Richard L. Sweet

    2011-01-01

    Full Text Available Pelvic inflammatory disease (PID, one of the most common infections in nonpregnant women of reproductive age, remains an important public health problem. It is associated with major long-term sequelae, including tubal factor infertility, ectopic pregnancy, and chronic pelvic pain. In addition, treatment of acute PID and its complications incurs substantial health care costs. Prevention of these long-term sequelae is dependent upon development of treatment strategies based on knowledge of the microbiologic etiology of acute PID. It is well accepted that acute PID is a polymicrobic infection. The sexually transmitted organisms, Neisseria gonorrhoeae and Chlamydia trachomatis, are present in many cases, and microorganisms comprising the endogenous vaginal and cervical flora are frequently associated with PID. This includes anaerobic and facultative bacteria, similar to those associated with bacterial vaginosis. Genital tract mycoplasmas, most importantly Mycoplasma genitalium, have recently also been implicated as a cause of acute PID. As a consequence, treatment regimens for acute PID should provide broad spectrum coverage that is effective against these microorganisms.

  11. A 3-year study of patients with Kawasaki's disease complicated with coronary artery lesion%超声随访川崎病并发冠状动脉病变的3年分析

    Institute of Scientific and Technical Information of China (English)

    莫汉有; 周润华; 刘雅妮; 石宇红; 许佳; 杨敏; 廖东; 李明; 张华

    2011-01-01

    Objective To investigate the prognosis of patients with Kawasaki's disease complicated with coronary artery lesion and to provide evidence for diagnosis and treatment of these patients. Methods This study was conducted during January 2002 to June 2007. All patients diagnosed as Kawasaki's disease complicated with coronary artery lesions were from the Affiliated Hospital of Guilin Medical College, the Second People's Hospital of Guangxi Province and Guilin Women and Childrens' Hospital. All cases were echocardiogram examined in month 3, 6, 12, 24 and 36 in the purpose of observing the morphology of coronary artery. The study subjects were re-categorized to the groups of mild, moderate dilatation of coronary artery and giant coronary aneurysm, based on the severity of coronary artery lesion. The results of these results of the three groups were compared. Results Eighty-four cases in the mild group, 27 cases in the moderate group and 8 cases in the giant coronary aneurysm. The recovery cases were 23 (27%), 3 (11%) and 0 in the 3 groups respectively at month 3. The above numbers were 44 (52%), 8 (30%) and 0 respectively at month 6.The numbers were 69(82%), 13 (48%) and 1 (13%) at month 12. The numbers were 78 (93%), 19 (70%), 3 (38%) at month 24. The numbers were 82(98%), 20(74%) and 4(50%) at month 36. Thirteen patients were treated with adenosine-triphosphate (ATP) stress echocardiography examiantion, 5 patients were evaluated by coronary angiography,and 4 patients were tested by 64-slice CT coronary reconstruction. Part of the patients were found to have coronary stenosis or occlusion. Conclusion Patients of Kawasaki's disease often have concurrent coronary artery lesions. Patients with mild dilatation of the coronary artery are the most commonly seen and have the best prognosis. On the contrast, patients with giant coronary aneurysm are the lest common situation and is the worst in prognosis. Part of them will develop coronary artery stenosis or occlusion in late

  12. The Value of Heart Fatty Acid Binding Protein in Early Diagnosis of Kawasaki Disease with Myocardial Ischemia%心肌脂肪酸结合蛋白在川崎病心肌缺血损伤早期诊断中的应用价值

    Institute of Scientific and Technical Information of China (English)

    段庆宁

    2013-01-01

    目的:探讨心肌脂肪酸结合蛋白(h-FABP)在川崎病患儿心肌缺血损伤早期诊断中的应用价值。方法分别测定54例川崎病患儿在急性期、亚急性期、恢复期的h-FABP,并选择50例健康患儿为对照组。应用SPSS13.0软件对结果进行统计学分析。结果川崎病患儿各期h-FABP水平均明显高于对照组(P<0.05)。结论h-FABP可作为川崎病心肌缺血损伤早期判断指标。%Objective To investigate the value of heart fat y acid binding protein in early diagnosis of kawasaki disease (KD) complicated with myocardial ischemia. Methods Heart fat y acid binding protein (h-FABP) levels were measured in acute stage, subacute stage and recovery stage of KD Patients, and 50 healthy controls as wel .The results were analyzed by statistical package for the social science 13.0 (SPSS 13.0). Results The h-FABP levels of al stages in KD were obviously higher than control group (P<0.05). Conclusion H-FABP can be used in early diagnosis of myocardial ischemia in KD.

  13. Chagas' Disease: an acute transfusional case report

    Directory of Open Access Journals (Sweden)

    Dalva Marli Valério Wanderley

    1988-12-01

    Full Text Available Report of a case of acute transfusional Chagas'disease in a four-year-old child with a previous diagnosis of acute lymphocytic leukemia, transmitted in São Paulo, the Capital of São Paulo State, Brazil. Epidemiological investigation disclosed the donor's serological positivity and his previous residence in an area where Chagas' disease is endemic. The importance of adequate sorological screening in blood donors is evident. It should be stressed that this is the first case notified to the Superintendência de Controle de Endemias (SUCEN (Superintendency for the Endemy Control of the State Secretariat of Health, São Paulo, for the last five years.

  14. Acute recurrent pancreatitis: An autoimmune disease?

    Institute of Scientific and Technical Information of China (English)

    Raffaele Pezzilli

    2008-01-01

    In this review article,we will briefly describe the main characteristics of autoimmune pancreatitis and then we will concentrate on our aim,namely,evaluating the clinical characteristics of patients having recurrence of pain from the disease.In fact,the open question is to evaluate the possible presence of autoimmune pancreatitis in patients with an undefined etiology of acute pancreatitis and for this reason we carried out a search in the literature in order to explore this issue.In cases of recurrent attacks of pain in patients with "idiopathic"pancreatitis,we need to keep in mind the possibility that our patients may have autoimmune pancreatitis.Even though the frequency of this disease seems to be quite low,we believe that in the future,by increasing our knowledge on the subject,we will be able to diagnose an ever-increasing number of patients having acute recurrence of pain from autoimmune pancreatitis.

  15. Clinical Analysis of 3 Cases with Abdominal Distension as the Main Manifestation of Kawasaki Disease%3例以腹胀为首发表现川崎病的临床特点分析

    Institute of Scientific and Technical Information of China (English)

    徐凤琴; 毕良学; 杨泽玉

    2013-01-01

    Objective To analysis clinical features of Kawasaki disease (KD) with abdomen distension as the main manifestations, and offer references to clinical diagnosis. Methods Analyze clinical information of 3 cases with KD, to understand especial clinical appearance of KD presented abdomen distension as the main manifestations. Results All the 3 cases of abdominal distension accompanied by fever, and abdomen distension was the first clinical manifestation. Conclusion The individual difference of clinical symptoms for KD was great , children with abdominal distension and fever of unknown origin probably be considered as KD.%  目的分析以腹胀为主要表现川崎病的临床特点,为不完全川崎病的诊断提供参考。方法对3例川崎病患儿病例资料进行综合分析,了解腹胀为主要甚至首发表现的川崎病临床特点。结果3例川崎病患儿腹胀伴有发热,腹胀为首要临床表现。结论川崎病临床症状个体差异变化很大,腹胀并有不明原因发热的患儿应该考虑川崎病的可能。

  16. 川崎病患儿冠状动脉的CT血管造影及磁共振血管造影%Computed tomography angiography and magnetic resonance angiography evaluate coronary artery in children with Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    胡喜红; 黄国英

    2014-01-01

    Kawasaki disease can affect the coronary arteries,including coronary artery dilation,aneurysm,stenosis and thrombus.Conventional coronary angiography is the gold standard for coronary artery evaluation,but there are risks associated with its invasive nature and with the exposure to contrast agents and radiation.With the rapid development,computed tomography angiography and magnetic resonance angiography become the noninvasive imaging modalities to evaluate the coronary artery.%川崎病容易累及冠状动脉,可导致冠状动脉扩张、冠状动脉瘤形成、狭窄或闭塞等.经导管冠状动脉血管造影是传统评价冠状动脉病变的方法,但其存在一定的侵入性.近年来CT和磁共振技术迅速发展,CT血管造影和磁共振血管造影逐渐成为评估冠状动脉病变的重要无创检查手段.

  17. Fatal acute Chagas Disease in a Chimpanzee

    Science.gov (United States)

    2009-08-01

    protozoan parasite belonging to the order Kinetoplastida, family Trypanosomatidae. Arthropod vectors (Reduviidae, assassin bugs or cone nosed bugs or...Laranja FS. Experimental Chagas’ disease in rhesus monkeys. I. Clinical, parasitological , hematological and anatomo- pathological studies in the acute...of infection and immunity from mother to young. Parasitology . 1972; 65:1–9. [PubMed: 4626452] 21. Miles MA, Marsden PD, Pettitt LE, Draper CC, Watson

  18. Treated with intravenous immunoglobulin in children with Kawasaki disease%川崎病的静脉注射免疫球蛋白治疗

    Institute of Scientific and Technical Information of China (English)

    杜军保; 李晓惠

    2007-01-01

    川崎病(Kawasaki disease,KD)的病因目前尚未完全明了,尽管许多证据表明其发病可能与感染有关,但是尚未被证实。已经证实的是本病急性期存在以免疫活化细胞激活为主要改变的免疫调节异常,主要病理变化为全身性非特异性血管炎,多侵犯冠状动脉,部分患儿形成冠状动脉瘤(Coronary aneurysm,CAA),其中少部分患儿冠状动脉可发生狭窄或血栓,甚至导致心肌梗死,因此成为小儿常见的获得性心脏病。目前本病尚无特异疗法,急性期治疗的目的是控制全身非特异性血管炎症,防止冠状动脉瘤形成及血栓性阻塞。治疗措施及药物包括:静脉注射丙种球蛋白(intravenous immune globulin,IVIG或intravenous gamma globulin,IVGG)、阿司匹林(Asprin,ASA)、糖皮质激素、其他治疗及IVIG无反应者治疗。现就川崎病的静脉注射免疫球蛋白治疗谈以下观点。

  19. Seasonality and temporal clustering of Kawasaki syndrome.

    Science.gov (United States)

    Burns, Jane C; Cayan, Daniel R; Tong, Garrick; Bainto, Emelia V; Turner, Christena L; Shike, Hiroko; Kawasaki, Tomisaku; Nakamura, Yosikazu; Yashiro, Mayumi; Yanagawa, Hiroshi

    2005-03-01

    The distribution of a syndrome in space and time may suggest clues to its etiology. The cause of Kawasaki syndrome, a systemic vasculitis of infants and children, is unknown, but an infectious etiology is suspected. Seasonality and clustering of Kawasaki syndrome cases were studied in Japanese children with Kawasaki syndrome reported in nationwide surveys in Japan. Excluding the years that contained the 3 major nationwide epidemics, 84,829 cases during a 14-year period (1987-2000) were analyzed. To assess seasonality, we calculated mean monthly incidence during the study period for eastern and western Japan and for each of the 47 prefectures. To assess clustering, we compared the number of cases per day (daily incidence) with a simulated distribution (Monte Carlo analysis). Marked spatial and temporal patterns were noted in both the seasonality and deviations from the average number of Kawasaki syndrome cases in Japan. Seasonality was bimodal with peaks in January and June/July and a nadir in October. This pattern was consistent throughout Japan and during the entire 14-year period. Some years produced very high or low numbers of cases, but the overall variability was consistent throughout the entire country. Temporal clustering of Kawasaki syndrome cases was detected with nationwide outbreaks. Kawasaki syndrome has a pronounced seasonality in Japan that is consistent throughout the length of the Japanese archipelago. Temporal clustering of cases combined with marked seasonality suggests an environmental trigger for this clinical syndrome.

  20. Phytotherapy of Acute Respiratory Viral Diseases

    Directory of Open Access Journals (Sweden)

    I.B. Ershova

    2017-02-01

    Full Text Available Nowadays phytotherapy is increasingly being implemented into medical practice, especially for the prevention and treatment of many diseases. Acute respiratory viral infections are most common in childhood and in adults. Acute rhinitis, pharyngitis, tonsillitis, sinusitis, nasopharyngitis and acute laryngitis refer to diseases of the upper respiratory tract. The main reason for respiratory diseases in recurrent respiratory infection child is disorders of mucociliary and immune protection. The therapeutic value of medicinal plants is determined by their biologically active substances. The method of application of phytotherpy is an integral part of traditional medicine. Herbal medicine can be used at home and does not require special equipment. The main indications for the herbal medicine use in pediatrics are the initial stage of the disease as a primary method of treatment due to mild and low toxicity; as a supporting treatment for enhancing the protective forces of the child’s body during the disease deterioration. During the recovery period herbal medicine again occupies a leading position, especially in case of chronic diseases because it can be used for a long time and is well combined with synthetic drugs. The terms of appointment of herbs for children: prescription of medicinal plants for children must be individual according to indications, taking into account the child’s age; it is recommended to take into account the form and nature of the course of the main disease and comorbidities as well; at the initial stage of the treatment it is better to use some medicinal plants or species consisting of 2–3 plants and in the future a more complex composition; therapy with medicinal plants requires a long period to be used use, especially in chronic diseases; in the treatment of chronic diseases a good effect preventive courses of herbal medicine was revealed, which are appointed during seasonal exacerbations; in case of intolerance

  1. 维生素D与川崎病的关系及作用机制研究进展%Research advances in association between vitamin D and Kawasaki disease and related mechanisms of action

    Institute of Scientific and Technical Information of China (English)

    杨雪(综述); 董湘玉(审校)

    2016-01-01

    维生素D是重要的类固醇激素,具有广泛的生物学效应,是心血管疾病及其他多种疾病的保护因素。目前川崎病(KD)的病因及发病机制尚不清楚,但近年来的研究发现,维生素D血清水平不足或缺乏与KD存在相关性。维生素D不足或缺乏可能通过影响炎性反应、脂肪细胞因子、内皮功能、血小板功能及DNA甲基化等途径影响KD,增加其冠脉损伤发生的风险。该综述就维生素D与KD的关系及其可能存在的多种作用机制的研究进展进行阐述。%Vitamin D is an important steroid hormone, which has a wide biological effect and is the protective factor against cardiovascular disease and other diseases. At present, the etiology and pathogenesis of Kawasaki disease (KD) remain unknown, but recent studies have shown that vitamin D insufifciency or deifciency is associated with KD. Vitamin D insufifciency or deifciency may affect KD via its inlfuence on inlfammatory response, adipokine, endothelial function, platelet function, and DNA methylation and increase the risk of coronary artery lesions. This article reviews the research advances in the association between vitamin D and KD and possible mechanisms of action.

  2. Crohn's disease and acute pancreatitis. A review of literature.

    Science.gov (United States)

    Jasdanwala, Sarfaraz; Babyatsky, Mark

    2015-03-20

    Crohn's disease, a transmural inflammatory bowel disease, has many well-known extra-intestinal manifestations and complications. Although acute pancreatitis has a higher incidence in patients with Crohn's disease as compared to the general population, acute pancreatitis is still relatively uncommon in patients with Crohn's disease. Patients with Crohn's disease are at an approximately fourfold higher risk than the general population to develop acute pancreatitis. The risk of developing acute pancreatitis is higher in females as compared to males. Acute pancreatitis can occur at any age with higher incidence reported in patients in their 20s and between 40-50 years of age. The severity and prognosis of acute pancreatitis in patients with Crohn's disease is the same as in general population. Acute pancreatitis can occur before onset of intestinal Crohn's disease, this presentation being more common in children than adults. It can also occur as the presenting symptom. However, most commonly it occurs after intestinal symptoms have manifest with a mean time interval between the initial presentation and development of acute pancreatitis being 2 years. There are several etiological factors contributing to acute pancreatitis in patients with Crohn's disease. It is not clear whether acute pancreatitis is a direct extra-intestinal manifestation of Crohn's disease; however, majority of the cases of acute pancreatitis in patients with Crohn's disease are due to GS and medications. Drugs used for the treatment of Crohn's disease that have been reported to cause acute pancreatitis include 5-ASA agents, azathioprine and 6 mercaptopurine, metornidazole and corticosteroids. Recent evidence has emerged correlating both type 1 and 2 autoimmune pancreatitis with Crohn's disease. Understanding the association between the two disease entities is key to effectively manage patients with Crohn's disease and acute pancreatitis.

  3. 基质金属蛋白酶及其抑制剂与川崎病冠脉损害的研究进展%ASSOCIATION OF MMPS AND TIMPS WITH CAL IN KAWASAKI DISEASE

    Institute of Scientific and Technical Information of China (English)

    黄芳芳; 饶晓红; 朱红枫

    2011-01-01

    The main complication of the Kawasaki disease (KD) is coronary artery lesions (CAL) , and it has become one of the most common acquired heart diseases in children. Recent studies show that CAL is related to the degradation of the vascular wall extracellular matrix, and the degradation of the vascular wall extracellular is related to the activation of MMPs and (or) the TIMPs out of balance. Therefore, matrix metalloproteinases (MMPs) and its inhibitors (TIMPs) play an important role in occurrence and development of coronary lesions. Application of TIMPs to regulaLe the level of MMPs is a new direction to treatment and prevention of coronary artery lesions.%冠状动脉损害(CAL)是川崎病(KD)的主要并发症,现已成为儿童最常见的后天性心脏病之一.近年来研究表明,川崎病冠状动脉损害与血管壁细胞外基质(ECM)降解有关,而血管壁细胞外基质降解与基质金属蛋白酶(matrix metalloproteinases,MMPs)激活和(或)与其组织抑制剂(tissue inhibitor of matrix metalloprotein-ases,TIMPs)的平衡被破坏有关.因此,基质金属蛋白酶(MMPs)及其抑制剂(TIMPs)在川崎病冠脉损害的发生发展中起着重要作用,应用TIMPs对MMPs水平进行调控是KD治疗及预防冠脉并发症的一个新方向.

  4. 川崎病冠状动脉病变心肌缺血的诊断方法%Diagnostic methods of myocardial ischemia in Kawasaki disease with coronary artery lesions

    Institute of Scientific and Technical Information of China (English)

    黄美容

    2014-01-01

    Kawasaki disease (KD) without proper treatment,the incidence of coronary artery lesions is up to 25%.Some of them develope of coronary artery aneurysms (CAA),obstructive coronary artery disease(stenosis,occlusion or thrombosis).KD with CAA in children need long term follow-up.Electrocardiogram(ECG) and two-dimensional echocardiography(2DE) are still the basic means of checking for follow-up.KD with CAA who should be preferred magnetic resonance imaging examination.Dobutamine stress echocardiography or coronary angiography should be done with suggestive of myocardial ischemia in ECG or 2DE.%川崎病(KD)未经适当治疗,冠状动脉病变的发生率可达25%.其中部分发展为冠状动脉瘤(CAA)及冠状动脉阻塞性病变(狭窄、闭塞或血栓形成).对KD合并CAA的患儿需进行长期随访.目前仍以心电图(ECG)和二维超声心动图(2DE)为基本检查手段,对KD合并CAA者,应首选磁共振成像检查,ECG和2DE提示有心肌缺血可能时可行多巴酚丁胺负荷超声心动图检查或直接行冠状动脉造影检查,及时发现冠状动脉狭窄,评估心肌缺血程度,为进一步治疗提供依据.

  5. Clarithromycin-induced acute interstitial nephritis and minimal change disease

    OpenAIRE

    Russell, Wendy; Smith, William

    2009-01-01

    Drug associated acute interstitial nephritis and minimal change disease has been well documented but the simultaneous presentation of both is rare and has not been reported with clarithromycin. We describe a case of simultaneous acute tubulointerstitial nephritis and minimal change disease induced by clarithromycin. The patient had acute kidney injury, nephrotic syndrome, eosinophilic pneumonitis and a maculopapular skin rash. The role of steroid therapy in acute interstitial nephritis is con...

  6. CT findings of acute pelvic inflammatory disease.

    Science.gov (United States)

    Lee, Mi Hee; Moon, Min Hoan; Sung, Chang Kyu; Woo, Hyunsik; Oh, Sohee

    2014-12-01

    To determine the computed tomographic (CT) findings of acute pelvic inflammatory disease (PID). This retrospective, single-institution case-control study was approved by our institutional review board, and the informed consent was waived owing to the retrospective nature of the study. CT images of 32 women with clinically proven acute PID and 32 control subjects with other conditions of similar presentation were retrospectively reviewed. Analysis of CT findings included hepatic capsular enhancement, pelvic fat haziness, complicated ascites, uterine serosal enhancement, tubal thickening, endometritis, and oophoritis. Comparison of CT findings was performed with the Chi square test or the Fisher exact test and logistic regression analysis was used to determine significant CT findings in predicting PID. The CT findings that showed a statistically significant difference were hepatic capsular enhancement on late arterial phase (p = 0.003), pelvic fat haziness (p = 0.045), and tubal thickening (p = 0.001). Subsequent multivariate logistic regression analysis revealed that the presence of hepatic capsular enhancement on late arterial phase and tubal thickening were significant predictors of PID (hepatic capsular enhancement on late arterial phase, p = 0.015, odds ratio [OR] = 4.8; tubal thickening, p = 0.005, OR = 10.5). Diagnostic morphological CT findings in women with clinically proven PID and acute abdominal pain include hepatic capsular enhancement on late arterial phase and tubal thickening.

  7. Transvaginal sonography of acute pelvic inflammatory disease

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Jin Soo; Kim, Young Hwa; Shin, Hyung Chul; Han, Gun Soo; Kim, Il Young [Chonan Hospital, Soonchunhyang University College of Medicine, Chonan (Korea, Republic of)

    1999-12-15

    To determine the value of transvaginal sonography in evaluating women with acute pelvic inflammatory disease (PID). Transvaginal sonography was performed in 25 patients with clinically suggested PID during recent 36 months. The sonographic findings of fallopian tubes and ovaries were analyzed and correlated with pathological findings of 2 fallopian tubes and 19 ovaries in 16 patients who had operations. The correct diagnosis of acute PID was made in 20/25 (80%) by transvaginal sonography. the abnormal sonographic findings of the fallopian tube include tubal thickening or dilatation with internal echo. The sensitivity, specificity, and accuracy for tubal abnormality were 88%, 96%, and 86% , respectively. Ovarian changes were seen on TVS in 14/19 (73%), which include multiple follicular enlargement in 5, tubo-ovarian complex in 9 (tubo-ovarian adhesion in 3, tubo-ovarian abscess in 6). At surgery, the ovay was not involved in all three women who showed tubo-ovarian adhesion on TVS. Among 6 women who showed tubo-ovarian abscess on TVS, tubo-ovarian abscess was confirmed in 3 and the remaining 3 had ovarian cysts. Trandvaginal sonography, a facilitative and accurate modality, is highly sensitive in detecting the abnormality of the tube and useful in differentiating the tubo-ovarian complex in patients with acute PID.

  8. Long term follow-up study on Kawasaki disease complicated by coronary arterial aneurysm by coronary angiography and echocardiography in children%儿童川崎病并冠状动脉瘤冠状动脉造影及心脏超声长期随访

    Institute of Scientific and Technical Information of China (English)

    成胜权; 刘建平; 刘丽文; 邓跃林; 孙立军; 牛永春

    2011-01-01

    @@ 川崎病(Kawasaki disease,KD)基本病理改变为全身性血管炎,主要侵犯大、中血管,其中冠状动脉(简称冠脉)是最常受损的血管,冠状动脉瘤(coro-nary artery aneurysm,CAA)和冠状动脉狭窄(简称冠脉狭窄)最为严重,可致缺血性心脏病、心肌梗死和猝死.

  9. Acute renal dysfunction in liver diseases

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Renal dysfunction is common in liver diseases, either as part of multiorgan involvement in acute illness or secondary to advanced liver disease. The presence of renal impairment in both groups is a poor prognostic indicator. Renal failure is often multifactorial and can present as pre-renal or intrinsic renal dysfunction. Obstructive or post renal dysfunction only rarely complicates liver disease. Hepatorenal syndrome (MRS) is a unique form of renal failure associated with advanced liver disease or cirrhosis, and is characterized by functional renal impairment without significant changes in renal histology. Irrespective of the type of renal failure, renal hypoperfusion is the central pathogenetic mechanism, due either to reduced perfusion pressure or increased renal vascular resistance. Volume expansion, avoidance of precipitating factors and treatment of underlying liver disease constitute the mainstay of therapy to prevent and reverse renal impairment. Splanchnic vasoconstrictor agents, such as terlipressin, along with volume expansion, and early placement of transjugular intrahepatic portosystemic shunt (TIPS) may be effective in improving renal function in HRS. Continuous renal replacement therapy (CRRT) and molecular absorbent recirculating system (MARS) in selected patients may be life saving while awaiting liver transplantation.

  10. 以无菌性脓尿为首发症状的婴儿不完全性川崎病%Infant with incomplete Kawasaki disease present with sterile pyuria

    Institute of Scientific and Technical Information of China (English)

    黄华; 茅幼英; 殷蕾; 周纬

    2013-01-01

    Objectives To explore the clinical features of incomplete Kawasaki disease (IKD) which presents with sterile pyuria in infant, in order to achieve early diagnosis and treatment. Methods A retrospective study of 15 infants with IKD was conducted in comparison with 29 patients who were diagnosed with KD according to the diagnostic criteria of American Heart Association 2004. Results Both KD and IKD groups had recurrent fever and poor response to antibiotic therapy. Hemoglobin level was signiifcantly lower in IKD group than in KD group at the onset or 7-d after onset (P<0.05). C-reaction protein (CRP) level was signiifcantly higher in IKD group than that in KD group at 7-d after onset (P<0.05). There were 4 patients with coronary artery abnormality, 2 patients with coronary artery dilation and 2 patients with coro-nary artery aneurysm. Conclusions For infant <1 year old who has pyuria ifrstly, continuous fever for over 5 days when treated by antibiotics and increasing serum inlfammatory markers, incomplete Kawasaki disease should be suspected and the cardiac ultrasound is recommended.%目的:探讨以无菌性脓尿为首发症状的婴儿不完全性川崎病(KD)的临床特点,以期早期诊断和治疗。方法回顾性分析以无菌性脓尿为首发症状的15例婴儿不完全性KD患儿的临床资料,与同期符合美国心脏协会2004年修订诊断标准的29例典型KD患儿进行比较。结果两组患儿均以反复高热、抗感染效果不佳为主要表现。与典型KD组相比,不完全性KD组在发病初期和发病7 d后血红蛋白较低,发病7 d后CRP较高,差异均有统计学意义(P<0.05)。4例不完全性KD患儿心脏超声检查发现冠状动脉变化,其中动脉扩张2例,冠脉瘤2例。结论对<1岁以尿路感染为首发症状的婴儿在治疗过程中如发热持续5d以上,伴血清炎症指标明显增高,且抗生素治疗无效时,应警惕不完全性川崎病可能,并尽早做心

  11. Clinical Research in Kawasaki Disease with Mycoplasma Pneumoniae Infection in Children%川崎病合并肺炎支原体感染患儿的临床研究

    Institute of Scientific and Technical Information of China (English)

    周益平; 符辰璐

    2016-01-01

    Objective To analyze clinical characteristics and laboratory data of children with mycoplasma pneumoniae (MP) infection of Kawasaki disease (KD).MethodsTotally 62 KD children with MP infection who were hospitalized from January 2010 to January 2016 in the First People's Hospital of Changzhou were divided into KD with MP group (18 children) as the MP group, and KD without MP group (44 children) as the control group. The blood routine, blood sedimentation, CRP and thermal were determined.Results The neutrophilic granulocyte count, leucocyte count, hematocrit, platelet count and erythrocyte sedimentation rate showed no difference between MP group and control group (P>0.05). CRP and thermal process showed significant difference between MP group and control group (P<0.05). Also the coronary artery expansion (coronary artery dilated, CAD) was signiifcantly different between the two groups (P<0.05). In the course of 5 to 10 days, both groups were given intravenous immunoglobulin (IVIG) 1 g/(kg•d) for 2 days, and aspirin 50 mg/(kg•d) (oral administration of three times). Then the MP group was given azithromycin for the sequential therapy.Conclusion MP infection may be one of the causes of KD. MP infection can induce or worsen the condition of KD children, and increase the risk of coronary artery expansion, ifnally would prolong the course and affect the prognosis.%目的:探讨川崎病(Kawasaki disease, KD)患儿合并肺炎支原体(mycoplasma pneumoniae,MP)感染患儿的临床特征。方法回顾性分析于2010年1月—2016年1月收治常州市第一人民医院儿科临床诊断为KD的62例患儿,其中合并MP感染的18例为MP组,其余44例为对照组。比较两组血常规、血沉、C反应蛋白(CRP)、热程等指标。结果 MP组与对照组患儿血白细胞总数、中性粒细胞计数、红细胞压积、血小板计数及血沉差异均没有统计学意义(P>0.05)。MP组热程较对照组长,CRP高于对照组

  12. A case of acute viral hepatitis interfering with acute fatty liver disease of pregnancy

    Directory of Open Access Journals (Sweden)

    Abdulkadir Turgut

    2013-03-01

    Full Text Available Acute hepatitis A is a rarely seen infection during pregnancy.In terms of clinical and laboratory findings, it can beinterfere with acute fatty liver disease which can be quitemortal during pregnancy. Since liver function tests are elevatedin both conditions, hepatitis A infection should alsobe kept in mind in differential diagnosis. We present a 30year-old pregnant woman with 35 weeks of gestation whopresented to our clinic with a suspection of acute fattyliver disease but finally diagnosed as acute hepatitis A infection.J Clin Exp Invest 2013; 4 (1: 123-125Key words: Hepatitis A, pregnancy, acute fatty liver disease

  13. Pathophysiology of acute small bowel disease with CT correlation

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    Sarwani, N., E-mail: nsarwani@hmc.psu.ed [Department of Radiology, Section of Abdominal Imaging, Penn State Milton Hershey Medical Center, Hershey, PA (United States); Tappouni, R.; Tice, J. [Department of Radiology, Section of Abdominal Imaging, Penn State Milton Hershey Medical Center, Hershey, PA (United States)

    2011-01-15

    The objective of this article is to review the pathophysiology of acute small bowel diseases, and to correlate the mechanisms of disease with computed tomography (CT) findings. Disease entities will be classified into the following: immune mediated and infectious causes, vascular causes, mechanical causes, trauma, and others. Having an understanding of acute small bowel pathophysiology is a useful teaching tool, and can lead to imaging clues to the most likely diagnosis of acute small bowel disorders.

  14. 川崎病冠状动脉血栓患儿8例药物治疗分析%Analysis of drug treatment of the coronary embolism in Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    俞惠娟; 朱卫华

    2014-01-01

    目的:观察川崎病(KD)冠状动脉瘤并发血栓药物治疗的效果。方法回顾性分析8例KD冠状动脉瘤合并血栓患儿的临床资料。结果8例KD患儿年龄0.2~5.2岁;男6例,女2例。冠状动脉瘤瘤体内径8.3~13.8 mm,血栓发生时间在冠状动脉瘤形成后19 d至5月余。初发症状为突发胸痛1例,有心肌梗死伴心力衰竭症状;休克样症状1例;无症状6例。心脏超声检查发现瘤内血栓最大径2.8 mm×15.4 mm,呈长段条索状。冠状动脉血栓右侧4例,左侧2例,双侧2例。使用肝素、尿激酶溶栓,华法林、阿司匹林及潘生丁抗凝,抗血小板治疗。7例消溶成功,完全消溶所需要时间7d至4月余。1例4个月后血栓复发;1例发病12 h死亡。结论 KD冠状动脉瘤合并血栓好发于KD发病半年内,药物溶栓治疗需要时间较长,血栓脱落不多见。%Objective To observe the efficacy of drug treatment of coronary aneurysm complicated with embolism in Kawasaki disease (KD). Methods The clinical data of eight KD children with coronary aneurysm and embolism were retrospectively analyzed. Results Eight KD children (six males and two females) at age of 0.25-5.2 years (mean=2.89) ,were diagnosed with gigantic coronary artery aneurysms. The diameter of aneurysm was around 8.3-13.8mm. Thrombosis appeared from 19 days to five months after coronary aneurysms formation. The onset manifestations included sudden chest pain and myocardial infarction with symptoms of heart failure in one case, shock in one case and no symptom in six cases. The maximum diameter of the thrombus was 2.8 mm×15.4 mm in the shape of funicular. Four cases had thrombus in the right coronary artery, two cases in the left coronary artery, and two cases in both sides. The patients underwent anticoagulant therapy taking heparin, urokinase, warfarin, aspirin and dipyridamole. Anticoagulant therapy was successful in 7 cases and the thrombus was completely

  15. Acute rheumatic fever and rheumatic heart disease.

    Science.gov (United States)

    Carapetis, Jonathan R; Beaton, Andrea; Cunningham, Madeleine W; Guilherme, Luiza; Karthikeyan, Ganesan; Mayosi, Bongani M; Sable, Craig; Steer, Andrew; Wilson, Nigel; Wyber, Rosemary; Zühlke, Liesl

    2016-01-14

    Acute rheumatic fever (ARF) is the result of an autoimmune response to pharyngitis caused by infection with group A Streptococcus. The long-term damage to cardiac valves caused by ARF, which can result from a single severe episode or from multiple recurrent episodes of the illness, is known as rheumatic heart disease (RHD) and is a notable cause of morbidity and mortality in resource-poor settings around the world. Although our understanding of disease pathogenesis has advanced in recent years, this has not led to dramatic improvements in diagnostic approaches, which are still reliant on clinical features using the Jones Criteria, or treatment practices. Indeed, penicillin has been the mainstay of treatment for decades and there is no other treatment that has been proven to alter the likelihood or the severity of RHD after an episode of ARF. Recent advances - including the use of echocardiographic diagnosis in those with ARF and in screening for early detection of RHD, progress in developing group A streptococcal vaccines and an increased focus on the lived experience of those with RHD and the need to improve quality of life - give cause for optimism that progress will be made in coming years against this neglected disease that affects populations around the world, but is a particular issue for those living in poverty.

  16. Acute Respiratory Distress: from syndrome to disease.

    Science.gov (United States)

    Cardinal-Fernández, P; Correger, E; Villanueva, J; Rios, F

    2016-04-01

    The acute respiratory distress syndrome (ARDS) is currently one of the most important critical entities given its high incidence, rate of mortality, long-term sequelae and non-specific pharmacological treatment. The histological hallmark of ARDS is diffuse alveolar damage (DAD). Approximately 50% of ARDS patients present DAD, the rest is made up of a heterogeneous group of histological patterns, many of which correspond to a well-recognized disease. For that reason, if these patterns could be diagnosed, patients could benefit from a treatment. Recently, the effect of DAD in clinical and analytical evolution of ARDS has been demonstrated, so the classical approach to ARDS as an entity defined solely by clinical, radiological and gasometrical variables should be reconsidered. This narrative review aims to examine the need to evolve from the concept of ARDS as a syndrome to ARDS as a specific disease. So we have raised 4 critical questions: a) What is a disease?; b) what is DAD?; c) how is DAD considered according to ARDS definition?, and d) what is the relationship between ARDS and DAD? Copyright © 2015 Elsevier España, S.L.U. and SEMICYUC. All rights reserved.

  17. 综合性护理干预对川崎病患儿临床预后的影响分析%Analysis on the Effect of Comprehensive Nursing Intervention on the Prognosis of Children with Kawasaki Disease

    Institute of Scientific and Technical Information of China (English)

    徐霞

    2014-01-01

    Objective To explore the effect of comprehensive nursing intervention on the curative effect of children with Kawasaki disease (KD). Methods 145 cases of children with KD were selected and randomly divided into two groups. 72 cases of control group were given routine nursing, while 73 cases of intervention group were given comprehensive nursing intervention. The incidences of complications, nursing satisfaction and disease cognitive score of children's parents, and psychological score of children were analyzed and compared between two groups. Results The incidences of complications of intervention group were significantly lower than those of control group (P<0.05). The psychological score of children and disease cognitive score of children's parents of intervention group were significantly better than those of control group (P <0.05). Conclusions Comprehensive nursing intervention can promote the recovery of children with KD, reduce the incidence of complications, relax children's emotion, and enhance the trust between patient and physician.%目的:探究综合性护理方式对川崎病患儿临床治疗效果的影响。方法选取川崎病患儿145例并随机分为两组,73例干预组患儿给予综合性护理措施,72例对照组患儿给予常规护理。分析比较两组患儿的并发症发生情况,患儿家长对护理服务的满意度及疾病的认知评分,以及患儿的心理认知评分。结果干预组患儿的并发症发生率显著低于对照组(孕<0.05);干预组的患儿心理评分和疾病知识认知均优于对照组(孕<0.05)。结论有效的综合性护理能促进川崎病患儿临床的康复,减少并发症的发生,缓解患儿情绪,提高医患间的信任。

  18. The clinical features and follow-up study of 100 cases of Kawasaki disease%儿童川崎病100例临床特征及随访分析

    Institute of Scientific and Technical Information of China (English)

    刘玉玲; 付四毛; 李小琳; 林汉炼; 陈明; 李性希

    2011-01-01

    Objective To explore the clinical features, etiological factors, treatments and prognosis of 100 cases of Kawasaki disease(KD). Methods Totally 100 patients with KD in our department treated from 2000 to 2005 was included in the study. A retrospective study was carried ont to analyze clinical characteristics, treatment and outcomes of these patients. Results Clinical features: age of onset (2.01 ± 1.35) years, the age of follow-up (8.2 t 1.68) years;male to female ratio was 1.7∶ 1. Tipical KD (88), including recurrence KD (2), incomplete KD (12). Cardiova-scular complications existed in 25 patients,including coronary artery complications(22), pericardial effusion(2), myocarditis( 1 ) ,transient coronary artery ectasia (18) and coronary aneurysm formation (4), (small tumors in 1 case, medium-sized tumor in 2 cases and a huge tumor in 1 case). Non-cardiovascular complications cases included hepatitis or impaired liver function (34), gallbladder hydrops (3), paralytic intestinal obstruction ( 1 ), aseptic meningitis (3) , facial palsy (5), pneumonia (53), urethritis (12) and arthritis (6). Treatment and efficacy: 89 patients within 10 days of the course were given IVIG 2 g/kg; 12 patients without response were given a 2nd IVIG; 2 IVIG non-responsive patients were added the use of glucocorticoid; 11 cases diagnosed as subacute were given IVIG 1 ~ 2 g/(kg-d), 3 cases with coronary aneurysm formation (P < 0.05 ).Those without coronary artery lesions withdrawed the treatment at the course of 6 to 8 weeks; 18 patients with coronary artery dilation were tracked from 6 months to 1 year to discontinuation; 3 cases of small and medium-sized coronary aneurysm withdrawed in the course of 1 to 3 years; 1 case of giant coronary aneurysm was given aspirin for 7 years. Follow-up and prognosis: 56 patients were followed up to ≥ 5 years, up to 10 years. Abnormal follow-up echocardiography occurred in 10 cases (17.8% ).Among them, 1 case of giant coronary artery aneurysms

  19. Addison's Disease Mimicking as Acute Pancreatitis: A Case Report.

    Science.gov (United States)

    Chaudhuri, Sayani; Rao, Karthik N; Patil, Navin; Ommurugan, Balaji; Varghese, George

    2017-04-01

    Over past two decades there has been significant improvement in medical field in elucidating the underlying pathophysiology and genetics of Addison's disease. Adrenal insufficiency (Addison's disease) is a rare disease with an incidence of 0.8/100,000 cases. The diagnosis may be delayed if the clinical presentation mimics a gastrointestinal disorder or psychiatric illness. We report a case of Addison's disease presenting as acute pain in abdomen mimicking clinical presentation of acute pancreatitis.

  20. Apply Astragalus Injecta to Children with Kawasaki Disease(KD) after Fever%川崎病患儿热退后应用黄芪注射液的疗效分析

    Institute of Scientific and Technical Information of China (English)

    王红丽

    2013-01-01

    [目的]评价黄芪注射液辅助治疗热退后川崎病(Kawasaki disease,KD)患儿的临床疗效.[方法]将82例经治疗热退后的KD患儿随机分成两组.对照组40例,应用阿司匹林(Asprin,ASA)3mg/(kg·d),治疗组42例,在对照组基础上加用黄芪注射液,14d为1疗程,共1疗程.[结果]治疗组治疗后C反应蛋白(CRP)、血小板(PLT)、血沉(ESR)较对照组下降明显(P<0.05),冠状动脉病变(Coronary artery lesions,CAL)发生率明显降低(P<0.05);两组临床总疗效比较,治疗组优于对照组(P<0.05).[结论]黄芪注射液配合治疗热退后的KD患儿,可缩短病程,提高临床疗效,降低CAL的发生率并且有较好的安全性.%[Objective] To observe the cure effect of Astragalus Injecta treating children of KD after fever in assistance. [Method] Randomly divide 82 cases into 2 groups; control group 40 cases take Asprin, the treatment group 42, added with Astragalus Injecta on the basis of control group; 14d as a course. [Result] In treatment group, CRP, PLT and ESR reduced much more than control one, CAI decreased a lot; comparison of clinical cure effect of both groups showed the treatment group was better than control one. [Conclusion] The Astragalus Injecta in combination treating children of KD after fever can ihorten disease course, improve clinical effect and reduce CAI occurrence rate with good safety.