WorldWideScience

Sample records for atypical kawasaki disease

  1. 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

  2. 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.

  3. Unusual imaging presentation of infantile atypical Kawasaki disease

    Directory of Open Access Journals (Sweden)

    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.

  4. 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.

  5. Kawasaki Disease

    Science.gov (United States)

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

  6. 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.

  7. [Kawasaki's disease].

    Science.gov (United States)

    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?

    Science.gov (United States)

    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 80 percent of people with ...

  9. 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.

  10. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  12. Genetics Home Reference: Kawasaki disease

    Science.gov (United States)

    ... Me Understand Genetics Home Health Conditions Kawasaki disease Kawasaki disease Enable Javascript to view the expand/collapse boxes. Download PDF Open All Close All Description Kawasaki disease is a sudden and time-limited (acute) illness ...

  13. How Is Kawasaki Disease Diagnosed?

    Science.gov (United States)

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

  14. History of Kawasaki disease.

    Science.gov (United States)

    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.

  15. 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。

  16. 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

  17. Diagnosis and management of kawasaki disease.

    Science.gov (United States)

    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.

  18. Vaccines and Kawasaki disease.

    Science.gov (United States)

    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.

  19. Kawasaki Disease: Complications, Treatment and Prevention

    Science.gov (United States)

    ... Peripheral Artery Disease Venous Thromboembolism Aortic Aneurysm More Kawasaki Disease: Complications, Treatment and Prevention Updated:Apr 27,2016 ... possibility of heart and coronary artery involvement makes Kawasaki disease unpredictable, but these problems usually are not serious ...

  20. Diagnosis of incomplete Kawasaki disease

    Directory of Open Access Journals (Sweden)

    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.

  1. [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.

  2. 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

  3. 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

  4. 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.

  5. Serum adipokine profiles in Kawasaki disease.

    Science.gov (United States)

    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.

  6. Kawasaki Disease (For Parents)

    Science.gov (United States)

    ... Development Infections Diseases & Conditions Pregnancy & Baby Nutrition & Fitness Emotions & Behavior School & Family Life First Aid & Safety Doctors & ... soles of the feet with a purple-red color swollen lymph nodes During the second phase, which ...

  7. 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

  8. 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.

  9. 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.

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

    Science.gov (United States)

    ... 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 fever ...

  11. Kawasaki Disease: A Clinician’s Update

    Directory of Open Access Journals (Sweden)

    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.

  12. Sensorineural hearing loss in Kawasaki disease

    Directory of Open Access Journals (Sweden)

    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.

  13. 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.

  14. 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.

  15. Influenza infection and Kawasaki disease

    Directory of Open Access Journals (Sweden)

    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.

  16. 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

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

    Science.gov (United States)

    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.

  18. Analysis of Children's Typical and Atypical Kawasaki Disease with Coronary Artery Damage in 72 Cases%儿童典型及不典型川崎病72例临床及冠脉损害分析

    Institute of Scientific and Technical Information of China (English)

    邹武军

    2013-01-01

    Objective:This paper aims to study the clinical features of kawasaki disease (KD), and the dangerous factors and probability of coronary artery lesion.Methods According to the clinical manifestation, 72 KD cases are divided into KD Group and atypical KD Group firstly. Secondly, the following data is gathered including auxiliary laboratory examination result, major clinical manifestation, age, gender and cardiac ultrasonography result of each patient. Thirdly, the information is collected and used for comparative analysis. Results:Clinical manifestations of KD Group and atypical KD Group show no obvious difference. In addition, gender compositions of patients in the two groups are almost equal, while the probability of CAL complication in atypical KD Group is obviously higher. Proportions of patients below 1-year-old are quite different in the 2 groups.Conclusion:Atypical KD is apt to occur on patient below 1-year-old, and atypical KD will cause higher rate of coronary artery lesion.%目的:探讨儿童川崎病( KD)的临床特征、冠脉损害的危险因素以及发生概率。方法:将KD患儿72例依照临床表现,分为KD组、不典型KD组。收集每位患儿的实验室检查辅助检查结果、主要临床表现、年龄、性别以及心脏超声。进行汇总并比较分析。结果:KD组与不典型KD组的患儿在临床表现上并无明显差异。2组的患儿性别组成无明显差异。不典型KD组的患儿并发CAL的几率明显高于KD组的患儿。2组<1岁的患儿在年龄分布上有明显的差异。结论:不典型KD好发于<1岁的患儿,不典型KD冠脉损害率高。

  19. Extremely refractory Kawasaki disease with disseminated intravascular coagulation.

    Science.gov (United States)

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

    2017-03-07

    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.

  20. [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.

  1. 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.

  2. Cardiac magnetic resonance feature tracking in Kawasaki disease convalescence

    Directory of Open Access Journals (Sweden)

    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.

  3. Coronary artery bypass grafting for Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    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.

  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. 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].

  6. 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.

  7. 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.

  8. 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.

  9. [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.

  10. 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.

  11. 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.)

  12. 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.

  13. 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.

  14. 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.

  15. 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.

  16. 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...

  17. Resistant Kawasaki disease, case report and literatures review

    Directory of Open Access Journals (Sweden)

    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.

  18. 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.

  19. Natriuretic Peptides in Kawasaki Disease: the Myocardial Perspective

    Directory of Open Access Journals (Sweden)

    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.

  20. Clinical aspects of 100 patients with Kawasaki disease.

    Science.gov (United States)

    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.

  1. 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.

  2. 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).

  3. 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.

  4. 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.

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

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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. 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.

  8. 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.

  9. [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.

  10. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  12. 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].

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

    Directory of Open Access Journals (Sweden)

    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.

  14. 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.

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

    Science.gov (United States)

    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.

  16. 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.

  17. 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.

  18. 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.

  19. 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.

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

    OpenAIRE

    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...

  1. 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...

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

  4. [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.

  5. 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.

  6. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  8. Atypical presentations of celiac disease

    Directory of Open Access Journals (Sweden)

    Balasa Adriana Luminita

    2016-08-01

    Full Text Available In this study we evaluated the association of celiac disease in 81 children with autoimmune disease and genetic syndromes over a two years periods (January 2014 to July 2016 in Pediatric Clinic in Constanta. Because the extraintestinal symptoms are an atypical presentation of celiac disease we determined in these children the presence of celiac disease antibodies: Anti-tissue Transglutaminase Antibody IgA and IgA total serum level as a screening method followeds in selective cases by Anti-tissue Transglutaminase Antibody IgG, anti-endomysial antibodies, deamidated gliadin antibodies IgA and IgG and intestinal biopsia. In our study 8 patients had been diagnosed with celiac disease with extraintestinal symptoms, of which 4 with type 1 diabetes, 1 patient with ataxia, 2 patients with dermatitis herpetiformis and 1 patient with Down syndrome that associate also autoimmune thyroiditis, alopecia areata, enamel hypoplasia.

  9. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  11. Clinical and laboratory characteristics of children with Kawasaki disease

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

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

    Science.gov (United States)

    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 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.

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

    Science.gov (United States)

    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.

  15. Variations in ORAI1 Gene Associated with Kawasaki Disease.

    Science.gov (United States)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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

  20. 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. Questionna

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

    Directory of Open Access Journals (Sweden)

    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.

  2. 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.

  3. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  5. 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.

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

    Science.gov (United States)

    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.

  10. Atypical disease phenotypes in pediatric ulcerative colitis

    DEFF Research Database (Denmark)

    Levine, Arie; de Bie, Charlotte I; Turner, Dan

    2013-01-01

    Definitive diagnosis of pediatric ulcerative colitis (UC) may be particularly challenging since isolated colitis with overlapping features is common in pediatric Crohn's disease (CD), while atypical phenotypes of UC are not uncommon. The Paris classification allows more accurate phenotyping...

  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.

  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. 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.

  14. 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.

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

    Directory of Open Access Journals (Sweden)

    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. 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

  17. 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

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

    Science.gov (United States)

    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.

  19. 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.

  20. 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.

  1. 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.

  2. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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)

  4. 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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Science.gov (United States)

    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.

  7. Wilson’s disease: Atypical imaging features

    Directory of Open Access Journals (Sweden)

    Venugopalan Y Vishnu

    2016-10-01

    Full Text Available Wilson’s disease is a genetic movement disorder with characteristic clinical and imaging features. We report a 17- year-old boy who presented with sialorrhea, hypophonic speech, paraparesis with repeated falls and recurrent seizures along with cognitive decline. He had bilateral Kayser Flescher rings. Other than the typical features of Wilson’s disease in cranial MRI, there were extensive white matter signal abnormalities (T2 and FLAIR hyperintensities and gyriform contrast enhancement which are rare imaging features in Wilson's disease. A high index of suspicion is required to diagnose Wilson’s disease when atypical imaging features are present.

  8. 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.

  9. 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.

  10. 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

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

    Science.gov (United States)

    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).

  12. 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.

  13. 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].

  14. 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.

  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. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  19. 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.

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

    Science.gov (United States)

    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.

  1. 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.

  2. 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

  3. 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.)

  4. 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.

  5. Atypical Celiac Disease: From Recognizing to Managing

    Directory of Open Access Journals (Sweden)

    B. Admou

    2012-01-01

    Full Text Available The nonclassic clinical presentation of celiac disease (CD becomes increasingly common in physician’s daily practice, which requires an awareness of its many clinical faces with atypical, silent, and latent forms. Besides the common genetic background (HLA DQ2/DQ8 of the disease, other non-HLA genes are now notably reported with a probable association to atypical forms. The availability of high-sensitive and specific serologic tests such as antitissue transglutuminase, antiendomysium, and more recent antideamidated, gliadin peptide antibodies permits to efficiently uncover a large portion of the submerged CD iceberg, including individuals having conditions associated with a high risk of developing CD (type 1 diabetes, autoimmune diseases, Down syndrome, family history of CD, etc., biologic abnormalities (iron deficiency anemia, abnormal transaminase levels, etc., and extraintestinal symptoms (short stature, neuropsychiatric disorders, alopecia, dental enamel hypoplasia, recurrent aphtous stomatitis, etc.. Despite the therapeutic alternatives currently in developing, the strict adherence to a GFD remains the only effective and safe therapy for CD.

  6. 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.

  7. 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.

  8. 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.

  9. 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.

  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.

  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. 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

  13. 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.

  14. 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.

  15. 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.

  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. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  1. 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。

  2. 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。

  3. 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.

  4. 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.

  5. 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.

  6. 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.

  7. 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.

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

    Science.gov (United States)

    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

  9. 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.)

  10. 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

  11. 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.

  12. 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.

  13. 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.

  14. 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.

  15. 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.

  16. 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.)

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

    Energy Technology Data Exchange (ETDEWEB)

    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)

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

    Directory of Open Access Journals (Sweden)

    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.

  19. 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 ...

  20. 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.

  1. 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.

  2. 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.

  3. 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

  4. 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.

  5. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    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.

  8. 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.

  9. [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.

  10. 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.

  11. 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.

  12. 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

  13. 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

  14. 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.

  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. 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.

  17. Clinical outcome of patients with refractory Kawasaki disease based on treatment modalities

    Science.gov (United States)

    Kim, Hyun Jung; Lee, Hyo Eun; Yu, Jae Won

    2016-01-01

    Purpose Although a significant number of reports on new therapeutic options for refractory Kawasaki disease (KD) such as steroid, infliximab, or repeated intravenous immunoglobulin (IVIG) are available, their effectiveness in reducing the prevalence of coronary artery lesions (CAL) remains controversial. This study aimed to define the clinical characteristics of patients with refractory KD and to assess the effects of adjuvant therapy on patient outcomes. Methods We performed a retrospective study of 38 refractory KD patients from January 2012 to March 2015. We divided these patients into 2 groups: group 1 received more than 3 IVIG administration+ steroid therapy, (n=7, 18.4%), and group 2 patients were unresponsive to initial IVIG and required steroid therapy or second IVIG (n=31, 81.6%). We compared the clinical manifestations, laboratory results, and echocardiographic findings between the groups and examined the clinical utility of additional therapies in both groups. Results A significant difference was found in the total duration of fever between the groups (13.0±4.04 days in group 1 vs. 8.87±2.30 days in group 2; P=0.035). At the end of the follow-up, all cases in group 1 showed suppressed CAL. In group 2, coronary artery aneurysm occurred in 2 patients (6.4 %). All the patients treated with intravenous corticosteroids without additional IVIG developed CALs including coronary artery aneurysms. Conclusion No statistical difference was found in the development of CAL between the groups. Prospective, randomized, clinical studies are needed to elucidate the effects of adjunctive therapy in refractory KD patients. PMID:27610181

  18. 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

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

    Directory of Open Access Journals (Sweden)

    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.

  20. 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

  1. 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.

  2. 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

  3. Atypical Cogan's syndrome associated with coronary disease

    Institute of Scientific and Technical Information of China (English)

    Ivanovic Branislava; Tadic Marijana; Damjanov Nemanja; Simic Dragan; Zlatanovic Maja

    2011-01-01

    Cogan's syndrome (CS) is a rare inflammatory disorder characterized by interstitial keratitis and vestibuloauditory abnormalities often associated with various systemic manifestations. Involvement of cardiovascular system resembling systemic vasculitis may lead to severe complications and death. The present report describes a case of a female patient with atypical Cogan's syndrome presented with systemic manifestations and severe coronary and femoral artery stenosis.Despite the clinical improvement after glucocorticoids and cyclophosphamide, the patient required double aortocoronal bypass grafting one year letter. During three years follow-up, she was in stable condition, without stenocardial symptoms and claudication and her inflammatory parameters remain normal. This case highlights the rare involvement of coronary arteries without associated large-vessel vasculitis of the aortic arch in CS.

  4. Atypical form of cat scratch disease in immunocompetent patient

    Directory of Open Access Journals (Sweden)

    Kojić Miroslav

    2013-01-01

    Full Text Available Introduction. Cat scratch disease (CSD is an acute infectious disease with benign course caused by the bacteria Bartonella henselae. Clinically, it is usually manifested as regional lymphadenopathy and mild infective syndrome. Rare forms of the disease which usually occur in immunocompromised presons are: encephalitis, transverse myelitis, neuroretinitis, granulomatosus conjunctivitis, arthritis, hepatitis etc. Case report. We presented an atypical form of cat scratch disease in a young immunocompetent female person. The disease was manifested with prolonged fever, rash, purulent lymphadenitis and hepatitis. The diagnosis was based on characteristic patohystological finding and exclusion of the other causes of lymphadenopathy. The patient was treated by antibiotics for a few weeks, with surgical incision and drainage of the purulent lymphadenitis. Conclusion. Atypical forms of CSD could be an important differential-diagnostic problem, especially if there is no opportunity for serological confirmation of the disease.

  5. 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.

  6. 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.

  7. Atypical Depression

    Science.gov (United States)

    Diseases and Conditions Atypical depression By Mayo Clinic Staff Any type of depression can make you feel sad and keep you from enjoying life. However, atypical depression — also called depression with atypical features — means that ...

  8. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  10. 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...... of the disease is abrupt onset of high fever, followed by conjunctivitis, lymphadenopathy, rash, oral mucosal erythema and erythema of palms and soles. The diagnosis of KS is based on the presence of fever of at least four-five days' duration, plus four of the remaining criteria. Udgivelsesdato: 2009-Feb-2...

  11. Adult-onset Still's disease with atypical cutaneous manifestations

    Science.gov (United States)

    Narváez Garcia, Francisco Javier; Pascual, María; López de Recalde, Mercè; Juarez, Pablo; Morales-Ivorra, Isabel; Notario, Jaime; Jucglà, Anna; Nolla, Joan M.

    2017-01-01

    Abstract The diagnosis of adult-onset Still's disease (AOSD) can be very difficult. There are no specific tests available, and diagnosis is usually based on a symptom complex and the well-described typical evanescent rash seen in the majority of patients. However, in recent years, other atypical cutaneous manifestations of AOSD have been reported. These atypical skin eruptions often present in addition to the typical evanescent rash but may also be the only skin manifestation, resulting in delayed diagnosis because of under-recognition. In this study, we present 3 new cases of AOSD with atypical cutaneous manifestations diagnosed during a 30-year period in our department and review 78 additional cases previously reported (PubMed 1990–2016). These 81 patients form the basis of the present analysis. The overall prevalence of atypical cutaneous manifestations in our AOSD population was 14%. These manifestations may appear at any time over the course of the disease, and usually occur in patients who have persistent and severe disease, with a considerable frequency of clinical complications (23%), including serositis, myopericarditis, lung involvement, abdominal pain, neurologic involvement, and reactive hemophagocytic syndrome. The most representative and frequent lesion among the nonclassical skin rashes is the development of persistent pruritic papules and/or plaques. Interestingly, these lesions show a distinctive histological pattern. Other, less frequently observed lesions include urticaria and urticaria-like eruptions, generalized or widespread non-pruritic persistent erythema, vesiculopustular eruptions, a widespread peau d’orange appearance of the skin, and edema of the eyelids mimicking dermatomyositis without any accompanying skin lesion. The great majority of these patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy

  12. 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

  13. 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.

  14. 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.)

  15. 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.)

  16. Posterior cortical atrophy: an atypical variant of Alzheimer disease.

    Science.gov (United States)

    Suárez-González, Aida; Henley, Susie M; Walton, Jill; Crutch, Sebastian J

    2015-06-01

    Posterior cortical atrophy (PCA) is a neurodegenerative syndrome characterized by striking progressive visual impairment and a pattern of atrophy mainly involving posterior cortices. PCA is the most frequent atypical presentation of Alzheimer disease. The purpose of this article is to provide a summary of PCA's neuropsychiatric manifestations. Emotional and psychotic symptoms are discussed in the context of signal characteristic features of the PCA syndrome (the early onset, focal loss of visual perception, focal posterior brain atrophy) and the underlying cause of the disease. The authors' experience with psychotherapeutic intervention and PCA support groups is shared in detail.

  17. Atypical Clinical Manifestations of Graves' Disease: An Analysis in Depth

    Directory of Open Access Journals (Sweden)

    Mohamed Osama Hegazi

    2012-01-01

    Full Text Available Over the past few decades, there has been an increase in the number of reports about newly recognized (atypical or unusual manifestations of Graves' disease (GD, that are related to various body systems. One of these manifestations is sometimes the main presenting feature of GD. Some of the atypical manifestations are specifically related to GD, while others are also similarly seen in patients with other forms of hyperthyroidism. Lack of knowledge of the association between these findings and GD may lead to delay in diagnosis, misdiagnosis, or unnecessary investigations. The atypical clinical presentations of GD include anemia, vomiting, jaundice, and right heart failure. There is one type of anemia that is not explained by any of the known etiological factors and responds well to hyperthyroidism treatment. This type of anemia resembles anemia of chronic disease and may be termed GD anemia. Other forms of anemia that are associated with GD include pernicious anemia, iron deficiency anemia of celiac disease, and autoimmune hemolytic anemia. Vomiting has been reported as a presenting feature of Graves' disease. Some cases had the typical findings of hyperthyroidism initially masked, and the vomiting did not improve until hyperthyroidism has been detected and treated. Hyperthyroidism may present with jaundice, and on the other hand, deep jaundice may develop with the onset of overt hyperthyroidism in previously compensated chronic liver disease patients. Pulmonary hypertension is reported to be associated with GD and to respond to its treatment. GD-related pulmonary hypertension may be so severe to produce isolated right-sided heart failure that is occasionally found as the presenting manifestation of GD.

  18. 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。

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

    Energy Technology Data Exchange (ETDEWEB)

    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.

  20. 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。

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

    Directory of Open Access Journals (Sweden)

    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.

  3. 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.

  4. 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

  5. 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.

  6. 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

  7. Single-nucleotide polymorphism rs7251246 in ITPKC is associated with susceptibility and coronary artery lesions in Kawasaki disease.

    Directory of Open Access Journals (Sweden)

    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.

  8. 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.

  9. 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.

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

    Energy Technology Data Exchange (ETDEWEB)

    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.)

  11. 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

  12. 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

  13. Atypical localizations of hydatid disease: Experience from a single institute

    Directory of Open Access Journals (Sweden)

    Majid Mushtaque

    2012-01-01

    Full Text Available Introduction: The hydatid disease most often involves the liver and the lungs. The disease can involve any part of the body except the hair, teeth and nails. Primary extrahepatico-pulmonary hydatid cysts are rare and only a few sporadic cases have been reported. Materials and Methods: Two hundred and forty-four patients with hydatid cysts managed surgically from January 2005 to December 2009 were evaluated retrospectively. Fourteen (5.7% patients had isolated involvement of the atypical sites, while six (2.4% also had a primary involvement of liver. Results: The cysts were present in gall bladder (0.4%, peritoneum (1.6%, spleen (1.6%, ovary (0.4%, subcutaneous (0.8%, seminal vesicle (0.4%, spinal (0.4%, pancreas (0.4%, kidney (0.4%, mediastinal (0.4%, muscle (0.4%, and brain (0.8%. Discussion and Conclusions: Involvement of sites other than liver and lungs by hydatid disease is rare. Symptoms are related to size, location or possible complication of the cyst. It should be strongly suspected in differential diagnosis of all abdominal cysts especially in an endemic area. Proper surgical and medical management to avoid any recurrences, and a regular follow-up, are of utmost importance to detect any late complications such as local recurrence of the disease and development of hydatidosis at the primary sites.

  14. 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

    Energy Technology Data Exchange (ETDEWEB)

    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).

  15. 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

  16. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  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. 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.

  20. 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.

  1. Biosignatures for Parkinson's disease and atypical parkinsonian disorders patients.

    Directory of Open Access Journals (Sweden)

    Judith A Potashkin

    Full Text Available Diagnosis of Parkinson' disease (PD carries a high misdiagnosis rate due to failure to recognize atypical parkinsonian disorders (APD. Usually by the time of diagnosis greater than 60% of the neurons in the substantia nigra are dead. Therefore, early detection would be beneficial so that therapeutic intervention may be initiated early in the disease process. We used splice variant-specific microarrays to identify mRNAs whose expression is altered in peripheral blood of early-stage PD patients compared to healthy and neurodegenerative disease controls. Quantitative polymerase chain reaction assays were used to validate splice variant transcripts in independent sample sets. Here we report a PD signature used to classify blinded samples with 90% sensitivity and 94% specificity and an APD signature that resulted in a diagnosis with 95% sensitivity and 94% specificity. This study provides the first discriminant functions with coherent diagnostic signatures for PD and APD. Analysis of the PD biomarkers identified a regulatory network with nodes centered on the transcription factors HNF4A and TNF, which have been implicated in insulin regulation.

  2. [Atypical celiac disease in an adolescent girl--case report].

    Science.gov (United States)

    Hozyasz, Kamil; Czerwińska, Barbara

    2004-11-01

    Coeliac disease is characterized by life-long gluten intolerance. There are a wide variety of clinical presentations, which range from severe diarrhoea and weight loss to asymptomatic forms. The primary treatment for coeliac disease is the removal of gluten from the diet to prevent both immediate and long-term complications. The case of 16-year-old girl with coeliac disease was presented. At the age of 2 years the patient with impaired growth and abnormal stools was suspected to have coeliac disease. She experienced symptomatic improvement on gluten-free diet, but after 3 years the treatment was discontinued. The patient denied gastrointestinal or skin problems. At the age of 14 years Raynaud's phenomenon was observed for the first time. Two years later episodes of Raynaud's phenomenon involved all fingers and toes. Body mass index (BMI) was 23.8 kg/m2. Levels of free-carnitine, tocopherol, vitamin B12 were below normal limits and homocysteine level was increased. Antiendomysial IgA, antireticulin IgA, antigliadin IgA and IgG antibodies were positive. The duodenal mucosa showed total villous atrophy. Gluten free-diet and multivitamin supplementation provided some benefit in reducing Raynaud's phenomenon. The patient's well being has improved markedly. Atypical coeliac disease is usually seen in adolescents and adults in whom features of overt malabsorption are often absent. In cases of health problems occurring in persons with history of malabsorption syndrome in childhood suspicion of coeliac disease should be heightened and appropriate evaluation undertaken.

  3. 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。

  4. 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

  5. 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.

  6. 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.

  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. Atypical Cutaneous Manifestations in Adult Onset Still’s Disease

    Directory of Open Access Journals (Sweden)

    Champa Nataraja

    2016-01-01

    Full Text Available Adult Onset Still’s Disease (AOSD, an adult variant of systemic onset juvenile idiopathic arthritis, is a rare systemic inflammatory disorder of unknown aetiology. The rarity of this disease is associated with low index of suspicion and delayed diagnosis in patients suffering from it and in the presence of atypical features the diagnosis can be further challenging. This is a case report on a 24-year-old woman, who was a diagnostic dilemma for 2 years due to the nonspecific symptoms of recurrent fever, generalized maculopapular persistent pruritic and tender rash, and polyarthralgia. She was initially diagnosed as leukocytoclastic vasculitis on a skin biopsy and was managed by a dermatologist with various medications including NSAIDs, hydroxychloroquine, dapsone, colchicine, cyclosporine, and high doses of oral steroids with minimal response. Subsequently, she has had multiple admissions with similar symptoms with raised inflammatory markers and negative septic workup. On one occasion, her iron study revealed hyperferritinaemia which led to the suspicion of AOSD. Once the rheumatic fever and infectious, malignant, autoimmune, and lymphoproliferative disorders were excluded, she was diagnosed as probable AOSD and managed successfully with IL-1 (interleukin-1 receptor antagonist, Anakinra, with remarkable and lasting response both clinically and biochemically.

  9. 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.

  10. 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。

  11. 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.

  12. Atypical motor neuron disease and related motor syndromes.

    Science.gov (United States)

    Verma, A; Bradley, W G

    2001-06-01

    There is an imperative need for the early diagnosis of amyotrophic lateral sclerosis/motor neuron disease (ALS/MND) in the current era of emerging treatments. When evaluating the patient with ALS/MND, the neurologist must consider a number of other motor neuron disorders and related motor syndromes that may have clinical features resembling ALS/MND. The revised Airlie House-El Escorial diagnostic criteria have been established through the consensus of experts meeting at workshops. However, by definition, using these criteria a patient is likely to have fairly advanced disease at the time of a definitive ALS/MND diagnosis. The reasons for the difficulty in making an early ALS/MND diagnosis are several. No surrogate diagnostic marker currently exists for ALS/MND. ALS/MND at its onset is heterogeneous in clinical presentation, its clinical course is variable, and several clinical variants are recognized. In addition, certain motor syndromes, such as monomelic amyotrophy, postpolio muscular atrophy, and multifocal motor neuropathy, can clinically mimic ALS/MND. Therefore, not only may the diagnosis of ALS/MND be clinically missed in the early stages, but worse, the patient may be wrongly labeled as having ALS/MND. The diagnosis of ALS/MND requires a combination of upper motor neuron (UMN) and lower motor neuron (LMN) involvement. Motor syndromes in which the deficit is restricted to the UMN or LMN through the entire course of the disease are described as atypical MND in this review. Approximately 5% of patients with ALS/MND have overt dementia with a characteristic frontal affect. ALS/MND with parkinsonism and dementia is rare outside the western Pacific region. The clinical course of motor disorder in these overlap syndromes does not differ from that in typical ALS/MND.

  13. 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。

  14. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  16. 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.

  17. 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

  18. 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.

  19. 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

  20. 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.

  1. 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.

  2. 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。

  3. 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

  4. 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.

  5. 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.

    Science.gov (United States)

    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

  6. 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.

  7. A rare disease in an atypical location - Kimura's Disease of the upper extremity

    Energy Technology Data Exchange (ETDEWEB)

    Lam, Alan Cheuk Si; Lau, Vince Wing Hang [Queen Mary Hospital, Department of Radiology, Hong Kong (China); Au Yeung, Rex Kwok Him [University of Hong Kong, Department of Pathology, Li Ka Shing Faculty of Medicine, Hong Kong (China)

    2015-12-15

    Kimura's disease is a rare chronic inflammatory disorder predominantly affecting young Asian male patients, occurring mainly in the head and neck regions. Kimura's disease of the upper extremity is extremely rare, and previous case reports in the literature show similar imaging characteristics with consistent location at the medial epitrochlear region, predominantly with unilateral involvement. We present the first reported case of Kimura's disease affecting the anterolateral aspect of the upper arm, sparing the medial epitrochlear region, illustrating that with typical MR appearance and serology, the involvement of this rare disease in an atypical location still warrants consideration of this diagnosis. There was also bilateral asymmetrical involvement in our patient, suggesting the possibility of a propensity for Kimura's disease affecting the upper extremities to have bilateral involvement, which may necessitate imaging of the clinically asymptomatic contralateral limb in these patients for early lesion identification and treatment. (orig.)

  8. 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

  9. 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.

  10. Diseases of the gastrointestinal tract in individuals diagnosed as children with atypical autism

    DEFF Research Database (Denmark)

    Mouridsen, Svend Erik Birkebæk; Isager, Torben; Rich, Bente

    2013-01-01

    with atypical autism, a total of 22 (24.7%) were registered with at least one diagnosis of any disease of the gastrointestinal tract, against 47 of 258 (18.2%) in the comparison group (p = 0.22; odds ratio = 1.5; 95% confidence interval = 0.8-2.6). Without reaching statistical significance, the rate of diseases...... of the gastrointestinal tract was particularly high (odds ratio = 1.2) in those with intelligence quotient people with atypical autism had about the same frequency of gastric, intestinal and hepatic diseases as had controls....

  11. 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

  12. 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.

  13. Atypical lymphocytes in dengue: role in diagnosis and prognosis of disease. A systematic review of literature

    Directory of Open Access Journals (Sweden)

    Luz Aida Rey-Caro

    2012-09-01

    Full Text Available Dengue is the arbovirus infection with the highest incidence in the world and a major cause of morbidity and mortality. Disease onset presents as a nonspecific febrile illness, and therefore, tools are required to identify and classify dengue patients at early stage. An useful parameter is the presence of atypical lymphocytes. Objective: to collect and analyze results from existing published studies on atypical lymphocytes and dengue infection, and its effect on diagnosis and prognosis of disease. Materials and methods: was conducted a PubMed and LILACS search under the terms: Dengue AND Atypical lymphocyte (OR Reactive lymphocyte, OR Turk cell, limiting the findings to human studies. The information was classified by its content. Only studies relevant to the topic were included in this study. Results: 68 references were identified using the search strategy, but only 12 met the inclusion criteria. All included studies reported the presence of atypical lymphocytes as particular hematologic finding in patients with dengue, and although it is not a classical specific finding of the disease, their concentrations were significantly higher in these patients, especially in severe forms of the disease. Conclusions: according to this evidence, there may be an association between the presence of atypical lymphocytes and dengue virus infection, but the intensity and usefulness of this finding requires further study and analysis.

  14. 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....

  15. 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.

  16. 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.

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

    Directory of Open Access Journals (Sweden)

    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

  18. 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.

  19. Atypical scrapie prions from sheep and lack of disease in transgenic mice overexpressing human prion protein.

    Science.gov (United States)

    Wadsworth, Jonathan D F; Joiner, Susan; Linehan, Jacqueline M; Balkema-Buschmann, Anne; Spiropoulos, John; Simmons, Marion M; Griffiths, Peter C; Groschup, Martin H; Hope, James; Brandner, Sebastian; Asante, Emmanuel A; Collinge, John

    2013-11-01

    Public and animal health controls to limit human exposure to animal prions are focused on bovine spongiform encephalopathy (BSE), but other prion strains in ruminants may also have zoonotic potential. One example is atypical/Nor98 scrapie, which evaded statutory diagnostic methods worldwide until the early 2000s. To investigate whether sheep infected with scrapie prions could be another source of infection, we inoculated transgenic mice that overexpressed human prion protein with brain tissue from sheep with natural field cases of classical and atypical scrapie, sheep with experimental BSE, and cattle with BSE. We found that these mice were susceptible to BSE prions, but disease did not develop after prolonged postinoculation periods when mice were inoculated with classical or atypical scrapie prions. These data are consistent with the conclusion that prion disease is less likely to develop in humans after exposure to naturally occurring prions of sheep than after exposure to epizootic BSE prions of ruminants.

  20. 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.

  1. 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

  2. 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

  3. Examination of Huntington's disease with atypical clinical features in a Bangladeshi family tree.

    Science.gov (United States)

    Al-Mamun, Md Mahfuz; Sarker, Suprovath Kumar; Qadri, Syeda Kashfi; Shirin, Tahmina; Mohammad, Quazi Deen; LaRocque, Regina; Karlsson, Elinor K; Saha, Narayan; Asaduzzaman, Muhammad; Qadri, Firdausi; Mannoor, Md Kaiissar

    2016-12-01

    Atypical manifestation of Huntington's disease (HD) could inform ongoing research into HD genetic modifiers not present in the primarily European populations studied to date. This work demonstrates that expanding HD genetic testing into under-resourced healthcare settings can benefit both local communities and ongoing research into HD etiology and new therapies.

  4. Atypical MR presentation of Wilson disease: a possible consequence of paramagnetic effect of copper

    Energy Technology Data Exchange (ETDEWEB)

    Brugieres, P.; Combes, C.; Ricolfi, F.; Gaston, A. (Dept. of Neuroradiology, Henri Mondor Hospital, Creteil (France)); Degos, J.D. (Dept. of Neurology, Henri Mondor Hospital, Creteil (France)); Poirier, J. (Dept. of Pathology, Henri Mondor Hospital, Creteil (France))

    1992-06-01

    A 53-year-old patient with Wilsons's disease and without autonomic dysfunction presented on T2-weighted MR study an atypical decreased signal intensity of the putamina and the caudate nuclei. Possible explanations of such a signal abnormalities are discussed. (orig.).

  5. Atypical early-onset Alzheimer's disease caused by the Iranian APP mutation

    DEFF Research Database (Denmark)

    Lindquist, Suzanne Granhøj; Nielsen, Jørgen Erik; Stokholm, Jette

    2008-01-01

    Approximately 1% of all cases of Alzheimer's disease are inherited autosomal dominantly, and to date, three causative genes have been found, the Presenilin 1 (PSEN1) gene, the Presenilin 2 (PSEN2) gene and the Amyloid precursor protein (APP) gene. We describe atypical phenotypic features in a fam...

  6. Diagnostic accuracy of Parkinson's disease and atypical parkinsonism in nursing homes

    NARCIS (Netherlands)

    Weerkamp, N. J.; Tissingh, G.; Poels, P. J. E.; Zuidema, S. U.; Munneke, M.; Koopmans, R. T. C. M.; Bloem, B. R.

    2014-01-01

    Introduction: Management of Parkinson's disease (PD) and atypical parkinsonism in nursing homes depends on a timely and accurate diagnosis. However, little is known about the diagnostic accuracy of these parkinsonian syndromes in nursing homes. We examined this issue in a large group of Dutch nursin

  7. 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.

  8. Atypical presentation of acute and chronic coronary artery disease in diabetics

    Institute of Scientific and Technical Information of China (English)

    Hadi; AR; Hadi; Khafaji; Jassim; M; Al; Suwaidi

    2014-01-01

    In patients with diabetes mellitus, cardiovascular disease is the principal cause of mortality and chest pain is the most frequent symptom in patients with stable and acute coronary artery disease. However, there is little knowledge concerning the pervasiveness of uncommon presentations in diabetics. The symptomatology of acute coronary syndrome, which comprises both pain and non-pain symptoms, may be affected by traditional risk factors such as age, gender, smoking, hypertension, diabetes, and dyslipidemia. Such atypical symptoms may range from silent myocardial ischemia to a wide spectrum of non-chest pain symptoms. Worldwide, few studies have highlighted this under-investigated subject, and this aspect of ischemic heart disease has also been under-evaluated in the major clinical trials. The results of these studies are highly diverse which makes definitive conclusions regarding the spectrum of atypical presentation of acute and even stable chronic coronay artery disease difficult to confirm. This may have a significant impact on the morbidity and mortality of coronary artery disease in diabetics. In this up-to-date review we will try to analyze the most recent studies on the atypical presentations in both acute and chronic ischemic heart disease which may give some emphasis to this under-investigated topic.

  9. 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.

  10. Fahr disease with atypical presentation: A report of two cases

    Directory of Open Access Journals (Sweden)

    Suzan Tunç

    2010-05-01

    Full Text Available Fahr’s disease is a rare disorder where bilateral, almost symmetric, calcium and other mineral deposits occur in basal ganglia, cerebellar dentate nucleus and white matter. Common clinical findings of the disease are characterizing parkinsonism, dystonia, chorea, ataxia and psychiatric symptoms. Fahr’s disease is associated with various metabolic disorders especially with parathyroid disorders. In this article a 65 year old female patient with vision loss and headache, bilateral basal ganglia and cerebellar calsification on Computerized Tomography examination and a 45 year old female patient with convulsive state and bilateral caudat nucleus calcification on Computerized Tomography examination were reported.

  11. Atypical early-onset Alzheimer's disease caused by the Iranian APP mutation

    DEFF Research Database (Denmark)

    Lindquist, S.G.; Nielsen, J.E.; Stokholm, J.

    2008-01-01

    BACKGROUND: Approximately 1% of all cases of Alzheimer's disease are inherited autosomal dominantly, and to date, three causative genes have been found, the Presenilin 1 (PSEN1) gene, the Presenilin 2 (PSEN2) gene and the Amyloid precursor protein (APP) gene. We describe atypical phenotypic...... features in a family with a pathogenic APP gene mutation and discuss possible explanations for these atypical features. METHODS AND RESULTS: We report a family with a history of dementia compatible with autosomal dominant transmission. The disease course in the proband was not typical for Alzheimer......'s disease as the diagnosis was preceded by 8 years of an isolated amnesia. Further, the proband had epilepsy with complex partial seizures and central degenerative autonomic failure as determined by clinical physiology. Sequencing the three known causative Alzheimer genes revealed a pathogenic missense...

  12. Review of Kawasaki Patients in Yazd

    Directory of Open Access Journals (Sweden)

    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 .

  13. Free-water imaging in Parkinson's disease and atypical parkinsonism.

    Science.gov (United States)

    Planetta, Peggy J; Ofori, Edward; Pasternak, Ofer; Burciu, Roxana G; Shukla, Priyank; DeSimone, Jesse C; Okun, Michael S; McFarland, Nikolaus R; Vaillancourt, David E

    2016-02-01

    Conventional single tensor diffusion analysis models have provided mixed findings in the substantia nigra of Parkinson's disease, but recent work using a bi-tensor analysis model has shown more promising results. Using a bi-tensor model, free-water values were found to be increased in the posterior substantia nigra of Parkinson's disease compared with controls at a single site and in a multi-site cohort. Further, free-water increased longitudinally over 1 year in the posterior substantia nigra of Parkinson's disease. Here, we test the hypothesis that other parkinsonian disorders such as multiple system atrophy and progressive supranuclear palsy have elevated free-water in the substantia nigra. Equally important, however, is whether the bi-tensor diffusion model is able to detect alterations in other brain regions beyond the substantia nigra in Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy and to accurately distinguish between these diseases. Free-water and free-water-corrected fractional anisotropy maps were compared across 72 individuals in the basal ganglia, midbrain, thalamus, dentate nucleus, cerebellar peduncles, cerebellar vermis and lobules V and VI, and corpus callosum. Compared with controls, free-water was increased in the anterior and posterior substantia nigra of Parkinson's disease, multiple system atrophy, and progressive supranuclear palsy. Despite no other changes in Parkinson's disease, we observed elevated free-water in all regions except the dentate nucleus, subthalamic nucleus, and corpus callosum of multiple system atrophy, and in all regions examined for progressive supranuclear palsy. Compared with controls, free-water-corrected fractional anisotropy values were increased for multiple system atrophy in the putamen and caudate, and increased for progressive supranuclear palsy in the putamen, caudate, thalamus, and vermis, and decreased in the superior cerebellar peduncle and corpus callosum. For all disease

  14. Case Report of Arm Kimura Disease with Atypical MR Finding

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hee Young; Kim, Baek Hyun; Lee, Young Hen; Kang, Chang Ho; Lee, Guen Young; Seo, Bo Kyoung; Lee, Ju Han [Korea University Ansan Hospital, Ansan (Korea, Republic of); Kim, Ye Lim; Hong, Suk Ju [Korea University Guro Hospital, Seoul (Korea, Republic of)

    2010-03-15

    Kimura disease is a rare chronic inflammatory condition of unknown origin that occurs mainly in young Asian male patients. Kimura disease is characterized by painless subcutaneous swelling and lymphadenopathy of the head and neck, associated with peripheral blood eosinophilia and increased serum IgE. According to some previous reports about a rare form of Kimura disease of the upper arm, soft tissue masses of the subcutaneous fat layer were demonstrated in the medial trochlear area, which showed strong and homogenous enhancement with marked perilesional edema and fat infiltrations on enhanced magnetic resonance imaging (MRI). In this case report, we introduce a case of Kimura disease of the upper arm in a 13-year male patient, which was seen as a well-marginated soft tissue mass without perilesional edema or fatty infiltrations on MRI. In this case study, we discuss the characteristics of the clinical and pathologic findings associated with unusual MRI findings, and report the Kimura disease with a different pathophysiology by anti-inflammatory drugs.

  15. Atypical refsum disease with pipecolic acidemia and abnormal catalase distribution.

    Science.gov (United States)

    Baumgartner, M R; Jansen, G A; Verhoeven, N M; Mooyer, P A; Jakobs, C; Roels, F; Espeel, M; Fourmaintraux, A; Bellet, H; Wanders, R J; Saudubray, J M

    2000-01-01

    We describe an 18-year-old patient with psychomotor retardation and abnormally short metatarsi and metacarpals but no other signs of classic Refsum disease. Molecular analysis of the phytanoyl-coenzyme A hydroxylase gene revealed a homozygous deletion causing a frameshift. Surprisingly, L-pipecolic acid was elevated in plasma, and microscopy of the liver showed a reduced number of peroxisomes per cell and a larger average peroxisome size. These abnormal peroxisomes lacked catalase as did peroxisomes in fibroblasts of this patient. Such generalized peroxisomal abnormalities are not present in classic Refsum disease.

  16. Atypical presentation of Creutzfeldt-Jakob disease: a rare but important cause of rapidly progressive dementia.

    Science.gov (United States)

    Taillefer, Marguerite S; Tangarorang, Glendo L; Kuchel, George A; Menkes, Daniel L

    2011-09-01

    We report an atypical presentation of sporadic Creutzfeldt-Jakob disease (CJD) in a 74-year-old woman that illustrates the difficulty in diagnosing this rare, but important, cause of rapidly progressive dementia. Despite well-established criteria, this diagnosis is often missed or substantially delayed (Table 1). In this case, a precipitous cognitive decline associated with a urinary tract infection initiallysuggested delirium. Although atypical CJD was considered as a cause when symptoms persisted, a definitive diagnosis was established postmortem when the cerebrospinal fluid (CSF) prion protein 14-3-3 tested positive. Creutzfeldt-Jakob disease must be considered in the differential diagnosis of rapidly progressive dementia as Connecticut accounts for approximately three of the more than 200 cases diagnosed nationally.

  17. [Cases of atypical pulmonary diseases under influence of industrial aerosols].

    Science.gov (United States)

    Liubchenko, P N; Stashuk, G A; Terpigorev, S A; Atamanchuk, A A; Massarygin, V V

    2014-01-01

    The article includes discharge epicrises of patients working in contact with quartz-containing dust, welding aerosol, cotton dust, irritant gases emitted in wood burning. Description covers cases of lung fibrosis, idiopathic interstitial pneumonia, systemic vasculitis with lungs involvement, sarcoidosis. The authors suggest that further accumulation of materials, further investigations of lung tissue state, epidemiologic studies will help to widen the list of occupational pulmonary diseases.

  18. Atypical presentation of infantile-onset farber disease with novel ASAH1 mutations.

    Science.gov (United States)

    Kim, Soo Yeon; Choi, Sun Ah; Lee, Sangmoon; Lee, Jin Sook; Hong, Che Ry; Lim, Byung Chan; Kang, Hyoung Jin; Kim, Ki Joong; Park, Sung-Hye; Choi, Murim; Chae, Jong-Hee

    2016-11-01

    Farber disease is a very rare autosomal recessive disease caused by mutation of ASAH1 that results in the accumulation of ceramide in various tissues. Clinical symptoms of classic Farber disease comprise painful joint deformity, hoarseness of voice, and subcutaneous nodules. Here, we describe a patient with Farber disease with atypical presentation of early onset hypotonia, sacral mass, congenital heart disease, and dysmorphic face since birth. Severe cognitive disability, failure to gain motor skills, failure to thrive, and joint contractures developed. Using whole-exome sequencing, we identified the compound heterozygote missense mutations of ASAH1 (p.R333C and p.G235R). Because of the diagnostic delay, she underwent sacral mass excision, which revealed enlarged lysosomes and zebra bodies. We report an atypical presentation of Farber disease with her pathology and associated genetic defect. This case expands the phenotypic spectrum of Farber disease to include novel mutations of ASAH1, which pose a diagnostic challenge. We also discuss the clinical utility of whole-exome sequencing for diagnosis of ultra-rare diseases. © 2016 Wiley Periodicals, Inc.

  19. 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

  20. 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.

  1. 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.

  2. 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

  3. Atypical velogenic Newcastle disease in a commercial layer flock in Japan.

    Science.gov (United States)

    Umali, Dennis V; Ito, Hiroshi; Shirota, Kazutoshi; Ito, Toshihiro; Katoh, Hiromitsu

    2015-05-01

    In 2002, a commercial layer flock in Japan was initially diagnosed as being infected with infectious bronchitis (IB) based on clinical signs, virus isolation, and serological analysis but was later found to be atypically infected with velogenic Newcastle disease virus (NDV) following molecular diagnosis. The flock had slightly decreased egg production and an increased occurrence of soft-shelled eggs without significant mortality. IB-like viruses were isolated, which caused dwarfing and curling in 12-day-old chicken embryos. Ten years after this case, retrospective genetic analyses showed that apart from IB virus (IBV), the flock was also infected with NDV. Mean death time (MDT), intracerebral pathogenicity index (ICPI), and deduced amino acid sequence of the cleavage site of the fusion (F)-protein gene revealed that the NDV isolate was velogenic ((112)RRQKR(116)). These results indicate that poultry clinicians should look out for atypical velogenic ND, especially in vaccinated commercial chicken flocks, which may harbor hidden NDV infection.

  4. 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。

  5. 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。

  6. 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.

  7. 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.

  8. 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.

  9. 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.

  10. Atypical presentation of Charcot-Marie-Tooth disease 1A: A case report.

    Science.gov (United States)

    Kulkarni, Shilpa D; Sayed, Rafat; Garg, Meenal; Patil, Varsha A

    2015-11-01

    Charcot-Marie-Tooth (CMT) 1A is the most common form of CMT disease and is characterized by duplication of Peripheral myelin protein 22 (PMP22) gene. We report a boy with genetically confirmed CMT1A disease having clinical involvement of hypoglossal and glossopharyngeal nerves, as well as asymmetrical and primarily upper limb involvement. These atypical features widen the clinical spectrum of CMT1A, leading to interesting observations about PMP22 gene related disorders and varied clinical expression of similar genetic mutations.

  11. Atypical Cat-Scratch Disease in Children: Report of Seven Presentations Ranging From Hepatosplenic Disease to Horner Syndrome

    Directory of Open Access Journals (Sweden)

    Gilliaux

    2016-01-01

    Full Text Available Introduction Cat scratch disease (CSD is an infectious disease caused by the Gram-negative rod Bartonella henselae (BH. It usually leads to subacute loco-regional lymphadenitis occasionally associated with fever. In most of the cases, it resolves spontaneously within 4 - 6 weeks. However, CSD has also been associated with other atypical presentations. Case Presentation We reported a series of seven children with unusual symptoms of CSD. In particular, we described the case of a child with ptosis, miosis and enophtalmy, suggesting Horner syndrome, associated with cervical lymphadenitis. Cat scratch was mentioned in only one patient, while four of them mentioned a recent contact with cats. We reviewed and discussed the incidence of these atypical presentations of CSD as well as the therapeutic approaches recommended and the available diagnostic tools. Conclusions This paper highlighted the need to exclude CSD in children with unexplained symptoms such as prolonged fever, hepatosplenic lesion and osteomyelitis.

  12. 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%.

  13. Vesicular erythema migrans: an atypical and easily misdiagnosed form of Lyme disease.

    Science.gov (United States)

    Mazori, Daniel R; Orme, Charisse M; Mir, Adnan; Meehan, Shane A; Neimann, Andrea L

    2015-08-15

    Erythema migrans is the initial sign in the majority of patients infected with Borrelia, the genus of spirochetes that causes Lyme disease. Early identification and treatment decrease the risk of progression to later stages of disease. Although a "bull's eye" appearance owing to lesional clearing is considered classic for erythema migrans, this feature is surprisingly often lacking among patients in the United States. Furthermore, cutaneous Lyme disease can exhibit a wide range of morphologic variability in a minority of patients. Herein, we describe the case of a patient with Lyme disease in which the presence of atypical vesicular features, in conjunction with the initial absence of clearing, resulted in multiple misdiagnoses and delayed treatment. We also review the literature on the epidemiology and management of erythema migrans for cases in which the diagnosis may pose a challenge.

  14. Epilepsy and other central nervous system diseases in atypical autism: a case control study

    DEFF Research Database (Denmark)

    Mouridsen, Svend Erik; Rich, Bente; Isager, Torben

    2011-01-01

    There is an increased but variable risk of epilepsy in autism spectrum disorders. The objective of this study is to compare the prevalence and types of epilepsy and other central nervous system (CNS) diseases in a clinical sample of 89 individuals diagnosed as children with atypical autism (AA......) with 258 matched controls from the general population. Diagnoses were based on data from the nationwide Danish National Hospital Register. The average observation time was 32.9 years, and mean age at follow-up was 48.5 years. Of the 89 individuals with AA, 20 (22.5%) were registered with at least one...

  15. Detection of atypical bile acids in disease states and their identification by gas chromatography-mass spectrometry-computer techniques

    Energy Technology Data Exchange (ETDEWEB)

    Szczepanik-Van Leeuwen, P. A.; Stellaard, F.

    1978-01-01

    The study of the bile acid constituents of serum, bile, urine, and stool of patients exhibiting liver disease has increased in importance with the availability of newer methods for their detection and identification. A cogent question for study has been whether specific bile acids are toxic and thus are the cause of liver disease, or whether they accumulate as a result of disease-induced alteration in metabolism. Examining a wide variety of clinical samples, we have observed that many patients with diagnosed cholestasis show the presence of atypical bile acids due to metabolic aberrations in either the side chain or in the steroid ring. Because cholestasis represents a spectrum of diseases with differing metabolic and/or anatomic defects and because our studies cover a variety of cholestatic states, we have sought to establish a correlation between the presence of these atypical bile acids and the disease state. The complexity of the bile acid mixtures to be examined requires that gas chromatographic-mass spectrometric-computer techniques be used to provide a reliable analysis. It is believed that atypical bile acids can be readily identified by GC/CI mass spectrometry with great sensitivity. It is also believed that such bile acid analysis may prove useful to the study and diagnosis of liver disease. Present data suggest that the identification of atypical bile acids in biological samples may enable differentiation between different types of intrahepatic cholestasis. Such analyses may prove useful to distinguish specific diseases, such as Byler's disease (and Byler's-like cholestasis) from other types of cholestasis and may distinguish diseases involving mitochondrial defects. Finally, the presence of atypical bile acids may indicate, by the particular compounds formed, where and what kind of damage occurs in a disease and may ultimately establish if these atypical bile acids are a cause or effect of the liver damage.

  16. 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。

  17. Clinical and Etiological Characteristics of Atypical Hand-Foot-and-Mouth Disease in Children from Chongqing, China: A Retrospective Study

    Directory of Open Access Journals (Sweden)

    Xiang Yan

    2015-01-01

    Full Text Available Background. Hand-foot-and-mouth disease (HFMD is a disease that had similar manifestations to chickenpox, impetigo, and measles, which is easy to misdiagnose and subsequently causes delayed therapy and subsequent epidemic. To date, no study has been conducted to report the clinical and epidemiological characteristics of atypical HFMD. Methods. 64 children with atypical HFMD out of 887 HFMD children were recruited, stool was collected, and viral VP1 was detected. Results. The atypical HFMD accounted for 7.2% of total HFMD in the same period (64/887 and there were two peaks in its prevalence in nonepidemic seasons. Ten children (15.6% had manifestations of neurologic involvement, of whom 4 (6.3% were diagnosed with severe HFMD and 1 with critically severe HFMD, but all recovered smoothly. Onychomadesis and desquamation were found in 14 patients (21.9% and 15 patients (23.4%, respectively. The most common pathogen was coxsackievirus A6 (CV-A6 which accounted for 67.2%, followed by nontypable enterovirus (26.6%, enterovirus 71 (EV-A71 (4.7%, and coxsackievirus A16 (A16 (1.5%. Conclusions. Atypical HFMD has seasonal prevalence. The manifestations of neurologic involvement in atypical HFMD are mild and usually have a good prognosis. CV-A6 is a major pathogen causing atypical HFMD, but not a major pathogen in Chongqing, China.

  18. Analysis of Risk Factors of Incomplete Kawasaki Disease with Coronary Artery Lesion%不完全川崎病冠状动脉损害高危因素分析

    Institute of Scientific and Technical Information of China (English)

    胡建芬; 罗海燕

    2012-01-01

    [目的]对比川崎病(KD)与不完全川崎病患者的临床特征,分析不完全川崎病并冠状动脉损害(CAL)的高危因素.[方法]根据有无CAL分组,比较两组间的临床表现;根据KD诊断标准分为不完全KD组和典型KD组,比较两组的临床特征;将KD并CAL的病例分为不完全KD组和典型KD组,分析不完全KD并发CAL的高危因素.[结果]比较有无CAL两组的临床表现,有CAL组患者出现皮疹与口唇皲裂的例数较低;不完全KD组患者年龄较小,确诊时间较晚,使用IVIG的时间也延迟,而炎症指标CRP、ESR水平不如典型KD组增高显著;不完全KD并CAL发生率高于典型KD组,年龄与使用静脉用两种球蛋白(IVIG)时的热程是不完全KD并发CAL发生率较高的重要因素.[结论]不完全KD并CAL的发生率高于典型KD者,对于年龄较小,症状不典型的患者,应早期行心脏彩超检查,及时诊断和治疗.%[Objective]To compare the clinical features of Kawasaki disease(KD) vs incomplete KD, and to analyze the risk factors of incomplete KD with coronary artery lesion(CAL). [ Methods]The patients were divided into the group of KD patients with CAL and the group of KD without CAL, and the clinical manifestations between these two groups were compared. According to the diagnosis criteria of KD, the patients were divided into incomplete KD group and typical KD group, and the clinical manifestations between these two groups were compared. The KD patients with CAL were divided into incomplete KD group and typical KD group. The risk factors of incomplete KD with CAL were analyzed. [Results]Clinical manifestations between the groups with and without CAL were compared. The case number with skin rash and lip chap in the group with CAL was less. The age of patients in incomplete KD group was lower, and their definite diagnosis was later, and the time of IVIG usage was delayed. The increasing of inflammatory indicators such as CRP and KSR in incomplete KD group was

  19. 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。

  20. 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

  1. Atypical Huntington's disease with the clinical presentation of behavioural variant of frontotemporal dementia.

    Science.gov (United States)

    Sutovsky, Stanislav; Smolek, Tomas; Alafuzoff, Irina; Blaho, Andrej; Parrak, Vojtech; Turcani, Peter; Palkovic, Michal; Petrovic, Robert; Novak, Michal; Zilka, Norbert

    2016-12-01

    Huntington's disease is an incurable, adult-onset, autosomal dominant inherited disorder caused by an expanded trinucleotide repeat (CAG). In this study, we describe a Huntington's disease patient displaying clinical symptoms of the behavioural variant of frontotemporal dementia in the absence of tremor and ataxia. The clinical onset was at the age of 36 years and the disease progressed slowly (18 years). Genetic testing revealed expanded trinucleotide CAG repeats in the Huntingtin gene, together with a Glu318Gly polymorphism in presenilin 1. Neuropathological assessment revealed extensive amyloid β (Aβ) aggregates in all cortical regions. No inclusions displaying hyperphosphorylated tau or phosphorylated transactive response DNA-binding protein 43 (TDP43) were found. A high number of p62 (sequestosome 1) immunopositive intranuclear inclusions were seen mainly in the cortex, while subcortical areas were affected to a lesser extent. Confocal microscopy revealed that the majority of p62 intranuclear lesions co-localised with the fused-in-sarcoma protein (FUS) immunostaining. The morphology of the inclusions resembled intranuclear aggregates in Huntington's disease. The presented proband suffered from Huntington's disease showed atypical distribution of FUS positive intranuclear aggregates in the cortical areas with concomitant Alzheimer's disease pathology.

  2. 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.

  3. 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

  4. Complement Factor B Mutations in Atypical Hemolytic Uremic Syndrome—Disease-Relevant or Benign?

    Science.gov (United States)

    Marinozzi, Maria Chiara; Vergoz, Laura; Rybkine, Tania; Ngo, Stephanie; Bettoni, Serena; Pashov, Anastas; Cayla, Mathieu; Tabarin, Fanny; Jablonski, Mathieu; Hue, Christophe; Smith, Richard J.; Noris, Marina; Halbwachs-Mecarelli, Lise; Donadelli, Roberta; Fremeaux-Bacchi, Veronique

    2014-01-01

    Atypical hemolytic uremic syndrome (aHUS) is a genetic ultrarare renal disease associated with overactivation of the alternative pathway of complement. Four gain-of-function mutations that form a hyperactive or deregulated C3 convertase have been identified in Factor B (FB) ligand binding sites. Here, we studied the functional consequences of 10 FB genetic changes recently identified from different aHUS cohorts. Using several tests for alternative C3 and C5 convertase formation and regulation, we identified two gain-of-function and potentially disease-relevant mutations that formed either an overactive convertase (M433I) or a convertase resistant to decay by FH (K298Q). One mutation (R178Q) produced a partially cleaved protein with no ligand binding or functional activity. Seven genetic changes led to near-normal or only slightly reduced ligand binding and functional activity compared with the most common polymorphism at position 7, R7. Notably, none of the algorithms used to predict the disease relevance of FB mutations agreed completely with the experimental data, suggesting that in silico approaches should be undertaken with caution. These data, combined with previously published results, suggest that 9 of 15 FB genetic changes identified in patients with aHUS are unrelated to disease pathogenesis. This study highlights that functional assessment of identified nucleotide changes in FB is mandatory to confirm disease association. PMID:24652797

  5. 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。

  6. Atypical Hallervorden-Spatz disease with preserved cognition and obtrusive obsessions and compulsions.

    Science.gov (United States)

    Nicholas, Anthony P; Earnst, Kelly S; Marson, Daniel C

    2005-07-01

    We describe the case of an adult female with Hallervorden-Spatz disease (HSD), "eye-of-the-tiger" sign on cranial magnetic resonance imaging scan, and two mutations in the pantothenate kinase 2 (PANK2) gene. Symptomatic presentation included stuttering dysarthria, dystonic posturing, increased limb and axial muscle tone, choreoathetosis, stereotyped motor behaviors, and obsessive-compulsive symptomatology since adolescence. Extensive neuropsychological testing at 40 and 44 years of age revealed a relatively normal IQ and stable cognitive pattern overall. This case demonstrates that HSD patients who survive into middle age should not be assumed to have a progressive dementia. In such cases, atypical behavioral problems such as persistent obsessions and compulsions may be present instead.

  7. Atypical Vascular Involvement in a Case of Behçet's Disease

    Directory of Open Access Journals (Sweden)

    Alejandro Rodríguez Morata

    2012-01-01

    Full Text Available Introduction. Behçet's disease (BD is a form of vasculitis of unknown etiology which is rare in our environment. It is characterized by a variety of clinical manifestations and usually affects young adults. Recurrent oral and genital ulcers are a characteristic and extremely frequent symptom, but mortality is linked with more significant symptoms such as aortic pseudoaneurysm, pulmonary pseudoaneurysm, and cerebral venous thrombosis. Patient and Method. We present a case of a young male with atypical BD and severe polyvascular involvement (previous cerebral venous thrombosis and current peripheral venous thrombosis, acute ischemia, and peripheral arterial pseudoaneurysm who required urgent surgical intervention due to a symptomatic external iliac pseudoaneurysm. Result. The pseudoaneurysm was successfully treated, we performed an iliofemoral bypass, and we treated it with steroids and immunosuppressive therapy. Conclusions. These rare clinical manifestations highlight the importance of considering BD in young patients, even in usual cases of vascular intervention, whether arterial or venous in nature.

  8. 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.

  9. Typical and atypical symptoms of gastro esophageal reflux disease: Does Helicobacter pylori infection matter?

    Institute of Scientific and Technical Information of China (English)

    Laurino; Grossi; Antonio; Francesco; Ciccaglione; Leonardo; Marzio

    2015-01-01

    AIM: To analyze whether the presence of Helicobacter pylori(H. pylori) infection could affect the quality of symptoms in gastro-esophageal reflux disease(GERD) patients. METHODS: one hundred and forty-four consecutive patients referred to our Unit for suspected GERD were recruited for the study. All patients underwent esophageal p H-metric recording. For those with a positive test, C13 urea breath test was then performed to assess the H. pylori status. GERD patients were stratified according to the quality of their symptoms and classified as typical, if affected by heartburn and regurgitation, and atypical if complaining of chest pain, respiratory and ears, nose, and throat features. H. pylori-negative patients were also asked whether they had a previous diagnosis of H. pylori infection. If a positive response was given, on the basis of the time period after successful eradication, patients were considered as "eradicated"(E) if H. pylori eradication occurred more than six months earlier or "recently eradicated" if the therapy had been administered within the last six months. Patients without history of infection were identified as "negative"(N). χ2 test was performed by combining the clinical aspects with the H. pylori status.RESULTS: one hundred and twenty-nine of the 144 patients, including 44 H. pylori-positive and 85 H. pylori-negative(41 negative, 21 recently eradicated, 23 eradicated more than 6 mo before), were eligible for the analysis. No difference has been found between H. pylori status and either the number of reflux episodes(138 ± 23 vs 146 ± 36, respectively, P = 0.2, not significant) or the percentage of time with pH values < 4(6.8 ± 1.2 vs 7.4 ± 2.1, respectively, P = 0.3, not significant). The distribution of symptoms was as follows: 13 typical(30%) and 31 atypical(70%) among the 44 H. pylori-positive cases; 44 typical(52%) and 41 atypical(48%) among the 85 H. pylori-negative cases,(P = 0.017 vs H. pylori +; OR = 2.55, 95%CI: 1

  10. Expression and function of the atypical cadherin FAT1 in chronic liver disease

    Energy Technology Data Exchange (ETDEWEB)

    Valletta, Daniela; Czech, Barbara [Department of Internal Medicine I, University Hospital Regensburg, Regensburg (Germany); Thasler, Wolfgang E. [Grosshadern Tissue Bank and Center for Liver Cell Research, Department of Surgery, Ludwig-Maximilians-University Munich (Germany); Mueller, Martina [Department of Internal Medicine I, University Hospital Regensburg, Regensburg (Germany); Bosserhoff, Anja-Katrin [Institute of Pathology, University of Regensburg, Regensburg (Germany); Hellerbrand, Claus, E-mail: claus.hellerbrand@ukr.de [Department of Internal Medicine I, University Hospital Regensburg, Regensburg (Germany)

    2012-09-28

    Highlights: Black-Right-Pointing-Pointer The expression of the atypical cadherin FAT1 is increased in chronic liver disease. Black-Right-Pointing-Pointer FAT1 expression goes up during the activation of hepatic stellate cells (HSCs). Black-Right-Pointing-Pointer Activated HSCs are the cellular source of enhanced FAT1 expression in diseased livers. Black-Right-Pointing-Pointer FAT1 enhanced NFkB activity and resistance to apoptosis in activated HSCs. Black-Right-Pointing-Pointer FAT1 is a new therapeutic target for prevention and treatment of hepatic fibrosis. -- Abstract: Hepatic fibrosis can be considered as wound healing process in response to hepatocellular injury. Activation of hepatic stellate cells (HSCs) is a key event of hepatic fibrosis since activated HSCs are the cellular source of enhanced extracellular matrix deposition, and reversion of liver fibrosis is accompanied by clearance of activated HSCs by apoptosis. The atypical cadherin FAT1 has been shown to regulate diverse biological functions as cell proliferation and planar cell polarity, and also to affect wound healing. Here, we found increased FAT1 expression in different murine models of chronic liver injury and in cirrhotic livers of patients with different liver disease. Also in hepatic tissue of patients with non-alcoholic steatohepatitis FAT1 expression was significantly enhanced and correlated with collagen alpha I(1) expression. Immunohistochemistry revealed no significant differences in staining intensity between hepatocytes in normal and cirrhotic liver tissue but myofibroblast like cells in fibrotic septa of cirrhotic livers showed a prominent immunosignal. Furthermore, FAT1 mRNA and protein expression markedly increased during in vitro activation of primary human and murine HSCs. Together, these data indicated activated HSCs as cellular source of enhanced FAT1 expression in diseased livers. To gain insight into the functional role of FAT1 in activated HSCs we suppressed FAT1 in these

  11. 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

  12. 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

  13. 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

  14. 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

  15. 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

  16. 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

  17. Late onset of atypical paroxysmal non-kinesigenic dyskinesia with remote history of Graves′ disease

    Directory of Open Access Journals (Sweden)

    Abdul Qayyum Rana

    2013-01-01

    Full Text Available Paroxysmal non-kinesigenic dyskinesia (PNKD is a rare hyperkinetic movement disorder and falls under the category of paroxysmal movement disorders. In this condition, episodes are spontaneous, involuntary, and involve dystonic posturing with choreic and ballistic movements. Attacks last for minutes to hours and rarely occur more than once per day. Attacks are not typically triggered by sudden movement, but may be brought on by alcohol, caffeine, stress, fatigue, or chocolate. We report a patient with multiple atypical features of PNKD. She had a 7-year history of this condition with onset at the age of 59, and a remote history of Graves′ disease requiring total thyroidectomy. The frequency of attacks in our case ranged from five to six times a day to a minimum of twice per week, and the duration of episode was short, lasting not more than 2 min. Typically, PNKDs occur at a much younger age and have longer attack durations with low frequency. Administering clonazepam worked to reduce her symptoms, although majority of previous research suggests that pharmacological interventions have poor outcomes.

  18. Late onset of atypical paroxysmal non-kinesigenic dyskinesia with remote history of Graves' disease.

    Science.gov (United States)

    Rana, Abdul Qayyum; Nadeem, Ambreen; Yousuf, Muhammad Saad; Kachhvi, Zakerabibi M

    2013-10-01

    Paroxysmal non-kinesigenic dyskinesia (PNKD) is a rare hyperkinetic movement disorder and falls under the category of paroxysmal movement disorders. In this condition, episodes are spontaneous, involuntary, and involve dystonic posturing with choreic and ballistic movements. Attacks last for minutes to hours and rarely occur more than once per day. Attacks are not typically triggered by sudden movement, but may be brought on by alcohol, caffeine, stress, fatigue, or chocolate. We report a patient with multiple atypical features of PNKD. She had a 7-year history of this condition with onset at the age of 59, and a remote history of Graves' disease requiring total thyroidectomy. The frequency of attacks in our case ranged from five to six times a day to a minimum of twice per week, and the duration of episode was short, lasting not more than 2 min. Typically, PNKDs occur at a much younger age and have longer attack durations with low frequency. Administering clonazepam worked to reduce her symptoms, although majority of previous research suggests that pharmacological interventions have poor outcomes.

  19. 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.

  20. Using atypical symptoms and red flags to identify non-demyelinating disease.

    LENUS (Irish Health Repository)

    Kelly, Siobhan B

    2012-01-01

    Red flags and atypical symptoms have been described as being useful in suggesting alternative diagnoses to multiple sclerosis (MS) and clinically isolated syndrome (CIS); however, their diagnostic utility has not been assessed. The aim of this study was to establish the predictive value of red flags and the typicality\\/atypicality of symptoms at presentation in relation to the final diagnosis of patients referred with suspected MS.

  1. 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,

  2. 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.

  3. 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

  4. 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.

  5. Atypical pneumonia

    Science.gov (United States)

    Walking pneumonia; Community-acquired pneumonia - atypical ... Bacteria that cause atypical pneumonia include: Mycoplasma pneumonia is caused by the bacteria Mycoplasma pneumoniae . It often affects people younger than age 40. Pneumonia due ...

  6. 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.

  7. Side effects induced by the acute levodopa challenge in Parkinson’s Disease and atypical parkinsonisms

    Science.gov (United States)

    Mostile, Giovanni; Dibilio, Valeria; Sciacca, Giorgia; Contrafatto, Donatella; Cicero, Calogero Edoardo; Raciti, Loredana; Luca, Antonina; Zappia, Mario

    2017-01-01

    Introduction Acute levodopa challenge may be performed to predict levodopa chronic responsiveness. The aim of the study was to investigate frequency of side effects during the acute levodopa challenge in PD and atypical parkinsonisms. Methods We enrolled 34 de novo PD patients and 29 patients affected by atypical parkinsonisms (Multiple System Atrophy, MSA, n = 10; Progressive Supranuclear Palsy, PSP, n = 12 and Corticobasal Degeneration, CBD, n = 7) who underwent an acute levodopa challenge. Side effects occurring during test were recorded. Results Side effects were more frequent among atypical parkinsonisms as unique group when compared to PD patients (64.3% versus 23.5%; p-value 0.002) with an adjusted OR of 4.36 (95%CI 1.40–13.5). Each atypical parkinsonisms showed almost double occurrence of side effects (MSA 90%, PSP 41.7% and CBD 57%). Conclusions Side effects during acute levodopa challenge may be frequent in atypical parkinsonisms. This information could be useful in order to better prepare the patient for the test. Furthermore, it could represent a useful cue in differential diagnosis with PD. PMID:28207803

  8. Association between Serum Atypical Fibroblast Growth Factors 21 and 19 and Pediatric Nonalcoholic Fatty Liver Disease.

    Directory of Open Access Journals (Sweden)

    Anna Alisi

    Full Text Available Atypical fibroblast growth factors (FGF 21 and 19 play a central role in energy metabolism through the mediation of Klotho coreceptor. Contradictory findings are available about the association of FGF21 and FGF19 with nonalcoholic fatty liver disease (NAFLD in humans. We investigated the association of serum FGF21, FGF19 and liver Klotho coreceptor with non-alcoholic steatohepatitis (NASH and fibrosis in children with NAFLD. Serum FGF21 and FGF19 were measured in 84 children with biopsy-proven NAFLD and 23 controls (CTRL. The hepatic expression of Klotho coreceptor was measured in 7 CTRL, 9 patients with NASH (NASH+ and 11 patients without NASH (NASH-. FGF21 and FGF19 showed a tendency to decrease from CTRL (median FGF21 = 196 pg/mL; median FGF19 = 201 pg/mL to NASH- (FGF21 = 89 pg/mL; FGF19 = 81 pg/mL to NASH+ patients (FGF21 = 54 pg/mL; FGF19 = 41 pg/mL (p<0.001 for all comparisons and were inversely associated with the probability of NASH and fibrosis in children with NAFLD. The hepatic expression of Klotho coreceptor was inversely associated with NASH (R(2 = 0.87, p<0.0001 and directly associated with serum FGF21 (R(2 = 0.57, p<0.0001 and FGF19 (R(2 = 0.67, p<0.0001. In conclusion, serum FGF19 and FGF21 and hepatic Klotho expression are inversely associated with hepatic damage in children with NAFLD and these findings may have important implications for understanding the mechanisms of NAFLD progression.

  9. Association between Serum Atypical Fibroblast Growth Factors 21 and 19 and Pediatric Nonalcoholic Fatty Liver Disease.

    Science.gov (United States)

    Alisi, Anna; Ceccarelli, Sara; Panera, Nadia; Prono, Federica; Petrini, Stefania; De Stefanis, Cristiano; Pezzullo, Marco; Tozzi, Alberto; Villani, Alberto; Bedogni, Giorgio; Nobili, Valerio

    2013-01-01

    Atypical fibroblast growth factors (FGF) 21 and 19 play a central role in energy metabolism through the mediation of Klotho coreceptor. Contradictory findings are available about the association of FGF21 and FGF19 with nonalcoholic fatty liver disease (NAFLD) in humans. We investigated the association of serum FGF21, FGF19 and liver Klotho coreceptor with non-alcoholic steatohepatitis (NASH) and fibrosis in children with NAFLD. Serum FGF21 and FGF19 were measured in 84 children with biopsy-proven NAFLD and 23 controls (CTRL). The hepatic expression of Klotho coreceptor was measured in 7 CTRL, 9 patients with NASH (NASH+) and 11 patients without NASH (NASH-). FGF21 and FGF19 showed a tendency to decrease from CTRL (median FGF21 = 196 pg/mL; median FGF19 = 201 pg/mL) to NASH- (FGF21 = 89 pg/mL; FGF19 = 81 pg/mL) to NASH+ patients (FGF21 = 54 pg/mL; FGF19 = 41 pg/mL) (p<0.001 for all comparisons) and were inversely associated with the probability of NASH and fibrosis in children with NAFLD. The hepatic expression of Klotho coreceptor was inversely associated with NASH (R(2) = 0.87, p<0.0001) and directly associated with serum FGF21 (R(2) = 0.57, p<0.0001) and FGF19 (R(2) = 0.67, p<0.0001). In conclusion, serum FGF19 and FGF21 and hepatic Klotho expression are inversely associated with hepatic damage in children with NAFLD and these findings may have important implications for understanding the mechanisms of NAFLD progression.

  10. 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。

  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. 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.

  13. 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。

  14. Low-Dose Atypical Antipsychotic Risperidone Improves the 5-Year Outcome in Alzheimer's Disease Patients with Sleep Disturbances.

    Science.gov (United States)

    Yin, You; Liu, Yan; Zhuang, Jianhua; Pan, Xiao; Li, Peng; Yang, Yuechang; Li, Yan-Peng; Zhao, Zheng-Qing; Huang, Liu-Qing; Zhao, Zhong-Xin

    2015-01-01

    Sleep disturbances (SD) accelerate the progression of Alzheimer's disease (AD) and increase the stress of caregivers. However, the long-term outcome of disturbed nocturnal sleep/wake patterns in AD and on increased stress of spousal caregivers is unclear. This study assessed the 5-year effect of nocturnal SD on the long-term outcome in AD patients. A total of 156 donepezil-treated mild-moderate AD patients (93 AD + SD and 63 AD - SD as a control group) were recruited. The AD + SD patients were formed into 4 subgroups according to the preferences of spousal caregivers for treatment with atypical antipsychotics (0.5-1 mg risperidone, n = 22), non-benzodiazepine hypnotic (5-10 mg zolpidem tartrate, n = 33), melatonin (2.55 mg, n = 9), or no-drug treatment (n = 29). SD were evaluated by polysomnography, sleep scale, and cognitive scale examinations. Moreover, all spousal caregivers of AD patients were assessed using a series of scales, including sleep, anxiety, mood, and treatment attitude scales. Our data showed that nocturnal sleep/wake disturbances were significantly associated with lower 5-year outcomes for AD patients, earlier nursing home placement, and more negative emotions of spousal caregivers. Treatment with low-dose atypical antipsychotic risperidone improved the 5-year outcome in AD + SD patients. In conclusion, low-dose atypical antipsychotic risperidone improves the 5-year outcome in AD patients with SD. Moreover, improvement of nocturnal sleep problems in AD patients will also bring better emotional stability for AD caregivers.

  15. 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。

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

    Directory of Open Access Journals (Sweden)

    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.

  17. 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.

  18. 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.

  19. 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。

  20. 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.

  1. 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.

  2. 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

  3. 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

  4. 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

  5. Atypical sporadic CJD-MM phenotype with white matter kuru plaques associated with intranuclear inclusion body and argyrophilic grain disease.

    Science.gov (United States)

    Berghoff, Anna S; Trummert, Anita; Unterberger, Ursula; Ströbel, Thomas; Hortobágyi, Tibor; Kovacs, Gabor G

    2015-08-01

    We describe an atypical neuropathological phenotype of sporadic Creutzfeldt-Jakob disease in a 76-year-old man. The clinical symptoms were characterized by progressive dementia, gait ataxia, rigidity and urinary incontinence. The disease duration was 6 weeks. MRI did not show prominent atrophy or hyperintensities in cortical areas, striatum or thalamus. Biomarker examination of the cerebrospinal fluid deviated from that seen in pure Alzheimer's disease. Triphasic waves in the EEG were detected only later in the disease course, while 14-3-3 assay was positive. PRNP genotyping revealed methionine homozygosity (MM) at codon 129. Neuropathology showed classical CJD changes corresponding to the MM type 1 cases. However, a striking feature was the presence of abundant kuru-type plaques in the white matter. This rare morphology was associated with neuropathological signs of intranuclear inclusion body disease and advanced stage of argyrophilic grain disease. These alterations did not show correlation with each other, thus seemed to develop independently. This case further highlights the complexity of neuropathological alterations in the ageing brain.

  6. 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。

  7. 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

  8. Diseases of the Gastrointestinal Tract in Individuals Diagnosed as Children with Atypical Autism: A Danish Register Study Based on Hospital Diagnoses

    Science.gov (United States)

    Mouridsen, Svend Erik; Isager, Torben; Rich, Bente

    2013-01-01

    The purpose of this study is to compare the prevalence and types of diseases (International Classification of Mental and Behavioural Disorders, 10th Edition codes K20-K93) relating to the gastrointestinal tract in a clinical sample of 89 individuals diagnosed as children with atypical autism/pervasive developmental disorder not otherwise specified…

  9. 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.

  10. 儿童病毒感染与川崎病发病及冠状动脉病变的相关性%Relationship between virus infection in children with Kawasaki disease and coronary artery disease

    Institute of Scientific and Technical Information of China (English)

    彭俊娟; 王锦; 朱伟; 徐丽琴

    2016-01-01

    目的:探究病毒感染与川崎病( KD)发病及冠状动脉病变的相关性。方法选取KD儿童105例为KD组,同期选取发热性疾病但非KD患儿210例为非KD组。对两组患儿的病毒感染情况进行比较,并行超声心动图检查,比较两组儿童冠状动脉病变情况。结果两组患儿中病毒感染232例,其中,KD组感染96例,占91.43%,非KD组感染136例,占64.76%。两组EB病毒感染率比较,KD组患儿明显高于非KD组患儿,差异具有统计学意义(χ2=32.874,P<0.001);呼吸道合胞病毒、副流感病毒、流感病毒、腺病毒、单纯疱疹病毒、冠状病毒感染率两组比较,差异均无统计学差异(均P>0.05)。分别将KD组和非KD组分为感染组与未感染组,其中KD组中感染组患儿冠状动脉损伤率最高,为46.55%(43/96),显著高于KD组的未感染组(11.11%),差异均具有统计学意义(确切概率法得出P=0.04),亦高于非KD组的感染组(7.59%)和未感染组(8.22%),差异均具有统计学意义(χ2值分别为42.45、27.41,均P<0.001);而KD组中未感染组(11.11%)、非KD组的感染组(7.95%)和未感染组(8.11%)比较差异无统计学意义(P>0.05)。结论患儿病毒感染与KD发病有关,且病毒感染的KD患儿更易发生冠状动脉病变。%Objective To investigate the relationship between virus infection in children with Kawasaki disease ( KD) and coronary artery disease.Methods Totally 105 cases of children with KD were selected in KD group , and 210 children with febrile disease but without KD were chosen in non-KD group.The status of virus infection and coronary artery injury were compared between two groups by ultrasonic cardiogram examination.Results There were 232 cases with virus infection, including 96 cases (91.43%) in KD group and 136 cases (64.76%) in non-KD group.EB virus

  11. Clinical Significance of the Platelet Parameter in Children with Kawasaki Disease%川崎病患儿血小板参数变化及临床意义

    Institute of Scientific and Technical Information of China (English)

    梁慕琼

    2015-01-01

    目的:观察川崎病(KD)患儿血小板参数的变化,探讨其对冠状动脉病变(CAL)的提示作用。方法:将2013年1月至2014年6月收治的急性期川崎病患儿为观察组(n=42),再根据心脏彩超结果将川崎病患儿分为有 CAL 组(n=24)和无 CAL 组(n=18),以同期年龄匹配的健康体检儿童为对照组(n=40),比较各组血小板数量(PLT)、平均血小板体积(MPV)、血小板分布宽度(PDW)。结果:与对照组相比,观察组PLT显著增高,MPV和PDW 均显著下降(P 均<0.05);而川崎病并 CAL 组与无 CAL 组的 PLT、MPV 及 PDW 均无明显差异。结论:血小板参数中PLT、MPV及PDW 可协助诊断KD ,但不能预测CAL的发生。%Objective:To observe the changes of platelet parameters and the indicate action of coronary artery lesion (CAL)in patients with Kawasaki disease (KD). Method:42 patients with KD were classified as observation group(n=42) and divided into CAL group(n=24) and no CAL group(n=18) according to UCG,while health children were classified as control group(n=40).The platelet count(PLT),mean platelet volume(MPV) and platelet distribution width (PDW) were compared among the groups. Results:Compared with the control group,the PLT were significantly higher,but MPV and PDW were significantly lower in the observation group,all P<0.05. Meanwhile,the platelet parameters were no significant differences between the CAL group and no CAL group. Conclusions:The changes of platelet parameters may be help to the diagnose of KD, but had nothing to do with CAL.

  12. [Atypical symptoms of Fabry's disease: sudden bilateral deafness, lymphoedema and Lown-Ganong-Levine syndrome].

    Science.gov (United States)

    Undas, Anetta; Ryś, Donata; Wegrzyn, Wojciech; Musiał, Jacek

    2002-11-01

    A 40-year-old man with Fabry disease, confirmed by decreased leukocyte alpha-galactosidase A activity in 2001, complained of sudden bilateral deafness, as evidenced by clinical history and audiometry. Magnetic resonance of the brain revealed features typical of Fabry disease. Other clinical manifestations of the disease included: angiokeratoma, mild proteinuria with normal renal function, lymphoedema of the lower limbs, pre-excitation syndrome, myocardial hypertrophy.

  13. A case of Refsum disease with atypical clinical picture in family members.

    Science.gov (United States)

    Marano, R; Soliveri, P; Garavaglia, B; Antonelli, A; Girotti, F

    1989-08-01

    A typical case of Refsum disease with high phytanic acid plasma levels is described. Two siblings showed some features but not the entire clinical spectrum of the disease. The unusual condition of the patient's father, a presumed heterozygotic carrier with characteristic bone abnormalities and a delayed onset retinopathy, is discussed.

  14. Atypical Cities

    Science.gov (United States)

    DiJulio, Betsy

    2011-01-01

    In this creative challenge, Surrealism and one-point perspective combine to produce images that not only go "beyond the real" but also beyond the ubiquitous "imaginary city" assignment often used to teach one-point perspective. Perhaps the difference is that in the "atypical cities challenge," an understanding of one-point perspective is a means…

  15. Chronic wasting disease and atypical forms of bovine spongiform encephalopathy and scrapie are not transmissible to mice expressing wild-type levels of human prion protein.

    Science.gov (United States)

    Wilson, Rona; Plinston, Chris; Hunter, Nora; Casalone, Cristina; Corona, Cristiano; Tagliavini, Fabrizio; Suardi, Silvia; Ruggerone, Margherita; Moda, Fabio; Graziano, Silvia; Sbriccoli, Marco; Cardone, Franco; Pocchiari, Maurizio; Ingrosso, Loredana; Baron, Thierry; Richt, Juergen; Andreoletti, Olivier; Simmons, Marion; Lockey, Richard; Manson, Jean C; Barron, Rona M

    2012-07-01

    The association between bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) has demonstrated that cattle transmissible spongiform encephalopathies (TSEs) can pose a risk to human health and raises the possibility that other ruminant TSEs may be transmissible to humans. In recent years, several novel TSEs in sheep, cattle and deer have been described and the risk posed to humans by these agents is currently unknown. In this study, we inoculated two forms of atypical BSE (BASE and H-type BSE), a chronic wasting disease (CWD) isolate and seven isolates of atypical scrapie into gene-targeted transgenic (Tg) mice expressing the human prion protein (PrP). Upon challenge with these ruminant TSEs, gene-targeted Tg mice expressing human PrP did not show any signs of disease pathology. These data strongly suggest the presence of a substantial transmission barrier between these recently identified ruminant TSEs and humans.

  16. Review: A review on classical and atypical scrapie in caprine: Prion protein gene polymorphisms and their role in the disease.

    Science.gov (United States)

    Curcio, L; Sebastiani, C; Di Lorenzo, P; Lasagna, E; Biagetti, M

    2016-10-01

    Scrapie is a naturally occurring transmissible spongiform encephalopathy in sheep and goat. It has been known for ~250 years and is characterised by the accumulation of an abnormal isoform of a host-encoded prion protein that leads to progressive neurodegeneration and death. Scrapie is recognised in two forms, classical and atypical scrapie. The susceptibility to both types of scrapie is influenced by polymorphisms of the prion protein gene (PRNP). Sheep susceptibility or resistance to classical scrapie is strongly regulated by the polymorphisms at codons 136, 154 and 171 of the PRNP. The genetic role in atypical scrapie in sheep has been defined by polymorphisms at codons 141, 154 and 171, which are associated with different degrees of risk in the occurrence of the ovine disease. Progress has been achieved in the prevention of scrapie in sheep due to efficient genetic breeding programmes based on eradication and control of the disease. In Europe, the success of these programmes has been verified by applying eradication and genetic selection plans. In general terms, the ovine selection plans aim to eliminate and reduce the susceptible allele and to enrich the resistant allele ARR. During outbreaks all susceptible animals are slaughtered, only ARR/ARR resistant rams and sheep and semi-resistant females are preserved. In the occurrence of scrapie positive goats a complete cull of the flock (stamping out) is performed with great economic loss and severe risk of extinction for the endangered breeds. The ability to select scrapie-resistant animals allows to define new breeding strategies aimed to boost genetic progress while reducing costs during scrapie outbreaks. Allelic variants of PRNP can be protective for caprine scrapie, and the knowledge of their distribution in goats has become very important. Over the past few years, the integration of genetic information on goat populations could be used to make selection decisions, commonly referred to as genetic selection

  17. “Sickle Cell Disease in the Emergency Department: Atypical Complications and Management”

    OpenAIRE

    Brandow, Amanda M.; Liem, Robert

    2011-01-01

    Sickle cell disease is the most common inherited blood disorder in the United States. This disorder of hemoglobin structure leads to a chronic hemolytic anemia and complex chronic disease manifested by sudden, severe, and life-threatening complications. These acute complications can occur in any organ system beginning in early childhood and lasting throughout life. The intermittent nature and acuity of these complications lend the emergency department to be an important site of care. The hall...

  18. Should post-mortem brain dissection be performed in Parkinson's disease with atypical dementia?

    Science.gov (United States)

    Lauretani, F; Ceda, G P; Nardelli, A; Maggio, M

    2015-02-01

    We describe a patient affected by PD with a rapid progression of cognitive decline. This case could suggest the coexistence of many neurodegenerative diseases, which is a common condition in older patients. We propose an hypothetical trajectory of the cognitive impairment usually associated with motor symptoms in the later phase of Parkinsonian patients. The trajectory is almost linear in classical Parkinson's disease dementia (PDD), while a constant acceleration of the cognitive decline with a subsequent change of the slope of the direction could suggest the coexistence of PD with other neurodegenerative disease. Finally, if the cognitive decline in PD is comparable to a "stepped" decline, vascular lesions could be the cause of the change of the slope. This case could suggest to request an autopsy in all cases of unexplained PDD, for better understanding the mixture of non-motor symptoms in PD.

  19. Atypical Parkinsonism Revealing a Late Onset, Rigid and Akinetic Form of Huntington's Disease

    Directory of Open Access Journals (Sweden)

    A. Ciammola

    2011-01-01

    Full Text Available Huntington's disease (HD is a rare hereditary neurodegenerative disorder characterized in over 90 percent of cases by chorea as the presenting motor symptom. We report a 54-year-old male who presented with Parkinsonism as the initial symptom of the disease. Genetic analysis revealed expansion of 40 CAG repeats, and brain MRI showed both severe caudate nuclei and cortical atrophy. Single-photon emission computed tomography (SPECT imaging of the dopamine transporter showed nigrostriatal pathway degeneration. Here, we also describe his 2 years of clinical followup after ensuing dopaminergic stimulation.

  20. [Immunofluorescence assay with Crithidia luciliae for the detection of anti-DNA antibodies. Atypical images and their relationship with Chagas' disease and leishmaniasis].

    Science.gov (United States)

    Griemberg, Gloria; Ferrarotti, Nidia F; Svibel, Graciela; Ravelli, Maria R; Taranto, Nestor J; Malchiodi, Emilio L; Pizzimenti, Maria C

    2006-01-01

    Anti-native DNA antibodies can be detected by indirect immunofluorescence assay with Crithidia luciliae, displaying an annular image due to a kinetoplast containing double stranded DNA. Other structures such as membrane, flagellum and basal corpuscle can be stained as well, showing what is called atypical fluorescent images. As C. luciliae belongs to the Trypanosomatidae family, which include the human pathogens Trypanosoma cruzi and Leishmania spp., it was considered that these atypical images could be caused by cross-reactions. Serological studies for Chagas' disease were performed in 105 serum samples displaying atypical images. Sixty four percent of the samples from non endemic and 78.3% from endemic areas for Chagas' disease showed fluorescence in both, membrane and flagellum (joint image). Fifty samples from normal blood donors and 57 samples from patients with conective tissue diseases were tested with C. luciliae. None of them presented the joint image except for two patients with lupus who were also chagasic. In addition, 54 samples from chagasic patients were studied and all of them presented the joint image. We also studied 46 samples from patients with leishmaniasis from whom 28 were coinfected with T. cruzi. The joint image was observed in 88.0% of the samples with leishmaniasis and in 89.3% of the co-infected samples. The results suggest that C. luciliae could be used as an economical, and of low risk, alternative substrate for the serological diagnosis of Chagas' disease, even though it does not discriminate for Leishmania spp. infection. This study also suggests that whenever atypical images are observed in C. luciliae during the search for anti-DNA antibodies, it would be convenient to submit the patient to clinical and serological tests for the diagnosis of leishmaniosis and Chagas' disease.

  1. Cat-scratch disease in Northern Italy: atypical clinical manifestations in humans and prevalence of Bartonella infection in cats.

    Science.gov (United States)

    Brunetti, E; Fabbi, M; Ferraioli, G; Prati, P; Filice, C; Sassera, D; Dalla Valle, C; Bandi, C; Vicari, N; Marone, P

    2013-04-01

    In this paper, we report an investigation on cat-scratch disease (CSD) in Northern Italy. Seventy-four cases of CSD were diagnosed at the San Matteo hospital, Pavia, during the period 2005-2010. Of these 74 patients, 18 (24.3 %) reported atypical clinical manifestations such as ocular papillitis, maculopapular eruptions, vertebral infection, pulmonary infiltrates, and granulomatous hepatitis. Contact with cats was documented for 61 patients (82.4 %), while cat-related trauma was reported for 49 patients (66.2 %). We subsequently investigated the presence of Bartonella infection in cats belonging to the above patients and in other domestic and stray cats from three provinces of Northern Italy. Among the 27 domestic cats tested, nine of the 11 belonging to the CSD patients and two of the remaining 16 were infected by B. henselae (81.8 % vs. 12.5 %). Out of over 1,300 stray cats examined, 23.1 % were seropositive for B. henselae; after culturing and genotyping, 17 % were found to be infected by B. henselae (15.5 %) or B. clarridgeiae (1.5 %).

  2. Characterisation of an atypical manifestation of black band disease on Porites lutea in the Western Indian Ocean

    Science.gov (United States)

    Wilkinson, David A.; Schleyer, Michael H.; Chabanet, Pascale; Quod, Jean-Pascal; Tortosa, Pablo

    2016-01-01

    Recent surveys conducted on Reunion Island coral reefs revealed an atypical manifestation of black band disease on the main framework building coral, Porites lutea. This BBD manifestation (PorBBD) presented a thick lighter-colored band, which preceded the typical BBD lesion. Whilst BBD aetiology has been intensively described worldwide, it remains unclear if corals with apparently similar lesions across coral reefs are affected by the same pathogens. Therefore, a multidisciplinary approach involving field surveys, gross lesion monitoring, histopathology and 454-pyrosequencing was employed to provide the first comprehensive characterization of this particular manifestation. Surveys conducted within two geomorphological zones over two consecutive summers and winters showed spatial and seasonal patterns consistent with those found for typical BBD. Genetic analyses suggested an uncharacteristically high level of Vibrio spp. bacterial infection within PorBBD. However, microscopic analysis revealed high densities of cyanobacteria, penetrating the compromised tissue as well as the presence of basophilic bodies resembling bacterial aggregates in the living tissue, adjacent to the bacterial mat. Additionally, classical BBD-associated cyanobacterial strains, genetically related to Pseudoscillatoria coralii and Roseofilum reptotaenium were identified and isolated and the presence of sulfate-reducers or sulfide-oxidizers such as Desulfovibrio and Arcobacter, previously shown to be associated with anoxic microenvironment within typical BBD was also observed, confirming that PorBBD is a manifestation of classical BBD. PMID:27441106

  3. Characterisation of an atypical manifestation of black band disease on Porites lutea in the Western Indian Ocean

    Directory of Open Access Journals (Sweden)

    Mathieu Séré

    2016-07-01

    Full Text Available Recent surveys conducted on Reunion Island coral reefs revealed an atypical manifestation of black band disease on the main framework building coral, Porites lutea. This BBD manifestation (PorBBD presented a thick lighter-colored band, which preceded the typical BBD lesion. Whilst BBD aetiology has been intensively described worldwide, it remains unclear if corals with apparently similar lesions across coral reefs are affected by the same pathogens. Therefore, a multidisciplinary approach involving field surveys, gross lesion monitoring, histopathology and 454-pyrosequencing was employed to provide the first comprehensive characterization of this particular manifestation. Surveys conducted within two geomorphological zones over two consecutive summers and winters showed spatial and seasonal patterns consistent with those found for typical BBD. Genetic analyses suggested an uncharacteristically high level of Vibrio spp. bacterial infection within PorBBD. However, microscopic analysis revealed high densities of cyanobacteria, penetrating the compromised tissue as well as the presence of basophilic bodies resembling bacterial aggregates in the living tissue, adjacent to the bacterial mat. Additionally, classical BBD-associated cyanobacterial strains, genetically related to Pseudoscillatoria coralii and Roseofilum reptotaenium were identified and isolated and the presence of sulfate-reducers or sulfide-oxidizers such as Desulfovibrio and Arcobacter, previously shown to be associated with anoxic microenvironment within typical BBD was also observed, confirming that PorBBD is a manifestation of classical BBD.

  4. Effects of Different dose of Immunoglobulin for Children with Kawasaki disease and Effect on Coronary Artery Lesion%不同剂量丙种球蛋白对川崎病患儿的疗效及对冠状动脉病变的影响

    Institute of Scientific and Technical Information of China (English)

    刘芳; 陈嫕; 秦蔚; 张慧

    2013-01-01

      目的探讨静脉注射不同剂量丙种球蛋白(IVIG)对治疗川崎病的疗效及对冠状动脉病变的近期和远期影响。方法选取2008年3月至2009年10月期间在我院和新华医院儿科接受治疗的64例川崎病患儿,分为IVIG 1g/kg观察组与IVIG 2g/kg对照组,观察两种治疗方法的疗效,并对患儿进3年左右的随访,观察对冠状动脉病变的远期影响。结果两组患者退热时间、颈淋巴结肿大消退时间、黏膜充血、手足肿胀和总热程等情况基本相同;各项实验室检查结果与治疗前比较,两组患者血小板计数、血沉、外周血白细胞计数、C反应蛋白明显降低(P<0.05),两组间比较差异无显著性。随访两组对冠状动脉病变的远期影响无明显差异。结论治疗川崎病过程中应用丙种球蛋白1g/kg单次静脉注射,可起到与丙种球蛋白2 g/kg单次静脉注射治疗川崎病相似的效果,有效降低冠状动脉病变的发生率。%  Objective To investigate the effect of different doses of intravenous immunoglobulin in treatment of Kawasaki disease and effect on coronary artery lesion. Methods From 2008 March to 2009 October treated in our hospital and XinHua Hospital 64 cases of Kawasaki disease in children, were divided into IVIG 1g/kg observation group and IVIG 2g/kg control group, compared the effect of two kinds of treatment methods. Children with total duration of fever, neck lymph node swelling subsided, hand and foot swelling and membrane hyperaemia were observed, in addition shall also monitor coronary artery lesion, platelet count, white blood cell counts, C reactive protein and blood sedimentation occurred. And 3 years follow-up was done to observe remote effect of CAL in two groups. Results The cooling time, two groups of patients with cervical lymphadenopathy subsided, mucosal hyperemia, swelling of the hands and feet and total heat process is basically the same

  5. [Typical and atypical ocular manifestations of Behçet's disease].

    Science.gov (United States)

    Stübiger, N; Pleyer, U

    2012-06-01

    The first ocular symptom in 50-87% of patients with Behçet's disease (BD) is unilateral anterior uveitis. As a characteristic sign of ocular BD the appearance of hypopyon iritis was originally described by Adamantiades and Behçet, but nowadays, probably due to an earlier and more aggressive treatment, this rarely occurs. In the further course of the disease up to 75% of BD patients develop a bilateral chronic relapsing form of posterior uveitis or panuveitis. In addition occlusive retinal vasculitis, which involves arteries as well as veins, is frequently present. A typical complication consists of a cystoid macular edema, which is, besides the retinal vasculitis, responsible for the limited prognosis of visual acuity.

  6. Atypical Creutzfeldt-Jakob Disease Evolution after Electroconvulsive Therapy for Catatonic Depression

    Directory of Open Access Journals (Sweden)

    Iria Grande

    2011-01-01

    Full Text Available We describe a case report of an 80-year-old woman who presented with symptomatology compatible with an episode of major depression with catatonia. After psychiatric admission, electroconvulsive therapy (ECT was applied, but symptoms progressed with cognitive impairment, bradykinesia, widespread stiffness, postural tremor, and gait disturbance. After compatible magnetic resonance imaging (MRI, diffusion changes, and electroencephalogram (EEG findings the case was reoriented to Creutzfeldt-Jakob disease (CJD. The genetic study found a methionine/valine heterozygosity at codon 129 of the prion protein gene PrPSc. On followup, a significant clinical recovery turned out. For this reason, EEG and MRI were repeated and confirmed the findings. The patient subsequently demonstrated progressive clinical deterioration and died 21 months later. The diagnosis was verified postmortem by neuropathology. The vCJD subtype MV2 is indeed characterized by early and prominent psychiatric symptoms and a prolonged disease duration however no frank clinical recovery has before been reported.

  7. Cavitary pulmonary nodules in atypical collagen disease and lupoid drug reaction. Report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Muren, C.; Strandberg, O.

    The case histories of two patients with cavitary pulmonary nodules and the findings at chest radiography are reviewed. The first patient had a connective tissue disease with features common to systematic lupus erythematosus and Wegener's granulomatosis. In the second patient the lung changes developed as part of a drug reaction to carbamezapine and/or phenytoin. The common denominator of the cavitating nodules is probably the presence of granulomas, developing as a sequela of pulmonary vasculitis. (orig.).

  8. Late endothelial function in children with coronary aneurysm due to Kawasaki disease%川崎病冠脉瘤患儿远期血管内皮功能的研究

    Institute of Scientific and Technical Information of China (English)

    段超; 杜忠东; 王玉; 贾立群

    2011-01-01

    Objective To evaluate the late endothelial function in children with coronary aneurysm due to Kawasaki disease (KD). Methods Thirty-one children with coronary aneurysms duc to KD who had the disease course for more than 1 year and twenty-one age-matched healthy children were enrolled. Brachial artery endothelium-dependent and -independent flow-mediated dilation (FMD), carotid arterial stiffness index (SI) and intima-media thickness (IMT) were measured by high-frequency ultrasound. Results There were 9 cases of medium and 22 cases of giant coronary aneurysms in the KD group. Twelve KD patients had evidence of myocardial ischemia. Compared to the normal controls, the endotheliumdependent FMD decreased (P < 0. 05), the carotid arterial SI increased (P < 0.05), and the carotid arterial intima-media thickness increased significantly (P <0.05) in children with coronary aneurysms due to KD. The endothelium-dependent FMD decreased more significantly in 12 KD patients with myocardial ischemia than in those without any evidence of myocardial ischemia (P < O. 05 ). Conclusions Late endothelial dysfunction exists in children with coronary aneurysms due to KD, especially in those with myocardial ischemia.%目的 评估川崎病(KD)冠脉瘤患儿远期血管内皮功能.方法 选择病程大于1年的合并中型以上冠脉瘤的川崎病患儿31例为研究对象,采用高分辨率超声仪进行肱动脉内皮依赖性和非依赖性舒张功能(FMD)、颈动脉僵硬度指数(SI)和颈动脉内中膜厚度(IMT)的检测.选择年龄相近的正常儿童21例作为对照.结果 31例KD冠脉瘤患儿中,中型冠脉瘤9例,冠脉巨大瘤22例,其中12例有心肌缺血.与对照组比较,KD冠脉瘤组患儿肱动脉血管内皮依赖性FMD减低(P<0.05),颈动脉SI增加(P<0.05),颈动脉IMT增厚(P<0.05).与无心肌缺血患儿相比,有心肌缺血患儿血管内皮依赖性FMD显著降低(P<0.05).结论 KD冠脉瘤患儿远期存在血管内皮功能障碍,

  9. Critical Overview of the Risk Scoring Systems to Predict Non-Responsiveness to Intravenous Immunoglobulin in Kawasaki Syndrome

    Directory of Open Access Journals (Sweden)

    Donato Rigante

    2016-02-01

    Full Text Available Kawasaki syndrome (KS is the most relevant cause of heart disease in children living in developed countries. Intravenous immunoglobulin (IVIG has a preventive function in the formation of coronary artery abnormalities and a poor strictly-curative action in established coronary damage. More than two decades ago, the Harada score was set to assess which children with KS should be subject to administration of IVIG, evaluating retrospectively a large cohort of patients with regard to age, sex and laboratory data. Nowadays, high dose IVIG is administered to all children with a confirmed diagnosis of KS, but a tool for predicting non-responsiveness to the initial infusion of IVIG has not been found. The prediction of IVIG resistance is a crucial issue, as recognising these high-risk patients should consent the administration of an intensified initial treatment in combination with IVIG in order to prevent coronary injuries. Few reports have focused on factors, referring to both clinical parameters and laboratory data at the onset of KS, in order to predict which patients might be IVIG non-responsive. We have analysed three different risk scores which were formulated to predict IVIG resistance in Japanese children with typical KS, but their application in non-Japanese patients or in those with incomplete and atypical patterns of the disease has been studied in a fragmentary way. Overall, our analysis showed that early and definite ascertainment of likely IVIG non-responders who require additional therapies reducing the development of coronary artery involvement in children with KS is still a challenge.

  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. 川崎病患儿血小板膜糖蛋白Ibα基因多态性分析%Investigation of platelet membrane glycoprotein Ibα gene polymorphisms in children with Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    李卓颖; 黄麟; 杨作成

    2013-01-01

    Objective To investigate the association between platelet glycoprotein Ibα (GPIbα) gene HPA-2a/b polymorphisms and the risk of Kawasaki disease (KD) and that complicated with coronary artery lesion (CAL).Methods A total of 30 patients with KD and 60 healthy controls were genotyped by polymerase chain reaction-restriction fragment length polymorphism and agarose gel electrophoresis for the HPA-2a/b polymorphism in GPIbα gene.Results For HPA-2a/b polymorphism in GPIbα gene,there was only genotype TC and CC of HPA-2a/b polymorphism in GPIbα gene in children with KD and healthy controls,and genotype TF was not found in both groups.There were no significant differences between KD patients and the controls in genotype frequencies of CC,TC and TT and allele frequencies of C and T (x2 =0.052,0.048,all P > 0.05) ; also there was no significant difference between KD patients with CAL and that without CAL in genotype and allele frequencies (x2 =2.672,2.481,all P > 0.05).Conclusion No association is found between HPA-2a/b polymorphism in GPIbα gene and the risk of KD or its complication of CAL in this study.%目的 研究血小板膜糖蛋白Ibα(GPIbα)基因HPA-2a/b多态性与KD发病及并发冠状动脉损伤(CAL)之间的关系.方法 应用聚合酶链反应-限制性内切酶片断长度多态性分析技术结合琼脂糖凝胶电泳技术,检测30例KD患儿和60例健康对照组儿童GPIbα基因HPA-2a/b多态性位点的基因型和等位基因分布.结果 本组KD病例和健康对照组儿童GPIbα基因HPA-2a/b的基因型均只有TC型和CC型,而未发现TT型.KD组GPIbα基因HPA-2a/b多态性的CC、TC、TT基因型分布频率和C、T等位基因频率与健康对照组比较差异均无统计学意义(x2=0.052、0.048,P均>0.05),KD组中合并CAL组与无CAL组基因型分布频率和等位基因频率比较差异亦无统计学意义(x2=2.672、2.481,P均>0.05).结论 GPIbα基因的HPA-2a/b多态性与KD及其CAL的发生均无明显相关性.

  12. 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.

  13. 临床护理路径在川崎病患儿护理工作中的应用研究%Application study of clinical nursing path in nursing work of children with Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    代宗琴; 何秋谊

    2015-01-01

    Objective To investigate the implementation effect of clinical nursing path (CNP) among children with Kawasaki disease (KD) to establish the best nursing model. Methods From January 2013 to December 2014,60 chil-dren with KD were randomly divided into observation group and control group and there were 30 patients in each group.Observation group was given nursing model guided by CNP,while control group was given traditional nursing model.Defervescence time,white blood cell count,C-reactive protein (CRP) level,score of satisfaction degree,mastering degree score of health knowledge about KD and hospital stay between two groups was compared respectively. Results Defervescence time in observation group was shorter than that of control group,white blood cell count and CRP level was lower than that of control group respectively and there was a statistical difference (P<0.05);score of satisfaction de-gree,mastering degree of health knowledge about KD in observation group was higher than that of control group,while hospital stay was shorter than that of control group,and there was a statistical difference (P<0.05). Conclusion Nursing model guided by CNP can improve clinical symptom and inflammatory indicator of patients with KD,improve satisfac-tion degree for medical personnel and awareness rate for health knowledge,and shorteren hospital stay at the same time, thus it is better than traditional nursing model.%目的:探讨临床护理路径(CNP)对川崎病(KD)患儿的实施效果,建立KD患儿的最佳护理模式。方法2013年1月~2014年12月,将60例KD患儿随机分为观察组和对照组,各30例。观察组予以CNP指导的护理模式,对照组予以传统护理模式。比较两组的退热时间、白细胞数、C反应蛋白(CRP)水平、满意度评分、KD健康知识掌握程度评分和住院时间。结果观察组的退热时间短于对照组,白细胞数和CRP水平低于对照组,差异有统计学意义(P<0.05);

  14. CT in nontraumatic acute thoracic aortic disease: typical and atypical features and complications.

    Science.gov (United States)

    Castañer, Eva; Andreu, Marta; Gallardo, Xavier; Mata, Josep Maria; Cabezuelo, María Angeles; Pallardó, Yolanda

    2003-10-01

    Thoracic aortic dissection is the most frequent cause of aortic emergency, and unless it is rapidly diagnosed and treated, the result is death. Helical computed tomography (CT) permits the diagnosis of acute aortic dissection with a sensitivity and specificity of nearly 100%. This imaging modality also enables differentiation between proximal aortic dissection (type A in the Stanford classification) and distal aortic dissection (Stanford type B), which are treated differently and have different prognoses. In 70% of patients in whom nontraumatic acute thoracic aortic dissection is diagnosed after evaluation with helical CT, scans show the typical signs of aortic dissection, with rupture and displacement of the intima. CT also can depict other pathologic entities with similar clinical manifestations, such as intramural hematoma and penetrating atherosclerotic ulcer. Awareness of the different radiologic appearances of these disease entities is essential for differential diagnosis. More than one-third of patients with aortic dissection show signs and symptoms indicative of systemic involvement. Because branch-vessel involvement may increase morbidity and mortality, in this group of patients it is important to evaluate the entire aorta so as to determine the distal extent of the dissection and detect any systemic involvement.

  15. Atypical Complications of Graves’ Disease: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Khaled Ahmed Baagar

    2017-01-01

    Full Text Available Graves’ disease (GD may display uncommon manifestations. We report a patient with rare complications of GD and present a comprehensive literature review. A 35-year-old woman presented with a two-week history of dyspnea, palpitations, and edema. She had a raised jugular venous pressure, goiter, and exophthalmos. Laboratory tests showed pancytopenia, a raised alkaline phosphatase level, hyperbilirubinemia (mainly direct bilirubin, and hyperthyroidism [TSH: 46.08 pmol/L (reference values: 2.6–5.7]. Her thyroid uptake scan indicated GD. Echocardiography showed a high right ventricular systolic pressure: 60.16 mmHg. Lugol’s iodine, propranolol, cholestyramine, and dexamethasone were initiated. Hematologic investigations uncovered no reason for the pancytopenia; therefore, carbimazole was started. Workup for hepatic impairment and pulmonary hypertension (PH was negative. The patient became euthyroid after 3 months. Leukocyte and platelet counts and bilirubin levels normalized, and her hemoglobin and alkaline phosphatase levels and right ventricular systolic pressure (52.64 mmHg improved. This is the first reported single case of GD with the following three rare manifestations: pancytopenia, cholestatic liver injury, and PH with right-sided heart failure. With antithyroid drugs treatment, pancytopenia should resolve with euthyroidism, but PH and liver injury may take several months to resolve.

  16. Atypical Complications of Graves' Disease: A Case Report and Literature Review

    Science.gov (United States)

    Siddique, Mashhood Ahmed; Arroub, Shaimaa Ahmed; Ebrahim, Ahmed Hamdi; Jayyousi, Amin Ahmed

    2017-01-01

    Graves' disease (GD) may display uncommon manifestations. We report a patient with rare complications of GD and present a comprehensive literature review. A 35-year-old woman presented with a two-week history of dyspnea, palpitations, and edema. She had a raised jugular venous pressure, goiter, and exophthalmos. Laboratory tests showed pancytopenia, a raised alkaline phosphatase level, hyperbilirubinemia (mainly direct bilirubin), and hyperthyroidism [TSH: 46.08 pmol/L (reference values: 2.6–5.7)]. Her thyroid uptake scan indicated GD. Echocardiography showed a high right ventricular systolic pressure: 60.16 mmHg. Lugol's iodine, propranolol, cholestyramine, and dexamethasone were initiated. Hematologic investigations uncovered no reason for the pancytopenia; therefore, carbimazole was started. Workup for hepatic impairment and pulmonary hypertension (PH) was negative. The patient became euthyroid after 3 months. Leukocyte and platelet counts and bilirubin levels normalized, and her hemoglobin and alkaline phosphatase levels and right ventricular systolic pressure (52.64 mmHg) improved. This is the first reported single case of GD with the following three rare manifestations: pancytopenia, cholestatic liver injury, and PH with right-sided heart failure. With antithyroid drugs treatment, pancytopenia should resolve with euthyroidism, but PH and liver injury may take several months to resolve. PMID:28348902

  17. Utility of susceptibility-weighted MRI in differentiating Parkinson's disease and atypical parkinsonism

    Energy Technology Data Exchange (ETDEWEB)

    Gupta, Deepak [Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Department of Neurology, Trivandrum, Kerala (India); Saini, Jitender; Kesavadas, Chandrasekharan [Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Department of Imaging Sciences and Interventional Radiology, Trivandrum, Kerala (India); Sarma, P.S. [Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Achutha Menon Centre for Health Sciences, Trivandrum, Kerala (India); Kishore, Asha [Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Department of Neurology, Trivandrum, Kerala (India); Sree Chitra Tirunal Institute for Medical Sciences and Technology (SCTIMST), Comprehensive Care Centre for Movement Disorders, Trivandrum, Kerala (India)

    2010-12-15

    Neuropathological studies report varying patterns of brain mineralization in Parkinson's diseases (PD), progressive supranuclear palsy (PSP), and Parkinson variant of multiple system atrophy (MSA-P). Susceptibility-weighted imaging (SWI) is the ideal magnetic resonance imaging (MRI) technique to detect mineralization of the brain. The purpose of this study was to test if SWI can differentiate PD, PSP, and MSA-P. Eleven patients with PD, 12 with PSP, 12 with MSA-P, and 11 healthy controls underwent SWI of the brain. Hypointensity of putamen, red nucleus, substantia nigra, and dentate nucleus in all groups were measured using an objective grading scale and scored from 0 to 3. In PSP, hypointensity score of red nucleus was higher than that in MSA-P (p = 0.001) and PD (p = 0.001), and a score of {>=}2 differentiated the PSP group from the PD and MSA-P groups. Putaminal hypointensity score was higher in PSP when compared to that in PD (p = 0.003), and a score of {>=}2 differentiated PSP from PD groups. SWI hypointensity scores of red nucleus and putamen had an excellent intrarater and interrater correlation. Substantia nigra hypointensity score of the PSP group was higher than that of the MSA-P (p = 0.004) and PD (p = 0.006) groups, but the scores had only a moderate intrarater and interrater correlation. SWI shows different patterns of brain mineralization in clinically diagnosed groups of PD, PSP, and MSA-P and may be considered as an additional MR protocol to help differentiate these conditions. (orig.)

  18. Carotid Artery Disease

    Science.gov (United States)

    ... Kawasaki Disease Long Q-T Syndrome Marfan Syndrome Metabolic Syndrome Mitral Valve Prolapse Myocardial Bridge Myocarditis Obstructive Sleep Apnea Pericarditis Peripheral Vascular Disease Rheumatic Fever Sick Sinus Syndrome Silent Ischemia Stroke Sudden ...

  19. Subcellular localization of SREBP1 depends on its interaction with the C-terminal region of wild-type and disease related A-type lamins

    Science.gov (United States)

    Duband-Goulet, Isabelle; Woerner, Stephanie; Gasparini, Sylvaine; Attanda, Wikayatou; Kondé, Emilie; Tellier-Lebègue, Carine; Craescu, Constantin T.; Gombault, Aurélie; Roussel, Pascal; Vadrot, Nathalie; Vicart, Patrick; Östlund, Cecilia; Worman, Howard J.; Zinn-Justin, Sophie; Buendia, Brigitte

    2011-01-01

    Lamins A and C are nuclear intermediate filament proteins expressed in most differentiated somatic cells. Previous data suggested that prelamin A, the lamin A precursor, accumulates in some lipodystrophy syndromes caused by mutations in the lamin A/C gene, and binds and inactivates the sterol regulatory element binding protein 1 (SREBP1). Here we show that, in vitro, the tail regions of prelamin A, lamin A and lamin C bind a polypeptide of SREBP1. Such interactions also occur in HeLa cells, since expression of lamin tail regions impedes nucleolar accumulation of the SREBP1 polypeptide fused to a nucleolar localization signal sequence. In addition, the tail regions of A-type lamin variants that occur in Dunnigan-type familial partial lipodystrophy of (R482W) and Hutchison Gilford progeria syndrome (Δ607–656) bind to the SREBP1 polypeptide in vitro, and the corresponding FLAG-tagged full-length lamin variants co-immunoprecipitate the SREBP1 polypeptide in cells. Overexpression of wild-type A-type lamins and variants favors SREBP1 polypeptide localization at the intranuclear periphery, suggesting its sequestration. Our data support the hypothesis that variation of A-type lamin protein level and spatial organization, in particular due to disease-linked mutations, influences the sequestration of SREBP1 at the nuclear envelope and thus contributes to the regulation of SREBP1 function. PMID:21993218

  20. Subcellular localization of SREBP1 depends on its interaction with the C-terminal region of wild-type and disease related A-type lamins

    Energy Technology Data Exchange (ETDEWEB)

    Duband-Goulet, Isabelle; Woerner, Stephanie [Laboratoire du Stress et Pathologies du Cytosquelette, Universite Paris Diderot-Paris 7, CNRS, Institut de Biologie Fonctionnelle et Adaptative, 4 rue M.A. Lagroua Weill Halle, 75205 Paris cedex 13 (France); Gasparini, Sylvaine [Laboratoire de Biologie Structurale et Radiobiologie, URA CNRS 2096, Commissariat a l' Energie Atomique Saclay, 91190 Gif-sur-Yvette (France); Attanda, Wikayatou [Laboratoire du Stress et Pathologies du Cytosquelette, Universite Paris Diderot-Paris 7, CNRS, Institut de Biologie Fonctionnelle et Adaptative, 4 rue M.A. Lagroua Weill Halle, 75205 Paris cedex 13 (France); Konde, Emilie; Tellier-Lebegue, Carine [Laboratoire de Biologie Structurale et Radiobiologie, URA CNRS 2096, Commissariat a l' Energie Atomique Saclay, 91190 Gif-sur-Yvette (France); Craescu, Constantin T. [INSERM U759, Institut Curie/Universite de Paris-Sud, 91405 Orsay Cedex (France); Gombault, Aurelie [Laboratoire du Stress et Pathologies du Cytosquelette, Universite Paris Diderot-Paris 7, CNRS, Institut de Biologie Fonctionnelle et Adaptative, 4 rue M.A. Lagroua Weill Halle, 75205 Paris cedex 13 (France); Roussel, Pascal [Institut Jacques Monod, UMR 7592, Universite Paris Diderot-Paris 7, CNRS, 15 rue Helene Brion, 75205 Paris (France); Vadrot, Nathalie; Vicart, Patrick [Laboratoire du Stress et Pathologies du Cytosquelette, Universite Paris Diderot-Paris 7, CNRS, Institut de Biologie Fonctionnelle et Adaptative, 4 rue M.A. Lagroua Weill Halle, 75205 Paris cedex 13 (France); Oestlund, Cecilia; Worman, Howard J. [Department of Medicine and Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York, NY (United States); and others

    2011-12-10

    Lamins A and C are nuclear intermediate filament proteins expressed in most differentiated somatic cells. Previous data suggested that prelamin A, the lamin A precursor, accumulates in some lipodystrophy syndromes caused by mutations in the lamin A/C gene, and binds and inactivates the sterol regulatory element binding protein 1 (SREBP1). Here we show that, in vitro, the tail regions of prelamin A, lamin A and lamin C bind a polypeptide of SREBP1. Such interactions also occur in HeLa cells, since expression of lamin tail regions impedes nucleolar accumulation of the SREBP1 polypeptide fused to a nucleolar localization signal sequence. In addition, the tail regions of A-type lamin variants that occur in Dunnigan-type familial partial lipodystrophy of (R482W) and Hutchison Gilford progeria syndrome ( Increment 607-656) bind to the SREBP1 polypeptide in vitro, and the corresponding FLAG-tagged full-length lamin variants co-immunoprecipitate the SREBP1 polypeptide in cells. Overexpression of wild-type A-type lamins and variants favors SREBP1 polypeptide localization at the intranuclear periphery, suggesting its sequestration. Our data support the hypothesis that variation of A-type lamin protein level and spatial organization, in particular due to disease-linked mutations, influences the sequestration of SREBP1 at the nuclear envelope and thus contributes to the regulation of SREBP1 function.

  1. Atypical GTPases as drug targets.

    Science.gov (United States)

    Soundararajan, Meera; Eswaran, Jeyanthy

    2012-01-01

    The Ras GTPases are the founding members of large Ras superfamily, which constitutes more than 150 of these important class of enzymes. These GTPases function as GDP-GTP-regulated binary switches that control many fundamental cellular processes. There are a number of GTPases that have been identified recently, which do not confine to this prototype termed as "atypical GTPases" but have proved to play a remarkable role in vital cellular functions. In this review, we provide an overview of the crucial physiological functions mediated by RGK and Centaurin class of multi domain atypical GTPases. Moreover, the recently available atypical GTPase structures of the two families, regulation, physiological functions and their critical roles in various diseases will be discussed. In summary, this review will highlight the emerging atypical GTPase family which allows us to understand novel regulatory mechanisms and thus providing new avenues for drug discovery programs.

  2. 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

  3. Lifting of a sector block for YE-2 at Kawasaki.

    CERN Multimedia

    R. Loveless/U. of Wisconsin

    2000-01-01

    YE-2 is build from machined sector blocks. Trial assembly is carried out horizontally. This picture represents the lifting of a machined sector block destined to the trial assembly of a half disk YE-2 at Kawasaki (KHI) Kobe, Japan.

  4. Effect of intravenous infusion of high does gamma globulin therapy on preventing Kawasaki disease children from eoronary artery damage and nursing%大剂量静脉滴注丙种球蛋白防止川崎病患儿冠伏动脉损害的效果评价及护理

    Institute of Scientific and Technical Information of China (English)

    郭志芬

    2012-01-01

    目的 探讨大剂量静脉滴注丙种球蛋白防止川崎病患儿冠状动脉损害的效果.方法 将82例川崎病患儿依据治疗方法分为观察组和对照组各41例,观察组患儿采用大剂量丙种球蛋白冲击治疗,对照组应用常规用量进行治疗.对照组采取常规护理,观察组在对照组的基础上按症状实施针对性的护理措施.比较2组的治疗及护理效果.结果 观察组发生冠状动脉扩张1例,对照组发生8例,观察组发生率显著低于对照组.主要观察指标如发热、淋巴结肿大及皮肤黏膜改变的恢复时间观察组明显较对照组缩短.结论 良好细致的护理和健康教育指导,可增加患儿及家属对川崎病的了解,积极配合医疗各个环节,可减少并发症,加快疾病康复.%Objective To explore the effect and nursing of high dose gamma globulin therapy on preventing Kawasaki disease children from coronary artery damage.Methods 82 cases of children with Kawasaki disease were divided into two groups according to the treatment methods,each with 41 cases.The observation group was treated by high dose shock treatment of gamma globulin,the control group was given conventional dosage of gamma globulin.The control group received routine nursing care.The observation group was given targeted nursing measures according to the symptoms on the basis of the control group.The treatment and nursing effect were compared between two groups.Results 1 cases of coronary artery dilatation appeared in the observation group,while 8 cases in the control group,the difference was statistically significant.The recovery time of main observation index such as fever,lymphadenopathy,skin and mucous membrane alterations was significantly shorter in the observation group than the control group,the differences were statistically significant.Conclusions Careful nursing and health education guidance can increase the understanding of disease for Kawasaki disease children and their

  5. Clinical analysis of incomplete Kawasaki disease in a single centre in 9 years%单中心9年不完全川崎病回顾性分析

    Institute of Scientific and Technical Information of China (English)

    付培培; 杜忠东; 潘岳松

    2012-01-01

    目的 探讨不完全川崎病(KD)的临床特征,以提高临床诊治水平.方法 回顾性分析2002年1月至2010年12月KD住院患儿的临床资料,比较不完全KD与典型KD在发病年龄、性别、临床表现、实验室检查、治疗及冠状动脉损害等方面的差异.结果 1 484例KD患儿进入分析,其中不完全KD 262例(17.6%),典型KD 1 222例;<1岁患儿中不完全KD占24.9%.不完全KD和典型KD患儿的平均发热时间分别为(7.8±5.0)和(6.7±3.6)d,差异有统计学意义.不完全KD四肢改变、多形皮疹、眼结膜充血、口唇改变、颈部淋巴结肿大和肛周改变的发生率显著高于典型KD;卡疤改变、扁桃体肿大、阴囊或外阴改变、呕吐和腹泻的发生率与典型KD差异无统计学意义.两组CRP、ESR、Hb、WBC、PLT、ALT、AST、CK-MB和LDH等实验室指标差异无统计学意义.不完全KD与典型KD患儿对IVIG无反应的发生率差异无统计学意义(14.1% vs 17.5%);不完全KD患儿冠状动脉扩张、冠状动脉瘤和巨大冠状动脉瘤的发生率分别为57.5%、14.1%和1.9%,典型KD患儿分别为31.5%、5.9% 和0.6%,两组差异有统计学意义.结论 不完全KD较典型KD发热时间长,且冠状动脉损害发生率高,但实验室指标差别不大.%Objective To summarize the clinical feature, diagnosis, treatment and prognosis of incomplete Kawasaki disease ( KD ) cases from Beijing Children's Hospital and improve the levels of diagnosis and treatment. Methods A retrospective review of patients with KD from January 2002 to December 2010 in Beijing Children's Hospital was performed. Demographic and clinical data included gender, age, recurrence rate, clinical manifestation ( including fever, bilateral conjunctival injection, changes in the lips and oral cavity, nonpurulent cervical lymph ad eno pat hy, polymorphous exanthema, changes in the extremities, recurrent redness and erythema around the BCG scar, changes around anus, vomiting, diarrhea

  6. Application comparison between bundle nursing and traditional nursing for children with Kawasaki disease%集束化护理与传统护理在川崎病患儿中的应用比较

    Institute of Scientific and Technical Information of China (English)

    夏蓓南

    2015-01-01

    Objective To compare the application effect of bundle nursing and traditional nursing in children with Kawasaki disease (KD). Methods Totals of 120 KD children had been randomly divided into two groups on average: experimental group and control group. The children were given conventional nursing combined bundle nursing in the experimental group, while the children acquired routine treatment in the control group. Bundle nursing involved service attitude, technical capacity, communication, health education and ward management. The satisfaction degree of nursing job and children clinical index were analyzed. Results The level of the service attitude, technical capacity, communication, health education and ward management were (4. 24 ± 0. 64),(4. 37 ± 0. 56),(4. 28 ± 0. 64),(4. 34 ± 0. 60) and (4. 34 ± 0. 64) respectively in the experimental group and that of them in the control group were (3. 67 ± 0. 83),(3. 99 ± 0. 81), (3. 64 ± 0. 81), (3. 52 ± 1. 10) and (3. 55 ± 1. 04) (t = 4. 21,2. 99,4. 80,5. 07,5. 01, respectively;P < 0. 05). The average time of defervescence, the value of hemameba and C-reactive protein and the days of hospitalization in the experimental group(3. 67 ± 1. 73)d, (9. 17 ± 1. 79) × 109 / L, (30. 66 ± 2. 81) μg/ ml and (7. 39 ± 2. 65) d respectively, which were lower than that of the control group (t = 2. 34,5. 72,10. 42,2. 21, respectively;P <0. 05). Conclusions The bundle nursing can dramatically improve the treatment effect in children with KD.%目的:比较集束化护理及传统护理在川崎病患儿治疗中的应用效果。方法将120例川崎病患儿采用随机数字表法随机分为研究组及对照组各60例。对照组给予传统护理,研究组在此基础上给予集束化护理,从服务态度、技术能力、关怀沟通、健康教育和病房管理5个部分来分析护理工作的满意度,并分析患者临床指标改善状况。结果研究组患儿在服务态度、技术能力、

  7. 川崎病恢复期颈动脉内膜-中膜厚度变化及相关因素分析%Study of carotid intima media thickness and its correlated factors in children at the convalescent phase of Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    牛峰海; 王瑜; 蔡文仙; 范国贞; 任雪云

    2012-01-01

    Objective To observe the change of carotid intima-media thickness and analysis of its correlated factors at the convalescent phase in children with Kawasaki disease, and to provide a scientific basis for prevention and treatment of vascular lesions. Methods A cohort of 60 children during the age of 2 to 4 years old was studied, which comprised 30 children at the convalescence phase of Kawasaki disease (KD group) and 30 healthy age-matched children (control group). Carotid arterial intima-media thickness (IMT) , malondialdehyde ( MDA) , interleukin-1 beta (IL-1|3) , interferon-gamma (IFN-7) and body mass index (BMI) were evaluated. Results IMT in KD group and control group was (0.40 ± 0.03) mm and (0.37 ± 0.04) mm respectively. IMT in KD group was higher than that in control group (P 0.05). IMT was positively correlated with the plasma level of MDA (r = 0.463, P < 0.05), but not significantly correlated with the levels of IL-iβ, IFN-7 and BMI. Conclusions The increased carotid IMT in children at the convalescent phase of Kawasaki disease is associated with oxidative stress.%目的 观察川崎病(KD)患儿恢复期颈动脉内膜-中膜厚度变化及相关因素,为防治KD血管病变提供科学依据.方法 收集30例2 ~ 4岁KD恢复期患儿及30例同年龄健康儿童,测定其颈动脉内膜-中膜厚度(IMT)、丙二醛(MDA)、白介素-1β(IL-1β)、γ-干扰素(IFN-γ)和体质指数(BMI).结果 KD患儿颈动脉IMT为(0.40 ± 0.03)mm,对照组为(0.37 ± 0.04)mm,两者比较差异有统计学意义.KD患儿MDA 为(2.56±0.18)nmol/ml,对照组为(2.09±0.24)nmol/ml,两者比较差异有统计学意义.两组间IL-1β、IFN-γ、BMI比较,差异均无统计学意义.直线相关分析显示,颈动脉IMT与MDA呈正相关(r = 0.463,P < 0.05),而与IL-1β、IFN-γ和 BMI水平均无相关性(P均> 0.05).结论 KD恢复期患儿颈动脉内膜-中膜增厚,与氧化应激有关.

  8. Atypical form of Alzheimer's disease with prominent posterior cortical atrophy: a review of lesion distribution and circuit disconnection in cortical visual pathways

    Science.gov (United States)

    Hof, P. R.; Vogt, B. A.; Bouras, C.; Morrison, J. H.; Bloom, F. E. (Principal Investigator)

    1997-01-01

    In recent years, the existence of visual variants of Alzheimer's disease characterized by atypical clinical presentation at onset has been increasingly recognized. In many of these cases post-mortem neuropathological assessment revealed that correlations could be established between clinical symptoms and the distribution of neurodegenerative lesions. We have analyzed a series of Alzheimer's disease patients presenting with prominent visual symptomatology as a cardinal sign of the disease. In these cases, a shift in the distribution of pathological lesions was observed such that the primary visual areas and certain visual association areas within the occipito-parieto-temporal junction and posterior cingulate cortex had very high densities of lesions, whereas the prefrontal cortex had fewer lesions than usually observed in Alzheimer's disease. Previous quantitative analyses have demonstrated that in Alzheimer's disease, primary sensory and motor cortical areas are less damaged than the multimodal association areas of the frontal and temporal lobes, as indicated by the laminar and regional distribution patterns of neurofibrillary tangles and senile plaques. The distribution of pathological lesions in the cerebral cortex of Alzheimer's disease cases with visual symptomatology revealed that specific visual association pathways were disrupted, whereas these particular connections are likely to be affected to a less severe degree in the more common form of Alzheimer's disease. These data suggest that in some cases with visual variants of Alzheimer's disease, the neurological symptomatology may be related to the loss of certain components of the cortical visual pathways, as reflected by the particular distribution of the neuropathological markers of the disease.

  9. Treatment options for atypical optic neuritis

    Directory of Open Access Journals (Sweden)

    Amina Malik

    2014-01-01

    Full Text Available Context: Optic neuritis (ON is defined as inflammation of the optic nerve and can have various etiologies. The most common presentation in the US is demyelinating, or "typical" ON, usually associated with multiple sclerosis. This is in contrast to "atypical" causes of ON, which differ in their clinical presentation, management, and prognosis. These atypical cases are characterized by lack of eye pain, exudates, and hemorrhages on exam, very severe, bilateral or progressive visual loss, or with failure to recover vision. Aims: The aim was to describe the clinical presentations of atypical ON and their treatments. Settings and Design: Review article. Materials and Methods: Literature review. Results: Types of atypical ON identified include neuromyelitis optica, autoimmune optic neuropathy, chronic relapsing inflammatory optic neuropathy, idiopathic recurrent neuroretinitis, and optic neuropathy associated with systemic diseases. Atypical ON usually requires corticosteroid treatment and often will require aggressive immunosuppression. Conclusions: Unlike demyelinating ON, atypical ON requires treatment to preserve vision.

  10. TRAF6 promotes atypical ubiquitination of mutant DJ-1 and alpha-synuclein and is localized to Lewy bodies in sporadic Parkinson's disease brains.

    Science.gov (United States)

    Zucchelli, Silvia; Codrich, Marta; Marcuzzi, Federica; Pinto, Milena; Vilotti, Sandra; Biagioli, Marta; Ferrer, Isidro; Gustincich, Stefano

    2010-10-01

    Parkinson's disease (PD) is a neurodegenerative disorder characterized by loss of dopaminergic neurons in the Substantia Nigra and the formation of ubiquitin- and alpha-synuclein (aSYN)-positive cytoplasmic inclusions called Lewy bodies (LBs). Although most PD cases are sporadic, families with genetic mutations have been found. Mutations in PARK7/DJ-1 have been associated with autosomal recessive early-onset PD, while missense mutations or duplications of aSYN (PARK1, PARK4) have been linked to dominant forms of the disease. In this study, we identify the E3 ubiquitin ligase tumor necrosis factor-receptor associated factor 6 (TRAF6) as a common player in genetic and sporadic cases. TRAF6 binds misfolded mutant DJ-1 and aSYN. Both proteins are substrates of TRAF6 ligase activity in vivo. Interestingly, rather than conventional K63 assembly, TRAF6 promotes atypical ubiquitin linkage formation to both PD targets that share K6-, K27- and K29- mediated ubiquitination. Importantly, TRAF6 stimulates the accumulation of insoluble and polyubiquitinated mutant DJ-1 into cytoplasmic aggregates. In human post-mortem brains of PD patients, TRAF6 protein colocalizes with aSYN in LBs. These results reveal a novel role for TRAF6 and for atypical ubiquitination in PD pathogenesis.

  11. The Application of Comprehensive Nursing Intervention in Children with Kawasaki Disease and Its Clinical Application%综合护理干预在小儿川崎病中的应用及临床使用参考价值研究

    Institute of Scientific and Technical Information of China (English)

    谢新曲

    2015-01-01

    目的:研究在小儿川崎病的临床护理中应用综合护理干预的临床参考价值。方法:选取2013年7月-2015年7月本院住院治疗的小儿川崎病患者60例,按照住院号采用随机数字表法分为综合护理干预组(观察组)30例、常规护理组(对照组)30例,对比两组的临床护理效果。结果:观察组与对照组的总有效率(100% vs 93.33%)比较差异无统计学意义(P>0.05),但观察组的治愈率高于对照组(90.00% vs 60.00%),差异有统计学意义(P0.05), but the cure rate (90.00% vs 60.00%)of the observation group was significantly higher than that of the control group (P<0.05). The incidence of complications in the observation group was significantly lower than that in the control group (P<0.05). The nursing intervention was statistically significant [(90.24±4.45)vs(82.25±3.23)] and the control group [(97.86±4.32)vs(90.11±3.65)], and the observation group was better than the control group (P<0.05).Conclusion: Comprehensive nursing intervention in the treatment of children with Kawasaki disease can improve the degree of parents of children with Kawasaki disease and nursing satisfaction, improve clinical efficacy, reduce the incidence of complications, it is worthy of popularization and application.

  12. Diseases of the Tricuspid Valve

    Science.gov (United States)

    ... Kawasaki Disease Long Q-T Syndrome Marfan Syndrome Metabolic Syndrome Mitral Valve Prolapse Myocardial Bridge Myocarditis Obstructive Sleep Apnea Pericarditis Peripheral Vascular Disease Rheumatic Fever Sick Sinus Syndrome Silent Ischemia Stroke Sudden ...

  13. Peripheral Vascular Disease

    Science.gov (United States)

    ... Murmurs High Blood Pressure (Hypertension) Infective Endocarditis Kawasaki Disease Long Q-T Syndrome Marfan Syndrome Metabolic Syndrome Mitral Valve Prolapse Myocardial Bridge Myocarditis Obstructive Sleep Apnea Pericarditis Rheumatic Fever Sick Sinus Syndrome Silent Ischemia Stroke Sudden ...

  14. 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

  15. 川崎病患儿血清抗β2糖蛋白Ⅰ抗体和抗心磷脂抗体的检测及其意义%The levels and relationship of anticardiolipin antibody and anti β2 lycoprotein I antibody in the serum of children with kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    刘桂英; 谭岩; 杜军保

    2005-01-01

    严重的心血管系统损害是川崎病(kawasaki disease,KD)主要的并发症.冠状动脉瘤(coronary arteries aneurysms,CAA)是KD患儿致死的主要原因.不治疗的KD发生冠状动脉并发症的发生率为25%~30%,病死率为1%~2%,其中6%以上死于心肌梗死.自从1990年发现β2糖蛋白Ⅰ(β2-glycoprotein I,β2GPⅠ)是抗心磷脂抗体(anticardiolipid antibody,ACA)与心磷脂体外结合所必需的辅助因子以来,β2GPⅠ引起人们广泛的注意.我们探讨了抗β2GPⅠ抗体和ACA在KD患儿血清中的水平及其临床意义。

  16. 川崎病冠状动脉病变的随访及超声心动图和冠状动脉造影价值的研究%Follow-up of coronary artery lesions caused by Kawasaki disease and the val ue of coronary angiography

    Institute of Scientific and Technical Information of China (English)

    龚方戚; 白石裕比湖; 桃井真里子

    2002-01-01

    Objective To investigate the course of coronary artery lesions caused by Kawasaki disease, and the value of coronary angiography (CAG) and two-dimensional echocardiograp hy (2-D Echo) in the evaluation and follow-up of coronary artery lesions. Methods Eighty seven patients with coronary artery lesions caused by Kawasaki disease fr om 1979 to 1997 were retrospectively analyzed. One hundred and sixty-seven CA Gs were performed in 87 patients during follow-up. CAG was repeated every 1- 3 years in each patient until complete regression was confirmed. 2-D Echo was performed before CAG each time. The longest period of follow-up was 16 years and 6 months. Patients were treated with aspirin or aspirin and warfarin. Results During follow-up, the coronary artery lesions regressed in 48/87 (55%) patients , however, they developed into severe coronary artery lesions in 6/87 (7%) patie nts in whom coronary artery bypass surgery was performed. The coronary artery aneurysm regressed in some patients, while stenotic lesions remained or develope d. The ratio of coronary artery stenotic lesions to aneurysms increased progre ssively. This study showed that Echo diagnosis of coronary artery lesions has "false positives" and "false negatives". Only 76% of coronary aneurysms and 18% of stenotic lesions could be found by 2-D Echo. No stenotic lesion could be found in distal segments of the coronary artery. Conclusions Long term follow up revealed spontaneous regression occurred in 55% of patients and development into severe coronary artery stenosis in 7%. It is necessary to perform long-term follow-up in patients with coronary artery lesions caused b y Kawasaki disease. 2-D Echo can not completely replace CAG during follow-up of coronary artery lesions caused by Kawasaki disease.%目的探讨川崎病后冠状动脉病变及转归,以及二维超声心动图和选择性冠状动脉造影在冠状动脉病变判断和长期随访中的作用.方法 1979-1997年因川崎病在治疗和随

  17. 不同剂量丙种球蛋白对川崎病患儿的疗效及对冠状动脉病变的影响%The clinical effect on different dosage of intravenous immune globulin in the treatment of Kawasaki disease and its influence on coronary artery lesion

    Institute of Scientific and Technical Information of China (English)

    何俊峰

    2012-01-01

    目的 评价静脉注射不同剂量的丙种球蛋白(intravenous immune globulin,IVIG)治疗川崎病(Kawasaki disease,KD)的临床效果及对冠状动脉病变的影响.方法 选取我院儿科2006年4月~2011年10月收治的102例KD患儿,随机分为观察组和对照组,每组各51例.观察组患儿给予IVIG 1 g/kg静脉注射,对照组患儿给予IVIG 2 g/kg静脉注射,对两种治疗方法的疗效进行比较.观察患儿总热程、退热时间、黏膜充血、手足肿胀和颈淋巴结肿大消退时间,监测外周血白细胞计数(white blood cells count,WBC)、血小板计数(platelet count,PLT)、C反应蛋白(creacting prorein,CRP)、血沉(erythrocyte sedimentation rate,ESR)和冠状动脉病变(coronary artery lesion,CAL)发生情况.结果 两组住院时间、退热时间、黏膜充血、手足肿胀和颈淋巴结肿大消退时间及总热程比较,差异无统计学意义(P>0.05);两组WBC、PLT、CRP、ESR与治疗前比较明显降低(P<0.05),两组间比较,差异无统计学意义(P> 0.05).观察组CAL发生率为25.5%,对照组CAL发生率为21.6%,两组比较,差异亦无统计学意义(P>0.05).结论 IVIG 1 g/kg单次静脉注射治疗KD,可有效缓解临床症状,降低CAL的发生率,且与IVIG 2g/kg单次静脉注射治疗KD比较治疗效果相似.%Objective To evaluate the clinical effect on different dosage of intravenous immune globulin (IVIG) in the treatment of Kawasaki disease and its influence on coronary artery lesion. Methods 102 cases of patients with Kawasaki disease in our hospital from April 2010 to October 2011 were randomly divided into the observation group and the control group, and each group had 51 cases. The children of observation group were treated with 1 g/kg of IVIG in intravenous injection, the children of control group were treated with 2 g/kg of IVIG in intravenous injection, the curative effect of two kinds of treatment methods were comparative studied. The total heat process

  18. [Therapy for atypical facial pain].

    Science.gov (United States)

    Ishida, Satoshi; Kimura, Hiroko

    2009-09-01

    Atypical facial pain is a pain in the head, neck and the face, without organic causes. It is treated at departments of physical medicine, such as dental, oral and maxillofacial surgery, otolaryngology, cerebral surgery, or head and neck surgery. In primary care, it is considered to be a medically unexplained symptom (MUS), or a somatoform disorder, such as somatization caused by a functional somatic syndrome (FSS) by psychiatrists. Usually, patients consult departments of physical medicine complaining of physical pain. Therefore physicians in these departments should examine the patients from the holistic perspective, and identify organic diseases. As atypical facial pain becomes chronic, other complications, including psychiatric complaints other than physical pain, such as depression may develop. Moreover, physical, psychological, and social factors affect the symptoms by interacting with one another. Therefore, in examining atypical facial pain, doctors specializing in dental, oral and maxillofacial medicine are required to provide psychosomatic treatment that is based on integrated knowledge.

  19. Atypical manifestations of leptospirosis.

    Science.gov (United States)

    Rajapakse, Senaka; Rodrigo, Chaturaka; Balaji, Krishan; Fernando, Sumadhya Deepika

    2015-05-01

    Leptospirosis is an illness with a wide spectrum of clinical manifestations and severe illness affects nearly all organ systems. Serious and potentially life-threatening clinical manifestations of acute leptospirosis are caused by both direct tissue invasion by spirochaetes and by the host immune responses. In its severe form, leptospirosis can cause multi-organ dysfunction and death in a matter of days. Therefore it is critical to suspect and recognize the disease early, in order to initiate timely treatment. While the classical presentation of the disease is easily recognized by experienced clinicians practising in endemic regions, rarer manifestations can be easily missed. In this systematic review, we summarize the atypical manifestations reported in literature in patients with confirmed leptospirosis. Awareness of these unusual manifestations would hopefully guide clinicians towards early diagnosis.

  20. Atypical case of Wilson's disease with psychotic onset, low 24 hour urine copper and the absence of Kayser-Fleischer rings

    Directory of Open Access Journals (Sweden)

    Krstić Dragan

    2014-01-01

    Full Text Available Introduction. Wilson's disease is typically manifested in two clinical forms, neurological and hepatic and in rare cases it starts with psychiatric symptoms exclusively. We presented a rare atypical case of Wilson's disease with psychotic onset. Case report. A 22-year-old male patient was initially presented with predominant signs and symptoms of psychiatric disorder and then later with the development of neurological signs and symptoms. Neuroimaging, detected metal deposits in central nervous system (CNS but not in peripheral organs, while serum analysis excluded pantothenate-kinase associated neurodegeneration (PKAN and aceruloplasminemia. In favor of the diagnosis of Wilson's disease there were reduced concentrations of copper and ceruloplasmin concentrations and metal deposits in CNS, but other pathognomonic signs and symptoms were absent: in-creased copper in urine, Kayser-Fleischer rings in Descemet’s corneal membrane and deposits of copper in liver. Introduction of penicillamine treatment resulted in improvement in mental and general health of the patient. Molecular genetic analysis definitely confirmed the diagnosis of Wilson's disease. Conclusion. Wilson's disease can remain undetected for a long period of time if masked with dominant or exclusive psychiatric symptoms. If clear clinical symptoms and signs, and unambiguous laboratory findings are not present, it is necessary to perform molecular genetic analysis to confirm the definitive diagnosis.

  1. Distinguishing Parkinson’s disease from atypical parkinsonian syndromes using PET data and a computer system based on support vector machines and Bayesian networks

    Directory of Open Access Journals (Sweden)

    Fermín eSegovia

    2015-11-01

    Full Text Available Differentiating between Parkinson's disease (PD and atypical parkinsonian syndromes (APS is still a challenge, specially at early stages when the patients show similar symptoms. During last years, several computer systems have been proposed in order to improve the diagnosis of PD, but their accuracy is still limited. In this work we demonstrate a full automatic computer system to assist the diagnosis of PD using 18F-DMFP PET data. First, a few regions of interest are selected by means of a two-sample t-test. The accuracy of the selected regions to separate PD from APS patients is then computed using a support vector machine classifier. The accuracy values are finally used to train a Bayesian network that can be used to predict the class of new unseen data. This methodology was evaluated using a database with 87 neuroimages, achieving accuracy rates over 78%. A fair comparison with other similar approaches is also provided.

  2. An Atypical Clinical Course of Anti-MDA5 Antibody-positive Interstitial Lung Disease in a Patient with Three Deteriorations in 9 years

    Science.gov (United States)

    Sato, Yuki; Otsuka, Kojiro; Tamai, Koji; Ono, Yuichiro; Hamaguchi, Yasuhito; Tomii, Keisuke

    2017-01-01

    Anti-MDA5 antibody-positive patients with clinically amyopathic dermatomyositis (CADM) are at high risk of developing rapidly progressive interstitial lung disease (ILD), which is associated with a high mortality rate. Approximately half of the patients with ILD recover; however, the long-term clinical course of these patients has not been fully reported and is not completely understood. This report describes the atypical clinical course of an anti-MDA5 antibody-positive CADM patient who experienced three deteriorations of ILD in 9 years. These findings indicate that the ILD in anti-MDA5 antibody-positive patients may not only be rapidly progressive, but may also be chronic and recurrent. PMID:28154280

  3. Absence of atypical haplotype and presence of Senegal haplotype sickle cell disease in African-descent population in the northern Brazil

    Directory of Open Access Journals (Sweden)

    Rafael E. Nascimento

    2015-04-01

    Full Text Available Introduction: Sickle cell anemia (SCA is the most severe form of sickle cell disease; it presents variants that are called haplotypes βS. There are five major haplotypes βS gene: Arab-Indian/Saudi, Senegal, Benin, Bantu, and Camaroon. Objective: Characterize the presence of haplotypes in patients with SCA in Amapá. Methods: 46 sample were studied, all samples were amplified and analyzed by polymerase chain reaction and restriction fragment length polymorphism (PCR-RFLP. Results: Bantu (61.2%, followed by Benin (26.5% and Senegal (12.2%. Conclusion: We identified three haplotypes characteristic of African ethnicity, with the presence of Senegal. In our study we found the presence of atypical haplotype, suggesting concentration and semi-isolation of the founding groups with little mixing.

  4. Transpupillary thermotherapy for atypical central serous chorioretinopathy

    Directory of Open Access Journals (Sweden)

    Kawamura R

    2012-01-01

    Full Text Available Ryosuke Kawamura1,2, Hidenao Ideta1, Hideyuki Hori1, Kenya Yuki2, Tsuyoshi Uno1, Tatsurou Tanabe1, Kazuo Tsubota2, Tsutomu Kawasaki11Ideta Eye Hospital, Kumamoto, Japan; 2Keio University, School of Medicine, Department of Ophthalmology, Tokyo, JapanBackground: Central serous chorioretinopathy (CSC has been traditionally treated with laser photocoagulation. We thought that transpupillary thermotherapy (TTT utilizing a lower temperature than that of conventional laser photocoagulation might minimize permanent retinal and choroidal damage. Studies suggest that undesirable effects on vision due to TTT are minimal even if it is applied to foveal and/or parafoveal lesions when TTT requires a larger irradiation spot. The aim of this study was to evaluate the efficacy of TTT in the management of atypical CSC.Methods: We defined atypical CSC as bullous retinal detachment with diffuse or several leakages, severe leakage with fibrin formation under serous retinal detachment, or leakage within a pigment epithelium detachment. Eight consecutive patients with atypical CSC underwent visual acuity testing, ophthalmic examination, color photography, fluorescein angiography, and optical coherence tomography to evaluate the results of transpupillary thermotherapy. Retreatment of atypical CSC was based on ophthalmic examination, optical coherence tomography, and fluorescein angiography. TTT was performed on the leaking spots shown in fluorescein angiography, with a power of 50–250 mW, spot size of 500–1200 µm, and exposure time of 13–60 seconds to minimize retinal damage.Results: In five of eight affected eyes, serous detachments completely resolved within 1 month after the initial TTT. One eye had persistent subretinal fluid and required a second TTT treatment. Two eyes showed no resolution of CSC and were treated by conventional photocoagulation. Initial best-corrected visual acuity (BCVA ranged from 20/600 to 20/20 (mean, 20/40; median, 20/30. Final BCVA

  5. α-Synuclein and anti-α-synuclein antibodies in Parkinson's disease, atypical Parkinson syndromes, REM sleep behavior disorder, and healthy controls.

    Directory of Open Access Journals (Sweden)

    Lynnae M Smith

    Full Text Available α-synuclein is thought to play a key role in Parkinson's disease (PD because it is the major protein in Lewy bodies, and because its gene mutations, duplication, and triplication are associated with early-onset PD. There are conflicting reports as to whether serum and plasma concentrations of α-synuclein and anti-α-synuclein antibodies differ between PD and control subjects. The objectives of this study were to compare the levels of α-synuclein and its antibodies between individuals with typical PD (n=14, atypical Parkinson syndromes (n=11, idiopathic rapid eye movement sleep behavior disorder (n=10, and healthy controls (n=9, to assess the strength of association between these serum proteins, and to determine group sizes needed for a high probability (80% power of detecting statistical significance for 25% or 50% differences between typical PD and control subjects for these measurements. Analysis of log-transformed data found no statistically significant differences between groups for either α-synuclein or its antibodies. The concentrations of these proteins were weakly correlated (Spearman rho=0.16. In subjects with typical PD and atypical Parkinson syndromes, anti-α-synuclein antibody levels above 1.5 µg/ml were detected only in subjects with no more than four years of clinical disease. Power analysis indicated that 236 and 73 samples per group would be required for an 80% probability that 25% and 50% differences, respectively, in mean α-synuclein levels between typical PD and control subjects would be statistically significant; for anti-α-synuclein antibodies, 283 and 87 samples per group would be required. Our findings are consistent with those previous studies which suggested that serum concentrations of α-synuclein and its antibodies are not significantly altered in PD.

  6. The relationship of age, blood pressure, serum cholesterol and smoking habits with the risk of typical and atypical coronary heart disease death in the European cohorts of the Seven Countries Study

    NARCIS (Netherlands)

    Menotti, A.; Lanti, M.; Nedeljkovic, S.; Nissinen, A.; Kafatos, A.; Kromhout, D.

    2006-01-01

    Objective: To explore whether "typical" coronary heart disease (CHD) such as fatal myocardial infarction and sudden death relate to major cardiovascular risk factors in the same way as the "atypical" CHD, such as fatal heart failure and chronic arrhythmias. Design and setting: Ten cohorts (6633 card

  7. Atypical presentation of atypical amyloid.

    Science.gov (United States)

    Holanda, Danniele G; Acharya, Veena K; Dogan, Ahmet; Racusen, Lorraine C; Atta, Mohamed G

    2011-01-01

    Amyloidosis is a group of diseases categorized by precipitation of a group of protein aggregates (amyloid) in tissues, including the kidney, and proteinuria is usually the commonest, though not exclusive, hallmark of clinical presentation. AL and AA are the most commonly recognized forms of amyloidosis involving the kidney, but other forms have been described. We present a case of renal amyloidosis due to a novel amyloidogenic protein, leucocyte cell-derived chemotaxin 2, without proteinuria at presentation or on subsequent follow-up.

  8. A recently isolated Lassa virus from Mali demonstrates atypical clinical disease manifestations and decreased virulence in cynomolgus macaques.

    Science.gov (United States)

    Safronetz, David; Strong, James E; Feldmann, Friederike; Haddock, Elaine; Sogoba, Nafomon; Brining, Douglas; Geisbert, Thomas W; Scott, Dana P; Feldmann, Heinz

    2013-04-15

    The virulence of Soromba-R, a Lassa virus strain recently isolated from southern Mali, was assessed in 2 animal models of Lassa fever: inbred strain 13 guinea pigs and cynomolgus macaques. In both models, the Malian isolate demonstrated tissue tropism and viral titers similar to those of historical Lassa virus isolates from Sierra Leone (Josiah) and Liberia (Z-132); however, the Soromba-R isolate was found to be less pathogenic, as determined by decreased mortality and prolonged time to euthanasia in macaques. Interestingly, in addition to the classic indicators of Lassa fever, Soromba-R infection presented with moderate to severe pulmonary manifestations in the macaque model. Analysis of host responses demonstrated increased immune activation in Soromba-R-infected macaques, particularly in neutrophil-activating or -potentiating proinflammatory cytokines or growth factors, including tumor necrosis factor α, macrophage inflammatory protein 1α, interleukin 1β, and granulocyte colony-stimulating factor, as well as interleukin 5, which may be responsible for the decreased lethality and uncharacteristic clinical presentation. These results suggest that the strain of Lassa virus circulating in Mali might be less pathogenic than strains circulating in the historical region of endemicity and may result in an atypical presentation for Lassa fever, which could complicate clinical diagnosis.

  9. 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

  10. 川崎病心型脂肪酸结合蛋白及血小板活化因子的变化及临床意义%Serum heart-type fatty acid-binding protein and platelet activating factor levels in Kawasaki disease and their clinical significance

    Institute of Scientific and Technical Information of China (English)

    洪泽; 孙兴珍

    2011-01-01

    46例川崎病患儿(观察组)根据超声心动图结果分为冠状动脉损伤(CAL)组19例和非冠状动脉损伤(NCAL)组27例,另选择正常健康者50例为对照组.检测所有对象的心型脂肪酸结合蛋白(h-FABP)、血小板活化因子(PAF)、肌钙蛋白Ⅰ及肌酸磷酸激酶同工酶等.结果显示,观察组h-FABP、肌钙蛋白Ⅰ水平和阳性率均显著高于对照组(P<0.05),两组间肌酸磷酸激酶同工酶水平和阳性率比较差异无统计学意义(P>0.05),观察组h-FABP阳性率显著高于肌钙蛋白Ⅰ和肌酸磷酸激酶同工酶阳性率(P<0.05),PAF水平及血小板、中性粒细胞计数显著高于对照组(P<0.05);CAL组h-FABP、PAF水平显著高于NCAL组(P<0.05).提示,h-FABP和PAF对预测川崎病患儿冠状动脉病变有重要的临床价值.%Forty six children with Kawasaki disease (observer group) were classified as coronary artery lesions (CAL) subgroup (19 cases) and non-coronary artery lesions (NCAL) subgroup (27 cases)according to echocardiography; 50 healthy children served as control group.Serum heart-type fatty acidbinding protein (h-FABP), platelet activating factor (PAF), cardiac tropnin Ⅰ (cTnI) and creatine kinase isoenzyme MB (CK-MB) levels of all subjects were detected.The results showed that the h-FABP and cTnI levels and positive rate in observer group were higher those in control group (P < 0.05 ), while there were no differences in CK-MB level and positive rate between two groups ( P > 0.05 ).The h-FABP positive rate in observation group was higher than the positive rates of cTnI and CK-MB (P <0.05).The PAF level, PLT and polymorphonuclear neutrophil count in observer group were higher than those in control group ( P <0.05).The h-FABP and PAF levels in CAL subgroup were higher than those in NCAL subgroup (P <0.05).The results suggest that serum h-FABP and PAF can be used as diagnostic indicators for Kawasaki disease complicated with coronary artery lesions.

  11. 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.

  12. Atypical presentation of CLIPPERS syndrome: a new entity in the differential diagnosis of central nervous system rheumatologic diseases.

    Science.gov (United States)

    Gul, Maryam; Chaudhry, Ammar A; Chaudhry, Abbas A; Sheikh, Mubashir A; Carsons, Steven

    2015-04-01

    Numerous autoimmune diseases can affect the central nervous system (CNS), and variable clinical presentations confound the differential diagnosis. The challenging task of properly characterizing various CNS autoimmune diseases enables patients to be rapidly triaged and appropriately treated. In this review article, we aim to explore different CNS manifestations of rheumatologic diseases with emphasis on the utility of imaging and cerebrospinal fluid findings. We review the classic physical examination findings, characteristic imaging features, cerebrospinal fluid results, and serum biomarkers. In addition, we also present a unique case of newly described autoimmune entity CLIPPERS syndrome. Our case is unique in that this is the first case which demonstrates involvement of the supratentorial perivascular spaces in addition to the classic infratentorial involvement as initially described by Pittock et al (Brain. 2010;133:2626-2634).

  13. A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia

    Directory of Open Access Journals (Sweden)

    Francesco Rapisarda

    2015-01-01

    Full Text Available The IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4. Idiopathic CD4 lymphocytopenia is a heterogenic and rare syndrome characterized by the detection of a persistent absolute CD4 T cells count <300 cells/mm3 (or <20% of total T cells in more than one occasion and no evidence of HIV infection in absence of immunodeficiency or therapy associated with depressed levels of CD4 T cells. We report the case of a 50-year-old man with a multiorgan IgG4-related disease presenting in a temporal association with a profound and symptomatic idiopathic CD4 lymphocytopenia. Both clinical pictures improved after steroid treatment. Idiopathic CD4 lymphocytopenia has been associated with a number of autoimmune conditions but, to the best of our knowledge, this is the first case in which an association with the IgG4-related disease is reported.

  14. Atypical case of Wolfram syndrome revealed through targeted exome sequencing in a patient with suspected mitochondrial disease

    Directory of Open Access Journals (Sweden)

    Lieber Daniel S

    2012-01-01

    Full Text Available Abstract Background Mitochondrial diseases comprise a diverse set of clinical disorders that affect multiple organ systems with varying severity and age of onset. Due to their clinical and genetic heterogeneity, these diseases are difficult to diagnose. We have developed a targeted exome sequencing approach to improve our ability to properly diagnose mitochondrial diseases and apply it here to an individual patient. Our method targets mitochondrial DNA (mtDNA and the exons of 1,600 nuclear genes involved in mitochondrial biology or Mendelian disorders with multi-system phenotypes, thereby allowing for simultaneous evaluation of multiple disease loci. Case Presentation Targeted exome sequencing was performed on a patient initially suspected to have a mitochondrial disorder. The patient presented with diabetes mellitus, diffuse brain atrophy, autonomic neuropathy, optic nerve atrophy, and a severe amnestic syndrome. Further work-up revealed multiple heteroplasmic mtDNA deletions as well as profound thiamine deficiency without a clear nutritional cause. Targeted exome sequencing revealed a homozygous c.1672C > T (p.R558C missense mutation in exon 8 of WFS1 that has previously been reported in a patient with Wolfram syndrome. Conclusion This case demonstrates how clinical application of next-generation sequencing technology can enhance the diagnosis of patients suspected to have rare genetic disorders. Furthermore, the finding of unexplained thiamine deficiency in a patient with Wolfram syndrome suggests a potential link between WFS1 biology and thiamine metabolism that has implications for the clinical management of Wolfram syndrome patients.

  15. A Case Report of an Atypical Presentation of IgG4-Related Disease and Idiopathic CD4 Lymphocytopenia.

    Science.gov (United States)

    Rapisarda, Francesco; Zanoli, Luca; Portale, Grazia; Scuto, Salvo; Castellino, Pietro

    2015-01-01

    The IgG4-related disease is a fibroinflammatory disease characterized by tumefactive lesions, a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and, often but not always, elevated serum levels of IgG4. Idiopathic CD4 lymphocytopenia is a heterogenic and rare syndrome characterized by the detection of a persistent absolute CD4 T cells count <300 cells/mm(3) (or <20% of total T cells) in more than one occasion and no evidence of HIV infection in absence of immunodeficiency or therapy associated with depressed levels of CD4 T cells. We report the case of a 50-year-old man with a multiorgan IgG4-related disease presenting in a temporal association with a profound and symptomatic idiopathic CD4 lymphocytopenia. Both clinical pictures improved after steroid treatment. Idiopathic CD4 lymphocytopenia has been associated with a number of autoimmune conditions but, to the best of our knowledge, this is the first case in which an association with the IgG4-related disease is reported.

  16. Gendered Uncertainty and Variation in Physicians' Decisions for Coronary Heart Disease: The Double-Edged Sword of "Atypical Symptoms"

    Science.gov (United States)

    Welch, Lisa C.; Lutfey, Karen E.; Gerstenberger, Eric; Grace, Matthew

    2012-01-01

    Nonmedical factors and diagnostic certainty contribute to variation in clinical decision making, but the process by which this occurs remains unclear. We examine how physicians' interpretations of patient sex-gender affect diagnostic certainty and, in turn, decision making for coronary heart disease. Data are from a factorial experiment of 256…

  17. PAPP2C Interacts with the Atypical Disease Resistance Protein RPW8.2 and Negatively Regulates Salicylic Acid-Dependent Defense Responses in Arabidopsis

    Institute of Scientific and Technical Information of China (English)

    Wen-Ming Wang; Xian-Feng Ma; Yi Zhang; Ming-Cheng Luo; Guo-Liang Wang; Maria Bellizzi; Xing-Yao Xiong; Shun-Yuan Xiao

    2012-01-01

    Many fungal and oomycete pathogens differentiate a feeding structure named the haustorium to extract nutrition from the plant epidermal cell.The atypical resistance (R) protein RPW8.2 activates salicylic acid (SA)-dependent,haustorium-targeted defenses against Golovinomyces spp.,the causal agents of powdery mildew diseases on multiple plant species.How RPW8.2 activates defense remains uncharacterized.Here,we report that RPW8.2 interacts with the phytochrome-associated protein phosphatase type 2C (PAPP2C) in yeast and in planta as evidenced by coimmunoprecipitation and bimolecular fluorescence complementation assays.Down-regulation of PAPP2C by RNA interference (RNAi) in Col-0 plants lacking RPW8.2 leads to leaf spontaneous cell death and enhanced disease resistance to powdery mildew via the SA-dependent signaling pathway.Moreover,down-regulation of PAPP2C by RNAi in the RPW8.2 background results in strong HR-like cell death,which correlates with elevated RPW8.2 expression.We further demonstrate that hemagglutinin (HA)-tagged PAPP2C prepared from tobacco leaf cells transiently transformed with HA-PAPP2C possesses phosphatase activity.In addition,silencing a rice gene (Os04g0452000) homologous to PAPP2C also results in spontaneous cell death in rice.Combined,our results suggest that RPW8.2 is functionally connected with PAPP2C and that PAPP2C negatively regulates SA-dependent basal defense against powdery mildew in Arabidopsis.

  18. Atypical charles bonnet syndrome.

    Science.gov (United States)

    Arun, Priti; Jain, Rajan; Tripathi, Vaibhav

    2013-10-01

    Charles Bonnet syndrome (CBS) is not uncommon disorder. It may not present with all typical symptoms and intact insight. Here, a case of atypical CBS is reported where antipsychotics were not effective. Patient improved completely after restoration of vision.

  19. ATYPICAL ANTIPSYCHOTICS FROM SCRATCH TO THE PRESENT

    Directory of Open Access Journals (Sweden)

    Ashish Chauhan*, Amit Mittal, Pradeep Kumar Arora

    2013-01-01

    Full Text Available Mental illness constitutes the second-largest disease burden in the United States. Psychosis is one of the most common and severe mental illnesses. It is an extremely devastating condition characterised by delusions, hallucinations, distortion of thoughts and deteriorating social functioning experiences. Psychosis in all human societies has approximately same incidence of occurrence as in accordance to “anthropo-parity principle.” It has large economic impact on various aspects of cognition, health, and quality of life which has devastated effects on its sufferers and facing them large economic burden. Psychosis (Schizophrenia is associated with an imbalance of the dopaminergic system, entailing hyper-stimulation of dopamine function in the brain, particularly in the mesolimbic pathway. Consequences of antipsychotic treatment are far reaching and expensive. Detrimental extrapyramidal side effects associated with conventional antipsychotics and non-compliance among patients limits long term treatment with conventional antipsychotics. It gives rise to a new class, atypical antipsychotics owning low propensity to cause EPS, efficacy against refractory cases and better control over negative symptoms, better tolerance and compliance along with lower relapse rate and safer adverse effect profile. Atypical antipsychotics have revolutionized the treatment of psychosis, now being the treatment of choice for patients with psychosis. The positive therapeutic experience with the atypical antipsychotics in the treatment of psychosis and their favourable effects outweighs their unfavourable adverse effects. Though atypical antipsychotics are widely prescribed in the treatment of schizophrenia, however not a single atypical antipsychotic drug having any exceptional efficacy and safety profile. Thus, there is still a lot of research needed to be carried out in the development of novel atypical antipsychotics. This review is comprehensive appraisal about

  20. NOVEL ATYPICAL ANTIPSYCHOTIC AGENTS

    Directory of Open Access Journals (Sweden)

    Vijay Vinay

    2011-05-01

    Full Text Available Antipsychotics are a group of drugs commonly but not exclusively used to treat psychosis. Antipsychotic agents are grouped in two categories: Typical and Atypical antipsychotics. The first antipsychotic was chlorpromazine, which was developed as a surgical anesthetic. The first atypical anti-psychotic medication, clozapine, was discovered in the 1950s, and introduced in clinical practice in the 1970s. Both typical and atypical antipsychotics are effective in reducing positive and negative symptoms of schizophrenia. Blockade of D2 receptor in mesolimbic pathway is responsible for antipsychotic action. Typical antipsychotics are not particularly selective and also block Dopamine receptors in the mesocortical pathway, tuberoinfundibular pathway, and the nigrostriatal pathway. Blocking D2 receptors in these other pathways is thought to produce some of the unwanted side effects. Atypical antipsychotics differ from typical psychotics in their "limbic-specific" dopamine type 2 (D2-receptor binding and high ratio of serotonin type 2 (5-HT2-receptor binding to D2. Atypical antipsychotics are associated with a decreased capacity to cause EPSs, TD, narcoleptic malignant syndrome, and hyperprolactinemia. Atypical antipsychotic agents were developed in response to problems with typical agents, including lack of efficacy in some patients, lack of improvement in negative symptoms, and troublesome adverse effects, especially extrapyramidal symptoms (EPSs and tardive dyskinesia (TD.

  1. Study on the risk factors for coronary artery lesions in children with Kawasaki disease in Lanzhou%兰州地区川崎病患儿并发冠状动脉损害的危险因素研究

    Institute of Scientific and Technical Information of China (English)

    朱琳; 牛少敏; 董湘玉; 杨轶男; 倪倩

    2014-01-01

    目的:探讨兰州地区川崎病(KD)患儿并发冠状动脉损害(CAL)的危险因素。方法对确诊的174例KD患儿,根据其是否并发CAL分为CAL组和NCAL组,分析比较其年龄、性别、热程、静脉用丙种球蛋白(IVIG)开始使用时间、IVIG使用剂量、C反应蛋白(CRP)、血清白蛋白、红细胞沉降率(ESR)、血小板(PLT)、血红蛋白等资料的差异。结果174例KD患儿并发CAL者46例(占26.44%),无并发CAL者128例;两组患儿平均热程、自发热到开始IVIG治疗的时间、IVIG使用剂量,以及PLT、CRP、ESR、红细胞计数(RBC)的差异均有统计学意义(P10 d、发热10 d后开始使用IVIG,PLT、CRP、ESR升高,RBC降低的KD患儿应警惕并发CAL的危险性,对于KD的治疗及预后判断有一定的参考价值。%Objective To investigate the risk factors for coronary artery lesions (CALs) in children with Kawasaki disease (KD) in Lanzhou. Methods One hundred and seventy-four children with diagnosed KD were divided into CAL group and non-CAL group based on the existence of concurrent CALs. The age, gender, fever duration, intravenous immunoglobulin (IVIG) start time, IVIG dose, C-reactive protein (CRP), serum albumin, erythrocyte sedimentation rate (ESR), platelet (PLT), red blood cell count (RBC), hemoglobin and so on were compared. Results Among the 174 children, 46 children (26.44%) were complicated by CALs and 128 children were not. The differences of average fever duration, IVIG starting time, IVIG dose, PLT, CRP, ESR and RBC were statistically signiifcant (P10 d, start of IVIG af-ter 10 days of fever, increase of PLT, CRP and ESR and decrease of RBC, clinicians should be alert to the risk of concurrent CAL.

  2. Molecular analysis and associated pathology of beak and feather disease virus isolated in Italy from young Congo African grey parrots (Psittacus erithacus) with an "atypical peracute form" of the disease.

    Science.gov (United States)

    Robino, Patrizia; Grego, Elena; Rossi, Giacomo; Bert, Elena; Tramuta, Clara; Stella, Maria Cristina; Bertoni, Pierfrancesco; Nebbia, Patrizia

    2014-01-01

    This study is the first report on the genetic and pathogenic characterization of beak and feather disease virus (BFDV) occurring in Italy. Twenty BFDV strains isolated in Italy from juvenile Congo African grey parrots (Psittacus erithacus) were investigated. Seventeen strains showed an "atypical peracute form" (aPF) of the disease, and three a chronic form (CF). The birds with aPF had been weaned, were independent as far as food and protection were concerned and apparently were without lesions. The gene coding for the putative coat protein was amplified in all isolates while the BFDV genome was sequenced completely in 10 samples, eight of them belonging to aPF affected birds and two from CF of the disease. All full genomes clustered into the J strain of BFDV, where two new subtypes were identified. Recombination analyses showed evidence of genetic exchanges in two BFDV genomes. In addition, a correlation between viral isolate and origin of the breeding material was shown, while an association between the genetic features of the virus and the clinical form was not observed. Histologically, apoptosis was detected frequently in aPF samples and sporadically in CF samples. Interestingly, BFDV antigens were detected in the nuclei and cytoplasm of such apoptotic cells. The data presented here support the hypothesis that, in the absence of a defined BFDV genetic variant accountable for a specific clinical form of psittacine beak and feather disease, differences in the apoptotic rate between aPF and CF are strictly host related.

  3. Diagnosis and Treatment of Atypical Newcastle Disease Coinfected with E.coli in Red-crested Crane%丹顶鹤非典型新城疫与大肠杆菌混合感染的诊治

    Institute of Scientific and Technical Information of China (English)

    李静姬; 李福军; 梁玉嬉

    2013-01-01

    Avian Newcastle disease had high incidence and mortality rate in poultry. The disease was mainly prevented with immune vaccination. Newcastle disease was controlled, but atypical Newcastle was occurred at the same time. The disease was epidemical in Red-rested crane in Jilin city zoo, atypical Newcastle disease infection mixed with E. coli was diagnosed. The epidemic was controlled by specific therapy of high immune yolk antibody and antibiotic therapy.%新城疫(Newcastle disease,ND)是危害禽类的一种接触性致死性传染病,OIE将其列为A类疫病.各养殖户对该病高度重视,主要采用全程免疫接种的方式加以预防,使急性新城疫得到有效控制,但随之而来的是,非典型新城疫发病率有所提高.吉林市某公园观赏动物丹顶鹤发生疫情,经临床剖检及实验室诊断确诊为非典型新城疫(CND)与大肠杆菌(E.col)混合感染,经高免卵黄抗体特异性疗法配合抗菌治疗及有效的消毒防疫措施,使疫情得到控制.

  4. ITPKC基因功能性SNP rs28493229与中国人群川崎病的相关性研究%Association study of a functional SNP rs28493229 of ITPKC gene and Kawasaki disease in a Chinese population

    Institute of Scientific and Technical Information of China (English)

    彭茜; 张渝; 陈昌辉; 吴青; 李波; 廖静; 罗彩丹; 胡小平; 郑植; 何海兰

    2011-01-01

    Objective Kawasaki disease (KD) is a form of acute multi-systemic vasculitis with unknown etiology.It is the leading cause of acquired heart disease in children due to the frequent occurrence of coronary artery lesions (CALs). Recently,a C allele of rs28493229 (G/C) in inositol 1,4,5-trisphosphate 3-kinase C (ITPKC) gene was found to significantly increase the risk for KD/CALs in Japanese population. It is important to confirm such finding in Chinese population to enable prognosis and personalized therapy for KD.Methods A case-control study was performed.The patient group has included 206 unrelated patients with KD,and the control group included 285 age,gender and ethnically matched children who never had KD. Genotyping of rs28493229 was performed using polymerase chain reactionrestriction fragment length polymorphism(PCR-RFLP) and DNA sequencing.The allele,genotype and C allele carrier frequencies were compared between the two groups,patients with or without CALs,and patients who were resistant or responsive to (intravenous immunoglobulin,IVIG) treatment. Results Frequency of the C allele of rs28493229 was significantly lower in both groups than that in the Japanese population (P<0.01).No significant difference was detected between the two groups in terms of allele,genotype and C carrier of rs28493229 frequencies.Such frequencies were also similar between patients with or without CALs,resistant or responsive to IVIG treatment.Conclusion Our study has failed to prove any association between rs28493229 and KD/CALs in Chinese patients,which indicated that the C allele of rs28493229 may not be used as a molecular marker for determining KD susceptibility,prognosis and effect of treatment.The much lower frequency of C allele does not support its significance in the occurrence of KD/CALs in Chinese population.It is still necessary to find functional SNPs in ITPKC gene which is associated with KD/CALs in Chinese population.%目的 分析中国人群中1,4,5

  5. Atypical meningococcal meningitis with rashless presentation:A case report

    Institute of Scientific and Technical Information of China (English)

    Sunita; Singh Manpreet; Kapoor Dheeraj

    2012-01-01

    Meningococcal disease is the major health problem in developing world. The clinical presentation is varied, ranging from transient fever and bacteraemia to fulminant disease with death ensuing within hours of the onset of clinical symptoms. The classical clinical manifestations of meningococcal disease have been well described, but atypical presentations if unrecognized, may lead to a delay in treatment and fatal outcome. We here report a case presented with atypical presentation of meningococcal meningitis without classical rash, which was diagnosed and managed successfully.

  6. Atypical Optic Neuritis.

    Science.gov (United States)

    Gaier, Eric D; Boudreault, Katherine; Rizzo, Joseph F; Falardeau, Julie; Cestari, Dean M

    2015-12-01

    Classic demyelinative optic neuritis is associated with multiple sclerosis and typically carries a good prognosis for visual recovery. This disorder is well characterized with respect to its presentation and clinical features by baseline data obtained through the optic neuritis treatment trial and numerous other studies. Atypical optic neuritis entails clinical manifestations that deviate from this classic pattern of features. Clinical signs and symptoms that deviate from the typical presentation should prompt consideration of less common etiologies. Atypical features to consider include lack of pain, simultaneous or near-simultaneous onset, lack of response to or relapse upon tapering from corticosteroids, or optic nerve head or peripapillary hemorrhages. The most important alternative etiologies to consider and the steps towards their respective diagnostic evaluations are suggested for these atypical features.

  7. Atypical Pyoderma Gangrenosum Mimicking an Infectious Process

    Directory of Open Access Journals (Sweden)

    Derek To

    2014-01-01

    Full Text Available We present a patient with atypical pyoderma gangrenosum (APG, which involved the patient’s arm and hand. Hemorrhagic bullae and progressive ulcerations were initially thought to be secondary to an infectious process, but a biopsy revealed PG. Awareness of APG by infectious disease services may prevent unnecessary use of broad-spectrum antibiotics.

  8. Atypical pyoderma gangrenosum mimicking an infectious process.

    Science.gov (United States)

    To, Derek; Wong, Aaron; Montessori, Valentina

    2014-01-01

    We present a patient with atypical pyoderma gangrenosum (APG), which involved the patient's arm and hand. Hemorrhagic bullae and progressive ulcerations were initially thought to be secondary to an infectious process, but a biopsy revealed PG. Awareness of APG by infectious disease services may prevent unnecessary use of broad-spectrum antibiotics.

  9. Causes and control of atypical newcastle disease in free-range chicken%放养土鸡非典型新城疫的病因分析及防控对策

    Institute of Scientific and Technical Information of China (English)

    高婷; 周五朵; 吴异健

    2015-01-01

    低发病率、低死亡率、高淘汰率、散发性的非典型新城疫是放养土鸡的主要疫病之一,其流行病学、临床症状、剖检病变等不同于传统典型新城疫,易被误诊,给土鸡养殖业造成巨大的经济损失。文中对放养土鸡非典型新城疫的流行特点、成因及防控措施等进行概述,以期为土鸡养殖业者提供参考。%The atypical Newcastle disease is one of the main diseases in free-range chickens with low morbidity and mortality and high attrition rate, and has caused huge economic losses in free-range chicken production. It is usually sporadic and different from typical Newcastle disease in epidemiology, clinical symptoms and pathological lesions. Here we summarized epidemic characteristics, causes, and control measures of atypical Newcastle disease to provide references for free-range chicken breeders.

  10. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

    Wallner-Blazek, Mirja; Rovira, Alex; Fillipp, Massimo;

    2013-01-01

    Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can be class...

  11. Atypical charles bonnet syndrome

    Directory of Open Access Journals (Sweden)

    Priti Arun

    2013-01-01

    Full Text Available Charles Bonnet syndrome (CBS is not uncommon disorder. It may not present with all typical symptoms and intact insight. Here, a case of atypical CBS is reported where antipsychotics were not effective. Patient improved completely after restoration of vision.

  12. Study on the involvement of microtubule-associated protein l light chain 3B in the inlfammatory response of Kawa-saki disease%微管相关蛋白LC3-II参与川崎病炎症反应的研究

    Institute of Scientific and Technical Information of China (English)

    冯思琪; 高放; 易岂建

    2015-01-01

    ObjectiveTo investigate the relationship between microtubule-associated protein l light chain 3B (LC3-II) and the inlfammatory response of Kawasaki disease (KD).MethodsThirty-nine cases of acute KD before intravenous admin-istration of immunoglobulin were enrolled. According to the results of echocardiography, the 39 cases were furtherly divided into KD with coronary artery lesion (CAL, 20 cases) group and KD with non-CAL (NCAL, 19 cases) group. At the same time, 12 healthy children were selected as controls. Serum samples were collected and cultured in vitro by human coronary artery endothe-lial cells (HCAEC) for 12 h. The LC3-II protein and mRNA expression of HCAEC were detected by Western-blotting and Q-PCR respectively.ResultsThe LC3-II protein and mRNA expression in CAL group and NCAL group were signiifcantly higher than those in control group, the LC3-II protein and mRNA expression in CAL group was higher than those in NCAL group, and both differences were statistically signiifcant (P<0.05).ConclusionsAutophagy may be involved in the inlfammatory response of KD in acute phase, which may be related to endothelial cell lesions of coronary artery in children with KD.%目的:探讨微管相关蛋白LC3-II与川崎病(KD)炎症反应的关系。方法选入39例急性期静脉注射丙种球蛋白前的KD患儿,根据心脏彩超结果,20例KD伴冠状动脉损伤(CAL)组,19例不伴冠状动脉损伤(NCAL)组;同时以12例正常儿童作为对照组。采集血清标本,加入人冠状动脉内皮细胞(HCAEC)体外培养体系干预12 h。采用Western-blotting、Q-PCR分别测定HCAEC中LC3-II蛋白及mRNA的表达。结果干预12 h后,CAL组、NCAL组的LC3-II蛋白及mRNA表达均较对照组明显升高,且CAL组更高于NCAL组,差异均有统计学意义(P<0.05)。结论自噬参与了急性期KD的炎症反应,可能与KD患儿冠状动脉内皮细胞损害有关。

  13. 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.

  14. Clinical Significance of ENA-78 and Visfatin and Their Association with Kawasaki Disease%川崎病患儿血清中性粒细胞激活肽-78和内脂素水平变化及其意义研究

    Institute of Scientific and Technical Information of China (English)

    邓灵芝; 周永勤; 黄友敏

    2011-01-01

    目的 探讨川崎病患儿血清中性粒细胞激活肽-78(ENA-78)和内脂素水平的变化及临床意义.方法 采用酶联免疫法检测90例川崎病患儿和30例健康体检儿童的血清ENA-78和内脂素水平.结果 川崎病组患儿血清中ENA-78水平[(163±22) ng/L]明显高于对照组[(64±12) ng/L],内脂素水平[(32±5) μg/L]也明显高于对照组[(17±4) μg/L],差异均有统计学意义(P<0.05).川崎病组中冠状动脉损伤患儿血清ENA-78水平[(217±30) ng/L]明显高于冠状动脉正常患儿[(129±20) ng/L],内脂素水平[(37±5) μg/L]也明显高于冠状动脉正常患儿[(28±5) μg/L],差异均有统计学意义(P<0.05).川崎病组患儿血清ENA-78水平与内脂素水平呈正相关(r=0.67,P<0.05).结论 ENA-78和内脂素水平在川崎病尤其伴冠状动脉损伤时明显升高,可作为判断川崎病冠状动脉损伤的参考指标.%Objective To explore the levels and clinical significance of epithelial neutrophil-activing peptide- 78 ( ENA -78 ), visfatin in the serum of children with Kawasaki diseases ( KD ) . Methods Serum level of ENA -78 and visfatin was measured in 90 children with KD and 30 healthy children by ELISA, and the outcome was estimated in combination with clinical symptoms. Results The serum levels of ENA -78 and visfatin in children with KD were significantly higher compared with that of the control group ( P < 0. 05 ); The serum levels of ENA - 78 and visfatin in children of KD with coronary artery lesion ( CAL ) were significantly higher compared with that of children of KD without CAL ( P < 0. 05 ). In children with KD, the serum levels of ENA -78 and visfatin had a positive correlation ( r = 0. 67, P < 0. 05 ). Conclusion Serum levels of ENA - 78 and visfatin are increased in the acute phase of KD, and are significantly higher in those KD children with CAL. ENA - 78 and visfatin may be used as an important serological indicator of KD with CAL.

  15. Changes of Plasma MMP-9,NT-proBNP Levels in Children with Kawasaki Disease and Its Clinic Significance%川崎病患儿血浆MMP-9、NT-proBNP水平变化及其临床意义

    Institute of Scientific and Technical Information of China (English)

    吴镇宇; 姚丽萍

    2016-01-01

    Objective:To explore the changes and clinic significance of plasma matrix metalloproteinase-9(MMP-9) and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels in children with Kawasaki disease(KD).Method:Forty two children with KD in our hospital from January 2015 to January 2016 were collected and divided into two groups according to the results of echocardiography,the coronary artery lesion(CAL) group(n=18) and the non-coronary artery lesion(NCAL) group (n=24).Thirty febrile children with respiratory tract infection and twenty healthy children in the same period were chosen as the control group.The plasma MMP-9 and NT-proBNP protein levels were measured respectively.Result:The plasma MMP-9 and NT-proBNP levels in the acute phase of KD patients were all higher than those in the two control group(P<0.01),and which were significantly higher in the CAL group than in the NCAL group,the differences were statistically significant(P<0.05 ).The plasma MMP-9 and NT-proBNP levels in remission stages were siginificantly lower after treatment,and the protein level of NT-proBNP in the CAL group was still higher than that of the NCAL group,the differences were statistically significant(P<0.05).There was a positive correlation between plasma MMP-9 and NT-proBNP levels in the acute phase of KD(r=0.57,P<0.05).Conclusion:The plasma MMP-9 and NT-proBNP levels are closely related to KD cardiovascular damage,combined detection of them may have critical value for the early diagnosis of KD and the prediction of CAL.%目的:探讨基质金属蛋白酶-9(MMP-9)与N末端脑利钠肽(NT-proBNP)在川崎病(KD)患儿血浆中的变化及其意义。方法:选取2015年1月-2016年1月本院明确诊断的KD患儿42例为研究对象,根据心脏彩超分为冠状动脉损伤(CAL)组18例、无冠状动脉损伤(NCAL)组24例,另选取同期伴有发热的呼吸道感染患儿30例和门诊健康体检儿童20例作为对照组,分别测定各组MMP-9、NT-proBNP蛋白水平

  16. Clinical Diagnosis of the Diseases with Atypical Parkinsonian Disorders%非典型帕金森病相关疾病的临床诊断思路

    Institute of Scientific and Technical Information of China (English)

    胡智伟; 王浩; 邹小东; 王百辰; 呙登俊

    2013-01-01

    目的 探讨非典型帕金森病(APD)相关疾病的临床诊断思路.方法 通过回顾性分析笔者医院2006~2011年收治的48例APD相关疾病患者的临床资料包括病史、体格检查、临床表现、实验室检查、影像学检查、认知功能评估、左旋多巴(L-dopa)试验结果,复习文献,总结分析临床资料特征,并依据APD相关疾病的诊断标准作出临床诊断.结果 48例患者中,肌张力增高42例、震颤40例、运动迟缓39例、姿势反射异常32例、步态异常29例、直立性低血压13例、腱反射亢进11例、认知功能障碍11例、垂直性凝视麻痹和球麻痹各10例、共济失调和病理征阳性各8例、角膜K-F环7例、视幻觉5例、一侧肢体忽略并失用3例;肝功能异常9例、血小板减少8例、血清铜蓝蛋白降低7例、凝血功能异常5例;头颅CT:基底核区低密度影19例,脑室扩大17例,脑萎缩14例;头颅MRI:基底节区异常信号30例,脑室扩大20例,弥漫性脑萎缩18例,脑干和小脑萎缩15例,海马萎缩7例,双侧苍白球T2高信号4例.壳核“裂隙征”8例,脑桥“十字征”7例,中脑“蜂鸟征”6例;L-dopa试验反应不良46例;蒙特利尔认知评估量表(MoCA)总分<26分23例.出院诊断:多系统萎缩(MSA)18例、进行性核上性麻痹(PSP)10例、Wilson病(WD)7例、路易体痴呆(DLB)6例、伴帕金森综合征(PS)的阿尔茨海默病(AD)5例和皮质基底核变性(CBD)2例.结论 APD相关疾病间,虽然临床表现有许多相似之处,但临床特征、诊断标准及治疗转归各不相同,正确区分十分必要.%Objective To investigate the ideas of the clinical diagnosis of the diseases with atypical parkinsonian disorders(APD).Methods The clinical data of 48 patients with APD admitted to our hospital during 2006 to 2011 were analyzed retrospectively,including medical history,physical examinations,clinical manifestations,laboratory tests,imagings,cognitive assessment tests

  17. Atypical Presentation of Atypical Teratoid Rhabdoid Tumor in a Child

    Directory of Open Access Journals (Sweden)

    Y. T. Udaka

    2013-01-01

    Full Text Available Atypical Teratoid Rhabdoid Tumor (ATRT is a rare malignant intracranial neoplasm more commonly diagnosed in young children. The authors report the case of an 11-year-old boy with a long standing history of slowly progressive weight loss, fatigue, and weakness over 1.5 years whose magnetic resonance imaging revealed a large heterogeneous enhancing dorsally exophytic lower brainstem mass. Examination revealed extreme cachexia, gaze-evoked nystagmus, dysphagia, dysarthria, bilateral dysmetria, and global weakness without ambulation. The protracted history and neuroimaging features were most suggestive of a low grade glioma. However, pathology revealed a hypercellular tumor with large hyperchromatic nucleoli and loss of INI-1 staining on immunohistochemistry consistent with a diagnosis of an ATRT. The child died shortly after surgery due to complications from his brainstem infiltrative disease. This case illustrates the diverse presentation of ATRT in childhood that can clinically and radiographically mimic that of low grade glioma.

  18. Psychiatric syndromes associated with atypical chest pain

    Directory of Open Access Journals (Sweden)

    Nikolić Gordana

    2010-01-01

    Full Text Available Background/Aim. Chest pain often indicates coronary disease, but in 25% of patients there is no evidence of ischemic heart disease using standard diagnostic tests. Beside that, cardiologic examinations are repeated several times for months. If other medical causes could not be found, there is a possibility that chest pain is a symptom of psychiatric disorder. The aim of this study was to determine the presence of psychiatric syndromes, increased somatization, anxiety, stress life events exposure and characteristic of chest pain expression in persons with atypical chest pain and coronary patients, as well as to define predictive parameters for atypical chest pain. Method. We compared 30 patients with atypical chest pain (E group to 30 coronary patients (K group, after cardiological and psychiatric evaluation. We have applied: Mini International Neuropsychiatric Interview (MINI, The Symptom Checklist 90-R (SCL-90 R, Beck Anxiety Inventory (BAI, Holms-Rahe Scale of stress life events (H-R, Questionnaire for pain expression Pain-O-Meter (POM. Significant differences between groups and predictive value of the parameters for atypical chest pain were determined. Results. The E group participants compared to the group K were younger (33.4 ± 5.4 : 48.3 ± 6,4 years, p < 0.001, had a moderate anxiety level (20.4 ± 11.9 : 9.6 ± 3.8, p < 0.001, panic and somatiform disorders were present in the half of the E group, as well as eleveted somatization score (SOM ≥ 63 -50% : 10%, p < 0.01 and a higher H-R score level (102.0 ± 52.2 : 46.5 ± 55.0, p < 0.001. Pain was mild, accompanied with panic. The half of the E group subjects had somatoform and panic disorders. Conclusion. Somatoform and panic disorders are associated with atypical chest pain. Pain expression is mild, accompained with panic. Predictive factors for atypical chest pain are: age under 40, anxiety level > 20, somatization ≥ 63, presence of panic and somatoform disorders, H-R score > 102

  19. Association of Sterile Pyuria and Coronary Artery Aneurysm in Kawasaki Syndrome

    Directory of Open Access Journals (Sweden)

    Mohsen Akhavan Sepahi

    2011-09-01

    Full Text Available "nKawasaki disease (KD is an inflammatory multiorgan disease of unknown etiology. The most dramatic organ involved is the heart. There were a few studies about cardiac involvement and sterile pyuria. This study guides to determine if sterile pyuria is associated with coronary artery aneurysm (CAA in KD patients and to consider it as a predicting factor for coronary artery involvement. Forty seven patients with KD were studied by echocardiography in admission and one month later. Urine analysis, complete blood count, erythrocyte sedimentation rate and C-reactive protein were measured in admission. Data were analyzed using SPSS-14 software. Patients' age was ranged from 13 month to 7 years old (mean age of 3.43 ± 1.54 years. Thirty patients (63.8% were male and 17 patients (36.1% were female. Cardiac involvement was detected in 32 patients (68% using echocardiography, of which CAA was reported in 8 cases (17%. Six of CAA (75% were in association with sterile pyuria, although it was statistically insignificant (P>0.05. Although the majority of patients with CAA had sterile pyuria, this association is not statistically significant, thus it couldn't be considered as a predicting factor for CAA.

  20. Intracranial Tuberculoma Presenting as Atypical Eclampsia: A Case Report

    Science.gov (United States)

    Murugesan, Sharmila; Pradeep, Sunitha; John, Lopamudra; Kolluru, Vasavi

    2016-01-01

    Occurrence of eclampsia before 20 weeks of pregnancy and after 48 hours of delivery in the absence of typical signs of hypertension and or proteinuria is termed as atypical eclampsia. Atypical or non-classic eclampsia will have some symptoms of eclampsia but without the usual proteinuria or hypertension. All patients with atypical onset should undergo neurological evaluation to rule out neurologic causes of seizures. Cerebral tuberculosis is a rare and serious form of disease secondary to haematogenous spread of Mycobacterium tuberculosis. Here we present a case of cerebral tuberculoma with seizures in late pregnancy mimicking eclampsia. PMID:27504359

  1. [Atypical presentation of preeclampsia].

    Science.gov (United States)

    Ditisheim, A; Boulvain, M; Irion, O; Pechère-Bertschi, A

    2015-09-09

    Preeclampsia is a pregnancy-related syndrome, which still represents one of the major causes of maternal-fetal mortality and morbidity. Diagnosis can be made difficult due to the complexity of the disorder and its wide spectrum of clinical manifestations. In order to provide an efficient diagnostic tool to the clinician, medical societies regularly rethink the definition criteria. However, there are still clinical presentations of preeclampsia that escape the frame of the definition. The present review will address atypical forms of preeclampsia, such as preeclampsia without proteinuria, normotensive preeclampsia, preeclampsia before 20 weeks of gestation and post-partum preeclampsia.

  2. Meta-analysis of the risk factors for coronary artery lesion secondary to Kawasaki disease in Chinese children%中国川崎病患儿并发冠状动脉病变高危因素的Meta分析

    Institute of Scientific and Technical Information of China (English)

    赵丽丽; 王一彪; 索琳

    2011-01-01

    Objective Coronary artery lesion (CAL)is a serious complication of Kawasak disease (KD).Whether there is CAL and the severity arc the most critical factors of the prognosis of KD.The incidence of KD is currently increasing year by year.KD has replaced rheumatic fever as the main entity of acquired heart disease of children.This study aimed to identify risk factors of CAL secondary to KD and take early interventions to prevent CAL or reduce its incidence.MethOd Literature search was performed at Chinese Academic Literature Main Database,Chinese Science and Technology Periodical Database,Wanfang Periodicals and Dissertation Database.and the Chinese Biomedical Literature Database comprehensively,besides,retrospective retrieval and manual retrieval were also performed from the domestic public actions and the dissertations dating from January,2000 to December, 2009.RayMan 4.2 provided by Cochrane was used for meta analysis.Fixed or random model was selected according to the results of heterogeneity test.Sensitivity analysis was done according to the different results.The publication bias was evaluated by funnel plots.Odds ratio(OR)and 95% confidence interval(CI)were estimated in the dissertation.Result Twenty studies were confirmed to be eligible.A11 the 20 studies were retrospective.OR and 95%CI of the risk factors were as follows:age≤1 year,OR=1.58,and 95%CI(1.23,2.04),P=0.0004;male gender, OR=1.48,95%CI(1.29,1.71),P<0.000 01;WBC>20×109/L,OR=1.73,95% CI(1.32,2.26),P<0.000l;C-reactive protein(CRP)>100 mg/L,OR=2.37,95%CI(1.49,3.77),P=0.0003:fever duration>10 d,OR=3.23,95%CI(2.08,5.02),P<0.000 01;use of intravenous gamma globulin(IVIG)>10 d,OR=2.50,95%CI(1.98,3.16),P<0.000 01.Conclusion The high risk factors for coronary artery lesion secondary to Kawasaki disease are age≤1 year,male,WBC>20×109/L,CRP>100 mg/L,fever duration>10 d,and use of intravenous gamma globulin(IVIG)>10 d.%目的 探讨中国川崎病患儿并发冠状动

  3. An Atypical Case of Pityriasis Rosea Gigantea after Influenza Vaccination

    Directory of Open Access Journals (Sweden)

    Dimitrios Papakostas

    2014-04-01

    Full Text Available Pityriasis rosea is a common erythematosquamous eruption, typically presenting along the cleavage lines of the skin. A wide spectrum of atypical manifestations may challenge even the most experienced physician. Here we report a rare case of a suberythrodermic pityriasis rosea with gigantic plaques after an influenza vaccination, and we discuss the possible triggers of atypical manifestations of such a common dermatological disease in the setting of an altered immunity.

  4. Evaluation of seasonal patterns of Kawasaki Syndrome- and rotavirus-associated hospitalizations in California and New York, 2000-2005

    Directory of Open Access Journals (Sweden)

    Parashar Umesh D

    2009-10-01

    Full Text Available Abstract Background Kawasaki Syndrome (KS is an uncommon childhood disease with unknown etiology. It has been suggested that rotavirus infection may play a causative role in the development of KS. Methods To examine potential temporal associations between KS and rotavirus infection, seasonal patterns of KS- and rotavirus-associated hospitalizations among children in California and New York during 2000-2005 were compared. Results Rotavirus hospital admissions were markedly winter seasonal, with very few summer hospitalizations. KS hospitalizations occurred year-round but also peaked slightly during winter and spring. Conclusion The strong winter seasonal pattern of rotavirus clearly differed from the year-round pattern of KS hospitalizations. While the present study cannot completely rule out rotavirus as having a role in the development of KS, other agents must be involved in the etiology of KS.

  5. Atypical depression in the structure of organic mental disorders (literature review

    Directory of Open Access Journals (Sweden)

    Leonov S.F.

    2014-09-01

    Full Text Available The review of literature presents current data on cli¬nical picture and diagnostics of atypical depression. Rubric “atypical depression” includes a variety of depressive states characterized by reactively caused changes of mood, sensitivity to interpersonal contacts, inverted vegetative and somatic symptoms such as increased appetite and hypersomnia. The article considers the place of atypical depression in the structure of organic mental disorders. Positions of foreign authors that produce atypical depression as a clinical entity in the structure of Bipolar affective disorder II type are represented, the views of other authors on the structure of atypical depression are considered. The analysis of national concept of non-circular depression is carried out. Questions of atypical affective conditions acquire special significance due to preparation of International Classification of Diseases of the 11th revision, because inclusion in it of Bipolar affective disorder II type, a manifestation of which is considered to be atypical depressions, is under discussion.

  6. 320排动态容积CT冠状动脉成像在川崎病冠状动脉瘤远期随访中应用%Application of 320-detector row dynamic volume CT angiography in follow up of coronary artery aneurysms caused by Kawasaki disease

    Institute of Scientific and Technical Information of China (English)

    刘俊; 于明华; 许开元; 向峰; 周乾潮

    2015-01-01

    目的:探讨320排动态容积CT冠状动脉成像(CTA)及经胸超声心动图(TTE)对川崎病(KD)合并冠状动脉瘤(CAA)中远期随访的应用价值。方法采用320排CTA、TTE进行中远期追踪随访8例KD合并CAA患儿。结果8例患儿平均发病年龄(41.63±22.70)月,随访时间(43.50±10.99)月。急性期TTE诊断冠状动脉巨大瘤(GCAA)3例,中小瘤5例,共累及冠状动脉16/32支(50.0%)。随访观察终点时TTE示GCAA 3例及中小瘤2例仍存在,另3例小瘤消退,仍累及冠状动脉6/32支(18.6%)。随访观察终点时320排CTA检查示累及冠状动脉7/32支(21.9%),分布部位与TTE基本一致;另发现左回旋支扩张1例,右冠状动脉(RCA)远段GCAA 1处、血栓2处、狭窄1处、钙化2处。结论KD引起的CAA可长期存在, GCAA后期可发生冠状动脉血栓、狭窄或钙化。TTE观察近中段冠脉改变敏感可靠,但对于中远段冠脉损害的观察有局限性。320排CTA能较全面地观察各支冠状动脉的病变,尤其对急性期后冠状动脉血栓,钙化及近、远端狭窄的观察敏感可靠。%ObjectiveTo assess the values of 320-detector row dynamic volume CT angiography (CTA) and transthoracic echocardiography (TTE) in follow up of coronary artery aneurysm (CAA) caused by Kawasaki disease (KD).Methods320-de-tector row CTA and TTE were applied in long-term follow-up of 8 patients with CAA caused by KD.ResultsIn 8 patients, the mean age at onset was 41.63±22.70 months and the mean follow up time was 43.50±10.99 months. In acute phase, 3 cases of giant coronary artery aneurysms (GCAA) and 5 cases of mid-small CAA were diagnosed by TTE. A total of 16/32 arteries (50%) were involved. At the end of follow-up, 3 cases of GCAA and 2 cases of mid-small CAA were still diagnosed by TTE, and small CAAs were regressed in another 3 cases. A total of 6/32 arteries (18.75%) were involved. Simultaneously at the end of follow-up, a total of 7/32 arteries (21

  7. C反应蛋白在静脉丙种球蛋白不敏感川崎病患儿中的临床意义%C-reactive protein level in children with Kawasaki disease unresponsive to initial intravenous immunoglobulin therapy

    Institute of Scientific and Technical Information of China (English)

    李伙德; 苏海浩

    2013-01-01

    Objective To analyze the serum C reactive protein (CRP) level in children with Kawasaki disease (KD) during the treatment with intravenous immunoglobulin (IVIG).Methods The study objects were collected from January 2009 to December 2012 in the two women and children hospitals.All the patients received IVIG of 2 g/kg daily.The children afebrile in 48 hours after initial administration of IVIG were defined as group A.The febrile children were given second IVIG.Patients who were afebrile in 48 hours after second IVIG were defined as group B.The rest was assigned to group C.CRP levels were detected before initial IVIG and 48 hours after initial IVIG in all the children.Then the data were analyzed statistically.Results 146 children were enrolled and 84 were male and 62 were female.Group A had 110 children with 8 developing CALs (7.3%),group B had 26 children with 5 CALs (19.2%),group C had 10 with 7 CALs (70%).There was significant difference in CRP level 48 hours after initial IVIG between the CALs and the negative children but no difference before initial IVIG.There were no significant differences in both CRP level and CALs between each group pairs before initial IVIG.48 hours after administration of initial IVIG,there were significant differences in both CRP level and CALs among groups A,B,and C.It showed that there was correlation between CRP level and the development of CALs.Conclusions Patients unresponsive to IVIG are liable to develop CALs.Patients with high CRP level 48 hours after initial IVIG are liable to develop to CALs as well.The patients who are the non-responder with high CRP are likely to fail in the additional IVIG treatment.These patients may need other powerful anti-inflammation therapy earlier.%目的 分析川崎病患儿血清C反应蛋白(CRP)在静脉丙种球蛋白(IVIG)治疗过程中的变化,对血清CRP变化水平预测IVIG治疗不敏感患儿的疗效进行评价.方法 收集2009年1月至2012年12月在我院与广东省妇幼

  8. Expression of MMP-9, AECA and ANCA in peripheral blood of patients with Kawasaki disease and its relationship with coronary artery lesions%MMP-9、AECA、ANCA 蛋白在川崎病外周血的表达及其与冠状动脉损害的关系

    Institute of Scientific and Technical Information of China (English)

    王晓华; 王倩; 赵建美

    2016-01-01

    Objective To investigate the potential role and clinical significance of matrix metalloproteinase 9 ( MMP-9), anti-endothelial cell antibodies (AECA) and anti-neutrophil cytoplasmic antibodies (ANCA) in vasculitis and coro-nary artery lesions ( CAL) of patients with Kawasaki disease ( KD) .Methods Forty-two children with KD were divided into two groups:CAL group (n=16) and non-coronary artery lesion (NCAL) group (n=26).Twenty febrile children and 15 children for selective operation were chosen as the control group.Serum levels of MMP-9, AECA and ANCA were meas-ured by enzyme-linked immunosorbent assay ( ELISA) , fluorescence quantitative-PCR and gelatin zymography were respec-tively used to detect MMP-9 mRNA expression and enzyme activity.Results The MMP-9 mRNA expression, protein level and enzymatic activity in the acute phase of KD patients were all higher than those in the two control groups ( all P<0.01), and they were significantly higher in the CAL group than in the NCAL group (all P<0.05).Moreover, MMP-9 mRNA expression, enzymatic activity and protein level were decreased significantly in the remission phase (all P<0.01). Serum AECA and ANCA protein levels of KD patients in the acute phase were significantly higher as compared with those of the two control groups (all P<0.01).The protein level of ANCA in the CAL group was significantly higher than that of the NCAL group (P<0.01).There was a positive correlation between serum MMP-9 and AECA protein in the acute phase of KD (r=0.77, P<0.01).The serum MMP-9 and ANCA protein levels of the CAL group in the acute phase were both positively correlated with LCA/AAO ratio (r=0.57, P<0.05;r=0.88, P<0.01).Conclusions MMP-9, AECA and ANCA are potential pathological factors in the formation of KD vasculitis and CAL.The serum level detection has a certain value for early prediction of CAL.%目的:探讨基质金属蛋白酶-9(MMP-9)、抗内皮细胞抗体(AECA)和抗中性粒细胞胞

  9. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome.

    NARCIS (Netherlands)

    Legendre, C.M.; Licht, C.; Muus, P.; Greenbaum, L.A.; Babu, S.; Bedrosian, C.; Bingham, C.; Cohen, D.J.; Delmas, Y.; Douglas, K.; Eitner, F.; Feldkamp, T.; Fouque, D.; Furman, R.R.; Gaber, O.; Herthelius, M.; Hourmant, M.; Karpman, D.; Lebranchu, Y.; Mariat, C.; Menne, J.; Moulin, B.; Nurnberger, J.; Ogawa, M.; Remuzzi, G.; Richard, T.; Sberro-Soussan, R.; Severino, B.; Sheerin, N.S.; Trivelli, A.; Zimmerhackl, L.B.; Goodship, T.; Loirat, C.

    2013-01-01

    BACKGROUND: Atypical hemolytic-uremic syndrome is a genetic, life-threatening, chronic disease of complement-mediated thrombotic microangiopathy. Plasma exchange or infusion may transiently maintain normal levels of hematologic measures but does not treat the underlying systemic disease. METHODS: We

  10. A Generalized Kawasaki-Type Dynamics with Spin-Pair Redistribution Mechanism

    Institute of Scientific and Technical Information of China (English)

    朱建阳; 朱涵

    2002-01-01

    We generalize Kawasaki's dynamics, spin-pair exchange mechanism, to a spin-pair redistribution mechanism,and we present a normalized redistribution probability. As an application, we treat the one-dimensional kinetic Gaussian model and obtain the exact diffusion equation and the temperature-dependent diffusion coefficient. We find that the diffusion process can slow down infinitely near the critical point and obtain the critical dynamic exponent z = 2 that is independent of the assumed mechanism, either Glauber-type or Kawasaki-type.

  11. Atypical presentations of neuromyelitis optica

    Directory of Open Access Journals (Sweden)

    Douglas Sato

    2011-10-01

    Full Text Available Neuromyelitis optica (NMO is an inflammatory disease of central nervous system classically characterized by acute, severe episodes of optic neuritis and longitudinally extensive transverse myelitis, usually with a relapsing course. The identification of an autoantibody exclusively detected in NMO patients against aquaporin-4 (AQP-4 has allowed identification of cases beyond the classical phenotype. Brain lesions, once thought as infrequent, can be observed in NMO patients, but lesions have different characteristics from the ones seen in multiple sclerosis. Additionally, some AQP-4 antibody positive patients may present with a variety of symptoms not being restricted to optic neuritis and acute myelitis during the first attack or in a relapse. Examples are not limited to, but may include patients only with brain and/or brainstem lesions, narcolepsy with hypothalamic lesions or patients with intractable hiccups, nausea and vomiting. The prompt identification of NMO patients with atypical presentations may benefit these patients with institution of early treatment to reduce disability and prevent further attacks.

  12. Inherited complement regulatory protein deficiency predisposes to human disease in acute injury and chronic inflammatory statesthe examples of vascular damage in atypical hemolytic uremic syndrome and debris accumulation in age-related macular degeneration.

    Science.gov (United States)

    Richards, Anna; Kavanagh, David; Atkinson, John P

    2007-01-01

    In this chapter, we examine the role of complement regulatory activity in atypical hemolytic uremic syndrome (aHUS) and age-related macular degeneration (AMD). These diseases are representative of two distinct types of complement-mediated injury, one being acute and self-limited, the other reflecting accumulation of chronic damage. Neither condition was previously thought to have a pathologic relationship to the immune system. However, alterations in complement regulatory protein genes have now been identified as major predisposing factors for the development of both diseases. In aHUS, heterozygous mutations leading to haploinsufficiency and function-altering polymorphisms in complement regulators have been identified, while in AMD, polymorphic haplotypes in complement genes are associated with development of disease. The basic premise is that a loss of function in a plasma or membrane inhibitor of the alternative complement pathway allows for excessive activation of complement on the endothelium of the kidney in aHUS and on retinal debris in AMD. These associations have much to teach us about the host's innate immune response to acute injury and to chronic debris deposition. We all experience cellular injury and, if we live long enough, will deposit debris in blood vessel walls (atherosclerosis leading to heart attacks and strokes), the brain (amyloid proteins leading to Alzheimer's disease), and retina (lipofuscin pigments leading to AMD). These are three common causes of morbidity and mortality in the developed world. The clinical, genetic, and immunopathologic understandings derived from the two examples of aHUS and AMD may illustrate what to anticipate in related conditions. They highlight how a powerful recognition and effector system, the alternative complement pathway, reacts to altered self. A response to acute injury or chronic debris accumulation must be appropriately balanced. In either case, too much activation or too little regulation promotes

  13. Atypical Odontalgia (Phantom Tooth Pain)

    Science.gov (United States)

    ... atypical facial pain, phantom tooth pain, or neuropathic orofacial pain, is characterized by chronic pain in a ... such as a specialist in oral medicine or orofacial pain. The information contained in this monograph is ...

  14. Occurrence and significance of atypical Aeromonas salmonicida in non-salmonid and salmonid fish species : A review

    DEFF Research Database (Denmark)

    Wiklund, T.; Dalsgaard, Inger

    1998-01-01

    information is available about the ecology, spread and survival of atypical strains in water. The commonly used therapeutic methods for the control of diseases in farmed fish caused by atypical A. salmonicida are generally effective against the atypical strains. Resistance to different antibiotics......, non-salmonids as well as salmonids, inhabiting fresh water, brackish water and marine environments in northern and central Europe, South Africa, North America, Japan and Australia. In non-salmonid fish species, infections with atypical strains often manifest themselves as superficial skin ulcerations....... The best known diseases associated with atypical A. salmonicida are carp Cyprinus carpio erythrodermatitis, goldfish Carassius auratus ulcer disease, and ulcer disease of flounder Platichthys flesus, but atypical strains are apparently involved in more disease outbreaks than previously suspected...

  15. Atypical Light Curves

    CERN Document Server

    Steenwyk, Steven D; Molnar, Lawrence A

    2013-01-01

    We have identified some two-hundred new variable stars in a systematic study of a data archive obtained with the Calvin-Rehoboth observatory. Of these, we present five close binaries showing behaviors presumably due to star spots or other magnetic activity. For context, we first present