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Sample records for acute kawasaki disease

  1. Kawasaki Disease

    ... Content Article Body What is Kawasaki disease? Kawasaki disease is a serious and perplexing disease, the cause of which is ... influenza) with aspirin has been linked with a serious disease called Reye syndrome. Always consult your pediatrician before ...

  2. Kawasaki Disease Mimicking Retropharyngeal Abscess

    Srividhya, Vazhkudai Sridharan; Vasanthi, Thiruvengadam; Shivbalan, Somu

    2010-01-01

    Kawasaki disease is an acute, self-limiting febrile mucocutaneous vasculitis of infants and young children. Retropharyngeal lymphadenopathy is a rare presentation of Kawasaki disease. We present a case of Kawasaki disease mimicking a retropharyngeal abscess, with upper airway obstruction resulting in delayed diagnosis. PMID:20635457

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

    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.

  4. Acute myocardial ischemia in adults secondary to missed Kawasaki disease in childhood

    Rizk, SRY; El Said, G; Daniels, LB; Burns, JC; El Said, H; Sorour, KA; Gharib, S; Gordon, JB

    2015-01-01

    © 2015 Elsevier Inc. All rights reserved. Coronary artery aneurysms that occur in 25% of untreated Kawasaki disease (KD) patients may remain clinically silent for decades and then thrombose resulting in myocardial infarction. Although KD is now the most common cause of acquired heart disease in children in Asia, the United States, and Western Europe, the incidence of KD in Egypt is unknown. We tested the hypothesis that young adults in Egypt presenting with acute myocardial ischemia may have ...

  5. Living with Kawasaki Disease

    ... all children, not just those who have Kawasaki disease). Children treated with immune globulin should wait 11 months before having measles and chicken pox vaccines. Immune globulin can prevent these vaccines ...

  6. Seven-year national survey of Kawasaki disease and acute rheumatic fever.

    Taubert, K A; Rowley, A H; Shulman, S T

    1994-08-01

    To assess the frequency of hospital encoded diagnoses of acute rheumatic fever (ARF) and Kawasaki disease (KD), the two leading causes of acquired heart disease in children in the United States, we performed a survey of the medical record departments of United States children's hospitals and of general hospitals that have at least 400 beds and a pediatric ward. With a simple questionnaire, data were gathered for the years 1984 through 1990 by ICD.9CM codes, with a 58% response rate. About 8000 diagnoses of KD and 6000 diagnoses of ARF were encoded during the study period. Encoded diagnoses of both KD and ARF showed yearly fluctuations in the earlier years (1984 through 1987). For KD there was a general trend toward increasing numbers after 1986. These data are consistent with increased physician awareness and diagnosis of KD. For ARF a gradual decline was observed between 1986 and 1990. About 80% of ARF diagnoses were reported from general hospitals. The much smaller pool of encoded diagnoses of ARF at the children's hospitals showed a 56% increase from 1985 to 1986. These data suggest that the highly publicized increase in cases of acute rheumatic fever in the United States during the mid-1980s may reflect focal rather than nationwide increased activity and that nationally the number of diagnoses of ARF actually may have continued to decline gradually from 1984 through 1990. PMID:7970970

  7. Acute myocardial ischemia in adults secondary to missed Kawasaki disease in childhood.

    Rizk, Sherif R Y; El Said, Galal; Daniels, Lori B; Burns, Jane C; El Said, Howaida; Sorour, Khaled A; Gharib, Soliman; Gordon, John B

    2015-02-15

    Coronary artery aneurysms that occur in 25% of untreated Kawasaki disease (KD) patients may remain clinically silent for decades and then thrombose resulting in myocardial infarction. Although KD is now the most common cause of acquired heart disease in children in Asia, the United States, and Western Europe, the incidence of KD in Egypt is unknown. We tested the hypothesis that young adults in Egypt presenting with acute myocardial ischemia may have coronary artery lesions because of KD in childhood. We reviewed a total of 580 angiograms of patients ≤40 years presenting with symptoms of myocardial ischemia. Coronary artery aneurysms were noted in 46 patients (7.9%), of whom 9 presented with myocardial infarction. The likelihood of antecedent KD as the cause of the aneurysms was classified as definite (n = 10), probable (n = 29), or equivocal (n = 7). Compared with the definite and probable groups, the equivocal group had more traditional cardiovascular risk factors, smaller sized aneurysms, and fewer coronary arteries affected. In conclusion, in a major metropolitan center in Egypt, 6.7% of adults aged ≤40 years who underwent angiography for evaluation of possible myocardial ischemia had lesions consistent with antecedent KD. Because of the unique therapeutic challenges associated with these lesions, adult cardiologists should be aware that coronary artery aneurysms in young adults may be because of missed KD in childhood. PMID:25555655

  8. Pulmonary Artery Dilation and Right Ventricular Function in Acute Kawasaki Disease.

    Numano, Fujito; Shimizu, Chisato; Tremoulet, Adriana H; Dyar, Dan; Burns, Jane C; Printz, Beth F

    2016-03-01

    Coronary artery inflammation and aneurysm formation are the most common complications of Kawasaki disease (KD). Valvulitis and myocarditis are also well described and may lead to valvar regurgitation and left ventricular dysfunction. However, functional changes in the right heart have rarely been reported. We noted several acute KD patients with dilated pulmonary arteries (PA) and thus sought to systematically characterize PA size and right-heart function in an unselected cohort of KD patients cared for at a single clinical center. Clinical, laboratory, and echocardiographic data from 143 acute KD subjects were analyzed. PA dilation was documented in 23 subjects (16.1 %); these subjects had higher median right ventricle myocardial performance index (RV MPI), higher ratio of early tricuspid inflow velocity to tricuspid annular early diastolic velocity (TV E/e'), and lower median TV e' velocity compared to the non-PA dilation group (0.50 vs 0.38 p < 0.01, 4.2 vs 3.6 p < 0.05, and 13.5 vs 15.2 cm/s p < 0.01, respectively). Almost all subjects with PA dilation had improved PA Z-score, RV MPI, and TV E/e' in the subacute phase (p < 0.01). There were no significant differences in indices of left ventricle function between PA dilation group and non-PA dilation group. In summary, PA dilation was documented in 16 % of acute KD subjects. These subjects were more likely to have echocardiographic indices consistent with isolated RV dysfunction that improved in the subacute phase. The long-term consequence of these findings will require longitudinal studies of this patient population. PMID:26681305

  9. How Is Kawasaki Disease Treated?

    ... all children, not just those who have Kawasaki disease.) Children who have had immune globulin should wait 11 months before having the measles and chicken pox vaccines. Immune globulin can prevent those vaccines ...

  10. Kawasaki disease with Glucose-6-Phosphate Dehydrogenase deficiency, case report

    Obeidat, Hesham Radi; Al-Dossary, Sahar; Asseri, Abdulsalam

    2014-01-01

    Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and children younger than 5 years of age. Coronary artery abnormalities are the most serious complication.

  11. Erythema Marginatum as an Unusual Presentation for Kawasaki Disease

    I. Sedighi; H. Bazmamoun; A. Amanati

    2008-01-01

    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 Repo...

  12. Kawasaki disease following Rocky Mountain spotted fever: a case report

    Bal Aswine K; Kairys Steven W

    2009-01-01

    Abstract Introduction Kawasaki disease is an idiopathic acute systemic vasculitis of childhood. Although it simulates the clinical features of many infectious diseases, an infectious etiology has not been established. This is the first reported case of Kawasaki disease following Rocky Mountain spotted fever. Case presentation We report the case of a 4-year-old girl who presented with fever and petechial rash. Serology confirmed Rocky Mountain spotted fever. While being treated with intravenou...

  13. Chest X-ray findings in the acute phase of Kawasaki disease

    We reviewed the chest X-ray (CXR) findings and clinical courses of 129 patients with Kawasaki disease and found abnormal CXR findings in 14.7% of the patients. Reticulogranular pattern was the most frequent abnormality (89.5%), while peribronchial cuffing (21.1%), pleural effusion (15.8%), atelectasis (10.5%) and air trapping (5.3%) were also seen. In each of these patients, CXR abnormalities appeared within 10 days after the onset of illness. In the group with abnormal CXR findings, a statistically significant increase was noted in duration of fever, incidence of adventitious sounds, serum CPR levels and incidence of coronary arterial lesions and pericardial effusion, as compared with the group having normal CXR findings. The pathological basis of these CXR changes is not clear, since no biopsy or autopsy specimen was obtained from these patients. Since none of these patients showed definite heart failure, it is difficult to consider that abnormal CXR findings were due to heart failure. On the other hand, physical signs and previous pathological reports suggested that the causes of abnormal CXR findings were lower respiratory tract inflammation and/or pulmonary arteritis. (orig.)

  14. Kawasaki disease following Rocky Mountain spotted fever: a case report

    Bal Aswine K

    2009-07-01

    Full Text Available Abstract Introduction Kawasaki disease is an idiopathic acute systemic vasculitis of childhood. Although it simulates the clinical features of many infectious diseases, an infectious etiology has not been established. This is the first reported case of Kawasaki disease following Rocky Mountain spotted fever. Case presentation We report the case of a 4-year-old girl who presented with fever and petechial rash. Serology confirmed Rocky Mountain spotted fever. While being treated with intravenous doxycycline, she developed swelling of her hands and feet. She had the clinical features of Kawasaki disease which resolved after therapy with intravenous immune globulin (IVIG and aspirin. Conclusion This case report suggests that Kawasaki disease can occur concurrently or immediately after a rickettsial illness such as Rocky Mountain spotted fever, hypothesizing an antigen-driven immune response to a rickettsial antigen.

  15. Myocardial ischemia in Kawasaki disease

    The detection of myocardial ischemia is essential for evaluation of patients with Kawasaki disease, especially who have had coronary artery lesions. To evaluate the clinical efficacy of Tl-201 single photon emission computed tomography (SPECT) after dipyridamole infusion (maximum dose 0.70 mg/kg) for detecting myocardial ischemia, 44 patients with Kawasaki disease aged 7.7±4.8 years at the study and 10 age matched controls were observed. In the Kawasaki disease group, significant coronary artery stenosis was observed in 14, coronary aneurysm without stenosis in 18, the regression of the coronary aneurysms in 2 and without coronary lesions in 10 patients. In 24 of 44 patients, treadmill exercise stress test was also performed at the same period. Myocardial ischemic changes were observed in 11 patients, all combined with significant coronary artery stenosis. The sensitivity of SPECT for detection of overall coronary stenosis was 79%, coronary that of treadmill exercise test was only 33% (p<0.001). Furthermore, among the patients having significant coronary stenosis, the severity score was significantly elevated in patients who had electrocardiographic abnormal Q wave compared to those without abnormal Q wave (51.0±38.8 versus 20.0±12.1, p<0.05). These data suggest that the pharmacological stress scintigraphy using dipyridamole injection provides not only the accurate detection but quantitative evaluation of myocardial ischemia in these patients. This noninvasive technique may become one of the most useful index for detection and follow-up of myocardial ischemia in Kawasaki disease. (author)

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

    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. PMID:26897142

  17. Primary Treatment of Kawasaki Disease with Corticosteroids

    Singhal, Swati; Mishra, Devendra; Kurien, Sumod; Juneja, Monica

    2014-01-01

    Kawasaki disease (KD), is the leading cause of acquired heart disease in children in the developed world. The use of aspirin and intravenous immunoglobulin as the initial therapy in KD is the standard of care, as they reduce the incidence of coronary artery aneurysms, the major cardiac morbidity from this disease. The place of corticosteroids in the initial therapy is; however, controversial. We describe the course of a one-year-old child with Kawasaki disease who was treated with aspirin and...

  18. Segmented Coronary Artery Aneurysms and Kawasaki Disease

    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.

  19. Myocardial infarction in a 35-day-old infant with incomplete Kawasaki disease and chicken pox.

    Kossiva, Lydia; Papadopoulos, Marios; Lagona, Evangelia; Papadopoulos, George; Athanassaki, Corina

    2010-10-01

    Kawasaki disease is an acute febrile vasculitis of infancy and early childhood. It is uncommon in early infancy, because a significant proportion of these children do not meet the classical diagnostic criteria at this age. Infants younger than 6 months with persistent fever and some of the criteria of Kawasaki disease should always raise suspicion for Kawasaki disease early to avoid delayed diagnosis with severe cardiac complications. We present a 35-day-old infant with incomplete Kawasaki disease complicated with myocardial infarction during chicken pox. PMID:20633312

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

    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. PMID:27399018

  1. Kawasaki disease: review of 21 cases

    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

  2. Vascular health late after Kawasaki disease: implications for accelerated atherosclerosis

    Cheung, Yiu-Fai

    2014-01-01

    Kawasaki disease (KD), an acute vasculitis that primarily affects young children, is the most common acquired paediatric cardiovascular disease in developed countries. While sequelae of arterial inflammation in the acute phase of KD are well documented, its late effects on vascular health are increasingly unveiled. Late vascular dysfunction is characterized by structural alterations and functional impairment in term of arterial stiffening and endothelial dysfunction and shown to involve both ...

  3. Sensorineural hearing loss in Kawasaki disease.

    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. PMID:27011703

  4. Cardiospect (CS) in Pediatrics. Kawasaki Disease (KD)

    The first publication of the EK was in the year 1967, by T. Kawasaki, referring to 50 cases and establishing clinical diagnostic criteria of the disease, including cardiac involvement. In 1968 T. Yamamoto published a series of cases of EK, in which 48% had electrocardiographic abnormalities, establishing that the cardiac involvement was a common occurrence in this disease. The international incidence is between 1 and 5 per 100 000 and mortality between 0.1% and 5% difference related to the study and better recognition and hence early and appropriate treatment in countries where EK is more frequent. Mortality is higher in men and in children under one year old. The cardiac manifestations are one of the most important EK: approximately 15-25% of untreated children develop coronary abnormalities, acute myocardial infarction and myocarditis. Studies of CS can be used to assess myocardial ischemia or viability within the context of stress with dipyridamole or rest study in patients with acute symptoms or follow-up post revascularization. Thus it follows a forecast or defining complications. We present the cases of three patients with KD seen in the past year at our institution who had related heart disease and who underwent CS studies. It describes the technical findings and local acquisition, processing and diagnostic and clinical relevance. (author)

  5. A diagnostic algorithm combining clinical and molecular data distinguishes Kawasaki disease from other febrile illnesses

    Ling Xuefeng B

    2011-12-01

    Full Text Available Abstract Background Kawasaki disease is an acute vasculitis of infants and young children that is recognized through a constellation of clinical signs that can mimic other benign conditions of childhood. The etiology remains unknown and there is no specific laboratory-based test to identify patients with Kawasaki disease. Treatment to prevent the complication of coronary artery aneurysms is most effective if administered early in the course of the illness. We sought to develop a diagnostic algorithm to help clinicians distinguish Kawasaki disease patients from febrile controls to allow timely initiation of treatment. Methods Urine peptidome profiling and whole blood cell type-specific gene expression analyses were integrated with clinical multivariate analysis to improve differentiation of Kawasaki disease subjects from febrile controls. Results Comparative analyses of multidimensional protein identification using 23 pooled Kawasaki disease and 23 pooled febrile control urine peptide samples revealed 139 candidate markers, of which 13 were confirmed (area under the receiver operating characteristic curve (ROC AUC 0.919 in an independent cohort of 30 Kawasaki disease and 30 febrile control urine peptidomes. Cell type-specific analysis of microarrays (csSAM on 26 Kawasaki disease and 13 febrile control whole blood samples revealed a 32-lymphocyte-specific-gene panel (ROC AUC 0.969. The integration of the urine/blood based biomarker panels and a multivariate analysis of 7 clinical parameters (ROC AUC 0.803 effectively stratified 441 Kawasaki disease and 342 febrile control subjects to diagnose Kawasaki disease. Conclusions A hybrid approach using a multi-step diagnostic algorithm integrating both clinical and molecular findings was successful in differentiating children with acute Kawasaki disease from febrile controls.

  6. Kawasaki disease with Glucose-6-Phosphate Dehydrogenase deficiency, case report.

    Obeidat, Hesham Radi; Al-Dossary, Sahar; Asseri, Abdulsalam

    2015-09-01

    Kawasaki disease (KD) is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and children younger than 5 years of age. Coronary artery abnormalities are the most serious complication. Based on the literatures infusion of Intravenous Immunoglobulin of 2 g/kg and a high dose of oral aspirin up to 100 mg/kg/day are the standard treatment for Kawasaki disease in the acute stage, and should be followed by antiplatelet dose of aspirin for thrombocytosis. Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is an inherited X-linked hereditary disorder, and aspirin can induce hemolysis in patients with G6PD deficiency. We report a case of a 5 year and 8 month old male with KD and G6PD deficiency. PMID:27134550

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

    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

  8. Detection of coronary artery damage at sub-acute phase of Kawasaki disease with myocardial perfusion imaging and two-dimensional echocardiography

    Objective: To compare the diagnostic values of rest 99Tcm-MIBI MPI and two-dimensional echocardiography (2-DE) for the detection of coronary artery damage at sub-acute phase of Kawasaki disease (KD). Methods: Twenty-four children (14 males and 10 females, mean age: (2.50±2.19) years) with KD at sub-acute phase were studied between August 1999 and March 2012. All patients underwent rest 99Tcm-MIBI MPI and 2-DE. χ2 and Wilcoxon rank sum tests with SPSS 13.0 were used for data analysis. Results: The positive rate of MPI was 66.67% (16/24), significantly higher than that of 2-DE (37.50%,9/24; χ2=4.00, P<0.05). There was no significant difference between the duration for definite diagnosis by MPI and 2-DE ((13.79±2.86) vs (15.89±5.60) d; Z=-0.746, P>0.05). Eight of 24 patients (33.33%) had positive results for both MPI and 2-DE, and 7 patients (29.17%) had negative findings for both methods. Eight patients (8/24, 33.33%) were positive on MPI but negative on 2-DE, and 1 patient (1/24, 4.17%) was positive on 2-DE but negative on MPI. The areas of myocardial ischemia detected by MPI in 4 patients were consistent with the findings by 2-DE. Conclusions: Rest 99Tcm-MIBI MPI is a valuable noninvasive method to evaluate the coronary circulation and myocardial ischemia in KD patients at sub-acute phase. In combination with MPI, 2-DE might provide more comprehensive information for the evaluation of KD. (authors)

  9. Genetics Home Reference: Kawasaki disease

    ... abnormal response are unknown. Because cases of the disorder tend to cluster geographically and by season, researchers have suggested that an infection may be involved. However, no infectious agent (such as a virus or bacteria) has been ... disease . The ITPKC gene provides instructions for making an ...

  10. Kawasaki disease: report of 34 cases Original Article

    YAVUZ, Taner; Öner, Naci; Ömeroğlu, Rukiye Eker Eker; Dindar, Aygün; Aydoğan, Ümrah; Ertuğrul, Türkan

    2006-01-01

    Aim: In this study we present children with Kawasaki disease and compare with the other series of patients reported in Turkey Material and Method: This is a retrospective study of 34 children admitted and treated with the clinical diagnosis of Kawasaki disease at our Pediatric Cardiology Unit in the last five years Complete blood count erythrocyte sedimentation rate routine urine examination measurements serum hepatic transaminase and C reactive protein and echocardiography were the studies p...

  11. Giant aortic arch aneurysm complicating Kawasaki disease: an original case report

    Kaouthar, Hakim; Rafik, Boussaada; Jihen, Ayari; Imen, Hamdi; Lilia, Chaker; Fatma, Ouarda; Hela, Msaad

    2013-01-01

    Kawasaki disease (KD) is a common acute vasculitis in pediatric population that usually involves small and middle-sized arteries, commonly coronary arteries. Although the incidence and natural course of coronary aneurysms after KD are well documented, related reports on peripheral arterial and aortic aneurysms are scarce.

  12. Common variants in CASP3 confer susceptibility to Kawasaki disease

    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

    2010-01-01

    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 × 10−8 in the Japanese and P = 3.7 × 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. PMID:20423928

  13. Kawasaki Disease in Ghana: Case Reports From Korle Bu Teaching Hospital

    Badoe, E V; Neequaye, J; Oliver-Commey, J O; Amoah, J; Osafo, A; Aryee, I; Nyarko, M Y

    2011-01-01

    Kawasaki disease, an acute febrile vasculitis, predominantly affects children under the age of 5 years and is thought to be a rare disease in the developing world. It has previously never been reported in Ghana. We report 3 cases from February, 2007 to February, 2008. This potentially serious disease has no definitive diagnostic test and it is not unusual for diagnosis to be delayed with serious consequences. Any child with irritability and persisting fever (>5 days) not responding to antipyr...

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

    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.

  15. Coronary CT angiography and MR angiography of Kawasaki disease

    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.)

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

    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

  17. Role of TGF-β signaling in remodeling of noncoronary artery aneurysms in kawasaki disease

    Lee, AM; Shimizu, C.; Oharaseki, T; K. Takahashi; Daniels, LB; Kahn, A.; Adamson, R.; Dembitsky, W; Gordon, JB; Burns, JC

    2015-01-01

    © 2015 Society for Pediatric Pathology. Coronary artery aneurysms (CAA) remain an important complication of Kawasaki disease (KD), the most common form of pediatric acquired heart disease in developed countries. Potentially life-threatening CAA develop in 25% of untreated children and 5% of children treated with highdose intravenous immunoglobulin during the acute phase of the self-limited vasculitis. Noncoronary artery aneurysms (NCAA) in extraparenchymal, muscular arteries occur in aminorit...

  18. Role of TGF beta signaling in Remodeling of Non-Coronary Artery Aneurysms in Kawasaki disease /

    Lee, Aaron Ming

    2014-01-01

    Coronary artery aneurysms remain a life-threatening complication of Kawasaki disease (KD), the most common form of pediatric acquired heart disease in developed countries (1). Potentially life-threatening coronary artery aneurysms (CAA) develop in 25% of untreated children and 5% of children treated with high dose intravenous immunoglobulin during the acute phase of the self-limited vasculitis (2). Non-coronary artery aneurysms (NCAA) in extra-parenchymal, muscular arteries occur in a minorit...

  19. Diagnosis of myocardial ischemia in Kawasaki disease

    Thallium-201 myocardial imaging was performed at rest in 131 children with coronary arterial lesions due to Kawasaki disease. The coronary arterial lesions were assessed by selective coronary angiography within a few days of the isotope study. Twenty-one children ha d occlusive lesions, and segmental stenotic lesions were seen in 16 children. Perfusion defects of the myocardial images were detected in nine of the former and in three of the latter. The locations of the perfusion defects coincided with the perfusion areas of the affected vessels on coronary angiography. Twelve patients with initial perfusion defects at rest had a follow-up study and the defects disappeared in five. Myocardial imaging with exercise was performed in 27 patients including four with coronary arterial occlusion and two with segmental stenosis on coronary angiography. All with coronary artery lesions showed perfusion defects on the imaging with exercise, while the resting study showed the defects only in one patient, in whom more extensive perfusion defects were observed after exercise. Myocardial imaging following intravenous injection of dipyridamole was carried out in 43 patients. Perfusion defects after the injection were noted in 15 of 17 patients with coronary occlusion and in nine of 13 patients with segmental stenosis. In four patients with perfusion defects at rest, additional or more extensive defects were revealed by this drug in the areas of additional coronary arterial involvements. In 20 patients with perfusion defects only after dipyridamole injection, the perfusion defects coincided with the angiographic findings very well. A perfusion defect was documented following dipyridamole injection in one exceptional patient who had no stenotic lesions, but had three giant coronary aneurysms of the right coronary artery. Thus the dilated coronary lesions seemed to give a perfusion defect. (J.P.N.)

  20. Consortium-Based Genetic Studies of Kawasaki Disease in Korea: Korean Kawasaki Disease Genetics Consortium.

    Lee, Jong-Keuk; Hong, Young Mi; Jang, Gi Young; Yun, Sin Weon; Yu, Jeong Jin; Yoon, Kyung Lim; Lee, Kyung-Yil; Kil, Hong-Rang

    2015-11-01

    In order to perform large-scale genetic studies of Kawasaki disease (KD) in Korea, the Korean Kawasaki Disease Genetics Consortium (KKDGC) was formed in 2008 with 10 hospitals. Since the establishment of KKDGC, there has been a collection of clinical data from a total of 1198 patients, and approximately 5 mL of blood samples per patient (for genomic deoxyribonucleic acid and plasma isolation), using a standard clinical data collection form and a nation-wide networking system for blood sample pick-up. In the clinical risk factor analysis using the collected clinical data of 478 KD patients, it was found that incomplete KD type, intravenous immunoglobulin (IVIG) non-responsiveness, and long febrile days are major risk factors for coronary artery lesions development, whereas low serum albumin concentration is an independent risk factor for IVIG non-responsiveness. In addition, we identified a KD susceptibility locus at 1p31, a coronary artery aneurysm locus (KCNN2 gene), and the causal variant in the C-reactive protein (CRP) promoter region, as determining the increased CRP levels in KD patients, by means of genome-wide association studies. Currently, this consortium is continually collecting more clinical data and genomic samples to identify the clinical and genetic risk factors via a single nucleotide polymorphism chip and exome sequencing, as well as collaborating with several international KD genetics teams. The consortium-based approach for genetic studies of KD in Korea will be a very effective way to understand the unknown etiology and causal mechanism of KD, which may be affected by multiple genes and environmental factors. PMID:26617644

  1. Resistant Kawasaki disease, case report and literatures review

    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.

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

    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.

  3. Changes in Coronary Perfusion after Occlusion of Coronary Arteries in Kawasaki Disease

    Kwak, Ji Hee; Song, Jinyoung; Kang, I-Seok; Huh, June; Lee, Heung-Jae

    2014-01-01

    Purpose Myocardial infarction in children with total occlusion of a coronary artery after Kawasaki disease is rare due to multiple collateral vessels. We aimed to investigate the changes in coronary perfusion associated with coronary artery occlusion after Kawasaki disease. Materials and Methods Eleven patients with coronary artery occlusion after Kawasaki disease were investigated. Serial coronary angiographies after total occlusion of a coronary artery were reviewed and the changes were des...

  4. Prediction of nonresponsiveness to medium-dose intravenous immunoglobulin (1 g/kg) treatment: an effective and safe schedule of acute treatment for Kawasaki disease

    Moon, Kyung Pil; Kim, Beom Joon; Lee, Kyu Jin; Oh, Jin Hee; Han, Ji Whan; Lee, Kyung Yil

    2016-01-01

    Purpose Medium-dose (1 g/kg) intravenous immunoglobulin (IVIG) is effective in the majority of patients with Kawasaki disease (KD) but some patients who do not respond to medium-dose IVIG are at high risk for the development of coronary artery lesions (CALs). The purpose of this study was to identify the clinical predictors associated with unresponsiveness to medium-dose IVIG and the development of CALs. Methods A retrospective study was performed in 91 children with KD who were treated with medium-dose IVIG at our institution from January 2004 to December 2013. We classified the patients into responders (group 1; n=68) and nonresponders (group 2; n=23). We compared demographic, laboratory, and echocardiographic data between the 2 groups. Results Multivariate logistic regression analysis identified 6 variables as predictors for resistance to medium-dose IVIG. We generated a predictive scoring system assigning 1 point each for percentage of neutrophils ≥65%, C-reactive protein≥100 mg/L, aspartate aminotransferase≥100 IU/L, and alanine aminotransferase≥100 IU/L, as well as 2 points for less than 5 days of illness, and serum sodium level≤136 mmol/L. Using a cutoff point of ≥4 with this scoring system, we could predict nonresponsiveness to medium-dose IVIG with 74% sensitivity and 71% specificity. Conclusion If a patient has a low-risk score in this system, medium-dose IVIG can be recommended as the initial treatment. Through this process, we can minimize the adverse effects of high-dose IVIG and incidence of CALs.

  5. Establishment of Kawasaki disease database based on metadata standard

    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

    2016-01-01

    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

  6. [Kawasaki disease and cranial nerve involvement: two cases].

    Delafay, M-C; Matoussi, Z; Remy-Piccolo, V; Gay, C; Veyrier, M; Stéphan, J-L

    2015-08-01

    CNS involvement, except classical lymphocytic meningitis, is exceptionally rare in Kawasaki disease. Herein, we report on two atypical cases of KD with cranial nerve inflammation. The first case presented supranuclear vertical palsy and the second case Bell palsy. Outcome was promptly favorable with IV immunoglobulins, with no long-term complications. No specific KD biomarkers are available and diagnosis of atypical forms is difficult. KD can mimic a systemic illness and appropriate therapy may be delayed. PMID:26141803

  7. 9 cases of coronary stenotic lesion due to Kawasaki disease

    Myocardial Tl-201 SPECT was undertaken in 9 children with selective coronary arteriographically (S-CAG) proven stenotic lesions associated with Kawasaki disease. A decrease in perfusion and/or perfusion defect were seen on SPECT in 6 patients, corresponding to the findings of S-CAG. Among six patients undergoing SPECT 4 hr after the iv injection of dipyridamole, only one had incomplete redistribution. SPECT may be of value for evaluating the presence of stenotic lesions and myocardial viability. (Namekawa, K.)

  8. Lipoprotein Particle Concentrations in Children and Adults following Kawasaki Disease

    J. Lin; Jain, S; X. Sun; Liu, V.; Sato, YZ; Jimenez-Fernandez, S; Newfield, RS; Pourfarzib, R; Tremoulet, AH; Gordon, JB; Daniels, LB; Burns, JC

    2014-01-01

    Objective: To test the hypothesis that children and adults with a history of Kawasaki disease (KD) are more likely to have abnormal lipoprotein particle profiles that could place them at increased risk for developing atherosclerosis later in life. Study design: Fasting serum samples were obtained from 192 children and 63 adults with history of KD and 90 age-similar healthy controls. Lipoprotein particle concentrations and sizes were measured by nuclear magnetic resonance spectroscopy (LipoSci...

  9. Lipoprotein particle concentrations in children and adults following kawasaki disease

    J. Lin; Jain, S; X. Sun; Liu, V.; Sato, YZ; Jimenez-Fernandez, S; Newfield, RS; Pourfarzib, R; Tremoulet, AH; Gordon, JB; Daniels, LB; Burns, JC

    2014-01-01

    © 2014 Elsevier Inc. Objective To test the hypothesis that children and adults with a history of Kawasaki disease (KD) are more likely to have abnormal lipoprotein particle profiles that could place them at increased risk for developing atherosclerosis later in life. Study design Fasting serum samples were obtained from 192 children and 63 adults with history of KD and 90 age-similar healthy controls. Lipoprotein particle concentrations and sizes were measured by nuclear magnetic resonance sp...

  10. Lipoprotein particle concentrations in children and adults following kawasaki disease

    Lin, J.; S. Jain; X. Sun; Liu, V; Sato, YZ; Jimenez-Fernandez, S; Newfield, RS; Pourfarzib, R; Tremoulet, AH; Gordon, JB; Daniels, LB; Burns, JC

    2014-01-01

    Objective: To test the hypothesis that children and adults with a history of Kawasaki disease (KD) are more likely to have abnormal lipoprotein particle profiles that could place them at increased risk for developing atherosclerosis later in life. Study design: Fasting serum samples were obtained from 192 children and 63 adults with history of KD and 90 age-similar healthy controls. Lipoprotein particle concentrations and sizes were measured by nuclear magnetic resonance spectroscopy (LipoSci...

  11. Infliximab Treatment for Refractory Kawasaki Disease in Korean Children

    Song, Min Seob; Lee, Sang Bum; Sohn, Sejung; Oh, Jin Hee; Yoon, Kyung Lim; Han, Ji Whan; Kim, Chul Ho

    2010-01-01

    Background and Objectives This was a multicenter study to evaluate the usefulness of the tumor necrosis factor-alpha (TNF-α) blocker infliximab for treatment of Korean pediatric patients with refractory Kawasaki disease (KD). Subjects and Methods Data from 16 patients throughout Korea who were diagnosed with refractory KD and received infliximab were collected retrospectively. Results Complete response to therapy with cessation of fever occurred in 13 of 16 patients. C-reactive protein (CRP) ...

  12. Identification of Novel Susceptibility Loci for Kawasaki Disease in a Han Chinese Population by a Genome-Wide Association Study

    Tsai, Fuu-Jen; Lee, Yi-Ching; Chang, Jeng-Sheng; Huang, Li-Min; Huang, Fu-Yuan; Chiu, Nan-Chang; Chen, Ming-Ren; CHI, Hsin; Lee, Yann-Jinn; Chang, Li-Ching; Liu, Yi-Min; Wang, Hsiang-Hua; Chen, Chien-Hsiun; Chen, Yuan-Tsong; Wu, Jer-Yuarn

    2011-01-01

    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 Ch...

  13. Usefulness of natriuretic peptide for the diagnosis of Kawasaki disease: a systematic review and meta-analysis

    Lin, Kuan-Ho; Chang, Shy-Shin; Yu, Chin-Wei; LIN, SHEN-CHE; Liu, Shu-Chun; Chao, Hsiao-yun; Lee, Meng-Tse Gabriel; Wu, Jiunn-Yih; Lee, Chien-Chang

    2015-01-01

    Objective To examine the diagnostic value of serum B-type natriuretic peptide (BNP) in acute Kawasaki disease (KD). Design Systematic review and meta-analysis. Data sources A systematic literature search strategy was designed and carried out using MEDLINE, EMBASE and the Cochrane Library from inception to December 2013. We also performed manual screening of the bibliographies of primary studies and review articles, and contacted authors for additional data. Study eligibility criteria We inclu...

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

    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

    2010-01-01

    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. PMID:18084290

  15. Usefulness of anterior uveitis as an additional tool for diagnosing incomplete Kawasaki disease

    Lee, Kyu Jin; Kim, Hyo Jin; Kim, Min Jae; Yoon, Ji Hong; Lee, Eun Jung; Lee, Jae Young; Oh, Jin Hee; Lee, Soon Ju; Lee, Kyung Yil; Han, Ji Whan

    2016-01-01

    Purpose There are no specific tests for diagnosing Kawasaki disease (KD). Additional diagnostic criteria are needed to prevent the delayed diagnosis of incomplete Kawasaki disease (IKD). This study compared the frequency of coronary artery lesions (CALs) in IKD patients with and without anterior uveitis (AU) and elucidated whether the finding of AU supported the diagnosis of IKD. Methods This study enrolled patients diagnosed with IKD at The Catholic University of Korea, Uijeongbu St. Mary's ...

  16. Characterization of the gut microbiota of Kawasaki disease patients by metagenomic analysis

    Akiko eKinumaki

    2015-08-01

    Full Text Available Kawasaki disease (KD is an acute febrile illness of early childhood. Previous reports have suggested that genetic disease susceptibility factors, together with a triggering infectious agent, could be involved in KD pathogenesis; however, the precise etiology of this disease remains unknown. Additionally, previous culture-based studies have suggested a possible role of intestinal microbiota in KD pathogenesis. In this study, we performed metagenomic analysis to comprehensively assess the longitudinal variation in the intestinal microbiota of twenty-eight KD patients. Several notable bacterial genera were commonly extracted during the acute phase, whereas a relative increase in the number of Ruminococcus bacteria was observed during the non-acute phase of KD. The metagenomic analysis results based on bacterial species classification suggested that the number of sequencing reads with similarity to five Streptococcus spp. (S. pneumonia, pseudopneumoniae, oralis, gordonii, and sanguinis, in addition to patient-derived Streptococcus isolates, markedly increased during the acute phase in most patients. Streptococci include a variety of pathogenic bacteria and probiotic bacteria that promote human health; therefore, this further species discrimination could comprehensively illuminate the KD-associated microbiota. The findings of this study suggest that KD-related Streptococci might be involved in the pathogenesis of this disease.

  17. Bacillus Calmette-Guérin reactivation as a sign of incomplete Kawasaki disease.

    Novais, Cristina; Fortunato, Fabiana; Bicho, Anabela; Preto, Luísa

    2016-01-01

    Kawasaki disease (KD) is an acute, self-limited, systemic vasculitis of unknown aetiology, extremely rare in infants younger than 6 months old. Younger infants are more likely to present with incomplete KD (IKD) and are at higher risk of developing coronary abnormalities. An early and specific clinical sign, not included in the classical diagnosis criteria, but that can be very useful in the diagnosis of KD, is the reaction at the Bacillus Calmette-Guérin (BCG) inoculation site. We describe a case of a 4-month-old boy, fully immunised, whose BCG scar reactivation led to the diagnosis of IKD. This case-report emphasises the importance of BCG site reactivation in establishing a diagnosis of IKD that clinicians should be aware of, especially in countries where BCG vaccination is still part of the immunisation schedule. PMID:27033285

  18. Clinical and laboratory characteristics of children with Kawasaki disease

    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.

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

    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.

  20. Variations in ORAI1 Gene Associated with Kawasaki Disease.

    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. PMID:26789410

  1. Myocardial performance and perfusion during exercise in patients with coronary artery disease caused by Kawasaki disease

    For a study of the natural history of coronary artery lesions after Kawasaki disease and their effect on myocardial blood flow reserve with exercise, five such patients underwent exercise testing on a bicycle. Oxygen consumption, carbon dioxide production, minute ventilation, and electrocardiograms were monitored continuously. Thallium-201 scintigraphy was performed for all patients. One patient stopped exercise before exhaustion of cardiovascular reserve but had no evidence of myocardial perfusion abnormalities. Four patients terminated exercise because of exhaustion of cardiovascular reserve; one had normal cardiovascular reserve and thallium scintiscans, but the remaining patients had diminished cardiovascular reserve. Thallium scintigrams showed myocardial ischemia in two and infarction in one. No patient had exercise-induced electrocardiographic changes. These results indicate that patients with residual coronary artery lesions after Kawasaki disease frequently have reduced cardiovascular reserve during exercise. The addition of thallium scintigraphy and metabolic measurements to exercise testing improved the detection of exercise-induced abnormalities of myocardial perfusion

  2. Mycoplasma pneumoniae infection in patients with Kawasaki disease

    Mi Na Lee

    2011-03-01

    Full Text Available Purpose : Kawasaki disease (KD is the main cause of acquired heart disease in children. In addition to cardiovascular involvement, many complications have been recognized in KD. However, respiratory complications have been rarely reported. We investigated the differences in clinical characteristics, laboratory findings, radiography findings, and echocardiography findings of Mycoplasma pneumoniae infection and other types of pneumonia in KD patients. Methods : Among 358 patients with KD, 54 developed concurrent pneumonia. Among the 54 patients, 12 (22.2% with high titers of anti-M. pneumoniae antibody (AMA (&gt;1:640 were grouped in the M. pneumoniae group and 42 were included in the control group. Serum AMA was measured in each patient. Clinical laboratory findings and total duration of fever were analyzed. Results : The duration of fever, serum hemoglobin, white blood cell count, platelet count, erythrocyte sedimentation rate, C-reactive protein level, albumin level, and the incidence of coronary arterial lesions showed no statistical difference in the 2 groups. Neutrophil count was significantly higher in the M. pneumoniae group than in the control group. Among various radiography findings observed in pneumonia, consolidation and pleural effusion were more frequent in the M. pneumoniae group than in the control group. On the other hand, parahilar peribronchial opacification, diffuse interstitial lesion, and normal findings prevailed in the control group. Conclusion : KD patients can have concurrent infections, especially pulmonary symptoms. The cause of KD is likely to be associated with M. pneumoniae infection. Thus, immediate treatment of M. pneumoniae infection in KD patients is very important.

  3. Ascending Aorta Elastography After Kawasaki Disease Compared to Systemic Hypertension.

    Nandlall, Ian; Maurice, Roch L; Fournier, Anne; Merouani, Aïcha; Dahdah, Nagib

    2015-10-01

    Kawasaki disease (KD) is a systemic vasculitis, classically affecting large- and medium-size arteries. The coronary arteries draw most of the clinical attention, whereas few studies have taken interest in the ascending aorta. Using a proprietary imaging-based mechanical biomarker (ImBioMark), we sought to determine aortic stiffness in KD compared to systemic hypertension (HTN) and healthy children. We evaluated parasternal long-axis views focused on the ascending aorta in 20 controls, 12 KD, and 8 HTN as a comparative clinical model of vascular stiffness. We calculated systolic and diastolic aortic wall strain with ImBioMark. Strain was tested for normality against height, systolic, and diastolic blood pressure in normal subjects. Strain from KD and HTN was normalized (Z score) accordingly. Z score comparisons were performed using nonparametric statistics. Age was similar between KD and HTN (9.1 ± 5.3 and 9.9 ± 5.3 years old; p = NS). Systolic and diastolic strain values were normally distributed against height, systolic blood pressure, and diastolic blood pressure in healthy subjects. HTN subjects had abnormal systolic and diastolic strain values (p < 0.0001). Whereas KD subjects had normal diastolic strain, systolic strain was significantly lower (p < 0.001), and systolic strain was intermediate between controls and HTN. There were no significant differences in aortic strain among KD, however, according to the presence of coronary artery aneurysms. Despite normal blood pressure, the ascending aorta in KD exhibits reduced strain during systole. This may reflect in situ rigidity of the aorta. The normal diastolic strain in KD may, in contrast, reflect normal peripheral vascular resistance. PMID:25921428

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

    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

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

    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

  6. Exploring the genes associated with the response to intravenous immunoglobulin in patients with Kawasaki disease using DNA microarray analysis.

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

    2015-02-01

    In this study we aimed to screen genes associated with intravenous immunoglobulin (IVIG) responding in patients with Kawasaki disease (KD) and thus explore the underlying molecular mechanism of IVIG resistance. The differentially expressed genes (DEGs) were identified by samr package in R. Then, protein-protein interaction (PPI) networks were constructed by STRING software. We further collected the regulatory data from TRANSFAC database, followed by regulatory interaction network construction. A total 194 of DEGs, including 185 up- and 9 down-regulated DEGs, were identified between IVIG-responding and non-responding patients with KD at acute stage. In contrast, no DEGs were found at convalescent stage. PPI networks and regulatory networks were constructed based on the 185 up-regulated genes at acute stage. The degrees of TFRC (transferrin receptor protein 1) and GADD45A (growth arrest and DNA-damage-inducible alpha) were higher than other genes, and meanwhile MYC (V-Myc Myelocytomatosis Viral Oncogene Homolog) and E2F1 (E2F Transcription Factor 1) were found to be two TFs (transcription factors) with the highest degrees. In conclusions, the response to IVIG in Kawasaki disease patients may be involved in the expression of TFRC, GADD45A, MYC and E2F1. PMID:25449331

  7. Distribution and Protective Role of HLAB40 in Iranian Patients with Kawasaki Disease; a Report from Southern Iran

    Ajami, Gholamhossein; Aflaki, Khashayar; Alyasin, Soheila; Gharesi-fard, Behrooz; BORZOUEE, Mohammad; Amoozgar, Hamid

    2014-01-01

    Objective: Kawasaki disease (KD) clinically presents as a systemic vasculitis syndrome with significant cardiovascular involvement. With different incidence among different ethnic groups, the role of certain human leukocyte antigens and their products has been considered as a crucial predisposing factor in the immune responses in this disease. Methods: We determined the distribution of human leukocyte antigens type B for 90 Iranian patients with Kawasaki disease in order to evaluate a possibl...

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

    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 hepcidin levels (both p hepcidin levels were positively and significantly correlated with urine hepcidin levels (p hepcidin and hemoglobin levels significantly decreased (both p hepcidin induces transient anemia and hypoferremia during KD's acute inflammatory phase. PMID:27187366

  9. Kawasaki Disease: Laboratory Findings and an Immunopathogenesis on the Premise of a "Protein Homeostasis System"

    Lee, Kyung-Yil; Rhim, Jung-Woo; Kang, Jin-Han

    2012-01-01

    Kawasaki disease (KD) is a self-limited systemic inflammatory illness, and coronary artery lesions (CALs) are a major complication determining the prognosis of the disease. Epidemiologic studies in Asian children suggest that the etiologic agent(s) of KD may be associated with environmental changes. Laboratory findings are useful for the diagnosis of incomplete KD, and they can guide the next-step in treatment of initial intravenous immunoglobulin non-responders. CALs seem to develop in the e...

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

    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.

  11. A Combination of Cross Correlation and Trend Analyses Reveals that Kawasaki Disease is a Pollen-Induced Delayed-Type Hyper-Sensitivity Disease

    Akira Awaya; Chiaki Nishimura

    2014-01-01

    Based on ecological analyses we proposed in 2003 the relation of Kawasaki Disease (KD) onset causing acute febrile systemic vasculitis, and pollen exposure. This study was aimed at investigating the correlation between pollen release and the change in the numbers of KD patients from 1991 to 2002 in Kanagawa, Japan. Short-term changes in the number of KD patients and medium- to long-term trends were analyzed separately. Short-term changes in the number of KD patients showed a significant pos...

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

    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.

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

    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.

  14. Uveitis as an important ocular sign to help early diagnosis in Kawasaki disease

    Choi, Han Seul; Lee, Seul Bee; Kwon, Jung Hyun; Kim, Hae Soon; Sohn, Sejung; Hong, Young Mi

    2015-01-01

    Purpose Incomplete Kawasaki disease (KD) is frequently associated with delayed diagnosis and treatment. Delayed diagnosis leads to increasing risk of coronary artery aneurysm. Anterior uveitis is an important ocular sign of KD. The purpose of this study was to assess differences in laboratory findings, including echocardiographic measurements, clinical characteristics such as fever duration and treatment responses between KD patients with and those without uveitis. Methods We conducted a pros...

  15. Genetic variants of glutamate receptor gene family in Taiwanese Kawasaki disease children with coronary artery aneurysms

    Lin, Ying-Ju; Chang, Jeng-Sheng; Liu, Xiang(Research Center for Hadron and CSR Physics, Lanzhou University and Institute of Modern Physics of CAS, 730000, Lanzhou , China); Tsang, Hsinyi; Lin, Ting-Hsu; Liao, Chiu-Chu; Huang, Shao-Mei; Chien, Wen-Kuei; Chen, Jin-Hua; Wu, Jer-Yuarn; Chen, Chien-Hsiun; Chang, Li-Ching; Lin, Cheng-Wen; Ho, Tsung-Jung; Tsai, Fuu-Jen

    2014-01-01

    Background Patients with Kawasaki disease (KD), a pediatric systemic vasculitis, may develop coronary artery aneurysm (CAA) as a complication. To investigate the role of glutamate receptors in KD and its CAA development, we performed genetic association studies. Methods and results We examined the whole family of glutamate receptors by genetic association studies in a Taiwanese cohort of 262 KD patients. We identified glutamate receptor ionotropic, kainate 1 (GRIK1) as a novel susceptibility ...

  16. Risk Factors of Coronary Involvement in Kawasaki Disease in Recent Outbreak in South Iran, Shiraz.

    Amoozgar, Hamid; Mohammadi, Hamid; Shakibazad, Nader

    2012-01-01

    Background: During the past year, Kawasaki disease (KD) had an outbreak with a high inci-dence of coronary involvement in Shiraz, Iran. This study focuses on risk factors and their correlation with aneurysm formation. Methods: All patients who were admitted with the diagnosis of KD in the pediatric units of Shiraz, Iran between 2009 and 2010 were included in this study. Data analysis was performed on demographic as well as clinical features, laboratory data, echocardiography, and electroca...

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

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

    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 metab...

  18. Cardiopulmonary Function, Exercise Capacity, and Echocardiography Finding of Pediatric Patients With Kawasaki Disease

    Tuan, Sheng-Hui; Li, Min-Hui; Hsu, Miao-Ju; Tsai, Yun-Jeng; Chen, Yin-Han; Liao, Tin-Yun; Lin, Ko-Long

    2016-01-01

    Abstract Coronary artery (CA) abnormalities influence exercise capacity (EC) of patients with Kawasaki disease (KD), and Z-score of CA is a well established method for detecting CA aneurysm. We studied the influence of KD on cardiopulmonary function and EC; meanwhile we analyzed echocardiographic findings of KD patients. We also assessed the correlation between CA Z-score and EC of KD patients to see if CA Z-score of KD patients could reflect EC during exercise. Sixty-three KD patients were recruited as KD group 1 from children (aged 5–18 y) who received transthoracic echocardiographic examinations and symptom-limited treadmill exercise test for regular follow-up of KD from January 2010 to October 2014 in 1 medical center. We then divided KD group 1 into KD group 2 (pressure products (PRPPs) in KD group 1 to 3 were all lower than corresponding control groups significantly (P = 0.010, 0.020, and 0.049, respectively). PRPPs correlated with Max-Z of CA by both equations modest inversely (by Dallaire, P = 0.017, Spearman rho = −0.301; by Fuse, P = 0.014, Spearman rho = −0.309). Our study recruited larger number of KD patients and provided a newer data of EC of KD patients. Our finding suggests that after acute stage of KD, patients could maintain normal cardiorespiratory fitness. Therefore, we believe that it is important to promote cardiovascular health to KD patients and KD patients should exercise as normal peers. However, since KD patients might still have compromised coronary perfusion during exercise, it remains crucial to assess and monitor cardiovascular risk of KD patients. Max-Z of CA correlates with PRPP modest inversely and might be used as a follow-up indicator of CA reserve during exercise after acute stage of KD. PMID:26765431

  19. Cardiopulmonary Function, Exercise Capacity, and Echocardiography Finding of Pediatric Patients With Kawasaki Disease: An Observational Study.

    Tuan, Sheng-Hui; Li, Min-Hui; Hsu, Miao-Ju; Tsai, Yun-Jeng; Chen, Yin-Han; Liao, Tin-Yun; Lin, Ko-Long

    2016-01-01

    Coronary artery (CA) abnormalities influence exercise capacity (EC) of patients with Kawasaki disease (KD), and Z-score of CA is a well established method for detecting CA aneurysm. We studied the influence of KD on cardiopulmonary function and EC; meanwhile we analyzed echocardiographic findings of KD patients. We also assessed the correlation between CA Z-score and EC of KD patients to see if CA Z-score of KD patients could reflect EC during exercise.Sixty-three KD patients were recruited as KD group 1 from children (aged 5-18 y) who received transthoracic echocardiographic examinations and symptom-limited treadmill exercise test for regular follow-up of KD from January 2010 to October 2014 in 1 medical center. We then divided KD group 1 into KD group 2 (pressure products (PRPPs) in KD group 1 to 3 were all lower than corresponding control groups significantly (P = 0.010, 0.020, and 0.049, respectively). PRPPs correlated with Max-Z of CA by both equations modest inversely (by Dallaire, P = 0.017, Spearman rho = -0.301; by Fuse, P = 0.014, Spearman rho = -0.309).Our study recruited larger number of KD patients and provided a newer data of EC of KD patients. Our finding suggests that after acute stage of KD, patients could maintain normal cardiorespiratory fitness. Therefore, we believe that it is important to promote cardiovascular health to KD patients and KD patients should exercise as normal peers. However, since KD patients might still have compromised coronary perfusion during exercise, it remains crucial to assess and monitor cardiovascular risk of KD patients. Max-Z of CA correlates with PRPP modest inversely and might be used as a follow-up indicator of CA reserve during exercise after acute stage of KD. PMID:26765431

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

    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.

  1. Urinary Colorimetric Sensor Array and Algorithm to Distinguish Kawasaki Disease from Other Febrile Illnesses

    Jin, Bo; Deng, Xiaohong; Hu, Guang; Liu, Xiaodan; Zhang, Jie; Jin, Hua; Huang, Min; Kanegaye, John T.; Tremoulet, Adriana H.; Burns, Jane C.; Wu, Jianmin; Cohen, Harvey J.; Ling, Xuefeng B.

    2016-01-01

    Objectives Kawasaki disease (KD) is an acute pediatric vasculitis of infants and young children with unknown etiology and no specific laboratory-based test to identify. A specific molecular diagnostic test is urgently needed to support the clinical decision of proper medical intervention, preventing subsequent complications of coronary artery aneurysms. We used a simple and low-cost colorimetric sensor array to address the lack of a specific diagnostic test to differentiate KD from febrile control (FC) patients with similar rash/fever illnesses. Study Design Demographic and clinical data were prospectively collected for subjects with KD and FCs under standard protocol. After screening using a genetic algorithm, eleven compounds including metalloporphyrins, pH indicators, redox indicators and solvatochromic dye categories, were selected from our chromatic compound library (n = 190) to construct a colorimetric sensor array for diagnosing KD. Quantitative color difference analysis led to a decision-tree-based KD diagnostic algorithm. Results This KD sensing array allowed the identification of 94% of KD subjects (receiver operating characteristic [ROC] area under the curve [AUC] 0.981) in the training set (33 KD, 33 FC) and 94% of KD subjects (ROC AUC: 0.873) in the testing set (16 KD, 17 FC). Color difference maps reconstructed from the digital images of the sensing compounds demonstrated distinctive patterns differentiating KD from FC patients. Conclusions The colorimetric sensor array, composed of common used chemical compounds, is an easily accessible, low-cost method to realize the discrimination of subjects with KD from other febrile illness. PMID:26859297

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

    Ying-Hsien Huang

    2016-05-01

    Full Text Available 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.

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

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

    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

  5. Cardiopulmonary Function, Exercise Capacity, and Echocardiography Finding of Pediatric Patients With Kawasaki Disease

    Tuan, Sheng-Hui; LI, Min-hui; Hsu, Miao-Ju; Tsai, Yun-Jeng; Chen, Yin-Han; Liao, Tin-Yun; Lin, Ko-Long

    2016-01-01

    Abstract Coronary artery (CA) abnormalities influence exercise capacity (EC) of patients with Kawasaki disease (KD), and Z-score of CA is a well established method for detecting CA aneurysm. We studied the influence of KD on cardiopulmonary function and EC; meanwhile we analyzed echocardiographic findings of KD patients. We also assessed the correlation between CA Z-score and EC of KD patients to see if CA Z-score of KD patients could reflect EC during exercise. Sixty-three KD patients were r...

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

    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.

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

    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.

  8. Cardiac involvement in Kawasaki disease in Pakistani children

    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

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

    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.

  11. Adjunct cyclosporine therapy for refractory Kawasaki disease in a very young infant.

    Okada, Seigo; Azuma, Yoshihiro; Suzuki, Yasuo; Yamada, Hiroko; Wakabayashi-Takahara, Midori; Korenaga, Yuno; Akase, Hideaki; Hasegawa, Shunji; Ohga, Shouichi

    2016-04-01

    Herein we describe the case of a 6-week-old boy who developed complete Kawasaki disease (KD). The cytokine profile and activation of monocytes and subsequent T cells matched the typical feature of refractory KD. The patient received a total of three courses of i.v. immunoglobulin (IVIG), but did not achieve clinical relief. Adjunctive therapy with oral cyclosporine A (CsA) led to prompt defervescence. This was continued for 7 days without serious adverse events. Coronary artery dilatations regressed within 3 months of follow up. KD infants aneurysm than the older ones. To our knowledge, oral CsA treatment has not been reported in such young infants with KD. The diagnosis and treatment of very young infants with KD are challenging. Adjunctive use of CsA in IVIG treatment could be effective for refractory KD in infants <3 months of age. PMID:26670024

  12. Fatal Kawasaki disease with incomplete criteria: Correlation between optical coherence tomography and pathology.

    Dionne, Audrey; Kokta, Victor; Chami, Rose; Morissette, Geneviève; Dahdah, Nagib

    2015-12-01

    Coronary artery aneurysm is a serious complication of Kawasaki disease (KD). A 3-month-old infant presented with severe KD 27 days after onset of fever. The patient presented with shock, inferolateral ischemia on electrocardiogram and high troponin. Echocardiography showed severe myocardial dysfunction with diffuse coronary dilation and right coronary artery aneurysm. Arterial Doppler demonstrated thrombosis of aneurysmal axillary and iliac arteries. Withdrawal of support was implemented due to multi-organ failure. Post-mortem optical coherence tomography correlated with pathology. The pulmonary artery was normal on OCT and histology. Coronary arteries showed aneurysmal dilatation, with intimal hyperplasia and preserved media on OCT. Pathology confirmed these findings, with destruction of the internal elastic lamina, luminal myofibroblastic proliferation, neovascularization, and partial disappearance of the media. This is the first report of pathologic correlation in KD with OCT at the subacute stage, which adequately identified structural wall changes. PMID:26711918

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

    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 99mTc-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.)

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

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

    2010-12-15

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

  15. Kawasaki disease

    ... mucous membranes in the mouth Strawberry tongue, white coating on the tongue, or visible red bumps on ... damage to the coronary arteries and heart. Intravenous gamma globulin is the standard treatment. It is given ...

  16. Association between GRIN3A Gene Polymorphism in Kawasaki Disease and Coronary Artery Aneurysms in Taiwanese Children

    Ying-Ju Lin; Jeng-Sheng Chang; Xiang Liu; Chien-Hui Hung; Ting-Hsu Lin; Shao-Mei Huang; Kuan-Teh Jeang; Chia-Yen Chen; Chiu-Chu Liao; Cheng-Wen Lin; Chih-Ho Lai; Ni Tien; Yu-Ching Lan; Mao-Wang Ho; Wen-Kuei Chien

    2013-01-01

    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 compl...

  17. Myocardial Strain and Strain Rate in Kawasaki Disease: Range, Recovery, and Relationship to Systemic Inflammation/Coronary Artery Dilation

    Frank, Benjamin; Davidson, Jesse; Tong, Suhong; Martin, Blake; Heizer, Heather; Anderson, Marsha S; Glode, Mary P; Dominguez, Samuel R; Jone, Pei-Ni

    2016-01-01

    Background Kawasaki Disease (KD), a systemic vasculitis of medium sized vessels, is the most common cause of acquired heart disease among children in the developed world. Some KD patients demonstrate echocardiographic evidence of depressed myocardial mechanics. However, the incidence, etiology, and reversibility of abnormal mechanics in KD patients remain undefined. Methods and results We retrospectively studied 41 KD patients and measured myocardial strain and strain rate by velocity vector imaging from pre-treatment and convalescent echocardiograms. Pre-treatment procalcitonin, C-reactive protein (CRP), and coronary artery z-scores were obtained in all patients and compared between the groups with preserved versus depressed acute phase mechanics. The change in mechanics between the acute and convalescent phases was also assessed. Patients with initially low longitudinal strain improved by the convalescent period (mean difference - 4.0%; p<0.005) with the greatest improvement occurring in patients with the lowest initial strain (−7.3%; p<0.05). Patients with higher initial strain did not change significantly by the convalescent period. Patients with lower longitudinal and circumferential strain demonstrated higher median procalcitonin levels (1.2 vs. 0.3 ng/mL; p<0.05 and 1.8 vs. 0.4 ng/mL; p<0.05 respectively) and a trend towards higher CRP, but no difference in coronary artery z-scores. Strain rate was not associated with inflammatory markers or coronary artery z-scores. Conclusions The range of strain found in our cohort was large. Improvement in mean strain was driven primarily by patients with lower initial strain. Lower strain was associated with increased markers of systemic inflammation, but not proximal coronary artery changes.

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

    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.

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

    Angela Esposito Ferronato; Selma Betta Ragazzi; Maki Hirose; Débora Morais Cardoso; Alfredo Elias Gilio

    2010-01-01

    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 estud...

  20. Combination Thrombolytic and Anti-Platelet Therapies in an Infant with Incomplete Kawasaki Disease and Coronary Aneurysms

    Johnson, Peter N.; Kuhn, Robert J.

    2008-01-01

    A 3-month-old infant was transferred to our facility with persistent fever and concerns for septic shock. A 2-D echocardiogram revealed multiple coronary aneurysms and axillary and coronary artery thrombi, and a diagnosis of incomplete Kawasaki disease (KD) was established. Aggressive therapies including intravenous immunoglobulins, enoxaparin, abciximab, aspirin, and alteplase were used to decrease the size of the coronary aneurysms and inhibit further thrombus formation. After minimal chang...

  1. Giant coronary aneurysm caused by Kawasaki disease: consistency between catheter angiography and electrocardiogram gated dual-source computed tomography angiography

    Hwang, Eun-Ha; Ju, Jung-Ki; Cho, Min-Jung; Lee, Ji-Won; Lee, Hyoung-Doo

    2015-01-01

    We present the case of a 5-year-old child with coronary complications due to Kawasaki disease; this patient unintentionally underwent both dual-source computed tomography (DSCT) coronary angiography and invasive coronary angiographic examination in 2 months. This case highlights the strong consistency of the results between DSCT coronary angiography and invasive coronary angiography. Compared to conventional invasive coronary angiography, DSCT coronary angiography offered additional advantage...

  2. Galectin-3 is a marker of myocardial and vascular fibrosis in Kawasaki disease patients with giant aneurysms

    Numano, F; Shimizu, C.; Jimenez-Fernandez, S; Vejar, M; Oharaseki, T; K. Takahashi; Salgado, A; Tremoulet, AH; Gordon, JB; Burns, JC; Daniels, LB

    2015-01-01

    © 2015 Elsevier Ireland Ltd. Backgrounds Galectin-3 (Gal-3) is a multifunctional matricellular protein associated with heart failure and cardiovascular events. Gal-3 is required for transforming growth factor-β pathway-mediated myofibroblast activation that is a key process in coronary artery aneurysm formation in Kawasaki Disease (KD). Autopsies from young adults late after KD onset (AKD) have demonstrated bridging fibrosis throughout the myocardium and arteries. In this study, we postulated...

  3. Giant coronary aneurysm caused by Kawasaki disease: consistency between catheter angiography and electrocardiogram gated dual-source computed tomography angiography.

    Hwang, Eun-Ha; Ju, Jung-Ki; Cho, Min-Jung; Lee, Ji-Won; Lee, Hyoung-Doo

    2015-12-01

    We present the case of a 5-year-old child with coronary complications due to Kawasaki disease; this patient unintentionally underwent both dual-source computed tomography (DSCT) coronary angiography and invasive coronary angiographic examination in 2 months. This case highlights the strong consistency of the results between DSCT coronary angiography and invasive coronary angiography. Compared to conventional invasive coronary angiography, DSCT coronary angiography offered additional advantages such as minimal invasiveness and less radiation exposure. PMID:26770226

  4. Non-Responders to Intravenous Immunoglobulin and Coronary Artery Dilatation in Kawasaki Disease: Predictive Parameters in Korean Children

    Kim, Bo Young; Kim, Dongwan; Kim, Yong Hyun; Ryoo, Eell; Sun, Yong Han; Jeon, In-sang; Jung, Mi-Jin; Cho, Hye Kyung; Tchah, Hann; Choi, Deok Young

    2016-01-01

    Background and Objectives In Kawasaki disease (KD), high dose intravenous immunoglobulin (IVIG) significantly lowers the coronary complications. However, some patients either do not respond to initial therapy or develop coronary complications. We aimed to identify the predictive factors for unresponsiveness to initial IVIG therapy and coronary artery dilatation (CAD; defined by Z-score≥2.5) in the acute phase and convalescent phase. Subjects and Methods A retrospective review was conducted of 703 patients with KD, admitted to Gachon University Gil Medical Center between January 2005 and June 2013. The patients were divided into two groups—IVIG responders vs. non-responders—based on the IVIG treatments, and presence of fever after treatment. Further, these groups were divided into two subgroups based on their CAD. Results Among the 703 patients with KD, the rate of non-responders to initial IVIG was 16.8%. Serum total bilirubin, platelet count, and neutrophil proportion were independent predictive parameters of unresponsiveness (p<0.05). CAD was found in 234 patients (33.3%) in the acute phase, and in 32 patients (4.6%) in the convalescent phase. Male gender, fever duration, serum C-reactive protein, and white blood cell count were related to CAD (p<0.05). CAD was detected more frequently in non-responders than in the responders (47.5% vs. 31.5%, p=0.001). Kobayashi, Egami, and Sano scoring systems applied to our study population reflected low sensitivities (28.0-33.9%). Conclusion Several independent parameters were related to unresponsiveness to the initial IVIG or CAD. These parameters might be helpful in establishing more focused and careful monitoring of high-risk KD patients in Korea.

  5. 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

    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.

  6. 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

    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.

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

    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

  8. 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.

    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. PMID:24730632

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

    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.

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

    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.

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

    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.)

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

    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

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

    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.

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

    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.

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

    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.

  17. A Combination of Cross Correlation and Trend Analyses Reveals that Kawasaki Disease is a Pollen-Induced Delayed-Type Hyper-Sensitivity Disease

    Akira Awaya

    2014-03-01

    Full Text Available Based on ecological analyses we proposed in 2003 the relation of Kawasaki Disease (KD onset causing acute febrile systemic vasculitis, and pollen exposure. This study was aimed at investigating the correlation between pollen release and the change in the numbers of KD patients from 1991 to 2002 in Kanagawa, Japan. Short-term changes in the number of KD patients and medium- to long-term trends were analyzed separately. Short-term changes in the number of KD patients showed a significant positive cross correlation (CC with 9- to 10-month delay following pollen releases, and a smaller but significant CC with 3- to 4-month delay. Further, a temporal relationship revealed by positive CC distribution showed that pollen release preceded KD development, suggesting that pollen release leads to KD development. A trend in patient numbers was fitted by an exponential curve with the time constant of 0.005494. We hypothesized that the trend was caused by the cumulative effects of pollen exposure for elapsed months on patients who may develop KD. By comparing the time constants of fitted exponential curve for each pollen accumulation period with 0.005494, the exposure period was estimated to be 21.4 months, which explains why approximately 50% of patients developed KD within 24 months from birth.

  18. Kawasaki Disease With Giant Coronary Aneurysms Requiring a Ventricular Assist Device to Separate From Extracorporeal Membrane Oxygenation: Coronary Issues Can Be a Pediatric Problem Too!

    Adler, Adam C; Kodavatiganti, Ramesh

    2016-08-15

    Kawasaki disease, although common in children, may rarely affect the coronary arteries, leading to aneurysm formation and potential for coronary thrombus formation. Extremely rarely, coronary aneurysms from Kawasaki disease can thrombose, resulting in ischemic myocardium. We present a case of a 31-month-old patient requiring a left ventricular assist device after thrombosis of giant coronary aneurysms led to ischemic cardiomyopathy. At the termination of the surgical procedure, we encountered 2 periods of ventricular assist device dropout requiring intervention. With the increase in the number of pediatric patients with assist devices, we review the basic care for a patient requiring emergent surgery. PMID:27310902

  19. Does Vitamin C improve endothelial function in patients with Kawasaki disease?

    Mohammad Reza Sabri

    2015-01-01

    Full Text Available Background: Improvement of endothelial dysfunction could prevent or delay the occurrence of the atherosclerosis process in patients with Kawasaki disease (KD. It is suggested that Vitamin C could improve endothelial dysfunction. In this study, we investigated whether administration of Vitamin C as a water-soluble antioxidant could be effective in this regard among patients with KD. Materials and Methods: In this case control analytic-experimental study, children aged 3-18 years with KD, and a group of healthy children evaluated. Vitamin C (250 mg/daily administrated for the two studied groups for 1 month. Endothelial function evaluated by flow-mediated dilatation (FMD and intima-media thickness (IMT measurement using vascular Doppler ultrasonography, before and after trial. Results: In this study, 16 patients with KD and 19 normal children were studied. At baseline mean of IMT and FMD was not different in the two studied groups (P > 0.05. After Vitamin C administration IMT decreased significantly in two studied groups (from 27.06 ± 6.27 to 21.56 ± 3.77 in KD group and from 27.66 ± 5.66 to 23.33 ± 3.66 in control group [P < 0.05]. FMD increased in two studied groups, but the difference was significant in the control group (from 6.84 ± 2.51 to 7.03 ± 2.87 in KD group and from 6.53 ± 2.36 to 7.82 ± 2.14 in the control group. Conclusion: Vitamin C might improve the endothelial function of patients with KD.

  20. Systematic Confirmation Study of GWAS-Identified Genetic Variants for Kawasaki Disease in A Chinese Population

    Lou, Jiao; Zhong, Rong; Shen, Na; Lu, Xu-zai; Ke, Jun-tao; Duan, Jia-yu; Qi, Yan-qi; Wang, Yu-jia; Zhang, Qing; Wang, Wei; Gong, Fang-qi; Miao, Xiao-ping

    2015-01-01

    Genome-wide association studies (GWASs) have identified multiple single nucleotide polymorphisms (SNPs) associated with Kawasaki disease (KD). In this study, we replicated the associations of 10 GWAS-identified SNPs with KD in a Han Chinese population. Odds ratios (ORs) and 95% confidence intervals (CIs) were calculated by logistic regression, and cumulative effect of non-risk genotypes were also performed. Although none of the SNPs reached the corrected significance level, 4 SNPs showed nominal associations with KD risk. Compared with their respective wild type counterparts, rs1801274 AG+GG genotypes and rs3818298 TC+CC genotypes were nominally associated with the reduced risk of KD (OR = 0.77, 95% CI = 0.59–0.99, P = 0.045; OR = 0.74, 95% CI = 0.56–0.98, P = 0.038). Meanwhile, rs1801274 GG genotype, rs2736340 CC genotype or rs4813003 TT genotype showed a reduced risk trend (OR = 0.57, 95% CI = 0.35–0.93, P = 0.024; OR = 0.46, 95% CI = 0.26–0.83, P = 0.010; OR = 0.64, 95% CI = 0.43–0.94, P = 0.022), compared with rs1801274 AG+AA genotypes, rs2736340 CT+TT genotypes or rs4813003 TC+CC genotypes, respectively. Furthermore, a cumulative effect was observed with the ORs being gradually decreased with the increasing accumulative number of non-risk genotypes (Ptrend<0.001). In conclusion, our study suggests that 4 GWAS-identified SNPs, rs2736340, rs4813003, rs3818298 and rs1801274, were nominally associated with KD risk in a Han Chinese population individually and jointly. PMID:25645453

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

    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.

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

    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.

  3. Detection of coronary artery stenosis in children with Kawasaki disease. Usefulness of pharmacologic stress 201Tl myocardial tomography

    This study determined the feasibility and accuracy of quantitative 201Tl myocardial single-photon emission computed tomography (SPECT) after dipyridamole infusion to detect coronary obstructive lesions in children with Kawasaki disease. 201Tl distribution after dipyridamole infusion was measured in 23 normal children, and with these normal values, quantitative analysis of SPECT was performed in 49 patients. Thirty-four patients had coronary stenosis 90% or greater on angiograms. Side effects resulting from systemic vasodilation were observed in about 70%. Angina pectoris and ischemic ST changes were observed only in patients with coronary stenosis. These symptoms disappeared after aminophylline infusion. Results of visual and quantitative analysis of SPECT were compared. SPECT data were shown on two-dimensional polar maps, and the extent and severity scores were calculated. The sensitivity of SPECT for detection of overall coronary stenosis was 91% (visual analysis) and 88% (quantitative analysis). The specificity of SPECT was 60% visually and 93% quantitatively. The sensitivity of quantitative analysis to detect individual coronary stenosis was similar to that of visual analysis. However, the specificity of visual analysis to detect individual coronary artery stenosis was significantly less than that of quantitative analysis. From these data, we conclude that quantitative analysis of myocardial SPECT after dipyridamole infusion is a safe and accurate diagnostic method for identifying coronary stenosis in children with Kawasaki disease

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

    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

  5. Facial Palsy in Kawasaki Syndrome; Review and a Case Report

    MH. Moradi Nejad

    2003-07-01

    Full Text Available Facial nerve palsy, a very rare complication of Kawasaki syndrome, has been reported in only 25 patents. None of the 25 previous reported cases with this complication was treated with IVIG. We treated a 24-month-old boy with bilateral coronary artery aneurysms due to Kawasaki syndrome. In our patient, whom we treated with Aspirin and IVIG, duration of disease was 7-9 days. He developed marked unilateral peripheral facial nerve palsy on day 3 of illness. This treatment led to complete resolution of facial nerve palsy within 48 hours. Review of pervious cases demonstrates that children with Kawasaki–associated facial nerve palsy have more than twice the risk for coronary artery aneurysm (52% vs 25% as that of children without facial nerve palsy. Unexplained facial nerve palsy in young children with a prolonged febrile illness should provoke consideration of Kawasaki syndrome and Echocardiography to exclude coronary artery aneurysms. Although facial nerve palsy appears likely to be resolved in all patients surviving the acute phase of Kawasaki syndrome, treatment with IVIG appears to shorten considerably the time for full recovery and provides an important clue to the mechanism of neurological injury in this illness.

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

    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.

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

    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

  8. Genome-Wide Association Study Identifies Novel Susceptibility Genes Associated with Coronary Artery Aneurysm Formation in Kawasaki Disease

    Guo, Mindy Ming-Huey; Huang, Ying-Hsien; Yu, Hong-Ren; Huang, Fu-Chen; Jiao, Fuyong; Kuo, Hsing-Chun; Andrade, Jorge

    2016-01-01

    Kawasaki disease (KD) or Kawasaki syndrome is known as a vasculitis of small to medium-sized vessels, and coronary arteries are predominantly involved in childhood. Generally, 20–25% of untreated with IVIG and 3–5% of treated KD patients have been developed coronary artery lesions (CALs), such as dilatation and aneurysm. Understanding how coronary artery aneurysms (CAAs) are established and maintained in KD patients is therefore of great importance. Upon our previous genotyping data of 157 valid KD subjects, a genome-wide association study (GWAS) has been conducted among 11 (7%) CAA-developed KD patients to reveal five significant genetic variants passed pre-defined thresholds and resulted in two novel susceptibility protein-coding genes, which are NEBL (rs16921209 (P = 7.44 × 10−9; OR = 32.22) and rs7922552 (P = 8.43 × 10−9; OR = 32.0)) and TUBA3C (rs17076896 (P = 8.04 × 10−9; OR = 21.03)). Their known functions have been reported to associate with cardiac muscle and tubulin, respectively. As a result, this might imply their putative roles of establishing CAAs during KD progression. Additionally, various model analyses have been utilized to determine dominant and recessive inheritance patterns of identified susceptibility mutations. Finally, all susceptibility genes hit by significant genetic variants were further investigated and the top three representative gene-ontology (GO) clusters were regulation of cell projection organization, neuron recognition, and peptidyl-threonine phosphorylation. Our results help to depict the potential routes of the pathogenesis of CAAs in KD patients and will facilitate researchers to improve the diagnosis and prognosis of KD in personalized medicine. PMID:27171184

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

    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

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

    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.

  11. Etanercept Suppresses Arteritis in a Murine Model of Kawasaki Disease: A Comparative Study Involving Different Biological Agents

    Ryuji Ohashi

    2013-01-01

    Full Text Available Coronary arteritis, a complication of Kawasaki disease (KD, can be refractory to immunoglobulin (IVIG treatment. To determine the most effective alternative therapy, we compared the efficacy of different agents in a mouse model of KD. Vasculitis was induced by injection of Candida albicans water-soluble fractions (CAWS into a DBA/2 mouse, followed by administration of IVIG, etanercept, methylprednisolone (MP, and cyclosporine-A (CsA. At 2 and 4 weeks, the mice were sacrificed, and plasma cytokines and chemokines were measured. CAWS injection induced active inflammation in the aortic root and coronary arteries. At 2 weeks, the vasculitis was reduced only by etanercept, and this effect persisted for the subsequent 2 weeks. At 4 weeks, IVIG and CsA also attenuated the inflammation, but the effect of etanercept was more significant. MP exerted no apparent effect at 2 or 4 weeks. The suppressive effect exerted by etanercept on cytokines, such as interleukin- (IL-6, IL-12, IL-13, and tumor necrosis factor-α (TNF-α, was more evident than that of others. The extent of arteritis correlated with the plasma TNF-α levels, suggesting a pivotal role of TNF-α in KD. In conclusion, etanercept was most effective in suppressing CAWS-induced vasculitis and can be a new therapeutic intervention for KD.

  12. Severe vitamin D deficiency in patients with Kawasaki disease: a potential role in the risk to develop heart vascular abnormalities?

    Stagi, Stefano; Rigante, Donato; Lepri, Gemma; Matucci Cerinic, Marco; Falcini, Fernanda

    2016-07-01

    Twenty-five-hydroxyvitamin D (25(OH)-vitamin D) is crucial in the regulation of immunologic processes, but-although its deficiency has been reported in patients with different rheumatological disorders-no data are available for Kawasaki disease (KD). The goals of this study were to assess the serum levels of 25(OH)-vitamin D in children with KD and evaluate the relationship with the eventual occurrence of KD-related vascular abnormalities. We evaluated serum 25(OH)-vitamin D levels in 79 children with KD (21 females, 58 males, median age 4.9 years, range 1.4-7.5 years) in comparison with healthy sex-/age-matched controls. A significantly higher percentage of KD patients (98.7 %) were shown to have reduced 25(OH)-vitamin D levels (vitamin D than controls (9.17 ± 4.94 vs 23.3 ± 10.6 ng/mL, p vitamin D levels correlated not only with erythrosedimentation rate (p vitamin D might have a contributive role in the development of coronary artery complications observed in children with KD. PMID:25994612

  13. Acute radiation disease

    Features of clinical trends in acute period of radiation disease at personnel who suffered from Chernobyl accident are considered. The main attention is paid to the results of 10 year observation of organs, systems and metabolic processes in patients. Used therapeutic, rehabilitation and preventive actions in stationary, ambulatory and sanatorium - health resort stages are described

  14. Granulocyte colony-stimulating factor ameliorates coronary artery elastin breakdown in a mouse model of Kawasaki disease

    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.

  15. Effect of pravastatin on endothelial dysfunction in children with medium to giant coronary aneurysms due to Kawasaki disease

    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.

  16. Analysis of the Clinical Nursing Experience of Children Kawasaki disease%浅谈小儿川崎病的临床护理体会

    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。

  17. 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

    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 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.

  18. Treatment response in Kawasaki disease is associated with sialylation levels of endogenous but not therapeutic intravenous immunoglobulin G.

    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.

  19. The Validity of a Scoring System in Predicting Intravenous Immunoglobulin Treatment Failure in Children With Kawasaki Disease

    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

  20. 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

    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.

  1. Measurements of Coronary Artery Aneurysms Due to Kawasaki Disease by Dual-Source Computed Tomography (DSCT).

    Tsujii, Nobuyuki; Tsuda, Etsuko; Kanzaki, Suzu; Kurosaki, Kenichi

    2016-03-01

    Diameters of coronary artery aneurysms (CAAs) complicating acute phase KD can strongly predict the long-term prognosis of coronary artery lesions (CAL). Recently, computed tomographic angiography (CTA) has been used to detect CAL, and the purpose of this study was to determine whether coronary artery diameters measurements by CTA using dual-source computed tomography (DSCT) can be used instead of coronary angiogram (CAG) measurements. Twenty-five patients (22 males and three females) with CAL due to KD, who had undergone both CTA and CAG within one year, were retrospectively evaluated between 2007 and 2013. A prospective electrocardiogram-triggered CTA was performed on a DSCT (SOMATOM(®) Definition, Siemens Healthcare, Germany). Two pediatric cardiologists independently measured the diameters of CAAs twice in each maximum intensity projection (MIP), curved multiplaner reconstruction (MPR) and CAG. We measured 161 segments in total (segment 1-3, 5-7, 11, 13). Diagnostic accuracy was expressed as κ coefficient. A Bland-Altman analysis was also used to assess the intra-observer, inter-observer and inter-modality agreement. The diagnostic quality of CTA was excellent (κ = 0.93). Excellent inter-observer agreement for the diameters of CAAs was obtained for MIP, MPR and CAG and for the intra-observer agreement. The inter-modality agreement was also excellent in measurements of CAA (MPR-CAG: y = 0.9x + 0.40, r = 0.97, p < 0.0001 MIP-CAG: y = x + 0.1, r = 0.94, p < 0.0001). These values in normal coronary arteries were also obtained. We found a significant correlation between CTA and CAG in measuring the coronary arteries. We conclude that measuring coronary artery diameters by CTA is reliable and useful. PMID:26515298

  2. Usefulness of 64-slice MDCT for follow-up of young children with coronary artery aneurysm due to Kawasaki disease: Initial experience

    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.

  3. 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.

    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 countries of origin seems due to underdiagnosis, which represents a public health concern in those countries. PMID:27336001

  4. Changes in endothelium-derived vascular regulatory factors during dobutamine-stress-induced silent myocardial ischemia in patients with Kawasaki disease.

    Hino, Y; Ohkubo, T; Katsube, Y; Ogawa, S

    1999-07-01

    The changes in endothelium-derived vascular regulatory factors during dobutamine (DOB)-induced myocardial ischemia (MI) were investigated in 21 patients with Kawasaki disease aged from 11 months to 18 years. They were classified into an ischemia group (8 patients) and a non-ischemia group (13 patients) based on the results of 99mTc myocardial scintigraphy and DOB stress 99mTc myocardial scintigraphy. In the ischemia group, MI was relatively mild, because there were ischemic changes on the electrocardiogram and no significant symptoms during DOB stress. Catheters were positioned near the orifice of the coronary artery (Ao) and at the coronary sinus (CS). Hemodynamics and the blood concentrations of lactic acid and endothelin-1, as well as NO3-, 6-keto-prostaglandin F1alpha, and thromboxane B2, (which are inactive metabolites of nitric oxide, prostaglandin I2 and thromboxane A2, respectively), were measured at rest and after DOB stress (maximum dose: 30 microg x kg(-1) x min(-1)). The CS/Ao ratio was determined for all parameters. The rate-pressure product, an index of work load, and the cardiac index were significantly increased by DOB stress in both groups. Coronary angiography showed no vasospasm of the epicardial coronary arteries before or after DOB stress in either group. The plasma concentrations of endothelin-1 and 6-keto-prostaglandin F1alpha were significantly increased after DOB stress in the ischemia group, but the serum concentration of NO did not increase. The lack of an increase in NO production during DOB stress may have contributed to the worsening of MI in patients with Kawasaki disease. PMID:10462014

  5. 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

    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.

  6. Kawasaki Disease (For Parents)

    ... dry, cracked lips swollen tongue with a white coating and big red bumps (called "strawberry tongue") sore, ... child is treated with intravenous (IV) doses of gamma globulin (purified antibodies), an ingredient of blood that ...

  7. 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

    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.

  8. Usefulness of Tc-99m HMPAO-labeled WBC heart scan to predict impaired ventricular function and coronary artery dilation in children with Kawasaki disease.

    Hsu, Hsiu-Bao; Fu, Yun-Ching; Tsai, Shih-Chuan; Yen, Ruoh-Fang; Hwang, Betau

    2003-11-01

    Forty-nine children with Kawasaki disease were included in this study. Based on the severity of carditis as determined by Tc-99m HMPAO-labeled WBC heart scan, the children were separated into two groups. Group A (n=24) had significant carditis and group B (n=25) did not have significant carditis. The left and right ventricular ejection fractions (LVEF and RVEF) were evaluated by equilibrium multigated blood pooling ventriculography. Diameters of the left and right coronary arteries (LCA and RCA) were measured by two-dimensional echocardiography. The results showed that group A had lower LVEF and RVEF, but larger diameters of the LCA and RCA than those of group B patients. The sensitivity of significant carditis to predict left ventricular dysfunction, right ventricular dysfunction, LCA dilation and RCA dilation was 88.9, 70.6, 75, and 71.4%, respectively; the specificities were 100, 100, 100, and 67.9%, respectively. Our findings suggest that significant carditis determined by Tc-99m HMPAO-labeled WBC heart scan can accurately predict impaired ventricular function and coronary artery dilation. PMID:14602219

  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

    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. Ultrasound in Acute Kidney Disease.

    Meola, Mario; Nalesso, Federico; Petrucci, Ilaria; Samoni, Sara; Ronco, Claudio

    2016-01-01

    Kidneys' imaging provides useful information in acute kidney injury (AKI) diagnosis and management. Today, several imaging techniques give information on kidneys anatomy, urinary obstruction, differential diagnosis between AKI and chronic kidney disease (CKD), renal blood flow and glomerular filtration rate. Ultrasound is a safe, non-invasive and repeatable imaging technique so it is widely used in the first level work-up of AKI. The utility of contrast-enhanced computed tomography and magnetic resonance imaging in AKI or in AKI during CKD is limited because of renal toxicity associated with contrast agents used. PMID:27169556

  11. Enfermedad de Kawasaki

    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%.

  12. Acute Chagas Disease in a Returning Traveler

    Carter, Yvonne L.; Juliano, Jonathan J; Montgomery, Susan P.; Qvarnstrom, Yvonne

    2012-01-01

    Acute Chagas disease is rarely recognized, and the risk for acquiring the disease is undefined in travelers to Central America. We describe a case of acute Chagas disease in a traveler to Costa Rica and highlight the need for increased awareness of this infection in travelers to Chagas-endemic areas.

  13. Focus on acute diarrhoeal disease

    Fabio Baldi; Maria Antonia Bianco; Gerardo Nardone; Alberto Pilotto; Emanuela Zamparo

    2009-01-01

    Diarrhoea is an alteration of normal bowel movement characterized by an increase in the water content,volume, or frequency of stools. Diarrhoea needs to be classified according to the trends over time (acute or chronic) and to the characteristics of the stools (watery, fatty, inflammatory). Secretory diarrhoeas,mostly acute and of viral aetiology in more than 70% of cases, are by far the most important subtype of diarrhoeas in terms of frequency, incidence and mortality (over 2.5 million deaths/year in developing countries). Natural and synthetic opiates such as morphine, codeine, and loperamide which react with endogenous opiates (enkephalins, beta-endorphins,dynorphins) mainly act on intestinal motility and slow down transit. An antidiarrhoeal drug developed in recent years, racecadotril, acts as an enkephalinase inhibitor.Clinical studies have shown that it is just as effective as loperamide in resolving acute diarrhoea but with greater reduction in pain and abdominal distension.Some studies have explored the prevalence of diarrhoea in old age. An epidemiological study carried out in Italy by 133 General Practitioners on 5515 elderly outpatients reported a prevalence of diarrhoea, defined according to the Rome criteria, of 9.1%. Infectious diseases (19%) and drug use (16%) were the most commoncauses of diarrhoea in old age. Regardless of the cause,the treatment of elderly patients with diarrhoea must include rehydration and nutritional support. Every year,more than 50 million tourists travel from industrialized countries to places where hygiene levels are poor. At least 75% of those travelling for short periods mention health problems, and in particular traveller's diarrhoea.

  14. Is acute recurrent pancreatitis a chronic disease?

    Mariani, Alberto; Testoni, Pier Alberto

    2008-01-01

    Whether acute recurrent pancreatitis is a chronic disease is still debated and a consensus is not still reached as demonstrated by differences in the classification of acute recurrent pancreatitis. There is major evidence for considering alcoholic pancreatitis as a chronic disease ab initio while chronic pancreatitis lesions detectable in biliary acute recurrent pancreatitis (ARP) seem a casual association. Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation, hereditary a...

  15. Obstructive lung disease in acute medical patients.

    Seemungal, T.; Harrinarine, R.; Rios, M.; Abiraj, V.; Ali, A.; Lacki, N.; Mahabir, N.; Ramoutar, V.; King, C. P.; Bhowmik, A.; Wedzicha, J A

    2008-01-01

    OBJECTIVES: To determine the proportion of adult medical patients who have chronic obstructive pulmonary disease (COPD), using the Global initiative for Chronic Obstructive Lung Disease guidelines (GOLD), and its relation to vascular disease. METHODS: This is a prospective cross-sectional study of adult patients admitted to acute medical wards. Interviewer administered questionnaire, anthropometric and spirometric measurements were done. RESULTS: Spirometry was performed in 720 acute admissio...

  16. Study on the Relationship Between Plasma Procalcitonin and Kawasaki Dis-ease Related with Bacterial Infection%血浆前降钙素与细菌性川崎病的关系研究

    徐小桥

    2014-01-01

    Objective To study the relationship between the level of plasma procalcitonin and kawasaki disease related with bacte-rial infection and its clinical value. Methods 86 cases of kawasaki disease diagnosed and treated in our hospital from June, 2011 to December, 2013 were selected as the object of the study by sampling and analyzed professionally and retrospectively. Results The plasma procalcitonin level of group with positive pathogens was significantly higher than that of group with negative pathogens. And plasma procalcitonin level of patients with bacterial infection was also significantly higher than that of patients with non-bac-terial infection, and the difference was statistically significant (P<0.05). Conclusion Plasma procalcitonin level is one of the main indicators for the diagnosis of kawasaki disease caused by bacterial infection or non-bacterial infection. It provides important refer-ence for the scientific diagnosis and treatment of the patients.%目的:研究血浆前降钙素水平与细菌感染相关川崎病的关系及临床价值。方法从该院2011年6月-2013年12月诊治的川崎病病例中,抽样选取86例作为该研究的对象,进行专业的回顾性分析。结果病原体阳性组的血浆前降钙素水平明显高于病原体阴性组,且细菌感染患者的血浆前降钙素水平也明显高于非细菌感染患者,差异有统计学意义(P<0.05)。结论血浆前降钙素水平是诊断细菌感染相关川崎病与非细菌感染相关川崎病的主要指标之一,为患者的科学诊治提供了重要的参考依据。

  17. Diarrheal Diseases - Acute and Chronic

    ... and drinking contaminated or raw foods and beverages. Screening/Diagnosis Most episodes of acute diarrhea resolve quickly without antibiotic therapy and with simple dietary modifications. See a ...

  18. Is acute recurrent pancreatitis a chronic disease?

    Alberto Mariani; Pier Alberto Testoni

    2008-01-01

    Whether acute recurrent pancreaUtis is a chronic disease is still debated and a consensus is not still reached as demonstrated by differences in the classification of acute recurrent pancreatitis.There is major evidence for considering alcoholic pancreatitis as a chronic disease ab initio while chronic pancreatitis lesions detectable in biliary acute recurrent pancreatitis (ARP) seem a casual association.Cystic fibrosis transmembrane con ductance regulator (CFTR) gene mutation,hereditary and obstructive pancreatitis seem an acute disease that progress to chronic pancreatitis,likely as a consequence of the activation and proliferation of pancreatic stellate cells that produce and activate collagen and therefore fibrosis.From the diagnostic point of view,in patients with acute recurrent pancreatitis Endoscopic ultrasound (EUS) seems the more reliable technique for an accurate evaluation and follow-up of some ductal and parenchymal abnormalities suspected for early chronic pancreatitis.

  19. 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

    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.

  20. Thrombolysis in Acute Cerebrovascular Disease

    刘泽霖

    2003-01-01

    @@ Large-scale trials have shown that thrombolytic therapy reduces mortality and preserves left ventricular function in patients with acute myocardial infarction (AMI). That's a rationale for the use of thrombolytic agents in the management of ischemic stroke.

  1. Consideraciones orales del síndrome de Kawasaki: Descripción de un caso clínico Oral considerations of Kawasaki syndrome: A case report

    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

  2. 运用Delphi法确立川崎病中医基本证候分类的研究%Using Delphi Method to Establish TCM syndrome classification of Kawasaki disease

    王文革; 申广生; 汪受传

    2011-01-01

    Objective: To screen out the TCM syndrome classification indices of Kawasaki disease by Delphi method.Methods:Two rounds of questionnaire survey were carried out by 40 experts according to Delphi method. The data was analyzed with SPSS software for positive degree, concentricity and coordination. Results:The recovery rate of the first round questionnaire was 90.0% and that of the second round was 92.5%. The variance coefficient of syndrome of disease involving weifen and qifen, syndrome of flaring heat in qifen and yingfen, syndrome of deficiency of both qi and yin were smaller than 0.20. The variance coefficient of syndrome of lingering heat due to deficiency of yin was 0.576 of the second round questionnaire. Conclusions:Syndrome of disease involving weifen and qifen, syndrome of flaring heat in qifen and yingfen, syndrome of deficiency of both qi and yin could be taken as the TCM syndrome classification indices of Kawasaki disease.%目的:运用德尔菲法(Delphi法)筛选川崎病中医基本证候分类的指标.方法:通过Delphi法对全国40位专家进行两轮问卷调查,利用SPSS软件,对问卷反馈的数据进行积极度、集中程度、协调程度的统计与评价.结果:第1轮问卷回收率90.0%,第2轮问卷回收率92.5%.第2轮问卷邪在卫气证、气营两燔证、气阴两伤证3项指标的变异系数(CV)均在0.20以内,热恋阴伤证的CV值为0.576.结论:邪在卫气证、气营两燔证、气阴两伤证可作为川崎病的基本证候分类.

  3. The diagnostic value of ultrasonic cardiogram in coronary artery lesions resulting from Kawasaki disease%超声心动图对小儿川崎病冠状动脉病变的诊断价值

    余启军

    2015-01-01

    Objectives:To explore the diagnostic value of ultrasound cardiogram in coronary artery lesions resulting from Kawasaki disease. Methods:Doppler ultrasound technique was used to detect 26 cases of coronary artery lesions resulting from Kawasaki disease treated from October 2006 to March 2014 and to observe the inner diameter and wall thickness of the coronary artery, echo intensity and whether there was an aneurysm formed. Results:The display rate was 100 % for the left main coronary artery and right coronary artery in the healthy group;15 cases (57. 7 %) of coronary artery lesions were detected in the group with coronary artery disease , including 14 cases of coronary artery diameter expansion, 2 cases of coronary artery inner diameter expansion with intimal thickening and pericardial effusion respectively, 1 case of right coronary artery aneurysm;the earliest onset of coronary artery lesions occurred in the first 5-7 days. Conclusion:It is safe, convenient, fast and visual to use ultrasound car-diogram for diagnosing coronary artery lesions resulting from Kawasaki disease, which is of diagnostic value. It can be considered to have the priority in the imaging test , but it's a bit difficult to check the damage to the distal coronary artery.%目的:探讨超声心动图对小儿川崎病冠状动脉病变的诊断价值。方法:以50例健康儿童的超声监测值为参照,应用多普勒超声技术对2006年10月至2014年3月进行治疗的26例川崎病冠状动脉病变的患儿进行检测,观察冠状动脉内径、冠状动脉壁厚度、回声的强度及有无动脉瘤形成。结果:健康组左冠状动脉主干及右冠脉显示率为100%,病变组检出冠状动脉病变15例(57.7%),其中冠状动脉内径扩张为14例,冠状动脉内径扩张合并内膜增厚为2例,合并心包积液2例,1例为右冠状动脉瘤。冠脉病变最早发生在起病5~7d。结论:超声心动图用于诊断和随访小儿川崎病冠状动脉病

  4. An Acute Hemorrhagic Infectious Disease: Ebola Virus Disease

    Lei JIAO

    2014-09-01

    Full Text Available Ebola virus disease (EVD is an acute hemorrhagic infectious disease caused by ebola virus, with high infectivity and fatality rate. At present, it mainly occurs in areas of Central Africa and West Africa and no effective vaccine and antiviral drugs are available for the clinical treatment.

  5. Microcirculation in Acute and Chronic Kidney Diseases.

    Zafrani, Lara; Ince, Can

    2015-12-01

    The renal microvasculature is emerging as a key player in acute and chronic kidney diseases. Renal microvascular disease involves alterations in endothelial barrier permeability, exaggerated inflammation, impairment of endothelium-dependent vasorelaxation involving the nitric oxide system, increased oxidative stress, and loss of angiogenic factors. Moreover, evidence suggests that there is a microvascular component to the pathogenesis of renal scarring. New technology is being developed to explore renal microcirculation in vivo in experimental models and humans. This technology will provide a better understanding of the pathogenesis of kidney diseases and will help guide specific therapeutic strategies aimed at restoring the renal microcirculation. This article reviews the cellular and molecular mechanisms of renal microvascular dysfunction in acute and chronic kidney diseases and the potential diagnostic and therapeutic implications of these findings. Recent developments in the monitoring of renal microcirculation are described with respect to their advantages and limitations, and future directions are outlined. PMID:26231789

  6. Crohn's Disease and Acute Pancreatitis: A Review of Literature

    Sarfaraz Jasdanwala; Mark Babyatsky

    2015-01-01

    Crohn's disease, a transmural inflammatory bowel disease, has many well-known extra-intestinal manifestations and complications. Although acute pancreatitis has a higher incidence in patients with Crohn's disease as compared to the general population, acute pancreatitis is still relatively uncommon in patients with Crohn's disease. Patients with Crohn's disease are at an approximately fourfold higher risk than the general population to develop acute pancreatitis. The risk of developing acute ...

  7. Strain Echocardiography in Acute Cardiovascular Diseases.

    Favot, Mark; Courage, Cheryl; Ehrman, Robert; Khait, Lyudmila; Levy, Phillip

    2016-01-01

    Echocardiography has become a critical tool in the evaluation of patients presenting to the emergency department (ED) with acute cardiovascular diseases and undifferentiated cardiopulmonary symptoms. New technological advances allow clinicians to accurately measure left ventricular (LV) strain, a superior marker of LV systolic function compared to traditional measures such as ejection fraction, but most emergency physicians (EPs) are unfamiliar with this method of echocardiographic assessment. This article discusses the application of LV longitudinal strain in the ED and reviews how it has been used in various disease states including acute heart failure, acute coronary syndromes (ACS) and pulmonary embolism. It is important for EPs to understand the utility of technological and software advances in ultrasound and how new methods can build on traditional two-dimensional and Doppler techniques of standard echocardiography. The next step in competency development for EP-performed focused echocardiography is to adopt novel approaches such as strain using speckle-tracking software in the management of patients with acute cardiovascular disease. With the advent of speckle tracking, strain image acquisition and interpretation has become semi-automated making it something that could be routinely added to the sonographic evaluation of patients presenting to the ED with cardiovascular disease. Once strain imaging is adopted by skilled EPs, focused echocardiography can be expanded and more direct, phenotype-driven care may be achievable for ED patients with a variety of conditions including heart failure, ACS and shock. PMID:26823931

  8. Acute dysautonomia associated with Hodgkin's disease.

    van Lieshout, J. J.; Wieling, W.; van Montfrans, G A; Settels, J J; Speelman, J D; Endert, E.; Karemaker, J. M.

    1986-01-01

    A patient is described with acute dysautonomia associated with Hodgkin's disease. Testing of cardiovascular reflex control showed that this patient had a rare manifestation of autonomic cardiovascular neuropathy, namely intact parasympathetic heart rate control in combination with a sympathetic postganglionic lesion affecting the control of the vascular tree.

  9. Minimal Residual Disease in Acute Myeloid Leukemia

    Ommen, Hans Beier; Nederby, Line; Toft-Petersen, Marie;

    2014-01-01

    This chapter discusses how minimal residual disease (MRD) is detected and managed in acute myeloid leukemia (AML) patients. The most commonly used techniques to detect residual leukemia in patients in complete remission (CR) are quantitative PCR (qPCR) and multicolor flow cytometry (MFC). While q...

  10. Cine-CT diagnosis of coronary artery aneurysms in patients with Kawasaki

    Coronary artery aneurysms (CAA) are the most serious late sequelae of Kawasaki disease, and periodic screening examinations are necessary. Two-dimensional (2D) echocardiography represents the standard screening method; however, visualization of the distal coronary arteries is often limited. This report documents the complimentary role of cine-CT with 2D echocardiography in the evaluation of CAA induced by Kawasaki disease. Six pediatric patients with coronary aneurysms were evaluated with both 2D echocardiography and cine-CT. Cine-CT detected ten of 11 lesions. The one misdiagnosed CAA was one of the two contiguous aneurysms. Owing to intersection thickness, these two discrete aneurysms were interpreted as a solitary aneurysm. Cine-CT detected one CAA not initially visualized on echocardiography. Cine-CT is effective and complementary to 2D echocardigraphy as a noninvasive method for the evaluation of coronary artery aneurysms occurring as sequelae of Kawasaki disease

  11. Kawasakis syndrom hos voksne

    Roed, Casper; Skinhøj, Peter

    2009-01-01

    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...

  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

    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. Treated with intravenous immunoglobulin in children with Kawasaki disease%川崎病的静脉注射免疫球蛋白治疗

    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。

  14. Acute recurrent pancreatitis: An autoimmune disease?

    Raffaele Pezzilli

    2008-01-01

    In this review article,we will briefly describe the main characteristics of autoimmune pancreatitis and then we will concentrate on our aim,namely,evaluating the clinical characteristics of patients having recurrence of pain from the disease.In fact,the open question is to evaluate the possible presence of autoimmune pancreatitis in patients with an undefined etiology of acute pancreatitis and for this reason we carried out a search in the literature in order to explore this issue.In cases of recurrent attacks of pain in patients with "idiopathic"pancreatitis,we need to keep in mind the possibility that our patients may have autoimmune pancreatitis.Even though the frequency of this disease seems to be quite low,we believe that in the future,by increasing our knowledge on the subject,we will be able to diagnose an ever-increasing number of patients having acute recurrence of pain from autoimmune pancreatitis.

  15. MINIMAL RESIDUAL DISEASE IN ACUTE LYMPHOBLASTIC LEUKEMIA

    Campana, Dario

    2009-01-01

    In patients with acute lymphoblastic leukemia (ALL), monitoring of minimal residual disease (MRD) offers a way to precisely assess early treatment response and detect relapse. Established methods to study MRD are flow cytometric detection of abnormal immunophenotypes, polymerase chain reaction (PCR) amplification of antigen-receptor genes, and PCR amplification of fusion transcripts. The strong correlation between MRD levels and risk of relapse in childhood ALL is well established; studies in...

  16. Crohn's Disease and Acute Pancreatitis: A Review of Literature

    Sarfaraz Jasdanwala

    2015-03-01

    Full Text Available Crohn's disease, a transmural inflammatory bowel disease, has many well-known extra-intestinal manifestations and complications. Although acute pancreatitis has a higher incidence in patients with Crohn's disease as compared to the general population, acute pancreatitis is still relatively uncommon in patients with Crohn's disease. Patients with Crohn's disease are at an approximately fourfold higher risk than the general population to develop acute pancreatitis. The risk of developing acute pancreatitis is higher in females as compared to males. Acute pancreatitis can occur at any age with higher incidence reported in patients in their 20s and between 40- 50 years of age. The severity and prognosis of acute pancreatitis in patients with Crohn's disease is the same as in general population. Acute pancreatitis can occur before onset of intestinal Crohn's disease, this presentation being more common in children than adults. It can also occur as the presenting symptom. However, most commonly it occurs after intestinal symptoms have manifest with a mean time interval between the initial presentation and development of acute pancreatitis being 2 years. There are several etiological factors contributing to acute pancreatitis in patients with crohn's disease. It is not clear whether acute pancreatitis is a direct extra-intestinal manifestation of Crohn's disease; however majority of the cases of acute pancreatitis in patients with Crohn's disease are due to GS and medications. Drugs used for the treatment of Crohn's disease that have been reported to cause acute pancreatitis include 5-ASA agents, azathioprine and 6 mercaptopurine, metornidazole and corticosteroids. Recent evidence has emerged correlating both type 1 and 2 autoimmune pancreatitis with Crohn's disease. Understanding the association between the two disease entities is key to effectively manage patients with Crohn's disease and acute pancreatitis.

  17. 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

    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.

  18. Transvaginal sonography of acute pelvic inflammatory disease

    To determine the value of transvaginal sonography in evaluating women with acute pelvic inflammatory disease (PID). Transvaginal sonography was performed in 25 patients with clinically suggested PID during recent 36 months. The sonographic findings of fallopian tubes and ovaries were analyzed and correlated with pathological findings of 2 fallopian tubes and 19 ovaries in 16 patients who had operations. The correct diagnosis of acute PID was made in 20/25 (80%) by transvaginal sonography. the abnormal sonographic findings of the fallopian tube include tubal thickening or dilatation with internal echo. The sensitivity, specificity, and accuracy for tubal abnormality were 88%, 96%, and 86% , respectively. Ovarian changes were seen on TVS in 14/19 (73%), which include multiple follicular enlargement in 5, tubo-ovarian complex in 9 (tubo-ovarian adhesion in 3, tubo-ovarian abscess in 6). At surgery, the ovay was not involved in all three women who showed tubo-ovarian adhesion on TVS. Among 6 women who showed tubo-ovarian abscess on TVS, tubo-ovarian abscess was confirmed in 3 and the remaining 3 had ovarian cysts. Trandvaginal sonography, a facilitative and accurate modality, is highly sensitive in detecting the abnormality of the tube and useful in differentiating the tubo-ovarian complex in patients with acute PID.

  19. 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

    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.

  20. Acute renal dysfunction in liver diseases

    2007-01-01

    Renal dysfunction is common in liver diseases, either as part of multiorgan involvement in acute illness or secondary to advanced liver disease. The presence of renal impairment in both groups is a poor prognostic indicator. Renal failure is often multifactorial and can present as pre-renal or intrinsic renal dysfunction. Obstructive or post renal dysfunction only rarely complicates liver disease. Hepatorenal syndrome (MRS) is a unique form of renal failure associated with advanced liver disease or cirrhosis, and is characterized by functional renal impairment without significant changes in renal histology. Irrespective of the type of renal failure, renal hypoperfusion is the central pathogenetic mechanism, due either to reduced perfusion pressure or increased renal vascular resistance. Volume expansion, avoidance of precipitating factors and treatment of underlying liver disease constitute the mainstay of therapy to prevent and reverse renal impairment. Splanchnic vasoconstrictor agents, such as terlipressin, along with volume expansion, and early placement of transjugular intrahepatic portosystemic shunt (TIPS) may be effective in improving renal function in HRS. Continuous renal replacement therapy (CRRT) and molecular absorbent recirculating system (MARS) in selected patients may be life saving while awaiting liver transplantation.

  1. Association between polymorphism ofGRIN3A gene and clinical characteristic in children with Kawasaki disease%GRIN3A基因多态性与川崎病的相关性分析

    金向群; 熊志; 祝莹; 童丽娜; 王立琼

    2015-01-01

    ObjectiveTo investigate the association between nucleotide polymorphisms ofGRIN3A gene and clinical characteristic of Kawasaki disease (KD) in children in Han population in central Chinese.MethodsA case-control study was performed. A total of 191 children with KD were recruited and 217 healthy children were served as controls. The distribution of SNP was determined by PCR-RFLP. Arterial lesions were detected by echocardiographic.ResultsThe distribution of three genotypes (CC, CG, GG) in SNP (rs7849782) was statistically difference between KD and control groups (P=0.034), and C allele was associated with KD susceptibility (OR=1.46, 95%CI: 1.10-1.92,P=0.007). In children with KD, the polymorphism of SNP loci was signiifcantly associated with oral mucosa lesions and coronary artery lesion (P0.05). The polymorphism of SNP loci was also associated with the levels of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) (P0.05);该位点的多态性与患者的血沉以及C反应蛋白(CRP)水平相关(P<0.05)。结论 GRIN3A基因SNP位点(rs7849782)多态性与KD的易感性相关,C等位基因为风险因子;且该SNP位点可能与KD患儿的口腔黏膜以及冠状动脉损伤等特征相关,并可能影响血沉以及CRP水平。

  2. A case of acute viral hepatitis interfering with acute fatty liver disease of pregnancy

    Abdulkadir Turgut

    2013-03-01

    Full Text Available Acute hepatitis A is a rarely seen infection during pregnancy.In terms of clinical and laboratory findings, it can beinterfere with acute fatty liver disease which can be quitemortal during pregnancy. Since liver function tests are elevatedin both conditions, hepatitis A infection should alsobe kept in mind in differential diagnosis. We present a 30year-old pregnant woman with 35 weeks of gestation whopresented to our clinic with a suspection of acute fattyliver disease but finally diagnosed as acute hepatitis A infection.J Clin Exp Invest 2013; 4 (1: 123-125Key words: Hepatitis A, pregnancy, acute fatty liver disease

  3. Pathophysiology of acute small bowel disease with CT correlation

    The objective of this article is to review the pathophysiology of acute small bowel diseases, and to correlate the mechanisms of disease with computed tomography (CT) findings. Disease entities will be classified into the following: immune mediated and infectious causes, vascular causes, mechanical causes, trauma, and others. Having an understanding of acute small bowel pathophysiology is a useful teaching tool, and can lead to imaging clues to the most likely diagnosis of acute small bowel disorders.

  4. Pathophysiology of acute small bowel disease with CT correlation

    Sarwani, N., E-mail: nsarwani@hmc.psu.ed [Department of Radiology, Section of Abdominal Imaging, Penn State Milton Hershey Medical Center, Hershey, PA (United States); Tappouni, R.; Tice, J. [Department of Radiology, Section of Abdominal Imaging, Penn State Milton Hershey Medical Center, Hershey, PA (United States)

    2011-01-15

    The objective of this article is to review the pathophysiology of acute small bowel diseases, and to correlate the mechanisms of disease with computed tomography (CT) findings. Disease entities will be classified into the following: immune mediated and infectious causes, vascular causes, mechanical causes, trauma, and others. Having an understanding of acute small bowel pathophysiology is a useful teaching tool, and can lead to imaging clues to the most likely diagnosis of acute small bowel disorders.

  5. Analysis of drug treatment of the coronary embolism in Kawasaki disease%川崎病冠状动脉血栓患儿8例药物治疗分析

    俞惠娟; 朱卫华

    2014-01-01

    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 dissolved in 7 days to more than 4 months. One case had recurrent coronary thrombosis after 4 months. One case died in 12 hours after thrombolysis. Conclusions Coronary thrombosis usually appears in the half year after the onset of KD coronary aneurysm. Thrombolysis therapy takes long time in Kawasaki patients. Breaking off of thrombus are rarely seen.%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

  6. 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

    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

  7. Acute rheumatic fever and rheumatic heart disease.

    Carapetis, Jonathan R; Beaton, Andrea; Cunningham, Madeleine W; Guilherme, Luiza; Karthikeyan, Ganesan; Mayosi, Bongani M; Sable, Craig; Steer, Andrew; Wilson, Nigel; Wyber, Rosemary; Zühlke, Liesl

    2016-01-01

    Acute rheumatic fever (ARF) is the result of an autoimmune response to pharyngitis caused by infection with group A Streptococcus. The long-term damage to cardiac valves caused by ARF, which can result from a single severe episode or from multiple recurrent episodes of the illness, is known as rheumatic heart disease (RHD) and is a notable cause of morbidity and mortality in resource-poor settings around the world. Although our understanding of disease pathogenesis has advanced in recent years, this has not led to dramatic improvements in diagnostic approaches, which are still reliant on clinical features using the Jones Criteria, or treatment practices. Indeed, penicillin has been the mainstay of treatment for decades and there is no other treatment that has been proven to alter the likelihood or the severity of RHD after an episode of ARF. Recent advances - including the use of echocardiographic diagnosis in those with ARF and in screening for early detection of RHD, progress in developing group A streptococcal vaccines and an increased focus on the lived experience of those with RHD and the need to improve quality of life - give cause for optimism that progress will be made in coming years against this neglected disease that affects populations around the world, but is a particular issue for those living in poverty. PMID:27188830

  8. 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

    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

  9. Efficacy of iodine-123-15-(p-iodophenyl)-3-R,S-methylpentadecanoic acid single photon emission computed tomography imaging in detecting myocardial ischemia in children with Kawasaki disease

    To evaluate its efficacy in detecting myocardial ischemia in children, iodine-123-labeled 15-(p-iodophenyl)-3-R,S-methylpentadecanoic acid (BMIPP) myocardial single photon emission computed tomography (SPECT) imaging was performed in 16 pediatric patients with Kawasaki disease (KD, 11 male, 5 female; mean age and range: 13 years 8 months and 8 years 11 months to 17 years 7 months). Five children with chest pain and no cardiac disease were studied as controls (2 male, 3 female; mean age and range: 13 years 4 months and 9 years 4 months to 17 years 11 months). Selective coronary angiography was also performed in the 16 patients to evaluate the location of coronary stenosis and coronary aneurysms. The SPECT images were expressed as polar maps (Bull's eye maps) and the 'defect' area was defined as where the uptake of BMIPP was less than the standardized BMIPP images of the 5 control children. In the 16 patients, 33 segments had coronary aneurysms and 10 (10/33: 30.3%) had significant coronary stenosis on selective coronary angiography. Nine of the 10 (90%) segments with significant coronary stenosis showed a defect on the BMIPP image whereas only 6 of the 23 (26.1%) segments without coronary stenosis showed a defect on BMIPP imaging. The sensitivity of BMIPP SPECT imaging for detection of coronary stenosis was 90% (9/10) and its specificity was 73.9% (17/23), whereas the sensitivity of 201Tl SPECT imaging was 80% (8/10) and its specificity was 60% (14/23). There was no significant difference between the BMIPP and 201Tl SPECT images in either the sensitivity or specificity for the detection of coronary stenosis. In the present series, only one case had discordant BMIPP uptake (BMIPP uptake201Tl uptake) in which there was a large coronary aneurysm and re-canalization after complete obstruction at segment 1 of the right coronary artery. This discordant BMIPP uptake reflects the possibility of ischemic but viable myocardium after re-canalization of a large aneurysm in KD

  10. A case of acute viral hepatitis interfering with acute fatty liver disease of pregnancy

    Abdulkadir Turgut; Ali Özler; Neval Yaman Görük; Senem Yaman Tunç; Nurullah Peker; Recep Tekin

    2013-01-01

    Acute hepatitis A is a rarely seen infection during pregnancy.In terms of clinical and laboratory findings, it can beinterfere with acute fatty liver disease which can be quitemortal during pregnancy. Since liver function tests are elevatedin both conditions, hepatitis A infection should alsobe kept in mind in differential diagnosis. We present a 30year-old pregnant woman with 35 weeks of gestation whopresented to our clinic with a suspection of acute fattyliver disease but finally diagnosed ...

  11. Acute Diarrhoeal Diseases Among Preschool Children in Western Maharashtra, India.

    Mahesh B Tondare , Vaishali V Raje, Satish V Kakade , Madhavi V Rayate

    2014-01-01

    "Background: Malnutrition and infectious diseases both occur in the same unfortunate children and together they play a major role in causing the high morbidity and mortality in them. Out of all the childhood illnesses, acute respiratory tract infections, diarrhoeal diseases and malnutrition are the principle causes of illness and death in the developing countries. Acute Diarrhoeal diseases (ADD’s) are reported to be the 2nd leading cause of child morbidity and mortality. Objectives: ...

  12. Giant aortic arch aneurysm complicating Kawasaki′s disease

    Kaouthar Hakim; Rafik Boussada; Lilia Chaker; Fatma Ouarda

    2014-01-01

    Kawasaki disease (KD) is a common acute vasculitis in pediatric population that usually involves small- and middle-sized arteries, commonly coronary arteries. Although the incidence and natural course of coronary aneurysms after KD are well documented in studies, related reports on peripheral arterial and aortic aneurysms are scarce. We report the occurrence of a giant aortic aneurysm involving the horizontal part of aortic arch in a 28-month-old boy diagnosed with KD. This complication was m...

  13. Minimal residual disease in acute lymphoblastic leukemia.

    Campana, Dario

    2009-01-01

    In patients with acute lymphoblastic leukemia (ALL), monitoring of minimal residual disease (MRD) offers a way to precisely assess early treatment response and detect relapse. Established methods to study MRD are flow cytometric detection of abnormal immunophenotypes, polymerase chain reaction (PCR) amplification of antigen-receptor genes, and PCR amplification of fusion transcripts. The strong correlation between MRD levels and risk of relapse in childhood ALL is well demonstrated; studies in adult patients also support its prognostic value. Hence, results of MRD studies can be used to select treatment intensity and duration, and to estimate the optimal timing for hematopoietic stem cell transplantation. Practical issues in the implementation of MRD assays in clinical studies include determining the most informative time point to study MRD and the levels of MRD that will trigger changes in treatment intensity, as well as the relative cost and informative power of different methodologies. The identification of new markers of leukemia and the use of increasingly refined assays should further facilitate routine monitoring of MRD and help to clarify the cellular and biologic features of leukemic cells that resist chemotherapy in vivo. PMID:19100372

  14. Pathophysiology of coronary artery disease leading to acute coronary syndromes

    Ambrose, John A; Singh, Manmeet

    2015-01-01

    Acute myocardial infarction (AMI) and sudden cardiac death (SCD) are among the most serious and catastrophic of acute cardiac disorders, accounting for hundreds of thousands of deaths each year worldwide. Although the incidence of AMI has been decreasing in the US according to the American Heart Association, heart disease is still the leading cause of mortality in adults. In most cases of AMI and in a majority of cases of SCD, the underlying pathology is acute intraluminal coronary thrombus f...

  15. ACUTE RESPIRATORY DISEASE AS THE DEBUT OF SYSTEMIC LUPUS ERYTHEMATOSUS

    A. Yu. Ischenko

    2015-01-01

    Full Text Available Systemic lupus erythematosus — a chronic autoimmune disease that is often associated with infectious processes. The paper presents two clinical cases of systemic lupus erythematosus , debuted with acute respiratory infection.

  16. [Acute pancreatitis with hypertriglyceridemia--an underestimated disease?].

    Wild, Wolfgang; Tajjiou, Morad; Ferschke, Melanie; Bormann, Fabian; Dörr, Pius; Schwarzbach, Matthias

    2016-01-01

    Hypertriglyceridemia is a rare, but since a long time well known etiology for acute pancreatitis. It could occure alone or coactive with other triggers like alcohlic excess. Nevertheless it found no approach to the current classifications and parameters of prognosis of the acute pancreatitis. We refer about two patients with hypertriglyceridemia and acute pancreatitis, whose initial disease was limited on the tail of the pancreas with just a circumscripted or--in the other case--no necrosis. However, in both cases and although a consequent treatment started immediately, a serious process developed including a life-threatening acute respiratory distress syndrome in one case, which necessitated an extracorporal membrane oxygenation. PMID:26710203

  17. Acute Demyelinating Disease after Oral Therapy with Herbal Extracts

    Alex Kostianovsky

    2011-06-01

    Full Text Available Central nervous system demyelinating processes such as multiple sclerosis and acute disseminated encephalomyelitis constitute a group of diseases not completely understood in their physiopathology. Environmental and toxic insults are thought to play a role in priming autoimmunity. The aim of the present report is to describe a case of acute demyelinating disease with fatal outcome occurring 15 days after oral exposure to herbal extracts.

  18. 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

    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,

  19. [Demyelinating diseases in children with acute neurological symptoms].

    Olofsson, Isa Amalie; Skov, Liselotte; Miranda, Maria Jose

    2015-12-01

    Demyelinating diseases in children is a broad group of illnesses, which affect the central nervous system. Demyelinating diseases can be monophasic or chronic and comprise acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, multiple sclerosis and neuromyelitis optica. Demyelinating diseases are rare, but it is important for the physician to recognize these diseases, as well as to understand the differential diagnoses. This review summarizes the current knowledge of demyelinating disorders in children, focusing on an approach to diagnosis and management. PMID:26651911

  20. BLK基因多态性与川崎病及其临床特点的相关性%Association between gene polymorphism ofBLK gene and clinical characteristic in Kawasaki disease

    董启忠; 张超; 李月茹

    2015-01-01

    ObjectiveTo investigate the association of two single nucleotide polymorphisms (SNPs) (rs2736340 and rs2618476) inBLK gene with Kawasaki disease (KD) and coronary artery lesion in Chinese Han children.MethodsIn the case-control study, 179 children with KD were selected and 182 healthy check-up children during the same period were selected as normal controls. The genotypes of two SNPs inBLK gene were detected using PCR-RFLP and the data were analyzed. ResultsThere was no difference in distribution of three genotypes (TT, CT, and CC) of SNP rs2736340 between KD group and control group (P=0.093). However, T allele frequency in KD group was signiifcantly higher than that in control group (P=0.021). The distribution of three genotypes (CC, CT, and TT) of SNP rs2618476 between KD group and control group was signiifcantly different (P=0.021). C allele frequency in KD group was signiifcantly higher than that in control group (P=0.006). The two SNPs inBLK gene were not associated with rash, hand-foot edema and coronary artery lesion (CAL), but SNP (rs2618476) was asso-ciated with oral mucosa lesions (P=0.018).ConclusionsThe SNP (rs2736340) inBLK gene was not associated with KD, but the T-allele was associated with KD. The SNP (rs2618476) was associated with KD in Han Chinese, and was also associated with oral mucosa lesions in KD patients.%目的:探讨汉族人群中BLK基因2个SNP位点rs2736340和rs2618476的多态性与川崎病(KD)以及动脉损伤的相关性。方法采取病例对照研究方法,分别选取179例KD患儿和同期182例体检正常儿童作为研究对象。利用PCR-RFLP的方法测定BLK基因两个SNP位点多态性分布,并进行统计分析。结果 SNP位点(rs2736340)3种基因型(TT、CT和CC)在KD组与对照组之间分布的差异无统计学意义(P=0.093);但KD患儿T等位基因频率高于对照组,差异有统计意义(P=0.021)。SNP位点(rs2618476)3种基因型(CC、CT、TT)分布,在KD患儿与对照组之间

  1. Cerebrospinal Fluid Proteome of Patients with Acute Lyme Disease

    Angel, Thomas E.; Jacobs, Jon M.; Smith, Robert P.; Pasternack, Mark S.; Elias, Susan; Gritsenko, Marina A.; Shukla, Anil K.; Gilmore, Edward C.; McCarthy, Carol; Camp, David G.; Smith, Richard D.

    2012-10-05

    Acute Lyme disease results from transmission of and infection by the bacterium Borrelia burgdorferi following a tick bite. During acute infection, bacteria can disseminate to the central nervous system (CNS) leading to the development of Lyme meningitis. Here we have analyzed pooled cerebrospinal fluid (CSF) allowing for a deep view into the proteome for a cohort of patients with early-disseminated Lyme disease and CSF inflammation leading to the identification of proteins that reflect host responses, which are distinct for subjects with acute Lyme disease. Additionally, we analyzed individual patient samples and quantified changes in protein abundance employing label-free quantitative mass spectrometry based methods. The measured changes in protein abundances reflect the impact of acute Lyme disease on the CNS as presented in CSF. We have identified 89 proteins that differ significantly in abundance in patients with acute Lyme disease. A number of the differentially abundant proteins have been found to be localized to brain synapse and thus constitute important leads for better understanding of the neurological consequence of disseminated Lyme disease.

  2. Blocking Interleukin-1β in Acute and Chronic Autoinflammatory Diseases

    Dinarello, Charles A

    2011-01-01

    An expanding spectrum of acute and chronic inflammatory diseases are considered “autoinflammatory” diseases. This review considers autoinflammatory diseases as being distinct from “autoimmune” diseases. Autoimmune diseases are associated with dysfunctional T-cells and treated with “biologicals” including anti-TNFα, CTLA-Ig, anti-IL-12/23, anti-CD20, anti-IL-17 and anti-IL-6 receptor. In contrast, autoinflammatory diseases are uniquely due to a dysfunctional monocyte caspase-1 activity and sec...

  3. Acute Chagas Disease Induces Cerebral Microvasculopathy in Mice

    Nisimura, Lindice Mitie; Estato, Vanessa; de Souza, Elen Mello; Reis, Patricia A.; Lessa, Marcos Adriano; de Castro-Faria-Neto, Hugo Caire; Pereira, Mirian Claudia de Souza; Tibiriçá, Eduardo; Garzoni, Luciana Ribeiro

    2014-01-01

    Cardiomyopathy is the main clinical form of Chagas disease (CD); however, cerebral manifestations, such as meningoencephalitis, ischemic stroke and cognitive impairment, can also occur. The aim of the present study was to investigate functional microvascular alterations and oxidative stress in the brain of mice in acute CD. Acute CD was induced in Swiss Webster mice (SWM) with the Y strain of Trypanosoma cruzi (T. cruzi). Cerebral functional capillary density (the number of spontaneously perf...

  4. Intestinal acute graft versus host disease

    Rovani, C; Danse, E; Dragean, C

    2010-01-01

    Background: We present a case of a 23-year-old man having drug addiction. He is managed by the department of hematology for an idiopathic medullar aplasia. Two months before his admission, he was treated with bone marrow transplantation. During follow-up, we noted a nonspecific recent cutaneous lesion. He was admitted for acute digestive symptoms including anorexia, nausea, vomiting and abdominal pain.

  5. Relationship between acute and chronic disease epidemiology.

    Kuller, L.H. (Lewis H.)

    1987-01-01

    Epidemiology is the study of epidemics. The primary goal of epidemiological studies should be the identification of the determinants of disease in order to decrease morbidity and mortality. Epidemiological studies evolve through descriptive, analytical, and experimental approaches. The traditional infectious disease epidemiology studies were primarily concerned with identification of an agent, incubation period, mode of transmission, population at risk, and methods of disease control. Chronic...

  6. Spectrum of diseases in acute intestinal obstruction

    To determine the etiological spectrum of acute intestinal obstruction in our clinical setup Military Hospital Rawalpindi. Study Design: Descriptive study. Place and Duration of Study: Surgical department of Military Hospital, Rawalpindi from Jul 2012 to Jul 2013, over a period of about 1 year. Material and Methods: A total of 120 patients with acute mechanical intestinal obstruction who underwent laparotomy were included in our study while those with non-mechanical intestinal obstruction like history of trauma and paralytic ileus were excluded from the study. All the patients were selected by non-probability purposive sampling technique. Emergency laparotomy was done and operative findings were recorded. Results: A total of 120 patients with mechanical intestinal obstruction were included in this study out of which 93 (69.17%) were female and remaining 27 (30.83%) were males. Male to female ratio was 1:2.24. Age range of patients was 22-85 years. Out of 120 patients operated for acute intestinal obstruction post-op adhesions were found in 37 (30.83%) patients followed by intestinal tuberculosis in 23 (19.17%) patients, obstructed inguinal hernias in 13 (10.83%), gut malignancies in 15 (12.5%) , Meckel's diverticulum with bands in 7 (5.83%), volvulus in 7 (5.83%), perforated appendix in 6 (5%), intussusception in 2 (1.7%), inflammatory bands in 5 (4.17%), trichobezoar and faecal impaction in 2 (1.7%) while in 3 (2.5%) patients no definite cause was found. Conclusion: Post-op adhesions are the commonest cause of mechanical intestinal obstruction in our setup followed by intestinal tuberculosis as second most common clinical pattern of presentation. (author)

  7. Blood transfusion for the treatment of acute anaemia in inflammatory bowel disease and other digestive diseases

    García-Erce, José Antonio; Gomollón, Fernando; Muñoz, Manuel

    2009-01-01

    Allogeneic blood transfusion (ABT) is frequently used as the first therapeutic option for the treatment of acute anaemia in patients with inflammatory bowel disease (IBD), especially when it developed due to gastrointestinal or perioperative blood loss, but is not risk-free. Adverse effects of ABT include, but are not limited to, acute hemolytic reaction (wrong blood or wrong patient), febrile non-hemolytic transfusional reaction, bacterial contamination, transfusion-related acute lung injury...

  8. Changes of myeloid related protein-8/myeloid related protein-14 expressions in children with Kawasaki disease%川崎病患儿外周血髓细胞相关蛋白-8/髓细胞相关蛋白-14的表达变化

    钱为国; 范秋红; 严文华; 吕海涛; 孙凌; 黄洁

    2013-01-01

    目的 观察髓细胞相关蛋白-8(MRP-8)/髓细胞相关蛋白-14(MRP-14)异二聚体在川崎病(KD)患儿血清中的表达变化,寻求KD的实验室诊断指标及药物治疗新靶点.方法 选择2009年7月至2010年12月确诊为KD的患儿46例为KD组,根据心脏彩超评定标准又分为冠状动脉扩张组(15例)和冠状动脉未扩张组(31例).选择同期住院有呼吸道感染而无心脏、血液、免疫系统等疾病的发热患儿25例为非KD发热组,选择同期本院门诊体检健康儿童20例为健康对照组.急性期及亚急性期分别采集外周静脉血,采用ELISA法测定血清MRP-8/MRP-14水平,采用半定量RT-PCR法检测白细胞中MRP-8 mRNA和MRP-14 mRNA相对水平.结果 KD组急性期和亚急性期MRP-8/MRP-14水平、MRP-8 mRNA和MRP-14 mRNA相对水平均明显高于非KD发热组和健康对照组(P均<0.05),非KD发热组与健康对照组比较差异无统计学意义(P>0.05).KD组急性期MRP-8/MRP-14水平、MRP-8 mRNA和MRP-14 mRNA相对水平均明显高于亚急性期(P均<0.001).在急性期及亚急性期冠状动脉扩张组血清MRP-8/MRP-14水平及白细胞MRP-8 mRNA和MRP-14 mRNA相对水平均明显高于同期冠状动脉未扩张组(P<0.05).结论 MRP-8/MRP-14可能参与KD血管炎的病理过程,并参与冠状动脉损害,可作为KD诊断及预测冠状动脉损害的指标之一,可为KD的治疗提供新靶点.%Objective To investigate the expression changes of myeloid-related protein-8 (MRP-8) and myeloid-related protein-14 (MRP-14) in children with Kawasaki disease (KD) and to obtain laboratory diagnostic serum markers and new targets for its drug therapy.Methods A total of 46 patients with KD(KD group) were enrolled from Jul.2009 to Dec.2010 and divided into the coronary artery dilatation(CAD) group(n =15) and the normal coronary artery group(n =31) ;Meanwhile,25 febrile patients with acute respiratory tract infection but without disease in the circulatory

  9. Is Progressive Chronic Kidney Disease a Slow Acute Kidney Injury?

    Cowgill, Larry D; Polzin, David J; Elliott, Jonathan; Nabity, Mary B; Segev, Gilad; Grauer, Gregory F; Brown, Scott; Langston, Cathy; van Dongen, Astrid M

    2016-11-01

    International Renal Interest Society chronic kidney disease Stage 1 and acute kidney injury Grade I categorizations of kidney disease are often confused or ignored because patients are nonazotemic and generally asymptomatic. Recent evidence suggests these seemingly disparate conditions may be mechanistically linked and interrelated. Active kidney injury biomarkers have the potential to establish a new understanding for traditional views of chronic kidney disease, including its early identification and possible mediators of its progression, which, if validated, would establish a new and sophisticated paradigm for the understanding and approach to the diagnostic evaluation, and treatment of urinary disease in dogs and cats. PMID:27593574

  10. Acute Diarrhoeal Diseases Among Preschool Children in Western Maharashtra, India.

    Mahesh B Tondare , Vaishali V Raje, Satish V Kakade , Madhavi V Rayate

    2014-01-01

    Full Text Available "Background: Malnutrition and infectious diseases both occur in the same unfortunate children and together they play a major role in causing the high morbidity and mortality in them. Out of all the childhood illnesses, acute respiratory tract infections, diarrhoeal diseases and malnutrition are the principle causes of illness and death in the developing countries. Acute Diarrhoeal diseases (ADD’s are reported to be the 2nd leading cause of child morbidity and mortality. Objectives: To study the attack rate of Acute Diarrhoeal Disease among pre-school children and to study the socio-demographic variables of pre-school children suffering from Acute Diarrhoeal Disease. Methods: A Longitudinal study was conducted among preschool children (3-5years who were selected from Private pre-primary school of urban area and followed for the period of one year. Mother/guardian/teacher was interviewed by using pre-tested proforma during this period. Results: About 56% of children found suffering from ADD with 0.6 episodes per children per year among private pre-primary school. Higher proportions of ADD affected children were residing in nuclear type of family, belonging to middle socio-economic class, mothers were literate & housewives, born with order >2 compared to non ADD affected children. Conclusion: Maximum number of children from private pre-primary schools suffered with nearly one attack of Acute Diarrhoeal Disease with maternal illiteracy and working mothers found favorable factors. Immunization coverage, EBF and proper weaning play a very important role in prevention of infections."

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

    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.

  12. Oxygen therapy in acute exacerbations of chronic obstructive pulmonary disease

    Wedzicha, Wisia

    2014-01-01

    Simon E Brill, Jadwiga A Wedzicha Airway Disease Section, National Heart and Lung Institute, Imperial College, London, UK Abstract: Acute exacerbations of chronic obstructive pulmonary disease (COPD) are important events in the history of this debilitating lung condition. Associated health care utilization and morbidity are high, and many patients require supplemental oxygen or ventilatory support. The last 2 decades have seen a substantial increase in our understanding of the best way to ma...

  13. Acute type II cryoglobulinaemic vasculitis mimicking atherosclerotic peripheral vascular disease.

    Saeed, A

    2012-01-31

    Atherosclerotic peripheral vascular disease is a common presenting cause for digital ischaemia in life long smokers. Acute severe Type II Cryoglobulinaemic vasculitis is a rare yet important cause, which may present with similar clinical features and which if undiagnosed may be rapidly fatal. Following the instigation of therapy with intravenous methylprednisolone and cyclophosphamide this patient made an excellent recovery.

  14. [Acute cardiovascular disease and job retention].

    Fantoni-Quinton, Sophie; Tellart, Anne-Sophie; Cambier-Langrand, Evodie; Fassier, Jean Baptiste; Mounier-Vehier, Claire

    2016-05-01

    Since it allows a better quality of life, return to work must be considered ever since the early stages of the health care pathway following a cardiovascular disease. Seeing the occupational physician beforehand, so as to anticipate the return to work, is crucial. Dialogue between cardiologists, general practitioners and occupational physician, still observing medical confidentiality, must allow a better quality of return to work. Being recognized as a handicapped worker is a key element in the prevention of socio-professional exclusion. Even when dealing with long sick leave, permanent functional injuries or job loss, guiding the patients towards the appropriate person can improve return to work and job retention in the long term. PMID:27021479

  15. Pulmonary thromboembolic disease. Clinical management of acute and chronic disease.

    Torbicki, Adam

    2010-07-01

    Pulmonary thromboembolism falls between the areas of pulmonology and cardiology, internal medicine and intensive care, radiology and nuclear medicine, and hematology and cardiothoracic surgery. Depending on their clinical background, physicians faced with a patient with a pulmonary thromboembolism may speak different languages and adopt different treatment approaches. Now, however, there is an opportunity to end the Tower of Babel surrounding pulmonary thromboembolism. There is a growing acknowledgement that the key clinical problems in both acute pulmonary embolism and chronic thromboembolic pulmonary hypertension are linked to right ventricular pressure overload and right ventricular failure. As a result, cardiologists and cardiac intensive care specialists are taking an increasing interest in understanding and combating these conditions. The European Society of Cardiology was the first to elaborate comprehensive clinical practice guidelines for pulmonary thromboembolism and chronic thromboembolic pulmonary hypertension. The task forces involved in producing these guidelines included radiologists, pulmonologists, hematologists, intensive care physicians and surgeons, which ensured that the final document was universally acceptable. The aim of this article was to provide an overview of the epidemiology, risk factors, diagnosis, treatment, prognosis and prevention of acute pulmonary thromboembolism and chronic thromboembolic pulmonary hypertension, while taking into account European Society of Cardiology guidelines and incorporating new evidence where necessary. PMID:20609317

  16. Acute renal failure: outcomes and risk of chronic kidney disease.

    Block, C A; Schoolwerth, A C

    2007-09-01

    Acute renal failure (ARF) is a common condition, especially among the critically ill, and confers a high mortality. The incidence of ARF is increasing. Efforts such as the Acute Dialysis Quality Initiative (ADQI) are being undertaken to establish a consensus definition of ARF, and to distinguish between varying degrees of acute kidney injury that might confer a different prognosis. Data are emerging to allow comparison of the epidemiology of ARF across institutions internationally. There is ongoing recognition of the important interaction between ARF and chronic kidney disease and more information regarding recovery from ARF is available. Controversy exists regarding the optimal management of ARF. Recent publications emphasize the importance of timing and dose of renal replacement therapy rather than the modality of treatment (intermittent hemodialysis vs continuous therapies). These issues are explored in this review. PMID:17912228

  17. Managing acute and chronic renal stone disease.

    Moran, Conor P; Courtney, Aisling E

    2016-02-01

    Nephrolithiasis, or renal stone disease, is common and the incidence is increasing globally. In the UK the lifetime risk is estimated to be 8-10%. On a population level, the increase in stone incidence, erosion of gender disparity, and younger age of onset is likely to reflect increasing prevalence of obesity and a Western diet with a high intake of animal protein and salt. Stones can be detected by a variety of imaging techniques. The gold standard is a non-contrast CT of kidneys, ureters and bladder (CT KUB) which can identify > 99% of stones. CT KUB should be the primary mode of imaging for all patients with colic unless contraindicated. In such instances, or if a CT KUB is not available, an ultrasound KUB is an alternative. This has advantages in terms of radiation exposure and cost, but is limited in sensitivity, particularly for ureteric stones. Once diagnosed, a plain film KUB can be used for follow-up of radiopaque stones. For most patients diclofenac is a reasonable first choice of analgesia, e.g. 50-100 mg rectally, or 75 mg IM. Opioid medication can worsen nausea and be less effective, but should be used if there is a contraindication to NSAIDs. A combination of diclofenac, paracetamol, and/or codeine regularly can provide adequate pain control in many cases. Failure of this analgesic combination should prompt consideration of secondary care support. If a ureteric stone 10 mm in diameter should be discussed with the urology service as they are unlikely to pass spontaneously. PMID:27032222

  18. Type 2 Gaucher Disease (Acute Infantile Gaucher Disease or Neuropathic Type

    Mohammad Mehdi TAGHDIRI

    2012-12-01

    Full Text Available How to Cite this Article: Taghdiri MM. Type 2 Gaucher Disease (Acute Infantile Gaucher Disease or Neuropathic Type. Iran J Child Neurol Autumn 2012; 6:4 (suppl. 1:12. Pls see PDF. 

  19. Minimal Residual Disease Assessment in Childhood Acute Lymphoblastic Leukemia

    Thörn, Ingrid

    2009-01-01

    Traditionally, response to treatment in hematological malignancies is evaluated by light microscopy of bone marrow (BM) smears, but due to more effective therapies more sensitive methods are needed. Today, detection of minimal residual disease (MRD) using immunological and molecular techniques can be 100 times more sensitive than morphology. The main aim of this thesis was to compare and evaluate three currently available MRD methods in childhood acute lymphoblastic leukemia (ALL): (i) real-t...

  20. Aspirin as Primary Prevention of Acute Coronary Heart Disease Events

    Glasser, Stephen P.; Hovater, Martha; Brown, Todd M.; Howard, George; Safford, Monika M.

    2014-01-01

    Background/Objective Aspirin for primary prophylaxis is controversial. This study evaluated associations between prophylactic aspirin use and incident acute coronary heart disease (CHD) events. Methods and Results The Reasons for Geographic and Racial Differences in Stroke (REGARDS) Study was accessed for aspirin use examining black and white hazards for incident CHD, for men and women, each adjusting incrementally for sampling, sociodemographics, and CHD risk factors. Stratified models exami...

  1. Investigation of the acute inflammatory response in Crohn's disease.

    MARKS, D. J. B.

    2006-01-01

    Most theories concerning the primary cause of Crohn's disease focus on over-activation of the immune response. Paradoxically, the defect may instead relate to diminished acute inflammation. Neutrophil accumulation to sites of dermal trauma has been shown to be reduced. Were the same phenomenon to occur in the gut, it might impair bacterial clearance thus provoking granuloma formation. In this thesis, a novel technique demonstrated attenuated neutrophil accumulation following trauma to the bow...

  2. Assessment of Coronary Artery Aneurysms Cased By Kawasaki Diesease Using Transluminal Attenuation Gradient Analysis of CT Angiograms

    Gutierrez, Noelia Grande

    2015-01-01

    Patients with coronary artery aneurysms (CAA) resulting from Kawasaki Disease (KD) are at risk for thrombosis and myocardial infarction. Current guidelines recommend using CAA diameter >8 mm as the criterion for initiating systemic anticoagulation, but there is little outcome data to support this choice. Transluminal Attenuation Gradient (TAG) has been proposed as a non-invasive method for evaluating the functional significance of coronary stenoses using CT Angiography (CTA). However TAG has ...

  3. Metabolomics and Its Application to Acute Lung Diseases.

    Stringer, Kathleen A; McKay, Ryan T; Karnovsky, Alla; Quémerais, Bernadette; Lacy, Paige

    2016-01-01

    Metabolomics is a rapidly expanding field of systems biology that is gaining significant attention in many areas of biomedical research. Also known as metabonomics, it comprises the analysis of all small molecules or metabolites that are present within an organism or a specific compartment of the body. Metabolite detection and quantification provide a valuable addition to genomics and proteomics and give unique insights into metabolic changes that occur in tangent to alterations in gene and protein activity that are associated with disease. As a novel approach to understanding disease, metabolomics provides a "snapshot" in time of all metabolites present in a biological sample such as whole blood, plasma, serum, urine, and many other specimens that may be obtained from either patients or experimental models. In this article, we review the burgeoning field of metabolomics in its application to acute lung diseases, specifically pneumonia and acute respiratory disease syndrome (ARDS). We also discuss the potential applications of metabolomics for monitoring exposure to aerosolized environmental toxins. Recent reports have suggested that metabolomics analysis using nuclear magnetic resonance (NMR) and mass spectrometry (MS) approaches may provide clinicians with the opportunity to identify new biomarkers that may predict progression to more severe disease, such as sepsis, which kills many patients each year. In addition, metabolomics may provide more detailed phenotyping of patient heterogeneity, which is needed to achieve the goal of precision medicine. However, although several experimental and clinical metabolomics studies have been conducted assessing the application of the science to acute lung diseases, only incremental progress has been made. Specifically, little is known about the metabolic phenotypes of these illnesses. These data are needed to substantiate metabolomics biomarker credentials so that clinicians can employ them for clinical decision-making and

  4. 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

    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。

  5. 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

    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

  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

    刘俊; 于明华; 许开元; 向峰; 周乾潮

    2015-01-01

    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 (21.9%) were involved by 320-detector row CTA. The distribution was consistent with that of TTE. Mean-while, there were one case of left circumlfex artery, one case of GCAA at distal of the right coronary artery, 2 cases of thrombus, 1 case of coronary stenosis and 2 cases of calciifcation.ConclusionsCAA caused by KD may be persistent for a long time. The thrombus, stenosis, and calciifcation of coronary can occurr at late phase in GCAA. TTE is sensitive and reliable to detect proxi-mal and middle segment of coronary lesions, but has limitations in detection of distal segment of coronary arteries. 320-detector row CTA has more comprehensively view of each coronary artery lesions and is especially sensitive and reliable to detect coro-nary thrombosis, calciifcation and narrowing in proximal and distal coronary arteries after acute phase.%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

  7. 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

    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

  8. Noninvasive imaging in acute coronary disease. A clinical perspective

    Numerous highly complex and sensitive noninvasive imaging techniques have enhanced the care of patients with acute myocardial infarction. Optimum use requires specific objectives to be defined in advance, including a review of the potential impact of the test on subsequent decisions. An additional issue that is subject to scrutiny in the current climate of cost containment relates to the incremental value of a specific examination. The imaging modality to be used will partially depend on other issues, including accessibility, cost, and interindividual or institutional expertise with a particular technique. Major applications in noninvasive imaging in the acute coronary syndromes include the following: (1) diagnosis, including identification of associated diseases and contraindications for acute reperfusion; (2) evaluation and management of complications; (3) determination of prognosis (both early and late); (4) estimation of myocardial viability; (5) assessment of therapeutic efficacy; (6) investigational approaches, including 99mTc-sestamibi tomographic imaging, ultrafast cine computed tomographic scanning, and nuclear magnetic resonance imaging. Previous studies in the prethrombolytic era have documented the powerful impact of radionuclide stress testing on prognosis, but this needs to be reevaluated in the light of the changing current population undergoing stress testing. Preliminary data imply that the prognostic accuracy of stress testing after thrombolytic therapy is diminished. Moreover, the role of the open infarct-related artery in traditional estimates of prognosis requires further study. Noninvasive imaging has multiple applications in the diagnosis and management of patients with acute coronary disease, but the decision to use a specific technology in a particular circumstance mandates good clinical judgment and selectivity. 82 references

  9. Compared with parenteral nutrition, enteral feeding attenuates the acute phase response and improves disease severity in acute pancreatitis

    Windsor, A; Kanwar, S; Li, A.; Barnes, E.; Guthrie, J; Spark, J; Welsh, F.; Guillou, P; Reynolds, J

    1998-01-01

    Background—In patients with major trauma and burns, total enteral nutrition (TEN) significantly decreases the acute phase response and incidence of septic complications when compared with total parenteral nutrition (TPN). Poor outcome in acute pancreatitis is associated with a high incidence of systemic inflammatory response syndrome (SIRS) and sepsis. 
Aims—To determine whether TEN can attenuate the acute phase response and improve clinical disease severity in patients with ac...

  10. Lithium-induced minimal change disease and acute kidney injury

    Parul Tandon

    2015-01-01

    Full Text Available Context: Lithium carbonate is a psychiatric medication commonly used in the treatment of bipolar disorder. It has been implicated in inducing nephrogenic diabetes inspidus, chronic tubulointerstitial nephropathy, and acute tubular necrosis. We describe a case of lithium-induced minimal change disease (MCD and acute kidney injury (AKI. Case Report: A 32-year-old female with a medical history of bipolar disorder treated with chronic lithium therapy presented with anasarca, fatigue, and tremors. Work-up revealed supra-therapeutic lithium levels, hypoalbuminemia, and significant proteinuria. The patient was treated conservatively with fluids and discontinuation of lithium therapy. Subsequently, she developed significant AKI and persistent proteinuria. She underwent a renal biopsy that demonstrated effacement of podocyte foot processes consistent with lithium-induced MCD. This was treated with corticosteroids, which decreased the proteinuria and resolved all the patient′s symptoms. Conclusion: Lithium-induced MCD is a rare disease that affects patients of all ages. It is often associated with therapeutic lithium and is typically resolved with discontinuation of lithium. In some cases, concurrent AKI may result due to vascular obstruction from hyperalbuminuria and associated renal interstitial edema. Corticosteroids may be needed to reduce the proteinuria and prevent progression to chronic kidney disease. As such, patients on lithium therapy may benefit from monitoring of glomerular function via urinalysis to prevent the onset of nephrotic syndrome.

  11. Relationship Between Periodontal Disease and Acute Myocardial Infarction

    M Zamirian

    2008-07-01

    Full Text Available Background: Conventional risk factors for coronary artery disease and myocardial infarction do not explain all of the clinical and epidemiological features of the disease. Periodontal disease is a common bacterial and destructive disorder of oral tissues. Many studies demonstrate close association between chronic periodontitis and development of generalized inflammation, vascular endothelial injury, and atherosclesis. Periodontal disease has been convincingly emerging as an important independent risk factor for ischemic heart disease. A case - control study was carried out to assess the prevalence of periodontitis in patients with Acute myocardial Infarction (AMI and evaluate the possible relationship between AMI and chronic periodontitis. Patients and Methods: A number of 160 patients, aged 35 to 70 years old, enrolled in the study. Eighty patients (43 men, 37 women were examined four days after hospitalization due to AMI. Control group consisted of 80 persons (38 men, 42 women with normal coronary angiography. The following periodontal parameters were examined: Plaque index (PI, gingiral index (GI, bleeding on probing (BOP, probing depth (PD, clinical attachment loss (CAL and number of sites with CAL.Results: The case, compared to control showed significantly worse results for some periodontal variables studied: The mean of PD and PD > 3 mm, CAL, and number of sites with CAL, had worse results compared to control despite similar oral hygiene and frequency of brushing. The confounding factors for the present study were found to be hypertension and diabetes. Conclusion: The association between periodontitis and acute myocardial infarction was significant after adjusting for conventional risk factors for AMI.

  12. Pathogenic mechanisms of Acute Graft versus Host Disease

    Ferrara James L.M.

    2002-01-01

    Full Text Available Graft-versus-host-disease (GVHD is the major complication of allogeneic Bone Marrow Transplant (BMT. Older BMT recipients are a greater risk for acute GVHD after allogeneic BMT, but the causes of this association are poorly understood. Using well-characterized murine BMT models we have explored the mechanisms of increased GVHD in older mice. GVHD mortality and morbidity, and pathologic and biochemical indices were all worse in old recipients. Donor T cell responses were significantly increased in old recipients both in vivo and in vitro when stimulated by antigen-presenting cells (APCs from old mice. In a haploidential GVHD model, CD4+ donor T cells mediated more severe GVHD in old mice. We confirmed the role of aged APCs in GVHD using bone marrow chimera recipient created with either old or young bone marrow. APCs from these mice also stimulated greater responses from allogeneic cells in vitro. In a separate set of experiments we evaluated whether alloantigen expression on host target epithelium is essential for tissue damage induced by GVHD. Using bone marrow chimeras recipients in which either MHC II or MHC I alloantigen was expressed only on APCs, we found that acute GVHD does not require alloantigen expression on host target epithelium and that neutralization of tumor necrosis factor-alpha and interleukin-1 prevents acute GVHD. These results pertain to CD4-mediated GVHD and to a lesser extent in CD8-mediated GVHD, and confirm the central role of most APCs as well as inflammatory cytokines.

  13. Role of anaerobes in acute pelvic inflammatory disease

    Saini S

    2003-01-01

    Full Text Available Pouch of Douglas aspirates were collected from 50 women with history and examination suggestive of acute pelvic inflammatory disease (PID and 20 healthy women admitted for tubal ligation served as control. A total of 57 microorganisms were isolated from 37 patients out of 50 in study group. Of 37 positive cultures 21(56.7% were monomicrobial and 16(43.2% were polymicrobial. Most common symptom in study group was lower abdominal pain (90%, vaginal discharge (70% and irregular bleeding (40% and 30% patients had history of intrauterine contraceptive device (IUCD implantation. The predominant aerobic isolates were Escherichia coli, Coagulase Negative Staphylococcus (CONS, Staphylococcus aureus, Klebsiella pneumoniae while common anaerobes were Bacteroides fragilis, Prevotella melaninogenica, Fusobacterium nucleatum and Peptostreptococcus spp. Our study shows that cefotaxime, cefuroxime and gentamicin may be used for gram negative aerobic bacilli; cloxacillin, cephaloridine and erythromycin for aerobic gram positive cocci and amikacin and ceftazidime for Pseudomonas aeruginosa. Thus for optimum therapy of acute PID it is beneficial to keep in mind major conceptual changes and therapeutic realities that have influenced current understanding of acute PID and have affected the choice of therapy.

  14. Role of anaerobes in acute pelvic inflammatory disease.

    Saini, S; Gupta, N; Batra, G; Arora, D R

    2003-01-01

    Pouch of Douglas aspirates were collected from 50 women with history and examination suggestive of acute pelvic inflammatory disease (PID) and 20 healthy women admitted for tubal ligation served as control. A total of 57 microorganisms were isolated from 37 patients out of 50 in study group. Of 37 positive cultures 21(56.7%) were monomicrobial and 16(43.2%) were polymicrobial. Most common symptom in study group was lower abdominal pain (90%), vaginal discharge (70%) and irregular bleeding (40%) and 30% patients had history of intrauterine contraceptive device (IUCD) implantation. The predominant aerobic isolates were Escherichia coli, Coagulase Negative Staphylococcus (CONS), Staphylococcus aureus, Klebsiella pneumoniae while common anaerobes were Bacteroides fragilis, Prevotella melaninogenica, Fusobacterium nucleatum and Peptostreptococcus spp. Our study shows that cefotaxime, cefuroxime and gentamicin may be used for gram negative aerobic bacilli; cloxacillin, cephaloridine and erythromycin for aerobic gram positive cocci and amikacin and ceftazidime for Pseudomonas aeruginosa. Thus for optimum therapy of acute PID it is beneficial to keep in mind major conceptual changes and therapeutic realities that have influenced current understanding of acute PID and have affected the choice of therapy. PMID:17643017

  15. Formas agudas de periodontitis Acute conditions of periodontal disease

    L. Pérez-Salcedo

    2008-04-01

    Full Text Available La clasificación de las Enfermedades Periodontales ha cambiado en las últimas décadas. En la clasificación la AAP de 1989 la periodontitis necrotizante ocupaba el cuarto lugar. En el Workshop Europeo de 1993 la periodontitis necrotizante aparece en el grupo de los descriptores primarios. Según el Internacional Workshop for a Classification of Periodontal Diseases and Conditions 1999 en el que se revisó y se modificó la clasificación de las patologías periodontales, las enfermedades periodontales necrotizantes ocupan el punto cinco, diferenciándose entre Gingivitis Necrotizante y Periodontitis Necrotizante. Y se añade en la clasificación el grupo de abscesos periodontales. En este artículo de revisión vamos a profundizar acerca de las formas agudas de periodontitis.The Periodontal Diseases classification had changed in the last decades. In AAP classification of 1989 the necrotize was in the 4th position. In the European Workshop was in the group of primary descriptors. According to the International Workshop for a Classification of Periodontal Diseases and Conditions 1999, review and modificated the classification of periodontal pathologies, the periodontal necrotize diseases are in the 5th position, distinguishing between Necrotize Gingivitis and Necrotize Periodontitis. And Peridontal Abscesses was add to the classification. In this paper we are going to review about the acute forms of Periodontal Diseases.

  16. Acute exacerbations of chronic obstructive pulmonary disease: causes and impacts.

    Chhabra, Sunil K; Dash, Devi Jyoti

    2014-01-01

    Acute exacerbations of chronic obstructive pulmonary disease (AECOPD) are recognised clinically as episodes of increased breathlessness and productive cough requiring a more intensive treatment. A subset of patients with this disease is especially prone to such exacerbations. These patients are labelled as 'frequent exacerbators'. Though yet poorly characterised in terms of host characteristics, including any genetic basis, these patients are believed to represent a distinct phenotype as they have a different natural history with a more progressive disease and a poorer prognosis than those who get exacerbations infrequently. Most exacerbations appear to be associated with infective triggers, either bacterial or viral, although 'non-infective' agents, such as air pollution and other irritants may also be important. Susceptibility to exacerbations is determined by multiple factors. Several risk factors have been identified, some of which are modifiable. Chronic obstructive pulmonary disease (COPD) exacerbations are major drivers of health status and patient-centered outcomes, and are a major reason for health care utilisation including hospitalisations and intensive care admissions. These are associated with considerable morbidity and mortality, both immediate and long-term. These episodes have a negative impact on the patient and the disease including high economic burden, increased mortality, worsening of health status, limitation of activity, and aggravation of comorbidities including cardiovascular disease, osteoporosis and neuro-psychiatric complications. Exacerbations also increase the rate of progression of disease, increasing the annual decline in lung function and leading to a poorer prognosis. Evaluation of risk of exacerbations is now included as a major component of the initial assessment of a patient with COPD in addition to the traditionally used lung function parameter, forced expiratory volume in one second (FEV1). Decreasing the risk of exacerbations

  17. Detection of acute hepatopancreatic necrosis disease (AHPND) in Mexico.

    Nunan, Linda; Lightner, Donald; Pantoja, Carlos; Gomez-Jimenez, Silvia

    2014-08-21

    Acute hepatopancreatic necrosis disease (AHPND), which has also been referred to as early mortality syndrome (EMS), initially emerged as a destructive disease of cultured shrimp species in Asia in 2009. The pathogen associated with the disease, Vibrio parahaemolyticus, subsequently spread to the Western Hemisphere and emerged in Mexico in early 2013. The spread to the Western Hemisphere is a major concern to shrimp producers in the region. To date, the only peer-reviewed published method for determining whether mortalities are due to AHPND is through histological examination. A novel PCR detection method was employed to assess samples from Mexico in order to confirm the presence of the pathogen in this country. This manuscript details the detection methods used to confirm the presence of AHPND in Mexico. Both immersion and per os challenge studies were used to expose the Penaeus vannamei to the bacteria in order to induce the disease. Histological analysis confirmed AHPND status following the challenge studies. Also provided are the details of the molecular test by PCR that was used for screening candidate V. parahaemolyticus isolates. A rapid PCR assay for detection of AHPND may help with early detection and help prevent the spread of AHPND to other countries. PMID:25144120

  18. Managing the acutely ill adult with sickle cell disease.

    Brown, Marvelle

    Sickle cell disease (SCD) is an autosomal recessively inherited condition, affecting the structure of the haemoglobin. SCD is a long-term chronic condition which is manifested by periods of acute painful sickling crisis, known as vaso-occlusive crisis (VOC) and is the cause of 90% of sickle cell-related hospital admissions. SCD is one of the most common genetic conditions worldwide and in the UK there are approximately 12,500 people living with it (Streetly et al,1997; Howard et al, 2008), making it more common than cystic fibrosis, yet there still remains many challenges in managing these patients when they become acutely ill. Lack of awareness and understanding of the illness, concerns regarding addiction and limited attention to the psycho-social implications of the illness, leads to less than effective care for this patient group when they are hospitalized. The aims of this article are to outline the pathophysiology of SCD, identify the causes of VOC and discuss the key principles of nursing management for patients experiencing a VOC. PMID:22306637

  19. Oxygen therapy in acute exacerbations of chronic obstructive pulmonary disease

    Brill SE

    2014-11-01

    Full Text Available Simon E Brill, Jadwiga A Wedzicha Airway Disease Section, National Heart and Lung Institute, Imperial College, London, UK Abstract: Acute exacerbations of chronic obstructive pulmonary disease (COPD are important events in the history of this debilitating lung condition. Associated health care utilization and morbidity are high, and many patients require supplemental oxygen or ventilatory support. The last 2 decades have seen a substantial increase in our understanding of the best way to manage the respiratory failure suffered by many patients during this high-risk period. This review article examines the evidence underlying supplemental oxygen therapy during exacerbations of COPD. We first discuss the epidemiology and pathophysiology of respiratory failure in COPD during exacerbations. The rationale and evidence underlying oxygen therapy, including the risks when administered inappropriately, are then discussed, along with further strategies for ventilatory support. We also review current recommendations for best practice, including methods for improving oxygen provision in the future. Keywords: chronic obstructive pulmonary disease (COPD, exacerbation, oxygen therapy, respiratory failure, hypercapnia

  20. Mitochondrial dysfunction in inherited renal disease and acute kidney injury.

    Emma, Francesco; Montini, Giovanni; Parikh, Samir M; Salviati, Leonardo

    2016-05-01

    Mitochondria are increasingly recognized as key players in genetic and acquired renal diseases. Most mitochondrial cytopathies that cause renal symptoms are characterized by tubular defects, but glomerular, tubulointerstitial and cystic diseases have also been described. For example, defects in coenzyme Q10 (CoQ10) biosynthesis and the mitochondrial DNA 3243 A>G mutation are important causes of focal segmental glomerulosclerosis in children and in adults, respectively. Although they sometimes present with isolated renal findings, mitochondrial diseases are frequently associated with symptoms related to central nervous system and neuromuscular involvement. They can result from mutations in nuclear genes that are inherited according to classic Mendelian rules or from mutations in mitochondrial DNA, which are transmitted according to more complex rules of mitochondrial genetics. Diagnosis of mitochondrial disorders involves clinical characterization of patients in combination with biochemical and genetic analyses. In particular, prompt diagnosis of CoQ10 biosynthesis defects is imperative because of their potentially reversible nature. In acute kidney injury (AKI), mitochondrial dysfunction contributes to the physiopathology of tissue injury, whereas mitochondrial biogenesis has an important role in the recovery of renal function. Potential therapies that target mitochondrial dysfunction or promote mitochondrial regeneration are being developed to limit renal damage during AKI and promote repair of injured tissue. PMID:26804019

  1. CT appearance of acute inflammatory disease of the renal interstitium

    Today, infection remains the most common disease of the urinary tract and constitutes almost 75% of patient problems requiring urologic evaluation. There have been several major factors responsible for our better understanding of the nature and pathophysiology of urinary tract infection. One has been quantitated urine bacteriology and another, the discovery that a significant part of the apparently healthy adult female population has asymptomatic bacteriuria. Abnormal conditions such as neurogenic bladder, bladder malignancy, prolonged catheter drainage and reflux, altered host resistance, diabetes mellitus, and urinary tract obstruction, as well as pregnancy, may either predispose to or be implicated in the pathogenesis of urinary tract infection. There is a wide range of conditions that result in acute renal inflammation and those under discussion affect primarily the interstitium. This term refers to the connective tissue elements separating the tubules in the cortex and medulla. Hence, the interstitial nephritides are to be distinguished from the glomerulonephritides and fall into two general etiologic categories: infectious and noninfectious

  2. Acute Psychosis as Major Clinical Presentation of Legionnaires’ Disease

    Ricardo Coentre

    2016-01-01

    Full Text Available We report a case of a 61-year-old woman who presented with acute psychosis as a major manifestation of Legionnaires’ disease in the absence of other neuropsychiatric symptoms. Clinical history revealed dry cough and nausea. Observation showed fever and auscultation crackles in the lower lobe of the right lung. Laboratory testing demonstrated elevated C-reactive protein and lung chest radiograph showed patchy peribronchial and right lower lobe consolidation. Soon after admission, she started producing purulent sputum. Epidemiological data suggested Legionella pneumophila as possible cause of the clinical picture that was confirmed by urinary antigen detection and polymerase chain reaction of the sputum. She was treated with levofloxacin 750 mg/day for 10 days with complete remission of pulmonary and psychiatric symptoms. She has not had further psychotic symptoms.

  3. Invasive fungal diseases in patients with acute lymphoid leukemia.

    Nicolato, Andrea; Nouér, Simone A; Garnica, Marcia; Portugal, Rodrigo; Maiolino, Angelo; Nucci, Marcio

    2016-09-01

    Invasive fungal disease (IFD) represents an important complication in patients with acute lymphoid leukemia (ALL). The objectives of this study were to determine the prevalence of IFD in ALL patients with neutropenia, identify factors associated with IFD, and estimate the impact of IFD on the outcome. All patients with ALL who developed febrile neutropenia from 1987 to 2013 were evaluated. Cases of IFD were classified as proven or probable. Factors associated with IFD were evaluated by comparing episodes with and without a diagnosis of IFD. Among 350 episodes of febrile neutropenia, 31 IFDs were diagnosed (8.8%). Prolonged neutropenia was the only factor associated with IFD caused by yeasts. Factors associated with IFD caused by molds by multivariate analysis were the period after 2008, receipt of allogeneic transplant, relapsed ALL and prolonged neutropenia. Patients in relapse should receive induction chemotherapy in rooms with HEPA filter and receive antifungal prophylaxis. PMID:26949001

  4. Preventive measures for Acute Rheumatic Fever/ Rheumatic Heart Disease : A literature review

    Shrestha, Usha; Kunwar, Nabina

    2013-01-01

    Acute rheumatic disease is a major burden in the developing countries and also a major cause of premature death in children and young adults every year. Aim: The aim of this study is to investigate the factors contributing to prevention of acute rheumatic fever and rheumatic heart disease in developing countries. This will offers appropriate knowledge to the care provider to identify risk factors for acute rheumatic fever and implement in-terventions timely. The research questions are followi...

  5. Acute Kidney Injury due to Crescentic Glomerulonephritis in a Patient with Polycystic Kidney Disease

    Maggard, Reuben; Makary, Raafat; Monteiro, Carmela l.; James, Leighton R.

    2013-01-01

    Polycystic kidney disease is an inherited condition, characterized by the development of cysts in the kidney, as well as in other organs. Patients with polycystic kidney can suffer from the same causes of acute kidney injury as the general population. Nephritic syndrome is an uncommon cause of acute kidney injury in the general population and less common in patients with polycystic kidney disease. We report the second case of crescentic glomerulonephritis, causing acute kidney injury, in a pa...

  6. Uric acid levels and their relation to incapacities in acute cerebrovascular disease

    Julio López Argüelles; Joan Rojas Fuentes; Ricardo Verdecia Fraga

    2010-01-01

    Background: cerebrovascular disease and ischemic cardiopathy can be considered as an epidemic and constitute the first cause of incapacities in developed countries. Multiple studies have shown the association between uric acid levels and cerebrovascular diseases. Objective: To correlate the levels of serum uric acid and incapacities in the acute phase of cerebrovascular disease. Methods: A correlational study was carried out with 217 patients with acute cerebrovascular disease. The patient’s ...

  7. Coronary heart disease is not significantly linked to acute kidney injury identified using Acute Kidney Injury Group criteria

    Yayan J

    2012-01-01

    Josef YayanDepartment of Internal Medicine, Vinzentius Hospital, Landau, GermanyBackground: Patients with unstable angina or myocardial infarction are at risk of acute kidney injury, which may be aggravated by the iodine-containing contrast agent used during coronary angiography; however, the relationship between these two conditions remains unclear.Objective: The current study investigated the relationship between acute kidney injury and coronary heart disease prior to coronary angiography.M...

  8. Diagnostic criteria for acute liver failure due to Wilson disease

    Christoph Eisenbach; Olivia Sieg; Wolfgang Stremmel; Jens Encke; Uta Merle

    2007-01-01

    AIM: To describe the diagnostic criteria for acute liver failure due to Wilson disease (WD), which is an uncommon cause of acute liver failure (ALF).METHODS: We compared findings of patients presenting with ALF due to WD to those with ALF of other etiologies.RESULTS: Previously described criteria, such as low alkaline phosphatase activity, ratio of low alkaline phosphatase to total bilirubin or ratio of high aspartate aminotransferase (AST) to alanine aminotransferase (ALT), failed to identify patients with ALF due to WD. There were significant differences in low ALT and AST activities (53 ± 43 vs 1982 ± 938, P < 0.0001 and 87 ± 44 vs 2756 ± 2941, P = 0.037, respectively), low choline esterase activity (1.79 ± 1.2 vs 4.30 ± 1.2, P = 0.009), high urine copper concentrations (93.4 ± 144.0 vs 3.5 ± 1.8, P = 0.001) and low hemoglobin (7.0 ± 2.2 vs 12.6 ± 1.8, P < 0.0001) in patients with ALF caused by WD as compared with other etiologies. Interestingly, 4 of 7 patients with ALF due to WD survived without liver transplantation.CONCLUSION: In ALF, these criteria can help establish a diagnosis of WD. Where applicable, slit-lamp examination for presence of Kayser-Fleischer rings and liver biopsy for determination of hepatic copper concentration still remain important for the diagnosis of ALF due to WD. The need for liver transplantation should be evaluated carefully as the prognosis is not necessarily fatal.

  9. Pathogenesis of acute hepatopancreatic necrosis disease (AHPND) in shrimp.

    Lai, Hung-Chiao; Ng, Tze Hann; Ando, Masahiro; Lee, Chung-Te; Chen, I-Tung; Chuang, Jie-Cheng; Mavichak, Rapeepat; Chang, Sheng-Hsiung; Yeh, Mi-De; Chiang, Yi-An; Takeyama, Haruko; Hamaguchi, Hiro-o; Lo, Chu-Fang; Aoki, Takashi; Wang, Han-Ching

    2015-12-01

    Acute hepatopancreatic necrosis disease (AHPND), also called early mortality syndrome (EMS), is a recently emergent shrimp bacterial disease that has resulted in substantial economic losses since 2009. AHPND is known to be caused by strains of Vibrio parahaemolyticus that contain a unique virulence plasmid, but the pathology of the disease is still unclear. In this study, we show that AHPND-causing strains of V. parahaemolyticus secrete the plasmid-encoded binary toxin PirAB(vp) into the culture medium. We further determined that, after shrimp were challenged with AHPND-causing bacteria, the bacteria initially colonized the stomach, where they started to produce PirAB(vp) toxin. At the same early time point (6 hpi), PirB(vp) toxin, but not PirA(vp) toxin, was detected in the hepatopancreas, and the characteristic histopathological signs of AHPND, including sloughing of the epithelial cells of the hepatopancreatic tubules, were also seen. Although some previous studies have found that both components of the binary PirAB(vp) toxin are necessary to induce a toxic effect, our present results are consistent with other studies which have suggested that PirB(vp) alone may be sufficient to cause cellular damage. At later time points, the bacteria and PirA(vp) and PirB(vp) toxins were all detected in the hepatopancreas. We also show that Raman spectroscopy "Whole organism fingerprints" were unable to distinguish between AHPND-causing and non-AHPND causing strains. Lastly, by using minimum inhibitory concentrations, we found that both virulent and non-virulent V. parahaemolyticus strains were resistant to several antibiotics, suggesting that the use of antibiotics in shrimp culture should be more strictly regulated. PMID:26549178

  10. Review of Elephant Endotheliotropic Herpesviruses and Acute Hemorrhagic Disease.

    Long, Simon Y; Latimer, Erin M; Hayward, Gary S

    2016-02-24

    More than 100 young captive and wild Asian elephants are known to have died from a rapid-onset, acute hemorrhagic disease caused primarily by multiple distinct strains of two closely related chimeric variants of a novel herpesvirus species designated elephant endotheliotropic herpesvirus (EEHV1A and EEHV1B). These and two other species of Probosciviruses (EEHV4 and EEHV5) are evidently ancient and likely nearly ubiquitous asymptomatic infections of adult Asian elephants worldwide that are occasionally shed in trunk wash secretions. Although only a handful of similar cases have been observed in African elephants, they also have proved to harbor their own multiple and distinct species of Probosciviruses-EEHV2, EEHV3, EEHV6, and EEHV7-found in lung and skin nodules or saliva. For reasons that are not yet understood, approximately 20% of Asian elephant calves appear to be susceptible to the disease when primary infections are not controlled by normal innate cellular and humoral immune responses. Sensitive specific polymerase chain reaction (PCR) DNA blood tests have been developed, routine monitoring has been established, the complete large DNA genomes of each of the four Asian EEHV species have now been sequenced, and PCR gene subtyping has provided unambiguous evidence that this is a sporadic rather than epidemic disease that it is not being spread among zoos or other elephant housing facilities. Nevertheless, researchers have not yet been able to propagate EEHV in cell culture, determine whether or not human antiherpesvirus drugs are effective inhibitors, or develop serology assays that can distinguish between antibodies against the multiple different EEHV species. PMID:26912715