The envelope gene of ADOL-4817 strain of avian leukosis viru s subgroup J (ALV-J) was amplified by polymerase chain reaction （PCR） and clo ned into TA vector. The sequence analysis results showed that the envelope gene is composed of 1?746 bp, 1?554 bp of which could be translated into 517 amino acids for gp85 and gp37. The molecular weight of envelope protein is 57.7kD. T here are 15 potential glycosylation sites in the envelope protein, 13 of which i s located in gp85. Analysis of sequences of envelope gene indicate that ADOL -4817 showed high degree of sequence identity to other ALV-J strains, and m ost close ly related to the like-envelope gene of endogenous virus EAV-HP but divergent from these of other ALV subgroup A-E . These data support the hypothesis that envelope gene of avian leukosis virus subgroup J maybe acquired by recombination with expressed sequences.

Kawasaki disease (KD) is a common febrile disease in East Asia. Because KD with coronary artery aneurysm (CAA) may predispose to thrombosis, children with KD-associated CAA may need anticoagulation in addition to aspirin. In this report, we describe a 6-year-old girl with KD and CAA who was found to have unexpected warfarin-induced coagulopathy caused by CYP2C9 and VKORC1 genotype variants, which affect warfarin metabolism.

Kawasaki disease (KD) is a common febrile disease in East Asia. Because KD with coronary artery aneurysm (CAA) may predispose to thrombosis, children with KD-associated CAA may need anticoagulation in addition to aspirin. In this report, we describe a 6-year-old girl with KD and CAA who was found to have unexpected warfarin-induced coagulopathy caused by CYP2C9 and VKORC1 genotype variants, which affect warfarin metabolism.

Coronary artery involvement remains the most important complication with Kawasaki disease (KD). Additional myocardial injury can be caused by inflammatory response and ischemic event. However, the long-term outcome of myocardial function has not been fully known in KD. The purpose of this study is to evaluate myocardial function in school-aged children who had the past history of KD. Sixty-seven children in the second grade of elementary schools, who had the past history of KD, were included. Echocardiographic measurements of each coronary artery and myocardial function were obtained as the long-term follow-up data, and compared with the baseline data at the time of initial presentation of KD. The mean age of the subjects was 8.6 ± 2.4 years, and 4.3 ± 3.4 years have passed since the diagnosis of KD. Among the echocardiographic data, interventricular septum thickness at end-diastole (IVSd), LV internal diameters at end-systole (LVIDs), maximal velocity of late diastolic filling across mitral valve (mitral A) flow, maximal velocity of early diastolic filling across mitral valve (mitral E)/A ratio, mitral inflow E wave to peak early diastolic tissue velocity (E/E') ratio showed significant differences between the baseline and follow-up measurements. Coronary Z-score of left main artery (LMA), left anterior descending (LAD), and right coronary artery (RCA) showed no significant difference. The school-aged children with the past history of KD may have diastolic dysfunction. Therefore, appropriate assessment of myocardial function would be recommended during the follow-up period in children with KD.
Arteries ; Child* ; Coronary Vessels ; Diagnosis ; Echocardiography ; Follow-Up Studies ; Humans ; Mitral Valve ; Mucocutaneous Lymph Node Syndrome*

PURPOSE: Children with mitochondrial disease (MD) have clinical phenotypes that are more severe than those found in adults. In this study, we assessed cardiac function in children with MD using conventional and advanced echocardiographic measurements, explored any unique patterns present, and investigated the development of early cardiomyopathy (CMP). MATERIALS AND METHODS: We retrospectively reviewed the medical records of 33 children with MD. All patients underwent transthoracic echocardiography with conventional and advanced myocardial analysis. We compared all data between patients and an age-matched healthy control group. RESULTS: Conventional echocardiographic diastolic measurements of mitral E, E/A, and tissue Doppler E′ were significantly lower and E/E′ was significantly higher in children with MD, compared with the measurements from the control group. There was no significant difference in longitudinal and radial strain between the groups. Circumferential strain in the endocardium (p=0.161), middle myocardium (p=0.008), and epicardium (p=0.042) were lower in patients, compared to the values in controls. Circumferential strain was correlated with E′ (p < 0.01, r>0.60). CONCLUSION: In children with MD, myocardial circumferential strain may develop early in all three layers, even with normally preserved longitudinal and radial strain. This may be an early diagnostic indicator with which to predict CMP in this patient population.
Biomechanical Phenomena ; Child ; Echocardiography, Doppler ; Female ; Humans ; Male ; Mitochondrial Diseases/diagnostic imaging ; Mitochondrial Diseases/pathology ; Mitochondrial Diseases/physiopathology ; Myocardium/pathology

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises group of small vessel vasculitides, including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In 2022, the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR) jointly proposed new classification criteria for AAV (the 2022 ACR/EULAR criteria). In this review, we briefly summarize the 2022 ACR/EULAR criteria for GPA, MPA, and EGPA, and introduce our clinical experience with applying them to patients who were previously diagnosed with AAV based on three criteria: firstly, the classification criteria for GPA and EGPA proposed by the ACR in 1990; secondly, the algorithm for the classification of AAV and polyarteritis nodosa proposed by the European Medicines Agency algorithm in 2007 (the 2007 EMA algorithm); and thirdly, the revised International Chapel Hill Consensus Conference nomenclature of vasculitides in 2012 (the 2012 CHCC definitions). We found that concordance rate was highest in patients with MPA (96.6%), followed by those with EGPA (86.3%) and GPA (73.8%). In addition, compared to previous criteria, we noted several issues of the undervalued or overvalued items in the 2022 ACR/EULAR criteria for classifying AAV and provided several suggestions. To increase the diagnostic accuracy and reduce the discordance rate among the new and previous criteria for AAV, we suggest that the previous criteria should be considered together with the 2022 ACR/EULAR criteria when applying the classification criteria for AAV to patients suspected of AAV.

PURPOSE: Cardiomyopathy is becoming the leading cause of death in patients with Duchenne muscular dystrophy because mechanically assisted lung ventilation and assisted coughing have helped resolve respiratory complications. To clarify cardiopulmonary function, we compared cardiac function between the home ventilator-assisted and non-ventilator-assisted groups. METHODS: We retrospectively reviewed patients with Duchenne muscular dystrophy from January 2010 to March 2016 at Gangnam Severance Hospital. Demographic characteristics, pulmonary function, and echocardiography data were investigated. RESULTS: Fifty-four patients with Duchenne muscular dystrophy were divided into 2 groups: home ventilator-assisted and non-ventilator-assisted. The patients in the home ventilator group were older (16.25±1.85 years) than those in the nonventilator group (14.73±1.36 years) (P=0.001). Height, weight, and body surface area did not differ significantly between groups. The home ventilator group had a lower seated functional vital capacity (1,038±620.41 mL) than the nonventilator group (1,455±603.12 mL). Mean left ventricular ejection fraction and fractional shortening were greater in the home ventilator group, but the data did not show any statistical difference. The early ventricular filling velocity/late ventricular filling velocity ratio (1.7±0.44) was lower in the home ventilator group than in the nonventilator group (2.02±0.62). The mitral valve annular systolic velocity was higher in the home ventilator group (estimated β, 1.06; standard error, 0.48). Patients with Duchenne muscular dystrophy on a ventilator may have better systolic and diastolic cardiac functions. CONCLUSION: Noninvasive ventilator assistance can help preserve cardiac function. Therefore, early utilization of noninvasive ventilation or oxygen may positively influence cardiac function in patients with Duchenne muscular dystrophy.
Body Surface Area ; Cardiomyopathies ; Cause of Death ; Cough ; Echocardiography ; Heart Function Tests ; Humans ; Lung ; Mitral Valve ; Muscular Dystrophy, Duchenne ; Noninvasive Ventilation ; Oxygen ; Retrospective Studies ; Stroke Volume ; Ventilation ; Ventilators, Mechanical ; Vital Capacity

In this paper, the fourth author’s name and institution were given incorrectly.

Objective: The total haemolytic complement activity (CH50) assay evaluates the functioning of the complement system. Accumulating evidence indicates that the activation of the complement system plays a critical role in the pathogenesis of antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Therefore, this study aimed to investigate whether CH50 levels at diagnosis could reflect the baseline activity of AAV. Methods: This retrospective study included 101 immunosuppressive drug-naïve patients with AAV. At diagnosis, all patients underwent clinical assessments for disease activity, including measurement of the Birmingham Vasculitis Activity Score (BVAS) and Five Factor Score (FFS), and laboratory evaluations, such as tests for CH50, C3, and C4 levels. The association between CH50 levels and disease activity was determined. Results: The median BVAS and FFS at diagnosis were 12.0 and 1.0, respectively, whereas the median CH50 level was 60.4 U/mL. There was a negative correlation between the CH50 level and BVAS (r=−0.241; p=0.015). A CH50 cut-off value of 62.1 U/mL was used to classify the patients into two groups: patients with CH50 levels ＜62.1 U/mL (low-CH50 group) and those with CH50 levels ≥ 62.1 U/mL (high-CH50 group). The low-CH50 group had a higher proportion of patients with high disease activity, based on the BVAS, than the high-CH50 group (52.5% vs. 23.8%, p=0.004). Additionally, the low-CH50 group exhibited a lower relapse-free survival rate than the high-CH50 group; however, this difference was not statistically significant (p=0.082). Conclusion Low CH50 levels at diagnosis may reflect high baseline activity of AAV.

We report a case of a cardiac lipoma arising from the free wall of right atrium, which was diagnosed as the presumed source of angina by compressing the right coronary artery in a 54-year-old woman. Surgical excision of the tumor was performed well. She continues to do well for six months without angina.
Coronary Vessels ; Female ; Heart Atria ; Humans ; Lipoma ; Middle Aged