[Clinical and myopathological features of Jo-1 syndrome].

OBJECTIVE: To report the clinical and myopathological features of 16 patients with Jo-1 syndrome. METHODS: Sixteen patients were recruited in this study, who were diagnosed as Jo-1 syndrome in Department of Neurology of Peking University First Hospital from January, 2011 to July, 2015. The clinical data and myopathological data were analyzed. RESULTS: The mean onset age was 41±14 (21-68) years old. 87.5% was female. The median duration was 9.5 months (1-192 months). The main clinical manifestations were weakness in 13 cases (81.2%), arthritis in 10 cases (62.5%), interstitial lung diseases in 8 cases (50%), dermatomyositis-like skin lesions in 5 cases (31.2%), fever in 3 cases (18.8%), Raynaud's phenomenon in 2 cases (12.5%) and mechanic's hands in 2 cases (12.5%). There were 3 cases with other connective tissue diseases and 1 case with non-Hodgkin's lymphoma. Mean serum CK was 3 054±2 058(470-5 222) U/L. All patients had anti-Jo-1antibody, combined with anti- Mi-2 antibody in 1 case, anti-Ro-52 antibody in 5 cases, and anti-nuclear antibody in 5 cases. 4/5 cases showed myopathic changes for electromyography (EMG) tests. Myopathological changes included edema, fragmentation and inflammatory infiltration in perimysium in 14 cases (87.5%), muscle atrophy in 13 cases including 7 cases(43.8%) predominantly in perifascicular field. Muscle fiber necrosis appeared in 8 cases with predominantly in perifascicular area in 4 cases (25%). Muscle fiber regeneration occurred in 11 cases with predominantly in perifascicular field in 5 cases (31.2%). CD8 positive T-lymphocytes, CD20 positive B-lymphocytes and CD68 positive macrophages infiltrated in various degrees, most of which were located in perimysium. MHC-Ⅰ were expressed on muscle fiber membranes in different degrees, including 7 cases (43.8%) predominantly in the cytoplasm of perifascicular muscle fibers. C5b-9 deposited in perifascicular muscle fiber membranes in 7 cases (43.8%) and perifascicular capillaries in 2 cases (12.5%). CONCLUSIONS: The main manifestations of this group of Jo-1 syndrome are weakness, arthritis and interstitial lung diseases, and dermatomyositis-like skin lesions, fever, Raynaud's phenomenon, and mechanic's hands can also be seen. Edema, fragmentation and inflammatory infiltration in perimysium are common. Pathological changes in perifascicular fields appear in some cases.

Preparation, thermal storage properties and application of sodium acetate trihydrate/expanded graphite composite phase change materials.

The development of energy storage technology is beneficial for the efficient use of energy and sustainable development. As an effectual approach for storing and transporting thermal energy, latent heat storage using phase change materials (PCMs) has attracted tremendous attention. However, low thermal conductivity, poor stability, and leakages are considerable challenges to the widespread application of solid-liquid PCMs. Composite phase change materials (CPCMs) were prepared by combining expanded graphite (EG) and sodium acetate trihydrate (CH3COONa·3H2O, SAT). EG as a supporting material plays a crucial part in both enhancing the thermal conductivity and preventing the melted PCMs from leakage. The chemical structure, micromorphology, thermal stability, thermal conductivity, phase change behavior and heat storage performance of SAT/EG CPCMs have been extensively investigated by scanning electron microscopy (SEM), Fourier transform infrared spectroscopy (FTIR), X-ray diffraction (XRD), thermal conductivity analysis, differential scanning calorimetry (DSC), and cycling stability measurement. The results of SEM indicate that EG with a loose and porous layered structure has a good molding effect and can adsorb SAT well. XRD and FTIR results show that only a simple physical combination between EG and SAT exists, and no new substances have been produced. Compared with pure SAT, thermal conductivity and supercooling tests show that the supercooling degree of the CPCMs was decreased and the thermal conductivity was increased by 205.1%. In addition, the addition of 2 wt% of disodium hydrogen phosphate dodecahydrate (Na2HPO4·12H2O, DHPD) as a nucleating agent and 0.5 wt% of gelatin as a thickening agent to SAT could reduce the supercooling degree and inhibit the phase separation well. Based on SAT/EG-8% CPCMs, an oven with phase change energy storage was designed and the heat storage/release performance of the oven was investigated under different operating conditions.

Intrinsic Capacity Impairment Patterns and their Associations with Unfavorable Medication Utilization: A Nationwide Population-Based Study of 37,993 Community-Dwelling Older Adults.

OBJECTIVES: Our aim was to explore the patterns of intrinsic capacity (IC) impairments among community-dwelling older adults and the associations of these different patterns with excessive polypharmacy, potentially inappropriate medications, and adverse drug reactions in a nationwide population-based study. DESIGN: A cross-sectional study included older adults from the Taiwan Integrated Care for Older People (ICOPE) program in 2020. SETTING AND PARTICIPANTS: The study subjects comprised 38,308 adults aged 65 years and older who participated in the ICOPE Step 1 screening and assessed six domains of IC following the World Health Organization (WHO) ICOPE approach. METHODS: Latent class analysis was adopted to identify distinct subgroups with different IC impairments patterns. The associations between different IC impairments patterns and unfavorable medication utilization, including excess polypharmacy (EPP), potentially inappropriate medications (PIMs), and adverse drug reactions (ADRs), were assessed by multivariate logistic regression models. RESULTS: Latent class analysis identified five distinct subgroups with different IC impairment patterns: robust (latent class prevalence: 59.4%), visual impairment (17.7%), physio-cognitive decline (PCD) with sensory impairment (12.3%), depression with cognitive impairment (7.7%), and impairments in all domains (2.9%). Compared to the robust group, all other groups were at higher odds for unfavorable medication utilization. The "depression with cognitive impairment" group (EPP: aOR=4.35, 95% CI 3.52-5.39, p<0.01; PIMs: aOR=2.73, 95% CI 2.46-3.02, p<0.01) and the "impairment in all domains" group (EPP: aOR=9.02, 95% CI 7.16-11.37, p<0.01; PIMs: aOR=3.75, 95% CI 3.24-4.34, p<0.01) remained at higher odds for EPP and PIMs after adjustment. CONCLUSIONS: We identified five distinct impairment patterns of IC, and each impairment pattern, particularly the "depression with cognitive impairment" and "impairment in all domains", was associated with higher odds of EPP and PIMs. Further longitudinal and intervention studies are needed to explore long-term outcomes of different impairment pattern and their reversibility.

Seeding Homologous Adipose-Derived Stem Cells and Bladder Smooth Muscle Cells Into Bladder Submucosa Matrix for Reconstructing the Ureter in a Rabbit Model.

BACKGROUND: Congenital or acquired abnormalities may result in ureteral malformation, trauma, or defect. Traditional reconstructive methods are often associated with numerous complications. Tissue engineering technology may provide an alternate avenue for ureteral reconstruction. In this study, we constructed tissue-engineered tubularized grafts (TETGs) by seeding homologous adipose-derived stem cells (ADSCs) and bladder smooth muscle cells (SMCs) into bladder submucosa matrix (BSM) for ureteral reconstruction in rabbit models. METHODS: ADSCs and bladder SMCs were seeded onto 2 sides of the BSM, respectively. Then the grafts were used to construct TETGs of 4.0 cm length and 8.0 mm diameter and were transplanted into the omentum of rabbits for 2 weeks before ureteral reconstruction. The 4.0-cm segment of the ureter was replaced by the TETG. Evolutionary formation of tissue structures and degree of epithelization were evaluated with the use of histologic and immunohistochemical techniques at 2, 4, 8, and 16 weeks after implantation. RESULTS: All of the rabbits were alive until they were killed. Histologic and immunohistochemical analyses showed consistent regeneration of mature and functional urothelium. At 16 weeks after TETG implantation, multilayered urothelium covered the entire lumen, with visible neovascularization in the center and formation of organized smooth muscle bundles. CONCLUSIONS: We successfully constructed a tissue-engineered transplanted graft by seeding ADSCs and SMCs onto the BSM for ureteral repair and reconstruction in a rabbit model.

The Brain Bench: virtual tools for stereotactic frame neurosurgery.

We present a suite of neurosurgery supporting tools developed around (i) the Virtual Workbench, a productive environment for the control of 3-D data, in which delicate work can be performed for hours on end without strain, and (ii) the Electronic Brain Atlas, integrating the major print brain atlases in day-to-day clinical use. We describe in detail the Brain Bench, a surgical planning system for stereotactic frame neurosurgery. Its objective is to prepare faster plans; have a better and more accurate choice of target points; improve the avoidance of sensitive structures; have fewer sub-optimal frame attachments and speedier, more effective planning and training. If validated by a clinical study now under way, this will improve medical efficacy and reduce costs.

Positron emission tomography for the assessment of myocardial viability in Kawasaki disease using different therapies.

13N-ammonia and 18F-fluorodeoxyglucose positron emission tomography (PET) of the heart were performed on 30 children with a history of Kawasaki disease. The results indicated PET abnormalities in 61.1% of patients during the acute and subacute stages and in 41.2% of patients in the convalescent stage of Kawasaki disease. Two-dimensional echocardiography and coronary angiography could not predict the myocardial viability and perfusion as well as PET. Different therapies during the acute stage of the disease did not effectively prevent myocardial damage, despite the absence of coronary arterial abnormalities. The patients who received 400 mg x kg(-1) x day(-1) of intravenous immunoglobulin (IVIG) for 5 days had a significantly lower incidence of PET abnormalities than those who received a single dose of 2000 mg x kg(-1) IVIG (P < 0.05).

Transcatheter stent treatment for congenital peripheral pulmonary arterial stenosis.

A total of 5 Johnson and Johnson stents were implanted in two patients with significant residual peripheral pulmonary arterial stenosis. These were a 15-year-old boy with post-open heart surgery for tetralogy of Fallot and a 3 8/12 year-old boy with D-transposition of great vessels. Immediately after balloon dilatation and implantation of the stents, the diameter of the narrowing pulmonary arteries increased significantly from 6.0 +/- 0.8 mm to 13.5 +/- 1.7 mm (P < 0.001) and the systolic pressure gradients across the stenosis of peripheral pulmonary artery dropped significantly from 33.0 +/- 16.0 mmHg to 10.2 +/- 4.4 mmHg (P < 0.01). One year later, repeated cardiac catheterization was performed on both patients. In the patient with tetralogy of Fallot, a 20 mmHg pressure gradient was found between the main and left pulmonary artery. This patient then received another stent implantation to release the residual stenosis. The boy with D-transposition of great vessels had only 9 mmHg gradient between main and right pulmonary artery. Transcatheter placement of the stent is a feasible and effective method to treat certain patients with significant pulmonary arterial stenosis if surgical correction can not be performed.

Aortic stenosis in children: 19-year experience.

BACKGROUND: In the Taiwanese literature, few articles describe the pertinent features of aortic stenosis (AS). This study explores the features of AS in Chinese children. METHODS: 3808 children with congenital heart diseases have undergone cardiac catheterization at our institution over the past 19 years. Among them, 51 (1.3%) cases were AS. The clinical, electrocardiographic, echocardiographic and catheterization findings, the methods of treatment and outcomes were reviewed. RESULTS: Valvular AS occurred in 39 children (76.5%), subvalvular AS in 5 (9.8%), and supravalvular AS in 7(13.7%). Male was predominant (M/F ratio, 2.6) except in supravalvular type. Forty-three patients had associated cardiovascular defects. Aortic regurgitation (AR) was the most common one. Most patients (56.9%) were asymptomatic. Classic symptoms included exertional dyspnea (17.6%), syncope (9.8%), and chest pain (7.8%), etc. Left ventricular hypertrophy was noted in 31.2% of cases. The mean duration of follow-up was 3.9 +/- 3.4 years. Ten patients received open-heart surgery and 2 received balloon dilation. The pressure gradients across the stenotic area dropped from 95.3 +/- 29.3 to 51.4 +/- 35.8 and 53.1 +/- 12.3 mm Hg in early and late Doppler follow-up studies, respectively (p < 0.05). The average gradient increased from 36.9 +/- 25.3 to 40.8 +/- 32.6 mm Hg in nonsurgical patients. The result was insignificant. No mortality occurred following open-heart surgery. One child expired due to heart failure after the ligation of the patent ductus arteriosus and dilation of the stenotic aortic valve on the surgical table under general anesthesia. Autopsy revealed valvular rupture. In the nonsurgical group, no mortality occurred, but one patient was brought home by parents in critical condition and later died. CONCLUSIONS: We found that some clinical features of AS in Chinese children were different from those in occidental populations. (1) The incidence of AS was relatively low. (2) Subvalvular AS was the least common type in contrast to supravalvular AS in western studies. (3) Male predominance was not present in the supravalvular type, which lacked sexual proclivity. (4) Williams syndrome was a more frequently associated anomaly. Turner syndrome was not present in our study. (5) Isolated AS was less frequent. (6) The unusual finding such as right ventricular hypertrophy on EKG was present due to associated cardiac anomalies. Open-heart surgery is effective and safe, but the efficacy of balloon dilation requires further investigation.

Cytomegalovirus pericarditis with cardiac tamponade in a young infant.

The principal viruses implicated in pericarditis are enteroviruses. Cytomegalovirus pericarditis is quite rare and has been reported in immunocompromised patients with acquired immunodeficiency syndrome, malignant neoplasm or organ transplantation. We report a three-month-old male infant who suffered from cough and rhinorrhea for two weeks. He developed shortness of breath for three days, and fever for one day, prior to admission. Physical examination revealed tachycardia, tachypnea, pale conjunctiva, hepatomegaly, and a muffled heart sound without significant murmur. Chest radiography showed marked enlargement of the cardiac silhouette. Echocardiography demonstrated a large amount of pericardial effusion with impaired diastolic ventricular function. After pericardial drainage and supportive treatment, the fluid gradually disappeared. Viral culture of the pericardial fluid and serologic data confirmed a cytomegalovirus infection. Cytomegalovirus pericarditis should be included in the differential diagnosis of pericardial effusion in a young infant.

Transcatheter closure of atrial septal defect with a CardioSEAL device.

Transcatheter closure of an interatrial septal defect (ASD) with a CardioSEAL device was successfully performed on 12 Taiwanese children. There were five boys and seven girls, aged from 3.6-13.9 (8.3+/-3.2) years and with body weight of 15-57 (33.7+/-14.7) kgs. After one year of follow-up studies, which included physical examination, ECG, chest X-ray and echocardiography, complete closure of ASD was achieved in nine (75%) patients. Two children with a trivial residual shunt were asymptomatic and without audible cardiac murmur. A girl had a small residual left to right atrial shunt by color Doppler echocardiography, but without audible cardiac murmur. There were no immediate or intermediate complications. Transcatheter implantation of the CardioSEAL device is a safe and proper treatment for children with non-complicated small to medium secundum ASD.