Adult T cell leukemia/lymphoma with massive involvement of cardiac muscle and valves

An autopsy case of a 58-year-old woman with massive cardiac Involvement of adult T cell leukemia/lymphoma (ATLL) is reported. She developed cardiac failure due to aortic and mitral regurgitation with cardiac infiltration of ATLL cells, and underwent replacement of both aortic and mitral valves. Studies of the cut-surfaces revealed diffuse thickening of the subendocardial wall of the left chamber with widespread whitish-brown tumor infiltrates. In the regions surrounding the replaced aortic and mitral valves there was also massive tumor cell infiltration. The tumor cells infiltrating the cardiac muscle wall were T cell in origin and exhibited Leu-3a (CD4)-positive immunoreaction. Ultrastructurally, tumor cells contained markedly indented nuclei and some were attached directly to the muscle cells. These findings suggest that this was an unusual form of ATLL with widespread involvement of the heart.     

Plasticity of the central nervous system—a neurosurgeon's experience of cerebral compensation and decompensation

Summary Cerebral plasticity constitutes one of the most decisive factors in recovery and readaptation after cerebral lesions. In contrast to the considerable progress in current studies on normal neuronal plasticity including the idea of l'homme neuronal, the concept of plasticity postulated by Albrecht Bethe in 1929 received little attention. The author, as a neurosurgeon, has tried to describe cranial morphological plasticity, morphological and functional plasticity in infantile encephalopathies and especially in hemiatrophic lesions. It is supposed that a true morphological substrate exists due to compensatory hyperplasia of the uninvolved hemisphere.Modern neurosurgical techniques have demonstrated that the functional plastic capacity is much larger than has been supposed, even in the elderly. Some aspects of the mechanisms of compensation and decompensation of cortical and subcortical structures as well as of the central regulation systems are discussed. The full extent of the amazing recovery and functional reorganization is reached by plastic capacity, personal motivation, adequate training and sufficient time.The contribution ends with an exposition of a personal philosophy concerning psycho-somatic dualism, the body-mind problem, the future of the human brain and the ethical outlook, based on the progressive biological evolution of the basal neocortex and the immanent functional development (H. Spatz).In grateful memory of my paternal friends, the great German brain researchers Julius Hallervorden (1882–1965) and Hugo Spatz (1888–1969).     

Beta-2-adrenoceptor agonists as inhibitors of lung vascular permeability to radiolabelled transferrin in the adult respiratory distress syndrome in man

Increased lung vascular permeability leading to increased plasma protein extravasation and accumulation (PPA) is a characteristic feature of acute lung injury. Using a previously described technique, PPA was monitored in the lungs of patients with the adult respiratory distress syndrome (ARDS) — an extreme example of acute lung injury in man. An external radiation probe detector was used to monitor the pulmonary accumulation of the plasma protein transferrin radiolabelled in-vivo with ^113mIn. Ten patients with ARDS exhibiting increased PPA indices (>1.0x10^-3/min) were given an intravenous infusion of terbutaline (7 g/kg) over 30 min. Of the four patients in whom the post-drug PPA indices remained within the ARDS range, none survived, whilst five of the six patients in whom the post-drug PPA indices were reduced to below 1.0x10^-3/min survived. PPA indices prior to the administration of terbutaline were not significantly different between the survivor (n=5) and non-survivor (n=5) groups. There was a significant decrease in the PPA indices following terbutaline in survivors (p<0.01) but not in non-survivors. Thus beta-2-agonists in therapeutic doses can inhibit increased lung vascular permeability in man. These findings may have prognostic and therapeutic implication for beta-2-agonists in ARDS.     

Prevalence of mental disorder in an urban population in central Sweden

The principal reason for this epidemiological study was the lack of psychiatric morbidity studies in a predominantly urban population, by psychiatrists in direct interviews. The psychiatric examination, covering 1970-71, included a representative selection of 2,283 persons, 18-65 years old from "former" Stockholm County, and the 12-month prevalence of mental disorders was measured. The total of non-participants was 12%. Forty-seven percent had a psychiatric diagnosis - significantly more women (54%) than men (40%). Excluding the psychosomatic diagnoses, 31% of the population received a psychiatric diagnosis, which agrees closely with other contemporary studies of mental disorder in the Nordic countries. The primary diagnoses were: neuroses 26%, psychosomatic diagnoses 16%, schizophrenic/paranoid conditions or other psychoses 0.6%, affective disorders 0.2%, psychoorganic syndromes 1.2%, psychopathy 0.2%, character neurosis 1%, drug dependence 0.2% (as a primary or a secondary diagnosis 0.6%), alcoholism 1.4% (as a primary or a secondary diagnosis 3.1%) and mental retardation 0.4% (as a primary or a secondary diagnosis 0.8%).     

健康成年人接种两剂新型冠状病毒灭活疫苗后序贯加强免疫重组新型冠状病毒疫苗(CHO细胞)的免疫原性和安全性研究

目的 评价在接种两剂已上市新冠病毒灭活疫苗的人群中序贯加强免疫重组新型冠状病毒疫苗(CHO细胞)后的免疫原性和安全性,为制定新型冠状病毒疫苗的加强免疫策略提供科学依据。 方法 采用开放性试验设计,筛选入组360例已接种两剂新冠病毒灭活疫苗3～4个月、6～8个月、11～13个月的18周岁及以上研究对象并接种1剂重组新型冠状病毒疫苗(CHO细胞)。采集所有研究对象研究用疫苗接种前、接种后14 d血样,用于体液免疫检测,收集研究用疫苗接种后1个月内的所有不良事件。 结果 本研究入组360例研究对象,按研究对象加强免疫与基础免疫间隔时间分3组(A组91～120 d,B组181～240 d,C组331～390 d),各组120例,无研究对象脱落。三组年龄均值分别为38.13、40.22和45.73岁,各组间年龄差异有统计学意义(F=13.516,P＜0.001),A、B组差异无统计学意义(P=0.168),C组和A、B组间差异均有统计学意义(P＜0.001)。三组 IgG GMC(几何平均浓度)免疫前分别为4.81、4.23、2.12 AU/ml,差异有统计学意义(F=10.054,P＜0.001),C组和A、B组间差异均有统计学意义(P＜0.001),A、B组间差异无统计学意义(P=0.520);三组免疫后 IgG GMC 分别为106.69、124.05、80.04 AU/ml,差异无统计学意义(F=2.028,P=0.133)。三组 IgG 抗体阳转率分别为84.17%、87.50%、79.17%,差异无统计学意义(χ²=3.081,P=0.214)。免疫前血清针对Delta变异株和原型株不同组别的中和抗体滴度比较,三组原型株中和抗体GMT(几何平均滴度)为1∶2.18、1∶2.18、1∶2.19,差异无统计学意义(F=0.011,P=0.990);三组Delta变异株中和抗体GMT为1∶2.09、1∶2.17、1∶2.16,差异无统计学意义(F=0.378,P=0.686)。免疫后血清三组原型株中和抗体 GMT为1∶31.09、1∶34.90、1∶21.98,差异无统计学意义(F=2.262,P=0.106);三组Delta变异株中和抗体GMT为1∶61.46、1∶77.44、1∶43.71,差异无统计学意义(F=2.105,P=0.123)。序贯加强免疫后血清对新型冠状病毒Delta变异株和原型株的中和抗体阳转率分别达到82.78%、83.33%。三组 SARS-CoV-2原型株中和抗体阳转率分别为86.67%、87.50%、75.83%,差异有统计学意义(χ²=7.320,P=0.026),其中C组低于A组和B组;三组 SARS-CoV-2 Delta变异株中和抗体阳转率分别为87.50%、84.17%、76.67%,差异无统计学意义(χ²=5.183,P=0.075)。发生不良事件人数为124人,不良事件总体发生率为34.44%。各组发生率分别为35.83%、40.83%和26.67%,差异无统计学意义(χ²=5.487,P=0.064),所有研究对象出现的不良事件以接种部位不良事件为主,主要表现为疫苗接种部位疼痛(23.89%);全身不良事件主要表现为疲劳/乏力(6.94%),未发生与疫苗接种有关的SAEs。结论 接种两剂新型冠状病毒灭活疫苗后序贯加强免疫重组新型冠状病毒疫苗(CHO细胞)具有良好的免疫原性和安全性。较长间隔期的免疫前IgG和免疫后原型株中和抗体阳转率较低,不同间隔期的免疫后IgG 、Delta变异株和原型株中和抗体水平比较差异均无统计学意义,建议基础免疫后6~8个月为最佳的接种间隔。     

Scanning and synthesizing Canadian policies that address the school-to-work transition of youth and young adults with disabilities

BackgroundDisability faced by a young person can impact the school-to-work transition and shape health and well-being over the life course. Unique barriers to entry and advancement within the labor market that are relevant to young people with disabilities underscore the need for tailored policy-level supports.ObjectivesTo examine and describe policies that support the school-to-work transition of young people with disabilities in Canada.MethodsA scan of policies which focused on the school-to-work transition of young people with disabilities across Canada was conducted between June 2019 and January 2020. Searches were completed within federal, provincial and territorial policy portals. Each policy relating to employment participation of people with disabilities was summarized. Policies that focused on the school-to-work-specific were synthesized using Bemelmans-Vidic, Rist and Vedung's policy tool framework.ResultsA total of 36 policies were identified by our scan that focused on the employment of people with disabilities. Only five policies explicitly addressed the school-to-work transition. All existing policies were implemented at the provincial level and aimed to promote entry into employment. The synthesis of policies revealed that financial policy tools were primarily used to incentivize employment, provision of workplace accommodations, or the development and implementation of job readiness programs.ConclusionOur analysis of federal, provincial and territorial policies in Canada uncovered a limited number of policies that specifically support the school-to-work transition. Addressing these policy gaps can increase the inclusion of young people with disabilities in the labor market.     

Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team

We aimed to report the implementation of a phenylketonuria (PKU) transition program and study the effects of follow-up with an adult team on metabolic control, adherence, and loss of follow-up. Fifty-five PKU patients were analysed in the study periods (SP): 2 years before (SP1) and after the beginning of adult care (SP2). Retrospective data on metabolic control and number of clinic appointments were collected for each SP, and protein intakes were analysed. In SP2, three patients (6%) were lost to follow-up. There was a small but statistically significant increase in median number of annual blood spots from SP1 to SP2: 11 (7–15) vs. 14 (7–20); p = 0.002. Mean ± SD of median blood Phe remained stable (525 ± 248 µmol/L vs. 552 ± 225 µmol/L; p = 0.100); median % of blood Phe < 480 µmol/L decreased (51 (4–96)% vs. 37 (5–85)%; p = 0.041) and median number of clinic appointments increased from SP1 to SP2: (5 (4–6) vs. 11 (8–13); p < 0.001). No significant differences were found regarding any parameter of protein intake. Our results suggest that the implementation of an adult service was successful as impact on metabolic control was limited and attendance remained high. Continuous dietetic care likely contributed to these results by keeping patients in follow-up and committed to treatment.     

Diagnostic Testing in Long-Term Care and Resident Emergency Department Visits: A Retrospective Cohort Study

ObjectivesTo investigate the association between rapid access to radiographs, blood tests, urine cultures, and intravenous (IV) therapy in a long-term care (LTC) home with resident transfers to the emergency department (ED).DesignRetrospective cohort study.Setting and Participants21,811 residents living in 162 LTC homes in Ontario, Canada.MethodsWe administered a survey to LTC homes to collect wait times for radiographs, basic blood tests, urine culture, and IV therapy. Rapid availability was defined as typically receiving test results within 1 or 2 days, or same-day IV therapy. We linked the survey results to administrative data and defined a cohort of residents living in survey-respondent homes between January and May 2017. We followed residents in the linked administrative databases for 6 months, until discharge, or death. Two physicians identified diagnostic codes for ED visits that were potentially preventable with rapid availability of each of the 4 resources. Multilevel logistic regression models estimated associations between potentially preventable ED visits and rapid diagnostic tests and intravenous access while controlling for demographic characteristics, illness severity, LTC home size, chain status, and physician availability.ResultsRapid blood tests, radiographs, urine culture, and IV therapy were available in 55%, 47%, 34%, and 45% of LTC homes, respectively. LTC homes that were part of multihome chains were less likely to have rapid access to the 4 resources. Of the 4736 residents (27%) who visited an ED during follow-up, individuals from homes with rapid access to radiographs (odds ratio 0.79, 95% confidence interval 0.66-0.97), urine culture (0.88, 0.72-1.08), blood tests (0.83, 0.69-1.00), and IV therapy (0.93, 0.70-1.23) tended to have fewer potentially preventable ED visits.Conclusions and ImplicationsRapid access to diagnostic testing and IV therapy in LTC reduced ED visits. Improving access to these resources may prevent ED visits and allow residents to stay home.     

成人游戏力与3~8岁儿童脑功能发展的关系

3~8岁是儿童脑功能发育的重要时期,该阶段儿童大脑及其功能具有较强可塑性,而早期经验的剥夺会导致中枢神经系统发育停滞,甚至萎缩并构成永久性损害。儿童早期经验的获得大部分来自游戏,而游戏的开展离不开成年人的参与,越来越多的研究揭示了游戏力对脑功能的重要作用。本文旨在综述成人游戏力对儿童脑功能发育,如在感觉和动作、注意力和意识、学习和记忆中的作用。本综述还简述了游戏类型对脑功能的影响及现有的挑战,以期从父母、教师等成人参与游戏的角度来促进儿童脑功能健康发展。