Pancreatic INI1-deficient undifferentiated rhabdoid carcinoma achieves complete clinical response on gemcitabine and nab-paclitaxel following immediate progression on FOLFIRINOX: a case report

Introduction: INI1-deficient undifferentiated rhabdoid carcinoma is a rare pancreatic carcinoma for which the optimal treatment is unknown. Pancreatic ductal adenocarcinoma, the most common histology of pancreas cancer, is treated with combination chemotherapy in the advanced setting, a strategy supported by strong evidence in well powered studies. In patients with excellent performance status, first-line treatment usually consists of the three-drug regimen FOLFIRINOX, with the combination of gemcitabine with nab-paclitaxel, typically less toxic than the three-drug regimen, reserved for second-line therapy. Given the lack of published reports describing treatment outcomes for patients with rare forms of pancreatic cancer, the same treatment approach used for pancreatic ductal adenocarcinoma is typically employed. Observation: This case describes a patient with metastatic pancreatic INI1-deficient undifferentiated rhabdoid carcinoma who was primarily resistant to FOLFIRINOX therapy but who then achieved an immediate, marked and sustained response to gemcitabine with nab-paclitaxel. Conclusion: Given the lack of data informing on optimal management of INI1-deficient pancreatic undifferentiated rhabdoid carcinoma, and the exceptional response achieved by gemcitabine with nab-paclitaxel, this case report highlights a surprising and potentially informative anecdote. Additional studies are needed to confirm responses observed in this report which when taken together may strongly influence first-line therapy choice for this rare malignancy. Given the difficult in acquiring sufficient numbers of these rare histologies in any one institution, multi-institution collaboration in studying outcomes of rare pancreatic malignancies is likely essential.     

以血液系病变为首诊的系统性红斑狼疮20例临床分析

目的：探讨系统性红斑狼疮(SLE)的血液系统改变。方法：20例以血液系统病变为首诊的SLE患，分析其临床表现、体征及外周血象、骨髓象改变。结果：20例患中贫血16例，白细胞减少10例，血小板减少12例，骨髓细胞学检查示：缺铁性贫血(IDA)2例，再生障碍性贫血(AA)1例，溶血性贫血(HA)3例，环形铁粒幼细胞性贫血1例，原发性血小板减少性紫癜(ITP)2例，余骨髓象为非特异性改变。结论：以血液系统改变为首发或突出症状的SLE患，早期临床症状不典型，故对于年轻女性，若伴有骨髓象示ITP、AIHA或血细胞减少而骨髓细胞形态无异常，应进行免疫学筛选及随访，警惕SLE可能。     

Using a site visit to a contaminated location as a focus for environmental health education for academic and public health nurses

We describe a conference initiative that is distinguished by the use of a "community case study" to increase the knowledge and skills of nursing faculty and public health nurses in environmental health and to provide networking support to facilitate infusion of environmental health into nursing curricula and public health nursing practice. The Institute of Medicine's (1995) general environmental health competencies for nurses provided the conference framework. Woburn, Massachusetts, a Superfund site, served as the community case study to illustrate a complex environmental health problem. Over an extended period of time, Woburn was contaminated with multiple chemicals that eventually contaminated the drinking water supply; a cluster of childhood leukemia cases was linked subsequently to the Superfund site contaminants. A 6-hr interpreted walking and bus tour of the Superfund site enabled us to visit the premises of responsible parties, the vapor extraction fields, the capped Well H in the wooded wetlands, and to tour the affected neighborhood. This intensive, hands-on approach to learning environmental health content and skills that incorporated multiple learning strategies serves as a model for developing future conferences for public health nurses and nursing faculty.     

Association of cardiovascular emerging risk factors with acute coronary syndrome and stroke: A case‐control study

In this study, we estimated the risk of acute coronary syndrome and stroke associated with several emerging cardiovascular risk factors. This was a case‐control study, where an age ‐ and sex‐matched acute coronary syndrome group and stroke group were compared with controls. Demographic and clinical data were collected through patient interviews, and blood samples were taken for analysis. In the bivariate analysis, all cardiovascular risk factors analyzed showed as predictors of acute coronary syndrome and stroke, except total cholesterol and smoking. In the multivariate logistic regression model for acute coronary syndrome, hypertension and body mass index, N‐terminal section brain natriuretic peptide and pregnancy‐associated plasma protein‐A were independent predictors. For stroke, the predictors were hypertension, diabetes mellitus, body mass index, and N‐terminal section brain natriuretic peptide. Controlling for age, sex, and classical cardiovascular risk factors, N‐terminal section brain natriuretic peptide and pregnancy‐associated plasma protein‐A were independent emerging cardiovascular risk factors for acute coronary syndrome, but pregnancy‐associated plasma protein‐A was not for stroke. High levels of cardiovascular risk factors in individuals with no episodes of cardiovascular disease requires the implementation of prevention programs, given that at least half of them are modifiable.     

修订的阿尔茨海默病诊断标准适用于中低收入国家吗?（英文）

阿尔茨海默病(Alzheimer’s Disease,AD)是老年人主要疾病负担之一。在中等收入国家,如中国,整体健康状况改善、寿命延长以及其他一些因素使得人口迅速老龄化,AD所致的负担日益加重。近来AD诊断标准的修订,则反映了过去三十年中对该病认识的新进展。不同国际组织提出不同的AD诊断标准,这些标准都将AD疾病谱细分为若干阶段,所分的阶段彼此有些重叠。某些情况下,诊断标准要求同时存在记忆受损的表现以及特定的生物标记物。然而,这些修订的标准有几大不足:需要有训练有素的临床医生才能明确区分不同的阶段;诊断标准提及的生物标记物在AD的发生和发展中的作用仍然不明确;需要由训练有素的技术人员使用昂贵的高科技设备来评估这些生物标记物。上述问题限制了这些诊断标准的临床应用,资源匮乏的地方受限更甚,因为那里负责诊断和治疗AD患者的临床医生获得的培训有限,并且还缺少设备来确定上述生物标记物。     

‘Too much for one day’: a case study of disclosure in the paediatric HIV/AIDS clinic

Background

Despite acknowledgement of the complexity and challenges of the process of disclosure of HIV status to adolescents, little work has been carried out on the communication features which facilitate or hinder the process. This qualitative case study reflects the interactional dynamics of an interaction between caregiver, physician and counsellor around the topic of disclosure in a South African clinic.

Methods

A 40‐minute encounter between a doctor, the grandmother of an adolescent with HIV/AIDS and a counsellor around the process of disclosure was transcribed and examined in detail in relation to its structure, topics, timing and sequence as well as its movement towards a collaborative ending. Analysis was based on elements of conversational and thematic analysis.

Results

The session was characterized by distinct stages and the emergence of multiple voices and perspectives from the participants. The negotiated management of the session sheds light on multiple barriers to care as well as the influence of contextual factors on the process. The important mediating role of the counsellor in the triad emerges. The analysis highlights both unique features of the consultation and common challenges to clinicians when disclosing.

Discussion and Conclusions

The task of disclosure is complex and intricate. Results suggest team processes are critical, as are temporal and contextual factors and the need for an understanding of communication. Implications for the clinic team are discussed and suggestions made for the development of collaborative partnership based on an understanding of roles and responsibilities, time factors and enhancement of features such as trust and communication.     

Attachment-Based Family Therapy for Adolescent Self-Injury

There is growing awareness in the popular media and in clinical reports of the increasing prevalence of self-injury, especially among adolescents. Self-injury affects the entire family and is, in turn, affected by the family system. Thus, family therapy is an important component when working with adolescents who are still dependent on their families. However, available treatments, for the most part, do not address the relational-systemic aspects of self-harm, and treat only the individual. This article situates self-harm within the relational framework of adolescent attachment theory and presents a case study demonstrating the treatment of adolescent self-injury with attachment-based family therapy.