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Context: Huntington's disease (HD) is a neurodegenerative disorder characterized by mid-life onset, cognitive decline, and behavioral disturbance. Objective: We conducted a review of the end-of-life (EOL) experience of HD patients and their families. Methods: We searched 5 electronic databases. Eligible studies were published in English and contained outcomes related to PC, end-of-life (EOL), advance directives (ADs), symptom management, or hospice use for HD adults. Results: We screened 1566 studies, assessed 244 studies, and included 27 studies. Symptom Prevalence: Decedent data showed greater likelihood of pneumonia, choking, nutritional deficiencies, and skin ulcers. HD patients in hospice experienced pain, anxiety, nausea, and dyspnea. Psychiatric symptoms included dysphoria, agitation, irritability, apathy, and anxiety. Psychosis is associated with worse cognition, function, and behavioral disturbance. Symptom Management: Electroconvulsive therapy (ECT) and venlafaxine improved depression. Suicidal ideation improved with talking, self-management, medication, and discussing EOL wishes. Tetrabenazine improved chorea. Experience as Illness Progressed: HD patients require home care within two years of diagnosis. Only one study reported use of palliative care services (4%). HD patients are admitted to the hospital late in disease course and are often discharged to long-term care facilities (LTCF). Advance Care Planning: Two studies created tools to navigate EOL decisions. Most HD patients had EOL wishes; only familiarity with HD predicted having EOL wishes. Few had ADs or discussed EOL wishes with their families. Clinicians drive EOL discussions. Views on physician-assisted death (PAD) and euthanasia varied widely. Conclusions: Research is needed to further assess the PC needs of HD patients and to provide care recommendations.

Context Little is known about advance care planning (ACP) and palliative care needs among adults with congenital heart disease (ACHD). Objectives The objective of this study was to identify and synthesize studies concerning palliative care among ACHD patients. Methods We searched five electronic databases (PubMed, Embase, SCOPUS, Web of Science, and CINAHL) using the keywords palliative care and congenital heart disease. Inclusion criteria were adults (age > 18 years) with congenital heart disease and publications in English through March 3, 2019. Results Our search yielded 2872 studies, and after removal of duplicates, we screened 2319 abstracts and identified seven for inclusion. Study findings were grouped into three domains: ACP, symptomatology, and end-of-life care. Among the five cross-sectional studies, only 1%–28% of ACHD patients recalled participating in ACP discussions with their doctors but 69%–78% reported a strong interest and desire to participate in ACP. In one study, 46% (n = 67) of patients had elevated anxiety symptoms (Hospital Anxiety and Depression Scale [HADS-A] ≥ 8) and 11% (n = 15) had elevated depressive symptoms (HADS-A ≥ 8). ACHD patients who had a documented goals of care conversation before cardiac decompensation had a lower incidence of resuscitation and aggressive treatments at end of life (12% [n = 3] vs. 100% [n = 12], P Conclusion While few ACHD patients complete advance directives, our findings support that many ACHD patients recognize the value of initiating end-of-life and goals of care conversations early on in the course of illness. Future studies investigating communication and implementation strategies of ACP as well as the symptom experience of patients with ACHD are needed.

OBJECTIVE To compare complications and functional outcomes of treatment with primary distal femoral replacement (DFR) versus open reduction and internal fixation (ORIF). DATA SOURCES PubMed, Embase, and Cochrane databases were searched for English language studies up to May 19, 2020, identifying 913 studies. STUDY SELECTION Studies that assessed complications of periprosthetic distal femur fractures with primary DFR or ORIF were included. Studies with sample size ≤5, mean age

Objectives A systematic review to analyze communication rates of complementary and integrative health (CIH) and analyze how communication terms, such as “disclosure,” are measured and operationalized. Methods We searched seven databases for studies published between 2010 and 2018 with quantitative measurements of patients’ communication of CIH to a biomedical clinician. We analyzed communication terms used to describe patients reporting CIH usage. We also examined the conceptual and operational definitions of CIH provided and whether those terms were explicitly operationalized. We aggregated the percentage, rate, or ratio of CIH users that communicated about CIH with their clinicians by disease type and geographical region. Results 7882 studies were screened and 89 included in the review. Studies used a wide range of conceptual and operational definitions for CIH, as well as 23 different terms to report communication related to reporting CIH usage. Usage varied by disease type and geographical region. Conclusions Studies of CIH and CIH communication may measure different kinds of social and communicative phenomena, which makes comparison across international studies challenging. Practice Implications Future studies should employ standardized, replicable measures for defining CIH and for reporting CIH communication. Clinicians can incorporate questions about prior, current, and future CIH use during the medical visit.

There is growing interest in providing palliative care (PC) in the emergency department (ED), but relatively little is known about the efficacy of ED-based PC interventions. A 2016 systematic review on this topic found no evidence that ED-based PC interventions affect patient outcomes or health care utilization, but new research has emerged since the publication of that review.This systematic review provides a concise summary of current literature addressing the impact of ED-based PC interventions on patient-reported or family reported outcomes, health care utilization, and survival.We searched PubMed, Embase, Web of Science, Scopus, and the Cumulative Index to Nursing and Allied Health Literature from inception until September 1, 2018 and reviewed references. Eligible articles evaluated the effects of PC interventions in the ED on patient-reported or family reported outcomes, health care utilization, or survival.We screened 3091 abstracts and 98 full-text articles with 13 articles selected for final inclusion. Two articles reported the results of a single randomized controlled trial, whereas the remaining 11 studies were descriptive or quasi-experimental cohort studies. More than half of the included articles were published after the previous systematic review on this topic. Populations studied included older adults, patients with advanced malignancy, and ED patients screening positive for unmet PC needs. Most interventions involved referral to hospice or PC or PC provided directly in the ED. Compared with usual care, ED-PC interventions improved quality of life, although this improvement was not observed when comparing ED-PC to inpatient PC. ED-PC interventions expedited PC consultation; most studies reported a concomitant reduction in hospital length of stay and increase in hospice utilization, but some data were conflicting. Short-term mortality rates were high across all studies, but ED-PC interventions did not decrease survival time compared with usual care.Existing data support that PC in the ED is feasible, may improve quality of life, and does not appear to affect survival.

OBJECTIVEAdjuvant radiotherapy has become a common addition to the management of high-grade meningiomas, as immediate treatment with radiation following resection has been associated with significantly improved outcomes. Recent investigations into particle therapy have expanded into the management of high-risk meningiomas. Here, the authors systematically review studies on the efficacy and utility of particle-based radiotherapy in the management of high-grade meningioma.METHODSA literature search was developed by first defining the population, intervention, comparison, outcomes, and study design (PICOS). A search strategy was designed for each of three electronic databases: PubMed, Embase, and Scopus. Data extraction was conducted in accordance with the PRISMA guidelines. Outcomes of interest included local disease control, overall survival, and toxicity, which were compared with historical data on photon-based therapies.RESULTSEleven retrospective studies including 240 patients with atypical (WHO grade II) and anaplastic (WHO grade III) meningioma undergoing particle radiation therapy were identified. Five of the 11 studies included in this systematic review focused specifically on WHO grade II and III meningiomas; the others also included WHO grade I meningioma. Across all of the studies, the median follow-up ranged from 6 to 145 months. Local control rates for high-grade meningiomas ranged from 46.7% to 86% by the last follow-up or at 5 years. Overall survival rates ranged from 0% to 100% with better prognoses for atypical than for malignant meningiomas. Radiation necrosis was the most common adverse effect of treatment, occurring in 3.9% of specified cases.CONCLUSIONSDespite the lack of randomized prospective trials, this review of existing retrospective studies suggests that particle therapy, whether an adjuvant or a stand-alone treatment, confers survival benefit with a relatively low risk for severe treatment-derived toxicity compared to standard photon-based therapy. However, additional controlled studies are needed.

Context Immune checkpoint inhibitors (ICIs) are increasingly used to treat a variety of cancers, but comparatively little is known about patient-reported outcomes (PROs) and health-related quality of life (HRQoL) among patients receiving these novel therapies. Objectives We performed a systematic review to examine PROs and HRQoL among cancer patients receiving ICIs as compared to other anticancer therapies. Methods We systematically searched PubMed, CINAHL, Embase, Web of Science, and Scopus, using search terms representing ICIs, PROs, and HRQoL on August 10, 2018. Eligible articles were required to involve cancer patients treated with ICIs and to report PROs and/or HRQoL data. Results We screened 1453 references and included 15 publications representing 15 randomized controlled trials in our analysis. Studies included several cancer types (melanoma, lung cancer, genitourinary cancer, and head/neck cancer), used four different ICIs (nivolumab, pembrolizumab, atezolizumab, and ipilimumab), and compared ICIs to a wide range of therapies (chemotherapy, targeted therapies, other immunotherapy strategies, and placebo). Studies used a total of seven different PROs to measure HRQOL, most commonly the European Organisation for the Research and Treatment of Cancer core quality of life questionnaire (EORTC QLQ-C30) (n = 12, 80%). PRO data were reported in a variety of formats and at a variety of time points throughout treatment, which made direct comparison challenging. Some trials (n = 11, 73%) reported PROs on specific symptoms. In general, patients receiving ICIs had similar-to-improved HRQoL and experiences when compared to other therapies. Conclusion Despite the broad clinical trials experience of ICI therapies across cancer types, relatively few randomized studies reported PROs and patient HRQoL data. Available data suggest that ICIs are well tolerated in terms of HRQoL compared to other anticancer therapies although the conclusions are limited by the heterogeneity of trial designs and outcomes. Currently used instruments may fail to capture important symptomatology unique to ICIs, underscoring a need for PROs designed specifically for ICIs.

Context Children with cancer and their families have complex needs related to symptoms, decision-making, care planning, and psychosocial impact extending across the illness trajectory, which for some includes end of life. Whether specialty pediatric palliative care (SPPC) is associated with improved outcomes for children with cancer and their families is unknown. Objective We conducted a systematic review following PRISMA guidelines to investigate outcomes associated with SPPC in pediatric oncology with a focus on intervention delivery, collaboration, and alignment with National Quality Forum domains. Methods We searched PubMed, Embase, Scopus, Web of Science, and CINAHL databases from inception until April 2020 and reviewed references manually. Eligible articles were published in English, involved pediatric patients aged 0-18 years with cancer, and contained original data regarding patient and family illness and end-of-life experiences, including symptom management, communication, decision-making, quality of life, satisfaction, and healthcare utilization. Results We screened 6682 article abstracts and 82 full-text articles; 32 studies met inclusion criteria, representing 15,635 unique children with cancer and 342 parents. Generally, children with cancer who received SPPC had improved symptom burden, pain control, and quality of life with decreased intensive procedures, increased completion of advance care planning and resuscitation status documentation, and fewer end-of-life intensive care stays with higher likelihood of dying at home. Family impact included satisfaction with SPPC and perception of improved communication. Conclusion SPPC may improve illness experiences for children with cancer and their families. Multisite studies utilizing comparative effectiveness approaches and validated metrics may support further advancement of the field.

Context Patients with significant burn injuries likely have palliative care needs. Objectives We performed a systematic review of existing evidence concerning the palliative care needs of burn patients. Methods Through November 26, 2018, we systematically searched PubMed, CINAHL, Embase, Web of Science, and Scopus, using terms representing burn injuries and the eight domains of quality palliative care as outlined by the National Consensus Project for Quality Palliative Care. Eligible articles involved burn-injured patients treated with an intervention targeting at least one of the eight domains. Results Our searches yielded 7532 unique records, which led to 238 articles for full review and 88 studies that met inclusion criteria. Seventy-five studies addressed the domain physical aspects of care and merit a separate systematic review; 13 studies were included in our final review. Four of the seven domains—processes of care, psychologic symptoms, social aspects, and end of life—were addressed by studies but three domains—spiritual, cultural, or ethics—were unaddressed. Included studies highlight potential benefits from peridischarge self-care education programs, peer support, and group therapy in improving quality of life. In patients with severe injuries, end-of-life decision-making protocols were associated with increased utilization of comfort-focused treatments. Conclusion Most existing palliative care-related research in burn patients addresses interventions for physical symptoms with minimal literature concerning other domains. Opportunities exist for further research of palliative care in burn populations with emphasis on addressing interventions for all domains and better standardizing the language and outcomes for the palliative care interventions.

Context Individuals with cystic fibrosis (CF) frequently survive into adulthood, and many have multifaceted symptoms that impair quality of life. Objective We conducted a systematic review to investigate opportunities to improve utilization of palliative care among adults with CF. Methods We searched PubMed, Embase, Scopus, Web of Science, and CINAHL databases from inception until September 27, 2018, and reviewed references manually. Eligible articles were published in English, involved adults aged 18 years and older with CF, and contained original data regarding patient outcomes related to presence of advance care planning (ACP), symptom experience, and preferred and/or received end-of-life (EOL) care. Results We screened 652 article abstracts and 32 full-text articles; 12 studies met inclusion criteria. All studies were published between 2000 and 2018. Pertinent findings include that although 43% to 65% of adults with CF had contemplated completing ACP, the majority only completed ACP during their terminal hospital admission. Patients also reported high prevalence of untreated symptoms, with adequate symptom control reported in 45% among those with dyspnea, 22% among those with pain, and 51% among those with anxiety and/or depression. Prevalence of in-hospital death ranged from 62% to 100%, with a third dying in the intensive care unit. The majority received antibiotics and preventative treatments during their terminal hospitalization. Finally, treatment from a palliative care specialist was associated with a higher prevalence of patient completion of advanced directives, decreased likelihood of death in intensive care unit, and decreased use of mechanical ventilation at EOL. Conclusion Adults with CF often have untreated symptoms, and many opportunities exist for palliative care specialists to improve ACP completion and quality of EOL care.

This study investigated the prevalence of bisexual behaviors and marital status among Chinese men who have sex with men (MSM) and the associations between MSMs condom use and their heterosexual behaviors. Eight hundred ninety-six adult males in Yunnan Province China who reported to ever have engaged in MSM behaviors were interviewed. Data were acquired through the China-UK HIV/AIDS Prevention and Care Project from 2003 to 2006. Of all respondents one-third had ever been married 59% had ever engaged in bisexual behaviors and 31% had done so in the past 6 months. High prevalence of inconsistent condom use was reported in heterosexual behaviors (71.9%) as well as with those who had engaged in MSM sexual behaviors in the past 6 months (30.8% with commercial sex workers and 54.7% with noncommercial sex partners) in the past 6 months. Those who did not use condoms with MSM partners were also more likely than others to not use condoms with their female sex partners (FSP). Those who had voluntary counseling and testing services were more likely than others to have used a condom in the last episode of sex with their FSP (multivariate odds ratio = 1.66). The clustering of unprotected sexual behaviors with male and FSP among bisexual MSM is revealed. The bridging effects of the risk for human immunodeficiency virus transmission from the MSM population to the female population are evident. (authors)

Swimming is a popular activity and sport among people of all ages. Outbreaks of illness caused by infectious organisms associated with swimming pool use are reported with regularity (Yoder et al. 2004). Proper pool maintenance, especially appropriate water disinfection procedures, and monitoring of pool water quality are key factors in the prevention of infectious diseases associated with use of swimming pools (Nemery et al. 2002). However, although chlorine kills many potential pathogens, it can also react with human wastes such as perspiration, urine, skin particles, and lotion in pool water to form chloramines and trihalomethanes. Chloramines may remain in the pool water or volatilize into the air, where they create the pungent smell and acutely irritating properties of air above swimming pools (Aggazzotti et al. 1990; Goyder 2000; Hery et al. 1995). Chloramines have been suspected as a cause of occupational asthma and pneumonitis among lifeguards (Massin et al. 1998; Nemery et al. 2002; Thickett et al. 2002). Also, an outbreak associated with a swimming pool and attributed to chloramine exposure led to cough or ocular symptoms in > 70% of pool patrons interviewed (Goyder 2000). Chloramine levels in pool water depend on chlorine and nitrogen concentrations, pool pH, temperature, and water circulation patterns (Hery et al. 1995; Massin et al. 1998; Nemery et al. 2002). However, chloramine levels in the air above swimming pools are also influenced by ventilation and the pool water chemistry (Hery et al. 1995; Massin et al. 1998). Indoor pools are likely to be less well ventilated than outdoor pools, so the risks associated with chloramine exposure are likely to differ between indoor and outdoor swimming pools. We investigated two outbreaks of acute ocular and respiratory symptoms associated with exposure to indoor swimming pools. In January 2004, the Illinois Department of Public Health was notified of two outbreaks of acute ocular and respiratory symptoms associated with indoor swimming pool exposure among patrons of two hotels located in noncontiguous central Illinois counties. We investigated these outbreaks during January 2004 to describe illness syndromes, determine risk factors for illness, and develop recommendations to prevent future incidents.

Background: Ethnic variations in breast cancer epidemiology and genetics have necessitated investigation of the spectra of BRCA1 and BRCA2 mutations in different populations. Knowledge of BRCA mutations in Chinese populations is still largely unknown. We conducted a multi-center study to characterize the spectra of BRCA mutations in Chinese breast and ovarian cancer patients from Southern China. Methodology/Principal Findings: A total of 651 clinically high-risk breast and/or ovarian cancer patients were recruited from the Hong Kong Hereditary Breast Cancer Family Registry from 2007 to 2011. Comprehensive BRCA1 and BRCA2 mutation screening was performed using bi-directional sequencing of all coding exons of BRCA1 and BRCA2. Sequencing results were confirmed by in-house developed full high resolution DNA melting (HRM) analysis. Among the 451 probands analyzed, 69 (15.3%) deleterious BRCA mutations were identified, comprising 29 in BRCA1 and 40 in BRCA2. The four recurrent BRCA1 mutations (c.470_471delCT, c.3342_3345delAGAA, c.5406+1_5406+3delGTA and c.981_982delAT) accounted for 34.5% (10/29) of all BRCA1 mutations in this cohort. The four recurrent BRCA2 mutations (c.2808_2811delACAA, c.3109C>T, c.7436_7805del370 and c.9097_9098insA) accounted for 40% (16/40) of all BRCA2 mutations. Haplotype analysis was performed to confirm 1 BRCA1 and 3 BRCA2 mutations are putative founder mutations. Rapid HRM mutation screening for a panel of the founder mutations were developed and validated. Conclusion: In this study, our findings suggest that BRCA mutations account for a substantial proportion of hereditary breast/ovarian cancer in Southern Chinese population. Knowing the spectrum and frequency of the founder mutations in this population will assist in the development of a cost-effective rapid screening assay, which in turn facilitates genetic counseling and testing for the purpose of cancer risk assessment. © 2012 Kwong et al., published_or_final_version