Cancer patients' expectations of advance care planning: A typological content analysis of qualitative interviews.

BACKGROUND: Advance care planning (ACP) can help to elicit cancer patients' preferences in a discussion process to promote person-centred medical decision-making. Expectations are known to be highly relevant determinants of decisional processes. So far, however, little is known about cancer patients' expectations of ACP that lead to acceptance or refusal of the programme. The presented study, therefore, aims to explore cancer patients' expectations of ACP. METHODS: Semi-structured interviews were conducted with a purposeful sample of 27 cancer patients consenting to or refusing a newly implemented ACP programme in a German university hospital. Data were analysed using typological content analysis. RESULTS: We identified five different expectation clusters in relation to ACP. Consenting participants held expectations about the impact of ACP that were either 'ego-centred' or 'family-centred'. Refusers had expectations based on ignorance and misinformation, or-if they had already completed an advance directive-expectations to avoid unpleasant redundancy, perceiving no additional benefit but a burden from ACP. Finally, refusers in particular expressed expectations of delegated responsibility at the end of life, including anticipation of proxy decision-making. CONCLUSION: Our study results suggest that expectation-modifying measures could be taken to positively influence cancer patients' expectations and thus the acceptance of ACP. In this respect, reducing ignorance and misguided expectations plays a decisive role. Especially in family constellations with expected delegation of responsibility and dependence at the end of life, it might be important to promote ACP as a family-intervention to improve family outcomes.

Non-lesional treatments for tremor in Parkinson's disease: A systematic review and meta-analysis.

INTRODUCTION: Tremor is often perceived as severely disabling by patients with idiopathic Parkinson's disease (iPD) and yet ranges among the most difficult symptoms to treat. To date, no comprehensive analysis of non-lesional therapies to manage tremor in iPD exists to base recommendations upon. We therefore present a systematic literature review and meta-analysis assessing the efficacy/effectiveness and safety of non-lesional treatments for tremor in iPD. METHODS: Three electronic databases were searched using a combination of title/abstract keywords complemented by hand-searching of reference lists. A random-effects meta-analysis of standardized mean change scores was conducted where appropriate. RESULTS: Some 114 studies met inclusion criteria involving 8045 patients. The meta-analysis revealed an overall reduction of standardized mean change scores by (-0.93 [CI: -1.42; -0.43], p < 0.001) by 14 different dopaminergic and non-dopaminergic classes of agents. No significant differences were identified between direct comparisons. Subgroup analysis comparing dopamine receptor agonists resulted in superior effects of pramipexole and rotigotine compared with ropinirole. There was little cumulative evidence to support the use of individual non-pharmacological interventions for tremor, except for electrical stimulation. CONCLUSIONS: The results of this meta-analysis suggest a large but nonspecific effect of established pharmacological therapies on tremor in iPD. Based on high-quality studies, there is sufficient evidence to support that levodopa, dopamine receptor agonists, and monoamine oxidase inhibitors provide tremor relief in most patients, while evidence supporting other treatments is less well established. Sufficient evidence to draw conclusions on effects of non-lesional treatments in cases with refractory tremor is lacking.

Neutrophil gelatinase-associated lipocalin as a biomarker for lupus nephritis.

BACKGROUND: One of the challenges of treating patients with lupus nephritis (LN) is to accurately assess disease activity and predict its outcome. Since renal-biopsy cannot be performed routinely, new surrogate biomarkers are needed. METHODS: We evaluated neutrophil gelatinase-associated lipocalin (NGAL), to predict renal outcome in LN. Serum and urinary NGAL levels, measured by the enzyme-linked immunosorbent assay, and the fractional excretion (FE) of NGAL relative to the FE of proteins (FE NGAL/FE protein ratio) were determined in a cross-sectional (n = 199) and longitudinal (n = 45) cohort of systemic lupus erythematosus (SLE) patients. Global and renal disease activity was assessed by the SLE disease activity indices, SLEDAI and rSLEDAI, respectively. Correlations between traditional biomarkers were established. Sensitivity, specificity and predictive values of NGAL for renal flare, response to therapy and progression to chronic kidney disease were calculated. RESULTS: The FE NGAL/FE protein ratio exhibited the best sensitivity and specificity to discriminate patients with active LN from those with non-renal flare and inactive SLE. In the prospective study, this biomarker was found to be the best candidate to predict proteinuric flares with an 87% sensitivity and 62% specificity for ratios >14.56 and complete response with a 61% sensitivity and 78% specificity for ratios >26.54 in the presence of a simultaneous worsening or improving rSLEDAIs, respectively. In both conditions, the FE NGAL/FE protein ratio outperformed the anti-dsDNA antibody titres and C3 predictive value. Progression to chronic kidney disease was best predicted by estimated glomerular filtration rate levels, but persistently high levels of serum NGAL (>444.4 ng/mL, P = 0.0001 by Kaplan-Meier) predicted a faster progression. CONCLUSIONS: The FE NGAL/FE protein ratio is a reliable marker of disease activity in patients with SLE and could be used as an indicator of response to therapy, although further studies are required to confirm these results.

The Quality in Quality of Life in Parkinson's Disease: A Qualitative Meta-Synthesis.

BACKGROUND: Quality of life (QoL) is known to be impaired in people with Parkinson's disease (PwPD). Not surprisingly, a considerable effort of health interventions is aimed at maintaining or improving QoL. Yet, little is known about its determinants from a PwPD perspective to inform person-centered health care interventions. OBJECTIVES: This systematic review aims to overcome this information gap by synthesizing existing evidence on factors associated with PwPD' self-perceived QoL. METHODS: We searched six electronic databases (MEDLINE, EMBASE, PsycINFO, CINAHL, Web of Science, Cochrane Library) from inception to January 2022 for eligible qualitative studies of QoL in PwPD, supplemented by citation tracking and hand searching. Study quality was assessed using the QualSyst tool. In order to characterize the determinants of QoL in PwPD, we conducted a qualitative meta-synthesis. RESULTS: Our analysis revealed a wide range of facilitators and barriers to QoL relating to seven overarching themes: Illness experience, health care, everyday life, social life, identity, spirituality/religion, and environment. CONCLUSIONS: Our systematic review reinforces the impact of symptom experience on PwPD's QoL. However, it also highlights the need to consider the non-physical dimensions of PD when assessing patients' QoL. It is therefore essential that health care professionals acknowledge the psychological, social and spiritual repercussions of PD and endeavor to respond to these concerns through a comprehensive and patient-centered strategy. Further research is needed to gain a deeper understanding of these facets of PD and to formulate successful interventions aimed at improving the QoL of PwPD.

Factors Associated with Preferred Place of Care and Death in Patients with Parkinson's Disease: A Cross-Sectional Study.

Background: A significant proportion of people with Parkinson's disease (PwPD) die in hospital settings. Although one could presume that most PwPD would favor being cared for and die at home, there is currently no evidence to support this assumption. Objective: We aimed at exploring PwPD's preferences for place of end-of-life care and place of death, along with associated factors. Methods: A cross-sectional study was conducted to investigate PwPD's end-of life wishes regarding their preferred place of care and preferred place of death. Using different approaches within a generalized linear model framework, we additionally explored factors possibly associated with preferences for home care and home death. Results: Although most PwPD wished to be cared for and die at home, about one-third reported feeling indifferent about their place of death. Preferred home care was associated with the preference for home death. Furthermore, a preference for dying at home was more likely among PwPD's with informal care support and spiritual/religious affiliation, but less likely if they preferred institutional care towards the end of life. Conclusions: The variation in responses regarding the preferred place of care and place of death highlights the need to distinguish between the concepts when discussing end-of-life care. However, it is worth noting that the majority of PwPD preferred care and death at home. The factors identified in relation to preferred place of care and death provide an initial understanding of PwPD decision-making, but call for further research to confirm our findings, explore causality and identify additional influencing factors.

Safety perception in patients with advanced idiopathic Parkinson's disease - a qualitative study.

Background: A fundamental cornerstone of quality of healthcare is patient safety, which many people with life-limiting illnesses feel is being compromised. Perceptions of impaired safety are associated with the occurrence of psychological distress and healthcare utilization. However, little is known about how people with idiopathic Parkinson's disease (iPD) perceive their own safety toward the end of life. The aim of our study was therefore to investigate factors that influence the perception of safety of patients with advanced iPD. Methods: We conducted semi-structured interviews with a purposeful sample of 21 patients with advanced iPD. Participants were recruited at the neurology department of a tertiary care hospital in Germany between August 2021 and June 2022. Data were analyzed using reflexive thematic analysis. Results: iPD-patients reported relevant impairment of their safety. While most participants expressed safety concerns based on the manifestation of disease, our analysis identified enablers and barriers to establishing safety in patients with advanced iPD, in 10 additional domains: relationship to the disease, informedness, self-perception, utilization of support and care structures, healthcare professionals and structures, treatment, social interaction, social security, spirituality, and environment. Conclusion: This study provides new insights into safety perceptions of patients with advanced iPD, which extend well beyond the physical realm. The findings suggest that clinicians and policy makers should consider a holistic and multidisciplinary approach to assessing and improving patients' safety taking into account the enablers and barriers identified in this study.

Rare case of nephrotic syndrome: Schimke syndrome.

Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case.

Resumo A síndrome Schimke corresponde à displasia imuno-óssea, associada à doença renal progressiva secundária à síndrome nefrótica córtico-resistente, podendo haver outras anormalidades como hipotireoidismo e aplasia de medula óssea. Trata-se de uma patologia genética rara, com poucos relatos na literatura. O acometimento renal mais frequente é uma síndrome nefrótica por glomeruloesclerose segmentar e focal e falência renal progressiva. O objetivo deste estudo foi relatar um caso de síndrome de Schimke, investigação diagnóstica e condução do caso.

Blood transfusion reactions in children: associated factors.

OBJECTIVE: To analyze the profile of blood transfusion reactions in children and to identify the involved factors. METHODS: This was a cross-sectional study in a tertiary pediatric teaching hospital from the public healthcare system, involving all children admitted from January to July of 2011 (5,437), of which 1,226 received blood transfusions, constituting the sample. A documental study was performed by analyzing files from the hemovigilance service and notification forms of transfusion reactions. The variables investigated were: number and type of blood components transfused, transfusion site, reaction site, age, gender, type of blood components involved, type of incident, and previous history of multiple transfusions. A descriptive and inferential analysis was performed, using statistical tests to establish the association between the variables. RESULTS: There were 57 transfusion incidents involving 47 children and 72 different blood products, thus constituting a prevalence of reactions of 3.8%. At the inferential analysis, the chi-squared test showed that the following variables were significantly associated (p<0.05) with the type of reaction: age range and type of blood component. Similarly, the patient's underlying disease was associated with previous history of transfusion incident and type of blood component. CONCLUSIONS: The prevalence of transfusion reactions in children is high, and the intervening factors are: type of blood component, age, patient comorbidity, and multiple transfusions; type of blood component and age are also associated with type of reaction.

Rare case of nephrotic syndrome: Schimke syndrome / Caso raro de síndrome nefrótica: síndrome de Schimke

Abstract Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case.

Resumo A síndrome Schimke corresponde à displasia imuno-óssea, associada à doença renal progressiva secundária à síndrome nefrótica córtico-resistente, podendo haver outras anormalidades como hipotireoidismo e aplasia de medula óssea. Trata-se de uma patologia genética rara, com poucos relatos na literatura. O acometimento renal mais frequente é uma síndrome nefrótica por glomeruloesclerose segmentar e focal e falência renal progressiva. O objetivo deste estudo foi relatar um caso de síndrome de Schimke, investigação diagnóstica e condução do caso.

Reações transfusionais em crianças: fatores associados / Blood transfusion reactions in children: associated factors

OBJETIVO: Analisar o perfil das reações transfusionais em crianças e identificar os fatores intervenientes. MÉTODOS: Estudo transversal realizado em um hospital-escola pediátrico terciário da rede pública, envolvendo todas as crianças internadas de janeiro a julho de 2011 (5437), das quais 1.226 foram hemotransfundidas, constituindo, assim, a amostra. Realizado estudo documental dos relatórios do serviço de hemovigilância e das fichas de notificação de reações transfusionais. As variáveis investigadas foram: número e tipo de hemocomponente transfundido, local de transfusão, local da reação, idade, sexo, tipo de hemocomponente envolvido, tipo de incidente e história prévia de politransfusão. Realizada análise descritiva e inferencial, utilizando-se testes estatísticos para estabelecer associação entre as variáveis. RESULTADOS: Verificou-se 57 incidentes transfusionais envolvendo 47 crianças distintas e 72 hemocomponentes, constituindo uma prevalência de reações de 3,8%. Na análise inferencial, pelo teste do Qui-quadrado, as seguintes variáveis apresentaram associação estatisticamente significativa (p < 0,05) com o tipo de reação: faixa etária e tipo de hemocomponente envolvido. Analogamente, a doença de base do paciente associou-se com a história prévia de incidente transfusional e o tipo de hemocomponente. CONCLUSÕES: A prevalência de reações transfusionais em crianças é elevada, e os fatores intervenientes são o tipo de hemocomponente, a faixa etária, comorbidade do paciente e politransfusão, sendo que os dois primeiros associam-se, também, com tipo de reação ocorrida.

OBJECTIVE:To analyze the profile of blood transfusion reactions in children and to identify the involved factors. METHODS: This was a cross-sectional study in a tertiary pediatric teaching hospital from the public healthcare system, involving all children admitted from January to July of 2011 (5,437), of which 1,226 received blood transfusions, constituting the sample. A documental study was performed by analyzing files from the hemovigilance service and notification forms of transfusion reactions. The variables investigated were: number and type of blood components transfused, transfusion site, reaction site, age, gender, type of blood components involved, type of incident, and previous history of multiple transfusions. A descriptive and inferential analysis was performed, using statistical tests to establish the association between the variables. RESULTS: There were 57 transfusion incidents involving 47 children and 72 different blood products, thus constituting a prevalence of reactions of 3.8%. At the inferential analysis, the chi-squared test showed that the following variables were significantly associated (p < 0.05) with the type of reaction: age range and type of blood component. Similarly, the patient's underlying disease was associated with previous history of transfusion incident and type of blood component. CONCLUSIONS: The prevalence of transfusion reactions in children is high, and the intervening factors are: type of blood component, age, patient comorbidity, and multiple transfusions; type of blood component and age are also associated with type of reaction.