Connectedness of healthcare professionals involved in the treatment of patients with Parkinson's disease: a social networks study

<p>Abstract</p> <p>Background</p> <p>Patients with chronic illness typically receive ambulatory treatment from multiple health professionals. Connectedness between these professionals may influence their clinical decisions and the coordination of patient care. We aimed to describe and analyze connectedness in a regional network of health professionals involved in ambulatory treatment of patients with Parkinson's disease (PD).</p> <p>Methods</p> <p>Observational study with 104 health professionals who had joined a newly established network (ParkinsonNet) were asked to complete a pre-structured form to report on their professional contacts with others in the network. Using social networks methods, network measures were calculated for the total network and for the networks of individual health professionals. We planned to test differences between subgroups of health professionals regarding 12 network measures, using a random permutation method.</p> <p>Results</p> <p>Ninety-six health professionals (92%) provided data on 101 professionals. The reciprocity of reported connections was 0.42 in the network of professional contacts. Measures characterizing the individual networks showed a wide variation; <it>e.g</it>., density varied between 0 and 100% (mean value 28.4%). Health professionals with ≥10 PD patients had higher values on 7 out of 12 network measures compare to those with < 10 PD patients (size, number of connections, two step reach, indegree centrality, outdegree centrality, inreach centrality, betweenness centrality). Primary care professionals had lower values on 11 out of 12 network measures (all but reach efficiency) compared to professionals who were affiliated with a hospital.</p> <p>Conclusions</p> <p>Our measure of professional connectedness proved to be feasible in a regional disease-specific network of health professionals. Network measures describing patterns in the professional contacts showed relevant variation across professionals. A higher caseload and an affiliation with a hospital were associated with stronger connectedness with other health professionals.</p

Gcase and limp2 abnormalities in the liver of niemann pick type c mice

Funding Information: This work was supported by the NWO-Building Blocks of Life: GlcCer grant to J.M.F.G.A: BBOL-2007247202. Publisher Copyright: © 2021 by the authors. Licensee MDPI, Basel, Switzerland.The lysosomal storage disease Niemann–Pick type C (NPC) is caused by impaired cholesterol efflux from lysosomes, which is accompanied by secondary lysosomal accumulation of sph-ingomyelin and glucosylceramide (GlcCer). Similar to Gaucher disease (GD), patients deficient in glucocerebrosidase (GCase) degrading GlcCer, NPC patients show an elevated glucosylsphingosine and glucosylated cholesterol. In livers of mice lacking the lysosomal cholesterol efflux transporter NPC1, we investigated the expression of established biomarkers of lipid-laden macrophages of GD patients, their GCase status, and content on the cytosol facing glucosylceramidase GBA2 and lysoso-mal integral membrane protein type B (LIMP2), a transporter of newly formed GCase to lysosomes. Livers of 80-week-old Npc1−/− mice showed a partially reduced GCase protein and enzymatic activity. In contrast, GBA2 levels tended to be reciprocally increased with the GCase deficiency. In Npc1−/− liver, increased expression of lysosomal enzymes (cathepsin D, acid ceramidase) was observed as well as increased markers of lipid-stressed macrophages (GPNMB and galectin-3). Im-munohistochemistry showed that the latter markers are expressed by lipid laden Kupffer cells. Earlier reported increase of LIMP2 in Npc1−/− liver was confirmed. Unexpectedly, immunohistochemistry showed that LIMP2 is particularly overexpressed in the hepatocytes of the Npc1−/− liver. LIMP2 in these hepatocytes seems not to only localize to (endo)lysosomes. The recent recognition that LIMP2 harbors a cholesterol channel prompts the speculation that LIMP2 in Npc1−/− hepatocytes might mediate export of cholesterol into the bile and thus protects the hepatocytes.publishersversionpublishe

Personal health communities: A phenomenological study of a new health-care concept

Context: Fragmentation of care, complexity of diseases and the need to involve patients actively in their individual health care led to the development of the personal health community (PHC). In a PHC, patients can -regardless of the nature of their condition- invite all professionals that are involved in their health care process. Once gathered, the patient and health care team can exchange information about the patient's health and communicate through several functionalities, in a secured environment. Objectives: Exploring the use, first experiences and potential consequences of using PHCs in health care. Design: Qualitative phenomenological study. Participants: Eighteen respondents, consisting of women experiencing infertility (n = 5), persons with Parkinson's disease (n = 6), a gynaecologist, a fertility doctor, a fertility nurse, three Parkinson's specialist nurses and a neurologist. Results: First experiences with PHCs showed that patients use their PHC differently, dependending on their condition and people involved. Various (potential) advantages for future health care were mentioned relating to both organizational aspects of care (e.g. continuity of care) and the human side of care (e.g. personal care). Patient involvement in care was facilitated. Disadvantages were the amount of work that it took and technological issues. Conclusions: Using PHCs leads to promising improvements in both the organization of care and care experience, according to the participants in this study. They indicate that patients with different diseases and in different circumstances can benefit from these improvements. The PHC seem to be an online tool that can be applied in a personalized way. When (technically) well facilitated, it could stimulate active involvement of patients in their own health and health care. It warrants further research to study its effect on concrete health outcomes

Using a smartphone-based self-management platform to support medication adherence and clinical consultation in Parkinson's disease.

The progressive nature of Parkinson's disease, its complex treatment regimens and the high rates of comorbid conditions make self-management and treatment adherence a challenge. Clinicians have limited face-to-face consultation time with Parkinson's disease patients, making it difficult to comprehensively address non-adherence. Here we share the results from a multi-centre (seven centres) randomised controlled trial conducted in England and Scotland to assess the impact of using a smartphone-based Parkinson's tracker app to promote patient self-management, enhance treatment adherence and quality of clinical consultation. Eligible Parkinson's disease patients were randomised using a 1:1 ratio according to a computer-generated random sequence, stratified by centre and using blocks of variable size, to intervention Parkinson's Tracker App or control (Treatment as Usual). Primary outcome was the self-reported score of adherence to treatment (Morisky medication adherence scale -8) at 16 weeks. Secondary outcomes were Quality of Life (Parkinson's disease questionnaire -39), quality of consultation for Parkinson's disease patients (Patient-centred questionnaire for Parkinson's disease), impact on non-motor symptoms (Non-motor symptoms questionnaire), depression and anxiety (Hospital anxiety and depression scale) and beliefs about medication (Beliefs about Medication Questionnaire) at 16 weeks. Primary and secondary endpoints were analysed using a generalised linear model with treatment as the fixed effect and baseline measurement as the covariate. 158 patients completed the study (Parkinson's tracker app = 68 and TAU = 90). At 16 weeks Parkinson's tracker app significantly improved adherence, compared to treatment as usual (mean difference: 0.39, 95%CI 0.04-0.74; p = 0.0304) with no confounding effects of gender, number of comorbidities and age. Among secondary outcomes, Parkinson's tracker app significantly improved patients' perception of quality of consultation (0.15, 95% CI 0.03 to 0.27; p = 0.0110). The change in non-motor symptoms was -0.82 (95% CI -1.75 to 0.10; p = 0.0822). 72% of participants in the Parkinson's tracker app group continued to use and engage with the application throughout the 16-week trial period. The Parkinson's tracker app can be an effective and novel way of enhancing self-reported medication adherence and quality of clinical consultation by supporting self-management in Parkinson's disease in patients owning smartphones. Further work is recommended to determine whether the benefits of the intervention are maintained beyond the 16 week study period

Correction to: A nonsynonymous mutation in PLCG2 reduces the risk of Alzheimer's disease, dementia with Lewy bodies and frontotemporal dementia, and increases the likelihood of longevity.

The IPDGC (The International Parkinson Disease Genomics Consortium) and EADB (Alzheimer Disease European DNA biobank) are listed correctly as an author to the article, however, they were incorrectly listed more than once

Correction to: A nonsynonymous mutation in PLCG2 reduces the risk of Alzheimer’s disease, dementia with Lewy bodies and frontotemporal dementia, and increases the likelihood of longevity

The IPDGC (The International Parkinson Disease Genomics Consortium) and EADB (Alzheimer Disease European DNA biobank) are listed correctly as an author to the article, however, they were incorrectly listed more than once

Correction to: A nonsynonymous mutation in PLCG2 reduces the risk of Alzheimer’s disease, dementia with Lewy bodies and frontotemporal dementia, and increases the likelihood of longevity

The IPDGC (The International Parkinson Disease Genomics Consortium) and EADB (Alzheimer Disease European DNA biobank) are listed correctly as an author to the article, however, they were incorrectly listed more than once

Patient-centeredness in PD care: Development and validation of a patient experience questionnaire

AbstractIntroductionPatient-centeredness is increasingly recognized as a crucial element of quality of care. A suitable instrument to assess the level of patient-centeredness for Parkinson's disease (PD) care is lacking. Here we describe the development and validation of the Patient-Centered Questionnaire for PD (PCQ-PD), and its initial application in a large patient sample.MethodsBased on the outcomes of eight focus groups we composed a questionnaire that measures patient-centeredness by assessing patients' care experiences. The questionnaire was sent to 1112 Dutch PD patients, and face-, content- and construct-validity and reliability were assessed. The level of patient-centeredness was determined by calculating scores for overall patient-centeredness [0–3], subscale experiences [0–3], item experience, item priority and quality improvement.Results895 PD patients (net response 82.0%) completed the questionnaire. After the validation procedure, the PCQ-PD addressed 46 care aspects in six different subscales of patient-centeredness. The internal consistency of the instrument, expressed in Cronbach's α per subscale, ranged from 0.62 to 0.84. The overall patient-centeredness score was 1.69 (SD 0.45). ‘Emotional support’ (1.05, SD 0.90) and ‘provision of tailored information’ (1.18, SD 0.57) subscales received the lowest experience ratings. ‘Access to medical records’ obtained the highest item quality improvement score (5.44).ConclusionsThis study produced a valid instrument to measure patient-centeredness in PD care. Psychometric properties of the instrument were good. Application of the PCQ-PD revealed the level of patient-centeredness in the care for PD patients in The Netherlands. The main outcome was a compelling call for the provision of tailored information and emotional support

HEPES-buffering of bicarbonate-containing culture medium perturbs lysosomal glucocerebrosidase activity

Glucocerebrosidase (GCase), encoded by the GBA gene, degrades the ubiquitous glycosphingolipid glucosylceramide. Inherited GCase deficiency causes Gaucher disease (GD). In addition, carriers of an abnormal GBA allele are at increased risk for Parkinson's disease. GCase undergoes extensive modification of its four N-glycans en route to and inside the lysosome that is reflected in changes in molecular weight as detected with sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Fluorescent activity-based probes (ABPs) that covalently label GCase in reaction-based manner in vivo and in vitro allow sensitive visualization of GCase molecules. Using these ABPs, we studied the life cycle of GCase in cultured fibroblasts and macrophage-like RAW264.7 cells. Specific attention was paid to the impact of 4-(2-hydroxyethyl)-1-piperazineethanesulfonic acid (HEPES) supplementation to bicarbonate-buffered medium. Here, we report how HEPES-buffered medium markedly influences processing of GCase, its lysosomal degradation, and the total cellular enzyme level. HEPES-containing medium was also found to reduce maturation of other lysosomal enzymes (α-glucosidase and β-glucuronidase) in cells. The presence of HEPES in bicarbonate containing medium increases GCase activity in GD-patient derived fibroblasts, illustrating how the supplementation of HEPES complicates the use of cultured cells for diagnosing GD