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Wetensch. publicatieFaculteit der Wijsbegeert

Case report. Perioperatieve anticoagulantia voor de behandeling van een vena cava inferior tumor trombus bij patiënten met niercelcarcinoom

A 71-year-old patient was diagnosed with a renal cell carcinoma with extension in the IVC. Patient was admitted to the intensive care one day before his planned surgery because of massive pulmonary embolism. Trombolysis was eventually effective and later a radical nephrectomy was successfully performed. Inferior vena caval (IVC) tumor thrombus occurs in 4–10% of patients diagnosed with renal cell carcinoma. These patients have an increased risk of venous thromboembolism. There is no randomized trial investigating the role of anticoagulants in this patient group. Based on expert opinion it is advised to start Low Molecular Weight Heparins (LMWH), in a therapeutic dose, at time of diagnosis up to three to six months following surgery. Based on a literature search on this subject immediate treatment with therapeutic LMWH is recommended in patients with a renal cell carcinoma extending in the IVC

Present readiness of, and white spots in the Dutch national system for greenhouse gas reporting of the land use, land-use change and forestry sector (LULUCF)

Current LULUCF greenhouse gas reporting by the Netherlands is incomplete. The few sections that are included in the current reporting are done at lower Tiers. Thirty nine existing monitoring systems or databases were identified as possibly relevant for setting up a national system. The systems are grouped as follows : 1: systems for land use and area (mapping), including soil maps (13); 2: data on agricultural and forestry practices that may serve to derive emission factors (16) 3: data on emission factors including weather and groundwater data (8) 4: modelling systems (2). Based on the identified exisiting systems, the report concludes on the main discrepancies between the ongoing reporting/current monitoring systems and the requirements as set out by the Marrakesh Accords and the 1st draft of the Good Practice Guidance

Klimaatverandering en de functies van het landelijk gebied : resultaten van vier jaar klimaatonderzoek

Vier thema's komen in deze brochure aan bod: koolstofvastlegging in bos en landbouw; emissies van lachgas en methaan uit de landbouw; landgebruik als actor en reactor in het klimaatsysteem; klimaatverandering en het landelijk gebied: een integrale kijk. Uitleg over een actueel onderwerp, aan de hand van 31 vrage

Abnormal aortic wall properties in women with Turner syndrome

Background Turner syndrome (TS) is associated with aortic dilatation and dissection, but the underlying process is unclear. The aim of this study was to investigate the elastic properties and composition of the aortic wall in women with TS. Methods In this cross-sectional study, 52 women with TS aged 35 ± 13 years (50% monosomy, 12 with bicuspid aortic valve [BAV] and 4 with coarctation) were investigated using carotid-femoral pulse wave velocity (CF-PWV) by echocardiography and ascending aortic distensibility (AAD) and aortic arch pulse wave velocity (AA-PWV) by magnetic resonance imaging (MRI). As control group, 13 women with BAV without TS and 48 healthy patients were included. Results Women with TS showed a higher AA-PWV (β = 1.08, confidence interval [CI]: 0.54–1.62) after correcting for age and comorbidities compared with controls. We found no significant difference in AAD and CF-PWV. In women with TS, the presence of BAV, coarctation of the aorta, or monosomy (45, X) was not associated with aortic stiffness. In addition, aortic tissue samples were investigated with routine and immunohistochemical stains in five additional women with TS who were operated. The tissue showed more compact smooth muscle cell layers with abnormal deposition and structure of elastin and diminished or absent expression of contractile proteins desmin, actin, and caldesmon, as well as the progesterone receptor. Conclusion Both aortic arch stiffness measurements on MRI and histomorphological changes point toward an inherent abnormal thoracic aortic wall in women with TS

Inter-method reliability of the modified Rankin Scale in patients with subarachnoid hemorrhage

Background and objectives The modified Rankin Scale (mRS) is one of the most frequently used outcome measures in trials in patients with an aneurysmal subarachnoid hemorrhage (aSAH). The assessment method of the mRS is often not clearly described in trials, while the method used might influence the mRS score. The aim of this study is to evaluate the inter-method reliability of different assessment methods of the mRS.Methods This is a prospective, randomized, multicenter study with follow-up at 6 weeks and 6 months. Patients aged >= 18 years with aSAH were randomized to either a structured interview or a self-assessment of the mRS. Patients were seen by a physician who assigned an mRS score, followed by either the structured interview or the self-assessment. Inter-method reliability was assessed with the quadratic weighted kappa score and percentage of agreement. Assessment of feasibility of the self-assessment was done by a feasibility questionnaire.Results The quadratic weighted kappa was 0.60 between the assessment of the physician and structured interview and 0.56 between assessment of the physician and self-assessment. Percentage agreement was, respectively, 50.8 and 19.6%. The assessment of the mRS through a structured interview and by self-assessment resulted in systematically higher mRS scores than the mRS scored by the physician. Self-assessment of the mRS was proven feasible.Discussion The mRS scores obtained with different assessment methods differ significantly. The agreement between the scores is low, although the reliability between the assessment methods is good. This should be considered when using the mRS in clinical trials.Scientific Assessment and Innovation in Neurosurgical Treatment Strategie

Self-reported work productivity in people with multiple sclerosis and its association with mental and physical health

Purpose This study aimed to identify mental health, physical health, demographic and disease characteristics relating to work productivity in people with multiple sclerosis (MS). Methods In this cross-sectional study, 236 employed people with MS (median age = 42 years, 78.8% female) underwent neurological and neuropsychological assessments. Additionally, they completed questionnaires inquiring about work productivity (presenteeism: reduced productivity while working, and absenteeism: loss of productivity due to absence from work), mental and physical health, demographic and disease characteristics. Multiple linear and logistic regression analyses were performed with presenteeism and absenteeism as dependent variables, respectively. Results A model with mental and physical health factors significantly predicted presenteeism F(11,202) = 11.33, p < 0.001, R-2 = 0.38; a higher cognitive (p < 0.001) and physical impact (p = 0.042) of fatigue were associated with more presenteeism. A model with only mental health factors significantly predicted absenteeism; chi(2)(11)=37.72, p < 0.001, with R-2 = 0.27 (Nagelkerke) and R-2 = 0.16 (Cox and Snell). Specifically, we observed that more symptoms of depression (p = 0.041) and a higher cognitive impact of fatigue (p = 0.011) were significantly associated with more absenteeism. Conclusions In people with MS, both cognitive and physical impact of fatigue are positively related to presenteeism, while symptoms of depression and cognitive impact of fatigue are positively related to absenteeism.Neurological Motor Disorder

Expanding the clinical and genetic spectrum of ALPK3 variants: Phenotypes identified in pediatric cardiomyopathy patients and adults with heterozygous variants

Introduction: Biallelic damaging variants in ALPK3, encoding alpha-protein kinase 3, cause pediatric-onset cardiomyopathy with manifestations that are incompletely defined. Methods and Results: We analyzed clinical manifestations of damaging biallelic ALPK3 variants in 19 pediatric patients, including nine previously published cases. Among these, 11 loss-of-function (LoF) variants, seven compound LoF and deleterious missense variants, and one homozygous deleterious missense variant were identified. Among 18 live-born patients, 8 exhibited neonatal dilated cardiomyopathy (44.4%; 95% CI: 21.5%-69.2%) that subsequently transitioned into ventricular hypertrophy. The majority of patients had extracardiac phenotypes, including contractures, scoliosis, cleft palate, and facial dysmorphisms. We observed no association between variant type or location, disease severity, and/or extracardiac manifestations. Myocardial histopathology showed focal cardiomyocyte hypertrophy, subendocardial fibroelastosis in patients under 4 years of age, and myofibrillar disarray in adults. Rare heterozygous ALPK3 variants were also assessed in adult-onset cardiomyopathy patients. Among 1548 Dutch patients referred for initial genetic analyses, we identified 39 individuals with rare heterozygous ALPK3 variants (2.5%; 95% CI: 1.8%-3.4%), including 26 missense and 10 LoF variants. Among 149 U.S. patients without pathogenic variants in 83 cardiomyopathy-related genes, we identified six missense and nine LoF ALPK3 variants (10.1%; 95% CI: 5.7%-16.1%). LoF ALPK3 variants were increased in comparison to matched controls (Dutch cohort, P = 1.6×10−5; U.S. cohort, P = 2.2×10−13). Conclusion: Biallelic damaging ALPK3 variants cause pediatric cardiomyopathy manifested by DCM transitioning to hypertrophy, often with poor contractile function. Additional extracardiac features occur in most patients, including musculoskeletal abnormalities and cleft palate. Heterozygous LoF ALPK3 variants are enriched in adults with cardiomyopathy and may contribute to their cardiomyopathy. Adults with ALPK3 LoF variants therefore warrant evaluations for cardiomyopathy