A deadly capillary leak attack. Clarkson's disease: a narrative review.

A previously 42-year-old healthy man was brought in by an ambulance to the emergency department with symptoms of a distributive shock. He experienced a rapid decline in his clinical state that evolved into a cardiac arrest. Despite all the performed measures and a prolonged resuscitation, the patient died a few hours later without an initial clear diagnosis. Lab results showed an extremely high haemoconcentration leading to further investigations which suggested the possibility of Clarkson's disease, although septic shock as an alternative diagnosis could not be excluded. Nevertheless, because of its presentation, especially emergency and intensive care physicians should be aware of the existence of this condition in the event of an unexplained refractory distributive shock in combination with haemoconcentration and hypoalbuminemia given its possible fatal outcome.

Community-acquired bacterial meningitis in adults: emergency department management protocol.

Acute bacterial meningitis (ABM) is a rare but disabling infectious condition that requires a performant multidisciplinary management approach. Between 70 and 90 adult patients are diagnosed with community-acquired ABM in Belgium annually, and reported case fatality rates range from 17 to 40%. The currently available guidelines provide evidence-based guidance on how to manage this disease. However, these guidelines do not translate the evidence to the daily practice at the emergency department in a Belgian healthcare context. We created a taskforce in University Hospitals Leuven consisting of experts with complementary expertise in managing this disease: neurology, neurosurgery, intensive care medicine, microbiology and infectious diseases. The taskforce agreed upon a flowchart containing seven management steps encompassing all relevant phases in emergency ABM management. In addition to the focus on timely and adequate initiation of antimicrobial treatment, the flowchart and protocol also provide guidance on practical hurdles such as how to assess the safety of performing a lumbar puncture and when to refer patients to the intensive care department. This protocol was implemented in University Hospitals Leuven and fosters inter-disciplinary coordination of ABM care.

Access to expensive cancer drugs in Dutch daily practice: should we be concerned?

BACKGROUND: To investigate whether equal access to bortezomib has been achieved under the Dutch policy regulations that guarantee equal access to expensive inpatient drugs. METHODS: We investigated accessibility to bortezomib treatment at national and regional levels by (i) conducting interviews with stakeholders in the Dutch healthcare system to explore prescription barriers and (ii) tabulating sales data from 2004-2009 and trial participation rates. RESULTS: Interviews revealed awareness of the high treatment costs, although prescription barriers were not encountered. National use of bortezomib increased slowly (treating 2% of patients in 2004 to 17% in 2009), indicating a long adjustment period. Furthermore, use remains below the rate estimated by the professional association of haematologists (27%). Regional differences were found for both daily practice use (e.g. ranging from 13-27% in 2009) and clinical trial participation (e.g. ranging from 1-12% in 2006). CONCLUSION: Our results were somewhat conflicting: interviews did not reveal any prescription barriers, but quantitative methods showed regional differences, signs of underutilisation, and access inequality. Investigating use and accessibility, based on data triangulation, provides valuable feedback which can enhance evidence-based decision making for both physicians and policymakers. This could improve appropriate and efficient use and ensure equal access to expensive drugs.

Review of health-related quality of life data in multiple myeloma patients treated with novel agents.

In multiple myeloma (MM), health-related quality of life (HRQoL) data is becoming increasingly important, owing to improved survival outcomes and the impact of treatment-related toxicity on HRQoL. Researchers are more frequently including HRQoL assessments in clinical trials, but analysis and reporting of this data has not been consistent. A systematic literature review assessed the effect of novel agents (thalidomide, bortezomib and lenalidomide) on HRQoL in MM patients, and evaluated the subsequent reporting of these HRQoL results. A relatively small body of literature addresses HRQoL data in MM patients treated with novel MM therapeutic agents: 9 manuscripts and 15 conference proceedings. The literature demonstrates the complementary value of HRQoL when assessing clinical response, progression, overall survival and toxicity. However, weaknesses and inconsistencies in analysis and presentation of HRQoL data were observed, often complicating interpretation of the impact of treatment on HRQoL in MM. Further evaluation of HRQoL in MM patients treated with novel agents is required in larger cohorts, and ideally in head-to-head comparative studies. Additionally, the development of standardised MM-specific best practice guidelines in HRQoL data collection and analysis is recommended. These would ensure that future data are more useful in guiding predictive models and clinical decisions.

Drug-related admissions due to interaction with an old drug, lithium.

Lithium is one of the oldest psychotropic drugs with a well-known narrow therapeutic range and the drugs that interact with lithium elimination are well established. However, patients are still admitted to the emergency department with lithium toxicity due to often overlooked interactions with concomitant drugs. We report on two patients, admitted to the emergency department, with lithium toxicity. One patient presented with aphasia and ataxia, showing moderate toxicity. The other was referred due to coma, illustrating severe lithium toxicity. In both cases, a non-steroidal anti-inflammatory drug was the underlying cause. We highlight the mechanism of this drug-drug interaction and underline the need for thoughtful use of other medications in patients taking lithium. Special attention has to be paid for the non-steroidal antiinflammatory drugs due to the low threshold of prescribing them for the control of acute pain and its availability as free over-the-counter drugs.

Real-world costs of autologous and allogeneic stem cell transplantations for haematological diseases: a multicentre study.

Haematopoietic stem cell transplantation (SCT) is an expensive lifesaving procedure, which is increasingly performed in patients with haematological diseases. Developments in the protocol for SCT have resulted in cost estimates that require updating. We aimed to calculate actual costs for SCT and to identify major cost drivers by means of a daily practice cost study. We randomly selected 191 patients, treated at three university hospitals, who underwent an autologous (auto) SCT or allogeneic (allo) SCT in 2007, 2008 or 2009. Allo-SCT included sibling donors, matched unrelated donors (MUD) and umbilical cord blood (UCB). Resource use was collected from the hospital registration systems and medical files. The total costs included selection and harvesting of stem cells, transplantation and 1-year follow-up. The average costs per patient were 45,670 € for auto-SCT and 101,919 € for sibling allo-SCT. The costs of transplantations from unrelated donors were much higher: 171,478 € for allo-SCT-MUD and 254,689 € for allo-SCT-UCB. Hospital inpatient days together with laboratory and other activities were the main cost drivers across all types of SCT. Besides, donor search costs were a large cost component in allo-SCT-sib (18 %) and allo-SCT-MUD (12 %). Real-world costs were above routine reimbursement and appropriate financing is necessary to guarantee the continuation of SCT. The costs calculated in this study provide reliable up-to-date input for cost-effectiveness studies and budget revision.

[Experience of family members with the decision concerning artificial nutrition and hydration in people with dementia in nursing homes]. / Ervaringen van familie met de besluitvorming rond kunstmatige toediening van vocht en voedsel (ktvv) bij mensen met dementie in het verpleeghuis.

The purpose of this study is to investigate how relatives of nursing home patients with dementia, for whom the decision whether to start or forgo artificial nutrition and hydration (ANH) was made, assess the decision-making process. Furthermore we evaluated the information given by the nursing home staff to the relatives and the care provided by them to the patient. The study was an observational study based on written questionnaires. Relatives of 99 nursing home patients with dementia filled in a questionnaire at the time of the decision-making and 6 weeks after the decision was made or 6 weeks after the patient had died. Almost all relatives assessed the 4 aspects of the decision-making process (number of consults, content of the consults, carefulness of the decision-making and there own part in the decision-making process) at both times as 'good' or 'satisfactory'. Furthermore it appeared that they judged the carefulness of the decision-making process significantly more often as 'good' when they also assessed the other three aspects as 'good'. Especially their own influence on the decision-making process appeared to play a part in this matter. Information about the possible consequences of starting ANH as well as about the condition of the patient were experienced sufficient by almost all relatives. This was also the case for the care offered to the patient. In general almost all relatives reported satisfaction with the decision and with the carefulness of the decision-making process. This satisfaction did not differ between the moment of the decision and afterwards. For this it did not make any difference whether the patient had or had not died.

Primary leiomyosarcoma of bone: clinicopathologic, immunohistochemical, and molecular biologic aspects.

BACKGROUND: Primary leiomyosarcoma of bone is a very rare malignant tumor with uncertain pathogenicity. METHODS: The authors studied five cases of surgically treated primary leiomyosarcoma of bone. Clinical histories and radiographic findings were recorded. Regular clinical and radiographic controls were obtained postoperatively. In all cases, immunohistochemical studies were used to confirm the diagnosis. Molecular biologic examinations, using the polymerase chain reaction technique with microsatellite DNA markers from regions of tumor-relevant genes, were performed to determine the stability of the genome or to detect some typical genomic changes. RESULTS: The study included three women and two men, with an average age of 42 years. The tumor was located in the pelvis in two patients, in the femur in two patients, and in the proximal tibia in one patient. All tumors were classified as high-grade tumors (four stage IIB, one stage IIA). Radiographically, all tumors appear as purely osteolytic lesions, with a geographic or moth-eaten appearance and without any sclerotic margin. Three patients underwent limb salvage surgery followed by endoprosthetic replacement. The other two patients required amputation. The mean follow-up was 19 months (range, 8-29 months). Three patients died of disease, with a mean postoperative survival period of 18 months (range, 6-27 months). Four patients developed diffuse pulmonary metastases after an average of 10.5 months. One of those patients responded well to chemotherapy. In all cases, immunohistochemistry showed strong reactivity of the tumor cells for (alpha-SMA and vimentin. Molecular biologic investigations revealed a high rate of genomic instabilities in all of the stage IIB tumors. CONCLUSION: Clinical follow-up suggests that primary osseous leiomyosarcoma has an aggressive biologic behavior. The immunohistochemical studies are useful tools and suggest that osseous leiomyosarcoma arise from the vascular smooth muscle cells within the bone. The molecular biologic findings of a high rate of genomic instability confirm the hypothesis that this rare entity is of an aggressive nature.