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When clozapine appears at a dance event…. 当氯氮平出现在舞会上....
IF 1.6 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2020-12-01 Epub Date: 2019-06-14 DOI: 10.1080/17843286.2019.1630068
Lisbeth Patteet, Kristof Maudens, Sarah Wille, Peter Blanckaert, Hugo Neels, Paul Calle

Objectives: The content of substances sold and consumed as party drugs is often unknown. They may contain inactive, contaminated or unexpected ingredients, and the dosage of the active components may vary considerably. Obviously, these phenomena increase the chances of a wrong or delayed therapy. To illustrate this point, we report 3 cases of clozapine intoxication at a dance event where most likely clozapine tablets were sold as party drugs.Methods: The three cases were part of a prospective toxicology study at a nocturnal indoor dance event.Results: One patient had to be intubated after obstructive breathing with desaturation and bradycardia, while the 2 other patients presented with syncope and altered mental status. All patients recovered after 20 minutes to 8 hours. Systematic toxicological analysis of the blood samples revealed the presence of clozapine (73-244 ng/ml) and its metabolite norclozapine (9-59 ng/ml). A pill, found in a pocket of one patient, was identified as Leponex® 100 mg (clozapine). This neuroleptic drug is mainly prescribed for treatment-resistant schizophrenia. In clozapine-naive subjects, orthostatic hypotension, bradycardia and syncope have been reported with a single 25 mg oral dose. Serum clozapine concentrations of the 3 cases were below the defined therapeutic range (350-600ng/ml) and the clozapine:norclozapine ratios were suggestive for recent drug intake.Conclusion: Routine drug screening may be unable to detect the toxic agent(s) involved. Whenever unusual symptoms are observed in an intoxicated patient, blood and urine samples should be sent to a reference toxicology laboratory.

目的:作为聚会毒品出售和消费的物质的含量通常是未知的。它们可能含有非活性的、受污染的或意想不到的成分,活性成分的剂量可能相差很大。显然,这些现象增加了错误或延迟治疗的机会。为了说明这一点,我们报告了3例氯氮平中毒的舞蹈活动,其中最有可能的氯氮平片作为派对药物出售。方法:这三个病例是夜间室内舞蹈活动前瞻性毒理学研究的一部分。结果:1例患者因呼吸梗阻性失血、心动过缓需插管,2例患者出现晕厥、精神状态改变。所有患者均在20分钟至8小时后恢复。血液样本系统毒理学分析显示存在氯氮平(73 ~ 244ng /ml)及其代谢物去氯氮平(9 ~ 59ng /ml)。在一名患者的口袋中发现一颗药片,经鉴定为Leponex®100 mg(氯氮平)。这种抗精神病药物主要用于治疗难治性精神分裂症。在氯氮平初始受试者中,单次口服25mg剂量有直立性低血压、心动过缓和晕厥的报道。3例患者血清氯氮平浓度均低于规定的治疗范围(350 ~ 600ng/ml),氯氮平与去氯氮平比值提示近期服药。结论:常规药物筛选可能无法检测到所涉及的毒性物质。每当在中毒患者身上观察到异常症状时,应将血液和尿液样本送到参考毒理学实验室。
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引用次数: 5
A case report of successful eradication of new isolates of Burkholderia cenocepacia in a child with cystic fibrosis. 成功根除新分离的伯克霍尔德菌囊性纤维化儿童的病例报告。
IF 1.6 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2020-12-01 Epub Date: 2019-06-15 DOI: 10.1080/17843286.2019.1630585
V Sputael, G Van Schandevyl, L Hanssens

Chronic respiratory infection with Burkholderia cenocepacia (Bc) in patients with cystic fibrosis (CF) is associated with accelerated decline in lung function and increased mortality. It is therefore important to attempt to eradicate new isolates, especially in children. However, there are no standardized guidelines to eradicate Bc. We report a case of successful eradication of new isolates of Bc in a 2-year-old child with CF using a combination of IV, nebulized antibiotics and sinus surgery.

囊性纤维化(CF)患者的慢性呼吸道感染伴随结核杆菌(Bc)与肺功能加速下降和死亡率增加相关。因此,重要的是努力根除新的分离株,特别是在儿童中。然而,没有标准化的指南来根除Bc。我们报告一例成功根除新分离Bc在一个2岁的CF儿童使用静脉注射,雾化抗生素和鼻窦手术的组合。
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引用次数: 3
Application of the GheOP3S-tool in nursing home residents: acceptance and implementation of pharmacist recommendations. gheop3s工具在养老院居民中的应用:药师建议的接受与实施。
IF 1.6 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2020-12-01 Epub Date: 2019-06-26 DOI: 10.1080/17843286.2019.1634323
Katrien Foubert, Peter Muylaert, Els Mehuys, Annemie Somers, Mirko Petrovic, Koen Boussery
ABSTRACT Background and objective: The prevalence of potentially inappropriate prescribing (PIP) among nursing home (NH) residents is high. This study aimed to investigate the acceptance and implementation of pharmacist recommendations based on a screening tool for PIP, the Ghent Older People’s Prescriptions community Pharmacy Screening (GheOP3S)-tool. Setting and method: Prospective observational study in NH residents (≥ 70 years, using ≥ 5 medications) with a 3-month follow-up period. A pharmacist screened the medication lists using the GheOP3S-tool and formulated recommendations to reduce PIP. The acceptance of recommendations discussed during face-to-face pharmacist-general practitioner (GP) meetings was recorded. Implementation was examined by comparing baseline and follow-up medication lists. A pre-post comparison of the number of chronic medications and GheOP3S-criteria; the anticholinergic and sedative burden quantified by the Drug Burden Index (DBI); and medication costs was performed. Results: Screening with the GheOP3S-tool resulted in 168 pharmacist recommendations for 50 NH residents, mainly to stop (78.0%) and to substitute (14.3%) medications. Ninety-three % (156/168) of recommendations were considered relevant. GPs acceptance rate was 44.9%. Fifty-four % of all accepted recommendations were implemented. At follow-up, the number of chronic medications (p = 0.007), and DBI scores (p = 0.004) significantly differed from baseline. There was no significant decrease in the number of GheOP3S-criteria (p = 0.075) and medication costs (p > 0.05). Conclusion: The acceptance and implementation of pharmacist recommendations were relatively low. Future studies should increase the involvement of patients and all health-care providers. Interdisciplinary collaboration with sufficient education for all disciplines and patients is essential.
背景与目的:潜在不当处方(PIP)在养老院(NH)居民中的患病率很高。本研究旨在调查基于PIP筛选工具,根特老年人处方社区药房筛选(GheOP3S)工具的药剂师建议的接受和实施情况。背景和方法:前瞻性观察研究在NH居民(≥70岁,使用≥5种药物)中进行,随访3个月。药剂师使用gheop3s工具筛选药物清单,并制定减少PIP的建议。在面对面的药剂师-全科医生(GP)会议上讨论的建议的接受情况被记录下来。通过比较基线和随访药物清单来检查实施情况。慢性药物数量与gheop3s标准的前后比较用药物负担指数(DBI)量化抗胆碱能和镇静负担;并计算了药物费用。结果:使用gheop3s工具筛选50名NH居民,得到168名药剂师建议,主要是停药(78.0%)和替代药物(14.3%)。93%(156/168)的建议被认为是相关的。全科医生接受率为44.9%。所有接受的建议中有54%得到了实施。随访时,慢性药物使用次数(p = 0.007)和DBI评分(p = 0.004)与基线有显著差异。两组间gheop3s标准个数(p = 0.075)和用药费用(p > 0.05)均无显著差异。结论:药师推荐的接受度和执行度较低。未来的研究应增加患者和所有卫生保健提供者的参与。跨学科合作以及对所有学科和患者的充分教育是必不可少的。
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引用次数: 10
Necrotizing myositis case report and brief literature study. 坏死性肌炎1例报告及简要文献研究。
IF 1.6 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2020-12-01 Epub Date: 2019-07-03 DOI: 10.1080/17843286.2019.1637388
Arthur Basso, Filip Moerman, Christophe Ronsmans, Martine Demarche

Necrotizing myositis is an extremely rare soft tissue infection, mainly caused by Group A Streptococci. Although its presentation is nonspecific and seems harmless, it quickly leads to death in almost all cases. Therefore, diagnosis and treatment of necrotizing myositis are considered as medical emergencies. The 27 years old patient we report benefited from early diagnosis and care. Necrotic tissues were surgically removed 24 hours after the appearance of the first clinical signs. Intravenous antibiotherapy as well as immunoglobulin therapy were also given on the first day. Starting from this clinical case, we present a brief explanation of the pathogenesis, the key clinical features and appropriate tools for diagnosis. Then, adequate antibiotherapy, role of immunoglobulin therapy and interest of hyperbaric oxygenotherapy will be discussed.

坏死性肌炎是一种极为罕见的软组织感染,主要由A群链球菌引起。虽然它的表现是非特异性的,似乎是无害的,但它几乎在所有病例中都会迅速导致死亡。因此,坏死性肌炎的诊断和治疗被视为医疗急救。我们报告的这名27岁患者受益于早期诊断和护理。坏死组织在出现第一个临床症状24小时后手术切除。第一天给予静脉抗生素治疗和免疫球蛋白治疗。从这个临床病例出发,我们对其发病机制、主要临床特征和适当的诊断工具进行了简要的解释。然后,适当的抗生素治疗,免疫球蛋白治疗的作用和高压氧治疗的兴趣将讨论。
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引用次数: 1
Survival prediction in patients with cutaneous melanoma by tumour lymphangiogenesis. 通过肿瘤淋巴管生成预测皮肤黑色素瘤患者的生存。
IF 1.6 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2020-12-01 Epub Date: 2019-06-18 DOI: 10.1080/17843286.2019.1629076
Zorica Špirić, Milka Vještica, Mirela Erić

Objectives: Melanoma induces lymphangiogenesis by secreting lymphangiogenic growth factors. The aim of this study was to examine the role of tumour lymphangiogenesis in survival of patients with cutaneous melanoma. Methods: Immunostaining of one hundred melanoma specimens was done with lymphatic-specific antibody D2-40. The quantification of tumour lymphangiogenesis - lymphatic vessel density (LVD) and lymphatic vessel area (LVA) - was calculated by computer-assisted morphometric analysis. Results: High intratumoural LVD, high peritumoural LVD, male gender, greater tumour thickness and Clark level IV/V were significantly associated with shorter disease-free survival (p= 0.001, p= 0.004, p= 0.004, p= 0.000 and p= 0.008, respectively) and melanoma-specific survival (p= 0.002, p= 0.002, p= 0.001, p= 0.000 and p= 0.017, respectively), while the trunk melanoma site was significantly associated only with shorter disease-free survival (p= 0.033). No significant association of LVA with survival was found. At multivariate analysis, peritumoural LVD [hazard ratio (HR) = 2.143, 95% confidence interval (CI) 1.097-4.189, p= 0.026)] and melanoma thickness (HR = 1.276, 95%CI 1.106-1.473, p= 0.001) were independent predictors of disease-free survival, while intratumoural LVD (HR = 3.446, 95%CI 1.465-8.109, p= 0.005), peritumoural LVD (HR = 2.742, 95%CI 1.313-5.725, p= 0.007) and gender (HR = 2.880, 95%CI 1.304-6.362, p= 0.009) were independent predictors of melanoma-specific survival. Conclusion: Тhis study shows that LVD enables better prediction of survival than melanoma thickness and other clinical-pathological parameters. Intratumoural LVD is the most significant predictor of melanoma-specific survival, while only peritumoural LVD has a significant impact on both, a disease-free survival and a melanoma-specific survival.

目的:黑色素瘤通过分泌淋巴管生成生长因子诱导淋巴管生成。本研究的目的是研究肿瘤淋巴管生成在皮肤黑色素瘤患者生存中的作用。方法:采用淋巴特异性抗体D2-40对100例黑色素瘤标本进行免疫染色。肿瘤淋巴管生成的量化-淋巴管密度(LVD)和淋巴管面积(LVA) -通过计算机辅助形态计量学分析计算。结果:高瘤内LVD、高瘤周LVD、男性、较大肿瘤厚度和Clark IV/V水平与较短的无病生存期(p= 0.001、p= 0.004、p= 0.004、p= 0.000和p= 0.008)和黑色素瘤特异性生存期(p= 0.002、p= 0.002、p= 0.001、p= 0.000和p= 0.017)显著相关,而主干黑色素瘤部位仅与较短的无病生存期显著相关(p= 0.033)。LVA与生存率无显著相关性。在多因素分析中,瘤周LVD[危险比(HR) = 2.143, 95%可信区间(CI) 1.097 ~ 4.189, p= 0.026)]和黑色素瘤厚度(HR = 1.276, 95%CI 1.106 ~ 1.473, p= 0.001)是无病生存的独立预测因子,而瘤内LVD (HR = 3.446, 95%CI 1.465 ~ 8.109, p= 0.005)、瘤周LVD (HR = 2.742, 95%CI 1.313 ~ 5.725, p= 0.007)和性别(HR = 2.880, 95%CI 1.304 ~ 6.362, p= 0.009)是黑色素瘤特异性生存的独立预测因子。结论:Тhis研究表明,LVD比黑色素瘤厚度和其他临床病理参数更能预测患者的生存。瘤内LVD是黑色素瘤特异性生存的最重要预测因子,而只有瘤周LVD对无病生存和黑色素瘤特异性生存都有显著影响。
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引用次数: 4
Dyspnea in homosexual male patients: throwback to an occasionally forgotten but severe clinical presentation of HIV/AIDS. 男同性恋患者的呼吸困难:倒退到偶尔被遗忘但严重的HIV/AIDS临床表现。
IF 1.6 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2020-12-01 Epub Date: 2019-05-25 DOI: 10.1080/17843286.2019.1622880
Koen Verbeke, Roel Verbuyst, Christiane Brands, Hans Slabbynck

Pneumocystis jirovecii pneumonia (PJP) can be a severe indicator disease of acquired immunodeficiency syndrome (AIDS). We present two cases of homosexual male patients who came to the emergency unit of a Belgian hospital because of shortness of breath. Both men had been sent back home, initially diagnosed with a benign viral infection. Because of worsening symptoms and gradually evolving hypoxemia, both patients came back and were admitted to the hospital with a diagnosis of (microbiology proven) Pneumocystis jirovecii pneumonia. HIV serology in both men was tested and was clearly positive, indicating a new diagnosis of HIV infection. In this article, we provide an overview of this possibly severe AIDS defining condition. First, we give an introduction of the history of HIV/AIDS and its occurrence in homosexual males in Europe. Secondly, we provide an overview of the diagnosis and treatment of Pneumocystis jirovecii pneumonia. Finally, since the first case reports of Pneumocystis jirovecii pneumonia at the beginning of the AIDS epidemic also included homosexual men, we emphasize the potential importance of a sexual anamnesis in young male patients with an initial complaint of dyspnea.

乙基肺囊虫肺炎(PJP)是获得性免疫缺陷综合征(艾滋病)的一种严重指示性疾病。我们提出了两例同性恋男性患者谁来到比利时医院急诊室,因为呼吸短促。两人都被送回家,最初被诊断为良性病毒感染。由于症状恶化和逐渐发展的低氧血症,两名患者返回并被诊断为(经微生物学证实的)乙氏肺囊虫肺炎入院。对这两名男子进行了艾滋病毒血清学检测,结果明显呈阳性,表明艾滋病病毒感染的新诊断。在这篇文章中,我们概述了这种可能严重的艾滋病定义条件。首先,我们介绍了艾滋病毒/艾滋病的历史及其在欧洲同性恋男性中的发生情况。其次,我们提供的诊断和治疗的乙氏肺囊虫肺炎概述。最后,由于艾滋病流行初期的第一例乙氏肺囊虫肺炎也包括男同性恋者,我们强调在以呼吸困难为首发主诊的年轻男性患者中,性记忆缺失的潜在重要性。
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引用次数: 0
Efficacy and toxicity of sorafenib in patients with adenoid cystic carcinoma of the head and neck: a case series of five patients. 索拉非尼治疗头颈部腺样囊性癌的疗效和毒性:5例患者的病例系列。
IF 1.6 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2020-10-01 Epub Date: 2019-06-23 DOI: 10.1080/17843286.2019.1633490
Karen Couvreur, Jacobs Celine, Bock Marlies, D'Hondt Randal, Deron Philippe, Duprez Frederic, Rottey Sylvie

Introduction: Adenoid cystic carcinoma (ACC) of the head and neck is a rare malignancy of the salivary glands that accounts for approximately 10% of salivary gland carcinoma. Despite aggressive local therapy, local recurrence and distant metastases occur frequently. Response rates (RR) to potential curative and palliative chemotherapy are limited, so new strategies are needed.

Case reports: We describe five case reports of patients with unresectable locally advanced or metastatic ACC of the head and neck who have been treated with sorafenib, a multi-tyrosine kinase inhibitor (mTKI).

Results: In this case series, we found that three out of five patients treated with sorafenib survived, respectively, 16, 35 and 35 months. Two patients showed a partial response (PR) and one patient had a prolonged stable disease (SD) for almost three years. Grade 3 adverse events (AE) occur under sorafenib so adequate toxicity management is essential. This retrospective case series hints towards the possibility of clinical benefit for treating ACC patients with sorafenib. Efficacy of sorafenib should be studied in a prospective-randomized clinical trial which is a challenging task due to the rarity of the disease.

头颈部腺样囊性癌(ACC)是一种罕见的唾液腺恶性肿瘤,约占唾液腺癌的10%。尽管积极的局部治疗,局部复发和远处转移是经常发生的。对潜在的治愈性和姑息性化疗的反应率(RR)有限,因此需要新的策略。病例报告:我们描述了5例不可切除的局部晚期或转移性头颈部ACC患者,他们接受了索拉非尼(一种多酪氨酸激酶抑制剂(mTKI))的治疗。结果:在这个病例系列中,我们发现5名接受索拉非尼治疗的患者中有3名分别存活了16个月、35个月和35个月。2例患者表现出部分缓解(PR), 1例患者的病情延长稳定(SD)近3年。3级不良事件(AE)发生在索拉非尼,因此充分的毒性管理是必不可少的。本回顾性病例系列提示索拉非尼治疗ACC患者的临床获益可能性。索拉非尼的疗效应该在前瞻性随机临床试验中进行研究,由于这种疾病的罕见性,这是一项具有挑战性的任务。
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引用次数: 3
Orthostatic blood pressure drop and cardiovascular and psychotropic medication dosages in acutely ill geriatric inpatients. 老年急性病住院患者体位血压下降与心血管和精神药物剂量的关系。
IF 1.6 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2020-10-01 Epub Date: 2019-05-29 DOI: 10.1080/17843286.2019.1623516
J Paul, F Vaillant, O Vanden Bossche, T Pepersack, S Henrard, B Boland

Objectives: . To investigate the dose-response relationship between cardiovascular or psychotropic medication dosages and falling orthostatic blood pressure in geriatric inpatients.

Methods: . This cross-sectional study included 100 consecutive geriatric inpatients of a Belgian hospital. The end points were the maximum changes of systolic (sBP) and diastolic (dBP) blood pressure in a standing up position at one or three minutes. The dosages of six classes of vascular and five classes of psychotropic medications were expressed in terms of a proportion of defined daily doses (DDD). Bivariate and multivariate linear regression models were used.

Results: . The 100 geriatric patients (85 ± 5 years, 58 % women) received 7.7 ± 4 medications (mean DDD: vascular = 1.0, psychotropic = 0.74) on the day of an orthostatic test (lying sBP: 136 ± 21; dBP: 72 ± 14 mm Hg). In a standing position, sBP and dBP fell by 12 ± 17 and 11 ± 5 mmHg, respectively. At the individual level, BP change was not correlated with vascular DDD (sBP: p = 0.07, r2 = 0.04; dBP: p = 0.59; r2 = 0.004) nor with psychotropic DDD (sBP: p = 0.14, r2 = 0.02; dBP: p = 0.82; r2 = 0.0). In multivariate analysis, sBP drop was positively associated with age, diabetes, falls history, and number of medications, but not with the DDD of any of the medication classes, while dBP drop was positively associated with age, diabetes, stroke and anaemia, but again with the DDD of any of the medication classes.

Conclusion: . No correlation was found between vascular and psychotropic medication dosages and the orthostatic blood pressure drop expressed as a continuous variable.

目的:。探讨老年住院患者心血管或精神药物剂量与体位血压下降的剂量-反应关系。方法:。本横断面研究包括100名连续在比利时一家医院住院的老年患者。终点是站立位1分钟或3分钟时收缩压(sBP)和舒张压(dBP)的最大变化。六类血管药物和五类精神药物的剂量以限定日剂量(DDD)的比例表示。采用双变量和多变量线性回归模型。结果:。100例老年患者(85±5岁,58%为女性)在直立试验当天接受了7.7±4种药物治疗(平均DDD:血管= 1.0,精神药物= 0.74)(卧位收缩压:136±21;dBP: 72±14mmhg)。站立时收缩压和舒张压分别下降12±17和11±5 mmHg。在个体水平上,血压变化与血管DDD无相关性(sBP: p = 0.07, r2 = 0.04;dBP: p = 0.59;r2 = 0.004)与精神类DDD无关(sBP: p = 0.14, r2 = 0.02;dBP: p = 0.82;r2 = 0.0)。在多变量分析中,收缩压下降与年龄、糖尿病、跌倒史和药物数量呈正相关,但与任何药物类别的DDD无关,而舒张压下降与年龄、糖尿病、中风和贫血呈正相关,但与任何药物类别的DDD呈正相关。结论:。血管和精神药物剂量与体位血压降之间没有相关性,体位血压降表现为连续变量。
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引用次数: 1
Dissatisfaction and Burnout among Flemish emergency physicians: a qualitative study. 佛兰德急诊科医师的不满与倦怠:一项质性研究。
IF 1.6 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2020-10-01 Epub Date: 2019-06-11 DOI: 10.1080/17843286.2019.1629077
Ine Van Bogaert, Catheline Depuydt, Said Hachimi-Idrissi

Objectives: Emergency physicians have shown difficulties to combine their private and professional life. In addition to a very stressful environment, they have to deal with multiple sources of uncertainty. These factors may lead to psychological distress. The aim of this study is to determine the factors leading to dissatisfaction and burnout among Flemish emergency physicians (EPs). Which are the barriers hindering EPs to seek help and what are the potential solutions for this problem? Methods:This is a prospective, explorative qualitative study where seven Flemish EPs were interviewed. These were in-depth semi-structured interviews. The information from the interviews was analyzed using NVivo 12. Results:The common contributors were the working schedule, the increasing number of patients, the mental and physical impact of the job and the relatively low income. The most important barrier to seek help is the existence of an unforgiving medical culture. The solutions provided by the EPs are less patients and a better financing. Conclusion:Dissatisfaction and burnout are common among the EP profession. There is a need for awareness and a change in the unforgiving medical culture to a 'no shame no blame culture'. Improvement of EPs' work schedule and income to the level of other medical specialties are other important needs. Finally, reducing the number of patient contact at the emergency department, by highlighting the difference between medical urgency rather than personal convenience.

目的:急诊医生很难将他们的私人生活和职业生活结合起来。除了压力很大的环境外,他们还必须处理各种不确定因素。这些因素可能导致心理困扰。本研究的目的是确定导致佛兰德急诊医生(EPs)的不满和倦怠的因素。有哪些障碍阻碍易犯案人士寻求帮助?这个问题有什么可能的解决办法?方法:这是一个前瞻性的,探索性质的研究,其中七个佛兰德EPs采访。这些都是深入的半结构化采访。使用NVivo 12对访谈信息进行分析。结果:常见的影响因素为工作时间安排、就诊人数增加、工作对身心的影响以及收入相对较低。寻求帮助的最重要障碍是存在一种无情的医疗文化。EPs提供的解决方案是减少患者和更好的融资。结论:不满意和职业倦怠在EP职业中普遍存在。有必要提高认识,并将无情的医疗文化转变为“不羞耻,不责备”的文化。另外一个重要的需求是,将普通医生的工作时间表和收入提高到其他医学专业的水平。最后,通过强调医疗急迫性而不是个人便利性之间的区别,减少患者在急诊科的接触次数。
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引用次数: 1
Coombs-positive refractory acquired thrombotic thrombocytopenic purpura in a patient with chronic myelomonocytic leukemia successfully treated with rituximab. 康姆斯阳性难治性获得性血栓性血小板减少性紫癜在慢性髓单细胞白血病患者成功治疗利妥昔单抗。
IF 1.6 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2020-10-01 Epub Date: 2019-04-22 DOI: 10.1080/17843286.2019.1608395
Ivan Krecak, Marijana Grgic Medic, Velka Gveric-Krecak, Pavle Roncevic, Sandra Bašić Kinda, Jaksa Babel, Radovan Radonic

Objectives: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare autoimmune disorder characterized by auto-antibodies to Willebrand factor (vWF) cleaving enzyme (ADAMTS13), resulting in unusually large vWF multimers that lead to platelet aggregation, microthrombi formation and microangiopathic hemolytic anemia. Hemolysis in aTTP is mechanical; thus, direct antiglobulin test (Coombs test) is usually negative. Multiple autoimmune conditions and various auto-antibodies have been described in the context of chronic myelomonocytic leukemia (CMML). In this paper, we describe the first case of CMML with auto-antibodies to ADAMTS13, presenting initially as plasmapheresis-refractory Coombs-positive aTTP. Results: Although our patient was not treated for CMML, a complete remission of aTTP was eventually achieved with rituximab. Conclusion; We propose that aTTP should be in the differential diagnosis of CMML patients with thrombocytopenia and anemia (Coombs positive or not) who develop signs of thrombotic microangiopathy. Further studies are much needed to decipher the immune-mediated processes in CMML.

目的:获得性血栓性血小板减少性紫癜(aTTP)是一种罕见的自身免疫性疾病,其特征是对Willebrand因子(vWF)切割酶(ADAMTS13)的自身抗体,导致异常大的vWF多聚体,导致血小板聚集、微血栓形成和微血管病溶血性贫血。aTTP的溶血是机械性的;因此,直接抗球蛋白试验(库姆试验)通常为阴性。在慢性髓细胞白血病(CMML)的背景下,多种自身免疫性疾病和各种自身抗体已被描述。在本文中,我们描述了第一例具有ADAMTS13自身抗体的CMML,最初表现为血浆分离难固性coombs阳性aTTP。结果:虽然我们的患者没有接受CMML治疗,但使用利妥昔单抗最终实现了aTTP的完全缓解。结论;我们建议,aTTP应作为CMML合并血小板减少和贫血(Coombs阳性或阴性)的鉴别诊断,这些患者有血栓性微血管病变的迹象。需要进一步的研究来破译CMML中免疫介导的过程。
{"title":"Coombs-positive refractory acquired thrombotic thrombocytopenic purpura in a patient with chronic myelomonocytic leukemia successfully treated with rituximab.","authors":"Ivan Krecak,&nbsp;Marijana Grgic Medic,&nbsp;Velka Gveric-Krecak,&nbsp;Pavle Roncevic,&nbsp;Sandra Bašić Kinda,&nbsp;Jaksa Babel,&nbsp;Radovan Radonic","doi":"10.1080/17843286.2019.1608395","DOIUrl":"https://doi.org/10.1080/17843286.2019.1608395","url":null,"abstract":"<p><strong>Objectives: </strong>Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare autoimmune disorder characterized by auto-antibodies to Willebrand factor (vWF) cleaving enzyme (ADAMTS13), resulting in unusually large vWF multimers that lead to platelet aggregation, microthrombi formation and microangiopathic hemolytic anemia. Hemolysis in aTTP is mechanical; thus, direct antiglobulin test (Coombs test) is usually negative. Multiple autoimmune conditions and various auto-antibodies have been described in the context of chronic myelomonocytic leukemia (CMML). In this paper, we describe the first case of CMML with auto-antibodies to ADAMTS13, presenting initially as plasmapheresis-refractory Coombs-positive aTTP. <b>Results:</b> Although our patient was not treated for CMML, a complete remission of aTTP was eventually achieved with rituximab. Conclusion; We propose that aTTP should be in the differential diagnosis of CMML patients with thrombocytopenia and anemia (Coombs positive or not) who develop signs of thrombotic microangiopathy. Further studies are much needed to decipher the immune-mediated processes in CMML.</p>","PeriodicalId":48865,"journal":{"name":"Acta Clinica Belgica","volume":"75 5","pages":"357-361"},"PeriodicalIF":1.6,"publicationDate":"2020-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1080/17843286.2019.1608395","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37173030","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 1
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Acta Clinica Belgica
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