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Prevention of burnout syndrome in physicians: a systematic review and meta-analysis. 医师职业倦怠综合症的预防:一项系统回顾和荟萃分析。
IF 2.1 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-11-10 DOI: 10.1007/s00508-025-02601-y
Lea Krebs, Laura Jung, Jasmin Arrich

Background: Physicians are at high risk of developing burnout and several studies have evaluated burnout prevention programs. This meta-analysis and systematic review aimed to assess the effectiveness of burnout prevention programs and to evaluate whether one approach is superior to other programs.

Patients, material and methods: The methods were based on the Cochrane Handbook of Systematic Reviews. We searched the literature in five medical databases from October 2019 to June 2022. We included randomized controlled trials that examined the effect of burnout prevention programs on physicians.

Results: A total of 22 studies were included in our analysis, with 20 studies targeting individual interventions and 2 studies targeting structural interventions. Mindfulness-based stress reduction (MBSR) or similar programs were the most common interventions. The main analysis showed a significant reduction of burnout in the intervention groups with an standardised mean difference (SMD) of -0.32 (95% confidence interval, CI: -0.41 to -0.22), which suggests a small to moderate effect. MBSR did not appear superior to other interventions in the subgroup analysis SMD -0.25 (95% CI: -0.48 to -0.02) versus SMD -0.61 (95% CI: -1.19 to 0.03).

Discussion: This analysis shows that individual burnout prevention programs may reduce burnout rates in physicians; however, the effect was relatively small. The effect was reduced even further when removing studies causing severe heterogeneity and those with a high risk of bias. Future research programs should focus on structural programs that address the lack of mentoring, rising administrative tasks, or long working hours, which may be more effective in reducing burnout in physicians.

背景:医生有很高的职业倦怠风险,一些研究已经评估了职业倦怠预防方案。本荟萃分析和系统回顾旨在评估职业倦怠预防方案的有效性,并评估一种方法是否优于其他方案。患者、材料和方法:方法基于Cochrane系统评价手册。我们从2019年10月至2022年6月在五个医学数据库中检索了文献。我们纳入了随机对照试验,以检验倦怠预防项目对医生的影响。结果:我们的分析共纳入了22项研究,其中20项研究针对个体干预,2项研究针对结构干预。正念减压(MBSR)或类似的项目是最常见的干预措施。主要分析显示,干预组的职业倦怠显著减少,标准化平均差异(SMD)为-0.32(95%置信区间,CI: -0.41至-0.22),表明影响小到中等。在亚组分析中,MBSR并没有表现出优于其他干预措施(SMD -0.25 (95% CI: -0.48至-0.02)和SMD -0.61 (95% CI: -1.19至0.03)。讨论:这一分析表明,个人倦怠预防计划可能会降低医生的倦怠率;然而,这种影响相对较小。当排除导致严重异质性和高偏倚风险的研究时,效果进一步降低。未来的研究项目应该集中在解决缺乏指导、管理任务增加或工作时间过长的结构性项目上,这可能更有效地减少医生的职业倦怠。
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引用次数: 0
[177Lu]Lu-PSMA radioligand therapy as an initial approach in hormone-sensitive metastatic prostate cancer: a case report. [177]Lu-PSMA放射配体治疗激素敏感性转移性前列腺癌1例报告。
IF 2.1 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-11-06 DOI: 10.1007/s00508-025-02642-3
Ilva Kristiana Langrate, Elisabeth Kretschmer-Chott, Stefan Schmitl, Shahrokh F Shariat, Marcus Hacker, Gero Kramer, Sazan Rasul

The [177Lu]Lu-PSMA radioligand therapy is a prostate-specific membrane antigen (PSMA) targeting treatment approved for patients with metastatic castration-resistant prostate cancer, after failure of androgen deprivation therapy or taxane-based chemotherapy. We report a unique case of a patient with metastatic hormone-sensitive prostate cancer (mHSPC) who received PSMA radioligand therapy as the sole treatment, without any prior or additional systemic treatment. Over a span of 8 years, the patient underwent 12 cycles of [177Lu]Lu-PSMA radioligand therapy, demonstrating favorable therapeutic response and a prolonged androgen deprivation therapy-free period. This case highlights the potential of long-term PSMA radioligand therapy as a standalone option in selected mHSPC patients.

[177Lu]Lu-PSMA放射配体治疗是一种前列腺特异性膜抗原(PSMA)靶向治疗,被批准用于转移性去势抵抗性前列腺癌患者在雄激素剥夺治疗或紫杉烷化疗失败后的治疗。我们报告一例独特的转移性激素敏感性前列腺癌(mHSPC)患者,接受PSMA放射配体治疗作为唯一治疗,没有任何先前或额外的全身治疗。在8年的时间里,患者接受了12个周期的[177Lu]Lu-PSMA放射配体治疗,显示出良好的治疗反应和延长的雄激素剥夺无治疗期。该病例强调了长期PSMA放射配体治疗在选定的mHSPC患者中作为独立选择的潜力。
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引用次数: 0
Nodular changes in the tracheobronchial mucosa in nonasthmatic eosinophilic bronchitis. 非哮喘性嗜酸性支气管炎气管支气管黏膜结节性改变。
IF 2.1 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-11-06 DOI: 10.1007/s00508-025-02647-y
Hui Chen, Shengyu Wang, Yanyun Jia
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引用次数: 0
Reply: comment on "Acute liver failure in patients admitted to the intensive care unit-A Viennese retrospective single-center analysis". 回复:关于“重症监护病房住院患者急性肝功能衰竭——维也纳回顾性单中心分析”的评论。
IF 2.1 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-11-01 Epub Date: 2025-08-29 DOI: 10.1007/s00508-025-02600-z
Patrick Haselwanter, Mathias Schneeweiss-Gleixner
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引用次数: 0
Markers for the angiogenic potential of fat grafts. 脂肪移植血管生成潜能的标志物。
IF 2.1 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-11-01 Epub Date: 2025-04-15 DOI: 10.1007/s00508-025-02532-8
Maryana Teufelsbauer, Sandra Stickler, Marie-Therese Eggerstorfer, Dennis C Hammond, Clemens Lang, Gerhard Hamilton

Background: Fat grafting is widely utilized in reconstructive and esthetic plastic surgery, typically with minimal complications. Nevertheless, the occurrence of fat necrosis is dependent on the technique used for fat extraction, tissue processing and the volume of the graft. The longevity of the graft critically depends on the presence of adipose-derived stromal cells (ADSC) and their promotion of a reconstituted vascular supply.

Objective: This study seeks to determine whether there are differences in 13 angiogenesis-related adipokines based on their grouping by vascular endothelial growth factor (VEGF) expression levels.

Methods: The expression of 14 adipokines related to angiogenesis in 12 cultured ADSCs was evaluated using Human Adipokine Profiler kits, which simultaneously detect 58 mediators. Adipokines of the high and low VEGF expression groups were evaluated for their expression of the remaining 13 angiogenic proteins.

Results: We were able to show that there are significant differences in VEGFlow and VEGFhigh ADSCs regarding fibroblast growth factor 19 (p = 0.043) and insulin like growth factor binding protein 3 (p = 0.028). Furthermore, ADSCs with differentially highly expressed VEGF show a different pattern in the amount of protein levels regarding the 13 other adipokines observed.

Conclusion: The VEGF has been described as a key angiogenic factor in fat grafts that may be linked to successful grafting; however, two of the fat samples analyzed exhibited high expression of VEGF but lacked significant co-expression of a range of other angiogenic factors. Thus, the assessment of the expression of predisposing mediators for graft angiogenesis for wound healing or contouring should include further angiogenesis promoters aside VEGF as parameters.

背景:脂肪移植广泛应用于重建和美容整形手术,通常并发症很少。然而,脂肪坏死的发生取决于脂肪提取技术、组织处理和移植物的体积。移植物的寿命在很大程度上取决于脂肪源性基质细胞(ADSC)的存在及其对血管供应重建的促进作用。目的:本研究旨在通过血管内皮生长因子(VEGF)表达水平分组来确定13种血管生成相关脂肪因子是否存在差异。方法:使用Human Adipokine Profiler试剂盒,同时检测58种介质,评估14种与血管生成相关的脂肪因子在12个培养的ADSCs中的表达。评估VEGF高表达组和低表达组的脂肪因子对其余13种血管生成蛋白的表达。结果:我们能够证明VEGFlow和VEGFhigh ADSCs在成纤维细胞生长因子19 (p = 0.043)和胰岛素样生长因子结合蛋白3 (p = 0.028)方面存在显著差异。此外,对于观察到的其他13种脂肪因子,VEGF高表达差异的ADSCs在蛋白质水平上表现出不同的模式。结论:VEGF已被描述为脂肪移植中一个关键的血管生成因子,可能与移植成功有关;然而,分析的两种脂肪样本显示VEGF高表达,但缺乏一系列其他血管生成因子的显著共表达。因此,评估移植物血管生成的易感介质对伤口愈合或轮廓的表达应包括除VEGF外的其他血管生成促进因子作为参数。
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引用次数: 0
MUW researcher of the month: Dipl.-Ing.in Anja Agneter. 本月最佳MUW研究员:Dipl.-Ing。在Anja Agneter。
IF 2.1 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-11-01 DOI: 10.1007/s00508-025-02657-w
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引用次数: 0
Completely ulcerated infantile hemangioma: a diagnostic challenge. 完全溃烂的婴儿血管瘤:一个诊断挑战。
IF 2.1 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-11-01 Epub Date: 2025-05-22 DOI: 10.1007/s00508-025-02543-5
Elias Marquart, Doris Weiss, Klaudija Batinic, Thomas Wiesner, Johannes Rohrbeck, Tamara Arnoldner, Wolfgang Weninger, Tamar Kinaciyan

The present case report describes a completely ulcerated infantile hemangioma (UIH) in a 5-month-old infant on the left proximolateral thigh initially misdiagnosed as pyoderma gangrenosum, sporotrichosis or atypical mycobacterial infection. Clinical assessment, histological findings, and GLUT‑1 immunohistochemistry confirmed the diagnosis of UIH. Systemic propranolol treatment led to rapid ulcer healing within 3 weeks and complete recovery without relapse after 18 months of treatment. The report emphasizes the diagnostic challenges, effective propranolol treatment and the importance of considering UIH in the differential diagnoses of solitary pediatric ulcers.

本病例报告描述了一个完全溃疡的婴儿血管瘤(UIH)在5个月大的婴儿左大腿近外侧最初误诊为坏疽性脓皮病,孢子毛病或非典型分枝杆菌感染。临床评估、组织学检查和GLUT‑1免疫组织化学证实了UIH的诊断。全身心得安治疗3周内溃疡迅速愈合,治疗18个月后完全恢复无复发。该报告强调了诊断的挑战、有效的心得安治疗以及在儿童孤立性溃疡鉴别诊断中考虑uh的重要性。
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引用次数: 0
Acute liver failure in patients admitted to the intensive care unit-a Viennese retrospective single-center analysis. 重症监护病房住院患者的急性肝衰竭——维也纳回顾性单中心分析
IF 2.1 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-11-01 Epub Date: 2025-05-26 DOI: 10.1007/s00508-025-02539-1
Patrick Haselwanter, Seanna Fairfield, Marlene Riedl-Wewalka, Monika Schmid, Albert Friedrich Stättermayer, Thomas Reiberger, Michael Trauner, Christian Zauner, Mathias Schneeweiss-Gleixner

Background: Acute liver failure (ALF) is characterized by a rapid deterioration of liver function and a high mortality without transplantation depending on etiology and onset. Immediate transfer to a dedicated intensive care unit (ICU) and evaluation for high-urgency liver transplantation (HU-LTx) is recommended to maximize chances of survival. Data on ALF epidemiology are limited, particularly for Central Europe.

Methods: This retrospective single-center study included all ALF patients admitted to the ICU of the Department of Gastroenterology and Hepatology at the Vienna General Hospital between 2012 and 2024.

Results: Overall, 31 patients (median age of 44 [interquartile range, IQR 32-56] years, 20 [65%] female) were included. The primary causes of ALF were viral infections (n = 8; 26%), autoimmune hepatitis (n = 5; 16%), drug-induced liver injury (DILI; n = 3; 10%), and Wilson's disease (n = 4; 13%), while in 8 patients (26%) no cause was identified. Median length of ICU stay was 12 (IQR 4-21) days, with mean sequential organ failure assessment (SOFA) and simplified acute physiology score II (SAPS II) scores of 10.55 ± 4.56 and 40.97 ± 14.84. Overall ICU survival was 61% (n = 19). Non-HU-LTx patients (n = 18) had an ICU survival of 44%. HU-LTx was performed in 13 patients (42%), with 12 patients (92%) surviving 28 days. The 6‑month overall survival of HU-LTx patients was 85%.

Conclusion: The diverse causes of ALF in Central Europe include most commonly viral infections, autoimmune hepatitis, and DILI. HU-LTx was required and performed in almost half of patients and was associated with favorable survival rates, underscoring the importance of ICU management and early transfer to liver transplantation centers in the management of ALF.

背景:急性肝衰竭(Acute liver failure, ALF)的特点是肝功能的迅速恶化和不需要移植的高死亡率,这取决于病因和发病。建议立即转移到专门的重症监护病房(ICU)并评估高急肝移植(HU-LTx),以最大限度地提高生存机会。关于ALF流行病学的数据有限,特别是在中欧。方法:本回顾性单中心研究纳入2012年至2024年在维也纳总医院胃肠病学和肝病科ICU住院的所有ALF患者。结果:共纳入31例患者(中位年龄44岁[四分位数范围,IQR 32-56]岁,女性20例[65%])。ALF的主要原因是病毒感染(n = 8;26%),自身免疫性肝炎(n = 5;16%),药物性肝损伤(DILI;n = 3;10%)和威尔逊氏病(n = 4;13%),而8例患者(26%)未确定病因。ICU的中位住院时间为12 (IQR 4-21)天,平均顺序器官衰竭评估(SOFA)和简化急性生理评分II (SAPS II)评分分别为10.55 ±4.56和40.97 ±14.84。ICU总生存率为61% (n = 19)。非hu - ltx患者(n = 18)的ICU生存率为44%。13例患者(42%)接受HU-LTx治疗,12例患者(92%)存活28天。HU-LTx患者的6个月总生存率为85%。结论:中欧地区ALF的病因多种多样,包括最常见的病毒感染、自身免疫性肝炎和DILI。几乎一半的患者需要进行HU-LTx治疗,并且与良好的生存率相关,这强调了ICU管理和早期转移到肝移植中心对ALF治疗的重要性。
{"title":"Acute liver failure in patients admitted to the intensive care unit-a Viennese retrospective single-center analysis.","authors":"Patrick Haselwanter, Seanna Fairfield, Marlene Riedl-Wewalka, Monika Schmid, Albert Friedrich Stättermayer, Thomas Reiberger, Michael Trauner, Christian Zauner, Mathias Schneeweiss-Gleixner","doi":"10.1007/s00508-025-02539-1","DOIUrl":"10.1007/s00508-025-02539-1","url":null,"abstract":"<p><strong>Background: </strong>Acute liver failure (ALF) is characterized by a rapid deterioration of liver function and a high mortality without transplantation depending on etiology and onset. Immediate transfer to a dedicated intensive care unit (ICU) and evaluation for high-urgency liver transplantation (HU-LTx) is recommended to maximize chances of survival. Data on ALF epidemiology are limited, particularly for Central Europe.</p><p><strong>Methods: </strong>This retrospective single-center study included all ALF patients admitted to the ICU of the Department of Gastroenterology and Hepatology at the Vienna General Hospital between 2012 and 2024.</p><p><strong>Results: </strong>Overall, 31 patients (median age of 44 [interquartile range, IQR 32-56] years, 20 [65%] female) were included. The primary causes of ALF were viral infections (n = 8; 26%), autoimmune hepatitis (n = 5; 16%), drug-induced liver injury (DILI; n = 3; 10%), and Wilson's disease (n = 4; 13%), while in 8 patients (26%) no cause was identified. Median length of ICU stay was 12 (IQR 4-21) days, with mean sequential organ failure assessment (SOFA) and simplified acute physiology score II (SAPS II) scores of 10.55 ± 4.56 and 40.97 ± 14.84. Overall ICU survival was 61% (n = 19). Non-HU-LTx patients (n = 18) had an ICU survival of 44%. HU-LTx was performed in 13 patients (42%), with 12 patients (92%) surviving 28 days. The 6‑month overall survival of HU-LTx patients was 85%.</p><p><strong>Conclusion: </strong>The diverse causes of ALF in Central Europe include most commonly viral infections, autoimmune hepatitis, and DILI. HU-LTx was required and performed in almost half of patients and was associated with favorable survival rates, underscoring the importance of ICU management and early transfer to liver transplantation centers in the management of ALF.</p>","PeriodicalId":23861,"journal":{"name":"Wiener Klinische Wochenschrift","volume":" ","pages":"720-729"},"PeriodicalIF":2.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12592254/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144152070","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
High lymphocyte count and bleeding risk in patients with chronic lymphocytic leukemia treated with Bruton's tyrosine kinase inhibitors. 布鲁顿酪氨酸激酶抑制剂治疗慢性淋巴细胞白血病患者的高淋巴细胞计数和出血风险
IF 2.1 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-11-01 Epub Date: 2025-04-11 DOI: 10.1007/s00508-025-02529-3
Ivan Krečak, Bruna Kvinta, Marina Paladin, Aron Grubešić, Marija Stanić Damić, Neven Franjić, Josipa Budimir, Josipa Antonija Bačić, Davor Galušić, Zinaida Perić, Marko Skelin

Bruton's tyrosine kinase inhibitors (BTKi) are being increasingly used to treat patients with chronic lymphocytic leukemia (CLL). Pathological bleeding is a well-known side effect of BTKi but identifying its predictors remains a challenge. This retrospective multicenter study analyzed whether baseline absolute lymphocyte count (ALC) may be associated with bleeding risk in CLL patients treated with BTKi. Time to bleeding (TTB) was the primary outcome of interest. A total of 108 CLL patients treated with BTKi (ibrutinib, n = 86, acalabrutinib, n = 22) were included. The median age was 70 years (range 41-88 years) and 48 (44.4%) were female. The median follow-up time was 32 months (range 1-108 months) and 17 (15.7%) bleeding events occurred during this time. Receiver operating curve analysis set the optimal cut-off value of the ALC at > 77.4 × 109/L. Patients with higher ALC presented with higher total white blood cell count (p < 0.001), lower hemoglobin (p = 0.012), higher Rai stages (p = 0.037) and higher total tumor mass (p < 0.001). Univariately, patients with higher ALC had an inferior TTB when compared to those with lower ALC (hazard ratio, HR 3.27, p = 0.016); this effect persisted in the multivariate Cox regression analysis where higher ALC (HR 4.59, p = 0.032), higher Cumulative Illness Rating Scale (CIRS, HR 4.21, p = 0.040) and the use of antiplatelets/anticoagulants (HR 3.96, p = 0.046) remained independently of each other associated with an inferior TTB. This study provides an important signal regarding the higher risk of bleeding in CLL patients treated with BTKi who present with higher ALC and higher CIRS. Further studies are needed to validate our findings and to unravel the exact pathophysiological mechanisms behind this interesting observation.

布鲁顿酪氨酸激酶抑制剂(BTKi)越来越多地用于治疗慢性淋巴细胞白血病(CLL)患者。病理性出血是众所周知的BTKi副作用,但确定其预测因素仍然是一个挑战。这项回顾性多中心研究分析了基线绝对淋巴细胞计数(ALC)是否可能与接受BTKi治疗的CLL患者出血风险相关。出血时间(TTB)是主要观察指标。共纳入108例接受BTKi治疗的CLL患者(ibrutinib, n = 86,acalabrutinib, n = 22)。年龄中位数为70岁(41 ~ 88岁),女性48例(44.4%)。中位随访时间为32个月(1-108个月),期间发生17例(15.7%)出血事件。受试者工作曲线分析确定ALC的最佳临界值为> 77.4 × 109/L。ALC高的患者总白细胞计数较高(p
{"title":"High lymphocyte count and bleeding risk in patients with chronic lymphocytic leukemia treated with Bruton's tyrosine kinase inhibitors.","authors":"Ivan Krečak, Bruna Kvinta, Marina Paladin, Aron Grubešić, Marija Stanić Damić, Neven Franjić, Josipa Budimir, Josipa Antonija Bačić, Davor Galušić, Zinaida Perić, Marko Skelin","doi":"10.1007/s00508-025-02529-3","DOIUrl":"10.1007/s00508-025-02529-3","url":null,"abstract":"<p><p>Bruton's tyrosine kinase inhibitors (BTKi) are being increasingly used to treat patients with chronic lymphocytic leukemia (CLL). Pathological bleeding is a well-known side effect of BTKi but identifying its predictors remains a challenge. This retrospective multicenter study analyzed whether baseline absolute lymphocyte count (ALC) may be associated with bleeding risk in CLL patients treated with BTKi. Time to bleeding (TTB) was the primary outcome of interest. A total of 108 CLL patients treated with BTKi (ibrutinib, n = 86, acalabrutinib, n = 22) were included. The median age was 70 years (range 41-88 years) and 48 (44.4%) were female. The median follow-up time was 32 months (range 1-108 months) and 17 (15.7%) bleeding events occurred during this time. Receiver operating curve analysis set the optimal cut-off value of the ALC at > 77.4 × 10<sup>9</sup>/L. Patients with higher ALC presented with higher total white blood cell count (p < 0.001), lower hemoglobin (p = 0.012), higher Rai stages (p = 0.037) and higher total tumor mass (p < 0.001). Univariately, patients with higher ALC had an inferior TTB when compared to those with lower ALC (hazard ratio, HR 3.27, p = 0.016); this effect persisted in the multivariate Cox regression analysis where higher ALC (HR 4.59, p = 0.032), higher Cumulative Illness Rating Scale (CIRS, HR 4.21, p = 0.040) and the use of antiplatelets/anticoagulants (HR 3.96, p = 0.046) remained independently of each other associated with an inferior TTB. This study provides an important signal regarding the higher risk of bleeding in CLL patients treated with BTKi who present with higher ALC and higher CIRS. Further studies are needed to validate our findings and to unravel the exact pathophysiological mechanisms behind this interesting observation.</p>","PeriodicalId":23861,"journal":{"name":"Wiener Klinische Wochenschrift","volume":" ","pages":"702-706"},"PeriodicalIF":2.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144050467","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: 0
Wild-type transthyretin cardiac amyloidosis mimicking hypertrophic obstructive cardiomyopathy : A case report. 模拟肥厚性阻塞性心肌病的野生型转甲状腺素型心脏淀粉样变1例报告。
IF 2.1 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-11-01 Epub Date: 2025-05-13 DOI: 10.1007/s00508-025-02540-8
Nikol Kubinova, Tomas Paleček, Marek Mika, Radek Jaksa, Ales Linhart

Background: Wild-type transthyretin cardiac amyloidosis (ATTRwt CA) is increasingly recognized as an important cause of heart failure and arrhythmias in older people. There are several clinical, echocardiographic, electrocardiographic (ECG) and laboratory features that increase the suspicion for ATTRwt CA. Presentation and phenotype can, however, be associated with atypical findings making it difficult to make a correct diagnosis. A 65-year-old man was admitted for an acute coronary syndrome. Echocardiography revealed diffuse concentric left ventricular (LV) thickening. Because of a history of bilateral carpal tunnel syndrome and polyneuropathy, the patient underwent dedicated laboratory testing and diphosphonate scintigraphy the results of which were suggestive of transthyretin cardiac amyloidosis. Also, a dynamic LV outflow tract obstruction due to the systolic anterior motion of the anterior mitral valve was noted on echocardiography during the initial investigations. Genetic testing for hypertrophic cardiomyopathy was negative. Seeking a conclusive diagnosis, endomyocardial biopsy was performed. This confirmed the diagnosis of ATTRwt CA.

Discussion: The presence of dynamic LV outflow tract obstruction is typically seen in patients with sarcomeric hypertrophic cardiomyopathy. It can be rarely seen also in individuals with cardiac amyloidosis, including ATTR-wt CA. The presence of so-called red flags in patients' history, physical examination, laboratory test, ECG and imaging should raise suspicion for other etiologies of LV wall thickening than hypertrophic cardiomyopathy. Although noninvasive diagnosis of ATTRwt CA is possible in most patients, endomyocardial biopsy remains necessary in cases with diagnostic ambiguity.

背景:野生型转甲状腺素型心脏淀粉样变(ATTRwt CA)越来越被认为是老年人心力衰竭和心律失常的重要原因。有几个临床、超声心动图、心电图(ECG)和实验室特征增加了对ATTRwt CA的怀疑。然而,表现和表型可能与非典型表现有关,因此难以做出正确的诊断。一名65岁男子因急性冠状动脉综合征入院。超声心动图显示左室弥漫性同心性增厚。由于有双侧腕管综合征和多神经病变的病史,患者接受了专门的实验室检查和二膦酸盐显像检查,结果提示甲状腺素型心脏淀粉样变性。此外,在最初的调查中,超声心动图显示由于前二尖瓣收缩前运动引起的动态左室流出道阻塞。肥厚性心肌病基因检测呈阴性。为寻求结论性诊断,进行了心内膜肌活检。讨论:动态左室流出道梗阻在肌瘤性肥厚性心肌病患者中很常见。在心脏淀粉样变患者(包括atr -wt型CA)中也很少见。在患者的病史、体格检查、实验室检查、心电图和影像学中出现所谓的“危险信号”,应引起对左室壁增厚的其他病因的怀疑,而不是肥厚性心肌病。虽然在大多数患者中,无创诊断ATTRwt CA是可能的,但在诊断不明确的情况下,仍有必要进行心内膜活检。
{"title":"Wild-type transthyretin cardiac amyloidosis mimicking hypertrophic obstructive cardiomyopathy : A case report.","authors":"Nikol Kubinova, Tomas Paleček, Marek Mika, Radek Jaksa, Ales Linhart","doi":"10.1007/s00508-025-02540-8","DOIUrl":"10.1007/s00508-025-02540-8","url":null,"abstract":"<p><strong>Background: </strong>Wild-type transthyretin cardiac amyloidosis (ATTRwt CA) is increasingly recognized as an important cause of heart failure and arrhythmias in older people. There are several clinical, echocardiographic, electrocardiographic (ECG) and laboratory features that increase the suspicion for ATTRwt CA. Presentation and phenotype can, however, be associated with atypical findings making it difficult to make a correct diagnosis. A 65-year-old man was admitted for an acute coronary syndrome. Echocardiography revealed diffuse concentric left ventricular (LV) thickening. Because of a history of bilateral carpal tunnel syndrome and polyneuropathy, the patient underwent dedicated laboratory testing and diphosphonate scintigraphy the results of which were suggestive of transthyretin cardiac amyloidosis. Also, a dynamic LV outflow tract obstruction due to the systolic anterior motion of the anterior mitral valve was noted on echocardiography during the initial investigations. Genetic testing for hypertrophic cardiomyopathy was negative. Seeking a conclusive diagnosis, endomyocardial biopsy was performed. This confirmed the diagnosis of ATTRwt CA.</p><p><strong>Discussion: </strong>The presence of dynamic LV outflow tract obstruction is typically seen in patients with sarcomeric hypertrophic cardiomyopathy. It can be rarely seen also in individuals with cardiac amyloidosis, including ATTR-wt CA. The presence of so-called red flags in patients' history, physical examination, laboratory test, ECG and imaging should raise suspicion for other etiologies of LV wall thickening than hypertrophic cardiomyopathy. Although noninvasive diagnosis of ATTRwt CA is possible in most patients, endomyocardial biopsy remains necessary in cases with diagnostic ambiguity.</p>","PeriodicalId":23861,"journal":{"name":"Wiener Klinische Wochenschrift","volume":" ","pages":"715-719"},"PeriodicalIF":2.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12592313/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144001501","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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