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Entirely Intramural Growth Pattern: A Rare Presentation of Esophageal Squamous Cell Carcinoma and Review of the Literature. 完全壁内生长模式:食管鳞状细胞癌的罕见表现及文献综述
IF 1.4 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-11-25 DOI: 10.1055/a-2442-9540
Jiayu Qiu, Yi Tu, Chen Yu, Xu Shu, Xiaolin Pan, Yanxia Zhang

Esophageal squamous cell carcinoma (ESCC) is a malignant tumor originating from the squamous epithelium. In contrast, esophageal submucosal tumors are common benign lesions arising from mesenchymal tissues. To date, an entirely intramural growth of ESCC is very rare. This study described a case of an esophageal submucosal tumor resected by endoscopic submucosal dissection (ESD) that was finally diagnosed as ESCC.A 51-year-old woman presented with progressive dysphagia and was provisionally diagnosed with esophageal leiomyoma by further diagnostic modalities. The patient did not have any obvious suspicious malignant features and underwent ESD. However, the histopathology of the resected specimen was reported as poorly differentiated infiltrating squamous cell carcinoma with normal overlying squamous epithelium. Consequently, the patient received additional chemoradiotherapy, and no recurrence was observed during the 2-year follow-up.A comprehensive literature search related to ESCC with entirely intramural growth was performed in PubMed and Embase from their inception up to November 2023, and 12 articles including 13 cases were finally included in the literature review. Subsequently, we extracted information about these cases.It is concluded that ESCC may masquerade as a submucosal tumor with a complete submucosal growth pattern and is easily misdiagnosed because endoscopic biopsy and iodine staining are always negative. Therefore, if a patient with a submucosal tumor has dysphagia or weight loss in the short term, clinicians should be alert to the possibility of ESCC with a complete submucosal growth pattern. Endoscopic ultrasonography (EUS), chest computed tomography (CT), or positron emission tomography-computed tomography (PET-CT) may help assist in the diagnosis, and EUS-guided fine-needle aspiration (EUS-FNA) could be used to confirm the diagnosis.

食管鳞状细胞癌(ESCC)是一种起源于鳞状上皮的恶性肿瘤。相比之下,食管粘膜下肿瘤是常见的良性病变,源于间质组织。迄今为止,完全由间质内生长的 ESCC 非常罕见。本研究描述了一例通过内镜下粘膜下剥离术(ESD)切除的食管粘膜下肿瘤,该肿瘤最终被诊断为ESCC。一名51岁的女性患者出现进行性吞咽困难,通过进一步诊断,患者被初步诊断为食管子宫肌瘤。患者没有任何明显的可疑恶性特征,并接受了ESD检查。然而,切除标本的组织病理学报告为分化较差的浸润性鳞状细胞癌,其上覆有正常的鳞状上皮。我们在PubMed和Embase上进行了从开始到2023年11月的有关完全壁内生长的ESCC的全面文献检索,最终包括13例病例在内的12篇文章被纳入文献综述。结论是 ESCC 可伪装成完全粘膜下生长的粘膜下肿瘤,由于内镜活检和碘染色总是阴性,因此很容易被误诊。因此,如果粘膜下肿瘤患者在短期内出现吞咽困难或体重减轻,临床医生应警惕具有完整粘膜下生长模式的 ESCC 的可能性。内镜超声检查(EUS)、胸部计算机断层扫描(CT)或正电子发射断层扫描-计算机断层扫描(PET-CT)可帮助辅助诊断,EUS 引导下的细针穿刺术(EUS-FNA)可用于确诊。
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引用次数: 0
Effect of Bacteroides on Crohn's disease. Bacteroides 对克罗恩病的影响。
IF 1.4 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-11-25 DOI: 10.1055/a-2435-2659
Xuanyu Wei, Dong Tang

Crohn's disease (CD), also known as cicatrizing enteritis, is an inflammatory bowel disease that occurs in the distal ileum and right colon of unknown cause and is also called inflammatory bowel disease (IBD) with ulcerative colitis (UC). In recent years, intestinal biota have been confirmed to play a significant role in various gastrointestinal diseases. Studies have found that intestinal microbiota disorders are closely associated with the onset and progression of Crohn's disease. Bacteroidetes, the second largest microbiota in the intestine, are crucial for equilibrium in the microbiota and intestinal environment. Certain Bacteroides can induce the development of Crohn's disease and aggravate intestinal inflammation directly or through their metabolites. Conversely, certain Bacteroides can reduce intestinal inflammation and symptoms of Crohn's disease. This article reviews the effect of several intestinal Bacteroides in the onset and progression of Crohn's disease and their impact on its treatment.

克罗恩病(Crohn's disease,CD)又称糜烂性肠炎,是一种发生在回肠远端和右侧结肠的原因不明的炎症性肠病,也被称为炎症性肠病(IBD)伴溃疡性结肠炎(UC)。近年来,肠道生物群已被证实在各种胃肠道疾病中发挥着重要作用。研究发现,肠道微生物群紊乱与克罗恩病的发病和进展密切相关。类杆菌是肠道中第二大微生物群,对微生物群和肠道环境的平衡至关重要。某些类杆菌能直接或通过其代谢产物诱导克罗恩病的发生并加重肠道炎症。相反,某些乳酸菌可减轻肠道炎症和克罗恩病症状。本文综述了几种肠道乳杆菌在克罗恩病发病和发展过程中的作用及其对治疗的影响。
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引用次数: 0
[Percutaneous endoscopic gastrostomy in a 42-year-old patient with suspected variant of Creutzfeld Jakob disease using a disposable bronchoscope]. [使用一次性支气管镜为一名 42 岁疑似克雅氏病变异型患者进行经皮内镜胃造瘘术]。
IF 1.4 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-11-25 DOI: 10.1055/a-2406-9419
Michael Klausner, Roman Feller, Wolfgang Vogt, Henning Wege

Prion diseases pose a challenge for flexible reusable endoscopes. Especially for the new variant of Creutzfeld Jakob disease (vCJD) there is currently no suitable processing procedure. In these patients only disposable endoscopes are hygienically safe. The case report shows a PEG placement in a 42-year-old patient with suspected vCJD using a disposable bronchoscope.

朊病毒疾病给可重复使用的柔性内窥镜带来了挑战。尤其是克雅氏病的新变种(vCJD),目前还没有合适的处理程序。对于这些患者,只有一次性内窥镜才是卫生安全的。本病例报告展示了使用一次性支气管镜为一名 42 岁疑似 vCJD 患者进行 PEG 置入术的情况。
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引用次数: 0
[Significant increase in newly diagnosed hepatitis B and C cases in Germany due to screening]. [德国新诊断的乙型和丙型肝炎病例因筛查而显著增加]。
IF 1.4 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-11-25 DOI: 10.1055/a-2435-5069
Dietrich Hüppe, Yvonne Serfert, Markus Cornberg, Heiner Wedemeyer

In 2016, the World Health Organization (WHO) decided on an initiative to identify 90% of the world's existing hepatitis B and C virus infections by 2030, treat 80% and reduce the mortality by 65%. In 2016, the federal government endorsed these goals. From Oct 1, 2021, the G-BA included a one-time screening for hepatitis B and C in the health examination (GU) for people aged 35 and over who have statutory health insurance. The previous participation rates in the GU were evaluated and the effectiveness of the program is documented using the reporting data on hepatitis B and C of Robert Koch Institute (RKI).Notifications from Central Institute of Statutory Health Insurance (ZI) and Scientific Institute of the local health insurance companies (WIdO) were compiled with regard to the GU, the reporting data from the RKI were collected and analyzed using SURVSTAT@RKI 2.0 as well as billing data from the Association of Statutory Health Insurance Physicians (KBV).Between 2007 and 2021, around 83% of all insured persons took part in a GU at least once. From the fourth quarter of 2021, the number of newly diagnosed cases of hepatitis B and C increased. In 2022, the increase for hepatitis B was 91.9% and for hepatitis C 68%. This trend continued in 2023, with new diagnoses of hepatitis B increasing by 160% (to 22,795 cases) and hepatitis C increasing by 121% (to 10,508 cases) compared to 2021. The analysis of the KBV billing data showed that the increase in new diagnoses shows a strong correlation between the number of new diagnoses within the GU and the total number of diagnoses.The decision of the Federal Joint Committee (G-BA) to include hepatitis B and C screening in the GU seems to be a success in detecting previously undetected cases of the disease. Based on the billing data from the KBV, a high level of agreement could be demonstrated between the number of new diagnoses within the GU and the increase in the total number of diagnoses. The goals of the WHO may be achievable.

2016 年,世界卫生组织(WHO)决定采取一项举措,在 2030 年前发现全球 90% 的现有乙型和丙型肝炎病毒感染者,治疗 80%,并将死亡率降低 65%。2016 年,联邦政府批准了这些目标。自 2021 年 10 月 1 日起,G-BA 将在 35 岁及以上拥有法定医疗保险的人群的健康检查(GU)中加入一次性乙型和丙型肝炎筛查。通过罗伯特-科赫研究所(RKI)的乙型肝炎和丙型肝炎报告数据,对以往的健康检查参与率进行了评估,并记录了该计划的有效性。我们汇编了法定医疗保险中央研究所(ZI)和地方医疗保险公司科学研究所(WIdO)有关 GU 的通知,并使用 SURVSTAT@RKI 2.0 收集和分析了 RKI 的报告数据以及法定医疗保险医生协会(KBV)的账单数据。从 2021 年第四季度开始,新确诊的乙型肝炎和丙型肝炎病例数量有所增加。2022 年,乙型肝炎病例增加了 91.9%,丙型肝炎病例增加了 68%。这一趋势在 2023 年仍在继续,与 2021 年相比,乙型肝炎新诊断病例增加了 160%(达到 22,795 例),丙型肝炎增加了 121%(达到 10,508 例)。对 KBV 账单数据的分析表明,新诊断病例的增加表明,GU 内新诊断病例数与诊断病例总数之间存在很强的相关性。联邦联合委员会(G-BA)决定将乙型肝炎和丙型肝炎筛查纳入 GU,这似乎成功地发现了以前未被发现的疾病病例。联邦联合委员会(G-BA)将乙型肝炎和丙型肝炎筛查纳入 GU 的决定似乎成功地发现了以前未被发现的病例。根据 KBV 的账单数据,GU 新诊断病例数与诊断病例总数的增长之间存在高度的一致性。世卫组织的目标是可以实现的。
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引用次数: 0
Palliative treatment of malignant intestinal obstruction with EUS-guided ileosigmoidostomy - a case report. 通过 EUS 引导回肠乙状结肠造口术对恶性肠梗阻进行姑息治疗--病例报告。
IF 1.4 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-11-25 DOI: 10.1055/a-2452-4337
Sebastian Zundler, Kerstin Wolff, Moritz Leppkes, Anne Gaza, Sophie Haberkamp, Deike Strobel, Jürgen Siebler, Markus F Neurath, Maximilian Waldner

We present the case of a 70-year-old patient of Caucasian origin under palliative chemotherapy for metastasized colorectal cancer, who presented with signs of complete small bowel obstruction six months after placement of a diverting ileostomy. Following previous tumor perforation, he was not considered a candidate for further surgical intervention.Thus, endoscopic ultrasound-guided enterocolostomy (i.e., ileosigmoidostomy) with a lumen-apposing metal stent was successfully performed in a coloenteric approach to restore the passage.Oral nutrition could be resumed the next day, and the patient could be discharged in substantially improved condition after three days.

我们介绍了一例 70 岁的白种人结直肠癌转移姑息化疗患者的病例,该患者在实施回肠分流造口术 6 个月后出现完全性小肠梗阻症状。因此,在内窥镜超声引导下,通过结肠途径成功实施了肠结肠造口术(即回肠乙状结肠造口术),并在肠腔内放置了金属支架,恢复了肠道的通畅。第二天就可以恢复口服营养,三天后患者的情况大为好转,可以出院了。
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引用次数: 0
Severe gastroparesis complicated by gastric perforation caused by lightchain amyloidosis. 轻链淀粉样变性引起的严重胃痉挛并发胃穿孔。
IF 1.4 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-11-25 DOI: 10.1055/a-2442-7944
Michael Rietz, Tobias Weber, Tina Schaller, Jan Hendrik Luitjens, Luise Uhrmacher, Helmut Messmann, Andreas Probst

AL-Amyloidosis is a rare systemic disease that can occur in patients with monoclonal gammopathy or multiple myeloma. As multiple organs may be affected by deposition of amyloid fibrils, the clinical presentation varies considerably, and the diagnostic process may be challenging.We report on a 59-year-old female who suffered from gastroesophageal reflux symptoms, nausea, epigastric pain, and meteorism over several years. Repeated upper GI endoscopies including biopsies and CT scans were unremarkable except for unspecific enlargement of mesenterial lymph nodes.A few weeks after a surgical hiatal hernia repair with fundoplication, the patient developed massive distension of the stomach and the proximal duodenum resulting in gastric perforation. Histopathological staining of gastric biopsies and mesenterial lymph nodes using hematoxylin and eosin was unremarkable. Because of endoscopic findings (submucosal hematomas, and ulcerations) and the unexplained severe motility disorder, histopathological staining was performed using Congo red. Extensive amyloid deposits were seen. Further workup confirmed AL amyloidosis caused by monoclonal gammopathy. Specific oncological treatment was started.The rare differential diagnosis of amyloidosis should be taken into account in patients with unexplained motility disorders, unspecific gastrointestinal symptoms, and abdominal lymphadenopathy. In the presented case, delayed diagnosis of AL amyloidosis in the gastrointestinal tract led to severe gastroparesis resulting in gastric perforation. Specific histopathologic staining can confirm the diagnosis.

谷丙转氨酶淀粉样变性是一种罕见的全身性疾病,可发生在单克隆丙种球蛋白病或多发性骨髓瘤患者身上。由于淀粉样蛋白纤维沉积可能影响多个器官,因此临床表现差异很大,诊断过程可能具有挑战性。我们报告了一名 59 岁女性的病例,她数年来出现胃食管反流症状、恶心、上腹痛和流食。反复进行的上消化道内窥镜检查(包括活组织检查和 CT 扫描)除发现肠系膜淋巴结无特异性肿大外,其他检查结果均无异常。用苏木精和伊红对胃活检组织和肠系膜淋巴结进行了组织病理学染色,结果并无异常。由于内镜检查结果(粘膜下血肿和溃疡)和无法解释的严重运动障碍,医生使用刚果红进行了组织病理学染色。结果发现了广泛的淀粉样沉积。进一步检查证实了由单克隆抗体病引起的 AL 淀粉样变性。对于不明原因的运动障碍、非特异性胃肠道症状和腹部淋巴结病的患者,应考虑到淀粉样变性这一罕见的鉴别诊断。在本病例中,胃肠道中的AL淀粉样变性被延误诊断,导致严重胃瘫,造成胃穿孔。特定的组织病理学染色可以确诊。
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引用次数: 0
[Staffing situation and basics requirement calculations in the inpatient and outpatient sector in Germany - a representative survey by the Working Group of Senior Gastroenterology Hospital Physicians (ALGK)]. [德国住院病人和门诊病人的人员配置情况和基本需求计算--高级消化内科医院医生工作组(ALGK)的代表性调查]。
IF 1.4 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-11-25 DOI: 10.1055/a-2435-4967
Thomas Frieling, Dieter Schilling, Birgit Terjung, Ludger Leifeld, Torsten Kucharzik, Ahmed Madisch, Kerstin Schütte, Jochen Labenz, Alexander Dechêne, Felix Gundling, Markus Dollhopf, Carsten Gartung, Jörg G Albert, Carl Christoph Schimanski, Franz Ludwig Dumoulin, Josef Menzel

In Germany, there are no uniform hospital standards for the transparent calculation of medical staffing requirements in hospitals. This is particularly true for outpatient services.The ALGK conducted a member survey via the link https://de.surveymonkey.com/r/H7TTJYZ from March 21 to May 1, 2024. 400 members with a valid email address were contacted. The 119 extraordinary members who had ended their professional activity were not included. 17 questions were asked in the survey.The response rate was 24%. 37% represented basic and standard, 47% specialized and 16% maximum care providers. Municipal hospitals were represented by 42%, denominational providers by 34% and private providers by 24%. 7% of the hospitals had fewer than 200 beds, 28% 200 to 400 beds, 37% 400 to 600 beds, 15% 600 to 800 beds and 13% more than 800 beds. Up to 1.500 patients were treated annually in 15%, up to 2.500 patients in 15%, each up to 3.000 or 4.000 patient in 22%s, up to 5,000 patientsin 16% and more than 5.000 patients per year in 12%. The average casemix was 2373 ± 999 and the casemix index 0.70 ± 0.11. On average, 17.6 ± 7.6 doctor's posts were available and one consultant treated 14 ± 2.7 patients per day. A transparent personnel calculation was not available in 56% of the departments or clinics, in 82% there was no internal cost allocation or cost recovery calculation, in 54% there was no internal cost allocation. In 54%, there was no feedback on outpatient services from the administration, in 93% there was no transparent calculation of physician positions based on outpatient services, in 97% the training and further training of young colleagues was not taken into account in the calculation of positions, in 75% there was no communicated and recognizable strategic plan by the hospital operator for the senior gastroenterologists with regard to outpatient services. 49% of those surveyed feared that their hospital operator would not be able to cope with the restructuring in the healthcare sector with outpatient services, centralization, minimum volume compliance and would even jeopardize its existence.Transparent job calculation and communication about inpatient and outpatient gastroenterology services and the strategic objectives of the healthcare structural reform are very patchy in German hospitals. This leads to a high degree of uncertainty and existential fears.

在德国,没有统一的医院标准来透明地计算医院的医疗人员需求。2024 年 3 月 21 日至 5 月 1 日,ALGK 通过链接 https://de.surveymonkey.com/r/H7TTJYZ 进行了一项会员调查。我们联系了 400 名拥有有效电子邮件地址的会员。其中不包括已结束职业活动的 119 名特殊会员。调查共提出 17 个问题,回复率为 24%。基本和标准医疗服务提供者占 37%,专业医疗服务提供者占 47%,最高医疗服务提供者占 16%。市级医院占 42%,教派医疗机构占 34%,私营医疗机构占 24%。7%的医院床位数少于 200 张,28%的医院床位数在 200 到 400 张之间,37%的医院床位数在 400 到 600 张之间,15%的医院床位数在 600 到 800 张之间,13%的医院床位数在 800 张以上。15% 的医院每年收治的病人不超过 1 500 人,15% 的医院每年收治的病人不超过 2 500 人,22% 的医院每年收治的病人不超过 3 000 或 4 000 人,16% 的医院每年收治的病人不超过 5 000 人,12% 的医院每年收治的病人超过 5 000 人。平均病例组合为 2373 ± 999,病例组合指数为 0.70 ± 0.11。平均有 17.6 ± 7.6 个医生职位,一名顾问每天治疗 14 ± 2.7 名病人。56%的科室或诊所没有进行透明的人事计算,82%的科室或诊所没有进行内部成本分配或成本回收计算,54%的科室或诊所没有进行内部成本分配。54%的管理部门没有对门诊服务进行反馈,93%的管理部门没有根据门诊服务对医生职位进行透明的计算,97%的管理部门在计算职位时没有考虑到年轻同事的培训和进修,75%的医院经营者没有向高级消化科医生传达和宣传有关门诊服务的战略计划。49%的受访者担心,他们所在医院的运营者将无法应对医疗行业的重组,包括门诊服务、集中化、最低诊疗量等,甚至会危及医院的生存。在德国的医院中,有关住院和门诊消化内科服务以及医疗结构改革战略目标的透明职位计算和沟通非常零散。这导致了高度的不确定性和生存恐惧。
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引用次数: 0
Alveolar Echinococcosis - Diagnostic challenges of a parasitic disease on the rise in Europe - a case report. 肺泡棘球蚴病--一种在欧洲呈上升趋势的寄生虫病的诊断难题--病例报告。
IF 1.4 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-11-25 DOI: 10.1055/a-2414-1320
Jonas Schumacher, Henning Trawinski, Hans-Michael Tautenhahn, Hendrik Bläker, Johannes Wiegand

A 45-year-old male presented with jaundice, abdominal discomfort, and weight loss. Abdominal ultrasound revealed intrahepatic cholestasis and cholelithiasis indicative of Caroli-syndrome. Subsequent magnetic resonance imaging demonstrated a corresponding 5 × 4 cm polycyclic, calcified mass and a distant 12 mm subhilar stenosis of the common bile duct resembling cholangiocarcinoma. Calcifications seemed unusual and brought up differential diagnosis of echinococcosis. Serology revealed an echinococcus antibody-titer of 33 U (cut-off > 11.5 U). However, the clinical presentation with jaundice, weight loss, and a distant subhilar stenosis of the common bile duct without association to the intrahepatic process led to underestimation of the positive antibody-titer. Thus, the patient underwent right hemihepatectomy for potential cholangiocarcinoma. Dense infiltrative small cysts interspersed the resected right lobe of the liver. Histopathology revealed a granulomatous inflammation surrounding typical cuticulas, confirming hepatobiliary alveolar echinococcosis. Alveolar echinococcosis is considered as one of the neglected diseases by the World Health Organization, although incidence is rising worldwide and specifically in Germany. Our case illustrates hurdles in diagnosing alveolar echinococcosis and underlines the importance to be aware of classical and rare clinical manifestations.

一名 45 岁的男性因黄疸、腹部不适和体重减轻前来就诊。腹部超声波检查发现肝内胆汁淤积和胆石症,表明患有卡洛里综合征。随后的磁共振成像显示,有一个相应的 5 × 4 厘米多环状钙化肿块和一个远端 12 毫米的胆总管近端狭窄,类似胆管癌。钙化似乎并不常见,因此提出了棘球蚴病的鉴别诊断。血清学检查显示,棘球蚴抗体滴度为 33 U(临界值大于 11.5 U)。然而,黄疸、体重减轻和远端胆总管近端狭窄等临床表现与肝内病变无关,导致抗体阳性滴度被低估。因此,患者因可能患有胆管癌而接受了右半肝切除术。在切除的肝脏右叶中,密布着浸润性小囊肿。组织病理学显示,典型的切口周围有肉芽肿性炎症,确诊为肝胆泡状棘球蚴病。泡状棘球蚴病被世界卫生组织视为被忽视的疾病之一,尽管其发病率在全球,尤其是在德国呈上升趋势。我们的病例说明了在诊断肺泡棘球蚴病时遇到的障碍,并强调了了解典型和罕见临床表现的重要性。
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引用次数: 0
Sub-optimal therapy of patients with primary biliary cholangitis (PBC) in the real-life stetting of the German PBC cohort. 德国原发性胆汁性胆管炎(PBC)队列中原发性胆汁性胆管炎(PBC)患者的次优治疗。
IF 1.4 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-09-03 DOI: 10.1055/a-2382-7720
Johannes Wiegand, Annegret Franke, Tobias Müller, Kerstin Stein, Heike Bantel, Rainer Günther, Gerald Denk, Philipp A Reuken, Jörn M Schattenberg, Uwe Naumann, Tobias Böttler, Andreas Weber, Stefan Zeuzem, Matthias Hinz, Robin Greinert, Christoph Berg, Thaddäus Till Wissniowski, Karl-Georg Simon, Jonel Trebicka, Rüdiger Behrens, Harald Grümmer, Wolf Peter Hofmann, Nektarios Dikopoulos, Christoph Sarrazin, Elke Roeb, Andreas E Kremer, Marion Muche, Marc Ringelhan, Andreas Teufel, Patrick Michl, Verena Keitel, Jens U Marquardt, Achim Kautz, Frank Tacke, Katja Piotrowski, Nicole Köppe-Bauernfeind, Christian Trautwein, Thomas Berg

Real-world data on the management of patients with primary biliary cholangitis (PBC) are so far scarce in Germany. Therefore, we aimed to establish a nationwide registry and describe the clinical characteristics and therapy of PBC patients.Three different cohorts defined as ursodeoxycholic acid (UDCA) responders, as inadequate responders according to Paris II criteria, and as newly diagnosed patients were prospectively recruited.This manuscript includes the baseline data of the project.In total, 33/77 (43%) contacted centres (58% of university hospitals, 38% of non-university hospitals, and 24% of private practices) recruited 515 patients including 204 UDCA responders, 221 inadequate responders to UDCA, and 90 newly diagnosed patients.All patients were treated with UDCA; however, a UDCA dosage below the recommended dosage of 13 mg/kg/d was observed in 38.5% of individuals after 12 months of treatment. UDCA dosages were lower in nonacademic compared to academic centres.Only 75/219 (38.5%) of inadequate responders to UDCA received a second-line therapy with obeticholic acid (OCA) and/or bezafibrate (BZF). OCA (13% vs. 4.5%) and BZF (14% vs. 6.5%) were significantly more often prescribed by academic vs. nonacademic centres.Pruritus (27% vs. 15.5%), fatigue (23% vs. 4.5%), and sicca syndrome (14% vs. 1%) were significantly more often reported by academic centres.The German PBC registry could be established, which indicates suboptimal therapy in a relevant proportion of patients and shows significant differences between academic and nonacademic centres. Results are fundamental to improving clinical management at different levels of care.

迄今为止,德国有关原发性胆汁性胆管炎(PBC)患者治疗的真实数据还很少。因此,我们旨在建立一个全国范围的登记处,并描述 PBC 患者的临床特征和治疗方法。我们前瞻性地招募了熊去氧胆酸(UDCA)应答者、根据巴黎 II 标准应答不足者和新诊断患者三个不同的组群。33/77(43%)家联系中心(58%为大学医院,38%为非大学医院,24%为私人诊所)共招募了 515 名患者,其中包括 204 名 UDCA 反应者、221 名 UDCA 反应不足者和 90 名新诊断患者。与学术中心相比,非学术中心的 UDCA 剂量更低。在对 UDCA 反应不足的患者中,只有 75/219 人(38.5%)接受了奥贝胆酸 (OCA) 和/或贝扎贝特 (BZF) 的二线治疗。OCA(13% vs. 4.5%)和 BZF(14% vs. 6.5%)在学术中心的处方率明显高于非学术中心。瘙痒(27% vs. 15.5%)、疲劳(23% vs. 4.5%)和眼综合征(14% vs. 1%)在学术中心的报告率明显高于非学术中心。这些结果对于改善不同级别医疗机构的临床管理至关重要。
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引用次数: 0
A case of gastric granulosa cell tumor resected by endoscopic submucosal dissection. 一例通过内镜黏膜下剥离术切除的胃颗粒细胞瘤。
IF 1.4 4区 医学 Q4 GASTROENTEROLOGY & HEPATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-06-25 DOI: 10.1055/a-2321-1446
Shijie Lu, Chengyi Xu, Lian Zhang, Junan Li

Granular cell tumors, uncommon soft tissue growths, predominantly manifest in the subcutaneous and tongue areas, while those in the gastrointestinal tract are scarce and develop slowly. Patients typically show no distinct clinical symptoms and are hard to differentiate from gastrointestinal mesenchymal tumors, smooth muscle tumors, neural sheath tumors, and rhabdomyosarcomas using endoscopy. This paper details a case of a granular cell tumor in the stomach addressed through endoscopic submucosal dissection, focusing on its endoscopic attributes and clinicopathological traits.

颗粒细胞瘤是一种不常见的软组织增生,主要发生在皮下和舌头部位,而胃肠道中的颗粒细胞瘤很少且发展缓慢。患者通常没有明显的临床症状,而且很难通过内窥镜将其与胃肠间质瘤、平滑肌瘤、神经鞘瘤和横纹肌肉瘤区分开来。本文详细介绍了一例通过内镜黏膜下剥离术治疗的胃颗粒细胞瘤,重点是其内镜特征和临床病理特征。
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Zeitschrift fur Gastroenterologie
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