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VIPoma: An Unusual Cause of Chronic Diarrhea. VIPoma:慢性腹泻的不寻常原因
Pub Date : 2024-01-01 DOI: 10.14712/18059694.2024.22
Sutharin Suteetorn, Krit Kitisin, Natcha Wanpiyarat, Supaksorn Kunjan, Thiti Snabboon

Chronic diarrhea is a significant challenge in clinical practice because of its high prevalence and various causes. Comprehensive clinical assessment and stepwise laboratory approach are crucial for an accurate diagnosis. This report presents a case of an adult woman who experienced chronic watery diarrhea, complicated by renal impairment and multiple electrolyte imbalances, including hypokalemia, hypophosphatemia, and metabolic acidosis. The diagnosis of a vasoactive intestinal polypeptide-secreting tumor (VIPoma) with liver metastases was confirmed by elevated serum levels of a vasoactive intestinal polypeptide (VIP) and imaging findings of a pancreatic mass with multiple hepatic lesions. Preoperative management, including fluid rehydration, electrolyte correction, and somatostatin analog therapy, significantly improved her clinical symptoms. Subsequent surgical tumor removal and radiofrequency ablation of the hepatic lesions resulted in complete resolution of symptoms and normalized VIP levels. This case emphasizes the importance of early recognition of this rare tumor in patients with chronic diarrhea to improve clinical outcomes.

慢性腹泻发病率高,病因多样,是临床实践中的一大难题。全面的临床评估和循序渐进的实验室方法是准确诊断的关键。本报告介绍了一例成年女性慢性水样腹泻病例,该病例并发肾功能损害和多种电解质失衡,包括低钾血症、低磷血症和代谢性酸中毒。血清中血管活性肠多肽(VIP)水平升高,影像学检查发现胰腺肿块伴有多处肝脏病变,确诊为血管活性肠多肽分泌性肿瘤(VIP瘤)伴肝脏转移。术前处理,包括液体补液、电解质纠正和体泌素类似物治疗,明显改善了她的临床症状。随后通过手术切除肿瘤并对肝脏病灶进行射频消融,症状完全缓解,VIP 水平恢复正常。本病例强调了在慢性腹泻患者中早期识别这种罕见肿瘤以改善临床疗效的重要性。
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引用次数: 0
P(A)SH Syndrome: Case Presentation and Short Update of Related Disorders. P(A)SH综合征:病例介绍和相关疾病的简短更新。
Pub Date : 2024-01-01 DOI: 10.14712/18059694.2025.9
Simona Kordeva, Alice Hristova, Valentina Broshtilova, Georgi Tchernev

Hidradenitis suppurativa (HS) is a chronic inflammatory disease that is frequently associated with syndromes, such as those within the PAPA spectrum. Syndromic HS presents unique management challenges, as it often shows resistance to conventional therapies. Pyoderma gangrenosum is a rare inflammatory neutrophilic dermatosis that is often seen in association within the spectrum of autoinflammatory diseases. The PAPA spectrum disorders include a group of autoinflammatory diseases characterized by mutations in the PSTPIP1 gene or by clinical manifestations that closely resemble or overlap with those of PAPA syndrome. Each syndrome (PASH, PAPASH, PsAPASH, PASS, PAC, and PAMI syndrome) in this spectrum highlights specific inflammatory pathways and symptoms, providing insight into targeted therapeutic approaches. Here, we present a rare case of incomplete PASH (pyoderma gangrenosum and hidradenitis suppurativa) syndrome successfully managed with a standard combination of antibiotics (ceftriaxone and metronidazole) and corticosteroids (methylprednisolone), followed by immunosuppressant (azathioprine) and corticosteroids (dexamethasone). We review both novel and established/standard treatment options, with an emphasis on treatment outcomes. Conventional therapies remain both effective and affordable, providing valuable alternatives for patients.

化脓性汗腺炎(HS)是一种慢性炎症性疾病,通常与综合征相关,如PAPA谱内的综合征。综合征型HS提出了独特的管理挑战,因为它往往表现出对常规治疗的耐药性。坏疽性脓皮病是一种罕见的炎症性中性粒细胞性皮肤病,通常与自身炎症性疾病有关。PAPA谱系障碍包括一组以PSTPIP1基因突变或临床表现与PAPA综合征非常相似或重叠为特征的自身炎症性疾病。该谱中的每种综合征(PASH、PAPASH、PsAPASH、PASS、PAC和PAMI综合征)都突出了特定的炎症途径和症状,为有针对性的治疗方法提供了见解。在这里,我们报告了一例罕见的不完全PASH(坏疽性脓皮病和化脓性汗腺炎)综合征,通过抗生素(头孢曲松和甲硝唑)和皮质类固醇(甲基强的松龙)的标准组合成功治疗,然后是免疫抑制剂(硫唑嘌呤)和皮质类固醇(地塞米松)。我们回顾了新的和建立/标准的治疗方案,重点是治疗结果。传统疗法仍然有效且负担得起,为患者提供了有价值的替代疗法。
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引用次数: 0
Imaging Findings of Solitary Fibrous Tumors of the Gallbladder. 胆囊孤立性纤维性肿瘤的影像学表现。
Pub Date : 2024-01-01 DOI: 10.14712/18059694.2025.5
Trinh Anh Tuan, Le-Thi Mai Huong, Nguyen Thu Minh Chau, Ngo Quang Duy, Nguyen-Thi Hai Anh, Nguyen Duy Hung, Luc Ceugnart, Nguyen Minh Duc

Solitary Fibrous Tumor (SFT) is a rare mesenchymal tumor with a higher incidence of benign than malignant, most common location in the pleura. Although this tumor has been found in other locations in the body such as the head and neck region, retroperitoneal space, and intra-abdominal omentum, SFT of the gallbladder remains extremely rare in the medical literature. In this article, we present the imaging characteristics of Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) of gallbladder SFT, thereby contributing to providing information in the study of this rare pathology.

孤立性纤维瘤(SFT)是一种罕见的间质肿瘤,良性发生率高于恶性发生率,最常见于胸膜。虽然这种肿瘤在身体的其他部位如头颈部、腹膜后间隙和腹内网膜也有发现,但胆囊的SFT在医学文献中仍然极为罕见。在本文中,我们介绍了胆囊SFT的计算机断层扫描(CT)和磁共振成像(MRI)的成像特征,从而有助于为研究这种罕见的病理提供信息。
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引用次数: 0
Academic Responsibilities, Obligations, and Freedom: A Call for Review - In Memory of Professor Dr. Tomáš Hudlický. 学术责任、义务和自由:呼吁回顾--纪念 Tomáš Hudlický 教授博士。
Pub Date : 2024-01-01 DOI: 10.14712/18059694.2024.17
Consolato M Sergi

The advent and dominance of social media in our daily lives is not a matter of discussion, and very few minimalistic individuals have tried to decrease this technological dependency, which can become toxic and noxious for the development of an autonomous personality and free thinking. Academic faculties claim a depauperation in terms of their freedom but are also not free from duties, responsibilities, and obligations. Here, duties, responsibilities, obligations, and freedom are addressed in historical terms as the university as an institution developed over the centuries after its founding in the 11th century is currently under attack. We premonish that these concepts must still be reiterated and divulgated to students and fellows in academia. Galilei's "Eppur si muove" ("and yet it moves") are the words pronounced by the Italian mathematician, physicist, and philosopher Galileo Galilei that should resonate in censorship bodies now and in the future.

社交媒体在我们日常生活中的出现和主导地位并不是一个值得讨论的问题,极少有极简主义者试图减少这种技术依赖,因为这种依赖会对自主人格和自由思考的发展产生毒害。学术界声称他们的自由是一种解脱,但也无法摆脱责任、职责和义务。在此,我们将从历史的角度来探讨责任、职责、义务和自由,因为大学作为一个自 11 世纪创建以来历经数百年发展起来的机构,目前正受到冲击。我们预感到,这些概念仍然必须向学术界的学生和研究人员重申和传播。伽利略的 "Eppur si muove"("然而它在移动")是意大利数学家、物理学家和哲学家伽利略-伽利莱(Galileo Galilei)的名言,现在和将来都应在审查机构中引起共鸣。
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引用次数: 0
Visual Acuity Screening of Refugees and Immigrants with a Web-Based Digital Test: A Pilot Study. 基于网络的数字测试对难民和移民的视力筛查:一项试点研究。
Pub Date : 2024-01-01 DOI: 10.14712/18059694.2025.2
Minas Bakirtzis, Eirini Michaleakou, Maria-Eleni Martidou, Eleni Lahana, Petros Kostagiolas, Dimitris Niakas, Georgios Labiris

Purpose: To screen visual acuity in two refugee camps in Greece and explore the feasibility of replicating these methods on a nationwide scale.

Methods: Visual acuity was assessed in all participants using web-based Democritus Digital Acuity & Reading Test (DDART). Furthermore, the immigrants responded to a structured questionnaire regarding their demographics and medical history.

Results: A total of 330 adult refugees and immigrants were recruited. A total of 47.3% of the patients had never undergone ophthalmological examination. A significant negative correlation was detected between age (r = -0.207, p < 0.001) and educational background (r = -0.135, p = 0.014), suggesting that younger immigrants who had attended compulsory education were more likely to have their eyes checked in their home country. A total of 6.97% of patients presented with impaired vision and were referred for further care. All remote DDART measurements presented no differences from the corresponding hospital-based data in the referred cases.

Conclusions: Visual acuity screening using DDART provides valuable information regarding the visual capacity of refugees. The study outcomes suggest that pilot methods can be replicated on a nationwide scale.

Clinical trials: Gov number NCT05209581; date of registration: January 13, 2022. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. The authors have no funding or conflicts of interest to disclose. Patients Consent Statement: The patients sign written consent form.

目的:对希腊两个难民营的视力进行筛查,探讨在全国范围内复制这些方法的可行性。方法:使用基于web的Democritus Digital acuity对所有参与者的视力进行评估;阅读测试(dart)。此外,移民还回答了一份关于他们的人口统计和病史的结构化问卷。结果:共招募了330名成年难民和移民。47.3%的患者从未做过眼科检查。年龄(r = -0.207, p < 0.001)与教育背景(r = -0.135, p = 0.014)之间呈显著负相关,表明接受过义务教育的年轻移民更有可能在原籍国接受眼科检查。共有6.97%的患者出现视力受损,并被转介进一步治疗。在转诊病例中,所有远程dart测量结果与相应的基于医院的数据没有差异。结论:使用dart进行视力筛查提供了有关难民视力的宝贵信息。研究结果表明,试点方法可以在全国范围内复制。临床试验:政府编号NCT05209581;注册日期:2022年1月13日。这项研究没有从公共、商业或非营利部门的资助机构获得任何具体的资助。作者没有资金或利益冲突需要披露。患者同意声明:患者签署书面同意书。
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引用次数: 0
Atypical Manifestation of X-linked Agammaglobulinemia - the Importance of Genetic Testing. X 连锁阿加球蛋白血症的非典型表现--基因检测的重要性。
Pub Date : 2024-01-01 DOI: 10.14712/18059694.2024.21
Adam Markocsy, Daniela Kapustová, Andrej Čereš, Eva Froňkova, Miloš Jeseňák

X-linked agammaglobulinemia (XLA) was one of the first inborn errors of immunity to be described. It is caused by pathogenic variants in the gene for Bruton tyrosine kinase (BTK), which has important functions in B cell development and maturation. Recurrent bacterial infections in the first two years of life and hypogammaglobulinemia with absent B cells in male patients are the most common symptoms. A four-month-old male patient underwent surgical removal of urachus persistens complicated with recurrent scar abscesses. Hypogammaglobulinemia (IgG, IgA, and IgM), low phagocytic activity, mild neutropenia, and a normal percentage of B cells were observed in the patient's immune laboratory profile. Over time, he suffered recurrent respiratory infections (otitis media and rhinosinusitis) and developed B cell depletion, but interestingly, this was with a normalisation of IgG and IgA levels along with undetectable IgM. Molecular-genetic testing confirmed the presence of the pathogenic variant c.1843C>T in the BTK gene, which is associated with a milder phenotype of XLA. Molecular-genetic testing uncovers the variability of clinical and laboratory features of apparently well-known inherited disorders. Patients with mild "leaky" XLA may have normal levels of non-functional or oligoclonal immunoglobulins.

X 连锁丙种球蛋白血症(XLA)是最早被描述的先天性免疫错误之一。它是由布鲁顿酪氨酸激酶(BTK)基因的致病变体引起的,该基因在 B 细胞发育和成熟过程中具有重要功能。最常见的症状是男性患者在出生后头两年内反复出现细菌感染,以及低丙种球蛋白血症伴 B 细胞缺失。一名四个月大的男性患者接受了尿道持续存在并发复发性疤痕脓肿的手术切除。在患者的免疫实验室检查中发现了低丙种球蛋白血症(IgG、IgA 和 IgM)、低吞噬活性、轻度中性粒细胞减少以及正常比例的 B 细胞。随着时间的推移,他出现了反复呼吸道感染(中耳炎和鼻炎),并出现了 B 细胞耗竭,但有趣的是,IgG 和 IgA 水平趋于正常,IgM 也检测不到。分子遗传学检测证实了 BTK 基因中存在致病变体 c.1843C>T,该变体与较轻的 XLA 表型有关。分子遗传学检测揭示了表面上众所周知的遗传性疾病的临床和实验室特征的变异性。轻度 "漏型 "XLA 患者的非功能性或少克隆免疫球蛋白水平可能正常。
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引用次数: 0
Zirconia Ceramics. 氧化锆陶瓷。
Pub Date : 2024-01-01 DOI: 10.14712/18059694.2024.18
Bedirhan Savas Yigit, Marwan Al-Akkad, Radek Mounajjed

Zirconia ceramics have become popular among other dental ceramics thanks to their biological, mechanical, optical, and aesthetic properties. CAD/CAM (computer-aided design/ computer-aided manufacturing) technology improvement has played a vital role in the increased popularity of zirconia ceramics; easy computer manipulation significantly expanded the possibility of using different types of restorations. Zirconia ceramics have a broad spectrum of indications in prosthetic dentistry, from simple restorations to complex structures supported by dental implants. A good orientation in the classification, features, and manipulation of zirconia ceramics is the main key to success.

氧化锆陶瓷具有生物、机械、光学和美学特性,因此在其他牙科陶瓷中颇受欢迎。CAD/CAM(计算机辅助设计/计算机辅助制造)技术的改进对氧化锆陶瓷的普及起到了至关重要的作用;简便的计算机操作大大扩展了使用不同类型修复体的可能性。氧化锆陶瓷在牙科修复中具有广泛的适应症,从简单的修复体到由牙科种植体支撑的复杂结构。掌握氧化锆陶瓷的分类、特点和操作方法是成功的关键。
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引用次数: 0
Sensorineural Hearing Loss in Otosclerosis Surgery. 耳硬化症手术中的感音神经性听力损失。
Pub Date : 2024-01-01 DOI: 10.14712/18059694.2025.1
Kristýna Néma, Viktor Chrobok, Jan Mejzlík, Vladimír Pavlík, Lukáš Školoudík

Background: During otosclerosis surgery, operative trauma can lead to decreased bone conduction.

Aims: The study aims to observe the bone conduction changes after otosclerosis operations and analyse possible factors affecting the postoperative decrease in bone conduction.

Material and methods: Authors retrospectively processed the data of 109 patients and evaluated pure tone audiometry before surgery and consequently 2 days, 1 month and 1 year after surgery.

Results: We noted a deterioration of bone conduction >5 dB on the second postoperative day in 28% (30/109) of patients, which persisted one year after the surgery in 9% (10/109) cases. Analysis of individual factors affecting bone conduction loss revealed a higher risk of permanent loss of bone conduction in patients with early postoperative loss in higher frequencies, in older patients and patients with a preoperative threshold of bone conduction >20 dB. Revision surgery was not a statistically significant factor.

Conclusion and significance: The bone conduction decrease after otosclerosis surgery is usually temporary. The recovery of bone conduction is influenced by the age of patients and the level of bone conduction before the surgery. The early postoperative decrease of bone conduction in higher frequencies is a negative predictive factor for permanent hearing loss.

背景:在耳硬化手术中,手术创伤可导致骨传导减少。目的:观察耳硬化术后骨传导的变化,分析影响术后骨传导降低的可能因素。材料与方法:对109例患者术前、术后2天、1个月、1年的纯音听力进行回顾性分析。结果:我们注意到28%(30/109)的患者术后第二天骨传导bbb50 dB恶化,9%(10/109)的患者术后1年持续恶化。对影响骨传导丧失的个体因素的分析显示,术后早期高频骨传导丧失患者、老年患者和术前骨传导阈值为bbb20 dB的患者发生永久性骨传导丧失的风险更高。翻修手术并无统计学意义。结论及意义:耳硬化术后骨传导降低通常是暂时性的。骨传导恢复受患者年龄和术前骨传导水平的影响。术后早期高频骨传导减少是永久性听力损失的负面预测因素。
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引用次数: 0
Colloid Cyst of the Third Ventricle: A Case Report. 第三脑室胶体囊肿:病例报告
Pub Date : 2024-01-01 DOI: 10.14712/18059694.2025.4
Aleš Kopal, Jiří Preis, Leoš Ungermann, Edvard Ehler, Ivana Štětkářová

Colloid cyst of the third ventricle (CC) represents approximately 1% of intracranial tumours and 20% of intraventricular tumours. CC usually occurs between 20 and 50 years of age. During the first decade of life, it is diagnosed very rarely (1-2%). It can be most commonly found in the anterior part of the third ventricle at the foramen of Monro (1). It is often visualised during the computed tomography (CT) examination as a hyperdense focal lesion, it has variable change of the signal during magnetic resonance imaging (MRI) (2). CC has a benign character, however, a strategic position which may lead to acute hydrocephalus, intracranial hypertension syndrome, consciousness disorder, and even sudden death. This peracute hydrocephalus is an indication to an acute neurosurgical procedure (3).

第三脑室胶质囊肿约占颅内肿瘤的1%和脑室内肿瘤的20%。CC通常发生在20至50岁之间。在生命的头十年中,很少被诊断出来(1-2%)。最常见于第三脑室前部的Monro孔处(1)。CT检查时常表现为高密度局灶性病变,磁共振成像(MRI)时信号变化多端(2)。CC为良性,但其战略性位置可导致急性脑积水、颅内高压综合征、意识障碍,甚至猝死。过急性脑积水是急性神经外科手术的指征(3)。
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引用次数: 0
Two World Wars and Belgium: Missing Births and Birth Sex Ratio Changes. 两次世界大战与比利时:缺失出生和出生性别比变化。
Pub Date : 2024-01-01 DOI: 10.14712/18059694.2024.15
Victor Grech, Tania Borg

Introduction: The sex ratio at birth (male/total births, M/T) is expected to approximate 0.515. M/T is influenced by many factors including stress. Both World Wars have been implicated as influencing birth rates and M/T. This study was carried out to analyse the effects of two World Wars on Belgium vis-à-vis missing births as well as M/T changes.

Methods: Belgian male and female births were available for 1830-2019 and annual population was available from Statista. ARIMA models were used to estimate and project birth losses. The effect of wars was assumed to begin in the years following the commencement of each war and extend to the year after cessation of hostilities i.e., 1915-1919 and 1940-1946 for the First and Second World Wars respectively.

Results: This study included 27,346,178 live births for 1830-2019, M/T 0.5124. There was a decreasing trend in births for 1830-2019, significant for 1950-2019. There were dips in births in association with both Wars resulting in over 440,000 missing births, 3.80% of the Belgian population for the First World War and 1.91% for the Second World War. M/T rose non-significantly for the First World War and significantly for the Second World War.

Discussion: The declining birth rate and M/T in developed countries is a recognised phenomenon. The missing births in relation to wars are of demographic importance but are often overlooked with emphasis usually on casualties and deaths. M/T may rise in wars, possibly due to increased coital activity as well as other factors.

导言:出生性别比(男性/总出生人数,M/T)预计约为 0.515。M/T 受许多因素的影响,包括压力。两次世界大战都被认为影响了出生率和 M/T。本研究旨在分析两次世界大战对比利时出生率和 M/T 变化的影响:方法:比利时男性和女性出生人数可追溯到 1830-2019 年,年度人口数可从 Statista 上获得。使用 ARIMA 模型来估计和预测出生损失。假定战争的影响始于每场战争开始后的几年,并延续到敌对行动停止后的一年,即第一次和第二次世界大战分别为 1915 年至 1919 年和 1940 年至 1946 年:这项研究包括 1830-2019 年的 27,346,178 例活产,M/T 为 0.5124。1830-2019 年的出生人数呈下降趋势,1950-2019 年的下降趋势显著。两次大战期间的出生人数均出现下降,导致超过 44 万名出生人口失踪,其中第一次世界大战期间占比利时人口的 3.80%,第二次世界大战期间占比利时人口的 1.91%。在第一次世界大战中,出生率/出生率上升不明显,而在第二次世界大战中,出生率/出生率上升明显:讨论:发达国家出生率和 M/T 下降是一个公认的现象。与战争有关的出生缺失具有重要的人口学意义,但往往被忽视,人们通常把重点放在伤亡和死亡上。在战争中,M/T 可能会上升,这可能是由于同居活动增加以及其他因素造成的。
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引用次数: 0
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Acta medica (Hradec Kralove)
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