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Primary renal leiomyosarcoma in adult patients: a systematic review and individual patient data analysis 成年患者的原发性肾脏良性肉瘤:系统综述和个体患者数据分析
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2024-03-20 DOI: 10.1186/s12301-024-00418-1
Kannan Periasamy, Treshita Dey, Shikha Goyal, Renu Madan, Santosh Kumar, Sudheer Kumar Devana, Thiraviyam Elumalai, Prashanth Giridhar, Sushmita Ghoshal, Rakesh Kapoor, Chandan K. Das
The optimal management of primary renal leiomyosarcomas is unknown owing to its rarity and minimal available information about their primary, adjuvant treatment and clinical outcomes. This study systematically reviews treatment evidence and effects in terms of survival for leiomyosarcomas arising primarily from kidney, renal pelvis and renal vessels. PubMed and Embase databases were searched from inception to March 2023, with manual searches of reference lists. Two investigators independently reviewed the studies reporting management and survival outcomes of renal leiomyosarcomas. A total of 85 publications met inclusion criteria, reporting on 188 cases. The median age was 55.5 years, predominantly female [52.7%]. Pain was the most common presenting symptom [41.5%], and most tumors were high grade [45.8%]. Complete surgical resection with negative margins forms definitive treatment. The median disease-free survival and overall survival (OS) for all reviewed patients were 24 months [95%CI 4.1–43.9] and 42 months [95%CI 32.5–51.4], respectively. The OS of 1 year, 2 year, 3 year and 5 year was 78.8%, 64.4%, 53.8% and 38.9%, respectively. On univariate analysis, favorable factors for OS included tumor size ≤ 5 cm, low-grade histology, tumors of renal vascular origin and non-metastatic disease at presentation. Neoadjuvant or adjuvant treatment with either radiotherapy or chemotherapy has been shown to improve OS (NR vs. 36 months, p < 0.001), especially for high-grade tumors > 5 cm in size. Radical nephrectomy with en bloc tumor resection with negative margins forms the mainstay of treatment for renal leiomyosarcomas. Adjuvant radiotherapy or chemotherapy appears to improve OS. To validate this treatment strategy, prospective multicentric efforts are required to acquire reliable data from randomized trials.
原发性肾脏良性肌瘤的最佳治疗方法尚不清楚,因为这种肿瘤非常罕见,而且有关其原发性、辅助治疗和临床结果的可用信息极少。本研究系统回顾了主要发生于肾脏、肾盂和肾血管的肾脏细肌瘤的治疗证据和生存效果。研究人员检索了 PubMed 和 Embase 数据库中从开始到 2023 年 3 月的所有研究,并人工检索了参考文献列表。两名研究人员独立审阅了报告肾脏细肌瘤管理和生存结果的研究。共有 85 篇文献符合纳入标准,报告了 188 个病例。中位年龄为 55.5 岁,以女性为主[52.7%]。疼痛是最常见的症状[41.5%],大多数肿瘤为高级别[45.8%]。阴性边缘的完全手术切除是最终治疗方法。所有复查患者的中位无病生存期和总生存期(OS)分别为24个月[95%CI 4.1-43.9]和42个月[95%CI 32.5-51.4]。1年、2年、3年和5年的OS分别为78.8%、64.4%、53.8%和38.9%。单变量分析显示,肿瘤大小≤5厘米、低级别组织学、肾血管源性肿瘤和发病时非转移性疾病是OS的有利因素。放疗或化疗的新辅助治疗或辅助治疗已被证明可改善患者的生存期(5 厘米大小的肿瘤可改善患者的生存期(NR vs. 36 个月,P)。根治性肾切除术和阴性边缘肿瘤全切术是治疗肾脏细肌瘤的主要方法。辅助放疗或化疗似乎可以改善患者的生存期。为了验证这种治疗策略,需要开展前瞻性多中心工作,从随机试验中获取可靠数据。
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引用次数: 0
Feasibility of partial penectomy under local anesthesia: a case–control study 局部麻醉下阴茎部分切除术的可行性:病例对照研究
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2024-03-12 DOI: 10.1186/s12301-024-00416-3
Madhur Anand, Apul Goel, Bhupendra Pal Singh, Swati Aggarwal, Manoj Kumar, Vivek Kumar Singh, Vishwajeet Singh
To assess the feasibility of partial penectomy under local anesthesia and penile nerve blocks. A total of 45 patients underwent partial penectomy under local anesthesia over the last 15 years at our institute. These patients were included in group A. We took 45 age-matched controls for comparison in group B. All patients underwent partial penectomy with the standard technique. The control group underwent partial penectomy under general or spinal anesthesia, and the intervention group underwent partial penectomy under local anesthesia. Patients’ tolerance to anesthesia and surgery was compared, and postoperative pain and other complications were assessed. Mean age was 53.5 years in group A (case) and 52.8 years in group B (controls). Out of 45 patients in group A, 9 were ASA I, 16 were ASA II, 8 were ASA III, and 12 were ASA IV patients. Out of control patients, 35 underwent surgery under spinal and 10 underwent surgery under general anesthesia. All patients tolerated the anesthesia and surgery well. The duration of anesthesia and surgery was shorter in group A (p < 0.05). Postoperative pain scores between the two groups were comparable after 6 h. Postoperative recovery was comparable in both groups, and hospital stays were shorter in local anesthesia/nerve block group but were statistically insignificant. There was no positive margin in any group. Partial penectomy under local anesthesia is a satisfactory alternative in selected cases or with limited availability of anesthesia services.
评估在局部麻醉和阴茎神经阻滞下进行阴茎部分切除术的可行性。在过去的 15 年中,共有 45 名患者在我院接受了局部麻醉下的阴茎部分切除术。所有患者均采用标准技术进行了阴茎部分切除术。对照组在全身或脊髓麻醉下进行阴茎部分切除术,干预组在局部麻醉下进行阴茎部分切除术。比较了患者对麻醉和手术的耐受性,并对术后疼痛和其他并发症进行了评估。A组(病例)的平均年龄为53.5岁,B组(对照组)的平均年龄为52.8岁。A 组 45 名患者中,9 人为 ASA I 级,16 人为 ASA II 级,8 人为 ASA III 级,12 人为 ASA IV 级。在对照组患者中,35 人接受了脊髓麻醉手术,10 人接受了全身麻醉手术。所有患者都能很好地耐受麻醉和手术。A 组的麻醉和手术时间较短(P < 0.05)。两组术后恢复情况相当,局麻/神经阻滞组住院时间较短,但无统计学意义。各组均无阳性边缘。在局部麻醉下进行阴茎部分切除术是一种令人满意的替代方法,适用于部分病例或麻醉服务有限的病例。
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引用次数: 0
Prevalence of burnout among urologist and its risk factors in Indonesia 印度尼西亚泌尿科医生的职业倦怠及其风险因素
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2024-03-12 DOI: 10.1186/s12301-024-00417-2
Reginald Rustandi, Fakhri Rahman, Raga Manduaru, Chaidir Arif Mochtar
To evaluate burnout syndrome among Indonesian urologists by describing its prevalence and risk factors. This is a cross-sectional study conducted from February to April 2020. A validated Indonesian version of the Maslach Burnout Inventory that consists of 22 questions addressing emotional exhaustion, depersonalization, and personal achievement was utilized. The questionnaire was constructed using Google Forms (Google, Inc.) and was distributed via e-mail and WhatsApp Messenger (Facebook, Inc.). Burnout was defined as either high score on emotional exhaustion, or high score on depersonalization, or low score on personal achievement. Several variables were analyzed as risk factors to burnout using multivariate analysis. All statistical analyses were carried out using Statistical Software for Social Science (SPSS) version 23. From 486 eligible members of IUA, 184 (37%) respondents completed the questionnaire. Burnout syndrome was detected in 43 respondents (23.4%). From the results, most of the respondents have low EE (54.9%), low DP (77.7%), and moderate PA (45.7%). Bivariate analysis showed that age of less than 44 years old and being single were associated with increased risk of burnout with OR 2.2 (1.0–5.1, 95% CI, p-value 0.04) while being married decreases risk of burnout with OR 0.2 (0.1–0.8, 95% CI, p-value < 0.01). Lower work load in COVID-19–19 era was related to protective results with OR 0.4 (0.2–0.9, 95% CI, p-value 0.03). Multivariate analysis showed that being married decreases risk of burnout with OR 0.3 (0.1–0.9, 95% CI, p-value 0.04). Multivariate analysis showed that being married was the only significantly protective factor from burnout. Burnout syndrome among majority of urologists in Indonesia has a moderate degree of burnout, with being married the only significant factor influencing burnout in this study.
通过描述倦怠综合征的发生率和风险因素,评估印度尼西亚泌尿科医生的倦怠综合征。这是一项横断面研究,研究时间为 2020 年 2 月至 4 月。研究采用了经过验证的印尼版马斯拉奇职业倦怠量表,该量表由22个问题组成,涉及情绪衰竭、人格解体和个人成就感。问卷使用谷歌表格(谷歌公司)制作,并通过电子邮件和 WhatsApp Messenger(Facebook 公司)分发。职业倦怠的定义是情绪衰竭得分高,或人格解体得分高,或个人成就感得分低。我们使用多变量分析法对导致职业倦怠的几个风险因素进行了分析。所有统计分析均使用社会科学统计软件(SPSS)第 23 版进行。在 486 名符合条件的国际大学协会成员中,有 184 名(37%)受访者完成了问卷调查。有 43 名受访者(23.4%)发现了职业倦怠综合症。从结果来看,大多数受访者的 EE 值偏低(54.9%),DP 值偏低(77.7%),PA 值适中(45.7%)。双变量分析显示,年龄小于 44 岁和单身与倦怠风险增加有关,OR 值为 2.2(1.0-5.1,95% CI,p 值为 0.04),而已婚会降低倦怠风险,OR 值为 0.2(0.1-0.8,95% CI,p 值<0.01)。COVID-19-19 时代较低的工作负荷与保护性结果有关,OR 值为 0.4(0.2-0.9,95% CI,p 值为 0.03)。多变量分析表明,已婚会降低职业倦怠的风险,OR 值为 0.3(0.1-0.9,95% CI,p 值为 0.04)。多变量分析表明,已婚是唯一对职业倦怠有明显保护作用的因素。印尼大多数泌尿科医生的职业倦怠综合症属于中度倦怠,而已婚是本研究中影响职业倦怠的唯一重要因素。
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引用次数: 0
Human papillomavirus and bladder cancer: literature review and meta-analysis 人类乳头瘤病毒与膀胱癌:文献综述与荟萃分析
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2024-03-11 DOI: 10.1186/s12301-024-00414-5
Daria S. Dolgasheva, Marina K. Ibragimova, Matvey M. Tsyganov, Nikolai V. Litviakov
The aim of this study was to evaluate data over the past 25 years to assess the prevalence of the virus in patients with bladder cancer and to evaluate the association between human papillomavirus (HPV) and bladder cancer risk. Major databases were searched for published studies from October 1995 to May 2022. The present study evaluated the prevalence of HPV in patients with bladder cancer and the prevalence according to patients' ethnicity. The incidence of HPV in bladder cancer patients varies widely, ranging from complete absence of the virus in tissues to 64.6%. The meta-analysis was performed using Meta-Essentials_1.5 software. Begg’s and Egger’s methods were used to assess publication bias. Cochran’s Q test was used to assess heterogeneity and the I2 index was employed for calculating the variation in the pooled estimations. A weak association of HPV infection with the risk of bladder cancer was found. The risk of bladder cancer with HPV infection has clear ethnic characteristics and is statistically significant in Arabs and Asians.
本研究旨在评估过去 25 年来的数据,以评估病毒在膀胱癌患者中的流行情况,并评估人类乳头瘤病毒(HPV)与膀胱癌风险之间的关联。研究人员在主要数据库中搜索了 1995 年 10 月至 2022 年 5 月期间发表的研究。本研究评估了 HPV 在膀胱癌患者中的流行率以及与患者种族有关的流行率。HPV在膀胱癌患者中的发病率差异很大,从组织中完全没有病毒到64.6%不等。荟萃分析使用 Meta-Essentials_1.5 软件进行。采用 Begg's 和 Egger's 方法评估发表偏倚。Cochran's Q 检验用于评估异质性,I2 指数用于计算汇总估计值的差异。研究发现,HPV感染与膀胱癌风险的关系不大。HPV感染引发膀胱癌的风险具有明显的种族特征,在阿拉伯人和亚洲人中具有显著的统计学意义。
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引用次数: 0
The impact of uro-oncology multidisciplinary team meeting (MDTM) on clinical decision-making and adherence to MDTM recommendation: experience from a tertiary referral centre in Malaysia 泌尿肿瘤多学科小组会议(MDTM)对临床决策和遵守MDTM建议的影响:马来西亚一家三级转诊中心的经验
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2024-03-11 DOI: 10.1186/s12301-024-00415-4
Norshuhada Amat, Lim Jeffery, Chai C. Ann, Yeoh W. Sien, Ahmad N. Fadzli, Shanggar Kuppusamy, Marniza Saad, Ong T. Aik
Multidisciplinary team meeting (MDTM) has become an increasingly important part of disease management model, particularly in cancer care. MDTM consists of a group of doctors to provide independent opinions on diagnostic and treatment decisions, as well as personalized therapeutic plan for patients. By selecting the most suitable treatment for patients from multiple perspectives, management by multidisciplinary team (MDT) have been shown to have advantages over traditional treatment models. The objective of this study is to determine the impact of MDTM on the management of uro-oncological cases and adherence to MDTM plans. We retrospectively collected patients’ clinical information discussed in MDTM from 1st January 2021 to 31st December 2022 at our institution. The pre-MDTM treatment plan by the clinicians and the MDTM consensus plans were compared to assess the overall MDTM impact on patient management. Adherence to MDTM recommendations was also analyzed. Data on 432 patients discussed in MDTM from 1st January 2021 to 31st December 2022 were collected and analyzed. Prostate cancer was the most common type of cancer discussed (n = 212, 48.8%). MDTM had a significant impact on decision-making in 276 (63.6%) cases, with changes to patient management being observed in more than half of all cases. Adherence to MDTM outcomes was high with 383 (90.5%) of patients eventually had treatment according to the MDTM recommendation. The study highlights the importance of MDTM in the management of genitourinary malignancies, particularly in cases where no original plan exists. Patient’s compliance and adherence to the MDTM consensus plan are also very encouraging.
多学科团队会议(MDTM)已成为疾病管理模式中日益重要的一部分,尤其是在癌症护理方面。多学科团队会议由一组医生组成,就诊断和治疗决策提供独立意见,并为患者制定个性化治疗方案。与传统治疗模式相比,多学科团队(MDT)通过从多角度为患者选择最合适的治疗方法,已被证明具有优势。本研究旨在确定多学科团队管理对泌尿肿瘤病例管理的影响以及多学科团队管理计划的依从性。我们回顾性地收集了本院 2021 年 1 月 1 日至 2022 年 12 月 31 日期间在 MDTM 中讨论的患者临床信息。我们比较了临床医生在MDTM前的治疗计划和MDTM共识计划,以评估MDTM对患者管理的整体影响。此外,还分析了对 MDTM 建议的遵守情况。收集并分析了 2021 年 1 月 1 日至 2022 年 12 月 31 日期间在 MDTM 中讨论的 432 名患者的数据。前列腺癌是最常讨论的癌症类型(n = 212,48.8%)。在 276 例(63.6%)病例中,MDTM 对决策产生了重大影响,超过半数的病例观察到患者管理发生了变化。对 MDTM 结果的坚持率很高,383 例(90.5%)患者最终根据 MDTM 建议进行了治疗。这项研究强调了 MDTM 在泌尿生殖系统恶性肿瘤治疗中的重要性,尤其是在没有原始计划的情况下。患者对MDTM共识计划的依从性和坚持性也非常令人鼓舞。
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引用次数: 0
Retroperitoneal hernia following laparoscopic living-donor nephrectomy: a case report and review of literature 腹腔镜活体肾切除术后腹膜后疝:病例报告和文献综述
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2024-03-01 DOI: 10.1186/s12301-024-00411-8
Marzuki Panji Wijaya, Ahmad Zulfan Hendri
The laparoscopic approach is the standard of care for living-donor nephrectomy. A rare postoperative complication is small bowel obstruction due to a retroperitoneal hernia. We present a case of an incidental finding of a retroperitoneal hernia in a patient with a history of laparoscopic donor nephrectomy. An adult male presented with diffuse abdominal pain, vomiting, and obstipation for 12 h. He had undergone laparoscopic donor nephrectomy two months prior. Plain abdominal radiograph revealed a dilated small bowel with homogenous opacity in the left lumbar region. Due to worsening diffuse abdominal pain and a high clinical suspicion of intestinal strangulation and ischemia after conservative management, an exploratory laparotomy was performed. Intraoperatively, an incarcerated small bowel segment herniating through an 8 cm descending mesocolon defect into the retroperitoneal space was discovered, forming a closed-loop obstruction. The bowel segment was reduced, and the mesocolon defect was repaired. The patient was discharged five days postoperatively with good recovery. Retroperitoneal hernia following laparoscopic living-donor nephrectomy is a rare but significant complication. Mitigation to prevent mesenteric defect creation, routine inspection, and closure of the defect can reduce the risk of hernia. Non-specific abdominal pain in patients with a history of laparoscopic donor nephrectomy prompts rapid imaging evaluation to aid in the early diagnosis of possible retroperitoneal hernia and its intervention.
腹腔镜方法是活体肾切除术的标准护理方法。腹膜后疝导致小肠梗阻是一种罕见的术后并发症。我们介绍了一例偶然发现腹膜后疝的患者,该患者曾接受过腹腔镜供体肾切除术。一名成年男性因腹部弥漫性疼痛、呕吐和便秘 12 小时前来就诊。腹部平片显示小肠扩张,左腰部有同质不透明物。由于弥漫性腹痛加剧,且临床高度怀疑保守治疗后出现肠绞窄和缺血,因此进行了探查性开腹手术。术中发现,一段嵌顿的小肠通过 8 厘米长的降结肠系膜缺损疝入腹膜后间隙,形成闭环梗阻。医生缩小了肠段,并修复了结肠系膜缺损。患者术后五天康复出院。腹腔镜活体供肾切除术后腹膜后疝是一种罕见但严重的并发症。防止肠系膜缺损的措施、常规检查和闭合缺损可降低疝气的风险。有腹腔镜供体肾切除术病史的患者出现非特异性腹痛时,应迅速进行影像学评估,以帮助早期诊断可能存在的腹膜后疝并进行干预。
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引用次数: 0
Combined surgical and chemotherapy treatment for invasive primary urethral cancer: a case presentation 浸润性原发性尿道癌的手术和化疗联合治疗:一个病例展示
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2024-02-28 DOI: 10.1186/s12301-024-00413-6
Alper Simsek, Salih Butun, Mesut Berkan Duran, Kursat Kucuker, Sinan Celen, Yusuf Ozlulerden, Omer Levent Tuncay
The European Association of Urology (EAU) defines primary urethral carcinoma (PUC) as a carcinoma that arises in the urethra without a previous diagnosis of carcinoma elsewhere in the urinary system. It is considered as a rare cancer, accounting for less than 1% of all malignant tumors and 5% of malignant tumors of the urinary system. The difficulty in diagnosis and its rarity can lead to delayed diagnosis and decreased survival. We think that a case report to be made in the literature for this rare disease with no consensus on treatment will contribute to disease management. In this article, we describe the diagnosis and treatment process of a 75-year-old patient who was diagnosed with primary urethral cancer and had urethral discharge and difficulty in urination. The biopsy result from the suspected hyperemic area in the urethra in cystourethroscopy was primary urethral cancer. Urethrectomy followed by urethroplasty was performed on the patient's 2-cm primary urethral cancerous tissue. In the postoperative first month, an F-18 FDG whole-body PET scan for oncological evaluation showed increased pathological F-18 FDG uptake in the periphery of the mass in the penile urethra and indistinguishable boundaries from the prostatic urethra. After this, adjuvant gemcitabine and carboplatin therapy was planned by the oncology team. Based on our outcome in this case, we believe that chemotherapy combined with surgery increases the chance of successful treatment in locally advanced urethral cancer.
欧洲泌尿学协会(EAU)将原发性尿道癌(PUC)定义为尿道中出现的癌,且之前未确诊为泌尿系统其他部位的癌。它被认为是一种罕见癌症,在所有恶性肿瘤中占不到 1%,在泌尿系统恶性肿瘤中占不到 5%。其诊断困难和罕见性可能导致诊断延误和生存率下降。我们认为,在文献中对这种治疗方法尚未达成共识的罕见疾病进行病例报告,将有助于疾病的管理。本文描述了一位 75 岁患者的诊断和治疗过程,该患者被诊断为原发性尿道癌,并伴有尿道分泌物和排尿困难。在膀胱尿道镜检查中,尿道疑似充血区的活检结果为原发性尿道癌。对患者 2 厘米的原发性尿道癌组织进行了尿道切除和尿道成形术。术后第一个月,用于肿瘤评估的 F-18 FDG 全身 PET 扫描显示,阴茎尿道肿块外围的病理 F-18 FDG 摄取增加,与前列腺尿道的边界无法区分。之后,肿瘤科团队计划对患者进行吉西他滨和卡铂辅助治疗。根据本病例的治疗结果,我们认为化疗联合手术可增加局部晚期尿道癌的治疗成功率。
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引用次数: 0
Xanthogranulomatous pyelonephritis presenting as giant hydronephrosis in young women: a very rare case report 表现为年轻女性巨大肾积水的黄疽性肾盂肾炎:一份非常罕见的病例报告
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2024-02-16 DOI: 10.1186/s12301-023-00406-x
Babasaheb Dhakne, Venkat Arjunrao Gite
Xanthogranulomatous pyelonephritis (XGP) is common in middle-age female, involved kidney usually is hydronephrotic, non-functioning and associated with stones but giant hydronephrotic presentation is a very rare. We report a case of 25-year-old female presented as huge cystic abdominal lump involving left hemi-abdomen and crossing midline associated with pain. On radiological evaluation, she had giant left non-functioning hydronephrotic kidney pushing small and large bowel on right side for which she underwent open simple nephrectomy. To our surprise, her histopathology report was Xanthogranulomatous pyelonephritis. Only one case reported till date of Xanthogranulomatous pyelonephritis presented as giant hydronephrosis in adult. Xanthogranulomatous pyelonephritis is very rare cause of giant hydronephrosis with a varying clinical and radiological presentation and difficult to diagnose preoperatively. Diagnosis of XGP should be entertained in case long-standing gross hydronephrotic obstructed, infected kidney with stone disease.
黄疽性肾盂肾炎(XGP)常见于中年女性,受累肾脏通常为肾积水、无功能并伴有结石,但巨型肾积水非常罕见。我们报告了一例 25 岁女性的病例,她因腹部巨大囊性肿块而就诊,肿块累及左半腹并越过中线,伴有疼痛。经放射学评估,她的左侧巨大无功能肾积水肾脏推挤右侧小肠和大肠,为此她接受了开放性单纯肾切除术。令我们惊讶的是,她的组织病理报告是黄疽性肾盂肾炎。迄今为止,只有一例成人黄粒肿性肾盂肾炎表现为巨大肾积水。黄粒肿性肾盂肾炎是导致巨大肾积水的非常罕见的原因,其临床和影像学表现各不相同,很难在术前诊断出来。如果长期存在巨大肾积水梗阻、感染性肾脏和结石病,应考虑诊断为 XGP。
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引用次数: 0
IgG4-related retroperitoneal fibrosis mimicking renal pelvis tumor: a case report and literature review 模仿肾盂肿瘤的IgG4相关腹膜后纤维化:病例报告和文献综述
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2024-02-13 DOI: 10.1186/s12301-024-00408-3
Sahin Gokhan, Dundar Mehmet, Senturk Taskin
Retroperitoneal fibrosis (RPF) is a rare disease characterized by the development of a fibroinflammatory mass in the retroperitoneum. Immunoglobulin-G4 related RPF was suggested as a secondary form of RPF and thought to be part of the spectrum of Immunoglobulin-G4 related diseases (IgG4-RD). Patients often present to the clinic because of flank pain. Ranging from mild to end-stage renal failure can be observed. The main purpose of treatment is to preserve renal function. As it is a rare condition, there is no definite treatment strategy. We report a case of 39-year-old man with left flank pain and diagnosis of IgG4-related RPF mimicking a renal pelvis tumor. A 39-year-old male patient presented with left flank pain. MRI suggested solid retroperitoneal mass associated with hydronephrosis in the left kidney collecting system. Upon identifying the retroperitoneal origin of the mass during nephroureterectomy, the procedure was concluded following the acquisition of frozen section and routine pathological samples from the lesion. In the histopathological examination, inflammatory cells were observed and specific immunohistochemistry for IgG-4 was detected focally positive. Following the placement of a DJ stent, immunosuppressive therapy was initiated with Prednol and Azathioprine. After a one-year follow-up period, during which the patient received immunosuppressive treatment and underwent tri-monthly DJ stent replacements, the DJ stent was subsequently removed, revealing complete regression of hydronephrosis. With the correct diagnosis and treatment of IgG4-related RPF, it is possible to prevent irreversible complications of the disease. Because it is a rare disease, case reports in the literature will be useful for treatment.
腹膜后纤维化(RPF)是一种罕见疾病,其特征是腹膜后出现纤维炎性肿块。免疫球蛋白-G4相关的腹膜后纤维化被认为是腹膜后纤维化的一种继发形式,也被认为是免疫球蛋白-G4相关疾病(IgG4-RD)的一部分。患者通常因腹部疼痛而就诊。可观察到从轻度到终末期的肾衰竭。治疗的主要目的是保护肾功能。由于该病十分罕见,目前尚无明确的治疗策略。我们报告了一例 39 岁男性左侧腹痛病例,诊断为 IgG4 相关 RPF(模仿肾盂肿瘤)。一名 39 岁男性患者因左侧腹痛就诊。核磁共振成像提示腹膜后实性肿块伴有左肾集合系统肾积水。在肾切除术中确定肿块来源于腹膜后后,在获取病变部位的冰冻切片和常规病理样本后,手术结束。在组织病理学检查中,观察到炎性细胞,IgG-4 的特异性免疫组化检测呈灶性阳性。植入 DJ 支架后,开始使用泼尼松和硫唑嘌呤进行免疫抑制治疗。在为期一年的随访期间,患者接受了免疫抑制治疗,并每三个月更换一次 DJ 支架,随后拆除了 DJ 支架,发现肾积水完全消退。通过对 IgG4 相关 RPF 的正确诊断和治疗,有可能避免该病出现不可逆转的并发症。由于这是一种罕见疾病,文献中的病例报告将有助于治疗。
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引用次数: 0
Outcome and predictors of failure of abdominal surgical repair of high vesico-vaginal and vesico-uterine fistulae at Gezira Hospital for Renal Disease and Surgery 杰济拉肾病和外科医院腹部手术修补高位膀胱阴道瘘和膀胱子宫瘘失败的结果和预测因素
IF 0.5 Q4 UROLOGY & NEPHROLOGY Pub Date : 2024-01-23 DOI: 10.1186/s12301-024-00409-2
Muzafr Shakir Ali Yousif, Ismail Gareeballah Alhag Mohamad, Mohamed Elimam Mohamed Ahmed, Yassin Mohammed Osman, Ahmed Shakir Ali Yousif, Mustafa Omran
Urogenital fistula can arise from various causes, leading to the development of diverse surgical procedures. The prevention and treatment of obstetric fistula continue to pose challenges in low-income countries. To assess the outcomes and predictors of failure of surgical repair for urogenital fistulas, specifically high vesico-vaginal fistula (VVF) and vesico-uterine fistula (VUF), within our context. Conducted a prospective hospital-based study involving 100 female patients with urogenital fistula (95 VVF and 5 VUF) who underwent abdominal surgical repair at Gezira Hospital for Renal Diseases and Surgery from 2018 to 2023. Collected data encompassing demographics, obstetric history, fistula etiologies, Swab test, cystoscopy findings, urine diversion, ureteric re-implantation, and repair outcomes. The majority of women were aged 20–29 years (39%), illiterate (62%), and had a low socio-economic status (87%). Lack of antenatal care was noted in 77% of patients. Fistula etiologies were predominantly obstetric (70%), mainly due to spontaneous vaginal delivery (SVD), with the remaining 30% attributed to gynecological causes (hysterectomy). In terms of fistula characteristics, all patients had a high-level fistula, 95% had a single fistula, and 69% had a posterior wall fistula. Successful closure was achieved in 84% of cases, with 11% experiencing ureteric involvements. The analysis of failures pointed to recurrent fistulae (50%), larger fistula size (31%), and the presence of multiple fistulae (19%) as notable predictors of unsuccessful repair. VVF was the prevalent type of urogenital fistula in our population. Risk factors included being in the third decade of life, illiteracy, low socioeconomic status, and a lack of prenatal care. Obstetric causes, particularly prolonged and obstructed labor through SVD, dominated the etiology. Surgical procedures resulted in successful closure in 84% of cases. Recurrent fistulae, larger size, and multiple occurrences emerged as predictors of surgical repair failure.
泌尿生殖系统瘘管病的病因多种多样,导致了各种外科手术的发展。在低收入国家,产科瘘管病的预防和治疗仍是一项挑战。在我国范围内评估泌尿生殖道瘘,特别是高位膀胱阴道瘘(VVF)和膀胱子宫瘘(VUF)手术修复失败的结果和预测因素。开展了一项基于医院的前瞻性研究,涉及 2018 年至 2023 年期间在杰济拉肾病和外科医院接受腹部手术修复的 100 名女性泌尿生殖道瘘患者(95 名 VVF 和 5 名 VUF)。收集的数据包括人口统计学、产科病史、瘘管病因、Swab 测试、膀胱镜检查结果、尿液分流、输尿管再植和修复结果。大多数妇女的年龄在 20-29 岁之间(39%),文盲(62%),社会经济地位较低(87%)。77%的患者缺乏产前护理。瘘管的病因主要是产科(70%),主要是阴道自然分娩(SVD),其余 30% 的病因是妇科(子宫切除术)。就瘘管的特征而言,所有患者都有高位瘘管,95%为单发瘘管,69%为后壁瘘管。84%的病例成功闭合,11%的病例输尿管受累。对失败病例的分析表明,复发性瘘管(50%)、瘘管较大(31%)和存在多个瘘管(19%)是导致修复失败的显著预测因素。VVF是我国人群中最常见的泌尿生殖道瘘类型。高危因素包括年过三旬、文盲、社会经济地位低下以及缺乏产前护理。产科病因,尤其是通过SVD导致的产程延长和难产,在病因学中占主导地位。84%的病例通过手术成功关闭了瘘管。复发性瘘管、较大的瘘管和多次瘘管是手术修复失败的预测因素。
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African Journal of Urology
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