Xanthogranulomatous

黄色肉芽肿
  • 文章类型: Case Reports
    及时诊断黄色肉芽肿性肾盂肾炎(XGP),一种罕见的慢性肾病,连同其适当的管理,是使肾脏免于终末期肾病(ESRD)的必要条件。大多数疾病早期诊断的主要障碍,包括XGP,是没有特定和特征性的症状,which,如果存在,会使患者更早地寻求医疗援助,并诱使临床医生考虑可变的鉴别诊断。我们在此报告一例20岁的男性患者,没有特定的症状提示肾脏病理,这耽误了他考虑咨询医疗保健专业人员,因此,在他的受累肾脏几乎无法挽救时,他的病情被诊断为XGP。通过这个案例报告,我们希望谦卑地要求世界各地的临床医生在处理非特异性症状的患者时,扩大他们的鉴别诊断范围,以便有更好的预后。
    The timely diagnosis of xanthogranulomatous pyelonephritis (XGP), a rare and chronic kidney condition, along with its appropriate management, is a must to spare the kidney from end-stage renal disease (ESRD). The main hurdle in early diagnosis of most medical conditions, including XGP, is the absence of specific and characteristic symptoms, which, if present, would make the patient seek medical aid earlier and tempt the clinician to think of variable differential diagnosis. We hereby report a case of a 20-year-old male patient who had no specific symptoms suggestive of a renal pathology, which delayed him from considering consulting a healthcare professional, thereby making his condition diagnosed as XGP at a time when his involved kidney was hardly salvageable. Through this case report, we wish to humbly request clinicians all across the globe to kindly broaden their range of differential diagnoses while dealing with patients with nonspecific symptomatology, in order to have a better prognosis.
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  • 文章类型: Case Reports
    我们报告了一名患者,该患者最初因诊断为Erdheim-Chester病(ECD)的视力丧失而就诊于眼科诊所。
    ECD是一种罕见的非朗格汉斯细胞组织细胞增生症,其特征是多系统器官受累和预后不良。在出现任何全身症状之前,我们的患者由于突出的眼眶受累而完全视力丧失。该病例表现出不同的ECD临床表现。
    对类固醇治疗反应差的无痛性双侧眼球突出应提示考虑ECD和系统评估。此外,在没有典型临床表现的情况下,对活检的全面评估对于准确诊断至关重要。
    UNASSIGNED: We report a patient who initially visited the ophthalmology clinic for a vision loss diagnosed with Erdheim-Chester Disease (ECD).
    UNASSIGNED: ECD is a rare non-Langerhans cell histiocytosis characterized by multisystemic organ involvement and poor prognosis. Our patient had complete vision loss due to prominent orbital involvement before any systemic symptoms appeared. This case demonstrates variable clinical manifestations of ECD.
    UNASSIGNED: Painless bilateral proptosis with poor response to steroid treatment should prompt consideration for ECD and systemic evaluation. In addition, in the absence of typical clinical manifestations, a thorough evaluation of the biopsy can be crucial for an accurate diagnosis.
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  • 文章类型: Case Reports
    垂体炎是一种极其罕见的炎症性疾病,可以模仿垂体腺瘤的临床和放射学特征。在这个案例报告中,我们描述了一名45岁女性,患有继发性黄色肉芽肿性垂体炎(XGH),表现为垂体大腺瘤.病人抱怨头痛,视力障碍,闭经-溢乳综合征.体检正常。实验室调查显示促肾上腺皮质激素,促甲状腺激素,和促性腺激素缺乏。她的右眼视力也很低,视野也改变了。垂体磁共振成像显示鞍内和鞍上肿块13×11×16mm,伴有出血性坏死,这对患者的视交叉和垂体柄有离散的质量影响。患者接受了氢化可的松和左甲状腺素治疗,然后转到神经外科进行全蝶窦切除。肿瘤的组织学检查允许对重塑的Rathke囊囊肿进行XGH诊断。系统性疾病,如结核病,结节病,排除其他肉芽肿性疾病。XGH的病因仍然不明确,但它可能是淋巴细胞性垂体炎的进行性形式或重塑的Rathke囊囊肿。筛查自身免疫性病理学和全身性疾病对于指导适当的管理至关重要。
    Hypophysitis is an extremely rare inflammatory disease that can mimic the clinical and radiological features of a pituitary adenoma. In this case report, we describe a 45-year-old woman with secondary xanthogranulomatous hypophysitis (XGH) who presented with signs of a pituitary macroadenoma. The patient complained of headaches, visual impairment, and amenorrhea-galactorrhea syndrome. Her physical examination was normal. Laboratory investigation revealed corticotropin, thyrotropin, and gonadotropin deficiencies. She also had low visual acuity in her right eye and an altered visual field. Pituitary magnetic resonance imaging revealed an intra and suprasellar mass measuring 13 × 11 × 16 mm, with hemorrhagic necrosis, that was having a discrete mass effect on the patient\'s optic chiasm and pituitary stalk. The patient was treated with hydrocortisone and levothyroxine, and then transferred to the Neurosurgery department for total transsphenoidal resection of the mass. Histological examination of the tumor permitted a diagnosis of XGH of a remodeled Rathke\'s pouch cyst to be made. Systemic conditions such as tuberculosis, sarcoidosis, and other granulomatous diseases were excluded. The etiopathogenesis of XGH remains poorly characterized, but it may be a progressive form of lymphocytic hypophysitis or a remodeled Rathke\'s pouch cyst. Screening for autoimmune pathology and systemic diseases is essential to guide appropriate management.
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  • 文章类型: Journal Article
    OBJECTIVE: To present the results of our case series on laparoscopic nephrectomy in xanthogranulomatous pyelonephritis (XGP).
    METHODS: A retrospective study was conducted that included 143 patients treated with laparoscopic nephrectomy for non-functioning kidney, of whom 15 had XGP, within the time frame of 2011 to 2019. The demographic and clinical data were collected, along with the intraoperative results, complications, and days of hospital stay.
    RESULTS: Transperitoneal laparoscopic nephrectomy was successfully performed on 15 patients with XGP, with no need for conversion. Mean intraoperative time was 124.4 minutes (range 70-240) and intraoperative blood loss was 148.5 ml (range 30-550), with no blood transfusion required. No intraoperative complications occurred but there was one postoperative complication (6.6%), classified as Clavien-Dindo I (surgical wound infection). Mean hospital stay was 2.85 days (range 2-7).
    CONCLUSIONS: Nephrectomy is the definitive management for XGP, and the laparoscopic approach should be considered a treatment modality, despite the fact that the pathology involves a severe chronic inflammatory process. Its benefits are reduced surgery duration, less blood loss, a lower complication rate, and fewer days of hospital stay, when performed by a skilled and experienced surgeon.
    OBJECTIVE: Presentar los resultados de nuestra serie de nefrectomía laparoscópica en pielonefritis xantogranulomatosa (PXG).
    UNASSIGNED: Se realizó un estudio retrospectivo que incluyó 143 pacientes tratados con nefrectomía laparoscópica por exclusión renal, de los cuales 15 fueron por PXG, en el periodo comprendido de 2011 a 2019. Se recolectaron datos demográficos y clínicos, resultados transoperatorios, complicaciones y días de estancia hospitalaria.
    RESULTS: Se realizó nefrectomía laparoscópica transperitoneal de forma exitosa en 15 pacientes con PXG, sin necesidad de conversión. El tiempo transoperatorio promedio fue de 124.4 minutos (rango: 70-240). El sangrado transoperatorio fue de 148.5 ml (rango: 30-550), sin requerimiento de transfusión sanguínea. No se reportaron complicaciones transoperatorias; se presentó una complicación en el posoperatorio (6.6%) clasificada como Clavien-Dindo I (infección de la herida quirúrgica). La estancia hospitalaria promedio fue de 2.85 días (rango: 2-7).
    CONCLUSIONS: El manejo definitivo de la PXG es la nefrectomía, y el abordaje laparoscópico debe ser considerado como una modalidad de tratamiento a pesar de ser una patología que presenta un proceso inflamatorio grave y crónico, obteniéndose beneficios como disminución en el tiempo quirúrgico, menor sangrado, menor tasa de complicaciones y menos días de estancia hospitalaria cuando es realizado por un cirujano experimentado.
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  • 文章类型: Journal Article
    目的:黄色肉芽肿性肾盂肾炎(XGP)是一种罕见的肾盂肾炎,常见于女性和糖尿病患者。常伴有肾结石,通常很难诊断,因为它可以在临床和放射学上模仿其他疾病。大多数病例使用抗生素和肾切除术治疗。我们审查的目的是总结和分析当前以管理为重点的证据。
    方法:进行了文献检索,以确定有关成人黄色肉芽肿性肾盂肾炎的文献。包含10名或更多XGP患者的研究被纳入描述性分析,以及一项队列研究的荟萃分析,对开放性肾切除术和微创肾切除术进行了比较。其他论文也包括在叙述性审查中。
    结果:确定了52项研究,纳入20项进行叙述性回顾,纳入32项包含868例患者的回顾性观察性研究进行描述性分析。99.8%的患者接受了肾切除术,大约三分之一的腹腔镜手术。最常见的培养生物是大肠杆菌和奇异变形杆菌。60%的患者,据报道,术前引流。包括211例患者的七项研究被纳入荟萃分析,发现术后并发症,接受微创手术的患者的住院时间和输血需求均显著降低.
    结论:XGP的主要管理是抗生素治疗和肾切除术。一些研究强调了术前上尿路引流的作用,但是支持这一点的证据是有限的。我们提出了第一个荟萃分析,以检查接受XGP肾切除术的患者的手术方法。尽管受现有数据的限制,我们的荟萃分析显示,XGP微创肾切除术提供了更好的术后结局.
    OBJECTIVE: Xanthogranulomatous pyelonephritis (XGP) is a rare form of pyelonephritis more commonly seen in females and diabetics. Frequently associated with renal tract calculi, it is often difficult to diagnose, as it can clinically and radiologically mimic other disorders. Most cases are treated with antibiotics and nephrectomy. The aim of our review is to summarise and analyse the current evidence focusing on management.
    METHODS: A literature search was conducted to identify papers relating to xanthogranulomatous pyelonephritis in adults. Studies containing ten or more patients with XGP were included for descriptive analysis, and a meta-analyses of cohort studies conducted comparing open and minimally invasive nephrectomy undertaken. Other papers were included for narrative review.
    RESULTS: 52 studies were identified, 20 were included for narrative review and 32 retrospective observational studies containing 868 patients were included for descriptive analysis. 99.8% of patients underwent nephrectomy, about one-third laparoscopically. The most commonly cultured organisms were Escherichia coli and Proteus mirabilis. 60% of patients, where reported, underwent preoperative drainage. Seven studies containing 211 patients were included for meta-analysis which found that postoperative complications, length of stay and transfusion requirements were all significantly reduced in those who underwent minimally invasive surgery.
    CONCLUSIONS: The mainstay management of XGP is antibiotic therapy and nephrectomy. Some studies highlight a role for preoperative upper urinary tract drainage, but evidence supporting this is limited. We present the first meta-analyses examining operative approach for patients undergoing nephrectomy for XGP. Though limited by the data available, our meta-analysis indicates minimally invasive nephrectomy for XGP provides better postoperative outcomes.
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  • 文章类型: Journal Article
    目的:黄色肉芽肿性胆囊炎(XGC)是慢性胆囊炎的一种罕见变种。这种罕见的病理特征是胆囊壁严重和进行性纤维化以及富含脂肪的巨噬细胞浸润。
    方法:回顾性分析2011-2019年8213例胆囊切除术的最终病理报告,将病理结果报告为XGC的患者纳入研究。患者的人口统计学特征,病理结果,并对手术方法进行了评价。对中转开腹胆囊切除术的危险因素进行Logistic回归分析。
    结果:胆囊切除术中XGC的发生率为0.91%。患者的平均年龄为57.32岁。92%(n:69)的患者采用了腹腔镜胆囊切除术。这些患者在术前都没有怀疑癌症,但是在手术中发现10.6%的患者怀疑癌症。有了冷冻测试,这些患者避免了不必要的手术。开腹胆囊切除术的转化率为26.09%。转换为开腹胆囊切除术的最常见原因(66.7%)是强烈的纤维化。超声检查发现胆囊壁厚度增加和急性胆囊炎是有统计学意义的危险因素(p<0.05)。XGC病例的总并发症发生率为3.9%。
    结论:XGC是一种极其罕见的疾病,在胆囊切除术前难以诊断。特别是在术前USG中,在没有怀疑恶性肿瘤的情况下,但手术期间怀疑是恶性肿瘤,在大范围手术前使用冷冻方法进行组织病理学检查可以防止不必要的解剖和相关的并发症。
    OBJECTIVE: Xanthogranulomatous cholecystitis (XGC) is a rare variant of chronic cholecystitis. This rare pathology is characterized by severe and progressive fibrosis of the gallbladder wall as well as infiltration of fat-laden macrophages.
    METHODS: The final pathology report of 8213 cholecystectomies performed between 2011 and 2019 was evaluated retrospectively, and patients whose pathology result was reported as XGC were included in the study. Patients\' demographic characteristics, pathology results, and surgical methods were evaluated. Logistic regression analysis was performed for risk factors on conversion to open cholecystectomy.
    RESULTS: The rate of XGC among cholecystectomies was 0.91%. Mean age of the patients was 57.32 years. Laparoscopic cholecystectomy was applied to 92% (n: 69) of the patients. None of the patients had cancer suspicion in the preoperative period, but cancer suspicion was found in 10.6% of the patients during the operation. With the frozen test, unnecessary surgeries were prevented in these patients. Conversion rate to open cholecystectomy was found to be 26.09%. The most common reason for conversion to open cholecystectomy (66.7%) was intense fibrosis. Increased gallbladder wall thickness and acute cholecystitis were found to be statistically significant risk factors in ultrasonography (p <0.05). Total complication rate in XGC cases was 3.9%.
    CONCLUSIONS: XGC is an extremely rare disease and is difficult to diagnose before cholecystectomy. Especially in preoperative USG, in cases with no suspicion of malignancy, but with suspected malignancy during the operation, histopathological examination with frozen method before extensive surgery may prevent unnecessary dissection and related morbidities.
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  • 文章类型: Journal Article
    内窥镜超声(EUS)比其他诊断成像方式具有更大的空间分辨率。此外,如果发现胆囊病变并怀疑胆囊癌,EUS是一种不可或缺的模态,使用多普勒模式和超声试剂进行详细的侵入深度评估测试。此外,胆囊病变,EUS细针穿刺(EUS-FNA)可用于区分良性和恶性疾病,如黄色肉芽肿性胆囊炎,在化疗前收集证据.EUS-FNA也可用于高度精确和特异性的诊断。然而,预防胆漏,一个意外的症状,是非常重要的。下一代测序(NGS)技术的进步促进了对EUS-FNA样品的多重平行测序的应用。几种生物标志物有望对胆囊癌的治疗进行分层;然而,NGS可以揭示治疗反应的潜在预测性基因组生物标志物。据认为,使用EUS-FNA获得的样品可能是可行的,进一步增加了对EUS-FNA的需求。
    Endoscopic ultrasonography (EUS) has greater spatial resolution than other diagnostic imaging modalities. In addition, if gallbladder lesions are found and gallbladder cancer is suspected, EUS is an indispensable modality, enabling detailed tests for invasion depth evaluation using the Doppler mode and ultrasound agents. Furthermore, for gallbladder lesions, EUS fine-needle aspiration (EUS-FNA) can be used to differentiate benign and malignant forms of conditions, such as xanthogranulomatous cholecystitis, and collect evidence before chemotherapy. EUS-FNA is also useful for highly precise and specific diagnoses. However, the prevention of bile leakage, an accidental symptom, is highly important. Advancements in next-generation sequencing (NGS) technologies facilitate the application of multiple parallel sequencing to EUS-FNA samples. Several biomarkers are expected to stratify treatment for gallbladder cancer; however, NGS can unveil potential predictive genomic biomarkers for the treatment response. It is believed that NGS may be feasible with samples obtained using EUS-FNA, further increasing the demand for EUS-FNA.
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  • 文章类型: Journal Article
    未经证实:黄色肉芽肿性肾盂肾炎(XPGN)是一种罕见的肾脏病理,发生在所有肾脏感染病例的0.6%至1%,在男人和女人。其特征在于肾实质的严重炎症,导致含有脂质负载的巨噬细胞的肉芽肿组织的形成。这种情况可能模仿较不积极或良性的情况,但如果不积极治疗,可能会恶化或致命。
    方法:我们的患者是一名54岁的白种人女性,表现为5天的左侧腹疼痛,血尿,发冷,恶心和呕吐。影像学和活检结果显示患者患有XPGN。
    UASSIGNED:XPGN是一种难以诊断的疾病,因为症状相对于肾细胞癌或其他常见的肾脏感染是非特异性的。明确的诊断是通过活检;然而,各种成像方式中的线索用于进行初步诊断。目前尚不清楚早期手术干预是否会改善患者的总体预后。目前,部分或完全肾切除术是唯一有效的治疗方法。
    结论:积极的管理,包括早期诊断,抗生素和肾切除术似乎对预防XPNG的进展和并发症至关重要。
    UNASSIGNED: Xanthogranulomatous pyelonephritis (XPGN) is a rare pathology of the kidneys occurring in 0.6 to 1% of all cases of renal infections, in both men and women. It is characterized by severe inflammation of the renal parenchyma leading to formation of granulomatous tissue containing lipid-laden macrophages. This condition may mimic less aggressive or benign conditions but may worsen or be fatal if not treated aggressively.
    METHODS: Our patient is a 54 year old Caucasian female who presented with five days of left flank pain, hematuria, chills, nausea and vomiting. Imaging and biopsy results showed that the patient had XPGN.
    UNASSIGNED: XPGN is a difficult condition to diagnose as the symptoms are non-specific relative to renal cell carcinoma or other common renal infections. Definitive diagnosis is made with a biopsy; however, clues in various imaging modalities are used to make a tentative diagnosis. It is unclear whether earlier surgical intervention would have improved overall patient outcomes. Currently, a partial or complete nephrectomy is the only effective treatment.
    CONCLUSIONS: Aggressive management including early diagnosis, antibiotics and nephrectomy appears to be critical in preventing progression and complications of XPNG.
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  • 文章类型: Journal Article
    OBJECTIVE: In this study, we aimed to compare development of complications, malignancy and confusion rates in the preliminary diagnosis in patients with xanthogranulomatous cholecystitis identified.
    METHODS: In this study, 2803 patients undergone cholecystectomy between January 2010 and December 2016 were retrospectively evaluated. Patients with xanthogranulomatous cholecystitis identified in the histopathological examination were classified as Group 1 and patients with cholelithiasis, cholecystitis, and malignancy detected were classified as Group 2.
    RESULTS: Forty-five patients with xanthogranulomatous cholecystitis were classified as group 1 and 2758 patients as group 2. of group 1, 18 were male and group 2 consisted of 2758 patients with 707 (26%) being male (p=0.04). In the ultrasonographic examination, the wall thickness was increased in 40 patients in Group 1 and 662 patients in Group 2 (p<0.0001). The operation was converted to the open type in 24 patients in Group 1 and 61 patients in Group 2 (p<0.0001). Five patients in Group 1 and 32 patients in Group 2 developed complications in the postoperative period (p<0.0001).
    CONCLUSIONS: Xanthogranulomatous cholecystitis should be considered for the differential diagnosis and the operation should be performed, especially by carefully exposing the anatomy in these patients.
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  • 文章类型: Journal Article
    Background Affections of the gallbladder remain exceedingly ubiquitous and often warrant surgical intervention. The histopathological patterns represent a spectrum, ranging from cholecystitis to gallbladder carcinoma. The present study aims to delineate the occurrence of various gallbladder histopathologies in a tertiary care hospital in Pakistan. Methods A retrospective study was conducted at Maroof International Hospital, Islamabad, Pakistan. Histopathological records of 442 gallbladder specimens obtained from cholecystectomy were analysed. The prevalence of various histopathological outcomes was assessed. The data were eventually analysed using the SPSS 23.0 software (Armonk, NY: IBM Corp.). Thereafter, the distribution of various gallbladder histopathologies was tabulated across gender.  Results Of the 442 patients included, 330 were females and 112 were males, with the mean age hovering at 45.77±14.65 years. The most common histopathological findings were chronic cholecystitis and cholesterolosis, observed in 78.6% and 32.8% of the patients, respectively. While only one case of gallbladder adenocarcinoma was observed, multiple specimens divulged premalignant lesions including reactive atypia and intestinal metaplasia. Conclusions Diseases of the gallbladder often mandate prompt surgical intervention. Of these, chronic cholecystitis, which is an established risk factor for gallbladder carcinoma, is exceedingly common. The employment of histopathological techniques remains imperative in the detection of premalignant and malignant lesions that might otherwise evade macroscopic detection and thus progress to adenocarcinoma.
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