Adrenocortical

肾上腺皮质
  • 文章类型: Journal Article
    高血压导致多种合并症和死亡风险增加。内分泌失调有助于高血压的发展,包括原发性醛固酮增多症(PA)。本文讨论了PA的评估和管理。
    Hypertension leads to multiple comorbidities and increased risk for mortality. Endocrine disorders contribute to the development of hypertension, including primary aldosteronism (PA). This article discusses the evaluation and management of PA.
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  • 文章类型: Case Reports
    肾上腺皮质嗜酸细胞腺瘤通常表现为无功能性肾上腺肿瘤,但可能导致患者出现库欣综合征。本文报道1例由右肾上腺皮质嗜酸细胞瘤引起的库欣综合征。患者在武川市人民医院泌尿外科(遵义,中国),2022年11月,由于多毛症,体重增加和高血压。使用腹部入路进行腹腔镜右肾上腺肿瘤切除术。手术后,患者的血压和心率在健康范围内波动,月经恢复正常。腹腔镜肾上腺切除术较开腹肾上腺切除术有明显优势,比如更少的创伤,恢复时间短,并发症少。因此,这种罕见疾病的治疗是安全可行的。
    Adrenal cortical eosinophilic adenoma usually presents as non-functional adrenal tumor but may lead to Cushing\'s syndrome in patients. The present article reports a patient with Cushing\'s syndrome caused by right adrenocortical oncocytoma. The patient was treated in Urology Department of Wuchuan People\'s Hospital (Zunyi, China) in November 2022 because of hirsutism, weight gain and hypertension. A laparoscopic right adrenal tumor resection was performed using an abdominal approach. Following surgery, blood pressure and heart rate of the patient fluctuated within a healthy range and menstruation returned to normal. Laparoscopic adrenalectomy has obvious advantages over open adrenalectomy, such as less trauma, shorter recovery time and fewer complications. Thus, this treatment for this rare disease is safe and feasible.
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  • 文章类型: Journal Article
    衰老的特征是生理功能逐渐下降。这个过程影响到包括肾上腺皮质在内的所有器官,它通常能产生必需的类固醇激素,包括盐皮质激素,糖皮质激素,和雄激素。随着年龄的增长,特征,如减少肾上腺皮质大小,改变的分区,髓样免疫细胞浸润的增加实质上改变了肾上腺皮质的结构和功能。肾上腺皮质衰老的许多特征都发生在男性和女性中,但在男性中却更加增强。荷尔蒙,肾上腺雄激素的大量减少是衰老的关键特征,伴随着醛固酮和皮质醇的适度变化。这些荷尔蒙的变化与各种病理后果有关,包括受损的免疫反应。骨骼健康下降,和加速与年龄有关的疾病。肾上腺衰老最显著的变化之一是肾上腺肿瘤的发病率增加,这是性别二态,女性患病率更高。随着年龄的增长,肾上腺肿瘤发生的增加可能是由于基因突变的增加以及组织微环境的重塑所致。新的抗衰老策略提供了一个有希望的途径,以减轻肾上腺衰老和减轻年龄相关的病理,包括肾上腺肿瘤.
    Aging is characterized by a gradual decline in physiological function. This process affects all organs including the adrenal cortex, which normally functions to produce essential steroid hormones including mineralocorticoids, glucocorticoids, and androgens. With increasing age, features such as reduced adrenal cortex size, altered zonation, and increased myeloid immune cell infiltration substantially alter the structure and function of the adrenal cortex. Many of these hallmark features of adrenal cortex aging occur both in males and females, yet are more enhanced in males. Hormonally, a substantial reduction in adrenal androgens is a key feature of aging, which is accompanied by modest changes in aldosterone and cortisol. These hormonal changes are associated with various pathological consequences including impaired immune responses, decreased bone health, and accelerated age-related diseases. One of the most notable changes with adrenal aging is the increased incidence of adrenal tumors, which is sex dimorphic with a higher prevalence in females. Increased adrenal tumorigenesis with age is likely driven by both an increase in genetic mutations as well as remodeling of the tissue microenvironment. Novel antiaging strategies offer a promising avenue to mitigate adrenal aging and alleviate age-associated pathologies, including adrenal tumors.
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  • 文章类型: Case Reports
    背景:经前鉴定的肾上腺上肿块最常发现为肾上腺出血。这种情况下最常见的肿瘤是神经母细胞瘤。
    方法:我们报告一例罕见的肾上腺皮质肿瘤在产前超声发现。虽然大多数肾上腺皮质肿瘤患者存在男性化症状,我们的患者没有男性化的证据,推测患有神经母细胞瘤.
    结论:经过一段时间的观察,由于肿瘤生长,我们的患者接受了手术切除,显示出意外的诊断。
    BACKGROUND: Prenatally identified suprarenal masses are most often found to be adrenal hemorrhage. The most common tumor in this situation is neuroblastoma.
    METHODS: We report the case of a rare adrenocortical tumor found prenatally on ultrasound. While most patients with adrenocortical tumors present with virilizing symptoms, our patient did not have evidence of virilization and was presumed to have neuroblastoma.
    CONCLUSIONS: Following a period of observation, our patient underwent surgical resection due to tumor growth revealing the unexpected diagnosis.
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  • 文章类型: Journal Article
    肾上腺皮质肿瘤是儿童的罕见肿瘤,缺乏结果数据,尤其是在资源受到挑战的环境中。
    进行了一项回顾性研究以评估其表现,管理,以及1992年1月至2019年12月到我们研究所就诊的肾上腺皮质肿瘤儿童的结局。
    在研究期间,包括28名儿童。9名儿童存在腺瘤,其余19名儿童患有肾上腺皮质癌。报告的中位年龄为48个月。将近一半的患有癌的儿童表现出肿块,而只有10%的腺瘤(P=0.049)。在60%的患者中存在病毒化,并且在大多数情况下与Cushingoid特征有关。3名儿童(15.7%)出现转移。所有儿童均接受了手术切除,其中6例溢出,两名患者残留。在平均11.8个月后,在5例患者中观察到复发。腺瘤患者的4年总生存率和无事件生存率分别为100%和75%和44%。分别,在癌症中。接受完全手术切除而没有任何溢出的儿童具有相对有利的结果。
    肾上腺皮质肿瘤是儿童的少见肿瘤。腺瘤有良好的结局。在不完全切除或转移性肿瘤中没有无病生存。肿瘤溢出在完全切除的肿瘤中也可能具有不良结果。
    UNASSIGNED: Adrenocortical tumors are rare tumors in children with a paucity of outcome data, especially in a resource-challenged setting.
    UNASSIGNED: A retrospective study was conducted to evaluate the presentation, management, and outcomes of children with adrenocortical tumors who presented to our institute from January 1992 to December 2019.
    UNASSIGNED: During the study period, 28 children were included. Adenoma was present in nine children and the remaining 19 children had adrenocortical carcinoma. The median age of presentation was 48 months. Nearly half of the children with carcinomas presented with a mass compared to only 10% adenomas (P = 0.049). Virilization was present in 60% of the patients and in most cases was associated with Cushingoid features. Three children (15.7%) had metastasis at presentation. All children underwent surgical excision with a spill in 6 and gross residue in two patients. Recurrence was observed in five patients after a mean duration of 11.8 months. The 4-year overall survival and event-free survival were 100% in patients with adenomas compared to 75% and 44%, respectively, in carcinoma. Children who underwent complete surgical excision without any spill had a relatively favorable outcome.
    UNASSIGNED: Adrenocortical tumors are uncommon tumors in children. Adenomas have a favorable outcome. There was no disease-free survival in incompletely resected or metastatic tumors. Tumor spill may also have an adverse outcome in completely resected tumors.
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  • 文章类型: Case Reports
    肾上腺嗜酸细胞肿瘤是一种极其罕见的由肾上腺引起的肿瘤,在肾上腺偶发瘤的鉴别诊断中应予以考虑。因为它经常不起作用。当肾上腺偶发瘤大于4厘米时,对恶性肿瘤的怀疑很高;然而,肾上腺嗜酸细胞瘤很大,平均测量8厘米,是圆形和封装的,通常有良性行为。我们介绍了一例55岁的男性血脂异常患者,服用辛伐他汀。在抱怨腹痛时,全科医生要求腹部超声检查显示肾上腺病变,进一步以计算机断层扫描为特征,显示49×64×56mm的肾上腺病变和钙化灶。患者转诊至普外科和内分泌科。分析研究对嗜铬细胞瘤或库欣综合征呈阴性,允许手术进行,如建议。本病例报告的目的是帮助了解肾上腺嗜酸细胞瘤,因为基于独特经验的信息很少。
    An adrenal oncocytic neoplasm is an extremely rare tumour arising from the adrenal gland and it should be considered in the differential diagnosis of an adrenal incidentaloma, since it is frequently non-functioning. The suspicion for malignancy is high when an adrenal incidentaloma is >4 cm in size; however, adrenal oncocytomas are large, measuring an average of 8 cm, are round and encapsulated, and normally have a benign behaviour. We present a case of a 55-year-old male patient with dyslipidaemia, medicated with simvastatin. Upon complaints of abdominal pain, the general physician asked for an abdominal ultrasound that revealed an adrenal lesion, further characterized with a computed tomography scan, which showed an adrenal lesion measuring 49 × 64 × 56 mm and a calcification focus. The patient was referred to the general surgery and endocrinology department. The analytical study was negative for pheochromocytoma or Cushing\'s syndrome, which allowed surgery to be conducted, as is recommended. The aim of this case report is to contribute to the knowledge on adrenal oncocytomas, since there is scarce information based on singular experiences.
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  • 文章类型: Journal Article
    原发性醛固酮增多症(PA)是一种常见且高度可治疗的疾病,通常由肾上腺皮质肿瘤生长或增生引起。PA由于其复杂而漫长的诊断程序,目前未被诊断。简化的基于生物标志物的测试在降低心血管发病率和死亡率方面将是非常有价值的。循环微小RNA由于其稳定性和可及性而成为许多疾病的潜在生物标志物。已知PA会改变肾上腺皮质组织中的microRNA表达;如果这些变化或其作用反映在循环miRNA谱中,那么这可以通过诊断测试来利用。然而,由于一系列技术挑战,鉴定生物标志物-循环microRNAs的研究的可重复性已被证明难以用于其他疾病.因此,任何寻求最终确定PA循环microRNA生物标志物的研究都必须在其设计中解决这个问题.为此,我们目前正在进行正在进行的ENS@T-HT研究的循环microRNA部分.在这篇评论文章中,我们提供的证据支持循环microRNAs作为PA生物标志物的效用,描述这种方法的实际挑战,以ENS@T-HT为例,讨论如何克服这些问题。
    Primary aldosteronism (PA) is a common and highly treatable condition, usually resulting from adrenocortical tumorous growth or hyperplasia. PA is currently underdiagnosed owing to its complex and protracted diagnostic procedures. A simplified biomarker-based test would be highly valuable in reducing cardiovascular morbidity and mortality. Circulating microRNAs are emerging as potential biomarkers for a number of conditions due to their stability and accessibility. PA is known to alter microRNA expression in adrenocortical tissue; if these changes or their effects are mirrored in the circulating miRNA profile, then this could be exploited by a diagnostic test. However, the reproducibility of studies to identify biomarker-circulating microRNAs has proved difficult for other conditions due to a series of technical challenges. Therefore, any studies seeking to definitively identify circulating microRNA biomarkers of PA must address this in their design. To this end, we are currently conducting the circulating microRNA arm of the ongoing ENS@T-HT study. In this review article, we present evidence to support the utility of circulating microRNAs as PA biomarkers, describe the practical challenges to this approach and, using ENS@T-HT as an example, discuss how these might be overcome.
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  • 文章类型: Journal Article
    目的:胎盘异位组织的报道较少,主要包括肝脏和肾上腺细胞。我们报告在胎盘中发现的脂肪组织。病例报告:我们介绍了一个在25岁的胎盘中发现的病例,该胎盘在妊娠期间患有高血压疾病。在常规显微镜研究中,我们观察到了异位,良性,脂肪组织的外切和绒毛间结节。结论:据我们所知,绒毛膜绒毛中没有其他报道的脂肪细胞病例。为什么外来组织出现在胎盘中仍然未知;然而,一些新的理论提供了解释。
    Objective: Reports of heterotopic tissue in the placenta are few and mainly include liver and adrenal cells. We report on adipose tissue found in the placenta. Case report: We present the case of a microscopic finding in a 25-year-old\'s placenta who suffered from hypertensive disorder in pregnancy. During routine microscopic study, we observed a heterotopic, benign, circumscribed and intervillous nodule of adipose tissue. Conclusion: To our knowledge, there is no other reported case of adipocytes among chorionic villi. Why foreign tissues show up in the placenta remains unknown; however, several new theories offer explanations.
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  • 文章类型: Journal Article
    异位肾上腺组织,定义为肾上腺组织在异常解剖位置的形成,不是罕见的实体,可能具有临床意义。尽管它们出现的机制还没有被完全理解,已经报道了许多异位肾上腺组织的病例,大部分在肾上腺的原始位置附近,如肾脏和性腺。在这些情况下,大多数作者将它们的出现归因于可能的迁移缺陷。然而,这种机制并不能简单地解释偏远地区的异位组织,如垂体或肺部。这篇综述总结了这些报告,描述了许多不同位置的异位肾上腺组织,以及他们建议的机制。
    Ectopic adrenal tissue, defined as the formation of adrenal tissue in an abnormal anatomical location, is not a rare entity and may have clinical significance. Even though the mechanism for their emergence has not been fully understood, numerous cases of ectopic adrenal tissue have been reported, mostly in the vicinity of the original location of adrenal gland, such as in kidneys and gonads. In these cases, most authors attributed their emergence to a probable migration defect. However, this mechanism does not simply explain the ectopic tissues in remote locations, such as in the hypophysis or lungs. This review summarizes these reports, describing many different locations in which ectopic adrenal tissues were encountered, together with their suggested mechanisms.
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  • 文章类型: Journal Article
    尽管在阐明肾上腺皮质癌(ACC)的分子通路方面取得了最新进展,这种孤儿恶性肿瘤与低生存率有关.可靶向基因组改变的鉴定对于改善结果至关重要。这项研究的目的是表征一大群患者ACC样品的基因组谱,以鉴定可操作的基因组改变。分析了三百六十四个个体患者ACC肿瘤。该队列的中位年龄为52岁,60.9%(n=222)为女性。ACC样本在表观遗传途径上有常见的改变,38%的肿瘤携带参与组蛋白修饰的基因改变。端粒延长21%,和21%的SWI/SNF复合物。肿瘤抑制基因和WNT信号通路在51%的肿瘤中各自突变。50个(13.7%)ACC肿瘤在参与DNA错配修复(MMR)途径的基因中具有基因组改变,其中许多肿瘤还显示异常高数目的突变和相应的MMR突变特征。此外,观察到一些以前与ACC无关的基因的基因组改变,包括IL7R,LRP1B,FRS2在6%,8%和4%的肿瘤中突变,分别。总的来说,58.5%的ACC(n=213)在46个不同基因中具有至少一个潜在可操作的基因组改变。由于超过一半的ACC具有一个或多个潜在的可操作的基因组改变,这凸显了靶向测序对这种预后不良的孤儿癌的价值.此外,MMR基因改变的显著发生率表明,对于相当一部分ACC患者,免疫治疗是一种有希望的治疗方法.
    Despite recent advances in elucidating molecular pathways underlying adrenocortical carcinoma (ACC), this orphan malignancy is associated with poor survival. Identification of targetable genomic alterations is critical to improve outcomes. The objective of this study was to characterize the genomic profile of a large cohort of patient ACC samples to identify actionable genomic alterations. Three hundred sixty-four individual patient ACC tumors were analyzed. The median age of the cohort was 52 years and 60.9% (n = 222) were female. ACC samples had common alterations in epigenetic pathways with 38% of tumors carrying alterations in genes involved in histone modification, 21% in telomere lengthening, and 21% in SWI/SNF complex. Tumor suppressor genes and WNT signaling pathway were each mutated in 51% of tumors. Fifty (13.7%) ACC tumors had a genomic alteration in genes involved in the DNA mismatch repair (MMR) pathway with many tumors also displaying an unusually high number of mutations and a corresponding MMR mutation signature. In addition, genomic alterations in several genes not previously associated with ACC were observed, including IL7R, LRP1B, FRS2 mutated in 6, 8 and 4% of tumors, respectively. In total, 58.5% of ACC (n = 213) had at least one potentially actionable genomic alteration in 46 different genes. As more than half of ACC have one or more potentially actionable genomic alterations, this highlights the value of targeted sequencing for this orphan cancer with a poor prognosis. In addition, significant incidence of MMR gene alterations suggests that immunotherapy is a promising therapeutic for a considerable subset of ACC patients.
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