Adrenal disorders

肾上腺疾病
  • 文章类型: Journal Article
    背景:肾上腺功能不全(AI)是一种罕见的疾病,其特征是肾上腺的糖皮质激素分泌受损。随着时间的推移,随着糖皮质激素替代剂量的减少和昼夜节律暴露方案的改善,AI的治疗策略已经发展起来。但这是否导致更好的生存是未知的。本系统综述的主要目的是收集和综合有关AI患者长期死亡率的现有证据。次要目的是研究死亡原因,专注于心血管和传染病,在AI患者中。
    方法:从各个数据库开始发表的研究(Medline,Embase,Cochrane和WebofScience)将在2023年5月底之前进行系统合成。将包括参考人群的观察性研究,它们的质量将使用纽卡斯尔-渥太华量表进行评估。收集的数据将进行叙述整合,并进行荟萃分析,以汇集被认为是同质研究的数据。系统审查将按照系统审查和荟萃分析指南的首选报告项目进行报告。这将是第一个评估AI患者死亡率和死亡原因的系统评价。这项系统评价的结果将对患者和医疗保健提供者都有价值。
    背景:该系统评价不需要伦理批准或知情同意,因为它将仅基于先前发布的数据,并且不涉及与单个患者的任何直接接触。研究结果将在科学会议上发表,并提交国际公认的同行评审科学期刊上发表。
    CRD42023416253。
    Adrenal insufficiency (AI) is a rare disorder characterised by an impaired secretion of glucocorticoids from the adrenal glands. Treatment strategies for AI have developed over time with reduced glucocorticoid replacement doses and improved circadian exposure regimens, but whether this has resulted in better survival is unknown. The main purpose of this systematic review is to gather and synthesise available evidence on long-term mortality in patients with AI. The secondary aim is to study causes of death, with focus on cardiovascular and infectious diseases, in AI patients.
    Studies published from the inception of respective databases (Medline, Embase, Cochrane and Web of Science) until the end of May 2023 will be systematically synthetised. Observational studies with a reference population will be included, and their quality will be assessed using the Newcastle-Ottawa scale. Data collected will be narratively integrated and a meta-analysis will be performed to pool data from studies considered homogeneous. The systematic review will be reported following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. This will be the first systematic review assessing mortality and causes of death in AI patients. The findings of this systematic review will be of value for both patients and healthcare providers.
    This systematic review does not require ethical approval or informed consent because it will be based on previously published data only and does not implicate any direct contact with individual patients. The research results will be presented at scientific conferences and submitted for publication in an internationally recognised peer-reviewed scientific journal.
    CRD42023416253.
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  • 文章类型: Journal Article
    背景:原发性醛固酮增多症(PA)的治疗策略包括单侧肾上腺切除术和盐皮质激素受体(MR)拮抗剂的药物治疗。这两种不同的治疗策略在减轻PA对结果的不利影响方面是否具有可比性仍存在争议。
    目的:本系统评价的主要目的是确定,评估和综合现有文献,比较PA患者治疗后的临床结果。
    方法:将使用PubMed进行系统和全面的搜索,WebofScience和EMBASE,对于2022年12月之前发表的研究。观察性和介入性研究将有资格纳入。将使用纽卡斯尔-渥太华量表评估观察性研究的质量,而介入研究将使用CochraneEffectivePracticeOrganizationofCare工具进行评估。收集的证据将以叙述方式综合。我们将进行荟萃分析,以汇集被认为是同质的研究的估计。系统评价和荟萃分析的报告将根据系统评价和荟萃分析指南的流行病学首选报告项目的观察性研究的荟萃分析。
    背景:由于本研究仅基于已发表的文献,不需要道德批准。这次审查的目的是提供一些关于结果的估计,包括生存,临床和生化控制率,心血管和脑血管事件,以及有关生活质量和肾功能的数据,在接受手术治疗或MR拮抗剂治疗的PA患者中。研究结果将在科学会议上发表,并将发表在国际同行评审的科学杂志上。
    CRD42022362506。
    Treatment strategies for primary aldosteronism (PA) include unilateral adrenalectomy and medical treatment with mineralocorticoid receptor (MR) antagonists. Whether these two different treatment strategies are comparable in mitigating the detrimental effect of PA on outcomes is still debated.
    The primary aim of this systematic review is to identify, appraise and synthesise existing literature comparing clinical outcomes after treatment in patients with PA.
    A systematic and comprehensive search will be performed using PubMed, Web of Science and EMBASE, for studies published until December 2022. Observational and interventional studies will be eligible for inclusion. The quality of observational studies will be assessed using the Newcastle-Ottawa Scale, while interventional studies will be assessed using the Cochrane Effective Practice Organization of Care tool. The collected evidence will be narratively synthesised. We will perform meta-analysis to pool estimates from studies considered to be homogeneous. Reporting of the systematic review and meta-analysis will be in accordance with the Meta-analysis of Observational Studies in Epidemiology Preferred Reporting Items for Systematic reviews and Meta-Analysis guidelines.
    As this study is based solely on the published literature, no ethics approval is required. This review will aim to provide some estimates on outcomes, including survival, rates of clinical and biochemical control, cardiovascular and cerebrovascular events, as well as data on quality of life and renal function, in patients with PA treated surgically or with MR antagonists. The study findings will be presented at scientific meetings and will be published in an international peer-reviewed scientific journal.
    CRD42022362506.
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  • 文章类型: Case Reports
    抗磷脂综合征(APS)是一种罕见的自身抗体介导的疾病,其特征是获得性血栓形成,导致反复的动脉和静脉血栓形成。一个激发因子允许内皮磷脂的暴露,导致抗原形成和随后产生的抗体。一名70多岁的妇女在车辆创伤后出现,肋骨骨折,气胸和偶然发现的左肾上腺出血。两周后,她出现了急性腹痛,在CT成像中发现右肾上腺出血,没有发生间隔创伤。该患者进行了抗磷脂抗体实验室研究,并在出院时给予静脉内肝素与华法林的桥接。实验室检查结果为狼疮抗凝物阳性,β-2糖蛋白和抗心磷脂抗体表明三阳性,在12周内反复进行实验室检查呈阳性,确认诊断。双侧肾上腺出血,而不是传统的静脉血栓栓塞,是该患者诊断为APS的病理表现。
    Antiphospholipid syndrome (APS) is an uncommon autoantibody-mediated condition characterised by acquired thrombophilia resulting in recurrent arterial and venous thrombosis. An inciting factor allows for the exposure of endothelial phospholipids, causing antigen formation and subsequent creation of antibodies. A woman in her 70s presented after vehicular trauma, suffering broken ribs, pneumothorax and incidentally discovered left adrenal haemorrhage. Two weeks later she presented with acute-onset abdominal pain and was found to have a right adrenal gland haemorrhage on CT imaging without interval trauma occurring. The patient had antiphospholipid antibody laboratory studies drawn and was given intravenous heparin with a bridge to warfarin at discharge. Laboratory results returned positive for lupus anticoagulant, beta-2 glycoprotein and anticardiolipin antibodies indicating triple positivity, with repeated laboratory tests positive in 12 weeks\' time, confirming the diagnosis. Bilateral adrenal haemorrhage, rather than traditional venous thromboembolism, was the presenting pathology in this patient\'s diagnosis of APS.
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  • 文章类型: Case Reports
    免疫检查点抑制剂显著改善转移性黑色素瘤的预后,但是转移到肾上腺仍然对这些新疗法有很强的抵抗力。黑色素瘤的肾上腺转移可以以不寻常的方式出现,比如在这份报告中,使其在诊断和治疗上具有挑战性。在这个案例报告中,我们介绍了一个组织学证实的肾上腺转移性黑色素瘤患者,怀疑转移性疾病的巨大心内肿块,和下腔静脉血栓.我们回顾了现有的文献来解释其独特的特征,临床相关性,发病机制,诊断,和黑色素瘤肾上腺转移的治疗。
    Immune checkpoint inhibitors have significantly improved the prognosis of metastatic melanoma, but metastases to the adrenal glands remain highly resistant to these new treatments. Adrenal gland metastases from melanoma can present in an unusual manner, such as in this report, making it diagnostically and therapeutically challenging. In this case report, we present a patient with histologically confirmed metastatic melanoma to the adrenal glands, a large intracardiac mass suspicious for metastatic disease, and an inferior vena cava thrombus. We review the existing literature to explain the unique characteristics, clinical relevance, pathogenesis, diagnosis, and treatment of adrenal gland metastases from melanoma.
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  • 文章类型: Case Reports
    自发性肾上腺出血(SAH)是一种罕见的疾病。目前尚不清楚妊娠期肾上腺出血的发生率;据报道与怀孕有关。严重的背部或侧腹疼痛的急性表现应引起对这种情况的怀疑。诊断基于影像学。超声扫描是怀孕期间的基本且容易获得的检查,以排除肾脏和肾上腺病理学,而CT或MRI扫描可以帮助确认诊断。多学科团队(MDT)方法,涉及产科,麻醉,医疗和内分泌团队,在这种情况的管理中至关重要。我们提出了一个SAH的案例;保守地管理,在一个健康和低风险的孕妇中,并描述了关于这种罕见情况的文献综述,包括病理生理学和管理。
    Spontaneous adrenal haemorrhage (SAH) is a rare condition. The incidence of adrenal haemorrhage in pregnancy is currently not known; however, an association with pregnancy has been reported.An acute presentation with severe back or flank pain should raise suspicion of this condition. Diagnosis is based on imaging. An ultrasound scan is a basic and readily available investigation in pregnancy to rule out renal and suprarenal pathology while CT or MRI scan can help to confirm the diagnosis. A multidisciplinary team (MDT) approach, involving the obstetric, anaesthetic, medical and endocrine team, is essential in management of this condition.We present a case of an SAH; managed conservatively, in an otherwise healthy and low-risk pregnant woman and describe the literature review on this rare condition, including pathophysiology and management.
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  • 文章类型: Case Reports
    一名68岁的妇女在没有事先接触肝素的情况下被送往医院进行双膝选择性全膝关节置换术。她在术后第12天出现肾上腺出血和血小板减少症,随后出现右腿动脉闭塞和多个静脉腹内部位血栓形成。在给予普通肝素治疗动脉闭塞后,血小板计数逐渐下降。自发性肝素诱导的血小板减少症通过肝素诱导的血小板活化试验和光透射聚集测定法诊断。患者成功接受磺达肝素和静脉注射免疫球蛋白治疗。血小板计数恢复后给予阿哌沙班。治疗2个月后,整个腹部的CT显示沿主动脉的血栓和肾上腺出血的消退。我们的案例表明,这种严重的并发症很重要,但很少早期发现。
    A 68-year-old woman was admitted to the hospital for elective total knee arthroplasty in both knees without preceding heparin exposure. She developed adrenal haemorrhage and thrombocytopaenia on postoperative day 12, followed by right leg arterial occlusion and multiple venous intra-abdominal sites thrombosis. After given unfractionated heparin to treat arterial occlusion, platelet count was gradually declined. Spontaneous heparin-induced thrombocytopaenia was diagnosed by heparin-induced platelet activation test with light transmission aggregometry. The patient was successfully treated with fondaparinux and intravenous immunoglobulin. Apixaban was given after recovery of platelet count. Resolution of both thrombus along aorta and adrenal haemorrhage were shown by CT of whole abdomen after 2 months of treatment. Our case demonstrates that this serious complication is important but seldom recognised early.
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  • 文章类型: Case Reports
    甲基丙二酰辅酶A变位酶缺乏症是以甲基丙二酸积累为特征的支链氨基酸代谢中罕见的常染色体遗传性先天性错误。关于这些儿童进行腹部大手术的麻醉管理的文献相对较少。术前管理包括以目标为导向的脱水纠正,代谢性酸中毒和高氨血症。麻醉目标包括避免可能引发代谢危机的因素,如高碳酸血症,体温过低,缺氧,手术应激,低血容量,低血压等。在这里,我们正在报告一名17个月大的患有甲基丙二酸血症(MMA)的儿童的麻醉管理,该儿童因肾上腺肿块切除而进行了上腹部大手术,入院时偶然诊断为代谢危象。我们的目标是强调疾病的病理生理学的具体细微差别,术前优化,麻醉方面的考虑,高级监测和区域麻醉的作用以及有关MMA患者管理的最新文献。
    Methyl malonyl coenzyme A mutase deficiency is a rare autosomal inherited inborn error in branched-chain amino acid metabolism characterised by the accumulation of methylmalonic acids. There is relative paucity of literature regarding anaesthetic management of these children presenting for incidental major abdominal surgery. Preoperative management includes goal-directed correction of dehydration, metabolic acidosis and hyperammonemia. Anaesthetic goals include avoidance of factors that can trigger metabolic crisis like hypercapnia, hypothermia, hypoxia, surgical stress, hypovolaemia, hypotension and so on. Herein, we are reporting the anaesthetic management of a 17-month-old child with methylmalonic acidemia (MMA) posted for a major upper abdominal surgery for excision of an adrenal mass, which was incidentally diagnosed during admission for an episode of metabolic crisis. We aim to highlight the specific nuances of pathophysiology of the disease, preoperative optimisation, anaesthetic considerations, role of advanced monitoring and regional anaesthesia and current literature on the management of patients with MMA.
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  • 文章类型: Journal Article
    Pregnancy in women with associated endocrine conditions is a therapeutic challenge for clinicians. These disorders may be common, such us thyroid disorders and diabetes, or rare, including adrenal and parathyroid disease and pituitary dysfunction. With the development of assisted reproductive techniques, the number of pregnancies with these conditions has increased. It is necessary to recognize symptoms and correct diagnosis for a proper pharmacotherapeutic management in order to avoid adverse side effects both in mother and fetus. This review summarizes the pharmacotherapy of these clinical situations in order to reduce maternal and fetal morbidity.
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  • 文章类型: Journal Article
    Aiming to validate the use of a single poststimulus sampling protocol for cosyntropin test short standard high-dose test (SST) in our institution, our primary objectives were (1) to determine the concordance between 30 and 60 min serum cortisol (SC) measurements during SST; and (2) to evaluate the diagnostic agreement between both sampling times when using classic or assay-specific and sex-specific SC cut-off values. The secondary objectives included (1) estimating the specificity and positive predictive value of 30 and 60 min sampling times while considering the suspected origin of adrenal insufficiency (AI); and (2) obtaining assay-specific cut-off values for SC after SST in a group of subjects with normal hypothalamic-pituitary-adrenal (HPA) axis.
    This is a retrospective chart review study conducted at a Spanish academic hospital from 2011 to 2015.
    Two groups were evaluated: (1) a main study group including 370 patients in whom SC was measured at 30 and 60 min during SST; and (2) a confirmative group that included 150 women presenting with a normal HPA axis in whom SST was conducted to rule out late-onset congenital adrenal hyperplasia. Diagnostic agreement between both sampling times was assessed by considering both classic (500 nmol/L) and assay-specific SC cut-off concentrations.
    Diagnostic agreement between both sampling times was greater when applying sex-specific and assay-specific cut-off values instead of the classic cut-off values. For suspected primary AI, 30 min SC determination was enough to establish a diagnosis in over 95% of cases, without missing any necessary treatment. When central AI is suspected, 60 min SC measurement was more specific, establishing a diagnosis in over 97% of cases.
    Sex-specific and assay-specific SC cut-off values improve the diagnostic accuracy of SST. For primary disease, a subnormal SC response at 30 min is a reliable marker of adrenal dysfunction. On the contrary, when central AI is suspected, 60 min SC measurement improves the diagnostic accuracy of the test.
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