Pituitary Diseases

垂体疾病
  • 文章类型: Review
    基础促肾上腺皮质激素(ACTH)浓度的测量是垂体中层功能障碍(PPID)最常用的诊断测试。尽管已经报道了几种分析前和分析因素会影响同类动物的基础ACTH浓度,在PPID诊断中对这些评估的程度尚不清楚.本范围审查的目的是确定并系统地绘制有关影响成年家畜基础ACTH浓度的分析前和分析因素的当前证据。电子数据库和会议记录的系统搜索于2022年6月进行,2022年10月重复,2023年8月更新。包括在这些日期之前出版的英文出版物。筛选和数据提取由作者单独进行,使用预定义的条件和修改的范围审查数据提取模板。删除重复项后,确定了903种出版物,其中235份摘要经筛选合格,134份出版物符合纳入标准.一年中的时间,锻炼,品种/类型和运输是与ACTH浓度显着增加最常见的因素(n=26、16、13和10出版物,分别)。只有25份出版物报告在研究人群中包含PPID病例,因此,影响基础ACTH浓度的许多因素与PPID诊断准确性之间的关系尚不明确.然而,确定了可能影响基础ACTH结果解释的几个因素.调查结果还强调,需要在未来的研究中详细报告分析前和分析条件,以促进将证据转化为实践。
    Measurement of basal adrenocorticotropic hormone (ACTH) concentration is the most commonly used diagnostic test for pituitary pars intermedia dysfunction (PPID). Although several pre-analytical and analytical factors have been reported to affect basal ACTH concentrations in equids, the extent to which these have been evaluated in the context of PPID diagnosis is unclear. The objectives of this scoping review were to identify and systematically chart current evidence about pre-analytical and analytical factors affecting basal ACTH concentrations in adult domestic equids. Systematic searches of electronic databases and conference proceedings were undertaken in June 2022, repeated in October 2022 and updated in August 2023. English language publications published prior to these dates were included. Screening and data extraction were undertaken individually by the authors, using predefined criteria and a modified scoping review data extraction template. After removal of duplicates, 903 publications were identified, of which 235 abstracts were screened for eligibility and 134 publications met inclusion criteria. Time of year, exercise, breed/type and transportation were the factors most frequently associated with significant increases in ACTH concentration (n = 26, 16, 13 and 10 publications, respectively). Only 25 publications reported inclusion of PPID cases in the study population, therefore the relationship between many factors affecting basal ACTH concentration and diagnostic accuracy for PPID remains undefined. However, several factors were identified that could impact interpretation of basal ACTH results. Findings also highlight the need for detailed reporting of pre-analytical and analytical conditions in future research to facilitate translation of evidence to practice.
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  • 文章类型: Review
    垂体柄中断综合征(PSIS)是一种先天性疾病,常见于合并垂体激素缺乏症(CPHD)的患者。大多数PSIS患者表现为生长迟缓和青春期延迟。我们报道了一例罕见的身材高大的PSIS病例,肝硬化和糖尿病,可能是KCNJ11基因突变所致.
    一名37岁女性患者最初表现为肝硬化和糖尿病,没有第二性征.内分泌检查提示CPHD。小垂体前叶,在磁共振成像(MRI)中观察到的蝶鞍中看不见的垂体柄和无在位后叶高信号证实了PSIS的诊断。尽管没有接受生长激素或性激素治疗,她最终达到了186厘米的高度。肝组织病理学提示非酒精性脂肪性肝硬化。遗传测试鉴定了KCNJ11基因中的杂合p.Arg301Cys突变。
    这是一例罕见的与KCNJ11基因突变相关的肝硬化和糖尿病的PSIS病例。据推测,早期高胰岛素血症是由KCNJ11基因突变引起的,以及由于雌激素缺乏而导致的骨phy闭合延迟,促成了病人异常高大的身材。未经治疗的生长激素缺乏症(GHD)导致内脏脂肪增加,导致非酒精性脂肪性肝病(NAFLD)和肝硬化。β细胞功能随着年龄的增长而下降,结合NAFLD,可能在糖尿病的发展中发挥了作用。
    Pituitary stalk interruption syndrome (PSIS) is a congenital disease commonly found in patients with combined pituitary hormone deficiency (CPHD). Most PSIS patients manifest growth retardation and delayed puberty. We report a rare case of PSIS with tall stature, liver cirrhosis and diabetes, possibly caused by an inactivating KCNJ11 gene mutation.
    A 37-year-old female patient initially presented with liver cirrhosis and diabetes, without any secondary sexual characteristics. Endocrine investigation indicated CPHD. Small anterior pituitary, invisible pituitary stalk and no eutopic posterior lobe hypersignal in the sella turcica viewed in magnetic resonance imaging (MRI) confirmed the diagnosis of PSIS. Despite receiving no growth hormone or sex hormone therapy, she reached a final height of 186 cm. Liver histopathology revealed nonalcoholic fatty cirrhosis. Genetic testing identified a heterozygous p.Arg301Cys mutation in the KCNJ11 gene.
    This is a rare case of PSIS with liver cirrhosis and diabetes associated with an inactivating KCNJ11 gene mutation. It\'s supposed that early hyperinsulinism caused by the KCNJ11 gene mutation, as well as delayed epiphyseal closure due to estrogen deficiency, contributed to the patient\'s exceptionally tall stature. Untreated growth hormone deficiency (GHD) resulted in increased visceral fat, leading to nonalcoholic fatty liver disease (NAFLD) and cirrhosis. The decline in β cell function with age, combined with NAFLD, may have played a role in the development of diabetes.
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  • 文章类型: Review
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  • 文章类型: Systematic Review
    最近的文献表明,内窥镜垂体手术存在学习曲线。然而,这些研究报告结果的方式存在显著差异.本研究旨在系统地回顾有关内镜垂体手术的结果以及这可能与手术学习曲线有关的文献。对Medline数据库的电子搜索,Scopus,Embase,进行了WebofScience和CochraneLibrary数据库,并根据2020年系统评论和荟萃分析(PRISMA)声明的首选报告项目提取数据。这篇综述包括了十篇文章,因为他们检查了以下内容:总切除率,平均手术时间,脑脊液漏出率,视觉结果,内分泌结局以及这些结果如何受到手术经验的影响.我们已经证明,内镜垂体手术的某些结果变量存在学习曲线。然而,目前的文献存在显著的异质性,这使得明确的比较变得困难。
    Recent literature demonstrates that a learning curve exists for endoscopic pituitary surgery. However, there is significant variability in the way these studies report their outcomes. This study aims to systematically review the literature regarding outcomes for endoscopic pituitary surgery and how this may be related to a surgical learning curve. An electronic search of the databases Medline, Scopus, Embase, Web of Science and Cochrane Library databases was performed and data extracted according 2020 Preferred Reporting Items of Systematic Reviews and Meta-Analyses (PRISMA) statement. Ten articles were included in the review as they examined the following: rates of gross total resection, average operative time, CSF leak rate, visual outcomes, endocrine outcomes and how these results were influenced by surgical experience. We have demonstrated that a learning curve exists for some outcome variables for endoscopic pituitary surgery. However, there is significant heterogeneity in the current body of literature which makes clear comparisons difficult.
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  • 文章类型: Journal Article
    垂体腺复制是一种罕见的异常,而垂体柄的孤立复制却没有任何垂体异常是一种罕见的实体,这种情况是迄今为止第一例记录在案的病例。尽管偶然发现的脑垂体(DPG)重复病例已有报道,有时会有重复的垂体柄,患有这种疾病的患者通常会出现其他颅面异常。因此,使用DPGplus综合征,因为它通常伴有内分泌紊乱和病理,例如正中裂面部综合征,眼部疾病,颅颈骨异常,血管异常和结核乳腺肿块。由于这是第一例报告的病例,没有任何额外的脑垂体异常,我们建议使用首字母缩写DPS(垂体柄复制)来统一这个实体,因为我们可以肯定的是,就像前面描述的垂体复制障碍一样,更多的病例将独立记录,而不是在垂体复制障碍的保护下。这是至关重要的,因为与现有的垂体重复综合征相比,我们研究中审查的病例的预期寿命(诊断年龄)与正常人群一样好,死亡率没有明显增加。我们提供了一个2岁7个月大的女孩的病例报告,该女孩被儿科医生转诊以评估早产。垂体柄的重复以及大型大池和结核融合是唯一的阳性影像学发现,垂体绝对正常。
    Pituitary gland duplication is a rare abnormality and isolated duplication of the pituitary stalk without any pituitary anomaly is an even rarer entity with this case being the first documented case till date. Although incidentally discovered cases of duplication of pituitary gland (DPG) have been reported, sometimes with a duplicated pituitary stalk, patients with this disorder usually present with other craniofacial abnormalities. Consequently, DPG plus syndrome is used as it is often accompanied by endocrine disturbances and pathologies such as median cleft face syndrome, ocular disorders, craniocervical bony abnormalities, vascular anomalies and tuberomammillary masses. Since this is the first reported case without any additional pituitary gland anomaly, we propose the acronym DPS (duplication of pituitary stalk) to be used to unify this entity as we are certain that much like the previously described pituitary duplication disorders, more cases will be documented independently rather than under the umbrella of pituitary duplication disorders. This is critical as the life expectancy (age of diagnosis) in the cases reviewed in our study is as good as normal population with no obvious increase in mortality as compared to existing pituitary duplication syndromes. We present a case report of a 2 year 7 month old girl who was referred by the paediatrician for evaluation of premature thelarche. The duplication of the pituitary stalk along with mega cisterna magna and tuberomammillary fusion was the only positive finding on imaging with the pituitary gland being absolutely normal.
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  • 文章类型: Review
    酒精诱导的皮质醇增多症(AIH)未被认识到,可能伪装成肿瘤性皮质醇增多症[库欣综合征(CS)]掩盖了其诊断。
    为了表征AIH,我们对8名患者(4名男性和4名女性;2014-2022年)进行了图表审查,以评估和治疗肿瘤性皮质醇增多症-6名用于下岩窦采样,一个是由于单侧肾上腺切除术后持续性CS,还有一项用于库欣病(CD)的垂体手术。5人接受了dDAVP刺激试验。
    所有8例患者的临床特征是皮质醇增多症和血浆ACTH水平在或高于参考区间,证实下丘脑-垂体介导。所有患者均有异常的低剂量地塞米松抑制试验和深夜唾液皮质醇升高。只有一个人的尿皮质醇排泄增加。与CD相比,测试的5例患者对去氨加压素的ACTH和皮质醇反应减弱或缺失。两个有肾上腺结节,一个有异常的垂体成像。大多数患者报告饮酒不足,其中一名患者否认饮酒。一名患者需要升高血磷脂酰乙醇(PEth)以确认过量饮酒。所有患者的肝功能检查(LFTs)均升高,AST>ALT。
    AIH是一个被低估的人,非肿瘤性皮质醇增多症的可逆原因与肿瘤性CS无法区分。偶然的垂体和肾上腺影像学异常以及饮酒报告不足进一步混淆了诊断。PEth的测量有助于确认酒精使用障碍。LFTs的升高(AST>ALT)和对dDAVP的低常ACTH和皮质醇反应有助于区分AIH和肿瘤性皮质醇增多症。
    Alcohol-induced hypercortisolism (AIH) is underrecognized and may masquerade as neoplastic hypercortisolism [Cushing syndrome (CS)] obscuring its diagnosis.
    In order to characterize AIH, we performed a chart review of eight patients (4 males and 4 females; 2014-2022) referred for evaluation and treatment of neoplastic hypercortisolism - six for inferior petrosal sinus sampling, one due to persistent CS after unilateral adrenalectomy, and one for pituitary surgery for Cushing disease (CD). Five underwent dDAVP stimulation testing.
    All eight patients had clinical features of hypercortisolism and plasma ACTH levels within or above the reference interval confirming hypothalamic-pituitary mediation. All had abnormal low-dose dexamethasone suppression test and increased late-night salivary cortisol. Only one had increased urine cortisol excretion. In contrast to CD, the 5 patients tested had blunted or absent ACTH and cortisol responses to desmopressin. Two had adrenal nodules and one had abnormal pituitary imaging. Most patients underreported their alcohol consumption and one denied alcohol use. Elevated blood phosphatidyl ethanol (PEth) was required in one patient to confirm excessive alcohol use. All patients had elevations of liver function tests (LFTs) with AST>ALT.
    AIH is an under-appreciated, reversible cause of non-neoplastic hypercortisolism that is indistinguishable from neoplastic CS. Incidental pituitary and adrenal imaging abnormalities as well as under-reporting of alcohol consumption further confound the diagnosis. Measurement of PEth helps to confirm an alcohol use disorder. Elevations of LFTs (AST>ALT) and subnormal ACTH and cortisol responses to dDAVP help to distinguish AIH from neoplastic hypercortisolism.
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  • 文章类型: Systematic Review
    背景:垂体脓肿(PA)是一种罕见的疾病,尚不清楚。我们的目的是描述一个病例,并进行全面的系统评价,以探索目前的症状,放射学发现,内分泌异常和死亡率。
    目的:为了识别出现的症状,放射学发现,内分泌异常和PA死亡率的预测因子。
    方法:我们系统回顾了文献,以确定所有PA病例报告。关于陈述的数据,死亡率,放射学发现,内分泌异常和治疗被提取。
    结果:我们从218篇文章中确定了488例符合纳入标准的患者。死亡率为5.1%,报告天数(OR1.0005,95%CI1.0001-1.0008,p<0.01)是唯一确定的死亡率独立预测因子。死亡率随着时间的推移而下降,2000年前发表的病例死亡率较高(OR6.92,95%CI2.80-17.90,p<0.001)。最常见的症状是头痛(76.2%),其次是视野缺陷(47.3%)。只有43%的人出现经典的感染迹象。磁共振成像(MRI)上最常见的成像特征是垂体的高T2和低T1信号,并伴有周围对比增强。超过一半(54.8%)为文化阴性,最常见的细菌是金黄色葡萄球菌(7.8%),真菌是曲霉菌(8.8%)。最常见的内分泌异常是垂体功能减退(41.1%),其次是尿崩症(24.8%)。虽然大多数患者的症状得到了缓解,超过一半(61.0%)的患者存在持续性内分泌异常.
    结论:PA与显著的死亡率相关,延迟就诊会增加死亡风险。持续的内分泌异常是常见的。鉴于非特异性临床表现,MRI上垂体高T2,低T1和周围对比增强的出现应提示考虑这种罕见疾病。
    Pituitary abscess (PA) is a rare condition and not well understood. We aimed to describe a case and perform a comprehensive systematic review to explore presenting symptoms, radiological findings, endocrine abnormalities and mortality.
    To identify presenting symptoms, radiological findings, endocrinological abnormalities and predictors of mortality for PA.
    We systematically reviewed the literature to identify all case reports of PA. Data regarding presentation, mortality, radiological findings, endocrinological abnormalities and treatment was extracted.
    We identified 488 patients from 218 articles meeting the inclusion criteria. Mortality was 5.1%, with days to presentation (OR 1.0005, 95% CI 1.0001-1.0008, p < 0.01) being the only identified independent predictor of mortality. Mortality rates have decreased over time, with cases published prior to 2000 having higher mortality rates (OR 6.92, 95% CI 2.80-17.90, p < 0.001). The most common symptom was headache (76.2%), followed by visual field defects (47.3%). Classical signs of infection were only present in 43%. The most common imaging feature on magnetic resonance imaging (MRI) was high T2 and low T1 signal of the pituitary gland with peripheral contrast enhancement. Over half (54.8%) were culture negative, with the most common bacterial organism being staphylococcus aureus (7.8%) and fungal organism being aspergillus (8.8%). The most common endocrine abnormality was hypopituitarism (41.1%), followed by diabetes insipidus (24.8%). Whilst symptoms resolved in most patients, persistent endocrine abnormalities were present in over half of patients (61.0%).
    PA is associated with significant mortality, with delayed presentation increasing risk of mortality. Ongoing endocrinological abnormalities are common. Given the non-specific clinical presentation, the appearance of high T2, low T1 and peripheral contrast enhancement of the pituitary on MRI should prompt consideration of this rare disease.
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  • 文章类型: Meta-Analysis
    背景:预测垂体手术后长期肾上腺功能不全的可靠策略可以降低糖皮质激素过度暴露或缺失垂体功能不全个体的风险。为此,我们旨在评估术后早期早晨血清皮质醇水平对垂体手术患者下丘脑-垂体-肾上腺轴功能障碍的预测价值.
    方法:进行了一项基于PRISMA的系统评价,包括研究垂体损伤手术后早晨血液皮质醇水平的文章,作为长期补充糖皮质激素给药的决定因素。使用贝叶斯统计来汇集敏感性和特异性率。在术后前两天(POD),还确定了每种潜在皮质醇水平的敏感性和特异性。
    结果:本研究共纳入17篇文献,包括1648名患者。POD-1和POD-2的早晨皮质醇水平对预测术后长期糖皮质激素替代的合并敏感性为86.4%和86.6%,合并特异性为73.1%和78.2%。分别。2.1ug/dl的皮质醇水平显示出最高的敏感性(98.78%),和22.5ug/dl显示对POD-1的最高特异性率(72.5%)。
    结论:在这篇综述和贝叶斯荟萃分析中,我们发现,在进行垂体手术的患者中,术后血清皮质醇测定在预测长期需要使用糖皮质激素方面可能具有较高的准确性.
    A reliable strategy for predicting long-term adrenal insufficiency after pituitary surgery can reduce the risk of glucocorticoid overexposure or missing patients with pituitary insufficiency. For this purpose, we aimed to assess the predictive value of early postoperative morning serum cortisol level for the detection of hypothalamic-pituitary-adrenal axis dysfunction in patients who underwent pituitary surgery.
    A Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-based systematic review was conducted to include articles investigating morning blood cortisol levels after pituitary surgery for lesions of the pituitary gland as a determinant for administration of long-term supplemental glucocorticoids. Bayesian statistics were used to pool the sensitivity and specificity rates. Sensitivity and specificity were also determined for each potential cortisol level on postoperative day (POD) 1 and POD 2.
    The study included 17 articles encompassing 1648 patients. Morning cortisol levels on POD 1 and POD 2 showed pooled sensitivity rates of 86.4% and 86.6% and pooled specificity rates of 73.1% and 78.2%, respectively, for predicting long-term glucocorticoid replacement after surgery. A cortisol level of 2.1 μg/dL showed the highest sensitivity rate (98.78%), and 22.5 μg/dL showed the highest specificity rate (72.5%) on POD 1.
    In this review and Bayesian meta-analysis, we found that postoperative serum cortisol measurement may have high accuracy in prediction of the long-term need for glucocorticoid administration in patients who underwent pituitary surgery.
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  • 文章类型: Review
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  • 文章类型: Systematic Review
    目的:目前对于阻塞性睡眠呼吸暂停(OSA)患者内镜垂体手术后恢复无创正压通气(PPV)的合适时机尚无共识。我们对文献进行了系统回顾,以更好地评估OSA患者术后早期使用PPV的安全性。
    方法:本研究遵循系统评价和荟萃分析(PRISMA)指南的首选报告项目。使用以下关键字搜索英语数据库:“睡眠呼吸暂停,\"\"CPAP,“内窥镜”,\"\"颅底,\"\"经蝶窦\"和\"垂体手术。\"病例报告,社论,reviews,荟萃分析,未发表和仅抽象的文章都被排除在外。
    结果:确定了五项回顾性研究,包括267例接受内镜鼻内垂体手术的OSA患者。在4项研究(n=198)中,患者的平均年龄为56.3岁(SD=8.6),最常见的手术指征是垂体腺瘤切除术。4项研究报告了手术后PPV恢复的时间(n=130),29例患者在两周内接受PPV治疗。在3项研究(n=27)中,与PPV恢复相关的术后CSF漏合并率为4.0%(95%CI:1.3-6.7%),并且在术后早期(<2周)没有与PPV使用相关的气颅报告。
    结论:OSA患者经鼻内镜垂体手术后早期恢复PPV似乎相对安全。然而,目前的文献是有限的。有必要进行更严格的结果报告的其他研究,以评估该人群术后重新开始PPV的真正安全性。
    There is currently no consensus on the appropriate timing of noninvasive positive pressure ventilation (PPV) resumption in patients with obstructive sleep apnea (OSA) after endoscopic pituitary surgery. We performed a systematic review of the literature to better assess the safety of early PPV use in OSA patients following surgery.
    The study followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Databases in English were searched using the keywords: \"sleep apnea,\" \"CPAP,\" \"endoscopic,\" \"skull base,\" \"transsphenoidal\" and \"pituitary surgery.\" Case reports, editorials, reviews, meta-analyses, unpublished and abstract-only articles were all excluded.
    Five retrospective studies were identified, comprising 267 patients with OSA who underwent endoscopic endonasal pituitary surgery. The mean age of patients in four studies (n = 198) was 56.3 years (SD = 8.6) and the most common indication for surgery was pituitary adenoma resection. The timing of PPV resumption following surgery was reported in four studies (n = 130), with 29 patients receiving PPV therapy within two weeks. The pooled rate of postoperative cerebrospinal fluid leak associated with PPV resumption was 4.0% (95% CI: 1.3-6.7%) in three studies (n = 27) and there were no reports of pneumocephalus associated with PPV use in the early postoperative period (<2 weeks).
    Early resumption of PPV in OSA patients after endoscopic endonasal pituitary surgery appears relatively safe. However, the current literature is limited. Additional studies with more rigorous outcome reporting are warranted to assess the true safety of re-initiating PPV postoperatively in this population.
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