diabetes insipidus

尿崩症
  • 文章类型: Journal Article
    目的:中心性尿崩症或加压素缺乏症(AVP-D)是经蝶窦手术(TSS)后最常见的水分平衡障碍,在研究中患病率不同。我们旨在确定使用TSS治疗的垂体肿瘤患者中新出现的短暂性或永久性AVP-D的发生率。
    方法:我们对Medline,Embase和CochraneLibrary于2000/01-2021/01/01/31之间进行,用于报告垂体腺瘤结局的研究,TSS术后颅咽管瘤和Rathke裂隙囊肿(RCC),并提供术后AVP-D的定义。我们使用Freeman-Tukey转化随机效应荟萃分析将结果合并为具有95%置信区间(CI)的比例。
    结果:来自11694项研究,包括51个。短暂性或永久性AVP-D的发生率为:全组的17%(95%CI,13-21)和3%(95%CI,2-5),16%(95%CI,12-21)和2%(95%CI,2-3)在垂体腺瘤,颅咽管瘤的31%(95%CI,24-39)和30%(95%CI,22-39),RCC中35%(95%CI,16-57)和14%(95%CI,6-23),分别。根据诊断标准,短暂性或永久性AVP-D的发生率为:低渗多尿,14%(95%CI,8-22)和3%(95%CI,1-4),低渗多尿和高钠血症,21%(95%CI,13-29)和5%(95%CI,2-11),去氨加压素给药,22%(95%CI,15-29)和9%(95%CI,0-30),分别。
    结论:在TSS之后,一小部分垂体腺瘤患者具有永久性AVP-D(2%),但是颅咽管瘤的患病率达到30%,RCC的患病率达到14%。术后AVP-D的诊断标准仍然可变,影响报告的这种情况的发生率。
    OBJECTIVE: Central diabetes insipidus or vasopressin deficiency (AVP-D) is the most frequent water balance disorder after transsphenoidal surgery (TSS) with variable prevalence amongst studies. We aimed to determine rates of newly-developed transient or permanent AVP-D in patients with pituitary tumours treated with TSS.
    METHODS: We performed systematic review of Medline, Embase and Cochrane Library between 01/01/2000-31/01/2021 for studies reporting on outcomes for pituitary adenoma, craniopharyngioma and Rathke\'s cleft cyst (RCC) after TSS and providing definition of post-operative AVP-D. We pooled the results as proportions with 95% confidence intervals (CIs) using Freeman-Tukey transformation random effects meta-analysis.
    RESULTS: From 11694 studies, 51 were included. Rates of transient or permanent AVP-D were: 17% (95% CI, 13-21) and 3% (95% CI, 2-5) in total group, 16% (95% CI, 12-21) and 2% (95% CI, 2-3) in pituitary adenomas, 31% (95% CI, 24-39) and 30% (95% CI, 22-39) in craniopharyngiomas, 35% (95% CI, 16-57) and 14% (95% CI, 6-23) in RCCs, respectively. Based on diagnostic criteria, rates of transient or permanent AVP-D were: for hypotonic polyuria, 14% (95% CI, 8-22) and 3% (95% CI, 1-4), for hypotonic polyuria and hypernatraemia, 21% (95% CI, 13-29) and 5% (95% CI, 2-11), for desmopressin administration, 22% (95% CI, 15-29) and 9% (95% CI, 0-30), respectively.
    CONCLUSIONS: Following TSS, a small proportion of patients with pituitary adenoma have permanent AVP-D (2%), but prevalence reaches 30% in ones with craniopharyngioma and 14% in those with RCC. Diagnostic criteria for post-operative AVP-D remain variable affecting reported rates of this condition.
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  • 文章类型: Case Reports
    尿崩症是一种特征为在血清高渗透压下不适当稀释尿液的病症。两种主要亚型包括中枢型(缺乏加压素产生)和肾性尿崩症(从肾脏抵抗到循环加压素)。常见的表现是过量多尿引起的严重口渴。我们提供了一个病例报告和文献综述,该病例报告和文献综述了一种罕见的中央性尿崩症,称为脂肪(下丘脑)尿崩症,其特点是没有口渴,继发于破裂的前交通动脉瘤的盘绕。由于口渴的丧失,患者发生高钠血症和继发于脱水的并发症的风险增加.我们的病人的病程并发多尿和高钠血症,需要固定剂量去氨加压素方案。关于后续行动,只有部分口渴的感觉得以恢复。我们提供了文献综述,将我们的病例报告与现有的文献进行比较,以扩大对这种罕见的认识,危险,表现。
    Diabetes insipidus is a condition characterized by inappropriately dilute urine in the setting of serum hyperosmolality. The two predominant subtypes include central (from lack of vasopressin production) and nephrogenic diabetes insipidus (from renal resistance to circulating vasopressin). A common manifestation is the significant pursuant thirst from excessive polyuria. We present a case report and literature review of an infrequent variation of central diabetes insipidus known as adipsic (hypothalamic) diabetes insipidus, characterized by the absence of thirst, secondary to coiling of a ruptured anterior communicating artery aneurysm. Due to the loss of thirst, patients are at a heightened risk for hypernatremia and complications secondary to dehydration. Our patient\'s course was complicated by recurrent polyuria and hypernatremia, requiring a fixed-dose desmopressin regimen. On follow-up, only partial thirst sensation was restored. We provide a literature review to compare our case report to the scant literature available to broaden the awareness of this infrequent, perilous, manifestation.
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  • 文章类型: Case Reports
    背景:朗格汉斯细胞组织细胞增生症(LCH)以尿崩症为特征,是一种罕见的发生。病理活检仍有一定的诊断概率。我们介绍了LCH最初影响垂体的情况。这导致病理检查时慢性炎症的误诊。
    方法:一名25岁女性出现尿崩症症状。磁共振成像显示垂体中的病灶增强。手术切除垂体病变后,病理诊断为慢性炎症。然而,患者后来经历了颅骨和下肢骨的破坏。将下肢骨病变与最初的垂体病变进行比较后,最终诊断改为LCH.患者接受了多个化疗疗程。然而,病人的病情逐渐恶化,她最终还是在家里去世了.
    结论:当患者表现为尿崩症,在矢状T1加权图像上垂体腺中没有高信号强度和垂体区域异常增强时,应考虑LCH。
    BACKGROUND: Langerhans cell histiocytosis (LCH) is characterized by diabetes insipidus and is an uncommon occurrence. Pathological biopsies still have a certain degree of diagnostic probability. We present a case in which LCH initially affected the pituitary gland. This resulted in a misdiagnosis of chronic inflammation upon pathological examination.
    METHODS: A 25-year-old female exhibited symptoms of diabetes insipidus. Magnetic resonance imaging revealed an enhanced foci in the pituitary gland. After surgical resection of the pituitary lesion, the pathological diagnosis was chronic inflammation. However, the patient later experienced bone destruction in the skull and lower limb bones. After the lower limb bone lesion was compared with the initial pituitary lesion, the final diagnosis was modified to LCH. The patient was treated with multiple chemotherapy courses. However, the patient\'s condition gradually worsened, and she eventually passed away at home.
    CONCLUSIONS: LCH should be considered when patients exhibit diabetes insipidus and absence of high signal intensity in the pituitary gland on sagittal T1-weighted image and abnormal enhancement in the pituitary region.
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  • 文章类型: Case Reports
    患者男性60岁,病程1年余。以垂体占位引起的中枢性尿崩症起病,伴主动脉周围软组织包绕、腹膜后病变、渗出性多浆膜腔积液、长骨受累,临床表现类似IgG4相关性疾病。查血IgG4浓度正常(0.928 g/L)。左胫骨上段骨病变组织活检病理检查,确诊脂质肉芽肿病(Erdheim-Chester病)。予泼尼松(50 mg每日1次)、沙利度胺(100 mg每晚1次)、去氨加压素(0.05 g每日1次)治疗,1个月后患者气促、多尿症状改善,随访半年患者胸腔积液未再增多。通过对本例的分析和总结,临床医生应提高对脂质肉芽肿病这类少见疾病的关注和认识,了解其与IgG4相关性疾病的鉴别要点,降低误诊和漏诊率。.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    血管加压素输注因其血管收缩特性而在晚期血管舒张性休克状态下常用于重症监护。血管加压素还作用于肾脏收集管中的肾小管细胞受体以允许水重吸收。突然停止加压素输注可导致短暂性尿崩症(DI)的发展,并有多尿的经典发现,稀释尿液,和高钠血症.我们报告了一名59岁的男性,该男性因甲状腺乳头状癌继发于紧急床边环膜切开术,随后发生感染性休克,需要开始输注加压素以支持血液动力学。在临床改善后停止输注之前,他继续使用加压素五天。在加压素停药12小时内,患者出现多尿(>3L/天尿量),尿量高达每小时1L.他的血清钠水平从137增加到149mmol/L,超过10mmol/L。这个案例与以前的报道不同,因为我们的患者没有任何神经系统或神经外科合并症,这将使他成为DI的器质性中心原因。此外,患者的大量利尿和血清异常在24小时内自发自我改善,没有显著的药物干预。总之,该病例增加了越来越多的血管加压素停药后的短暂性DI报告,证明有必要正式认识到这种情况是重症监护中使用加压素的潜在后果。
    Vasopressin infusion is commonly used in intensive care settings during states of advanced vasodilatory shock for its vasoconstrictive properties. Vasopressin also acts on renal tubular cell receptors in the collecting ducts of kidneys to allow for water reabsorption. The sudden discontinuation of vasopressin infusion can lead to the development of transient diabetes insipidus (DI) with classic findings of polyuria, dilute urine, and hypernatremia. We report the case of a 59-year-old male who underwent an emergent bedside cricothyrotomy procedure secondary to papillary carcinoma of the thyroid and subsequently developed septic shock requiring initiation of vasopressin infusion for hemodynamic support. He remained on vasopressin for five days before the infusion was discontinued after clinical improvement. Within 12 hours of vasopressin discontinuation, the patient developed polyuria (> 3 L/day urine output) with volumes as high as 1 L per hour. His serum sodium levels increased more than 10 mmol/L from 137 to 149 mmol/L. This case is unique from prior reports, as our patient was without any neurological or neurosurgical comorbidities that would predispose him to an organic central cause of DI. Furthermore, the patient\'s large-volume diuresis and serum abnormalities spontaneously self-improved within 24 hours without significant medical intervention. In conclusion, this case adds to a growing number of reports of transient DI following vasopressin withdrawal, demonstrating the need to formally recognize this occurrence as a potential consequence of vasopressin use in intensive care settings.
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  • 文章类型: Journal Article
    目的:我们最近通过使用3,4-亚甲二氧基-甲基苯丙胺(MDMA)作为新的激发试验,证明了精氨酸加压素(AVP)缺乏症(中枢性尿崩症)患者的催产素(OT)缺乏症。然而,MDMA激发试验在临床实践中的意义可能具有挑战性.胰高血糖素有效刺激血管加压素能神经元,血浆和肽素强烈增加。因此,我们假设这种挑衅测试也可能刺激OT。
    方法:这是对前瞻性双盲的预定义二次分析,随机化,在巴塞尔大学医院进行的安慰剂对照交叉试验,涉及10名AVP缺乏症患者和10名性别和体重指数匹配的健康参与者,瑞士。每位参与者都接受胰高血糖素测试(皮下注射1mg胰高血糖素)和安慰剂测试(皮下注射0.9%生理盐水)。在基线测量血浆OT水平,注射后60、120和180分钟。主要目的是确定胰高血糖素是否刺激OT以及AVP缺乏患者和健康参与者之间的OT水平是否不同。使用线性混合效应模型在组和条件之间比较主要结果(180分钟内OT的最大变化)。
    结果:在健康参与者中,基线时的中位OT为82.7pg/ml[62.3-94.3],注射胰高血糖素后略有增加至最大93.3pg/ml[87.2-121.1],导致24.9pg/ml的变化增加[5.1-27.8]。同样,在AVP缺乏的患者中,基线时的中位OT为73.9pg/ml[65.3-81.6],注射胰高血糖素后略有增加至114.9pg/ml[70.9-140.9],导致36.8pg/ml的变化增加[-2.2至51.2]。混合模型的结果显示,与安慰剂相比,胰高血糖素对OT没有影响(差异:-0.5pg/ml;95%-CI[-25,24];p=0.97),并且与健康参与者相比,患者之间没有明显的治疗组交互作用(交互作用:28pg/ml;95%-CI[-7,62];p=0.13)。
    结论:我们发现胰高血糖素对血浆OT水平没有影响,AVP缺乏患者和健康参与者之间也没有差异。
    OBJECTIVE: We recently demonstrated an additional oxytocin (OT) deficiency in patients with arginine vasopressin (AVP) deficiency (central diabetes insipidus) by using 3,4-methylenedioxy-methamphetamine (MDMA) as a novel provocation test. However, the implication of the MDMA provocation test in clinical practice might be challenging. Glucagon effectively stimulates vasopressinergic neurons with a strong increase in plasma copeptin. We therefore hypothesized that this provocation test might also stimulate OT.
    METHODS: This is a predefined secondary analysis of a prospective double-blind, randomised, placebo-controlled cross-over trial involving ten patients with AVP deficiency and ten sex- and body-mass index-matched healthy participants at the University Hospital Basel, Switzerland. Each participant underwent the glucagon test (s.c. injection of 1 mg glucagon) and placebo test (s.c. injection of 0.9% normal saline). Plasma OT levels were measured at baseline, 60, 120 and 180 min after injection. The primary objective was to determine whether glucagon stimulates OT and whether OT levels differ between patients with AVP deficiency and healthy participants. The primary outcome (maximum change in OT within 180 min) was compared between groups and conditions using a linear mixed effects model.
    RESULTS: In healthy participants, the median OT at baseline was 82.7 pg/ml [62.3-94.3] and slightly increased to a maximum of 93.3 pg/ml [87.2-121.1] after injection of glucagon, resulting in a change increase of 24.9 pg/ml [5.1-27.8]. Similarly, in patients with AVP deficiency, the median OT at baseline was 73.9 pg/ml [65.3-81.6] and slightly increased after glucagon injection to 114.9 pg/ml [70.9-140.9], resulting in a change increase of 36.8 pg/ml [-2.2 to 51.2]. The results from the mixed model showed no effect between glucagon compared to placebo on OT (difference: -0.5 pg/ml; 95%-CI [-25, 24]; p = 0.97) and no significant treatment-by-group interaction effect between patients compared to healthy participants (interaction: 28 pg/ml; 95%-CI [-7, 62]; p = 0.13).
    CONCLUSIONS: We found no effect of glucagon on plasma OT levels and no difference between patients with AVP deficiency and healthy participants.
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  • 文章类型: Case Reports
    盐酸舍曲林属于选择性5-羟色胺再摄取抑制剂类抗抑郁药,会导致呼吸抑制,低血压,恶性呕吐,肝功能损害,以及其他过量服用时的症状。据我们所知,盐酸舍曲林过量导致患者尿崩症的报告很少见。本报告描述了一个17岁女性患者的独特病例,该患者在后期治疗过程中一次性口服20片盐酸舍曲林片(50mg/片)后出现尿崩症。经垂体后叶素治疗后,患者症状得到有效缓解。
    Sertraline hydrochloride belongs to the selective serotonin reuptake inhibitor class of antidepressants, which can cause respiratory depression, hypotension, malignant vomiting, liver function impairment, and other symptoms when taken in excess. To our knowledge, reports of sertraline hydrochloride overdose causing diabetes insipidus in patients are rare. This report describes a unique case of a 17-year-old female patient who developed diabetes insipidus after a one-time oral intake of 20 sertraline hydrochloride tablets (50 mg/tablet) during the later course of treatment. Her symptoms were effectively relieved after treatment with pituitrin.
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  • 文章类型: Case Reports
    继发性自发性气胸发生在已知潜在肺部疾病的患者中。肺气肿患者,bullae,肺部的囊性病变有发生气胸的高风险。像朗格汉斯细胞组织细胞增生症(LCH)这样的囊性肺病可能会出现气胸等并发症。其他常见的表现特征包括斑丘疹和骨病变。它也可能与内分泌疾病有关,最常见的中枢尿崩症(CDI)。我们在这里介绍一个22岁的男性,他出现了气胸,多尿,和多饮。他在经支气管肺活检时被诊断为LCH,与CDI相关,并采用胸腔镜引导自体血补片治疗持续性漏气和皮下阿糖胞苷。
    Secondary spontaneous pneumothoraces occur in patients with known underlying lung disease. Patients with emphysema, bullae, and cystic lesions in the lungs are at high risk of developing pneumothorax. Cystic lung diseases like Langerhans cell histiocytosis (LCH) can present with complications like pneumothorax. Other common presenting features include maculopapular rashes and bone lesions. It can also be associated with endocrinopathies, most commonly central diabetes insipidus (CDI). We here present a case of a 22-year-old male who presented with pneumothorax, polyuria, and polydipsia. He was diagnosed with LCH on transbronchial lung biopsy, associated with CDI, and was treated with thoracoscopy-guided autologous blood patch for persistent air leak and subcutaneous cytarabine.
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  • 文章类型: Journal Article
    目的:恶性颅内生殖细胞肿瘤(GCT)是西方国家罕见的疾病。它们出现在中线结构中,诊断通常会延迟。我们在MRI上评估了鞍上和双焦点GCT的影像学特征和早期肿瘤征象。
    方法:包括诊断为生殖细胞瘤或非生殖细胞性GCT(NGGCT)的患者,在MRI治疗前接受非对比矢状T1WI。垂体后叶亮点(PPBS)的丧失,肿瘤的扩大和大小,并对周围结构的膨胀和渗透进行了评价。进行组织学和定位的组比较。
    结果:共102例GCT患者(诊断时的中位年龄12.3岁,范围4.4-33.8;57位男性;67位在鞍上定位)被纳入研究。在鞍上队列中,NGGCT(n=20)明显大于生殖细胞瘤(n=47;p<.001)。每个肿瘤均显示后叶或垂体柄受累。在超过90%的每个定位和实体中观察到PPBS损失(总计n=98),并且与尿崩症有关。仅在鞍上GCT中观察到骨浸润(在NGGCT中明显更频繁;p=.004)。在鞍上队列中,第一次MRI和治疗开始之间的时间明显更长(p=0.005),与NGGCT相比,生殖细胞瘤的延迟甚至更大(p=0.002)。最长的治疗间隔是有限的鞍上生殖细胞瘤(中位数312天)。
    结论:PPBS的丢失是肿瘤起源的提示,显示神经垂体中的小肿瘤。在尿崩症患儿中使用此体征可避免诊断延迟。
    OBJECTIVE: Malignant intracranial germ cell tumors (GCTs) are rare diseases in Western countries. They arise in midline structures and diagnosis is often delayed. We evaluated imaging characteristics and early tumor signs of suprasellar and bifocal GCT on MRI.
    METHODS: Patients with the diagnosis of a germinoma or non-germinomatous GCT (NGGCT) who received non-contrast sagittal T1WI on MRI pre-therapy were included. Loss of the posterior pituitary bright spot (PPBS), the expansion and size of the tumor, and the expansion and infiltration of surrounding structures were evaluated. Group comparison for histologies and localizations was performed.
    RESULTS: A total of 102 GCT patients (median age at diagnosis 12.3 years, range 4.4-33.8; 57 males; 67 in suprasellar localization) were enrolled in the study. In the suprasellar cohort, NGGCTs (n = 20) were noticeably larger than germinomas (n = 47; p < .001). Each tumor showed involvement of the posterior lobe or pituitary stalk. A PPBS loss (total n = 98) was observed for each localization and entity in more than 90% and was related to diabetes insipidus. Osseous infiltration was observed exclusively in suprasellar GCT (significantly more frequent in NGGCT; p = .004). Time between the first MRI and therapy start was significantly longer in the suprasellar cohort (p = .005), with an even greater delay in germinoma compared to NGGCT (p = .002). The longest interval to treatment had circumscribed suprasellar germinomas (median 312 days).
    CONCLUSIONS: A loss of the PPBS is a hint of tumor origin revealing small tumors in the neurohypophysis. Using this sign in children with diabetes insipidus avoids a delay in diagnosis.
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