Diabetes Insipidus, Neurogenic

尿崩症,神经性
  • 文章类型: Case Reports
    背景:希恩综合征是一种罕见的疾病,其典型特征是产后休克或出血后垂体前叶功能减退。虽然尿崩症(DI)通常与希汉综合征无关,我们介绍了一例罕见的经产女性在严重产后出血后发展为快速发作的全垂体功能减退症和DI的病例。
    方法:一位以前健康的39岁女性,gravida5,para4,表现为阴道分娩后低血容量性休克,归因于严重的产后出血,需要紧急子宫切除术。尽管她的休克发作在住院期间得到了缓解,她出现了间歇性发热,后来被诊断为肾上腺功能不全。给予氢化可的松有效地解决了发烧。然而,她随后发展为尿崩症。通过功能性激素检查和MRI检查结果证实了Sheehan综合征合并中枢性尿崩症的诊断。治疗包括激素替代疗法,在10年的随访期内发现持续性全垂体功能减退。
    结论:希汉综合征是产后出血的罕见并发症。应该怀疑中枢尿崩症,虽然不常见,患者出现多尿和多饮。此外,应考虑长期激素替代疗法的潜在必要性.
    BACKGROUND: Sheehan\'s syndrome is a rare condition, which is classically characterized by anterior pituitary hypofunction following postpartum shock or hemorrhage. While diabetes insipidus (DI) is not commonly associated with Sheehan\'s syndrome, we present a rare case of a multiparous female developing rapid-onset panhypopituitarism and DI following severe postpartum hemorrhage.
    METHODS: A previously healthy 39-year-old woman, gravida 5, para 4, presented with hypovolemic shock after vaginal delivery, attributed to severe postpartum hemorrhage, necessitating emergent hysterectomy. Although her shock episodes resolved during hospitalization, she developed intermittent fever, later diagnosed as adrenal insufficiency. Administration of hydrocortisone effectively resolved the fever. However, she subsequently developed diabetes insipidus. Diagnosis of Sheehan\'s syndrome with central diabetes insipidus was confirmed through functional hormonal tests and MRI findings. Treatment consisted of hormone replacement therapy, with persistent panhypopituitarism noted during a ten-year follow-up period.
    CONCLUSIONS: Sheehan\'s syndrome is a rare complication of postpartum hemorrhage. Central diabetes insipidus should be suspected, although not commonly, while the patient presented polyuria and polydipsia. Besides, the potential necessity for long-term hormonal replacement therapy should be considered.
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  • 文章类型: Journal Article
    背景:术后中枢性尿崩症(CDI)通常见于颅咽管瘤(CP)患者,炎症反应在CPs中起重要作用。我们旨在评估术前外周炎症标志物及其组合对CPsCDI发生的预测价值。
    方法:回顾性分析208例接受手术治疗的CP患者的临床资料,包括术前外周炎症指标。术前外周血白细胞(WBC),中性粒细胞,淋巴细胞,单核细胞,血小板(PLT),中性粒细胞与淋巴细胞比率(NLR),派生NLR(dNLR),在总共208例CP患者以及不同年龄和手术方式的CP患者亚组中评估了单核细胞与淋巴细胞比率(MLR)和PLT与淋巴细胞比率(PLR).通过受试者操作员特征曲线分析评估其预测值。
    结果:术前外周白细胞,中性粒细胞,NLR,dNLR,MLR,与PLR呈正相关,淋巴细胞与CP患者术后CDI的发生呈负相关,特别是当白细胞≥6.66×109/L或淋巴细胞≤1.86×109/L时同时,多因素logistic回归分析显示,年龄>18岁的患者WBC>6.39×109/L,经颅入路患者WBC>6.88×109/L或淋巴细胞≤1.85×109/L与术后CDI发生率升高密切相关。此外,从受试者操作特征曲线分析获得的曲线下面积显示,炎症标志物的最佳预测因子是总CP患者的NLR,≤18岁年龄组和经蝶窦组的MLR,>18岁年龄组的NLR和经颅组的dNLR。值得注意的是,NLR+dNLR组合指数在所有组中显示出最有价值的预测因子。
    结论:术前外周炎症标志物,尤其是WBC,淋巴细胞和NLR+dNLR,是CP术后CDI的有希望的预测因子。
    BACKGROUND: Postoperative central diabetes insipidus (CDI) is commonly observed in craniopharyngioma (CP) patients, and the inflammatory response plays an important role in CPs. We aimed to evaluate the predictive value of preoperative peripheral inflammatory markers and their combinations regarding CDI occurrence in CPs.
    METHODS: The clinical data including preoperative peripheral inflammatory markers of 208 CP patients who underwent surgical treatment were retrospectively collected and analyzed. The preoperative peripheral white blood cells (WBC), neutrophils, lymphocytes, monocytes, platelet (PLT), neutrophil-to-lymphocyte ratio (NLR), derived-NLR (dNLR), monocyte-to-lymphocyte ratio (MLR) and PLT-to-lymphocyte ratio (PLR) were assessed in total 208 CP patients and different age and surgical approach CP patient subgroups. Their predictive values were evaluated by the receiver operator characteristic curve analysis.
    RESULTS: Preoperative peripheral WBC, neutrophils, NLR, dNLR, MLR, and PLR were positively correlated and lymphocyte was negatively associated with postoperative CDI occurrence in CP patients, especially when WBC ≥ 6.66 × 109/L or lymphocyte ≤ 1.86 × 109/L. Meanwhile, multiple logistic regression analysis showed that WBC > 6.39 × 109/L in the > 18 yrs age patients, WBC > 6.88 × 109/L or lymphocytes ≤ 1.85 × 109/L in the transcranial approach patients were closely associated with the elevated incidence of postoperative CDI. Furthermore, the area under the curve obtained from the receiver operator characteristic curve analysis showed that the best predictors of inflammatory markers were the NLR in total CP patients, the MLR in the ≤ 18 yrs age group and the transsphenoidal group, the NLR in the > 18 yrs age group and the dNLR in the transcranial group. Notably, the combination index NLR + dNLR demonstrated the most valuable predictor in all groups.
    CONCLUSIONS: Preoperative peripheral inflammatory markers, especially WBC, lymphocytes and NLR + dNLR, are promising predictors of postoperative CDI in CPs.
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  • 文章类型: Case Reports
    背景:尿崩症是一种以多尿为特征的综合征,这几乎总是与多饮有关。最常见的原因是中枢性尿崩症,这是抗利尿激素分泌不足的结果,诊断包括将其与多尿和多饮的其他原因区分开来。
    方法:这里,我们提供了一个以前健康的13岁尼泊尔男孩的临床病例,谁,在2022年12月,被发现有强烈的多饮并伴有多尿。他在就诊时患有双侧下肢无力。生化评估显示血清钠升高(181mEq/L),血清肌酐(78μmol/L),和血清尿酸(560μmol/L)与抑制血清钾(2.7mEq/L),这是临床医生最关心的问题。进一步的实验室检查显示,血清渗透压增加(393.6mOsm/kg),尿液渗透压降低(222.7mOsm/kg)。对比大脑的磁共振成像,伴有双侧孔阻塞的厚壁第三脑室囊肿,Sylvius输水管顶部的薄膜状结构伴严重阻塞性脑积水(不活跃),垂体压缩变薄,无亮点。实验室的发现,放射学发现,和病例介绍提供了由于脑积水和第三脑室囊肿引起的尿崩症的临时诊断。
    结论:脑积水引起的中心性尿崩症,虽然罕见,可能有严重的并发症,包括其他垂体激素缺乏的倾向。因此,即使脑积水处于正常颅内压的休眠状态,在对中枢性尿崩症进行调查时必须加以解决.
    BACKGROUND: Diabetes insipidus is a syndrome characterized by polyuria, which is almost always associated with polydipsia. The most frequent cause is central diabetes insipidus, which is the result of an inadequate secretion of the antidiuretic hormone, and diagnosis involves differentiating it from other causes of polyuria and polydipsia.
    METHODS: Here, we present a clinical case of a previously healthy 13-year-old Nepali boy, who, in December 2022, was found to have intense polydipsia accompanied by polyuria. He had bilateral lower limb weakness at the time of presentation. Biochemical evaluation demonstrated raised serum sodium (181 mEq/L), serum creatinine (78 μmol/L), and serum uric acid (560 μmol/L) with suppressed serum potassium (2.7 mEq/L), which was the major concern to the clinicians. Further laboratory workup revealed an increased serum osmolarity (393.6 mOsm/kg) with reduced urine osmolarity (222.7 mOsm/kg). On contrast magnetic resonance imaging of the brain, a thick-walled third ventricular cyst with bilateral foramen obstruction, thin membrane-like structure at top of aqueduct of Sylvius with gross obstructive hydrocephalus (inactive), and compressed and thinned pituitary gland with no bright spot was observed. The laboratory findings, radiological findings, and case presentation provided the provisional diagnosis of diabetes insipidus due to hydrocephalus and third ventricular cyst.
    CONCLUSIONS: Central diabetes insipidus due to hydrocephalus, though rare, can have serious complications including the predilection to develop a deficit of other pituitary hormones. Thus, even if hydrocephalus is dormant with normal intracranial pressure, it must be addressed during investigations of central diabetes insipidus.
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  • 文章类型: Case Reports
    中心性尿崩症(CDI)通常表现为多尿-多饮综合征,其中正常血压通常通过多饮维持。一名53岁的妇女出现糖尿病酮症和高渗性高血糖综合征。她的病史包括疱疹性脑膜脑炎,这与混乱和健忘症有关。在体检时,她是个脱衣舞者,困惑,有细胞外脱水的迹象.她的毛细血管葡萄糖浓度很高,尿液中的酮呈阳性。实验室检查显示严重的高血糖,高钠血症(血浆高渗透压为393.6mOsm/L),轻度急性肾功能衰竭.此外,她患有无症状的COVID-19感染。等渗盐溶液静脉补液和胰岛素治疗可有效控制酮症和改善高血糖。但未能使高钠血症和高渗透压正常化。她不渴,尿量为1升/天,尿渗过少。给药去氨加压素将高钠血症和高渗透压降低到正常范围内,患者的尿渗透压增加到743mOsm/L。因此,诊断为adipsicCDI。内分泌检查显示孤立的中枢甲状腺功能减退症。垂体磁共振成像结果正常。因此,口渴受损的患者可能有不典型的CDI表现.此外,脂肪CDI的诊断尤其具有挑战性.
    Central diabetes insipidus (CDI) typically manifests as a polyuria-polydipsia syndrome, in which normonatremia is generally maintained through the polydipsia. A 53-year-old woman presented with diabetic ketosis and hyperosmolar hyperglycemic syndrome. Her medical history included herpes meningoencephalitis, which was associated with confusion and amnesia. On physical examination, she was apyretic, confused, and had signs of extracellular dehydration. Her capillary glucose concentration was high and her urine was positive for ketones. Laboratory investigations revealed severe hyperglycemia, hypernatremia (plasma hyperosmolarity of 393.6 mOsm/L), and mild acute renal failure. In addition, she had a paucisymptomatic COVID-19 infection. Intravenous rehydration with isotonic saline solution and insulin therapy were effective at controlling the ketosis and ameliorating the hyperglycemia, but failed to normalize the hypernatremia and hyperosmolarity. She was not thirsty and had a urine output of 1 L/day, with urinary hypotonicity. Desmopressin administration reduced the hypernatremia and hyperosmolarity to within their normal ranges, and the patient\'s urinary osmolarity increased to 743 mOsm/L. Therefore, adipsic CDI was diagnosed. Endocrine investigations revealed isolated central hypothyroidism. The results of pituitary magnetic resonance imaging were normal. Thus, patients with impaired thirst may have an atypical presentation of CDI. In addition, the diagnosis of adipsic CDI is particularly challenging.
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  • 文章类型: Case Reports
    背景:伴有大脑皮质受累的渗透性脱髓鞘综合征(ODS)是严重低钠血症矫正的罕见并发症。精心管理低钠血症至关重要,特别是在有危险因素的患者中,如酒精使用障碍和尿崩症。
    方法:一名40多岁的患者在治疗低钠血症期间出现24mEq/L渗透压升高后,出现ODS,有酒精使用障碍和中枢性尿崩症的病史。患者病情进展为锁定综合征,然后在皮质基底节ODS改善后改善为痉挛性四瘫。
    结论:皮质脱髓鞘的鉴别诊断包括层状皮质坏死,解释表观扩散系数(ADC)MRI序列是一个有用的工具。该病例强调需要研究和改进ODS患者的诊断和治疗策略。它还强调了认真纠正低钠血症和频繁监测的重要性,特别是在已知ODS危险因素的患者中。
    BACKGROUND: Osmotic demyelination syndrome (ODS) with cerebral cortical involvement is a rare complication of severe hyponatremia correction. Careful management of hyponatremia is crucial, particularly in patients with risk factors, such as alcohol use disorder and diabetes insipidus.
    METHODS: A patient in his 40s with a history of alcohol use disorder and central diabetes insipidus developed ODS after a 24 mEq/L osmolar increase during the treatment of hyponatremia. The patient\'s condition progressed into locked-in syndrome and then improved to spastic tetraparesis after cortical basal ganglia ODS improved.
    CONCLUSIONS: The differential diagnosis of cortical demyelination includes laminar cortical necrosis, being the interpretation of Apparent Diffusion Coefficient (ADC) MRI sequence is a useful tool.This case underscores the need to investigate and improve diagnosis and treatment strategies in patients with ODS. It also emphasises the significance of careful hyponatremia correction and frequent monitoring, particularly in patients with known risk factors for ODS.
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  • 文章类型: Observational Study
    延迟的术后低钠血症(DPH)是垂体手术后再入院的最常见原因。在这项研究中,我们旨在评估血清和肽素的临界值,并确定和肽素测量的最佳时机,以预测接受内镜经蝶入路(eTSA)手术和肿瘤切除术的患者中DPH的发生.
    这是一项前瞻性观察性研究,对73例患者进行了垂体或柄病变的eTSA手术。术前测量和肽素水平,拔管后1小时,以及术后第1、2、7和90天。
    在73名患者中,23例(31.5%)患者发生DPH。和肽素与血清钠水平的基线比率显示出最高的预测性能(曲线下面积[AUROC],0.699),使Youden指数最大化的最佳临界值为2.5×10-11,灵敏度为91.3%,阴性预测值为92.0%。对于短暂性精氨酸加压素(AVP)缺乏的患者,没有发现明显的预测因素。然而,对于无短暂性AVP缺乏的患者,基线时和肽素与尿摩尔渗透压比表现出最高的预测性能(AUROC,0.725).6.5×10-12的最佳截止值最大化尤登指数,敏感性为92.9%,阴性预测值为94.1%。
    仅在垂体手术后无短暂性AVP缺乏的患者中,可以使用基线和肽素及其与血清钠或尿渗透压的比值来预测DPH的发生。
    BACKGROUND: Delayed postoperative hyponatremia (DPH) is the most common cause of readmission after pituitary surgery. In this study, we aimed to evaluate the cutoff values of serum copeptin and determine the optimal timing for copeptin measurement for the prediction of the occurrence of DPH in patients who undergo endoscopic transsphenoidal approach (eTSA) surgery and tumor resection.
    METHODS: This was a prospective observational study of 73 patients who underwent eTSA surgery for pituitary or stalk lesions. Copeptin levels were measured before surgery, 1 hour after extubation, and on postoperative days 1, 2, 7, and 90.
    RESULTS: Among 73 patients, 23 patients (31.5%) developed DPH. The baseline ratio of copeptin to serum sodium level showed the highest predictive performance (area under the curve [AUROC], 0.699), and its optimal cutoff to maximize Youden\'s index was 2.5×10-11, with a sensitivity of 91.3% and negative predictive value of 92.0%. No significant predictors were identified for patients with transient arginine vasopressin (AVP) deficiency. However, for patients without transient AVP deficiency, the copeptin-to-urine osmolarity ratio at baseline demonstrated the highest predictive performance (AUROC, 0.725). An optimal cutoff of 6.5×10-12 maximized Youden\'s index, with a sensitivity of 92.9% and a negative predictive value of 94.1%.
    CONCLUSIONS: The occurrence of DPH can be predicted using baseline copeptin and its ratio with serum sodium or urine osmolarity only in patients without transient AVP deficiency after pituitary surgery.
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  • 文章类型: Journal Article
    生活质量(QoL)一直是肿瘤学中的一个多因素问题。我们旨在检查颅咽管瘤患者的术前QoL,并探讨QoL参数与临床指标之间的潜在相关性。
    我们共纳入109例颅咽管瘤患者。我们使用了简短表格36(SF-36),症状检查表-90,广义焦虑症问卷量表(GAD7),患者健康问卷抑郁(PHQ9)和匹兹堡睡眠质量指数前瞻性评估他们的QoL。在根据Puget分类划分的亚组之间比较了QoL参数和临床指标。进行了相关分析和回归分析,以检测对自我报告的健康有影响的决定因素。
    与普通人群相比,患者的QoL受损(p<0.001),由SF-36评估。相关性分析表明,由中心性尿崩症(CDI)引起的有害影响。多元线性回归揭示了CDI对精神成分汇总的不利影响(系数=-13.869,p=0.007),GAD7总分(系数=2.072,p=0.049)和PHQ9总分(系数=3.721,p=0.001)。多因素logistic回归分析证实CDI是抑郁症状发生的危险因素(OR=6.160,p=0.001)。
    颅咽管瘤患者手术前QoL明显受损。CDI对患者的QoL产生有害影响,它可能作为早期识别有抑郁症风险的患者的标志。
    UNASSIGNED: Quality of Life (QoL) has been a multifactorial concerning issue in oncology. We aimed to inspect the pre-operative QoL among patients with craniopharyngioma and to explore the potential correlations between parameters of QoL and clinical indices.
    UNASSIGNED: We enrolled a total of 109 patients with craniopharyngioma. We utilized Short Form 36 (SF-36), Symptom Check List-90, Generalized Anxiety Disorder Questionnaire scale (GAD7), Patient Health Questionnaire Depression (PHQ9) and Pittsburgh Sleep Quality Index to prospectively evaluated their QoL. Parameters of QoL along with clinical indices were compared among sub-groups divided according to Puget classification. Correlation analyses and regression analyses were performed to detect influential determinants to self-reported wellness.
    UNASSIGNED: Patients presented impaired QoL compared with general population (p < 0.001), as assessed by SF-36. Correlation analyses indicated the detrimental influence resulting from central diabetes insipidus (CDI). Multivariate linear regression unveiled the adverse effect of CDI on Mental Component Summary (coefficient = -13.869, p= 0.007), GAD7 total score (coefficient = 2.072, p = 0.049) as well as PHQ9 total score (coefficient = 3.721, p = 0.001). Multivariate logistic regression verified CDI as a risk factor of developing depressive symptoms (OR = 6.160, p = 0.001).
    UNASSIGNED: QoL of patients with craniopharyngioma was remarkably compromised before operation. CDI exerted detrimental influences on patients\' QoL and it might serve as a marker for early identification of patients at risk of depression.
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  • 文章类型: Review
    背景:在IgG4相关疾病(IgG4-RD)的初始治疗方面有相对丰富的经验,但是对复发合并多器官和组织受累的病例的治疗措施知之甚少。
    方法:一名43岁的男性,因复发性右泪腺肿大伴眼睑红斑伴尿崩症而先前诊断为IgG4-RD。
    方法:我们进行了垂体磁共振成像,显示垂体后叶边缘变化,垂体柄不均匀增强和结节样增厚,并进行了断水加压素试验,证实了中央性尿崩症,并结合患者的升高的IgG4水平和过去的医疗条件,我们诊断出了中心性尿崩症,IgG4相关的垂体炎,和IgG4-RD。
    方法:患者入院后,我们每天一次静脉注射甲基强的松龙500mg,连续4天,间隔10天后连续4天。在此期间,与霉酚酸酯250毫克每天两次和醋酸去氨加压素0.1毫克,每天3次。
    结果:对患者进行持续6个月的随访,未发现糖皮质激素治疗的副作用,没有复发的迹象,每日尿量稳定在正常范围内。
    结论:我们认识到IgG4水平不能反映复发或长期控制,糖皮质激素休克疗法是复发患者的可选和可靠的治疗策略。
    BACKGROUND: There is a relative wealth of experience in the initial treatment of IgG4-related disease (IgG4-RD), but little is known about therapeutic measures for recurrent cases combined with multiple organ and tissue involvement.
    METHODS: A 43-year-old man with a previous diagnosis of IgG4-RD due to recurrent right lacrimal gland enlargement with eyelid erythema presented with diabetes insipidus.
    METHODS: We performed a pituitary Magnetic Resonance Imaging which revealed posterior pituitary rim changes with inhomogeneous enhancement and nodular-like thickening of the pituitary stalk, and performed a water-deprivation-vasopressin test confirmed central diabetes insipidus, and in combination with the patient\'s elevated IgG4 levels and past medical conditions, we diagnosed central diabetes insipidus, IgG4-related hypophysitis, and IgG4-RD.
    METHODS: After the patient was admitted to the hospital we gave methylprednisolone 500 mg intravenously once daily for 4 days and again for 4 consecutive days after a 10-day interval. During this period combined with mycophenolate mofetil 250 mg twice daily and desmopressin acetate 0.1 mg 3 times daily.
    RESULTS: The patient was followed up for a sustained period of 6 months and no side effects of glucocorticoid therapy were noted, there were no signs of recurrence, and the daily urine output stabilized in the normal range.
    CONCLUSIONS: We recognized that IgG4 levels do not reflect relapse or long-term control, and that glucocorticoid shock therapy is an optional and reliable treatment strategy for relapsed patients.
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  • 文章类型: Journal Article
    和肽素从与精氨酸加压素相同的前体裂解,并响应于相同的刺激,与精氨酸加压素以等摩尔量从垂体后叶释放。它在血液中的稳定水平,快速简单的分析,和易于自动化使得分析比精氨酸加压素更容易,从而提供了在内分泌疾病中测量精氨酸加压素的合适替代方法。研究表明,成人中的和肽素适合于将精氨酸加压素抵抗和精氨酸加压素缺乏与原发性多饮区分开,除了垂体手术后精氨酸加压素缺乏的早期发现;然而,抗利尿激素不适当综合征(SIADH)和儿科人群仍需进一步研究.
    Copeptin is cleaved from the same precursor as arginine vasopressin and is released in equimolar amounts with arginine vasopressin from the posterior pituitary in response to the same stimuli. Its level of stability in the blood, quick and simple analysis, and ease of automation make it much easier to analyze than arginine vasopressin, thereby offering a suitable alternative to measuring arginine vasopressin in endocrine disorders. Research has demonstrated the suitability of copeptin in adults for the differentiation of arginine vasopressin resistance and arginine vasopressin deficiency from primary polydipsia, in addition to the early identification of arginine vasopressin deficiency following pituitary surgery; however, further research is still required in the Syndrome of Inappropriate Antidiuretic Hormone (SIADH) and the pediatric population.
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  • 文章类型: Case Reports
    背景:中心性尿崩症(CDI)是由各种基础疾病引起的罕见疾病,包括肿瘤,自身免疫性疾病,和浸润性疾病。区分CDI病因是困难的。最初被归类为“特发性”中枢性尿崩症实际上可能是各种致病机制的基础,这些机制迄今鲜为人知和/或在最初表现时并不明显。因此,即使特发性CDI在发病时被诊断出来,在监测过程中常见的是肿瘤,如生殖细胞瘤。至关重要的是,生殖细胞瘤的延迟诊断可能与预后较差有关。最近,抗狂犬病蛋白-3A抗体的存在已被发现是淋巴细胞性漏斗神经垂体炎的高度敏感和特异性标志物,自身免疫介导的CDI。
    方法:我们在此介绍两种情况,即,一名13岁的男孩(患者1)和一名19岁的年轻人(患者2)被诊断为特发性CDI。在这两个病人中,全垂体功能减退。磁共振成像显示,CDI发作后约11/2年,垂体柄增厚和垂体肿胀。Westernblotting未显示任何患者血清中存在抗rabphilin-3A抗体,提示可能不涉及自身免疫机制。两名患者随后在病理检查中均被诊断为生殖细胞瘤。他们接受了化疗,其次是放射治疗。值得注意的是,睾酮和胰岛素样生长因子-1水平恢复正常,2例患者放化疗后,性欲和胡须生长恢复。
    结论:我们的数据表明,在临床诊断为特发性CDI的年轻患者中缺乏抗狂犬病蛋白3A抗体可能会增加非淋巴细胞病变发展的可能性,包括生殖细胞瘤.因此,我们建议在这些疾病的发作时采取更专心的方法。
    BACKGROUND: Central diabetes insipidus (CDI) is a rare condition caused by various underlying diseases, including neoplasms, autoimmune diseases, and infiltrative diseases. Differentiating between CDI etiologies is difficult. What has initially been classified as \"idiopathic\" central diabetes insipidus might in fact underlie various pathogenic mechanisms that are less understood to date and/or are not obvious at initial presentation. Therefore, even if idiopathic CDI is diagnosed at the time of onset, it is common for tumors such as germinoma to develop during surveillance. Crucially, a delayed diagnosis of germinoma may be associated with a worse prognosis. Recently, the presence of anti-rabphilin-3A antibodies has been found to be a highly sensitive and specific marker of lymphocytic infundibuloneurohypophysitis, an autoimmune-mediated CDI.
    METHODS: We herein present two cases, namely, a 13-year-old boy (patient 1) and a 19-year-old young man (patient 2) who were diagnosed with idiopathic CDI. In both patients, panhypopituitarism developed. Magnetic resonance imaging revealed pituitary stalk thickening and pituitary swelling approximately 1 1/2 years after the onset of CDI. Western blotting did not reveal the presence of anti-rabphilin-3A antibodies in serum in either patient, suggesting that autoimmune mechanisms might not be involved. Both patients were subsequently diagnosed with germinoma on pathological examination. They received chemotherapy, followed by radiation therapy. Notably, testosterone and insulin-like growth factor-1 levels normalized, and libido and beard growth recovered after chemoradiotherapy in patient 2.
    CONCLUSIONS: Our data suggest that the absence of anti-rabphilin-3A antibodies in young patients clinically diagnosed with idiopathic CDI may increase the probability of the development of non-lymphocytic lesions, including germinoma. We thus recommend a more attentive approach at the onset of these diseases.
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