肢端肥大症型垂体腺瘤的临床及病理免疫分析

目的探讨肢端肥大症型垂体腺瘤病理免疫反应特点及与临床表现的关系。方法回顾性分析2008年1月至2013年6月采用单鼻孔直达蝶窦入路显微手术切除的84例肢大垂体腺瘤的临床资料,运用免疫化学发光法测定术前基础血内分泌激素水平,采用免疫组织化学的方法检测腺瘤内各种激素免疫反应阳性细胞。结果生长激素(GH)病理免疫反应平均光密度(AOD)值高于泌乳素(PRL)、促卵泡激素(FSH)、促肾上腺皮质激素(ACTH)、促黄体生成素(LH)(P0.05),GH与促甲状腺激素(TSH)比较差异无统计学意义(P0.05)。肿瘤内分泌激素免疫反应阳性表达数量依次为GH62例(74%)、PRL36例(43%)、TSH23例(27%);常见表达类型依次为GH 23例(27%)、GH+PRL 16例(19%)、PRL+TSH 8例(10%)。GH、PRL病理免疫阳性率、血液激素水平升高率及临床表现阳性率分别为74%、99%、100%及43%、15%、10%,差异有统计学意义(P0.05);TSH、ACTH、FSH、LH病理免疫阳性率分别为27%、18%、10%、8%,但临床内分泌激素测定均在正常范围内。结论GH、GH+PRL、PRL+TSH为肢大垂体瘤激素病理免疫反应常见类型,GH免疫表达与血内分泌激素水平及临床表现具有良好的相符性。肿瘤细胞分泌的TSH、PRL可能参与了肢大患者发病的病理生理过程。     

鞍区转移癌六例临床分析

目的鞍区转移癌为临床罕见病,探讨鞍区转移癌的转移来源、临床表现、诊断与鉴别诊断,总结鞍区转移癌的诊治经验。方法与结果共6例患者均为2014年1月至2019年10月行神经内镜或显微镜下经鼻蝶入路鞍区占位性病变切除术后经病理证实的鞍区转移癌,4例以鞍区占位效应首发,2例既往有恶性肿瘤史。临床表现以头痛、视力下降和视野缺损最为常见。病变主要累及海绵窦和垂体。3例病变累及腺垂体的患者表现为腺垂体功能减退症,其中2例入院诊断为无功能性垂体腺瘤。结论鞍区转移癌可作为恶性肿瘤的首发表现,与垂体腺瘤相比更易出现尿崩症、腺垂体功能减退症等,单纯依靠影像学诊断相对局限,临床实践中应结合多方面考虑鉴别诊断,降低误诊率。     

垂体腺瘤伽玛刀治疗对垂体正常功能的影响

目的分析垂体腺瘤病人在γ-刀治疗后垂体功能不全的发生率及时间变化情况。方法回顾性分析了92例垂体瘤病人。结果非功能性腺瘤61例,泌乳素腺瘤18例,生长激素腺瘤9例,促肾上腺皮质激素腺瘤4例。肿瘤平均体积3.8cm3 (0.2～14.6cm3),平均放射剂量15Gy,平均等剂量曲线50.7%,平均随访4.6年(1.2～10年)。治疗后新发生或原有垂体功能症状加重的发生情况如下:不需治疗者:FSH/LH19例(20.6%),TSH32例(34.8%),ACTH10例(10.9%),GH26例(28.3%);需激素替代治疗者:FSH/LH20例(21.7%),TSH22例(23.9%),ACTH8例(8.7%),GH12例(13%)。59例病人于下…     

丘脑出血继发肾上腺皮质功能减退12例分析

目的 探讨丘脑出血继发肾上腺皮质功能减退症的临床特点.方法 回顾性分析丘脑出血继发肾上腺皮质功能减退症12例患者的临床资料.结果 12例患者均出现意识障碍、血糖下降、血电解质紊乱, 24 h尿17-羟皮质类固醇及血浆ACTH 下降.激素替代治疗后所有患者均痊愈.结论 丘脑出血病情加重时应考虑继发肾上腺皮质功能减退症的可能,及时诊治,预后好.     

创伤性脑损伤后垂体功能低下

下丘脑-垂体特殊的解剖和血管结构易导致创伤性脑损伤后垂体功能低下发生,但其确切机制仍然不清楚。常见类型有GH缺乏、LH/FSH缺乏、TSH缺乏、ACTH缺乏、高泌乳素血症、持久性尿崩症等,可单独发生,亦可合并发生。临床表现与创伤性脑损伤后遗症相似,易被误诊。激素检查是诊断创伤性脑损伤后垂体功能低下的主要依据。     

肌萎缩侧索硬化伴发特发性ACTH缺乏一例

特发性ACTH缺乏的病因至今仍然不清楚,然而ACTH缺乏有时与几种自身免疫性疾病有关。因此自身免疫机制可能在其发病机理中发挥作用。有关肌萎缩侧索硬化(ALS)的免疫和内分泌功能障碍已有报道。作者报告一例伴发ACTH缺乏的ALS。 患者男性,70岁。全身疲乏、手肌无力1年,双腿无力近2月,近期发现手肌萎缩而就诊。无嗜酒、毒物接触或皮质类固醇治疗史。无ALS或其他神经疾病的家族史。查体:普通体检未发现异常。神经系统     

颅脑损伤患者血清甲状腺素和和肽素的变化

目的观察颅脑损伤(TBI)患者血清甲状腺激素和和肽素的变化。方法 2015年3月至2016年6月我院重症监护病房收治TBI患者56例,其中重型TBI(GCS评分6~8分)18例,特重型(GCS评分3~5分)38例。选取同期健康体检者30例作为正常对照组。入院24 h采集静脉血离心后取血清,化学发光法测定与垂体功能相关的激素分泌水平,包括游离三碘甲状腺原氨酸(FT3)、四碘甲状腺原氨酸(FT4)、促甲状腺素(TSH)和和肽素。结果与正常对照组相比,TBI患者血清TSH、FT3和FT4浓度分别降低52.60%、49.14%和40.10%,和肽素浓度增加775.45%,差异具有统计学意义(P0.05)。重型TBI患者血清TSH、FT3和FT4浓度均显著高于特重型患者(P0.05),而血清和肽素浓度显著高于特重型患者(P0.05)。结论急性重型、特重型TBI患者早期下丘脑-腺垂体-甲状腺轴功能降低,而下丘脑-神经垂体-抗利尿激素系统功能亢进;下丘脑-神经垂体-抗利尿激素系统功能亢进可能是导致TBI患者预后不良的原因之一。     

产后抑郁症患者HPA轴激素、甲状腺功能及性激素的水平分析

目的探讨产后抑郁患者下丘脑-垂体-肾上腺(HPA)轴激素、甲状腺功能及性激素的水平及其意义。方法选取产后6～7周经我院精神科门诊检测评估为产后抑郁的产妇100例作为抑郁组、100例同期产后未发生抑郁的产妇作为对照组;检测对比两组的HPA激素、甲状腺激素、性激素水平;并分析抑郁组患者各项激素水平与爱丁堡产后抑郁量表(EPDS)评分的关系。结果抑郁组患者的CHR、ACTH水平高于对照组(P0.05),抑郁组患者的CORT水平低于对照组(P0.05);抑郁组患者的TSH水平低于对照组(P0.05),抑郁组患者的TG-Ab、TPO-Ab、FT3、FT4水平与对照组比较,无统计学意义的差异(P0.05);抑郁组患者的E2水平低于对照组(P0.05),抑郁组患者的PRL、P值高于对照组(P0.05);抑郁组患者的E2、TSH、CORT水平与EPDS评分负相关(P0.05),抑郁组患者的PRL、CHR、ACTH测定值与EPDS评分正相关(P0.05)。结论产后抑郁患者自身激素水平异于正常产妇,调节产后激素水平可能有利于改善产后抑郁。     

垂体腺瘤经蝶窦手术中垂体柄和垂体组织的辨认与保护效果

目的分析采用经蝶窦手术治疗垂体腺瘤对垂体柄和垂体组织的辨认和保护,以改善治疗效果。方法对我院收治的35例经蝶窦手术治疗垂体腺瘤的患者进行回顾性分析,统计肿瘤切除率、FT3、FT4、TSH、ACTH 4种激素在手术前后和随访期的水平,及术后并发症。结果垂体腺瘤全切除和次全切除患者共34例,占97.1%;激素检测显示手术对FT3、ACTH并无显著性影响(P0.05),而FT4和TSH在术后及随访期的正常人数显著增加(P0.05);此外,手术后的并发症主要包括尿崩(22.8%)、电解质紊乱(8.6%)、脑脊液漏(2.8%)及垂体功能低下(2.8%)。结论采用经蝶窦入路手术切除垂体腺瘤具有较高的安全性和切除率,且对剩余的垂体组织损伤较小,能很好保护垂体组织的正常生理功能,并发症发生率较低,是一种有效治疗垂体腺瘤的手段。     

难治性抑郁症患者血清胰岛素和神经内分泌激素的对照研究

目的研究难治性抑郁症患者治疗前后血清胰岛素和神经内分泌激素变化。方法难治性抑郁症患者(TRD组)、非难治性抑郁症患者(NTRD组)、健康者(正常对照组)各60例为研究对象,使用HAMD量表评估患者的临床症状,检测患者血清胰岛素、皮质醇(CORT)、促肾上腺皮质激素(ACTH)、三碘甲状腺原氨酸(T3)、游离三碘甲状腺原氨酸(FT3)、甲状腺素(T4)、游离甲状腺素(FT4)、促甲状腺激素刺激激素(TSH)等神经内分泌激素水平,并进行治疗前后各组上述指标的统计学处理。结果 TRD组血清胰岛素、CORT、ACTH、T3、FT3、T4、FT4、TSH与正常对照组存在显著差异,血清胰岛素、CORT、ACTH、FT4与NTRD组存在显著差异;NTRD组血清CORT、ACTH、T3、FT3、T4、FT4、TSH等与正常对照组存在显著差异(P0.05);治疗后,TRD组胰岛素、CORT、ACTH、FT3、FT4、TSH与对照组存在显著差异,而NTRD组CORT与对照组存在显著差异(P0.05)。结论 TRD患者HPA轴功能亢进、HPT轴功能低下等较NTRD患者更为严重,药物治疗后仍存在显著差异。     

脑干海绵状血管瘤术中扩散张量成像联合电生理学监测研究

目的探讨术中扩散张量成像(DTI)联合电生理学监测在脑干海绵状血管瘤切除术中的应用价值。方法共39例脑干海绵状血管瘤患者术中采用DTI和扩散张量纤维束示踪成像(DTT)追踪并重建锥体束,电生理学监测体感诱发电位、运动诱发电位和脑干听觉诱发电位变化。结果 39例患者均顺利完成脑干海绵状血管瘤切除术,术中体感诱发电位异常5例(12.82%);运动诱发电位异常6例(15.38%),2例(5.13%)经DTI证实锥体束体积减少;脑干听觉诱发电位无明显变化。术中MRI显示病变全切除36例(92.31%),次全切除3例(7.69%),术后临床症状改善29例(74.36%)、无明显变化4例(10.26%)、新发面瘫3例(7.69%)、运动障碍加重2例(5.13%),意识障碍合并肺部感染致死亡1例(2.56%)。术后平均随访30个月,Glasgow预后分级5分27例(69.23%)、4分7例(17.95%)、3分4例(10.26%)、1分1例(2.56%)。结论术中联合应用DTI和电生理学监测有助于安全、有效地切除脑干海绵状血管瘤。     

经蝶窦显微外科手术治疗541例库欣病

目的 探讨经蝶窦入路显微外科手术治疗库欣病的疗效。方法 总结北京协和医院神经外科1981年至2007年收治的541例库欣病的临床资料。结果CT和（或）MRI显示微腺瘤、大腺瘤、巨大腺瘤和未见异常的比例分别为66.2％( 358/541)、14.0％( 76/541)、0.6％( 3/541)和19.2％(104/541)。病理为垂体ACTH腺瘤、增生和既未见肿瘤又未见增生的比例分别为75.4％(408/541)、8.3％ (45/541)和16.3％ (88/541)。病理明确腺瘤、增生以及病理阴性者术后近期（半年内）治愈率分别是85.0％( 347/408)、24.4％ (11/45)和45.5％(40/88)。随访0.5 -25.0年（平均3.1年）,复发率13.3％(53/398),复发时间0.5 -16.5年（平均3.4年）。结论 经蝶窦选择性垂体腺瘤切除加瘤周垂体组织大部分切除是治疗库欣病安全有效的首选方法。对首次手术无效或复发性库欣病,可以再次经蝶窦手术或垂体放疗。     

Three patients with hypopituitarism accompanied by primary empty sella presenting mental symptoms

We report here three patients with hypopituitarism accompanied by primary empty sella, whose first manifestations were various mental symptoms. Endocrine studies revealed that two patients showed panhypopituitarism and the other had isolated adrenocorticotropin (ACTH) deficiency. Although several different types of pituitary dysfunctions have been described in a mild form, empty sella is usually asymptomatic. Their first manifestations were mental symptoms; consciousness disturbance, psychomotor agitation, visual hallucination and delusion. Isolated ACTH deficiency is an uncommon disease which etiology is still undetermined. A case with isolated ACTH deficiency associated with an empty sella has been reported before. It is suggested that empty sella might have a role in pathogenesis of isolated ACTH deficiency. The empty sella was confirmed by metrizamide cisternography and magnetic resonance imaging (MRI). These imaging studies are good tools to disclose empty sella. Replacement with cortisone and levothyroxine resulted in an improvement in the mental symptoms in two patients with panhypopituitarism. No alteration was observed following cortisone administration in the patient with isolated ACTH deficiency. Delusion and visual hallucination in this patient poorly responded to treatment with neuroleptics.     

Thyroid axis activity and serotonin function in major depressive episode.

Recent studies in depression have reported alterations in both hypothalamic-pituitary-thyroid (HPT) axis activity and serotonin (5-HT) function; however, the functional relationships between the two systems have not been well defined in patients with major depressive episode. Thyrotropin (TSH) response to 0800 and 2300 h protirelin (TRH) challenges, and adrenocorticotropic hormone (ACTH), cortisol, and prolactin (PRL) responses to D-fenfluramine (D-FEN), a specific 5-HT releasing/uptake-inhibiting agent, were examined in 60 drug-free DSM-IV major depressed inpatients and 20 hospitalized controls. Compared with controls, patients showed lower basal serum 2300 h TSH, 2300 h maximum increment in serum TSH above baseline (delta TSH) and difference between 2300 h delta TSH and 0800 h delta TSH (delta delta TSH) levels. The hormonal responses to D-FEN (i.e. delta ACTH, delta cortisol and delta PRL) were interrelated. No significant difference in basal and post-D-FEN ACTH, cortisol or PRL values were found between controls and patients. A negative relationship between hormonal responses to D-FEN and 2300 h delta TSH and delta delta TSH values was observed in the depressed group. When patients were classified on the basis of their delta TSH test status, patients with reduced delta delta TSH values (i.e. with HPT axis abnormality) had hormonal D-FEN responses comparable to those of controls. Patients with normal delta delta TSH values (i.e. without HPT axis abnormality) showed lower ACTH, cortisol and PRL responses to D-FEN than controls and patients with abnormal delta delta TSH values. These results suggest that: (1) pathophysiological mechanisms other than 5-HT dysregulation may be involved in TSH blunting in major depressed patients; (2) 5-HT function is reduced in some depressed patients, especially those without HPT axis abnormality; and (3) HPT dysregulation may be regarded as a compensatory mechanism for diminished central 5-HT activity.     

垂体腺瘤经蝶窦入路术后继发迟发性低钠血症的危险因素

目的 探讨垂体腺瘤经蝶窦入路术后发生迟发性低钠血症的危险因素。方法 回顾性分析2012年3月~2020年7月经鼻蝶入路手术切除的671例垂体腺瘤的临床资料。结果 术后47例（7.0%）发生迟发性低钠血症,发生时间在术后3~13 d,中位数7.5 d;血钠浓度114~131 mmol/L,平均（124.3±4.8）mmol/L;治疗时间1~9 d,中位数3 d。多因素logistic回归分析显示,女性、术前存在垂体功能减退、术后ACTH轴异常、肿瘤腔高度显著变化是发生迟发性低钠血症的独立危险因素（P<0.05）。结论 迟发性低钠血症是垂体腺瘤术后常见的并发症,尤其是女性、垂体功能减退的病人;肿瘤腔高度显著变化与术后迟发性低钠血症有关,建议肿瘤切除后填塞瘤腔。     

发作性运动诱发性运动障碍临床表型分析

研究背景发作性运动诱发性运动障碍是一组由突然动作诱发的非随意性运动障碍性疾病,表现为反复发作的短暂性肌张力障碍或舞蹈样动作,具有高度临床和遗传异质性。本研究旨在总结中国发作性运动诱发性运动障碍临床表型特点。方法采集195例原发性发作性运动诱发性运动障碍患者临床资料,采用自行设计的发作性运动诱发性运动障碍登记表记录并整理,分析和总结发作性运动诱发性运动障碍临床表型特点,并比较家族性与散发性患者临床表型差异。结果 195例发作性运动诱发性运动障碍患者男女比例为4.42∶1,平均发病年龄为(12.32±3.49)岁,单纯型162例(83.08%)、复杂型33例(16.92%),16例(8.21%)合并特发性震颤,144例(73.85%)发作前有先兆,发作形式包括肌张力障碍(134例,68.72%)、舞蹈样动作(8例,4.10%)和二者混合形式(53例,27.18%),134例(68.72%)发作时面部受累,115例(58.97%)发作频率10次/d、54例(27.69%)10~20次/d、26例(13.33%)20~30次/d,117例(60%)发作持续时间10 s、58例(29.74%)10~30 s、20例(10.26%)30~60 s,散发性131例(67.18%)、家族性64例(32.82%),78例(40%)未服用药物,117例(60%)服用抗癫药物患者中106例症状完全控制、8例偶有发作、3例未见明显缓解。其中,家族性组发病年龄低于(t=2.376,P=0.019)、发作持续时间短于(χ~2=7.731,P=0.021)散发性组。结论通过大样本临床数据分析和总结中国发作性运动诱发性运动障碍临床表型特点,以期为临床诊断与治疗提供帮助。     

Subclinical hyperthyroidism: Physical and mental state of patients

Summary We investigated whether subclinical hyperthyroidism [subnormal basal thyroid-stimulating hormone (TSH) level, attenuated TSH response to thyrotropin-releasing hormone (TRH) stimulation, peripheral thyroid hormones within normal range] is accompanied by physical and mental changes. Thirty-five subclinically hyperthyroid patients (27 female, 8 male) were compared with 60 overtly hyperthyroid patients (51 female, 9 male) and with 28 euthyroid control patients (18 female, 10 male) with respect to physical symptoms, affective state, short-term memory, ability to concentrate and psychomotor performance. Patients with subclinical hyperthyroidism ranged between the other two groups. The major difference between controls and subclinically hyperthyroid patients was an increase in frequency of nervous symptoms and symptoms due to an increase of metabolic rate and thermal regulation changes. The major differences between subclinically hyperthyroid and overtly hyperthyroid patients were psychomotor impairment and symptoms of increased metabolic rate. Self-ratings of affective state tended to be similar in patients with subclinical and overt hyperthyroidism. The ability to concentrate and short-term memory were not impaired in any group. Symptoms in patients with subclinical hyperthyroidism probably result from central changes which lead to attenuated TSH responses to TRH, or from elevated but still normal thyroxine levels, which possibly enhance the effect of catecholamines.     

Dissociation of TSH and adrenocortical disturbance in endogenous depression

To evaluate a possible interaction between the pituitary-thyroid axis and the pituitary-adrenal axis, we measured serum levels of thyrotropin (thyroid stimulating hormone; TSH), the maximal TSH response (Δ max TSH) to thyrotropin releasing hormone (TRH), serum and cerebrospinal fluid (CSF) levels of cortisol, urinary excretion of cortisol, and the plasma levels of adrenocorticotropic hormone (ACTH) in 20 patients with endogenous depression before and after electroconvulsive therapy. No significant correlations were found between serum TSH or Δ max TSH on the one hand, and serum and CSF cortisol or urinary excretion of cortisol on the other hand. A direct correlation was found between serum cortisol and plasma ACTH.     

Psychiatric symptoms in a patient with isolated adrenocorticotropin deficiency: case report and literature review

We report a 59-year-old man with isolated adrenocorticotropin (ACTH) deficiency. The patient presented with sudden onset of delusions and hallucinations at the age of 54, which resolved gradually without treatment. Subsequently, the patient manifested stereotypy, wandering, hypobulia, and autistic symptoms, and was treated with antipsychotics for 1 year without any improvement. He suffered from neuroleptic malignant syndrome-like symptoms at the age of 59. A thorough endocrine assessment revealed isolated ACTH deficiency. After hydrocortisone supplementation, the physical and psychiatric symptoms improved dramatically. Clinicians should consider this rare disease when diagnosing patients with refractory psychiatric symptoms and unique physical symptoms of isolated ACTH deficiency.     

Oxcarbazepine-induced hyponatremia and the regulation of serum sodium after replacing carbamazepine with oxcarbazepine in children

While severe hyponatremia is reported to be more frequent in adults treated with oxcarbazepine (OXC) than with carbamazepine (CBZ), there is not sufficient data about the incidence of hyponatremia in childhood during treatment with OXC. We evaluated changes in serum electrolyte balance in 75 children with epilepsy before and during treatment with OXC and after replacing carbamazepine (CBZ) therapy with OXC therapy. All patients had normal sodium serum levels at the onset of OXC. During treatment with OXC we found hyponatremia (Na +< 135 mmol/l) without clinical symptoms in 26.6 % of the children (n = 20), sodium levels below 125 mmol/l were observed in 2 children (2.6 %). Clinically relevant hyponatremia occurred in one girl only (1.3 %). In a subgroup of 27 children, in whom CBZ was directly replaced with OXC, hyponatremia without symptoms was found in one child under CBZ (3.7 %) and in six children under OXC (22.2 %). Dosage of OXC, serum levels of the active metabolite of OXC, antiepileptic comedication or patients' age and gender were of no predictive value for the development of hyponatremia. Electrolytes should be measured before establishing OXC and if clinically relevant side effects occur.