颅咽管瘤切除术后水钠代谢紊乱机制研究

目的探讨颅咽管瘤术后水钠代谢紊乱的原因和最佳处理方式。方法对102例经胼胝体切开穹窿间入路切除巨大颅咽管瘤的病人，记录术后尿量、血电解质、抗利尿激素（ADH）、醛固酮（ALD）、皮质醇水平，比较术后激素水平变化与水、钠代谢紊乱的关系。结果本组均出现水、钠代谢紊乱，术后2周完全恢复52例，4周基本恢复33例，6周恢复12例，需长时间人工调整电解质水平5例。术后ADH、ALD和皮质醇的不足是导致术后水钠代谢紊乱的主要原因。结论颅咽管瘤切除术后水、钠代谢紊乱与手术损伤下丘脑有关，紊乱类型与ADH、ALD和醛固酮的缺乏情况有关；及时给予相应激素及对症治疗，可获满意疗效。     

颅咽管瘤切除术后水钠代谢紊乱机制研究(附102例分析)

目的探讨颅咽管瘤术后水钠代谢紊乱的原因和最佳处理方式.方法对102例经胼胝体切开穹窿间入路切除巨大颅咽管瘤的病人,记录术后尿量、血电解质、抗利尿激素(ADH)、醛固酮(ALD)、皮质醇水平,比较术后激素水平变化与水、钠代谢紊乱的关系.结果本组均出现水、钠代谢紊乱,术后2周完全恢复52例,4周基本恢复33例,6周恢复12例,需长时间人工调整电解质水平5例.术后ADH、ALD和皮质醇的不足是导致术后水钠代谢紊乱的主要原因.结论颅咽管瘤切除术后水、钠代谢紊乱与手术损伤下丘脑有关,紊乱类型与ADH、ALD和醛固酮的缺乏情况有关;及时给予相应激素及对症治疗,可获满意疗效.     

颅咽管瘤全切除术后钠代谢紊乱及处理

目的分析颅咽管瘤全切除术后钠代谢紊乱的病理生理、诊断及处理。方法对我科86例巨大颅咽管瘤(直径>3.5cm)全切除患者进行回顾性分析,根据血钠、尿钠、渗透压以及中心静脉压等确定钠代谢紊乱类型,并进行相应处理。结果发生低钠血症29例(33.7％,29/86)、高钠血症13例(15.1％,13/86)、高钠及低钠血症交替9例(10.5％,9/86)。高钠及低钠血症平均开始时间分别为术后(2.3±0.7)d和(4.8±1.6)d。2例死于严重下丘脑反应,其余钠代谢紊乱均纠正良好,平均住院时间26 d。结论钠代谢紊乱是颅咽管瘤全切除术后常见表现之一。高钠血症与尿崩症关系密切,而低钠血症主要有两个原因:脑性盐耗综合征和抗利尿激素不适当分泌综合征,二者要仔细区分,前者着重补液、补盐,而后者应在补盐的基础上要适当限水和利尿。     

颅咽管瘤术后水电解质平衡紊乱的治疗

目的探讨颅咽管瘤切除术后水电解质平衡紊乱的临床特点及治疗方法。方法36例行颅咽管瘤切除术患者,手术当日及术后每日定时检测电解质并观察尿量变化,根据其变化进行治疗。结果25例术后并发尿崩症,其中18例两周内恢复,6例4周内恢复,1例1年内恢复。电解质紊乱30例中,24例术后4周恢复,5例2个月内恢复,1例半年内恢复。结论颅咽管瘤患者术后绝大多数会出现水电解质代谢紊乱,尿崩症大多数在术后早期恢复,电解质异常需严密监测,针对监测结果进行治疗,效果满意。     

颅咽管瘤全切术后抗利尿激素分泌异常综合征的治疗

目的 探讨颅咽管瘤全切术后抗利尿激素分泌异常综合征(SIADH)的临床特点及治疗方法。方法 120例颅咽管瘤切除术病人，112例肿瘤全切除，手术当日及术后每日定时检测血钠及观察尿量变化。17例病人术前、术后当日、术后3d、7d、14d检测血清抗利尿激素(ADH)水平，并进行比较观察。结果 ①72例(60％)术后出现以低血钠为主要特征的抗利尿激素分泌异常综合征。其中37例(51.4％)单纯性低血钠，29例(40.3％)尿崩症伴高血钠后转为低血钠，6例(8.3％)低血钠后转为尿崩症伴高血钠。②17例病人术前、术后当日、术后3d、7d、14d血清ADH放射免疫学测定，示术后SIADH病人血清ADH水平与术前相比有所增高，但是经统计学分析无显性差别。结论 颅咽管瘤全切术后SIADH为常见术后合并症，可能是由于颅咽管瘤术后下丘脑ADH的释放不能随着体内晶体渗透压变化而改变所致。术前和术后2周内血清ADH水平变化没有显性差异。     

儿童颅咽管瘤手术治疗和长期随访

目的探求在保护下丘脑功能的条件下，安全地伞切或近全切儿童颅咽管瘤。并长期随访术后病儿的生存情况。方法手术切除儿童颅咽管瘤202例。采用经胼胝体一透明隔间隙一穹隆问入路、经额部纵裂入路等八种入路切除鞍区和第三脑室内的颅咽管瘤。术前和术后采取积极措施预防和治疗下丘脑功能紊乱。并对其中的105例进行了12个月到6年的长期随访。结果全切＋近全切184例（91．1％），死亡2例（0．9％）。术后主要并发症为：尿崩症发生率80．7％，长期尿崩症率11．4％；血电解质紊乱74．8％；癫痫9．4％。术后第1年和第4年是死亡高峰。手术后5年生存率68．2％。结论选择合适的手术入路在直视下尽可能全切除颅咽管瘤，并保护第三脑室前下外侧壁（下丘脑）。术后积极防治血钠紊乱和癫痫是确保手术安全的重要措施。术后放疗可明显降低颅咽管瘤的复发率。     

颅咽管瘤术后并发症的防治

目的探讨颅咽管瘤切除术后并发症的特点和处理原则。方法回顾性分析82例颅咽管瘤病人的临床资料,均经手术治疗,术后监测水、电解质和血糖变化,并记录24 h尿量。结果肿瘤全切除75例,次全切除7例。颅咽管瘤术后并发症较多,其中尿崩症78例,钠代谢紊乱74例,垂体功能低下67例,高热17例,精神障碍11例,意识障碍4例,深静脉血栓4例,癫癎3例。随访17-36个月,平均28个月,恢复正常生活和学习68例,仅能生活自理8例,不能生活自理4例,死亡2例。结论颅咽管瘤术后并发症较多,早期监测和及时处理可进一步提高该病的治愈率。     

儿童颅咽管瘤术后水电解质紊乱的管理方案

目的 探讨儿童颅咽管瘤术后水电解质紊乱的管理方案。方法 回顾性分析2016年12月到2019年5月手术治疗的56例儿童颅咽管瘤的临床资料。术后高钠血症使用生理盐水补充每日钠需要量+5%葡萄糖溶液降血钠,术后出现重度低钠血症使用3%盐水微量泵持续补钠。结果 术后出现尿崩症40例（71.4%）,电解质紊乱30例（53.6%）。高钠血症患儿治疗第一天血钠波动幅度平均为（7.8±2.0）mmol/L,第二天平均为（5.9±2.2）mmol/L,控制时间平均（76.5±11.7）h。重度低钠血症患儿治疗第一天波动幅度平均为（7.7±2.0）mmol/L,第二天平均为（7.5±3.1）mmol/L,控制时间平均（89.8±14.2）h。治疗过程中,未出现意识障碍、癫痫、脱髓鞘等并发症。出院时,40例尿崩症患儿的尿量得到控制,30例电解质紊乱患儿连续3次血清Na+正常。结论 儿童颅咽管瘤术后电解质紊乱发生率高,稳定血清钠波动幅度,有助于改善病人预后。     

颅咽管瘤切除术后常见并发症的处理

目的 探讨颅咽管瘤切除术后常见并发症的特点和处理。方法 对我科80例颅咽管瘤切除患者的临床资料进行回顾性分析。结果 术后出现尿崩72例，出院时39例尿量恢复正常，仍有尿崩者33例；钠代谢紊乱58例，其中低钠血症31例，高钠血症14例，高钠、低钠交替13例，出院时血钠正常者51例，仍有钠代谢紊乱者7例；高热34例，出院时体温均正常；意识障碍20例，1例因长期昏迷死于循环功能衰竭，出院时11例意识清楚，8例仍有意识障碍；癫痫5例。1例死于癫痫持续状态，余4例得到良好控制。结论 处理尿崩时要合理使用抗利尿制剂。引起低钠的原因有脑性盐耗综合征和抗利尿激素异常分泌综合征，治疗前者应补液、补盐。后者应适当限水、利尿。高钠血症要限钠、补液并给予抗利尿制剂。发生高热应及时降温。出现意识障碍，除做CT检查确定有无颅内血肿和脑积水外。还应从电解质和激素用药方面查找原因。癫痫是颅咽管瘤术后极危险的并发症，要及时控制。避免出现癫痫持续状态。     

颅咽管瘤围手术期内分泌变化的影响因素及激素替代治疗

目的 研究颅咽管瘤围手术期内分泌变化影响因素及激素替代治疗.方法 回顾分析2002年1月至2006年8月手术治疗的47例颅咽管瘤患者.术前采血,测定TSH、T3、T4、PRL、FSH、LH、E2、T值,8 am、4 pm分别检测血皮质醇1次,并于术后1周对上述指标进行常规复查.手术前后监测尿量、尿比重.将所得数据进行统计学分析.结果 术后TSH、性腺激素低下和尿崩的出现率显著高于术前,高PRL出现率明显低于术前;手术前、后甲状腺功能低下差异无统计学意义.结论 颅咽管瘤术后内分泌功能紊乱加重;开颅手术及肿瘤全切除明显影响术后内分泌变化;颅咽管瘤术后需常规激素替代治疗,围手术期肾上腺皮质激素的替代治疗可明显减少低皮质醇血症的发生.     

Long-term effects of treatment on endocrine function in children with brain tumors

Fourteen children with brain tumors received endocrine evaluations at least one year following completion of cranial irradiation. Treatment consisted of operation (13 patients), craniospinal irradiation (6), whole brain irradiation (5), posterior fossa irradiation (3), and chemotherapy (10). Endocrine evaluation included bone age roentgenography and measurement of growth hormone (using sequential arginine and insulin stimulation), thyroxine, thyroid-stimulating hormone, plasma cortisol, testosterone, prolactin, and urinary follicle-stimulating hormone and luteinizing hormone. Ten of 12 children (83%) had abnormal responses to both tests of growth hormone stimulation. All growth hormone-deficient patients treated prior to puberty and tested at least 2 years following completion of cranial irradiation had decelerated linear growth. Results of thyroid function tests were abnormal in 4 patients: 2 patients had evidence of primary hypothyroidism, and 2 showed secondary or tertiary hypothyroidism. Two patients had inadequate cortisol responses to insulin hypoglycemia. Urinary follicle-stimulating hormone and luteinizing hormone, serum prolactin, and serum testosterone levels were appropriate for age in all patients.     

垂体影像学特征在无功能性垂体腺瘤术后内分泌功能评估中的作用

目的 探讨垂体影像学特征在无功能性垂体腺瘤（NFPA）术后内分泌功能评估中的作用。方法 回顾性分析2010年3月~2021年3月经鼻蝶入路神经内镜手术切除的96例NFPA的临床资料。术前、术后2个月、术后12个月评估激素功能。结果 术前54例（56.3%）出现垂体功能减退,术后2、12个月分别有67例（69.8%）和55例（57.3%）存在垂体功能减退。与术前相比,术后2、12个月,性腺功能减退、甲状腺功能减退、生长激素缺乏无明显变化（P>0.05）,高泌乳素血症明显减少（P<0.05）,但是肾上腺皮质功能减退、尿崩症术后2个月明显增加（P<0.05）、术后12个月又明显减少（P<0.05）。19例（19.8%）术后12个月出现少一个激素轴功能恶化。单因素分析显示,术前垂体柄是否可见与术后垂体功能恶化无明显关系（P>0.05）,术前垂体腺可见程度、肿瘤切除程度、肿瘤体积与术后激素恶化有关（P<0.05）。多因素logistic回归分析显示术前垂体腺不可见是术后激素恶化的独立影响因素（P<0.05）。结论 垂体功能减退是NFPA常见的临床表现,术前垂体功能评估具有重要意义。术后垂体激素轴可能恶化,垂体影像学特征,例如垂体腺可见程度、肿瘤大小等,可作为术后垂体功能恶化的评估指标。     

Seizures, hormones and sexuality.

Sexual disorders (both hyposexuality and sexual dysfunction) are common in people with epilepsy, occurring in up to two-thirds of patients. However, characteristically, patients do not spontaneously report these problems. Nocturnal penile tumescence testing suggests that the erectile dysfunction has a neurophysiological component. The aetiology remains uncertain but is likely to be multifactorial, involving neurological, endocrine, iatrogenic, cognitive, psychiatric and psychosocial factors. Epilepsy-related factors include the age of onset/duration of epilepsy along with the seizure type and focus. In addition, seizure frequency might be relevant as successful epilepsy surgery can result in an improvement in sexual functioning despite remaining on anticonvulsant medication. Endocrine changes (raised sex hormone binding globulin and reduced free testosterone) have been reported in men with epilepsy, especially when treated with hepatic-enzyme inducing antiepileptic drugs. Studies have not been performed evaluating anticonvulsants that do not induce hepatic enzymes such as lamotrigine. The association between these endocrine changes and hyposexuality is not known. The relationship between seizures, hormones and anticonvulsant medication in women is explored, focusing on issues such as catamenial epilepsy, the menopause, hormone replacement therapy and the polycystic ovarian syndrome. Suggestions for future research and treatment issues are discussed.     

Endocrine disorders in two sisters affected by MELAS syndrome

A variety of endocrine and metabolic defects, including hypothalamopituitary hypofunction and diabetes mellitus, has been reported in association with mitochondrial disorders. We describe two sisters affected by mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes (MELAS) syndrome in whom DNA analysis showed an A-->G transition at the 3243rd nucleotide position on the transfer RNALeu(UUR) gene with 65% and 45% of mutant-type mitochondrial DNA present in the blood cells of the younger and the older sister, respectively. The younger sister had severe involvement of the central nervous system with mental retardation, epilepsia partialis continua, and strokelike episodes. Endocrine investigations showed an extensive neuroendocrine dysfunction with growth hormone deficiency, hypothalamopituitary hypothyroidism, prepubertal gonadotropin levels, and absence of any secondary sexual characteristics at the age of 12 6/12 years. The neurologically normal older sister was affected by diabetes mellitus and had normal hypothalamopituitary function. Our report confirms that the endocrine system can be affected differently by the same mitochondrial DNA mutation, depending on the heteroplasmia phenomenon. A complete endocrine evaluation must be performed in patients affected by mitochondrial disease and the existence of a mitochondrial disorder should be taken into account in patients with endocrine abnormalities, even if neuromuscular signs are lacking.     

Myopathies of endocrine disorders: A prospective clinical and biochemical study

Introduction:

Major categories of endocrine myopathy include those associated with: Adrenal dysfunction (as in Cushing''s disease or steroid myopathy); thyroid dysfunction (as in myxedema coma or thyrotoxic myopathy); vitamin D deficiency; parathyroid dysfunction; and pituitary dysfunction. Steroid myopathy is the most common endocrine myopathy.

Objective:

To study the etiology, varied presentations, and outcome after therapy of patients with endocrine myopathies.

Materials and Methods:

Myopathy was evaluated by the standard clinical procedures: Detailed clinical history, manual muscle strength testing, and creatine phosphokinase (CPK). Endocrine disorders were diagnosed as per clinical features and biochemical parameters. The treatment was given to patients as per underlying endocrine disease. Myopathy was assessed before and after treatment.

Results:

Out of the 37 patients who were diagnosed with endocrine myopathies, thyroid dysfunction was the most common cause (17 cases), followed by vitamin D deficiency in nine, adrenal dysfunction in six, parathyroid dysfunction in three, and pituitary dysfunction in two. Some patients had atypical presentation (repeated falls in one, tongue fasciculations in one, neck weakness in five, one with ptosis and facial weakness, asymmetrical onset in one, and calf hypertrophy in one. The serum creatine kinase (CK) concentration did not correlate with muscle weakness. Following the treatment regimen which was specific for a given myopathy, 26 patients recovered fully.

Conclusion:

We found varied clinical presentations of endocrine myopathies. All the patients with neuromuscular complaints should be investigated for endocrine causes because significant number of them recovers fully with specific treatment.     

Endocrine alterations in critically III patients with stroke during the early recovery period

Introduction: Endocrine abnormalities in critically ill patients with stroke during the early recovery period have not been well characterized. Methods: To investigate this issue, 33 consecutive mechanically ventilated patients (27 men) with hemorrhagic (n=21) or ischemic (n=12) stroke having a mean age of 57 ± 12 years were studied. Glasgow Coma Scale score on admission in the hospital was 8 ± 3. The following basal hormones were measured within 72 hours postextubation: cortisol, corticotropin (ACTH), free thyroxine, tri-iodothyronine, thyroid-stimulating hormone, testosterone, estradiol, follicle-stimulating hormone, luteinizing hormone, growth hormone, and insulin-like growth factor (IGF)-1. Subsequently, a low-dose (1 μg) ACTH stimulation test was performed. Results: Twenty-six (79%) patients showed endocrine alterations. The most common change was low IGF-1 levels compatible with growth hormone deficiency (45%), followed by hypogonadism (39%), thyroid dysfunction (36%), and cortisol hyporesponsiveness (33%). Conclusion: Neuroendocrine changes occur with high frequency in critically ill patients with stroke during the early recovery period. It remains to be determined whether these changes have implications for functional and/or clinical outcome.     

万拉法新对抑郁症患者血清性激素的影响

目的 探讨万拉法新对抑郁瘟患血清性激素的影响。方法 用放免法测定42例抑郁症患治疗前和治疗后1、6周末血清催乳素(PRL)、促滤泡成熟激素(FSH)、促黄体生成素(LH)、睾酮(T)、雌二醇(E2)，并与30例健康对照。结果 抑郁症组男性LH、E2和女性PRL、FSH与对照组比较明显升高。治疗后1、6周末男性患PRL、T、E2和女性PRL、T有显差异。结论 抑郁症患存在下丘脑—垂体—性腺轴内分泌异常，万拉法新可引起抑郁症患性激素分泌变异。     

Disturbances in the hypothalamo-pituitary-adrenal and other neuroendocrine axes in bulimia

Disturbances in the hypothalamo-pituitary-adrenal (HPA) and other endocrine axes were assessed in 24 women with bulimia and healthy controls. Overnight blood samples for measuring nocturnal plasma cortisol, prolactin (PRL), growth hormone (GH), luteinizing hormone (LH), and follicle stimulating hormone (FSH) were obtained at 30-min intervals. A 1.5 mg dexamethasone suppression test (DST) and a TRH-test were performed. Patients were monitored closely while their nutritional intake was recorded over 21 days. Compared with healthy controls, nocturnal cortisol plasma levels were not elevated in the bulimics. There was a trend toward insufficient cortisol suppression in the DST in patients with bulimia, which was most pronounced in patients with signs of restricted caloric intake. Plasma dexamethasone levels were significantly reduced in bulimics compared with healthy controls. There was a trend for blunted thyrotropin stimulating hormone (TSH) responses to thyrotropin releasing hormone (TRH) in bulimia. The prolactin response to TRH was significantly reduced in bulimics with a history of anorexia nervosa. Plasma LH and plasma FSH were significantly reduced in bulimics with signs of reduced caloric intake [low T3, high levels of beta-hydroxy-butyric acid (BHBA), reduced daily caloric intake, high number of fasting days] as compared with healthy controls. Bulimics with high BHBA levels had significantly reduced nocturnal prolactin plasma levels. Results show that multiple neuroendocrine disturbances exist in bulimia in a milder form than in anorexia nervosa. Evidence for the impact of caloric intake on endocrine functions is presented. Endocrine dysfunctions in our bulimic sample did not show a positive association with the presence of depressive symptoms.     

Plasma growth hormone response to insulin-induced hypoglycemia in infantile autism: A pilot study

Plasma growth hormone responses to insulin-induced hypoglycemia were examined in eight preschool-age autistic children. Six of these children were examined on two separate occasions: during the period of baseline evaluation and after 4 weeks of daily haloperidol administration. On at least one occasion, half of this small sample exhibited persistent elevation of growth hormone levels, with a failure to return to baseline values over the course of a 135-minute period postinsulin infusion. These data are consistent with the occurrence of hypothalamic dysfunction in subgroups of autistic children. Short-term administration of haloperidol did not diminish the ability of the pituitary to secrete growth hormone in response to insulin-induced hypoglycemia.This study was supported in part by Public Health Service Grant MH-32212 from the National Institute of Mental Health.