Behavioural changes due to pontine and extrapontine myelinolysis.

OBJECTIVE: To report a case of central and extrapontine myelinolysis with unusual behavioural manifestations that developed after slow (not more than 0.5 mmol sodium per litre per hour) correction of hyponatraemia. CLINICAL FEATURES: A 51-year-old Caucasian woman with hyponatraemia caused by psychogenic polydipsia experienced a delayed onset of behavioural changes with some ataxia, but no pyramidal signs, after correction of the hyponatraemia. INTERVENTION AND OUTCOME: The hyponatraemia was corrected with a combination of hypertonic saline and fluid restriction at the currently recommended rate of not more than 0.5 mmol of sodium per litre per hour. CONCLUSION: Central and extrapontine myelinolysis may develop after slow correction of hyponatraemia. Behavioural manifestations may be the most prominent clinical feature.     

Obliteration of empty sella for therapy of empty sella syndrome by transsphenoidal and tranfrontal approach

作者报道７例空蝶鞍综合征，其中原发性２例，继发性５例。经蝶窦进路充填法手术治疗５例，经额进路治疗２例。除１例因有原发性脑积水未愈外，余６例治愈。本病与鞍隔缺损、良性颅内高压、长期服用嗅隐亭、垂体手术或放疗后有关。临床以头痛、视力视野损害和垂体功能低下为主要特点。经蝶窦充填法治疗是简单有效的方法。     

Avoiding common problems associated with intravenous fluid therapy

Inappropriate intravenous fluid therapy is a significant cause of patient morbidity and mortality and may result from either incorrect volume (too much or too little) or incorrect type of fluid. Fluid overload has no precise definition, but complications usually arise in the context of pre-existing cardiorespiratory disease and severe acute illness. Insufficient fluid administration is readily identified by signs and symptoms of inadequate circulation and decreased organ perfusion. Administration of the wrong type of fluid results in derangement of serum sodium concentration, which, if severe enough, leads to changes in cell volume and function, and may result in serious neurological injury. In patients whose condition is uncomplicated, we recommend a restrictive approach to perioperative intravenous fluid replacement, with initial avoidance of hypotonic fluids, and regular measurement of serum concentration of electrolytes, especially sodium.     

42例儿童期起病的成人生长激素缺乏症的临床分析

目的 探讨儿童期起病的成人生长激素缺乏症(Co-AGHD)的病因分布、临床特点及治疗情况。方法 回顾性分析2012年7月至2014年12月北京协和医院内分泌矮小门诊定期随诊的42例Co-AGHD患者出生情况、病因、临床特点、影像检查及治疗情况。根据是否接受重组人生长激素(rhGH)替代治疗,分为治疗组(n=12例)和未治疗组(n=30例),评价治疗效果。结果 男性患者的比例高于女性。先天性病因39例,其中垂体发育不良20例,合并空泡蝶鞍3例,部分空泡蝶鞍1例,其他病因不详。获得性病因3例,均为颅咽管瘤术后患者。两组rhGH替代治疗在改善肝功能异常和血脂异常方面无明显差异(P>0.05)。结论 Co-AGHD病因多是先天性,其中大多患者出生史异常并伴有垂体发育不良。AGHD易合并多种代谢相关疾病。rhGH替代治疗在改善肝功能异常和血脂异常方面的有效性仍需进一步证实。     

Symptomatic hypopituitarism revealing a primary empty sella turcica.

A 64-year-old nulliparous woman presented with clinical signs of thyroid and adrenocortical insufficiency. Subsequent hormonal investigations demonstrated a failure of all anterior pituitary functions. Pneumotomo-encephalography revealed a large arachnoid herniation, leading to the diagnosis of primary empty sella turcica syndrome with secondary panhypopituitarism. This unusual observation emphasizes the necessity of ruling out an empty sella turcica syndrome in patients with pituitary insufficiency.     

Primary thyroid failure presenting as a pituitary tumour

A 20-year-old female was referred as a pituitary tumour with failure to grow. Primary hypothyroidism from the age of 8 had been diagnosed at the age of 11, when the patient was put on thyroid replacement therapy. Routine investigations showed normal anterior pituitary function, and confirmed the diagnosis of primary hypothyroidism. A needle biopsy of the pituitary gland revealed an empty fossa. The possible relation of the empty sella with the primary hypothyroidism is discussed. A chromosome anomaly, deletion of the short arm of an X chromosome, was found and its contribution to the delayed growth is discussed.     

枸橼酸钠置换液在血液滤过中的抗凝效果评估

目的评估枸橼酸钠置换液在血液滤过中的抗凝效果。方法随机对照的实验研究,每组健康犬6只。枸橼酸钠组:配制含1.5 mmol/L枸橼酸钠抗凝剂的置换液,在血滤管路静脉端持续补钙对抗枸椽酸根;肝素组:使用低分子肝素(20 U/kg.h)持续泵入的抗凝方法。分别对急性肾功能衰竭的动物行血液滤过治疗,血流量为50 ml/min,置换液速度为500 ml/h。观察使用枸橼酸钠置换液后及补钙后血滤管道的凝血功能(PT、PT%、PT/R、PT INR、APTT、AT3%及Fbg等)的变化,记录血滤管路使用时间,以及血液滤过结束时的血清肌酐、尿素、电解质、酸碱平衡,血滤器前、以及体内凝血功能指标的变化。结果血液滤过治疗过程中,使用枸橼酸钠置换液后血滤管道中血液的凝血指标(PT、PT%、PT INR、APTT)延长,补钙后恢复正常水平,体内静脉血的凝血指标均恢复正常范围内;两组均未有血滤管路和血滤器堵塞现象,实验犬的血浆电解质、酸碱平衡、肌酐、尿素等结果两组无明显差异(P<0.01),肝素组动物体内的部分凝血指标(PT、PT INR、APTT)延长(P<0.01)。结论经研究配制后的枸橼酸钠置换液应用血液滤过治疗时,通过血滤器时...     

Intrasellar abscess simulating a pituitary tumour

A case of an abscess of the sella turcica discovered during craniotomy for pituitary tumour in a 20-year-old man is presented. The clinical features of this unusual intrasellar lesion are discussed. The importance of early diagnosis, high-dosage antibiotics and surgical intervention in reducing the high mortality associated with abscesses in this location are emphasized. It is suggested that abscess of the sella turcica should be considered in the differential diagnosis of a sella mass in any patient with a recent history of cerebrospinal fluid rhinorrhea, sphenoid sinusitis or meningitis.     

MRI对原发性空蝶鞍综合征的诊断价值

目的探讨磁共振成像(magnetic resonance imaging,MRI)对原发性空蝶鞍综合征的诊断价值.方法对有头颅MRI检查并有完整临床资料的原发性空蝶鞍患者36例进行研究.采用矢状面T1WI、冠状面T1WI,横断面T1WI、T2WI,9例行钆对比增强扫描.结果原发性空蝶鞍综合征患者蝶鞍的MRI表现主要有鞍区增大、鞍底下陷、鞍内为典型的长T1、长T2脑脊液信号;垂体受压变扁紧贴于鞍底,受压垂体信号无异常改变,垂体柄延长直达鞍底.其临床表现主要有:顽固性头痛、头晕、视力障碍、内分泌紊乱等症状.结论原发性空蝶鞍综合征具有典型的MRI表现,MRI可清晰显示空蝶鞍,MRI应作为原发性空蝶鞍综合征的首选影像学检查方法.     

鞍区肿瘤术后低钠血症临床诊断和处理

目的探讨鞍区肿瘤手术后并发尿崩症以及低钠血症的病因、发病机制、诊断、治疗经验。方法回顾分析34例鞍区肿瘤术后尿崩症以及低钠血症患者,通过其临床表现以及实验室检查确立诊断,总结有效的治疗方法。结果除1例患者继发细胞毒性脑水肿,肢体瘫痪,记忆、定向神经功能低下外,其余33例病人恢复良好。结论鞍区肿瘤术后低钠血症出现后,尿量、尿比重的变化以及试验性限水治疗有助于鉴别抗利尿激素分泌异常综合征和脑性盐耗综合征;病因未明时,应首选限水治疗。因为绝大多数的病例为脑性耗盐综合征。     

MRI空泡蝶鞍与婴儿生产方式的相关性研究

目的通过对空泡蝶鞍患者顺产与剖腹产两种生产方式调查分析,探讨婴儿生产方式与MRI空泡蝶鞍的相关性。方法回顾性分析我院260例空泡蝶鞍MRI检查受检者婴儿生产方式、病史、相关疾病及临床症状,采用统计软件分析数据的相关性。结果顺产182例占70%,剖腹产78例占30%,空泡蝶鞍发生率对比P0.01,顺产、剖腹产两种生产方式与空泡蝶鞍发生率有显著差异。结论婴儿生产方式是空泡蝶鞍形成的重要原因,应引起临床的高度重视。     

溴隐亭治疗高泌乳素血症119例

１９８７年至１９９３年我院妇科内分泌门诊治疗１１９例高泌乳素血症。其中垂体腺瘤４６例，空泡蝶鞍２例，功能性高泌乳素血症７１例。１１９例均用溴隐亭治疗，其中９例行垂体腺瘤手术切除，术后继服溴隐亭。结果血清泌乳素值迅速下降至正常范围者占８８．２４％，泌乳明显减少或停止者占８７．６１％，月经复潮者为８０．６７％，妊娠率为６０．２０％。     

An experimental study on the role of fluid replacement in the prevention of organ failure after burn injury]

44 dogs with 50% third degree burn were divided into 4 groups. Those without fluid replacement died within 12 postburn hours with complication of organ dysfunction; whereas those with immediate fluid replacement according to the Parkland formulae survived without definite organ dysfunction. But low-perfusion in the first 8-12 hours and some pathologic changes in the organs were seen. The results suggested that it is necessary to infuse fluid more rapidly in the first 2-3 hours. In group 3, most of the dogs with fluid replacement 6 hours after burn survived longer than the dogs without fluid replacement, but they were all complicated by severe organ dysfunction. This evidence that delayed inadequate resuscitation may be the primary factor affecting the development of early organ failure. In group 4, the dogs were given fluid replacement 6 hours after burn. The replacement was very rapid during the 7th and 8th hour. Both mortality and incidence of organ dysfunction in this group were decreased markedly. This result demonstrated that rapid infusion to replace loss of fluid within 2 hours was effective to treat shock and prevent progressive hypoxic organ damage.     

Hyponatraemia in clinical practice

Hyponatraemia is defined as a serum sodium concentration below 135 mmol/l. It causes major diagnostic and management problems in practice. Hyponatraemic disorders are divided into euvolaemic, hypervolaemic and hypovolaemic. In the evaluation of the hyponatraemic patient, history taking should focus on identifying the potential cause, duration and symptomatology. Clinical examination should include assessment of volume status. Acute hyponatraemia of less than 48 h duration requires prompt correction. Treatment may involve hypertonic saline, isotonic saline and appropriate hormone replacement therapy depending on the aetiology. Chronic hyponatraemia should be treated with caution because of the risk of central pontine myelinolysis.     

Relationship between endogenous digitalis－like factor and fluid therapy in burn－blast combined injury

Ｒｅｌａｔｉｏｎｓｈｉｐｂｅｔｗｅｅｎｅｎｄｏｇｅｎｏｕｓｄｉｇｉｔａｌｉｓ－ｌｉｋｅｆａｃｔｏｒａｎｄｆｌｕｉｄｔｈｅｒａｐｙｉｎｂｕｒｎ－ｂｌａｓｔｃｏｍｂｉｎｅｄｉｎｊｕｒｙＺｈｕＰｅｉｆａｎｇ（朱佩芳）；ＸｕＹｏｕｑｉ（徐有奇）；ＺｈｏｕＪｉ...     

关于“渗透性脱髓鞘综合征”的临床进展——肝移植术后并发症机理的探讨

脑桥中央髓鞘溶解症(central pontine myelinolysis,CPM)自1970年代后期被联系于低血钠的快速纠正,约10年后又拓展至"渗透性脱髓鞘综合征(osmotic demyelination syndrome,ODS)[1]",及至2008年个案文献甚至提出了"可能的周围神经ODS[2]"。但不少迹象提示,诸如后述的问题或许尚无满意答案:CPM最可能的病因是什么?文献肝移植术后或严重呕吐相关的CPM或"ODS"预后极差之原因为何,如何优化其围手术期处理、如何优化重度低钠血症的治疗原则、改善预后?"ODS"累及的主要部位为何?病理机制为何?如何在纠正血钠时避免之?等等。兹对早期的、经典的或有一定代表性的病例文献的具体资料进行重新分析及商榷;在此基础上,对上述诸问题进行初步的探索和反思。     

Therapy with hypertonic saline in combination with anti-convulsants for hyponatremia-induced seizure: a case report and review of the literature

Seizures are an uncommon but serious complication of hyponatremia which can lead to permanent brain damage and even death. It is recommended that patients with hyponatremic-induced seizures be treated with 3% hypertonic saline, however, a rapid rate of correction may result in central pontine myelinolysis (CPM), a severe neurological disorder characterized by mutism, dysarthria, spastic quadriparesis, and pseudobulbar palsy. The patient in this case developed a hyponatremia-induced generalized tonic-clonic seizure which was aborted by rapid therapy with diazepam, followed by hypertonic saline and phenytoin. Subsequent replacement of hypertonic saline with normal saline and salt tabs in combination with phenytoin allowed gradual correction of serum sodium without any subsequent seizures or neurological complications.     

烧冲复合伤后液体治疗与内源性类洋地黄物质的关系

实验用狗50只,分5个组,均致成重度烧冲复合伤。伤后2h开始输注不等量的液体和钠盐。于伤后2、4、8、24h取血测EDLF、钠及血浆量。留尿测尿钠。结果显示:各组EDLF伤后均有不同程度的减少。给钠量越少,EDLF下降愈显著。本文对血清EDLF变化与血钠、尿钠等关系进行了讨论,并指出血清EDLF结合血钠和尿钠测定对了解机体水盐代谢有一定的参考价值。对液体治疗有一定的指导意义。     

Colobomata associated with Noonan's syndrome

A patient with Noonan's syndrome and fundal colobomata in both eyes is described. To our knowledge, this is the first report of the association of colobomata with Noonan's syndrome. Although the patient had poor sight since early childhood and dyspnea on exertion as a teenager, the diagnosis of Noonan's syndrome was not made until early adulthood. We hope this report will encourage recognition of this syndrome and its implications at an earlier stage.