Secondary paroxysmal dyskinesias.

Paroxysmal dyskinesias (PxDs) are involuntary, episodic movements that include paroxysmal kinesigenic (PKD), paroxysmal nonkinesigenic (PNKD), and paroxysmal hypnogenic (PHD) varieties. Although most PxDs are primary (idiopathic or genetic), we found 17 of our 76 patients with PxD (22%) to have an identifiable cause for their PxD (10 men; mean age, 41.4 years). Causes included peripheral trauma (in three patients), vascular lesions (in four), central trauma (in four), kernicterus (in two), multiple sclerosis (in one), cytomegalovirus encephalitis (in one), meningovascular syphilis (in one), and migraine (in one). The latency from insult to symptom onset ranged from days (trauma) to 18 years (kernicterus), with a mean of 3 years. Nine patients had PNKD, two had PKD, five had mixed PKD/PNKD, and one had PHD. Hemidystonia was the most common expression of the paroxysmal movement disorder, present in 11 patients. Both of the patients with PKD had symptom durations of <5 minutes. Symptom duration ranged from 10 seconds to 15 days for PNKD and from 5 minutes to 45 minutes for mixed PKD/PNKD. There were no uniformly effective therapies, but anticonvulsant drugs, clonazepam, and botulinum toxin injections were the most beneficial. Awareness of the variable phenomenology and the spectrum of causes associated with secondary PxD will allow for more timely diagnosis and early intervention.     

1 alpha(OH)D3 (ETALPHA) treatment and receptor studies in 16 patients with chronic and myeloproliferative disorders

10 patients with CLL and 2 with CML were treated with gradually increasing doses of 1 alpha(OH)D3, up to 4 micrograms daily during 6 wk. 3 patients with preleukemia and 1 with myelofibrosis were treated with 2 micrograms daily of 1 alpha(OH)D3 for a prolonged period up to 17 wk. The treatment with 1 alpha (OH)D3 did not result in changes of disease parameters in any of the patients under study. Receptor studies for 1,25(OH)2D3 were performed in 8 CLL patients and revealed only 1 patient with increased specific receptor binding capacity. The maximum tolerable dose of 1 alpha(OH)D3 varied individually, but was in the range of 2-4 micrograms daily.     

发作性睡病伴发的幻觉症——随访22年一例

患者十六岁开始有逐渐加重的睡眠发作,伴有轻度不典型猝倒。14岁后出现入睡前幻觉,主要为鲜明生动的听幻觉,偶有幻视、幻嗅。幻觉严重时继发关系妄想和被害妄想,失去自知力。22年随访结果:患者精神状态良好,仍有轻度睡眠发作及入睡前幻觉,不影响日常工作和生活。对诊疗作了评价。     

继发性唇裂鼻畸形硅胶植入整复术

本文介绍一种使继发性唇裂鼻畸形获得较好外形的新手术方法。术式包括切除偏曲的鼻中隔软骨,并将一片硅胶植入于中央作为鼻小柱支柱,从而矫正歪斜的鼻小柱和抬高鼻尖,使两侧鼻孔形态对称一致,用本法为11例单侧唇裂畸形较重者进行了整复,随访4个月至4年半。绝大多数(10/11)取得满意效果,未见有严重感染和变形等并发症。     

Sphingolipid metabolites in neural signalling and function

Sphingolipid metabolites, such as ceramide, sphingosine, sphingosine-1-phosphate (S1P) and complex sphingolipids (gangliosides), are recognized as molecules capable of regulating a variety of cellular processes. The role of sphingolipid metabolites has been studied mainly in non-neuronal tissues. These studies have underscored their importance as signals transducers, involved in control of proliferation, survival, differentiation and apoptosis. In this review, we will focus on studies performed over the last years in the nervous system, discussing the recent developments and the current perspectives in sphingolipid metabolism and functions.     

浅析中专护生文化休克的护理策略

文化休克(culture shock),又译为文化震撼或文化震惊,是指生活在某一种文化环境中的人初次进入到另一种文化环境,如到了不同的民族、社会群体中或地区甚至国家时所产生的思想混乱与心理上精神紧张综合征。新入校中专护生,由于文化环境的改变,往往会出现孤独、焦虑、无助等文化休克的种种表现,这些现象若不能及时得到解决,将不利于他们的全面健康状态的成长与发展。众所周知,WHO明确规定,健康不但是没有疾病和身体缺陷,还要有完整的生理、心理状态和良好的社会适应能力。因此,新入校中专护生这一特殊群体所产生的文化休克现象应引起学校有…     

MANAGEMENT OF UNCONTROLLED GLAUCOMA WITH THE MOLTENO SYSTEM

In glaucomatous eyes refractory to medication, laser techniques and conventional drainage surgery, intraocular pressure is often high, and visual loss rapid. In this situation a reliable, robust artificial outflow system is required. Molteno has evolved a plastic tube and plate device combined with a fibrosis suppression medication regimen. Thirty-eight eyes of 32 patients with uncontrolled glaucoma were treated with the Molteno system. Six months after operation mean intraocular pressure had been reduced from 41.0 ± 13.6 to 16.2 ± 5.6 mmHg. Eighteen eyes had pressures of 20 mmHg or less on no hypotensive therapy, 17 on reduced treatment. Three eyes had a pressure of 21 to 35 mmHg on treatment at six months. The 13 aphakic eyes responded as well as 25 phakic eyes. Five eyes with rubeotic glaucoma demonstrated pressures of less than 20 mmHg without therapy, four eyes with traumatic glaucoma required continuing medication with three having pressures below 22 mmHg. Of the seven eyes with uveitic glaucoma, one was lost, two required maintenance therapy; five of six surviving eyes had pressures below 20 mmHg. Fifteen eyes with congenital or juvenile glaucoma achieved pressures below 20 mmHg, three of these with timolol drops, three with timolol and acetazolamide, and nine with no treatment. While seven of seven eyes with refractory primary open-angle glaucoma attained pressures below 20 mmHg. all seven needed continuing mild hypotensive therapy. Eleven eyes underwent a one-stage procedure, while 27 eyes required a two-stage operation. Twenty-eight eyes received fibrosis suppression medication after the second stage, and 24 maintained or improved their preoperative visual acuity. Results have been encouraging: in general the Molteno system is recommended as the second drainage operation in all glaucomatous eyes in which conventional therapy has failed, and as the primary surgical procedure (after laser techniques) in eyes with rubeotic and uveitic glaucoma. Ciliary body destructive procedures should be restricted to control of symptoms in blind eyes.     

Expression of the calcium receptor CaR in the parathyroid of secondary hyperparathyroidism patients

The effects of calcium on parathyroid hormone (PTH) has further discovered in recent years. It has been known that calcium ion concentration in the extracellular fluid is a major determinant of PTH secretion. The relationship between serum intact PTH (iPTH) and calcium ion levels is described by a sigmoidal curve. The calcium concentration that produces half-maximal change in PTH release (the midpoint between maximal and minimal     

负载型丝光沸石催化剂的催化性能研究

运用二元碱氮置换吸附红外光谱法、分子筛孔径分布测定技术、XPS技术,考察了丝光沸石表面酸性与孔结构的相互关系、钯在丝光沸石上分布情况和价态及其与沸石的相互作用。结果表明,随着丝光沸石脱铝程度的增加,表面出现大量不规整晶格,存在较多的次级孔道。强质子酸中心主要分布在它的“外表面”。钯在其表面富集,并与“外表面”上的质子酸中心协同作用,促进了轻质烷烃异构化反应。