带血管蒂髂骨移植治疗青少年镰状细胞病股骨头缺血性坏死：短中期结果随访

背景：镰状细胞病是造成非洲青少年股骨头缺血性坏死的主要原因，带血管的髂骨移植用于股骨头缺血性坏死的报告已经很多，它也适用与镰状细胞病股骨头缺血性坏死的患者，可以使坏死区再血管化提高和阻止进行性发展。 目的：探讨带血管蒂髂骨移植治疗青少年镰状细胞性股骨头缺血性坏死的早期和中期疗效。 方法：纳入尼日尔马腊迪省治疗中心的青少年镰状细胞病并股骨头缺血性坏死的患者12例，均采带旋髂深血管髂骨瓣移植治疗，术后随访24~30个月(平均27个月)，观察移植后患者是否髋痛，功能活动和X射线骨盆平片结果。 结果与结论：移植后疼痛消失，无感染等并发症，无重复手术，近中期疗效满意，Harris评分平均从术前75分提高到术后90分，而X射线平片表现并不令人满意。对于Ficat III、IV期的镰状细胞性骨股头缺血性坏死青少年患者，采用带血管蒂髂骨移植治疗，可以解除疼痛，恢复髋关节功能。     

非骨水泥型髋关节假体用于老龄初次全髋关节置换：2年随访

背景：目前普遍认为非骨水泥型假体适用于年龄< 65岁的患者,骨水泥假体适用高龄、骨质情况欠佳的患者。但没有明确证据表明非骨水泥假体不适用于年龄> 70岁、既往身体健康、日常活动量满意、无骨质疏松或有轻度骨质疏松的患者。 目的：观察非骨水泥型髋关节假体用于老龄初次全髋关节置换患者的效果。 方法：纳入行非骨水泥假体初次全髋关节置换、年龄> 70岁的老龄患者57例65髋,平均年龄86.3岁;其中股骨颈骨折33例33髋,股骨头缺血性坏死18例25髋,髋关节发育不良6例7髋。记录置换时间、手术出血量、住院时间、置换后初次下床活动的时间、置换前及置换后3,6,12,24个月的髋关节Harris评分、X射线片结果、置换后并发症。 结果与结论：随访6~40个月,平均19.6个月。53例完成随访,4例失访,未发现假体松动和需翻修手术病例。Harris评分由置换前的平均(40.7±18.9)分提高到置换后的(89.2±5.5)分,优良率为93.7%。置换后并发症包括术口血肿1例、脑血管意外1例几泌尿系感染1例。提示老龄患者用非骨水泥型髋关节假体行初次全髋关节置换治疗能达到满意的近期效果。     

强直性脊柱炎非骨水泥型全髋关节置换27例

探讨强直性脊柱炎患者行人工非骨水泥型全髋关节置换的手术时机、手术方法及疗效分析。 方法：选择2000/2005湘雅医院收治强直性脊柱炎后髋关节强直患者27例33髋，均经Gibson入路行人工非骨水泥型全髋关节置换，全部采用Link公司Ribbed非骨水泥型人工全髋关节。比较髋关节置换前后Merle D’Aubigne评分及髋关节活动范围。 结果：27例33髋均获得随访，随访时间≥5个月，平均21个月。X射线平片示假体位置良好，无脱位，髋关节疼痛消失。各向运动良好。患者术后Merle D'Aubigne评分平均17.3分(16~18分)。 结论：对强直性脊柱炎进行人工非骨水泥型全髋关节置换可放宽手术年龄限制，根据患者的不同情况，在术中采取恰当的关节置换技术，均可恢复髋关节功能。     

应用髋关节镜髓芯减压并自体外周血干细胞移植治疗早期股骨头缺血性坏死38例★

目的：髋关节镜检查清理、髓芯减压已应用于股骨头缺血性坏死的治疗。观察髓芯减压术并自体外周血干细胞移植治疗早期股骨头缺血性坏死的疗效。 方法：①试验对象：选择2004-12/2006-12本科应用髋关节镜及自体外周血干细胞移植手术的股骨头缺血坏死患者38例(48髋)。男性28例，女性10例，年龄34～57岁。纳入标准：根据临床症状、体征、CT及MRI诊断为早期股骨头缺血性坏死；所有患者经谈话后自愿接受髋关节镜及自体外周血干细胞移植手术治疗并签署知情同意书。术前Ficat分期Ⅰ期25髋，Ⅱ期13髋。②试验方法及评估：采用Harris髋关节评分对术前及术后关节功能进行评估，优：> 80分，良：> 60分，可：> 40分。同时X射线观察患髋的术后恢复情况及有无并发症。 结果：38例患者，均进入结果分析。术后随访10~30个月，平均20个月。①术后80分以上(优)31例，60分以上(良)5例，40分以上(可)2例，平均(90.1±3.6)分，优良率达94.7%，与术前比较，差异有显著性意义（P < 0.01）。②所有患者疼痛消失，行走正常，髓关节活动范围正常或接近正常，X射线片示股骨头轮廓清晰，囊性变消失，骨密度均匀，关节间隙正常。无感染和血管神经损伤等并发症的发生。 结论：髋关节镜下清理、髓芯减压及自体外周血干细胞移植手术治疗早期股骨头缺血性坏死具有损伤小、简便、准确、有效的优点。     

髋臼内壁截骨在成人发育不良髋关节置换中的应用：8例分析

回顾性分析2004-09/2006-11南昌大学第二附属医院骨科收治的髋关节发育不良患者8例(8髋),男2例,女6例,年龄35~65岁,平均46岁;置换前行X射线摄片及CT检查,提示所有患者患侧髋臼角(Sharp角)均> 45°,股骨头包容不良,关节间隙缩窄,shenton线连续性欠佳。按Crowe分期,CroweⅡ期5例5髋,CroweⅢ期3例3髋。置换前Harris评分44~62分,平均50分。全部患者均采用全髋置换,假体采用非骨水泥或混合型固定模式,其中非骨水泥生物固定者6髋,水泥固定者2髋。所有患者髋臼侧均采用髋臼内壁截骨内移法安放髋臼假体。记录置换时间,置换过程中出血量,置换后住院天数,Harris评分及围置换期并发症;置换后临床随访观察截骨断面及植骨块愈合情况。8例患者均获得随访,平均6个月,全部患者关节功能恢复良好,随访病例中未出现感染、坐骨神经损伤、髋关节脱位、股骨干骨折、深静脉血栓、假体松动等并发症。置换后6个月Harris评分86~94分,平均88分。置换后3个月X射线平片提示截骨断面及植骨块愈合良好。提示髋臼内壁内移截骨全髋置换治疗髋关节发育不良作用明显,早中期效果良好。     

自体股骨头植骨生物型人工全髋关节置换治疗髋关节发育不良的疗效

背景：Crowe Ⅱ、Ⅲ、Ⅳ型髋关节发育不良患者真臼发育差,且受股骨头蚀损影响,外侧壁常有缺损,因此大部分患者均需采用自体植骨、假臼重建。 目的：观察生物型人工全髋关节置换治疗髋关节发育不良时采用自体股骨头植骨的临床效果。 方法：选择2007-03/2009-11四川省骨科医院髋部创伤科收治的髋关节发育不良继发骨性关节炎患者15例15髋,单侧患者双下肢不等长为(2.7±0.8) cm,置换前Harris评分为(41.6±12.8)分。术中15髋采用标准全髋关节置换,同时采用自体股骨头植骨重建髋臼旋转中心。置换后随访摄骨盆正位和患髋侧位X射线平片评价髋臼和股骨假体位置、植骨块愈合情况以及双下肢长度。 结果与结论：置换后切口均Ⅰ期愈合。患者均获随访,随访时间一两年,平均1年。置换后12个月X射线平片示植骨块均已愈合。末次随访时单侧患者双下肢不等长(0.8±0.3) cm,Harris评分为(89.3±6.5)分,与置换前相比,差异均有显著性意义(P < 0.05)。X射线平片示髋臼和股骨假体无移位,未见植骨块有明显移位和吸收塌陷征象。提示髋关节发育不良继发骨性关节炎行全髋关节置换时采用自体股骨头植骨有利于恢复髋臼旋转中心,提供良好髋臼固定。     

全髋关节置换治疗系统性红斑狼疮合并股骨头缺血性坏死6例

背景：全髋关节置换是目前治疗髋关节疾患终末期的主要方法,疗效确切,但是对于伴有股骨头缺血性坏死的系统性红斑狼疮患者,常合并如严重骨质疏松症等其他疾患,目前国内外对于此类患者接受髋关节置换的效果尚不清楚。 目的：探讨系统性红斑狼疮合并股骨头缺血性坏死行全髋关节置换的临床效果。 方法：回顾性分析本科1998-06/2008-06行全髋关节置换的系统性红斑狼疮合并股骨头缺血性骨坏死患者临床资料。共6例(共6髋)获得随访,其中男1例,女5例,年龄46(28~67)岁,5髋使用生物固定型假体,1髋使用骨水泥型假体。治疗效果评估选用WOMAC骨关节炎指数,SF-36和Harris评分标准。 结果与结论：平均手术时间为141 (85~210) min,平均出血量为522(180~1 500) mL;平均随访时间3.5(1~10)年,1例于术后1.5年死于颅内出血。术后早期出现1例泌尿系统感染,其他病例在随访期间无假体感染、松动、脱位及深静脉血栓等并发症出现。随访时Harris评分、WOMAC骨性关节炎指数、SF-36评分均较术前明显改善(P < 0.05)。结果提示全髋关节置换可明显提高系统性红斑狼疮合并股骨头缺血性坏死患者的生活质量,中期临床效果良好。     

多孔钽棒置入治疗股骨头坏死16例

背景：髓心减压及钽棒置入治疗早期股骨头坏死的手术方法,尤其适合于股骨头坏死SteinbergⅠ期和Ⅱ期,既可以解决疼痛又可以防治股骨头的塌陷延缓坏死的进展,是一种值得尝试的新方法。 目的：探讨多孔钽棒置入治疗股骨头坏死的适应症及其临床疗效。 方法：纳入股骨头坏死16例18髋,根据Steinberg分期：Ⅰ期5髋,Ⅱ期12髋,Ⅲ期A 1髋。采用侧方小切口入路,行髓心减压及多孔坦棒置入。置入后6周内禁止负重,6~10周部分负重,逐步过渡至完全负重。采用Harris评分系统评估患者置入后髋关节功能改善情况;置入后24 h、1,3,6,12个月摄正侧位X射线平片,观察股骨头修复情况及有无坏死进展。 结果与结论：16例18髋获得随访,随访时间7~13个月。置入后末次随访进行疗效评估,优：11例12髋,良：4例5髋,可：1例1髋。患者Harris评分由置入前49~83分,平均(65.3±8.6)分,提高至置入后75~97分,平均(88.2±9.3)分,治疗前后比较差异有显著性意义(P < 0.05)。随访摄片髋关节影像学表现稳定,股骨头无坏死进展。提示,多孔钽棒假体置入治疗股骨头坏死疗效确切,尤其是对于SteinbergⅠ期,Ⅱ期及塌陷较小的 ⅢA期股骨头坏死的患者。     

全陶瓷型人工髋关节置换治疗股骨头缺血性坏死23例☆

目的：全髋关节置换存在的主要问题是假体无菌性松动，为此许多不同特质的关节假体界面被研究利用，陶瓷对陶瓷型假体就是其中的一种。观察陶瓷-陶瓷人工髋关节置换治疗股骨头缺血性坏死的中短期疗效，并评估其临床应用价值。 方法：于2006-01/2008-12对23例(28髋)采用陶瓷-陶瓷非骨水泥型假体置换治疗的股骨头缺血性坏死患者进行随访，所有关节假体均使用BiCONTACT®全陶瓷关节假体。应用Harris评分标准进行功能评价，应用股骨Gruen与髋臼Delee-Charnly分区方法进行影像学评价。 结果：平均随访28个月。患者Harris评分从置换前平均39.45分提高至置换后平均88.57分，差异具有显著性意义(P < 0.05)。影像学上无明显的假体松动、下沉。 结论：对于股骨头缺血性坏死患者，新型陶瓷-陶瓷假体短期随访置换效果良好，影像学表现无明显松动。     

骨小梁金属AVN重建棒植入配合中药治疗早期股骨头坏死26例

背景：骨小梁金属 AVN重建棒为多孔钽金属假体,为Michael Christie设计并发明,自2001-11在欧洲上市以来已有大量的假体植入,取得良好效果。中医药治疗股骨头坏死具有一定优势,特别是对早、中期患者的疗效良好,关键在于早期诊断与早期治疗是取得良好疗效的关键。 目的：观察骨小梁金属AVN重建棒植入配合中药治疗早期股骨头缺血性坏死的临床效果。 方法：选择2005-02/2007-06河南省中医院骨科收治的早中期股骨头缺血性坏死患者26例,男16例,女10例;年龄18~56(40.0±2.2)岁;单侧股骨头坏死21例,双侧坏死5例;Ficat分期,Ⅰ期14例,Ⅱ期12例。所有患者均有患髋疼痛,腹股沟中点处深在性压痛,髋关节活动受限,能扶单拐行走者15例,扶双拐行走者8例,卧床不能行走者3例。26例术前采用Harris评分平均(61.5±1.0)分。全部患者均采用骨小梁金属AVN重建棒植入配合中药治疗。 结果与结论：26例患者均获得随访,随访时间1~2年。26例患者均顺利植入骨小梁金属AVN重建棒,植入时间10~18 min,平均15 min。植入后患者切口均Ⅰ期愈合。术后1年Harris评分平均(87.3±1.3)分,与植入前相比差异有显著性意义(P < 0.05),优21例,良3例,可2例,优良率92%。植入后均无深静脉栓塞、泌尿系统、肺部感染及压疮等发生。提示骨小梁金属AVN重建棒植入配合中药是治疗早期股骨头缺血性坏死的有效方法。     

Hepatic Considerations in the Use of Antiepileptic Drugs

Summary: Virtually all of the major antiepileptic drugs (AEDs) can cause hepatotoxicity, although fatal hepatic reactions are rare. The mechanisms, incidences, and risk profiles for such reactions differ from drug to drug. With carbamazepine and phenytoin, hepatotoxicity may be due to drug hypersensitivity. Although the profiles of patients at risk have not been well-defined for these two antiepileptic drugs, it would appear from reports in the literature that older adolescents and adults are at higher risk than children of developing serious or fatal hepatotoxicity. Once hepatotoxicity develops, mortality rates are 10–38% with phenytoin and 25% for carbamazepine. The risk profile for valproate fatal hepatotoxicity has been more clearly defined. Those at primary risk of fatal hepatic dysfunction are children under the age of 2 years who are receiving multiple anticonvulsants and also have significant medical problems in addition to severe epilepsy. The risk is considerably lower for patients over the age of 2 years on valproate monotherapy. In contrast to the risk profile with other AEDs, adults receiving valproate as monotherapy have the lowest risk of hepatotoxicity. Fatal hepatic dysfunction coincident with valproate may be the result of aberrant drug metabolism. Concomitant use of AEDs that induce microsomal P450 enzymes (e.g., phenytoin and phenobarbital) may enhance the production of a toxic metabolite, and hence the greater risk of hepatotoxicity with polypharmacy.     

Vascular Malformations and Epilepsy: Clinical Considerations and Basic Mechanisms

Summary: Vascular malformations (VMs) are associated with epilepsy. The natural history of the various VMs, clinical presentation, and tendency to provoke epilepsy determine treatment strategies. Investigations have probed the mechanisms of epileptogenesis associated with these lesions. Electrophysiologic changes are associated with epileptogenic cortex adjacent to VMs. Putative pathophysiologic mechanisms of epileptogenesis include neuronal cell loss, glial proliferation and abnormal glial physiology, altered neurotransmitter levels, free radical formation, and aberrant second messenger physiology.     

Diagnostic Difficulties and Treatment Implications

Summary: Differentiation between types of epileptic seizures has been aided in recent years by the introduction of intensive neurodiagnostic techniques and the development of increasingly detailed classification systems. Paradoxically, these developments have not simplified the task of matching the appropriate antiepileptic drug to a particular seizure type. It is reasonable to assume that anticonvulsant drugs will have different effects on different types of seizures, but faulty, circular reasoning can enter the picture if one also assumes that responses of seizures to different drugs signify different seizure types. There are several examples of differential diagnoses that can fall prey to this problem, including the diagnosis between partial seizures with secondary generalization and generalized tonic-clonic seizures, and the diagnosis between complex partial seizures and absence seizures with automatisms, among others. Considerations of etiology in future classification systems can further complicate the problem: should one then choose an anticonvulsant drug on the basis of individual seizure type or on the basis of the type of epilepsy? Ramifications of this issue extend even to the drug approval process. Official sanction is not given for use of a drug for a seizure type not included in the original efficacy studies, even if later scientific evidence shows that seizure type to be related to a type that is included. New trials must be undertaken. These problems arise from how we choose to classify seizures.     

Cognitive Dysfunction Associated with Antiepileptic Drug Therapy

Summary: Epilepsy is frequently associated with cognitive dysfunction. However, the reasons for this correlation are unclear. Possible influential factors include patient age; duration, frequency, etiology, and type of seizures; hereditary factors; psychosocial issues; and antiepileptic drug (AED) therapy. Whereas many of these factors are beyond the physician's control, AED therapy is one element that can be addressed in treatment decisions by recognizing the potential cognitive effects of particular AEDs. For example, phenobarbital impairs memory and concentration; phenytoin affects attention, problem solving ability, and performance of visuomotor tasks. In contrast, carbamazepine may affect concentration, while valproate would appear to have minimal effects on cognition. Moreover, cognitive effects of AEDs are amplified with coadministration of multiple anticonvulsants (polytherapy). A review of studies on the cognitive effects of monotherapy with AEDs, as opposed to those of polytherapy, provides evidence that drug-related cognitive dysfunction can be reversed if patients are switched to a simpler therapeutic regimen. Future research should be directed toward developing reliable measures for assessing and monitoring cognition, and understanding the particular cognitive side effects of each AED. Physicians also need to revise their opinions about which side effects are "tolerable" for epileptic patients.     

Epilepsy and anomalies of neuronal migration: MRI and clinical aspects

Neuronal migration disorders are the result of disturbed brain development. In such disorders, neurons are abnormally located. In diagnosing these conditions, magnetic resonance imaging is superior to any other imaging technique. This enables us to improve our knowledge of the clinical correlates of neuronal migration. With reference to migrational disorder, a retrospective study of all 303 patients with epileptic seizures referred for magnetic resonance imaging during a 3-year period was performed, 13 patients (aged 12-41, mean age 27) were identified. They represent 4.3% of the entire study group. Of the patients with known epilepsy, 6.7% and of the mentally retarded, 13.7% had migrational disorders. Four patients had schizencephaly as the dominant finding, one was classified as hemimegalencephaly, 2 had isolated heterotopias, and 6 had localized pachy- and/or poly-microgyria. The clinical pictures are complex. Ectopias of grey matter are recognised foci of epilepsy, but from an epileptological and a clinical viewpoint little attention has been given to these disorders. The present study shows that malmigration is not rare in epilepsy patients, especially not in the mentally retarded.     

Carbamazepine Efficacy and Utilization in Children

Summary: Carbamazepine is effective for preventing partial and generalized tonic-clonic seizures in children. Although absence epilepsies are more common in children than adults, an estimated 80% of children with epilepsy have seizure types or epilepsies that are potentially responsive to carbamazepine. The differential diagnosis of ictal staring is an especially important issue in children because absence and atypical absence seizures are more prevalent in children than adults. Age-related pharmacokinetic differences and drug interactions are major considerations in children. On average, children have higher clearance rates of carbamazepine, shorter half-lives, and higher ratios of carbamazepine-10, 11-epoxide to carbamazepine than adults. In addition, children with severe epilepsy are more likely to require multiple-drug therapy, which can lead to complex drug interactions. When carbamazepine is administered along with valproate, drug protein binding interactions can cause intermittent side effects.     

Carbamazepine Efficacy in Adults With Partial and Generalized Tonic-Clonic Seizures

Summary: Carbamazepine and phenytoin are drugs of choice in initial monotherapy for adult partial and secondarily generalized tonic-clonic seizures. These designations reflect the results of the Veterans Administration Epilepsy Cooperative Study Group of 1985. An earlier comparative study of carbamazepine and phenytoin by Ramsay and associates found both drugs equally effective in controlling new-onset seizures. Among the advantages of carbamazepine is that it causes relatively few cognitive and dysmorphic side effects. Its disadvantages are its unavailability in parenteral formulation and its metabolic autoinduction. The latter must be compensated for by planned dosage increases to maintain therapeutic plasma steady-state levels during the first 2 or 3 months of treatment. Carbamazepine is judged a drug of choice in the treatment of these secondarily generalized tonic-clonic seizures, and the drug of choice in children, adolescents, and women susceptible to the dysmorphic side effects associated with other anticonvulsant agents.     

Etiologic and Preventive Aspects of Epilepsy in the Child–Bridging the Gap Between Laboratory and Clinic

Summary: Four broad categories of basic phenomena are pertinent to developing ways to prevent epilepsy. These include mechanisms of epileptogenesis, ictal initiation and temporary entrainment by the seizure discharge of normally functioning brain, seizure propagation, and control mechanisms that function both to restrain the cascade of epileptic events culminating in a seizure and to arrest the epileptic event and restore the interictal state. In newborns and children, hypoxia-ischemia is a major factor leading to epileptogenesis, and several schemes are proposed to classify, quantify, and prevent hypoxic-ischemic encephalopathy. Control mechanisms must be better understood in order to develop prophylactic recommendations for epilepsy, and an experimental model of "kindling antagonism" may increase our understanding of these. Programs of prevention of seizures in children will evolve only if basic researchers and clinicians work productively together to develop an adequate understanding of factors important in epileptogenesis and antiepileptogenic control mechanisms.     

Classification of methods in transcranial Electrical Stimulation (tES) and evolving strategy from historical approaches to contemporary innovations

Transcranial Electrical Stimulation (tES) encompasses all methods of non-invasive current application to the brain used in research and clinical practice. We present the first comprehensive and technical review, explaining the evolution of tES in both terminology and dosage over the past 100 years of research to present day. Current transcranial Pulsed Current Stimulation (tPCS) approaches such as Cranial Electrotherapy Stimulation (CES) descended from Electrosleep (ES) through Cranial Electro-stimulation Therapy (CET), Transcerebral Electrotherapy (TCET), and NeuroElectric Therapy (NET) while others like Transcutaneous Cranial Electrical Stimulation (TCES) descended from Electroanesthesia (EA) through Limoge, and Interferential Stimulation. Prior to a contemporary resurgence in interest, variations of transcranial Direct Current Stimulation were explored intermittently, including Polarizing current, Galvanic Vestibular Stimulation (GVS), and Transcranial Micropolarization. The development of these approaches alongside Electroconvulsive Therapy (ECT) and pharmacological developments are considered. Both the roots and unique features of contemporary approaches such as transcranial Alternating Current Stimulation (tACS) and transcranial Random Noise Stimulation (tRNS) are discussed. Trends and incremental developments in electrode montage and waveform spanning decades are presented leading to the present day. Commercial devices, seminal conferences, and regulatory decisions are noted. We conclude with six rules on how increasing medical and technological sophistication may now be leveraged for broader success and adoption of tES.     

Predisposing and Causative Factors in Childhood Epilepsy

Summary: We review information from large studies of defined populations, examining the role of known factors and especially of prenatal and perinatal factors in contributing to nonfebrile seizure disorders of early childhood. We depend especially, but not exclusively, on the recently completed analyses from the Collaborative Perinatal Project of the National Institute of Neurological and Communicative Disorders and Stroke, the NCPP. About 4% of children in the NCPP who had at least one non-febrile nonsymptomatic seizure by the age of 7 years had a previous seizure during acute neurologic illness, such as meningitis or during the acute illness after trauma. Many such seizures should potentially be preventable. Of children with seizures, 10% had had a neonatal seizure and 13% had had a febrile seizure. Among the hundreds of prenatal and perinatal factors explored as predictors of childhood seizure disorders, the principal predictors identified were congenital malformations of the fetus, cerebral and noncerebral; family history of certain neurologic disorders; and neonatal seizures. In agreement with the British National Child Development Study, labor and delivery factors in the NCPP appeared to contribute very little to childhood seizure disorders. Maldevelopment, rather than damage at birth to an initially intact nervous system, appeared to be the more common mechanism. Most seizure disorders of early childhood remained unexplained by the large set of prenatal and perinatal characteristics examined.