Mono- or Polypharmacotherapy in Institutionalized Epileptic Children with Severe Mental Retardation?

ABSTRACT. Nine severely mentally retarded patients with severe epilepsy who were living in an institution were studied during a three-year period by a multi-disciplinary team. A seizure rate serum level chart was made for each patient. It served as the basis for monthly discussions in the team about medication changes. Drug plasma concentrations were monitored monthly. Statistical comparisons between seizure frequency on different drug regimens were made by χ² test. Withdrawal of all medication was possible in one case and reduction to monotherapy in two cases. In the remainder of the patients a combination of two or three anticonvulsants gave the best clinical effect. We thus found polypharmacotherapy necessary for some severely retarded patients with epilepsy. Our multidisciplinary approach and chart monitoring system has many advantages and is valuable for the medical care and drug treatment of this patient category.     

新型壳聚糖对血浆胆红素和细胞因子吸附性能的体外筛选初步研究

目的观察各类新型吸附剂对重型肝炎患者血浆中的胆红素和细胞因子的吸附性能。方法第一步,收集第一组重型肝炎患者的血浆各3ml,以8种不同的吸附剂(1#～3#为致孔剂浓度分别为3%、1%、5%的壳聚糖,4#为胺基化壳聚糖,5#为苯乙烯/二乙烯苯聚合物,6#为后交连苯乙烯/二乙烯苯聚合物,7#为壳聚糖-苯乙烯/二乙烯苯聚合物,8#为壳聚糖-后交连苯乙烯/二乙烯苯聚合物)各1ml进行吸附,检测对胆红素的吸附能力,做吸附剂筛选实验和吸附实验。第二步,用以上筛选出吸附率较好的两种吸附剂各1ml对第二组患者血浆(各3ml)进行胆红素和细胞因子的吸附实验。结果第一步实验显示胺基壳聚糖(4#)和苯乙烯/二乙烯苯聚合物(5#)有较好的吸附效果,4#吸附剂对总胆红素(TBiL)、直接胆红素(DBiL)、间接胆红素(IBiL)的吸附率分别为50.5%±3.4%、57.0%±11.3%、39.0%±7.2%;5#分别为30.1%±2.5%、32.6%±3.0%、27.6%±2.9%。第二步实验显示4#胺基化壳聚糖吸附后血浆中TBiL、DBiL、IBiL、白细胞介素6(IL-6)和肿瘤坏死因子α(TNF-α)水平下降比5#明显,有统计学意义(P<0.001)。结论胺基化壳聚糖(4#)体外吸附重型肝炎患者血浆中胆红素、细胞因子效果有明显优势。     

硫酸镁治疗新生儿重症肺炎的临床研究及对血气血镁的影响

目的　为探讨硫酸镁治疗新生儿重症肺炎的临床效果及对血气血镁的影响。方法　采用 96例新生儿重症肺炎患儿随机分成治疗组 4 8例 ,对照组 4 8例 ,治疗组加用硫酸镁 30～ 5 0mg/(kg·d)加入 5 %葡萄糖 2 0ml静滴 ,持续 1h ,疗程3～ 8d ,同时监测血气血镁。结果　治疗组总有效率 79.2 % ,对照组总有效率 5 0 .0 % (P <0 .0 1) ,硫酸镁在改善症状 ,消除肺部体征及缩短住院时间和提高治愈率等方面优于对照组 ,治疗组病死率下降 ,同时血气恢复正常优于对照组 ,血镁含量治疗前后无变化。结论　硫酸镁治疗新生儿重症肺炎疗效显著 ,未发现明显副作用。     

用猪肝细胞型混合生物人工肝支持系统治疗肝衰竭的初步研究

目的 对培养猪肝细胞型混合生物人工肝支持系统治疗肝衰竭的可行性进行初步评价。方法 采用自行研制的以新生实验小型猪肝细胞为基础的混合生物人工肝支持系统，对5例慢性重型肝炎患者进行体外人工肝支持，综合评价其临床疗效和安全性。结果 5例患者经混合人工肝支持后，肝衰竭均得到不同程度的控制，表现为临床症状和肝衰竭相关指标好转，肝性脑病改善。最终2例好转出院，1例经肝移植存活，2例 死亡，死亡原因与不良反应无关。结论 猪肝细胞型混合生物人工肝系统可用作重型肝炎肝衰竭的辅助支持和治疗。     

血清TBA评估慢性重型肝炎肝细胞损伤的临床价值

目的　进一步探讨血清总胆汁酸 (TBA)评估慢性重型肝炎患者肝细胞损伤程度及其预后的临床价值。方法　对慢性重型肝炎组 12 2例、慢性肝炎组 98例和正常对照组 4 8人同时测定TBA、总胆红素 (TBIL)及凝血酶原活动度 (PA) ,并分析它们之间的关系。结果　慢性重型肝炎患者血清TBA水平显著高于慢性肝炎组及正常对照组 (P <0 .0 1) ,TBA与TBIL呈正相关 ,与PA呈负相关 (分别为r =0 .712 ,P <0 .0 1;r =- 0 .832 ,P <0 .0 1) ;治愈 +好转组TBA明显低于恶化 +死亡组 (P <0 .0 1)。结论　慢性重型肝炎TBA显著升高 ,肝细胞损伤越严重 ,TBA水平越高。TBA是敏感的肝功能检测指标 ,对慢性重型肝炎的疗效及预后评估有一定的临床价值。     

An additional case of craniodigital syndrome: variable expression of the Filippi syndrome?

We report on a boy with severe growth and mental retardation, syndactyly of toes and facial anomalies. This phenotype can easily be classified in the group of craniodigital syndromes, but it is difficult to make a more clearly defined diagnosis, based on other minor anomalies, because of the presence of overlapping features. On the basis of various pathognomic features, we conclude that our patient could be an additional case of Filippi syndrome. Moreover, newly recognised features in this patient may be due to variability in phenotypic expression.     

普通外科危重患者围手术期的营养支持

目的：研究普通外科危重患者围手术期营养支持的临床疗效。方法：对1995年以来147例普通外科危重患者围手术期营养支持应用情况进行了分析，腹部手术138例（93.9%)，颈部手术9例（6.1%)，采用以全胃肠外营养（TPN）为主的阶段性营养支持方法补充营养。深静脉置管方法：经颈内静脉置管132例（89.8%)、腋静脉11例（7.5%)、股静脉4例（2.7%)，使用单腔导管127例（86.4%)，双腔20例（13.6%)，41例（27.9%)，放置肠内营养（EN）管。106例（72.1%)为TPN→PN 口服饮食→口服饮食，41例（27.9%)为TPN→PN EN→EN→EN 口服饮食→口服饮食。结果：本组营养支持期间无置管和代谢并发症。142例（96.6%)康复，5例（3.4%)死于原发病。结论：普通外科危重患者围手术期营养支持是重要辅助治疗方法，可保证手术安全，促进术后康复。     

退变性腰椎侧凸的外科治疗

目的探讨退变性腰椎侧凸的特点、诊断与治疗。方法2001年7月至2004年1月手术治疗退变性脊柱侧凸患者15例,行后路彻底椎板减压、椎弓根钉棒矫形固定,椎间融合器融合12例,后外侧植骨融合3例,回顾性分析其临床特点、手术方法与效果。结果术后侧凸平均矫正率为42.8%,腰腿痛均消失,下肢麻木等症状减轻,随访6~36个月,植骨融合良好,无融合器移位,矫正度数与椎间隙高度无丢失。结论成人退变性腰椎侧凸发病年龄大,多合并腰椎管狭窄、失稳等,腰腿痛原因复杂,治疗的主要目的是彻底减压,通过矫形使脊柱重新获得稳定,椎弓根钉棒固定及椎间融合是有效的治疗方法。     

Anterior lumbar instrumentation improves correction of severe lumbar Lenke C curves in double major idiopathic scoliosis

Fifteen skeletally immature patients with double major adolescent idiopathic scoliosis with large lumbar curves and notable L4 and L5 coronal plane obliquity were retrospectively studied. Seven patients who underwent anterior release and fusion of the lumbar curve with segmental anterior instrumentation and subsequent posterior instrumentation ending at L3 were compared with eight patients treated with anterior release and fusion without anterior instrumentation followed by posterior instrumentation to L3 or L4. At 4.5 years follow-up (range 2.5-7 years), curve correction, coronal balance and fusion rate were not statistically different between the two groups; however, the group with anterior instrumentation had improved coronal plane, near normalangulation in the distal unfused segment compared with the group without anterior instrumentation. In cases involving severe lumbar curvatures in the context of double major scoliosis, when as a first stage anterior release is chosen, the addition of instrumentation appears to restore normal coronal alignment of the distal unfused lumbar segment, and may in certain cases save a level compared with traditional fusions to L4.     

Evaluation and Treatment of Children with Primary Immune Deficiency in the Emergency Department

The potential for morbidity and mortality in patients who have PID with febrile and nonfebrile illness is extremely high. Familiarity with the clinical manifestations of PID and collaboration with a pediatric immunologist are prerequisites for optimal short-term care of these complex patients. Conservative management with empiric broad-spectrum antimicrobials, early and aggressive surgical debridement of abscesses, and admission at a tertiary pediatric care center are often indicated.