脑白质病变的研究进展

脑白质病变与临床上许多疾病密切相关,而且影响到了有关疾病的预后。本文就近年来脑白质病变的研究进展进行了综述,希望能为今后的研究提供新的思路。     

West syndrome associated with Down syndrome: Case report and literature review

West syndrome is the most frequent cause of epilepsy in Down syndrome. West syndrome is often associated with poor long-term prognosis in most of children. We report a girl with West syndrome associated with Down syndrome which occurred at 8 months of age for repetitive flexor spasms and electroencephalography (EEG) showed hypsarrhythmia. She had Down syndrome facies, microcephaly, psychomotor development delay and axial hypotonia. Computed tomography of the brain was normal. Her karyotype was 47, XX, +21. Phenobarbital therapy was immediately effective with good clinical control of seizures, while the EEG monitored after one month was unchanged. At 2 years of age, the patient had hypertonic status epilepticus following a lung infection. The EEG showed a persistence of hypsarrhythmia. Sodium valproate and hydrocortisone therapy was effective with good seizure control but her psychomotor development was severely impaired. After a follow-up of 7 years, the patient presents growth retardation, microcephaly, severe psychomotor development delay, generalized hypotonia and tetraparesis. Knowledge of West syndrome in Down syndrome allows the early detection and prompt management of this neurological complication in order to optimize psychomotor development and improve the quality of life of these children.     

儿童急性非特异性脊髓炎临床特点及预后

目的 总结儿童急性非特异性脊髓炎的临床特点,分析影响疾病预后的相关因素.方法 收集2003年1月至2012年12月于重庆医科大学附属儿童医院新诊为急性非特异性脊髓炎的75例患儿临床资料,总结疾病临床特点,在部分病例预后随访的基础上,对可能影响预后的危险因素采用SPSS 19.0软件行统计学分析,探讨疾病预后不良的相关因素.结果 本组患儿中,男37例,女38例,发病年龄有两个集中段,分别为1～4岁及9～13岁.75例(100.0％)患儿均有不同程度的肢体瘫痪,以截瘫最多见(62.7％,47/75例);44例(59.0％)伴感觉功能异常,39例有确切的感觉平面;60例(80.0％)有二便功能障碍;38例(50.7％)伴根痛,7例(9.3％)病程中出现球麻痹症状,50例(66.7％)病初1周内伴发热.70例患儿行脊髓磁共振成像(MRI)检查,61例提示信号异常,均有T2相长信号改变.40例成功随访患儿中,预后良好者32例,预后不良者8例.与预后不良相关的因素有发生脊髓休克(P=0.001)、脊髓休克持续时间≥2周(P=0.015)、极期神经功能缺损评分低[(9.25±0.89)比(14.03±2.87),P=0.000]、极期≥12 d(P =0.004)、疾病达峰时间≤24 h(P=0.029)、病初发热(P =0.029)以及女性(P=0.021).结论 急性非特异性脊髓炎以肢体瘫痪为主要表现,二便障碍较感觉异常发生率更高,部分患儿病程中可出现球麻痹表现,脊髓MRI检查常有T2相长信号改变.同时,临床工作中应早期识别本病的预后不良因素,以便对个体预后进行初步评估并指导临床治疗.     

Multicystic dysplastic kidney: Impact of imaging modality selection on the initial management and prognosis

ObjectiveTo evaluate the impact of imaging modalities on the evaluation and prognosis of children with multicystic dysplastic kidney (MCDK) disease.Patients and methodsA retrospective analysis of all children with MCDK diagnosed from 2004 until 2012 was performed. The study included 63 patients for whom all postnatal imaging modalities were available: renal bladder ultrasound (RBUS), dimercaptosuccinic acid scan (DMSA) and voiding cystourethrogram (VCUG). Cases with major congenital abnormalities or incomplete data were excluded. Abnormalities in the contralateral kidney and the fate of MCDK were also addressed.ResultsAt diagnosis, the average age was four-and-a-half months. The majority of cases were detected antenatally (87%). Postnatal RBUS and DMSA scans established the diagnosis of MCDK in 92% and 98% of patients, respectively. DMSA showed photopenic areas in the contralateral kidneys in 10% of patients; all of them had hydronephrosis and were confirmed to have vesicoureteral reflux (VUR). Contralateral VUR was detected in 16 patients; 63% of them had hydronephrosis. After a mean follow-up of three-and-a-half years, involution occurred in 62% of patients and the involution rate was inversely proportional to the initial size.ConclusionsThe classical appearance of MCDK on RBUS was sufficient to establish the diagnosis in most patients. DMSA scan was more accurate in confirming the diagnosis and evaluating the contralateral kidney. Selective screening for VUR in patients with contralateral hydronephrotic kidney should be considered.     

累及第二、三肝门巨大肝母细胞瘤手术切除41例

目的：探讨累及第二、三肝门的巨大肝母细胞瘤手术切除的可行性及手术方法,以期扩大手术适应证,提高小儿肝母细胞瘤的治疗效果。方法我们对41例累及第二、三肝门的巨大肝母细胞瘤施行肿瘤切除手术,患儿年龄3个月至14岁,平均年龄3.8岁;其中男25例,女16例;肿瘤直径8.5～21.6 cm,平均13.5 cm;均行手术前化疗,化疗3至7个疗程不等,直至肿瘤不再缩小。采用Kap-lan-Meier生存分析计算本组患儿生存率。结果41例均顺利实施肝脏肿瘤切除术,手术时间125～350 min（平均220 min）,术中出血40～500 mL,8例未输血。第一肝门阻断时间15～35 min （平均22 min）,其中阻断1次者12例,2次者23例,2次以上者6例,每次阻断时间一般不超过20 min。手术切除方式包括V-Ⅷ段切除8例;Ⅳ-Ⅷ段切除9例;Ⅰ-Ⅳ段切除4例;Ⅰ、Ⅳ、Ⅷ段切除9例;Ⅰ、Ⅳ、Ⅴ、Ⅷ段切除6例;Ⅰ段＋Ⅴ-Ⅷ段切除2例;Ⅰ段＋Ⅳ-Ⅷ段切除3例;合并下腔静脉瘤栓7例。41例患儿1年、3年、5年无瘤生存率分别为92.7％、72.8％和33.5％。其中最长无瘤生存期为90个月。41例术后均经规范化疗,化疗后定期门诊复查,其中20例（48.8％）复发,继续化疗。5例出现肺转移病例中,2例经术后化疗转移灶消失,3例于术后1年内死亡。结论累及第二、三肝门的巨大肝母细胞瘤患儿手术切除虽然具有一定挑战性,但在详细、准确的术前评估,及对肝脏解剖的熟练掌握下,肿瘤切除仍具有一定的可行性和安全性。完整切除肿瘤可有效提高肝母细胞瘤的长期生存率。     

儿童心肌致密化不全的临床表现及预后

心肌致密化不全是一种与基因相关的遗传性心肌病,以心室内异常粗大的肌小梁和交错的深隐窝为特征.心肌致密化不全病因及发病机制不清.心肌致密化不全临床少见,预后较差.该文就儿童心肌致密化不全的临床表现及预后进行阐述,加深对儿童心肌致密化不全的认识,提高临床诊治水平,提高患者生存质量,延长患儿生存期.     

降钙素原在儿童社区获得性肺炎中的诊断与预测价值

降钙素原作为严重细菌感染的早期诊断指标,是反映机体感染程度的一个生物学指标,可对细菌感染进行预测判断.儿童社区获得性肺炎是5岁以下儿童发病和死亡的主要病因,目前诊断主要依靠临床表现、胸片和血常规,但仍缺乏特异性,降钙素原在儿童的诊断和预测价值越来越得到认可.该文主要就降钙素原在儿童儿童社区获得性肺炎中的临床价值作一综述.     

儿童多发性硬化生活能力、神经心理和眼科预后长期随访观察

目的通过对儿童多发性硬化（MS）系统随访,总结其躯体、心理预后及视力特点。方法回顾性收集首都医科大学附属北京儿童医院近10年来经McDonald诊断标准评估确诊的MS病历,对病程3年以上的MS病例进行系统的随访,随访内容包括日常生活活动能力评估（ADL）,扩展的功能障碍量表评分（EDSS）,神经心理评估（抑郁、焦虑、注意力、社会生活能力）和智力检测,眼科检查［最佳矫正视力、光学相干断层扫描(OCT)测量视神经纤维层(RNFL)厚度、图形视觉诱发电位(P-VEP)］等,对预后进行综合分析。 结果23例MS患儿进入分析。男10例,女13例,平均起病年龄7.7岁;复发缓解型20例,进展复发型2例,继发进展型1例。23例均复发,次数1～10次。平均随访7.7（3.6～14）年,均行ADL评分,完全依赖和中度依赖各1例,均为进展复发型;16例行EDSS评分,EDSS 0分 11例,EDSS≤2.5分4例,EDSS 4.0分1例（为进展复发型）;15例完成神经心理检查,韦氏智力测验2例低于平常,2例边界,1例社会适应能力临界,6例存在注意力缺陷,4例存在轻度焦虑情绪,均无抑郁情绪。16例患儿32眼完善眼科检查,30眼矫正视敏度0.5～1.5,视野无异常,余2眼仅存光感;合并视神经炎MS患儿眼底均提示视神经萎缩,OCT检查结果显示视神经眼在各个象限的RNFL厚度与无视神经炎眼相比明显变薄（P＜0.001）;P-VEP检查中视神经炎眼较无视神经炎眼P100潜伏期明显延长,振幅明显减低（P＜0.05） 结论儿童MS复发缓解型预后尚好,多合并视神经萎缩,但无视力严重受损;其他类型预后差,多遗留躯体残疾及视力残疾,OCT检查可对视神经受累程度定量测定。儿童MS可出现认知损害、焦虑和注意力缺陷等多种神经心理问题。     

3种方式处理下颌骨对舌鳞癌患者手术预后的影响

目的：探讨舌癌病人下颌骨的不同处理方法与手术远期预后的关系,为舌癌患者的手术选择提供参考。方法：随访71例接受手术治疗的舌癌患者5年,分析术后影响预后因素,评价3种不同的下颌骨处理方式与患者预后的关系。结果：下颌骨处理方式对于舌鳞癌患者生存率有直接影响,其中未处理下颌骨患者5年总体生存率56.3%,边缘性去骨截骨患者的5年总体生存率为53.6%,而节段性截骨患者的仅为51.7%。结论：舌癌的预后不完全由下颌骨的切除方式决定的,而是由其侵袭性决定的,因此对于舌癌患者要根据不同情况选择不同方式处理下颌骨,在提高患者生存率的情况下,尽量保存下颌骨的完整性。     

Radiographic assessment of clinical root-crown ratios of permanent teeth in a healthy Korean population

PURPOSE

The aim of this study was to determine the absolute value of the root/crown ratio (R/C ratio) using panoramic radiographs (PRGs) in a healthy Korean population.

MATERIALS AND METHODS

In total, 99 patient radiographs (of 50 males and 49 females subjects; aged 16 to 24 years old) were examined, and 2,770 teeth were analyzed. Crown lengths and root lengths were measured with modified Lind''s measurements using PACS tools by two examiners in two separate sessions two months apart. All data were analyzed using SPSS. The independent t-test was used to assess for gender differences, and the paired t-test was used to compare both arches with a significance level of P<.05.

RESULTS

The mean R/C ratios varied from 1.29 to 1.89 (male: 1.28-1.84; females: 1.31-1.94). The highest R/C ratios were recorded for the mandibular canines (1.89), followed by the maxillary canines (1.79). The lowest R/C ratios were recorded for the maxillary second molars (1.31). In comparison with the maxillary teeth (1.29-1.78), the mandibular teeth yielded the higher R/C ratio (1.47-1.89), and this difference was significant in the females (P<.05). The difference between the genders was not statistically significant, except for the maxillary central incisors, mandibular canines and mandibular first premolars.

CONCLUSION

These data may enhance the understanding of the clinical R/C ratio as a useful guideline for determining the status of teeth and the ethnic difference.