先天性巨细胞病毒是导致儿童非遗传性感音神经性聋最常见的原因.由于缺乏针对孕妇和新生儿巨细胞病毒感染规范的筛查流程,且大多数患儿在出生时表现为无症状感染,临床上常常被忽略.而且在明确诊断后因缺乏特异性的治疗手段,导致患儿出现不可逆的听觉功能障碍.本文就先天性巨细胞病毒感染及其导致的感音神经性聋的临床特点、发病机制、治疗和预防等方面进行综述,以期引起临床医师和科研工作者的重视,早日制订出有效、规范的诊疗策略.

目的 探讨儿童横纹肌肉瘤(rhabdomyosarcoma,RMS)的临床特点、影像学检查及临床病理学特征,以期减少漏诊误诊,提高临床诊断和鉴别诊断水平.方法 回顾性分析3例不典型学龄前期儿童RMS的详细临床资料并复习相关文献.结果 2例以眼部症状为主诉收入院,1例罕见以反复鼻腔出血并睡觉打鼾为主诉收入院,相关影像学检查显示肿瘤多已广泛侵犯破坏周围结构,行相关手术病理确诊,均为胚胎性RMS.免疫组化显示3例患者Myogenin均为阳性,Ki-67(LIt分别约为90％、80％、70％),其中2例VIM阳性,2例DES(部分或者局灶性)阳性,2例CD99阳性.后转入肿瘤科规律治疗.结论 儿童RMS早期临床表现不典型呈现多样化,具有恶性程度高、侵袭性强、进展快等特点,需要根据临床特征结合影像学检查及免疫组化结果确诊.

Objective To investigate the clinical features,diagnosis and treatment of sinusitis with orbital complications and the indications for surgical treatment of sinusitis with subperiosteal abscess(Chandler Ⅲ).Methods In this retrospective study,33 patients with sinusitis and orbital complications were enrolled and the clinical characteristics and treatment outcomes of these patients were examined.Especially,the differences in clinical manifestations were analyzed between patients who underwent conservative treatment and surgical treatment after failure of conservative treatment.Results Thirty-three patients were grouped according to the Chandler stage system:5 cases in stage Ⅰ,11 cases in stage Ⅱ,16 cases in stage Ⅲ and 1 case in stage Ⅳ.The effective rates of conservative treatment for patients in stage I,stage Ⅱ and stage Ⅲ were 20.0%,27.3% and 31.3%,respectively.Patients in stage Ⅲ were more likely to have proptosis(> 4 mm),decreased vision,ophthalmoplegia,diplopia and elevated intraocular pressure in conservative plus surgical treatment group than in conservative treatment group.For patients in stage Ⅲ,the length,width and height of subperiosteal abscess measured on the CT image were significantly less in conservative treatment group than in conservative plus surgical treatment group.All patients recovered well except two patients with impaired visual acuity.No patients relapsed in 6-month follow-up.Conclusion For patients with sinusitis and orbit complications in stage Ⅰ and Ⅱ,conservative treatment could be tried for the first 48 h.Surgery should be performed if the conservative treatment is not effective.The conservative treatment could first be attempted in adult patients at stage Ⅲ who have no manifestations of proptosis(> 4 mm),decreased vision,ophthalmoplegia,diplopia,elevated intraocular pressure,or abscess height > 0.50 cm on CT images.During 48 h surveillance,surgery should be performed quickly for those who have no improvement or the above-mentioned symptoms.Patients at stage Ⅳ should be given surgical treatment in priority.

Objective To investigate the expression of Cav 1.3 calcium channel in adult rat cochlea and study its role in auditory physiology and pathology.Methods The sprague-dawley rats were used as experimental subjects.The distribution of Cav1.3 calcium channel in the cochlea was detected by immunofluorescence technique.The expression of Cav1.3 was measured with Western blot (WB) and RT-PCR.Results Immunofluorescence photographs revealed that Cav 1.3 calcium channel localized in the lateral wall membrane,hair cells,stria vascularis,spiral ganglion cell,spiral ligment,spiral prominence,and limbus laminae spiralis.The results of WB and RT-PCR inform Cav1.3 calcium channel gene (CACNA1D) were measured in the cochlea and kidney.The expression of Cav1.3was mainly in the basilar membrane.Moderate expression was observed in the spiral ganglion and stria vascularis.Conclusion The preliminary study revealed the distribution of Cav 1.3 calcium channel gene(CACNA1D)in adult rat cochle possesses tissue specificity,providing a theoretical basis for further research in auditory physiology and pathology.

Age-related hearing loss (AHL) is one of the most common sensory disorders among elderly persons. The inwardly rectifying potassium channel 5.1 (Kir5.1) plays a vital role in regulating cochlear K+ circulation which is necessary for normal hearing. The distribution of Kir5.1 in C57BL/6J mice cochleae, and the relationship between the expression of Kir5.1 and the etiology of AHL were investigated. Forty C57BL/6J mice were randomly divided into four groups at 4, 12, 24 and 52 weeks of age respectively. The location of Kir5.1 was detected by immunofluorescence technique. The mRNA and protein expression of Kir5.1 was evaluated in mice cochleae using real-time polymerase-chain reactions (RT-PCR) and Western blotting respectively. Kir5.1 was detected in the type II and IV fibrocytes of the spiral ligament in the cochlear lateral wall of C57BL/6J mice. The expression levels of Kir5.1 mRNA and protein in the cochleae of aging C57BL/6J mice were down-regulated. It was suggested that the age-related decreased expression of Kir5.1 in the lateral wall of C57BL/6J mice was associated with hearing loss. Our results indicated that Kir5.1 may play an important role in the pathogenesis of AHL.

Objective To study the expression of plasma membrane Ca2 + -ATPase isoforms 1 -4 and the splice variants at sites A and C in the neonatal rat vestibular organ.Methods Ten rats at postnatal 2 days (P2 ) were decapitated and their vestibular organs (macula utriculi and macula sacculi)were isolated.The total proteins of the vestibular organs were extracted.The expression of PMCA1-4 splice variants at sites A and C was detected by RT-PCR.Results The splice variants of PMCA1-4 at sites A and C in macula utriculi and macula sacculi of neo-natal rat vestibular organs were PMCA1x/b,PMCA2w/(a,b),PMCA3z/(a,b,c)and PMCA4 (x,z)/b.Conclusion The splice variants at sites A and C among PMCA1,PMCA2,PMCA3 and PMCA4 were different in the vestibu-lar organs of neonatal rats,which could be explained that macula utriculi and macula sacculi had different require-ments of Ca2 + turning for these PMCA isoforms.

Objective To study the expression of plasma membrane Ca2+-ATPase isoforms 1~3 (PMCA 1~3 )in the basilar membrane (BM)of the neonatal rat cochlea by Western blot.The PMCA2 content in single BM of the neonatal rat was also examined.Methods Four rats at postnatal 2 days (P2)and 8 days (P8)were respective-ly decapitated and their BMs were isolated.The total proteins of BMs were extracted.The 20μg total proteins were respectively loaded to the gel.The expression of PMCA1-3 was detected by Western blot.Likewise,3μg total proteins from P2 and P8 rat BM were loaded.The expression of PMCA2 was detected by Western blot.Four rats at P8 were decapitated and their BM was isolated.The 5μg,10μg and 20μg total proteins of P8 rat BM were added to the gel and 100 ng,400 ng and 800 ng bovine serum albumin (BSA)were also loaded as reference.After electro-phoresis,the gel was separated into two parts.One part was used for SYPRO staining and the other part was used for PMCA2 detection by Western blot.Results In the 20μg BM total proteins of P2 and P8 rats,the expression of PMCA1 was weak (0.126±0.024,0.131±0.012,respectively),PMCA2 was strong (4.16±0.528,4.25±0.319, respectively),and PMCA3 was barely expressed (0 ).There was a statistical difference among PMCA1 ,PMCA2 and PMCA3(P<0.05).In the 3μg BM total proteins of P2 and P8 rats,the expression of PMCA2 in P8 (4.571± 0.336)was higher than P2 (3.622±0.285).There was a statistical difference(P<0.05).The PMCA2 content in the BM of a P8 rat was about 2 .5 ng.Conclusion There was a different-level expression of PMCA1~3 in the neonatal rat BM with highest expression of PMCA2 ,which could be explained that cochlear hair cells had different requirements of Ca2+ turning for these PMCA isoforms.

目的 观察鼻内镜下“挖隧道”法治疗复杂性鼻中隔偏曲的临床疗效及安全性.方法 对术前经鼻窦CT和鼻内镜检查确认为复杂性鼻中隔偏曲的48例患者,采取全麻鼻内镜黏-软骨膜下隧道技术行鼻中隔偏曲矫正术,术后双侧鼻腔均使用高吸水性膨胀海绵填塞,观察术后患者的手术并发症及不良反应发生情况.结果 术后鼻内镜检查鼻中隔偏曲骨性组织均得到矫正,症状明显改善.所有患者均未发生鼻中隔血肿及外鼻塌陷,平均住院时间约10.5 d,其中5例患者术后有鼻中隔黏膜纵向撕裂伤,长度均小于1.0 cm,发生率为10.41％,随访观察1个月,未见鼻中隔穿孔、鼻腔粘连、鼻梁塌陷发生.结论 黏-软骨膜下隧道解剖技术符合鼻中隔偏曲产生的生物力学特点,对于减少复杂性鼻中隔偏曲手术并发症具有一定优势,值得临床推广应用.

Objective To investigate the expression of α1D subunit of L-type calcium channels (Cav1.3) in C57BL/6 mice cochlea and its correlation with presbycusis.Methods Auditory function was measured with auditory brainstem response(ABR) in C57BL/6 mice at 4,14,24 and 48 weeks.Morphological changes of the inner ear were studied by using hematoxylin and eosin staining.The expression of Cav1.3 channels was detected by immunofluorescence and the expression of CACNA1D mRNA was determined by reverse transcription-polymerase chain reaction (RT-PCR).Results Immunofluorescence photographs revealed that Cav1.3 channels were mainly localized in the hair cell,spiral ganglion cell,spiral ligment,stria vascularis,spiral limbus.The ABR threshold at click(27.08±9.19) dB SPL,4 KHz(23.79± 13.31)dB SPL,8 KHz(18.83± 12.18)dB SPL,was higher in 14-,24-,and 48-week group than in 4-week group (all P＜0.05).The ABR threshold at click(87.81±8.36)dB SPL,4KHz(85.63±9.88)dB SPL,8KHz(79.50±9.83) dB SPL,was significantly higher in 48-week group than in 4-,14-,24-week groups (all P＜0.01).Along with aging,the missing hair cells and spiral ganglion cells were increased,stria vascularis and spiral ligament were atrophied in a certain degree,and the expression of Cav1.3 channels was gradually decreased (4 weeks 218.94 ± 11.29 ; 14 weeks 184.67 ± 11.92 ; 24 weeks 148.18 ± 8.35 ; 48 weeks 98.04±4.52; all P＜0.01).Conclusions The expression of Cav1.3 channels is decreased along with age.Dysfunction or missing of Cav1.3 calcium channels may be related to presbycusis.

ObjectiveTo investigate the expression and distribution of KCNQ1 in stria vascularis of murine cochlea with laser scanning confocal microscopy (LSCM) and immunohistochemistry staining technique. Methods: With LSCM and immunohistochemistry staining, the location of KCNQ1 in cochlea of the CBA/CaJ and KCNQ1 -/ -mice were observed. Results: Immunohistochemical staining showed that the expression of KCNQ1 protein was found at the apical membrane wall of marginal cells in stria vascularis of CBA/CaJ mice. There was no KCNQ1 protein expression in marginal cells of KCNQ1 -/-mice. The fluorescence of KCNQl protein was mainly expressed in the cell membrane of marginal cells of CBA/CaJ mice with LSCM. Conclusion:The expression and distribution of KCNQ1 protein in marginal cells of stria vascularis could be observed with LSCM.

AIM:To investigate the morphological and functional responses of the cochlear lateral wall following sensorineural hearing loss in mouse.METHODS: CBA/J mice of 3-4 weeks old were deafened by co-administration of kanamycin and furosemide.The endocochlear potential(EP) were measured at 0.5,1,2,7,28 and 112 days after administration of the drugs.The morphological changes of the lateral wall,the expression of NKCC1 and α2 Na,K-ATPase were assessed and verified by histological examination.RESULTS: The EP displayed a significant decline 12 h after treatment,followed by complete recovery on the 2nd day.Then the EP maintained at near normal level in the animals deafened for a period up to 112 days.Loss of the hair cells and atrophy of the lateral wall were the main pathological changes observed.The expression of NKCC1 and α2 Na,K-ATPase in the lateral wall was significantly reduced after a long-term deafening.There was also a significant and progressive decrease in stria vascularis thickness while the major cause appeared predominantly to be a reduction in the volume of the marginal cells.CONCLUSION: Atrophic lateral wall is capable of maintaining a normal EP under the condition of severe hair cell loss.