喉部神经纤维瘤的生物学特征及治疗

神经纤维瘤是起源于雪旺氏细胞、周围神经细胞或成纤维细胞的一种良性肿瘤。喉部神经纤维瘤极少见,于1929年由Jackson和Coates首次报道。肿瘤多位于杓会厌襞、披裂或喉部富含周围神经丛处,它可以为无特征性表现的孤立病变,但多数都与1型或2型神经纤维瘤病有关。神经纤维瘤病1型     

神经纤维瘤病Ⅰ型

神经纤维瘤病Ⅰ型(neurofibromatosis typeⅠ,NFⅠ)是一种神经皮肤综合征,表现为皮肤、软组织、骨骼、神经系统及内分泌系统的各种损伤,是原发于神经施万细胞及神经内、外束膜细胞的良性肿瘤,为常染色体显性遗传。     

头颈部神经源性肿瘤的诊治结果

[英]／KempfHG…／／ORL．1995，57（5）-273~278对头颈部神经源性肿瘤患者诊断及治疗结果的回顾性分析。总结了25例，16例为神经鞘瘤，5例为神经纤维瘤及4例为神经源性肉瘤。在13例起源于面神经的神经鞘瘤中，2例位于中耳及乳突并呈现面瘫。有4例表现为1型多发性神经纤维瘤病。有23例作了肿瘤切除术，18例在术中发现来自颅神经主干而7例来自小分支或表现为弥散的多发性神经纤维瘤。所有良性的神经源性肿瘤均作了完全切除而无复发。2例用耳神经自体移植的中耳面神经鞘痛中有1例获得足够的面肌张力。有3例在12～24个月内死于恶性者，其1例…     

头颈部非颅内神经鞘膜瘤的临床特点

神经鞘膜瘤（神经细胞瘤、神经瘤或神经鞘瘤）是神经鞘细胞衍生、有包膜的神经鞘良性肿瘤，神经鞘膜瘤通常是孤立性肿瘤，但在神经纤维瘤病的患者中可见多发性神经纤维瘤。有报道发病率为1／3000。高发年龄20～50岁，男女比例相同。     

头颈部遗传性肿瘤概述

头颈部遗传性肿瘤主要包括家族性头颈部副神经节瘤、多发性内分泌腺瘤病1型、多发性内分泌腺瘤病2型、家族性甲状旁腺功能亢进、家族性甲状腺乳头状癌、神经纤维瘤病I型、神经纤维瘤病II型、家族性鼻咽癌及家族性鳞状细胞癌。本文从头颈部遗传性肿瘤的分类、临床特征、遗传学和治疗等方面做一综述。     

听神经瘤分子生物学研究

听神经瘤起源于前庭神经雪旺细胞。单侧与双侧听神经瘤即Ⅱ型神经纤维瘤病（neurofibromatosis type2,NF2）,其肿瘤形成机制基本相同。大量研究表明NF2基因突变是发病的主要分子机制,其编码蛋白产物的多种表达调控与肿瘤发生密切相关,有研究指出,其它肿瘤相关基因、生长调节基因、神经生长因子、细胞凋亡转录物等可能参与听神经瘤的形成。本文对听神经瘤分子生物学研究的进展加以综述。     

听神经瘤分子生物学研究

听神经瘤起源于前庭神经雪旺细胞。单侧与双侧听神经瘤即Ⅱ型神经纤维瘤病(neurofibromatosis type2,NF2),其肿瘤形成机制基本相同。大量研究表明NF2基因突变是发病的主要分子机制,其编码蛋白产物的多种表达调控与肿瘤发生密切相关,有研究指出,其它肿瘤相关基因、生长调节基因、神经生长因子、细胞凋亡转录物等可能参与听神经瘤的形成。本文对听神经瘤分子生物学研究的进展加以综述。     

误诊为甲状腺肿物的颈部喉返神经神经鞘瘤一例

正神经鞘瘤又称雪旺细胞瘤,是起源于神经鞘膜的良性肿瘤。发生于头颈部者占神经鞘瘤25%~45%~([1]),主要来源于交感神经(占26.9%)、臂丛(占23.6%)、迷走神经(占21.8%)、颈丛(占9.8%)、舌下神经(占3.6%)~([2])。发生在喉返神经者极少见~([3-4]),特别是以呼吸困难为首发症状且无喉返神经受损表现声音嘶哑者极易误诊。2017年7月4日,我们收治从甲状腺外科转入我科的喉返神经神经鞘瘤患者1例,现报告如下。     

Ⅰ型和Ⅱ型复合型神经纤维瘤病一例

神经纤维瘤病（neurofibromatosis，NF）是一种主要由常染色体显性遗传引起的疾病，包括多种基因病变。NF可分为Ⅰ型、Ⅱ型和神经鞘膜瘤病三种。其中Ⅰ型NF相对较多见，Ⅱ型较少见，神经鞘膜瘤病罕见。Ⅰ型和Ⅱ型复合型神经纤维瘤病也较为少见。本文介绍一例Ⅰ型合并Ⅱ型的神经纤维瘤病病例。     

伴有神经纤维瘤病的蝶骨发育不良的有关影象

蝶骨发育不良症是一种罕见,系神经纤维瘤病的典型发现,其原因尚不清楚。虽已证实常染色体占优势的遗传模式,仅占50%,诊断是根据临床特征。骨损害占30～50%,其中有些伴有软组织肿瘤,而另一些是单独发生的。与邻近肿瘤压迫无关的骨发育不良可能是营养紊乱、失神经、血供不足、废用等所致。1933年LeWald首先描述神经纤维瘤病人蝶骨发育不良的X线表现,强调了认识眼眶X线缺损的     

Comparative study on the morphology and the composition of the otoliths in the teleosts

Conclusion. Saccular otoliths of teleosts were mostly larger than utricular otoliths, which might relate to the three-dimensional movement. The large and heavy otolith may be better suited in saccules of the bottom and reef fishes. The quantities of iron in lagenar otoliths were found to be lower than those of birds. The function of the fish lagena remains to be elucidated by further studies. Objective. To evaluate the morphological characteristics and the chemical composition of the otoliths in fishes as related to behaviour and habitat. Materials and methods. We studied the morphology of the otoliths of 18 genera of fishes (81 samples) divided into 3 groups: saltwater fish (13 genera), freshwater fish except for the carp family (3 genera) and carp family fish (2 genera). The otoliths and the living environments were compared. The chemical composition was analysed using a synchrotron X-ray fluorescence analyser. Results. Bottom fishes generally have larger saccular otoliths, and migrating fishes have smaller saccular otoliths. In comparing the bottom/reef fishes and the migrating fishes in salt water, the former tended to have larger saccular otoliths. In saltwater bottom fishes the tendency was found that the thinner the head, the larger was the saccular otolith. We found significant quantities of iron, zinc and manganese in the lagenar otoliths.     

Observations on the stria vascularis of the guinea pig cochlea and the changes resulting from the administration of the diuretic furosemide

An electron microscope examination of the stria vascularis of guinea pigs and the structural changes occurring after administration of furosemide has been made. The use of ruthenium-red, which stains cell coats, has shown that the stria is impermeable to the passive diffusion of material from both the endolymph and the spiral ligament. The first changes after administration of furosemide are observed 4 hours after injection; small spaces develop between the cells. After 5–6 hours the spaces have enlarged but the tight junctions between marginal cells remain intact. The intermediate cells show signs of atrophy. In severely affected animals the intermediate cells have so shrunk that the spaces are very large and the marginal cells clearly resolved. These latter cells show no structural damage. In this severely affected tissue it is seen that the blood vessels are entirely surrounded by marginal cell extensions. It is suggested that the intermediate cells may have a regulatory function. The damage appears to be recoverable, since 6 hours after injection the spaces have decreased in size although some signs of intermediate cell atrophy are still apparent.     

On the High Frequency Transfer of Mechanical Stimuli from the Surface of the Head to the Macular Neuroepithelium of the Mouse

Vestibular macular sensors are activated by a shearing motion between the otoconial membrane and underlying receptor epithelium. Shearing motion and sensory activation in response to an externally induced head motion do not occur instantaneously. The mechanically reactive elastic and inertial properties of the intervening tissue introduce temporal constraints on the transfer of the stimulus to sensors. Treating the otoconial sensory apparatus as an overdamped second-order mechanical system, we measured the governing long time constant (Τ_L) for stimulus transfer from the head surface to epithelium. This provided the basis to estimate the corresponding upper cutoff for the frequency response curve for mouse otoconial organs. A velocity step excitation was used as the forcing function. Hypothetically, the onset of the mechanical response to a step excitation follows an exponential rise having the form Vel_shear = U(1-e^−t/TL), where U is the applied shearing velocity step amplitude. The response time of the otoconial apparatus was estimated based on the activation threshold of macular neural responses to step stimuli having durations between 0.1 and 2.0 ms. Twenty adult C57BL/6 J mice were evaluated. Animals were anesthetized. The head was secured to a shaker platform using a non-invasive head clip or implanted skull screws. The shaker was driven to produce a theoretical forcing step velocity excitation at the otoconial organ. Vestibular sensory evoked potentials (VsEPs) were recorded to measure the threshold for macular neural activation. The duration of the applied step motion was reduced systematically from 2 to 0.1 ms and response threshold determined for each duration (nine durations). Hypothetically, the threshold of activation will increase according to the decrease in velocity transfer occurring at shorter step durations. The relationship between neural threshold and stimulus step duration was characterized. Activation threshold increased exponentially as velocity step duration decreased below 1.0 ms. The time constants associated with the exponential curve were Τ_L = 0.50 ms for the head clip coupling and T_L = 0.79 ms for skull screw preparation. These corresponded to upper −3 dB frequency cutoff points of approximately 318 and 201 Hz, respectively. T_L ranged from 224 to 379 across individual animals using the head clip coupling. The findings were consistent with a second-order mass-spring mechanical system. Threshold data were also fitted to underdamped models post hoc. The underdamped fits suggested natural resonance frequencies on the order of 278 to 448 Hz as well as the idea that macular systems in mammals are less damped than generally acknowledged. Although estimated indirectly, it is argued that these time constants reflect largely if not entirely the mechanics of transfer to the sensory apparatus. The estimated governing time constant of 0.50 ms for composite data predicts high frequency cutoffs of at least 318 Hz for the intact otoconial apparatus of the mouse.     

胃镜联合食管镜取出胃内异物1例

患者,男,49岁,因＂反复中上腹疼痛6年＂于2012年6月12日就诊于我院消化内科。患者有反复胃痛、胃部不适症状,长期按胃病治疗效果不佳。既往有吸毒史、丙型肝炎病史。6年前患者毒瘾发作时曾吞入牙刷,在当地医院复查未发现异物,未进一步诊治。     

Contact between the tectorial membrane and the cochlear sensory hairs in the human and the monkey

European Archives of Oto-Rhino-Laryngology - The surface structure of the organ of Corti and the overlying tectorial membrane were studied in human and monkey cochleas under a scanning electron...     

膈神经与面神经吻合的解剖学研究

目的:探讨膈神经移位与面神经干吻合的解剖学基础。方法:解剖11具22侧成年尸体头颈部,观察并测量:①膈神经根与面神经总干腮腺区分叉处之间的距离;②膈神经干在颈部可利用长度;③面神经总干腮腺区分叉处游离至水平半规管下缘可利用长度,面神经总干腮腺区分叉处游离至水平半规管下缘较游离至茎乳孔处增加的面神经干可利用长度;④面神经总干腮腺区分叉处至水平半规管下方切断处膈神经干及面神经干神经移位后并列放置重合长度。结果:①膈神经根与面神经总干腮腺区分叉处之间的距离为(7.2±0.9)cm。②膈神经起点至锁骨下静脉上缘平面为(7.2±1.6)cm。③面神经总干腮腺区分叉处至水平半规管下方切断处的长度2.7~3.5 cm,较面神经干自茎乳孔下方切断增加1.0~1.5 cm。④神经移位后重合长度为0.4~1.8 cm。结论:在锁骨下静脉上缘平面切断20侧标本膈神经可与自乳突内切取的面神经干无张力吻合,两侧膈神经不能与自乳突内切取的面神经干无张力吻合。