多功能验光试镜装置的研制

本文介绍了一种多功能验光试镜装置的设计和应用，通过比较，得出该试镜架比之传统器械具有的优势，在可调散光轴位、可调瞳距、可调镜腿、可调鼻托方面更方便、简捷，且重量轻更加契合人体生理特点，进一步满足临床对眼镜度数的检查需要。最后提出了一些改进意见。     

鼻中隔偏曲矫正手术40例临床体会

目的：探讨鼻中隔偏曲矫正术的临床应用效果。方法：回顾性分析40例鼻中隔偏曲矫正术的手术方式、效果、并发症防治措施。结果：40例患者均一次完成手术,术中出血约3—5ml不等,1例出现穿孔,1例中隔血肿,无脓肿、塌鼻、脑脊液鼻漏、矫正不全等其他并发症出现。结论：鼻内镜下鼻中隔局部偏曲的手术,骨膜分离的少,黏膜的损伤小,术后填塞少,患者的痛苦小,手术时问短,达到了微创的手术目的,提高了手术的疗效。     

原发性结外淋巴瘤在耳鼻及咽部的临床特点

目的 总结原发性结外淋巴瘤在耳、鼻及咽部的临床表现,以提高及时确诊率。方法 回顾性分析16例原发性结外淋巴瘤在耳、鼻及咽部的临床表现特点,特别是罕见的中耳淋巴瘤和少见的副鼻窦淋巴瘤的临床表现。结果 中耳淋巴瘤1例,以伴有疼痛的慢性分泌性中耳炎(传导性聋)、轻度面瘫为特点;扁桃体淋巴瘤7例,以咽异物感及单侧扁桃体肿大为特点;鼻及副鼻窦淋巴瘤2例,以血涕、鼻臭、下鼻甲黏膜粗糙,增厚为特点;鼻咽部淋巴瘤以血涕、头痛为特点;口咽部淋巴瘤以咽痛、发热、软腭溃疡、口臭为特点。结论 在发现耳、鼻及咽部病变时,掌握原发性结外淋巴瘤在耳、鼻及咽部的临床表现特点,及时行病理检查,是及时确诊的关键。     

Myosite à inclusions trompeuse : savoir répéter la biopsie musculaire

Inclusion body myositis, although rare, is the commonest cause of myopathy in patients aged over 55 years. We report a 62-year-old man presenting a glycogen storage disease type 2 mimicking an inclusion body myositis.     

Analysis of the cleft lip–nose in the submental-vertical view. Part II. Panel study: Which is the most important deformity?

BACKGROUND: In part I, we presented an anthropometric measurement instrument that uses standardized facial pictures in the submental-vertical view, Adobe Photoshop 7.0, and Scion Software for Windows to determine both form and symmetry of the cleft nose. This instrument was found to be both reliable and universally suitable for comparing results of cleft rhinoplasties. However, the quality of the overall result needs to be based on weights assigned to different measurable parameters/deformities. Therefore, we carried out a panel study to evaluate the relative importance of the different parameters/deformities according to the parents of the patients. MATERIALS AND METHODS: A questionnaire with both pictorial and textual representations of 10 distinct nasal cleft deformities was sent twice to the parents of 14 complete unilateral and 14 complete bilateral cleft lip-nose patients. The deformities were rank-ordered by the parents, and the responses were analyzed for intraobserver and interobserver reliabilities. RESULTS: The most important deformity according to both groups was the asymmetric position of the nose within the facial frame. Alar position was also important whereas nostril form was least important. There was fair test reliability for both intraobserver and interobserver rankings. CONCLUSION: Rankings of the different nasal cleft deformities by the parents of cleft patients are reproducible and, hence, a useful preparatory guide for surgeons.     

Proboscis lateralis

Proboscis lateralis is a rare craniofacial malformation for which no embryological basis has been established. The condition presents with a tubular, nose like structure arising from the medial canthal area or orbit roof. We describe here such a case that was surgically treated.     

Nablus syndrome: Easy to diagnose yet difficult to solve

Nablus syndrome was first described by the late Ahmad Teebi in 2000, and 13 individuals have been reported to date. Nablus syndrome can be clinically diagnosed based on striking facial features, including tight glistening skin with reduced facial expression, blepharophimosis, telecanthus, bulky nasal tip, abnormal external ear architecture, upswept frontal hairline, and sparse eyebrows. However, the precise genetic etiology for this rare condition remains elusive. Comparative microarray analyses of individuals with Nablus syndrome (including two mother–son pairs) reveal an overlapping 8q22.1 microdeletion, with a minimal critical region of 1.84 Mb (94.43–96.27 Mb). Whereas this deletion is present in all affected individuals, 13 individuals without Nablus syndrome (including two mother–child pairs) also have the 8q22.1 microdeletion that partially or fully overlaps the minimal critical region. Thus, the 8q22.1 microdeletion is necessary but not sufficient to cause the clinical features characteristic of Nablus syndrome. We discuss possible explanations for Nablus syndrome, including one-locus, two-locus, epigenetic, and environmental mechanisms. We performed exome sequencing for five individuals with Nablus syndrome. Although we failed to identify any deleterious rare coding variants in the critical region that were shared between individuals, we did identify one common SNP in an intronic region that was shared. Clearly, unraveling the genetic mechanism(s) of Nablus syndrome will require additional investigation, including genomic and RNA sequencing of a larger cohort of affected individuals. If successful, it will provide important insights into fundamental concepts such as variable expressivity, incomplete penetrance, and complex disease relevant to both Mendelian and non-Mendelian disorders.     

Valvular aortic stenosis, left anterior cusp hypoplasia and obstruction of left main coronary artery ostium by a congenital membrane

Aortic stenosis and obstruction of the left main coronary artery ostium is very rare. This report describes the case of one patient with valvular aortic stenosis, left anterior cusp hypoplasia and obstruction of the left main coronary artery by a congenital membrane. Surgical treatment with aortic valve removal and excision of the congenital membrane was successful.     

Percutaneous transluminal renal angioplasty in hypertension

The aim of our study was assess anatomical and functional results of renal artery angioplasty with and without stenting in 25 hypertensive patients (8 female and 16 male, 42.6 and 61.6 years old respectively) with significative renal artery stenosis (RAS) (atherosclerotic: 22; fibrodysplastic: 3). Eleven patients had simple angioplasty and 13 had stenting. The rate of angioplasty success was 96%. In the stent group, the anatomical result was better: 2% of residual stenosis versus 24% in the other group (p < 0.001). Restenosis occurred in 2 patients. Immediately after revascularisation arterial blood pressure decreased from 195/105 +/- to 150/85 +/- mmHg in-group without stent (p < 0.001) and from 190/100 to 145/85 mmHg in the group (p < 0.001). The value of serum creatinine concentration in patient with renal failure didn't change after revascularization.     

短鼻畸形自身软骨植入术后护理的研究进展

探讨短鼻畸形自身软骨植入手术的术后护理。术后护理是决定鼻部整形手术成败至关重要的环节。术后护理技术的充分、规范,通常会让短鼻畸形自身软骨手术取得一个意想不到的好效果。