Dentofacial features of a family with Crouzon syndrome. Case reports

Crouzon syndrome is an autosomal dominant condition characterized by craniosynostosis with associated dentofacial anomalies. This paper describes the variable clinical features in affected individuals over two generations of a family with particular reference to the dentofacial deformities and discussion of management strategies.     

Influences of interferon-gamma on cell proliferation and interleukin-6 production in Down syndrome derived fibroblasts

Objective

Down syndrome, a frequently encountered genetic disorder, is usually associated with medical problems related to infectious disease, such as periodontal diseases and prolonged wound healing. Although affected individuals are considered to have clinical problems related to high interferon (IFN) sensitivity, the molecular mechanisms of IFN activities are not completely understood.

Design

Down syndrome derived fibroblasts, Detroit 539 (D1) and Hs 52.Sk (D2) cells, were used. To analyse the expressions of interferon (IFN) receptors and downstream of IFN-γ, western blotting was performed. Cell proliferation was determined by counting cells following trypan blue staining. Media levels of IL-1β, TNF-α, and IL-6 were quantified using ELISA.

Results

IFN-γ receptor 2 and IFN-α receptor 1, but not IFN-γ receptor 1, were highly expressed in D1 and D2 cells, as compared to the control fibroblast cells. Cell proliferation by D1 and D2 cells was lower than that by the control fibroblasts, further, IFN-γ had a greater effect to inhibit cell proliferation by D1 and D2 cells. In addition, IFN-γ treatment increased the phosphorylation of STAT1 and MAPK in D1 cells as compared to normal fibroblasts. Also, the presence of exogenous IFN-γ in the growth medium significantly induced IL-6, but not IL-1β or TNF-α, in D1 and D2 cells.

Conclusion

Taken together, our results are consistent with hypersensitive reactions to IFN-γ seen in patients with Down syndrome and may provide useful information to elucidate the mechanisms of IFN-γ activities in those individuals.     

Dynamic magnetic resonance sialography as a new diagnostic technique for patients with Sjögren's syndrome

Objective: To evaluate the clinical utility of dynamic magnetic resonance (MR) sialographic images as a diagnostic tool for patients with Sjögren's syndrome. Methods: The morphological findings and various kinds of functional parameters in volunteers on dynamic MR sialographic images were compared with those in five patients with definite Sjögren's syndrome. Results: On the MR sialographs of all five patients with Sjögren's syndrome, the so‐called ‘apple‐tree appearance’ was seen. The difference in two functional parameters using the dynamic MR sialographic data was elucidated between the two groups. The maximum area of the detectable ducts in the group of patients was significantly smaller (P < 0.001) than that in the group of volunteers. The ratio of change in the detectable ducts in the group of patients was significantly lower (P = 0.011) than that in the group of volunteers. Conclusions: Our study suggests that dynamic MR sialographic data in addition to MR sialographic images might be useful for the diagnosis of Sjögren's syndrome.     

种植修复Combination综合征临床病例报告

目的探讨Combination综合征病例种植修复设计方法。方法选择上颌牙列缺失、下颌KenneyⅠ类缺损的患者,对满足种植修复条件的7例患者实施上颌种植覆盖义齿或固定桥、下颌种植支持式固定桥修复,观察患者满意度、后牙咬合变化。结果7例种植修复患者临床检查未见种植体松动、固位不良、损坏等问题,种植体周围软组织健康。种植修复患者满意程度较高。结论Combination综合征患者修复宜以种植修复方式提供足够后牙支持,减少前牙剪切力破坏。     

少汗型外胚层发育不全1例

本文报道1例外胚层发育不全病例,并分析外胚层发育不全的临床表现、发病原因及遗传方面的影响因素等.     

一种可调型下颌前移矫治器治疗OSAS患者的临床应用

目的观察一种可调节的下颌前移矫治器治疗阻塞性睡眠呼吸暂停综合征的情况并评价其临床疗效。方法6名UPPP手术效果不佳的OSAS患者戴用矫治器,治疗前后进行多导睡眠仪监测和X线头影测量。结果患者戴用矫治器感觉舒适并自觉戴用。使用该矫治器一个月后,患者的主观症状明显改善,AI、AHI均明显降低(P<0.01),SaO2明显上升(P<0.01)。X线片显示下颌前移(P<0.05)、气道增宽(P<0.05)、软腭和舌体缩短(P<0.05)、舌骨上移(P<0.01)。结论该下颌前移矫治器的设计针对个体,极易调改、轻便舒适、应用灵活,因此疗效好,且制作简单,适于临床推广应用。     

非肥胖型糖尿病小鼠自发性涎腺炎的发生与发展

目的 观察雌性非肥胖型糖尿病（NOD）小鼠自发性涎腺炎的发生发展过程。方法 选择5、10、15、20周龄雌性NOD小鼠各6只。测定刺激全唾液流率（STFR）、施墨试验、唾液总蛋白、常规HE切片及电镜超微结构观察涎腺组织的改变。以BALB／c小鼠作对照。结果 10、15、20周NOD小鼠STFR、唾液总蛋白均明显低于对照组（P〈0．05），相应的下颌下腺分泌颗粒减少。10周雌性NOD小鼠涎腺炎发病率为4／6，15、20周均为6／6。淋巴细胞浸润主要见于下颌下腺，舌下腺极少，腮腺未见。10周时NOD小鼠已有淋巴细胞浸润灶形成。15周显著增多而且面积增加。STFR与淋巴细胞浸润灶数呈负相关。结论 雌性NOD小鼠5～10周淋巴细胞开始浸润下颌下腺，刺激唾液和蛋白分泌降低。不同腺体受累情况不同。     

Zimmermann-Laband综合征概述

Zimmermann-Laband综合征（Zimmermann-Laband syndrome，ZLS）是以发病早期口内弥漫性牙龈纤维增生为特征的一种罕见的颅颌面部畸形，伴有指甲、远节或中节趾骨发育不良或缺失。文章通过文献回顾，就ZLS的可能基因突变位点做一分析，归纳整理ZLS的临床特点、诊断、鉴别诊断及治疗方案，以期为临床诊断治疗ZLS提供依据。     

Prevalence, phenotype and genotype of oral enterococci

This study investigated the prevalence, phenotype and genotype of oral enterococci. Enterococci were detected in oral rinse samples from 11% of 100 patients receiving endodontic treatment and 1% of 100 dental students with no history of endodontic treatment ( P  = 0.0027). All enterococcal isolates were identified as Enterococcus faecalis . Viable counts ranged from 1 × 10 to 6 × 10³ colony forming units per mL of oral rinse sample. Potential virulence traits expressed by oral E. faecalis strains included production of hemolysin ( n  = 4) and gelatinase ( n  = 4), and response to pheromones in E. faecalis culture filtrate ( n  = 1). Six strains produced bacteriocin. All strains were susceptible to ampicillin, benzylpenicillin, gentamicin and vancomycin. There was no evidence of metal-ion resistance. One isolate produced hemolysin, gelatinase and bacteriocin, was resistant to several antibiotics, and responded to the pheromone cPD1. Pulsed-field gel electrophoresis and plasmid analysis showed that oral E. faecalis exhibited widespread genetic polymorphism, with plasmids detected in seven strains.     

激素替代疗法治疗女性灼口综合征的临床观察

目的 :观察尼尔雌醇治疗女性灼口综合征的临床效果。方法 :采用尼尔雌醇辅以孕激素的雌激素替代疗法治疗已停经的女性灼口综合征患者。结果 :激素治疗组与维生素治疗组相比有显著性差异 (P <0 .0 1)。结论 :舌感觉异常多发生在妇女更年期 ,内分泌紊乱起着重要作用 ,激素替代疗法可以明显缓解症状