腭裂分子发病机制的新进展

Cleft palate represents one of the major groups of congenital birth defects in the human population.Despite recent advancements in medical intervention,babies born with cleft palate often suffer multiple handicaps that signifi-cantly compromise the quality of their lives.Investigations of craniofacial development and malformations utilizes     

RETINOIC ACID DOWN-REGULATES BONE MORPHOGENETIC PROTEIN 7 EXPRESSION IN RAT WITH CLEFT PALATE

Objective To evaluate the effects of retinoic acid （RA） on expression of bone morphogenetic protein 7 （ BMP-7 ） in rat fetus with cleft palate, and the effects of RA on proliferation and apoptosis of osteoblasts. Methods All-trans RA （ATRA） was used to induce congenital cleft palate in Wistar rat. BMP-7 mRNA expression in maxillary bone tissue of fetal rats was measured by Northern blotting analysis. Flow cytometry and MTF assay were used to measure the apoptosis and proliferation of ATRA-treated MC-3T3-E1 cells. BMP-7 mRNA and protein expressions in ATRA-treated MC-3T3-E1 cells were detected by RT-PCR and Western blotting analysis. Remilts ATRA could induce cleft palate of rat fetus. The incidence rate of cleft palate induced by 100 mg/kg AT-RA （45.5%） was significantly higher than 50 mg/kg ATRA （ 12.5%, P 〈 0. 05 ）. BMP-7 mRNA expression decreased in maxillary bone tissue of rat fetus with cleft palate. MC-3T3-E1 cells proliferation treated with 1 × 10^-6 mol/L ATRA decreased by 60%, the cell apoptosis increased by 2 times. BMP-7 mRNA and protein levels in MC-3T3-E1 cells treated with 1 × 10^-6 mol/L ATRA decreased by 60% and 80%, respectively, compared with ATRA-untreated cells （ P 〈 0.05 ）. Conclusions BMP-7 may play an important role in embryonic palate development. RA may possess the ability to down-regulate cell proliferation through regulation of BMP-7 gene expression.     

A RETROSPECTIVE STUDY OF BILATERAL ALVEOLAR BONE GRAFTING

Objective. To evaluate the treatment results of bilateral alveolar bone grafting (BABG) in patients with bilateral complete clefts of lip and palate. Methods. A retrospective study was performed in 66 bilateral complete cleft lip and palate patients who received the procedure of BABG, among them 15 were primary BABG and 51 were secondary BABG.The patients were further divided into three groups according to age and eruption stage of the canine at the time of surgery. The result of BABG was evaluated on the radiographs. Results. (1)The overall success rate of BABG was 75.0%, with 83.3% and 72.5% for primary and secondary BABG respectively; (2)The marginal bone level was found to be significantly higher in the youngest age group than in the other groups both for primary and secondary BABG; (3)For both primary and secondary BABG, Group C (patients‘ age more than 16 years) had the least optimal success rate, with 66.7% and 65.4% respectively. Conclusion. Simultaneous primary palate repair and BABG is safe and feasible procedure for treating unoperated bilateral complete cleft lip and cleft palate patients. For both primary and secondary BABG,significantly better results can be achieved if the operation is performed before eruption of the canine.     

Evaluation of speech function on repairing defects of maxilla and palate with temporalis muscle flap

Objective： To evaluate the speech function on repairing defects of maxilla and palate with temporalis muscle flap after benign or malignant turmor resection. Methods： The lateral cephalogram and speech intelligibility were detected in 19 cases with the operations of repairing defects of maxilla and palate by temporalis muscle flap, and their recovery of the speech function were analyzed. Results： Among the 19 patients, there were 15 cases （78.00%） with complete velopharynx, 3 cases （15.80%） with mafiginal velopharynx, and 1 case （5.26%） with insufficient velopharynx. The average speech intelligibility was 94.3%, close to the normal speech intelligibility. Conclusion： The operation of repairing defects of maxilla and palate with temporalis muscle flap can reconstruct the phonatory structure, preserve the palate function and restore the speech function after operation.     

Treatment of cleft lip and cleft palate.

The first case of cheiloplasty recorded was in China at about 200 BC. The technique of course has been vastly improved over the centuries. Cleft lip and/or cleft palate are among the most common congenital anomalies in China and the world. There is controversy over some of the surgical techniques used and age for operation, especially in cleft palate, but the condition requires surgery. Unilateral cleft lip is operated on at 3-6 month of age and bilateral cleft lip at 6-12 month of age. Views on palatoplasty are: 1. Cleft palate should be repaired at an early age; 2. Supplementary orthodontic treatment to expand the upper arch postoperatively is necessary, and orthognathic surgery is performed if needed when the patient has matured. This is the best choice at present.

     

Evaluation of airway obstruction at soft palate level in male patients with obstructive sleep apnea/hypopnea syndrome: dynamic 3-dimensional CT imaging of upper airway

This study examined the dynamic characteristics of upper airway collapse at soft palate level in patients with obstructive sleep apnea/hypopnea syndrome(OSAHS) by using dynamic 3-Dimensional(3-D) CT imaging.A total of 41 male patients who presented with 2 of the following symptoms,i.e.,daytime sleepiness and fatigue,frequent snoring,and apnea with witness,were diagnosed as having OSAHS.They underwent full-night polysomnography and then dynamic 3-D CT imaging of the upper airway during quiet breathing and in Muller’s maneuver.The soft palate length(SPL),the minimal cross-sectional area of the retropalatal region(mXSA-RP),and the vertical distance from the hard palate to the upper posterior part of the hyoid(hhL) were compared between the two breathing states.These parameters,together with hard palate length(HPL),were also compared between mild/moderate and severe OSAHS groups.Association of these parameters with the severity of OSAHS [as reflected by apnea hypopnea index(AHI) and the lowest saturation of blood oxygen(LSaO2)] was examined.The results showed that 31 patients had severe OSAHS,and 10 mild/moderate OSAHS.All the patients had airway obstruction at soft palate level.mXSA-RP was significantly decreased and SPL remarkably increased during Muller’s maneuver as compared with the quiet breathing state.There were no significant differences in these airway parameters(except the position of the hyoid bone) between severe and mild/moderate OSAHS groups.And no significant correlation between these airway parameters and the severity of OSAHS was found.The position of hyoid was lower in the severe OSAHS group than in the mild/moderate OSAHS group.The patients in group with body mass index(BMI)≥26 had higher collapse ratio of mXSA-RP,greater neck circumference and smaller mXSA-RP in the Muller’s maneuver than those in group with BMI<26(P<0.05 for all).It was concluded that dynamic 3-D CT imaging could dynamically show the upper airway changes at soft palate level in OSAHS patients.All the OSAHS patients had airway obstruction of various degrees at soft palate level.But no correlation was observed between the airway change at soft palate level and the severity of OSAHS.The patients in group with BMI≥26 were more likely to develop airway obstruction at soft palate level than those with BMI<26.     

BIRTH DEFECTS EPIDEMIOLOGY IN THE BEIJING- TIANJIN AREA

A survey and analysis of 208 801 deliveries in 16 hospitals in the Beijing-Tianjin Area discloses birth defects (BD) of 58 categories in the period of 1970- 1984. The overall BD prevalence rates varied from 8.7% t0 17.2%. They were significantly higher in rural than in urban and suburban areas. The 6 leading BD were: CNS defects, other skeletomuscular defects, cleft lip and/or cleft palate, GI tracUabdo minat wall defects, reduction deformity/other limb defects and polydactyly and syndactyly. Twelve common BD with prevalence rates higher than 0.2%。 were: spina bifida, anencephalus, hydrocephaly, varUq and vulgus deformity of the foot, cleft lip and cleft palate, cleft lip alone, cleft palate alone, omphalocele/ gastroschisis, other abdominal wall defects, polydacty- ly and deformity of multiple systems. Most common was neural tube defects which were sttrprising by as high as 6.6%. The male-to-female ratio of birth defects was 0.82, but those of individual categories varied considerably. Data from 10 hospitals show that seasonal variations of both overall BD and CNS defects have the same pattern, peaking in October. Seasonal distribution of BD in the cities, countryside, overall BD and some individual defects all differ.     

LncRNA H19 and Target Gene-mediated Cleft Palate Induced by TCDD

This study investigated the role of long non‐coding RNAs(lnc RNAs) in the development of the palatal tissues. Cleft palates in mice were induced by 2,3,7,8‐tetrachlorodibenzo‐p‐dioxin(TCDD). Expression levels of long non‐coding RNA H19(lncR NA H19) and insulin‐like growth factor 2(IGF2) gene were measured by quantitative real‐time polymerase chain reaction(q RT‐PCR). The rate of occurrence of cleft palate was found to be 100% by TCDD exposure, and TCDD could cause short upper limb, cerebral fissure, webbed neck, and short neck. The expression levels of lnc RNA H19 and IGF2 gene specifically showed embryo age‐related differences on E13, E14, and E15 in the palatal tissues. The expression levels of lnc RNA H19 and IGF2 gene showed an inverse relationship on E13, E14, and E15. These findings demonstrated that lnc RNA H19 and IGF2 can mediate the development of mouse cleft palate.     

Three-dimensional finite element analysls for external midface distraction after different osteotomy in patients with cleft lip and palate

<正>Objective To study three - dimensional finite element analysis for external midface distraction after different osteotomy in patients with cleft lip and palate ( Clp) . Methods Three - dimensional Fem models of Le Fort Ⅰ,Ⅱand Ⅲ,osteotomy in Clp patients were estabolished. External midface distraction were simulated. An anteriorly and inferiorly directed 900 g force was applied     

完全性唇腭裂婴儿期手术对上颌骨发育的影响

目的：观察婴儿早期行唇腭裂手术对完全性唇腭裂患儿上颌骨发育的近期影响。方法：18例完全性唇腭裂患儿在婴儿期接受唇腭裂手术作为唇腭裂修复组,随访时平均年龄（10.15±0.76）岁;10例未行唇腭裂手术的单纯唇裂患儿为单纯唇腭裂组,随访时平均年龄（10.25±0.75）岁;另20例正常的10岁龄学童作为正常对照组。三组分别取头颅侧位片与上牙颌模型,进行测量分析。结果：两组唇腭裂患儿之间的上颌骨发育无明显差异（P〉0.05）,与正常对照组比较,均有生长抑制（P〈0.05）。结论：早期对完全性唇腭裂患儿行唇裂修复术对上颌骨发育的影响不大,还可以使患者获得正常的语言功能。     

唇腭裂合并的单侧牙槽突裂大鼠模型的建立和稳定性研究

目的建立唇腭裂合并的上颌骨缺损（牙槽突裂）大鼠模型,并研究其稳定性。方法以7周龄SD大鼠作为实验对象,于右侧上颌制备4mm×4mm×3mm的牙槽突裂,模拟建立唇腭裂合并的上颌骨缺损大鼠模型。根据术后骨缺损区的处理方式将模型大鼠分为对照组（骨缺损区不予特殊处理）和实验组（骨缺损区予以骨蜡填塞）,每组10只。两组大鼠分别于术毕即刻和术后4、8周时点分批处死后制备模型标本;利用显微CT（MicroCT）进行三维重建,观察和定量检测骨缺损区新骨形成情况,分析和比较两组模型的稳定性。结果 MicroCT三维图像重建显示,在术后8周时点,对照组有大量新骨充填于缺损区,缺损区明显缩小;而实验组仅断端边缘有少量新骨形成,缺损区无明显缩小。定量分析显示,对照组和实验组术后4周时点的新骨形成百分比分别为54.35%和16.53%,术后8周时点的新骨形成百分比分别为93.12%和29.30%,两组间比较差异均有统计学意义（P〈0.05）。结论成功建立模拟唇腭裂合并的上颌骨缺损（牙槽突裂）大鼠模型,利用骨蜡填塞骨缺损区能显著提高模型的稳定性。     

骨劈开、牙槽嵴扩张结合GBR技术同期上颌前牙区种植的临床效果

目的：探讨骨劈开、牙槽嵴扩张结合GBR技术同期上颌前牙区种植的临床效果。方法便利选取2011年1月—2015年6月该院收治的96例上前牙种植区剩余骨量不足的患者采用骨劈开、牙槽嵴扩张结合GBR技术治疗,1年随访后评定治疗效果。结果术后初期、术后3个月、术后1年牙槽骨高度分别为(10.25±1.19)、(9.35±1.24)、(9.15±1.19)mm,ISQ分别为(76.88±4.51)、(91.88±4.91)、(92.98±2.19)。种植前牙槽骨唇腭向平均宽度为(3.2±0.3)mm,、治疗后平均增加骨量、牙槽骨唇腭向平均宽度分别为(4.4±0.1)、(7.5±0.2)mm。修复后1个月、修复后3个月、修复后1年满意度评分分别为(72.2±5.1)分、(90.4±5.3)分、(92.4±7.3)分。术后3个月和术后初期的牙槽骨宽度、牙槽骨高度比较结果差异有统计学意义(P<0.05),但术后1年与之相比,差异无统计学意义(P>0.05);种植后牙槽骨唇腭向平均宽度和平均增加骨量与种植前相比,差异有统计学意义(P<0.05);术后1年的满意度评分、种植体稳定性评分与术后初期进行统计比较,差异有统计学意义(P<0.05)。结论骨劈开、牙槽嵴扩张结合GBR技术同期上颌前牙区种植可取的理想的短期治疗效果,但需进一步观察和评定远期疗效。     

成年腭裂患者颅面形态的X线头影测量研究

采用X线头影测量的方法对60例成年腭裂患者进行颅颌面部软硬组织测量,并与60例正常成人进行对照。结果发现,与对照组比较,硬组织方面腭裂患者SNA(上牙槽座角)、N-A-Po(颌凸角)和ANS-Ptm(上颌长)等结果偏小,差异有统计学意义(P<0.01);而软组织方面腭裂患者G-Sn-Po’(面型角)、ULP(上唇突度)减少,差异有统计学意义(P<0.01)。表明硬组织方面腭裂患者的上颌骨存在明显的发育不足、硬腭长度过短、下颌颏部前伸现象明显,下颌骨向下旋转及后退。而软组织方面腭裂患者面中1/3凹陷明显,上唇突度不足。     

上颌前牵引联合腭部扩弓的三维有限元生物力学研究

目的：探讨上颌前牵引过程中,模拟腭部扩弓对上颌骨的位移变化的影响规律,为临床上唇腭裂患者的正畸治疗提供理论依据。方法：在自主探索建立的唇腭裂上颌骨三维有限元模型的基础上,利用ANSYS 10.0软件模拟上颌前牵引和上颌扩弓,分析比较前牵引力作用下上颌骨的位移变化。结果：模拟前牵引加腭部扩弓治疗,上颌骨发生向前、向下、向外的增长。有扩弓治疗的水平位移值明显减小。健侧和患侧牙弓均未出现牙弓内缩的变化。结论：上颌前牵引联合应用腭部扩弓治疗唇腭裂患者,有利于全面改善上颌骨的发育。     

鼻-牙槽突矫治器用于单侧完全性唇腭裂患儿的术前治疗效果观察

目的观察术前应用鼻一牙槽突矫治器在单侧完全性唇腭裂患儿正畸治疗中的疗效。方法选取我院2011年1月至2013年1月间收治的40例单侧完全性唇腭裂患儿为观察对象,随机均分两组,观察组患儿接受术前应用鼻一牙槽突矫治器正畸治疗,对照组患儿不进行术前正畸治疗。比较两组患儿治疗前后上唇裂隙、上前牙槽突裂隙、鼻底宽度、鼻小柱长度以及鼻翼外观满意度等。结果两组患儿术前上唇裂隙宽度、上前牙槽突裂隙宽度、鼻底宽度、鼻小柱长度比较差异均无统计学意义（P〉0．05）。观察组患儿治疗后上唇裂隙宽度、上前牙槽突裂隙宽度和鼻底宽度分别为（5．10±1．83）mm、（6．40±2．01）mm和（z3．29±2．19）mm,均小于治疗前,鼻小柱长度为（3．89±1．10）mm,大于治疗前,其比较差异均有统计学意义（P〈O．05）。观察组患儿治疗后上唇裂隙宽度、上前牙槽突裂隙宽度和鼻底宽度均小于对照组,鼻小柱长度大于对照组,其比较差异均有统计学意义（P〈O．05）。对照组患儿治疗后上唇裂隙宽度和上前牙槽突裂隙宽度分别为（8．93±1．73）mm和（9．49±1．81）mm,均大于治疗前,其比较差异均有统计学意义（P〈O．05）;对照组患儿治疗后鼻底宽度和鼻小柱宽度分别为（29．29±2．11）mm和（1．26±0．22）mm,均大于治疗前,其差异均无统计学意义（P〈0．05）。观察组患儿家属对鼻翼外观的满意度为（8．94±1．10）分,大于对照组的（4．09±1．13）分,比较差异有统计学意义（P〈0．05）。结论术前鼻一牙槽突矫治器治疗作为单侧完全性唇腭裂患儿手术治疗前重要的步骤,可明显减少患儿上唇裂隙宽度、上前牙槽突裂隙宽度,增加鼻小柱长度,减少鼻底宽度,提高手术治疗效果。     

完全性腭裂腭形态学定量统计分析研究

目的：研究完全性腭裂患者不同年龄段腭裂腭形态学定量测量统计分析,为完全性腭裂取得最佳的近期和远期效果而选择最佳的手术方案提供参考依据,并且对完全性腭裂上腭在各个年龄阶段的发育趋势进行初步探讨。方法对82例完全性腭裂患者根据手术时年龄段分成7个组,分别于手术前测量腭健侧和裂侧的直线长度、曲线长度、硬腭长、软腭长、上颌结节至裂隙距离、软腭最宽度、两侧上颌结节距离和上颌结节间裂隙宽度等指标,统计分析。结果≤12月组的上腭曲线长度健侧与裂侧的长度比较具有统计学意义,健侧长于裂侧。而在10～15周岁时,硬腭长度、上颌结节至裂隙缘长度在健侧与裂侧的比较均有统计学意义。不同年龄组的上颌结节间距与上颌结节间裂隙的宽度存在线性关系。结论不同年龄段完全性腭裂患者,上腭健侧和裂侧组织发育不一致。为完全性腭裂手术术式以及序列治疗方案,提供重要的参考依据。     

未经手术修复的恒牙列期腭裂患者牙颌模型分析

目的分析未经手术修复腭裂患者的牙颌形态学特征。方法以40例未接受手术修复的恒牙列期腭裂患者作为研究对象,其中单侧完全性唇腭裂（UCLP）患者15例,中位年龄20.2岁;单纯腭裂（ICP）患者25例,中位年龄21.8岁。分别对两组患者上颌牙颌模型进行相关指标测量和分析,以40例年龄、性别匹配的AngleⅠ类错畸形患者作为对照组。结果与对照组比较,UCLP组患者前段牙弓宽度缩窄,前段牙弓长度及总牙弓长度明显缩短,差异有统计学意义（P〈0.05）;ICP组患者后段牙弓宽度增宽,前段牙弓长度和总牙弓长度明显缩短,差异有统计学意义（P〈0.05）。结论未接受手术修复的腭裂患者均存在上颌矢状向发育障碍;其中UCLP患者前牙区存在一定的横向发育不足。     

牵引成骨技术在矫治唇腭裂继发重度上颌发育不全畸形中的应用

目的　探讨颌骨牵引成骨技术在矫治唇腭裂继发重度上颌发育不全畸形中的应用价值及不同牵引方式的选择。方法　 8例患者 (男 6例 ,女 2例 ) ,年龄 11～ 2 5岁。其中不全腭裂 1例 ,单侧完全腭裂 4例 ,双侧完全腭裂 3例。所有患者均伴有重度上颌发育不全畸形 ,7例患者采用改良高位台阶式LeFortⅠ型截骨术 ,1例儿童患者行LeFortⅠ型截骨后采用颅外固定牵引装置完成牵引。手术前术后均投照定位头颅正位、侧位片及曲面体层片、颞颌关节薛氏位片 ,并在模型外科上准确测量牵引距离 ,设计牵引方向 ,牵引完成后即开始术后正畸 4个月后拆除牵引器。结果　 8例患者均按设计要求完成预定牵引。除 1例右侧上颌窦外侧壁截骨线处约有 1cm× 0 .5cm的骨缺损外 ,其余病例牵引区均有致密新骨生成 ,术后咬合关系稳定 ,无感染及其他并发症发生。经平均 2 0个月的术后随访观察 ,上颌骨及咬合关系均稳定 ,无明显复发。 8例患者平均牵引上颌骨向前达 12mm(5～ 15mm) ,患者的上牙槽座角由术前的平均 71°增加到术后的 79° ,所有病例均达到容貌及咬合关系恢复正常。结论　牵引成骨技术是矫治唇腭裂继发重度上颌骨发育不全畸形的有效方法 ,且不需植骨、效果稳定、对腭咽闭合的影响较小 ,值得推广。     

上颌前牵引对唇腭裂上颌骨影响的三维有限元生物力学研究

目的:探讨不同方向的前牵引力,对上颌骨的应力、应变和位移变化的影响规律,为临床唇腭裂患者的正畸治疗提供科学依据.方法:在自主探索建立的唇腭裂上颌骨三维有限元模型的基础上.利用ANSYS 10.0软件模拟上颌前牵引,分析比较在相同力值,不同牵引方向前牵引力作用下上颌骨的应力和位移变化.结果:前牵引力为500 g/侧,前牵引方向向前下20°～45°之间时,上颌骨发生向前、向下及向外的位移变化,上颌腭部牙弓形状发生内缩变化.内缩变化程度与前牵引方向有密切关系;前牵引方向与功能(牙合)平平面夹角越小,牙弓内缩越明显,区域越大,牵引方向与(牙合)平面夹角越大,内缩程度越轻,区域越小.结论:在前牵引力作用下,上颌骨发生向前、向下及向外的增长,上颌牙弓形状发生内缩变化,变化的程度与前牵引方向有密切的关系.