紧闭环路系统在新生儿和婴儿唇腭裂修复术麻醉中的应用

【目的】评价紧闭环路系统在新生儿和婴儿唇、腭裂修复术麻醉应用中的安全性和有效性。【方法】49例唇、腭裂新生儿和婴儿拟在全麻下行修复术。全身麻醉气管插管后,行机械定压模式呼吸通气30min,监测动脉血气值、SpO2、肛温、PetCO2、气道压、呼吸频率、心电等参数。【结果】新生儿和婴儿唇、腭修复术全身麻醉控制呼吸过程中,根据PetCO2适当调节呼吸参数,采用定压呼吸模式,能够维持PaO2、PaCO2于正常范围。[结论]紧闭环路系统能安全有效地应用于新生儿和婴儿唇、腭裂修复术。     

50例成年唇腭裂患者及其父母心理状况调查

【目的】研究50例唇腭裂患者及其父母的心理健康状况,为改善患者及其父母的心理健康状况提供依据。【方法】采用SCL-90症状自评量表对50名成年患者及30名正常青少年的心理健康状况进行评价。同时采用生活事件量表（life event scale,LES）对唇腭裂患者组父母和正常青少年组父母,以及唇腭裂患者组父亲与母亲间心理状态进行定性和定量分析。【结果】唇腭裂患者SCL-90总分、阳性项目数及抑郁、焦虑和人际关系诸因子分高于对照组,差异有显著性。并且患者父母生活事件总刺激量、负性事件刺激量均高于对照组（P〈0．05）。其中唇裂（CL）、腭裂（CP）、唇腭裂（CLP）三亚组患者及其父母之间的心理健康状况差异均无显著性。【结论】唇腭裂患者及其父母的心理健康状况较差,对唇腭裂患者临床治疗的同时,也要重视患者家庭成员的社会、心理等因素。     

农村唇腭裂患者生活质量及其影响因素的研究

【目的】了解农村成年唇腭裂患者的生活质量状况,探讨影响生活质量的因素,为改善患者的生活质量提供理论依据。【方法】采用生活质量综合问卷(GQOLI-74)对78名农村成年唇腭裂患者及50名农村正常成年人的生活质量进行评价。【结果】农村成年唇腭裂患者生活质量总分及各维度均低于对照组。其中对生活质量影响作用最大的是:心理健康维度。农村唇腭裂患者的生活质量受多种因素影响。【结论】农村成年唇腭裂患者生活质量较差,患者的生活质量受多种因素影响。对唇腭裂患者临床治疗的同时,要重视心理、社会等因素。     

先天性唇腭裂综合序列治疗5O2例临床护理程序效果观察

目的：总结采取综合序列治疗5O2例先天性唇腭裂患者手术前后临床护理经验,旨在进一步提高护理质量。方法：对5O2例先天性唇腭裂患者行手术整复,并给予手术前后临床护理程序,包括健康宣教、心理护理、术前护理、术后护理、出院指导等。结果：本组患者临床效果均满意,414例患者唇部创口一期愈合,外貌明显改善,11例腭裂术后发生腭瘘或复裂,其余患者创口愈合良好,语音明显改善。结论：规范的手术前后临床护理程序有助于唇腭裂患者整复手术的成功。     

孕早期超声观察腭线筛查胎儿唇腭裂

目的 评价孕早期超声观察腭线筛查胎儿唇腭裂的价值。方法 回顾性分析14 360胎接受超声颈后透明层厚度（NT）检查的孕早期胎儿,观察胎儿腭线表现,记录胎儿转归,评价孕早期超声观察腭线筛查胎儿唇腭裂的效能。结果 孕早期超声提示14 327胎（14 327/14 360,99.77%）腭线正常,其中7胎经随访证实存在唇腭裂;33胎（33/14 360,0.23%）腭线异常,其中4胎腭线为小裂隙,随访证实无唇腭裂,29胎随访证实腭线异常,包括小裂隙8胎、大裂隙4胎、前部缺失11胎及腭线变细/变短6胎。孕中期超声提示36胎唇腭裂,并于出生后或经引产证实,包括4胎单纯唇裂、10胎单纯继发性腭裂、17胎单侧唇腭裂,5胎双侧唇腭裂。超声观察腭线预测胎儿唇腭裂的敏感度为80.56%（29/36）,特异度为99.97%（14 320/14 324）,阳性预测值为87.88%（29/33）,阴性预测值为99.95%（14 320/14 327）。结论 孕早期超声观察胎儿NT平面腭线可作为筛查胎儿唇腭裂的指标,值得推广。     

Bilateral cleft lip and palate diagnosed sonographically at 11 weeks of pregnancy

Cleft lip with or without cleft palate is the most common facial malformation in newborns. We report a case of bilateral cleft lip and palate that was detected via transvaginal sonography at 11 weeks and 5 days of gestation. The sonographic findings were confirmed at autopsy.     

Improving cleft palate/cleft lip antenatal diagnosis by 3-dimensional sonography: the "flipped face" view.

OBJECTIVE: Three-dimensional sonography has enhanced the diagnosis of congenital anomalies in the early stages of pregnancy. Both cleft lip and palate remain a diagnostic challenge for the sonographer because of the variable size of the defects as well as their location. Recently, a technique described by Campbell et al (Ultrasound Obstet Gynecol 2003; 22:552-554, 2005; 25:12-18) demonstrated an improved method called the "reverse face" view, which appears to assist in the diagnosis of clefts involving the palate. METHODS: The fetal face was initially examined with the fetus in the supine position. Using 3-dimensional sonography, a static volume was acquired. Following acquisition of the volume, it was rotated 90 degrees so that the cut plane was directed in a plane from the chin to the nose. The volume cut plane was then scrolled from the chin to the nose to examine in sequential order the lower lip, mandible, and alveolar ridge; tongue; upper lip, maxilla, and alveolar ridge; and hard and soft palates. RESULTS: This approach identified the full length and width of the structures of the mouth and palates and allows the examiner to identify normal anatomy as well as clefts of the hard and soft palates. CONCLUSIONS: The fetal hard and soft palates of the mouth can be accessed using a new technique, which we call the "flipped face" maneuver, when an adequate volume of the face can be obtained.     

超声诊断儿童鳃裂畸形

目的 探讨儿童鳃裂畸形的超声诊断价值.方法 回顾15例鳃裂畸形患儿的声像图表现,并与手术、病理结果对照分析.结果 6例为鳃裂囊肿,超声表现为囊性团块,内回声均匀;6例为鳃裂囊肿并瘘管形成,超声表现为囊性团块,并有条状低回声与体表或咽内相通;3例为鳃裂窦道形成.全部鳃裂畸形患儿中13例病灶位于颈前,1例位于锁骨下缘前胸壁,1例位于耳前腮腺区.超声诊断与手术病理符合率为86.70%.结论 超声诊断鳃裂畸形较准确、可靠,可明确其位置及毗邻关系,对临床手术有重要意义.     

OmniView algorithm: a novel 3-dimensional sonographic technique in the study of the fetal hard and soft palates

The purpose of this pictorial essay is to report on the application of OmniView (GE Healthcare, Zipf, Austria), new 3-dimensional sonographic software, and its application in the prenatal sonographic study of the fetal hard and soft palates. We will show that this novel technique is easy and feasible, requires a limited learning curve, and provides correct volume interrogation of the region of interest. The OmniView algorithm may be useful in training programs, and volume data sets can be interpreted by experts in remote sites. Future prospective studies with consecutive patients will be necessary to evaluate whether the routine application of OmniView will increase the prenatal diagnosis of facial clefting, especially those with isolated palate defects.     

鳃裂囊肿和瘘管的CT诊断

目的 提高对鳃裂囊肿CT表现的认识和诊断准确率,方法 回顾分析10例经手术,病理证实的鳃裂囊肿的CT表现,结果 CT表现为位于颈前三角区,胸锁乳突肌前缘的囊性肿块,CT值20－30Hu;合并感染,边界模糊,囊内密度增高2例,囊壁强化,囊性部分无强化,第一鳃裂瘘管1例,CT未显示,结论 CT检查对鳃裂囊肿可以清楚显示囊肿部位,形态,范围,强化特点及其与周围结构的关系,并能做出准确的诊断。     

2008-2011年广东省珠海市围产儿唇腭裂监测流行病学特征分析

目的 了解广东省珠海市2008-2011年围产儿唇腭裂的流行病学特征。 方法 利用珠海市出生缺陷监测系统获得围产儿唇腭裂资料并进行统计分析。 结果 珠海市2008-2011年围产儿总唇腭裂222例,发病率为21.77/万,各年度发病率差异无统计学意义;不同性别、产妇各年龄组发病率差异无统计学意义,城镇组发病率高于农村组;40.50%唇腭裂患儿在围生期死亡。 结论 珠海市围产儿唇腭裂发病率水平相对稳定,发病率较高,应采取综合措施加强围产儿唇腭裂的三级预防。     

Within-day and day-to-day intrarater reliability of ultrasonographic measurements of acromion-greater tuberosity distance in healthy people

Shoulder subluxation is subjectively assessed by a palpable increase in the distance between the acromion and the humerus. Diagnostic ultrasound has potential for objective assessment of this distance. We used portable ultrasound to measure the distance between the acromion and greater tuberosity (acromion-greater tuberosity [AGT] distance) and tested the intrarater reliability in healthy individuals prior to testing on stroke patients. Thirty-two healthy participants aged 51–85 years (mean 64.2±10.5) were recruited. Seated participants were scanned by a physiotherapist trained in shoulder ultrasound. Measurements were recorded on day 1 and again within 2 weeks. Reliability was assessed by intraclass correlation coefficients. The mean AGT distance was 1.68±0.41?cm for the left and 1.78±0.40?cm for the right shoulder. Within-day intrarater reliability coefficients were 0.99 and 0.98 for the left and right shoulders, respectively. Corresponding values for day-to-day reliability were 0.96 and 0.97. Portable diagnostic ultrasound is a quick and reliable method of assessing AGT distance in healthy individuals when measured by the same examiner.     

唇腭裂的超声筛查及诊断价值

目的：研究常规鼻唇冠状切面加特殊切面在胎儿唇腭裂畸形筛查中的应用价值.材料与方法：对11688例,孕24 ～ 30周胎儿唇腭部采用常规鼻唇冠状切面加特殊切面（经唇部冠状面、经双眼标准横断面、经鼻腔标准横断面、经上牙槽突标准横断面、经舌标准横断面、经下牙槽突标准横断面和经鼻正中矢状面）行产前超声筛查,并与出生儿颜面部检查结果进行对照分析,总结鼻唇冠状切面加特殊切面超声扫查法的诊断符合率.结果：11688例胎儿共查出59例阳性患者,阳性率0.50％（59/11688）,单纯唇裂13例,占阳性病例的22.03％ （13/59）,唇裂合并腭裂的46例,占阳性病例的77.97％（46/59）,左侧唇腭裂38例,占64.4％ （38/59）,右侧唇腭裂10例,占16.9％（10/59）,正中唇裂2例占3.39％（2/59）,双侧唇腭裂9例,占12.25％（9/59）.结论：超声诊断胎儿唇腭裂畸形通过观察鼻唇部冠状切面加特殊切面（上牙槽突标准横断面、下牙槽突标准横断面、鼻腔横断面、双眼标准横断面、舌标准横断面、胎儿头颅矢状面）及配合一定手法,可明显提高其诊断率.     

小儿腭裂修复术后低氧血症的观察及护理

用脉搏血氧饱和度仪观察了160例小儿腭裂修复术后低氧血症的发生率及特点,发现有40．6％的病例发生了低氧血症,而且大部分发生在术后30min内,及时氧疗并保持呼吸道通畅是预防和纠正术后低氧血症的关键。     

The premaxillary triangle: clue to the diagnosis of cleft lip and palate.

OBJECTIVE: The prenatal detection rate of cleft lip and palate is low, especially in low-risk patients who undergo targeted sonography. The reason is that evaluating surface anatomy is relatively difficult and requires operator expertise. Our purpose was to describe a technique to improve the diagnostic accuracy of facial clefts (lip and palate) and to assess the feasibility of including this technique as part of standard protocol during targeted imaging. METHODS: A prospective study was done during 2000 through 2002 to evaluate the accuracy of the "premaxillary triangle (PMT) sign": a new sign to diagnose unilateral cleft lip and palate in women referred for prenatal sonography at our center. Patients with only isolated unilateral cleft lip and palate and cleft lip were included in this study. Before this, all examiners were trained to image the PMT. The images were reviewed by a senior consultant. It was later decided to include this sign as part of the protocol of targeted sonography done between 18 and 22 weeks in our institution. However, depending on the fetal position, the PMT was documented even in patients referred for the first time in late second and third trimesters. RESULTS: Twenty-nine cases of isolated facial clefts were diagnosed during the study period, of which 2 had unilateral cleft lip and 27 had unilateral cleft lip and palate. The PMT sign was absent in all cases of unilateral cleft lip and palate but was present in 2 cases of isolated cleft lip without cleft palate. CONCLUSIONS: The PMT sign can be easily incorporated into targeted sonography at 18 to 22 weeks' gestation. Its inclusion would help in increasing the detection rate of unilateral cleft lip and palate. It may also be potentially used for differentiating between isolated cleft lip and cleft lip and palate, which helps in better prenatal counseling.     

Rathke裂囊肿的MRI诊断价值

目的:Rathke裂囊肿的MRI表现及诊断价值。材料与方法:回顾性分析21例经手术病理证实的Rathke裂囊肿的MRI表现,包括病灶的位置、大小、形态及边界,MR平扫、增强信号特点及比邻结构的改变。结果:21例Rathke裂囊肿中位于鞍内16例,位于鞍内及鞍上4例,位于鞍上1例。病灶最大径0.3cm~2.5 cm。病灶呈类圆形10例,椭圆形7例,不规则形4例。MRI平扫信号表现呈多样性,T1WI可呈低、等、高信号,T2WI多呈高信号或稍高、等信号;囊内见漂浮结节1例;增强后病灶均未见强化。部分病例可伴有垂体柄、视交叉及鞍底受压改变。结论:Rathke裂囊肿MRI平扫信号虽然呈多样性,但也有一定的特征性;结合MRI动态增强扫描及延迟增强扫描有助于与其它鞍区囊性病变的鉴别诊断,也有助于提高本病术前诊断准确率。     

MHC结构与功能的研究进展

ＭＨＣ分子首先作为主要组织相容性抗原被发现,进而依此命名。随着其抗原结合分子及Ｔ细胞信使生物学功能本质的揭示,ＭＨＣ为什么是主要组织相容性抗原的谜底就昭然于世。约１０年前,ＭＨＣ分子的抗原结合骨架结构首次被阐明,关于结构与功能的研究就进入了一个方兴未艾的崭新时代,并在这一领域中取得了许多可喜的成果。对此,本概述了近几年来ＭＨＣ结构与功能方面的令人振奋的研究进展。     

IRF6和RARA基因多态性与非综合征性唇腭裂的相关性研究

目的探讨干扰素调节因子-6(IRF6)基因rs2235371位点和视黄酸受体-α(RARA)基因rs2229773位点单核苷酸多态性(SNP)与非综合征性唇腭裂的关系,以及2个位点在患者和健康者之间的基因型和等位基因型频率差异。方法选取153例非综合征性唇腭裂(NSCL/P)患者作为NSCL/P组,体检健康者150例作为健康对照组。运用聚合酶链式反应-限制性片段长度多态性(PCR-RELF)技术,分析IRF6、RARA基因的多态性,比较2组研究对象基因型和等位基因型频率差异。结果 IRF6基因rs2235371位点基因型CC、TT和等位基因C、T频率在NSCL/P组和健康对照组的分布,差异有统计学意义(P0.05),NSCL/P组等位基因C频率高于健康对照组,差异有统计学意义(P0.05)。RARA基因rs2229773位点基因型CT、TT频率在NSCL/P组和健康对照组的分布,差异有统计学意义(P0.05),NSCL/P组基因型为CT杂合子,显著多于健康对照组,差异有统计学意义(P0.05)。结论 NSCL/P与IRF6基因rs2235371位点等位基因C及RARA基因rs2229773位点CT基因型具有相关性。     

牙槽突裂植骨修复术与护理

目的比较自体髂骨骨松质与OAM材料植入修复牙槽突裂的术后效果并做出评价,同时探讨护理因素对成功植骨的影响。方法从临床病例中选取牙槽突裂患者39例,年龄在7~20岁,男女不限。A组(25例)作为对照组用自体髂骨松质骨做牙槽突裂移植修复;B组(14例)则用OAM(BMP与牛松质骨复合材料)植入做牙槽突裂的修复。对两组治疗成功率及平均手术时间、临床住院日做出比较。结果A、B两组牙槽突裂植骨区X线片示均成骨良好,尖牙可在植骨区萌出。经X线片观察和统计学分析处理,可以认为A、B两组牙槽突裂植骨术的临床治疗成功率无显著性差别。B组手术时间,病人平均住院日较A组明显缩短。结论OAM(BMP与牛松质骨复合材料)修复牙槽突裂骨性裂隙区可以诱导成骨,尖牙可在移植区萌出。为牙槽突裂患者的修复,提供了一种新的治疗方法。同时科学的护理对牙槽突裂植骨术的成功至关重要。     

Real-time magnetic resonance imaging aids prenatal diagnosis of isolated cleft palate.

OBJECTIVE: Cleft of the secondary palate without cleft lip is difficult to visualize sonographically. This study was performed to assess the utility of sonography, standard magnetic resonance (MR) imaging, and real-time MR imaging in the diagnosis of isolated cleft palate. METHODS: We prospectively assessed 5 fetuses at risk for isolated cleft palate on the basis of family history, micrognathia, or both, using sonography and standard and real-time single-shot fast spin echo MR sequences. Written informed consent was obtained under our Institutional Review Board-approved Health Insurance Portability and Accountability Act-compliant protocol. Images were assessed for confidence in a diagnosis of cleft or normal palate. Prenatal and postnatal diagnoses were compared. RESULTS: In 3 fetuses, micrognathia was visualized by sonography and MR imaging with standard and real-time sequences. One fetus at 19 weeks had a wide cleft of the entire secondary palate, and another fetus at 33 weeks had a cleft of the soft palate; these defects were seen only with real-time MR imaging. One 35-week gestational age fetus had a cleft soft palate that was visualized on standard and real-time MR imaging. Two fetuses with no abnormalities had the normal midline secondary palate seen only on real-time MR imaging. In all fetuses, real-time images were helpful in assessing the secondary palate because the entire midline naso-oropharynx could be visualized. CONCLUSIONS: Real-time MR imaging allows for rapid assessment of the midline structures, providing accurate diagnosis of isolated cleft palate.