PAO治疗成人DDH的预后影响因素

目的：探讨影响泊尔尼髋臼周围截骨术（PAO）治疗成人发育性髋关节发育不良（DDH）预后的相关因素,为临床工作提供参考.方法：总结相关文献报道,全面研究及评估可能影响PAO治疗成人DDH预后的各种因素.结果：对于合并早期骨关节炎及关节匹配度良好的DDH患者,应用PAO手术可以获得相对令人满意的预后;对于DDH合并股骨近端畸形的患者,在术后获得股骨头良好覆盖及关节面匹配度良好的前提下,采用PAO治疗DDH预后良好;而术前合并髋关节半脱位及假臼形成的患者预后较差.结论：影响PAO治疗成人DDH预后是多种因素共同参与的,其临床参考价值亟待提高.     

髋关节发育不良伴股骨前倾角异常增大成人患者联合截骨术的疗效

目的 对比年龄≥30岁伴股骨前倾角异常增大（>40°）髋关节发育不良患者（developmental dysplasia of the hip, DDH）接受髋臼周围截骨术（periacetabular osteotomy, PAO）联合股骨近端去旋转截骨术（derotational osteotomy, PFDO）与单纯PAO的手术效果。方法 选取2012年1月至2020年1月在解放军总医院第四医学中心骨科医学部接受PAO联合PFDO的年龄≥30岁伴股骨前倾角异常增大（>40°）的DDH患者,共计55例,按照接受手术方式不同,分为联合手术组（n=29）和PAO组（n=26）。联合手术组接受PAO联合PFDO治疗,PAO组仅接受PAO治疗。比较两组患者的LCEA、ACEA、T?nnis角术前及术后1年的差异,比较两组患者术前、术后6个月、术后1年及末次随访（>2年）的改良Harris评分和iHot-12评分。结果 共计随访55例患者,随访时间为2年1个月～9年11个月。术后6个月,联合手术组与PAO组的改良Harris评分和iHot-12评分相近（P>0.05）...     

髋关节发育不良继发骨性关节炎的全髋置换术治疗现状

目的 总结全髋关节置换术(THA)在髋关节发育不良(DDH)继发性骨性关节炎治疗中的研究现状。方法 在万方数据、PubMed 数据库以“髋关节发育不良、全髋置换术”查阅2009 年1 月—2015 年9月收录的与DDH、骨性关节炎、THA相关的文献进行汇总分析。结果 DDH患者骨与软组织解剖个体差异较大,涉及的解剖异常包括髋臼异常、股骨侧畸形及软组织改变三大方面。THA术前对DDH患者的解剖异常的正确评估和术中髋臼、股骨端及软组织的得当处理是提高手术疗效的关键。结论 DDH患者的THA需要谨慎的术前计划,个体化地选择合理的治疗策略,提高手术疗效。     

基于CT数据的3D打印技术在儿童发育性髋关节脱位手术中的应用

目的探讨基于髋关节CT影像数据的3D打印技术在儿童发育性髋关节脱位(DDH)手术中的应用价值。方法选取行手术治疗的DDH患儿62例,根据制定的手术方案分为3D打印组(30例)和常规手术组(32例)。3D打印组术前根据患儿髋关节CT扫描数据进行3D模型打印,并制定手术方案、模拟手术操作,根据3D模型行Pemberton髋臼截骨联合股骨截骨术;常规手术组根据术前三维CT图像制定的手术方案进行Pemberton髋臼截骨联合股骨截骨术。记录并比较2组患者手术时间、髋臼指数矫正、沈通氏线是否连续、术中出血量等。结果 2组手术时间、术中出血量比较,差异均有统计学意义(P 0.05)。2组髋关节髋臼指数矫正、术后沈通氏线连续比较,差异无统计学意义(P 0.05)。结论基于髋关节CT影像数据的3D打印技术辅助行DDH手术能显著缩短手术时间,减少术中出血量,提高手术成功率,改善预后,为患儿提供了更精准、个体化的诊疗方案。     

31例发育性髋关节脱位的手术治疗

目的：探讨发育性髋关节脱位（DDH）手术治疗方法的选择及手术操作技巧。方法：术前充分骨牵引后,对其中25例30例髋采用Salter骨盆截骨术或改良Salter骨盆截骨术,4例5例髋行髋臼加盖术,2例2侧髋行沙氏手术（Zahradnicek手术）同时行Chiari骨盆内移截骨术（术中同时短缩股骨1.8-2cm),术后用双髋穗形石膏外固定4－6周,拆除石膏后康复训练。结果;全组31例患者均获3－5年随访。根据SeverinX线评定标准和Mckey临床评定标准,31例37例髋中优32例（86％）,仅一例为差（3％）,无一例伤口感染及截骨处不愈合。结论;对于闭合复位失败及年龄较大（6岁以上）的患儿根据其年龄及病理特点,选择合适的手术方法,其治疗效果令人满意。     

利用有限元方法对髋臼弧形截骨术进行术前规划

目的 对已截骨的髋臼块进行不同角度旋转,以模拟手术中髋臼块需调节的不同角度,通过分析髋关节周围软骨接触应力以及接触面积确定最佳角度,为髋臼截骨提供个体化方案.方法 建立髋关节发育不良(development dysplasia of hip,DDH)和正常有限元模型,探讨发育不良髋臼形态特征以及应力集中原因.对DDH模...     

文章导读

<正>发育性髋关节发育不良(developmental dysplasia of the hip,DDH)是一种与先天异常相关,并且随生长发育不断变化的髋关节疾病,包括髋臼发育不良、股骨头半脱位和全脱位。DDH是骨科较为常见的、在患者不同年龄阶段都有“标准化”手术治疗策略的疾病。DDH手术治疗策略大致分为保髋手术和关节置换手术。新生儿及婴幼儿DDH多无症状,如被确诊,往往采取保守治疗。但随着年龄增长,患者会出现髓痛、易于疲劳及活动受限等症状。不同患者的髋关节病变严重程度有很大不同,治疗方法也各异。同一患者在不同年龄阶段的治疗也不一样,从初期的吊带、石膏等保守治疗,到之后的截骨矫形手术、微创髋关节镜修复手术,直至最终的人工髋关节置换术,手术难度及手术创伤也随之增大。     

股骨大转子长斜形截骨联合人工全髋关节置换术治疗成人高脱位DDH的疗效分析

目的探讨股骨大转子长斜形截骨在治疗成人高脱位发育性髋关节脱位(developmental dislocation of hip DDH)中的临床疗效。方法实验组:选取2012年2月至2015年2月本院收治的11例高脱位DDH患者的临床资料,11例患者均采用全髋人工关节假体置换联合股骨大转子长斜形截骨钢丝内固定术。其中,男3例,女8例。年龄45～72岁,平均56.5岁,术后末次随访體关节功能按改良Merle d'Aubingne-Postel评分系统评估。对照组:通过医院病历管理平台获得自2009年1月至2012年1月本院采用全髋人工关节假体置换联合股骨大转子下横形截骨术治疗17例患者的临床资料,最后电话得到随访的有12例,获取病历数据得知12例患者的平均手术时间、出血量等相关数据。将实验组与对照组的数据进行对比分析。结果实验组11例患者均得到随访,随访时间12～18个月,平均15.8个月。平均手术时间(120.05±32.50) min,术中出血量(270.15±72.50) mL,末次随访髋关节功能按改良Merle d'Aubingne-Postel评分系统来评估:优7例,良3例,可1例,差0例,优良率90.91%。无1例发生坐骨神经损伤。对照组平均手术时间(160.08±95.50) min,术中出血量(380.02±75.70) mL,优良率91.67%。有1例坐骨神经损伤,末次随访时已恢复。两组病例治疗效果的优良率通过χ~2检验,P0.05。手术时间及出血量通过t检验,p0.05。结论采用股骨大转子长斜形截骨能够获得与转子下横形截骨相同的临床效果,但其手术时间、术中出血量明显减少,术中手术创伤减小、手术操作难度降低。     

Bernese髋臼周围截骨治疗髋臼发育不良

背景：改变髋臼朝向是纠正髋臼发育不良的最好方法,而Bernese髋臼周围截骨是当前最常应用也是最成功的治疗方法之一。 目的：总结分析Bernese髋臼周围截骨的适应证及技术和影响效果的因素,以期更好的应用于临床。 方法：使用“Bernese osteotomy,Ganz osteotomy,periacetabular ostetomy”作为关键词,采用计算机检索PubMed、Elsevier和Springer数据库中2012年12月以前的相关文章。纳入与Bernese髋臼周围截骨治疗在髋臼发育不良患者中临床应用的相关文献;排除重复研究和Meta分析类文章。重点对Bernese髋臼周围截骨治疗的适应证及技术和治疗效果的影响因素进行综合分析。 结果与结论：计算机初检得到483篇文献,根据纳入排除标准,对其中43篇文献进行分析。Bernese截骨术是采用改良的Smith-Peterson入路在髋臼周围进行多边形截骨。虽然手术技术逐渐改进,但是主要截骨步骤一直保持不变,只是改良了软组织松解方法。影响Bernese髋臼周围截骨治疗后效果的直接因素是影像学上髋关节退变和发育不良的严重程度。将来随着对髋关节畸形理解的深入,Bernese髋臼周围截骨治疗的指征、技术方式也将得到发展。     

全髋关节置换术中不同截骨方式行股骨短缩治疗Crowe Ⅳ型髋关节发育不良的疗效分析

目的 研究Crowe Ⅳ型髋关节发育不良(DDH)患者行人工全髋关节置换时采用转子间截骨与转子下截骨治疗的疗效及安全性。方法 回顾性分析1999年5月—2013年12月期间收治的33例(37髋)Crowe Ⅳ型DDH患者,年龄17～67岁,平均35.9岁。在行人工全髋关节置换术中时采取了不同的截骨方式,其中28例(30髋)接受了改进的转子间截骨、5例(7髋)采用了转子下截骨。两组患者均用改良的Merled Aubigné和Postel部分评分进行效果评定,术前平均分别为9.4分、9.7分,下肢长度差异平均分别为44.7 mm、57.5 mm,收集评估临床及影像学检查数据。 结果 平均随访时间为83.6个月,术后转子间截骨与转子下截骨患者下肢的平均长度差异分别是9.2 mm、9.4 mm,末次随访Merled Aubigné评分平均分别为15.9分、15.8分。转子间截骨组患者中有2例术后出现坐骨神经麻痹,但是6个月内症状完全缓解。末次随访中转子间截骨组有2例Trendelenburg征阳性。在随访期内均无假体翻修的患者。各组手术前后Merled Aubigné和Postel部分评分差异均有统计学意义(P值均＜0.05),两组间统计学差异均无统计学意义 (P＞0.05)。结论 人工全髋关节置换术中股骨缩短治疗Crowe Ⅳ型髋关节发育不良时,无论是转子间截骨还是转子下截骨均安全有效。     

Renal dysplasia and asplenia in two sibs

A family is reported in which two sibs, one male and the other female, both died within 24 hours of birth with enlarged polycystic kidneys. Postmortem histology in the second child showed gross renal dysplasia. In both children the pancreas was enlarged, nodular and cystic but the liver appeared macroscopically normal. In the second child, histological examination confirmed pancreatic fibrosis with cystic dilation of ducts, but showed portal fibrosis with bile duct proliferation in the liver.
This combination of findings is very reminiscent of those in a girl and her brother reported by Ivemark et al. (1959). The children reported here also showed absence or hypoplasia of the spleen, cardiac anomalies and other features of the Ivemark syndrome (Ivemark 1955), a quite different, usually sporadic, congenital disorder. It is suggested that the children described here have a distinct lethal congenital disorder, probably inherited in an autosomal recessive manner.     

Schizophrenia in a North Swedish geographical isolate, 1900–1977. Epidemiology, genetics and biochemistry

Over 200 schizophrenic patients belonging to three major and interrelated pedigree complexes have been investigated over the past 30 years in a North Swedish geographically isolated population, presently numbering about 6,000. An intensive investigation of a number of biochemical correlates and genetic markers in a few selected families belonging to one of the major pedigrees has indicated new strategies for the current research program.
Schizophrenia, as defined operationally, is significantly associated with decreased activities of two enzymes (1) blood platelet monoamine oxidase, (2) plasma dopamine-β-hydroxylase, and (3) with the genetic marker Gc² (group specific antigen). Both enzymes are subject to genetic variation. A positive score for linkage between schizophrenia and low plasma DBH activity has been calculated, but, so far, available data are insufficient for discrimination between linkage and partial contribution of genetically controlled low plasma DBH to the pathogenesis of the disease. Alternatively, both mechanisms could be involved.
As a model for continued research, schizophrenia is explained as based on a double dominant-recessive genotype (Aabb), representing a vulnerability which in about 50 % of cases develops into clinical schizophrenia. It is suggested that the dominant mutation (A) operates on or affects MAO activity, and that the recessive genotype (bb) is instrumental in low variates of DBH activity and very likely such variates within the normal range of physiological variation. Moreover, it is suggested that the combined effects of MAO- and DBH-reduced efficiency on the metabolism of e.g. dopamine could be an essential pathogenic mechanism for the schizophrenic illness which is segregating in this population.     

The dominant diseases of modernized societies as omega-3 essential fatty acid deficiency syndrome: Substrate beriberi

About 1900, modern food selection and processing caused widespread $\text{epidemics}$ of the B vitamin deficiency diseases of beriberi and pellagra which, for genetic reasons, often expressed as different diseases ranging from bowel and heart disease to dermatoses and psychoses. But the B vitamins merely help convert essential fatty acids (EFA) into the prostaglandin (PG) tissue regulators and it now turns out that, through hydrogenation, milling and selection of w3-poor southern foods, we have also been systematically depleting, by as much as 90%, a newly discovered trace Nordic EFA (w3) of special importance to primates and sole precursor of the PG3(4) series, even as a concurrent fiber deficiency increases body demand for EFA. Since substrate EFA is processed by many B vitamin catalysts, an EFA deficiency will mimic a $\text{panh}$ypovitaminosis B, i.e., a mixture of $\text{substrate}$ beriberi and $\text{substrate}$ pellagra resembling vitamin beriberi and pellagra but exhibiting as even more diverse $\text{endemic}$ disease. This would consitute a second stage of the Modern Malnutrition and explain why some workers now hold the dominant diseases of modermized societies to be new, nutritionally based, pellagraform yet lipid-related and to range, once again, from heart disease to psychosis. It is an assumption that our dominant diseases are unrelated to each other or are merely revealed by our diagnostic acumen and therapeutic success; and that hydrogenating millions of tons of food oils annually, to destroy the rancidity producing w3-EFA, is safe for $\text{primates}$. Extensive beriberiform disease is reported here in 32 typical cases taken from medical practice which responds strikingly to linseed oil supplements (60% w3-EFA) in confirmation of identical results in Capuchins.     

What will studies of Fulani individuals naturally exposed to malaria teach us about protective immunity to malaria?

There are an estimated over 200 million yearly cases of malaria worldwide. Despite concerted international effort to combat the disease, it still causes approximately half a million deaths every year, the majority of which are young children with Plasmodium falciparum infection in sub-Saharan Africa. Successes are largely attributed to malaria prevention strategies, such as insecticide-treated mosquito nets and indoor spraying, as well as improved access to existing treatments. One important hurdle to new approaches for the treatment and prevention of malaria is our limited understanding of the biology of Plasmodium infection and its complex interaction with the immune system of its human host. Therefore, the elimination of malaria in Africa not only relies on existing tools to reduce malaria burden, but also requires fundamental research to develop innovative approaches. Here, we summarize our discoveries from investigations of ethnic groups of West Africa who have different susceptibility to malaria.     

'Very sore nights and days': the child's experience of illness in early modern England, c.1580-1720

Sick children were ubiquitous in early modern England, and yet they have received very little attention from historians. Taking the elusive perspective of the child, this article explores the physical, emotional, and spiritual experience of illness in England between approximately 1580 and 1720. What was it like being ill and suffering pain? How did the young respond emotionally to the anticipation of death? It is argued that children’s experiences were characterised by profound ambivalence: illness could be terrifying and distressing, but also a source of emotional and spiritual fulfilment and joy. This interpretation challenges the common assumption amongst medical historians that the experiences of early modern patients were utterly miserable. It also sheds light on children’s emotional feelings for their parents, a subject often overlooked in the historiography of childhood. The primary sources used in this article include diaries, autobiographies, letters, the biographies of pious children, printed possession cases, doctors’ casebooks, and theological treatises concerning the afterlife.     

Guidelines for the Validation and Use of Immunoassays for Determination of Introduced Proteins in Biotechnology Enhanced Crops and Derived Food Ingredients

Recent advancements in agricultural biotechnology have created a need for analytical techniques to determine introduced proteins in crops enhanced through modern biotechnology techniques. These proteins are expressed in plant tissues and may be present in food ingredients. Immunoassays are ideally suited for protein detection and may be used as both quantitative and threshold methods. Microplate ELISA and lateral flow devices are two of the most commonly used immunoassay formats for agricultural biotechnology applications. This paper provides general background information and a discussion of criteria for the validation and application of immunochemical methods to the analysis of proteins introduced into plants and food ingredients using biotechnology methods. It is the result of a collaborative effort of members of the Analytical Environmental Immunochemical Consortium. This collaborative effort represents the combined expertise of several organizations to reach consensus on establishing guidelines for the validation and use of immunoassays. Further, the paper offers developers and users a consistent approach to adopting the technology as well as aid in producing accurate and meaningful results.     

HLA in North Colombia Chimila Amerindians

HLA-A,-B,-C,-DRB1 and -DQB1 alleles have been studied in Chimila Amerindians from Sabana de San Angel (North Colombian Coast) by using high resolution molecular typing. A frequent extended haplotype was found:HLA-A*24:02-B*51:10-C*15:02-BRB1*04:07-DQB1*03:02 (28.7%) which has also been described in Amerinndian Mayos Mexican population (Mexico, California Gulf, Pacific Ocean). Other haplotypes had already been found in Amerindians from Mexico (Pacific and Atlantic Coast), Peru (highlands and Amazon Basin), Bolivia and North USA. A geographic pattern according to HLA allele or haplotype frequencies is lacking in Amerindians, as already known. Also, five new extended haplotypes were found in Chimila Amerindians. Their HLA-A*24:02 high frequencies characteristic is shared with aboriginal populations of Taiwan; also, HLA-C*01:02 high frequencies are found in New Zealand Maoris, New Caledonians and Kimberly Aborigines from Australia. Finally, this study may show a model of evolutionary factors acting and rising one HLA allele frequency (-A*24:02), but not in others that belong to the same or different HLA loci.     

Ultracryotomy: development and application (with examples from the yeast cytology) (author's transl)

The preparation steps usually necessary for obtaining ultrathin frozen sections of biological material (chemical prefixation, enclosing, cryoprotective treatment, freezing, sectioning, and post-staining the sections for transmission electron microscopy) are submitted to a critical analysis. The application of cryo-ultramicrotomy, in particularly for cytochemical purposes, is reviewed. Fundamental considerations of chemical prefixation and poststaining are supported by examples from yeast cytology. Furthermore, the efficiency of the cryo-ultramicrotomy (electron optical resolution of ultrastructural details) is demonstrated on yeast cells and protoplasts.     

The universal muscular chamber. A basic unit of the genitourinary, cardiovascular, gastro-intestinal, skeletal, cutaneous, respiratory and other systems, endocameral hypertension; and spasm, the key to their idiopathic diseases and arthropathies

Starting with the integument, we see many organs are contractile sacs or multiples thereof, which tubes or bags constitute the major part of the entire body. Recognition of this basic unit and its characteristics sheds new light, individually and collectively, on many disorders previously considered unrelated. Muscular tears and perforations develop in the walls of these chambers, being no way peculiar to those organs, wherein, hydrochloric acid occurs. So, it is not necessary to explain the absence of excessive acid from patients who exhibit holes in the gastric, uterine, aortic, duodenal, rectal, pulmonary, retina, and other walls. Muscle, not acid is the great common factor relating idiopathic disorders in the gastrointestinal tract to each other and to similar diseases in other systems. When the units are linked together, the lesions tend to appear as arthropathies, i.e. at the joints. Rephrasing common-place observations, frees us from conventional, conceptual cul-de-sacs. An observation is only as good as its interpretation, so all possibilities must be considered, otherwise, we will remain blinded by our misconceptions.