妊高征合并胎儿发育迟缓患者分娩方式与围产儿预后-20年回顾分析

目的为了探讨妊高征(PIH)合并胎儿宫内发育迟缓(IUGR)患者分娩方式对围产儿预后的影响.方法回顾性分析近20年来在我院分娩的355例PIH合并IUGR患者分娩方式与围产儿预后的关系.结果355例IUGR围产儿,死亡24例(67.6‰),新生儿窒息75例(21.13%).其中阴道分娩组(n=230)围产儿死亡22例(95.7‰),新生儿窒息56例(24.35%);剖宫产组(n=125)围产儿死亡2例(16‰),新生儿窒息19例(15.2%).两组围产儿死亡率差异有显著意义(P=0.008),新生儿窒息率差异无显著意义(P=0.06).剖宫产组中,急诊剖宫产组(n=64)围产儿死亡2例(31.25‰),新生儿窒息15例(23.44%);择期剖宫产组(n=61)国产儿无死亡,新生儿窒息4例(6.56%).两组围产儿死亡率差异无显著意义(P=0.493),新生儿窒息率差异有显著意义(P=0.017).阴道分娩组围产儿死亡的主要原因为胎儿宫内缺氧,而剖宫产组为孕妇严重并发症.结论分娩方式是影响PIH合并IUGR围产儿预后的关键环节.控制PIH病情,减少孕妇并发症,胎儿适时脱离宫内缺氧环境,对改善围产儿预后致关重要.     

168例新生儿先天性心脏病的临床与病理

报道经尸检证实的活产新生儿心脏病168例,占同期活产新生儿尸检的14％。先心病占新生儿畸形的首位。心脏畸形比较发病率以室间隔缺损(VSD)最高,其次为房间隔缺损(ASD),两者共占尸检总数的40％,明显高于国外资料。ASD和VSD病人死于心脏外原因者占100％和84％,左心发育不良综合征、大动脉转位、肺动脉闭锁伴室间隔完整,死于心脏原因者分别占100％、80％和78％。本文30例伴有心外畸形(17.7％),其中Down's综合征、无脾综合征各4例。现公认孤立性先心畸形为多基因遗传,而伴有心外畸形或伴综合征分别为染色体或可能为单基因遗传。     

先天性心脏病缺陷监测及干预对策

目的分析本地区围产儿先天性心脏病缺陷发生的动态变化,为有关部门制定干预措施提供医学依据.方法按照国家出生缺陷监测方案,对江门市区1998年1月～2003年9月的围产儿出生缺陷监测分析.结果监测38421例围产儿,先天性心脏病缺陷104例,发生率2.71‰.6年的资料分析,先天性心脏病顺位由1998年的第4位,逐渐上升至近年排首位,总出生缺陷的构成比例呈上升趋势,先天性心脏病死亡发生占33.65%.Ⅲ级医院先心病比例最高,其次为Ⅱ级医院.讨论根据本地区的监测情况,提出先心病的三级预防、干预对策.     

177例围产儿先天性畸形病理尸检分析

目的提高临床对胎儿先天性畸形的检出率及准确率。方法回顾绍兴市妇幼保健院病理科2006年至2013年370例围产儿死亡资料,分析围产儿先天性畸形的发病率及发病类型。结果 8年来围产儿尸检共370例,其中先天性畸形围产儿177例,占47.84%,成为围产儿死亡的首要原因,其中排在前三位分别为中枢神经系统(31.64%)、心血管系统(24.29%)、颌面五官系统(15.83%),三者占71.76%,是胎儿先天性畸形的常见类型。结论尸检有助于明确先天性畸形的发生类型,了解围产儿畸形发生的规律,进一步证实、澄清及矫正诊断,为临床积累宝贵的经验。     

149例围产儿死亡原因分析

目的通过对我院2005～2009年间围产儿死亡原因和死亡构成比的分析,为制定有效的干预措施提供科学依据,从而提高围产保健质量,进一步降低围产儿死亡率。方法对我院2005～2009年围产儿死亡原因进行回顾性分析。结果 5年间我院住院分娩围产儿数18 581例,围产儿死亡数149例,围产儿死亡率平均为8.26‰,呈逐年下降的趋势。其中,本市户籍围产儿死亡占死亡率的8.75%,非本市户籍占91.25%。导致围产儿死亡的主要原因有胎儿因素、出生缺陷、脐带胎盘因素、母体因素、原因不明及其他。结论加强对围产儿保健者的培训,28周前发现畸形、预防早产、治疗妊娠合并症、加强监护有助于减少围产儿死亡的发生。     

深圳地区2008年~2013年围产儿出生缺陷监测资料分析

目的了解深圳地区围产儿出生缺陷的发生情况,为制定针对性干预措施、降低出生缺陷提供依据。方法依据《中国出生缺陷监测方案》,将深圳市妇幼保健信息系统中监测的2008~2013年围产儿出生缺陷资料导出后进行回顾性分析。结果深圳市2008~2013年围产儿出生缺陷总的发生率为22.53‰,随着年份增长围产儿出生缺陷发生率逐年增长(趋势χ2=286.47,P=0.000)。6年间出生缺陷发生率前5位依次为:先天性心脏病(6.29‰)、多指(趾)(2.11‰)、外耳其它畸形(1.07‰)、唇裂合并腭裂(1.03‰)和马蹄内翻足(0.77‰)。除先天性心脏病和多指(趾)都位于前两位外,不同年份的其它出生缺陷顺位有所不同。确诊时间在产前诊断的占34.47%,产后7天内占62.68%,产后7天后占2.85%;超声波(52.02%)、临床(39.41%)和染色体(2.18%)是诊断的主要措施。结论深圳市近几年出生缺陷发生率逐年增多,尤其以先天性心脏病比例最高,应制定一、二级预防措施,减少出生缺陷率。     

8579例围产儿中出生缺陷报告

总结辽河油田1986年10月至1991年12月共5年零3个月的围产儿出生缺陷监测结果。8579例围产儿中发现出生缺陷者70例,出生缺陷发生率为8.16‰,低于87年辽宁省平均水平(11.99‰),且发生率呈逐年下降趋势。不同人群的畸形发生率不同,农村高于城镇,孕_1产_1的畸形发生率低,科技人员及干部发生率低,母亲文化程度高发生率低;35岁以上年龄组发生率明显增高。70例畸形儿中单发畸形53例,多发畸形17例,畸形种类35种,受累部位共计99处。受累部位以中枢神经系统占首位,为33.3％,其次为肌肉骨骼系统及面颈部。畸形病种前5位依次为无脑儿、脊柱裂,唇 腭裂、脑积水马蹄内翻、足月睾丸未降。8579例围产儿中死亡138例,死亡率为16.09‰,低于87年辽宁省平均水平(22.01‰)。70例畸形儿中活婴43例,活产畸形发生率5.09‰。138例围产儿死亡中死因顺位前五位为畸形、脐带因素、早产、宫内窘迫、胎盘因素,其中畸形死亡居首位,占全部死亡例数的21.74％。     

APC法综合分析205例围产儿死因

目的：分析围产儿死亡原因，指导临床实践。方法：结合尸体解剖（autopsy,A），胎盘病理检查（placental parhologi-cal examination,P）及临床资料（clinical data，C）的分析方法，即APC法。结果：我院1993年8月至1998年12月共有205例围产Ⅱ期的围产儿死亡，除去先天异常所致围产儿死亡79例，效正围产儿死亡率18.4‰，围产儿死因中先天异常占38.5‰，缺氧50例，为24.4%，宫内感染19例，占9.3%，新生儿透明膜病18例，占8.8％，其它各种原因所致围产儿死亡39例，占19％。结论：引起围产儿死亡的主要原因是畸形、缺氧、宫内感染、早产。开展优生咨询，加强围产期保健，提高合并症及并发症的治愈率是降低围产儿死亡率的关键。     

海丰地区7402例围产儿出生缺陷监测分析

目的 探讨本地区出生缺陷发生态势及预防措施。方法 对1998年～2002年7402例围产儿监测结果进行回顾性临床分析。结果 5年出生缺陷平均发生率13.5‰,其顺位排列前5位是胎儿水肿,神经管畸形(脑积水、无脑儿、脑膨出)、多指、唇腭裂;农村的发生率明显高于城镇(P<0.05),经产妇高于初产妇(P<0.01),>35岁发生率高于其它年龄组(P<0.01);监测的围产儿死亡率25.3‰,出生缺陷儿的死亡率为68‰,占围产儿死亡35.8％。结论 本地区出生缺陷的高发类型与遗传、环境、营养相关,一、二级预防对减少出生缺陷的发生至关重要。     

121例围产儿死亡分析

本文回顾分析１２１例围产儿死亡，围产儿死亡率１６．４２‰。其中死胎７１例占５８．６８％，早期新生儿死亡４１例占３３．８８％，死产９例占７．４４％。畸形是引起围产儿死亡首要因素，共４５例占３７．１９％。而畸形儿中，又以α－地中海贫血巴氏水肿胎儿（ＨｂＢａｒｔ＇ｓ水肿儿）最多共１６例，占畸形的３５．５５％。本文指出畸形是围产儿致死的首要因素，影响优生。做好产前咨询和国产保健，开展产前诊断，都可以有效地预防畸形儿的出生，可以预测胎儿的健康情况，从而降低围产儿死亡率，更好做到优生。     

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.     

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.     

'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.     

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.