系统性红斑狼疮孕妇孕产次和受孕时机对新生儿的影响

将３４例妊娠合并系统性红斑狼疮（ＳＬＥ）孕妇分为初孕组和经孕组，比较其新生儿体重和出生时孕周，并对在疾病的不同状态下（缓解期、控制期、活动期和妊娠时初次发病）受孕后新生儿体重、分娩孕周进行比较。结果表明：初孕组新生儿体重明显高于经孕组，分娩孕周差异无显著性；在疾病活动期受孕，其新生儿体重、分娩孕周最低。提示：ＳＬＥ患者应在疾病缓解期或控制期，即停药后１年或用小剂量强的松（每日５～１５ｍｇ）维持量半年以上时受孕，其ＳＬＥ恶化率低，孕妇及新生儿安全性亦较大。同时应尽可能避免多次受孕，以免给下次妊娠带来不良影响。     

妊娠合并系统性红斑狼疮的临床研究

目的探讨妊娠合并系统性红斑狼疮对母婴结局的影响。 方法回顾性分析广州医学院第三附属医院2005年1月至2009年12月近5年收治的34例妊娠合并系统性红斑狼疮患者的临床资料。在医师指导下妊娠,为选择性妊娠组(14例);未在医师指导下妊娠,为非选择性妊娠组(20例),分析比较两组患者分娩孕周及母婴结局。连续变量补体C3、C4进行独立样本t检验。两组率的比较采用Fisher确切概率检验。 结果选择性妊娠组14例中11例病情平稳,足月分娩6例,早产6例,平均分娩孕周为(35.3±4.3)周,新生儿出生体质量(2076.9±192.4) g;非选择性妊娠组20例中12例出现系统性红斑狼疮活动,足月分娩4例,早产6例,平均分娩孕周为(33.6±4.2)周,新生儿出生体质量(1315.0±488.5) g;选择性妊娠的妊娠成功率86%,明显高于非选择性妊娠组的50% (P＝0.035)。 结论妊娠合并系统性红斑狼疮系高危妊娠,存在较高的母婴风险。非选择性妊娠有更多的胎儿丢失率,选择性妊娠和孕期多学科协作可显著改善妊娠结局。     

残角子宫内翻误诊黏膜下子宫肌瘤1例

患者21岁.孕2产2.因产后6d阴道持续出血并脱出肿物1d,于2006-12-07人院.患者1年前妊娠7个月早产,此系第2胎,妊娠39周+于家中阴道分娩一男婴,体质量约3 000g,自述产程进展顺利,胎盘胎膜娩出完整,分娩后出血不多.     

三胎与单胎妊娠胎儿生长发育的比较

目的　探讨三胎妊娠的胎儿发育规律及平均孕周的特点。方法　采用超声测量三胎妊娠的胎儿与单胎妊娠胎儿不同孕周的双顶径、股骨长 ,比较三胎妊娠胎儿的发育状况、出生体重及分娩孕周与单胎妊娠胎儿的差异值。结果　三胎妊娠的胎儿从孕 2 8周起 ,双顶径及股骨长的平均值低于单胎妊娠胎儿 (差异均值分别为 2 1mm和 3 1mm ,P <0 0 1)。三胎妊娠的新生儿出生体重明显低于单胎妊娠的新生儿 (P <0 0 1)。 3 6 2 %的三胎妊娠的新生儿出生体重低于同孕周的第十百分位数 ;95 7%的三胎妊娠胎儿在 3 6周之前分娩 ,平均分娩孕周数为 (3 4 0± 1 6)周 ,较单胎妊娠胎儿的 (3 9 0± 1 6)周明显提前。结论　三胎妊娠胎儿有着与单胎妊娠胎儿不同的发育规律 ,其平均孕周较单胎妊娠明显缩短 ,新生儿出生体重明显降低     

昆明地区正常单胎活产新生儿出生体重十年分析

目的:探讨不同孕龄正常新生儿出生体重经验百分位数,建立新生儿出生体重修匀曲线及百分位数值,获得不同孕龄正常新生儿出生体重标准以及不同孕龄男、女新生儿出生体重标准。方法:回顾分析1999年1月1日~2008年12月31日在我院产科住院分娩,母亲无并发症且新生儿存活无畸形的15830例的出生体重。以4次方方程y=a+bx+cx2+dx3+ex4分别修匀新生儿实际出生体重及男婴、女婴10th、25th、50th、75th、90th百分位数曲线,分别计算修匀后出生体重。结果:(1)获得我院正常妊娠28~42周单胎活产新生儿出生体重10th、25th、50th、75th、90th经验百分位数与修匀后百分位数;(2)我院10年间单胎活产中小于孕龄儿和大于孕龄儿的总体发生率分别为10.16%和10.29%;(3)在我院出生的新生儿中男婴的平均出生体重较女婴高104g。结论:(1)我院正常妊娠28~42周单胎活产新生儿出生体重修匀曲线为监测评估胎儿生长发育和围产儿结局提供了重要正常参考值;(2)我院小于孕龄儿与大于孕龄儿发生率与国外报道近似;(3)男婴平均出生体重大于女婴。     

双胎妊娠结局及分娩时机与分娩方式对新生儿窒息的影响

目的:探讨双胎妊娠结局及不同分娩时机与分娩方式对新生儿窒息的影响。方法:回顾性分析本院462例双胎妊娠孕妇晚期流产及分娩结局资料,以1分钟Apgar评分≤7分为标准诊断新生儿窒息,对比分析不同孕周的晚期流产率,及在不同分娩孕周采用不同分娩方式的新生儿窒息率。结果:(1)双胎妊娠孕妇孕28周前流产42例,主要集中于孕26～27~(+6)周(18例)。(2)孕28周后分娩的活产新生儿共834例,其剖宫产新生儿窒息率(2. 16%)低于阴道分娩(12. 77%)(P0. 05),其中孕28～29~(+6)、孕32～33~(+6)周剖宫产与阴道分娩的新生儿窒息率差异有统计学意义(P0. 05)。新生儿窒息率在孕36～37~(+6)周(0. 44%)明显低于其他孕周(P0. 05)。(3)大胎儿和小胎儿的剖宫产新生儿窒息率均低于阴道分娩(P0. 05),剖宫产中大胎儿新生儿窒息率低于小胎儿(P0. 05)。剖宫产中大胎儿在孕34～35~(+6)周的新生儿窒息率(0)明显低于其他孕周(P0. 05),剖宫产中小胎儿在孕36～37~(+6)周的新生儿窒息率(0)明显低于其他孕周(P0. 05)。结论:双胎妊娠应加强孕期监护,防止晚期流产的发生。双胎妊娠无明显并发症时可尽量延长孕周至36～37~(+6)周,但不宜过迟,采取剖宫产方式可降低新生儿窒息率的发生。     

妊高征早期防治成功病例分析

1　病历摘要　　患者 38岁 ,孕 3产 2 ,住院号 12 92 1。既往两次妊娠均患肾病综合征型妊高征伴胎儿宫内生长迟缓 (ＩＵＧＲ) ,分别于 33孕周分娩 ,新生儿预后不良。首次妊娠 ,于 2 8孕周发病 ,出现全身水肿 ,33孕周时血压 17/ 13ｋＰａ ,与基础血压相比收缩压增加 4 0ｋＰａ,舒张压增高 2 0ｋＰａ。尿蛋白 ( ) ,自然发动宫缩 ,早产一男活婴 135 0ｇ ,属该孕龄低体重儿 ,3天后死亡。第 2次妊娠 ,于 7孕周出现水肿 ,2 5孕周时尿蛋白 ( ) ,30孕周全身水肿 ,2 4小时尿蛋白总量 12 7ｇ ,住院治疗 3周后出现肾功受累 ,致胎儿窘迫 ,行剖宫产…     

连续三次胎盘早剥成功分娩一例

患者35岁,孕4产2.因孕28周+5,腹胀4 h于2007年12月1日入院.末次月经2007年5月13日,月经规律.停经20周时出现胎动,未行正规产前检查.门诊B超示胎儿双顶径77 mm,羊水深54 mm,胎心率156次,胎盘附着在子宫底前壁.患者2001年5月孕37周时因胎盘早剥在外院剖宫产分娩一女婴,新生儿死亡,体重不详.2004年8月孕35周因胎盘早剥在外院剖宫产分娩一死男婴,体重不详.     

脐动脉舒张末期血流缺失35例的分娩时机及处理方案

目的 分析胎儿脐动脉舒张末期血流缺失(absent end-diastolic velocity,AEDV)出现时间,探讨其临床处理与围产儿结局的关系以及终止妊娠时机对围产儿预后的影响. 方法 对首都医科大学附属北京妇产医院2008年1月至2009年12月间35例发生AEDV的母儿临床资料进行分析,以妊娠28周为界,并按治疗与否分为4组.第1组5例(14.3％)为发现AEDV孕周＜28周未治疗组;第2组13例(37.1％)为发现AEDV孕周＜28周治疗组;第3组11例(31.4％)为发现AEDV孕周≥28周末治疗组;第4组6例(17.1％)为发现AEDV孕周≥28周治疗组.35例孕妇中,除第2组有3例孕妇外,其余均伴有不同程度的各种妊娠合并症.收集的资料用频数、率或均数±标准差表示. 结果 35例孕妇中,19例分娩,占54.3％,共获23例新生儿,结局良好.第1组出现AEDV孕周＜28周,平均(22.8±2.2)周.其中双胎输血综合征1例及溶血、肝酶升高和血小板减少综合征并胎盘早剥1例,发现AEDV后即引产或剖宫取胎,余3例均未治疗,2周后AEDV自然恢复,新生儿存活.第2组出现AEDV孕周＜28周,平均(24.2±2.0)周,平均终止妊娠孕周(31.4±5.5)周,平均治疗时间(10.7±5.5)d.6例治疗后AEDV未恢复,其中5例引产,1例早产;余7例中,6例剖宫产,1例足月自然分娩.除早产1例家属放弃胎儿、1例新生儿失访外,余新生儿结局良好.第3组出现AEDV孕周＞28周,平均(30.9±2.8)周,平均终止妊娠孕周(31.2±2.9)周,均未治疗.5例因严重母儿合并症引产,余6例剖宫产分娩,其中1例因胎盘早剥新生儿死亡,另6例新生儿(1例双胎)结局良好.第4组出现AEDV孕周＞28周,平均(29.5±0.8)周,平均终止妊娠孕周(32.8±2.9)周,平均治疗时间(i0.8±6.7)d.2例治疗后予引产;4例治疗后AEDV恢复正常,剖宫产分娩,新生儿结局良好. 结论 对于AEDV伴有严重并发症的孕妇,积极治疗后,围产儿预后与终止妊娠的孕周有关.AEDV如不伴严重并发症者,部分可自行恢复,且围产儿结局良好.     

双胎妊娠的分娩方式与妊娠结局探讨

目的　探讨双胎妊娠的分娩方式与妊娠结局的关系。方法　将 1995年 1月至 2 0 0 3年 5月 98例双胎妊娠产妇按分娩方式分为阴道分娩组及剖宫产组 ,对其临床资料进行回顾性分析。结果　两组的孕周、产后出血率比较差异无显著性意义 (P >0 0 5 ) ,剖宫产组新生儿体重显著高于阴道分娩组 (P <0 0 1) ,两组第 1胎分娩新生儿窒息率差异无显著性意义 (P >0 0 5 ) ,而第 2胎分娩新生儿窒息率剖宫产组显著低于阴道分娩组(P <0 0 5 )。结论　正确选择双胎妊娠的分娩方式 ,将有助于降低剖宫产率及新生儿窒息率     

The role of hepatic ornithine transcarbamylase deficiency in the orotic aciduria of pregnant mice

Groups of normal and heterozygote sparse-fur (spf) mutant mice were studied at various stages of gestation, to assess the effects of normal pregnancy on orotate excretion, hepatic mitochondrial urea cycle enzymes and any predisposition to the development of fatty liver. Results show a higher total daily excretion of urinary orotate by normal pregnant mice on the 8th and 15th days of gestation, which came to within the usual basal range of excretion of non-pregnant mutant heterozygotes with hereditary ornithine transcarbamylase deficiency. Liver ornithine transcarbamylase and carbamyl phosphate synthetase-I activities were reduced in pregnant mice on the 16th day of gestation (P less than 0.05). No fatty change, bile stasis or glycogen depletion was discernible on optical microscopy in normal or mutant mice. Nonspecific changes were seen on ultrastructural examination. Orotic aciduria seen in pregnant mice may be directly related to a physiological deficiency of liver ornithine transcarbamylase. However, the depletion of both the mitochondrial urea cycle enzymes, seen on the 16th day of pregnancy, may be indicative of a metabolic stress at the mitochondrial level.     

Delivery of a healthy twin after intrauterine demise and expulsion of a first twin

We report on a diamniotic-dichorionic twin pregnancy after in vitro fertilization. The first twin was diagnosed with Down syndrome and spontaneously aborted at 24 weeks of gestation after intrauterine death at week 18. The second healthy twin was delivered by cesarean section 11 weeks later. We discuss management aspects and review the literature.     

First report of a successful pregnancy by preimplantation genetic testing for Beckwith-Wiedemann syndrome

ObjectiveBeckwith-Wiedemann syndrome (BWS) is a rare imprinting gene disorder. Maternal CDKN1C mutation comprises 5% of etiologies of BWS. There is no successful report of preventing BWS by preimplantation genetic testing for monogenic disease (PGT-M) in the literature. Is PGT-M applicable for preventing BWS ?Case reportThis 39-year-old woman conceived naturally and delivered a boy who was diagnosed of BWS. The genetic testing of her son revealed CDKN1C gene mutation, and of the mother showed a carrier of the same mutation. She underwent controlled ovarian stimulation, oocyte pickup, and intracytoplasmic sperm injection. Trophectoderm biopsies were performed and samples were checked for PGT. Two wild-type and euploid embryos were thawed and transferred. One intrauterine pregnancy was achieved. The patient delivered a healthy female baby at 37 weeks of gestation.ConclusionIn this case, we first report a successful pregnancy with a wild-type CDKN1C gene baby achieved by PGT-M.     

Hyperammonemic coma in an ornithine transcarbamylase mutation carrier following antepartum corticosteroids

Women who are carriers of the ornithine transcarbamylase (OTC) mutation are at risk for developing hyperammonemia during the postpartum period and at times of metabolic stress. We present a unique case of hyperammonemic coma occurring in an OTC mutation carrier during the antepartum period. Multiple factors, including the administration of antenatal corticosteroids, likely precipitated this critical condition. Clinicians should be aware of this life-threatening clinical presentation and be prepared to identify, treat, and prevent hyperammonemia in affected individuals.     

IVF/ICSI in a woman with active acromegaly: Successful outcome following treatment with pegvisomant

A 29 year old woman with difficult to control acromegaly and a pituitary macroadenoma responded to pegvisomant therapy and subsequently conceived with her first cycle of in-vitro fertilization and intra-cytoplasmic sperm injection. Pregnancy was complicated by gestational diabetes, pituitary gland enlargement and deteriorating visual fields. Conservative management with elective cesarean section was performed at 32 weeks gestation. A healthy boy was delivered who remains developmentally normal at 1 year. This complex case required intricate care by a multi-disciplinary team and is likely to represent the first in many cases of assisted conception on pegvisomant therapy for active acromegaly.     

First specific preimplantation genetic diagnosis for ornithine transcarbamylase deficiency

Ornithine transcarbamylase (OTC) deficiency is an X-linked dominant metabolic disorder with partial penetrance in heterozygous females. Affected boys usually die from hyperammonemia in the first few days of life, while clinical expression in carrier females ranges from no symptoms to neonatal death. A young couple whose boy had died of OTC deficiency in the neonatal period was referred to our genetic department for their subsequent pregnancy. The fetus was found to be affected, and after genetic counseling the pregnancy was terminated. Prenatal diagnosis of the third pregnancy identified a heterozygous female, who died after a normal birth at age 11 days from hyperammonemia. After this, the couple asked for preimplantation genetic diagnosis (PGD). We have developed a duplex nested PCR assay allowing the amplification of both the mutation and an informative restriction fragment length polymorphism (RFLP) located in the 3' end of the OTC gene. After nested amplification, allele identification was carried out for both loci by double restriction digestion and electrophoresis gel analysis. The co-amplification of both loci provided a means of detecting potential allele dropout or incomplete digestion. Two PGD cycles were carried out, a total of 14 embryos were analysed and a diagnosis could be obtained in 13/14 embryos. There were four unaffected male embryos, four heterozygous females and four unaffected females; the final embryo was an affected one of undetermined gender. In both cycles, three unaffected embryos could be transferred early on Day 4 post-insemination. The second cycle resulted in the birth of a baby boy devoid of the OTC mutation. This constitutes the first birth following PGD carried out by a French team.     

Three consecutive pregnancies in a patient with glycogen storage disease type IA (von Gierke's disease)

Glycogen storage disease type IA is associated with metabolic abnormalities that can compromise fetal outcome. Normal outcome can be achieved by maintaining euglycemia throughout gestation. We report three consecutive pregnancies in a patient with glycogen storage disease type IA. The patient, a 35-year-old woman, has been maintained on a regimen of nightly nasogastric or cornstarch feedings for the past 12 years with improving metabolic control, reduced liver size, and no progression of multiple hepatic adenomas. On confirmation of each pregnancy, early in the first trimester nightly feeding was changed from cornstarch ingestion to Polycose by nasogastric intubation, with good metabolic control. During the last trimester of each pregnancy metabolic control showed further improvement, with lowering of lactate, urate, and triglyceride levels. During the first pregnancy unexpected fetal death occurred at 33 weeks. During the last two pregnancies, the patient was admitted at 33 and 34 weeks, respectively, for closer supervision of metabolic status and fetal monitoring. She underwent a cesarean section at 35 weeks 4 days of gestation and was delivered of a girl. She underwent a repeat cesarean section at 35 weeks 2 days for the subsequent gestation and was delivered of a boy. Both infants are healthy and appear to be unaffected by von Gierke's disease. Hepatic adenomas did not enlarge during the pregnancies. Meticulous management resulted in normal pregnancy outcomes in two consecutive gestations. Rapid fetal growth late in the third trimester may require particularly careful supervision to maintain euglycemia. (AM J Obstet Gynecol 1994;170:1687-91.)     

Ornithine transcarbamylase deficiency diagnosed in pregnancy

Urea cycle enzymes deficiencies are rare metabolic disorders. Ornithine transcarbamylase (OTC) deficiency is the most common type. The syndrome results from a deficiency of the mitochondrial enzyme OTC which catalyses the conversion of ornithine and carbamoyl phosphate to citrulline. It shows X-linked inheritance and typically remains asymptomatic until late infancy or early childhood. The severity of the symptoms depends on the age of the patient and the duration of hyperammonemia. Female heterozygotes are more difficult to diagnose. They suffer from hyperammonemic periods which can be triggered by trauma, infections, surgery, childbirth, parenteral nutrition, and by the initiation of sodium valproate therapy. The prognosis of OTC deficiency is better for those with an onset after infancy, but morbidity from brain damage does not appear to be linked to the number of episodes of hyperammonemia that have occurred. However, early diagnosis and prompt initiation of ammonia-lowering treatment are essential for survival of these patients. This case presents a patient who was diagnosed with OTC deficiency following mental confusion during pregnancy.     

Intercellular adhesion molecule-1 concentration,in utero,decreases after antibiotic treatment

A parturient suffering from preterm premature rupture of membranes at 29-weeks of gestation was hospitalized and staphylococcus was detected in her amniotic fluid. After treatment with antibiotics she delivered a healthy neonate three weeks later. ICAM-1 levels decreased by 20 fold correlating with elimination of the bacteria and prolongation of the pregnancy.     

Intercellular adhesion molecule-1 concentration, in utero, decreases after antibiotic treatment.

A parturient suffering from preterm premature rupture of membranes at 29-weeks of gestation was hospitalized and staphylococcus was detected in her amniotic fluid. After treatment with antibiotics she delivered a healthy neonate three weeks later. ICAM-1 levels decreased by 20 fold correlating with elimination of the bacteria and prolongation of the pregnancy.