The impact of pregnancy-prolonging management on maternal and neonatal morbidity in HELLP syndrome]

OBJECTIVE: Until recently, delivery immediately after diagnosing HELLP syndrome was recommended due to the life-threatening risk to mother and child. Prolongation at least until lung maturation is being increasingly considered because of the high rate of premature births characterized by extreme immaturity. We investigated the influence of the time of delivery on maternal and neonatal morbidity at a gestational age of less than 34 + 0 weeks of pregnancy. - MATERIAL AND METHODS: The disease course was reevaluated in 37 patients who developed HELLP syndrome (thrombocytes < 100 000/microl, transaminase > 70 U/l, haptoglobin < 0.5 g/l) between 1994 and 1999. An attempt was made to stabilize the mother's condition under therapeutic volume expansion. Pregnancy was terminated with the onset of a renewed HELLP episode. -RESULTS: HELLP syndrome occurred with an incidence of 1 : 310 births. There were no maternal or neonatal deaths or any severe complications. Prolonging pregnancy until completing drug-induced lung maturity was successful in 16 of 25 patients before the 34(th) week of pregnancy. In the case of immediate delivery with inadequate stabilization, 5 of 9 patients had postpartum complications. A severe RDS occurred in 3 premature babies without drug-induced maturity. - CONCLUSION: If there is no life-threatening risk to the fetus or mother in patients with HELLP syndrome, the objective is the prolongation of pregnancy in a perinatal center until lung maturation. Stabilization is successful in a high percentage of patients under therapeutic volume expansion with optimal monitoring of mother and child.     

妊娠合并重度特发性血小板减少性紫癜的诊治

妊娠合并特发性血小板减少性紫癜(idiopathic thrombocytopenic purpura,ITP)是孕妇妊娠期间常见的血液系统疾病,临床表现以出血为主,贫血及感染风险轻度增加,ITP在整体上并不影响母儿结局,但会使出血的风险增加。本文重点就妊娠合并重度ITP的诊治、对母儿影响及孕期管理进行讨论。     

Acute intermittent porphyria complicated by pregnancy

The case of a woman with acute intermittent porphyria complicated by two pregnancies is described. Both pregnancies gave an exacerbation of the disease. Premature delivery were necessary for both children due to intrauterine growth retardation. The first child died from respiratory distress syndrome, but the second child was healthy apart from transient abstinence symptoms due to pethidine given to the mother during pregnancy. The effect of pregnancy on acute intermittent porphyria is discussed.     

发热合并肝功能异常的妊娠期干燥综合征一例

干燥综合征（Sjögren′s syndrome）是一种系统性自身免疫性疾病,不影响女性患者的生育能力,但影响胎儿在宫内的生长发育,导致自然流产和早产。由于鉴别诊断困难,妊娠期首次发病的干燥综合征孕妇易延误治疗,导致疾病加重,出现严重不良母儿结局的风险随之升高。报告1例以发热及肝功能异常为首要表现的原发性干燥综合征患者,旨在总结该疾病的诊断依据及治疗方案,阐述临床监测指标及治疗效果。     

妊娠合并主动脉夹层的诊治

妊娠合并主动脉夹层病情凶险,容易误诊、漏诊,母儿不良结局风险极高,且与夹层分型密切相关,病因主要与原发高血压、先天性和遗传性血管性疾病相关,妊娠是其好发因素。胸痛为主要临床表现,诊断主要依靠影像学检查。治疗包括支持治疗、药物治疗、传统手术治疗、主动脉瘤腔内修复术等,治疗方式、分娩时机、分娩方式根据主动脉夹层累及范围与类型、孕周、患者及家属意愿和医疗条件综合决定。加强孕前与孕期保健、多学科评估与救治是降低不良母胎结局的关键。     

Obstetric and perinatal consequences of human immunodeficiency virus (HIV) infection: a review

Pediatric acquired immune deficiency syndrome (AIDS) was known to be a new disease that could be acquired from the mother even before human immunodeficiency virus (HIV) was identified. The suggested routes of transmission of infection are intrauterine, perinatal--from contact with infected maternal genital secretions, or through breastfeeding. At this time the problem in Europe concerns primarily women in high-risk groups: intravenous drug abusers, prostitutes, women from countries where the prevalence of HIV is high, and women whose sexual partners are in a high-risk group. In the future, the infection may extend beyond women in high-risk groups as the disease becomes more prevalent in the community. It has been claimed that pregnancy accelerates symptoms in women who are HIV positive, yet this is based on only a series of case reports of severe infection in pregnancy and on the development of AIDS in asymptomatic women in pregnancy subsequent to the birth of an AIDS child. The only data capable of shedding some light on this issue would be a prospective followup of both pregnant and nonpregnant HIV-positive women from similar high-risk groups. Such a study is ongoing in the US. An increasing number of case reports suggest intrauterine transmission of infection. The following 3 case reports provide clear evidence of intrauterine transmission. Sprecher et al. (1986) detected HIV antigen in amniotic fluid and fetal tissues from a pregnancy termination at 15 weeks gestation in a woman with stage IV AIDS and Kaposi sarcoma. Lapointe et al. (1985) reported an infant born by cesarean section at 28 weeks gestation to a mother with terminal aids. A new dysmorphic syndrome recently has been described in children with symptomatic HIV infection (Marion et al., 1986). HIV has been isolated from cervical secretions (Fogt et al., 1986; Wofsy et al., 1986), which suggests that this cold be another source of infection. There is 1 report of isolation of HIV from the noncellular fraction of breast milk (Thirty et al., 1985). Several case reports have described acquired immunodeficiency in infants for whom the only known risk factor was neonatal transfusion from an individual later found to be suffering from AIDS. The risk of transmission from an infected mother to her infant is unknown, but the best available evidence comes from a study of children born to women who had previously given birth to a child with AIDS (Scott et al, 1985). Of 12 children, 4 developed AIDS or Aids-related complex. Clinical problems among children with AIDS or AIDS-related complex have been fully described. The fatality rate of children with AIDS is high, but the ultimate progress of children with less severe disease or who have asymptomatic infection is known.     

Multidisciplinary management of aortic dissection complicating pregnancy

BACKGROUND: Most cases of aortic dissection observed in women under 40 years of age occur as a complication of pregnancy in patients with other risk factors. Case report. We report a case of dissection of the ascending aorta in a young primigravida at 35 weeks gestation. The risk factor was aortic regurgitation. Multidisciplinary management enabled fetal extraction followed by repair of the aorta. Outcome was favorable for both mother and child. DISCUSSION: A review of the literature shows a variety of etiological factors leading to this disease. Emergency diagnosis and management is mandatory. Obstetricians should be aware that pregnancy can be a triggering factor in patients with a predisposition, e.g. connective tissue disease. A complete cardiovascular evaluation should be conducted before conception and a suitable surveillance planned for the entire pregnancy. The aim of this careful follow-up it to avoid surgery in an emergency setting that could compromise prognosis for both mother and child.     

Pregnancy in renal transplant recipients

Women with renal disease face increasing infertility and high-risk pregnancy as they approach end-stage renal disease due to uremia. Renal transplantation has provided these patients the ability to return to a better quality of life, and for a number of women who are of child bearing age with renal disease, it has restored their fertility and provided the opportunity to have children. But, although fertility is restored, pregnancy in these women still harbors risk to the mother, graft, and fetus. Selected patients who have stable graft function can have successful pregnancies under the supervision of a multidisciplinary team involving maternal fetal medicine specialists and transplant nephrologists. Careful observation and management are required to optimize outcome for mother and fetus.     

Marfan syndrome in pregnancy: presentation of four cases and discussion

The coincidence of Marfan syndrome and pregnancy means a high risk for mother and child, as it is associated with cardiovascular and obstetric complications. We report our experience of four pregnancies with the Marfan syndrome. The course of pregnancy, the peripartum management and both the maternal and neonatal outcomes of four pregnant women with the Marfan syndrome, who were treated in our department between 1995 and 2005, were retrospectively analysed. The pregnancies of two women were complicated by premature rupture of membranes (36 (th) gestational week) and premature uterine contractions with cervical incompetence (30 (th) gestational week), respectively. One patient developed class 3 (NYHA) heart failure in the 3 (rd) trimenon. Two out of four women had mild cardiovascular disease and could deliver vaginally. In the other two cases a primary Caesarean section was performed at the 36 (th) week of gestation because of severe cardiovascular morbidity. No patient had a progressive aortic dilatation, dissection or rupture. The neonatal outcome was uneventful in all cases. Three newborns underwent a genetic evaluation for the Marfan syndrome, in two of them mutations in the fibrillin 1 gene were detected. Women with the Marfan syndrome should be counselled pre-conception and observed by an interdisciplinary team during pregnancy. If the aortic root diameter is < 40 mm, without progression in pregnancy, and in the absence of severe valve insufficiency, then pregnancy is in most cases well tolerated and vaginal delivery can be performed.     

HLA relationships of pregnancy, microchimerism and autoimmune disease

Recent studies have established that there is bi-directional cell traffic between mother and fetus during pregnancy. Suprisingly, fetal cells have been found to persist in the maternal circulation for years after pregnancy. Maternal cells can also persist into adult life in her progeny. When cells from one individual are present in the body of another the term chimerism is used and a low level of non-host cells is referred to as microchimerism. Chronic graft-versus-host disease often occurs after stem cell transplantation, is a known condition of chimerism, and resembles spontaneously occurring autoimmune diseases including systemic sclerosis, Sj?gren's syndrome, primary biliary cirrhosis and sometimes myositis and systemic lupus. Of central importance to the development of chronic graft-versus-host disease is the HLA relationship of host and donor cells. Considering this constellation of observations together led to the hypothesis that microchimerism and HLA-relationships are involved in the pathogenesis of some autoimmune diseases. Although much additional work is needed, results of initial studies provide support to the concept that non-host cells could participate in the pathogenesis of some autoimmune diseases.     

子痫前期的预测和预警

子痫前期可直接影响妊娠结局甚至母儿远期健康。预测子痫前期的方法包括母体危险因素、平均动脉压、子宫动脉多普勒指标以及血清生化标志物等,尤以妊娠早期联合筛查预测价值高。重视子痫前期的早期预警信息及时做出诊断,预测疾病严重程度、妊娠不良结局,有助于及时给予恰当的治疗包括决定终止妊娠的时机,最终改善母儿结局。     

Maternal obesity and pregnancy outcome

The increasing prevalence of maternal obesity worldwide provides a major challenge to obstetric practice from preconception to postpartum. Maternal obesity can result in unfavorable outcomes for the woman and fetus. Maternal risks during pregnancy include gestational diabetes and chronic hypertension leading to preeclampsia. The fetus is at risk for stillbirth and congenital anomalies. Intrapartum care, normal and operative deliveries, anesthetic and operative interventions in the obese demand extra care. Obesity in pregnancy can also affect health later in life for both mother and child. For women, these risks include heart disease and hypertension. Children have a risk of future obesity and heart disease. Women and their offspring are at increased risk for diabetes. Obstetrician-gynecologists should be well informed to prevent and treat this epidemic. Interventions directed at weight loss and prevention of excessive weight gain during pregnancy must begin in the preconception period.     

Two cases of prenatal analysis for the pathogenic T to G substitution at nucleotide 8993 in mitochondrial DNA

We report the outcome of two prenatal analyses for the T to G mutation at nucleotide 8993 in the mitochondrial DNA. This mutation is associated with neurogenic muscle weakness, ataxia and retinitis pigmentosa (NARP) and the neurodegenerative condition, Leigh syndrome. One prospective mother was the sister of a severely affected individual, and had previously had an unaffected child and a stillborn child. The second prospective mother had two unaffected children and two affected children. The mutation was not detected in the chorionic villus sample from one fetus nor in the amniocytes from the other fetus. Both pregnancies were continued, and the resulting children were healthy at two years and five years of age. Prenatal diagnosis of this mitochondrial DNA mutation is an option likely to be acceptable to some families to prevent the birth of a child at high risk for neurological disease.     

妊娠合并甲状腺功能异常:子痫前期风险与防范

妊娠期甲状腺功能异常性疾病通常表现为妊娠期甲状腺功能亢进(甲亢)或妊娠期甲状腺功能减退症(甲减),未经治疗的妊娠期甲状腺功能异常可导致多种母儿并发症并引起不良妊娠结局,无论是甲亢还是甲减均可增加子痫前期的发病风险,且子痫前期合并甲状腺功能异常使病情变得更为复杂,增加母儿不良预后的风险。故在孕前和孕早期对妊娠期甲状腺功能进行筛查,通过以药物为主的治疗手段使孕期甲状腺激素水平保持在正常范围,以及严密的监护等有助于降低妊娠期子痫前期的发病风险,改善子痫前期合并甲状腺功能异常患者的妊娠结局。     

高血压患者孕前和孕期风险评估及处理

高血压患者妊娠易发生重度子痫前期及多脏器损害,导致母儿严重并发症。高血压患者孕前应综合评估妊娠风险,孕前应将血压控制在正常范围,选择合适妊娠时间。孕期加强监护,应用阿司匹林预防子痫前期。终止妊娠时机及方式应根据病情严重程度及母儿情况综合决定。分娩过程中注意监测血压,产后加强随访。     

Pregnancy and inflammatory bowel disease: a review of the literature

This review encompasses fertility with inflammatory bowel disease, risk of inheritance, effects of inflammatory bowel disease on pregnancy, effects of pregnancy on inflammatory bowel disease, effects of treatment on the mother and child, and pregnancy after proctocolectomy with ileostomy. Fertility is minimally, if at all, compromised. The fetus is likely to survive despite disease activity. Inactive disease favors a normal pregnancy. Active disease may worsen during the first trimester or the puerperium. Close medical supervision allows therapy to be instituted promptly. Patients with Crohn's disease who have medically induced remissions may fare less well than those who have undergone a successful operation. Previous surgery does not preclude vaginal delivery. Sulfasalazine and corticosteroids may be used. Patients with extensive fistulas may require cesarean section. There are few reasons to consider therapeutic abortion.     

The medically certified inability to work in relation to prenatal care

The specificity of medical care is analyzed by investigations of causes for disablement during pregnancy in 4,742 cases of working pregnant women. The diagnoses of disablement with a frequency of 9.31% are distributed by equal parts of causes dependent on pregnancy or not. The percentage of sick pregnant women not treated by the gynecologist is very high with 36.4% of causes dependent on pregnancy. For an improvement of the health protection of mother and child the pregnant women with these types of diseases should be referred to the gynecologist for further diagnostic and treatment.     

Long-term outcome of children born from mothers with autoimmune diseases

Autoimmune diseases often affect young women and this may represent a problem in family planning. Pregnancies in these patients may carry several complications but nowadays the continued amelioration in treatment and management has greatly improved the pregnancy outcome. The main concern of these women obviously is the short- and long-term outcome of their children. A child born from a woman with autoimmune disease is potentially exposed in utero to maternal autoantibodies, cytokines, and drugs, and each item could impair his or her development. In addition, the maternal genetic heritage can favor autoimmunity. All these items could have a role, for example, in the development of autoimmune diseases (the same as the mother or different ones) or neurological disorders. Data in literature are controversial. This review will gather the available data possibly providing a useful tool for counseling future mothers.     

Endokrine Erkrankungen in der Schwangerschaft

Thyroid diseases and diabetes are the most common endocrine diseases in pregnancy. Besides the enhanced iodine demand in pregnancy physicians should take notice of autoimmune thyroiditis as a common disease. Screening of proper thyroid function is necessary as the symptoms are unspecific. A substitution of thyroid hormones should be based on pregnancy-specific standard values. If a rare hyperthyroidism occurs, both mother and fetus have to be treated with antithyroid drugs and be under constant surveillance. Preexisting diabetes is still a rare disease in childbearing women. It requires risk stratification by properly taking the diabetic and obstetric history to organize adequate interdisciplinary care. Physiological changes in pregnancy require constant adaptation to the variable insulin demands. As glucose metabolism is important for fetal development, especially fetal growth should be taken into account to assure individualized therapy.     

Prenatal diagnosis of congenital myasthenia with arthrogryposis in a myasthenic mother

We studied two children born to a myasthenic mother. The first child, a female, had multiple flexion contractures. She died 1 h after birth. In the second pregnancy, 3 years later, ultrasonographic examination at 20 weeks showed decreased fetal movements and multiple flexion contractures. The pregnancy was interrupted. Eight other cases of congenital myasthenia with arthrogryposis are known; four of them are siblings. The recurrence risk may be as high as 100 per cent. Our second case demonstrates that prenatal diagnosis is possible early enough to allow termination of pregnancy.