Genetic factors in common obstetric disorders

Genetic research of disease has recently turned from individual genes for rare but highly penetrant diseases (like cystic fibrosis) to focus on common, multigene disorders with polygenic inheritance patterns, such as preterm labor, preeclampsia, gestational diabetes, placental abruption, and thromboembolism. These conditions are characterized by multiple etiologies, chronicity, fetal involvement, adaptive clinical manifestations, and gene-environment interactions. As we understand genetic contributions to complex disease and build upon the genetic data and technology available, more effective and specific management and treatment options will become available for clinicians and their patients.     

The obstetric patient and disseminated intravascular coagulation

Disseminated intravascular coagulation remains a major problem for the practicing obstetrician. The various etiologies, the pathophysiology, and suggested therapeutic regimens are reviewed.     

The unbooked patient: a lingering obstetric pathology in Jos, Nigeria.

There were 297 unbooked patients constituting 2% of all deliveries in the Jos Teaching Hospital. Mean age and parity were 26.7 +/- 6.8 years and 3.7 +/- 3.0, respectively. Non-literate women accounted for 36.9%, secondary education 26.2% and tertiary education 3.8%. Housewives constituted 81.2% of the mothers. The majority (93%) were married, while 21 (7.1%) were unmarried. Maternal morbidity included pre-term labour (40.1%), intrauterine fetal death (9.1%), abruptio placenta (4.2%) and ante-partum eclampsia (2.8%). Vaginal delivery occurred in 246 (82.8%) and caesarean section in 45 (15.1%). There were five maternal deaths and all died undelivered. There were 78 perinatal deaths (260/1,000 births), and over 65% were due to low birth weight and prematurity. Mean birth weight was 2.5 +/- 0.8 kg and 38.3% were of low birth weight. Unbooked patients remain significant obstetric pathologies in our maternity unit. Community health education is needed to reduce the number of unbooked patients and the associated pathologies.     

Gynecologic laparoscopy. Personal experience]

The paper reports the Author's personal experience over the past 10 years and gives the most common indications for gynecology endoscopy. An increasingly wide range of possible uses for laparoscopy has been identified and the technique is now an essential element of day-to-day gynecological practice.     

组织因子、组织因子途径抑制物与产科凝血关系的初步研究

目的 检测正常足月妊娠胎盘、蜕膜和子宫肌层中组织因子（tissue factor，TF）和组织因子途径抑制物（tissue factor pathway inhibitor，TFPI）含量，探讨TF、TFPI与产科凝血间的关系。方法 选择正常足月妊娠孕妇30例，用ELISA方法分别检测血浆、胎盘、蜕膜、子宫肌层中TF、TFPI抗原含量。结果 胎盘、蜕膜和子宫肌层中TF抗原含量分别为（14.17±0.60）ng／mg总蛋白、（15.60±0．75）ng／mg总蛋白和（4．37±1．28）ng／mg总蛋白。TFPI抗原含量分别为（11．15±3．63）ng／mg总蛋白、（39.17±15.32）ng／mg总蛋白和（8．91±10.00）ng／mg总蛋白。血浆中TF、TFPI分别为0．013ng／mg总蛋白和（34．00±10．75）ng／mg总蛋白。结论正常足月妊娠胎盘、蜕膜和子宫肌层的高TF，低TFPI抗原水平可能与胎盘剥离面凝血止血及产科凝血障碍有关。     

Eclampsia: a continuing obstetric catastrophe the experience in Ile-Ife, Nigeria.

All cases of eclampsia over a 15-year period in Ile-Ife University Teaching Hospital, Nigeria, were reviewed in order to highlight the problems associated with this condition. The incidence was 0.76% of the total deliveries and showing increasing trend during the period of study. Nulliparas, mainly teenagers and young adults, who were of lower socioeconomic group and who lacked antenatal supervision constituted the majority of cases. Many patients presented following numerous convulsions. Diazepam in combination with hydralazine were the most commonly used drugs. The associated maternal and perinatal mortality and morbidity were unacceptably high. Suggestions as to how the incidence might be reduced are included.     

Atelectasis following cesarean section. Personal experience

Purpose of this paper is to evaluate the risk of ateletasis in patients that underwent surgery of the abdomen (cesarean section), since the reflex of cough in these patients is notably altered for the limitation of the diaphragmatic excursions, due to analgesic contracture. The inhibition of cough's reflex causes retention of secretions with consequent bronchial obstruction and atelectasis. Personal experience in 6 cases of atelectasis following cesarean section is reported.     

产科弥散性血管内凝血的临床诊治

弥散性血管内凝血是由不同原因引起的广泛凝血系统激活,导致弥散性微血管内纤维蛋白沉积、微血栓形成,造成全身出血及微循环衰竭为特征的综合征。本文对产科弥散性血管内凝血的概念、发病因素、病理机制、临床表现、实验室检查、治疗方法及预防进行讨论。     

Gynaecological and obstetric management of women with inherited bleeding disorders.

OBJECTIVE: The prevalence of bleeding disorders, notably von Willebrand disease (vWD), among adult women with objectively documented menorrhagia is consistently reported to be 10% to 20% and is even higher in adolescents presenting with menorrhagia. This consensus document has been developed by a multidisciplinary committee consisting of an anesthesiologist, 2 hematologists, and an obstetrician/gynaecologist and has been endorsed by their relevant specialty bodies. It has been prepared with the express purpose of providing guidelines for both women with inherited bleeding disorders and for their caregivers regarding the gynaecological and obstetric management of these women, including appropriate anesthesia support where indicated. OPTIONS: Diagnostic tools and specific medical and, where appropriate, surgical alternatives to management are reviewed and evidence-based recommendations presented. EVIDENCE: A MEDLINE search of the English literature between January 1975 and November 2003 was performed using the following key words: menorrhagia, uterine bleeding, pregnancy, von Willebrand, congenital bleeding disorder, desmopressin/DDAVP, tranexamic acid, oral contraceptives, medroxyprogesterone, therapy, hysterectomy, anesthesia, epidural, spinal. Recommendations from other society guidelines were reviewed. RECOMMENDATIONS: 1. Inherited bleeding disorders should be considered in the differential diagnosis of all patients presenting with menorrhagia (II-2B). The graphical scoring system presented is a validated tool which offers a simple yet practical method that can be used by patients to quantify their blood loss (II-2B). 2. Because underlying bleeding disorders are frequent in women with menorrhagia, physicians should consider performing a hemoglobin/hematocrit, platelet count, ferritin, PT (INR) and APTT in women with menorrhagia. In women who have a personal history of other bleeding or a family history of bleeding, further investigation should be considered, including a vWD workup (factor VIII, vWF antigen, and vWF functional assay) (II-2B). 3. Treatment of menorrhagia in women with inherited bleeding disorders should be individualized (III-B). 4. An inherited bleeding disorder is not a contraindication to hormonal therapy (oral contraceptives [II-1B], depot medroxyprogesterone acetate (DMPA) [II-3B], danazol [II-2B], GnRH analogs [II-3B]) or local treatments (levonorgestrel-releasing IUS [II-1B]) and non-hormonal therapy (antifibrinolytic drug tranexamic acid [II-1B]) as well as desmopressin (II-1B). These therapies represent first line treatment. Blood products should not be used for women with mild bleeding disorders (III-A). 5. In women who no longer want to preserve their fertility, conservative surgical therapy (ablation) and hysterectomy may be options (III-B). Clinicians may consult the "SOGC Clinical Practice Guideline: Guidelines for the Management of Abnormal Uterine Bleeding" for an in-depth discussion of the available therapeutic modalities, both medical and surgical. To minimize the risk of intraoperative and post-operative hemorrhage, coagulation factors should be corrected preoperatively with post-operative monitoring (II-1B). 6. Girls growing up in families with a history of vWD or other inherited bleeding disorders should be tested pre-menarchally to determine whether or not they have inherited the disease to allow both the patient and her family to prepare for her first and subsequent menstrual periods (III-C). 7. In adolescents presenting with menorrhagia, an inherited bleeding disorder should be excluded (III-B). When possible, investigation should be undertaken before oral contraceptive therapy is instituted, as the hormonally induced increase in factor VIII and vWF may mask the diagnosis (II-B). 8. Pregnancy in women with inherited bleeding disorders may require a multidisciplinary approach. A copy of their recommendations should be given to the patient and she should be instructed to present it to the health care provider admitting her to the birthing centre. Women with severe bleeding disorders or with a fetus at risk for a severe bleeding disorder should deliver in a hospital (level three) or where there is access to consultants in obstetrics, anesthesiology, hematology, and pediatrics (III-C). 9. Vacuum extraction, forceps, fetal scalp electrodes, and fetal scalp blood sampling should be avoided if the fetus is known or thought to be at risk for a congenital bleeding disorder. A Caesarean section should be performed for obstetrical indications only (II-2C). 10. Epidural and spinal anesthesia are contraindicated if there is a coagulation defect. There is no contraindication to regional anesthesia if coagulation is normalized. The decision to use regional anesthesia should be made on an individual basis (III-C). 11. The risk of early and late postpartum hemorrhage is increased in women with bleeding disorders. Women with inherited bleeding disorders should be advised about the possibility of excessive postpartum bleeding and instructed to report this immediately (III-B). 12. Intramuscular injections, surgery, and circumcision should be avoided in neonates at risk for a severe hereditary bleeding disorder until adequate workup/preparation are possible (III-B). The quality of evidence reported in this document has been described using the Evaluation of Evidence criteria outlined in the Report of the Canadian Task Force on the Periodic Health Exam (Table 1).     

Diagnosis and management of obstetric acute disseminated intravascular coagulation]

Twenty-seven in-patients with obstetric DIC in our hospital from Jan. 1971 to Dec. 1990 were analysed retrospectively. The incidence was 0.12% in the first decade and 0.02%, in the second, showing a difference of significance between them. The most common predisposing factors included amniotic fluid embolism, abruptio placenta and hemorrhagic shock. Bleeding from multi-organs in various extent and coagulation disorders occurred in all those 27 cases. [Besides anti-shock treatment, heparin was employed together with fibrinogen in 4 postpartum and 1 antepartum DIC patients, fibrinogen alone in 8 cases, and hysterectomy in 11 cases. 17 patients were saved and 9 died. It is important that early diagnosis and much attention paid to clinical characteristics together with serial laboratory tests. Key management should include prompt treatment and eradication of predisposing factors. Quick decision spite of to terminate the pregnancy and even hysterectomy should be done in some risks.     

Menorrhagia and inherited disorders of coagulation

Menorrhagia, a common complaint among women, may be a manifestation of an underlying inherited disorder of coagulation. In this review, the most frequent hereditary bleeding disorders associated with menorrhagia are briefly analyzed. Particularly, the epidemiological, clinical and diagnostic characteristics of von Willebrand disease, factor VIII, factor IX and factor XI deficiencies are examined. The influence of external factors (age, phase of menstrual cycle and hormonal therapy) on coagulation factor levels is also analyzed. Finally, the main therapeutic options (such as hormonal therapy, tranexamic acid and desmopressin), for the treatment of menorrhagia in women with hereditary bleeding disorders are reviewed. Since inherited bleeding disorders are frequently associated with menorrhagia, the conclusion is drawn that an underlying congenital bleeding disorder should be ruled out in any woman with menorrhagia.     

产科凝血功能障碍高危因素与处理策略

在各种疾病基础上或某些特殊条件下，在疾病的发生和发展过程中引发了凝血系统激活，引发弥散性血管内凝血（DIC），由此而导致的凝血功能障碍并不是一种独立的疾病，是多种疾病的共同病理表现。由产科因素引发的凝血功能障碍占总的凝血功能障碍的4％～12％，是产科多种高危妊娠常见的严重并发症。因其临床表现隐匿、疾病经过与发展迅速、凶险，往往预后较差，以至于发生多脏器系统功能衰竭乃致死亡。因此，早期发现并迅速、正确处理产科凝血功能障碍，是降低孕产妇死亡率的关键。     

Optimizing obstetric venous thromboembolism protocol adherence: The experience of a hospital system

The purpose of this review is to explore the role of hospital systems in reliably providing high quality obstetric venous thromboembolism (VTE) prophylaxis focusing on the example of the Kaiser Permanente Southern California hospital system. While providers ultimately administer thromboprophylaxis on a patient-by-patient basis, hospital-level protocols, practices, and resources may be the most important determinants of whether a patient receives appropriate care. In comparison to the complex maternal and fetal emergencies that obstetricians are routinely called on to manage, VTE prophylaxis can often be simplified and integrated into the workflow, making decision-making time efficient and straightforward for the provider. Not having protocols (i.e. the provider being on their own) is associated with desired management occurring in only 40% of cases. Enhanced VTE protocols with complementary strategies to encourage use and identification of oversights addressed in real time can result in appropriate care in >90% of cases.     

Surviving a malpractice suit. Personal experience and general information

Few CNMs to date have been sued and fewer yet have been through the trauma of a malpractice suit. The following article is a synopsis of the malpractice process from summons through trial. Interspersed throughout are personal recollections and responses of one CNM who endured a two-week trial.     

Episiotomy suture using the Shute method. Personal experience

The suture of episiotomy is certainly a problem in modern obstetrics. The Shute seam, still largely unknown in Europe, is a good technique and offers a much cleaner wound healing and a significant reduction of pain during childbed with very satisfactory functional and cosmetic results.