Intrahepatic cholestasis of pregnancy

Intrahepatic cholestasis of pregnancy is a pregnancy-specific liver disease characterised by maternal pruritus and raised serum bile acids. It is of multifactorial aetiology with genetic influences. The disease has potential implications for the fetus, linked to the serum bile acid concentration in the blood, including preterm labour, stillbirth and effects on fetal organ systems including the cardiovascular and respiratory systems. The mainstay of pharmacological management is the drug ursodeoxycholic acid which improves pruritus and decreases serum bile acid concentrations. In the UK, management of delivery is usually induction of labour between 37 and 38 weeks gestation, balancing the risks associated with preterm delivery against that of fetal demise which is greater as gestation advances.     

Potential clinical applications of placental stem cells for use in fetal therapy of birth defects

Placental stem cells are of growing interest for a variety of clinical applications due to their multipotency and ready availability from otherwise frequently discarded biomaterial. Stem cells derived from the placenta have been investigated in a number of disease processes, including wound healing, ischemic heart disease, autoimmune disorders, and chronic lung or liver injury. Fetal intervention for structural congenital defects, such as spina bifida, has rapidly progressed as a field due to advances in maternal-fetal medicine and improving surgical techniques. In utero treatment of structural, as well as non-structural, congenital disorders with cell-based therapies is of particular interest given the immunologic immaturity and immunotolerant environment of the developing fetus. A comprehensive literature review was performed to assess the potential utilization of placenta-derived stem cells for in utero treatment of congenital disorders. Most studies are still in the preclinical phase, utilizing animal models of common congenital disorders. Future research endeavors may include autologous transplantation, gene transfers, induced pluripotent stem cells, or cell-free therapies derived from the stem cell secretome. Though much work still needs to be done, placental stem cells are a promising therapeutic agent for fetal intervention for congenital disease.     

Update on the etiologies and management of neonatal cholestasis

The early detection of cholestatic liver disease is one of the major challenges facing pediatricians when evaluating the jaundiced infant. Early recognition of liver disease greatly facilitates the care and outcome of infants, because several serious life-threatening disorders may have cholestasis as a major presenting sign of underlying neonatal liver disease. A key component of the work-up is measurement of serum conjugated bilirubin levels, which if elevated should prompt the clinician to initiate a work-up to determine the cause of neonatal cholestasis. In general, if a patient is developing progressive jaundice soon after birth, is still jaundiced at 2 weeks of life, or develops jaundice within the first month of life, a work-up for neonatal cholestasis should begin. A number of previously undiagnosed causes of neonatal cholestasis are beginning to be assigned genetic and infectious etiologies, with significant implications for the work-up and management of cholestatic infants.     

PRIMARY CARE FOR WOMEN: Management and Treatment of Gastrointestinal Disorders

This article outlines the clinical management of common gastrointestinal disorders encountered in the primary care setting. The general assessment of a woman presenting with a gastrointestinal concern is reviewed. Diagnosis and management of acute and chronic diarrhea, constipation, irritable bowel syndrome, and anorectal disorders are covered with emphasis on client education. Gastroesophageal reflux disease and peptic ulcer disease is discussed with the latest treatment recommendations for Helicobacter pylori infection outlined. Diagnosis of gallbladder disease and gallstones with alternative treatment options is reviewed. Finally, the diagnosis and management of viral hepatitis is outlined. A case study is given to illustrate the basic principles needed by the nurse-midwifes in the assessment, diagnosis, and management of a woman with a gastrointestinal concern.     

Modified ISTH pregnancy-specific DIC score in parturients with liver rupture: population-based case series

Objectives: Liver rupture and hematoma are rare life-threatening complications of pregnancy. The aims of the current study are to: (1) characterize in a population-based study all cases of liver hematoma and/or rupture; and (2) validate the utility of the International Society on Thrombosis and Haemostasis (ISTH) modified pregnancy specific disseminated intravascular coagulation (DIC) score in those cases.

Study design: A retrospective cohort study including all patients with liver subcapsular hematoma or rupture between the years 1996 and 2012 was conducted. Information on maternal characteristics, clinical presentation, diagnostic studies, therapeutic modalities, as well as maternal and fetal outcomes was collected. The pregnancy-specific modified ISTH DIC scores were calculated from admission to discharge, a score >26 is suggestive of DIC.

Results: Out of 175,000 births in our database, seven patients were identified with liver rupture or subcapsular hematoma, representing a prevalence of 4:100,000 deliveries. Of those, six had liver rupture and one had subcapsular liver hematoma. One patient died of hemorrhagic shock. Four patients underwent surgical liver packing and one also underwent hepatic artery ligation. Four out of seven patients were diagnosed during the immediate postpartum period with severe features of preeclampsia or with hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Modified ISTH pregnancy-specific DIC scores were calculated for five out of seven patients, and three (60%) had a score higher than 26. Patients with higher scores received more blood product transfusions, had longer hospitalizations, and their neonates had lower 1 and 5?minutes Apgar scores.

Conclusions: Elevated pregnancy-specific modified ISTH DIC score (>26) in patients with liver hematoma or rupture was associated with adverse maternal and neonatal outcomes and appeared to perform well in distinguishing high and low-risk cases. Postpartum preeclampsia may be associated with severe features and a more complicated disease course.     

Hyperemesis,gastrointestinal and liver disorders in pregnancy

Pregnant women may be affected by diseases of the gastrointestinal tract or liver. These disorders can be related or unrelated to pregnancy. Conditions unrelated to pregnancy can be pre-existing or co-incident. These diseases have varying effects on obstetric outcome. Severe liver disease in pregnancy is rare. We present some common gastrointestinal and liver disorders focussing on the diagnosis, management and effects on pregnancy outcomes.     

Connective tissue and dermatological conditions in pregnancy

Connective tissue disorders are common in women of reproductive age, and hence are seen frequently in maternal medicine clinics. The disorders, and their treatments, may have significant adverse effects on fertility, the developing fetus, and on pregnancy outcomes. In turn pregnancy may affect the natural course of the illness. Pre-pregnancy planning and multidisciplinary management are vital to optimise maternal and fetal outcomes. This review will cover a general approach and specific management points of common autoimmune and genetic conditions.Alterations in the maternal immune system affect the disease course of pre-existing skin conditions during pregnancy, and pharmacotherapy may be limited due to effects on the fetus. Some dermatological conditions arise de novo during pregnancy. Women may present to maternity services directly; therefore obstetricians must be able to diagnose, investigate and initiate management of pregnancy dermatoses, with input from dermatologists where available.     

Hydatid cyst of urinary bladder associated with pregnancy:a case report

Echinococcosis or hydatid disease which is caused by Echinococcus group of cestodes is very rare in pregnancy. While liver and lungs are commonly involved, other sites can be rarely affected. The management of hydatid disease in pregnancy is challenging in view of varied presentation and manifestation. We report a case of hydatid cyst arising from the bladder associated with pregnancy and presenting with abdominal pain. The cyst was surgically removed and the bladder wash was given with povidone–iodine. The postoperative recovery was uneventful with ongoing pregnancy. This is to our knowledge, the first case of hydatid cyst arising from the bladder associated with pregnancy to be reported.     

Acute liver failure in pregnancy. A case report

BACKGROUND: Liver disease in pregnancy can be grossly divided into those disorders coincidentally occurring during the pregnant state and hepatic diseases limited to pregnancy. Numerous infectious agents can result in acute hepatitis and include not only the hepatitis viruses--A, B, C and E--but herpesvirus and cytomegalovirus as well. Coxsackie B viruses can cause several clinical presentations, ranging from asymptomatic to mild febrile illness to myocarditis and meningitis. Rarely has coxsackievirus infection been associated with fulminant hepatic failure. CASE: A Coxsackie B virus infection resulted in acute liver failure in a gravid woman. The patient was managed expectantly, with resolution of the liver disease and delivery five weeks after discharge. CONCLUSION: The onset of hepatic disease is insidious, with only vague symptoms or minor complaints often heralding the progression to liver failure. A careful history, physical examination and appropriate diagnostic tests can help determine the etiology of hepatic disease and help decide whether expectant management of the gravid patient or immediate delivery is appropriate.     

Family history of gynaecological cancers

Women are presenting to primary and secondary care with concerns about a family history of ovarian and breast cancer, or ovarian, endometrial and bowel cancer. Although most ovarian and endometrial cancer is sporadic, about 5−10% is due to mutations in genes which predispose to breast/ovarian cancers, BRCA1 and BRCA2, and ovarian/endometrial and bowel cancer, the mismatch repair genes of hereditary non-polyposis colon cancer (HNPCC). This review considers different scenarios in women presenting with a family history of ovarian and endometrial cancer. It uses these family histories to illustrate the ways in which families at high risk of ovarian and endometrial cancer can be identified by pedigree analysis. There will be further discussion about these genes and the different management options available to families, including surveillance, chemoprevention and prophylactic surgery.     

氧化应激对胎盘生命的影响

氧化应激不仅是氧化剂的产生与抗氧化剂防御系统之间的不平衡状态,也是众多细胞功能障碍和组织损伤的原因。氧化应激相关因子在多种疾病中发挥重要作用,通过多种途径调节细胞内环境稳定,维持细胞存活。近年研究发现,氧化应激与相关的氧化损伤是导致多种疾病的主要因素,氧化应激相关指标在妊娠特有疾病(如子痫前期、胎儿生长受限)孕妇的血清及胎盘滋养细胞中高度表达。氧化应激同时与肿瘤、高血压等多种慢性疾病有关,与多种疾病的发生、发展密切相关。氧化应激作为妊娠期特有疾病的"共同通路"被深入研究,其中氧化应激对胎盘功能的影响备受关注。分析与总结胎盘在不同缺氧状态下与妊娠相关疾病的关系,探索氧化应激相关因子是否具有预测作用,为围生期相关疾病的预防及诊疗提供新方向、新思路。     

Immunoreactive diseases in pregnancy

Immunoreactive diseases in pregnancy are not frequent and it is important to guarantee an optimal and interdisciplinary treatment. The herpes gestationis is a pregnancy-specific disease of the skin with unknown origin and a good prognosis for mother and child. The progressional lupus erythematodes visceralis has a high risk for mother and child, but the rheumatoid arthritis shows remissions during pregnancy. The Basedow's disease and the autoimmune thyroiditis are needed a pregnancy-associated therapy to prevent a hyperthyreotic syndrome of newborns. The autoimmune haemolytic anemia and the thrombocytopenia have a high fetal risk although by intensive management. Myasthenia gravis may cause a transient neonatal syndrome.     

Maple syrup urine disease: tailoring a plan for pregnancy

Maple syrup urine disease (MSUD) is an autosomal metabolic condition that can cause significant morbidity in pregnant women and their infants. Advances in nutritional management mean more mothers with congenital metabolic disorders are becoming pregnant, and presenting challenges in obstetric care. In particular, the increase in protein requirements during pregnancy and metabolic stress of childbirth require careful planning and caloric titration. We report a case of a second time mother with MSUD and demonstrate a treatment scaffold to help achieve successful pregnancies for women with this and similar conditions.     

Pyogenic liver abscess caused by Burkholderia pseudomallei in Taiwan.

Pyogenic liver abscess in Taiwan is a well-known disease entity, commonly associated with a single pathogen, Klebsiella pneumoniae. Melioidosis is an endemic disease in Taiwan that can manifest as multiple abscesses in sites including the liver. We report three cases of liver abscesses caused by Burkholderia pseudomallei. The first patient was a 54-year-old diabetic woman, who presented with liver abscess and a left subphrenic abscess resulting from a ruptured splenic abscess, co-infected with K. pneumoniae and B. pseudomallei. The second patient, a 58-year-old diabetic man, developed bacteremic pneumonia over the left lower lung due to B. pseudomallei with acute respiratory distress syndrome, and relapsed 5 months later with bacteremic abscesses of the liver, spleen, prostate and osteomyelitis, due to lack of compliance with prescribed antibiotic therapy. The third patient was a 61-year-old diabetic man with a history of travel to Thailand, who presented with jaundice and fever of unknown origin. Liver and splenic abscesses due to B. pseudomallei were diagnosed. A high clinical alertness to patients' travel history, underlying diseases, and the presence of concomitant splenic abscess is essential to early detection of the great mimicker, melioidosis. The treatment of choice is intravenous ceftazidime for at least 14 days or more. An adequate duration of maintenance oral therapy, with amoxicillin-clavulanate or trimethoprim-sulfamethoxazole for 12-20 weeks, is necessary to prevent relapse. Liver abscess in Taiwan is most commonly due to K. pneumoniae, but clinicians should keep in mind that this may be a presenting feature of melioidosis.     

Hyperemesis gravidarum,gastrointestinal and liver disease in pregnancy

Women of childbearing age may be affected by diseases of the gastrointestinal tract or liver; some have no effect on obstetric outcome, some are improved in pregnancy and some deteriorate. Gastrointestinal or liver disease may be caused by pregnancy and resolve following delivery e.g. hyperemesis gravidarum, pre-eclampsia, acute fatty liver of pregnancy, HELLP syndrome and obstetric cholestasis, or may present for the first time in pregnancy e.g. inflammatory bowel disease, cholelithiasis and hepatitis. These examples and other common gastrointestinal and liver disorders will be discussed giving details of diagnosis, management and effects on pregnancy outcome and also background information on normal liver physiology in pregnancy.     

Amniotic fluid pregnancy-specific beta 1-glycoprotein (SP1) in fetal developmental disorders

Concentration of pregnancy-specific beta 1-glycoprotein (SP1) was studied in second and third trimester amniotic fluid from pregnancies with various fetal developmental disorders. The material consisted of 26 cases with chromosomal disorders and 19 cases with non-chromosomal fetal malformations. The SP1 concentration was elevated in two cases of Meckel's syndrome (mean +2.7-4.0 S.D.) as well as in one case of fetal triploidy (mean +22 S.D.), while it was normal in all other 14 different fetal disorders.     

Preeclampsia - What is to blame? The placenta,maternal cardiovascular system or both?

Preeclampsia (PE) is a pregnancy-specific syndrome, complicating 2%-8% of pregnancies. PE is a major cause of maternal mortality throughout the world with 60000 maternal deaths attributed to hypertensive disorders of pregnancy. PE also results in fetal morbidity due to prematurity and fetal growth restriction. The precise aetiology of PE remains an enigma with multiple theories including a combination of environmental, immunological and genetic factors. The conventional and leading hypotheses for the initial insult in PE is inadequate trophoblast invasion which is thought to result in incomplete remodelling of uterine spiral arteries leading to placental ischaemia, hypoxia and thus oxidative stress. The significant heterogeneity observed in pre-eclampsia cannot be solely explained by the placental model alone. Herein we critically evaluate the clinical (risk factors, placental blood flow and biomarkers) and pathological (genetic, molecular, histological) correlates for PE. Furthermore, we discuss the role played by the (dysfunctional) maternal cardiovascular system in the aetiology of PE. We review the evidence that demonstrates a role for both the placenta and the cardiovascular system in early- and late-onset PE and highlight some of the key differences between these two distinct disease entities.     

Management of chronic pelvic pain

Chronic pelvic pain (CPP) is a common complaint of women presenting for gynecologic and primary care. Evaluation of CPP requires obtaining a careful history including not only obstetrical and gynecologic information but also screening for gastrointestinal, urologic, musculoskeletal, and neurological disorders. A detailed physical examination is also necessary. Management of CPP depends largely on the cause. Gynecologic causes include endometriosis, pelvic inflammatory disease, adhesive disease, pelvic congestion syndrome, ovarian retention syndrome, ovarian remnant syndrome, adenomyosis, and leiomyomas. Some non-gynecologic causes are interstitial cystitis/painful bladder syndrome, irritable bowel syndrome, pelvic floor tension myalgia, and abdominal myofascial pain syndrome. Treatments may be directed toward specific causes or may be targeted to general pain management. The most effective therapy may involve using both approaches. The diagnosis and treatment of each of the above disorders, and the management of CPP itself, is discussed.