Jacques Bernuau

Pregnancy in women with known and treated Budd–Chiari syndrome: Maternal and fetal outcomes

BACKGROUND/AIMS Budd-Chiari syndrome (BCS) mainly affects women of childbearing age. We aimed to clarify whether pregnancy, a thrombotic risk factor, should be contraindicated in patients with known and treated BCS. METHODS A retrospective study of pregnancy in women with known and treated BCS. RESULTS Sixteen women had 24 pregnancies. Nine women had undergone surgical or radiological treatment. Anticoagulation was administered during 17 pregnancies. Seven fetuses were lost before gestation week 20. Deliveries occurred between week 20 and 31 in two patients, week 32 and 36 in eleven and after week 37 in four. There was one stillbirth, but 16 infants did well. Factor II gene mutation was a factor for a poor outcome of pregnancies. In two patients, symptomatic thrombosis recurred during pregnancy or postpartum. All patients were alive after a median follow-up of 34 months after the last delivery. Bleeding at delivery, although non-lethal, occurred only on anticoagulation therapy. CONCLUSIONS When known and treated BCS is well controlled, pregnancy should not be contraindicated as maternal outcome, and fetal outcome beyond gestation week 20, are good. The risk-benefit ratio of anticoagulant therapy needs to be further clarified. Patients should be fully informed of the persistent risks of such pregnancies.

Pregnancy: a risk factor for Budd–Chiari syndrome?

In a review on Recent Advances in Clinical Practice for Budd–Chiari syndrome (BCS) ( Gut 2008; 57 :1469–78), one of us emphasised that multiple thrombotic risk factors are usually present in patients with BCS. Below, we report recent results from our group which further support this paradigm and extend it to the context of pregnancy. An influence of female sex hormones on the risk of BCS has long been recognised on the basis of an increased risk of BCS among oral contraceptive users.1 2 However, the role of pregnancy in causing BCS remains unclear: although 6–47% of reported BCS cases encountered in women presented in pregnancy or postpartum (hereafter referred to as time-related to pregnancy), other risk factors for thrombosis were not investigated.3–6 Among 96 patients with primary BCS diagnosed in our centre between January 1995 and December 2005, there were …

Foie et prééclampsie

HELLP syndrome complicates PE in 5 to 20 % of cases. The clinical manifestations (i.e. epigastric pain, elevated liver enzymes, thrombocytopenia and hemolysis) are secondary to the fibrin deposit within the peri-portal sinusoids. The clinical presentation of HELLP syndrome can be misleading. It is therefore necessary to suspect this complication whenever a PE patient develops gastro-intestinal pain. The interruption of pregnancy is the only effective treatment against HELLP syndrome. If it can be safely performed passed the 34(th) week of amenorrhea, a protective attitude should be adopted prior to reaching this date. This consists of the administration of corticosteroid therapy for fetal pulmonary maturation, intensive clinical, biological and sonographic monitoring of the mothers parameters. The administration of corticosteroids or performing a plasmapharesis is not recommended for the treatment of established HELLP syndrome because neither improves the maternal or neonatal outcome. The differential diagnosis may also include acute fatty liver of pregnancy. An early liver impairment, polyuria-polydipsia syndrome and a rise in INR support this diagnosis.

Amniocentèse et risque viral (hépatites virales B, C et VIH)

Very few studies have properly addressed to the risk of fetal hepatitis B (HBV), hepatitis C (HCV) or human immunodeficiency virus (HIV) infection through amniocentesis. For HBV, this risk is low. However, knowledge of the maternal hepatitis B e antigen status is valuable in the counselling of risks associated with amniocentesis. For HCV, the risk is not well known but cannot be excluded. For HIV, it seems rational to propose a viral test before amniocentesis for patients with contaminations risk and to postpone the sampling in cases with positive results in order to obtain an undetectable HIV-1 RNA viral load. For these reasons, it can be useful to analyse for each virus the benefit of amniocentesis and the risk of mother-to-infant transmission, and to inform the patient.

Transient excess of liver fat detected by magnetic resonance imaging in women with acute fatty liver of pregnancy

OBJECTIVE: Acute fatty liver of pregnancy (AFLP) is a lifethreatening complication of pregnancy characterized by acute development of intrahepatocyte microvesicular steatosis. Recovery is associated with normalization of liver blood test results in early postpartum weeks 1 and full restitution of normal liver tissue. Diagnosis of AFLP is accepted when at least 6 of the 14 Swansea criteria are present in the absence of another explanation. 2,3 The aim of this study was to show that magnetic resonance (MR) imaging (MRI) with quantification of intrahepatic fat allows detection of transient liver steatosis in patients with AFLP noninvasively.

État des connaissancesCirrhose biliaire primitive et grossessePrimary biliary cirrhosis and pregnancy

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease, asymptomatic during a protracted time, characterized by changes in the small-sized bile ducts near portal spaces. The etiology of PBC is undefined, but immunologic and environmental disturbances may contribute to the disease. Infertility is often associated with PBC and cirrhosis, but pregnancy may well occur in women with PBC and without cirrhosis or in some others with compensated cirrhosis. A pluridisciplinary approach including gastroenterologists and obstetricians is recommended. The patient must be closely monitored throughout her pregnancy with maternal and routine antenatal care. Medical treatment requires ursodeoxycholic acid (UDCA). In non-cirrhotic UDCA-treated women with PBC, pregnancy often follows a normal course with vaginal delivery. In cirrhotic patients, UDCA must be continued during pregnancy, esophageal and gastric varices must be evaluated before pregnancy, and endoscopic ligature is recommended for treating large varices. Additionally, beta-blocker therapy may be associated, especially when variceal rupture occurred previously. Elective cesarean section is recommended in patients with large esophageal or gastric varices because of the potentially increased risk of variceal bleeding during maternal expulsive efforts in case of vaginal delivery.

Cirrhose biliaire primitive et grossesse

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease, asymptomatic during a protracted time, characterized by changes in the small-sized bile ducts near portal spaces. The etiology of PBC is undefined, but immunologic and environmental disturbances may contribute to the disease. Infertility is often associated with PBC and cirrhosis, but pregnancy may well occur in women with PBC and without cirrhosis or in some others with compensated cirrhosis. A pluridisciplinary approach including gastroenterologists and obstetricians is recommended. The patient must be closely monitored throughout her pregnancy with maternal and routine antenatal care. Medical treatment requires ursodeoxycholic acid (UDCA). In non-cirrhotic UDCA-treated women with PBC, pregnancy often follows a normal course with vaginal delivery. In cirrhotic patients, UDCA must be continued during pregnancy, esophageal and gastric varices must be evaluated before pregnancy, and endoscopic ligature is recommended for treating large varices. Additionally, beta-blocker therapy may be associated, especially when variceal rupture occurred previously. Elective cesarean section is recommended in patients with large esophageal or gastric varices because of the potentially increased risk of variceal bleeding during maternal expulsive efforts in case of vaginal delivery.

Mise au pointTransplantation hépatique et grossesseLiver transplantation and pregnancy

Patients with liver failure have menstrual cycle irregularities or amenorrhea. Liver transplantation restores menstrual pattern among women with cirrhosis in childbearing years. It is now accepted that a planned pregnancy is possible among liver transplant recipients at least 1 year after liver transplantation, with stable allograft function and under immunosuppressive regimens, to minimize the risks of preterm delivery and pregnancy-induced hypertension. After 1 year, the risk of graft loss decreases and is not related to pregnancy. It is a high-risk pregnancy which requires a specific and regular multidisciplinary joint follow-up (obstetrician, hepatologist, and anaesthesiologist), which leads in most cases to successful outcome for mother and child. But, early prevention and multidisciplinary management of the most common complications (pregnancy-induced hypertension, preeclampsia, and fetal growth restriction) is essential. The prematurity rate, maternal morbidity and mortality are higher than in the general population. Usual immunosuppressive treatments (corticoids, cyclosporine, tacrolimus, azathioprine or mycophenolate mofetil) may require dose adaptation during pregnancy. Immunosuppressive drugs are not teratogenic, but breast feeding is not allowed.

Transplantation hépatique et grossesse

Patients with liver failure have menstrual cycle irregularities or amenorrhea. Liver transplantation restores menstrual pattern among women with cirrhosis in childbearing years. It is now accepted that a planned pregnancy is possible among liver transplant recipients at least 1 year after liver transplantation, with stable allograft function and under immunosuppressive regimens, to minimize the risks of preterm delivery and pregnancy-induced hypertension. After 1 year, the risk of graft loss decreases and is not related to pregnancy. It is a high-risk pregnancy which requires a specific and regular multidisciplinary joint follow-up (obstetrician, hepatologist, and anaesthesiologist), which leads in most cases to successful outcome for mother and child. But, early prevention and multidisciplinary management of the most common complications (pregnancy-induced hypertension, preeclampsia, and fetal growth restriction) is essential. The prematurity rate, maternal morbidity and mortality are higher than in the general population. Usual immunosuppressive treatments (corticoids, cyclosporine, tacrolimus, azathioprine or mycophenolate mofetil) may require dose adaptation during pregnancy. Immunosuppressive drugs are not teratogenic, but breast feeding is not allowed.