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Summary Background Pregnancies in women with pre-existing cardiomyopathies are considered at high risk for complications. However, few data are available to characterize their natural history and predict the outcome. Aims Our aim was to evaluate the prevalence and predictors of acute cardiac and obstetric events in women with a cardiomyopathy during pregnancy, excluding peripartum cardiomyopathy. Methods In this retrospective study in a referral centre for cardiomyopathies, we included 43 consecutive pregnancies in 36 women with dilated, hypertrophic, arrhythmogenic right ventricular or tachycardia-induced cardiomyopathy, or left ventricular non-compaction. Results We observed a major cardiovascular event during 15 pregnancies (35%), including three cardiac deaths, which occurred in patients who did not follow our usual early multidisciplinary protocol. The Carpreg score was predictive of maternal complication rate (67%, 36% and 31% in women with a Carpreg score of 2, 1 and 0, respectively). However, major cardiac complications occurred in four women with no risk factors. Left ventricular ejection fraction alone, gradient in hypertrophic cardiomyopathy, the Zahara score and the modified World Health Organization score appeared to be less discriminant than Carpreg for maternal outcome. There were two intrauterine fetal deaths, nine premature deliveries (23%), 17 low neonatal birth weights (40%) and 11 cases of hypoglycaemia (26%). Conclusions Pregnancy in women with a cardiomyopathy is at high risk for both women and neonates. The highest risks are observed in women who do not benefit from early multidisciplinary team management, and in patients with dilated cardiomyopathy. Our findings suggest that the Carpreg score is the most appropriate predictor of maternal complications, although the stratification might be improved.

The aim of the study was to evaluate the usefulness of therapeutic window for delivery for patients under prophylactic anticoagulation by low molecular weight heparin (LMWH) by comparing those who had a planned delivery versus those who delivered spontaneously.This retrospective monocentric study included pregnant patients with prophylactic anticoagulation (one injection per day of enoxaparin 4000UI), who delivered after 24 weeks of gestation. We collected data on pregnancy characteristics and on the delivery. We divided patients in two groups: GROUP S were patients who delivered spontaneously and GROUP P were patients who had a planned delivery for a therapeutic anticoagulant window. Our primary endpoint was the neuraxial anaesthesia rate. We compared the two groups with the student test for quantitative data and the Chi-2 test or the exact test of Fisher for qualitative data.Over a 3.5 years period, we included 100 pregnant patients with prophylactic anticoagulation. Indications of prophylactic LMWH were patients with a low risk of thrombosis, mostly personal history of cured deep venous thrombosis (36%) prior to pregnancy or non-thrombotic antiphospholid syndrome (37%). The overall rate of neuraxial anaesthesia during delivery was 86%. Twenty-six patients were included in GROUP S and 74 in GROUP P. In GROUP P, all patients had access to neuraxial anaesthesia versus 88.4% (23/26) in the GROUP S (p0.0001). For the three remaining patients, the contraindication was a short delay between the last injection of LMWH and the need for anaesthesia. The mean delay between the last injection of LMWH and neuraxial anaesthesia was 42.6+/-24.1 (13-123)h in the GROUP P and 22.7+/-9.9 (11-42)h in the GROUP S (p0.0001). One patient had deep venous thrombosis in the 6 weeks postpartum, in the GROUP P.With a planned induced delivery between 38 and 39 weeks of gestation, 11.5% of patients who went into spontaneous labour before induction would not have a neuraxial anaesthesia when respecting the 12h delay between the last injection of low molecular weight heparin and the need for anaesthesia.

International audience; Introduction : Heart failure during systemic lupus erythematosus has various causes.Case report : A 29-year-old female presented with a systemic lupus flare and a nephrotic syndrome, followed by cardiogenic shock requiring extra-corporeal membranous oxygenation. Ventricular dysfunction was related to massive myocardial infarction due to an anterior interventricular artery thrombosis and an underlying atheroma. The young age and the absence of chest pain were not suggestive of coronary artery disease initially. Coronary thrombosis was probably favored by the nephrotic syndrome, in which the arterial thrombotic risk is increased.Conclusion : Coronary artery disease should be systematically evoked in the presence of ventricular dysfunction in patients with systemic lupus, including when they are young and in the absence of chest pain. Nephrotic syndrome should be identified as a risk factor for arterial thrombosis.; Introduction : Les atteintes cardiaques au cours du lupus systémique ont des causes variées.Observation : Une patiente de 29 ans présentait 3 mois après une grossesse une poussée de lupus systémique précédemment connu, avec un syndrome néphrotique, puis un choc cardiogénique nécessitant la pose d’une assistance cardiaque extracorporelle. La dysfonction ventriculaire était liée à un infarctus myocardique massif par thrombose de l’artère interventriculaire antérieure, associée à un athérome sous-jacent. L’âge jeune et l’absence de douleur thoracique étaient peu évocateurs d’une atteinte coronarienne initialement. La thrombose coronarienne était probablement favorisée par le syndrome néphrotique, au cours duquel le risque thrombotique artériel est augmenté, et moins connu que le risque thrombotique veineux.Conclusion : Une atteinte coronarienne doit être systématiquement évoquée devant une dysfonction ventriculaire chez les patients lupiques, y compris lorsqu’ils sont jeunes et en l’absence de douleur thoracique. Le syndrome néphrotique doit être identifié comme un facteur de risque de thrombose artérielle.

International audience; Introduction : Heart failure during systemic lupus erythematosus has various causes.Case report : A 29-year-old female presented with a systemic lupus flare and a nephrotic syndrome, followed by cardiogenic shock requiring extra-corporeal membranous oxygenation. Ventricular dysfunction was related to massive myocardial infarction due to an anterior interventricular artery thrombosis and an underlying atheroma. The young age and the absence of chest pain were not suggestive of coronary artery disease initially. Coronary thrombosis was probably favored by the nephrotic syndrome, in which the arterial thrombotic risk is increased.Conclusion : Coronary artery disease should be systematically evoked in the presence of ventricular dysfunction in patients with systemic lupus, including when they are young and in the absence of chest pain. Nephrotic syndrome should be identified as a risk factor for arterial thrombosis.; Introduction : Les atteintes cardiaques au cours du lupus systémique ont des causes variées.Observation : Une patiente de 29 ans présentait 3 mois après une grossesse une poussée de lupus systémique précédemment connu, avec un syndrome néphrotique, puis un choc cardiogénique nécessitant la pose d’une assistance cardiaque extracorporelle. La dysfonction ventriculaire était liée à un infarctus myocardique massif par thrombose de l’artère interventriculaire antérieure, associée à un athérome sous-jacent. L’âge jeune et l’absence de douleur thoracique étaient peu évocateurs d’une atteinte coronarienne initialement. La thrombose coronarienne était probablement favorisée par le syndrome néphrotique, au cours duquel le risque thrombotique artériel est augmenté, et moins connu que le risque thrombotique veineux.Conclusion : Une atteinte coronarienne doit être systématiquement évoquée devant une dysfonction ventriculaire chez les patients lupiques, y compris lorsqu’ils sont jeunes et en l’absence de douleur thoracique. Le syndrome néphrotique doit être identifié comme un facteur de risque de thrombose artérielle.

Objective To describe the interplay between Behcet's disease (BD) and pregnancy. Methods This retrospective study included 76 pregnancies in 46 patients fulfilling the international criteria for BD. The median age of the patients at the time of entry into the study was 28.4 years (interquartile range 22.8–30.9 years). Patients were used as their own historical controls to assess the incidence of BD flares during pregnancy and before or after pregnancy. Factors associated with the occurrence of complications during pregnancy were assessed. Results Among the 76 pregnancies with BD analyzed, 27 (35.5%) were associated with worsening of the symptoms of BD flare; oral and genital ulcerations (78.4% and 67.6%, respectively) as well as ocular complications (32.4%) were the most frequent. The mean ± SD annual rates of BD flares were 0.49 ± 0.72 during pregnancy and 1.46 ± 2.42 during the nonobstetric period (P = 0.018). The proportion of BD flares tended to be lower in patients treated with colchicine (27.9% versus 45.4% of patients not treated with colchicine; P = 0.11). The overall rate of complications during pregnancy was 15.8%. The complications included miscarriage (5 patients), cesarean delivery (3 patients), medical termination of pregnancy (2 patients), hemolysis, elevated liver enzymes, and low platelets syndrome (1 patient), and immune thrombocytopenia (1 patient). There was a statistically significant association between a history of deep vein thrombosis in BD and the risk of obstetric complications (odds ratio 7.25, 95% confidence interval 1.21–43.46, P = 0.029). Neither gestational age at delivery nor neonatal outcome was influenced by BD. Conclusion The disease course in BD seems to improve during pregnancy, mostly in patients who are treated with colchicine. Pregnancy in patients with BD appears not to be associated with an increased rate of pregnancy-related complications.

Antiphospholipid syndrome (APS) is associated with a risk of obstetrical complications, affecting both the mother and the fetus. Obstetrical APS is defined by a history of three consecutive spontaneous miscarriages before 10 weeks of gestation (WG), an intra-uterine fetal death after 10 WG, or a premature birth before 34 WG because of severe pre-eclampsia, eclampsia or placental adverse outcomes (intrauterine growth retardation, oligohydramnios). Pregnancy in women with a diagnosis of obstetric APS is at increased risk for placental abruption, HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome and thrombosis that may be part of a catastrophic antiphospholipid syndrome (CAPS). A previous thrombosis and the presence of a lupus anticoagulant are risk factors for pregnancy failure. A multidisciplinary approach, associating the internist, the anesthesiologist and the obstetrician, is recommended for these high-risk pregnancies. Preconception counseling is proposed to identify pregnancy contraindications, and to define and adapt the treatment prior and during the upcoming pregnancy. Heparin and low-dose aspirin are the main treatments. The choice between therapeutic or prophylactic doses of heparin will depend on the patient's medical history. The anticoagulant therapeutic window for delivery should be as narrow as possible and adapted to maternal thrombotic risk. There is a persistent maternal risk in the postpartum period (thrombosis, HELLP syndrome, CAPS) justifying an antithrombotic coverage during this period. We suggest a monthly clinical and biological monitoring which can be more frequent towards the end of pregnancy. The persistence of notches at the Doppler-ultrasound evaluation seems to be the best predictor for a higher risk of placental vascular complications. Treatment optimization and multidisciplinary antenatal care improve the prognosis of pregnancies in women with obstetric APS, leading to a favorable outcome most of the time.

Objective Women with antiphospholipid (aPL) biology present obstetric complications. The α-fetoprotein (AFP) serum levels of these patients are higher than in general population. Because AFP is involved in the calculation of the risk of trisomy 21 (T21), we studied the effect of AFP variations in the presence of aPL during T21 screening. Methods The study group (aPL group) was comprised of 64 pregnancies in women with aPL antibodies. The control group was comprised of 21 655 pregnancies included in the national program for routine Down syndrome (DS) screening by maternal serum markers [human chorionic gonadotrophin (hCG) and AFP] between 14 + 0 and 18 + 6 weeks of gestation. Results AFP values, converted in logarithm of multiples of the median (MoM), were significantly higher in the aPL group (0.03 vs 0.10; p = 0.018). After a matricial transformation of AFP MoM and hCG MoM in the aPL group, new T21 risks presented a median of one in 1665 versus one in 2574 (p < 0.0001 with a rank-sign test). Conclusion Our results highlight the fact that in the presence of aPL antibodies, the calculated risk of T21 is underestimated. Therefore, clinicians should interpret the screening borderline results in aPL patients with caution. Copyright © 2009 John Wiley & Sons, Ltd.

Adrenal necrosis, a rare life threatening complication of antiphospholipid syndrome, is difficult to diagnose during pregnancy. We report the case of a 33-year-old woman with bilateral adrenal necrosis which started during the third trimester of her second pregnancy. Antiphospholipid syndrome had been diagnosed few years ago, after a thrombotic event. The pregnancy was uneventful until 36 weeks plus five days, when the patient was admitted for bilateral back ache, initially considered as uterine contractions. Labour was induced because pain persisted and was associated with major thrombocytopenia. A healthy infant was delivered vaginally on the second day, adrenal failure was diagnosed based on intense asthenia, persistent severe lumbar pain, low blood sodium and cortisol. Bilateral adrenal oedema was documented by CT scan and MRI. Symptoms resolved following administration of hydrocortisone and fludrocortisone. This case illustrates the difficulty to diagnose adrenal necrosis in the third trimester of pregnancy.

Dear Editor, Reactive hemophagocytic syndrome (RHS) is a benign histiocytic disorder characterized by abnormal phagocytosis of the hematopoietic elements [9]. Its occurrence has been documented in association with infections [1, 5, 9, 10], malignancy, and hematological diseases [5], and, less frequently, systemic diseases [3, 8, 11]. RHS has been reported during pregnancy in only four cases [2, 4, 6, 7]. We report the case of a pregnant patient with systemic lupus erythematosus (SLE), presenting with RHS. The evolution was complicated by preeclampsia and intracerebral hemorrhage. We discuss the putative causes of RHS in our patient with a review of the literature. A 28-year-old nurse was admitted at 22 weeks’ gestation (WG) for persistent fever. She had a 2-year history of SLE featuring arthritis, skin involvement, positive antinuclear, and anti-double-stranded DNA antibodies. She had been occasionally treated with nonsteroidal anti-inflammatory drugs. She suddenly developed high fever. Physical examination showed no signs of infection, hematological disease, or SLE flare. Laboratory studies showed hemoglobin of 9 g/dl with 80×10/l platelet and normal white blood cell, C-reactive protein (CRP) of 83 mg/l, triglyceride of 9.7 g/l, ferritin up to 15,000 ng/ml, and lactate dehydrogenase (LDH) up to 2,000 U/l. Serum uric acid and liver functions were normal. Fraction complement C3 was elevated. Antinuclear antibodies were 1/5,120, antidouble-stranded DNA antibodies were stable at 15 IU, and antiphospholipid antibodies remained negative. The bone marrow aspiration showed histiocytes engulfing myeloid cells, platelets, and nucleated red cells, and RHS was diagnosed. There was no evidence of malignancy associated to RHS on abdominal ultrasonography, pulmonary computed tomography (CT) scan, and lymphocyte immunophenotyping. Repeated searches for bacterial, viral, and fungal infections remained negative, including mycobacteria, Epstein–Barr virus (EBV), cytomegalovirus, herpes simplex virus, rubella virus, blood, urine cultures, and bronchoalveolar lavage. Antibiotics (amoxicillin–clavulanic acid and roxithromycin) were not efficient. At 25 WG, Ann Hematol (2007) 86:541–544 DOI 10.1007/s00277-007-0277-7

Mature oocytes are rare and highly specialized cells. In vitro maturation of human oocytes is an emerging assisted reproductive technology allowing to produce more mature oocytes without ovarian stimulation. Whereas in vitro maturation is technically more demanding than conventional in vitro fertilization for the laboratory, it carries many potential advantages, for example, in terms of lower treatment heaviness and removal of risk of severe ovarian hyperstimulation syndrome for the patients. Although the technology is still experimental, oocytes in vitro maturation has been successfully used and pregnancies and live births have been reported. Despite these successes, the overall efficiency of in vitro maturation remains low and this procedure must still be improved. The different steps of in vitro maturation process are shown and discussed as well as results in terms of pregnancy and live birth rates.

OBJECTIVE—To study maternal and fetal outcome in women with past or present histologically proven systemic lupus erythematosus (SLE) nephritis. METHOD—Retrospective study of 32 pregnancies in 22 women with past or present histologically proven SLE nephritis in a single French centre. RESULTS—Pregnancy (25 planned and 7 not planned) occurred in a mean (SD) of 8 (5) years after SLE diagnosis and 6 (4) years after renal disease onset. Seven occurred in women with antiphospholipid syndrome. At pregnancy onset, all but one woman had creatininaemia below 100 µmol/l, five had proteinuria >0.5 g/day, none had hypertension. Twelve pregnancies occurred in women previously treated with immunosuppressant drugs. Treatment comprised prednisone (n=31), hydroxychloroquine (n=11), aspirin (n=22), heparin (n=12), and azathioprine in one patient with steroid resistant nephrotic syndrome disclosing SLE. No therapeutic abortion was done. During pregnancy or the postpartum period, or both, proteinuria >0.5 g/day occurred in 10 women (five related to pre-eclampsia, four to renal flare, one to stable nephrotic syndrome). One flare consisted of mild arthralgias. Pregnancy outcome comprised one feto-maternal death in SLE disclosed by pregnancy, five embryonic losses, two fetal deaths, and 18 premature (one neonatal death) and six full term births. No criterion appeared to influence fetal survival significantly. At long term, one patient died during an SLE flare, three women had renal relapses. At the last visit, all had creatininaemia below 100 µmol/l except one woman with creatinine level 115 µmol/l, nine had proteinuria >0.5 g/day, and one was treated for hypertension. CONCLUSION—Pregnancy need not be discouraged in women with a history of SLE nephritis with normal or mildly impaired renal function. Deterioration of renal function rarely occurs. However, these pregnancies are at high risk of pre-eclampsia and prematurity.

The prognosis of systemic lupus erythematosus (SLE) has greatly improved during the last two decades, now allowing most patients to have a very long survival including a satisfactory quality of life. Initially considered contraindicated in SLE due to its overwhelming risks, pregnancy is nowadays allowed in a majority of patients, and fair results are usually obtained under appropriate management (1-3). Consequently, patients thought to have infertility ask the question of a possible therapy, i.e. ovulation induction (OI) associated or not with in vitro fertilization (IVF). Considering the importance of estrogens in the pathogenesis of the disease, the use of such procedures raise several questions in SLE. Though data remain to date extremely scarce, the theoretical and practical aspects of OI in SLE will be briefly reviewed here.

We conducted a prospective study in order to determine planned pregnancy outcome in systemic lupus erythematosus followed in a tertiary referral centre. Pregnancy was authorized if disease was inactive on 20 mg/day prednisone or less for at least 1 yr. Upon the diagnosis of pregnancy, systematic corticosteroids consisting of 10 mg/day prednisone or more were started. In the case of antiphospholipid antibodies, 100 mg/day aspirin was added, replaced by heparin in the pre-partum period. In the case of antiphospholipid syndrome complicated by previous thrombotic events or fetal losses despite aspirin, heparin was prescribed. One woman with a history of atrioventricular block was treated with dexamethasone. Patients were monitored by medical and obstetrical examination, and laboratory tests carried out at least monthly and a quarterly echography. Among 62 pregnancies in 38 women, lupus flare was observed in 27% of the cases, 6% of which occurred in the post-partum period. Flares were moderate except in one renal involvement in a woman with prior diffuse proliferative glomerulonephritis. Therapy was not modified in half of the cases. Pregnancy ended in early spontaneous abortion not related to lupus flare (n = 10), stillbirth (n = 2). induced abortion (n = 2), preterm birth (n = 29) and full-term birth (n = 19). Caesarean section was performed in nine cases. A severe infection occurred in two premature neonates. Another premature neonate was growth retarded. Two children had cutaneous neonatal lupus. No child died, neither had atrioventricular block. Stillbirth and severe prematurity were more common in mothers with antiphospholipid syndrome. After exclusion of early spontaneous and induced abortions, the live birth rate was 96%, that is close to the French general population. The main problem remains a high rate of prematurity, but without maternal or neonatal death.

Background and objective Pregnancies in women with cardiomyopathies are considered at high risk for complications. However we lack data to characterize their natural history and to predict the outcome. Our aim was to evaluate the prevalence and predictors of acute cardiac and obstetrical events in pregnant women with a cardiomyopathy, excluding peripartum cardiomyopathy. Methods and results The monocentric retrospective survey includes 43 pregnancies in 36 women with dilated cardiomyopathy (DCM, n=10), hypertrophic cardiomyopathy (HCM, n=28), arrhythmogenic right ventricular cardiomyopathy (ARVC, n=3), tachycardia-induced-cardiomyopathy (TCM, n=1) and non-compacted left ventricle (NCLV, n=1). There were 1 fetal death (with maternal cardiac death), 10 (23%) preterm deliveries and 11 (26%) low neonatal birth weights. There were major maternal cardiovascular events in 14 pregnancies including 6 acute heart failures (5 DCM, 1HCM), and 3 cardiac deaths, which occurred in cases that did not follow our usual multidisciplinary protocol (1 pregnancy denial with DCM, 1 undiagnosed HCM, 1 DCM recognized near term). CARPREG score was predictive of maternal cardiac events that occurred in 67%, 33% and 25% of pregnancies with CARPREG scores of 2, 1 and 0 respectively. However major complications (heart failure, asymptomatic degradation of LVEF, and symptomatic aggravation of pulmonary artery hypertension) occurred in 3 women with no known risk factors. LV ejection fraction alone, gradient in HCM, ZAHARA or WHO scores were less discriminant than CARPREG for maternal outcome. Conclusion Pregnancy in women with a Cardiomyopathy is a high risk period for both major cardiac and fetal events. The worst complications occurred in cases that did not benefit from a multidisciplinary team management. The highest rate of events was observed in DCM patients but significant risk was also associated with HCM. Prediction of risk with CAPREG scoring is appropriate but need to be improved.

Background In up to 5% of pregnancies, ultrasound screening detects a “soft marker” (SM) that places the foetus at risk for a severe abnormality. In most cases, prenatal diagnostic work-up rules out a severe defect. We aimed to study the effects of false positive SM on maternal emotional status, maternal representations of the infant, and mother-infant interaction. Methodology and Principal Findings Utilizing an extreme-case prospective case control design, we selected from a group of 244 women undergoing ultrasound, 19 pregnant women whose foetus had a positive SM screening and a reassuring diagnostic work up, and 19 controls without SM matched for age and education. In the third trimester of pregnancy, within one week after delivery, and 2 months postpartum, we assessed anxiety, depression, and maternal representations. Mother-infant interactions were videotaped during feeding within one week after delivery and again at 2 months postpartum and coded blindly using the Coding Interactive Behavior (CIB) scales. Anxiety and depression scores were significantly higher at all assessment points in the SM group. Maternal representations were also different between SM and control groups at all study time. Perturbations to early mother-infant interactions were observed in the SM group. These dyads showed greater dysregulation, lower maternal sensitivity, higher maternal intrusive behaviour and higher infant avoidance. Multivariate analysis showed that maternal representation and depression at third trimester predicted mother-infant interaction. Conclusion False positive ultrasound screenings for SM are not benign and negatively affect the developing maternal-infant attachment. Medical efforts should be directed to minimize as much as possible such false diagnoses, and to limit their psychological adverse consequences.

Antiphospholipid syndrome (APS) is associated with a risk of obstetrical complications, affecting both the mother and the fetus. Obstetrical APS is defined by a history of three consecutive spontaneous miscarriages before 10 weeks of gestation (WG), an intra-uterine fetal death after 10 WG, or a premature birth before 34 WG because of severe pre-eclampsia, eclampsia or placental adverse outcomes (intrauterine growth retardation, oligohydramnios). Pregnancy in women with a diagnosis of obstetric APS is at increased risk for placental abruption, HELLP (Hemolysis, Elevated Liver enzymes, Low Platelet count) syndrome and thrombosis that may be part of a catastrophic antiphospholipid syndrome (CAPS). A previous thrombosis and the presence of a lupus anticoagulant are risk factors for pregnancy failure. A multidisciplinary approach, associating the internist, the anesthesiologist and the obstetrician, is recommended for these high-risk pregnancies. Preconception counseling is proposed to identify pregnancy contraindications, and to define and adapt the treatment prior and during the upcoming pregnancy. Heparin and low-dose aspirin are the main treatments. The choice between therapeutic or prophylactic doses of heparin will depend on the patient's medical history. The anticoagulant therapeutic window for delivery should be as narrow as possible and adapted to maternal thrombotic risk. There is a persistent maternal risk in the postpartum period (thrombosis, HELLP syndrome, CAPS) justifying an antithrombotic coverage during this period. We suggest a monthly clinical and biological monitoring which can be more frequent towards the end of pregnancy. The persistence of notches at the Doppler-ultrasound evaluation seems to be the best predictor for a higher risk of placental vascular complications. Treatment optimization and multidisciplinary antenatal care improve the prognosis of pregnancies in women with obstetric APS, leading to a favorable outcome most of the time.

The present study was carried out from in vitro fertilization (IVF) attempts to analyze further the total and specific protein contents of 47 follicular fluids yielding one oocyte. The aim was to find correlations between the follicular concentrations of such proteins and the occurrence of coupled oocyte cleavage. These findings would be used as markers of IVF outcome. Two groups of follicular samples were distinguished: one group with cleavage occurrence (25 cases) and another group without cleavage or even fertilization (22 cases). In the group with cleavage a significantly higher level was observed for six proteins: C3 complement fraction and ceruleoplasmin (P less than 0.02), alpha-antitrypsin and transferrin (P less than 0.01), and alpha 2-macroglobulin and beta 2-microglobulin (P less than 0.001). The data are discussed with respect to changes in follicle permeability with advancing maturity.

Adrenal necrosis, a rare life threatening complication of antiphospholipid syndrome, is difficult to diagnose during pregnancy. We report the case of a 33-year-old woman with bilateral adrenal necrosis which started during the third trimester of her second pregnancy. Antiphospholipid syndrome had been diagnosed few years ago, after a thrombotic event. The pregnancy was uneventful until 36 weeks plus five days, when the patient was admitted for bilateral back ache, initially considered as uterine contractions. Labour was induced because pain persisted and was associated with major thrombocytopenia. A healthy infant was delivered vaginally on the second day, adrenal failure was diagnosed based on intense asthenia, persistent severe lumbar pain, low blood sodium and cortisol. Bilateral adrenal oedema was documented by CT scan and MRI. Symptoms resolved following administration of hydrocortisone and fludrocortisone. This case illustrates the difficulty to diagnose adrenal necrosis in the third trimester of pregnancy.

G.P.1 Cytokine and chemokine profiling shows rapid activation of the immune system following enzyme replacement therapy in Pompe disease E. Masat *, P. Laforet , D. Amelin , P. Veron , B. Perniconi , N. Taouagh , O. Benveniste , F. Mingozzi 1 1 University Pierre and Marie Curie, INSERM, UMR974 Paris, France; 2 AP-HP, Pitie-Salpetriere University Hospital, Paris-Est Neuromuscular Center, Paris, France; 3 Genethon, Evry, France; 4 AP-HP, Pitie-Salpetriere University Hospital, Institute of Myology, Paris, France; 5 AP-HP, Pitie-Salpetriere University Hospital, Department of Internal Medicine and Clinical Immunology, Paris, France

Idiopathic hypereosinophilic syndrome (HES) is a condition of unknown origin characterized by clinical manifestations attributable to eosinophilia and eosinophilic infiltration of tissues. Cardiac involvement is rare and threatening accounting for 33% to 43% of death in HES. Management of pregnant patients with HES is challenging and have rarely been reported, particularly in the setting of heart failure. We here report on the case of a 29-year-old woman with HES who developed severe endomyocardial fibrosis with heart failure during pregnancy. Outcome was favorable under treatment with prednisone and azathioprine. This case illustrates a favorable outcome of endomyocardial fibrosis during pregnancy.

Objective: To study the characteristics of the haemolysis, elevated liver enzymes, low platelets (HELLP) syndrome in the antiphospholipid syndrome (APS) and its influence on the subsequent pregnancies. Methods: This was a retrospective analysis of 16 episodes of HELLP complicating APS in 15 women. Results: HELLP was complete in 10 cases and partial in six. It occurred during the second trimester in seven cases (the earliest at 18 weeks' gestation), the third trimester in seven cases, and the day following delivery in two cases. Pre-eclampsia was present in six cases and eclampsia in five. Outcome of pregnancies was: live birth (n = 8), stillbirth (n = 2) and fetal death (n = 6). APS was primary in nine women and secondary to systemic lupus erythematosus (SLE) in six. HELLP revealed primary APS in six cases. Seven women were not treated. Low dose aspirin was empirically prescribed in one woman whose APS had been undiagnosed despite a history of two fetal deaths. In the other women, therapy consisted of aspirin (n = 8), low molecular weight heparin with a dose varying between 3000 and 12 000 U daily (n = 5), and high dose immunoglobulin every 4 weeks (n = 2), hydroxychloroquine (n = 4), and prednisone (n = 6). Six women had seven subsequent pregnancies, 3–6 years after the complicated pregnancy. HELLP recurred at 33 weeks' gestation in one woman with SLE treated with prednisone, hydroxychloroquine, aspirin, and enoxaparin, and pregnancy ended in live birth. One woman became pregnant after in vitro fertilisation and embryo transfer, but pregnancy ended in fetal death despite prednisone, hydroxychloroquine, and enoxaparin. Four women had five uneventful pregnancies with 100 mg daily aspirin and heparin. Conclusions: APS may be revealed by HELLP. In APS, HELLP is associated with pre-eclampsia/eclampsia in most cases and seems to occur earlier than in the general population. Heparin plus aspirin may prevent obstetric complications in the subsequent pregnancies.

Pregnancy and Behçet's disease are both disorders with increased risks of cerebral venous thrombosis. However, we describe the first case of central venous thrombosis during the pregnancy of a woman with Behçet's disease, revealed by headaches, visual obscuring, and a slight increase in cerebrospinal fluid opening pressure. Treatment with heparin and corticosteroids led to rapid amelioration.

Mature oocytes are rare and highly specialized cells. In vitro maturation of human oocytes is an emerging assisted reproductive technology allowing to produce more mature oocytes without ovarian stimulation. Whereas in vitro maturation is technically more demanding than conventional in vitro fertilization for the laboratory, it carries many potential advantages, for example, in terms of lower treatment heaviness and removal of risk of severe ovarian hyperstimulation syndrome for the patients. Although the technology is still experimental, oocytes in vitro maturation has been successfully used and pregnancies and live births have been reported. Despite these successes, the overall efficiency of in vitro maturation remains low and this procedure must still be improved. The different steps of in vitro maturation process are shown and discussed as well as results in terms of pregnancy and live birth rates.

Objective: To analyze the results and complications of ovulation induction therapy (OIT) in women with systemic lupus erythematosus (SLE) and/or the antiphospholipid syndrome (APS). Methods: A retrospective study of 21 women followed in a single tertiary-referral French center who underwent 114 OIT cycles with or without in vitro fertilization and embryo transfer (IVFET). Results: Before OIT, SLE was present in 6 women, APS in 3, SLE-related APS in 3, and discoid lupus in 1. Eight women had no identified disease and underwent 36 cycles of OIT. Diagnosis (SLE, n = 3; primary APS, n = 5) was made after OIT complication: spontaneous abortion (n = 5), SLE flare (n = 2), and thrombophlebitis (n = 1). Five women with known disease intentionally concealed their history from their gynecologists and underwent 34 cycles. Forty-four cycles were planned in 11 women, in 3 of them after complications of prior OIT performed without particular therapy and monitoring. Eighteen pregnancies occurred, which ended in 9 live births, 4 fetal deaths, and 5 embryonic losses. The pregnancy rate was higher with gonadotropin and/or gonadotropin-releasing hormone analog (GnRHa) (25% of cycles) than with clomiphene (4% of cycles, P

Introduction Le depistage au 1 er trimestre (T1) par une glycemie a jeun (GAJ) est recommande quand il existe des facteurs de risque (FDR) de diabete gestationnel (DG) Analyse des complications perinatales en cas d'oubli du depistage du T1, rattrape par celui du 2 e trimestre (T2). Materiels et methodes Etude retrospective observationnelle du 1 er janvier au 31 decembre 2012 Inclusion : patientes diagnostiquees DG selon les criteres IADPSG (HGPO 75g de glucose a24/28 SA ou GAJ au T1≥a 0,92g/l (exclusion : grossesses gemellaires). FDR au T1 dans notre maternite : IMC>30, antecedent familial de diabete au 1 er degre, antecedent personnel DG, mort foetale in utero ou macrosomie ; Groupe A (GA) : FDR de DG, diagnostique au T1 ; Groupe B (GB) : FDR de DG oublie au T1, diagnostique au T2 ; Groupe C (GC) : FDR, GAJ au T1 normale, diagnostic auT2. Comparaison des complications materno-fœtales Resultats 205 patientes : GA (n=41) âge moyen (AM) : 33,9 ans, IMC 31,2kg/m 2 ; GB (n=124) AM: 33,8, IMC 26,8kg/m 2 ; GC (n=40) AM 33,6, IMC 29,2kg/m 2 Absence de difference significative entre GA et GB : poids de naissance moyen 3 253g ; 3 262g (p=0,67), macrosomie (> 90°p) 15 % ; 8,9 % (p=0,38) petit poids pour l'âge gestationnel ( Conclusion Il n'existe pas plus de complications perinatales chez les patientes avec FDR de DG selon les criteres de notre maternite si le depistage du DG est oublie lors du T1 et rattrape au T2.

Introduction Le depistage du diabete gestationnel (DG) en France est cible sur facteurs de risque (FDR) au contraire de l’IADGSP qui recommande un depistage systematique. Nous avons evalue les complications perinatales chez les patientes presentant un DG sans FDR comparees aux patientes avec FDR selon les criteres des recommandations du CNGOF de 2010 (IMC ≥ 25, antecedent familial de diabete au 1er degre, antecedent personnel de DG, mort foetale in utero ou macrosomie, âge ≥ 35 ans) Patients et methodes Etude retrospective observationnelle du 1er janvier au 31 decembre 2012 dans notre maternite. Inclusion de toutes les patientes diagnostiquees DG selon les criteres IADPSG (HGPO 75 g de glucose entre 24 et 28 SA ou glycemie au premier trimestre ≥ a 0,92 g/l (exclusion des grossesses gemellaires). Groupe 1 : DG sans FDR, Groupe 2 : DG avec FDR. Evaluations des complications maternofoetales Resultats 297 patientes, G1 (n = 48 âge moyen 28,5 ans, IMC moyen 21,7 kg/m 2 ) G2 (n = 249, âge moyen 32,6 ans, IMC moyen 27,4 kg/m 2 ). Aucune difference significative entre les Patientes de G1 comparees a celles de G2 pour les evenements suivants : poids de naissance moyen : 3 223 g vs 3 256 g (p = 0,67) ; macrosomie (> 90°p) 8,3 % versus 8,9 % (p = 1), petit poids pour l’âge gestationnel ( Conclusion Il n’existe pas de difference significative concernant les complications maternofoetales entre les patientes DG sans FDR et avec FDR

To describe a French tertiary referral center experience in the treatment of pregnancies in patients with the antiphospholipid syndrome (APS).Retrospective review of the data of 75 consecutive pregnancies in 47 women.After exclusion of induced abortions and pregnancies occurring before APS onset, the prior live birth rate was 7.9%. Forty-nine pregnancies occurred in women with history of vascular thrombosis, 17 with history of thrombocytopenia. Heparin was prescribed in 39 pregnancies, associated with aspirin in 35 cases, and aspirin alone was used in 36 as first-line therapy. Corticosteroids were prescribed in 38 pregnancies. Three pregnancies by in vitro fertilization led to one embryonic loss, one full term birth, and one premature birth. Six pregnancies treated with immunoglobulin ended in one fetal death, 2 premature and 3 full term deliveries. The outcome of the other 66 pregnancies was one embryonic loss, 8 fetal deaths, 16 prematurates, and 38 full term births. Use of corticosteroids correlated with severe prematurity (p = 0.005), preeclampsia (p = 0.014), intrauterine growth retardation (p = 0.005), and presence of disease associated to APS (p = 0.009). After exclusion of one fetal death associated with congenital anomaly, live birth rate was 72.9%. There was a trend for higher rate of fetal survival in patients without history of vascular thrombosis (84.6 vs 66.4%; p = 0.11).Obstetrical prognosis in APS was improved by antithrombotic therapy. Studies are needed to define individual risk and specific significance of the various antiphospholipid antibodies, in order to improve the respective indications for aspirin alone or with heparin in women without thrombotic events.

Five cases of pregnancy occurring in three women with previously diagnosed Wegener's granulomatosis are described. The disease was diffuse in one case and localised in the other. Initial treatment consisted of a combination of corticosteroids and intravenous cyclophosphamide in two women, and methotrexate in one. Four pregnancies ended in live births despite pre-eclampsia in two cases. One therapeutic abortion was induced because of encephalocele. Comparable reported cases were reviewed to examine the implications of immunosuppressive treatment on the fetus. A relapse occurred during pregnancy in 40% of the cases, but in 25% if only pregnancies beginning during inactive disease were taken into account. No other indicator for maternal and fetal outcome was obvious. Pregnancy should be planned after complete disappearance of disease activity. In the case of a relapse a combination of immunosuppressive drugs and corticosteroids should be chosen rather than corticosteroids alone because the outcome of pregnancy is poor in cases of undertreatment. Prematurity remains common.

Materno-fetal platelet allo-immunization causes fetal or neonatal thrombocytopenia and sometimes severe intracerebral bleeding. The HPA-1s antigen is most generally implicated. This accident can occur during the first pregnancy with a major risk of severe recurrence during the next pregnancy. These women require specific care in a specialized center although no consensus has been reached on management of second pregnancies. Proposed treatments include immunoglobulins and/or corticosteroids, fetal blood puncture and unique or iterative platelet transfusions.

SIR, Pregnant patients with APS may develop HELLP syndrome (haemolysis; elevated liver enzymes; low platelet count) that can manifest first by elevation of ALP level. However, other causes of ALP elevation must be discussed and include elevation of ALP of placental origin. We report for the first time an APS pregnant patient presenting with ALP increased over 15-fold of placental origin.

Use of serum markers alone for trisomy 21 screening programs leads to a high rate of amniocentesis. Adding a second parameter (ultrasonography during the second trimester) might reduce this rate yet retain satisfactory sensitivity. This work was conducted to evaluate the pertinence of associating serum hCG level between 16 and 17 weeks gestation and morphological ultrasonography between 18 ans 20 weeks gestation.A prospective study was conducted in 5,163 pregnant women aged over 38 years. A morphological ultrasonography was performed in all patients whose hCG level1/150 indicated a risk. An amniocentesis was proposed if an anomaly was detected at ultrasonography.Serum hCG was above the risk threshold retained in 11.9% of the patients and among these patients at least one anomaly was detected at ultrasonography in 12%. An amniocentesis was performed in 1.4% of all patients. The positive predictive value of the screening test was 20%.Combining a serum marker and ultrasonography during the second trimester allows a reduction in the rate of amniocentesis compared with screening with serum markers alone. Sensitivity for detecting trisomy 21 remains satisfactory.

This study had two objectives. Firstly we assessed the effects of multifetal pregnancy reduction on the mothers' emotional well-being and the relationship with the children during the 2 years following intervention. Secondly at 2 years we compared mothers who had a reduction with mothers who had not and had delivered triplets. The comparisons focused on the mothers' health and their relationship with the children. Women having had a reduction in two hospitals in Paris, between May 1992 and June 1993, were contacted just after intervention for a prospective study. In all, 18 women were included. At 1 and 2 years, 10 women participated. At 2 years, 10 additional women were included. The answers of these 20 mothers were compared to those of 11 consecutive mothers of 2 year old triplets, assessed by the same psychologist in a previous prospective study. Semi-structured interviews were conducted at home. The mothers' social characteristics, their parity, the children's condition at birth and 4 months were very similar between the reduction and triplet groups. One year after birth one-third of the women in the reduction group reported persistent depressive symptoms related to the reduction, mainly sadness and guilt. The others made medical and rational comments expressing no emotion. At 2 years all but two women seemed to have overcome the emotional pain associated with the reduction. The comparison with mothers of triplets indicated that the mothers' anxiety and depression, and difficult relationship with the children were less acute in the reduction group. These results presented some limitations, since a high number of women who miscarried or refused to participate in the follow-up were not assessed at 1 and 2 years. However, a majority of women who participated in the study 2 years after intervention seemed able to accept a multifetal pregnancy reduction to achieve parental goals.

We present a case of maternal and fetal virilization caused by an androgen-secreting ovarian tumor. Biopsy at caesarean section was required to determine the etiology (Krukenberg tumor) and orient diagnosis to a primary cancer (gastric adenocarcinoma).

A male fetus presenting with prenatal hydrocephalus is reported. The fetus died during labor. Pathological examination disclosed four accessory spleens without any abnormalities of the situs. Hydrocephalus was secondary to aqueductal stenosis. Histological features of the aqueduct were consistent with a developmental defect. The association of such malformations has already been reported and could be explained by common regulatory mechanisms which control the splenic and neural tube development.

Maternal and fetal risk is high during pregnancy for young women with systemic lupus erythematosus. We analyzed outcome after a planned pregnancy program for these patients.Between 1982 and 1994, 58 pregnancy were planned in 34 women with systemic lupus. Nine of them had renal and 5 central nervous system involvement; antiphospholipid syndrome was present in 8; steroid therapy was given in 24, immunosuppressors in 2 and plasma exchange in 1. At diagnosis of pregnancy, prednisone was prescribed (at least 10 mg/d), associated with aspirin in all non-symptomatic patients with antiphospholipid antibodies followed by heparin at pre partum. Heparin was used in case of antiphospholipid syndrome. Women with anti-SSA or B antibodies and no past history of congenital atrioventricular block were not given any specific treatment.An acute lupus flare-up occurred in 27% of the cases including 6% in post partum. The flare-up was mild in all cases and treatment had to be changed in half of the cases. There were 9 early abortions, 1 induced abortion for congenital malformation, 2 fetal deaths, 28 premature deliveries and 18 term deliveries. Cesarean section was indicated in 8 cases. Severe neonatal infection occurred in 2 premature infants and 1 other was growth retarded. Cutaneous neonatal lupus was observed in 2 infants. No atrioventricular blocks occurred.Fetal death or very premature birth were more frequent in patients with antiphospholipid syndrome. When pregnancy is planned in women with systemic lupus erythematosus, live birth rate reaches 96% after exclusion of early and therapeutic abortions. This rate is close to the rate in the general population. The high rate of premature birth is the main risk, but there were no maternal nor neonatal deaths.

The psychological effect of reducing the number of embryons was studied prospectively in two Paris hospitals. All women who had multifetal pregnancy reduction (n = 18) were contacted for interviews just after the operation, during pregnancy, and four months after delivery. The decision to reduce the number of embryons was painful and led to a guilty feeling. After operation, nearly one-half of the women suffered psychologically. To justify their decision, and contain their emotions, the women spoke of the problems related to having triplets and, most importantly, the medical arguements explained by the physician. Medical counselling is thus essential to help this couples at the time of the operation. The pregnancy period is almost always a period of calm for these women. Nevertheless, four months after delivery, 5 women expressed a psychological malaise related to the reduction, and 2 women refused to continue the study because they did not want to revive their suffering. This study shows that psychological problems are of major importance after multifetal pregnancy reduction and can occur several months after delivery. These negative conclusions should however be examined in light of the consequences if reduction had not been performed.

Three long protocols of ovarian stimulation for IVF using long acting GnRH agonist and hMG were compared. 3 molecules were used: Triptoreline (Décapeptyl* 3.75 mg), gosereline (Zoladex*) and leuproreline (Enantone* 3.75 mg). Assigning of these 3 protocols were randomised. 63, 68 and 67 cycles of stimulation were studied. Results in term of pregnancy are the same in the 3 groups. Only a tendency of lower frequency of ovarian hyperstimulation in Décapeptyl 3.75 mg group is found, associated with less embryo and less cycles with cryopreservation of supernumeraries embryos (p = 0.1).

Having carried out four cases of vaginal caesarean section the authors describe the technique they used. The advantages of the operation are: it is simple and can be carried out quickly, future obstetric behaviour is not compromised. It is important to avoid two complications of the operation: haemorrhage and injury to the bladder. These are reduced if the vertical incision in the cervix is made in the midline and long enough. The ideal indications for the operation are absence of cervical dilatation, or the occurrence of severe maternal haemorrhage during the operation to terminate a pregnancy, or while the uterine contents are being expelled towards the end of the second trimester. It is disputable whether there is a place for vaginal caesarean operation when the fetus is alive, in view of the recent studies on fetal prognosis.

The case reported consists of thrombosis of the left internal jugular vein occurring on the 22nd week of amenorrhea of a pregnancy achieved by IVF. No etiological factor other than biochemical ovarian hyperstimulation was found. Maternal outcome was positive but intrauterine fetal death occurred 7 weeks later at 29 weeks of amenorrhea. The late occurrence of this thrombosis after ovarian hyperstimulation need to be stressed.

The place of laparoscopic surgery continues to increase in the field of surgery in our specialty. Although the advantages would seem to be obvious, it seemed to us interesting to quantify, if possible, the parameters of operative stress and compare laparoscopic surgery with conventional surgery. Markers studied are Prolactin, Cortisol, Adrenaline, Nor-Adrenaline, Dopamine and the Beta-Endorphins. The only marker that shows any difference in the two procedures in our study is Beta-Endorphin which is significantly less raised in laparoscopic surgery directly after the operation (p less than 0.01). This was very specific for pain, which is one of the benefits of this technique and shown in this parameter which confirms the clinical impression. The curves of the changes in the different markers have been analysed and discussed.

Improvements in the management of patients with cardiac transplantation make it possible for these patients in the child-bearing age to expect a pregnancy. In fact, since 1987, several cases of pregnancy after cardiac transplantation have been reported. We report here two cases of successful pregnancy two years after cardiac transplantation. First Case. Pregnancy was uneventful until 36 weeks of gestation with no evidence of transplant rejection. At 38 weeks of gestation, a cesarean section was performed for increasing blood pressure, cholestatic pruritus and cephalopelvic disproportion. Cesarean section was performed under regional anesthesia and a healthy baby boy of 2680 gr was delivered. The patient was discharged one week after. Eighteen months after, both the mother and the baby are in good condition. Second case. Pregnancy was complicated by severe maternal anemia and fetal hypotrophia. Because of increasing renal insufficiency and pre eclampsia, a cesarean section was performed under regional anesthesia at 36 weeks of gestation. Delivery of a healthy baby girl of 1700 g. Five other cases have been reported. Cesarean section performed in september 1984 for a patient who underwent cardiac transplantation in 1980. Delivery of a healthy baby of 3280 gr. Death of the mother five months after following heart transplant rejection. Vaginal delivery performed in august 1986 for a patient who underwent cardiac transplantation in 1984. Delivery of a preterm baby at 31 weeks of gestation who survived without any sequelae. Vaginal delivery performed in august 1987 for a patient who underwent cardiac transplantation in april 1985. Delivery of a healthy baby of 2550 gr at 38 weeks of gestation. Vaginal delivery of twins (baby girl of 1200 and 1100 gr) in april 1988 of a patient who underwent cardiac transplantation in 1986. Vaginal delivery at 38 weeks of gestation performed in november 1990 for a patient who underwent cardiac transplantation in 1986. All the cases reported showed that: The foetus is not affected by the immuno-suppressive treatment of the mother. This was already known for pregnant patients with renal transplant. The cardiovascular changes associated with pregnancy are well tolerated by the heart transplant. Preexisting hypertension is increased, particularly during the third trimester of pregnancy and during labour. The incidence of preterm labor is increased in patients with heart transplant. Transplant rejection never occurred during pregnancy.

The authors used epidural anaesthesia to carry out laparoscopy in 220 patients. The chief indications for the laparoscopies were GIFT (intratubal transfer of gametes) and tubal sterilization. The technique used was slightly different according to the indications for the use but they had to be sure of anaesthetising up to T4. In 90% of cases the patients tolerated the procedure well. No change in ventilation or in metabolic measurements. As far as fertilization was concerned studies carried out in various parameters failed to show any untoward side effects due to the use of local anaesthetics. Finally so long as the anaesthesia is only used for a short length of time this technique seems to be suitable for day cases.