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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.

Objective.To compile and assess data about complication and success rates forin vitrofertilization (IVF) of women with systemic lupus erythematosus (SLE) and/or antiphospholipid syndrome (APS). To date, such data are sparse.Methods.This retrospective study described women with SLE and/or APS who have had at least 1 IVF cycle.Results.Thirty-seven women with SLE (n = 23, including 8 with antiphospholipid antibodies), SLE with APS (n = 4), or primary APS (n = 10) underwent 97 IVF procedures. For 43% of cases, the infertility was female in origin, for 19% male, 14% mixed, and 24% unexplained. No women had premature ovarian insufficiency because of cyclophosphamide. Median age at IVF was 34 years (range 26–46). The median number of IVF cycles was 2.6 (1–8). Patients were treated with hydroxychloroquine (72%), steroids (70%), azathioprine (3%), aspirin (92%), and/or low molecular weight heparin (62%). There were 27 (28%) pregnancies, 23 live births among 26 neonates (3 twin pregnancies), 2 miscarriages, and 2 terminations for trisomy 13 and 21. Six spontaneous pregnancies occurred during the followup. Finally, 26 women (70%) delivered at least 1 healthy child. Complications occurred in or after 8 IVF cycles (8%): SLE flares in 4 (polyarthritis in 3 and lupus enteritis in 1) and thromboembolic events in 4 others. One SLE flare was the first sign of previously undiagnosed SLE. Poor treatment adherence was obvious in 2 other flares and 2 thromboses. No ovarian hyperstimulation syndrome was reported.Conclusion.These preliminary results confirm that IVF can be safely and successfully performed in women with SLE and/or APS.

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.

RésuméLa survenue d’une grossesse au cours du lupus systémique est une situation fréquente qui reste associée à une morbi-mortalité maternelle et fœtale plus importante que dans la population générale. Les complications incluent les poussées lupiques, les complications obstétricales (pertes fœtales, retard de croissance in utero, pré-éclampsie, prématurité) et le lupus néonatal. Une biologie ou un syndrome des antiphospholipides augmente nettement le risque de complications obstétricales. L’amélioration de la prise en charge de ces grossesses est conditionnée par leur planification systématique, idéalement au cours d’une consultation pré-conceptionnelle, et par une prise en charge multidisciplinaire avec une collaboration étroite entre interniste/rhumatologue, obstétricien et anesthésiste. L’absence d’activité du lupus, un traitement compatible avec la grossesse adapté aux antécédents et aux risques ainsi qu’une surveillance régulière sont les principaux éléments permettant une issue favorable à ces grossesses à risque. Cette revue a pour objectif de proposer une mise au point sur la prise en charge actuelle de la grossesse au cours du lupus systémique ou du syndrome des antiphospholipides afin de limiter le risque de complications et d’assurer le meilleur pronostic tant maternel que fœtal.AbstractPregnancy in systemic lupus erythematosus patients is a common situation that remains associated with higher maternal and fetal mortality/morbidity than in the general population. Complications include lupus flares, obstetrical complications (fetal loss, in utero growth retardation, prematurity) and neonatal lupus syndrome. The association with antiphospholipid antibodies or antiphospholipid syndrome increases the risk of obstetrical complications. Improving the care of these pregnancies depends upon a systematic pregnancy planning, ideally during a preconception counseling visit and a multidisciplinary approach (internist/rheumatologist, obstetrician and anesthetist). The absence of lupus activity, the use of appropriate medications during pregnancy adjusted to the patient's medical history and risk factors, and a regular monitoring are the best tools for a favorable outcome for these high-risk pregnancies. The aim of this review article is to perform an update on the medical care of pregnancy in systemic lupus erythematosus or antiphospholipid syndrome to reduce the risk of complications and to ensure the best maternal and fetal prognosis.

Fertility is not impaired in systemic lupus erythematosus or antiphospholipid syndrome, but, similarly to the general population, these patients may undergo in vitro fertilization. This type of treatment increases the risk of lupus flare, thrombosis, and ovarian hyperstimulation syndrome. This review will focus on in vitro fertilization in systemic lupus erythematosus or antiphospholipid syndrome. Literature data are relatively scant with only 3 reported studies. The first one included 17 patients and 63 cycles of induction ovulation/in vitro fertilization leading to 25 % of lupus flare, no thrombosis, and 3 % of ovarian hyperstimulation syndrome. The second study included 10 patients and 40 cycles of in vitro fertilization showing 31 % of lupus flare, no thrombosis and no ovarian hyperstimulation syndrome. The last one included 34 patients and 83 procedures of in vitro fertilization leading to 8 % of flares, 5 % of thrombosis and no ovarian hyperstimulation syndrome. Interestingly, in this last study, half of the complications were explained by poor adherence to treatment. These data are reassuring but it is important to remember that in vitro fertilization should be scheduled and carefully supervised in the same way as the high-risk pregnancies occurring in these patients.

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

Resume Les fœtus ou les enfants des femmes porteuses d’un anticorps anti-SSA/Ro et/ou anti-SSB/La peuvent developper un « lupus erythemateux neonatal » comportant une atteinte cutanee, hematologique, hepatique et/ou un bloc auriculo-ventriculaire congenital (BAVc) survenant sur un cœur indemne de cardiopathie malformative majeure. La frequence du BAVc chez les femmes ayant une connectivite associee a un anticorps anti-SSA/Ro est estimee a 1 a 2 % et le risque de recurrence est de 10 a 17 %. Le BAVc complet est definitif et est associe a une morbidite (necessite d’implanter un pace maker dans 2/3 des cas) et une mortalite (16 a 19 %) faisant toute la gravite de ce syndrome. Une atteinte myocardique peut etre associee d’emblee ou apparaitre tardivement. Les autres atteintes sont egalement discutees. Lorsqu’une femme enceinte est porteuse d’un anticorps anti-SSA/Ro, la surveillance echographique obstetricale doit etre realisee tous les 15 jours entre 16 et 24 SA, voire toutes les semaines si elle a un antecedent de BAVc lors d’une grossesse precedente. Un electrocardiogramme est pratique de principe chez tous les enfants en periode neo-natale pour depister des BAVc incomplets. Le traitement curatif in utero du BAVc fait appel aux corticoides fluores, en privilegiant la betamethasone. Leur efficacite est inconstante.

Objective The use of hydroxychloroquine (HCQ) in pregnancy remains controversial. The recent demonstration that HCQ passes across the placenta, with cord blood concentrations nearly identical to those found in maternal blood, emphasizes the need for careful evaluation of pregnancies in women receiving HCQ. However, only small series of HCQ-treated pregnant women have been reported, and most of these studies had no control group. We now report our experience with 133 pregnancies in women being treated with HCQ, resulting in 117 live births. Results in the HCQ group are compared with those in a control group. Methods One hundred thirty-three consecutive pregnancies in 90 women treated with 200 mg of HCQ either twice daily (122 pregnancies) or once daily (11 pregnancies) were studied. These pregnancies were compared with 70 consecutive pregnancies in 53 women with similar disorders who did not receive HCQ. Electrocardiography was performed in 47 children of mothers treated with HCQ and in 45 children in the control group. Results Eighty-eight percent of pregnancies in the HCQ group and 84% of those in the control group ended successfully with a live birth. The outcomes of pregnancy were not statistically different between groups. One child in each group died of causes related to prematurity. Three malformations were observed in the HCQ group (1 hypospadias, 1 craniostenosis, and 1 cardiac malformation) versus 4 in the control group. On the electrocardiograms, the PR interval and the corrected QT interval were not statistically different between groups. No visual, hearing, growth, or developmental abnormalities were reported in any of the children at the last follow-up (ages 12–108 months; mean age 26 months). Conclusion Our findings support preliminary evidence for the safety of HCQ therapy during pregnancy. This treatment probably should be maintained throughout pregnancy in patients with systemic lupus erythematosus.

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.

Resume Le lupus erythemateux neonatal se manifeste par une atteinte cutanee, hematologique, hepatique ou cardiaque incluant un eventuel bloc auriculo-ventriculaire congenital (BAVc) survenant sur un cœur indemne de cardiopathie malformative, une cardiomyopathie dilatee et une fibroelastose endocardique. Ce syndrome rare est constamment associe a la presence chez la mere d’anticorps anti-SSA/Ro et/ou anti-SSB/La. La frequence du BAVc chez les femmes ayant une connectivite associee a un anticorps anti-SSA/Ro est estimee entre 1 et 2 %, et le risque de recurrence est evalue a environ 19 % lors d’une grossesse ulterieure. Les atteintes cutanees et systemiques sont transitoires, contrairement aux atteintes cardiaques et notamment au BAVc qui est definitif, associe a une morbidite et a une mortalite d’environ 18 %. La mise en place d’un pacemaker est necessaire dans 2/3 des cas. Une myocardite peut etre associee d’emblee ou apparaitre plus tardivement. Les meres d’enfants ayant un BAVc sont le plus souvent asymptomatiques au moment de la decouverte du BAVc. La physiopathologie n’est pas completement elucidee a ce jour. Lorsqu’une femme enceinte est porteuse d’un anticorps anti-SSA/Ro, une surveillance echographique obstetricale sera realisee tous les 15 jours entre 16 et 24 semaines d’amenorrhee et un electrocardiogramme sera fait de principe chez l’enfant. L’interet d’un traitement curatif in utero du BAVc par corticoides fluores n’est a ce jour pas demontre. Un traitement preventif par hydroxychloroquine semble diminuer le risque de recidive du BAVc chez les femmes ayant deja eu un enfant atteint. Une etude prospective est en cours pour confirmer ces resultats.

Pregnancy in systemic lupus erythematosus patients is a common situation that remains associated with higher maternal and fetal mortality/morbidity than in the general population. Complications include lupus flares, obstetrical complications (fetal loss, in utero growth retardation, prematurity) and neonatal lupus syndrome. The association with antiphospholipid antibodies or antiphospholipid syndrome increases the risk of obstetrical complications. Improving the care of these pregnancies depends upon a systematic pregnancy planning, ideally during a preconception counseling visit and a multidisciplinary approach (internist/rheumatologist, obstetrician and anesthetist). The absence of lupus activity, the use of appropriate medications during pregnancy adjusted to the patient's medical history and risk factors, and a regular monitoring are the best tools for a favorable outcome for these high-risk pregnancies. The aim of this review article is to perform an update on the medical care of pregnancy in systemic lupus erythematosus or antiphospholipid syndrome to reduce the risk of complications and to ensure the best maternal and fetal prognosis.

Fertility is not impaired in systemic lupus erythematosus or antiphospholipid syndrome, but, similarly to the general population, these patients may undergo in vitro fertilization. This type of treatment increases the risk of lupus flare, thrombosis, and ovarian hyperstimulation syndrome. This review will focus on in vitro fertilization in systemic lupus erythematosus or antiphospholipid syndrome. Literature data are relatively scant with only 3 reported studies. The first one included 17 patients and 63 cycles of induction ovulation/in vitro fertilization leading to 25 % of lupus flare, no thrombosis, and 3 % of ovarian hyperstimulation syndrome. The second study included 10 patients and 40 cycles of in vitro fertilization showing 31 % of lupus flare, no thrombosis and no ovarian hyperstimulation syndrome. The last one included 34 patients and 83 procedures of in vitro fertilization leading to 8 % of flares, 5 % of thrombosis and no ovarian hyperstimulation syndrome. Interestingly, in this last study, half of the complications were explained by poor adherence to treatment. These data are reassuring but it is important to remember that in vitro fertilization should be scheduled and carefully supervised in the same way as the high-risk pregnancies occurring in these patients.

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.

L'etude phenologique du debourrement vegetatif du sapin de Cephalonie a ete realisee sur le bourgeon terminal de la tige principale d'individus âges de 11 ans. L'echantillon, represente sur la plantation comparative de Saint-Lambert (Vaucluse), etait compose de 9 provenances, dont quatre etaient subdivisees en descendances maternelles. L'utilisation d'une methode d'observation qualitative en 5 points (Debazac, 1965 et 1967) apporte une precision supplementaire significative dans la discrimination des provenances par rapport a une methode indiquant seulement l'etat debourre ou non debourre du bourgeon. La precision des 2 methodes d'observation est relativement comparable pour le calcul des parametres genetiques

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.

Fetuses and infants of women with anti-SSA/Ro and anti-SSB/La antibodies are at risk of neonatal lupus syndrome, featuring skin lesions, hematological and hepatic disorders, and congenital heart block (CHB) in the absence of severe cardiac malformation. The prevalence of CHB in newborns of anti-SSA/Ro positive women with known connective tissue disease is 1 to 2% and the risk of recurrence ranges from 10 to 17%. CHB is definitive and is associated with significant morbidity (pacemaker must be implanted in 2/3 of cases) and mortality (16 to 19%). Myocardial involvement may either be associated or appear subsequently. Other manifestations are discussed. For anti-SSA/Ro positive pregnant women, echocardiograms should be performed every 2 weeks from 16 to 24 weeks of gestation, and every week in case of past history of CHB. Electrocardiogram should be performed in the first days of life for all children to detect incomplete CHB. Therapy for CHB detected in utero is based on fluorinated steroids, especially betamethasone. Its efficiency is variable.

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.

Objective Aside from congenital heart block (CHB), sinus bradycardia and prolongation of the corrected QT (QTc) interval have been reported in infants born to mothers with anti-SSA antibodies. To assess the pathologic nature of these manifestations, this study focused on electrocardiographic (EKG) variations in these children, comparing them with findings in a control group. Methods We studied 165 consecutive pregnancies in 106 anti-SSA–positive women with connective tissue diseases (CTDs). EKGs obtained on 58 children of this group were compared with those obtained on 85 infants born to mothers with CTD who were negative for both anti-SSA and anti-SSB. Results No statistically significant difference was seen between the 2 study groups with regard to gestational age, prematurity, birth weight, age of the children at the time of EKG, age of the mothers, or treatments received by the mothers during their pregnancies. Seven of 137 children developed cutaneous neonatal lupus syndrome; 1 child developed CHB (CHB risk of 1 in 99 [1%] if only the first prospectively observed pregnancy in women without a history of CHB is included in the analysis). For EKGs recorded during the first 2 months of life, the mean ± SD PR interval was 96 ± 16 msec in the anti-SSA–positive group and 96 ± 13 msec in the anti-SSA–negative group (P = 0.84), with mean QTc values of 397 ± 27 and 395 ± 25 msec (P = 0.57) and mean heart rates of 141 ± 23 and 137 ± 21 beats per minute (P = 0.20), respectively. No difference in the PR interval, QTc interval, or heart rate was observed for EKGs obtained between 2 and 4 months of life. When EKGs obtained at 0–2 months were compared with those obtained at 2–4 months, a physiologic prolongation of the QTc interval was observed in both study groups. No sudden infant death or symptomatic arrhythmia occurred during the first year of life. Conclusion The EKG findings in children of anti-SSA–positive and anti-SSA–negative mothers were not significantly different. Our results suggest that the prolongation of the QTc interval and sinus bradycardia that have recently been reported in children of mothers with anti-SSA antibodies occur independently of the anti-SSA antibodies. The pathologic nature of these EKG variations was not confirmed by our controlled study.

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.

Neonatal lupus syndrome include skin lesions, hematological and hepatic disorders, and congenital heart block (CHB) in the absence of severe cardiac malformation. This rare disorder is closely linked to transplacental transport of anti-SSA/Ro and anti-SSB/La maternal antibodies.The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is 2% and the risk of recurrence ranges from 10 to 17%. Skin and systemic lesions are transient, whereas CHB is definitive and is associated with significant morbidity and mortality (estimated at 16-19%). A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or may appeared secondarily. Mothers of children with CHB are usually asymptomatic or have Gougerot-Sjögren, or undifferentiated connective tissue disease. Mothers of children with cutaneous manifestations may present with more severe disease and systemic lupus erythematosus. In anti-Ro/SSA positive pregnant women, echocardiograms should be performed at least every 2 weeks from 16 to 24 weeks gestation. Electrocardiogram should be performed for all children.The efficiency of prophylactic treatment of CHB is not established. Therapy for CHB detected in utero is not standardized and involves fluorinated steroids (especially betamethasone).

Background: Mothers with anti-SSA/Ro antibodies who have had a previous fetus with congenital heart block (CHB) have a risk of recurrence estimated to be up to 16%. Objective: To improve the management of these “high risk patients” by determining ( a ) whether or not prophylactic treatment is efficient; ( b ) whether or not fluorinated steroids (betametasone and dexamethasone) that do cross the placenta in an active form are safe for the fetus; and ( c ) which prophylactic treatment should be used. Methods: Retrospective study performed on seven mothers sent to a university hospital owing to a past history of one (six mothers) or two children (one mother) with CHB. Results: 13 subsequent pregnancies occurred. No CHB was observed. All four pregnancies in women treated with 10 mg/day prednisone were uneventful. Three pregnancies in women receiving no steroids resulted in two early spontaneous abortions and one live birth. The six pregnancies in women treated with dexamethasone (4–5 mg/day) ended in one early and one late spontaneous abortion, two stillbirths, and two live births with intrauterine growth restriction and mild adrenal insufficiency. A histological study of one stillbirth disclosed intrauterine growth restriction and marked adrenal hypoplasia. Conclusion: Adverse obstetric outcomes were often seen here and major concerns have been raised by paediatricians about the safety of fluorinated steroids, owing to the results of animals studies, retrospective data, and randomised trials. Because fluorinated steroids have not been shown to improve prophylactic treatment of CHB in pregnant women at high risk, their use is questionable.

Summary Objectives To validate a diagnostic test for gestational diabetes which predicts the risk of macrosomia. Methods A prospective study was carried out among 354 women at risk for gestational diabetes to compare two glucose tests diagnosing pregnancies at risk of macrosomia. The "practical" test consisted in glucose measurement in the fasting state and two hours after an usual breakfast and the "reference" test was the test proposed in France (O'Sullivan test with or without a 100 g oral glucose tolerance test). Both tests were made between the 24 th and 28 th week of gestation. Women at high risk for macrosomia were treated. The first assessment criterion was macrosomia (babies large for gestational age). Because of the presence of women treated for gestational diabetes in our sample, the sensitivity and specificity of the tests in diagnosing pregnancies at risk of macrosomia were calculated using either the incidence of macrosomia observed in our population, or the incidence of macrosomia observed theoretically in the absence of treatment (22% in literature). Results Macrosomia was diagnosed in 49 neonates (14%). The "practical" test was significantly more sensitive than the reference test (respectively 46.9% versus 16.3%, p=0.0001 in the first case, and 54.3% versus 20.1%, p=0.0001 in the second case). The "reference" test was significantly more specific than the "practical" test (respectively 80% versus 68.2%, p=0.0001 in the first case, and 80.6% versus 70%, p=0.0001 in the second case). Conclusion Our study shows that the simplified "practical" test is more sensitive than the "reference" test currently used in France in screening women at risk of macrosomia.

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.