Prenatal diagnosis of congenital megalourethra associated with VACTERL sequence in twin pregnancy: favorable postnatal outcome.
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Biomedical subjects
Publications and source records attributed to F Puech.
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PURPOSE: Pregnancy complicated by the HELLP syndrome and antiphospholipid syndrome have rarely been reported. We report a study on anticardiolipin antibodies in HELLP syndrome. METHODS: Between March 1996 and September 1999, anticardiolipin antibodies were checked in all women with HELLP syndrome hospitalised in a maternity of the North of France. The women with positive anticardiolipin antibodies were seen month later in a internal medicine department. RESULTS: In the period 68 women with HELLP syndrome were checked for anticardiolipin antibodies. Apl were present in 9 patients (Incidence 42.8/1000 HELLP Year). They persisted after the accident only in 3 patients. Antiphospholipid syndrome was diagnosed in 2 patients, prevalence between the HELLP syndrome for the 42 month period was 0.03. CONCLUSIONS: HELLP syndrome may be a manifestation linked to the antiphospholipid syndrome and may revealed it.
BACKGROUND: Repeated plasmapheresis was used to prevent fetal death from severe anti-Kell alloimmunization until intrauterine transfusions were feasible. CASE: Repeated maternal plasma exchanges (N = 40) beginning at 7 weeks' gestation were used to treat severe anti-Kell alloimmunization. Ultrasound examination at 19 weeks' gestation revealed diffuse hydrops in this fetus (umbilical venous hemoglobin, 1.2 g/dL), which then required nine intrauterine transfusions through 34 weeks. A healthy 3840-g girl was delivered by cesarean delivery at 36 weeks. Despite aplastic anemia during the first 3 months of life, she is healthy and has no observable abnormalities at age 8. CONCLUSION: A highly aggressive course of plasmapheresis and intrauterine transfusions can successfully treat fetal anemia caused by anti-Kell alloimmunization even when fetal hemoglobin is extremely low.
OBJECTIVES: Our objectives were to link hyperemesis gravidarum with biochemical hyperthyroidism and hormonal modification (HCG and estradiol) and to test the hypothesis that biological abnormalities (ionogram and liver enzyme) are more often reported in hyperemesis gravidarum with biochemical hyperthyroidism. STUDY DESIGN: Thirty three patients admitted in "Hôpital Jeanne de Flandre" with hyperemesis gravidarum were studied prospectively. RESULTS: Twenty-two patients (66.7%) had biochemical hyperthyroïdsm (suppressed thyroid stimulating hormone or increased triiodothyronine index or tetraiodothyronine index). Hyperthyroid patients were more likely than euthyroid patients to have abnormal electrolyte levels (16/22 [72.7%] vs 3/1 [27.3%], P < 0.02) or increased liver enzyme levels (8/22 [36.4%] vs 3/11 [27.3%]). The severity of hyperemesis was found to vary directly with the degree of hyperthyroidism. We report a female predominance among the offspring of mothers with hyperemesis gravidarum. CONCLUSION: Our results are suggestive of the involvement of hyperthyroidism and fetal sex in the pathogenesis of hyperemesis gravidarum. Also these hypothesis are not clearly understood, human chorionic gonadotrophin occurred in the two mechanisms.
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Placental chorioangioma is a benign vascular tumor found in about 1% of pregnancies at routine histological examination of the placenta. The large placental tumor may cause serious fetal complications. We report 4 cases of placental chorioangioma diagnosed during the second trimester. Three pregnancies with rapidly growing tumors measuring more than 4 cm led to fetal hydrops and perinatal death despite thoracocentesis and cesarean delivery at 33 weeks (1 case) and repeated amniodrainage (1 case). The only surviving infant presented with a 3.3cm tumor which grew progressively to 8 cm at 38 weeks and did not induce polyhydramnios or hydrops. Although aggressive prenatal management of placental chorioangioma has been reported using amniodrainage, intrauterine transfusions, embolization, or ligation of the tumor vessel supply, prognosis remains poor for large tumors and largely depends on fetal hemodynamic tolerance.
During pregnancy, a number of maternal metabolic changes occur early and continue throughout pregnancy which help optimize the transfer of nutrients to the fetus. During normal pregnancy, there are a decrease in insulin sensibility which is physiological, progressive and reverse. For glucose tolerance to be maintained in pregnancy it is necessary for maternal insulin secretion to increase sufficiently to counteract the fall in insulin sensitivity. The metabolic characteristic of women with gestational diabetes is insufficient insulin secretion to counteract the pregnancy related fall in insulin sensitivity. There are a lot of factors that could explain the mechanism of insulin secretion and insulin sensitivity during normal pregnancy and gestational diabetes mellitus. Although glucose tolerance normalizes shortly after pregnancy with gestational diabetes in the majority of women, the risk of developing overt diabetes, especially type 2 diabetes is markedly increased. The mechanisms which could explain gestational diabetes are the same as type 2 diabetes mellitus. We could speculate that these two diseases are identical for alterations in carbohydrate metabolism, but at different stages.
Gestational diabetes, a glucose tolerance disorder of variable severity which occurs or is diagnosed for the first time during pregnancy, constitutes a public health problem because of its frequency (1 to 6% of all pregnancies) and its short-or long term consequences for the foetus and/or the mother. There is as yet still no consensus concerning screening and diagnosis criteria, therapeutic management and the reality of the disease. This population is a high risk population of diabetes mellitus, especially of type 2 diabetes. We could think that the introduction of specific prevention programs in this group could delay or avoid diabetes mellitus and its complications. The mechanisms which could explain gestational diabetes are the same as type 2 diabetes mellitus. We could speculate that these two diseases are identical for alterations in carbohydrate metabolism, but at different stages. It has been reported that the offspring of gestational diabetics mothers are at risk of obesity and glucose intolerance. Therapeutic management of the mother and/or the offspring should be better defined. The screening for gestational diabetes provides an opportunity of identify a large population of women and children at risk of diabetes. It should be possible to avoid diabetes mellitus by specific therapeutic programs in these populations.
STUDY AIM: Mechanisms that modulate fetal pulmonary circulation and transitional circulation at birth are incompletely understood. The aim of this experimental study was to describe an animal model in order to study the perinatal pulmonary circulation. MATERIAL AND METHODS: Pregnant ewes were operated on between 126 and 128 days gestation (term = 145 days). A skin incision was performed to the fetal lambs in utero and catheters were placed into the ascending aorta and the superior vena cava after insertion in the axillary artery and vein. Then, catheters were inserted into the left pulmonary artery (LPA), main pulmonary artery, and left atrium via a thoracotomy. Moreover, an ultrasonic flow transducer, and an inflatable vascular occluder were placed around the LPA and around the ductus arteriosus. During 10 days, studies were performed in utero (possibly continued when fetal lambs were delivered by caesarean section). This chronically prepared animal may be used to perform hemodynamic studies according to different protocols (drugs injection to the fetus or to the ewes, ductus arteriosus compression, oxygen test). The main pulmonary artery, aortic, left atrial and amniotic pressures, heart rhythm, and flow signal were continuously recorded. RESULTS: Eighteen pregnant ewes were operated on and nine only could be used for experimentation. This ovine model permitted several studies, particularly about effects of catecholamines on the pulmonary circulation, and about effects of ductus arteriosus compression on the pulmonary circulation. CONCLUSION: Chronically instrumented fetal lambs are an excellent model in order to study the perinatal pulmonary circulation.
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OBJECTIVE: To estimate the performance of the foetal fibronectin test as a predictor of preterm delivery. PATIENTS AND METHODS: This prospective study concern 61 patients who had a singleton pregnancy between 24 and 36 weeks of gestation and were hospitalized because of a threatened preterm labor without premature rupture of the membranes. For each patient the presence of foetal fibronectin in cervicovaginal secretions was determined with a rapid swab-test. RESULTS: Prematurity rate was 38% (23 patients). In case of positive result, delivery became before 37 weeks in 75% (12/16) against 24% in case of negative result (11/45). The prolongation of pregnancy after the test was on average 21 days in the positive group and 44 days in the negative group. About the prediction of preterm delivery, the results showed a sensibility of 52%, a specificity of 89%, a positive predictive value of 75%, a negative predictive value of 76%. To predict a delivery within the two weeks after the test, the sensibility was 88%, the specificity 83%, and the negative predictive value 98%. CONCLUSION: The presence of foetal fibronectin in cervicovaginal secretions represent an increased risk of preterm delivery, whereas its excellent negative predictive value allow to be reassuring, especially within a period of 15 days.
We describe, to our knowledge, the first case of a pulmonary malformation called acinar dysplasia occurring at a surviving fetus after selective embryo reduction in a bichorionic pregnancy. The chronological and histological observations suggest that this anomaly may be linked with a feticide achieved at 13 week's gestation. Literature review concerning selective embryo reduction shows rare cases of vascular connections in bichorionic pregnancies especially during the first half of gestation, that can explain in part the apparition of survivor's anomalies.
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OBJECTIVE: We describe the different ultrasound findings suggestive of trisomy 18. PATIENTS AND METHODS: We conducted a retrospective study in 40 cases of trisomy 18 diagnosed in the department of obstetrics at the Lille University Hospital between 1988 and 1998. RESULTS: Eighty percent of the women in this series were multiparous. Mean maternal age at discovery of the trisomy as 33.2 years and the mean gestational age was 20.4 weeks. Fifty-five percent of the cases were discovered during the second trimester of pregnancy, 22.5% during the third trimester and 22.5% during the first trimester. One ultrasound abnormality, at least, was detected in 36/40 cases (90%) a percentage that reached 96.8% taking into consideration the ultrasound examinations performed during the second and third trimesters (30/31 cases). The most frequently detected ultrasound abnormalities were: intra uterine growth retardation (IUGR: 50%), poly-hydramnios (42.5%), limb abnormalities (42.5%), cardiac defects (30%), facial abnormalities (37.5%), meningomyelocele (32.5%), digestive abnormalities (32.5%), urinary tract abnormalities (27.5%), lymphangiectasia and cystic hygroma (15%), and single umbilical artery (12.5%). Medical termination of pregnancy (TOP) was performed in 28 cases. There was one spontaneous miscarriage at 8 weeks and one in utero death (IUD) at 39 weeks in a patient who desired to continue her pregnancy. In 6 cases, the issue of the pregnancy was unknown because the patients were lost to follow-up. In 4 cases (10%), pregnancy was continued to delivery of live babies that only survived a few minutes to 7 days. CONCLUSION: The ultrasound signs suggestive of trisomy 18 change according to the term of pregnancy. At the first trimester, most of the signs are nonspecific, such as cystic hydroma or lymphangiectasia, and do not suggest the need for a karyotype. At the end of the second trimester, an association of various signs that alone would not be highly suspect suggest the need for further exploration in search of other signs: early IUGR, associated or not with poly-hydramnios, limb abnormalities, cardiac defects, omphalocele, diaphragmatic hernia, meningomyelocele, enlarged cisterna magna, choroid plexus cysts, single umbilical artery, facial dysmorphism, facial cleft, hydronephrosis.
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From October 1993 to February 1998, 33 cases of fetal cardiac arrhythmia were investigated by doppler-echocardiography at the Lille infantile and congenital cardiology department. Extrasystolic arrhythmias were the most frequently encountered disorder (25 fetuses, i.e., 76% of cases: 24 instances of extrasystolic auricular arrhythmia and one case of extrasystolic ventricular arrhythmia). They were invariably benign, and apart from one case only required standard monitoring. Tachycardia was observed in 15% of cases (three cases of supraventricular tachycardia [SVT] and two cases of auricular flutter [AF]). In no instance was a cardiopathic syndrome noted. A number of efficient treatments have been described, but the prognosis is often poor in the presence of hydrops fetalis. Direct fetal treatments (cordocentesis) are currently under evaluation, and at present can only be used as a last resort. In our series, one fetus died 15 minutes after transplacental Flecaine (flecainide) administration. Two of the three SVT and the two AF cases were successfully treated. Bradycardia, which was unassociated with extrasystolic arrhythmia, was found in 9% of cases. It is concluded that Flecaine is probably the treatment of choice for supraventricular and ventricular fetal tachycardia, as it has no teratogenic effect and crosses the placenta at a fetal concentration that is 80% of the maternal level. However, the administration of this drug is not without risk. It is known to possess certain negative side effects, and its pharmacological profile and maternal and fetal health risks have not yet been fully investigated. At present, no entirely safe and efficient treatment for fetal cardiac arrhythmia has been found.
The "limb body wall complex" or LBWC is a rare polymalformative syndrome. Two distinct phenotypes of which were recently described: one form with "placento-cranial" adhesion and the other with "placento-abdominal" adhesion. Coelosomia is found in all cases, it variably coexists with encephalic, vertebral, visceral or limb anomalies. Three pathogenesis are proposed: the "early amnion rupture" theory and the "vascular" theory can explain the form with "placento-cranial" adhesion, conversely, the "defective folding process" allows for a better explanation of the form with "placento-cranial" adhesion. These theories do not exclude each other if we accept that this syndrome could be split: the first form would then be the original "LBWC" et could belong to "amniotic band syndrome", the mechanism of which is not precisely known, whereas the second could be named "body stalk syndrome" - "syndrome du cordon court". In any case, this syndrome should be better known so as to be studied and give a prognosis for a later pregnancy, because there is no recurrence.
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