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Seven cases with the pathologic/autopsy diagnosis of caudal regression or sirenomelia in which antenatal sonography had been performed were reviewed. The three patients with caudal regression had similar findings on antenatal sonogram, including normal or increased amniotic fluid, mild dilation or normal urinary systems, nonfused extremities, and sacral agenesis. In the four patients with sirenomelia, common sonographic findings included marked oligohydramnios, suspected renal agenesis, and sacral agenesis. A history of maternal diabetes was elicited in all patients with caudal regression and in none of the patients with sirenomelia. Findings confirm recent articles in pediatric pathology suggesting that caudal regression is a separate entity, distinct from sirenomelia.
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PMID:Caudal regression versus sirenomelia: sonographic clues. 851 29

Transient neonatal diabetes mellitus is an uncommon disorder. Macroglossia in association with transient neonatal diabetes mellitus has been reported only twice before. We report the case of a 21-day-old male infant referred from a peripheral hospital for management of hyperglycemia. The mother was a 21-year-old primigravid in good health. There was no history of diabetes or drug or alcohol exposure. The pregnancy was complicated by intrauterine growth retardation and oligohydramnios from 30 weeks' gestation and the birth weight at 38 weeks' gestation was only 1480 gm. Physical examination revealed dysmorphic features and asymmetric growth retardation. The admission weight (1840 gm) and length (40.5 cm) were 5 SDs less than the mean and head circumference (32.5 cm) was 1 SD less than the mean. Dysmorphic features included macroglossia, large fontanelles, hypospadias, umbilical hernia, and bilateral inguinal hernias. Hyperglycemia had been noted on day 1 of life with an initial blood glucose value of 16 mmol/L (288 mg/dl). Despite treatment with regular insulin blood glucose control continued to be erratic. Therefore a regimen of daily NPH insulin was begun, which has a smoother action. Interestingly, from day 41 to day 47 the infant did not receive insulin and a crude control of the blood glucose was demonstrated. Peak levels of blood glucose in excess of 20 mmol/L (360 mg/dl) were followed by drops to levels less than 2 mmol/L (36 mg/dl) without insulin administration. This abnormal pattern of glucose control may represent poorly regulated release of endogenous insulin. However, because of unsatisfactory glucose levels administration of daily NPH insulin was reintroduced. The infant was discharged from the hospital on day 50 and administration of insulin was discontinued uneventfully at 9 months. At 1 year the hemoglobin A1c level was still normal and the infant's weight was at the 10th percentile. Macroglossia was less pronounced. Development showed mild delay in gross motor milestones.
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PMID:Transient neonatal diabetes mellitus and macroglossia. 886

The aim of this study was to test the hypothesis that isolated oligohydramnios in the otherwise normal term pregnancy does not indicate fetal compromise. Women undergoing labor induction for isolated oligohydramnios between 37 and 41-6/7 weeks gestation were matched by gestational age and parity to women with normal amniotic fluid index measurements who presented in spontaneous labor. Pregnancies complicated by hypertension, diabetes, fetal anomalies, or suspected fetal growth restriction were excluded. The primary outcome variable was route of delivery. Secondary outcomes examined included presence of meconium, acidosis, low Apgar score, and NICU admission. A total of 183 women underwent induction for isolated oligohydramnios. When compared to the control group, neonatal outcome measures did not differ in the group induced for oligohydramnios. However, the women who were induced had significantly more cesarean deliveries (15.8% vs. 6.6%, P < 0.01, odds ratio 2.7). The increased need for operative delivery was not attributable to more fetal distress in the oligohydramnios group. We conclude that isolated oligohydramnios in the otherwise normal term pregnancy may not be a marker for fetal compromise, and induction of labor may not be warranted in most cases.
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PMID:Isolated oligohydramnios in the term pregnancy: is it a clinical entity? 973 Apr 87

Aside from recognized overgrowth syndromes, instances of visceromegaly are not uncommon at perinatal autopsy. The database of the University of Michigan Teratology Unit was screened for individual viscera exceeding the 90th centile for body and brain weight standards. The data were stratified for several maternal (hypertension, diabetes, obesity), gestational (chorioamnionitis, oligohydramnios, amniorrhaea, polyhydramnios), and fetal (body wall defect, cardiac malformation, renal malformation, diaphragmatic hernia, nonimmune hydrops, twin transfusion syndrome) characteristics and tested for statistically significant excessive numbers of heavy organs. The most striking associations were heavy adrenal glands and liver with chorioamnionitis, heavy heart with polyhydramnios and in the twin transfusion syndrome, and heavy heart and liver with nonimmune hydrops. Excessive brain weight for body weight had a number of correlations, each most likely reflecting growth restriction with sparing of brain growth.
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PMID:Correlates of prenatal visceromegaly. 978 3

Preterm birth is the leading cause of perinatal morbidity and mortality, while preterm labor and delivery in diabetes mellitus (DM) carries an increased risk of perinatal complications. We investigated the hypothesis that DM (gestational and pregestational) is an independent risk factor for preterm birth and evaluated the hypothesis that the risk factors for preterm birth in diabetics are different from those in non-diabetics. The study population consisted of all singleton deliveries at this hospital between 1990-1997. Excluded were those of mothers who had not had prenatal care, or who had only partial care or multiple gestations. There were 3 subgroups: 834 women with pregestational DM, 3,841 with gestational DM, and 66,253 non-diabetics. The combined spontaneous and induced preterm delivery rate was determined in each subgroup. Potential risk factors for spontaneous preterm deliveries were assessed by a univariate model. A logistic regression model was used to assess the unique contribution of DM (gestational and pregestational) to preterm delivery in the presence of the other risk factors, and to compare risk factors for preterm delivery between subgroups. The prevalence of spontaneous preterm delivery was: 7.1% in non-diabetics, 10.0% in those with gestational DM and 25.5% in those with pregestational DM. When adjusted by a multivariate model for other risk factors for preterm delivery, DM still remained an independent risk factor for spontaneous preterm delivery (gestational DM: odds ratio 1.28, 95% CI: 1.1-1.48; pregestational diabetes: odds ratio 3.4, 95% CI: 2.65-4.36). The main difference in risk factors for preterm birth between the 3 subgroups was the amount of amniotic fluid. Polyhydramnios was an independent risk factor for preterm delivery in non-diabetics and in pregestational DM, but not in gestational DM. On the other hand, oligohydramnios was associated with a higher risk for preterm delivery only in gestational DM compared to non-diabetics. DM (gestational and pregestational) is an independent risk factor for spontaneous preterm delivery. Polyhydramnios is an independent risk factor for preterm delivery in pregestational but not in gestational DM. Oligohydramnios is a greater risk factor for preterm delivery in gestational DM compared to non-diabetics.
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PMID:[Premature delivery in diabetes: etiology and risk factors]. 1097 99

The obstetrical and neonatal courses in pregnancies following orthotopic liver transplantation were studied. Maternal and neonatal records were reviewed from six patients (eight pregnancies), cared for by a single practitioner, who had undergone orthotopic liver transplantation prior to pregnancy between 1984 and 1999. Demographic data, reason for transplantation, interval from transplantation to pregnancy, immunosuppressive agents, antepartum complications, and maternal and neonatal outcomes were reviewed. Many reasons for transplantation were noted, and no acute graft rejection occurred. Maternal complications noted were mild renal insufficiency, chronic hypertension, pregestational diabetes, and erythema nodosum. Antepartum complications included oligohydramnios, preterm labor, premature rupture of membranes, severe preeclampsia, fetal growth restriction, multiple congenital anomalies, and intra-amniotic infection. There was one miscarriage at 8 weeks, one previable and one periviable delivery, and the remainder delivered after 34 weeks. In our cohort of patients, once fetal viability was achieved, patients with a prior liver transplant had reasonable maternal and neonatal outcomes.
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PMID:Obstetrical and neonatal outcome in pregnancies after liver transplantation. 1114 11

Prevalence rates of birth defects in the Federal Republic of Germany are informative to assess the general background risk of having a child with a birth defect. They provide basic figures to determine temporal and regional prevalence trends, to evaluate and initiate preventive measures and to initiate research projects. To avoid observer, definition and collection bias, active monitoring systems are required. Data collected in the active monitoring system of the Mainz Birth Defects Registry are presented. From 1990-1998, 30,940 live-births, stillbirths and abortions underwent standardized physical and sonographic examinations. Anamnestic data were collected from prenatal care records, maternity files and hospital records. Major malformations were diagnosed in 2144 (6.9%) and mild errors of morphogenesis in 11,104 (35.8%) of all infants. Risk factors associated with the occurrence of major malformations were identified by comparing anamnestic data from infants with and without major malformations. Using multivariate regression models, statistically significant associations were established for 9 risk factors. Causally related risk factors were parents or siblings with malformations, parental consanguinity, more than 3 minor errors of morphogenesis in the proband, maternal diabetes mellitus and ingestion of antiallergic drugs in the first trimester of pregnancy. Conjunctional risk factors were polyhydramnios, oligohydramnios and gestational age <32 weeks at birth. Using these risk factors, populations at risk for the occurrence of major malformation can be identified.
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PMID:Malformations in newborn: results based on 30,940 infants and fetuses from the Mainz congenital birth defect monitoring system (1990-1998). 1219 58

Increased lipid peroxidation (LPO) and reduced antioxidant activity may contribute to the development of complications in pregnancy. The present study discusses the possibility of LPO and antioxidant activity in both maternal and umbilical cord blood as an indicator of oxygen radical activity. For this aim, pregnancies with hypertension and pre-eclampsia, diabetes mellitus (insulin dependent diabetes mellitus and gestational diabetes mellitus), oligohydramnios and abruptio placentae, as well as a healthy control group, were subjected in the present study. Simultaneous determination of glutathione S-transferase (GST), selenium dependent glutathione peroxidase (Se-GPx), catalase (CAT) activities and thiobarbituric acid reactive-substances (TBARs) levels were carried out in maternal erythrocyte and plasma in the antenatal period (in the third trimester) and immediately after the delivery. The same oxidative stress-related parameters were determined in umbilical cord blood as well. Erythrocyte GST activity was significantly increased in insulin-dependent diabetic pregnancy (IDDP) when compared to the control (P<0.05). Erythrocyte Se-GPx activity was found to be significantly increased in hypertensive preeclamptic pregnancy (HPP) (P<0.05) and in IDDP (P<0.05). Alterations in enzyme activities were accompanied by a simultaneous significant increase in the levels of TBARs in plasma samples of HPP (P<0.05), and IDDP (P<0.05). Enzyme activities were found to be significantly lower in cord blood samples than the maternal values, except GST. This enzyme represents about two- to threefold higher activity than those of the maternal activity in uncomplicated and complicated groups. Cord blood erythrocyte and plasma Se-GPx and CAT activities were decreased significantly in the HPP group when compared to the maternal value (P<0.05). Cord blood erythrocyte CAT activity was significantly decreased in the HPP group compared to the control (P<0.05). Cord blood TBARs levels were significantly lower than the before deliveries maternal value in the HPP group (P<0.05). No difference was detected between umbilical cord blood and maternal blood TBARs levels after delivery. The results of the present study suggest that oxidative stress and subsequent lipid peroxidation accompany the complications of hypertension, preeclampsia and diabetes mellitus in pregnancy. Maternal erythrocyte GST activity seems to be a sensitive indicator of oxidative stress in IDDP before delivery. The same enzyme can be used in cord blood as a biomarker of oxidative stress upon a sudden increase in oxygenation during delivery. These multiparameter biomarkers can also be used in monitoring the efficiency of antioxidant supplementation in complicated pregnant women, as has recently been suggested for diabetic and preeclamptic pregnancies.
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PMID:Circulating biomarkers of oxidative stress in complicated pregnancies. 1259 16

In twin pregnancies, the use of beta-adrenergics is associated with a significantly higher incidence of cardiovascular complications, and calcium channel blockers as well as oxytocin antagonists currently appear as first line agents. After extreme preterm delivery of the first twin and in selected patients, the birth of second twin may be delayed with a mean gain of 10-50 days. In cases of symptomatic placenta previa with mild-to-moderate bleeding, tocolytic agents may be associated with a prolongation of pregnancy and increased birth weight without significant impact on frequency or severity of bleeding. Calcium channel blockers are the drugs of choice in the event of diabetes. Indomethacin is a potent tocolytic, in particular in patients with polyhydramnios. However, it may cause oligohydramnios, premature closure of the ductus arteriosus and intrauterine fetal death when high doses are administered for a duration exceeding 48 to 72 hours, particularly beyond 32 weeks' gestation. The neonatal complications of indomethacin occur frequently. Tocolysis appears to reduce the failure rate of external cephalic version at term.
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PMID:[The therapeutic role of tocolysis]. 1496 18

Quantitative and qualitative alterations of the amniotic fluid complicate 7% of the pregnancies. Polyhydramnios complicates 1-3% while oligohydramnios involves 3-5% of the pregnancies. The most common causes of polyhydramnios are fetal abnormalities, maternal diabetes and twin pregnancies, but are idiopathic in the 60%. Perinatal mortality has been reported to range between 10-30% while the risk of preterm birth reaches up to 22% in pregnancies complicated by polyhydramnios. The neonatal outcome, in cases where polyhydramnios is due to fetal-neonatal abnormalities, depends on the underlying pathology. Polyhydramnios due to defects in intestinal canalisation in particular, has been correlated to good neonatal prognosis. In our experience no early postoperative deaths occurred in a group of 16 newborns consequtively admitted to our unit in the last two years, with abnormalities of the gastrointestinal tract with need of surgery within the second week of life. Most cases of oligohydramnios are due to premature rupture of membranes, other causes are fetal abnormalities, such as urinary tract malformations, or chromosomopaties and drugs e.g. NSAID's. Oligohydramnios of mild entities is often associated to preterm birth, fetal growth restriction. In some cases of oligohydramnios, neonatal survival is highly conditioned by pulmonary hypoplasia which develops with rates that range between 13 and 21%. Neonatal prognosis is often disastrous in cases with severe oligohydramnios, which however could be improved by amnioinfusion, which restores an amniotic fluid volume sufficient in reducing the adverse environmental effects and in prolonging, where possible, pregnancy. Beside the quantity also the quality of the amniotic fluid may be related to the neonatal outcome. Finding of some inflammatory factors (interleukines) in the amniotic fluid seems to be significantly correlated to periventricular leucomalacia (PVL), cerebral paralysis and long-term neurological abnormalities, both in the preterm and term neonate. Therefore, increase of the cytokines in the amniotic fluid could give information not only of the infection but also regarding the risk of developing neurological sequelae in neonatal period. Diagnosis and therapy for pathologies that alter the amniotic fluid have progressed, however efforts have still to be made in the identification and search for those quantitative-qualitative alterations of the amniotic fluid, for their potential implications on neonatal outcome.
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PMID:Alteration of the amniotic fluid and neonatal outcome. 1530 Dec 96


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