Hereditary antithrombin III deficiency and pregnancy: report of two cases and review of the literature.

The pregnancy and the serum antithrombin III levels during the antenatal and postpartum period of two patients with hereditary antithrombin III deficiency is described. Both antithrombin III antigen and activity levels dropped to their lowest levels immediately after delivery. A review of the literature emphasizes the high risk for thromboembolism in patients with hereditary antithrombin III deficiency. Important considerations for the obstetrician concerning hereditary antithrombin III deficiency are discussed, including: 1) the need to therapeutically anticoagulate these patients postpartum, 2) the need to consider prophylactic anticoagulation throughout pregnancy especially in patients with a history of thrombosis, 3) the practical aspects of assaying antithrombin III in plasma rather than serum, 4) the normally low antithrombin III levels in normal newborns, and 5) the need to provide prepregnancy counseling, including information about the autosomal dominant inheritance of hereditary antithrombin III deficiency.

Antepartum prediction of the postmature infant.

A prospective investigation was undertaken to determine whether present antepartum methods of fetal assessment were useful in predicting postmaturity. Thirty-two (22%) of 147 strictly defined postdate pregnancies produced infants with signs of postmaturity. Clinical findings, fetal heart rate testing (primarily nonstress tests), and fetal movement charting were not found to be reliable predictors. Single-voiding estrogen:creatinine (E:C) ratios were significantly (P less than .0001) lower in fetuses with subsequent findings of postmaturity than in those without such signs, and all subnormal values were associated with postmature infants. Twenty-four of 29 pregnancies with oligohydramnios diagnosed by ultrasonography produced postmature infants, whereas 110 of 118 pregnancies with either pockets or an adequate volume of amniotic fluid produced infants who were not postmature. Of the fetal surveillance methods used in the authors' clinic, subnormal E:C ratios and ultrasonic evidence of oligohydramnios were the most reliable predictors of postmaturity.

Association of prolonged, preterm premature rupture of the membranes and abruptio placentae.

Nine patients with prolonged, preterm premature rupture of the membranes (PROM) had associated abruptio placentae confirmed at delivery. Antepartum vaginal bleeding during the course of expectant management was the most common feature in the clinical course of the patients who developed abruptio placentae. Other clinical and laboratory findings suggesting the diagnosis were not present consistently. Retrospective analysis of all patients with prolonged, preterm PROM led to an estimated risk of 4% for the development of abruptio placentae during the course of expectant management of such patients. This risk estimate is three to eight times greater than that for the development of abruptio placentae in pregnancy generally. A high index of suspicion for abruptio placentae is appropriate when patients with prolonged, preterm PROM develop vaginal bleeding or any other suggestive clinical signs or symptoms of abruptio placentae during the course of expectant management.

Diagnosis of fetal death in utero with amniotic fluid creatine kinase.

There is a need for a widely available and accurate method of diagnosing fetal death in utero. The accepted standard technique for making this diagnosis is real-time ultrasonography, but this procedure is not available at all hospitals. However, almost every hospital laboratory has the capability of measuring creatine kinase in body fluids. One hundred eight pregnant women underwent real-time ultrasonography and analysis of amniotic fluid for creatine kinase. Ninety-one women with conditions that required amniocentesis during the second and third trimesters had a life fetus by ultrasonographic examination and a low level of amniotic fluid creatine kinase (0 to 3 sigma units per milliliter). Seventeen women with a dead fetus by ultrasonographic examination had an abnormally high level of amniotic fluid creatine kinase (5 to 9,800 sigma units per milliliter). The only erroneous diagnosis in this series involved a set of twins, one of whom was shown by real-time ultrasonography to be alive and the other dead. Both sacs contained abnormally high, although different, levels of creatine kinase. Analysis of creatine kinase in amniotic fluid is a reliable technique for diagnosing fetal death.

Urinary undiversion and pregnancy.

The case reports of two pregnant patients with myelodysplasia who had previously undergone ileocecal cystoplasty are presented. Obstetric and urologic complications of urinary diversion with or without subsequent undiversion are discussed.

Hyperlactacidemia associated with acute ritodrine infusion.

The use of beta-mimetic tocolytics has been associated with various metabolic derangements which have been the topic of investigational reports. Lactic acidosis has been reported to follow concomitant steroid administration. The purpose of this investigation was to delineate more clearly hyperlactacidemia in patients receiving various tocolytics with and without concomitant use of steroids in a prospective manner. Twenty patients in premature labor who received ritodrine had markedly increased blood levels of lactate (baseline, 1.0 +/- 0.1 mmol/L [SEM] versus 3.5 +/- 0.3 mmol/L [SEM] after 6 hours' intravenous infusion). In the other treatment groups, ritodrine plus hydrocortisone, hydrocortisone alone, and magnesium sulfate alone, lactate levels failed to change significantly. Clinical relevance and implications for metabolic alterations associated with beta-mimetic tocolysis are discussed.

Gastrointestinal malformations associated with prune belly syndrome: three cases and a review of the literature.

Prune belly syndrome (PBS), a triad consisting of abdominal musculature hypoplasia, urinary tract malformations, and cryptorchidism, is frequently associated with other congenital malformations. Although it is acknowledged that gastrointestinal (GI) malrotation and mesenteric anomalies are frequent in PBS, other GI anomalies are generally considered to be exceedingly rare. Here we describe 3 autopsy cases with severe malformations of both midgut and hindgut derivatives and review the world literature to evaluate the spectrum of GI malformations associated with this syndrome. The relatively high frequency of distal stenoses and atresias suggests that the anomalous mesenteric attachments may predispose to prenatal volvulus and subsequent anatomic bowel obstruction. Postnatal volvulus is also occasionally observed. Infants with PBS also appear to be at a higher risk for persistence of the common fetal cloaca.

Antenatal diagnosis of sacral agenesis syndrome in a pregnancy complicated by diabetes mellitus.

The sacral agenesis syndrome is a severe congenital abnormality consisting of agenesis of the lumbar spine, sacrum, and coccyx, as well as hypoplasia of the lower extremities. It is considered the most characteristic of all congenital anomalies associated with maternal diabetes mellitus. We describe the sonographic and radiologic findings of agenesis of the lumbosacrococcygeal spine with lower limb and genital hypoplasia in the offspring of a woman with both diabetic retinopathy and nephropathy. The diagnosis was established at 25 weeks' gestation and was confirmed by radiologic evaluation of the neonate.

Capillary gas chromatographic-mass spectrometric profiles of trimethylsilyl derivatives of organic acids from amniotic fluids of different gestational age.

Amniotic fluid from different gestational age patients was partitioned into neutral, acidic and basic fractions. The organic acids were trimethylsilylated and analyzed by glass capillary gas chromatography-mass spectrometry. A marked difference in the level of hippuric acid was observed between samples from early (15-22 weeks) and late (30-38.5 weeks) pregnancy. This difference probably reflects the degree of maturity in the fetal liver and kidney. The procedures establish amniotic fluid profiles of substances of varying gestational age and should be useful in determining alterations caused by diseases.