Uoamines A and B, piperidine alkaloids from the ascidian Aplidium uouo.

A specimen of the ascidian Aplidium uouo from Maui contained two piperidine alkaloids, uoamines A and B, that differed only in the geometry of a 3-thiomethylacrylate ester group. The alkaloids exhibit conformational mobility in the NMR time frame, which complicated the elucidation of their structures by interpretation of spectroscopic data.

Confined placental mosaicism for trisomy 8 and intra-uterine growth retardation.

This report describes a case of apparent confined placental mosaicism for trisomy 8 in a pregnancy which produced a male infant with intra-uterine growth retardation. Postnatal cytogenetic and molecular studies were consistent with biparental disomy 8. Postnatally, the infant experienced a period of rapid catch-up growth and exhibited no clinical features of trisomy 8 mosaicism. His development was age appropriate.

Aneurysm of the vein of Galen: prenatal diagnosis and perinatal management.

An aneurysm of the vein of Galen is a rare arteriovenous malformation of the central nervous system. Fetal manifestations have included nonimmune hydrops, hydrocephalus, and intracranial hemorrhage. This anomaly may be diagnosed prenatally by several imaging modalities. A cystic cranial mass was identified by ultrasound in a fetus at 30 weeks gestation. Both pulsed-wave Doppler and color-velocity imaging studies suggested aneurysm of the vein of Galen was the most likely diagnosis. The fetus demonstrated no evidence of hydrops on serial ultrasound examinations. A 2430 g female infant was delivered vaginally at 35 weeks gestation. Postnatal management included transarterial embolization of the vessels feeding the aneurysm with craniectomy, an intra-aneurysmal balloon, and vascular microcoils. Hydrocephalus developed and a ventriculo-peritoneal shunt was placed. The infant has grown appropriately in the first year of life. An aneurysm of the vein of Galen may be diagnosed prenatally by real-time ultrasound, pulsed-wave Doppler, color-velocity imaging, or magnetic resonance imaging. The presence of this malformation should prompt close follow-up for the remainder of the pregnancy. Careful obstetric management and early postnatal intervention may lead to a favorable outcome.

Transumbilical use of the endovaginal probe.

The endovaginal probe can be used in the umbilicus to improve fetal imaging. In 27 of 31 patients, an optimal ultrasound examination was achieved because of this added technique. The procedure is most useful in obese women and those with prior abdominal surgery.

Fatal lupus pleuritis presenting in pregnancy. A case report.

BACKGROUND: Lupus, one of the most common autoimmune diseases in pregnancy, may involve multiple organ systems with varying severity. The diagnosis and treatment of the disease may be complicated by the physiologic changes of pregnancy. CASE: A 22-year-old woman presented at 29 weeks' gestation with a 4-week history of dyspnea and mild hypertension. She was found to have large bilateral pleural effusions. Her antinuclear antibody level was 1:640, with a speckled pattern, and her complement levels were low. Her urine had 2+ hemoglobin and 2+ protein with hyaline casts. Over the next three days, respiratory compromise increased despite high-dose steroids. A cesarean delivery was performed for fetal compromise. The infant did well after moderate respiratory distress. The mother developed worsening respiratory distress with adult respiratory distress syndrome, and she could not be weaned from the ventilator. Bilateral chest tubes were placed to control her effusions. Urine output remained poor despite pressors and diuretics. Staphylococcal sepsis occurred on postoperative day 5 and precluded our use of other antiinflammatory agents. Over the next 14 days the mother developed seizures, hypotension and eventual respiratory collapse. Autopsy was notable for lupus nephritis and serositis. CONCLUSION: The diagnosis and management of systemic lupus in pregnancy may be extremely difficult. Serositis and nephritis may lead to maternal compromise despite early diagnosis and treatment.

Fetal ovarian torsion appearing as a solid abdominal mass.

Fetal and neonatal ovarian cysts can be small asymptomatic lucencies or large symptomatic echocomplex masses that can cause intestinal obstruction and peritonitis. Solid masses are the most rare form of ovarian cysts. A fetus, anatomically unremarkable at 16 weeks' development, was found at 35 weeks to have a 5 x 5 cm solid mass. After delivery a laparotomy was performed, and a torsed ovary measuring 8 x 7 cm was removed. The infant did well after the procedure. Over 400 cases of perinatal ovarian cysts have been reported in the literature. Only 1% of these are purely echodense. When a fetal abdominal mass is present, separate from bowels and kidneys, a torsed ovary should be considered in the differential diagnosis. Management of the pregnancy and infant may be guided by the fetus or neonate's symptoms.

Endoscopic management of biliary disease during pregnancy.

BACKGROUND: The transient but substantial alterations in the biliary system during pregnancy increase the risk of cholecystitis, choledocholithiasis, and pancreatitis. Traditionally, these disorders are managed conservatively or with operative cholecystectomy. Recent advances in fiber-optic technology allow endoscopy to be used as a safe, effective, and definitive treatment alternative for pancreaticobiliary disease in pregnancy. CASES: Three gravid women with cholelithiasis, acute cholecystitis, and/or gallstone pancreatitis were treated endoscopically with stone extraction and experienced rapid resolution of symptoms and successful pregnancy outcomes. CONCLUSION: Aggressive endoscopic intervention for biliary disorders in pregnancy appears to decrease morbidity, mortality, and costs. Endoscopy offers a safer, more effective treatment alternative to prolonged medical management and traditional surgical intervention during pregnancy. Because biliary abnormalities resolve rapidly in the postpartum period, it is unlikely these women will need subsequent treatment.

An association between fetal parvovirus B19 infection and fetal anomalies: a report of two cases.

The association between fetal parvovirus B19 infection and hydrops was first reported in 1984. The virus has a predilection for the erythroid cell line, which in the fetus may produce anemia. Recent cases of parvovirus infection in other fetal cell lines have raised concern that the infection may induce fetal anomalies in rare cases. We report two pregnancies complicated by parvovirus B19 infection. In each instance the patient had normal second trimester ultrasounds but subsequently developed fetal abnormalities--disruptions of normal structure. One infant has myocardial infarction, splenic calcifications, and mild hydrocephalus. The other had moderate hydrocephalus with central nervous system scarring. There are two possible mechanisms in which parvovirus may induce fetal anomalies. Both direct infection of fetal organs and vascular inflammation have been documented in association with B19 parvovirus. Although fetal abnormalities associated with parvovirus are rare, continued study of this organism may indicate a greater pathologic potential than is now thought.

Alpha 1-antitrypsin deficiency and pregnancy.

Alpha1-antitrypsin deficiency is an inherited pulmonary disorder which results from a deficiency of a major plasma protease inhibitor. The onset and severity of symptoms vary widely and depend on the genotype and whether the patient smokes cigarettes. Alpha1-antitrypsin in pregnancy has only been previously reported twice. Our patient had a functional serum alpha1-antitrypsin level which was 15% of normal but was clinically asymptomatic and she did not smoke. Her genotype revealed a non-ZZ pattern. Her obstetric history was complicated by preterm labor in each of her five ongoing pregnancies. Alpha1-antitrypsin deficiency is inherited via two codominant autosomal genes. Although there is great variability in severity of disease, seriously affected patients may have emphysema and hepatic abnormalities. Patients with non-ZZ genotypes or who are heterozygotes may have favorable pregnancy outcomes.

Effects of low-dose aspirin therapy on plasma levels of polyunsaturated fatty acids: a preliminary investigation.

Polyunsaturated fatty acids play an important yet poorly understood role in pregnancy complications. We are interested in the effects of aspirin therapy on the metabolism of these compounds. To determine the effects of low-dose aspirin on plasma levels of polyunsaturated fatty acid precursors, we assayed linoleic, linolenic, arachidonic, eicosapentaenoic, and docosahexaenoic acids using high-performance liquid chromatography. Seventeen pregnant women being treated with aspirin therapy (81 mg/day) were evaluated between 9 and 37 weeks' gestation. Blood was drawn before initiation of aspirin therapy and after 3 or 4 days, and again after 3 or 4 weeks of therapy. We found no significant change in the plasma levels of fatty acids during aspirin therapy at either 3 or 4 days or 3 or 4 weeks compared with baseline. In this group of women with preexisting disease, low-dose aspirin does not appear to change the plasma levels of polyunsaturated fatty acid precursors of eicosanoids. Data on pregnant women without hypertensive disorders is needed to help in understanding the role and physiology of these important compounds.

Pregnancy complicated by Guillain-Barré syndrome.

We report a case of a pregnant patient with Guillain-Barré syndrome. Her symptoms, including weakness, vague numbness, urinary incontinence, and unsteady gait, were initially mild and mimicked many of the changes seen in pregnancy. Her progressive weakness was not preceded by any known illness. Evaluation of symptoms revealed an elevated cytomegalovirus IgM titer. When symptoms became severe, electromyography was done, showing a severe demyelinating motor-sensory neuropathy. After plasma exchange and vaginal delivery, symptoms resolved. Neither the patient nor her infant have had any long-term sequelae. Guillain-Barré syndrome may be difficult to diagnose in pregnancy but may be effectively treated with plasmapheresis.

Successful treatment of a cervical pregnancy with a single low dose methotrexate regimen.

Until very recently cervical pregnancies have been treated with surgery, usually hysterectomy. The development of endovaginal ultrasound, which allows early diagnosis, and methotrexate chemotherapy have opened up new therapeutic options. A 45-year-old multigravida presented at 8 weeks' gestation with vaginal bleeding. Endovaginal ultrasound demonstrated a cervical pregnancy with a fetal pole, 1.2 x 1.4 cm sac, no cardiac pulsations, and an empty uterus. After discussion with the patient, single low dose methotrexate 1.5 mg/m2 was given intramuscularly. The patient's hCG titre was 5882 IU-(Third International Standard). Over a 5-week period the hCG titres fell, and the gestational sac disappeared. The patient experienced intermittent vaginal bleeding and cramping but was managed as an outpatient. Single low dose methotrexate may be a successful management option in selected cases of cervical pregnancy.

Bacterial vaginosis in pregnancy: an approach for the 1990s.

It is proposed that clinicians screen all pregnant women at high risk for preterm labor and premature rupture of membranes for bacterial vaginosis, and treat all women when it is diagnosed. This infection is associated with a two to three times increase in preterm labor and delivery, premature rupture of the membranes, and endometritis. Although cause and effect have not been conclusively documented, these associations must be considered in the practice of obstetrics at the present. The paucity of vaginal Lactobacillus spp is pivotal in allowing overgrowth of many other organisms of the vagina. Screening is suggested because 50 percent of bacterial vaginosis is asymptomatic. The diagnosis, which is rapidly made and inexpensive, remains defined by clue cells seen on wet prep, high vaginal pH, and amine odor of the vaginal discharge. Optimal treatment of pregnant women with bacterial vaginosis is via oral or intravaginal metronidazole or clindamycin.

Prenatal diagnosis and management of massive bilateral axillary cystic lymphangioma.

BACKGROUND: Fetal lymphangiomas can occur in many different anatomic locations, including the most commonly seen nuchal cystic hygroma. CASE: A fetus at 18 weeks' gestation was found to have a massive right axillary hygroma. The fetal karyotype was normal. Serial ultrasound examinations indicated progressive enlargement, but no hydrops. At 32 weeks' gestation, a left axillary hygroma was also diagnosed. The patient underwent cesarean delivery. CONCLUSION: Prenatal diagnosis of nuchal cystic hygromas has a high association with karyotypic abnormalities, hydrops, and fetal demise; however, this association may not apply to cystic lymphangiomas at other locations.

Combined abdominal-perineal sonography to assist in diagnosis of transverse vaginal septum.

BACKGROUND: Transperineal sonography has been used to detect incompetent cervices, placenta previa, cervical pregnancy, and vaginal and cervical atresia. CASE: Transperineal sonography was used to investigate primary amenorrhea in a 14-year-old girl. A mid-transverse septum was found and confirmed surgically. CONCLUSION: Transperineal or translabial sonography assisted in the differentiation of primary amenorrhea, when adequate vaginal access was prohibited.

Pelvic magnetic resonance imaging in the evaluation of uterine torsion.

BACKGROUND: Uterine torsion is defined as the rotation of more than 45 degrees around the long axis of the uterus. An uncommon but potentially fatal event, uterine torsion is rarely diagnosed until the time of surgery. With magnetic resonance imaging (MRI), however, an accurate diagnosis of uterine torsion may now be made preoperatively. CASE: We describe a patient with uterine torsion in whom the correct diagnosis was made prenatally with the use of MRI, by the demonstration of an X-shaped configuration of the upper vagina. CONCLUSION: Distinctive features suggestive of uterine torsion were demonstrated by MRI and enabled an accurate preoperative diagnosis. To our knowledge, this is the first reported case of uterine torsion diagnosed on MRI.

Non-immune hydrops after 20 weeks' gestation: review of 10 years' experience with suggestions for management.

OBJECTIVE: To analyze the etiologies and outcomes for a southeastern section of the United States, and to organize an efficient approach to evaluation. METHODS: We reviewed 82 cases of non-immune hydrops presenting after 20 weeks' gestation over a 10-year period. RESULTS: Overall perinatal mortality was 86.6%. Fetuses diagnosed with hydrops before 24 weeks' gestation had a perinatal mortality of 95%, with nearly one-third having abnormal karyotypes. The etiology of hydrops diagnosed after 24 weeks' gestation was more likely to remain idiopathic or to be related to cardiothoracic abnormalities. CONCLUSION: Before 24 weeks' gestation, the high risk of mortality and abnormal karyotype justifies offering families funipuncture in the hope of finding a treatable cause of non-immune hydrops. After 24 weeks' gestation, when fewer abnormal karyotypes are found, funipuncture may also be pivotal in diagnosing the cause of non-immune hydrops.

Outcome of pregnancies with elevation of both maternal serum alpha-fetoprotein and human chorionic gonadotropin.

We evaluated the pregnancy outcome of all patients with elevations of both maternal serum alpha-fetoprotein and human chorionic gonadotropin since institution of combined serum screening at our program. After analysis of 34,404 samples, 99 patients were found to have significant elevations of both maternal serum alpha-fetoprotein and human chorionic gonadotropin. The ultrasound findings, amniocentesis results, and pregnancy outcomes were determined in each case. Sixty-six patients with singleton gestations met entry criteria. Pregnancy outcome information is available for 63 of these patients, 60% of whom had at least one complication. Thirty-three patients with multiple gestations met inclusion criteria. Pregnancy outcome information is available for 31 of these, 81% of whom had at least one complication. These patients had a high incidence of pregnancy related complications. This group would appear to be at higher risk than women with elevation of either maternal serum alpha-fetoprotein or human chorionic gonadotropin alone.

Prenatal diagnosis of renal agenesis in a twin gestation.

Bilateral renal agenesis is a lethal congenital anomaly. It appears to be transmitted in a polygenic pattern. The prenatal ultrasound findings consist of severe oligohydramnios, absence of the fetal bladder, and failure to identify fetal kidneys. Twin gestations with renal agenesis have been described in the paediatric literature. We detail a case of a patient with two prior affected pregnancies with bilateral renal agenesis. Her latest pregnancy was diagnosed prenatally, with one fetus with bilateral and the other fetus with unilateral renal agenesis. The ultrasound findings should be differentiated from the stuck twin phenomenon.

A fetus with sirenomelia, omphalocele, and meningomyelocele, but normal kidneys.

Sirenomelia, which has been reported for more than 450 years, is the most severe form of caudal dysplasia. This disorder is classically associated with agenesis or severe dysplasia of the urinary tract. A 27-year-old gravida 3 para 0020 presented at 19 weeks' gestation with monoamniotic twins, one normal and one with sirenomelia. The pregnancy miscarried following invasive diagnostic testing. Autopsy confirmed the findings of sirenomelia, meningomyelocele, omphalocele, persistent cloaca, and two normal kidneys. The abnormalities accompanying sirenomelia appear as a spectrum. The pathogenic theories of sirenomelia are reviewed in light of this case with normal kidneys.