Vascular control by infrarenal aortic cross-clamping in placenta accreta spectrum disorders: description of technique.

We describe a novel surgical technique in 31 women with histopathologically confirmed placenta accreta spectrum (PAS) disorders managed by a multidisciplinary team using a prophylactic infrarenal abdominal aortic cross-clamping technique during caesarean hysterectomy. We conclude that this new surgical procedure is a relatively safe technique to potentially control operative blood loss. Our work may stimulate others to develop protocols assessing this innovative technique to improve the surgical outcome of PAS disorders.

Acute respiratory distress syndrome in a pregnant woman with systemic lupus erythematosus: a case report.

When the disease activity of systemic lupus erythematosus (SLE) is controlled appropriately, a pregnant woman who has lupus is able to carry safely to term and deliver a healthy infant. While the physiology of a healthy pregnancy itself influences ventilatory function, acute pulmonary distress may decrease oxygenation and influence both mother and fetus. Though respiratory failure in pregnancy is relatively rare, it remains one of the leading conditions requiring intensive care unit admission in pregnancy and carries a high risk of maternal and fetal morbidity and mortality, not to mention the complexity caused by lupus flare. We report a case of SLE complicated with lupus pneumonitis and followed by acute respiratory distress during pregnancy. Though there is a high risk of maternal and fetal morbidity and mortality, maternal respiratory function improved after cesarean section and treatment of the underlying causes. The newborn had an extremely low birth weight but was well at discharge.

A young male adolescent with feminine appearance: diagnosis of 46, XX syndrome neglected for 4 years with gynaecomastia presentation.

Gynaecomastia is common in infancy and adolescent boys, but other inciting causes should be kept in mind and necessitate further evaluation should be conducted to determine any underlying conditions. A 22-year-old unmarried male adolescent visited our endocrinology clinic for feminine appearance despite operations for bilateral gynaecomastia 4 years ago. Physical examination showed inverted triangular distribution of pubic hair, sparse beard, small-sized testes, flaccid short penis and surgical scar of the chest wall. Serum hormones study revealed primary hypergonadotropic hypogonadism, and cytogenetic study disclosed female complement (46, XX). The authors recommend that sexual chromosome abnormality should be considered in patients with hypogonadism to avert androgen deficiency-related complications early and that long-term team care should be provided to improve the patient's health-related quality of life.

Visfatin-induced expression of inflammatory mediators in human endothelial cells through the NF-kappaB pathway.

BACKGROUND: Visfatin is an adipokine that is highly expressed in visceral fat. Plasma levels of visfatin have been reported to be higher in subjects with obesity and/or type 2 diabetes mellitus. However, the role of visfatin in endothelial dysfunction has been largely unexplored. OBJECTIVES: We investigated the possible pathogenic role of visfatin in endothelial dysfunction, particularly focusing on its effect on inflammatory mediators. DESIGN: Primary human umbilical vein endothelial cells (HUVECs) pretreated with visfatin (1, 10 and 50 ng ml(-1)) were used to study the relationship between visfatin and endothelium dysfunction. Expressions of adhesion molecules (ICAM-1, VCAM-1 and E-selectin) and cytokines (interleukin (IL)-6 and IL-8) affected by visfatin were investigated by enzyme-linked immunosorbent assay, flow cytometry and real-time PCR. Activity of nuclear factor (NF)-kappaB was examined by electrophoretic mobility shift assay. RESULTS: At a visfatin concentration of 50 ng ml(-1), significant increases in IL-6, IL-8, ICAM-1, VCAM-1 and E-selectin gene expression along with increased IL-6, IL-8 and sE-selectin protein levels in the conditioned medium were detected. Flow cytometry showed that the addition of visfatin significantly increased ICAM-1 expression and VCAM-1 expression (10 and 50 ng ml(-1), respectively). Electrophoretic mobility shift assay confirmed that visfatin increased the DNA-binding activity of NF-kappaB. In addition, pretreatment with visfatin (10 and 50 ng ml(-1)) increased human monocyte cell line THP-1 adhesion to HUVECs. CONCLUSIONS: Our findings suggest that visfatin causes endothelial dysfunction by increasing inflammatory and adhesion molecule expression at least partly through the upregulation of NF-kappaB activity.

Differential expression of vascular endothelial growth factor, placenta growth factor and their receptors in placentae from pregnancies complicated by placenta accreta.

Placenta accreta is a pregnancy complication characterized by the presence of life-threatening uteroplacental neovascularization. The factors involving its development are unknown. Vascular endothelial growth factor (VEGF), placenta growth factor (PlGF) and their receptors (VEGFR) have important roles in vascular remodeling. We have investigated the differential expression of these proteins in placentae from placenta accreta (cases) and normal placentation (controls). Immunohistochemically, the expression of VEGFR-2 in the syncytiotrophoblast was significantly lower in cases than in controls during both the second and third trimesters (P = 0.005 and 0.002, respectively). However, VEGFR-2 expression in the cytotrophoblastic and extravillous trophoblastic cells and VEGFR-1, -3 and Ki-67 in the trophoblast populations were not significantly different between controls and cases (P > 0.05). Ki-67 immunostaining also showed that endothelial cells of the larger vessels were stained weaker in normal placenta than in placenta accreta. The majority of VEGFR-2 expression, as demonstrated by Western blot or reverse transcription polymerase chain reaction, was consistent with the immunohistochemical findings in the syncytiotrophoblast. Furthermore, enzyme-linked immunosorbent assay in the placental lysates showed that the women with placenta accreta demonstrated significantly higher VEGF (P = 0.001) and lower soluble VEGFR-2 (P = 0.015) concentrations than did women with normal pregnancy. PlGF and soluble VEGFR-1 levels did not show any significance in study groups (P > 0.05). These observations suggest that the participation of up-regulated VEGF and down-regulated VEGFR-2 (both membrane-bound and soluble forms) may be associated with the development of placenta accreta.

Fetal echocardiographic diagnosis of isolated ductus arteriosus aneurysm: a longitudinal study from 32 weeks of gestation to term.

OBJECTIVE: To investigate the echocardiographic characteristics of isolated fetal ductus arteriosus aneurysm (DAA) and the factors influencing its development. METHODS: Fetal echocardiograms for 509 low-risk singleton pregnancies were performed longitudinally from 32 weeks of gestation. The ventricular outflow tracts and great vessels were visualized, focusing on changes in the ductus arteriosus (DA), and fetuses with DAA or ductus arteriosus dilatation (DAD) were identified. Prenatal and perinatal findings were compared between those infants diagnosed postnatally with and those without neonatal DAA. RESULTS: Forty-one of the 509 fetuses (8.1%) studied had neonatal DAA; 2.2% (11/509) had DAA and 5.9% (30/509) DAD before delivery. The mean gestational age at the time of diagnosis of fetal DAA/DAD was 36.9 +/- 1.7 weeks. The mean maximal internal diameter of DAA/DAD was 8.0 +/- 0.8 mm at initial diagnosis, increasing to 10.8 +/- 1.6 mm before delivery. All cases originated from the aortic end of the DA. Compared with fetuses without neonatal DAA, the affected cases had a markedly more curved DA prior to diagnosis, and higher peak velocities at the aortic end of the DA (P < 0.05). Newborns with fetal DAA/DAD had a larger placental weight to birth body weight (BBW) ratio and were less likely to have a BBW appropriate for gestational age (P < 0.05). All cases of fetal DAA/DAD regressed spontaneously. CONCLUSION: Isolated neonatal DAA is a continuity of fetal DAA/DAD. The development of fetal DAA/DAD is likely to be related to the higher peak velocities in the more markedly curved DA occurring in the latter part of the third trimester. Fetal DAA/DAD usually initiates at the aortic end of the DA, followed by progression towards the pulmonary end.

Fetal axillary hemangiolymphangioma with secondary intralesional bleeding: serial ultrasound findings.

A case of fetal axillary hemangiolymphangioma coexisting with intralesional hemorrhage is presented. At 27 weeks' gestation, the fetus was found to have a 52 x 43-mm left axillary multilocular cystic mass which showed no signals on color Doppler. The mass was composed mostly of sonolucent spaces. At 29 weeks' gestation, an arterial flow signal (15 cm/s) was detected within the mass. In addition, two low-density echogenic cystic spaces with bidirectional flow waveforms were found, which raised the suspicion of intratumoral bleeding. Two weeks later, a fine-needle aspiration of the mass revealed both straw-colored and chocolate-colored fluid. The tumor size increased from 52 x 43 mm at 27 weeks to 100 x 79 mm at 37 weeks. Blood clots developed gradually in the hemorrhagic spaces. The pregnancy proceeded smoothly to term and at 38 weeks an elective Cesarean section was performed. After a surgical excision of the mass at the age of 4 days, a mixed cavernous hemangioma and cystic lymphangioma with secondary intralesional hemorrhage was confirmed histopathologically.

Three-dimensional color power Doppler imaging in the assessment of uteroplacental neovascularization in placenta previa increta/percreta.

A case of placenta previa increta/percreta was diagnosed at 18 weeks' gestation with the 3-dimensional color power Doppler imaging technique. Unusually extensive uteroplacental vascular network architecture was seen on the 3-dimensional angiohistogram. After appropriate counseling, the patient chose to terminate the pregnancy. A hysterectomy was performed with prophylactic preoperative embolization of internal iliac arteries at 21 weeks' gestation, and histopathologic examination revealed placenta previa increta/percreta. This new 3-dimensional angiohistogram technique allowed us to visualize all 3 orthogonal planes of the angioarchitectural information. It appears to be a useful complementary tool and is likely to play a more defining and clarifying role in assessing the quantification of abnormal uteroplacental neovascularization for patients with placenta previa increta/percreta.

In utero sonographic diagnosis of a communicating enteric duplication cyst in a giant omphalocele.

Enteric duplications are rare lesions, and relatively few cases have been diagnosed prenatally. We present, to our knowledge, the first case of an associated communicating ileal duplication cyst in a huge omphalocele diagnosed prenatally. The prenatal ultrasound findings revealed four features of the cystic lesion including peristaltic movements of the cystic wall, communication between the cyst and normal bowel lumen, intra-cystic echogenic contents, and echogenic mesenteric tissue (fat) close to the cyst. These distinct characteristics helped us to make a firm in utero diagnosis.

Primary structure and developmental expression of zebrafish sodium channel Na(v)1.6 during neurogenesis.

A zebrafish sodium channel cDNA encoding a 1949-amino acid polypeptide, Na(v)1.6, was isolated. Two transcripts were detected in zebrafish adult brain but not in cardiac or skeletal muscle. The RNase protection analysis confirmed the neural specificity of zebrafish Na(v)1.6 24 hours postfertilization (hpf) Na(v)1.6 was expressed in the trigeminal ganglion, anterior and posterior lateral line ganglia, rhombomeres, and Rohon-Beard neurons. This preferential localization suggests that Na(v)1.6 plays an important role in tactile sensitivity. The abundance of zebrafish Na(v) 1.6 mRNA in the central and peripheral nervous systems increased markedly between 48 and 72 hpf, during the maturation of the nervous system.

Congenital complete heart block.

Congenital complete heart blocks (CCHB) are mostly related to the neonatal lupus syndrome. The purpose of this paper was to assess the clinical spectrum of CCHB in our hospital. Nine patients were retrospectively enrolled between 1994 and 1999. The birth history, electrocardiography, 24-hour Holter monitoring, pacemaker insertion and its complications, maternal disease, and maternal and infant autoantibody levels were studied. All nine cases were diagnosed prenatally. Hydrops fetalis was noted in five (55.6%). Six cases were live births and the other three were terminated. No anatomical heart defects were noted. Initial electrocardiography revealed the atrial rates ranged from 150 to 166 beats per minute. The minimal ventricular rates ranged from 46 to 80 beats per minute. VVI mode pacemakers were inserted through xyphoid approach in all live-birth infants. Complications were noted in three of them (50%). Antinuclear antibody and anti-SSA/Ro antibody were positive in all 8 mothers (100%). The anti-SSB/La antibody was positive in 6 of the eight mothers (75%). Five infants tested positive for anti-SSA/Ro antibody. None of the infants tested positive for anti-SSB/La antibody. In conclusion, all CCHBs in our series were associated with maternal autoantibodies. Because of high complication rate of permanent pacemaker insertion during the neonatal period, it should be restricted in certain conditions.

Predictive factors for residual disease in subsequent hysterectomy following conization for CIN III.

OBJECTIVE: The aim of this study was to determine predictive factors for post-cone residual disease in subsequent hysterectomy for CIN III. METHODS: From June 1994 to June 1999, 120 patients with CIN III who received hysterectomy within 6 months of conization regardless of marginal status were identified from 1450 conization cases. The demographic features and pathologic parameters were analyzed for the predictive rate of post-cone residual disease. RESULTS: Age >==50 years and parity >==5 were significant factors associated with residual disease. The incidence of residual disease was 56.5 and 29. 3% in patients >==50 and <50 years, respectively, and 61.8 and 36.0% in patients with parity >==5 and <5. Post-cone endocervical curettage (ECC) and multiple-quadrant disease were the only pathologic predictive factors identified. The incidence of residual disease was 64.6 and 29.2% in patients with positive ECC and negative ECC, respectively, and 48.4 and 25.9% in patient with multiple-quadrant disease and one- or two-quadrant disease. Other pathologic parameters, including endocervical margins, ectocervical margins, endocervical gland involvement, and depth of conization, were not predictive of residual disease. When ECC was combined individually with age, endocervical margins, or multiple-quadrant disease, there was no increase of positive predictive rate. CONCLUSIONS: (1) Age 50 years or more and parity >==5 were two demographic features that predicted post-cone residual disease. (2) ECC and multiple-quadrant disease were the only pathologic parameters that predicted post-cone residual disease. (3) With the appropriate application of the predictive factors, post-cone hysterectomy may be further decreased.

Perinatal management of congenital complete heart block.

BACKGROUND: The perinatal management of congenital complete heart block (CCHB) remains controversial. The purpose of this study was to present a therapeutic modality for CCHB. METHODS: We collected retrospective cases of all pregnant women admitted to our hospital between January 1992 and June 1999 whose babies developed CCHB antenatally. After a series of examinations, maternal, fetal and neonatal data were analyzed. RESULTS: Nine fetuses from six mothers (cases 1-6) in nine different pregnancies were studied. In case 1, both consecutive fetuses had CCHB and in case 2, all three consecutive fetuses had CCHB. The other mothers (cases 3-6) had only one fetus each with CCHB. Of the seven fetuses with isolated CCHB, four underwent observation only due to late-onset, or nonimmunologic CCHB, two received dexamethasone and/or intravenous immunoglobulin therapy because of the presence of hydropic signs, and one received dexamethasone at 23 weeks' gestation due to early-onset CCHB. Shortening fractions of the right ventricle had good compensation in four fetuses, without any treatment, and improving compensation in two of three fetuses receiving dexamethasone therapy. All seven fetuses were delivered smoothly and pacemakers were implanted shortly after birth. Two other fetuses had a poor outcome due to associated ventricular septal defect or hemoglobin Bart's disease. Furthermore, we gave dexamethasone (2 mg/day) instead of prednisolone (10 mg/day) for the next pregnancies of patients 3 to 5, beginning at 12 weeks of gestation. No fetal CCHB developed again. CONCLUSIONS: For pregnant women with previous fetal immunologic CCHB, early initiation of dexamethasone instead of prednisolone might be effective to cross the placenta and avoid recurrences. Dexamethasone is also effective for fetal CCHB of early onset, fetal hydrops or heart failure. Observation only is suggested for nonimmunologic CCHB and remote or late-onset immunologic CCHB. Other modalities were tried for very sick fetuses, but their effectiveness was not predictable.

Prenatal diagnosis of intrahepatic arteriovenous shunts.

A case of mixed intrahepatic arteriovenous shunts in a fetus diagnosed at 35 weeks' gestation is presented. Color Doppler ultrasonography in the fetal liver demonstrated complicated vascular connections fed by the hepatic arterial branches and drained into the portal and middle hepatic veins. Pulsed Doppler ultrasonography identified a high cardiac output state by the detection of increased flow velocities of the great vessels. The contribution of Doppler ultrasonography to hemodynamic changes is highlighted. The management is briefly discussed.