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This study aimed to evaluate the perioperative outcomes of laparoscopic fundoplication (LF) for gastroesophageal reflux disease (GERD) in children with scoliosis. Data of consecutive patients with GERD who underwent LF from January 2015 to December 2020 at a single pediatric institution were retrospectively analyzed. Eighty-two patients underwent laparoscopic Nissen fundoplication. The median [interquartile range (IQR)] body weight was 9.3 [7; 14] kg. Seventy-five patients were neurologically impaired (91%), and other comorbidities included scoliosis (n = 33), lung disease (n = 39), and cardiac disease (n = 14). The median (IQR) operative time including the creation of the gastrostomy and volume of bleeding were 160 [143; 190] min and 2 [1; 5] mL, respectively. There were no significant differences between patients with and those without scoliosis (p = 0.17 and p = 0.90, respectively). Patients with cardiac disease had a longer operative time (167 [161; 193] vs. 157 [141; 190] min, p = 0.01). There were three post-operative complications in children with neurological impairment; however, there was no clear relationship between the severity of scoliosis and complications. Severity of scoliosis did not correlate with perioperative results and post-operative complications. This suggests that the same LF technique can be used regardless of the presence or absence of scoliosis in children.

AIM To evaluate the efficacy and safety of dinoprostone vaginal insert (PROPESS) in pregnant post-term Japanese women requiring cervical ripening. METHODS This randomized, double-blind, placebo-controlled study included 114 pregnant Japanese women at term (41 weeks of gestation) requiring cervical ripening (baseline Bishop score (BS) ≤ 4). The primary end-point was the proportion of subjects with successful cervical ripening defined as BS ≥ 7 or vaginal delivery in 12 h. The secondary end-points were changes in BS, proportion of women with vaginal delivery, proportion of women receiving mechanical cervical ripening procedure and use of oxytocic drugs. RESULTS PROPESS administration for a maximum of 12 h showed significantly higher successful cervical ripening rate (47.4% vs 14.3%, respectively; treatment contrast [TC]: 33.1%; P = 0.0002). The median time from administration to vaginal delivery was significantly shorter in the PROPESS group than in the placebo group (26.18 h vs 33.02 h; OR 2.51; 95% CI [1.60-3.92]; P

A 28-year-old woman was admitted during the eighth week of her pregnancy because her clinical course was consistent with rapid progressive glomerulonephritis (RPGN). Anti-glomerular basement membrane antibody (anti-GBM Ab) and myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) were positive, and the anti-GBM Ab titer being extremely high. She was treated with hemodialysis, plasma exchange and prednisolone. She survived the illness; however, neither the fetus nor her kidney function could be rescued. She had human leukocyte antigen (HLA)-DRB1*1502:01, which differs from the DRB1*1501 associated with anti-GBM GN. When patients have particular symptoms, we should check the urine and serum creatinine to exclude RPGN, even in cases of pregnancy.

Aim We aimed to evaluate the sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of noninvasive prenatal testing (NIPT) in high-risk pregnant women. Methods Pregnant women who underwent GeneTech NIPT, the most commonly used NIPT in Japan, between January 2015 and March 2019, at Japan NIPT Consortium medical sites were recruited for this study. The exclusion criteria were as follows: pregnant women with missing survey items, multiple pregnancy/vanishing twins, chromosomal abnormalities in the fetus other than the NIPT target disease, and nonreportable NIPT results. Sensitivity and specificity were calculated from the obtained data, and maternal age-specific PPV and NPV were estimated. Results Of the 45 504 cases, 44 263 cases fulfilling the study criteria were included. The mean maternal age and gestational weeks at the time of procedure were 38.5 years and 13.1 weeks, respectively. Sensitivities were 99.78% (95% confidence interval [95% CI]: 98.78-99.96), 99.12% (95% CI: 96.83-99.76), and 100% (95% CI: 88.30-100) for trisomies 21, 18, and 13, respectively. Specificities were more than 99.9% for trisomies 21, 18, and 13, respectively. Maternal age-specific PPVs were more than 93%, 77%, and 43% at the age of 35 years for trisomies 21, 18, and 13, respectively. Conclusion The GeneTech NIPT data showed high sensitivity and specificity in the detection of fetal trisomies 21, 18, and 13 in high-risk pregnant women, and maternal age-specific PPVs were obtained. These results could provide more accurate and improved information regarding NIPT for genetic counseling in Japan.

Introduction: Obstetric severe perineal laceration can frequently occur as a surgical site infection (SSI), which sometimes leads to rectovaginal fistula after repair. We encountered a rare case of a rectoperineal fistula 5 months after repair of a severe perineal laceration.Case presentation: The patient was a 39-year-old woman who underwent repair of a fourth-degree perineal laceration after vaginal delivery. Five months after primary repair, she presented with perineal swelling and pain followed by uncontrollable flatulence or passage of feces at the perineum, which was finally diagnosed as a rectoperineal fistula. Transperineal repair with fistulous tract excision was performed for the rectoperineal fistula. Closure of the rectum, perineal body, and vagina was performed layer-by-layer constructing a thick perineum to prevent anal dysfunction. The fistula was successfully closed, and the patient did not show any symptoms of fecal incontinence 6 months after surgery.Discussion: As the rectoperineal fistula might have resulted in SSI at the primary repair of the obstetric injury, the delayed occurrence of the rectoperineal fistula was unusual. A perineal approach should be performed for complete fistulous tract excision, reconstruction of a robust perineal structure, and preservation of anal sphincter function.

There is a paucity of data on the risk of preterm birth subcategorized by gestational age in pregnancies after the pre-pregnancy excisional treatment for cervical lesions. In addition, little is known about the effect of prophylactic cerclage on the risk of preterm birth. The aim of this study was to investigate the risk of preterm birth stratified by gestational period and its reduction by the prophylactic cerclage in women with prior excisional surgery.We retrospectively analyzed a cohort of singleton pregnancies in the Japan Perinatal Registry Network Database (2013-2014,In the propensity-score matched population (cases,Pre-pregnancy excisional cervical surgery was associated with the increased risk of preterm birth, especially before 32 weeks of gestation. The prophylactic cerclage did not reduce the risk of preterm birth.

Background Postpartum hemorrhage (PPH) is a major cause of maternal mortality. Even seemingly low-risk women may suddenly develop postpartum hemorrhage, and these cases are often unpredictable and threatens maternal life. We hypothesized that innate poor uterine contractility may be a risk factor of PPH. In this study, we examined the association between the innate poor uterine contractility, suggested by the characteristics of labor and PPH in obstetrically low-risk women.Methods We used the Japan Perinatal Registry database of the Japan Society of Obstetrics and Gynecology registered in 2013–2016. With exclusion of women with well-known risk factors for PPH (maternal basal disease of hematologic disease, uterine leiomyoma, pregnancy by assisted reproductive technology, placenta abruption, placenta accrete, low-lying placenta, hypertensive disorders of pregnancy, macrosomia, polyhydramnios, or epidural analgesia), we analyzed 174,082 primiparous women who had one live singleton birth via vaginal delivery in cephalic presentation at 37 weeks’ gestation. Information about abnormal labor patterns (hypotonic uterine dysfunction, prolonged labor, and arrest of labor) diagnosed by obstetricians were also used in this study. In order to focus on innate maternal poor uterine contractility, we classified subjects into four classes according to whether they were diagnosed with abnormal labor patterns and whether they had used uterotonics, and Odds ratios and their 95% CI were calculated as well.Results Among the enrolled women, 10,508 (6.0%) had PPH. Abnormal labor patterns, including hypotonic uterine dysfunction (adjusted OR 1.28, 95% CI 1.22 to 1.34), prolonged labor (adjusted OR 1.41, 95% CI 1.30 to 1.52) and arrest of labor (adjusted OR 2.02, 95% CI 1.78 to 2.29) were significantly associated with an increased risk of PPH. Compared to women who were not diagnosed any abnormal labor patterns and did not use any uterotonics, women who were diagnosed with abnormal labor patterns were at a significantly increased risk for PPH regardless of whether they had used uterotonics(adjusted OR 1.23, 95% CI 1.10 to 1.37) or not (adjusted OR 1.30, 95% CI 1.23 to 1.37).Conclusion Our study suggests that innate poor uterine contractility is a significant predisposing risk factor in otherwise low-risk women.Trial Registration: This study is retrospectively registered.

This study aimed to assess characteristics of pregnant women taking antiepileptic drugs with inadequate folic acid intake. This cross-sectional study examined pregnant women taking antiepileptic drugs who were registered in the Japanese Drug Information Institute in Pregnancy (JDIIP) database between October 2005 and December 2016. Participants were classified into two groups according to when they started folic acid supplementation (before pregnancy: 'adequate', after pregnancy or never: 'inadequate'). Logistic regression analysis was performed to investigate factors associated with inadequate folic acid intake. Of 12,794 registrants, 468 pregnant women were taking antiepileptics during the first trimester. Of these, we analysed data from 456 women who had no missing data. As a result, inadequate folic acid intake was noted among 83.3% of them, suggesting that the current level of folic acid intake is insufficient overall. Younger age, smoking, alcohol drinking, multiparity, unplanned pregnancy, and being prescribed AEDs by paediatric or psychiatric departments were independent factors associated with inadequate folic acid intake. As planned pregnancy was the strongest factor, healthcare professionals should ensure that childbearing women taking antiepileptics are informed of the importance of planned pregnancy. In addition, healthcare professionals must gain a better understanding of folic acid intake, as the prevalence of adequate intake differed according to which departments prescribed antiepileptic drugs.

To compare the prevalence of enlarged ovarian and intrapelvic parasitic arteries to the gravid uterus between cases of placenta accreta spectrum (PAS) and those with normal placentation using unenhanced magnetic resonance (MR) angiography.Unenhanced time-of-flight MR angiography was performed in 12 consecutive women with PAS (mean age, 34 years; range, 23-42 years) and 24 women with normal placentation (mean age, 31 years; range, 24-42 years) in their third trimester and reviewed by two independent observers. The consensus reading served as the reference standard. Findings of pelvic arteriography performed at cesarean hysterectomy were reviewed in all cases of PAS. The prevalence of enlarged ovarian and intrapelvic parasitic arteries was compared using Fisher's exact test. The interobserver agreement was assessed with Kappa statistics.The prevalence of enlarged ovarian arteries was not significantly different between cases of PAS and normal placentation (17% [4/24 pelvic sides] vs. 4% [2/48 pelvic sides], P = .091). The prevalence of intrapelvic parasitic arteries was significantly higher in cases of PAS than in those with normal placentation (67% [16/24 pelvic sides] vs. 0% [0/48 pelvic sides], P .0001). On a patient-by-patient basis, the intrapelvic parasitic artery was frequently present in women with PAS (92% [11/12 patients]). The Kappa values were 0.915 and 0.852 for detecting enlarged ovarian and intrapelvic parasitic arteries, respectively, indicating excellent interobserver agreement.The development of intrapelvic parasitic arteries was an anomalous phenomenon observed on unenhanced MR angiography in the majority of women with PAS but was not observed in those with normal placentation.

Aims The objective of this study was to determine how many pregnant Japanese women with diabetes mellitus (DM)/gestational diabetes mellitus (GDM) experience perinatal mortality in the presence of fetal anomalies. Methods Our investigation included data from 205 secondary/tertiary obstetric facilities located widely in Japan. The Japan Ministry of Health, Labour and Welfare Vital Statistics of Japan was used for comparison. Results Of 237 941 women giving birth at 205 hospitals, 1796 (0.8%) and 13 037 (5.5%) had DM and GDM, respectively. The perinatal mortality rates (per 1000 births) were 10.6 (19/1796) for women with DM, 5.2 (68/13037) for women with GDM, and 3.7 (7612/2039504) for the general Japanese population. Detailed information was available for 63 (72%) of the 87 perinatal deaths occurring in women with diabetes including DM and GDM; fetal anomalies were associated with 40% (25/63) of perinatal deaths, exceeding 16% (1211/7612) in the general Japanese population (P

Background and Aim Liver dysfunction with decreased antithrombin activity (AT) and/or thrombocytopenia is life-threatening in pregnant women. Whether AT is clinically useful for prediction of liver dysfunction remains unclear. Methods A total of 541 women were registered prospectively at gestational week 34.7 (20.0 – 41.4) with available data on antenatal AT and platelet count (PLC). Results Liver dysfunction defined as serum AST > 45 IU/L concomitant with lactate dehydrogenase (LDH) > 400 IU/L occurred in five women antenatally (≤ 2 weeks before delivery) and in 17 women postpartum (within 1 week postpartum). Median (5th – 95th) antenatal value was 85 (62 – 110)% for AT and 202 (118 – 315) × 109/L for PLC in the 541 women, and was significantly lower in women with than without perinatal liver dysfunction; 75 (51 – 108) vs. 86 (62 – 110)% and 179 (56 – 244) vs. 203 (121 – 316) × 109/L, respectively. Nineteen (86%) women with liver dysfunction showed AT ≤ 62% or thrombocytopenia (PLC ≤ 118 × 109/L) perinatally, but five lacked thrombocytopenia throughout the perinatal period. The best cut-off (AT, 77%; PLC, 139 × 109/L) suggested by receiver operating characteristic curve gave antenatal AT and PLC sensitivity of 59% and 41% with positive predictive value (PPV) of 8.6% and 14%, respectively, and combined use of AT and PLC improved sensitivity to 73% (16/22) with PPV of 9.2% for prediction of perinatal liver dysfunction. Conclusions Reduced AT not accompanied by thrombocytopenia can precede liver dysfunction. Clinical introduction of AT may enhance safety of pregnant women. This article is protected by copyright. All rights reserved.

This study aimed to assess characteristics of pregnant women taking antiepileptic drugs with inadequate folic acid intake. This cross-sectional study examined pregnant women taking antiepileptic drugs who were registered in the Japanese Drug Information Institute in Pregnancy (JDIIP) database between October 2005 and December 2016. Participants were classified into two groups according to when they started folic acid supplementation (before pregnancy: ‘adequate’, after pregnancy or never: ‘inadequate’). Logistic regression analysis was performed to investigate factors associated with inadequate folic acid intake. Of 12,794 registrants, 468 pregnant women were taking antiepileptics during the first trimester. Of these, we analysed data from 456 women who had no missing data. As a result, inadequate folic acid intake was noted among 83.3% of them, suggesting that the current level of folic acid intake is insufficient overall. Younger age, smoking, alcohol drinking, multiparity, unplanned pregnancy, and being prescribed AEDs by paediatric or psychiatric departments were independent factors associated with inadequate folic acid intake. As unplanned pregnancy was the strongest factor, healthcare professionals should ensure that childbearing women taking antiepileptics are informed of the importance of planned pregnancy. In addition, healthcare professionals must gain a better understanding of folic acid intake, as the prevalence of adequate intake differed according to which departments prescribed antiepileptic drugs.

Introduction Some pregnant women develop significant proteinuria in the absence of hypertension. However, clinical significance of isolated gestational proteinuria (IGP) is not well understood. This study aimed to determine the prevalence of IGP in singleton pregnancies and the proportion of women with IGP who subsequently developed preeclampsia (IGP-PE) among all PE cases. Material and methods This was an observational study of 6819 women with singleton pregnancies at 12 centers, including 938 women with at least once determination of protein-to-creatinine ratio (P/Cr). Significant proteinuria in pregnancy (SPIP) was defined as P/Cr (mg/mg) level >0.27. IGP was defined as SPIP in the absence of hypertension. Gestational hypertension (GH) preceding preeclampsia (GH-PE) was defined as preeclampsia (PE) in which GH preceded SPIP. Simultaneous PE (S-PE) was defined as PE in which both SPIP and hypertension occurred simultaneously. Results IGP and PE were diagnosed in 130 (1.9%) and 158 (2.3%) of 6819 women, respectively. Of 130 women with IGP, 32 (25%) progressed to PE and accounted for 20% of all women with PE. Hence, women with IGP had a relative risk of 13.1 (95% CI; 9.2–18.5) for developing PE compared with those without IGP [25% (32/130) vs. 1.9% (126/6689)]. At diagnosis of SPIP, P/Cr levels already exceeded 1.0 more often in women with S-PE than in those with IGP-PE [67% (33/49) vs. 44% (14/32), respectively, p = 0.031]. Conclusions IGP is a risk factor for PE, and IGP-PE accounts for a considerable proportion (20%) of all PE.

Perinatal care in Japan has progressed rapidly in recent decades, remarkably reducing maternal, perinatal and neonatal mortality rates. This is attributable not only to the sustained efforts and dedication of past obstetricians and midwives, but also to the collective results achieved by the Japan Society of Obstetrics and Gynecology and healthcare administration, including research on advanced medical care, education, medical care improvements and establishing perinatal care centers. Although the maternal mortality rate was in steady decline until 2007 (3.1/100 000 births), it repeatedly fluctuated thereafter, plateauing at 3.4 per 100 000 births in 2013 and 2.7 per 100 000 births in 2014. Thus, the Perinatology Committee has analyzed the current situation of maternal deaths and has proposed countermeasures to reduce such death. The items deliberated upon by related subcommittees in 2015 are presented herein. The addition of indications for ‘fibrinogen concentrate’, ‘eptacog alfa’ and approval of the PGE2 vaginal tablet for cervical ripening were discussed in the subcommittee for unapproved drug review. Thus, a request for approval for health insurance coverage was submitted to the ‘Evaluation committee on unapproved or off-label drugs with high medical needs’ of the Ministry of Health, Labour and Welfare. Maternal and late-maternal deaths from suicide during the 10 years from 2005 to 2014 in Tokyo's 23 wards were jointly examined with the Tokyo Medical Examiner's Office. The suicide rate in the 23 wards is very high, at 8.7 per 100 000 births. Thus, the subcommittee for the reduction of maternal death discussed countermeasures for the eradication of maternal death and maternal suicide and the revision of death certificates.

Recent studies have revealed unique biological characteristics of molecular hydrogen (H2) as an anti-inflammatory agent. We developed a novel haemodialysis (E-HD) system delivering an H2 (30–80 ppb)-enriched dialysis solution by water electrolysis, and conducted a non-randomized, non-blinded, prospective observational study exploring its clinical impact. Prevalent chronic HD patients were allocated to either the E-HD (n = 161) group or the conventional HD (C-HD: n = 148) group, and received the respective HD treatments during the study. The primary endpoint was a composite of all-cause mortality and development of non-lethal cardio-cerebrovascular events (cardiac disease, apoplexy, and leg amputation due to peripheral artery disease). During the 3.28-year mean observation period, there were no differences in dialysis parameters between the two groups; however, post-dialysis hypertension was ameliorated with significant reductions in antihypertensive agents in the E-HD patients. There were 91 events (50 in the C-HD group and 41 in the E-HD group). Multivariate analysis of the Cox proportional hazards model revealed E-HD as an independent significant factor for the primary endpoint (hazard ratio 0.59; [95% confidence interval: 0.38–0.92]) after adjusting for confounding factors (age, cardiovascular disease history, serum albumin, and C-reactive protein). HD applying an H2-dissolved HD solution could improve the prognosis of chronic HD patients.

Aim Monoacyglycerol acyltransferases (MGATs) are known to play important roles in intestinal TG absorption. In contrast, the role of MGATs in the liver is still unclear. We investigated the effects of JTP-103237, a novel MGAT inhibitor, on hepatic MGAT activity and hepatic lipid metabolism. Results JTP-103237 reduced hepatic triglyceride content and hepatic MGAT activity in a high sucrose very low fat (HSVLF) diet induced fatty liver model. Interestingly, JTP-103237 suppressed not only triglyceride (TG) and diacylglycerol (DG) synthesis, but also fatty acid (FA) synthesis (de novo lipogenesis) in this model. JTP-103237 also suppressed lipogenesis-related gene expression, such as sterol regulatory element-binding protein 1-c. Moreover, JTP-103237 decreased plasma glucose levels and total cholesterol and reduced the accumulation of epididymal fats in HSVLF diet fed mice. Conclusion In the present study, JTP-103237 prevented carbohydrate-induced fatty liver and suppressed both TG synthesis and de novo lipogenesis, suggesting MGAT inhibitor may prevent carbohydrate-induced metabolic disorders, including NAFLD, obesity and diabetes.

Objective To report a case of gas gangrene in a fetus at term, which was diagnosed by antenatal computed tomography (CT) imaging. Case Report A 23-year-old primiparous woman, who did not undergo any prenatal health checks until term, developed hypertension, proteinuria, and clouding of consciousness, and experienced intrauterine fetal death. A single, mature fetus with voluminous gas bubbles was observed on CT, which was consistent with a diagnosis of fetal gas gangrene. Following the induction of labor, a dead, malodorous infant was delivered, along with foul-smelling and frothy amniotic fluid. The patient's condition deteriorated, and intensive care support was required to treat the patient for septic shock and disseminated intravascular coagulation during the postpartum period. She died on the 2 nd postpartum day. Conclusion Fetal gas gangrene is a very rare and potentially lethal event in pregnant women. To our knowledge, this is the first report on the antenatal diagnosis of fetal gas gangrene in a term pregnancy through CT. CT was useful for evaluating the extent of emphysematous change in the fetal and maternal organs.

To clarify the effect of starvation due to hyperemesis gravidarum on the screening of gestational diabetes mellitus (GDM).A retrospective study was undertaken of pregnant women who delivered at Tsukuba University Hospital, Japan, between October 1, 2010, and September 30, 2013. GDM screening was performed in the first trimester using the random blood glucose test with a cutoff value of 5.2mmol/L and in the second trimester using a 50-g glucose challenge test with a cutoff value of 7.8mmol/L. If the screening was positive, a 75-g oral glucose tolerance test was performed for a definite diagnosis.Among 2112 eligible women, 33 (1.6%) required hospitalization for hyperemesis; the remaining 2079 women formed the control group. In the first trimester, the positive GDM screening rate was significantly higher in the hyperemesis group than in the control group (13 [39.4%] vs 115 [5.5%]; P0.001). Additionally, the positive predictive value was significantly lower in the hyperemesis group (23.1% vs 73.9%; P0.001). In the second trimester, no significant differences were observed between groups.Hyperemesis gravidarum affects the positive GDM screening rate in the first trimester.

Study objectives In humans and other mammals, sleep is altered during pregnancy. However, no studies have been conducted on sleep/wakefulness during pregnancy in mice. In this study, we examined sleep/wakefulness in female C57BL/6 mice during pregnancy. We also examined sleep/wake behaviors in an animal model of preeclampsia, pregnancy-associated hypertensive (PAH) mice, in which increased angiotensin causes hypertension. Methods Sleep/wake behaviors of female C57BL/6 and PAH mice were examined based on electroencephalogram (EEG) or electromyogram recordings before, during, and after pregnancy. To examine whether high blood pressure disrupts the integrity of the blood-brain barrier in PAH mice, Evans blue dye was injected intravenously. Angiotensin II receptor blocker (olmesartan)-administered PAH mice and female Tsukuba hypertensive mice were also examined. Results C57BL/6 mice showed a decreased total wake time and increased nonrapid eye movement (NREM) sleep time during late pregnancy. Rapid eye movement (REM) sleep time did not change during the course of pregnancy. PAH mice exhibited a general slowing of EEG during late pregnancy and subsequently returned to apparently normal sleep/wakefulness after delivery. All PAH mice exhibited multiple focal leakages of Evans blue dye in the brain. Spike-and-wave discharges were observed in 50% of PAH mice. Olmesartan-administered PAH mice did not show general slowing of EEG. Tsukuba hypertensive mice showed a normal time spent in wakefulness and NREM sleep and a decreased total REM sleep time. Conclusions This study showed pregnant-stage-specific changes in sleep/wakefulness in C57BL/6 mice. Furthermore, PAH mice may be useful as an animal model for eclampsia.

The original publication is available at www.springerlink.com, This questionnaire survey was conducted at 11 hospitals in Japan to determine vaccination coverage against seasonal influenza and the prevalence rate of influenza among pregnant Japanese women. Of 2,808 postpartum women who gave birth at the 11 hospitals during the study period from March 1, 2014, to July 31, 2014, 1,713 (61 %) participated in this study and 876 (51 %) reported having received vaccination against influenza in or after October 2013. Women aged < 25 years had a significantly lower vaccination rate than those aged a parts per thousand yen25 years (31 % vs. 53 %, respectively; p = 0.0000). Eighty-seven (5.1 %) and 1,626 (94.9 %) women did and did not contract influenza, respectively. Although prior birth did not affect overall vaccination coverage (50 % for primiparous vs. 53 % for multiparous), multiparous women had a significantly higher rate of contracting influenza than primiparous women, irrespective of vaccination status (5.6 % vs. 2.2 % [p = 0.0216] and 9.7 % vs. 3.5 % [p = 0.0003] for women with and without vaccination, respectively). The 2013-2014 vaccination program significantly reduced the influenza infection rate by 35 % (3.9 % vs. 6.3 % for women with and without vaccination, respectively; p = 0.0272). Seventy-two (83 %) of the 87 women took antiviral agents for the treatment of influenza and two (2.3 %) required hospitalization. These results suggested that pregnant Japanese women had a high level of concern regarding seasonal influenza. However, campaigns targeting young pregnant Japanese women, as well as multiparous women, for vaccination are needed in order to further reduce the incidence of influenza among pregnant Japanese women.

We evaluated the postnatal outcomes of patients with prenatally diagnosed agenesis of the corpus callosum (ACC), in conjunction with ventriculomegaly, as a tool for parental counseling. Through a retrospective review of maternal and infant records, we evaluated the postnatal outcomes of 21 patients with prenatally diagnosed ACC and fetal ventriculomegaly. Ten of the 21 patients (48 %) were diagnosed with isolated ACC. Among these 10 patients, neurodevelopmental outcomes were normal in four, uncertain in one, and five demonstrated mild or moderate disabilities. The remaining 11 patients had ACC associated with either central nervous system (CNS) anomalies (7 of 11) or chromosomal abnormalities (4 of 11). The outcomes were estimated in nine of the 11 children; all nine had severe disabilities. The mortality rate of ACC, associated with other anomalies, was 29 % (2 of 7) in children with CNS anomalies and 75 % (3 of 4) for those with chromosomal abnormalities. If ACC with fetal ventriculomegaly is associated with other malformations, a poor outcome is highly likely. Although the outcome of isolated ACC with fetal ventriculomegaly was generally better, >50 % of the patients had mild or moderate neurodevelopmental disabilities. These findings provide useful information for parental counseling.

Purpose To investigate whether planning of pregnancy in women with epilepsy affects seizure control during pregnancy and to compare the maternal and neonatal outcomes in planned and unplanned pregnancies. Methods This was a retrospective cohort study of 153 pregnant women with epilepsy who were treated at the University of Tsukuba Hospital and Hokkaido University Hospital between 2003 and 2011. Twenty-one pregnancies were excluded due to insufficient data. Data of patients followed by neurologists during their planned pregnancies (planned-pregnancy group, n =51) were compared to those of patients referred to neurologists after conception for managing epilepsy during pregnancy (unplanned-pregnancy group, n =81). The treatment profile for epilepsy, seizure control, and maternal and neonatal outcomes in both groups were compared using Chi-square test or Fisher's exact test and Mann–Whitney U test. Results Compared to the unplanned-pregnancy group, the planned-pregnancy group showed a significantly greater proportion of patients receiving monotherapy with antiepileptic drugs (80% vs. 61%: planned vs. unplanned, P =0.049) and those not requiring valproic acid (77% vs. 56%, P =0.031). Furthermore, the frequency of epileptic seizures (16% vs. 35%, P =0.018) and changes in antiepileptic drugs (24% vs. 41%, P =0.042) were significantly lower in the planned-pregnancy group than in the unplanned-pregnancy group. No significant intergroup differences were noted in the obstetric complications and neonatal outcomes, including congenital malformations. Conclusion For women with epilepsy, planning of pregnancy is associated with good seizure control during pregnancy and less fetal exposure to antiepileptic drugs.

Background: The dipstick test is widely used as a primary screening test for detection of significant proteinuria in pregnancy (SPIP). However, it often shows a false positive test result. This study was performed to determine which pregnant women should be recommended to undergo determination of urinary protein-to-creatinine ratio (mg/mg, P/Cr test) after dipstick test for confirmation of SPIP. Methods: This was a multicenter, prospective, and observational study of 2212 urine specimens from 1033 pregnant women who underwent simultaneous dipstick and P/Cr tests in the same spot urine samples at least once. SPIP was defined as P/Cr > 0.27. Preeclampsia was diagnosed in women with both hypertension and SPIP. Results: Preeclampsia, hypertension alone, and SPIP alone developed in 202 (20 %), 73 (7.1 %), and 120 (12 %) women, respectively. Creatinine concentration [Cr] varied greatly, ranging from 8.1 to 831 mg/dL in the 2212 urine samples. Rate of positive dipstick test results increased with increasing [Cr], while SPIP prevalence rate was lower in urine samples with higher [Cr], yielding higher false positive rates in samples with higher [Cr]. Postpartum urine samples had significantly lower [Cr] compared to those obtained antepartum (60 [8.7-297] vs. 100 [10-401] mg/dL, respectively). At the first P/Cr test among women with similar dipstick test results, the risk of having SPIP was consistently and significantly higher for hypertensive women than for normotensive women at any dipstick test result: 18 % (14/77) vs. 3.2 % (8/251), 47 % (26/55) vs. 8.7 % (37/425), 91 % (82/90) vs. 59 % (44/75) for negative/equivocal, 1+, and >= 2+ test results, respectively. The risk of SPIP was 16 % (9/55) for normotensive women when two successive antenatal urine samples showed a dipstick test result of 1 +. Conclusions: For prediction of SPIP, the dipstick test was more likely to show a false positive result in concentrated urine samples with higher [Cr]. Hypertensive women with >= 1+ as well as normotensive women with >= 2+ on dipstick test should be advised to undergo the P/Cr test.

To assess whether adiponectin gene (ADIPOQ) polymorphism is associated with intrauterine fetal growth and cord blood adiponectin, we investigated eight single-nucleotide polymorphisms (SNPs; rs182052, rs710445, rs16861205, rs12495941, rs1501299, rs3774261, rs2082940 and rs266729) in ADIPOQ and birth weight and cord blood adiponectin in 526 healthy neonates. We found that the neonates carrying the G allele of rs266729 had a significantly greater birth weight s.d. score than those homozygous for the C allele (CC: -0.06±0.75 versus CG: 0.20±0.64 versus GG: 0.07±0.78; P=1.65 × 10(-3), adjusted P=9.90 × 10(-3)). However, this difference was not significant after adjustment for cord blood adiponectin (P=0.04, adjusted P=0.26). The rs266729 SNP was strongly associated with cord blood adiponectin; neonates with rs266729 GG had the highest adiponectin (CC: 34.1±20.2 versus CG: 44.3±26.1 versus GG: 54.1±36.7 μg ml(-1), P=2.80 × 10(-9), adjusted P=1.68 × 10(-8)). This association remained after adjustment for birth weight s.d. score (P=6.63 × 10(-8), adjusted P=3.98 × 10(-7)). Our results suggest that the influence of the rs266729 SNP in ADIPOQ on birth weight may be dependent on circulating adiponectin.

Clinical guidelines for obstetrical practice were first published by the Japan Society of Obstetrics and Gynecology (JSOG) and the Japan Association of Obstetricians and Gynecologists (JAOG) in 2008, and a revised version was published in 2011. The aims of this publication include the determination of current standard care practices for pregnant women in Japan, the widespread use of standard care practices, the enhancement of safety in obstetrical practice, the reduction in burdens associated with medico-legal and medico-economical problems, and a better understanding between pregnant women and maternity-service providers. These guidelines include a total of 87 Clinical Questions followed by several Answers (CQ&A), a Discussion, a List of References, and some Tables and Figures covering common problems and questions encountered in obstetrical practice. Each answer with a recommendation level of A, B or C has been prepared based principally on 'evidence' or a consensus among Japanese obstetricians in situations where 'evidence' is weak or lacking. Answers with a recommendation level of A or B represent current standard care practices in Japan. All 87 CQ&A are presented herein to promote a better understanding of the current standard care practices for pregnant women in Japan.

膵癌の初発症状は腹痛，黄疸，背部痛の順に多く，腫瘍栓に伴う症状で発症することは極めて稀である．今回，上腸間膜静脈腫瘍栓により発症した膵癌を経験した．症例は77歳，男性．腹痛，下血を主訴に救急外来受診．強い心窩部痛と心電図上心房細動を認めたため上腸間膜動脈血栓症を疑い，動脈相中心の腹部造影computed tomography（以下CT）を施行．CTでは原因の特定に至らなかったが，症状が強いため試験開腹術を施行した．開腹所見では血性腹水，小腸と腸間膜の黒赤色変化，腸間膜静脈の血栓を認め，小腸切除，血栓除去，人工肛門造設を行った．術中採取した静脈壁の内部に腫瘍所見ありとの病理診断を得た．原発巣の検索を行い，膵頭体移行部に腫瘍を認め膵管細胞診でadenocarcinomaとの結果より，膵癌に伴う上腸間膜静脈腫瘍栓・血栓と診断した．自験例のように上腸間膜静脈腫瘍栓・血栓を初発症状とした膵癌の報告例は検索した限りではなく，極めて稀な症例と思われ報告する．

頭頸部癌の治療後にみられる摂食・嚥下障害に対する当科の取り組みを紹介した。嚥下造影検査，嚥下内視鏡検査を行う際には検査時状況画像ならびに嚥下時産生音を同時記録し，記録された患者の姿勢，体格，意識レベル，表情や意欲，摂食運動，発声，呼吸音，嚥下音むせなどの情報を利用することにより摂食・嚥下障害の効率的な臨床的対応を実現している。また摂食・嚥下障害の訓練法ならびに対応法を短期間に確実に習得させるために入院下集中リハビリテーションを行っており，その副次的効果として短期間での摂食レベルの改善を得ている。さらに頭頸部癌術後患者には摂食・嚥下障害の改善のために各種口腔内装置を適用しており，今回は舌接触補助床と下顎復位装置について紹介した。

Polymorphisms in cytokine genes can influence immune responses to human papillomavirus infection, possibly modifying risks of cervical cancer. Using an amplification refractory mutation system-polymerase chain reaction method, we analyzed a single nucleotide polymorphism (A/G) at position -1082 in interleukin-10 promoter region in 440 Japanese women: 173 women with normal cytology, 163 women with cervical intraepithelial neoplasia and 104 women with invasive cervical cancer. The carrier frequency of interleukin-10 -1082 G alleles associated with higher interleukin-10 production increased with disease severity: 9.8% for normal cytology; 19.6% for cervical intraepithelial neoplasia; 29.8% for invasive cervical cancer (P for trend < 0.001). Among cytologically normal women, human papillomavirus infections were more common in those who were positive for an interleukin-10 -1082 G allele (P = 0.04). In conclusion, our data suggest that interleukin-10 -1082 gene polymorphism may serve as a marker of genetic susceptibility to cervical cancer among Japanese women.

A diamniotic-monochorionic pregnancy of twins was revealed by transvaginal ultrasonography: one fetus with holoprosencephaly was diagnosed at 11 weeks of gestation. This is the first case of holoprosencephaly diagnosed in the first trimester. Prenatal diagnosis of this anomaly in the first trimester is now possible by transvaginal ultrasonography.

Background Fetal growth restriction (FGR) and low birth weight(LBW) are serious public health problems. In developing countries, the incidence of low birth weight is predominantly the result of FGR, and both low birth weight and FGR are associated with neonatal death and later growth and development. Fetal growth charts are important for assessing the size of the fetus during pregnancy. The aims of this study were to describe the fetal growth pattern of a population in rural Bangladesh where maternal undernutrition is prevalent and to compare the timing of FGR in that population with WHO and INTERGROWTH- 21st international reference values. Methods From November 2001 to October 2003, pregnant women were recruited in Matlab, a sub district of Bangladesh, and underwent three follow-up ultrasound examinations during pregnancy for measurement of the parameters of the fetal head, abdomen, and femur. The data were fitted to a linear-cubic model, and the derived values were compared with international reference values. Results A total of 2678 singleton pregnancies were included in the analyses. The mean (SD) maternal age was 25.9 (5.8) years (range, 14–47 years). The mean (SD) early pregnancy BMI was 20.1 (2.6) kg/m2, and 27.6% of the women were underweight (BMI

To obtain baseline data for human papillomavirus (HPV) screening and vaccination in Japan, we analyzed HPV DNA data from 2282 Japanese women (1517 normal cytology, 318 cervical intraepithelial neoplasia [CIN] grade 1, 307 CIN2-3, and 140 invasive cervical cancer [ICC]) that visited the University of Tsukuba Hospital or Ibaraki Seinan Medical Center Hospital for screening or treatment of cervical diseases between 1999 and 2007. An L1-based PCR method was used for individual HPV genotyping. The most common HPV types in ICC were, in order of decreasing prevalence, HPV16 (40.5%), HPV18 (24.4%), HPV52 (8.4%), HPV58 (3.1%), and HPV33 (3.1%). Based on the comparison of HPV type distributions between normal cytology and CIN2-3 and ICC, estimated risk of disease progression varied considerably by genotype: HPV16, HPV18, HPV31, HPV33, HPV35, HPV52, and HPV58 (prevalence ratio, 1.92; 95% confidence interval 1.58-2.34); other oncogenic types (0.31, 95% confidence interval 0.19-0.50); and non-oncogenic types (0.09, 95% confidence interval 0.03-0.43). HPV16 and/or HPV18, including coinfections with other types, contributed to 67.1% of ICC and 36.2% of CIN2-3 among Japanese women. More importantly, the overall prevalence of HPV16 and/or HPV18 varied greatly according to the women's age: highest in women aged 20-29 years (ICC, 90.0%; CIN2-3, 53.9%), decreasing with age thereafter, and lowest in women aged 60 years or older (ICC, 56.3%; CIN2-3, 25.0%). In conclusion, type-specific HPV testing may help identify Japanese women at high risk of progression to CIN2-3 and cancer. In Japan, current HPV vaccines are estimated to provide approximately 70% protection against ICC and may be more useful in reducing the incidence of cervical cancer and precancer in young women of reproductive age.

Nonalcoholic Steatohepatitis（以下，NASH）症例に対する肝切除においてはNASHによる肝予備能低下の可能性を念頭においた慎重な術前評価，切除術式の適応が必要である．症例は71歳の女性で，肝胆道系酵素の上昇を契機として左右肝管合流部から右肝管に主座をおく肝門部胆管癌と診断した．糖尿病，高脂血症，脂肪肝を有しておりNASHの合併が疑われた．ICGR15（％），99mTc-GSAシンチでのHH15，LHL15はいずれも正常値で肝予備能は良好と判断した．門脈塞栓術にて99mTc-GSAシンチによる推定残肝予備能は39.1％と増大が得られ，肝拡大右葉切除術を施行した．術後は肝不全の発症なく良好な経過であった．病理組織学的検査所見でもNASHと診断された．本症例はNASH症例においてもICGR15，99mTc-GSAシンチを中心とした肝硬変症例に準じた肝予備能評価，術式の適応が可能であることを示唆するものである．

症例は30歳，女性．左腹部の激痛を訴え当院受診．帝王切開と子宮筋腫核出術の既往あり．左腹部に腹膜刺激症状があり，腹部CTで左上腹部の限局性小腸ガス像を認めたことから，絞扼性イレウスの診断で緊急手術施行となる．Treitz靱帯より60cm肛門側の小腸間膜にリンパ節様結節性病変が付着し，そこから伸びる索状物で小腸の一部が絞扼されていた．索状物と結節性病変の切除によりイレウスは解除された．病理所見で，結節性病変は変性壊死と多量の好中球，組織球，好酸球浸潤を背景とする1mm径程度の好酸性構造物から成り，アニサキス虫体の一部との結果を得た．アニサキス症は大部分が胃・小腸を主とする消化管壁の急性炎症として発症するが，自験例は消化管外アニサキス症により絞扼性イレウスを生じた稀な症例であり，同様の報告は本邦で僅か6例である．非常に頻度は低いが，絞扼性イレウスの原因疾患として，本症を理解しておく必要がある．

Objective: To clarify the impact of a mild form of gestational diabetes mellitus (GDM) on neonatal birth size, and on insulin-related hormones and adiponectin (AdipoQ) in cord blood. Methods: Two hundred and sixteen Japanese pregnant women diagnosed as having normal glucose tolerance according to the JSOG criteria were enrolled. Of the 216 women, 38 women were reclassified into a mild GDM (mGDM) group according to the IADPSG criteria. Of the remaining 178 women, 135 women with normal 50-g glucose challenge test were reclassified into a normal glucose tolerance (NGT) group. Cord blood insulin-like growth factor (IGF)-1, IGF-binding proteins (IGFBPs) and AdipoQ were measured in the offspring of the two groups. Results: Birth weight and its SD scores were larger in the mGDM group than in the NGT group. The incidence of large-for-gestational age (LGA) newborns was higher in the mGDM than in the NGT group. No differences in cord blood free IGF-1, IGFBP-1, IGFBP-2 or AdipoQ levels were observed between the mGDM and NGT groups. Conclusions: Our study suggests that mild GDM reclassified according to the IADPSG criteria influences neonatal birth size, but neither the IGF-IGFBP axis nor AdipoQ can account for the changes of birth size in offspring of women with mild GDM.

Aims: This study aimed to determine effective predictive factors for primary postpartum hemorrhage (PPH) among clinical blood parameters associated with coagulation and fibrinolysis and demographic characteristics. Methods: We retrospectively studied 1032 women who underwent determinations of clinical blood parameters at gestational week (GW) 29–32 and GW 35–37 and gave birth to singleton infants at our hospital between January 2011 and December 2013. PPH was defined as estimated blood loss ≥700 mL. Multivariate logistic regression analyses were used to determine independent risk factors and odds ratios (OR) for PPH. Results: PPH occurred in 104 of 1032 women (10%). Three blood variables, fibrinogen level 2.7 μg/mL (2.03 [1.29–3.19]) at GW 35–37, and three demographic characteristics, maternal age ≥35 years (1.75 [1.15–2.68]), BMI >28.2 kg/m2 on admission for childbirth (1.95 [1.20–3.16]), and previous cesarean delivery (2.77 [1.31–5.83]), were identified as independent risk factors for PPH. Conclusion: Among blood parameters, higher D-dimer levels and lower levels of antithrombin activity and fibrinogen in late gestation were independent risk factors for PPH.

Fetal magnetocardiography (fMCG) is useful for analysis of fetal cardiac events. However, fetal presentation and movement affect the fMCG waveform, making it difficult to standardize the waveform. The aim of this study was to investigate whether the use of vector magnetometers can compensate for these limitations. We studied 59 fetuses (gestational age, 22-40 wk, median, 32), including 41 with uncomplicated pregnancies and 18 with fetal cardiac disease. fMCG was recorded twice in each case, and the two waveforms were compared with each other in uncomplicated subjects to investigate the effects of fetal presentation. The superconducting quantum interference device (SQUID) system used in this study was a 12-channel vector magnetometer, by which the three components of the magnetic field (Bx, By, Bz) could be detected simultaneously at four recording points. By constructing the three components, a composite waveform (Bxyz) was obtained. The configuration of the composite waveforms was similar among normal fetuses always with positive polarity, independent of fetal presentation and movement. The difference in the time intervals (PR, QRS, and ventricular activation time [VAT]) between the first and second measurements was minimal in the composite waveforms (Bxyz) compared with that in each channel (Bx, By, Bz). Even before signal averaging, waveforms with high time resolution were recorded in at least one of the three components, making it possible to analyze fetal arrhythmias precisely. Our results indicate that vector magnetocardiography is potentially useful for standardization of the fMCG waveforms and to provide a more complete and accurate analysis of fetal arrhythmias.

Monoacylglycerol acyltransferase 2 (MGAT2) plays an important role in intestinal fat absorption. We discovered the novel MGAT2 inhibitor, JTP-103237, and evaluated its pharmacological profile. JTP-103237 selectively inhibited MGAT2 without remarkable species differences and reduced absorbed lipids in circulation. After lipid administration, JTP-103237 slightly but significantly decreased triglyceride content in proximal small intestine and significantly increased the lipids content in the distal small intestine. In addition, JTP-103237 significantly increased MGAT substrate (monoacylglycerol and fatty acid) content in the small intestine. JTP-103237 increased plasma peptide YY levels after lipid loading and reduced food intake in a dietary fat-dependent manner. After chronic treatment, JTP-103237 significantly decreased body weight and increased O2 consumption in the early dark phase in high fat diet induced obese (DIO) mice. Moreover, JTP-103237 improved glucose tolerance and decreased fat weight and hepatic triglyceride content in DIO mice. Our findings indicate that JTP-103237 prevents diet-induced obesity by inhibiting intestinal MGAT2 and has unique properties as a drug for the treatment of obesity.

腹部臓器虚血を合併したStanford B型急性大動脈解離に対し,右総腸骨動脈-上腸間膜動脈 (SMA) バイパス術を行い,良好な経過を得た症例を経験した.症例は59歳,男性.腹痛と左下肢痛を主訴に救急搬送され, Stanford B型の大動脈解離,左外腸骨動脈閉塞と診断し,緊急に左大腿動脈へのバイパス術を施行した.下肢の虚血は改善したが,術翌日に血中肝酵素の著明な上昇を認め,造影CTにて腹腔動脈の閉塞と診断,緊急手術を行った.開腹時,肝全域の虚血を認め,解離は腹腔動脈から総肝動脈にまで及んでいた.大伏在静脈グラフトを用いて右総腸骨動脈-SMAバイパス術を施行し,肝血流は速やかに改善した.術後経過中,急性腎不全および十二指腸潰瘍の穿孔を併発したが,発症4カ月後に自宅退院となった.急性大動脈解離に伴う腹部臓器虚血に対して,本術式は有効な術式であると考えられた.

We report a fetus at 33 weeks of gestation with supraventricular tachycardia, which was successfully managed by transplacental administration of an antiarrhythmic agent. Fetal magnetocardiography (fMCG) revealed supraventricular tachycardia of the long RP’ tachycardia type. Transplacental administration of sotalol, instead of digoxin, was selected as the first-line drug, and it successfully converted supraventricular tachycardia to sinus rhythm. The diagnosis of the type of supraventricular tachycardia was confirmed by electrocardiography after birth. Sotalol was also effective after birth to maintain sinus rhythm. This case demonstrates that fMCG is potentially useful for prenatal differentiation of the type of supraventricular tachycardia and for prenatal treatment of fetal tachyarrhythmias.

症例は73歳の男性。大量下血による出血性ショックのため来院した。腹部造影CT,血管造影にて内腸骨動脈瘤の破裂とその下部消化管穿破を疑い,止血と再破裂を防ぐために経カテーテル動脈塞栓術(TAE)を2度,施行した。5日後,腹部造影CTにて腹腔内遊離ガスが認められたため緊急開腹術を施行した。術中所見で直腸前壁に穿孔を認めたため,同部位を切除し,直腸断端を閉鎖して人工肛門を造設した(Hartmann手術)。この穿孔部位が血腫の消化管への穿破部位と考えられた。さらに4日後,腹腔内ドレーンから便臭を伴う多量の排液が観察されたため,再開腹下に直腸を再切断し,腹腔内洗浄ドレナージ術を施行した。その後,数度の腹腔内膿瘍を繰り返したが,第48病日に外泊可能となった。しかし外泊中に右下腹部痛が出現し,第50病日には救急車で搬送され,センター到着時は心肺停止であった。内腸骨動脈瘤再破裂による出血性ショックと思われた。心肺蘇生法を行ったが心拍再開せず,永眠された。近年,内腸骨動脈瘤破裂にTAEが有効な治療として用いられているが,消化管穿破例ではTAEのみでは生命予後を改善しないと考えられる。

The aim of this study was to evaluate the efficacy of Doppler ultrasonography (US) with very high frequency transducers on assessing soft-tissue masses.We performed sonographic evaluation with an ATL HDI-5000 equipped with a 12.5-MHz linear transducer. Images were obtained in 70 patients admitted to our hospital. We applied Sono-CT as a new variation of compound sonography. Sono-CT provides compound images by combining the images obtained by electronically directing the transducer to scan from multiple angles.In this study, we found that US was able to depict softest tissue masses in detail. All tumors were shown as hypoechoic masses, except lipoma. Particularly, US was useful to diagnose certain neural or fatty tumors, and it was proven to help modality the most in the diagnosis of glomus tumors, since its vascularity can easily be detected using this device. In addition, US was not hampered by metal-induced artifacts. Moreover power (color) Doppler imaging was a valuable adjunct in differentiating malignant tumors from benign ones. US offers many advantages in the assessment of soft-tissue masses. This examination is thought to be sensitive, widely available, noninvasive, and less expensive.

We here report the first case of discordant Pena-Shokeir phenotype observed in monoamniotic twins. A 34-year-old woman, pregnant with twins, was referred at 10 weeks' gestation because one of the twins had increased nuchal translucency. Serial ultrasonographic examinations suggested that twin A may have had several other abnormalities, including pleural effusion at 21 weeks' gestation, decreased movement and contracted limbs at 24 weeks, and fetal growth restriction at 26 weeks. No abnormalities were observed in twin B. At 34 weeks of gestation, the twins were delivered by cesarean section. There were cord entanglements, and although the resuscitation of twin A was attempted, it proved difficult due to lockjaw. Twin A died during the second hour of life, and autopsy findings were consistent with the diagnosis of Pena-Shokeir phenotype. We suggest that cord entanglement during early gestation is a possible cause for the occurrence of Pena-Shokeir phenotype through an anoxic-ischemic mechanism.

Background and aim It is supposed that enhanced oxidative stress and inflammation are involved with the poor clinical outcomes in patients on chronic dialysis treatment. Recent studies have shown that molecular hydrogen (H2) is biologically active as an anti-inflammatory agent. Thus, we developed a novel hemodialysis (E-HD) system which delivers H2 (30 to 80 ppb)-enriched dialysis solution, to conduct a prospective observational study (UMIN000004857) in order to compare the long-term outcomes between E-HD and conventional-HD (C-HD) in Japan. The present interim analysis aimed to look at potential clinical effects of E-HD during the first 12 months observation. Subjects and method 262 patients (140, E-HD; 122, C-HD) were subjected for analysis for comprehensive clinical profiles. They were all participating in the above mentioned study, and they had been under the respective HD treatment for 12 consecutive months without hospitalization. Collected data, such as, physical and laboratory examinations, medications, and self-assessment questionnaires on subjective symptoms (i.e., fatigue and pruritus) were compared between the two groups. Results In a 12-month period, no clinical relevant differences were found in dialysis-related parameters between the two groups. However, there were differences in the defined daily dose of anti-hypertensive agents, and subjective symptoms, such as severe fatigue, and pruritus, which were all less in the E-HD group. Multivariate analysis revealed E-HD was an independent significant factor for the reduced use of anti-hypertensive agents as well as the absence of severe fatigue and pruritus at 12 months after adjusting for confounding factors. Conclusion The data indicates E-HD could have substantial clinical benefits beyond conventional HD therapy, and support the rationale to conduct clinical trials of H2 application to HD treatment.

We report a case of fetal trisomy 18 with SCN5A R1193Q variant that presented with sinus bradycardia, 2 : 1 atrioventricular block (AVB), and QT interval prolongation. These complex arrhythmias were diagnosed by fetal magnetocardiography combined with ultrasound findings. Advanced AVB and ventricular arrhythmias were confirmed after birth. Genetic testing of the baby revealed a SCN5A R1193Q variant, which we considered could account for the various arrhythmias in this case.

We report a case of alveolar capillary dysplasia, wherein duodenal atresia was diagnosed during the third trimester. A 36-year-old mother was referred to our hospital for polyhydramnios at 31 weeks' gestation. Duodenal atresia was suspected from the ultrasonographic findings, which showed gastric dilation. Other findings noted were enlarged, highly echogenic lungs, a spherical heart and an increased lung-thorax transverse area ratio. A male infant was born at 37 weeks' gestation. The findings of serial radiography of the infant's upper gastrointestinal tract were compatible with the diagnosis of duodenal atresia; however, he developed persistent pulmonary hypertension of the newborn eight hours after birth and died at five days of age. The autopsy revealed alveolar capillary dysplasia and duodenal obstruction. We propose that the detection of duodenal atresia should prompt the physician to conduct a thorough ultrasonographic examination to rule out associated anomalies, such as alveolar capillary dysplasia, which can be detected by the presence of highly echogenic lungs.

Beckwith-Wiedemann syndrome is an inherited disorder most commonly characterized by prenatal or postnatal overgrowth, macroglossia, omphalocele, unusual earlobe creases, and increased risk of neoplasia. Several reported cases of this syndrome have been prenatally diagnosed, but no report has described the occurrence of this syndrome in association with a single umbilical artery. We report a case in which prenatal sonographic examination demonstrated fetal overgrowth, macroglossia, and omphalocele together with a single umbilical artery; our prenatal diagnosis of Beckwith-Wiedemann syndrome was confirmed after birth of the infant. The possibility of this syndrome should be considered when performing a detailed sonographic examination of a fetus with a single umbilical artery.

Preeclampsia is associated with thrombosis of the intervillous or spiral artery. A deletion/insertion polymorphism (4G or 5G) in the promoter of the plasminogen activator inhibitor type 1 (PAI-1) gene is suggested to be involved in regulating the synthesis of the inhibitor, 4G allele, being associated with the enhanced gene expression and plasma PAI-1 levels. We assessed the association between preeclampsia and the 4G/5G polymorphism of the PAI-1 gene in 115 preeclamptic patients, 210 pregnant controls, and 298 healthy volunteer controls. The frequency of the homozygotes for the 4G allele was significantly higher in the patients than in the control pregnant women (P = 0.04) or in the healthy volunteers (P = 0.02). The 4G allele frequency was also significantly higher in the patients than in the control group of pregnant women (P = 0.03) and in the healthy volunteers (P = 0.02). These results suggest that the presence of the 4G/4G genotype of the PAI-1 gene is one of the risk factors for preeclampsia.