Severe intrauterine adhesions after life-threatening event caused by Epstein-Barr virus-associated atraumatic spleen laceration: Case report.

This is the first known case report of severe intrauterine adhesion (IUA) following a life-threatening event caused by an Epstein-Barr virus-associated atraumatic spleen laceration. A 22-year-old nulligravid female suffered from infectious mononucleosis for approximately 1 month. Sudden severe hypovolemic shock with massive hemoperitoneum appeared and hemostasis was completely achieved by a splenectomy for an atraumatic spleen laceration, although that was followed by multiorgan failure and abdominal compartment syndrome. Complete recovery without any neurological sequelae was achieved by intensive treatment. A postoperative pathological evaluation revealed Epstein-Barr virus-associated splenomegaly. The patient was referred to our department because of secondary amenorrhea for approximately 5 months since the last menstruation, which occurred just prior to the event. Laboratory blood test results demonstrated normal thyroid and ovarian functions. Hysterofiberscopy revealed complete obstruction at the end of the cervical canal, indicating secondary uterine amenorrhea caused by severe IUA. Hysteroscopic adhesiolysis with a rigid hysteroscope reached the opening of the uterine cavity and menstruation was restored.

Germline TP53 c.566C>T mutation incidentally diagnosed during treatment for acute myeloid leukemia: A case report.

TP53 mutations in acute myeloid leukemia (AML) are associated with poor outcomes. The number of somatic and/or germline genetic tests for therapy is increasing. Patients with such incidental findings should undergo adequate genetic counseling.

Evaluation of the clinical performance of noninvasive prenatal testing at a Japanese laboratory.

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.

Vasa praevia with meandering fetal vessels despite placental and umbilical cord insertion on the same side of the uterine wall: A case report.

Vasa praevia with meandering fetal vessels is extremely rare, and it is difficult to diagnose this prenatally. When cesarean section is performed, a change in the site of uterine incision may be required for a safe delivery.

Abdominal pregnancy implanted on surface of pedunculated subserosal uterine leiomyoma: A case report.

Abdominal pregnancy is a rare form of ectopic pregnancy. Various sites of implantation in abdominal pregnancy have been reported. Uterine serosa is an extremely rare implantation site, with only a few cases reported to date. No case of abdominal pregnancy implanted on the surface of a subserosal uterine leiomyoma has been reported. We herein report the case of a 40-year-old primigravida woman who was diagnosed with abdominal pregnancy implanted on the surface of a pedunculated subserosal uterine leiomyoma. The uterine leiomyoma with gestational tissue was resected laparoscopically and the postoperative course was uneventful. It is necessary to remember the possibility of unexpected implantation sites and that laparoscopic surgery may be more difficult in such cases than that for fallopian tube pregnancy.

Ovarian abscess caused by Helicobacter cinaedi in a patient with endometriosis.

Helicobacter cinaedi is a rarely encountered pathogen that easily induces bacteremia. Various foci of H. cinaedi infection have been reported; however, no case of adnexal abscess caused by H. cinaedi has been reported in the English literature. We herein report a case of ovarian abscess caused by H. cinaedi. A 38-year-old nulligravid Japanese woman was admitted to our hospital with an adnexal abscess. Clinical findings included fever, leukocytosis, and elevated C-reactive protein. Laparoscopic right partial oophorectomy with abdominal lavage was performed. H. cinaedi was isolated from cultures of blood and ovarian abscess fluid after surgery. Intravenous ampicillin/sulbactam was administered for 2 weeks, followed by oral amoxicillin for an additional 2 weeks. The postoperative course was uneventful and clinical findings improved. There was no evidence of relapse. H. cinaedi can cause ovarian abscess and is likely an under-recognized pathogen.

Resolution of dopamine agonist-resistant hyperprolactinemia by hysterectomy: a case report.

Prolactin-producing uterine leiomyomas are very rare. Although hyperprolactinemia rapidly improves after removal of such leiomyomas, no preoperative diagnostic test has been established for prolactin-producing uterine leiomyomas. A 45-year-old Japanese woman, gravida 3 para 3, was referred to our hospital for further examination of hyperprolactinemia resistant to a dopamine agonist. A pituitary prolactinoma was undetectable by brain magnetic resonance imaging. A bromocriptine loading test revealed an increased serum prolactin concentration after loading. Examination for the detection of an ectopic prolactinoma revealed a 9.0 cm diameter uterine leiomyoma that had measured 6.6 cm in diameter about six months before the first visit to our hospital. The hyperprolactinemia rapidly improved after hysterectomy. A prolactin-producing uterine leiomyoma should be considered as a possible cause of hyperprolactinemia resistant to dopamine agonists. Responsiveness to dopamine agonists; deterioration of hyperprolactinemia may be diagnostic for prolactin-producing uterine leiomyomas, although further research is required.

Ultrastructural and Cytological Studies on Mycosphaerella pinodes Infection of the Model Legume Medicago truncatula.

Ascochyta (Mycosphaerella) blight on cultivated peas is primarily caused by infection through asexual spores (pycnospores) of Mycosphaerella pinodes (Berk. et Blox.) Vestergren [recently renamed Peyronellaea pinodes (Berk. & A. Bloxam) Aveskamp, Gruyter & Verkley]. Using a model pathosystem involving Medicago truncatula and Mycosphaerella pinodes strain OMP-1, we examined the histology and ultrastructure of early infection events and fungal development including penetration by appressoria, vegetative growth of infection hyphae, and host responses. On the susceptible ecotype R108-1, pycnospores germinated and grew over the surface of the epidermis, then formed an appressoria and penetrated the cuticle. Beneath the cuticle, the infection peg expanded into a hyphae that grew within the outer wall of the epidermis. Subsequently, the hyphae penetrated down within mesophyll cells and proliferated vigorously, eventually, forming asexual fruiting bodies (pycnidia). In contrast, successful penetration and subsequent growth of infection hyphae were considerably restricted in the ecotype Caliph. Detected by its reaction with cerium chloride (CeCl3) to generate electron-dense cerium perhydroxides in transmission electron micrographs, hydrogen peroxide (H2O2) accumulated in epidermal and mesophyll cells of Caliph challenged with pycnospores of M. pinodes. This intracellular localization was confirmed by energy-dispersive X-ray spectroscopy. Our observations thus indicate that the oxidative burst reaction leading to the generation of reactive oxygen species is associated with a local host defense response in Caliph, since no clear H2O2 accumulation was detectable in susceptible R108-1. Indeed, aberrant hyphae such as intrahyphal hyphae and dead hyphae, probably due to a local defense elicited by the fungus, were abundant in Caliph but not in R108-1. Our results on the cellular interactions between the fungus and host cells provide additional insights to understand foliar infection by M. pinodes on cultivated peas.

Current status of non-invasive prenatal testing in Japan.

AIM: The purpose of this study was to report the 3-year experience of a nationwide demonstration project to introduce non-invasive prenatal testing (NIPT) of maternal plasma for aneuploidy, and review the current status of NIPT in Japan. METHODS: Tests were conducted to detect aneuploidy in high-risk pregnant women, and adequate genetic counseling was provided. The clinical data, test results, and pregnancy outcomes were recorded. We discuss the problems of NIPT on the basis of published reports and meta-analyses. RESULTS: From April 2013 to March 2016, 30 613 tests were conducted at 55 medical sites participating in a multicenter clinical study. Among the 30 613 women tested, 554 were positive (1.81%) and 30 021 were negative (98.1%) for aneuploidy. Of the 289, 128, and 44 women who tested positive for trisomies 21, 18, and 13, respectively, and underwent definitive testing, 279 (96.5%), 106 (82.8%), and 28 (63.6%) were determined to have a true-positive result. For the 13 481 women with negative result and whose progress could be traced, two had a false-negative result (0.02%). The tests were performed on the condition that a standard level of genetic counseling be provided at hospitals. CONCLUSION: Here, we report on the 3-year nationwide experience with NIPT in Japan. It is important to establish a genetic counseling system to enable women to make informed decisions regarding prenatal testing. Moreover, a welfare system is warranted to support women who decide to give birth to and raise children with chromosomal diseases.

Steroid Pulse Therapy for De Novo Minimal Change Disease During Pregnancy.

BACKGROUND Nephrotic syndrome occurs very rarely, in only about 0.01%-0.02% of all pregnancies, and de novo minimal change disease during pregnancy is especially rare. Nephrotic syndrome and, especially, minimal change disease are highly responsive to steroids, and preterm labor may be avoidable if the maternal condition is improved with steroid therapy. Therefore, prompt diagnosis and proper management are critical to maternal and fetal outcome when severe proteinuria occurs during pregnancy. CASE REPORT A 30-year-old pregnant Japanese woman presented with systemic edema, oliguria, and severe proteinuria and hypoalbuminemia at 25 weeks of gestation, although she was normotensive. The patient had high urinary protein selectivity. Her illness was diagnosed as de novo nephrotic syndrome with high steroid responsiveness rather than pre-eclampsia. She began steroid pulse therapy the day after admission. Complete remission was confirmed after 3 weeks. The patient did not relapse during pregnancy and delivered a healthy male baby at 37 weeks of gestation. A renal biopsy at a relapse after delivery confirmed minimal change disease. CONCLUSIONS In pregnant women with de novo minimal change disease, serious maternal and/or fetal complications may occur if severe proteinuria and hypoalbuminemia are unabated for an extended time. Evaluation of urinary protein selectivity is noninvasive and useful for prediction of steroid responsiveness. Results of urinary protein selectivity can be obtained earlier than results of renal biopsy. Renal biopsy during pregnancy is not always necessary for initiation of steroid therapy. Rapid initiation of steroid pulse therapy may enable quicker achievement of remission and prevent serious perinatal complications.

Preeclampsia before 20 weeks of gestation: a case report and review of the literature.

The occurrence of preeclampsia before 20 weeks of gestation is rare and usually associated with trophoblastic diseases or antiphospholipid syndrome. Here, we report a case of preeclampsia before 20 weeks of gestation in the absence of the aforementioned disorders. A healthy 30-year-old nulliparous woman presented with new onset of hypertension and proteinuria at 18 weeks of gestation. Fetal ultrasound did not reveal any abnormalities. Empirical steroid treatment was initiated based on a tentative diagnosis of underlying renal disease. The clinical course of the disease was progressive despite steroid treatment and the fetus died in utero 8 days after the initiation of treatment. Following delivery, a renal biopsy was performed and provided a diagnosis of preeclampsia. All symptoms resolved postpartum. This report demonstrates that preeclampsia may occur before 20 weeks of gestation and should always be considered in the differential diagnosis of pregnant women with new onset of hypertension with proteinuria. Previous published cases are summarized briefly.

Encephalomalacia in surviving twin after single fetal death diagnosed at 18 weeks of gestation in monochorionic twin pregnancy.

PATIENT: Female, 28 FINAL DIAGNOSIS: Single intrauterine fetal death Symptoms: - MEDICATION: - Clinical Procedure: - Specialty: Obstetrics and Gynecology. OBJECTIVE: Rare disease. BACKGROUND: Single fetal death in monochorionic twin pregnancy may result in poor perinatal outcome of the surviving twin, including neurologic sequelae, other organ injury, and death. In most reported cases of poor perinatal outcome in the surviving twin, monochorionic co-twin death occured after more than 20 weeks of gestation, while few with earlier occurrence have been presented. CASE REPORT: A 28-year-old primigravid woman was referred to our hospital at 18 3/7 weeks of gestation for perinatal management of single fetal death in a monochorionic-diamniotic twin pregnancy. Our first evaluation by ultrasonography revealed a dead twin sized at 16 weeks of gestation, and an alive one with normal size and appearance, together with 1 placenta and 2 amniotic cavities with normal fluid amounts. At 20 3/7 weeks of gestation, ultrasonography showed that the surviving twin had bilateral ventriculomegaly and dilatation all around the subarachnoid cavity despite a normal head size. Fetal magnetic resonance imaging revealed remarkable atrophy of the cerebral cortex. After counseling, the patient and family chose termination of pregnancy, and artificial abortion was performed at 21 weeks of gestation. The aborted fetuses were not anomalous. Autopsy pathological findings confirmed encephalomalacia in the surviving twin. CONCLUSIONS: Recent development of imaging device make it possible that several abnormalities in central nervous system can be detected in detail at earlier gestational age. It is important to keep this condition in mind even though single fetal death occurred at early gestational age.

Pathways of metastases from primary organs to the ovaries.

To investigate the metastatic pathways from the primary organs to the ovaries, we examined the microscopic findings from 18 original and 18 metastatic ovarian tumors carefully. In addition, we examined the immunohistochemical findings (Victoria blue stain for vascular invasion and D2-40 expression for lymphangio invasion) of metastatic ovarian tumors carefully. There were 4 (57%) ovarian lymphangio invasion cases in the 7 gastric cancers, but there were no cases in the 6 colorectal cancers (P < 0.05). There were 4 (67%) ovarian vascular invasion cases and one (17%) liver metastasis case in the 6 colorectal cancers, while there were no ovarian vascular invasions (P < 0.05) or no liver metastases in the 7 gastric cancers. The patients with metastatic ovarian tumors originating from distant organs who were treated at the same time as the original cancers had a significantly poorer prognosis than the patients with ovarian tumors treated later (P < 0.05). The rate of lymphatic metastasis from the stomach to the ovary was significantly higher than from the colon to the ovary. In addition we hypothesized that the rate of intravascular metastasis from the colorectum to the ovary was relatively higher than from the stomach to the ovary.

Retroperitoneal lipoma arising from the urinary bladder.

Retroperitoneal benign lipomas are extremely rare and represent about 2.9% of all primary retroperitoneal tumors. About 80% of the tumors in the retroperitoneal cavities are malignant neoplasms. We experienced a case of a retroperitoneal lipoma simulating an ovarian mature cystic teratoma. A diagnosis was correctly made by magnetic resonance imaging (MRI) prior to surgery, and a total tumorectomy was performed. The retroperitoneal lipoma was recognized to have arisen from the urinary bladder. Histological sections revealed a tumor consisting of typical adipose cells without atypia. These types of lipomas should be carefully followed-up because they often recur and undergo malignant transformations.

Synthesis and characterization of rhenium-copper sulfide cluster complexes [(Ph3P)2N][Re3(CuX)(mu3-S)4Cl6(PMe2Ph)3] (X = Cl, Br, I).

The reaction of a trinuclear rhenium sulfide cluster compound Re3S7Cl7 with dimethylphenylphosphine and CuX2 (X = Cl or Br) or CuX (X = Cl, Br, or I) formed tetranuclear cluster complexes [(Ph3P)2N][Re3(CuX)(mu3-S)4Cl6(PMe2Ph)3] (X = Cl, Br, or I). Their solutions have the characteristic intense blue color with visible spectral bands near 600 nm. Single-crystal X-ray structures show that three mu-S atoms in the intermediate trinuclear rhenium complex coordinate to a copper atom, forming elongated tetrahedral structures in which Re-Cu bonding interaction is negligible (Re-Cu distances are 3.50 to approximately 3.54 A as compared with Re-Re distances ranging from 2.69 to 2.81 A).

Fetal hemolytic disease due to anti-Rh17 alloimmunization.

OBJECTIVE: To delineate clinical features of a case of fetal hemolytic disease due to anti-Rh17, along with a review of relevant studies published in English and Japanese. METHODS: We present clinical features of a -D-/-D- phenotype woman with anti-Rh17 alloimmunization during pregnancy. Relevant English literature in the MEDLINE database was reviewed, while Japanese studies were searched in the Japana Centra Revuo Medicina database. RESULTS: A Japanese -D-/-D- woman with anti-Rh17 (Hro) was treated during pregnancy. Serial ultrasonography, antibody titers, amniocenteses, and cordocenteses were conducted for perinatal management. Amniocentesis results demonstrated a high delta optical density level of 450 in the amniotic fluid, while cordocentesis revealed alloimmunization between the mother and the fetus as well as fetal hemolytic anemia. Blood flow velocity in the middle cerebral artery indicated a rapid development of fetal anemia. The newborn demonstrated severe anemia and hyperbilirubinemia, which were successfully treated with exchange transfusions. Two cases of prenatally diagnosed fetal hemolytic disease due to anti-Rh17 were found published in English and 5 in Japanese. CONCLUSION: A -D-/-D- phenotype patient with anti-Rh17 was successfully managed during pregnancy and a good outcome for the neonate was achieved. Our results and a review of related literature led to the following suggestions. The first pregnancy in a -D-/-D- woman may be affected, an anamnestic immune response can easily occur during pregnancy, the level of anti-Rh17 titer is indicative of the degree of fetal hemolysis, and appropriate intrauterine intervention is warranted for achievement of a good outcome.

In utero diagnosis of an aneurysm of the vein of Galen causing hydrocephalus and heart failure.

A prenatally diagnosed aneurysm of the vein of Galen was presented in the fetus of a patient referred to our hospital at 31 weeks of gestation. Ultrasonography demonstrated polyhydramnios, cardiomegaly, dilatation of the right atrium and superior vena cava, tricuspid valve regurgitation, hydrocephalus, and a large hypoechoic mass with blood flow in the suboccipital region. Skin edema was shown thereafter. A 3262-g male was delivered by cesarean at 35 weeks of gestation. Computed tomography imaging demonstrated a large mass in the suboccipital region, after which thrombocytopenia appeared and the neonate died at 18 days of age.

Targeted disruption of the mouse rho-associated kinase 2 gene results in intrauterine growth retardation and fetal death.

Rho-associated kinase (ROCK), including the ROCK-I and ROCK-II isoforms, is a protein kinase involved in signaling from Rho to actin cytoskeleton. However, in vivo functions of each ROCK isoform remain largely unknown. We generated mice deficient in ROCK-II by gene targeting. ROCK-II(-/-) embryos were found at the expected Mendelian frequency until 13.5 days postcoitum, but approximately 90% died thereafter in utero. ROCK-II(-/-) mice of both genders that survived were born runts, subsequently developed without gross abnormality, and were fertile. Whole-mount staining for a knocked-in lacZ reporter gene revealed that ROCK-II was highly expressed in the labyrinth layer of the placenta. Disruption of architecture and extensive thrombus formation were found in the labyrinth layer of ROCK-II(-/-) mice. While no obvious alteration in actin filament structures was found in the labyrinth layer of ROCK-II(-/-) placenta and stress fibers were formed in cultured ROCK-II(-/-) trophoblasts, elevated expression of plasminogen activator inhibitor 1 was found in ROCK-II(-/-) placenta. These results suggest that ROCK-II is essential in inhibiting blood coagulation and maintaining blood flow in the endothelium-free labyrinth layer and that loss of ROCK-II leads to thrombus formation, placental dysfunction, intrauterine growth retardation, and fetal death.