official journal of Congeital Anomalies Research Association of Japan
Online ISSN : 2433-1503
Print ISSN : 0037-2285
Volume 53 , Issue 3
Congenital Anomalies
Showing 1-9 articles out of 9 articles from the selected issue
  • 2013 Volume 53 Issue 3 Pages 1
    Published: 2013
    Released: August 20, 2021
    JOURNAL OPEN ACCESS
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  • 2013 Volume 53 Issue 3 Pages 101-108
    Published: 2013
    Released: August 20, 2021
    JOURNAL OPEN ACCESS
    Abstract Congenital anomalies of wingless-type mouse mammary tumor virus (MMTV) integration site family (Wnt) are frequently accompanied with tooth and dentin abnormality. The aim of this study was to investigate the effects of Wnt signaling on odontoblast differentiation of mouse dental papilla cells (MDPs). Mouse dental papilla cells were cultured in α-modified minimum essential medium containing 10% fetal bovine serum and antibiotics. Odontoblast differentiation was induced by bone morphogenic protein 2 (BMP2), and the expression of odontoblast-specific markers and Wnt-related signaling molecules was analyzed by real-time reverse transcription-polymerase chain reaction and immunohistochemistry. Odontoblast differentiation was evaluated by dentin sialophosphoprotein (Dspp) and dentin matrix protein (DMP) 1 expression. Localization of β-catenin in MDPs was detected by immunocytochemistry using an anti-β-catenin antibody. Dspp expression in MDPs was upregulated in the presence of BMP2. Wnt5a, Wnt11, Lef1 and Tcf4 expression was upregulated in BMP2-treated MDPs. Wnt11 expression was detected in rat dental pulp in vivo, and particularly strong expression of Wnt11 was detected in odontoblasts. Enhanced Dspp and DMP1 expression and alkaline phosphatase activity induced by BMP2 were completely negated by the Wnt antagonist: IWR-1-endo treatment. Nuclear translocation of β-catenin observed in BMP2-treated MDPs was also negated by IWR-1-endo treatment. These results indicate that Wnt signaling upregulates odontoblast marker expression in MDPs, suggesting a promoting effect of Wnt signaling on odontoblast differentiation.
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  • 2013 Volume 53 Issue 3 Pages 109-114
    Published: 2013
    Released: August 20, 2021
    JOURNAL OPEN ACCESS
    Abstract A myriad of factors have been linked to increased risk for intrauterine growth restriction and the associated complications; the majority of which are based on observational statistics of demographics, socioeconomics and patient history. Unfortunately, there is a paucity of factors available that can appropriately address the underlying anatomy and physiology responsible for intrauterine growth restriction. To this point, it becomes necessary to use data acquisition modalities capable of addressing both the etiology and pathology in an effort to improve clinical management strategies. Near-infrared spectroscopy, although not traditionally used in standard, clinical screening has proven valuable for risk assessment in a number of recent investigational studies. Simulations based on the current literature are presented to assess near infrared spectroscopy utility regarding the ability to distinguish between the normal fetus and the growth restricted fetus. Findings are presented for all simulated data as well as the equipment-specific data derived from the NIRO-100 system (Hamamatsu Photonics, Hamamatsu, Japan). Results suggest an overall sensitivity and specificity on the order of 62% and 58%, respectively, and NIRO-100 sensitivity and specificity on the order of 85% and 92%, respectively.
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  • 2013 Volume 53 Issue 3 Pages 115-121
    Published: 2013
    Released: August 20, 2021
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    Abstract Intermediate frequency magnetic fields (MFs) have widely been used in industrial machines and home appliances, such as induction heating cookers, although toxicity studies to evaluate the potential health risks of such fields are insufficient. In induction heating cookers, the MF source (i.e. hobs), is located near the abdominal position of a person cooking. Hence, developmental effects on the fetus may be a concern in case the person is a pregnant woman. Fertile White Leghorn eggs (60/group) were either exposed to 20 kHz, 1.1 mT(rms) or 60 kHz, 0.11 mT(rms) sinusoidal MFs for 19 days during embryogenesis. The same number of eggs served as a control group. In addition, a sham-sham experiment was conducted to validate the equality between exposure and control facilities. After exposure, embryos were examined for mortality rate and stage. Live embryos were evaluated for developmental stage and gross and skeletal anomalies. Length of upper beak and leg digits was also measured. Examinations were conducted in a blinded fashion to ensure quality assurance; experiments were triplicated for each frequency to confirm the outcome reproducibility. Mortality rate and stage, incidence of malformed embryos, and developmental variables in live embryos were found to be similar between the MF-exposed and corresponding control group. Incidence of gross anomalies such as mandibular edema and skeletal anomalies such as coccyx defects were low across the experiments, and no significant group differences were noted. In conclusion, exposure to 20 kHz or 60 kHz MF did not produce any significant teratogenic developmental effects in chick embryos.
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  • 2013 Volume 53 Issue 3 Pages 122-126
    Published: 2013
    Released: August 20, 2021
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    Abstract The spinal nerve, which is composed of dorsal root ganglion (DRG) sensory axons and spinal motor axons, forms the dorsal ramus projecting to the dorsal musculature. By using the free-floating immunohistochemistry method, we closely examined the spatiotemporal pattern of the formation of the dorsal ramus and the relationship between its projection to the myotome/dorsal musculature and semaphorin 3A (Sema3A), which is an axonal guidance molecule. In embryonic day (E) 10.5–E11.5 wild-type mouse embryos, we clearly showed the existence of a waiting period for the dorsal ramus projection to the myotome. In contrast, in E10.5–E11.5 Sema3A-deficient embryos, the dorsal ramus fibers projected beyond the edge of the myotome without exhibiting the waiting period for projection. These results strongly suggest that the delayed innervation by dorsal ramus fibers may be caused by Sema3A-induced axon repulsion derived from the myotome. Next, by performing culture experiments, we confirmed that E12.5 mouse axons responded to Sema3A-induced repulsion. Together, our results imply that Sema3A may play a key role in the proper development of the dorsal ramus projection.
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  • 2013 Volume 53 Issue 3 Pages 127-130
    Published: 2013
    Released: August 20, 2021
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    Abstract Cerebellar abnormalities in 4-week-old rats with a single whole body X-irradiation at a dose of 0.5, 1.0, or 1.5 Gy on embryonic day (ED) 15 were examined by magnetic resonance imaging (MRI) volumetry. A 3D T2W-MRI anatomical sequence with high-spatial resolution at 11.7-tesla was acquired from the fixed rat heads. By MRI volumetry, whole cerebellar volumes decreased dose-dependently. Multiple linear regression analysis revealed that the cortical volume (standardized β = 0.901; P <0.001) was a major explanatory variable for the whole cerebellar volume, whereas both volumes of the white matter and deep cerebellar nuclei also decreased depending on the X-irradiation dose. The present MRI volumetric analysis revealed a dose-related cerebellar cortical hypoplasia by prenatal exposure to X-irradiation on E15.
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  • 2013 Volume 53 Issue 3 Pages 131-133
    Published: 2013
    Released: August 20, 2021
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    Abstract Asymmetric and parasitic conjoined twins are extremely rare anomalies of monochorionic monoamniotic twins, consisting of an incomplete twin attached to the fully developed body of the co-twin. Sonographic examination is essential for prenatal diagnosis as early detection of fetal anomalies with poor prognosis provides a chance to deliver the infant vaginally for the mother. We herein describe a case with a prenatal diagnosis of a parasitic twin using three-dimensional (3-D) ultrasound. The clear images obtained with 3-D ultrasound helped in counseling the parents. Making an early prenatal diagnosis of severe fetal anomalies with careful and sophisticated sonographic examinations should be warranted.
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  • 2013 Volume 53 Issue 3 Pages 134-136
    Published: 2013
    Released: August 20, 2021
    JOURNAL OPEN ACCESS
    Abstract Heterotopic glial nodules are rare congenital cutaneous lesions; only 13 cases of scalp localized lesions of this kind are reported in the English medical literature. Herpes simplex virus is a rare cause of neonatal morbidity and mortality and is a rare cause of intrauterine infection. We report the first case of concurrent presence of a heterotopic glial nodule of the scalp and neonatal, in utero-acquired, fatal herpes simplex virus type-2 infection.
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  • 2013 Volume 53 Issue 3 Pages 137-140
    Published: 2013
    Released: August 20, 2021
    JOURNAL OPEN ACCESS
    Abstract An early second-trimester prenatal ultrasound diagnosis of an arthrogryposis multiplex congenita-like syndrome associated with median clefts is reported. A molecular biological work-up was performed to search for a potentially overlapping syndrome and dysostosis. Autopsy and postmortem radiogram were performed to confirm the ultrasound diagnosis. Prenatal diagnosis enabled early detection of multiple fetal malformations, thus allowing early termination of pregnancy. Moreover, three-dimensional ultrasound with volume rendering in the maximum surface mode demonstrated its value in diagnosing oro-facial clefts, even at an early stage of fetal development.
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