official journal of Congeital Anomalies Research Association of Japan
Online ISSN : 2433-1503
Print ISSN : 0037-2285
Volume 29 , Issue 2
Congenital Anomalies
Showing 1-5 articles out of 5 articles from the selected issue
  • 1989 Volume 29 Issue 2 Pages 51-58
    Published: 1989
    Released: July 22, 2021
    JOURNALS OPEN ACCESS
    ABSTRACT Crj:CD (Sprague-Dawley) rats were treated intraperitoneally with methylazoxymethanol acetate (MAM) at 0 and 30 mg/kg on day 13 of gestation and were allowed to deliver. On day 4 postpartum, the litter size was adjusted to 8 with an equal sex distribution. Two males and females from each litter were tested between 3 and 5 weeks of age for open field activity and rotorod performance. At week 7 postpartum, all offspring were sacrificed and examined for brain anomalies. The open field activity of MAM treated offspring tended to increase; number of ambulations and rearings were significantly increased at 3 weeks of age in male offspring treated with MAM when compared to those of male controls. At 3 weeks of age, the male offspring in the MAM treated group showed significantly reduced rotorod performance when compared to that in the control. Severe reductions of the cerebral hemispheres were observed at 7 weeks of age in the male and female offspring treated with MAM. These results indicate that the motor behavior of offspring is significantly impaired at weaning when they were exposed to MAM prenatally. The effects were more severe for males than females.
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  • 1989 Volume 29 Issue 2 Pages 59-71
    Published: 1989
    Released: July 22, 2021
    JOURNALS OPEN ACCESS
    ABSTRACT Dose-related changes of 5-azacytidine (5-AC) induced digital mal- formations are reported. Dosages of 0.4, 0.5, 0.6,0.7, and 1.0 mg/kg of 5-AC were injected intraperitoneally into pregnant rats on day 13 of gestation. The susceptibility order of each digit was the second < third < first < fourth < fifth in the forepaw and the second < first < third = fourth < fifth in the hindpaw. In the forepaw, the percentages of cutaneous syndactyly with closely apposed metacarpals or osseous syndactyly between the first and second digits (type FA defect ) and brachydactyly of the third digit (type FB defect) were increased in a clear dose- related trend. In the hindlimb, the percentages of osseous or cutaneous syndactyly between the first and second digits (type HA defect), brachydactyly or ametatarsia of the second digit (type HB defect), and osseous or cutaneous syndactyly, closely apposed metacarpals, lateral metacarpal fusion, or osseous or cutaneous syndactyly with lateral metacarpal fusion or closely apposed metacarpals between the third and fourth digits (type HC defect) reached a maximum at 0.7 mg/kg of 5-AC and then decreased. However, the percentages of ectrodactyly, 1 missing digit (type M1 defect) or 2 missing digits (type M2 defect), were increased from 0.6 mg/kg or 0.7 mg/kg, respectively. Ectrodactyly, missing 3 digits (type M3 defect), was rarely observed in the dose range used.
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  • 1989 Volume 29 Issue 2 Pages 73-82
    Published: 1989
    Released: July 22, 2021
    JOURNALS OPEN ACCESS
    ABSTRACT The rat with congenital osteochondrodysplasia with systemic sub- cutaneous edema (ocd/ocd) dies shortly after parturition. The cause of death has not been fully understood in the similar lethal disorders in other species. The present study describes the morphological abnormalities in the respiratory tract in relation to the cause of death in ocd/ocd. Mid saggital section of the respiratory tract of the ocd/ocd neonate revealed that the air flow from nose to lung is disturbed by occlusion of the pharynx and larynx area with the cleft palate and relatively large tongue. Smaller thoracic volume was also noted in the ocd/ocd neonate. Of intra-thoracic organs, only the lung showed significantly smaller weight in the ocd/ocd neonate than that of phenotypically normal (t/?). Histology of the fetal and neonatal lung showed atelectasis and many small alveolar cavities with abundant lung surfactants in the ocd/ocd. Disarrangement of the cells com- posing the alveolar wall was also revealed in the ocd/ocd. Electron microscopy of the fetal lung demonstrates the lamella bodies in the alveolar cavities and the pneumocyte type I1 cells in both genotypes. Insufficiency of the surfactant was excluded for the cause of death in the ocd/ocd.
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  • 1989 Volume 29 Issue 2 Pages 83-89
    Published: 1989
    Released: July 22, 2021
    JOURNALS OPEN ACCESS
    ABSTRACT The rat with congenital osteochondrodysplasia with systemic sub- cutaneous edema (ocd/ocd) dies shortly after birth. It has been considered that the cause of death would be disturbed air flow from nose to lung by morphological abnormalities in respiratory tract. Respiratory rhythm was studied in the isolated and perfused preparation of brain stem-spinal cord-thorax in the ocd/ocd and phenotypically normal (+I?). The response of the isolated and perfused prepara- tion of thorax and diaphragm to the electrical stimulation was also studied. The spontaneous depolarizations of cervical ventral root C4 or C5 in the brain stem- spinal cord-thorax preparation were very low and infrequent in the ocd/ocd. The both tensions of the thorax and diaphragm preparations to the electrical stimula- tion were lower in the ocd/ocd than those in the +I?. Those results show that the cause of death in the ocd/ocd is not only morpho- logical abnormalities but also functional defects in the respiratory system including the central nervous system and muscles.
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  • 1989 Volume 29 Issue 2 Pages 91-101
    Published: 1989
    Released: July 22, 2021
    JOURNALS OPEN ACCESS
    ABSTRACT Under real-time ultrasonographic guidance, chorionic villi sampling (CVS) was performed either by direct punch biopsy with FUJINON therapeutic flexible hysterofiberscope (HYS-FT), or by catheter aspiration biopsy after obser- vation with FUJINON hysterofiberscope (HYS-F). The former method was per- formed in 2 pregnant women, the latter in 11. The chorion frondosum was observed three dimensionally in all cases. Except for one unsuccessful sampling in our first cases in which CVSs were performed, all attempts were successful, and diagnoses duly completed (1 1 cases of chromosome analyses and one case of DNA analysis). No serious complications appeared in any case immediately after sampling or on the 1st and 7th days. Ultrasonography showed no adverse effects on fetal heart rate, no hematoma at the site of CVS and no injury to the gestational sac. Uterine bleeding was rarely witnessed. Except for one spontane- ous aborTion and one intra-uterine fetal death (IUFD) due to placenta previa, all other newborns were healthy. With some improvements, this CVS is believed to be an effective method.
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