日本先天異常学会会報
Online ISSN : 2433-1503
Print ISSN : 0037-2285
35 巻 , 4 号
Congenital Anomalies
選択された号の論文の7件中1~7を表示しています
  • 1995 年 35 巻 4 号 p. 411-423
    発行日: 1995年
    公開日: 2021/07/29
    ジャーナル オープンアクセス
    ABSTRACT A study of congenital malformations and deformations in the neonatal peri- od in 14 hospitals consist of 13 provincial government hospitals as teaching hospitals and one private children and maternity hospital is presented. The incidence of anomalies in the range of 6-15 per thousand of total births. The incidence in boys was higher than girls. Based on the ICD-10, among the 2,621 anomalies, the most frequent of malformations and deformations were those of the musculoskeletal system (19.6%), followed by the nervous system (18.8%), the digestive system (18.7%), the circulatory system (12.9%), and the cleft lip and cleft palate (8.2%). Corrective and reconstructive surgery were performed by the Subspecialistic Division of the Surgical Department.
  • 1995 年 35 巻 4 号 p. 425-434
    発行日: 1995年
    公開日: 2021/07/29
    ジャーナル オープンアクセス
    ABSTRACT We made an epiidemiological study of congenital anomalies of the face, hand and foot in newborns from 1973 to 1992 in Miyagi Prefecture which has a population of about two million. In these twenty years 579,766 babies were born in Miyagi Prefecture. Out of these newborns 3,416 babies with 3,759 congenital anomalies of the face, hand and foot were registered. Of all registered congenital anomalies, face anomalies were most commonly encountered, followed by hand and foot anomalies. Among face anomalies, in order of frequency, accessory ear was the most common, next cleft lip with or without cleft palate, cleft palate alone, cryptotia and microtia. The occurrence ratio per 10,000 live births was 9.6 in accessory ear, 6.7 in cleft lip, 6.1 in cleft lip with cleft palate. 4.2 in cleft palate alone, 2.9 in cryptotia, and 1.8 in microtia. In hand and foot anomalies, polydactyly was the most common and syndactyly the next. Incidence ratio of polydactyly was 5.8 in the hand and 6.4 in the foot. Polydactyly was the most frequent in the preaxial ray in the hand and in the po:staxial ray in the foot. About half of cases of postaxial polydactyly the foot was associated with syndactyly between the fourth and fifth toe. In addition, we reported on variation of the incidence ratio and familial occurrence of con- genital anomalies of the face, hand and foot.
  • 1995 年 35 巻 4 号 p. 435-445
    発行日: 1995年
    公開日: 2021/07/29
    ジャーナル オープンアクセス
    ABSTRACT To evaluate tests for behavioral teratology in animals, brain dysfunction caused by maternal environmental agents is compared both epidemiologically and experimentally. Ethanol: The features of CNS involvement in human FAS (fetal alcohol syndrome) and FAE (fetal alcolhol effects) are developmental delay and intellectual impairment. One of the most vulnerable structures in the rat fetus exposed to ethanol in utero is the synaptic formation in the hippocampus. What test for brain dysfunctions, such as deficits in learning, inhibition and attention, is there? Tobacco: Thirty-three percent of human FTS (fetal tobacco syndrome) cases have CNS involvement, which is characterized by developmental delay, and behavioral problems such as a short attention span and hyperactivity. As to the concomitant effects of ethanol and tobacco on human offspring, CNS involvement appeared to be most specific for alcohol exposure, but was also observed slightly with smoking without drinking. How can the effects of concomitant substances be discriminated in an animal test? Low-copper level in brain: Related to the development of their offspring, abnormal movements in brindled mutant mouse heterozygotes are observed. These findings may be influenced by both copper and oxygen radical metabolism. The teratogenic effects of triethiylene tetramine dihydrochloride, a chelating drug for cop- per on the fetal mouse brain are noted both grossly and microscopically. How can the abnormal CNS development in relation to a certain biochemical mechanism be detected by means of an animal test? In conclusion, to examine behavioral teratology in animals, specificity and accuracy should be considered in comparison with in humans.
  • 1995 年 35 巻 4 号 p. 447-454
    発行日: 1995年
    公開日: 2021/07/29
    ジャーナル オープンアクセス
    ABSTRACT In order to examine the distribution of different types of congenital anomalies of the elbow joint and discuss their classification, a total of 94 patients, 96 congenital anomalies of the elbow joint, wtere analyzed. They were classified according to the elbow deformity itself as follows; radiioulnar synostosis in 59 cases (61 %), radial head dislocation in 23 cases (24%), ankylosis of the elbow joint in eight cases (8%), severe flexion contracture of the elbow joint in three cases (3%), and complete or partial aplasia of the elbow joint in three cases (3%). On the other hand, congenital elbow deformities can be classified into three types accortding to the associated anomalies. In 78 patients, in which congenital elbow deformity appeared as an independent deformity, there were radio-ulnar synostosis in 56 patients, radial1 head dislocation in 19 patients, ankylosis of the elbow joint in two patients, and hypoplasia of trocholea in one patient. In the 13 patients, which congenital elbow deformity appeared as a part of congenital upper limb anomalies, the underlying congenital anomalies of the upper limb were ulnar deficiency in eight patients, radial deficiency in two patients, transverse deficiency in two patients, and phocomelia in one patient. In five patients, elbow deformity appeared as a part of congenital syndromes. Classification according to associated anomalies seems to be more suitable in order to examine the real distribution of different types of congenital deformities of the elbow joint as a part of the upper limb.
  • 1995 年 35 巻 4 号 p. 455-466
    発行日: 1995年
    公開日: 2021/07/29
    ジャーナル オープンアクセス
    ABSTRACT Gestational day (GD) 13 rat embryos from dams treated p.0. with albenda- zole (ABZ) on GD 10-12 were examined as an experimental model for the pathogenesis of embryotoxicity induced by benzimidazole compounds. Dosage levels were 0, 10, 20 and 30 mgikg b.w. of ABZ. At 10 mgikg b.w., most embryos appeared macroscopically normal. However, the histological examination of a representative sample showed subtle alterations including discrete necrotic foci in the lateral and rostra1 telencephalon, delayed differentiation of lens, increased wavyness of the notochord caudad to the forelimb bud and absent or poorly defined apical ectodermal ridge in the forelimb bud. At 20 and 30 mgikg b.w. a sharp, dose-related increase in embryolethality, developmental delays and macroscopic abnormalities were accompanied histologically by marked necrotic alterations of a number of embryonic tissues. The telencephalon and neural tube were severely affected, showing also a regeneration attempt through the disordered formation of tubules. Besides neuroectoderm, necrosi!; and tissue depletion were mostly evident in neural crest and mesoderm-derived structures, such as branchial bars, limb buds and paraxial mesoderm. Lesions in the somites showed a head-to-tail increasing gradient; the sclerotomes were consistently more affected than the dermatomes. Wavyness of the notochord cephalad to the forelimb bud and fragmentediabnormal nuclei in the erythroblasts were also noted. Examination of embryos, during in vivo organogenesis may be a useful tool for investigating the pathogenesis alf embryotoxicity.
  • 1995 年 35 巻 4 号 p. 467-475
    発行日: 1995年
    公開日: 2021/07/29
    ジャーナル オープンアクセス
    ABSTRACT The behavioral abnormalities of homozygotes (busibus) of mutant bustling mice, BUS/ldr, were characterized in comparison with those of heterozygotes (+/bus) and of chemically labyrinthectomized mice. Homozygotes were 4-fold more active than heterozygotes, as evaluated in an open field (11 x 17 cm) for 24 hr by means of Animex. Homozygotes revealed the lack of righting reflex and of head nystagmus on a rotating board, the frequent backward-rnoving and the inability to swim, in addition to characteristic behaviors such as circling, liead bobbing and head tossing. Bilaterally labyrinthectomized mice behaviorally mimicked BUS homozygotes: The behavioral patterns of operated animals were essentially the same as those of BUS homozygotes with respect to all reflex and locomotor indices employed, suggesting that some peripheral vestibular dysfunction, though unidentified yet, is attributed to abnormal behaviors of BUS mice. Unilaterally labyrinthectomized mice were not hyperactive, nor exhibited circling behavior.
  • 1995 年 35 巻 4 号 p. 477-480
    発行日: 1995年
    公開日: 2021/07/29
    ジャーナル オープンアクセス
    ABSTRACT To confirm the alpplicability of MTT assay and sperm quality analyzer (SQA) to the examination of sperm viability and motility in rats, epididymal spermatozoa derived from male rats (S1c:SD) treated with nitrobenzene at a dose of 60 mg/kg/day for 16 days were examined by these methods. Epididymal fluid was suspended in Dulbecco’s modified Eagle’s medium containing 25 mM fructose in ratio of 1:125. Sperm concentrations in the epididymal fluid were 2.48 2 0.55 (X 106/pl, mean * SD, n = 8) in the control and 2.20 ? 0.54 in the nitrobenzene group, and did not significantly differ be- tween the groups. Both absorbance unit of the MTT assay (1.497 ? 0.406 vs. 1.084 ? 0.350) and SMI (sperm motility index) value of the SQA (204.0 ? 15.3 vs. 182.5 * 21.6) were significantly decreased in nitrobenzene group. The results suggest that the examinations of sperm viability and motility with MTT assay and SQA are available for the reproductive toxicology in rats.
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