著者
岩永 庄吾
出版者
日本先天異常学会
雑誌
日本先天異常学会会報 (ISSN:00372285)
巻号頁・発行日
vol.5, no.2, pp.133-148, 1965

The pathogenetic relationship between so-called congenital glomerulosclerosis of the kidney and developmental anomalies of the heart and great vessels was studied histopathologically. For this purpose 58 human fetuses and newborns associated with cardiovascular anomalies were used. Fursher, 54 human fetuses and newborns were employed as the controls. These fetuses and newborns aged 5 months of gestation to 20 days after birth. The cardiovascular anomalies were clssified into four groups: 1) Hypoplasia of the aorta isolated and combined with ventricular septal defect, in part, including Eisenmenger's complex, 2) the aorta and pulmonary trunk of equal size complicated with ventricular septal defect and, in part, including Eisenmenger's complex, 3) hypoplasia or aplasia of the pulmonary trunk including Fallot's tetralogy and 4) anomalies of the valves, transposition of the great vessels and others. Microscopic findings of the kidney, particularly in relation to glomerulosclerotic changes were examined among these groups of cardiovascular anomalies. Hematoxylin-eosin, Mallory, Weigert and van Gieson stains, and when necessary, PAS, sudan III stain and Lilie's impregnation were made. The results wereas follows: 1) In the controls no glomerulosclerosis of the kidney was found up to the 7th month of gestation, but there were I case of glomerulosclerosis ofthe kidney respectively in. the 9th month of gestation and after birth. The newborn infants aged less than 7 days showed interstitial congestion and slight degeneration of the uriniferous epithelium frequently as compared with the fetuses. 2) Generally the appearance of glomerulosclerosis of the kidney in the fetal, especially in the neoratal period was remarkable when cardiovascular anomalies were associated. 3) When cardiovascular anomalies were combined with other organ anomalies, the incidence of glomerulosclerosis of the kidney was slightly higher than that in cardiovascular anomalies alone. 4) According to the groups of cardiovascular anomalies such as classified above, the occurrence of glomerulosclerosis of the kidney was found to be as follows: 1) most frequent in hypoplasia of the aorta combined with or without ventricular septal defect (69.6%), 2) less frequent in the aorta and pulmonary trunk of equal size and other anomalies (61.1-62.5%), but 3) hardly detected in hypoplasia or aplasia of the pulmonary trunk except for complicated with renal hypoplasia which caused glomerulosclerosis of the kidney in 55.5%. The formation of the intrarenal arteries is closely connected with the growth of the kidney and related hemodynamic shift of general blood circulation, and in cosequence, depends upon the correlation between a maturation of the glomeruli and an increase of circulating blood volume into the kidney. It is noted that hemodynamic circulation of fetus and newborn is not only different from that of adult but also varies according to the kinds and grades of developmental anomalies of the heart and great vessels. Based upon these above-mentioned results, it is pointed out that a hemodynamic vicissitude of blood circulation due to developmental anomalies of the heart and great vessels throuhgout fetal to neonatal period possibly play a major role in pathogenesis of congenital glomerulosclerosis of the kidney in human fetus and newborn.
著者
杉浦 保夫 鶴田 登代志
出版者
日本先天異常学会
雑誌
日本先天異常学会会報 (ISSN:00372285)
巻号頁・発行日
vol.21, no.4, pp.533-542, 1981-12-30 (Released:2019-02-01)

The term "bone dysplasias", or "constitutional diseases of bone" is used as a general term for congenital and generalized morphological and/or structural bone diseases that are caused by some failures of morphogenesis, growth or maturation of bone and/or cartilage. Most of bone dysplasias are genetic diseases. Pathogenesis of bone dysplasias is considered to be some congenital metabolic failures of bone and cartilage, but is still unknown except in few dysplasias. In 1964, Rubin advocated a dynamic classification of bone dysplasias. He considered that bone dysplasias were caused by hypoplasia or hyperplasia of anatomico-physiological function of tubular bones which he divided in 4- sections: epiphysis, physis, metaphysis and diaphysis. His classification was really unique and was easily accepted by many researchers. Since that time, however, many new dysplasias have been clarified as definite entities. In order to classify these new diseases, a draft of international nomenclature of bone dysplasias was published in 1970. Final draft of international nomenclature was published in 1978. Main purpose of this nomenclature is to standardize the criteria for diagnosis and to report under the same name all over the world. Japanese researchers must also be well acquainted with this nomenclature and have to report cases of bone dysplasias to international medical journals in English so as to join international research net-work.
著者
近藤 良
出版者
日本先天異常学会
雑誌
日本先天異常学会会報 (ISSN:00372285)
巻号頁・発行日
vol.20, no.1, pp.7-16, 1980-03-30 (Released:2019-02-01)

"Hinoe-Uma" is the name of a year recurring in a sixty-year cycle in the ancient Japanese calender, and is characterized by a superstition that it is an evil omen for girls to be born in this year. In 1966, the year of "Hinoe Uma" live births m Japan decreased to 1,360,974, in contrast with 1,823,697 and 1,935,647 in the preceding and the following years, respectively. In 1966, the infant mortality rate from congenital anomalies rose to 22.6 per 10,000 live births from 19.8 in the preceding year and it returned to 19.2 in the following year. Whether there was an actual rise of risk or not should be a problem, because when the number of live births changes rapidly from one year to the next, infant mortality rate computed by a conventional method does not provide an accurate measure of risk of death during the first year of life. The author tried to obtain accurate measures of the risk using data on the number of infant deaths by age subdivisions from Vital Statistics in Japan 1965 to 1967. Adjusted infant mortality rates from congenital anomalies were 20.0, 21.2 and 20.5 per 10,000 live births in 1965, 1966 and 1967, respectively. It can therefore be concluded that there was no significant rise in the actual risk of infant deaths from congenital anomalies as a whole and from each kind of anomalies in 1966.
著者
小林 登
出版者
日本先天異常学会
雑誌
日本先天異常学会会報 (ISSN:00372285)
巻号頁・発行日
vol.15, no.3, pp.163-168, 1975

Clinical significance of congenital anomaly syndromes is reviewed in relation to teratogen, mutagen and carcinogen. Congenital anomaly syndromes, especially malformations, are important for attracting the eyes of clinicians, particulary pediatricians, so that an effective epidemiological analysis may be conducted to detect teratogens, mutagens or carcinogens. This approach of study is attempting to clinically monitor teratogens, mutagens and carcinogens. Among the various types of congenital anomaly syndrome, the cancer-malformation syndrome (malignancy-congenital anomaly syndrome) has unique significance in clinics. The congenital anomaly syndromes of chromosomal anomalies, immunodeficiencies, inborn errors of metabolism and malformation are high-risk groups for malignancy, including leukemia and malignant lymphoma. The registry of childhood malignancy in Japan organized by the Japan Children's Cancer Association, revealed a statistically significant difference in the pattern of groups of patients with major malformation, minor malformation, both major and minor malformation and with no malformation between leukemia and Wilms' tumor, leukemia and malignant teratoma, and lymphoma and Wilms' tumor.
著者
古谷 博
出版者
日本先天異常学会
雑誌
先天異常 (ISSN:09143505)
巻号頁・発行日
vol.30, no.1, pp.p1-3, 1990-03