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1. |
In Memorium Ujihiro Murakami (1910‐1992) |
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Congenital Anomalies,
Volume 33,
Issue 1,
1993,
Page 2-3
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ISSN:0914-3505
DOI:10.1111/j.1741-4520.1993.tb00506.x
出版商:Blackwell Publishing Ltd
年代:1993
数据来源: WILEY
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2. |
Renal Cystic Disease : Classification and Pathogenesis* |
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Congenital Anomalies,
Volume 33,
Issue 1,
1993,
Page 5-13
Jay BERNSTEIN,
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ISSN:0914-3505
DOI:10.1111/j.1741-4520.1993.tb00507.x
出版商:Blackwell Publishing Ltd
年代:1993
数据来源: WILEY
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3. |
New Approach in Behavioral Teratology—Experimental Study on FAS* |
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Congenital Anomalies,
Volume 33,
Issue 1,
1993,
Page 15-30
Miyako OMOTO,
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摘要:
ABSTRACTFetal alcohol syndrome is known as an effect given by mothers. In connection with this, we so far observed rats from physical, biochemical, histological, and functional standpoints. Recently we observed synaptogenesis in brain together with behavior and learning ability of rats. Ethanol was given to mother rats during pregnancy and nursing periods, and learning ability of their offspring at 12 weeks of age was studied by the radial arm maze method. Also synaptogenesis in the hippocampus CA1 was observed at 2, 7, 14, 21, and 70 days of age by quantitative electron microscopy. The offspring were significantly deficient in learning ability tested with the maze compared with offspring of mother rats not exposed to ethanol. Densities of all synapses in the strata radiatum and lacunosum‐moleculare of CA1 in the hippocampus became significantly lower at every time of the observation. Both axo‐spinous and axo‐shaftic synapses significantly decreased. Thus we found that observations of learning ability agreed with observations of synaptogenesis in the hippocampus CA1 which has important association with memory. This fact suggests that both observations will be of great value in the research in behavioral terat
ISSN:0914-3505
DOI:10.1111/j.1741-4520.1993.tb00508.x
出版商:Blackwell Publishing Ltd
年代:1993
数据来源: WILEY
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4. |
Atrioventricular Septal Defect in Rat Fetuses Induced by Administration of Bisdiamine |
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Congenital Anomalies,
Volume 33,
Issue 1,
1993,
Page 31-44
Hiroyasu TASAKA,
Naomasa OKAMOTO,
Hitoshi TAKENAKA,
Yasunori KOGA,
Minoru HAMADA,
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摘要:
ABSTRACTA high incidence of atrioventricular septal defect was induced in rat fetuses by administration ofN, N‐his (dichloroacetyl)‐1, 8‐octamethylenediamine, bisdiamine, to pregnant Donryu rats. Induction of atrioventricular septal defect was highly dependent on day of bisdiamine administration. When bisdiamine was administered singly on day 9.4 of gestation or consecutively on days 9 and 10 of gestation, the incidence of atrioventricular septal defect in fetuses increased. Survival rate decreased with increase in the amount of bisdiamine administered. Normalized incidence5for atrioventricular septal defect, which was calculated as a product of incidence and survival rate, was as high as 54.5% when 175 mg bisdiamine was administered on day 9.4 of gestation. Atrioventricular septal defect thus formed was of the complete type associated with either persistent truncus arteriosus or tetralogy of Fallot, indicating that the conotruncal portion was unexceptionally malformed. The present model is useful for analysis of development and pathogenesis of atrioventricular septal defect and also contributes to clarifying the normal development of atrioventricular valves and
ISSN:0914-3505
DOI:10.1111/j.1741-4520.1993.tb00509.x
出版商:Blackwell Publishing Ltd
年代:1993
数据来源: WILEY
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5. |
Campomelic Syndrome Associated with Potter's Syndrome and Car diosplenic Syndrome |
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Congenital Anomalies,
Volume 33,
Issue 1,
1993,
Page 45-53
Na Hye Myong,
Je G. Chi,
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摘要:
ABSTRACTCampomelic syndrome is a heterogeneous group of disorders characterized by short‐limbed dwarfism with congenital bowing or bending of the long bones particularly in the lower limbs and is frequently associated with a variety of non‐skeletal manifestations. Two distinct types are (1) long‐limbed campomelic dysplasia with bent bones of normal length and width: (2) short‐limbed campomelic dysplasia in which the bent bones are short and wide. We reported an autopsy case of this syndrome.This male baby with gestational age of 32 weeks was born to a mother with placenta previa, showing abdominal distention and short‐limbed appearance. Postmortem examination revealed bilaterally enlarged Potter type 1 polycystic kidneys, a pancreatic cyst, and two spleens in the abdominal cavity. Both lungs were hypoplastic and the heart exhibited multiple congenital anomalies such as double outlet right ventricle, pulmonary artery hy‐poplasia, mitral atresia, absence of ductus arteriosus, and inferior vena cava draining into the left atrium. Characteristically both femur bones were curved at proximal two‐thirds with slightly shortened lengths. Both humeri also showed bending of distal one‐third. Other chondro‐osseous abnormalities such as small epiglottis, narrow thorax, dolichoce‐phaly, and bilateral equinovarus deformity were noted. These findings were compatible with campomelic dysplasia of long‐limbed type accompanying unusual sets of non‐skeletal manifestations of Potter's syndrome and cardiosplenic syndrome. Key words: campomelic syndrome, Potter's syndrome
ISSN:0914-3505
DOI:10.1111/j.1741-4520.1993.tb00510.x
出版商:Blackwell Publishing Ltd
年代:1993
数据来源: WILEY
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6. |
Histological Analysis of Triphalangism Associated with Polydactyly of the Thumb |
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Congenital Anomalies,
Volume 33,
Issue 1,
1993,
Page 55-62
Hitomi MAKINO,
Takayuki MIURA,
Ryogo NAKAMURA,
Hiroshi NOGAMI,
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摘要:
ABSTRACTWassel's classification of thumb polydactyly has some problems especially in categories IV and VII because it depends solely on X‐ray findings. In order to improve his thumb polydactyly classification, we examined histologically untreated 182 bifid thumbs from 171 patients at Branch Hospital, Nagoya University in the past eight years. Ninety‐five of these cases had proximal phalangeal separate type of polydactyly corresponding to Wassel's categories IV and VII. Forty‐five thumbs of these categories were excised from 44 patients and classified into type I (24 cases), type II (12). type III (4), and type IV (5) in accordance with Kanno's classification. In distal phalanges, normal cartilage development was observed in Kanno's type I, whereas proximally elongated cartilaginous tissue was formed in the type II. Three distal phalanges from types II and III showed depression at the ulnar side of elongated cartilage of the distal phalanx where immature chondrocytes transversely distributed. Three cases with incomplete and one case with complete joint formation were formed in elongated cartilage of the distal phalanx, and endochondral ossification developed in the central area of elongated cartilage of the distal phalanx in four cases from types III and IV. All of the cases showed only minimal mobility of their involved joints. Their tendon development did not correspond to their anatomical characteristics. Histological studies indicated that there was no distinct difference between Wassel's category IV and category VII. It was postulated that the triph
ISSN:0914-3505
DOI:10.1111/j.1741-4520.1993.tb00511.x
出版商:Blackwell Publishing Ltd
年代:1993
数据来源: WILEY
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7. |
Overgrowth and Enlarged Heart in Mouse Fetuses with Maternally Inherited ThPand twLub2: An Example of Genomic Imprinting in Animals |
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Congenital Anomalies,
Volume 33,
Issue 1,
1993,
Page 63-75
Shinichi MIYABARA,
Mark H. BOGART,
Heinz WINKING,
Hajime SUGIHARA,
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摘要:
ABSTRACTThp/+mouse is known to have differing phenotypes depending on gender of the ThPparent. In the present study, fetuses with maternally [Group A (until day 18 of pregnancy) and Group C (day 19)] and paternally [Group B]inherited ThPwere examined with particular reference to the developmental abnormalities of hearts. In addition, a small number of fetuses with maternally inherited twLub2on day 19 were compared with Thp. Group A ThPfetuses had greater body weight, possibly larger body size, generalized edema (100%), marked enlargement of the hearts (100%) and hypoplastic pulmonary trunk (73.7%). There were no such malformations in Group B ThPfetuses or in the controls (+/+) for both groups. The bilaterally thickened ventricular wall of fetal hearts in Group A and C ThPfetuses bulged into ventricular cavity. The pulmonary valve was also thickened. The labeling indices of the ventricular myocardial cells by BrdU were inclined to be higher in Group A ThPthan in the control (+/+) fetuses. Fetuses of twLub2had abnormalities of the cardiovascular system similar to Group C Thp. The results suggest that cardiac lesions in fetuses with maternally inherited ThPand twLub2correspond to cardiomyopathy. Overgrowth and enlarged heart indicate the possibility of ThPas an animal model for Wiedemann‐Beckwith syndrom
ISSN:0914-3505
DOI:10.1111/j.1741-4520.1993.tb00512.x
出版商:Blackwell Publishing Ltd
年代:1993
数据来源: WILEY
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8. |
Effects of N‐acetyl‐L‐cysteine on Teratogenicity of 5‐Fluorouracil in Mice |
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Congenital Anomalies,
Volume 33,
Issue 1,
1993,
Page 77-84
Masato NAYA,
Takashi DEGUCHI,
Mineo YASUDA,
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摘要:
ABSTRACTEmbryotoxicity and teratogenicity of 5‐fluorouracil (5‐FU) and modulation of its effect by N‐acetyl‐L‐cysteine (NAC) andor phorone were evaluated in mice. Pregnant ICR mice were intraperitoneally injected with 25 mglkg of 5‐FU on day 11 of gestation (vaginal plug = day 0). Pregnant mice were pretreated with NAC at dose levels of 80, 160, and 320 mg/kg injected intravenously 2 hours before dosing with 5‐FU. Pregnant mice were killed on day 17 of gestation. Fetuses were examined for external malformations, especially limb malformations. Pretreatment with 160 mg/kg and 320 mg/ kg of NAC decreased the incidence and severity of oligodactyly induced by 5‐FU. There was little difference in maternal body weight gain, fetal mortality, and fetal weight between the 5‐FU group and the 5‐FU plus NAC groups. Pretreatment with phorone, a glutathione depleting agent, at dose levels of 160 and 320 mg/kg injected intraperitoneally, 4 hours before dosing with 5‐FU, increased the incidence and severity of oligodactyly induced by 5‐FU. Cotreatment with NAC at dose levels of 160 and 320 mg/kg decreased the incidence and severity of oligodactyly induced by 5‐FU and 80 mg/kg of phorone. Cotreatment with NAC 160 mg/kg could not suppress the augmentative effect of phorone on 5‐FU teratogenicity under the severe condition, that is, the excess amount of phorone such as 320 mg/kg. These results indicate that the teratogenicity of 5‐FU is mitigated with NAC pretreatment, and also the level of endogenous glutathione is one of the factors which significantly a
ISSN:0914-3505
DOI:10.1111/j.1741-4520.1993.tb00513.x
出版商:Blackwell Publishing Ltd
年代:1993
数据来源: WILEY
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9. |
Clinical Features of Congenital Contractural Arachnodactyly |
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Congenital Anomalies,
Volume 33,
Issue 1,
1993,
Page 85-94
Toshihiko OGINO,
Hiroyuki KATO,
Itaru OHSHIO,
Masatoshi TAKAHARA,
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摘要:
ABSTRACTClinical features of 11 cases of congenital contractural arachnodactyly (CCA) were reported. Eight cases were male and 3 cases were female. Family history was positive in 6 cases of 3 families. As for hand deformity, flexion of the finger, adduction of the thumb and shortening of the palmar skin were observed in all cases, arachnodactyly in ten cases, flexion contracture of the finger joint in 6 cases. Dolichostenomelia and crumpled ear deformity were associated in all cases, spinal deformity in 6 cases, flexion contracture of the lower extremities in 5 cases, micrognathia in 2 cases and high palate, strabismus, pectus excavatum and polydactyly of the fifth toe in one case. Arachnodactyly was not an essential feature of CCA. Existence of multiple camptodactyly with or without arachnodactyly and/or ulnar drift of the fingers, and crumpled ear deformity were important findings for differential diagnosis of CCA. There was no association of cardiovascular anomalies in this series. CCA without ocular and cardiovascular involvement must be a definite clinical entity and should be distinguished from the other forms of marfanoid habitus.
ISSN:0914-3505
DOI:10.1111/j.1741-4520.1993.tb00514.x
出版商:Blackwell Publishing Ltd
年代:1993
数据来源: WILEY
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10. |
Announcement |
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Congenital Anomalies,
Volume 33,
Issue 1,
1993,
Page 95-96
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ISSN:0914-3505
DOI:10.1111/j.1741-4520.1993.tb00515.x
出版商:Blackwell Publishing Ltd
年代:1993
数据来源: WILEY
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